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Items 1 to 100 of about 249
1. Colombo P, Rahal D, Grizzi F, Quagliuolo V, Roncalli M: Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: a case report. World J Gastroenterol; 2005 Sep 7;11(33):5226-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: a case report.
  • Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract.
  • Although rare, it is the most common primary tumor of the mesentery and can develop at any age.
  • We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST).
  • Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Gastrointestinal Stromal Tumors / diagnosis. Intestinal Neoplasms / diagnosis. Intestine, Small
  • [MeSH-minor] Diagnosis, Differential. Humans. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 16127758.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC4320401
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2. Dômont J, Salas S, Lacroix L, Brouste V, Saulnier P, Terrier P, Ranchère D, Neuville A, Leroux A, Guillou L, Sciot R, Collin F, Dufresne A, Blay JY, Le Cesne A, Coindre JM, Bonvalot S, Bénard J: High frequency of beta-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management. Br J Cancer; 2010 Mar 16;102(6):1032-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High frequency of beta-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management.
  • BACKGROUND: Fibromatosis comprises distinct clinical entities, including sporadic extra-abdominal fibromatosis, which have a high tendency for recurrence, even after adequate resection.
  • We searched for beta-catenin mutations in a European multicentre series of fibromatosis tumours to relate beta-catenin mutational status to disease outcome.
  • METHODS: Direct sequencing of exon 3 beta-catenin gene was performed for 155 frozen fibromatosis tissues from all topographies.
  • Correlation of outcome with mutation rate and type was performed on the extra-abdominal fibromatosis group (101 patients).
  • Among 101 extra-abdominal fibromatosis, similar mutation rates (87%) were observed, namely T41A (39.5%), S45P (9%), S45F (36.5%), and deletion (2%).
  • CONCLUSION: A high frequency (87%) of beta-catenin mutation hallmarks extra-abdominal fibromatosis from a large multicentric retrospective study.
  • Moreover, wild-type beta-catenin seems to be an interesting prognostic marker that might be useful in the therapeutic management of extra-abdominal fibromatosis.
  • [MeSH-major] Fibroma / diagnosis. Fibroma / genetics. Mutation, Missense. beta Catenin / genetics
  • [MeSH-minor] Base Sequence. Biomarkers, Tumor / analysis. Biomarkers, Tumor / genetics. DNA Mutational Analysis. Female. Gene Frequency. Heterozygote. Humans. Male. Molecular Diagnostic Techniques. Outcome Assessment (Health Care). Prognosis. Retrospective Studies

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  • (PMID = 20197769.001).
  • [ISSN] 1532-1827
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / beta Catenin
  • [Other-IDs] NLM/ PMC2844024
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3. Stoidis CN, Spyropoulos BG, Misiakos EP, Fountzilas CK, Paraskeva PP, Fotiadis CI: Surgical treatment of giant mesenteric fibromatosis presenting as a gastrointestinal stromal tumor: a case report. J Med Case Rep; 2010;4:314

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical treatment of giant mesenteric fibromatosis presenting as a gastrointestinal stromal tumor: a case report.
  • INTRODUCTION: Intra-abdominal fibromatosis, usually located at the mesenteric level, is a locally invasive tumor of fibrous origin, with no ability to metastasize, but a tendency to recur.
  • Certain non-typical cases of intra-abdominal fibromatosis with involvement of the bowel wall can be misdiagnosed because of their different biological behavior.
  • CASE PRESENTATION: We describe the case of a 64-year-old Caucasian man presenting with mesenteric fibromatosis and involvement of the bowel wall, who was treated surgically.
  • The macroscopic and microscopic appearance of the lesion mimicked a gastrointestinal stromal tumor, a tumor with potential malignant behavior.
  • CONCLUSION: It is essential to make an early and correct diagnosis in such equivocal cases, so that the appropriate treatment can be chosen and suitable patients admitted to clinical trials if appropriate.
  • New and reliable criteria for discriminating between intra-abdominal fibromatosis and gastrointestinal stromal tumor should be proposed and established because novel sophisticated therapeutic strategies have been introduced in the international literature.

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  • (PMID = 20863383.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2955058
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4. Fülöp E, Marcu S, Milutin D, Borda A: Gastrointestinal stromal tumors: review on morphology, diagnosis and management. Rom J Morphol Embryol; 2009;50(3):319-26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gastrointestinal stromal tumors: review on morphology, diagnosis and management.
  • The diagnosis of GISTs relies on histological arguments--proliferation of spindle-shaped cells in 70% of cases, of epithelioid cells in 20%, histological variants are rare--, and on immunohistochemical arguments--expression of CD117 in 95%, usually associated with CD34 expression in 70% of cases.
  • The differential diagnosis of GISTs includes the other mesenchymal tumors of the gastrointestinal tract, such as leiomyomas, leiomyosarcomas, schwannomas and intra-abdominal fibromatosis.
  • The evaluation of the prognosis is essential and is based on a simple algorithm using two histoprognostic parameters, tumor size and mitotic index.
  • [MeSH-major] Gastrointestinal Stromal Tumors / diagnosis. Gastrointestinal Stromal Tumors / therapy
  • [MeSH-minor] Diagnosis, Differential. Humans

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  • (PMID = 19690756.001).
  • [ISSN] 1220-0522
  • [Journal-full-title] Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
  • [ISO-abbreviation] Rom J Morphol Embryol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Romania
  • [Number-of-references] 31
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5. Koeda S, Nagasaka H, Kumamoto H, Kawamura H: Extra-abdominal fibromatosis of the cheek: report of a case. J Oral Maxillofac Surg; 2005 Aug;63(8):1222-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extra-abdominal fibromatosis of the cheek: report of a case.
  • [MeSH-major] Cheek. Fibromatosis, Aggressive / pathology. Mouth Neoplasms / pathology

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  • (PMID = 16094595.001).
  • [ISSN] 0278-2391
  • [Journal-full-title] Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons
  • [ISO-abbreviation] J. Oral Maxillofac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 20
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6. Choi JY, Kang KM, Kim BS, Kim TH: Mesenteric fibromatosis causing ureteral stenosis. Korean J Urol; 2010 Jul;51(7):501-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis causing ureteral stenosis.
  • Mesenteric fibromatosis is a rare benign disease characterized by proliferating fibrous tissue in the bowel mesentery.
  • We report a case of aggressive mesenteric fibromatosis with ureteral stenosis arising in a 46-year-old woman who suffered from intermittent right abdominal pain.
  • Computed tomography revealed a right retroperitoneal mass with right ureteral stenosis at the level of the right common iliac vessel.
  • Pathological examination confirmed mesenteric fibromatosis.

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  • (PMID = 20664786.001).
  • [ISSN] 2005-6745
  • [Journal-full-title] Korean journal of urology
  • [ISO-abbreviation] Korean J Urol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2907502
  • [Keywords] NOTNLM ; Aggressive fibromatosis / Retroperitoneal neoplasms
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7. Whittle P C, Sánchez R L, Albarracin G J, Hepp K J: [Retroperitoneal fibromatosis: report of a case]. Rev Med Chil; 2006 Jan;134(1):85-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Retroperitoneal fibromatosis: report of a case].
  • [Transliterated title] Fibromatosis mesentérica: caso clínico-imagenológico y revisión bibliográfica.
  • We report a 42 years old female that presented with abdominal pain and no palpable mass.
  • Imaging abdominal ultrasound, CAT scan and magnetic resonance showed a solid tumor located in the retroperitoneum.
  • The patient was operated on excising the tumor.
  • Pathological examination of the surgical piece disclosed a retroperitoneal fibromatosis or desmoid tumor.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Retroperitoneal Neoplasms / diagnosis

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  • (PMID = 16532167.001).
  • [ISSN] 0034-9887
  • [Journal-full-title] Revista médica de Chile
  • [ISO-abbreviation] Rev Med Chil
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Chile
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8. Tamura K, Tani M, Kinoshita H, Yamaue H: Mesenteric desmoid tumor of the interposed jejunal pouch after total gastrectomy. World J Surg Oncol; 2006;4:27

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric desmoid tumor of the interposed jejunal pouch after total gastrectomy.
  • BACKGROUND: Desmoid tumor is a rare entity, and most desmoid tumors are located in abdominal wall or extra-abdominal tissues.
  • Occurrence of desmoid tumor in mesentry is extremely rare.
  • CASE PRESENTATION: we report a mesenteric desmoid tumor in a 73-years-old woman who had undergone total gastrectomy reconstructed with jejunal pouch interposition for gastric carcinoma.
  • After 1 year, a tumor was originating from mesentery of the interposed jejunal pouch was identified, and the patient underwent resection of the large mass which was found to invade pancreas.
  • Histological examination revealed desmoid tumor.
  • CONCLUSION: Desmoid tumor is rare, and it was difficult for the differential diagnosis of desmoid tumor or recurrent tumor.

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  • (PMID = 16740152.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1481628
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9. Gonçalves A, Monges G, Yang Y, Palmerini F, Dubreuil P, Noguchi T, Jacquemier J, Di Stefano D, Delpero JR, Sobol H, Bertucci F: Response of a KIT-positive extra-abdominal fibromatosis to imatinib mesylate and KIT genetic analysis. J Natl Cancer Inst; 2006 Apr 19;98(8):562-3
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  • [Title] Response of a KIT-positive extra-abdominal fibromatosis to imatinib mesylate and KIT genetic analysis.
  • [MeSH-minor] Adult. Benzamides. Biomarkers, Tumor / analysis. Biomarkers, Tumor / genetics. Female. Fibroma / chemistry. Humans. Imatinib Mesylate. Protein-Tyrosine Kinases / antagonists & inhibitors. Receptor, Platelet-Derived Growth Factor alpha / analysis. Receptor, Platelet-Derived Growth Factor alpha / genetics

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  • [CommentIn] J Natl Cancer Inst. 2006 Nov 1;98(21):1583-4 [17077361.001]
  • (PMID = 16622127.001).
  • [ISSN] 1460-2105
  • [Journal-full-title] Journal of the National Cancer Institute
  • [ISO-abbreviation] J. Natl. Cancer Inst.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Biomarkers, Tumor; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha
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10. Tamborini E, Negri T, Miselli F, Lagonigro MS, Pricl S, Pilotti S: Re: Response of a KIT-positive extra-abdominal fibromatosis to imatinib mesylate and KIT genetic analysis. J Natl Cancer Inst; 2006 Nov 1;98(21):1583-4
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  • [Title] Re: Response of a KIT-positive extra-abdominal fibromatosis to imatinib mesylate and KIT genetic analysis.
  • [MeSH-minor] Alanine. Benzamides. Biomarkers, Tumor / genetics. Cysteine. Humans. Imatinib Mesylate. Leucine. Methionine. Mutagenesis. Polymorphism, Genetic. Protein-Tyrosine Kinases / antagonists & inhibitors. Treatment Outcome

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  • [CommentOn] J Natl Cancer Inst. 2006 Apr 19;98(8):562-3 [16622127.001]
  • (PMID = 17077361.001).
  • [ISSN] 1460-2105
  • [Journal-full-title] Journal of the National Cancer Institute
  • [ISO-abbreviation] J. Natl. Cancer Inst.
  • [Language] eng
  • [Publication-type] Comment; Letter; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Biomarkers, Tumor; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; AE28F7PNPL / Methionine; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; GMW67QNF9C / Leucine; K848JZ4886 / Cysteine; OF5P57N2ZX / Alanine
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11. Röpke M, Kalinski T, Wördehoff H, Aumann V, Bürger T: [Multicentric extra-abdominal fibromatosis: a rare case]. Z Orthop Ihre Grenzgeb; 2006 Mar-Apr;144(2):223-7
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  • [Title] [Multicentric extra-abdominal fibromatosis: a rare case].
  • [Transliterated title] Multizentrische extraabdominale Fibromatose -- ein seltener Fall.
  • Extra-abdominal aggressive fibromatosis is a benign fibroblastic neoplasia with an infiltrative nature and a high tendency of local recurrence.
  • Here, we report on a very rare case of multicentric fibromatosis.
  • [MeSH-major] Fibroma / diagnosis. Fibroma / surgery. Leg / surgery. Limb Salvage. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Abdomen. Adult. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Humans. Radiotherapy, Adjuvant. Rare Diseases / diagnosis. Rare Diseases / surgery. Treatment Outcome

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  • (PMID = 16625455.001).
  • [ISSN] 0044-3220
  • [Journal-full-title] Zeitschrift für Orthopädie und ihre Grenzgebiete
  • [ISO-abbreviation] Z Orthop Ihre Grenzgeb
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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12. Karagulle E, Gokturk HS, Turk E, Yildirim E, Kiyici H, Karakayali H: Intestinal perforation from primary intra-abdominal fibromatosis. Saudi Med J; 2007 Apr;28(4):639-40
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  • [Title] Intestinal perforation from primary intra-abdominal fibromatosis.
  • [MeSH-major] Fibromatosis, Abdominal / complications. Intestinal Perforation / etiology

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  • (PMID = 17457496.001).
  • [ISSN] 0379-5284
  • [Journal-full-title] Saudi medical journal
  • [ISO-abbreviation] Saudi Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Saudi Arabia
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13. Tryphonopoulos P, Weppler D, Levi DM, Nishida S, Madariaga JR, Kato T, Mittal N, Moon J, Selvaggi G, Esquenazi V, Cantwell P, Ruiz P, Miller J, Tzakis AG: Transplantation for the treatment of intra-abdominal fibromatosis. Transplant Proc; 2005 Mar;37(2):1379-80
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  • [Title] Transplantation for the treatment of intra-abdominal fibromatosis.
  • MATERIALS AND METHODS: During the last 9 years we treated 14 patients with a diagnosis of intra-abdominal fibromatosis.
  • Three patients received an intestinal autograft after partial abdominal evisceration and ex vivo tumor resection.
  • Three patients additionally underwent an abdominal wall allograft.
  • Two patients developed desmoid tumor recurrence in their abdominal or thoracic wall.
  • CONCLUSION: Intestinal allo-, or autotransplantation combined with transplantation of the abdominal wall can be lifesaving for patients suffering from extensive intra-abdominal fibromatosis.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Intestines / transplantation. Viscera / transplantation

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  • (PMID = 15848726.001).
  • [ISSN] 0041-1345
  • [Journal-full-title] Transplantation proceedings
  • [ISO-abbreviation] Transplant. Proc.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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14. Kato Y, Tsuyuki A, Kikuchi K, Kurihara N, Fujishiro Y: Mesenteric fibromatosis successfully resected with duodeno-jejunectomy and nephrectomy. Hepatogastroenterology; 2005 Nov-Dec;52(66):1730-3
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  • [Title] Mesenteric fibromatosis successfully resected with duodeno-jejunectomy and nephrectomy.
  • We report a rare case of intra-abdominal fibromatosis of the jejunal mesentery.
  • A 71-year-old man, who was incidentally diagnosed with left hydronephrosis, was found to have a tumor near the duodeno-jejunal junction.
  • The tumor appeared to invade the intestinal wall and was obstructing the left ureter.
  • The tumor was indefinite in diagnosis preoperatively.
  • The patient underwent an en-bloc excision of the tumor by a partial duodeno-jejunectomy combined with a left nephrectomy.
  • Microscopic examination of the tumor showed that non-dysplastic fibroblasts proliferating in the jejunal mesentery had infiltrated into the adjacent small intestines and ureter, resulting in a diagnosis of intra-abdominal fibromatosis.
  • This diagnosis was supported by findings of immunohistochemical analyses showing positive staining for vimentin and smooth muscle actin and negativity for keratin, CD34, C-kit and S-100.
  • To our knowledge, this is the first documented case of intra-abdominal fibromatosis of the jejunal mesentery completely resected with the operative procedure described herein.
  • In cases of a preoperatively undiagnosed retroperitoneal or mesenteric tumor that invades adjacent structures, it is important to consider intra-abdominal fibromatosis as a possible differential diagnosis.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Kidney Neoplasms / secondary. Kidney Neoplasms / surgery. Neoplasm Invasiveness / pathology. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Aged. Anastomosis, Surgical. Biopsy, Needle. Duodenum / surgery. Follow-Up Studies. Humans. Hydronephrosis / diagnosis. Hydronephrosis / surgery. Immunohistochemistry. Jejunum / surgery. Magnetic Resonance Imaging / methods. Male. Mesentery / pathology. Neoplasm Staging. Nephrectomy / methods. Risk Assessment. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 16334767.001).
  • [ISSN] 0172-6390
  • [Journal-full-title] Hepato-gastroenterology
  • [ISO-abbreviation] Hepatogastroenterology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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15. Li A, Lui CY, Ying M, Mak KL, Lam HS: Case of fibromatosis of male breast. Australas Radiol; 2007 Oct;51 Spec No.:B34-6
MedlinePlus Health Information. consumer health - Mammography.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Case of fibromatosis of male breast.
  • Extra-abdominal fibromatosis of the breast is rare with the clinical and radiological features closely resembled malignant breast tumour.
  • We describe, in a 40-year-old Chinese male patient with a palpable left breast mass, the clinical, radiological and pathological features of this benign though locally aggressive tumour.
  • [MeSH-major] Breast Neoplasms, Male / diagnosis. Fibroma / diagnosis. Mammography

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  • (PMID = 17875152.001).
  • [ISSN] 0004-8461
  • [Journal-full-title] Australasian radiology
  • [ISO-abbreviation] Australas Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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16. Dômont J, Salas S, Lacroix L, Brouste V, Dufresne A, Terrier P, Blay J, Le Cesne A, Coindre J, Benard J, Bonvalot S: Frequency of β-catenin heterozygous mutations in extra-abdominal fibromatosis as a surrogate marker for disease management. J Clin Oncol; 2009 May 20;27(15_suppl):10501

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Frequency of β-catenin heterozygous mutations in extra-abdominal fibromatosis as a surrogate marker for disease management.
  • : 10501 Background: Fibromatosis, a rare soft-tissue and locally invasive neoplasm, consist of distinct clinical entities including sporadic extra-abdominal fibromatosis (EAF).
  • As dysregulation of β-catenin (βC) expression hallmarks fibromatosis, herein we searched for βC mutations in a large retrospective European multi-centric tumors series to relate with clinicopathologic parameters.
  • METHODS: From 1987 to 2007, 155 pts with fibromatosis from all sites were gathered by the Conticanet Network were analysed.
  • From this cohort, 101 pts with EAF surgically treated with curative intent and follow-up allowed us to relate βC mutational status to disease outcome.

