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1. Sidi V, Fragandrea I, Hatzipantelis E, Kyriakopoulos C, Papanikolaou A, Bandouraki M, Koliouskas DE: Alveolar soft-part sarcoma of the extremity: a case report. Hippokratia; 2008;12(4):251-3

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma of the extremity: a case report.
  • Alveolar soft-part sarcoma (ASPS) is a rare form of soft tissue sarcoma and is most often seen in adolescents and young adults.
  • An 11- year-old boy with ASPS which presented with a markedly vascular tumor in the left thigh, and multiple bilateral pulmonary metastases 8 months after diagnosis is described.
  • The patient has remained disease-free for over 5 years since the initial diagnosis.

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  • (PMID = 19158970.001).
  • [ISSN] 1790-8019
  • [Journal-full-title] Hippokratia
  • [ISO-abbreviation] Hippokratia
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Other-IDs] NLM/ PMC2580048
  • [Keywords] NOTNLM ; alveolar soft – part sarcoma / children / long term survival / metastases / pulmonary
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2. Silas OA, Adoga AA, Manasseh AN, Echejoh GO, Vhriterhire RA, Mandong BM: Persistent alveolar soft-part sarcoma with liver metastasis: a case report. J Med Case Rep; 2010;4:233

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Persistent alveolar soft-part sarcoma with liver metastasis: a case report.
  • INTRODUCTION: Alveolar soft-part sarcomas are rare, slow-growing tumors that metastasize commonly via vascular routes to the lungs, bones, lymph nodes and brain, causing morbidity and mortality.
  • It was initially diagnosed as soft-tissue sarcoma, and its associated systemic effects lead to his death before a histological diagnosis could be obtained.
  • CONCLUSIONS: Alveolar soft-part sarcoma with metastasis to the liver can occur in our region (northeast Africa), and a high index of suspicion is required to make an early diagnosis, followed by prompt surgical excision with clear margins in order to prevent mortality.

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  • (PMID = 20673324.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2922299
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3. Alkatan H, Al-Shedoukhy AA, Chaudhry IA, Al-Ayoubi A: Orbital alveolar soft part sarcoma: Histopathologic report of two cases. Saudi J Ophthalmol; 2010 Apr;24(2):57-61

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Orbital alveolar soft part sarcoma: Histopathologic report of two cases.
  • Alveolar soft part sarcoma is considered as a distinct histopathological entity with rare cases reported from the orbit area.
  • Two cases of alveolar soft part sarcomas occurring in the orbit of two patients along with their histopathologic findings are reported herewith.
  • The diagnosis was made by incisional biopsies and histopathology.
  • The literature is reviewed regarding occurrence of this tumor, its diagnosis and management.

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  • (PMID = 23960876.001).
  • [ISSN] 1319-4534
  • [Journal-full-title] Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society
  • [ISO-abbreviation] Saudi J Ophthalmol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Saudi Arabia
  • [Other-IDs] NLM/ PMC3729400
  • [Keywords] NOTNLM ; Alveolar soft part sarcoma / Diagnosis / Orbit / Treatment
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4. Vistica DT, Hollingshead M, Borgel SD, Kenney S, Stockwin LH, Raffeld M, Schrump DS, Burkett S, Stone G, Butcher DO, Shoemaker RH: Therapeutic vulnerability of an in vivo model of alveolar soft part sarcoma (ASPS) to antiangiogenic therapy. J Pediatr Hematol Oncol; 2009 Aug;31(8):561-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapeutic vulnerability of an in vivo model of alveolar soft part sarcoma (ASPS) to antiangiogenic therapy.
  • In vivo growth of alveolar soft part sarcoma (ASPS) was achieved using subcutaneous xenografts in sex-matched nonobese diabetic severe combined immunodeficiency mice.
  • One tumor, currently at passage 6, has been maintained in vivo for 32 months and has maintained characteristics consistent with those of the original ASPS tumor including (1) tumor histology and staining with periodic acid Schiff/diastase, (2) the presence of the ASPL-TFE3 type 1 fusion transcript, (3) nuclear staining with antibodies to the ASPL-TFE3 type 1 fusion protein, (4) maintenance of the t(X;17)(p11;q25) translocation characteristic of ASPS, (5) stable expression of signature ASPS gene transcripts and finally, the development and maintenance of a functional vascular network, a hallmark of ASPS.
  • The ASPS xenograft tumor vasculature encompassing nests of ASPS cells is highly reactive to antibodies against the endothelial antigen CD34 and is readily accessible to intravenously administered fluorescein isothiocyanate-dextran.
  • In summary, this study describes a preclinical in vivo model for ASPS which will facilitate investigation into the biology of this slow growing soft tissue sarcoma and demonstrates the feasibility of using an antiangiogenic approach in the treatment of ASPS.

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  • (PMID = 19636271.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / N01CO12400; United States / Intramural NIH HHS / / Z99 CA999999; United States / NCI NIH HHS / CO / N01-CO-12400
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antigens, CD34; 0 / Antineoplastic Agents; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / HIF1A protein, human; 0 / Hypoxia-Inducible Factor 1, alpha Subunit; 0 / Oncogene Proteins, Fusion; 0 / TFE3 protein, human; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; 2S9ZZM9Q9V / Bevacizumab; 7M7YKX2N15 / Topotecan
  • [Other-IDs] NLM/ NIHMS118018; NLM/ PMC2784654
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5. Wakely PE Jr, McDermott JE, Ali SZ: Cytopathology of alveolar soft part sarcoma: a report of 10 cases. Cancer; 2009 Dec 25;117(6):500-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytopathology of alveolar soft part sarcoma: a report of 10 cases.
  • BACKGROUND: Alveolar soft part sarcoma (ASPS) rarely is subjected to cytopathologic evaluation.
  • The objectives of the current study were to evaluate the cytomorphology of 10 ASPS cases on fine-needle aspiration (FNA) or imprint cytology, review the literature, and highlight potential diagnostic pitfalls.
  • METHODS: The authors searched their files for all lesions that were signed out as ASPS or suspicious for ASPS and searched the surgical pathology files for any cases of ASPS that had corresponding cytology.
  • RESULTS: Ten cases of ASPS were retrieved from 7 patients (male-to-female ratio, 4:3; mean age, 22 years).
  • All had subsequent tissue confirmation.
  • Nine cases were diagnosed correctly as ASPS/consistent with ASPS, and 1 specimen was diagnosed as "tumor, not otherwise specified".
  • CONCLUSIONS: The results of the current study indicated that ASPS has cytomorphology that overlaps with several other neoplasms, including renal cell carcinoma.
  • Nonetheless, the morphologic features, when combined with the clinical presentation, radiologic findings, and ancillary testing, may allow for a specific diagnosis.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / pathology
  • [MeSH-minor] Adult. Child. Diagnosis, Differential. Female. Humans. Male

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  • [Copyright] (c) 2009 American Cancer Society.
  • (PMID = 19787801.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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6. Kim HS, Lee HK, Weon YC, Kim HJ: Alveolar soft-part sarcoma of the head and neck: clinical and imaging features in five cases. AJNR Am J Neuroradiol; 2005 Jun-Jul;26(6):1331-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma of the head and neck: clinical and imaging features in five cases.
  • BACKGROUND AND PURPOSE: Alveolar soft-part sarcoma (ASPS) of the head and neck is an extremely rare malignancy.
  • The purpose of this study was to describe the clinical and imaging features of ASPS of the head and neck.
  • METHODS: Between January 1990 and May 2004 at our institution, five head and neck ASPS were diagnosed in five patients (two male and three female patients; age range, 4-22 years).
  • CONCLUSION: ASPS should be included in the differential diagnosis of head and neck tumor when a slow-growing, large mass with high signal intensity and flow voids on T2-weighted images and strong enhancement on contrast-enhanced CT or MR image is seen, particularly in young female patients.
  • [MeSH-major] Head and Neck Neoplasms / diagnosis. Magnetic Resonance Imaging. Sarcoma, Alveolar Soft Part / diagnosis. Tomography, X-Ray Computed


7. Baglam T, Kalender ME, Durucu C, Bakir K, Karatas E, Kara F, Kanlikama M: Alveolar soft part sarcoma of the tongue. J Craniofac Surg; 2009 Nov;20(6):2160-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the tongue.
  • Alveolar soft part sarcoma is a rare type of sarcoma that usually affects young adult women.
  • Herein, we present an unusual case of alveolar soft part sarcoma of the tongue in an 18-year-old woman.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / pathology. Tongue Neoplasms / pathology

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  • (PMID = 19884839.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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8. Kayton ML, Meyers P, Wexler LH, Gerald WL, LaQuaglia MP: Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg; 2006 Jan;41(1):187-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults.
  • PURPOSE: Alveolar soft part sarcoma is a rare soft tissue neoplasm that can affect children and adolescents.
  • To define the clinical presentation, treatment, and outcome of young people with this rare sarcoma, we reviewed our clinical experience.
  • METHODS: After institutional review board approval, we examined the records of all patients younger than 25 years old who received treatment at our institution for alveolar soft part sarcoma in the past 30 years.
  • Demographics, tumor sizes, sites and extent of disease, treatments used, progression-free survival, and overall follow-up were evaluated.
  • Primary disease sites were thigh (n = 8), trunk (n = 6), retroperitoneum (n = 2), and scalp, neck, forearm, and calf (n = 1 each).
  • Although wide local excision provided the best chance for a patient to remain free of disease, 14 (70%) of 20 patients exhibited metastases either at presentation or later.
  • Younger patients tended to have Intergroup Rhabdomyosarcoma Study group I disease.
  • CONCLUSIONS: Achievement of complete microscopic resection is critical in localized alveolar soft part sarcoma, but incomplete excision and misdiagnosis are often encountered.
  • [MeSH-major] Sarcoma / pathology. Sarcoma / surgery
  • [MeSH-minor] Adolescent. Adult. Age Factors. Child. Disease Progression. Embolization, Therapeutic. Female. Follow-Up Studies. Humans. Male. Neoplasm Metastasis. Retrospective Studies. Survival Analysis. Treatment Outcome


9. Eley KA, Afzal T, Shah KA, Watt-Smith SR: Alveolar soft-part sarcoma of the tongue: report of a case and review of the literature. Int J Oral Maxillofac Surg; 2010 Aug;39(8):824-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma of the tongue: report of a case and review of the literature.
  • Alveolar soft part sarcoma (ASPS) accounts for less than 1% of all sarcomas.
  • More frequently encountered within the lower limbs, the authors present a 24-year-old male with ASPS presenting as an asymptomatic swelling of the lateral tongue.
  • Histological and immunohistochemical features are diagnostic of ASPS.
  • Whilst rare, head and neck surgeons should be aware of ASPS as a potential cause of slow growing lesions, as early surgical resection is vital in view of the propensity for metastatic spread.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / pathology. Tongue Neoplasms / pathology

