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1. Mrad K, Sassi S, Smida M, Oubiche F, Mekni A, Romdhane KB: Osteosarcoma with rhabdomyosarcomatous component or so-called malignant mesenchymoma of bone. Pathologica; 2004 Dec;96(6):475-8
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  • [Title] Osteosarcoma with rhabdomyosarcomatous component or so-called malignant mesenchymoma of bone.
  • BACKGROUND: Primary malignant mesenchymoma of the bone is a rare neoplasm consisting of two or more unrelated malignant mesenchymal components.
  • OBSERVATION: We report an exceedingly rare case of primary malignant mesenchymoma of bone composed of rhabdomyosarcoma, osteosarcoma, and a minor chondrosarcoma component, arising in the right proximal humerus of a 15-year-old girl.
  • The rhabdomyosarcomatous component was present in the initial biopsy and persisted in surgical specimen following chemotherapy.
  • CONCLUSION: Effect of chemotherapy is enigmatic since rhabdomyosarcomatous component could appear, persist or disappear after chemotherapy according to literature.
  • [MeSH-major] Bone Neoplasms / pathology. Humerus / pathology. Mesenchymoma / pathology. Neoplasms, Multiple Primary / pathology. Osteosarcoma / pathology. Rhabdomyosarcoma / pathology
  • [MeSH-minor] Adolescent. Antimetabolites, Antineoplastic / therapeutic use. Chondrosarcoma / diagnosis. Chondrosarcoma / drug therapy. Chondrosarcoma / pathology. Chondrosarcoma / radiography. Chondrosarcoma / surgery. Combined Modality Therapy. Desmin / analysis. Diagnosis, Differential. Fatal Outcome. Female. Fibrosarcoma / diagnosis. Fibrosarcoma / drug therapy. Fibrosarcoma / pathology. Fibrosarcoma / radiography. Fibrosarcoma / surgery. Humans. Methotrexate / therapeutic use. Neoplasm Proteins / analysis. Osteolysis / etiology. Postoperative Complications / etiology. Pulmonary Embolism / etiology. Sarcoma, Ewing / diagnosis


2. Drabko K, Choma M, Zaucha-Prazmo A, Wójcik B, Gorczyńska E, Kałwak K, Turkiewicz D, Słociak M, Ussowicz M, Dyla A, Chybicka A, Styczyński J, Debski R, Wysocki M, Goździk J, Ratajczak M, Kowalczyk JR: [Megachemotherapy and autologous hematopoietic stem cell transplantation in children with solid tumours excluding neuroblastoma--experience of Polish paediatric centres]. Med Wieku Rozwoj; 2006 Jul-Sep;10(3 Pt 1):785-92
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  • 25 children were treated for Ewing Sarcoma, 13 for rhabdomyosarcoma embryonale (RMS), 7 for germinal tumours, 6 for medulloblastoma, 4 for PNET, 4 for Wilm's tumours, 2 for glioblastoma and single patients with mesenchymoma, astrocytoma, ependymoma, angioblastoma, carcinoma ovarian and carcinoma embryonale glutei.
  • In 29 children MCH was introduced in first complete remission, in 14 the procedure was performed in second or subsequent remission and 24 patients did not achieve remission before megachemotherapy was started.
  • RESULTS: 30 children are alive (44%), 28 of them in complete remission of disease.
  • 23 out of 29 (79%) patients were transplanted in first complete remission and median observation time in that group is 29 months (range 2-74 months).
  • 39 patients relapsed at a median time 11 months after MCT and 37 of them subsequently died of disease at a median time of 16 months.
  • One toxic death was noted--it was a boy, transplanted with progressive disease.
  • It is a safe procedure especially when performed in remission.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hematopoietic Stem Cell Transplantation. Neoplasms / drug therapy. Neoplasms / surgery
  • [MeSH-minor] Academic Medical Centers. Adolescent. Antineoplastic Agents, Alkylating / administration & dosage. Bone Neoplasms / drug therapy. Bone Neoplasms / surgery. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Dose-Response Relationship, Drug. Ependymoma / drug therapy. Ependymoma / surgery. Female. Glioblastoma / drug therapy. Glioblastoma / surgery. Glioma / drug therapy. Glioma / surgery. Humans. Infant. Male. Meningeal Neoplasms / drug therapy. Meningeal Neoplasms / surgery. Meningioma / drug therapy. Meningioma / surgery. Neoplasm Staging. Oncology Service, Hospital. Poland. Remission Induction. Retrospective Studies. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / surgery. Transplantation, Autologous. Treatment Outcome