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  • (PMID = 27963688.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Dumont AG, Lev D, Lazar A, Joensuu H, Trent J: Simultaneous gastrointestinal stromal tumor (GIST) and desmoid fibromatosis (DF). J Clin Oncol; 2009 May 20;27(15_suppl):10568

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Simultaneous gastrointestinal stromal tumor (GIST) and desmoid fibromatosis (DF).
  • : 10568 Background: Desmoid Fibromatosis (DF) is one of a group of rare fibrous tissue proliferations (2-4 cases per year per million) which tend to be locally aggressive but have no propensity for metastasis.
  • Gastrointestinal Stromal Tumor (GIST) is the most common mesenchymal tumors of the gastrointestinal tract and also rare (15-20 cases per year per million).
  • The primary site of the GIST was gastric in 4 cases and the others were jejunal and mesenteric.
  • The 3 others were intra-abdominal DF.

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  • (PMID = 27963790.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Alebouyeh M, Moussavi F, Tabari AK, Vossough P: Aggressive intra-abdominal fibromatosis in children and response to chemotherapy. Pediatr Hematol Oncol; 2005 Sep;22(6):447-51
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive intra-abdominal fibromatosis in children and response to chemotherapy.
  • Intra-abdominal fibromatosis (IAF) is a rare benign neoplasm arising from the abdominal fibrous tissue, mostly in the mesentery.
  • Within 6 months after initial presentation, he underwent 4 subsequent abdominal explorations for diagnosis, tumor reduction, and intestinal obstructions.
  • Due to accelerated tumor growth and deteriorated general condition, as a last resort, a chemotherapy trial with vincristin and methotrexate was carried out.
  • This regimen proved to be effective in reducing the tumor burden and improving the patient's general condition.
  • Outcome of IAF depends on early diagnosis and complete tumor resection, and, if indicated, timely employment of neo/adjuvant chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Fibromatosis, Abdominal / drug therapy. Fibromatosis, Aggressive / drug therapy
  • [MeSH-minor] Child, Preschool. Humans. Male. Methotrexate / administration & dosage. Tumor Burden / drug effects. Vincristine / administration & dosage

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  • (PMID = 16169811.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; YL5FZ2Y5U1 / Methotrexate
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19. Croce S, Letourneux C, Dale G, Mathelin C: [Breast fibromatosis: An uncommon benign breast disease]. Gynecol Obstet Fertil; 2009 May;37(5):442-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Breast fibromatosis: An uncommon benign breast disease].
  • [Transliterated title] La fibromatose mammaire : une lésion bénigne peu connue.
  • Extra-abdominal fibromatosis is an uncommon benign breast lesion resembling an infiltrative carcinoma in its clinical and radiological presentation.
  • Surgical excision with wide margins remains the treatment of choice to avoid recurrence of this locally aggressive tumor.
  • We present a case in which surgical biopsy allowed the diagnosis of a breast fibromatosis and we discuss its clinical, diagnostic, pathological and therapeutic particularities.

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  • (PMID = 19362506.001).
  • [ISSN] 1297-9589
  • [Journal-full-title] Gynécologie, obstétrique & fertilité
  • [ISO-abbreviation] Gynecol Obstet Fertil
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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20. Efstratiadis G, Garyfallos A, Tsiaousis G, Jiavazis I, Deretzi G, Leontsini M, Venizelos I, Memmos D: Necrotic vasculitis in a patient with abdominal fibromatosis and Guillain-Barré syndrome. Ren Fail; 2006;28(8):743-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Necrotic vasculitis in a patient with abdominal fibromatosis and Guillain-Barré syndrome.
  • Tomographic investigation revealed abdominal and retroperitoneal fibromatosis.
  • [MeSH-major] Fibromatosis, Abdominal / complications. Guillain-Barre Syndrome / complications. Vasculitis / pathology
  • [MeSH-minor] Aged. Antibodies, Antineutrophil Cytoplasmic / blood. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / blood. CA-125 Antigen / blood. Cyclophosphamide / administration & dosage. Female. Humans. Kidney / pathology. Methylprednisolone / administration & dosage. Necrosis / complications. Tomography, X-Ray Computed. Ultrasonography, Interventional


21. Giuliani A, Demoro M, Ciardi A, Scimó M, Galati F, Lonardo MT, Galati G: Mesenteric fibromatosis. Case report. J Exp Clin Cancer Res; 2007 Sep;26(3):425-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis. Case report.
  • Deep fibromatosis is a rare locally aggressive but not metastasizing proliferation.
  • Intra-abdominal fibromatosis (IAF) occurs either in association with Gardner's syndrome or as a sporadic event and presents in most cases differential diagnostic problems with myofibroblastic or fibroblastic tumors, characterized by a more aggressive biological behaviour such as gastrointestinal stromal tumors (GISTs).
  • In absence of loco-regional and/or distant metastasis differential diagnosis may be difficult and represents a topical issue, since it influences treatment choice.
  • We describe the case of a patient with sporadic IAF in which the tumor locally involved the mesentery and presented no loco-regional and distant spread.
  • Definitive diagnosis was made, postoperatively, on the basis of immunohistochemical findings.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibroma / diagnosis. Mesentery
  • [MeSH-minor] Diagnosis, Differential. Gardner Syndrome / pathology. Humans. Male. Middle Aged

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  • (PMID = 17987807.001).
  • [ISSN] 0392-9078
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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22. Weiss ES, Burkart AL, Yeo CJ: Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy. J Gastrointest Surg; 2006 May;10(5):679-88
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy.
  • Intra-abdominal fibromatosis or desmoid tumors are rare forms of connective tissue cellular dysplasia characterized by proliferation of fibroblasts and abundant collagen.
  • Sporadic intra-abdominal desmoids involving the pancreas are quite rare, as only six previously reported cases exist.
  • In this report we present a seventh case of a sporadic intraabdominal desmoid involving the pancreas.
  • The patient, a 63-year-old white man, developed the desmoid tumor following a pylorus-preserving pancreaticoduodenectomy for an insulinoma.
  • Finally, we present a complete review of the six previous cases of sporadic pancreatic fibromatosis.
  • [MeSH-minor] Fibromatosis, Aggressive / pathology. Humans. Male. Middle Aged. Tomography, X-Ray Computed / methods

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  • (PMID = 16773761.001).
  • [ISSN] 1091-255X
  • [Journal-full-title] Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
  • [ISO-abbreviation] J. Gastrointest. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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23. Basu S, Nair N, Banavali S: Uptake characteristics of fluorodeoxyglucose (FDG) in deep fibromatosis and abdominal desmoids: potential clinical role of FDG-PET in the management. Br J Radiol; 2007 Sep;80(957):750-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Uptake characteristics of fluorodeoxyglucose (FDG) in deep fibromatosis and abdominal desmoids: potential clinical role of FDG-PET in the management.
  • In this preliminary report, we explore the uptake pattern of fluorodeoxyglucose (FDG) in fibromatosis and hypothesize the potential clinical role of FDG-positron emission tomography (PET) in the management of this benign but locally aggressive heterogeneous group of soft-tissue tumours.
  • Five patients were studied (two men and three women, age range 23-35 years), among whom were three cases of deep musculoskeletal fibromatosis, one of abdominal fibromatosis (abdominal desmoid) associated with familial adenomatous polyposis (Gardner's syndrome) and one case of both deep musculoskeletal fibromatosis and abdominal desmoid.
  • The maximum standardized uptake value (SUV(max)) observed was up to 4.7; the avidity probably related to the biological aggressiveness and tendency for recurrence, characteristic of fibromatosis.
  • Hence, a baseline FDG-PET can serve a valuable role in monitoring the effect of systemic pharmacotherapy in patients with recurrent progressive disease after unsuccessful local-regional treatment.
  • The findings in this report can be extrapolated and have implications for studying the utility of FDG-PET in defining aggressiveness, guiding biopsy and defining excision site in a large tumour and in monitoring therapy in fibromatosis.
  • [MeSH-minor] Adult. Female. Fibromatosis, Abdominal / complications. Fibromatosis, Abdominal / radiography. Fibromatosis, Abdominal / radionuclide imaging. Gardner Syndrome / complications. Humans. Male. Positron-Emission Tomography. Recurrence. Retrospective Studies

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  • (PMID = 17709361.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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24. Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, Vanel D, Terrier P, Blay JY, Le Cesne A, Le Péchoux C: Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol; 2008 Apr;34(4):462-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients.
  • OBJECTIVE: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis.
  • Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases.
  • Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant.
  • Quality of resection according to margins and the tumour site were the only prognostic factors.
  • There was a significant correlation between tumour site and quality of surgery (p=0.0002).
  • CONCLUSIONS: A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17709227.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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25. Shindholimath VV, Kumar V, Chumber S, Bhuvnesh, Srivastava A: Mesenteric fibromatosis with involvement of the gastrointestinal tract. Trop Gastroenterol; 2006 Oct-Dec;27(4):177-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis with involvement of the gastrointestinal tract.
  • Primary mesenteric fibromatosis is a rare condition.
  • The aggressive nature of these tumors and the potential for major morbidity secondary to resection makes it a challenging disease for the surgeon.
  • We report a case of mesenteric fibromatosis with involvement of small bowel.
  • [MeSH-major] Duodenal Diseases / etiology. Fibromatosis, Abdominal / complications. Intestinal Fistula / etiology. Mesentery. Peritoneal Neoplasms / complications

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  • (PMID = 17542298.001).
  • [ISSN] 0250-636X
  • [Journal-full-title] Tropical gastroenterology : official journal of the Digestive Diseases Foundation
  • [ISO-abbreviation] Trop Gastroenterol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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26. Ng TY, Yang MD, Chen YF, Chang CH: Resolution of hydronephrosis due to massive mesenteric fibromatosis using cyclo-oxygenase 2 inhibitors. Urology; 2007 Sep;70(3):591.e3-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Resolution of hydronephrosis due to massive mesenteric fibromatosis using cyclo-oxygenase 2 inhibitors.
  • Mesenteric fibromatosis is currently a diagnostic and therapeutic challenge.
  • We report a case of mesenteric fibromatosis in a 29-year-old man with a 2-month history of periumbilical fullness who was discovered to have an abdominal mass with hydronephrosis.
  • To the best of our knowledge, we report the first case of hydronephrosis in a mesenteric fibromatosis with Gardner's syndrome, which was successfully treated with a cyclo-oxygenase 2 inhibitor without surgical removal of the mesenteric fibromatosis.
  • The hydronephrosis and mesenteric fibromatosis improved modestly with long-term celecoxib (200 mg twice daily).
  • [MeSH-minor] Adult. Celecoxib. Gardner Syndrome / diagnosis. Gardner Syndrome / genetics. Humans. Laparotomy. Lithotripsy. Male. Nephrostomy, Percutaneous. Ureteral Calculi / etiology. Ureteral Calculi / therapy

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  • (PMID = 17905129.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclooxygenase 2 Inhibitors; 0 / Pyrazoles; 0 / Sulfonamides; JCX84Q7J1L / Celecoxib
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27. Lath C, Khanna PC, Gadewar SB, Agrawal D: Inoperable aggressive mesenteric fibromatosis with ureteric fistula. Case report and literature review. Eur J Radiol; 2006 Jul;59(1):117-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Inoperable aggressive mesenteric fibromatosis with ureteric fistula. Case report and literature review.
  • The purpose of our report is to illustrate an aggressive case of mesenteric fibromatosis in a 17-year-old girl with a ureteric fistula and to review imaging and pathological features, natural history and treatment options of this disease.
  • Our patient underwent computed tomography that revealed a widespread intra-abdominal mass.
  • Fibromatoses represent a spectrum of uncommon benign conditions characterised by proliferating fibrous tissue.
  • The deep intra-abdominal form of mesenteric fibromatosis (MF), one of the rare subtypes of the 'fibromatoses' or 'desmoid tumours', grows rapidly and may become extensive.
  • Surgery provides good results in limited disease and non-surgical modalities in cases of unresectable and residual disease.
  • [MeSH-major] Fibromatosis, Abdominal / radiography. Fibromatosis, Aggressive / radiography. Mesentery / pathology. Tomography, X-Ray Computed. Ureteral Diseases / radiography. Urinary Fistula / radiography

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  • [ErratumIn] Eur J Radiol. 2006 Dec;60(3):480
  • (PMID = 16464556.001).
  • [ISSN] 0720-048X
  • [Journal-full-title] European journal of radiology
  • [ISO-abbreviation] Eur J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Ireland
  • [Number-of-references] 16
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28. Bertagnolli MM, Morgan JA, Fletcher CD, Raut CP, Dileo P, Gill RR, Demetri GD, George S: Multimodality treatment of mesenteric desmoid tumours. Eur J Cancer; 2008 Nov;44(16):2404-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multimodality treatment of mesenteric desmoid tumours.
  • BACKGROUND: Desmoid tumours are rare neoplasms characterised by clonal proliferation of myofibroblasts that do not metastasise, but often exhibit an infiltrative pattern and functional impairment.
  • A multidisciplinary treatment plan was developed based on primary disease extent, tumour behaviour and resectability.
  • Patients with stable but unresectable disease were observed without treatment.
  • Patients with resectable disease underwent surgery, and patients with unresectable progressing disease received chemotherapy, most commonly liposomal doxorubicin, followed by surgery if chemotherapy rendered the disease resectable.
  • RESULTS: At a median follow-up of 50.0 months (range 4.6-212), 50 patients (96%) have either no recurrence or radiographically stable disease.
  • CONCLUSION: These data indicate that the extent of disease; tumour behaviour and resectability are the important factors when defining a treatment plan for mesenteric desmoid tumours.
  • A multidisciplinary approach of surgery combined with chemotherapy is an effective and function-sparing strategy for managing this disease.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibromatosis, Aggressive / drug therapy. Fibromatosis, Aggressive / surgery. Mesentery. Peritoneal Neoplasms / drug therapy. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Combined Modality Therapy. Doxorubicin / administration & dosage. Female. Fibromatosis, Abdominal / drug therapy. Fibromatosis, Abdominal / surgery. Humans. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome. Vinblastine / administration & dosage. Vinblastine / analogs & derivatives. Young Adult

