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1. Nunes LF, Fiod NJ, Vasconcelos RA, Meohas W, Rezende JF: [Epithelioid sarcoma: clinical behavior, prognostic factors and survival]. Rev Col Bras Cir; 2010 Aug;37(4):251-5
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  • [Title] [Epithelioid sarcoma: clinical behavior, prognostic factors and survival].
  • [Transliterated title] Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida.
  • OBJECTIVE: To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas.
  • METHODS: Careful analysis of 25 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between june 1987 and july 2005.
  • The primary site of presentation was the upper extremity in twelve patients (48%).
  • Six received any type of chemotherapy and 14 received treatment with radiotherapy with mean dose of 46,5 Gy.
  • Six patients underwent cancer treatment in its entirety at the National Institute of Cancer.
  • CONCLUSION: Epithelioid sarcoma is a rare subset of soft tissue sarcoma with high rate of local recurrence, regional node and distant metastases.
  • Surgical treatment of epithelioid sarcoma consists of early wide local resection to negative microscopic margins.
  • [MeSH-major] Sarcoma / diagnosis. Sarcoma / mortality. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / mortality

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  • (PMID = 21085839.001).
  • [ISSN] 1809-4546
  • [Journal-full-title] Revista do Colégio Brasileiro de Cirurgiões
  • [ISO-abbreviation] Rev Col Bras Cir
  • [Language] por
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Brazil
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2. Kudva R, Perveen S, Janardhana A: Primary epithelioid angiosarcoma of bone: a case report with immunohistochemical study. Indian J Pathol Microbiol; 2010 Oct-Dec;53(4):811-3
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  • [Title] Primary epithelioid angiosarcoma of bone: a case report with immunohistochemical study.
  • Primary malignant vascular tumors of the bone are exceedingly rare and represent <1% of primary malignant bone tumors.
  • Epithelioid angiosarcoma (EA) is a rare variant of angiosarcoma that is characterized by large cells with an epithelioid morphology.
  • Here, we present a case of a 62-year-old man who had primary EA of the left tibia.
  • He was treated with amputation and chemotherapy.
  • After 1 month of chemotherapy, he developed pleural effusion and died.
  • [MeSH-major] Bone Neoplasms / diagnosis. Bone Neoplasms / pathology. Hemangiosarcoma / diagnosis. Hemangiosarcoma / pathology. Sarcoma / diagnosis. Sarcoma / pathology. Tibia / pathology
  • [MeSH-minor] Amputation. Antigens, CD34 / analysis. Antineoplastic Agents / therapeutic use. Drug Therapy / methods. Fatal Outcome. Histocytochemistry. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Microscopy. Middle Aged

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  • (PMID = 21045426.001).
  • [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 / Antigens, CD34; 0 / Antineoplastic Agents
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3. Kato K, Arai K, Tanaka Y, Ijiri R, Kato Y, Kigasawa H, Toyoda Y, Aida N, Ohama Y: Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors". Mod Pathol; 2000 Oct;13(10):1156-60
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  • [Title] Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors".
  • We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass.
  • The age, clinical presentation, and histologic findings mostly favored Ewing's sarcoma/primitive neuroectodermal tumor.
  • Silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells.
  • The diagnosis of epithelioid leiomyosarcoma was made.
  • Following reduction in tumor size by chemotherapy, the serum NSE level was normalized.
  • From the surgical finding, the primary site was presumed to be the urachus or the urinary bladder dome.
  • Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors. "
  • [MeSH-major] Abdominal Neoplasms / pathology. Immunocompromised Host. Leiomyosarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Humans. Infant. Magnetic Resonance Imaging

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  • (PMID = 11048812.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
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4. Wolf PS, Flum DR, Tanas MR, Rubin BP, Mann GN: Epithelioid sarcoma: the University of Washington experience. Am J Surg; 2008 Sep;196(3):407-12
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  • [Title] Epithelioid sarcoma: the University of Washington experience.
  • BACKGROUND: Epithelioid sarcoma is a rare sarcoma with a high local recurrence rate that frequently metastasizes to lymph nodes.
  • We reviewed our experience with adjuvant therapy in patients with this disease.
  • METHODS: Between 1990 and 2003, we treated 11 patients with epithelioid sarcoma.
  • Patient, tumor, and treatment characteristics were analyzed, and effect of treatment on survival was evaluated by the Kaplan-Meier method.
  • Five patients developed nodal disease.
  • All patients underwent surgery for the primary tumor, and 7 patients had nodal evaluation.
  • Ten patients underwent adjuvant chemotherapy, and 9 underwent radiotherapy.
  • Recurrence developed in 9 patients.
  • Chemotherapy and radiation therapy did not impact disease-free survival.
  • CONCLUSIONS: Although surgery remains the primary treatment modality, multi-institutional trials are needed to develop more effective adjuvant therapy for patients with epithelioid sarcoma.
  • [MeSH-major] Sarcoma / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Antineoplastic Agents / therapeutic use. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Radiotherapy, Adjuvant. Retrospective Studies. Survival Analysis. Washington