3. Kramm T, Gaumann A, Heussel CP, Dahm M, Oelert H, Mayer E: [Surgical management of pulmonary artery sarcoma]. Dtsch Med Wochenschr; 2001 Dec 14;126(50):1423-7
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  • [Transliterated title] Chirurgische Therapie des Pulmonalarteriensarkoms.
  • Malignancy was suspected in six of these patients by means of computed tomography (CT) and magnetic resonance tomography (MRT).
  • Operative procedures consisted of gross tumor resection with prosthetic replacement (n = 3) or reconstruction (n = 5) of central parts of the pulmonary vessels.
  • Seven patients received adjuvant radio- and/or chemotherapy.
  • Adjuvant therapy might bring additional benefit.
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Chemotherapy, Adjuvant. Female. Humans. Leiomyosarcoma / complications. Leiomyosarcoma / diagnosis. Leiomyosarcoma / surgery. Magnetic Resonance Imaging. Male. Mesenchymoma / complications. Mesenchymoma / diagnosis. Mesenchymoma / surgery. Middle Aged. Neoplasm Metastasis. Neoplasm Recurrence, Local / pathology. Pneumonectomy. Prognosis. Radiotherapy, Adjuvant. Tomography, X-Ray Computed

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  • (PMID = 11743678.001).
  • [ISSN] 0012-0472
  • [Journal-full-title] Deutsche medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Dtsch. Med. Wochenschr.
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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4. Coşkun HS, Göksu SS, Sahin M, Alanoğlu G: Bleomycin, etoposide and cisplatin (BEP) combination with concurrent imatinib mesylate (GLEEVEC) in chronic myeloid leukemia (CML) patient with mesenchymal tumor. Med Oncol; 2008;25(1):110-2
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  • [Title] Bleomycin, etoposide and cisplatin (BEP) combination with concurrent imatinib mesylate (GLEEVEC) in chronic myeloid leukemia (CML) patient with mesenchymal tumor.
  • Imatinib is now indicated as the first line therapy for chronic myeloid leukemia (CML).
  • Treatment of CML with imatinib is generally well tolerated and the risk of severe adverse affects is low.
  • Many new drugs including targeted therapy are combined with antineoplastic agents safely.
  • We here report a patient with CML who developed concurrent mesenchymal tumor while undergoing therapy with imatinib and treated with combination chemotherapy including bleomycin, etoposide, and cisplatin, as well as imatinib without severe toxicity.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia, Myelogenous, Chronic, BCR-ABL Positive / drug therapy. Mesenchymoma / drug therapy. Piperazines / administration & dosage. Pyrimidines / administration & dosage


5. de Bouyn-Icher C, Minard-Colin V, Isapof A, Khuong Quang DA, Redon I, Hartmann O: [Malignant solid tumors in neonates: a study of 71 cases]. Arch Pediatr; 2006 Dec;13(12):1486-94
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  • Clinical features, histologic types, prognosis were very different from those seen in older children, facing oncologists with diagnostic, therapeutic and ethical problems.
  • PATIENTS AND METHODS: In a retrospective study from January 1987 to January 2004, we reviewed the management of neonates treated at the Institute Gustave Roussy for a malignant solid tumor for whom symptoms started in the first month of life.
  • RESULTS: Seventy-one neonates were treated, comprising 1,2% of the overall patients treated during the same period of time.
  • Of these 71 patients, 42 (59%) presented with neuroblastomas, 12 (17%) with mesenchymal tumors, 6(8%) with cerebral tumors and 11 with various other types of tumors.
  • Thirty-eight neonates received chemotherapy, administered at a 30 to 50% reduced dose.
  • Hematologic toxicities and infections were the main therapeutic complications.
  • There has been no therapy-related mortality.
  • The young age of patients resulted in problems of treatment tolerance.
  • The therapeutic regimen should take into account the risk of acute iatrogenic toxicity and long term sequelae.
  • Surgery remains the treatment of choice but chemotherapy, with dose reduction, managed by expert teams, is essential and safer in a lot of case.
  • [MeSH-major] Brain Neoplasms / epidemiology. Mesenchymoma / epidemiology. Neuroblastoma / epidemiology
  • [MeSH-minor] Adolescent. Age Factors. Antineoplastic Agents / therapeutic use. Brain / pathology. Child. Child, Preschool. Female. France / epidemiology. Humans. Incidence. Infant. Infant, Newborn. Male. Neoplasm Staging. Prenatal Diagnosis. Prognosis. Radiotherapy Dosage. Retrospective Studies. Risk Factors. Sex Factors. Survival Analysis