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  • (PMID = 18706807.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 5V9KLZ54CY / Vinblastine; 80168379AG / Doxorubicin; Q6C979R91Y / vinorelbine
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29. Iwasa T, Sadamoto Y, Itaba S, Nasu T, Ihara Y, Misawa T, Nakamura K: [A case of mesenteric desmoid tumor]. Nihon Shokakibyo Gakkai Zasshi; 2007 Jun;104(6):804-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A case of mesenteric desmoid tumor].
  • Abdominal computed tomography showed a tumor, about 5 cm in diameter at the duodeno-jejunal junction.
  • The preoperative diagnosis was submucosal or mesenteric tumor.
  • The tumor derived from the mesenterium and involved the anal side of the small intestine.
  • The tumor was removed with partial excision of the upper jejunum.
  • The diagnosis of mesenteric desmoid tumor was confirmed, histologically.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Ileus / etiology. Mesentery. Peritoneal Neoplasms / diagnosis

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  • (PMID = 17548947.001).
  • [ISSN] 0446-6586
  • [Journal-full-title] Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
  • [ISO-abbreviation] Nihon Shokakibyo Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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30. Rajakannu M, Ananthakrishnan N, Madhavan M: Isolated mesenteric fibromatosis. Trop Gastroenterol; 2008 Jul-Sep;29(3):179-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Isolated mesenteric fibromatosis.
  • Fibromatosis is a rare, locally aggressive but non-metastasising fibrous mass often associated with familial adenomatous polyposis in Gardner's Syndrome (GS).
  • Although sporadic cases occur, unlike in GS, they are predominantly extra-abdominal.
  • We report the case of a 40-year-old lady who underwent laparotomy for a large isolated abdominal mass three years after a Whipple's procedure for adenocarcinoma of the distal common bile duct.
  • A spherical, football sized tumour was found in the jejunal mesentery.
  • Resection of the tumour with jejunum was carried out.
  • This case is peculiar in that mesenteric fibromatosis occurred in a patient with prior history of periampullary carcinoma and without history of familial adenomatous polyposis.
  • [MeSH-major] Fibroma / diagnosis. Jejunal Neoplasms / diagnosis

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  • (PMID = 19115615.001).
  • [ISSN] 0250-636X
  • [Journal-full-title] Tropical gastroenterology : official journal of the Digestive Diseases Foundation
  • [ISO-abbreviation] Trop Gastroenterol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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31. Colović R, Grubor N, Radak V, Micev M, Stojković M, Colović N: [Aggressive intraabdominal fibromatosis]. Vojnosanit Pregl; 2006 Sep;63(9):839-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Aggressive intraabdominal fibromatosis].
  • BACKGROUND: Intraabdominal or mesenteric fibromatosis is a rare benign nonmetastatic neoplasm that appears as a sporadic lesion or in patients with familiar polyposis.
  • CASE REPORT: We presented a 22-year-old woman in whom an aggressive intraabdominal fibromatosis had appeared during the first pregnancy as a well circumscribed ovoid tumor, involving the terminal ileum, the caecum, the ascending colon, the right kidney, the ureter, and the right common iliac artery.
  • The tumor was excised with right colectomy, nephroureterectomy and resection of the involved artery using arterial reconstruction with graft interposition.
  • Two years after the surgery the patient developed an inoperable tumor recurrency with a fatal outcome.
  • CONCLUSION: In spite of a successful surgical excision during the original surgery intraabdominal or mesenteric fibromatosis might have an aggressive evolution leading to an inoperable tumor recurrency and a fatal outcome.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology. Neoplasm Recurrence, Local. Pregnancy Complications, Neoplastic / pathology

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  • (PMID = 17039898.001).
  • [ISSN] 0042-8450
  • [Journal-full-title] Vojnosanitetski pregled
  • [ISO-abbreviation] Vojnosanit Pregl
  • [Language] srp
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Serbia and Montenegro
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32. Huerta S, Heubner DR, Marcus DR: Mesenteric fibromatosis in a young girl without familial adenomatous polyposis. J Pediatr Surg; 2005 May;40(5):e33-6
MedlinePlus Health Information. consumer health - Peritoneal Disorders.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis in a young girl without familial adenomatous polyposis.
  • Mesenteric fibromatosis (MF) is a rare, benign tumor commonly associated with Gardner's syndrome.
  • Patients may present with abdominal pain or discomfort when the tumors reach large sizes, which is typical at the time of diagnosis.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Omentum / surgery. Peritoneal Diseases / surgery


33. Holubar S, Dwivedi AJ, O'Connor J: Giant mesenteric fibromatosis presenting as small bowel obstruction. Am Surg; 2006 May;72(5):427-9
MedlinePlus Health Information. consumer health - Intestinal Obstruction.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant mesenteric fibromatosis presenting as small bowel obstruction.
  • Mesenteric fibromatosis is a proliferative fibroblastic neoplasia of the small intestine mesentery that may occur as a unique or multiple formation.
  • Mesenteric fibromatosis is a rare, locally aggressive neoplasm and may present with abdominal discomfort, abdominal pain, weight loss, or symptoms of ureteral obstruction, mesenteric ischemia, or intestinal obstruction.
  • It is of the utmost importance to distinguish mesenteric fibromatosis from gastrointestinal stromal tumors.
  • The involvement of important structures like the superior mesenteric artery and the superior mesenteric vein may pose a challenge during resection, but these tumors can be excised and the vessels repaired primarily.
  • [MeSH-major] Fibromatosis, Abdominal / complications. Fibromatosis, Abdominal / diagnosis. Intestinal Obstruction / etiology
  • [MeSH-minor] Diagnosis, Differential. Gastrointestinal Stromal Tumors / pathology. Humans. Male. Mesentery. Middle Aged. Neoplasm Invasiveness

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  • (PMID = 16719198.001).
  • [ISSN] 0003-1348
  • [Journal-full-title] The American surgeon
  • [ISO-abbreviation] Am Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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34. Spiridakis K, Panagiotakis G, Grigoraki M, Kokkinos I, Papadakis T, Kokkinakis T, Kandylakis S: Isolated giant mesenteric fibromatosis (intra-abdominal desmoid tumors). Case report. G Chir; 2008 Oct;29(10):413-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Isolated giant mesenteric fibromatosis (intra-abdominal desmoid tumors). Case report.
  • A rare case of isolated giant mesenteric fibromatosis is presented.
  • The tumor originated from the fibrous mesenteric tissue.
  • The patient underwent laparotomy because of abdominal discomfort and sub-occlusive symptoms due to the giant mass.
  • Differential diagnosis of mesenteric masses is discussed and the Authors also review the literature concerning this rare disease.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Abdominal / surgery. Peritoneal Neoplasms / diagnosis. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Adult. Diagnosis, Differential. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery. Humans. Male. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18947463.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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35. Ballardini P, Gulmini L, Margutti G, Lelli G: Mesenteric fibromatosis in a patient with Camurati-Engelmann disease. A case report and literature review. Tumori; 2005 Nov-Dec;91(6):552-4
The Weizmann Institute of Science GeneCards and MalaCards databases. gene/protein/disease-specific - MalaCards for camurati-engelmann disease .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis in a patient with Camurati-Engelmann disease. A case report and literature review.
  • Mesenteric fibromatosis is a rare type of desmoid tumor characterized by local aggressiveness and a tendency to relapse.
  • Camurati-Engelmann disease is a very rare form of bone dysplasia characterized by osteosclerosis of the diaphyses of the long bones.
  • Here we describe the case of a male patient affected by these two rare diseases in association with chronic inflammatory intestinal disease.

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  • (PMID = 16457156.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 21
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36. McCormack D, Kesha K, Tittle SL, Saldinger PF: Mesenteric fibromatosis mimicking a gastrointestinal stromal tumor. Conn Med; 2010 Apr;74(4):197-200
Genetic Alliance. consumer health - Fibromatosis.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis mimicking a gastrointestinal stromal tumor.
  • Mesenteric fibromatosis is a locally aggressive tumor of the mesentery with a high propensity for bowel involvement.
  • Mesenteric fibromatosis often mimics gastrointestinal stromal tumors in size, location and immunohistochemical features.
  • We report the case of a 30-year-old male who underwent resection of a mesenteric tumor, initially diagnosed as gastrointestinal stromal tumor.
  • The tumor was categorized as high-risk and the patient was treated with chemotherapy.
  • The tumor did not respond to medical management.
  • The patient underwent a second en bloc resection and pathology results were conclusive for mesenteric fibromatosis.
  • This case highlights the significance of accurately differentiating mesenteric fibromatosis from gastrointestinal stromal tumor.
  • Making a concrete diagnosis is often difficult because both gastrointestinal stromal tumors and mesenteric fibromatosis share a number of morphological and immunohistochemical features including CKIT expression.
  • [MeSH-major] Diagnostic Errors. Fibromatosis, Abdominal / diagnosis. Gastrointestinal Stromal Tumors / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Diagnosis, Differential. Humans. Male

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  • (PMID = 20440999.001).
  • [ISSN] 0010-6178
  • [Journal-full-title] Connecticut medicine
  • [ISO-abbreviation] Conn Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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37. Bethune R, Amin A: Mesenteric fibromatosis: a rare cause of acute abdominal pain. Ann R Coll Surg Engl; 2006 Mar;88(2):1-2
MedlinePlus Health Information. consumer health - Small Intestine Disorders.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis: a rare cause of acute abdominal pain.
  • A 45-year-old man, with a previous history of astrocytoma, presented with diffuse abdominal pain.
  • A CT scan was non-specific and the pain worsened; his abdomen became peritonitic on examination.
  • An exploratory laparotomy was performed, and a large small bowel mesenteric mass was found together with an associated haematoma.
  • Subsequent histology showed the tumour to be a benign fibromatosis, and the patient recovered uneventfully.
  • There were no reports in the literature of any association between astrocytomas and fibromatosis.
  • Mesenteric fibromatosis is a rare tumour often associated with Gardner's syndrome.
  • [MeSH-major] Abdominal Pain / etiology. Fibroma / complications. Ileal Diseases / complications. Mesentery
  • [MeSH-minor] Acute Disease. Astrocytoma / complications. Colonic Diseases / complications. Diagnosis, Differential. Gastrointestinal Hemorrhage / complications. Hematoma / complications. Humans. Male. Middle Aged

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  • (PMID = 16884606.001).
  • [ISSN] 1478-7083
  • [Journal-full-title] Annals of the Royal College of Surgeons of England
  • [ISO-abbreviation] Ann R Coll Surg Engl
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 5
  • [Other-IDs] NLM/ PMC1963641
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38. Jung SH, Paik CN, Jung JH, Lee KM, Chung WC, Yang JM: Simultaneous Colonic Obstruction and Hydroureteronephrosis due to Mesenteric Fibromatosis. Gut Liver; 2009 Sep;3(3):215-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Simultaneous Colonic Obstruction and Hydroureteronephrosis due to Mesenteric Fibromatosis.
  • Mesenteric fibromatosis (MF) is a rare benign mesenchymal lesion that can occur throughout the gastrointestinal tract, especially small bowel.
  • We described a patient that presented with colonic obstruction and hydroureteronephrosis due to MF at sigmoid colon which mimicked submucosal tumor such as gastrointestinal tumor.
  • This case resulted in a positive positron emission tomography scan suggesting malignant neoplasm, but beta-catenin positivity on immunohistochemical staining separated MF from gastrointestinal stromal tumor and sclerosing mesenteritis.

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  • [Cites] World J Gastroenterol. 2007 Dec 7;13(45):5985-8 [18023087.001]
  • [Cites] Dis Colon Rectum. 2007 Jun;50(6):924-6 [17205200.001]
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  • (PMID = 20431749.001).
  • [ISSN] 2005-1212
  • [Journal-full-title] Gut and liver
  • [ISO-abbreviation] Gut Liver
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2852704
  • [Keywords] NOTNLM ; Colonic obstruction / Hydroureteronephrosis / Mesenteric fibromatosis
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39. Schlager A, Altchek A, Kalir T, Deligdisch L, Weber KJ: Mesenteric fibromatosis masquerading as an ovarian neoplasm twenty years after Chernobyl radiation exposure. Gynecol Oncol; 2006 Sep;102(3):587-9
MedlinePlus Health Information. consumer health - Ovarian Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric fibromatosis masquerading as an ovarian neoplasm twenty years after Chernobyl radiation exposure.
  • BACKGROUND: Since their initial description in 1832, desmoid tumors have been reported to occur in virtually every part of the body.
  • Intra-abdominal desmoid tumors, or mesenteric fibromatosis, are often associated with Familial Adenomatous Polyposis mutation in a syndrome known as Gardner's Syndrome.
  • Although sporadic cases of desmoid tumors do occur, unlike Gardner's Syndrome, they predominantly occur extra-abdominally.
  • CASE: Case report of a 61-year-old female who presented with two months of abdominal pain, progressive lower abdominal distension and a 10-15 pound weight gain accompanied by one week of urinary hesitancy and frequency.
  • Patient underwent a diagnostic workup for an ovarian neoplasm, but was found at surgery to have mesentric fibromatosis.
  • CONCLUSION: Although uncommon, mesentric fibromatosis must be considered in the differential diagnosis when evaluating a patient with an abdominal mass of unknown origin.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Aggressive / diagnosis. Ovarian Neoplasms / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Middle Aged. Power Plants. Radioactive Hazard Release. Ukraine

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  • (PMID = 16678243.001).
  • [ISSN] 0090-8258
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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40. Batori M, Chatelou E, Mariotta G, Sportelli G, Straniero A, Casella G, Casella MC: Giant mesenteric fibromatosis. Eur Rev Med Pharmacol Sci; 2005 Jul-Aug;9(4):223-5
Genetic Alliance. consumer health - Fibromatosis.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Giant mesenteric fibromatosis.
  • Mesenteric fibromatosis is a proliferative fibroblastic neoplasia of the small intestine mesentery which may occur as a unique or multiple formation.
  • Mesenteric fibromatosis represents the 8% of all desmoid neoplasm.
  • Giant mesenteric fibromatosis is uncommon by itself (2-4 case/milion/year).
  • Since the rarity of this tumor and the difficulties in diagnostic and therapeutic ambit, we believe it justified to describe a case of giant mesenteric fibromatosis which came to our observation.

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  • (PMID = 16128042.001).
  • [ISSN] 1128-3602
  • [Journal-full-title] European review for medical and pharmacological sciences
  • [ISO-abbreviation] Eur Rev Med Pharmacol Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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41. Rajesh A, Sandrasegaran K: Mesenteric desmoid mimicking recurrent testicular cancer. Abdom Imaging; 2005 Nov-Dec;30(6):777-9
MedlinePlus Health Information. consumer health - Testicular Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric desmoid mimicking recurrent testicular cancer.
  • The detection of a solid abdominal mass after surgery for testicular cancer most frequently represents metastatic disease.
  • We report an unusual case of mesenteric desmoid tumor presenting after retroperitoneal lymph node dissection for metastatic testicular cancer.
  • [MeSH-major] Fibromatosis, Aggressive / radiography. Mesentery. Neoplasm Recurrence, Local / radiography. Peritoneal Neoplasms / radiography. Testicular Neoplasms / radiography

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  • (PMID = 16096867.001).
  • [ISSN] 0942-8925
  • [Journal-full-title] Abdominal imaging
  • [ISO-abbreviation] Abdom Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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42. Perenze B, Potì O, Margiotta A, Rega D, Marrano N, Minni F: [Mesenteric fibromatosis]. Chir Ital; 2005 Nov-Dec;57(6):717-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Mesenteric fibromatosis].
  • [Transliterated title] La fibromatosi mesenterica.
  • Mesenteric fibromatosis is a rare benign mesenteric tumour, characterised by infiltrative growth and high rates of recurrence.
  • Histological examination is the only diagnostic procedure capable of ruling out a malignant mesenteric neoplasm.
  • It is not always possible to perform a radical removal of the tumour mass because it may have infiltrated important anatomical structures.
  • In the present study the Authors report two cases of mesenteric fibromatosis and analyse the main clinical-diagnostic, therapeutic and prognostic problems posed by these tumours.