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  • (PMID = 18436180.001).
  • [ISSN] 1879-1883
  • [Journal-full-title] American journal of surgery
  • [ISO-abbreviation] Am. J. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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5. Abdulkader I, Cameselle-Teijeiro J, Fraga M, Caparrini A, Forteza J: Sclerosing epithelioid fibrosarcoma primary of the bone. Int J Surg Pathol; 2002 Jul;10(3):227-30
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  • [Title] Sclerosing epithelioid fibrosarcoma primary of the bone.
  • Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor originally described in soft tissues.
  • We report a case of SEF primary of the left iliac bone in a 42-year-old woman.
  • [MeSH-major] Bone Neoplasms / pathology. Fibrosarcoma / secondary. Sarcoma / secondary
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Drug Therapy. Female. Humans. Ilium. Immunohistochemistry. Sclerosis / pathology. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 12232581.001).
  • [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
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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6. Kurtkaya-Yapícíer O, Scheithauer BW, Dedrick DJ, Wascher TM: Primary epithelioid sarcoma of the dura: case report. Neurosurgery; 2002 Jan;50(1):198-202; discussion 202-3
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  • [Title] Primary epithelioid sarcoma of the dura: case report.
  • OBJECTIVE AND IMPORTANCE: Epithelioid sarcomas are rare mesenchymal neoplasms that occur most often in the extremities of young adults.
  • Despite isolated reports of epithelioid sarcomas arising in the head and neck region, these lesions have not been described previously, to our knowledge, in the central nervous system.
  • CLINICAL PRESENTATION: We present the case of an 18-year-old woman with a unique dural sarcoma that arose in the right frontotemporal region.
  • As visualized on magnetic resonance imaging studies, the 4.5-cm tumor focally traversed the cranium to penetrate the galea, the temporal muscle, and subcutaneous tissue.
  • INTERVENTION: Despite gross total removal and postoperative radiotherapy (59 Gy), a large recurrence was noted 5 months after surgery.
  • Histologically, the partly necrotic tumor consisted of epithelioid and spindle cells showing widespread vimentin and variable cytokeratin as well as epithelial membrane antigen immunoreactivity.
  • CONCLUSION: With the inclusion of epithelioid sarcoma, the spectrum of central nervous system sarcomas continues to expand.
  • [MeSH-major] Dura Mater / surgery. Meningeal Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / analysis. Chemotherapy, Adjuvant. Combined Modality Therapy. Female. Humans. Magnetic Resonance Imaging. Microscopy, Electron. Neoplasm Recurrence, Local / drug therapy. Radiotherapy, Adjuvant. Tomography, X-Ray Computed

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  • (PMID = 11852861.001).
  • [ISSN] 0148-396X
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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7. Fleshman R, Mayerson J, Wakely PE Jr: Fine-needle aspiration biopsy of high-grade sarcoma: a report of 107 cases. Cancer; 2007 Dec 25;111(6):491-8
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  • [Title] Fine-needle aspiration biopsy of high-grade sarcoma: a report of 107 cases.
  • BACKGROUND: To the authors' knowledge, few studies exist demonstrating the reliability of fine-needle aspiration (FNA) biopsy for high-grade sarcoma (HGS).
  • They also searched their tissue database for all HGS cases that had prior FNA biopsy findings.
  • Fifty-four cases were diagnosed as HGS, not otherwise specified, 8 as myxofibrosarcoma, 8 as osteosarcoma, 5 as malignant peripheral nerve sheath tumor, 5 as leiomyosarcoma, 4 as Ewing sarcoma, 4 as liposarcoma, 2 as epithelioid sarcoma, and 1 as angiosarcoma.
  • Approximately 71% of patients presented with a primary tumor, 23% with disease recurrence, and 7% with metastasis.
  • Sixteen of 19 patients received neoadjuvant chemotherapy based on the FNA diagnosis alone.
  • [MeSH-major] Biopsy, Fine-Needle. Cytodiagnosis. Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Bone Neoplasms / diagnosis. Female. Humans. Male. Middle Aged. Neoadjuvant Therapy. Predictive Value of Tests. Reproducibility of Results

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  • (PMID = 17941014.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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8. Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N: Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol; 2008 Sep;39(9):1385-95
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  • [Title] Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival.
  • Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm.
  • There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas.
  • Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation.
  • Tumor grade appeared to be prognostically important in cardiac sarcoma.
  • [MeSH-major] Heart Neoplasms / pathology. Sarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Male. Middle Aged. Survival Analysis