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  • (PMID = 17137765.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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6. Kim DY, Kim KH, Jung SE, Lee SC, Park KW, Kim WK: Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg; 2002 Oct;37(10):1419-23
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  • [Title] Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy.
  • BACKGROUND/PURPOSE: Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignancy found in older children, and the most appropriate treatment strategy has been controversial.
  • The authors report the tumor's clinical characteristics and the results of management in 6 children and recommend the best treatment plans based on these findings.
  • Location of the tumor varied from right lobe in 4 to left lobe in one, and both lobes in one.
  • Three children underwent primary complete resection without preoperative chemotherapy.
  • Partial resection of the tumor was done in one child because of encasing inferior vena cava and hepatic vein.
  • However, complete resection was possible at second-look operation after chemotherapy.
  • Two children underwent complete resection after chemotherapy.
  • Preoperative chemotherapy allowed successful resection of 3 USL, which were initially considered unresectable.
  • Chemotherapy according to the the Third Intergroup Rhabdomyosarcoma Study (IRS III) was administered to all except one child who refused further postoperative chemotherapy after having had severe complications during the first cycle of chemotherapy.
  • One child with partial resection died of sepsis at 22 months after diagnosis during postoperative chemotherapy after complete surgical removal of the tumor.
  • CONCLUSION: The combined therapy of surgery and chemotherapy in USL can improve the prognosis.
  • [MeSH-major] Liver Neoplasms / drug therapy. Liver Neoplasms / surgery. Mesenchymoma / drug therapy. Mesenchymoma / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Female. Hepatectomy. Humans. Male. Prognosis. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2002, Elsevier Science (USA). All rights reserved.
  • (PMID = 12378446.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Comparative Study; Journal Article
  • [Publication-country] United States
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7. Oppenheimer O, Athanasian E, Meyers P, Antonescu CR, Gorlick R: Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature. Int J Surg Pathol; 2005 Jan;13(1):113-6
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  • Malignant ectomesenchymomas, rare and potentially aggressive tumors, occur in children and exhibit mesenchymal and neuroectodermal components.
  • This report describes the first patient diagnosed with a malignant ectomesenchymoma of the hand.
  • The patient was a 17-month-old male who developed a hypothenar mass on his left hand that was surgically excised.
  • After 3 courses of chemotherapy the patient had a wide reexcision with no residual tumor.
  • The patient is 4 years from diagnosis, without evidence of disease.
  • [MeSH-major] Mesenchymoma / pathology. Soft Tissue Neoplasms / pathology. Wrist
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Infant. Male. Vincristine / administration & dosage

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  • (PMID = 15735865.001).
  • [ISSN] 1066-8969
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide
  • [Number-of-references] 17
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8. Verweij J, Judson I, van Oosterom A: STI571: a magic bullet? Eur J Cancer; 2001 Oct;37(15):1816-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Enzyme Inhibitors / therapeutic use. Gastrointestinal Neoplasms / drug therapy. Leukemia, Myelogenous, Chronic, BCR-ABL Positive / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use
  • [MeSH-minor] Benzamides. Humans. Imatinib Mesylate. Mesenchymoma / drug therapy. Protein-Tyrosine Kinases / antagonists & inhibitors

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  • (PMID = 11576833.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Enzyme Inhibitors; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases
  • [Number-of-references] 16
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9. Deslée G, Guillou PJ, Baehrel B, Lebargy F: Malignant mesenchymoma of the pleura. Interact Cardiovasc Thorac Surg; 2003 Sep;2(3):376-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant mesenchymoma of the pleura.
  • Malignant mesenchymomas are rare soft tissue tumors of mesenchymal origin.
  • The treatment associated surgical resection, chemotherapy and radiotherapy.