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  • [Copyright] Copyright 2010 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
  • (PMID = 20335005.001).
  • [ISSN] 1399-0020
  • [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; Review
  • [Publication-country] Denmark
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10. Lee GW, Kim TH, Min HJ, Kim HJ, Jung WT, Lee OJ, Ko GH: Unusual gastrointestinal metastases from an alveolar soft part sarcoma. Dig Endosc; 2010 Apr;22(2):137-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unusual gastrointestinal metastases from an alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS) is a rare subtype of soft tissue sarcoma that occurs predominantly in young patients.
  • The common metastatic sites from an ASPS include the lung, brain and bone.
  • However, metastasis of an ASPS to the gastrointestinal tract is extremely rare.
  • Here, we report a rare case of upper gastrointestinal bleeding and jejunal intussusception due to gastrointestinal metastases from an ASPS.
  • [MeSH-major] Jejunal Neoplasms / secondary. Sarcoma, Alveolar Soft Part / secondary. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Endoscopy, Digestive System. Humans. Intussusception / diagnosis. Intussusception / etiology. Intussusception / therapy. Male. Young Adult

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  • (PMID = 20447209.001).
  • [ISSN] 1443-1661
  • [Journal-full-title] Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy Society
  • [ISO-abbreviation] Dig Endosc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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11. Khanna P, Paidas CN, Gilbert-Barness E: Alveolar soft part sarcoma: clinical, histopathological, molecular, and ultrastructural aspects. Fetal Pediatr Pathol; 2008;27(1):31-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma: clinical, histopathological, molecular, and ultrastructural aspects.
  • Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor occurring mainly in the adolescents and young adults.
  • The histopathological, ultrastructural, immunohistochemical, and genetic aspects of ASPS are discussed.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 18568987.001).
  • [ISSN] 1551-3823
  • [Journal-full-title] Fetal and pediatric pathology
  • [ISO-abbreviation] Fetal Pediatr Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm; 0 / S100 Proteins; 0 / Synaptophysin
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12. Kanhere HA, Pai PS, Neeli SI, Kantharia R, Saoji RR, D'cruz AK: Alveolar soft part sarcoma of the head and neck. Int J Oral Maxillofac Surg; 2005 May;34(3):268-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the head and neck.
  • Alveolar soft part sarcoma (ASPS) of the head and neck region has been a rarely reported entity.
  • A retrospective study of the medical records at our institute, revealed thirty-eight cases of ASPS.
  • [MeSH-major] Head and Neck Neoplasms / pathology. Head and Neck Neoplasms / therapy. Sarcoma, Alveolar Soft Part / pathology. Sarcoma, Alveolar Soft Part / therapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Disease-Free Survival. Female. Humans. Male. Neoplasm Invasiveness. Neoplasm Metastasis. Neoplasm Recurrence, Local. Radiotherapy, Adjuvant. Retrospective Studies

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  • (PMID = 15741035.001).
  • [ISSN] 0901-5027
  • [Journal-full-title] International journal of oral and maxillofacial surgery
  • [ISO-abbreviation] Int J Oral Maxillofac Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
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13. do Nascimento Souza KC, Faria PR, Costa IM, Duriguetto AF Jr, Loyola AM: Oral alveolar soft-part sarcoma: review of literature and case report with immunohistochemistry study for prognostic markers. Oral Surg Oral Med Oral Pathol Oral Radiol Endod; 2005 Jan;99(1):64-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Oral alveolar soft-part sarcoma: review of literature and case report with immunohistochemistry study for prognostic markers.
  • OBJECTIVE: To describe a case of alveolar soft-part sarcoma (ASPS) affecting the tongue of a child and to study prognostic imunohistochemical markers for the disease.
  • STUDY DESIGN: Tissue sections were incubated with primary antibodies reactive to neuron-specific enolase (NSE), vimentin, desmin, S-100 protein, cytokeratins AE1-AE3, EMA, neurofilament, synaptophysin, and muscle-specific actin (MSA), and for prognostic markers, including Ki-67, p53, bcl-2, bax, and nm23.
  • RESULTS: Histologically, the tumor showed a proliferation of large polygonal cells with PAS-positive diastase-resistant intracytoplasmatic material, arranged in an alveolar growth pattern.
  • CONCLUSION: These immunohistochemical findings may reflect the less aggressive behavior of ASPS in oral tissues.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / pathology. Tongue Neoplasms / pathology

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  • (PMID = 15599350.001).
  • [ISSN] 1079-2104
  • [Journal-full-title] Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics
  • [ISO-abbreviation] Oral Surg Oral Med Oral Pathol Oral Radiol Endod
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, Neoplasm; 0 / BAX protein, human; 0 / Biomarkers, Tumor; 0 / Desmin; 0 / Ki-67 Antigen; 0 / Mucin-1; 0 / NM23 Nucleoside Diphosphate Kinases; 0 / Neurofilament Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / S100 Proteins; 0 / Synaptophysin; 0 / Tumor Suppressor Protein p53; 0 / Vimentin; 0 / bcl-2-Associated X Protein; 68238-35-7 / Keratins; EC 2.7.4.6 / NME1 protein, human; EC 2.7.4.6 / Nucleoside-Diphosphate Kinase; EC 4.2.1.11 / Phosphopyruvate Hydratase
  • [Number-of-references] 42
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14. Zhu FP, Lu GM, Zhang LJ, Wang JD, An XJ, Dong YC: Primary alveolar soft part sarcoma of vertebra: a case report and literature review. Skeletal Radiol; 2009 Aug;38(8):825-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary alveolar soft part sarcoma of vertebra: a case report and literature review.
  • Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor, which rarely occurs in bone.
  • We present a case of ASPS in a 23-year-old man with a 2-month history of back pain.
  • Computed tomography scanning and magnetic resonance images demonstrated a destructive process in the 12th thoracic vertebra associated with a unilateral soft tissue mass.
  • The tumor showed evidence of hypervascularity on MRI; it obviously was enhanced on T1-weighted images after injection of Gd-GDPA, and signal voids were shown on all pulse sequences which may help to differentiate ASPS from other tumors of the vertebra.
  • We believe that this is the first case of ASPS arising in a vertebra.
  • [MeSH-major] Adenocarcinoma, Bronchiolo-Alveolar / diagnosis. Magnetic Resonance Imaging / methods. Spinal Neoplasms / diagnosis. Thoracic Vertebrae / pathology. Thoracic Vertebrae / radiography. Tomography, X-Ray Computed / methods

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  • (PMID = 19347336.001).
  • [ISSN] 1432-2161
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 31
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15. Akiyama Y, Baba T, Ibayashi Y, Asai Y, Houkin K: Alveolar soft part sarcoma in brain with cardiac metastasis: a case report. Int J Cardiol; 2007 Jan 18;114(3):e93-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma in brain with cardiac metastasis: a case report.
  • Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm.
  • The coexistence of ASPS with cardiac metastasis is quite rare, in particular.
  • In general, the sarcoma is a malignant disease and grows very fast.
  • However, the mean survival time of patients with ASPS is relatively long.
  • Patients who are diagnosed with localized disease usually have a favorable prognosis, while those who present with widespread metastases usually have a poor prognosis and ultimately, succumb to their disease.
  • Because of the long-term period with symptomatic brain tumors in this disease, the patients should be treated even in the presence of multiple metastases in other organs.
  • [MeSH-major] Brain Neoplasms / secondary. Heart Neoplasms / secondary. Sarcoma, Alveolar Soft Part / secondary. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Intestinal Neoplasms / secondary. Intestinal Neoplasms / surgery. Lung Neoplasms / secondary. Lung Neoplasms / surgery. Male. Radiosurgery. Thigh

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  • (PMID = 17055085.001).
  • [ISSN] 1874-1754
  • [Journal-full-title] International journal of cardiology
  • [ISO-abbreviation] Int. J. Cardiol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Netherlands
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16. Hillyer S, Vicens JC, Levinson H, Bhayani R, Mesea L, Chaudhry R, Fayans E, Fogler R: Alveolar soft-part sarcoma of the tongue in a 17-month-old. Ear Nose Throat J; 2009 Oct;88(10):E4-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma of the tongue in a 17-month-old.
  • Alveolar soft-part sarcoma (ASPS) is a rare and often fatal tumor.
  • ASPS accounts for 5% of all pediatric soft-tissue sarcomas other than rhabdomyosarcomas.
  • In children, ASPS rarely occurs in the oral cavity, and to the best of our knowledge, only 12 cases of ASPS of the tongue occurring during the first decade of life have been previously reported in the literature.
  • Because of the rarity of lingual ASPS in children, no specific treatment protocols have been developed, which makes its management difficult.
  • We report a new case of lingual ASPS in a young child.
  • Postoperatively, she has remained disease-free during 4 years of follow-up.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / diagnosis. Tongue Neoplasms / diagnosis
  • [MeSH-minor] Biopsy. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Neoplasm Staging. Neoplasm, Residual / diagnosis. Neoplasm, Residual / pathology. Neoplasm, Residual / surgery. Radionuclide Imaging. Sodium Pertechnetate Tc 99m. Tomography, X-Ray Computed. Tongue / pathology. Tongue / surgery

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  • (PMID = 19826991.001).
  • [ISSN] 1942-7522
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] A0730CX801 / Sodium Pertechnetate Tc 99m
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17. Rocha LA, Rizo VH, Romañach MJ, de Almeida OP, Vargas PA: Oral metastasis of alveolar soft-part sarcoma: a case report and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod; 2010 Apr;109(4):587-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Oral metastasis of alveolar soft-part sarcoma: a case report and review of literature.
  • Alveolar soft-part sarcoma (ASPS) is a rare malignant neoplasm with uncertain histogenesis and with a distinctive morphology.
  • It has been described in the oral cavity, but this is the first report of ASPS metastasizing to the maxillary tuber region.
  • A 27-year-old male patient, who was under chemotherapy treatment for ASPS of the thigh, presented in our dental clinic with a painless and pedunculated nodule on the right tuber maxillae.
  • An incisional biopsy was performed and the diagnosis of metastatic ASPS was made.
  • Unfortunately, the patient died 2 months after the diagnosis of the oral metastasis.
  • Metastases of ASPS to the mouth are very rare and indicate a poor prognosis.
  • [MeSH-major] Gingival Neoplasms / secondary. Maxilla / pathology. Sarcoma, Alveolar Soft Part / secondary
  • [MeSH-minor] Adult. Brain Neoplasms / secondary. Cell Nucleolus / ultrastructure. Cell Nucleus / ultrastructure. Cytoplasm / ultrastructure. Diagnosis, Differential. Fatal Outcome. Gingival Diseases / diagnosis. Granuloma, Giant Cell / diagnosis. Granuloma, Pyogenic / diagnosis. Humans. Male. Muscle Neoplasms / pathology. Thigh / pathology