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  • (PMID = 18602663.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / R01 HL063954
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS522251; NLM/ PMC4081532
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9. Del Frari B, Zelger BG, Piza-Katzer H: [Epithelioid sarcoma of the hand, a seldomly recognized tumor]. Handchir Mikrochir Plast Chir; 2004 Oct;36(5):313-7
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  • [Title] [Epithelioid sarcoma of the hand, a seldomly recognized tumor].
  • BACKGROUND: The epithelioid sarcoma of the hand is often misdiagnosed.
  • PATIENTS AND METHOD: Three young patients (one male and two female) were operated on the hand for treatment of Dupuytren's contracture, synovialoma and fibromas, respectively.
  • The necessity for surgical revision in these patients led to a reevaluation of the primary histological data.
  • The original diagnosis having been found to be incorrect, the correct diagnosis was established namely, epithelioid sarcoma.
  • None of the patients underwent adjuvant radio- or chemotherapy.
  • Adequate treatment requires early radical excision; amputation may be required if the primary tumor is located in the fingers or treatment of recurrent growth.
  • Patients must be monitored with yearly lung X-rays since recurrence or metastasis may occur many years after the initial diagnosis and treatment.
  • [MeSH-major] Hand / surgery. Sarcoma / diagnosis. Sarcoma / surgery. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adult. Amputation. Biopsy. Child. Connective Tissue / pathology. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Male. Muscle, Skeletal / pathology. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / surgery

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  • (PMID = 15503263.001).
  • [ISSN] 0722-1819
  • [Journal-full-title] Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Mikrochirurgie der Peripheren Nerven und Gefässe : Organ der Vereinigung der Deutschen Plastischen Chirurgen
  • [ISO-abbreviation] Handchir Mikrochir Plast Chir
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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10. Kao SY, Tu HF, Chang KW, Chang CS, Yang AH, Li WY: Epithelioid sarcoma metastasis to the gingivae: a case report. Int J Oral Maxillofac Surg; 2004 Mar;33(2):205-8
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  • [Title] Epithelioid sarcoma metastasis to the gingivae: a case report.
  • We present a rare case of oral metastatic epithelioid sarcoma rapidly growing over the mandibular gingivae; the primary lesion occurred on the wrist and was treated 18 months earlier by surgery and radiotherapy.
  • The oral metastatic lesion was resected and controlled by chemotherapy.
  • This case has been followed for 2 years with good control of the resected oral metastatic lesion.
  • Histologically, round to oval-shaped tumour cells with abundant eosinophylic globular cytoplasm and eccentrically localized nuclei, lack of epithelial features by electron microscopic study, and the immunohistochemical and cytologic features of tumour cells led into the diagnosis of epithelioid sarcoma.
  • [MeSH-major] Gingival Neoplasms / secondary. Sarcoma / secondary. Skin Neoplasms / pathology

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  • (PMID = 15050080.001).
  • [ISSN] 0901-5027
  • [Journal-full-title] International journal of oral and maxillofacial surgery
  • [ISO-abbreviation] Int J Oral Maxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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11. Coulibaly B, Tasei AM, Payan-Defais MJ, Bouvier C, Trousse D, Doddoli C, Figarella-Branger D: [Pulmonary epithelioid haemangioendothelioma: two different clinical courses]. Rev Mal Respir; 2008 Sep;25(7):867-70
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  • [Title] [Pulmonary epithelioid haemangioendothelioma: two different clinical courses].
  • [Transliterated title] Hémangioendothéliome épithélioïde pulmonaire: à propos de deux observations.
  • In both cases, chest radiography and thoracic CT scans showed multiple, bilateral pulmonary nodules, bronchial endoscopy was normal and surgical biopsy revealed epithelioid haemangioendothelioma.
  • It is a rare primary pulmonary tumour which is usually found in soft tissue, bone or liver.
  • Epithelioid haemangioendothelioma is a vascular tumour usually affecting women less than 40 years of age.
  • Chemotherapy and radiotherapy are generally ineffective and surgery is sometimes impossible because of the multifocal lesions.
  • Epithelioid haemangioendothelioma is considered a sarcoma without precise histological prognostic criteria.
  • [MeSH-major] Hemangioendothelioma, Epithelioid. Lung Neoplasms
  • [MeSH-minor] Biopsy. Female. Follow-Up Studies. Humans. Lung / pathology. Male. Middle Aged. Radiography, Thoracic. Time Factors. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

