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1. Grunewald TG, von Luettichau I, Weirich G, Wawer A, Behrends U, Prodinger PM, Jundt G, Bielack SS, Gradinger R, Burdach S: Sclerosing epithelioid fibrosarcoma of the bone: a case report of high resistance to chemotherapy and a survey of the literature. Sarcoma; 2010;2010:431627
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  • [Title] Sclerosing epithelioid fibrosarcoma of the bone: a case report of high resistance to chemotherapy and a survey of the literature.
  • Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites.
  • SEF represents a clinically challenging entity especially because no standardized treatment regimens are available.
  • The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction.
  • Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy.
  • Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma.
  • To date (22 months after diagnosis), there is no evidence of relapse or metastasis.
  • Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.

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  • (PMID = 20396630.001).
  • [ISSN] 1369-1643
  • [Journal-full-title] Sarcoma
  • [ISO-abbreviation] Sarcoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2853979
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2. Cecchetto G, Alaggio R, Dall'Igna P, Bisogno G, Ferrari A, Gigante C, Casanova M, Sotti G, Zanetti I, Carli M: Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer; 2005 Nov 1;104(9):2006-12
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  • [Title] Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.
  • BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity.
  • The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period.
  • Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy.
  • Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1.
  • Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors.
  • Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor.
  • CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS.
  • [MeSH-major] Extremities. Sarcoma / diagnosis
  • [MeSH-minor] Adolescent. Age Factors. Chemotherapy, Adjuvant. Child. Female. Humans. Italy. Male. Neoadjuvant Therapy. Survival Analysis

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  • [Copyright] (c) 2005 American Cancer Society.
  • (PMID = 16161038.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Erguvan-Onal R, Onal C, Gürlek A, Alkan A, Erkal HS, Mizrak B: Metastatic fibrosarcoma of the brain: transformation from conventional to epithelioid form--case report. Neurol Med Chir (Tokyo); 2004 Sep;44(9):497-501
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  • [Title] Metastatic fibrosarcoma of the brain: transformation from conventional to epithelioid form--case report.
  • A 45-year-old woman presented with an extremely rare metastatic fibrosarcoma of the brain manifesting as persistent headache.
  • She had undergone surgery for a fibrosarcoma of the soft tissue of the thigh 2 months earlier.
  • Computed tomography and scintigraphy showed multiple metastatic lesions of the lung.
  • She refused radiotherapy or chemotherapy.
  • Follow-up computed tomography showed multiple intracranial metastases.
  • [MeSH-major] Brain Neoplasms / secondary. Fibrosarcoma / secondary. Frontal Lobe / pathology
  • [MeSH-minor] Fatal Outcome. Female. Humans. Lung Neoplasms / secondary. Lung Neoplasms / surgery. Middle Aged. Neurosurgical Procedures / methods. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Tomography, X-Ray Computed

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  • (PMID = 15600287.001).
  • [ISSN] 0470-8105
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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4. Staals EL, Bacchini P, Bertoni F: Dedifferentiated central chondrosarcoma. Cancer; 2006 Jun 15;106(12):2682-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHODS: In this retrospective study, the clinical, radiographic, and histologic features and the treatments in 123 patients from the Rizzoli Institute were reviewed in an attempt to define which factors may be related to outcome in patients with dedifferentiated central chondrosarcoma.
  • Radiographically, a soft tissue mass was present in 87% of patients, and a bimorphic pattern was appreciated in 53% of patients.
  • In most patients, the dedifferentiated component showed the features of an osteosarcoma (92 patients), followed by fibrosarcoma (19 patients), and malignant fibrous histiocytoma (9 patients).
  • For 101 patients, surgery was a component of their definitive management.
  • In 25 patients, surgery was combined with chemotherapy.
  • CONCLUSIONS: Metastatic disease at diagnosis, malignant fibrous histiocytoma dedifferentiation, and a high percentage of dedifferentiated component were related to poorer outcomes.
  • There was no statistical evidence of any beneficial effect from adjuvant chemotherapy.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cell Differentiation. Combined Modality Therapy. Drug Therapy. Female. Femur / pathology. Histiocytoma, Malignant Fibrous / pathology. Histiocytoma, Malignant Fibrous / radiography. Histiocytoma, Malignant Fibrous / therapy. Humans. Humerus / pathology. Male. Middle Aged. Neoplasm Metastasis. Pelvis / pathology. Prognosis. Retrospective Studies. Survival Analysis. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2006 American Cancer Society.
  • (PMID = 16691621.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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7. Minard-Colin V, Orbach D, Martelli H, Bodemer C, Oberlin O: [Soft tissue tumors in neonates]. Arch Pediatr; 2009 Jul;16(7):1039-48