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  • [Copyright] Copyright 2010 Mosby, Inc. All rights reserved.
  • (PMID = 20303057.001).
  • [ISSN] 1528-395X
  • [Journal-full-title] Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics
  • [ISO-abbreviation] Oral Surg Oral Med Oral Pathol Oral Radiol Endod
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. Benetos IS, Mavrogenis AF, Soultanis KCh, Zoubos AB, Papagelopoulos PJ, Soucacos PN: Alveolar soft part sarcoma of the forearm: a case report. J Surg Orthop Adv; 2006;15(4):209-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the forearm: a case report.
  • Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma that most commonly arises in the deep soft tissues of the lower extremities of adults.
  • Median survival of patients with metastatic ASPS has been reported to be from 3 to 3.3 years.
  • In this article, a case of an 11-year-old male with primary ASPS of the right forearm is presented.
  • Three years after diagnosis, the patient developed pulmonary metastases.
  • Chemotherapy was unsuccessfully used to control the metastatic disease.
  • This time to death after the development of metastases vastly exceeded the previously reported survival rates of patients with metastatic ASPS.
  • [MeSH-major] Forearm. Sarcoma, Alveolar Soft Part / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17313933.001).
  • [ISSN] 1548-825X
  • [Journal-full-title] Journal of surgical orthopaedic advances
  • [ISO-abbreviation] J Surg Orthop Adv
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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19. Moyano S, Aguilera P, Petit A, de Alava E, Mascaró JM, Palou J, Ferrando J, Alos L: Alveolar soft part sarcoma presenting with cutaneous metastases: report of a case with immunohistochemical and molecular characterization. J Am Acad Dermatol; 2009 Jul;61(1):117-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma presenting with cutaneous metastases: report of a case with immunohistochemical and molecular characterization.
  • Alveolar soft part sarcoma (ASPS) is an uncommon neoplasm of uncertain histogenesis that usually behaves as a painless, slow-growing mass that metastasizes early.
  • We report a 21-year-old woman with cutaneous metastases of ASPS, whose histologic characteristics gave rise to a wide range of differential diagnoses of both primary and metastatic cutaneous neoplasms.
  • The molecular study identified a type 2 alveolar soft part locus-transcription factor E3 (ASPL-TFE3) fusion, secondary to der(17)t(X;17)(p11.2;q25) translocation.
  • A computed tomography scan performed after the diagnosis was made disclosed a 13-cm primary tumor in the left buttock.
  • Cutaneous metastases presenting as the first sign of ASPS have not been reported previously.
  • We emphasize the difficulties in making the diagnosis of ASPS when it presents in an unusual manner.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / secondary. Skin Neoplasms / secondary

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  • (PMID = 19539846.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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20. Almansori M, Turner AR, Girgis S, Vethanayagam D: Alveolar soft-part sarcoma presenting with eosinophilia and shunt. Can Respir J; 2005 Oct;12(7):389-91
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  • [Title] Alveolar soft-part sarcoma presenting with eosinophilia and shunt.
  • Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumour found in young adults that usually arises in skeletal muscle and occurs most frequently in the lower limbs.
  • Radiological and pathological findings of ASPS in a 34-year-old man who presented with increasing shortness of breath over a period of four to six weeks with peripheral blood eosinophilia, hypoxemia and a significant arteriovenous shunt are reported.
  • The present article is the fourth report of eosinophilia in association with sarcoma, and the first involving ASPS.
  • [MeSH-major] Eosinophilia / complications. Pulmonary Circulation. Sarcoma, Alveolar Soft Part / complications. Soft Tissue Neoplasms / complications

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  • (PMID = 16307030.001).
  • [ISSN] 1198-2241
  • [Journal-full-title] Canadian respiratory journal
  • [ISO-abbreviation] Can. Respir. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
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21. Hoshino M, Ogose A, Kawashima H, Izumi T, Hotta T, Hatano H, Morita T, Otsuka H, Umezu H, Yanoma S, Tsukuda M, Endo N: Molecular analyses of cell origin and detection of circulating tumor cells in the peripheral blood in alveolar soft part sarcoma. Cancer Genet Cytogenet; 2009 Apr 15;190(2):75-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular analyses of cell origin and detection of circulating tumor cells in the peripheral blood in alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS) is a distinct, rare soft tissue tumor with an unknown histogenesis and a tendency for late widespread metastases to lung, bone, and brain.
  • It is now clear that they are caused by a specific unbalanced translocation, der(17)t(X;17)(p11;q25), which results in the formation of an ASPSCR1-TFE3 (alias ASPL-TFE3) fusion gene.
  • The rearrangement results in the expression of chimeric transcripts, which can be identified by means of reverse transcriptase-polymerase chain reaction (RT-PCR).
  • We investigated the histogenesis of ASPS and attempted to detect circulating ASPS tumor cells in peripheral blood.
  • The immunohistochemical and genetic details of four cases and one cell line of ASPS were examined.
  • An immunohistochemical analysis and RT-PCR did not detect myogenic differentiation gene MYOD1.
  • The sensitivity of nested RT-PCR for detection of circulating ASPS cells was assessed by demonstrating that the tumor cell-associated gene translocation could be detected in 50 tumor cells/2 mL of blood.
  • Clinically, it was detectable in a peripheral blood sample (2 mL) of ASPS patient with distant metastases.
  • The findings suggest that ASPS is not of skeletal muscle origin.
  • ASPS tumor cells in the peripheral blood could be monitored by RT-PCR.
  • [MeSH-major] Neoplastic Cells, Circulating. Sarcoma, Alveolar Soft Part / pathology

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  • (PMID = 19380023.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / TFE3 protein, human
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22. Rodríguez-Velasco A, Fermán-Cano F, Cerecedo-Díaz F: Rare tumor of the tongue in a child: alveolar soft part sarcoma. Pediatr Dev Pathol; 2009 Mar-Apr;12(2):147-51
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rare tumor of the tongue in a child: alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS) is a rare, malignant tumor of uncertain histogenesis that has no benign counterpart.
  • Because of the indolent growth and lack of pain associated with the mass, 20% of patients have metastases at the time of initial diagnosis.
  • When ASPS presents in the tongue region, however, the patients involved are usually children and have a better prognosis than patients affected in the extremities.
  • Because metastases may occur after several decades, children with ASPS should be followed throughout adolescence and well into adulthood.
  • Only 10 cases of ASPS occurring in the tongues of children younger than 5 years of age were indexed by MEDLINE between 1952 and 2006.
  • Here, we describe the 1st case consistent with typical ASPS of the tongue in 15 years at our hospital.
  • The patient is a 2-year-old girl who has been disease-free for 32 months.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology. Tongue Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Child, Preschool. Cyclophosphamide / therapeutic use. Dactinomycin / therapeutic use. Disease-Free Survival. Female. Humans. Vimentin / analysis. Vincristine / therapeutic use

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  • (PMID = 18630993.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Vimentin; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VAC protocol
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23. Kacerovska D, Michal M, Nemcova J, Vanecek T, Kreuzberg B, Mrazkova P, Koudela K Jr, Kazakov DV: Crystal-deficient alveolar soft-part sarcoma with cutaneous involvement: a case report. Am J Dermatopathol; 2009 May;31(3):272-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Crystal-deficient alveolar soft-part sarcoma with cutaneous involvement: a case report.
  • Alveolar soft-part sarcoma (ASPS) is a rare distinctive sarcoma, in most cases involving deep soft tissues of the extremities.
  • It is associated with a specific unbalanced translocation, der(17)t(X;17)(p11;q25) that results in the formation of an ASPL-TFE3 fusion gene.
  • Microscopically, it is typified by an alveolar growth of large cells containing typical periodic acid-Schiff-positive rod-shaped crystals, often serving as a diagnostic clue.
  • Other distinctive features include nuclear immunoreactivity for transcription factor 3 (TFE3) protein and a typical ultrastructural finding of large crystals with a rectangular or rhomboid shape.
  • The authors present an unusual case of ASPS with cutaneous involvement, which did not exhibit typical large crystals; there were striking round granules.
  • To the best of our knowledge, cutaneous involvement of a crystal-deficient ASPS has not been reported.
  • [MeSH-major] Cytoplasmic Granules / pathology. Sarcoma, Alveolar Soft Part / pathology. Skin / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 19384069.001).
  • [ISSN] 1533-0311
  • [Journal-full-title] The American Journal of dermatopathology
  • [ISO-abbreviation] Am J Dermatopathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / TFE3 protein, human
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24. Xu LB, Yu SJ, Shao YF, Zhang HT, Zhao ZG: [Clinical features and prognosis of alveolar soft part sarcoma]. Zhonghua Zhong Liu Za Zhi; 2007 Oct;29(10):778-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Clinical features and prognosis of alveolar soft part sarcoma].
  • OBJECTIVE: To investigate the clinical feature, suitable treatment and prognosis of alveolar soft part sarcoma.
  • CONCLUSION: Alveolar soft part sarcoma usually grows slowly.
  • To achieve microscopically complete resection is quite critical to localized alveolar soft part sarcoma.
  • [MeSH-major] Lung Neoplasms / secondary. Sarcoma, Alveolar Soft Part / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 18396693.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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25. Magnan HD, Chou T, LaQuaglia MP, Gerald W, Ladanyi M, Merchant MS: Elevated expression of VEGFR-2 and VEGFA in desmoplastic small round cell tumor (DSRCT) and activity of bevacizumab and irinotecan in a xenograft model of DSRCT. J Clin Oncol; 2009 May 20;27(15_suppl):10016

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHODS: RNA was extracted from frozen tumor samples (DSRCT, alveolar soft part sarcoma, alveolar rhabdomyosarcoma, synovial sarcoma, and Ewing sarcoma) and a human DSRCT cell line, JN-DSRCT.
  • RESULTS: Microarray data demonstrated an average of 4.5 times higher RNA expression of VEGFR-2 (KDR) in DSRCT tumor samples as compared to the other translocation-associated sarcomas (p = 3.6 E-12).
  • VEGFR-2 was highly expressed in the JN-DSRCT line as compared to other sarcoma lines.
  • VEGFA was also highly overexpressed in the DSRCT line and tumor samples when compared to the other translocation-associated sarcomas (2.5 times, p = 1.1E-10).