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  • (PMID = 18946414.001).
  • [ISSN] 0761-8425
  • [Journal-full-title] Revue des maladies respiratoires
  • [ISO-abbreviation] Rev Mal Respir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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12. Hornick JL, Jaffe ES, Fletcher CD: Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol; 2004 Sep;28(9):1133-44
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  • [Title] Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy.
  • Histiocytic sarcoma is a rare malignant neoplasm that occurs in lymph nodes, skin, and the gastrointestinal tract.
  • Many previously published cases were likely misdiagnosed examples of non-Hodgkin lymphoma.
  • Seven tumors arose in soft tissue (6 lower limb; 1 upper limb), 5 in the gastrointestinal tract (1 involving both stomach and colon, 1 ileum, 2 rectum, 1 anus), 1 in the nasal cavity, and 1 in the lung.
  • The tumors were generally composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin, and large nucleoli.
  • Six patients were treated with postoperative radiation and 7 with chemotherapy (CHOP or ProMACE-MOPP).
  • Two tumors recurred locally, and 5 patients developed distant spread: 3 to lymph nodes, 1 to lung, and 1 to bone.
  • The patients who died thus far had the largest primary tumors.
  • Histiocytic sarcoma may arise primarily in soft tissue and shows reproducible histologic features, including abundant eosinophilic cytoplasm and a prominent inflammatory infiltrate.
  • Metastatic carcinoma, metastatic melanoma, and large cell non-Hodgkin lymphomas should be excluded by immunohistochemistry.
  • Histiocytic sarcoma has the potential for an aggressive clinical course, most often with lymph node involvement.
  • [MeSH-major] Sarcoma / pathology

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  • (PMID = 15316312.001).
  • [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
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13. 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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  • [Title] Sclerosing epithelioid fibrosarcomas involving the neuraxis: report of three cases.
  • 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.
  • Accordingly, SEF has been regarded as a low-grade sarcoma.
  • We report three examples of SEF distinguished by primary involvement of the neuraxis at initial presentation.
  • 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.
  • INTERVENTION: All three affected patients experienced local recurrence and distant metastasis after resection of the primary site.
  • In no case was there a response to adjuvant chemotherapy or radiotherapy.
  • [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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14. Jeon IS, Yi DY: Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus. Pediatr Hematol Oncol; 2009 Mar;26(2):85-8
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  • [Title] Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus.
  • Acute lymphoblastic leukemia (ALL), a primary hematologic malignancy that is especially common in childhood, occurs relatively rarely as a secondary malignant neoplasm.
  • Available data indicate that ALL often follows chemoradiotherapy for soft tissue sarcoma.
  • Perivascular epithelioid tumor (PEComa), a primitive mesenchymal tissue origin, can be classified as a soft tissue sarcoma.
  • An 11-year-old girl was diagnosed with ALL secondary to chemoradiotherapy (vincristine, ifosfamide, and anthracycline) and radiotherapy comprising 45 Gy to the whole pelvis for PEComa.
  • ALL, FAB L2, and immunophenotypically pro-B developed 16 months after the final chemotherapy treatment.
  • Herein, the authors report a case of secondary ALL that might be related to a previously used intercalating DNA topoisomerase II inhibitor (anthracycline) for a very rare sarcoma, PEComa.
  • [MeSH-major] Neoplasms, Second Primary / etiology. Perivascular Epithelioid Cell Neoplasms / complications. Precursor Cell Lymphoblastic Leukemia-Lymphoma / etiology. Uterine Neoplasms / complications

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  • (PMID = 19322738.001).
  • [ISSN] 1521-0669
  • [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] 0 / Anthracyclines; 0 / Topoisomerase II Inhibitors
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15. Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, Donohue JH: Surgical treatment of adult primary hepatic sarcoma. Br J Surg; 2000 Nov;87(11):1500-5
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  • [Title] Surgical treatment of adult primary hepatic sarcoma.
  • BACKGROUND: Primary sarcomas of the liver are extremely rare in adults.
  • Optimal therapeutic approaches remain unclear.
  • METHODS: Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed.
  • Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent.
  • No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient.
  • RESULTS: Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three).
  • Thirteen patients developed a recurrence.
  • Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure.
  • Six patients received salvage chemotherapy.
  • CONCLUSION: Surgical resection is the only effective therapy for primary hepatic sarcomas at present.
  • Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.
  • [MeSH-major] Liver Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Preoperative Care / methods. Retrospective Studies