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  • (PMID = 16400766.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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43. Rakha EA, Kandil MA, El-Santawe MG: Gigantic recurrent abdominal desmoid tumour: a case report. Hernia; 2007 Apr;11(2):193-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gigantic recurrent abdominal desmoid tumour: a case report.
  • Deeply seated fibromatosis or desmoid tumour (DT) is a rare entity characterized by benign proliferation of fibroblasts.
  • Although non-malignant, this tumour can be life-threatening due to its invasive property and high recurrence rate.
  • We report a unique case of a huge recurrent abdominal DT (36 cm in diameter, 25 kg in weight) that caused pressure necrosis and sloughing of the overlying anterior abdominal wall, and produced a large fungating mass protruding outside the abdomen.
  • Although preoperative neoadjuvant therapies were all ineffective, radical surgical removal of the tumour was successful.
  • The procedure was followed by an excellent clinical recovery and the patient is still alive with no evidence of recurrent disease after a 6-year follow-up.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Neoplasm Recurrence, Local / pathology

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  • (PMID = 17149531.001).
  • [ISSN] 1265-4906
  • [Journal-full-title] Hernia : the journal of hernias and abdominal wall surgery
  • [ISO-abbreviation] Hernia
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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44. Eren S: A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction. Eur J Pediatr Surg; 2007 Aug;17(4):285-8
MedlinePlus Health Information. consumer health - Intestinal Obstruction.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.
  • Desmoid tumours, also known as aggressive fibromatosis, are rare lesions with an intermediate biological behaviour between benign fibrous lesions and fibrosarcomas.
  • Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in an isolated form.
  • We report the findings of a barium study, ultrasound, computed tomography and magnetic resonance imaging in a nine-year-old boy with intermittent nausea and vomiting, diagnosed as having a desmoid tumour.
  • Although intra-abdominal desmoids are usually detected as a solitary lesion in sporadic cases, the presented case had two mesenteric lesions in the left upper quadrant.
  • [MeSH-major] Abdominal Neoplasms / complications. Digestive System Surgical Procedures / methods. Fibromatosis, Aggressive / complications. Intestinal Obstruction / etiology
  • [MeSH-minor] Child. Diagnosis, Differential. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 17806029.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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45. Sun L, Wu H, Zhuang YZ, Guan YS: A rare case of pregnancy complicated by mesenteric mass: what does chylous ascites tell us? World J Gastroenterol; 2007 Mar 14;13(10):1632-5
MedlinePlus Health Information. consumer health - Health Problems in Pregnancy.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A rare case of pregnancy complicated by mesenteric mass: what does chylous ascites tell us?
  • Mesenteric fibromatosis is a rare benign nonmetastatic neoplasm that appears as a sporadic lesion or occurs in patients with familial polyposis, while chylous ascites associated with aggressive mesenteric fibromatosis during pregnancy has never been reported thus far.
  • Here we present the case of a 28-year old pregnant woman, in whom an aggressive mesenteric fibromatosis with chylous ascites was detected, involving the jejunum, superior mesenteric artery (SMA) and superior mesenteric vein (SMV) and pancreas.
  • The authors report the case for its rarity and emphasize on combining clinicopathological, radiological and immunohistochemistry analysis for management of the disease.
  • [MeSH-major] Chylous Ascites / etiology. Fibromatosis, Abdominal / complications. Fibromatosis, Abdominal / diagnosis. Pregnancy Complications / diagnosis
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging. Mesentery / pathology. Mesentery / radiography. Peritoneal Neoplasms / complications. Peritoneal Neoplasms / diagnosis. Peritoneal Neoplasms / pathology. Pregnancy. Tomography, Spiral Computed

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  • [Cites] Histopathology. 2001 May;38(5):387-402 [11422475.001]
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  • (PMID = 17461463.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Other-IDs] NLM/ PMC4146913
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46. Sakorafas GH, Nissotakis C, Peros G: Abdominal desmoid tumors. Surg Oncol; 2007 Aug;16(2):131-42

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Abdominal desmoid tumors.
  • Desmoid tumors are rare, benign, fibromatous lesions that are the result of abnormal proliferation of myofibroblasts.
  • Desmoid tumors can be classified as extra-abdominal and abdominal.
  • Abdominal desmoid tumors are either superficial or intraabdominal.
  • These tumors are associated with a high recurrence rates, even if their microscopic characters indicate a benign disease; their biologic behavior often indicates rather a "malignant" disease, which can cause even the death.
  • Intraabdominal desmoid tumors can engulf surrounding viscera and vessels, thereby greatly complicating their surgical treatment.
  • [MeSH-major] Abdominal Neoplasms / pathology. Abdominal Neoplasms / therapy. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Algorithms. Diagnosis, Differential. Diagnostic Imaging. Genetic Testing. Humans. Medical History Taking

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  • (PMID = 17719772.001).
  • [ISSN] 0960-7404
  • [Journal-full-title] Surgical oncology
  • [ISO-abbreviation] Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 86
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47. Eren S: A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction. Eur J Pediatr Surg; 2005 Jun;15(3):196-9
MedlinePlus Health Information. consumer health - Intestinal Obstruction.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.
  • Desmoid tumours, also known as aggressive fibromatoses, are rare lesions having intermediate biological behaviour between benign fibrous lesions and fibrosarcomas.
  • Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in isolated form.
  • Although intraabdominal desmoids are usually detected as a solitary lesion in sporadic cases, the case presented here had two mesenteric lesions in the left upper quadrant.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Intestinal Obstruction / complications. Mesentery. Peritoneal Neoplasms / diagnosis

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  • (PMID = 15999314.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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48. Ko SF, Lin JW, Ng SH, Huang CC, Wan YL, Huang HY, Sheen-Chen SM: Spontaneous isolated mesenteric fibromatosis: sonographic and computed tomographic findings with pathologic correlation. Ultrasound Med Biol; 2006 Aug;32(8):1141-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Spontaneous isolated mesenteric fibromatosis: sonographic and computed tomographic findings with pathologic correlation.
  • Eight cases of spontaneous isolated mesenteric fibromatosis (SIMF) were retrospectively reviewed.
  • Clinical presentations included palpable abdominal mass (n = 6), abdominal pain (n = 4), gastrointestinal bleeding (n = 2) and acute abdomen (n = 1).
  • On sonography and computed tomography (CT), eight SIMFs (size range 3 to 24 cm, mean 14.8 cm) were categorized into four morphologic patterns: well-defined inhomogeneous, well-defined homogeneous, well-defined cystic or infiltrative mesenteric mass patterns.
  • Well-defined inhomogeneous SIMF (n = 3) was correlated with the histopathologic finding of bundles of fibroblasts with unevenly intermingled hyaline and/or myxoid degeneration areas, whereas well-defined homogeneous SIMF (n = 2) showed scarce degenerative changes.
  • One SIMF presented as an infiltrative mesenteric mass and the patient died 10 months after diagnosis.
  • Both cases of cystic SIMFs showed tumor recurrences and one patient died after 84 months.
  • The other five patients were cured by radical tumor resection.
  • In summary, sonography, similar to CT, is also useful for evaluating SIMF with protean morphologic features, ascribing to the underlying histopathologic changes with varied degrees of hyaline, myxoid or cystic degenerations.
  • [MeSH-major] Fibromatosis, Abdominal / ultrasonography. Mesentery / ultrasonography

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  • (PMID = 16875948.001).
  • [ISSN] 0301-5629
  • [Journal-full-title] Ultrasound in medicine & biology
  • [ISO-abbreviation] Ultrasound Med Biol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Contrast Media; K2I13DR72L / Gadolinium DTPA
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49. Ezumi K, Yamamoto H, Takemasa I, Nomura M, Ikeda M, Sekimoto M, Monden M: Dacarbazine-Doxorubicin therapy ameliorated an extremely aggressive mesenteric desmoid tumor associated with familial adenomatous polyposis: report of a case. Jpn J Clin Oncol; 2008 Mar;38(3):222-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dacarbazine-Doxorubicin therapy ameliorated an extremely aggressive mesenteric desmoid tumor associated with familial adenomatous polyposis: report of a case.
  • After 10 months, we found an intra-abdominal tumor, which grew rapidly to 25 cm in diameter.
  • We performed an emergency operation, which revealed that it was a desmoid tumor derived mainly from colorectal mesenterium.
  • The tumor was removed with three short segments of intestine and the left ureter.
  • A computed tomography (CT) scan done 3 months later showed a 10 cm mesenteric desmoid tumor at the beginning of jejunum, approaching the root of the superior mesenteric artery (SMA).
  • Fortunately, we were able to remove the tumor without injuring the SMA.
  • To our distress, however, another recurrent mesenteric desmoid tumor was discovered in the pelvis one month later, which grew rapidly from 5 cm to 16 cm within 4 months.
  • Finally, when the desmoid occupied the pelvic space, we gave the patient dacarbazine (DTIC) and doxorubicin (DOX).
  • After seven courses, the mesenteric tumor showed an almost complete response (CR).
  • No evidence of further recurrence of mesenteric desmoid has been seen for 4 years.
  • This combination chemotherapy is a promising strategy, even against an extremely aggressive, life-threatening mesenteric desmoid associated with FAP.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dacarbazine / administration & dosage. Doxorubicin / administration & dosage. Fibromatosis, Aggressive / drug therapy. Mesentery. Peritoneal Neoplasms / drug therapy

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  • (PMID = 18304951.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 094ZI81Y45 / Tamoxifen; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; 8A1O1M485B / Imatinib Mesylate
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50. Shah M, Azam B: Case report of an intra-abdominal desmoid tumour presenting with bowel perforation. Mcgill J Med; 2007 Jul;10(2):90-2

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Case report of an intra-abdominal desmoid tumour presenting with bowel perforation.
  • Desmoid tumours are benign tumours originating from the musculoaponeurotic structures of the body.
  • Here we present a case report of a young gentleman who presented with an acute surgical abdomen and subsequently underwent a laparotomy and was found to have an inflammatory mass.
  • Histological analysis of this mass revealed mesenteric fibromatosis (desmoid tumour).
  • It is rare for mesenteric fibromatosis to present with intestinal perforation and only one case been reported in the literature thus far (1).

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  • [Cites] Front Radiat Ther Oncol. 2001;35:107-19 [11351941.001]
  • [Cites] Arch Pathol. 1961 Feb;71:214-21 [13706852.001]
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  • (PMID = 18523540.001).
  • [ISSN] 1201-026X
  • [Journal-full-title] McGill journal of medicine : MJM : an international forum for the advancement of medical sciences by students
  • [ISO-abbreviation] Mcgill J Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2323473
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51. Rampone B, Pedrazzani C, Marrelli D, Pinto E, Roviello F: Updates on abdominal desmoid tumors. World J Gastroenterol; 2007 Dec 7;13(45):5985-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Updates on abdominal desmoid tumors.
  • Desmoid tumor is a monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures.
  • Abdominal desmoid occurs sporadically, in association with some familial syndromes and often represents a clinical dilemma for surgeons.
  • The enigmatic biology and anatomical location of abdominal desmoids make treatment recommendations difficult.
  • [MeSH-major] Fibromatosis, Abdominal / therapy. Neoplasm Recurrence, Local

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  • (PMID = 18023087.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] China
  • [Number-of-references] 50
  • [Other-IDs] NLM/ PMC4250878
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52. Ozger H, Eralp L, Toker B, Ağaoğlu F, Dizdar Y: [Evaluation of prognostic factors affecting recurrences and disease-free survival in extra-abdominal desmoid tumors]. Acta Orthop Traumatol Turc; 2007 Aug-Oct;41(4):291-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Evaluation of prognostic factors affecting recurrences and disease-free survival in extra-abdominal desmoid tumors].
  • [Transliterated title] Ekstra-abdominal desmoid tümörlerde nüks ve hastaliksiz sağkalimi etkileyen prognostik faktörlerin değerlendirilmesi.
  • OBJECTIVES: We investigated treatment results and the role of potential prognostic factors in patients treated by surgery with or without adjuvant radiotherapy for primary or recurrent extra-abdominal desmoid tumors.
  • METHODS: The study included 38 patients (23 females, 15 males; mean age 24 years; range 5 to 61 years) who underwent surgical treatment for extra-abdominal desmoid tumors.
  • The mean disease-free survival was 38+/-8 months, and eight-year disease-free survival was 35.7+/-8.5%.
  • Disease-free survival did not differ significantly between patients receiving adjuvant radiotherapy (47.9+/-7.9 months) and those treated with surgery alone (37.9+/-12.4 months), and between patients who developed a recurrence at the resection site (12.1+/-4.7 months) or at a different site (24.3+/-1.0 months) (p>0.05).
  • None of the potential prognostic factors including gender, age, localization, surgical margin, or adjuvant irradiation were found to affect disease-free survival.
  • [MeSH-major] Fibromatosis, Aggressive / surgery. Neoplasm Recurrence, Local / surgery. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Axilla. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Extremities. Female. Humans. Male. Middle Aged. Prognosis. Turkey / epidemiology

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  • (PMID = 18180559.001).
  • [ISSN] 1017-995X
  • [Journal-full-title] Acta orthopaedica et traumatologica turcica
  • [ISO-abbreviation] Acta Orthop Traumatol Turc
  • [Language] tur
  • [Publication-type] English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Turkey
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53. Kourda N, Ben Slama S, Mrabet N, Sayari S, Zouache A, Ben Jilani SB, Zermani R: [Abdominal desmoid tumor: pathologic and therapeutic concepts]. Tunis Med; 2008 Oct;86(10):916-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Abdominal desmoid tumor: pathologic and therapeutic concepts].
  • [Transliterated title] Tumeur desmoide abdominale: concepts histopathologiques et therapeutiques.
  • BACKGROUND: Abdominal desmoid tumors arise in young adults between 20 and 40 years.
  • METHODS: From 1990 to 2004, three cases of abdominal desmoid tumors were diagnosed in the Pathologic laboratory of the Charles Nicole Hospital of Tunis.
  • CONCLUSION: Abdominal desmoid tumors are locally aggressive but never give metastasis.
  • [MeSH-major] Abdominal Neoplasms / pathology. Abdominal Neoplasms / therapy. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy

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  • (PMID = 19472812.001).
  • [ISSN] 0041-4131
  • [Journal-full-title] La Tunisie médicale
  • [ISO-abbreviation] Tunis Med
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Tunisia
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54. Kreuzberg B, Koudelova J, Ferda J, Treska V, Spidlen V, Mukensnabl P: Diagnostic problems of abdominal desmoid tumors in various locations. Eur J Radiol; 2007 May;62(2):180-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnostic problems of abdominal desmoid tumors in various locations.
  • BACKGROUND: Abdominal desmoid tumor is one of the forms of deep (musculoaponeurotic) fibromatosis.
  • It occurs more often as a desmoid tumor in the abdominal wall, less often in various intra-abdominal locations.
  • In this work, we performed retrospective study concerning diagnostic problems of this disease with the use of imaging techniques.
  • RESULTS: The findings were typical only in the case with the lesion located in the abdominal wall, three described cases of the intra-abdominal desmoid provided a broad range of differentially diagnostic possibilities (metastases, GIT tumors, lymphomas, etc.).
  • In particular, the findings in infiltrative processes in intra-abdominal location and retroperitoneal involvement were less typical.
  • CONCLUSION: The possibility of the occurrence of intra-abdominal desmoid tumor must be considered particularly in younger individuals with rapidly growing tumorous process, which does not immediately arise from the surrounding organs (digestive tract, internal genitalia, etc.
  • ), and is located in the abdominal wall or in the abdominal cavity.
  • [MeSH-major] Fibromatosis, Abdominal / diagnosis. Fibromatosis, Aggressive / diagnosis
  • [MeSH-minor] Abdominal Cavity / pathology. Abdominal Wall / pathology. Adult. Biopsy, Fine-Needle. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed

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  • (PMID = 17321093.001).
  • [ISSN] 0720-048X
  • [Journal-full-title] European journal of radiology
  • [ISO-abbreviation] Eur J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
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55. Lahat G, Nachmany I, Itzkowitz E, Abu-Abeid S, Barazovsky E, Merimsky O, Klauzner J: Surgery for sporadic abdominal desmoid tumor: is low/no recurrence an achievable goal? Isr Med Assoc J; 2009 Jul;11(7):398-402
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgery for sporadic abdominal desmoid tumor: is low/no recurrence an achievable goal?
  • BACKGROUND: Sporadic abdominal desmoid tumors are rare and data on these tumors as a distinct disease entity are lacking.
  • Previous abdominal surgery, trauma, pregnancy and estrogen intake are considered risk factors.
  • OBJECTIVES: To evaluate outcomes of surgery for this rare disease.
  • METHODS: Since 1995, 16 patients with pathologically confirmed desmoid tumor were operated on in our center.
  • All patients presented with an isolated mass; 7 (50%) originated in the abdominal wall, 6 (37.5%) were retroperitoneal and 3 were (18.8%) mesenteric.
  • CONCLUSIONS: The perception of sporadic abdominal desmoids as tumors with a high recurrence rate (20-70%) is probably incorrect.
  • [MeSH-major] Abdominal Neoplasms / surgery. Fibromatosis, Aggressive / surgery