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  • (PMID = 27962501.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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26. Chen YD, Hsieh MS, Yao MS, Lin YH, Chan WP: MRI of alveolar soft-part sarcoma. Comput Med Imaging Graph; 2006 Dec;30(8):479-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MRI of alveolar soft-part sarcoma.
  • Alveolar soft-part sarcoma (ASPS) is rare.
  • We present a case of a 30-year-old woman with a 6-month history of a palpable mass in her left thigh.
  • Histologic findings were compatible with ASPS.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 17056231.001).
  • [ISSN] 0895-6111
  • [Journal-full-title] Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society
  • [ISO-abbreviation] Comput Med Imaging Graph
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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27. Trabelsi A, Ben Abdelkrim S, Taher Yacoubi M, Mlika S, Hmissa S, Mokni M, Korbi S: [Primary alveolar soft part sarcoma of the lung]. Rev Mal Respir; 2009 Mar;26(3):329-32
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  • [Title] [Primary alveolar soft part sarcoma of the lung].
  • [Transliterated title] Sarcome alvéolaire primitif du poumon.
  • BACKGROUND: Alveolar soft part sarcoma is rare; it mainly appears in the lower extremities in adults and the head and neck in children.
  • Diagnosis of primary alveolar sarcoma of the lung was made by histological examination of a biopsy specimen and elimination of a primary soft tissue tumour elsewhere.
  • CONCLUSION: Primary alveolar sarcoma of the lung is exceptionally rare and the diagnosis should only be made after eliminating a soft tissue tumour elsewhere.
  • [MeSH-major] Lung Neoplasms / pathology. Sarcoma, Alveolar Soft Part / pathology

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  • (PMID = 19367208.001).
  • [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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28. Montgomery JR, Conrad GR, Sinha P, Absher K: FDG PET of alveolar soft part sarcoma. Clin Nucl Med; 2010 Oct;35(10):827-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] FDG PET of alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS) is a very rare, but distinctive type of soft tissue sarcoma, whose name is derived from the pseudoalveolar appearance of its histology.
  • In this report, the FDG PET/CT findings of ASPS are described in a 17-year-old asthmatic female who presented with worsening respiratory symptoms and a pelvic mass.
  • In concordance with other soft tissue sarcomas, PET/CT helped to confirm the anatomic origin of the ASPS, to direct its biopsy, and to assess the distribution of disease.
  • [MeSH-major] Fluorodeoxyglucose F18. Positron-Emission Tomography. Sarcoma, Alveolar Soft Part / radionuclide imaging

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  • (PMID = 20838301.001).
  • [ISSN] 1536-0229
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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29. Altuğ T, Inci E, Güvenç MG, Edizer DT, Dervişoğlu S: Alveolar soft part sarcoma of the larynx. Eur Arch Otorhinolaryngol; 2007 Apr;264(4):445-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the larynx.
  • Alveolar soft part sarcoma (ASPS) is a rare malignancy.
  • In this article, we report a case of ASPS occurring in the larynx, an extremely rare location for this rather unusual tumor.
  • To our knowledge, only three cases of laryngeal ASPS have been previously reported in the English literature.
  • [MeSH-major] Laryngeal Neoplasms / pathology. Sarcoma, Alveolar Soft Part / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Disease Progression. Female. Hoarseness / diagnosis. Humans. Laryngectomy / methods. Laryngoscopy. Respiratory Sounds / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 17063352.001).
  • [ISSN] 0937-4477
  • [Journal-full-title] European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
  • [ISO-abbreviation] Eur Arch Otorhinolaryngol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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30. Zarrin-Khameh N, Kaye KS: Alveolar soft part sarcoma. Arch Pathol Lab Med; 2007 Mar;131(3):488-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma.
  • This article provides an overview of the pathology of alveolar soft part sarcoma, focused on its morphology, special stains useful in diagnosis, and the clinical and radiographic features of the disease.
  • Alveolar soft part sarcoma is a rare neoplasm of unknown histogenesis with poor prognosis.
  • Although there are several immunohistochemical stains available to help reach the diagnosis, the morphology of the tumor should be considered the main diagnostic feature.
  • The periodic acid-Schiff stain is the best single stain that supports the diagnosis.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / pathology
  • [MeSH-minor] Adolescent. Adult. Diagnosis, Differential. Female. Humans. Male. Prognosis

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  • (PMID = 17516754.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 46
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31. Gitau GM, Shepherd JH, Hughes G, Oriolowo A, Yiannakis D: Alveolar soft part sarcoma of the uterine cervix. Int J Gynecol Cancer; 2008 Jul-Aug;18(4):853-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the uterine cervix.
  • Histology and immunohistochemistry on a wedge biopsy had features consistent with alveolar soft part sarcoma (ASPS).
  • We review literature on diagnosis and treatment of genital ASPS.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / diagnosis. Uterine Cervical Neoplasms / diagnosis


32. Günay C, Atalar H, Kaygusuz G, Yildiz Y, Sağlik Y: [Alveolar soft part sarcoma of the extremities: an evaluation of four cases]. Acta Orthop Traumatol Turc; 2007 Aug-Oct;41(4):326-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Alveolar soft part sarcoma of the extremities: an evaluation of four cases].
  • We presented four patients (3 women, 1 man; mean age 40 years; range 20 to 56 years) who had alveolar soft part sarcoma in the left arm, right elbow, right tibia, and left thigh, respectively.
  • Two patients had lung metastasis at the time of diagnosis.
  • T1- and T2-weighted magnetic resonance images of all the patients showed a soft tissue lesion with hyperintense signal changes and heterogeneous contrast enhancement.
  • Two patients with involvement of the left arm and right elbow died within three years after diagnosis.
  • One patient with tibial involvement remained disease-free during 10 months of follow-up.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adult. Arm. Diagnosis, Differential. Elbow. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Metastasis. Thigh. Tibia

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  • (PMID = 18180565.001).
  • [ISSN] 1017-995X
  • [Journal-full-title] Acta orthopaedica et traumatologica turcica
  • [ISO-abbreviation] Acta Orthop Traumatol Turc
  • [Language] tur
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Turkey
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33. Tapisiz OL, Gungor T, Ustunyurt E, Ozdal B, Bilge U, Mollamahmutoglu L: An unusual case of lingual alveolar soft part sarcoma during pregnancy. Taiwan J Obstet Gynecol; 2008 Jun;47(2):212-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An unusual case of lingual alveolar soft part sarcoma during pregnancy.
  • OBJECTIVE: We report a case of alveolar soft part sarcoma (ASPS) of the tongue that presented in the third trimester of pregnancy.
  • CASE REPORT: An 18-year-old, gravida 1, para 0, woman with ASPS of the tongue in the 31st week of pregnancy was described.
  • Approximately 4 months after her first diagnosis, she was referred to our clinic for the delivery of her baby and additional treatment.
  • CONCLUSION: ASPS of the tongue presenting in pregnancy is an extremely rare combination, and to our knowledge, this is the first reported case in the English literature.
  • The diagnostic and therapeutic management of the pregnant patient with cancer, like ASPS, is especially difficult, because it involves both the mother and fetus.
  • [MeSH-major] Pregnancy Complications, Neoplastic / diagnosis. Pregnancy Complications, Neoplastic / therapy. Sarcoma, Alveolar Soft Part / diagnosis. Sarcoma, Alveolar Soft Part / therapy. Tongue Neoplasms / diagnosis. Tongue Neoplasms / therapy

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  • (PMID = 18603509.001).
  • [ISSN] 1875-6263
  • [Journal-full-title] Taiwanese journal of obstetrics & gynecology
  • [ISO-abbreviation] Taiwan J Obstet Gynecol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] China
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34. Bodi I, Gonzalez D, Epaliyange P, Gullan R, Fisher C: Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion. Neuropathology; 2009 Aug;29(4):460-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion.
  • Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor.
  • Alveolar soft part sarcoma (ASPS) is a rare but well-studied subtype of sarcoma.
  • The histological features were strongly suggestive of ASPS.
  • At the molecular level RT-PCR and sequencing analysis demonstrated ASPCR1-TFE3 fusion confirming the histological diagnosis of ASPS.
  • ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course.
  • This case may represent primary meningeal ASPS although metastatic deposit from an undiscovered primary site cannot be entirely excluded.
  • [MeSH-major] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics. Biomarkers, Tumor / genetics. Gene Fusion / genetics. Meningeal Neoplasms / genetics. Sarcoma, Alveolar Soft Part / genetics

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  • (PMID = 18713311.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Biomarkers, Tumor; 0 / TFE3 protein, human
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35. Kim YD, Lee CH, Lee MK, Jeong YJ, Kim JY, Park DY, Sol MY: Primary alveolar soft part sarcoma of the lung. J Korean Med Sci; 2007 Apr;22(2):369-72
MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary alveolar soft part sarcoma of the lung.
  • Alveolar soft part sarcoma (ASPS) is a rare epithelial-like soft tissue sarcoma.
  • Primary pulmonary involvement of this sarcoma, without evidence of soft tissue tumor elsewhere, is very exceptional.
  • We present a case of primary ASPS of the lung in a 42-yr-old woman.
  • Histologically, the tumor consisted of alveolar nests of large polygonal tumor cells, the cytoplasm of which frequently revealed periodic acid-Schiff-positive, diastase-resistant intracytoplasmic rod-like structures.
  • [MeSH-major] Lung Neoplasms / pathology. Lung Neoplasms / radiography. Pulmonary Alveoli / pathology. Pulmonary Alveoli / radiography. Sarcoma / pathology. Sarcoma / radiography
  • [MeSH-minor] Adult. Female. Humans. Rare Diseases / pathology. Rare Diseases / radiography. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / radiography

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  • [Cites] Oncology. 2000 May;58(4):319-23 [10838498.001]
  • [Cites] Oncogene. 2001 Jan 4;20(1):48-57 [11244503.001]
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  • (PMID = 17449953.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2693611
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36. Lim HS, Heo SH, Park JG, Kang HK: Metastatic alveolar soft part sarcoma of the breast. J Ultrasound Med; 2006 Jul;25(7):929-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic alveolar soft part sarcoma of the breast.
  • [MeSH-major] Breast Neoplasms / secondary. Sarcoma / secondary. Soft Tissue Neoplasms / pathology