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  • (PMID = 11091236.001).
  • [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
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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16. Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky B, Uzieli B, Eid A: Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre. Eur J Surg Oncol; 2004 May;30(4):421-7
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  • [Title] Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.
  • BACKGROUND: Primary hepatic sarcoma is a rare tumour with a poor prognosis.
  • METHODS: From 1997 to 2002 eight patients had liver resection for primary sarcoma of the liver at our institution.
  • The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed.
  • There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS).
  • Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2).
  • Two patients developed complications and there was one death.
  • Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection.
  • CONCLUSIONS: The majority of patients with primary liver sarcoma present with right upper quadrant pain, fever and a liver mass.
  • Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy.
  • Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.
  • [MeSH-major] Liver Neoplasms / surgery. Sarcoma / surgery
  • [MeSH-minor] Adult. Aged. Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Hepatectomy / methods. Humans. Length of Stay. Liver Function Tests. Male. Middle Aged. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 15063896.001).
  • [ISSN] 0748-7983
  • [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
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17. Fujii H, Honoki K, Enomoto Y, Kasai T, Kido A, Amano I, Kumamoto M, Morishita T, Mii Y, Nonomura A, Takakura Y: Adamantinoma-like Ewing's sarcoma with EWS-FLI1 fusion gene: a case report. Virchows Arch; 2006 Nov;449(5):579-84
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  • [Title] Adamantinoma-like Ewing's sarcoma with EWS-FLI1 fusion gene: a case report.
  • Recent studies have advocated the genotypic and phenotypic delineation of a novel Ewing's sarcoma histologic variant showing epithelial features defined as "adamantinoma-like Ewing's sarcoma".
  • We described an 18-year-old girl with a primary small round-cell sarcoma of the right tibia showing polyphenotypic differentiation with epithelioid features.
  • The immunohistochemical analysis showed the epithelial markers of cytokeratin 5/6/18, AE1/AE3, and cytokeratin high molecular weight were stained especially in the foci with epithelioid features, as well as MIC2, S100, and NSE.
  • The diagnosis of the lesion was confirmed as Ewing's sarcoma by the presence of the EWS-FLI1 fusion transcript, and could be defined as the so-called "adamantinoma-like Ewing's sarcoma".
  • After wide excision and high-dose chemotherapy with peripheral blood stem cell transfusion, the patient has been well and continuously event-free for 3 years since the initial diagnosis.
  • [MeSH-major] Adamantinoma / pathology. Bone Neoplasms / pathology. Oncogene Proteins, Fusion / genetics. Proto-Oncogene Protein c-fli-1 / genetics. Sarcoma, Ewing / pathology

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  • (PMID = 17016721.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS
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18. Etienne-Mastroianni B, Falchero L, Chalabreysse L, Loire R, Ranchère D, Souquet PJ, Cordier JF: Primary sarcomas of the lung: a clinicopathologic study of 12 cases. Lung Cancer; 2002 Dec;38(3):283-9
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  • [Title] Primary sarcomas of the lung: a clinicopathologic study of 12 cases.
  • BACKGROUND: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms.
  • PURPOSE: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis.
  • The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma.
  • Four patients received chemotherapy and two patients had radiation therapy postoperatively.
  • CONCLUSIONS: Primary sarcomas of the lung are a rare and aggressive malignancy.
  • Treatment and prognosis do not differ from other soft tissue sarcomas.
  • [MeSH-major] Lung Neoplasms / pathology. Sarcoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Humans. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 12445750.001).
  • [ISSN] 0169-5002
  • [Journal-full-title] Lung cancer (Amsterdam, Netherlands)
  • [ISO-abbreviation] Lung Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Ireland
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19. Bien E, Kazanowska B, Dantonello T, Adamkiewicz-Drozynska E, Balcerska A, Madziara W, Rybczynska A, Nurzynska-Flak J, Solarz E, Kurylak A, Zalewska-Szewczyk B, Krawczyk M, Izycka-Swieszewska E, Rapala M, Koscielniak E: Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature. Ann Surg Oncol; 2010 Jul;17(7):1878-89
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  • [Title] Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.
  • BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival.
  • METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols.
  • Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis.
  • Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses.
  • Response to systemic treatment was poor (44%) and did not prevent local and distant relapses.
  • CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS.
  • An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy.
  • The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.
  • [MeSH-major] Hemangioendothelioma / mortality. Hemangiopericytoma / mortality. Hemangiosarcoma / mortality. Sarcoma / mortality. Vascular Neoplasms / mortality

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  • (PMID = 20333551.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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20. Collini P, Sorensen PH, Patel S, Blay JY, Issels RD, Maki RG, Eriksson M, del Muro XG: Sarcomas with spindle cell morphology. Semin Oncol; 2009 Aug;36(4):324-37
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  • [Title] Sarcomas with spindle cell morphology.
  • In the days before the term "high-grade undifferentiated pleomorphic sarcoma" came into use, one of the most common sarcoma diagnoses was "malignant fibrous histiocytoma," and before that, in an era before immunohistochemistry, "fibrosarcoma" was used to describe most sarcomas.
  • "Spindle cell" is a descriptive phrase that denotes the cellular shape of many of the sarcomas encountered in the adult population.
  • As a result, they are usually treated differently from small round cell sarcomas, and have different biological characteristics than those tumors and sarcomas with epithelioid morphology.
  • As a very broad generalization, sarcomas with a spindle cell microscopic morphology occur in adults and are treated primarily with surgery and often adjuvant or neoadjuvant radiation as primary therapy.
  • In comparison to small round cell sarcomas such as Ewing sarcoma, the use of adjuvant chemotherapy remains controversial, and the sensitivity of these tumors to chemotherapy in the metastatic setting is highly variable.
  • [MeSH-major] Sarcoma / pathology
  • [MeSH-minor] Chemotherapy, Adjuvant. Fibrosarcoma / genetics. Fibrosarcoma / pathology. Gene Fusion. Heat-Shock Response. Humans. Hyperthermia, Induced. Protein Kinase Inhibitors / therapeutic use. Sarcoma, Synovial / genetics. Sarcoma, Synovial / pathology. Translocation, Genetic