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Soft tissue tumors in neonates].
  • Soft tissue tumors account for approximately 25% of neonatal tumors and are most often benign (more than 2/3 of cases).
  • Malignant soft tissue tumors are, after neuroblastoma, the second cause of cancer in neonates.
  • Infantile fibrosarcoma (IF) is a rare tumor that most often affects the extremities of children aged 4 years or younger.
  • Chemotherapy is indicated when initial surgical removal cannot be accomplished without unacceptable morbidity.
  • Treatment is based on age-adapted chemotherapy and surgery.
  • [MeSH-major] Soft Tissue Neoplasms / congenital
  • [MeSH-minor] Fibrosarcoma / congenital. Fibrosarcoma / diagnosis. Fibrosarcoma / genetics. Fibrosarcoma / therapy. Gene Fusion / genetics. Gene Rearrangement / genetics. Hemangioendothelioma / congenital. Hemangioendothelioma / diagnosis. Hemangioendothelioma / therapy. Hemangioma / congenital. Hemangioma / diagnosis. Hemangioma / therapy. Humans. Infant, Newborn. Prognosis. Proto-Oncogene Proteins c-ets / genetics. Receptor, trkC / genetics. Repressor Proteins / genetics. Rhabdomyosarcoma / congenital. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy

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  • (PMID = 19398311.001).
  • [ISSN] 1769-664X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / ETS translocation variant 6 protein; 0 / Proto-Oncogene Proteins c-ets; 0 / Repressor Proteins; EC 2.7.10.1 / Receptor, trkC
  • [Number-of-references] 37
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8. López Almaraz R, Villafruela Alvarez C, Rodríguez Luis J, Doménech Martínez E: [Neonatal neoplasms: a single-centre experience]. An Pediatr (Barc); 2006 Dec;65(6):529-35

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVES: To describe the neoplasms diagnosed and treated in newborns (</= 28 days of life) in the Hospital Universitario de Canarias and their association with congenital abnormalities and to evaluate prenatal diagnosis of these tumors.
  • The variables analyzed were the percentage of neonatal neoplasms among the total number of cancer cases in children aged less than 14 years, their incidence among all the newborns in our hospital, sex, year of diagnosis, age at clinical diagnosis, the presence or absence of prenatal diagnosis, type of tumor (histologic diagnosis), association with syndromes or other congenital anomalies, treatment, and long-term outcome.
  • Males accounted for 43.8 % and females for 56.2 %, with a mean age at diagnosis of 5.5 days (range 1-28 days).
  • Five neonates (31.2 %) had a prenatal diagnosis, 60 % of which were made in the last 7 years of the study period.
  • Histologic diagnoses were neuroblastoma (n = 5; 31.2 %), teratoma/ germ cell tumor (n = 4; 25 %), soft tissue sarcoma (one fibrosarcoma of the thigh and two hemangiopericytoma of the back and heart; 18.8 %), and one case each of mesoblastic nephroma, cerebral tumor (ependymoblastoma), melanoma (associated with giant congenital melanocytic nevi), and acute leukemia (associated with Down syndrome).
  • Treatment consisted of surgery alone (n = 10; 62.5 %) and surgery plus chemotherapy (n = 5; 31.2 %); one patient received no treatment.
  • In the last 7 years, the prenatal diagnosis of these entities has improved.
  • Most of the neoplasms responded to therapy, mainly surgery, and long-term outcome was favorable.