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  • (PMID = 17670075.001).
  • [ISSN] 1569-9285
  • [Journal-full-title] Interactive cardiovascular and thoracic surgery
  • [ISO-abbreviation] Interact Cardiovasc Thorac Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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10. Lee JA, Kim TW, Min JH, Byon SJ, Jang SH, Choi SY, Kim HJ: [A case of undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult]. Korean J Gastroenterol; 2010 Feb;55(2):144-8
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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 undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult].
  • Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children.
  • It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma.
  • The pathologic finding revealed infiltrative growth of atypical cells with rhabdoid features.
  • These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.
  • [MeSH-minor] Aged. Bile Ducts, Intrahepatic / pathology. Diagnosis, Differential. Dilatation, Pathologic. Humans. Klatskin Tumor / diagnosis. Male. Positron-Emission Tomography. Tomography, X-Ray Computed. Tuberculosis / diagnostic imaging. Tuberculosis / drug therapy. Ultrasonography. Vimentin / metabolism


11. Weiss E, Albrecht CF, Herms J, Behnke-Mursch J, Pekrun A, Brockmann K, Hess CF: Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. Eur J Pediatr; 2005 Jun;164(6):345-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options.
  • Malignant ectomesenchymoma is a rare tumour that contains both ectodermal and mesenchymal elements.
  • Only three patients with a manifestation in the cerebrum and clinicopathological data have been reported until now.
  • We present a patient with an intracerebral ectomesenchymoma, review the literature and discuss currently available therapeutic options.
  • In a 10-year-old girl, a left suprasellar temporo-parieto-occipitally localised tumour was diagnosed.
  • The tumour was completely excised macroscopically in two surgical sessions.
  • For the mesenchymal part of the tumour she subsequently underwent multidrug chemotherapy followed by radiation therapy.
  • Considering the neuroectodermal element of the tumour, radiotherapy was applied to the craniospinal axis with a local boost.
  • Therapy was tolerated well without any severe side effects.
  • Six years from diagnosis, the patient is alive without a tumour relapse.
  • CONCLUSION: Due to the sparcity of reported cases with malignant ectomesenchymoma, the role of adjuvant therapy is unclear.
  • Multimodal therapy may be able to improve outcome.
  • [MeSH-major] Brain Neoplasms. Cerebral Cortex. Mesenchymoma. Neuroectodermal Tumors
  • [MeSH-minor] Child. Combined Modality Therapy. Female. Humans

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  • (PMID = 15747131.001).
  • [ISSN] 0340-6199
  • [Journal-full-title] European journal of pediatrics
  • [ISO-abbreviation] Eur. J. Pediatr.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 17
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12. Machaladze ZO, Davydov MI, Polotskiĭ BE, Karseladze AI, Savelov NA: [Surgery for mesenchymal tumours of the mediastinum]. Khirurgiia (Mosk); 2008;(4):43-7
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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 mesenchymal tumours of the mediastinum].
  • Results of treatment of 52 patients with mesenchymal tumours of the mediastinum.
  • Clinical finding is various and nonspecific.
  • Computer tomography and MRT are the most informative diagnostic techniques.
  • Among 52 patients with mesenchymal tumours of the mediastinum, 40 patients were treated surgically and 12 patients got conservative treatment.
  • Chemotherapy and radiotherapy were carried out in 7 patients in pre- and postoperative periods.
  • Radical surgical treatment was carried out in 21 patients with benign tumors.
  • Among 19 patients with malignant tumors, 42% of patients got radical surgical treatment, and 58 % of patients got palliative therapy in combination with chemotherapy.
  • Benign tumors have a favorable prognosis after surgical treatment.
  • Major factors of the prognosis of surgical treatment are dissemination and the histologic form of a tumor as well as type of surgical intervention.
  • [MeSH-major] Mediastinal Neoplasms / surgery. Mediastinoscopy / methods. Mesenchymoma / surgery. Thoracic Surgery, Video-Assisted / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 18454108.001).
  • [ISSN] 0023-1207
  • [Journal-full-title] Khirurgiia
  • [ISO-abbreviation] Khirurgiia (Mosk)
  • [Language] rus
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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13. Paramelle O, Croué A, Dupré F, Rialland X, Saint-André JP: [Pelvic malignant ectomesenchymoma: a case report]. Ann Pathol; 2001 Aug;21(4):344-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We report the case of a 19 month old boy referred to our institution because of a pelvic tumor initially identified as an embryonal rhabdomyosarcoma and treated with surgery and chemotherapy.
  • Eight years after the initial surgery, a local tumor recurrence with bone metastasis was found.
  • This double differentiation was retrospectively found in the initial tumor, then allowing the diagnosis of malignant ectomesenchymoma also called gangliorhabdomyosarcoma.
  • This rare tumor, occurring mainly during childhood, is composed of neuroblasts and / or ganglion cells and of malignant mesenchymal cells (usually rhabdomyosarcomatous cells).
  • [MeSH-major] Mesenchymoma / pathology. Pelvic Neoplasms / pathology. Rhabdomyosarcoma / pathology