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  • (PMID = 16798907.001).
  • [ISSN] 0278-4297
  • [Journal-full-title] Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine
  • [ISO-abbreviation] J Ultrasound Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 9
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37. Ward E, Doody O, d'Adhemar C, Swan N, Torreggiani WC: Answer to case of the month #149 alveolar soft-part sarcoma. Can Assoc Radiol J; 2009 Jun;60(3):143-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Answer to case of the month #149 alveolar soft-part sarcoma.
  • [MeSH-major] Lung Neoplasms / diagnosis. Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adult. Biopsy. Contrast Media. Cough / etiology. Diagnosis, Differential. Fatigue / etiology. Gadolinium. Humans. Image Enhancement / methods. Knee / pathology. Lung / radiography. Magnetic Resonance Imaging. Male. Rare Diseases

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  • (PMID = 19591765.001).
  • [ISSN] 0846-5371
  • [Journal-full-title] Canadian Association of Radiologists journal = Journal l'Association canadienne des radiologistes
  • [ISO-abbreviation] Can Assoc Radiol J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Contrast Media; AU0V1LM3JT / Gadolinium
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38. Bisogno G, Rosolen A, Carli M: Interferon alpha for alveolar soft part sarcoma. Pediatr Blood Cancer; 2005 Jun 15;44(7):687-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Interferon alpha for alveolar soft part sarcoma.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Interferon-alpha / therapeutic use. Sarcoma, Alveolar Soft Part / drug therapy

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  • (PMID = 15602705.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Interferon-alpha; 0 / Recombinant Proteins; 99210-65-8 / interferon alfa-2b
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39. Koguchi Y, Yamaguchi T, Yamato M, Osada D, Saotome K: Alveolar soft part sarcoma of bone. J Orthop Sci; 2005 Nov;10(6):652-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of bone.
  • [MeSH-major] Bone Neoplasms / diagnosis. Ilium / pathology. Sarcoma, Alveolar Soft Part / diagnosis

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  • (PMID = 16307193.001).
  • [ISSN] 0949-2658
  • [Journal-full-title] Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association
  • [ISO-abbreviation] J Orthop Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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40. Afshar AR, Abbasi F: Photoclinic. Primary alveolar soft part sarcoma of bone. Arch Iran Med; 2006 Apr;9(2):175-6, 181
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Photoclinic. Primary alveolar soft part sarcoma of bone.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / diagnosis. Sarcoma, Alveolar Soft Part / surgery

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  • (PMID = 16649367.001).
  • [ISSN] 1029-2977
  • [Journal-full-title] Archives of Iranian medicine
  • [ISO-abbreviation] Arch Iran Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Iran
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41. Ishikawa M, Ishizuka O, Nakayama T, Kobayashi S, Igawa Y, Nishizawa O, Sugimoto K, Yamashita T, Matsushita T: Alveolar soft-part sarcoma of the retroperitoneum. Int J Urol; 2006 Oct;13(10):1355-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma of the retroperitoneum.
  • The pathological diagnosis was alveolar soft-part sarcoma (ASPS).
  • Alveolar soft-part sarcoma is a rare soft-tissue tumor that accounts for approximately 0.5-1% of soft-tissue sarcomas.
  • Herein is reported a case of ASPS of the retroperitoneum with radiological and pathological findings.
  • [MeSH-major] Retroperitoneal Neoplasms / diagnosis. Sarcoma, Alveolar Soft Part / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 17010020.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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42. Jun HJ, Lee J, Lim DH, Park JO, Ahn G, Seo SW, Sung KS, Lim DH, Yoo KH, Choi YL: Expression of MET in alveolar soft part sarcoma. Med Oncol; 2010 Jun;27(2):459-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Expression of MET in alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma which is characterized by the presence of a specific chromosomal translocation encoding the chimeric transcription factor (ASPL-TFE3) that activates expression of MET.
  • We reviewed the clinical features and treatment outcome of 12 ASPS patients.
  • The high expression of MET in ASPL-TFE3 (+) ASPS may further support the potential role of targeted agents against MET in this rare, chemoresistant tumor.
  • [MeSH-major] Gene Expression Regulation, Neoplastic. Proto-Oncogene Proteins c-met / biosynthesis. Receptors, Growth Factor / biosynthesis. Sarcoma, Alveolar Soft Part / pathology

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  • (PMID = 19472090.001).
  • [ISSN] 1559-131X
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Growth Factor; EC 2.7.10.1 / MET protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-met
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43. Lazar AJ, Das P, Tuvin D, Korchin B, Zhu Q, Jin Z, Warneke CL, Zhang PS, Hernandez V, Lopez-Terrada D, Pisters PW, Pollock RE, Lev D: Angiogenesis-promoting gene patterns in alveolar soft part sarcoma. Clin Cancer Res; 2007 Dec 15;13(24):7314-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Angiogenesis-promoting gene patterns in alveolar soft part sarcoma.
  • PURPOSE: We examined a cohort of patients with alveolar soft part sarcoma (ASPS) treated at our institution and showed the characteristic ASPSCR1-TFE3 fusion transcript in their tumors.
  • EXPERIMENTAL DESIGN: Medical records of 71 patients with ASPS presenting at the University of Texas M.D.
  • RNA extracted from available fresh-frozen and formalin-fixed paraffin-embedded human ASPS tumors were analyzed for ASPSCR1-TFE3 fusion transcript expression using reverse transcription-PCR and by angiogenesis oligomicroarrays with immunohistochemical confirmation.
  • ASPSCR1-TFE3 fusion transcripts were identified in 16 of 18 ASPS samples.
  • In the three frozen samples subjected to an angiogenesis oligoarray, 18 angiogenesis-related genes were up-regulated in tumor over adjacent normal tissue.
  • Immunohistochemistry for jag-1, midkine, and angiogenin in 33 human ASPS samples confirmed these results.
  • Comparison with other sarcomas indicates that the ASPS angiogenic signature is unique.
  • CONCLUSION: ASPS is a highly vascular and metastatic tumor with a surprisingly favorable outcome; therapeutically resistant metastases drive mortality.
  • Future molecular therapies targeting overexpressed angiogenesis-promoting proteins (such as those identified here) could benefit patients with ASPS.
  • [MeSH-major] Neovascularization, Pathologic / genetics. Sarcoma, Alveolar Soft Part / genetics
  • [MeSH-minor] Adult. Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics. Female. Humans. Immunohistochemistry. Kaplan-Meier Estimate. Male. Neoplasm Proteins / genetics. Oncogene Proteins, Fusion / genetics. RNA, Messenger / analysis. Reverse Transcriptase Polymerase Chain Reaction. Survival Analysis. Tissue Array Analysis

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  • (PMID = 18094412.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion; 0 / RNA, Messenger; 0 / TFE3 protein, human
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44. Vistica DT, Krosky PM, Kenney S, Raffeld M, Shoemaker RH: Immunohistochemical discrimination between the ASPL-TFE3 fusion proteins of alveolar soft part sarcoma. J Pediatr Hematol Oncol; 2008 Jan;30(1):46-52
eagle-i research resources. PMID 18176180 (Special Collections) .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Immunohistochemical discrimination between the ASPL-TFE3 fusion proteins of alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS), a rare soft tissue sarcoma, is characterized by a chromosomal translocation der(17)t(X;17)(p11;q25) resulting in the production of 2 fusion proteins encoded by regions of the genes for alveolar soft part locus (ASPL) and the transcription factor E3 (TFE3).
  • Immunohistochemical staining of formalin-fixed, paraffin-embedded ASPS tumors with the fusion-specific antibodies resulted in intense nuclear staining and differentiation between tumors that express the type 1 protein and tumors that express the type 2 protein.
  • These antibodies will be useful for the differential diagnosis of type 1 and type 2 ASPS and also in the detection of the fusion proteins in biochemical and cell biologic investigations.
  • [MeSH-major] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / metabolism. Cell Nucleus / metabolism. Neoplasm Proteins / metabolism. Oncogene Proteins, Fusion / metabolism. Rare Diseases / metabolism. Sarcoma / metabolism
  • [MeSH-minor] Adolescent. Antibodies / chemistry. Antibodies / immunology. Child. Child, Preschool. Diagnosis, Differential. Enzyme-Linked Immunosorbent Assay. Female. Humans. Immunohistochemistry. Male. Recombinant Proteins / chemistry. Recombinant Proteins / genetics. Recombinant Proteins / immunology. Translocation, Genetic / immunology

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  • (PMID = 18176180.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CO / N01-CO-12400
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Antibodies; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion; 0 / Recombinant Proteins; 0 / TFE3 protein, human
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45. Xin FY, Rana N, Ming Z, Lang YB: Alveolar soft part sarcoma of the retro peritoneum. J Cancer Res Ther; 2010 Jan-Mar;6(1):117-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the retro peritoneum.
  • Alveolar Soft Part Sarcoma (ASPS), also called Alveolar Soft-Tissue Sarcoma, is a rare type of soft-tissue neoplasm with a poor long term prognosis.
  • The pathological diagnosis was ASPS.
  • [MeSH-major] Retroperitoneal Neoplasms / pathology. Sarcoma, Alveolar Soft Part / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 20479565.001).
  • [ISSN] 1998-4138
  • [Journal-full-title] Journal of cancer research and therapeutics
  • [ISO-abbreviation] J Cancer Res Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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46. Stacchiotti S, Tamborini E, Marrari A, Brich S, Rota SA, Orsenigo M, Crippa F, Morosi C, Gronchi A, Pierotti MA, Casali PG, Pilotti S: Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res; 2009 Feb 1;15(3):1096-104
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response to sunitinib malate in advanced alveolar soft part sarcoma.
  • PURPOSE: Alveolar soft part sarcoma (ASPS) is a rare, chemoresistant soft tissue sarcoma.
  • ASPS harbors the t(17-X) (p11.2;q25) translocation, resulting in the ASPACR1-TFE3 fusion protein, causing MET autophosphorylation and activation of downstream signaling.
  • EXPERIMENTAL DESIGN: Since July 2007, five patients with progressive metastatic ASPS have been treated with continuous SM 37.5 mg/d on a named basis.
  • One had a RECIST stable disease.
  • CONCLUSION: SM may have antitumor activity in ASPS, possibly through a mechanism involving PDGFR and RET.
  • [MeSH-major] Indoles / therapeutic use. Protein Kinase Inhibitors / therapeutic use. Pyrroles / therapeutic use. Sarcoma, Alveolar Soft Part / drug therapy