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  • [CommentIn] An Pediatr (Barc). 2007 Jul;67(1):85-6 [17663916.001]
  • (PMID = 17194321.001).
  • [ISSN] 1695-4033
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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9. Miettinen M: From morphological to molecular diagnosis of soft tissue tumors. Adv Exp Med Biol; 2006;587:99-113
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  • [Title] From morphological to molecular diagnosis of soft tissue tumors.
  • Cytogenetic discoveries of balanced translocations in soft tissue tumors have opened the way to molecular genetic definition of these translocations as gene fusions from the late 1980s.
  • Many sarcomas are known to have such fusions, and the demonstration of the fusion transcripts in tumor tissue is of great value in specific diagnosis of synovial sarcoma (SYT-SSX), Ewing sarcoma (EWS-Fli1), clear cell sarcoma (EWS-ATF1), myxoid liposarcoma (FUS-CHOP), and other sarcomas.
  • Demonstration of SYT-SSX and EWS-ATF1 fusion assists in the diagnosis of synovial and clear cell sarcomas in unusual locations, such as the gastrointestinal tract, where these tumors occur with low frequency.
  • In two exceptional instances, the same translocation and gene fusion occurs in two unrelated diseases: ETV6-NTRK fusion in infantile fibrosarcoma and secretory carcinoma of the breast, and ALK-TPM3 fusion in inflammatory myofibroblastic tumor and large cell anaplastic lymphoma.
  • Activating mutations in two related receptor tyrosine kinases (RTKs), KIT, and platelet-derived growth factor receptor alpha (PDGFRA) is central to the pathogenesis of gastrointestinal stromal tumors (GISTs), and countering the mutational activation by specific tyrosine kinase inhibitors, such as Imatinib mesylate, is now standard treatment for metastatic GISTs.
  • Mutation type influences therapy responsiveness, but fortunately very few GISTs carry primarily Imatinib-resistant mutations.
  • Secondary drug resistance acquired during Imatinib treatment based on new, Imatinib-resistant mutations is a major problem limiting treatment success.
  • Schwannoma types may differ in their pathogenesis: gastrointestinal schwannomas lack NF2 changes suggesting a different pathogenesis.
  • [MeSH-major] Genetic Testing. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / pathology

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  • (PMID = 17163160.001).
  • [ISSN] 0065-2598
  • [Journal-full-title] Advances in experimental medicine and biology
  • [ISO-abbreviation] Adv. Exp. Med. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 70
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10. Tsuchido K, Yamada M, Satou T, Otsuki Y, Shimizu S, Kobayashi H: Cytology of sclerosing epithelioid fibrosarcoma in pleural effusion. Diagn Cytopathol; 2010 Oct;38(10):748-53
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  • [Title] Cytology of sclerosing epithelioid fibrosarcoma in pleural effusion.
  • We report a case of sclerosing epithelioid fibrosarcoma focusing on its cytological features in pleural effusion.
  • A 32-year-old man had noticed a tumor in his left buttock 5 years earlier but had not sought treatment because the tumor had been painless.
  • The histological diagnosis was sclerosing epithelioid fibrosarcoma with a negative surgical margin.
  • A histological examination revealed a metastasis of sclerosing epithelioid fibrosarcoma, and chemotherapy was initiated.
  • Pleural disseminations were detected 4 years after the chemotherapy, and the pleural effusion was sampled for cytological examination.
  • The above cytological features, coupled with the clinical findings, enabled a diagnosis of sclerosing epithelioid fibrosarcoma.
  • To our knowledge, this is the first cytological description of sclerosing epithelioid fibrosarcoma.
  • [MeSH-major] Cytodiagnosis. Fibrosarcoma / secondary. Pleural Effusion, Malignant / pathology
  • [MeSH-minor] Adult. Buttocks / pathology. Epithelioid Cells / pathology. Humans. Immunohistochemistry. Lung Neoplasms / secondary. Male. Sclerosis / pathology. Soft Tissue Neoplasms / secondary