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  • (PMID = 11685134.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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14. Floris G, Debiec-Rychter M, Wozniak A, Magrini E, Manfioletti G, De Wever I, Tallini G, Sciot R: Malignant ectomesenchymoma: genetic profile reflects rhabdomyosarcomatous differentiation. Diagn Mol Pathol; 2007 Dec;16(4):243-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Retrospective review of the incision biopsy showed the presence of a few ganglion cells in an otherwise classic embryonal rhabdomyosarcoma (RMS), whereas in the resection specimen after chemotherapy the combined RMS and ganglioneuroma components were very obvious.
  • Cytogenetic analysis of the residual lesion showed an abnormal karyotype, 49, XY, +2, -6, +11, +20, +mar, with a hyperploidy in a subset of cells.
  • By fluorescence in situ hybridization analysis, the marker chromosome was identified as originating from chromosome 6, and the tumor cells were negative for PAX3/PAX7 disrupting translocations specific for alveolar RMS.
  • Gains of chromosomes 2, 11, and 20, found in the current case, are a common finding in embryonal RMS.
  • The genomic profiles of both specimens were basically the same including the presence of 2 distinctive chromosome 6p21.32-p21.2 and 6p11.2 amplification regions in the primary tumor, which vanished in the postchemotherapy specimen.
  • The pretreatment biopsy exhibited strong expression of HMGA1 and HMGA2 proteins in immunohistochemistry, with the shift toward the loss of expression of both genes in the posttreatment tumoral tissue.
  • This finding supports the oncogenic properties of the HMGA family of proteins and their role in the process of malignant transformation.
  • [MeSH-major] Gene Expression Profiling. Genital Neoplasms, Male / diagnosis. Mesenchymoma / diagnosis. Rhabdomyosarcoma, Embryonal / diagnosis. Scrotum

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  • (PMID = 18043289.001).
  • [ISSN] 1052-9551
  • [Journal-full-title] Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • [ISO-abbreviation] Diagn. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / HMGA2 Protein; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors; 124544-67-8 / HMGA1a Protein
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15. Müller HL, Marx A, Trusen M, Schneider P, Kühl J: Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature. Pediatr Hematol Oncol; 2002 Jan-Feb;19(1):9-17
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  • Malignant ectomesenchymoma (MEM) is a rare soft tissue tumor believed to arise from a pluripotent migratory neural crest cell and composed fo both a mesenchymal element and a neuroectodermal element.
  • The authors report the case of an 11-month-old male who presented with a local abdominal MEM and systemic metastases into lungs, liver, bones, and bone marrow.
  • The tumor consisted of a neuroblastoma component and a mesenchymal component with sarcomatous features.
  • Diagnosis and therapy were complicated by the histological heterogeneity of the tumor, which also influenced the clinical appearance and course in this case.
  • A literature search revealed 15 other evaluated cases that arose in soft tissue and had adequate clinicopathologic data.
  • Complete surgical resection was the mainstay of treatment, and chemotherapy also appeared to be important.
  • In patients with disseminated MEM, new therapeutic approaches such as high-dose chemotherapy followed by stem cell rescue should be considered, similar to the current strategy in patients with stage VI neuroblastoma or soft tissue sarcoma.
  • [MeSH-major] Abdominal Neoplasms / pathology. Mesenchymoma / secondary
  • [MeSH-minor] 3-Iodobenzylguanidine. Bone Marrow Neoplasms / radionuclide imaging. Bone Marrow Neoplasms / secondary. Bone Marrow Neoplasms / therapy. Fatal Outcome. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm Metastasis / pathology. Neoplasm Recurrence, Local / therapy