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  • (PMID = 19664493.001).
  • [ISSN] 0093-7754
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA47179
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors
  • [Number-of-references] 62
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21. 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.
  • Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases.
  • Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy.
  • Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5.
  • 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.
  • Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.
  • [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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22. Daigeler A, Kuhnen C, Moritz R, Stricker I, Goertz O, Tilkorn D, Steinstraesser L, Steinau HU, Lehnhardt M: Lymph node metastases in soft tissue sarcomas: a single center analysis of 1,597 patients. Langenbecks Arch Surg; 2009 Mar;394(2):321-9
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  • [Title] Lymph node metastases in soft tissue sarcomas: a single center analysis of 1,597 patients.
  • PURPOSE: The aim of this study was to examine the clinical course of patients with the rare finding of regional lymph node metastasis (RLNM) from soft tissue sarcoma.
  • MATERIALS AND METHODS: Data from 28 out of 1,597 consecutive soft tissue sarcoma patients with RLNM were from the patients' charts and interviewing patients and general practitioners.
  • RESULTS: RLNM was seen in 21.4% for epithelioid sarcoma and 17.6% for clear cell sarcoma.
  • Survival was independent from surgical resection status of the primary tumor and the RLNM as well as from adjuvant radiation and chemotherapy.
  • Tumor entity as well as the length of the time period from primary to RLNM affect survival.
  • CONCLUSIONS: Surgical treatment as well as radiation and chemotherapy may improve survival in selected cases but probably have their value much more in terms of local disease control and improvement life quality of patients who probably already suffer from an aggressive systemic disease at time of nodal involvement.
  • [MeSH-major] Lymphatic Metastasis / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Neoplasm Staging. Prospective Studies. Radiotherapy, Adjuvant. Young Adult

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  • (PMID = 18594854.001).
  • [ISSN] 1435-2451
  • [Journal-full-title] Langenbeck's archives of surgery
  • [ISO-abbreviation] Langenbecks Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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23. McGrory JE, Pritchard DJ, Arndt CA, Nascimento AG, Remstein ED, Rowland CM: Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience. Clin Orthop Relat Res; 2000 May;(374):247-58
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  • [Title] Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience.
  • Eighty-six children to 18 years of age were treated for nonrhabdomyosarcoma soft tissue sarcomas of the trunk and extremities.
  • Synovial sarcoma (31), fibrosarcoma (13), malignant fibrous histiocytoma (11), epithelioid sarcoma (10), and clear cell sarcoma (7) were the most common diagnoses.
  • Four patients presented with metastatic disease.
  • Patients were treated with wide removal of the tumor when possible, with judicious use of adjuvant radiation, or with chemotherapy in selected cases.
  • When compared with published data in adults, the prognosis of primary, localized nonrhabdomyosarcoma soft tissue sarcomas in children appears to be more favorable.
  • [MeSH-major] Fibrosarcoma / pathology. Fibrosarcoma / therapy. Histiocytoma, Benign Fibrous / pathology. Histiocytoma, Benign Fibrous / surgery. Sarcoma / pathology. Sarcoma / surgery. Sarcoma, Clear Cell / pathology. Sarcoma, Clear Cell / surgery. Sarcoma, Synovial / pathology. Sarcoma, Synovial / surgery. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Adolescent. Age Factors. Biopsy. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant. Survival Analysis. Treatment Outcome

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  • (PMID = 10818984.001).
  • [ISSN] 0009-921X
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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24. Mhanna T, Ranchere-Vince D, Hervieu V, Tardieu D, Scoazec JY, Partensky C: Clear cell myomelanocytic tumor (PEComa) of the duodenum in a child with a history of neuroblastoma. Arch Pathol Lab Med; 2005 Nov;129(11):1484-6
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  • [Title] Clear cell myomelanocytic tumor (PEComa) of the duodenum in a child with a history of neuroblastoma.
  • We report herein a case of digestive clear cell myomelanocytic tumor (PEComa) that is unique in its location and presentation.
  • The lesion, located in the duodenal wall, was diagnosed in a child with a history of cervical neuroblastoma that was in remission after surgical resection and chemotherapy.
  • The decision was made to perform surgical resection.
  • [MeSH-major] Duodenal Neoplasms / pathology. Epithelioid Cells / pathology. Melanocytes / pathology. Neuroblastoma / pathology. Smooth Muscle Tumor / pathology
  • [MeSH-minor] Adenocarcinoma / diagnosis. Biomarkers, Tumor / analysis. Child. Diagnosis, Differential. Endocrine Gland Neoplasms / diagnosis. Humans. Male. Neoplasms, Second Primary. Pancreaticoduodenectomy. Sarcoma, Clear Cell / diagnosis. Treatment Outcome