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  • [Copyright] © 2010 Wiley-Liss, Inc.
  • (PMID = 20091700.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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11. Cecchetto G, Carli M, Alaggio R, Dall'Igna P, Bisogno G, Scarzello G, Zanetti I, Durante G, Inserra A, Siracusa F, Guglielmi M, Italian Cooperative Group: Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979-1995). J Surg Oncol; 2001 Dec;78(4):225-31
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  • [Title] Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979-1995).
  • BACKGROUND AND OBJECTIVES: Fibrosarcoma is a rare soft tissue sarcoma (STS) that has two peaks of incidence in pediatric patients: one in infants and young children (infantile fibrosarcoma), another in older children ("adult type" fibrosarcoma).
  • The purpose of this study was to describe the clinical features and the treatment results in patients affected by fibrosarcoma enrolled in two consecutive studies run by the STS-Italian Cooperative Group (ICG) between 1979 and 1995.
  • The cut-off point between the two forms was considered the age of 2 years: 14 patients were affected by infantile and 11 by adult type of fibrosarcoma.
  • The therapeutic guidelines were not strict and not different for the two forms: patients with initial macroscopic excision (Gr I-II) were given IVA or VAC; Gr III had VAC or VAIA and subsequent excision, if feasible.
  • Radiation therapy (RT) was delivered in patients > 3 years with microscopic (42 Gy) and macroscopic (54 Gy) residuals.
  • One Gr III patient was lost at follow up, 2 years from diagnosis.
  • CONCLUSIONS: The complete excision at diagnosis was the treatment of choice and was related to the best outcome.
  • Microscopical residuals were difficult to treat with chemo-radiotherapy in both forms of fibrosarcoma.
  • Neoadjuvant chemotherapy (CT) obtained a partial remission (PR) only in three of eight cases, while no conclusions concerning the efficacy of CT for infantile forms are possible.
  • Patients < 2 years had a better outcome than the older ones: most of them had a tumor on extremities which was excised at diagnosis.
  • [MeSH-major] Fibrosarcoma / radiotherapy. Soft Tissue Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Age Factors. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Radiotherapy Dosage. Sarcoma / radiotherapy. Sarcoma / surgery. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2001 Wiley-Liss, Inc.
  • (PMID = 11745814.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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12. Méndez R, Arnáiz S, Montero M, Tellado M, País E, Ríos J, Vela D: [Clinical patterns of soft-tissue sarcoma in children]. Cir Pediatr; 2001 Jan;14(1):14-20
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  • [Title] [Clinical patterns of soft-tissue sarcoma in children].
  • INTRODUCTION AND OBJECTIVES: Soft tissue sarcomas are rare mesenchymal neoplasms that constitute less than 10% of all pediatric malignancies.
  • Half of these are rhabdomyosarcomas, the remaining 50% have a varied and heterogenous histologic and clinical patterns (fibrosarcoma, synovial cell sarcoma, extraskeletal Ewing's sarcoma, angiosarcoma, liposarcoma, leiomyosarcoma, ...).
  • The purpose of this work is to evaluate our clinical experience with soft tissue sarcomas in uncommon sites over the past 10 years in order to delimitate the prognostic factors in survival and modalities of treatment.
  • MATERIAL AND METHODS: Between 1989 and 1998, 10 patients were diagnosed with soft tissue sarcomas in uncommon sites and treated by us over a total number of 139 pediatric neoplasms (7.19%).
  • Variables investigated included histologic findings, tumor size, age at presentation, primary site, clinical group, radiologic test performed, surgical treatment, radiotherapy and adjuvant chemotherapy, complications and survival rates.
  • RESULTS: The following histologic types of these 10 tumors were identified: 1 hemangiopericytoma in oral cavity, 2 extraosseous Ewing's sarcoma, 1 botryoid rhabdomyosarcoma of the bladder, 1 mediastinal fibrosarcoma, 1 retroperitoneal rhabdomyosarcoma, 1 paratesticular rhabdomyosarcoma, 1 cervical condrosarcoma, 1 alveolar rhabdomyosarcoma and 1 deltoid rhabdomyosarcoma.
  • The mean age at diagnosis was 7 years (4.6 years accounted for rhabdomyosarcoma alone).
  • Adjuvant chemotherapy with IVA was followed by radiotherapy only in four patients.
  • All the children classified in clinical groups II, III or IV needed 2nd. line regimens of chemotherapy.
  • Three patients died in the follow-up instead of the multimodal treatment.
  • 2) radiotherapy will only be necessary if margins of resection cannot control the local disease, and 3) chemotherapy have not clearly demonstrated his benefits as adjuvant therapy in clinical group I lesions but his employ is recommended in all cases because of the poor prognosis due to local recurrence.
  • [MeSH-major] Sarcoma / therapy. Soft Tissue Neoplasms / therapy