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  • (PMID = 11787870.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 13
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16. Longley BJ, Reguera MJ, Ma Y: Classes of c-KIT activating mutations: proposed mechanisms of action and implications for disease classification and therapy. Leuk Res; 2001 Jul;25(7):571-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Classes of c-KIT activating mutations: proposed mechanisms of action and implications for disease classification and therapy.
  • Mutations causing constitutive activation of KIT have been shown to be causative in some forms of mastocytosis, and several types of mutations have been associated with myeloproliferative disorders (MPDs), acute myelogenous leukemia (AML), sinonasal lymphomas, and gastrointestinal stromal tumors (GIST).
  • We divide these activating mutation into two types - 'regulatory type' mutations, which affect regulation of the kinase molecule, and 'enzymatic pocket type' mutations, which alter the amino acid sequence directly forming the enzymatic site.
  • KIT inhibitors have been suggested as therapeutic drugs for these conditions, but different types of activating mutations respond differentially to KIT inhibitors, so classification of individuals on the basis of specific mutations is necessary to guide therapy.
  • [MeSH-minor] Acute Disease. Adult. Antineoplastic Agents / chemistry. Antineoplastic Agents / pharmacology. Binding Sites. Child. Drug Design. Enzyme Activation / genetics. Enzyme Inhibitors / chemistry. Enzyme Inhibitors / pharmacology. Gastrointestinal Neoplasms / genetics. Hematologic Neoplasms / genetics. Humans. Leukemia, Myeloid / genetics. Ligands. Lymphoma, Non-Hodgkin / genetics. Mesenchymoma / genetics. Myeloproliferative Disorders / genetics. Nose Neoplasms / genetics. Paranasal Sinus Neoplasms / genetics. Phosphorylation. Protein Processing, Post-Translational. Stem Cell Factor / antagonists & inhibitors. Stem Cell Factor / physiology

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  • (PMID = 11377682.001).
  • [ISSN] 0145-2126
  • [Journal-full-title] Leukemia research
  • [ISO-abbreviation] Leuk. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Enzyme Inhibitors; 0 / Ligands; 0 / Stem Cell Factor; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
  • [Number-of-references] 42
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17. Yeung SJ, McCutcheon IE, Schultz P, Gagel RF: Use of long-term intravenous phosphate infusion in the palliative treatment of tumor-induced osteomalacia. J Clin Endocrinol Metab; 2000 Feb;85(2):549-55
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  • [Title] Use of long-term intravenous phosphate infusion in the palliative treatment of tumor-induced osteomalacia.
  • Tumor-induced osteomalacia is characterized by paraneoplastic defects in vitamin D metabolism, proximal renal tubular functions, and phosphate transport.
  • Palliative treatment with calcium, vitamin D, and phosphate replacement is indicated for patients in whom the causative tumor cannot be completely resected.
  • In this report we describe a case of tumor-induced osteomalacia in whom adequate oral doses of phosphate could not be used because of gastrointestinal side-effects.
  • This patient received repeated (three times) phosphate infusions over 8 yr, resulting in laboratory and symptomatic improvement after each course.
  • [MeSH-major] Mesenchymoma / complications. Osteomalacia / drug therapy. Osteomalacia / etiology. Palliative Care. Phosphates / administration & dosage. Spinal Neoplasms / complications
  • [MeSH-minor] Calcium / therapeutic use. Drug Therapy, Combination. Female. Humans. Infusion Pumps. Injections, Intravenous. Middle Aged. Time Factors. Vitamin D / therapeutic use