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  • (PMID = 16253032.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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25. Malagón HD, Valdez AM, Moran CA, Suster S: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Am J Surg Pathol; 2007 Sep;31(9):1356-62
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  • [Title] Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases.
  • The clinicopathologic features of 46 patients with germ cell tumors with sarcomatous components (GCTSC) involving either the primary site or their metastases were studied.
  • The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1).
  • The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1).
  • All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery.
  • [MeSH-major] Immunohistochemistry. Mediastinal Neoplasms / diagnosis. Neoplasms, Germ Cell and Embryonal / diagnosis. Ovarian Neoplasms / diagnosis. Retroperitoneal Neoplasms / diagnosis. Sarcoma / diagnosis. Testicular Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Antineoplastic Combined Chemotherapy Protocols. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Metastasis. Neoplasm Staging. Orchiectomy. Ovariectomy. Time Factors. Treatment Outcome

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  • (PMID = 17721191.001).
  • [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
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26. Danikas D, Theodorou SJ, Singh R, Camal DE: Leiomyosarcoma of the gallbladder: a case report. Am Surg; 2001 Sep;67(9):873-4
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  • Primary sarcoma of the gallbladder is a rare disease.
  • Laparoscopic cholecystectomy was converted to open as a result of dense tissue in the middle to distal gallbladder.
  • Pathology revealed poorly differentiated epithelioid leiomyosarcoma of the gallbladder with extension to the liver.
  • The disease followed a very aggressive course and the patient died 3 weeks after the procedure.
  • Recommended treatment is extensive surgical resection that can be followed by radiotherapy or chemotherapy.

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  • (PMID = 11565767.001).
  • [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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27. Grossman EJ, Millis JM: Liver transplantation for non-hepatocellular carcinoma malignancy: Indications, limitations, and analysis of the current literature. Liver Transpl; 2010 Aug;16(8):930-42
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  • [Title] Liver transplantation for non-hepatocellular carcinoma malignancy: Indications, limitations, and analysis of the current literature.
  • Orthotopic liver transplantation (OLT) is currently incorporated into the treatment regimens for specific nonhepatocellular malignancies.
  • For patients suffering from early-stage, unresectable hilar cholangiocarcinoma (CCA), OLT preceded by neoadjuvant radiotherapy has the potential to readily achieve a tumor-free margin, accomplish a radical resection, and treat underlying primary sclerosing cholangitis when present.
  • Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin.
  • Hepatoblastoma is the most common primary hepatic malignancy in children.
  • There exist subtle differences in the timing of chemotherapy between US and European centers; however, the long-term survival rate after transplantation ranges from 66% to 77%.
  • In the treatment of either primary or metastatic hepatic sarcomas, unacceptable survival and recurrence rates currently prohibit the use of OLT.
  • [MeSH-major] Liver Neoplasms / therapy. Liver Transplantation / methods
  • [MeSH-minor] Aged. Cholangiocarcinoma / therapy. Hemangioendothelioma / therapy. Hepatoblastoma / therapy. Humans. Immunosuppressive Agents / therapeutic use. Liver / pathology. Medical Oncology / methods. Middle Aged. Neoplasm Metastasis. Neuroendocrine Tumors / therapy. Sarcoma / therapy. Treatment Outcome

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  • [Copyright] (c) 2010 AASLD.
  • (PMID = 20677284.001).
  • [ISSN] 1527-6473
  • [Journal-full-title] Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • [ISO-abbreviation] Liver Transpl.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunosuppressive Agents
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28. Carbone M, Rizzo P, Powers A, Bocchetta M, Fresco R, Krausz T: Molecular analyses, morphology and immunohistochemistry together differentiate pleural synovial sarcomas from mesotheliomas: clinical implications. Anticancer Res; 2002 Nov-Dec;22(6B):3443-8
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  • PATIENT AND METHODS: We demonstrated a primary pleural biphasic SS in a patient referred to us has having a biphasic MM.
  • RESULTS: Histology showed a spindle cell tumor with focal epithelioid differentiation, microcalcifications, and a hemangiopericytomatous vascular pattern.
  • Ultrastructurally, the epithelioid tumor cells had few blunt microvilli and occasional intercellular junctions.
  • Detection of the t(X:18) translocation using RT-PCR, Southern blot, and DNA sequencing definitively confirmed the diagnosis of SS.
  • This differential is critical because patients with pleural SS can be susceptible to chemotherapy, and accordingly are treated, while patients with sarcomatoid MM are resistant to chemotherapy and accordingly are not treated.
  • [MeSH-major] Mesothelioma / diagnosis. Pleural Neoplasms / diagnosis. Sarcoma, Synovial / diagnosis