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  • (PMID = 11339112.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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13. Antonescu CR, Rosenblum MK, Pereira P, Nascimento AG, Woodruff JM: Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor. Am J Surg Pathol; 2001 Jun;25(6):699-709
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor.
  • Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor of deep soft tissues, originally described in 1995 by Meis-Kindblom et al.
  • Treatment consisted of intralesional excision (n = 2), attempted wide local excision (n = 11), and amputation (n = 3), with either adjuvant radiation therapy (n = 9) or chemotherapy (n = 3).
  • Eight patients (57%) died of disease 16 to 86 months after diagnosis.
  • [MeSH-major] Fibrosarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 11395547.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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14. Stachowicz-Stencel T, Bień E, Stefanowicz J, Połczytńska K, Sierota D, Szołkiewicz A, Drozyńska E, Kosiak W, Stankiewicz C, Pietniczka M, Kukwa A, Czauderna P, Balcerska A: [Diagnostic and therapeutic difficulties in soft tissue sarcomas localized in nonparameningeal head and neck region--own experiences]. Med Wieku Rozwoj; 2005 Jul-Sep;9(3 Pt 2):487-94
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  • [Title] [Diagnostic and therapeutic difficulties in soft tissue sarcomas localized in nonparameningeal head and neck region--own experiences].
  • INTRODUCTION: Soft tissue sarcomas (MTM) localized within the nonorbital and non-parameningeal head and neck region in children are associated with favourable prognosis.
  • However in our material we have observed many therapeutic failures in this group of patients.
  • The aim of the study was to analyze the reasons for disappointing results of oncological therapy in children with MTM treated between 1992 and 2004.
  • Five patients were diagnosed with rhabdomyosarcoma, four--with non-rhlabdomyosarcoma, including: angiosarcoma, malignant triton tumour, fibrosarcoma and leiomyosarcoma.
  • In as many as five of nine patients initially a false histopathological diagnosis was made based on material obtained from aspiration biopsy of the tumour performed in non-oncological centres.
  • This resulted in a significant delay of the proper diagnosis of malignant disease ranging from 2 to 15 months (mean 7 months).
  • Therapy was conducted according to the schemes: MMT-89, CWS-91, CWS-96 and CWS-2002.
  • Durable complete remission after the first line therapy was obtained in one child only.
  • Six patients developed MTM relapse and two--progression during chemotherapy.
  • Finally five children remain disease-free after treatment termination with follow-up of 1 to 1,5 years.
  • In three of them the proper diagnosis was delayed significantly and they were diagnosed in the first or even second relapse of the tumour.
  • CONCLUSION: Unfavourable course and treatment results in MTM located in nonorbital and nonparameningeal head and neck region in our patients result from initial wrong histopathological diagnosis and delayed therapy institution.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / therapy. Sarcoma / diagnosis. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Diagnosis, Differential. Female. Fibrosarcoma / diagnosis. Fibrosarcoma / therapy. Hemangiosarcoma / diagnosis. Hemangiosarcoma / therapy. Humans. Infant. Leiomyosarcoma / diagnosis. Leiomyosarcoma / therapy. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Treatment Outcome