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  • (PMID = 10690854.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Phosphates; 1406-16-2 / Vitamin D; SY7Q814VUP / Calcium
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18. Chow LT, Kumta SM: Primary osteochondrorhabdomyosarcoma (malignant mesenchymoma) of the fibula: a rare tumour in an unusual site -- case report and review of the literature. APMIS; 2004 Sep;112(9):617-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary osteochondrorhabdomyosarcoma (malignant mesenchymoma) of the fibula: a rare tumour in an unusual site -- case report and review of the literature.
  • Malignant mesenchymoma, defined by Stout as sarcomas comprising two or more unrelated differentiated tissue elements other than a fibrosarcoma component, is rare.
  • We report a case of primary malignant mesenchymoma of the proximal fibula in a 10-year-old female student who presented with pain and swelling of the right knee for 2 months.
  • Including our present patient, 16 cases of primary malignant mesenchymoma of bone are found in the English literature, affecting mainly adolescents and young adults, with a slight male predominance and predilection for the metaphysis of long bones, especially around the knee.
  • The clinical features of primary malignant mesenchymoma of bone thus resemble those of conventional osteosarcoma.
  • Moreover, our case illustrates that, with combination chemotherapy targeted for individual elements, the prognosis of this rare tumour might be much improved, as in osteosarcoma.
  • [MeSH-major] Bone Neoplasms / pathology. Fibula / pathology. Mesenchymoma / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biopsy. Child. Female. Humans. Immunohistochemistry

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  • (PMID = 15601312.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Denmark
  • [Number-of-references] 22
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19. De Padua M, Bhandari TP, Pingle J: Primary osteoliposarcoma of the bone. Indian J Pathol Microbiol; 2009 Jan-Mar;52(1):80-2
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  • Some of the reports have referred to these tumors as malignant mesenchymoma.
  • Radiologically, a tumor in the lower end of the right femur was seen extending into the soft tissue.
  • The patient received three cycles of neo-adjuvant chemotherapy followed by limb-salvage surgery with provisions for a custom-made prosthesis.
  • Only 21% tumor necrosis (effects of chemotherapy) was observed.
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Biopsy. Femur / pathology. Femur / radiography. Humans. Male

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  • (PMID = 19136790.001).
  • [ISSN] 0974-5130
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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20. Stewart RJ, Martelli H, Oberlin O, Rey A, Bouvet N, Spicer RD, Godzinski J, Stevens MC, International Society of Pediatric Oncology: Treatment of children with nonmetastatic paratesticular rhabdomyosarcoma: results of the Malignant Mesenchymal Tumors studies (MMT 84 and MMT 89) of the International Society of Pediatric Oncology. J Clin Oncol; 2003 Mar 1;21(5):793-8
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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 children with nonmetastatic paratesticular rhabdomyosarcoma: results of the Malignant Mesenchymal Tumors studies (MMT 84 and MMT 89) of the International Society of Pediatric Oncology.
  • PURPOSE: To report the results of the Malignant Mesenchymal Tumors studies (MMT 84 and 89) of the International Society of Pediatric Oncology (SIOP) in males with nonmetastatic paratesticular rhabdomyosarcoma.
  • Disease was staged according to the SIOP tumor-node-metastasis staging system.
  • Treatment was stratified by stage.
  • In the MMT 89 study, males with completely resected tumors at diagnosis received less chemotherapy (vincristine and dactinomycin) than patients in the MMT 84 study (ifosfamide, vincristine, and dactinomycin).
  • Thirty-one tumors were larger than 5 cm, and 13 males were older than 10 years with a tumor larger than 5 cm.
  • CONCLUSION: Males with paratesticular RMS have an excellent prognosis except for a selected group of patients older than 10 years or with tumor greater than 5 cm.
  • Intensified chemotherapy incorporating alkylating agents for this subgroup may be preferred to the use of systematic lymphadenectomy to improve survival while minimizing the burden of therapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dactinomycin / therapeutic use. Ifosfamide / therapeutic use. Mesenchymoma / drug therapy. Rhabdomyosarcoma / drug therapy. Testicular Neoplasms / drug therapy. Vincristine / therapeutic use
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Humans. Infant. Lymph Node Excision. Male. Neoplasm Recurrence, Local / drug therapy. Neoplasm Recurrence, Local / surgery. Neoplasm Staging. Salvage Therapy. Survival Rate. Treatment Outcome