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  • (PMID = 12552937.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R0-1 CA-92657
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] Greece
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29. Stringer MD: The role of liver transplantation in the management of paediatric liver tumours. Ann R Coll Surg Engl; 2007 Jan;89(1):12-21
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  • In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy.
  • Total hepatectomy and liver transplantation has emerged as an effective treatment for the small proportion of children with unresectable hepatoblastoma limited to the liver.
  • In contrast, the results of liver transplantation in children with hepatocellular cancer remain poor because these tumours are usually advanced with evidence of major vascular invasion and/or extrahepatic spread at the time of presentation.
  • The role of liver transplantation in children with other primary hepatic malignancies remains uncertain because experience is very limited.
  • Liver transplantation is rarely needed in the management of children with benign liver tumours but, if other treatments have failed, it can be a life-saving intervention.
  • [MeSH-minor] Carcinoma, Hepatocellular / mortality. Carcinoma, Hepatocellular / surgery. Child. Hamartoma / mortality. Hamartoma / surgery. Hemangioendothelioma, Epithelioid. Hepatoblastoma / mortality. Hepatoblastoma / surgery. Humans. Liver Diseases / mortality. Liver Diseases / surgery. Neoplasms, Muscle Tissue / mortality. Neoplasms, Muscle Tissue / surgery. Sarcoma / mortality. Sarcoma / surgery. Survival Analysis. Treatment Outcome

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  • (PMID = 17316514.001).
  • [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] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 71
  • [Other-IDs] NLM/ PMC1963524
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30. Thompson LD, Wieneke JA, Miettinen M: Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system. Am J Surg Pathol; 2003 May;27(5):594-611
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  • Primary sinonasal tract mucosal malignant melanomas are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management.
  • Histologically, the tumors were composed of a variety of cell types (epithelioid, spindled, undifferentiated), frequently arranged in a peritheliomatous distribution (n = 39).
  • Sinonasal tract mucosal malignant melanomas need to be considered in the differential diagnosis of most sinonasal malignancies, particularly carcinoma, lymphoma, sarcoma, and olfactory neuroblastoma.
  • Surgery accompanied by radiation and/or chemotherapy was generally used.
  • The majority of patients developed a recurrence (n = 79), with 75 patients dying with disseminated disease (mean 2.3 years), whereas 40 patients are either alive or had died of unrelated causes (mean 13.9 years).
  • A TNM-type classification separated by anatomic site of involvement and metastatic disease is proposed to predict biologic behavior.

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  • (PMID = 12717245.001).
  • [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
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31. Gupta D, Deavers MT, Silva EG, Malpica A: Malignant melanoma involving the ovary: a clinicopathologic and immunohistochemical study of 23 cases. Am J Surg Pathol; 2004 Jun;28(6):771-80
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  • Ovarian malignant melanoma (MM), primary or metastatic, is an extremely rare tumor and in the absence of a previous diagnosis can represent a diagnostic challenge.
  • A previous history of MM was definitively obtained in 14 patients; in these cases, the interval between the primary MM and the ovarian metastasis ranged from 15 to 228 months (mean 77.7 months).
  • The tumor cell type was epithelioid in 19 cases, spindled in 2 cases, mixed epithelioid and spindled in 1 case, and small cell in 1 case.
  • In 8 cases, initial diagnoses included sex cord stromal tumor, germ cell tumor, sarcoma, or undifferentiated carcinoma.
  • Treatment performed in 18 of the cases are as follows: oophorectomy with/without chemotherapy (10); total abdominal hysterectomy with bilateral salpingo-oophorectomy with/without chemotherapy (6); vaginal hysterectomy, bilateral salpingo-oophorectomy, and chemotherapy (1); and total abdominal hysterectomy with salpingo-oophorectomy (1).
  • The tumor is most often metastatic from another site and is unilateral in most cases.
  • Nodular or diffuse pattern and epithelioid cell type are most frequently seen, and the tumor can be mistaken for germ cell and sex cord stromal tumors.
  • [MeSH-minor] Adolescent. Adult. Antigens, Neoplasm. Calbindin 2. DNA-Binding Proteins / analysis. Ethnic Groups. Female. Humans. Hysterectomy. Immunohistochemistry. Inhibins / analysis. MART-1 Antigen. Melanoma-Specific Antigens. Microphthalmia-Associated Transcription Factor. Middle Aged. Monophenol Monooxygenase / analysis. Neoplasm Metastasis. Neoplasm Proteins / analysis. S100 Calcium Binding Protein G / analysis. S100 Proteins / analysis. Teratoma / pathology. Transcription Factors / analysis. Treatment Outcome

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  • (PMID = 15166669.001).
  • [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 / Antigens, Neoplasm; 0 / CALB2 protein, human; 0 / Calbindin 2; 0 / DNA-Binding Proteins; 0 / MART-1 Antigen; 0 / MITF protein, human; 0 / MLANA protein, human; 0 / Melanoma-Specific Antigens; 0 / Microphthalmia-Associated Transcription Factor; 0 / Neoplasm Proteins; 0 / S100 Calcium Binding Protein G; 0 / S100 Proteins; 0 / Transcription Factors; 57285-09-3 / Inhibins; EC 1.14.18.1 / Monophenol Monooxygenase
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