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  • (PMID = 16719161.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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15. Antoniello L, Cecchetto G, Carli M, Dall'Igna P, Bisogno G, Lo Piccolo R, Gigante C, Zanetti I, Guglielmi M: [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88]. Pediatr Med Chir; 2003 Jul-Aug;25(4):255-60
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  • [Title] [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88].
  • Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies.
  • HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10.
  • Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST.
  • The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases.
  • It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88).
  • OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study.
  • CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients.
  • [MeSH-major] Sarcoma / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 15070267.001).
  • [ISSN] 0391-5387
  • [Journal-full-title] La Pediatria medica e chirurgica : Medical and surgical pediatrics
  • [ISO-abbreviation] Pediatr Med Chir
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
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16. Alaggio R, Ninfo V, Rosolen A, Coffin CM: Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases. Am J Surg Pathol; 2006 Mar;30(3):388-94

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma.
  • In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis.
  • PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass.
  • Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy.
  • One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up.
  • The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.
  • [MeSH-major] Fibrosarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Dermatofibrosarcoma / pathology. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Infant. Infant, Newborn. Male. Microscopy, Electron, Transmission. Neoplasm Recurrence, Local / pathology. Nerve Sheath Neoplasms / pathology. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16538060.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ETV6-NTRK3 fusion protein, human; 0 / Oncogene Proteins, Fusion
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17. Bilsky MH, Schefler AC, Sandberg DI, Dunkel IJ, Rosenblum MK: Sclerosing epithelioid fibrosarcomas involving the neuraxis: report of three cases. Neurosurgery; 2000 Oct;47(4):956-9; discussion 959-60
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  • OBJECTIVE AND IMPORTANCE: Sclerosing epithelioid fibrosarcoma (SEF) is a rare mesenchymal neoplasm composed of rounded, vimentin-immunoreactive tumor cells disposed in nests and cords within a hyalinized collagenous matrix.
  • CLINICAL PRESENTATION: Two tumors had intracranial, calvarial and extracalvarial, soft-tissue components, whereas the third tumor manifested as a paraspinal mass with extension into the T12-L1 neural foramen and invasion of the T12 nerve root.
  • In no case was there a response to adjuvant chemotherapy or radiotherapy.
  • [MeSH-major] Brain Neoplasms / diagnosis. Fibrosarcoma / diagnosis. Spinal Cord Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Neoplasm Invasiveness. Sclerosis. Tomography, X-Ray Computed

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  • (PMID = 11014436.001).
  • [ISSN] 0148-396X
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
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18. Nascimento AF, Bertoni F, Fletcher CD: Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. Am J Surg Pathol; 2007 Jan;31(1):99-105
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Myxofibrosarcoma (MFS) is one of the most common soft tissue sarcomas of elderly patients and has a predilection for the limbs.
  • In 10 cases, the tumor was located in subcutaneous tissue and in 6 cases it was subfascial.
  • Twelve patients were treated by surgery followed by chemotherapy and/or radiation (8 cases).
  • One patient received chemotherapy after an incisional biopsy and 1 patient was treated by surgery alone.
  • Ten patients (71.4%) developed local recurrences.
  • Seven patients (50%) developed metastases to lungs or retroperitoneum.
  • Differential diagnosis included carcinoma, melanoma, myoepithelial carcinoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma.
  • [MeSH-major] Epithelioid Cells / pathology. Fibrosarcoma / secondary. Sarcoma / secondary. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / analysis. Carcinoma / diagnosis. Combined Modality Therapy. Diagnosis, Differential. Extremities. Fatal Outcome. Female. Humans. Immunoenzyme Techniques. Liposarcoma / diagnosis. Male. Melanoma / diagnosis. Middle Aged. Myoepithelioma / diagnosis. Rhabdomyosarcoma / diagnosis