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  • (PMID = 19911489.001).
  • [ISSN] 1565-1088
  • [Journal-full-title] The Israel Medical Association journal : IMAJ
  • [ISO-abbreviation] Isr. Med. Assoc. J.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Israel
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56. Pho LN, Coffin CM, Burt RW: Abdominal desmoid in familial adenomatous polyposis presenting as a pancreatic cystic lesion. Fam Cancer; 2005;4(2):135-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Abdominal desmoid in familial adenomatous polyposis presenting as a pancreatic cystic lesion.
  • An abdominal CT scan detected a cystic pancreatic lesion of unknown etiology.
  • Histopathological examination of the resected specimen showed a benign pancreatic cyst and fibrous plaque with desmoid fibromatosis adherent to the surface of the pancreas, serosa of the stomach, and colon.
  • The fibrous plaque was histologically identical to the fibrous mesenteric plaque known to occur in FAP and associated mesenteric fibromatosis.
  • We present pathologic evidence that the pancreatic cyst formation was induced by FAP-associated desmoid invasion.
  • Desmoid growth should be considered in the differential diagnosis of a pancreatic cystic mass lesion in patients with FAP or its Gardner syndrome variant.
  • This case report provides the first pathologic evidence for benign epithelial cyst formation in the pancreas caused by fibromatosis invasion of that organ as a part of FAP.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Adenomatous Polyposis Coli / pathology. Fibromatosis, Aggressive / complications. Fibromatosis, Aggressive / diagnosis. Pancreatic Cyst / diagnosis. Pancreatic Cyst / etiology
  • [MeSH-minor] Adolescent. Chest Pain. Diagnosis, Differential. Humans. Male. Weight Loss

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  • (PMID = 15951964.001).
  • [ISSN] 1389-9600
  • [Journal-full-title] Familial cancer
  • [ISO-abbreviation] Fam. Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01 CA 73992; United States / NCI NIH HHS / CA / P30 CA 42014; United States / NCI NIH HHS / CA / R01 CA 40641
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] Netherlands
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57. Speake D, Evans DG, Lalloo F, Scott NA, Hill J: Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations. Br J Surg; 2007 Aug;94(8):1009-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Desmoid tumours in patients with familial adenomatous polyposis and desmoid region adenomatous polyposis coli mutations.
  • BACKGROUND: :The aim of this study was to determine the proportion of patients with familial adenomatous polyposis (FAP) who had mutations in the desmoid region of the adenomatous polyposis coli (APC) gene that phenotypically expresses desmoid disease, and to determine the role for surgery in these patients.
  • RESULTS: Of 363 patients with FAP, 47 from ten families had APC mutations in the desmoid region 3' to codon 1399.
  • Of 22 patients undergoing surgery, 16 developed desmoids, and of these 12 had mesenteric desmoid disease.
  • Complications from mesenteric desmoids were death (two patients), enterectomy (three), local resection (three), fistula (one), cholangitis and local resection (one), bowel obstruction (one) and bowel and ureteric obstruction (one).
  • CONCLUSION: In individuals with 3' APC mutations, abdominal surgery is associated with a 65 per cent risk of developing mesenteric desmoids.
  • [MeSH-major] Adenomatous Polyposis Coli / genetics. Fibromatosis, Aggressive / genetics. Genes, APC. Mesentery. Mutation / genetics. Peritoneal Neoplasms / genetics

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  • [Copyright] Copyright (c) 2007 British Journal of Surgery Society Ltd.
  • (PMID = 17410559.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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58. Rosato L, Mondini G, Serbelloni M, Bertone P, Orlassino R, Cossavella D: [Intra-abdominal desmoid tumors: rare but important disease]. G Chir; 2007 Jan-Feb;28(1-2):20-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Intra-abdominal desmoid tumors: rare but important disease].
  • Desmoid tumors are rare benign neoplasms with high tendency to local recurrence, and they can be divided into extra- and intra-abdominal types (mesenteric fibromatosis).
  • Six patients (3 men and 3 women) affected by extra-abdominal desmoid tumors have been treated with radical excision.
  • In two patients desmoid was intra-abdominal:.
  • 1) a 55 years old man admitted for acute abdomen and submitted, in emergency, to a laparotomy with excision of a mesenteric abscess including a jejunal loop at about one meter from Treitz;.
  • 2) 52 years old man, submitted to an elective excision of a capsulated neoplasm of the little omentum, which had caused an oppressive abdominal pain.
  • In both cases the hystological diagnosis has been desmoid tumor.
  • Surgical treatment of desmoid tumors must aim at radical excision to avoid frequent recurrences (25-65%); these have stimulated the research of other kinds of treatments, since a new surgical operation itself can lead to a further recurrence.
  • Radiotherapy has been investigated with results in 79-96% of cases, antiestrogenic therapy has been used with success in 51% of patients, and high dose tamoxifen seemed to obtain a stable disease in non operable cases.
  • Conclusive results on the efficacy of these treatments have not been obtained yet, because of the rarity of the desmoid tumors even in greater Centres.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Mesentery. Peritoneal Neoplasms / surgery
  • [MeSH-minor] Abdominal Neoplasms / pathology. Abdominal Neoplasms / surgery. Aged. Female. Fibromatosis, Aggressive / surgery. Humans. Male. Middle Aged. Retrospective Studies. Treatment Outcome

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  • (PMID = 17313728.001).
  • [ISSN] 0391-9005
  • [Journal-full-title] Il Giornale di chirurgia
  • [ISO-abbreviation] G Chir
  • [Language] ita
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Italy
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59. Jain P, Shah P, Bhansali M: Unusual presentation of an uncommon abdominal pathology. Ann R Coll Surg Engl; 2010 Oct;92(7):W19-21

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unusual presentation of an uncommon abdominal pathology.
  • Desmoid tumour is a non-encapsulated locally invasive tumour, originating from fibroblasts, which has ubiquitous distribution in the body.
  • A case of a 75-year-old man, presenting with high-grade fever, dull aching abdominal pain and weight loss, suspected to be due to malignancy, is reported.
  • Histological analysis revealed mesenteric fibromatosis (desmoid tumour).
  • To the best of our knowledge, this is the first case on colonic perforation secondary to desmoid tumour.
  • [MeSH-major] Colonic Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis
  • [MeSH-minor] Abdomen, Acute / etiology. Aged. Humans. Intestinal Perforation / etiology. Intestinal Perforation / surgery. Male. Tomography, X-Ray Computed

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  • (PMID = 20825702.001).
  • [ISSN] 1478-7083
  • [Journal-full-title] Annals of the Royal College of Surgeons of England
  • [ISO-abbreviation] Ann R Coll Surg Engl
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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60. Viriyaroj V, Yingsakmongkol N, Pasukdee P, Rermluk N: A large abdominal desmoid tumor associated with pregnancy. J Med Assoc Thai; 2009 Jun;92 Suppl 3:S72-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A large abdominal desmoid tumor associated with pregnancy.
  • Desmoid tumors are rare, benign tumors with locally aggressive behavior which originate from fascial or musculoaponeurotic structure.
  • The etiology of desmoid tumors are uncertain but may be related to operation, trauma or hormonal factors.
  • The authors report a 17-year old woman, gravida 1, para 1 with a mass at her lower abdominal wall during the fifth month of gestation.
  • She was biopsied during delivery in another hospital but was not given a definite diagnosis.
  • The tumor measured 28 x 21 x 18 centimeters in size and 4,900 g in weight.
  • Complete surgical excision was performed The pathological report was desmoid tumor (aggressive fibromatosis).
  • She had no post-operative complication and no recurrent tumor in the 8 months after operation.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Pregnancy Complications

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  • (PMID = 19702071.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Thailand
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61. Stengel G, Metze D, Dörflinger B, Luger TA, Böhm M: Treatment of extra-abdominal aggressive fibromatosis with pegylated interferon. J Am Acad Dermatol; 2008 Aug;59(2 Suppl 1):S7-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of extra-abdominal aggressive fibromatosis with pegylated interferon.
  • Aggressive fibromatosis (desmoid tumor) is a very rare neoplasm arising from the musculoaponeurotic structures.
  • We report on a young woman with a large desmoid tumor of the left foot.
  • Long-term immunointervention with pegylated interferon alfa-2b, however, led to marked clinical improvement of the patient's condition and a radiologically proven stabilization of the disease.
  • [MeSH-major] Antineoplastic Agents / administration & dosage. Fibromatosis, Aggressive / drug therapy. Foot. Interferon-alpha / administration & dosage. Soft Tissue Neoplasms / drug therapy

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  • (PMID = 18625396.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Interferon-alpha; 0 / Recombinant Proteins; 0 / peginterferon alfa-2b; 30IQX730WE / Polyethylene Glycols; 99210-65-8 / interferon alfa-2b
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62. Yang JL, Wang J, Zhou XY, Li XQ, Hou YY, Zhu XZ: [Clinicopathologic and genetic studies of desmoid-type fibromatosis]. Zhonghua Bing Li Xue Za Zhi; 2006 Mar;35(3):145-50
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  • [Title] [Clinicopathologic and genetic studies of desmoid-type fibromatosis].
  • OBJECTIVE: To study the clinicopathological and genetic features of desmoid-type fibromatosis, and to investigate the feasibility of detecting trisomy 8 in formalin fixed, paraffin embedded (FFPE) tissue by fluorescence in-situ hybridization (FISH).
  • Histopathologic and immunohistochemical evaluations were available in 69 cases, and ultrastructural evaluation was done in 2 cases of desmoid-type fibromatosis.
  • Clinically, there were 44 extra-abdominal tumors, 28 abdominal wall tumors and 23 intra-abdominal lesions mostly involving the mesentery.
  • Histologically, desmoid-type fibromatoses showed longitudinal fascicles of spindle fibroblasts and myofibroblasts in a predominantly collagenous background.
  • The tumor cells stained positive for vimentin, alpha-smooth muscle actin, desmin, and beta-catenin (47.8%, 33/69).
  • Ultrastructurally, most tumor cells had features of fibroblasts, including rich endoplasmic reticulum and Golgi apparatus.
  • Some tumor cells were myofibroblast-like cells exhibiting intercellular junctions, fibronexous junctions and stress fibers.
  • Trisomy 8 was detected in 6 of 20 cases of desmoid-type fibromatosis including 5 of the 8 recurrent tumors but only one of the 12 primary tumors.
  • The latter tumor also recurred three years later.
  • CONCLUSIONS: Desmoid-type fibromatosis is an intermediate (locally aggressive) tumor that occurs predominantly in young females.
  • Trisomy 8 can be detected in FFPE tissue by FISH, and its presence serves as a useful predictor of tumor recurrence and may define a subtype of desmoid-type fibromatosis with high recurrence rate.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology. Peritoneal Neoplasms / pathology. Trisomy

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  • (PMID = 16630502.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Actins; 0 / Desmin; 0 / Vimentin; 0 / beta Catenin
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63. Seinfeld J, Kleinschmidt-DeMasters BK, Tayal S, Lillehei KO: Desmoid-type fibromatosis involving the brachial plexus. Neurosurg Focus; 2007;22(6):E22
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  • [Title] Desmoid-type fibromatosis involving the brachial plexus.
  • Desmoid-type fibromatosis involving the brachial plexus is a rare and challenging disease.
  • The authors describe their experience in four surgically treated patients with desmoid-type fibromatosis involving the brachial plexus and review the relevant neurosurgical literature.
  • Three patients experienced tumor recurrence requiring reoperation.
  • Fractionated radiotherapy achieved local control in three patients, and the disease in one patient progressed beyond the treatment field.
  • One tumor with this mutation did not respond to treatment with imatimib mesylate.
  • Analysis of these cases emphasizes the need for careful resection in patients with desmoid-type fibromatosis and supports the conclusion that without adjuvant radiotherapy a high local recurrence rate can be anticipated.
  • For optimal local disease control, the authors recommend postsurgical radiation therapy regardless of the extent of resection achieved.
  • [MeSH-major] Brachial Plexus / pathology. Brachial Plexus / surgery. Fibromatosis, Abdominal / radiotherapy. Fibromatosis, Abdominal / surgery
  • [MeSH-minor] Adult. Aged. Female. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / radiotherapy. Fibromatosis, Aggressive / surgery. Humans. Male. Middle Aged. Retrospective Studies. Treatment Outcome

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  • (PMID = 17613214.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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64. Ramos-Font C, Santiago Chinchilla A, Rebollo Aguirre AC, Rodríguez Fernández A, Medina Benítez A, Llamas Elvira JM: [Desmoid tumor of the thoraco-abdominal wall characterized with 18F-fluorodeoxyglucose PET/ CT scan. Correlation with magnetic resonance and bone scintigraphy. Review of the literature]. Rev Esp Med Nucl; 2009 Mar-Apr;28(2):70-3
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  • [Title] [Desmoid tumor of the thoraco-abdominal wall characterized with 18F-fluorodeoxyglucose PET/ CT scan. Correlation with magnetic resonance and bone scintigraphy. Review of the literature].
  • [Transliterated title] Tumor desmoide de la pared torácico-abdominal. Caracterización con PET-TAC con 18F-fluorodesoxiglucosa y correlación con resonancia magnética y gammagrafía ósea. Revisión de la literatura.
  • Desmoid tumours are uncommon benign tumours but with aggressive behaviour, resulting from the proliferation of well-differentiated fibroblasts.
  • When planning treatment, it is important to assess the correct tumour extent to permit curative radical surgery minimizing possible local recurrence.
  • We present the case of a patient with a desmoid tumour of the abdominal-chest wall and we review the related literature.
  • [MeSH-major] Abdominal Neoplasms / radionuclide imaging. Abdominal Wall / radionuclide imaging. Bone Neoplasms / radionuclide imaging. Fibromatosis, Aggressive / radionuclide imaging. Fluorine Radioisotopes. Fluorodeoxyglucose F18. Muscle Neoplasms / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals. Rectus Abdominis / radionuclide imaging. Ribs / radionuclide imaging. Tomography, X-Ray Computed

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  • (PMID = 19406052.001).
  • [ISSN] 0212-6982
  • [Journal-full-title] Revista española de medicina nuclear
  • [ISO-abbreviation] Rev Esp Med Nucl
  • [Language] spa
  • [Publication-type] Case Reports; Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
  • [Number-of-references] 22
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65. Gouin F, Tesson A, Bertrand-Vasseur A, Cassagnau E, Rolland F: [Rating of tumoral growth in non-operated primary or recurrent extra-abdominal aggressive fibromatosis]. Rev Chir Orthop Reparatrice Appar Mot; 2007 Oct;93(6):546-54
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  • [Title] [Rating of tumoral growth in non-operated primary or recurrent extra-abdominal aggressive fibromatosis].
  • [Transliterated title] Etude de la croissance tumorale des tumeurs desmoïdes extra-abdominales (FDEA) primitives ou récidivées non opérées.
  • PURPOSE OF THE STUDY: Extra-abdominal aggressive fibromatosis (EAAF) is a benign desmoid tumor with a potentially aggressive behavior.
  • For eight, the tumor was a recurrence after surgical removal.
  • MRI showed progression for two tumors (12%) but with a short follow-up since diagnosis (9 and 14 months), in one case despite medical treatment.
  • On average the tumor growth lasted ten months.
  • DISCUSSION: Tumor growth was never noted beyond 36 months.
  • This notion of an interruption in tumor growth is mentioned sporadically in reports on EAAF, which have generally included recurrent tumors.
  • The high rate of spontaneous interruption of tumor growth must be counterbalanced with the difficult task of local treatment: the risk of recurrence is particularly high after surgery and functional sequelae can be significant when wide resection is proposed in an anatomically difficult localization.
  • [MeSH-major] Fibromatosis, Aggressive / physiopathology. Neoplasm Recurrence, Local / physiopathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biopsy. Chemotherapy, Adjuvant. Cohort Studies. Disease Progression. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Invasiveness. Palliative Care. Prospective Studies. Radiotherapy, Adjuvant. Remission Induction. Treatment Outcome