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  • (PMID = 12610176.001).
  • [ISSN] 0732-183X
  • [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
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; UM20QQM95Y / Ifosfamide; IVA protocol; SIOP protocol
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21. Büchner-Steudel P, Fleig WE: [Diagnosis and conservative treatment of gastrointestinal stromal tumors (GIST)]. Dtsch Med Wochenschr; 2004 Aug 20;129(34-35):1808-10
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  • [Title] [Diagnosis and conservative treatment of gastrointestinal stromal tumors (GIST)].
  • [Transliterated title] Diagnostik und konservative Therapie gastrointestinaler Stromatumoren.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Enzyme Inhibitors / therapeutic use. Gastrointestinal Neoplasms / diagnosis. Gastrointestinal Neoplasms / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use
  • [MeSH-minor] Benzamides. Diagnosis, Differential. Endoscopy, Gastrointestinal. Endosonography. Female. Humans. Imatinib Mesylate. Leiomyoma, Epithelioid / diagnosis. Leiomyoma, Epithelioid / drug therapy. Leiomyoma, Epithelioid / epidemiology. Leiomyosarcoma / diagnosis. Leiomyosarcoma / drug therapy. Leiomyosarcoma / epidemiology. Male. Mesenchymoma / diagnosis. Mesenchymoma / drug therapy. Mesenchymoma / epidemiology. Neurilemmoma / diagnosis. Neurilemmoma / drug therapy. Neurilemmoma / epidemiology. Prognosis. Protein-Tyrosine Kinases / antagonists & inhibitors. Sex Factors. Tomography, X-Ray Computed

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  • (PMID = 15314745.001).
  • [ISSN] 0012-0472
  • [Journal-full-title] Deutsche medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Dtsch. Med. Wochenschr.
  • [Language] ger
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Enzyme Inhibitors; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases
  • [Number-of-references] 16
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22. Paikos P, Papathanassiou M, Stefanaki K, Fotopoulou M, Grigorios S, Tzortzatou F: Malignant ectomesenchymoma of the orbit in a child: Case report and review of the literature. Surv Ophthalmol; 2002 Jul-Aug;47(4):368-74
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Malignant ectomesenchymoma is a rare soft tissue tumor of childhood composed of both mesenchymal and neuroectodermal elements.
  • The clinical picture of the tumor, radiological findings, and its histopathologic and immunohistochemical characteristics are described.
  • The patient was successfully treated with combined surgical resection and chemotherapy.
  • [MeSH-major] Mesenchymoma / pathology. Orbital Neoplasms / pathology
  • [MeSH-minor] Child. Combined Modality Therapy. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male

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  • (PMID = 12161212.001).
  • [ISSN] 0039-6257
  • [Journal-full-title] Survey of ophthalmology
  • [ISO-abbreviation] Surv Ophthalmol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 45
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23. Kutsal A, Tansal S, Okutan H, Tuncer I: Primary malignant mesenchymoma of the heart. Eur J Cardiothorac Surg; 2002 Jan;21(1):124-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary malignant mesenchymoma of the heart.
  • Primary malignant cardiac tumours are uncommon, and cardiac malignant mesenchymoma is extremely rare.
  • A case of primary malignant mesenchymoma in a 41-year-old woman arousing from the left atrial septum, obstructing the mitral orifice by passing through it into the left ventricle is described.
  • The tumour was fully resected, and adjuvant chemotherapy was applied, but the patient had died by tumour recurrence in 8 months.
  • [MeSH-major] Heart Neoplasms / surgery. Mesenchymoma / surgery

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  • (PMID = 11788281.001).
  • [ISSN] 1010-7940
  • [Journal-full-title] European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
  • [ISO-abbreviation] Eur J Cardiothorac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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24. Tochika N, Kumon M, Ogawa Y, Sugimoto T, Araki K: Stromal sarcoma of the breast with lung metastasis successfully treated by radiotherapy: report of a case. Surg Today; 2000;30(3):282-5
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  • The original breast tumor had been extirpated in a local hospital, and she was subsequently referred to our outpatient clinic for nonsurgical treatment.
  • Pathological examination of the breast tumor had confirmed a diagnosis of stromal sarcoma.
  • Radiation and systemic chemotherapy for the remnant disease were administered at our hospital; however, 8 months later, local recurrence and a solitary lung metastasis were recognized on a chest X-ray.
  • A second lumpectomy of the recurrent breast tumor along with radiation for the lung metastasis and systemic chemotherapy were performed.
  • After a 4-year disease-free period, there is still no sign of recurrence.
  • This case serves to demonstrate the effectiveness of radiotherapy for the treatment of lung metastasis from stromal sarcoma of the breast.
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans. Mastectomy, Segmental. Mesenchymoma / pathology. Treatment Outcome






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