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  • (PMID = 17197925.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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19. Gugatschka M, Beham A, Stammberger H, Schmid C, Friedrich G: First case of a myxofibrosarcoma of the vocal folds: case report and review of the literature. J Voice; 2010 May;24(3):374-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • These tumors represent a distinct clinicopathologic entity and are fibroblast-derived soft-tissue neoplasms usually arising in late adulthood.
  • Surgery is the most common form of therapy, only a limited number of papers dealing with radio/chemotherapy exist due to the small number of cases.
  • [MeSH-major] Fibrosarcoma / pathology. Fibrosarcoma / surgery. Laryngeal Neoplasms / pathology. Laryngeal Neoplasms / surgery. Vocal Cords / pathology. Vocal Cords / surgery
  • [MeSH-minor] Aged. Diagnosis, Differential. Endoscopy. Humans. Male. Treatment Outcome

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  • [Copyright] (c) 2010 The Voice Foundation. Published by Mosby, Inc. All rights reserved.
  • (PMID = 19664897.001).
  • [ISSN] 1873-4588
  • [Journal-full-title] Journal of voice : official journal of the Voice Foundation
  • [ISO-abbreviation] J Voice
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 12
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20. Chastagner P: [Malignant extraconal tumors of the orbit in childhood]. Neurochirurgie; 2010 Apr-Jun;56(2-3):281-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Both diagnosis and treatment must be undertaken as soon as possible.
  • Sometimes diagnosis should be clear with the association of an orbital tumor and deterioration of the general health status favoring metastatic disease.
  • Today both CT and MRI are highly valuable in assessing the diagnosis and starting the management of these tumors.
  • Biopsy is mandatory to confirm the diagnosis.
  • Among the primitive tumors, soft tissue sarcomas, especially rhabdomyosarcomas, are the most frequent.
  • The diagnosis is suggested when the onset of the disease is acute and the course is rapid.
  • Most respond to neoadjuvant chemotherapy.
  • In the event of a residual tumor, local treatment is indicated so that surgery and/or radiotherapy are used as second-line treatment.
  • It can be satisfactory (Langerhans' cell histiocytosis, lymphoma, meningioma, infantile fibrosarcoma) or poor (metastatic tumor, rhabdoid tumor).
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Exophthalmos / etiology. France. Humans. Incidence. Infant. Male. Neoplasm Metastasis. Prognosis. Rhabdomyosarcoma / epidemiology. Rhabdomyosarcoma / surgery. Tomography, X-Ray Computed

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  • [Copyright] Copyright 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20303550.001).
  • [ISSN] 1773-0619
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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21. Albert A, Cruz O, Montaner A, Vela A, Badosa J, Castañón M, Morales L: [Congenital solid tumors. A thirteen-year review]. Cir Pediatr; 2004 Jul;17(3):133-6
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  • This is an interesting group of tumors because their type, relative incidence, natural history and response to treatment differ from those seen in older children.
  • There were 8 teratomas (3 sacrocoxigeal, 1 retroperitoneal, 1 in the CNS, 1 orbitary and two oronasal), two hepatic tumors (1 hepatoblastoma, 1 hemangioendothelioma, two CNS tumors, two giant nevus (one on a hamartoma), and one each Wilms tumor, infantile fibrosarcoma and myofibroblastic tumor.
  • Treatment was surgical resection alone in 17 cases (68%) and surgery + chemotherapy in 8 (32%) (5 neuroblastomas, one CNS tumor, one Wilms tumor and one presacral teratoma who developed a yolk sac tumor); 3 patients died (11%): one at surgery, one of tumoural airway obstruction at birth and one with craniopharyngioma.
  • Among the 14 tumors that were initially not malignant, two can be locally agressive, one was an immature teratoma, the giant nevus with hamartoma developed in situ melanoma, the other nevus had meningeal melanosis with hydrocephalus, and one mature presacral teratoma developed a yolk sac tumor.
  • CONCLUSIONS: Diagnosis of congenital tumors is performed earlier in recent years due to the wide use of prenatal ultrasound.
  • Complete surgical excision is the treatment of choice, most cases not need adjuvant chemotherapy.
  • We ought to pass this message on to our colleagues in prenatal diagnosis, so parents get reliable information.
  • [MeSH-major] Central Nervous System Neoplasms / congenital. Kidney Neoplasms / congenital. Liver Neoplasms / congenital. Neuroblastoma / congenital. Skin Neoplasms / congenital. Soft Tissue Neoplasms / congenital. Teratoma / congenital. Wilms Tumor / congenital
  • [MeSH-minor] Female. Follow-Up Studies. Humans. Infant, Newborn. Male. Neoplasm Recurrence, Local. Postoperative Complications. Pregnancy. Prenatal Diagnosis. Time Factors