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  • (PMID = 18065863.001).
  • [ISSN] 0035-1040
  • [Journal-full-title] Revue de chirurgie orthopédique et réparatrice de l'appareil moteur
  • [ISO-abbreviation] Rev Chir Orthop Reparatrice Appar Mot
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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66. Gursoy A, Cesur M, Aktaş B, Utkan G, Gedik VT, Erdogan M, Kamel N: Intracranial aggressive fibromatosis presenting as panhypopituitarism and diabetes insipidus. Pituitary; 2005;8(2):123-6
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  • [Title] Intracranial aggressive fibromatosis presenting as panhypopituitarism and diabetes insipidus.
  • Aggressive fibromatosis (AF) is a rare, locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often, of the limbs and trunk.
  • The tumor was markedly isointense on both T2- and T1-weighted images relative to gray matter, and enhanced strongly after administration of gadolinium.
  • No other sites were found to be involved by thorax and abdominal tomography.
  • On the basis of reports that radiotherapy is an effective treatment for this kind of tumor, we administered radiation to the affected area, since chemotherapy and hormonal treatment of non-resectable tumors are not satisfactory.
  • [MeSH-major] Diabetes Insipidus / diagnosis. Fibromatosis, Aggressive / diagnosis. Hypopituitarism / diagnosis. Skull Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male


67. Ketelsen D, Hartmann J, Horger M: [MR-imaging of aggressive fibromatosis]. Rofo; 2008 Feb;180(2):83-6
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  • [Title] [MR-imaging of aggressive fibromatosis].
  • [Transliterated title] MR-Bildgebung der Aggressiven Fibromatose.
  • Aggressive fibromatosis is a rare mesenchymal benign tumor characterized histologically by proliferation of fibroblasts and myofibroblasts with marked production of intercellular collagen.
  • Predominant low signal intensity on all pulse sequences is said to be characteristic of, but not specific for, aggressive fibromatosis.
  • The principal role of imaging in the management of aggressive fibromatosis is preoperative planning an the detection of recurrence or disease progression in nonsurgically managed patients.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Fibromatosis, Aggressive / diagnosis. Image Enhancement / methods. Magnetic Resonance Imaging / methods

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  • (PMID = 18224580.001).
  • [ISSN] 1438-9029
  • [Journal-full-title] RöFo : Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
  • [ISO-abbreviation] Rofo
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 5
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68. Skubitz KM, Manivel JC, Clohisy DR, Frolich JW: Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors. Cancer Chemother Pharmacol; 2009 Aug;64(3):635-40
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  • [Title] Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors.
  • PURPOSE: Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype.
  • METHODS: We report a case of an aggressive multicentric extra-abdominal AF that was responsive to sunitinib, but resistant to imatinib.
  • [MeSH-major] Fibromatosis, Aggressive / drug therapy. Indoles / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Pyrroles / therapeutic use

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  • (PMID = 19404642.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Benzamides; 0 / Indoles; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 0 / Pyrroles; 0 / sunitinib; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 40
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69. Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E: Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol; 2006 Jan;186(1):247-54
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  • [Title] Aggressive fibromatosis: MRI features with pathologic correlation.
  • OBJECTIVE: We present the MRI features with pathologic correlation of aggressive fibromatosis, incorporating 203 cases over a 5-year period from the Royal Marsden Hospital Sarcoma Unit database.
  • MATERIALS AND METHODS: Sixty patients had imaging available for retrospective review of which 29 had preoperative MRI and final histopathologic diagnosis of aggressive fibromatosis.
  • RESULTS: The average age at diagnosis was 41.3 years with a female-to-male sex ratio of 1.2:1.
  • Twenty lesions were extraabdominal; six, intraabdominal; and three, in the abdominal wall (classic desmoid).
  • The average tumor size was 6.4 cm (range, 2.2-13.7 cm).
  • Intraabdominal aggressive fibromatosis produced the largest tumors, averaging 9.5 cm.
  • The lesions crossed major fascial boundaries in 31% of cases overall and in 66% of patients referred for recurrent disease.
  • Patients referred for recurrent disease were most likely to have a recurrence after surgery.
  • CONCLUSION: Accurate diagnosis and staging of aggressive fibromatosis by MRI have important treatment and prognostic implications.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Magnetic Resonance Imaging / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Female. Fibromatosis, Abdominal / pathology. Humans. Male. Middle Aged. Prognosis. Recurrence

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  • (PMID = 16357411.001).
  • [ISSN] 0361-803X
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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70. Guney Y, Hiçsönmez A, Andrieu MN, Kurtman C: Outcome of aggressive fibromatosis treated with radiation therapy. Scott Med J; 2007 Nov;52(4):11-4
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  • [Title] Outcome of aggressive fibromatosis treated with radiation therapy.
  • INTRODUCTION: The purpose of this study is to report the clinical course and outcome in 7 patients with aggressive fibromatosis.
  • Patients' demographic information, including age and gender, tumour characteristics, surgical resection, and the use of radiotherapy were recorded and evaluated.
  • At follow-up, three patients had no evidence of disease, three patients were alive with disease, and one patient died 15 days after radiotherapy.
  • CONCLUSION: Local control is the primary problem in aggressive fibromatosis.
  • There is no appropriate treatment for aggressive fibromatosis and the type of treatment depends on tumour characteristics and location as well as patient characteristics.
  • [MeSH-major] Fibromatosis, Abdominal / radiotherapy. Fibromatosis, Aggressive / radiotherapy

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  • (PMID = 18092630.001).
  • [ISSN] 0036-9330
  • [Journal-full-title] Scottish medical journal
  • [ISO-abbreviation] Scott Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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71. Levy AD, Rimola J, Mehrotra AK, Sobin LH: From the archives of the AFIP: benign fibrous tumors and tumorlike lesions of the mesentery: radiologic-pathologic correlation. Radiographics; 2006 Jan-Feb;26(1):245-64
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  • Mesenteric fibromatosis, sclerosing mesenteritis, inflammatory pseudotumor, and extrapleural solitary fibrous tumor constitute a loosely associated group of benign fibrous tumors and tumorlike lesions of the mesentery.
  • Mesenteric fibromatosis is a locally aggressive, benign proliferative process that may occur sporadically or in association with familial adenomatous polyposis.
  • It most frequently manifests as a focal mesenteric mass and may simulate lymphoma, metastatic disease, or a soft-tissue sarcoma.
  • Sclerosing mesenteritis is a rare idiopathic disorder that most commonly produces a stellate mass within the mesentery and should be differentiated from metastatic disease, specifically metastatic carcinoid, because it frequently responds to conservative or medical management.
  • Inflammatory pseudotumor (inflammatory myofibroblastic tumor) is a benign, chronic inflammatory disorder of unknown cause that manifests as a solid mesenteric mass, indistinguishable from malignancy.
  • Extrapleural solitary fibrous tumor is a tumor of submesothelial origin that is identical to the solitary fibrous tumor of the pleura.
  • When located in the mesentery or peritoneal cavity, extrapleural solitary fibrous tumor has an imaging pattern that must be differentiated from metastatic disease, soft-tissue sarcomas, and other benign and malignant neoplasms of the mesentery and peritoneum.
  • Knowledge of this group of benign fibrous tumors and tumorlike lesions of the mesentery is important in the preoperative evaluation of a mesenteric mass.
  • [MeSH-major] Mesentery. Peritoneal Diseases / diagnosis. Peritoneal Neoplasms / diagnosis
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Child. Female. Fibroma / diagnosis. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Panniculitis, Peritoneal / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 16418255.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 57
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72. Barbier O, Anract P, Pluot E, Larouserie F, Sailhan F, Babinet A, Tomeno B: Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. Orthop Traumatol Surg Res; 2010 Dec;96(8):884-9
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  • [Title] Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only.
  • INTRODUCTION: Extra-abdominal desmoid fibromatosis (EADF) is a benign tumoral condition, classically managed by more or less radical and sometimes mutilating excision.
  • One primary tumor showed spontaneous regression, and one recurrence still showed evolution at end of follow-up (23 months).
  • [MeSH-major] Fibroma / therapy. Fibromatosis, Aggressive / therapy. Neoplasm Recurrence, Local / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Disease Progression. Female. Humans. Kaplan-Meier Estimate. Magnetic Resonance Imaging. Male. Middle Aged. Observation. Retrospective Studies. Young Adult

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  • [Copyright] Copyright © 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 21075698.001).
  • [ISSN] 1877-0568
  • [Journal-full-title] Orthopaedics & traumatology, surgery & research : OTSR
  • [ISO-abbreviation] Orthop Traumatol Surg Res
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] France
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73. Ghidirim G, Mishin I, Gagauz I, Vozian M, Zastavnitsky G, Iakovleva I: Sporadic retroperitoneal aggressive fibromatosis: report of a case. Zentralbl Chir; 2010 Feb;135(1):79-82
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  • [Title] Sporadic retroperitoneal aggressive fibromatosis: report of a case.
  • We describe herein a case of sporadic retroperitoneal aggressive fibromatosis.
  • A 54-year-old man with a palpable abdominal mass was referred to our hospital for investigation and treatment.
  • Abdominal ultrasonography and computed tomography revealed a solid mass with relatively well-defined borders in the left abdominal retroperitoneum.
  • At surgery, a large tumor (14 x 13 x 11 cm) was found, arising from the retroperitoneal space and involving the wall of jejunum.
  • Histological examination of the resected specimen revealed the presence of changes consistent with aggressive fibromatosis.
  • Two years after surgery, the patient is without any signs of recurrent disease.
  • Although extremely rare, aggressive fibromatosis should be considered in the differential diagnosis of retroperitoneal masses.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Fibromatosis, Aggressive / surgery. Retroperitoneal Neoplasms / surgery
  • [MeSH-minor] Diagnosis, Differential. Follow-Up Studies. Humans. Male. Middle Aged. Tomography, X-Ray Computed

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  • [Copyright] Georg Thieme Verlag Stuttgart, New York.
  • (PMID = 19424942.001).
  • [ISSN] 1438-9592
  • [Journal-full-title] Zentralblatt für Chirurgie
  • [ISO-abbreviation] Zentralbl Chir
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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74. Ferenc T, Stalińska L, Turant M, Sygut J, Tosik D, Dziki A, Kulig A: Analysis of TGF-beta protein expression in aggressive fibromatosis (desmoid tumor). Pol J Pathol; 2006;57(2):77-81
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  • [Title] Analysis of TGF-beta protein expression in aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis, usually termed desmoid tumor, develops from muscle connective tissue, fasciae and aponeuroses.
  • Aggressive fibromatosis located in various parts of the body demonstrates differentiated biological behavior.
  • Abnormalities in TGF-beta expression are very common in many disease processes, including neoplasms.
  • Immunohistochemical analysis employing a monoclonal antibody against TGF-beta was performed on archival material, consisting of 38 cases of aggressive fibromatosis, among which 23 represented abdominal, 11 extra-abdominal and 4 intra-abdominal localizations.
  • The average percentage of cells positively stained for TGF-beta protein was 40.2% in the group of extra-abdominal, 58.5% in the group of abdominal and 72.8% in the group of intra-abdominal localizations.
  • There were significant differences observed between the analyzed groups of desmoid tumor (p<0.05).
  • A positive cytoplasmic reaction for TGF-beta was noted in 65.8% (25/38) of the aggressive fibromatoses.
  • Overexpression of TGF-beta protein was noted in 39.5% (15/38) of the aggressive fibromatoses.
  • High expression noticed in desmoid fibroblasts might indicate that this protein plays a crucial role in the development of aggressive fibromatosis.
  • [MeSH-major] Abdominal Neoplasms / metabolism. Fibromatosis, Aggressive / metabolism. Transforming Growth Factor beta / metabolism
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Cell Count. Cytoplasm / metabolism. Cytoplasm / pathology. Fibroblasts / metabolism. Fibroblasts / pathology. Humans. Image Processing, Computer-Assisted. Immunoenzyme Techniques

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  • (PMID = 17019969.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Transforming Growth Factor beta
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75. Durkin AJ, Korkolis DP, Al-Saif O, Zervos EE: Full-term gestation and transvaginal delivery after wide resection of an abdominal desmoid tumor during pregnancy. J Surg Oncol; 2005 Feb 1;89(2):86-90
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  • [Title] Full-term gestation and transvaginal delivery after wide resection of an abdominal desmoid tumor during pregnancy.
  • A 29-year-old female was diagnosed with a symptomatic, extra-abdominal desmoid tumor during the first trimester of pregnancy.
  • Preoperative diagnosis was confirmed by core-needle biopsy of the lesion.
  • At 20-weeks gestation, wide local resection of the tumor with disease-free margins, as well as abdominal wall reconstruction with polytetrafluoroethylene (PTFE) mesh was successfully undertaken.
  • Histological examination of the tumor ex vivo confirmed that the lesion was a desmoid tumor consisting of spindle cells with dense infiltrating collagenous fibers.
  • This case illustrates the probable contribution of estrogens towards desmoid tumor development, the durability of abdominal wall reconstruction when subjected to the extraordinary strain of both a gravid uterus and labor, as well as the safety and efficacy of aggressive surgical therapy during pregnancy.
  • [MeSH-major] Abdominal Wall / surgery. Delivery, Obstetric. Fibromatosis, Abdominal / surgery. Pregnancy Complications, Neoplastic / surgery


76. Kouchi M, Okimoto K, Matsumoto I, Michimae Y, Yamada T, Inoue T, Kimura T, Seki T, Yasuba M, Hino O: Postoperative fibromatosis-type fibromas in the Bhd gene mutant (Nihon) rat. Exp Toxicol Pathol; 2008 Mar;59(5):273-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Postoperative fibromatosis-type fibromas in the Bhd gene mutant (Nihon) rat.
  • Fibromatosis-type fibromas were found to develop at abdominal surgical sites in 4 heterozygous Nihon rats, a model for the human Birt-Hogg-Dubé syndrome.
  • In all 4 rats, solitary and firm nodules were located within the lateral abdominal musculature involving the full thickness of the abdominal wall at the sites of laparotomy.
  • These cells were surrounded by large amounts of collagen fibers, and appeared to infiltrate within the abdominal musculature.
  • These characteristics are consistent with desmoid tumors in human.
  • Although the etiology of desmoid tumors in human remains unclear, they are known to occur in association with hormonal factors, surgical trauma, and familial adenomatous polyposis.
  • Genetic factor should be involved in the occurrence of the tumor, since it was found only in the Nihon rats among many rats.
  • [MeSH-minor] Animals. Disease Models, Animal. Mutation. Rats. Rats, Mutant Strains

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  • (PMID = 18036795.001).
  • [ISSN] 0940-2993
  • [Journal-full-title] Experimental and toxicologic pathology : official journal of the Gesellschaft für Toxikologische Pathologie
  • [ISO-abbreviation] Exp. Toxicol. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Flcn protein, rat; 0 / Proteins
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77. Meazza C, Bisogno G, Gronchi A, Fiore M, Cecchetto G, Alaggio R, Milano GM, Casanova M, Carli M, Ferrari A: Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer; 2010 Jan 1;116(1):233-40
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  • [Title] Aggressive fibromatosis in children and adolescents: the Italian experience.
  • BACKGROUND: Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence.
  • METHODS: The authors retrospectively analyzed 94 patients aged < or =21 years, including 23 patients who underwent complete surgery (Group I), 42 patients who underwent incomplete surgery with microscopic residual tumor (Group II), and 29 patients who underwent either biopsy or macroscopically incomplete surgery (Group III).
  • Two of 7 patients with abdominal disease died of tumor progression, whereas none of the patients with extra-abdominal AF died of their disease.
  • Systemic treatment was given to 15 patients as first-line treatment and to 34 patients at time the time they developed recurrent disease: The response rate was 47% in the former patients and 50% in the latter patients.
  • The completeness of initial resection was the main factor that influenced EFS, whereas disease control after marginal resection was much the same as that achieved after intralesional surgery/biopsy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Fibromatosis, Aggressive / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Male. Methotrexate / administration & dosage. Neoplasm Recurrence, Local. Prognosis. Survival Rate. Vinblastine / administration & dosage. Vinblastine / analogs & derivatives