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  • (PMID = 19188185.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Indoles; 0 / Protein Kinase Inhibitors; 0 / Pyrroles; 0 / sunitinib; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor; EC 2.7.10.1 / Receptors, Vascular Endothelial Growth Factor; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt
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47. Aisner SC, Beebe K, Blacksin M, Mirani N, Hameed M: Primary alveolar soft part sarcoma of fibula demonstrating ASPL-TFE3 fusion: a case report and review of the literature. Skeletal Radiol; 2008 Nov;37(11):1047-51
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary alveolar soft part sarcoma of fibula demonstrating ASPL-TFE3 fusion: a case report and review of the literature.
  • Alveolar soft part sarcoma is a rare soft tissue tumor typically affecting young adults.
  • These tumors are most often seen in the deep soft tissues of the extremities and patients generally present with advanced disease.
  • This is the first case of alveolar soft part sarcoma in bone documenting the ASPL-TFE3 gene product.
  • Herein, we report a rare presentation of alveolar soft part sarcoma presenting as a primary bone neoplasm involving the proximal fibula in a 41-year-old woman.
  • [MeSH-major] Fibula / pathology. Sarcoma, Alveolar Soft Part / diagnosis
  • [MeSH-minor] Adult. Basic Helix-Loop-Helix Leucine Zipper Transcription Factors. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Oncogene Proteins, Fusion. Tomography, X-Ray Computed

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  • (PMID = 18594808.001).
  • [ISSN] 0364-2348
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / TFE3 protein, human
  • [Number-of-references] 23
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48. Runge M, Katranji H, Viennet G, Kantelip B, Bonneville JF: [Alveolar soft-part sarcoma]. J Radiol; 2008 Jan;89(1 Pt 1):64-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Alveolar soft-part sarcoma].
  • [Transliterated title] Sarcome alvéolaire des tissus mous.
  • [MeSH-major] Head and Neck Neoplasms / diagnosis. Parietal Bone / pathology. Sarcoma, Alveolar Soft Part / diagnosis. Scalp / pathology. Skin Neoplasms / diagnosis. Skull Neoplasms / diagnosis

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  • (PMID = 18288030.001).
  • [ISSN] 0221-0363
  • [Journal-full-title] Journal de radiologie
  • [ISO-abbreviation] J Radiol
  • [Language] fre
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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49. Noussios G, Chouridis P, Petropoulos I, Karagiannidis K, Kontzoglou G: Alveolar soft part sarcoma of the tongue in a 3-year-old boy: a case report. J Med Case Rep; 2010;4:130

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the tongue in a 3-year-old boy: a case report.
  • INTRODUCTION: Alveolar soft tissue sarcoma of the tongue is a very rare and aggressive tumor which occurs in infancy with a relatively controversial histogenesis.
  • CASE PRESENTATION: We present the case of a three-year-old Caucasian boy who was admitted to our department with a history of dysphagia and two episodes of oral bleeding which had lasted for a period of six months.
  • A thorough histological examination together with imaging techniques form the basis of a reliable diagnosis.
  • CONCLUSION: Alveolar soft tissue sarcoma of the tongue is a rare tumor which occurs in infancy and which is often misdiagnosed pre-operatively.
  • It should therefore be included in the differential diagnosis of oral pediatric lesions.

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  • [Cites] Am J Surg Pathol. 1986 Mar;10(3):212-8 [3513637.001]
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  • (PMID = 20459684.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2873458
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50. Gardner K, Judson I, Leahy M, Barquin E, Marotti M, Collins B, Young H, Scurr M: Activity of cediranib, a highly potent and selective VEGF signaling inhibitor, in alveolar soft part sarcoma. J Clin Oncol; 2009 May 20;27(15_suppl):10523

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Activity of cediranib, a highly potent and selective VEGF signaling inhibitor, in alveolar soft part sarcoma.
  • : 10523 Background: Alveolar soft part sarcoma (ASPS) is a rare entity making up <1% of soft tissue sarcomas (STS).
  • It is typically indolent but with a high incidence of metastatic disease, usually to lungs, but also to sites such as the brain.
  • This is a preliminary report of the activity of cediranib, a highly potent and selective VEGF signaling inhibitor, in this disease.
  • METHODS: Efficacy and tolerability data were collected for seven patients with ASPS.
  • RESULTS: Median age at diagnosis was 39 years (range: 26-49).
  • All patients had pulmonary metastases and two had additional sites of disease (brain, bone, intra-abdominal) at study entry.
  • Four patients had a best response of partial response, two patients had a confirmed reduction in maximum tumor diameter of ≥10% and <30% and one patient experienced stable disease.
  • CONCLUSIONS: These data demonstrate the promising preliminary activity and safety of chronic administration of cediranib in this disease.
  • Further investigation is warranted, particularly as there is no effective systemic treatment for patients with advanced ASPS.

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  • (PMID = 27963912.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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51. Bouzit K, Bonnaire B, Cordonnier C, Sevestre H, Remond A: [Alveolar soft part sarcoma: a case report]. J Radiol; 2009 Mar;90(3 Pt 1):327-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Alveolar soft part sarcoma: a case report].
  • [Transliterated title] Imagerie du sarcome alvéolaire des parties molles: à propos d'un cas.
  • [MeSH-minor] Angiography. Biopsy. Humans. Immunohistochemistry. Magnetic Resonance Angiography. Magnetic Resonance Imaging. Male. Reverse Transcriptase Polymerase Chain Reaction. Sarcoma, Alveolar Soft Part / diagnosis. Sarcoma, Alveolar Soft Part / genetics. Sarcoma, Alveolar Soft Part / pathology. Sarcoma, Alveolar Soft Part / radiography. Sarcoma, Alveolar Soft Part / surgery. Sarcoma, Alveolar Soft Part / ultrasonography. Tomography, X-Ray Computed. Ultrasonography, Doppler. Young Adult

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  • (PMID = 19421121.001).
  • [ISSN] 0221-0363
  • [Journal-full-title] Journal de radiologie
  • [ISO-abbreviation] J Radiol
  • [Language] fre
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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52. Van Buren R, Stewart J 3rd: Alveolar soft part sarcoma presenting as a breast mass in a 13-year-old female. Diagn Cytopathol; 2009 Feb;37(2):122-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma presenting as a breast mass in a 13-year-old female.
  • We report a clinically unsuspected case of alveolar soft part sarcoma (ASPS) presenting in the breast of a 13-year-old female.
  • ASPS is a rare neoplasm that is usually present in the head, neck, or lower extremities.
  • This rare case presentation gives us the opportunity to review the differential diagnosis of adolescent breast lesions.
  • We also will review diagnostic features of ASPS on fine-needle aspiration.
  • These are relatively rare disease processes about which many cytopathology professionals may be unfamiliar.
  • [MeSH-major] Breast Neoplasms / diagnosis. Sarcoma, Alveolar Soft Part / diagnosis
  • [MeSH-minor] Adolescent. Biopsy, Fine-Needle. Breast / pathology. Breast Diseases / diagnosis. Breast Diseases / pathology. Diagnosis, Differential. Female. Humans

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  • (PMID = 19021216.001).
  • [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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53. Guntupalli S, Anderson ML, Bodurka DC: Alveolar soft part sarcoma of the cervix: case report and literature review. Arch Gynecol Obstet; 2009 Feb;279(2):263-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the cervix: case report and literature review.
  • Alveolar soft part sarcoma (ASPS) is a rare mesenchymal malignancy typically found in the extremities or chest of young adults.
  • We present a case of an alveolar soft part sarcoma arising in the endocervix of a 38-year-old premenopausal woman.
  • No evidence of metastatic disease was found after extensive surgical staging.
  • Since treatment, she has since remained disease-free for more than 5 years without additional therapy.
  • This is the second case of ASPS arising in the endocervix of which we are aware.
  • Our observations suggest simple hysterectomy suffices for optimal clinical management of cervical ASPS and that surgical staging of this disease offers little prognostic benefit.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / diagnosis. Uterine Cervical Neoplasms / diagnosis


54. Folpe AL, Deyrup AT: Alveolar soft-part sarcoma: a review and update. J Clin Pathol; 2006 Nov;59(11):1127-32
SciCrunch. KEGG: Data: Disease Annotation .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma: a review and update.
  • Alveolar soft-part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young patients.
  • Recently, our understanding of the genetic events underlying the pathogenesis of ASPS has greatly increased.
  • The historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of ASPS are reviewed in this article.
  • [MeSH-major] Sarcoma, Alveolar Soft Part
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Diagnosis, Differential. Humans. Translocation, Genetic

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  • (PMID = 17071801.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 63
  • [Other-IDs] NLM/ PMC1860509
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55. Madrigrano A, Beach B, Wheeler A, Wapnir I: Metastases to the breast: alveolar soft part sarcoma in adolescents. Clin Breast Cancer; 2008 Feb;8(1):92-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastases to the breast: alveolar soft part sarcoma in adolescents.
  • This is a case report of an 18-year-old woman with an alveolar soft part sarcoma metastatic to the breast.
  • [MeSH-major] Breast Neoplasms / secondary. Sarcoma, Alveolar Soft Part / secondary. Skin Neoplasms / pathology


56. Anderson ME, Hornicek FJ, Gebhardt MC, Raskin KA, Mankin HJ: Alveolar soft part sarcoma: a rare and enigmatic entity. Clin Orthop Relat Res; 2005 Sep;438:144-8
Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma: a rare and enigmatic entity.
  • Alveolar soft part sarcoma is a rare malignant tumor with unusual clinical behavior.
  • Treatment of alveolar soft part sarcoma has been difficult to evaluate because of the small numbers of cases seen, but it seemed that although treatment of the primary tumor in alveolar soft part sarcoma often is successful, treatment of metastatic tumors is unsuccessful.
  • A review of outcome after treatment of primary and metastatic disease in the 15 patients in our database with alveolar soft part sarcoma was done in order to evaluate this issue.
  • Nine of 15 patients presented with metastatic disease and one further patient developed metastases.
  • Adjuvant chemotherapy was administered to one patient with localized disease and to six patients with metastatic disease.
  • All instances of the metastatic disease either recurred or progressed.
  • Overall survival was 75% at 5 years and 40% at 10 years with a mean survival of 6.5 years, despite the high number of patients with metastatic disease.
  • New approaches to treatment of metastatic alveolar soft part sarcoma must be investigated and applied.
  • [MeSH-major] Neoplasm Metastasis / pathology. Sarcoma, Alveolar Soft Part / secondary. Soft Tissue Neoplasms / pathology

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  • (PMID = 16131883.001).
  • [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
  • [Chemical-registry-number] 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; NR7O1405Q9 / Mesna; UM20QQM95Y / Ifosfamide; MAID protocol
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57. Park YJ, Huh SJ, Han J, Kim B, Lee JW, Bae DS: A case of alveolar soft part sarcoma with vaginal metastasis: successful control of vaginal bleeding with external beam irradiation. Int J Gynecol Cancer; 2005 Nov-Dec;15(6):1166-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of alveolar soft part sarcoma with vaginal metastasis: successful control of vaginal bleeding with external beam irradiation.
  • Alveolar soft part sarcoma (ASPS) of the vagina is an exceptionally rare neoplasm.
  • Furthermore, vaginal metastasis of ASPS has not been reported.
  • A 28-year-old woman with a history of a right thigh mass diagnosed as ASPS excised 8 years ago presented to the emergency room with massive vaginal bleeding and anemia.
  • Biopsy of a vaginal mass revealed that the tumor was a vaginal metastasis of ASPS.
  • This is the first case of vaginal metastasis of ASPS reported in the literature that was manifested by intractable vaginal bleeding, which was controlled successfully with radiation therapy.
  • [MeSH-major] Hemorrhage / radiotherapy. Lung Neoplasms / therapy. Sarcoma / therapy. Vaginal Neoplasms / radiotherapy