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  • (PMID = 15503950.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Spain
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22. Kebudi R, Görgün O, Ayan I: Oral etoposide for recurrent/progressive sarcomas of childhood. Pediatr Blood Cancer; 2004 Apr;42(4):320-4
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  • PROCEDURE: Twenty-one children (10 girls and 11 boys) with R/P sarcomas and a median age of 11 years (range 3-16 years) were enrolled in this study.
  • The diagnosis was Ewing sarcoma family tumor (ESFT) in seven, osteosarcoma in eight, rhabdomyosarcoma in four, clear cell sarcoma of soft tissue in one, fibrosarcoma in one patient.
  • They are alive with no evidence of disease (NED) 79 and 94 months from time of relapse/progressive disease (PD).
  • A patient developed acute myeloid leukemia and died.
  • CONCLUSIONS: Oral VP-16 therapy is simple, relatively nontoxic, and does not necessitate hospitalization.
  • Given the risk of second malignancy, especially in children with previous exposure to topoisomerase II inhibitors and alkylating agents, this regimen may be used as a palliative treatment or in patients with poor prognosis.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / administration & dosage. Etoposide / administration & dosage. Sarcoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Child. Child, Preschool. Disease Progression. Female. Humans. Male. Neoplasms, Second Primary / chemically induced. Palliative Care. Recurrence. Remission Induction. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 14966827.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide
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23. Lowichik A, Zhou H, Pysher TJ, Smith L, Lemons R, Coffin CM: Therapy associated changes in childhood tumors. Adv Anat Pathol; 2000 Nov;7(6):341-59
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  • [Title] Therapy associated changes in childhood tumors.
  • Contemporary treatment regimens for the common solid tumors of childhood have led to increased numbers of post-treatment pathologic specimens from survivors.
  • Current therapeutic strategies for childhood cancers in North America require an accurate pathologic diagnosis and stratify patients based on combinations of clinical, biological, and pathologic features.
  • In several tumor systems, the pathologic response to therapy also modifies the treatment regimen.
  • Accurate pathologic interpretation of such specimens is critical in providing useful prognostic information for therapeutic decisions.
  • Standardized handling of post-therapy pathologic specimens, appropriate use of molecular and genetic studies, consideration of the differential diagnoses, and assessment of the potential biologic significance of therapy-induced pathologic changes are, therefore, critical for patient management and determination of treatment protocols.
  • [MeSH-minor] Adolescent. Bone Neoplasms / drug therapy. Bone Neoplasms / pathology. Bone Neoplasms / surgery. Child. Child, Preschool. Fibrosarcoma / pathology. Fibrosarcoma / therapy. Hepatoblastoma / pathology. Hepatoblastoma / therapy. Histocytochemistry. Humans. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Liver Neoplasms / pathology. Liver Neoplasms / therapy. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Osteosarcoma / pathology. Osteosarcoma / surgery. Prognosis. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / surgery. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / drug therapy. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Wilms Tumor / drug therapy. Wilms Tumor / pathology. Wilms Tumor / surgery

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  • (PMID = 11078058.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 149
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