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  • [Copyright] Copyright 2010 American Cancer Society.
  • (PMID = 19950127.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5V9KLZ54CY / Vinblastine; Q6C979R91Y / vinorelbine; YL5FZ2Y5U1 / Methotrexate
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78. Stalinska L, Turant M, Tosik D, Sygut J, Kulig A, Kopczynski J, Dziki A, Ferenc T: Analysis of pRb, p16INK4A proteins and proliferating antigens: PCNA, Ki-67 and MCM5 expression in aggressive fibromatosis (desmoid tumor). Histol Histopathol; 2009 03;24(3):299-308
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  • [Title] Analysis of pRb, p16INK4A proteins and proliferating antigens: PCNA, Ki-67 and MCM5 expression in aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis (desmoid tumor) is a mesenchymal lesion originating from fascial, aponeurotic and muscular connective tissue.
  • In this study we analyzed the cell cycle regulation proteins: pRb, p16, and proliferating antigens: Ki-67, PCNA, MCM5 with immunohistochemical method in archival material derived from 27 extra-abdominal (E-AD), 18 abdominal (AD) and 5 intra-abdominal (I-AD) cases of desmoid tumor.
  • None of the examined cases (n=50) of aggressive fibromatosis was pRb-immunonegative.
  • The noted levels of pRb and p16 expression in desmoid cells reflect their function in cell cycle regulation.
  • Probably the unsettled cell cycle progression, especially in G1 phase, is not the cause of aggressive fibromatosis pathogenesis.
  • [MeSH-major] Cell Cycle Proteins / analysis. Cyclin-Dependent Kinase Inhibitor p16 / analysis. Fibromatosis, Abdominal / metabolism. Ki-67 Antigen / analysis. Proliferating Cell Nuclear Antigen / analysis. Retinoblastoma Protein / analysis

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  • (PMID = 19130399.001).
  • [ISSN] 1699-5848
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Ki-67 Antigen; 0 / MCM5 protein, human; 0 / Proliferating Cell Nuclear Antigen; 0 / Retinoblastoma Protein
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79. Ferenc T, Sygut J, Kopczyński J, Mayer M, Latos-Bieleńska A, Dziki A, Kulig A: Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. Pol J Pathol; 2006;57(1):5-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background.
  • Aggressive fibromatosis, usually called desmoid tumor develops from muscle connective tissue, fasciae and aponeuroses.
  • As regards the site, aggressive fibromatoses can be divided into: extra-abdominal in the area of the shoulder and pelvic girdle or chest and neck wall; abdominal in abdominal wall muscles; intra-abdominal concerning pelvis, mesentery connective tissue or retroperitoneal space.
  • Desmoid tumor is a neoplasm which rarely turns malignant and is non-metastasizing but demonstrates ability to local infiltration into tissue and is characterized by high risk of recurrence (25-65%) after surgical treatment.
  • Desmoid tumor etiology is uncertain.
  • Most sporadic cases of aggressive fibromatosis contain a somatic mutation in either the adenomatous polyposis coli (APC) or beta-catenin genes.
  • In about 10-15 per cent of patients with familial adenomatous polyposis (FAP), aggressive fibromatosis is a parenteral manifestation of this familial syndrome conditioned by APC gene mutation.
  • Abdomen injury--most frequently due to surgery is said to play an important role in the initiation of fibrous tissue proliferative process in the cases of abdominal and intra abdominal forms.
  • [MeSH-major] Fibromatosis, Aggressive. Mesoderm / pathology

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  • (PMID = 16739877.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Poland
  • [Number-of-references] 93
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80. Ferenc T, Sygut J, Tosik D, Kopczyński J, Sidor M, Góźdź S, Kulig A, Dziki A, Turant M, Stalińska L: Analysis of p27KIP1 protein and Ki-67 expression in aggressive fibromatosis (desmoid tumor). Pol J Pathol; 2006;57(4):187-91
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  • [Title] Analysis of p27KIP1 protein and Ki-67 expression in aggressive fibromatosis (desmoid tumor).
  • Aggressive fibromatosis (desmoid tumor) is an uncommon locally invasive non-metastasizing neoplasm lesion.
  • Desmoid tumor consists of fibroblasts, miofibroblasts and a significant amount of extracellular matrix. p27KIP1 (p27) protein is a member of the universal cyclin-dependent kinase inhibitor (CDKI) family that regulates progression through the cell cycle.
  • There were analysed 42 specimens of aggressive fibromatosis, in which there were 24 abdominal and 18 extra-abdominal cases.
  • There was no statistically significant difference between Ki-67 or p27 expression in abdominal and extra-abdominal location.
  • Analysis of p27 and Ki-67 expression levels might indicate that low proliferating activity of desmoid fibroblasts is connected with another mechanism than the one, in which p27 takes part.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p27 / metabolism. Fibromatosis, Abdominal / metabolism. Ki-67 Antigen / metabolism

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  • (PMID = 17285761.001).
  • [ISSN] 1233-9687
  • [Journal-full-title] Polish journal of pathology : official journal of the Polish Society of Pathologists
  • [ISO-abbreviation] Pol J Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27
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81. Huang PW, Tzen CY: Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases. Pathology; 2010 Feb;42(2):147-50
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  • [Title] Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases.
  • AIMS: To determine risk factors for recurrence of desmoid-type fibromatosis (aggressive fibromatosis).
  • METHODS: Forty-six cases of desmoid-type fibromatosis in Taiwanese patients were analysed for an association between tumour recurrence and clinical features, pathology, and the presence of p53 protein and beta-catenin on immunohistochemical staining.
  • The only factor significantly associated with tumour recurrence was positive surgical margin (p = 0.035).
  • CONCLUSIONS: A positive surgical margin is a risk factor for recurrence of desmoid-type fibromatosis.
  • [MeSH-major] Fibromatosis, Abdominal / pathology. Fibromatosis, Aggressive / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / metabolism. Female. Humans. Immunohistochemistry. Male. Neoplasm Recurrence, Local. Prognosis. Risk Factors. Tumor Suppressor Protein p53 / metabolism. beta Catenin / metabolism

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  • (PMID = 20085516.001).
  • [ISSN] 1465-3931
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CTNNB1 protein, human; 0 / Tumor Suppressor Protein p53; 0 / beta Catenin
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82. Coco DP, Hirsch MS, Hornick JL: Smoothelin is a specific marker for smooth muscle neoplasms of the gastrointestinal tract. Am J Surg Pathol; 2009 Dec;33(12):1795-801
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The purpose of this study was to determine whether immunostaining for smoothelin could help distinguish smooth muscle neoplasms from their morphologic mimics, particularly KIT-negative gastrointestinal stromal tumors (GISTs), desmin-positive GISTs, and desmoid fibromatosis.
  • A total of 150 mesenchymal neoplasms of the GI tract, abdominal cavity, and retroperitoneum were retrieved from consult and surgical pathology archives, including 54 GISTs (8 KIT-negative; 13 desmin-positive), 17 GI leiomyosarcomas (LMS), 11 GI mural leiomyomas, 13 leiomyomas of the muscularis mucosae, 12 gastric schwannomas, 15 inflammatory myofibroblastic tumors, 9 cases of mesenteric desmoid fibromatosis, 10 dedifferentiated liposarcomas, and 9 malignant peripheral nerve sheath tumors.
  • None of the GISTs, desmoid tumors, inflammatory myofibroblastic tumors, schwannomas, dedifferentiated liposarcomas, or malignant peripheral nerve sheath tumors showed cytoplasmic reactivity for smoothelin.
  • Nuclear expression of smoothelin was not detected in any of the other tumor types examined.
  • [MeSH-major] Biomarkers, Tumor / analysis. Cytoskeletal Proteins / analysis. Gastrointestinal Neoplasms / chemistry. Muscle Proteins / analysis. Smooth Muscle Tumor / chemistry
  • [MeSH-minor] Cell Differentiation. Cell Nucleus / chemistry. Cytoplasm / chemistry. Desmin / analysis. Diagnosis, Differential. Humans. Immunohistochemistry. Neoplasm Staging. Predictive Value of Tests. Proto-Oncogene Proteins c-kit / analysis. Sensitivity and Specificity

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  • (PMID = 19950405.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cytoskeletal Proteins; 0 / Desmin; 0 / Muscle Proteins; 0 / SMTN protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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83. Garonzik Wang JM, Leach SD: Truncal sarcomas and abdominal desmoids. Surg Clin North Am; 2008 Jun;88(3):571-82, vi-vii
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Truncal sarcomas and abdominal desmoids.
  • It also provides a general review of abdominal desmoids.
  • [MeSH-major] Fibromatosis, Abdominal / surgery. Fibromatosis, Aggressive / surgery. Sarcoma / surgery
  • [MeSH-minor] Algorithms. Humans. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / surgery. Prognosis

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  • (PMID = 18514699.001).
  • [ISSN] 0039-6109
  • [Journal-full-title] The Surgical clinics of North America
  • [ISO-abbreviation] Surg. Clin. North Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 29
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84. Amary MF, Pauwels P, Meulemans E, Roemen GM, Islam L, Idowu B, Bousdras K, Diss TC, O'Donnell P, Flanagan AM: Detection of beta-catenin mutations in paraffin-embedded sporadic desmoid-type fibromatosis by mutation-specific restriction enzyme digestion (MSRED): an ancillary diagnostic tool. Am J Surg Pathol; 2007 Sep;31(9):1299-309
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  • [Title] Detection of beta-catenin mutations in paraffin-embedded sporadic desmoid-type fibromatosis by mutation-specific restriction enzyme digestion (MSRED): an ancillary diagnostic tool.
  • Desmoid-type fibromatosis is a locally aggressive deep soft tissue tumor.
  • The aim of this study was to determine the specificity and sensitivity of these 3 most common beta-catenin mutations in the diagnosis of desmoid-type fibromatosis using paraffin-embedded material.
  • One hundred and thirty-three cases were analyzed, including 76 desmoid-type, and 18 superficial fibromatosis, in addition to a further 39 fibromatosis mimics.
  • Mutations were detected in 66 cases (87%) of 76 desmoid-type fibromatosis (71 extra-abdominal).
  • All desmoid-type fibromatosis cases and 72% of the mimics tested showed nuclear positivity for beta-catenin indicating immunohistochemistry is a sensitive but not a specific test for desmoid-type fibromatosis.
  • In contrast, to date, beta-catenin mutations have not been detected in any lesions which mimic desmoid-type fibromatosis.
  • Mutation-specific restriction enzyme digestion, a simple and efficient means of detecting the common beta-catenin mutations in desmoid-type fibromatosis, complements light microscopy in reaching a diagnosis.
  • [MeSH-major] DNA Mutational Analysis / methods. Fibromatosis, Aggressive / diagnosis. Gene Expression Regulation, Neoplastic. Mutation. Restriction Mapping. beta Catenin / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Base Sequence. Cell Nucleus / chemistry. Child. Codon. Diagnosis, Differential. Female. Humans. Immunohistochemistry / methods. Magnetic Resonance Imaging. Male. Middle Aged. Molecular Sequence Data. Paraffin Embedding. Predictive Value of Tests. Sensitivity and Specificity. Tissue Array Analysis

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  • (PMID = 17721184.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CTNNB1 protein, human; 0 / Codon; 0 / beta Catenin
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85. Ristić D, Tomasević Z: Therapy of aggressive fibromatosis is still an open question: a series of patients treated at a single institution. J BUON; 2005 Jul-Sep;10(3):381-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapy of aggressive fibromatosis is still an open question: a series of patients treated at a single institution.
  • PURPOSE: This study was an attempt to evaluate the possible role of chemo-hormonotherapy as a possible approach in managing inoperable, deep extra-abdominal aggressive fibromatosis.
  • PATIENTS AND METHODS: A series of patients with inoperable, deep extra-abdominal aggressive fibromatosis, were treated with combination chemo-hormotherapy.
  • Extremities were the most frequent localization (5/9), followed by chest wall in 3 and abdominal wall in one patient.
  • Tumor size in most patients was 5-10 cm, and 3 patients had bulky disease (over 10 cm).
  • Complete remission (CR) was observed in one patient, partial remission (PR) in 4 and stabilization of disease (SD) in 4 patients.
  • No relapse of disease was observed up until now.
  • CONCLUSION: Systemic treatment should be considered in patients with aggressive fibromatosis for whom local treatment approaches are not possible or have failed.

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  • (PMID = 17357193.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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86. Ferenc T, Wroński JW, Kopczyński J, Kulig A, Sidor M, Stalińska L, Dziki A, Sygut J: Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor). Pathol Res Pract; 2009;205(5):311-24
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  • [Title] Analysis of APC, alpha-, beta-catenins, and N-cadherin protein expression in aggressive fibromatosis (desmoid tumor).
  • The aims of this study were to analyze the cadherin/catenin adhesion complex in cells from abdominal and extra-abdominal aggressive fibromatosis tumors, and to estimate the correlation between the expression of the tested proteins and the clinical data of the desmoid patients.
  • Immunohistochemistry was used to examine the expression of the cadherin/catenin adhesion complex: APC protein, alpha-, beta-catenin, and N-cadherin in archival material derived from 15 cases of extra-abdominal desmoid tumor (E-AD) and 20 cases of abdominal (AD) desmoid tumor.
  • In the groups investigated, no statistically significant correlation was found between alpha-catenin, beta-catenin (c), (n) and (c+n) expression, and tumor size (p>0.1).
  • The results regarding beta-catenin expression obtained in our study confirm the previous findings that nuclear accumulation of this protein plays a crucial role in the pathogenesis of aggressive fibromatosis.
  • [MeSH-major] Adenomatous Polyposis Coli Protein / biosynthesis. Cadherins / biosynthesis. Fibromatosis, Aggressive / metabolism. alpha Catenin / biosynthesis. beta Catenin / biosynthesis
  • [MeSH-minor] Adult. Age Factors. Aged. Biomarkers, Tumor / analysis. Female. Gene Expression. Humans. Immunohistochemistry. Male. Young Adult

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  • (PMID = 19124205.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / alpha Catenin; 0 / beta Catenin
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87. Zhang ZL, Liang CH, Liu YB, Xie SF, Yu YX, Wang QS, Liu ZY, Li JL: [Computed tomography and magnetic resonance imaging features of desmoid-type fibromatosis: comparison with the pathological findings]. Nan Fang Yi Ke Da Xue Xue Bao; 2010 Nov;30(11):2495-7
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  • [Title] [Computed tomography and magnetic resonance imaging features of desmoid-type fibromatosis: comparison with the pathological findings].
  • OBJECTIVE: To explore the computed tomography (CT) and magnetic resonance imaging (MRI) features of desmoid-type fibromatosis, and improve the diagnostic accuracy and understanding of the disease.
  • METHODS: The CT and MRI features of 18 cases of surgically and pathologically confirmed desmoid-type fibromatosis were reviewed retrospectively.
  • RESULTS: In the extra abdominal cases, the tumors occurred in the head and neck in 3, in the dorsal part of the chest in 2, in the abdominal wall and groin area in 9, and in the peritoneal cavity in 4; concomitant Gardner syndrome was found in 1 case.
  • In 4 cases the tumor occurred within 1 to 3 years after abdominal surgeries.
  • The density and signal of the tumor were well distributed.
  • CONCLUSION: The CT and MRI features of desmoid-type fibromatosis are characteristic, and CT and MRI are valuable modalities for the diagnosis and differential diagnosis of the tumor.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / radiography

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  • (PMID = 21097415.001).
  • [ISSN] 1673-4254
  • [Journal-full-title] Nan fang yi ke da xue xue bao = Journal of Southern Medical University
  • [ISO-abbreviation] Nan Fang Yi Ke Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] China
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88. Catalano F, Furci M, Fancello R, Costanzo M: Giant recurrent fibromatosis of the breast: a case report. Clinical features and implications for treatment. Chir Ital; 2006 Jul-Aug;58(4):538-43
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  • [Title] Giant recurrent fibromatosis of the breast: a case report. Clinical features and implications for treatment.
  • Fibromatosis of the breast is a benign but locally aggressive neoplasm, which has been described under various names including extra-abdominal desmoid tumour and aggressive fibromatosis.
  • Clinically and radiologically the lesion mimics breast cancer and the definitive diagnosis is provided by histology.
  • A high risk of local recurrence is a characteristic of this disease.
  • Radical excision of the tumour with clear histological margins decreases the likelihood of recurrence.
  • The possible association between breast implants and mammary fibromatosis has been reported.
  • In this study we report a case of giant fibromatosis of the breast observed in a young patient and its extensive recurrence, involving all quadrants of the breast, the pectoralis major muscle, the rectus abdominis muscle sheath and the costal layer.
  • The main clinical and pathological characteristics of this rare disease are illustrated, emphasizing the difficulties encountered both in the diagnosis of the primary lesion and in the management of its giant recurrence, particularly as regards the possibility of obtaining a radical surgical excision and the option of performing breast reconstruction.
  • [MeSH-major] Breast Neoplasms / diagnosis. Breast Neoplasms / surgery. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery
  • [MeSH-minor] Adult. Female. Humans. Mastectomy. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / surgery. Pectoralis Muscles / pathology. Pectoralis Muscles / surgery. Rectus Abdominis / pathology. Rectus Abdominis / surgery. Treatment Outcome