58. Wu J, Brinker DA, Haas M, Montgomery EA, Argani P: Primary alveolar soft part sarcoma (ASPS) of the breast: report of a deceptive case with xanthomatous features confirmed by TFE3 immunohistochemistry and electron microscopy. Int J Surg Pathol; 2005 Jan;13(1):81-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary alveolar soft part sarcoma (ASPS) of the breast: report of a deceptive case with xanthomatous features confirmed by TFE3 immunohistochemistry and electron microscopy.
  • Alveolar soft part sarcoma (ASPS) is a rare neoplasm that most commonly presents in the lower extremities.
  • Although ASPS has distinctive histologic features, it may cause diagnostic problems when it arises in unusual locations.
  • To our knowledge, only 1 case of ASPS arising within the breast has previously been reported.
  • Here, we report a second case of primary mammary ASPS.
  • The excision more clearly revealed the lesion's alveolar architecture and demonstrated cells with more eosinophilic cytoplasm, along with the xanthomatous cells.
  • The diagnosis of ASPS was confirmed by electron microscopy, which revealed characteristic membrane-bound rhomboidal crystals, as well as by nuclear labeling for TFE3 protein by immunohistochemistry.
  • With this report, we confirm the utility of a novel immunohistochmical technique for the identification of an ASPS presenting in an unusual locale.
  • [MeSH-major] Breast Neoplasms / pathology. DNA-Binding Proteins. Deoxycytidine / analogs & derivatives. Sarcoma / secondary. Soft Tissue Neoplasms / pathology. Transcription Factors. Xanthomatosis / pathology

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  • (PMID = 15735860.001).
  • [ISSN] 1066-8969
  • [Journal-full-title] International journal of surgical pathology
  • [ISO-abbreviation] Int. J. Surg. Pathol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA88843
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, Differentiation, Myelomonocytic; 0 / Antimetabolites, Antineoplastic; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Biomarkers, Tumor; 0 / CD68 antigen, human; 0 / DNA-Binding Proteins; 0 / TFE3 protein, human; 0 / Transcription Factors; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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59. Kusafuka K, Muramatsu K, Yabuzaki T, Ishiki H, Asano R, Kamijo T, Iida Y, Ebihara M, Onitsuka T, Kameya T: Alveolar soft part sarcoma of the larynx: a case report of an unusual location with immunohistochemical and ultrastructural analyses. Head Neck; 2008 Sep;30(9):1257-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma of the larynx: a case report of an unusual location with immunohistochemical and ultrastructural analyses.
  • BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare mesenchymal neoplasm of uncertain origin.
  • In this article, we report a case of ASPS occurring in the larynx, an extremely rare location for this rather unusual tumor.
  • The tumor showed a proliferation of large polygonal cells with periodic-acid-Schiff-positive diastase-resistant intracytoplasmic granules, arranged in an alveolar growth pattern.
  • This case was finally diagnosed as ASPS of the larynx.
  • CONCLUSION: We discuss the histogenesis and differential diagnosis of ASPS with immunohistochemical and ultrastructural findings.
  • TFE3 immunohistochemistry was found to be a very useful marker for the diagnosis of ASPS.
  • [MeSH-major] Laryngeal Neoplasms / pathology. Laryngoscopy / methods. Sarcoma, Alveolar Soft Part / pathology
  • [MeSH-minor] Adult. Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / analysis. Biopsy, Needle. Disease Progression. Female. Follow-Up Studies. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Neoplasm Staging. Rare Diseases. Risk Assessment. Treatment Outcome

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  • (PMID = 18286485.001).
  • [ISSN] 1097-0347
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / TFE3 protein, human
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60. Hanzer M, Nebl A, Spendel S, Pilhatsch A, Urban C, Benesch M: Necrosis of a skin autograft after short-term treatment with sunitinib in a 14-year-old girl with metastatic alveolar soft part sarcoma of the thigh. Klin Padiatr; 2010 May;222(3):184-6
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  • [Title] Necrosis of a skin autograft after short-term treatment with sunitinib in a 14-year-old girl with metastatic alveolar soft part sarcoma of the thigh.
  • A 14-year-old girl was diagnosed with alveolar soft part sarcoma (ASPS) of the thigh and lung metastases.
  • Since ASPS do not respond to conventional chemotherapy antiangiogenic treatment with peginterferon alfa-2b and thalidomide was started.
  • Immunohistochemical analysis of tumor tissue showed expression of vascular endothelial growth factor receptors (VEGFR) 1, 2, 3, and platelet derived growth factor receptor (PDGFR)-alpha and -beta.
  • [MeSH-major] Angiogenesis Inhibitors / adverse effects. Antineoplastic Agents / adverse effects. Indoles / adverse effects. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Pyrroles / adverse effects. Sarcoma, Alveolar Soft Part / drug therapy. Sarcoma, Alveolar Soft Part / secondary. Soft Tissue Neoplasms / drug therapy. Surgical Flaps / blood supply. Surgical Flaps / pathology. Thigh

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  • (PMID = 20514624.001).
  • [ISSN] 1439-3824
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents; 0 / Indoles; 0 / Pyrroles; 0 / sunitinib
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61. Luo J, Melnick S, Rossi A, Burke RP, Pfeifer JD, Dehner LP: Primary cardiac alveolar soft part sarcoma. A report of the first observed case with molecular diagnostics corroboration. Pediatr Dev Pathol; 2008 Mar-Apr;11(2):142-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary cardiac alveolar soft part sarcoma. A report of the first observed case with molecular diagnostics corroboration.
  • A case of primary alveolar soft part sarcoma (ASPS) of the heart is reported in an 11-year-old female as 1 of 16 cases of ASPS presenting in the first 2 decades of life in our institutional 17-year review period.
  • The classic alveolar or organoid pattern was inconspicuous as compared to a more diffuse or formless pattern consisting of a population of uniform round cells with abundant eosinophilic cytoplasm, but in addition there was a second, minor population of gigantiform tumor cells with a variety of unusual shapes.
  • Other unusual features for ASPS in our case included a lymphohistocytic reaction and zonal necrosis.
  • The only other examples of ASPS involving the heart were 3 cases in the literature of metastatic disease from tumors arising in the soft tissues.
  • This initial case of primary cardiac ASPS joins the list of other types of sarcomas in children that have been reported as primary neoplasms of the heart.
  • [MeSH-major] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics. Heart Neoplasms / pathology. Neoplasm Proteins / genetics. Oncogene Proteins, Fusion / genetics. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 17378669.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion; 0 / RNA, Neoplasm; 0 / TFE3 protein, human
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62. Bu X, Bernstein L: A proposed explanation for female predominance in alveolar soft part sarcoma. Noninactivation of X; autosome translocation fusion gene? Cancer; 2005 Mar 15;103(6):1245-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A proposed explanation for female predominance in alveolar soft part sarcoma. Noninactivation of X; autosome translocation fusion gene?
  • BACKGROUND: Alveolar soft-part sarcoma (ASPS) is a rare malignant soft tissue tumor with both clinically and morphologically distinct features.
  • Recently, ASPS was found to have a nonreciprocal der(17)t(X;17) translocation with the corresponding fusion gene located in chromosome 17.
  • Because females have an extra X-chromosome, their likelihood of developing an X;autosome translocation is theoretically double that of males, and thus, this extra X-chromosome is a likely explanation for female predominance of ASPS.
  • METHODS: The authors used data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry program, which included 87 ASPS cases (33 males and 54 females), and published ASPS cases, which included 317 cases (121 males and 196 females), to test our hypothesis.
  • CONCLUSIONS: Therefore, the authors found a statistical association between the female predominance observed in ASPS and female possession of an extra X-chromosome/noninactivation of the ASPS X;autosome translocation fusion gene.
  • [MeSH-major] Chromosomes, Human, Pair 17. Sarcoma, Alveolar Soft Part / epidemiology. Sarcoma, Alveolar Soft Part / genetics. Soft Tissue Neoplasms / epidemiology. Soft Tissue Neoplasms / genetics. Translocation, Genetic
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. California / epidemiology. Child. Child, Preschool. Female. Genetic Predisposition to Disease. Humans. Incidence. Infant. Male. Middle Aged. Probability. Prognosis. Registries. Retrospective Studies. Risk Assessment. SEER Program. Sex Factors. Survival Analysis

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  • (PMID = 15693033.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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63. Rekhi B, Kumar R, Menon S, Medhi S, Desai SB: Calvarial metastasis of a renal cell carcinoma, mimicking a primary alveolar soft part sarcoma, in a young girl-a rare case report. Pathol Oncol Res; 2009 Mar;15(1):137-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Calvarial metastasis of a renal cell carcinoma, mimicking a primary alveolar soft part sarcoma, in a young girl-a rare case report.
  • We present a rare case of a disseminated RCC in a 15-year-old girl, who primarily presented with an occipital soft tissue mass.
  • Computed tomography (CT) of the head revealed a soft tissue mass in the scalp, eroding the occipital bone and extending intracranially.
  • Biopsy examination showed overlapping features of an alveolar soft part sarcoma (ASPS) and a RCC.
  • Diagnosis of a metastatic RCC was ascertained.
  • The present case represents a rare manifestation of a RCC metastasizing at an unusual location i.e. calvarium in the youngest patient known, so far and masquerading a primary ASPS.
  • It also highlights the value of clinico-patho-radiological correlation, including CD10 as a useful IHC marker in diagnosing a RCC in young patients, especially when histopathological features overlap with ASPS.
  • [MeSH-major] Carcinoma, Renal Cell / secondary. Kidney Neoplasms / pathology. Sarcoma, Alveolar Soft Part / radiography. Skull Neoplasms / secondary