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  • (PMID = 16999161.001).
  • [ISSN] 0009-4773
  • [Journal-full-title] Chirurgia italiana
  • [ISO-abbreviation] Chir Ital
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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89. Tulchinsky H, Keidar A, Strul H, Goldman G, Klausner JM, Rabau M: Extracolonic manifestations of familial adenomatous polyposis after proctocolectomy. Arch Surg; 2005 Feb;140(2):159-63; discussion 164
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  • Twelve patients had 14 desmoid tumors: 7 were treated surgically and 7 medically (these patients received celecoxib and tamoxifen citrate therapy).
  • Two patients died--one of a huge mesenteric desmoid tumor and the other of an aggressive mesenteric malignant fibrous histiocytoma.
  • CONCLUSIONS: Long-term morbidity and mortality were strongly related to the development of mesenteric tumors and ampullary-duodenal polyps.
  • Early detection of desmoid tumors, duodenal, pouch, and rectal cuff adenomas by periodic computed tomography, gastroduodenoscopy, and pouchoscopy, respectively, may allow control by medical therapy, endoscopy, or limited surgical procedures.
  • In most patients control of desmoid tumors was achieved using a combination of celecoxib and tamoxifen citrate therapy.
  • [MeSH-minor] Adolescent. Adult. Celecoxib. Colorectal Neoplasms / drug therapy. Colorectal Neoplasms / radiography. Comorbidity. Drug Therapy, Combination. Endoscopy, Digestive System. Female. Fibromatosis, Aggressive / drug therapy. Fibromatosis, Aggressive / radiography. Fibromatosis, Aggressive / surgery. Humans. Male. Middle Aged. Pyrazoles / therapeutic use. Retrospective Studies. Sulfonamides / therapeutic use. Tamoxifen / therapeutic use

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  • (PMID = 15723997.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Pyrazoles; 0 / Sulfonamides; 094ZI81Y45 / Tamoxifen; JCX84Q7J1L / Celecoxib
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90. Janitzky A, Porsch M, Daher M, Küster D, Liehr UB: [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma]. Urologe A; 2010 Jan;49(1):81-3
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  • [Title] [Aggressive fibromatosis (desmoid tumor) : A rare differential diagnosis of metastasis of renal cell carcinoma].
  • [Transliterated title] Aggressive Fibromatose (Desmoidfibromatose) : Seltene Differentialdiagnose einer Nierenzellkarzinommetastase.
  • We report the case of a 65-year-old woman with an aggressive fibromatosis of the rectus abdominis muscle suspicious for a metastasis of renal cell carcinoma after tumor nephrectomy 3 years previously.
  • Aggressive fibromatoses (desmoid tumors) are rare semimalignant tumors of the connective tissue with local infiltration and destruction of tissue.
  • Complete resection is essential to avoid tumor relapse.
  • Aggressive fibromatosis must be considered in the differential diagnosis of renal cell carcinoma metastasis.
  • [MeSH-major] Abdominal Muscles / pathology. Carcinoma, Renal Cell / diagnosis. Carcinoma, Renal Cell / secondary. Fibromatosis, Abdominal / diagnosis. Kidney Neoplasms / diagnosis. Muscle Neoplasms / pathology
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans


91. Bernathova M, Felfernig M, Rachbauer F, Barthi SD, Martinoli C, Zelger B, Bodner G: Sonographic imaging of abdominal and extraabdominal desmoids. Ultraschall Med; 2008 Oct;29(5):515-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sonographic imaging of abdominal and extraabdominal desmoids.
  • PURPOSE: To describe the sonographic imaging characteristics of abdominal and extraabdominal desmoids.
  • MATERIALS AND METHODS: We retrospectively investigated 12 histologically and pathologically proven abdominal and extraabdominal desmoids.
  • A typical sonographic feature of all intramuscular desmoids was the spindle-shaped margin at the tumor ends when scanned along the long axis of the affected muscle.
  • Tumor vascularity was rich in 6 cases and poor in 6 cases.
  • A fibrillar pattern within the tumor was found in 75% of the cases.
  • [MeSH-major] Abdominal Neoplasms / ultrasonography. Fibromatosis, Aggressive / ultrasonography

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  • (PMID = 19241508.001).
  • [ISSN] 0172-4614
  • [Journal-full-title] Ultraschall in der Medizin (Stuttgart, Germany : 1980)
  • [ISO-abbreviation] Ultraschall Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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92. Pérez García FJ, Pinto Blazquez J, Rodríguez Martínez JJ, Gutiérrez García R, Jorge Barreiro JI, Velasco Alonso J: [Mesenteric desmoid tumor mimicking a testicular cancer recurrence]. Arch Esp Urol; 2007 Jul-Aug;60(6):703-6
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  • [Title] [Mesenteric desmoid tumor mimicking a testicular cancer recurrence].
  • [Transliterated title] Tumor desmoide mesentérico simulando una recidiva de cáncer testicular.
  • OBJECTIVE: Report of one case of desmoid tumor in a patient who had been treated of a testicular seminoma 26 months before, with excision of a retroperitoneal mass and chemotherapy.
  • On followup he presented with a mesenteric abdominal mass which was clinically labeleled as a recurrence of the seminoma.
  • RESULTS: Histologically it was reported as a mesenteric desmoid tumor.
  • Differential diagnosis with gastrointestinal stromal tumor was performed with immunohistochemical studies.
  • CONCLUSIONS: Desmoid tumor is rare.
  • There are few cases reported in patients with history of previous testicular tumor.
  • It should be included in the differential diagnosis of testicular tumor recurrences.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Mesentery. Peritoneal Neoplasms / diagnosis. Seminoma / diagnosis. Testicular Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Neoplasm Recurrence, Local / diagnosis

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  • (PMID = 17847750.001).
  • [ISSN] 0004-0614
  • [Journal-full-title] Archivos españoles de urología
  • [ISO-abbreviation] Arch. Esp. Urol.
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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93. Zampieri N, Cecchetto M, Zorzi MG, Pietrobelli A, Ottolenghi A, Camoglio F: An unusual case of extra-abdominal desmoid tumour. Eur J Cancer Care (Engl); 2010 May;19(3):410-2

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An unusual case of extra-abdominal desmoid tumour.
  • Desmoid tumour is relatively rare and generally non-metastatisizing lesion of mesenchymal origin composed of fibrous tissue and fitting in the group of aggressive fibromatosis; it is a locally aggressive proliferative soft-tissue lesion with controversial nature.
  • This tumour accounts for 0.03% of all tumours and 3% of soft-tissue tumours with annual incidence of two to four cases per million.
  • Although desmoid tumours are more common in persons aged 10-40 years than in others, they do occur in young children and older adults; in children the sex incidence is equal.
  • This is a rare case of extra-abdominal desmoid tumour in a 14-year-old girl affected by spastic tetraparesis.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Soft Tissue Neoplasms / diagnosis

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  • (PMID = 19709174.001).
  • [ISSN] 1365-2354
  • [Journal-full-title] European journal of cancer care
  • [ISO-abbreviation] Eur J Cancer Care (Engl)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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94. Bhattacharya B, Dilworth HP, Iacobuzio-Donahue C, Ricci F, Weber K, Furlong MA, Fisher C, Montgomery E: Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol; 2005 May;29(5):653-9
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  • [Title] Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.
  • Deep fibromatoses (desmoid tumors) are clonal myofibroblastic proliferations that are prone to aggressive local recurrences but that do not metastasize.
  • Virtually all deep fibromatoses have somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations leading to intranuclear accumulation of beta-catenin.
  • Since low-grade sarcomas in general lack beta-catenin and since reactive proliferations would not be expected to have it, we predicted that nuclear beta-catenin expression would be detected in deep fibromatoses but absent in other entities in the differential diagnosis.
  • We evaluated the role of beta-catenin to help differentiate distinguish deep fibromatoses from congeners.
  • Formalin-fixed, paraffin-embedded sections from 21 lesions from 20 patients with deep fibromatoses were stained with monoclonal beta-catenin antibody (Transduction Laboratories) and compared with low-grade fibromyxoid sarcoma (n=12), leiomyosarcoma (n=10), various other fibrosarcoma variants (n=13, including 3 myofibrosarcomas, 3 sclerosing epithelioid fibrosarcomas, 5 low-grade fibrosarcomas, 1 classic fibrosarcoma arising in dermatofibrosarcoma protuberans, 1 inflammatory myxohyaline tumor/myxoinflammatory fibroblastic sarcoma), myofibroma/myofibromatosis (n=12), nodular fasciitis (n=11), and scars (n=9).
  • All 21 examples of deep fibromatosis displayed nuclear beta-catenin (focal nuclear staining in one case to 90% staining).
  • All other lesions tested (n=67) lacked nuclear labeling for beta-catenin, showing only cytoplasmic accumulation. beta-Catenin immunohistochemistry separates deep fibromatosis from entities in the differential diagnosis, a finding that can be exploited for diagnosis.
  • Most fibromatoses have diffuse nuclear staining although occasional examples only focally label.
  • [MeSH-major] Cell Nucleus / pathology. Cytoskeletal Proteins / metabolism. Fibroblasts / pathology. Fibromatosis, Abdominal / pathology. Sarcoma / pathology. Trans-Activators / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Child. Cicatrix / metabolism. Cicatrix / pathology. Cytoplasm / metabolism. Cytoplasm / pathology. Diagnosis, Differential. Fasciitis / metabolism. Fasciitis / pathology. Female. Humans. Male. Middle Aged. beta Catenin

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  • (PMID = 15832090.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CTNNB1 protein, human; 0 / Cytoskeletal Proteins; 0 / Trans-Activators; 0 / beta Catenin
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95. Soufi M, Lahlou MK, Bensaid M, Messrouri R, Benamer S, Essadel A, Mdaghri J, Mohammadine E, Taghy A, Settaf A, Chad B: [Desmoid tumors of the abdominal wall: three cases]. Rev Med Liege; 2009 Dec;64(12):633-8

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  • [Title] [Desmoid tumors of the abdominal wall: three cases].
  • [Transliterated title] Les tumeurs desmoïdes de la paroi abdominale. A propos de trois cas.
  • They often develop from the fascia and muscles of the abdominal wall.
  • WE report three cases of histology proven desmoids tumors of the abdominal wall treated between 2000 and 2007.
  • Etiologic factors, diagnosis, surgical management and adjuvant therapy in case of incomplete resection or reccurrence are discussed.
  • [MeSH-major] Abdominal Wall / surgery. Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / surgery

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  • (PMID = 20143748.001).
  • [ISSN] 0370-629X
  • [Journal-full-title] Revue médicale de Liège
  • [ISO-abbreviation] Rev Med Liege
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Belgium
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96. Sudan D, DiBaise J, Torres C, Thompson J, Raynor S, Gilroy R, Horslen S, Grant W, Botha J, Langnas A: A multidisciplinary approach to the treatment of intestinal failure. J Gastrointest Surg; 2005 Feb;9(2):165-76; discussion 176-7
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  • Fifty patients (children=30, adults=20) underwent surgical procedures to restore intestinal continuity (n=5), repair enterocutaneous fistulas (n=5), resect dysmotile or strictured/obstructed bowel segments or mesenteric desmoid tumors (n=7), stricturoplasty (n=2), Bianchi tapering and lengthening (n=20), serial transverse enteroplasty (n=8), and other operations (n=8).
  • Patient deaths occurred primarily in those listed or eligible for transplantation and were related to advanced liver disease (n=3), gastrointestinal hemorrhage (n=1), or line sepsis (n=1).

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  • (PMID = 15694812.001).
  • [ISSN] 1091-255X
  • [Journal-full-title] Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
  • [ISO-abbreviation] J. Gastrointest. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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97. Tan CH, Pua U, Liau KH, Lee HY: Mesenteric desmoid tumour masquerading as a fat-containing cystic mass. Br J Radiol; 2010 Oct;83(994):e200-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric desmoid tumour masquerading as a fat-containing cystic mass.
  • Mesenteric desmoid tumour (MDT) is an uncommon neoplasm that typically presents as a solid soft-tissue mass on cross-sectional imaging.
  • [MeSH-major] Cysts / radiography. Fibromatosis, Abdominal / radiography. Mesenteric Cyst / radiography
  • [MeSH-minor] Abdominal Pain / etiology. Diagnosis, Differential. Female. Humans. Middle Aged. Tomography, X-Ray Computed. Vomiting / etiology

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  • (PMID = 20846976.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
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98. Agrawal PS, Jagtap SM, Mitra SR: Extra-abdominal desmoid tumour of the leg. Singapore Med J; 2008 Jan;49(1):e6-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extra-abdominal desmoid tumour of the leg.
  • Extra-abdominal desmoid tumour is a rare tumour and only a few cases occurring in the limbs have been reported.
  • She had a mild, dull, aching pain in the tumour.
  • Wide local excision was done and the tumour was found mainly in the subcutaneous tissue, which histopathologically proved to be an extra-abdominal desmoid tumour.
  • This case had an abnormal radiological appearance of peripheral calcification of tumour and saucer-shaped lesion in the underlying tibial cortex.
  • [MeSH-major] Fibromatosis, Aggressive / diagnosis. Leg / pathology. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biopsy. Disease-Free Survival. Female. Humans. Treatment Outcome

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  • (PMID = 18204758.001).
  • [ISSN] 0037-5675
  • [Journal-full-title] Singapore medical journal
  • [ISO-abbreviation] Singapore Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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99. de Camargo VP, Keohan ML, D'Adamo DR, Antonescu CR, Brennan MF, Singer S, Ahn LS, Maki RG: Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer; 2010 May 1;116(9):2258-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor).
  • BACKGROUND: In the current study, the authors examined the outcomes of patients with desmoid tumors who received systemic therapy at a single institution to provide a basis for the examination of newer agents.
  • METHODS: Records of patients with desmoid tumors who were treated with chemotherapy at the study institution were reviewed.
  • Patients without measurable disease and those receiving therapy could not be documented, and those receiving prophylactic therapy were excluded.
  • At the time of last follow-up, 9 patients had died, 7 of progressive disease.
  • An intra-abdominal primary tumor location was the most common (44%).
  • On multivariate analysis, macroscopic nodular morphology and the presence of Gardner syndrome were the only tumor factors found to be associated with a greater time to disease progression.
  • CONCLUSIONS: Compared with other agents, antiestrogens and anthracycline-containing regimens appear to be associated with a higher radiological response rate against desmoid tumors.
  • Systemic therapy can be successful in patients with desmoid tumors, and is a viable option in lieu of morbid or disabling surgery.

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  • [Copyright] (c) 2010 American Cancer Society.
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  • (PMID = 20187095.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / RC2 CA148260-01; United States / NCI NIH HHS / CA / P01 CA47179; United States / NCI NIH HHS / CA / P01 CA047179-15A2; United States / CCR NIH HHS / RC / CA148260-01; United States / NCI NIH HHS / CA / P01 CA047179
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Other-IDs] NLM/ NIHMS219553; NLM/ PMC2925106
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100. Kujak JL, Liu PT, Johnson GB, Callstrom MR: Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors. Skeletal Radiol; 2010 Feb;39(2):175-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors.
  • OBJECTIVE: Surgical resection, radiation therapy and chemotherapy are all accepted as standard treatments for extra-abdominal desmoid (EAD) tumors, but their effectiveness has been limited by frequent local recurrence.
  • The purpose of this article is to describe our early experiences with using percutaneous cryoablation for local control of extra-abdominal desmoid tumors in five patients whose tumors had failed to respond to standard therapy.
  • Three of these patients had been referred for cryoablation for local tumor control, and two had been referred for palliation of inoperable tumors.
  • RESULTS: For the three patients referred for local control of EAD tumors, complete tumor coverage with the ablation zones was achieved.
  • The third patient, with a 6.1 cm mass, reported improved mild pain at 6 months, and imaging showed a moderate decrease of tumor size.
  • For the two patients referred for palliative therapy, initial partial pain relief was felt 2 weeks after the procedure, At long-term (58 months) follow-up of one patient with a 9.1 cm mass, the tumor was still present although reduced in size, and local pain had returned to its former moderate level.
  • [MeSH-major] Cryosurgery / methods. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery
  • [MeSH-minor] Abdominal Neoplasms / diagnosis. Abdominal Neoplasms / surgery. Adolescent. Adult. Child. Female. Humans. Male. Pilot Projects. Treatment Outcome. Young Adult

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  • (PMID = 19768644.001).
  • [ISSN] 1432-2161
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Germany
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