64. Genin O, Rechavi G, Nagler A, Ben-Itzhak O, Nazemi KJ, Pines M: Myofibroblasts in pulmonary and brain metastases of alveolar soft-part sarcoma: a novel target for treatment? Neoplasia; 2008 Sep;10(9):940-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Myofibroblasts in pulmonary and brain metastases of alveolar soft-part sarcoma: a novel target for treatment?
  • Alveolar soft-part sarcoma (ASPS) is a rare neoplasm with chromosomal translocation that results in ASPL-TFE3 fusion.
  • We demonstrated that the ASPS metastases include also stromal myofibroblasts.
  • The pulmonary ASPS myofibroblasts synthesize serum response factor (SRF), a repressor of Smad3-mediated TGFbeta signaling essential for myofibroblast differentiation and Smad3.
  • These results suggest that the myofibroblasts may serve as a novel target for treatment of ASPS metastases.
  • [MeSH-major] Brain Neoplasms / secondary. Lung Neoplasms / secondary. Muscle Neoplasms / pathology. Sarcoma, Alveolar Soft Part / genetics. Smad3 Protein / metabolism

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  • (PMID = 18714394.001).
  • [ISSN] 1476-5586
  • [Journal-full-title] Neoplasia (New York, N.Y.)
  • [ISO-abbreviation] Neoplasia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Oncogene Proteins, Fusion; 0 / SMAD3 protein, human; 0 / Serum Response Factor; 0 / Smad3 Protein; 0 / cytoglobin; 9004-22-2 / Globins; EC 1.14.11.2 / Procollagen-Proline Dioxygenase
  • [Other-IDs] NLM/ PMC2517638
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65. Petersson F, Michal M: Minute alveolar soft part sarcoma of the endocervix: the smallest ever published case. Appl Immunohistochem Mol Morphol; 2009 Dec;17(6):553-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Minute alveolar soft part sarcoma of the endocervix: the smallest ever published case.
  • Alveolar soft part sarcoma (ASPS) is a distinctive mesenchymal tumor of uncertain histogenesis, which is exceedingly rare in the uterine cervix.
  • We herein report the smallest (1.6 mm) ever published case of ASPS of the endocervix with the characteristic histomorphology and immunohistochemical findings.
  • Because of its seemingly much better prognosis, it is important in routine practice to distinguish ASPS from adenocarcinoma of the cervix.
  • This is facilitated by the awareness that ASPS can occur in this location and if there is doubt about the diagnosis on routinely stained sections, the appropriate immunohistochemical study should be performed.
  • Owing to the small number of published ASPSs in the cervix, the optimal treatment strategy has yet to be determined.

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  • (PMID = 19620840.001).
  • [ISSN] 1533-4058
  • [Journal-full-title] Applied immunohistochemistry & molecular morphology : AIMM
  • [ISO-abbreviation] Appl. Immunohistochem. Mol. Morphol.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / TFE3 protein, human
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66. Pennacchioli E, Fiore M, Collini P, Radaelli S, Dileo P, Stacchiotti S, Casali PG, Gronchi A: Alveolar soft part sarcoma: clinical presentation, treatment, and outcome in a series of 33 patients at a single institution. Ann Surg Oncol; 2010 Dec;17(12):3229-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma: clinical presentation, treatment, and outcome in a series of 33 patients at a single institution.
  • BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that usually affects young patients.
  • A comprehensive retrospective review was performed of clinical presentation, treatment, outcome, and patterns of failure in a consecutive series of patients with localized or metastatic ASPS between 1975 and 2008.
  • METHODS: Demographics, tumor sizes, sites and extent of disease, treatments provided, progression-free survival, and overall survival were evaluated.
  • CONCLUSIONS: Prognosis of ASPS is basically related to the characteristics of the disease and the quality of surgery.
  • New agents are eagerly needed to complement surgery to eradicate this disease.
  • [MeSH-major] Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / therapy. Sarcoma, Alveolar Soft Part / pathology. Sarcoma, Alveolar Soft Part / therapy

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  • (PMID = 20593242.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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67. Huang HY, Lui MY, Ladanyi M: Nonrandom cell-cycle timing of a somatic chromosomal translocation: The t(X;17) of alveolar soft-part sarcoma occurs in G2. Genes Chromosomes Cancer; 2005 Oct;44(2):170-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Nonrandom cell-cycle timing of a somatic chromosomal translocation: The t(X;17) of alveolar soft-part sarcoma occurs in G2.
  • Alveolar soft-part sarcoma (ASPS) is a rare malignant soft-tissue tumor of uncertain lineage that provides an opportunity to address this question.
  • The great majority of ASPSs have relatively simple near-diploid karyotypes characterized by an unbalanced der(17)t(X;17)(p11.2;q25), resulting in nonreciprocal fusion of TFE3 with ASPSCR1 (a.k.a.
  • The presence of a normal X along with the der(17)t(X;17) in ASPSs that occur in men has been well described in previous cytogenetic reports and is most readily explained by a translocation in the G2 phase of the cell cycle.
  • To establish whether formation in G2 is a general feature of the t(X;17), we examined polymorphic loci in Xp11.2-->qter in ASPS from 9 women, including 7 with an unbalanced t(X;17).
  • Given that the two derivative chromosomes of a translocation in G2 would be expected to segregate together half the time, the predominance of an unbalanced der(17)t(X;17) also raises the possibility of a selective advantage in ASPS cells for gain of Xp11.2-->pter or loss of 17q25.3-->qter or retention of an active copy of TFE3.
  • [MeSH-major] Chromosomes, Human, Pair 17. Chromosomes, Human, X. G2 Phase. Sarcoma / genetics. Translocation, Genetic

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  • (PMID = 15952162.001).
  • [ISSN] 1045-2257
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA95785
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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68. Goldberg J, Demetri GD, Choy E, Rosen L, Pappo A, Dubois S, Geller J, Chai F, Ferrari D, Wagner AJ: Preliminary results from a phase II study of ARQ 197 in patients with microphthalmia transcription factor family (MiT)-associated tumors. J Clin Oncol; 2009 May 20;27(15_suppl):10502

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • MiT tumors include clear cell sarcoma (CCS), alveolar soft part sarcoma (ASPS), and translocation-associated renal cell carcinoma (TLA RCC) and are linked biologically by a shared activated transcriptional mechanism which directly upregulates c-Met.
  • Tumors with this type of chromosomal abnormality are generally resistant to all approved therapies and, in the absence of complete surgical resection, prove invariably fatal.
  • RESULTS: To date, 28 pts (19 females, 9 males; median age = 21; 7 CCS, 17 ASPS, 4 RCC) have been treated.
  • One pt with CCS demonstrated a confirmed PR, 15 pts (10 ASPS, 2 CCS, 3 RCC) demonstrated stable disease (SD) for durations up to 29+ weeks, and 4 pts progressed.
  • An overall response rate of 5% and a disease control rate (CR+PR+SD) of 80% were demonstrated among 20 pts who were evaluable for efficacy.

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  • (PMID = 27963690.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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69. Pang LJ, Chang B, Zou H, Qi Y, Jiang JF, Li HA, Hu WH, Chen YZ, Liu CX, Zhang WJ, Li F: Alveolar soft part sarcoma: a bimarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues. Diagn Mol Pathol; 2008 Dec;17(4):245-52
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma: a bimarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues.
  • Alveolar soft part sarcoma (ASPS) is a malignancy with low incidence, but with poor prognosis if misdiagnosed.
  • Immunohistochemical assay using TFE3 antibody has been shown to be a sensitive technique for ASPS diagnosis.
  • A specific chromosomal translocation, t(X;17)(p11.2;q25), results in the ASPL-TFE3 fusion gene: it is detectable using reverse-transcription polymerase chain reaction (RT-PCR) in frozen tumor tissues of ASPS.
  • However, the diagnostic usefulness of these markers has not been investigated in Chinese ASPS patients.
  • Here, we report the first systematic study applying TFE3 immunoassay and ASPL-TFE3 fusion transcript detection to archival paraffin-embedded tissues in a large Chinese ASPS patient population.
  • Sixteen patients had been diagnosed with ASPS (age, 3 to 58 y; 3 male patients and 13 female patients).
  • ASPS tumors showed the best immunoreactivity to the TFE3 antibody (16/16).
  • Using a strategy of RT-PCR, followed by a nested PCR with a different primer set, we were able to detect the expression of the chimeric ASPL-TFE3 mRNA in 11 of the 16 ASPS tumors.
  • Our results demonstrate that the "bimarker strategy," a combination of TFE3 immunostaining and ASPL-TFE3 chimeric transcript detection, might have sufficient sensitivity and specificity in diagnosing most of the ASPSs.
  • [MeSH-major] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / analysis. Oncogene Proteins, Fusion / genetics. Pathology, Surgical / methods. RNA Precursors / analysis. Sarcoma, Alveolar Soft Part / diagnosis

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  • (PMID = 18382356.001).
  • [ISSN] 1533-4066
  • [Journal-full-title] Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • [ISO-abbreviation] Diagn. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / RNA Precursors; 0 / TFE3 protein, human
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70. Lai YC, Chiou HJ, Wu HT, Chou YH, Wang HK, Chen PC: Ultrasonographic and MR findings of alveolar soft part sarcoma. J Chin Med Assoc; 2009 Jun;72(6):336-9
Genetic Alliance. consumer health - Alveolar Soft Part Sarcoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ultrasonographic and MR findings of alveolar soft part sarcoma.
  • Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma, commonly occurring in children and adolescents.
  • We present a 19-year-old female with ASPS in her right lower leg.
  • In a mass with hypervascularity and very low RI on sonography and hypervascularity with flow voids on magnetic resonance imaging, ASPS should be considered.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Thigh

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  • (PMID = 19541571.001).
  • [ISSN] 1726-4901
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
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71. Marchac A, Picard A, Landman-Parker J, Larroquet M, Vazquez MP, Franchi G: [A pediatric case of Alveolar Soft Part Sarcoma]. Rev Stomatol Chir Maxillofac; 2007 Dec;108(6):547-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A pediatric case of Alveolar Soft Part Sarcoma].
  • [Transliterated title] Sarcome alvéolaire de la langue: un cas pédiatrique.
  • INTRODUCTION: Alveolar Soft Part Sarcoma (ASPS) is a rare malignant tumor arising from skeletal muscles and occurring usually in young adults.
  • At time of diagnosis the disease is usually advanced and lung metastasis are present in 65% of the cases.
  • The translocation (X; 17), producing an ASPL-TFE3 transcript fusion which is detected on tumoral cells wall, is specific of ASPS.
  • CLINICAL REPORT: We report the case of a child with an ASPS of the tongue and lung metastasis.
  • Firstly this type of sarcoma is very exceptional in a 2-year-old child.
  • Secondly ASPS is not usually susceptible to chemotherapy.
  • [MeSH-major] Sarcoma, Alveolar Soft Part / diagnosis. Tongue Neoplasms / diagnosis

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  • (PMID = 17964622.001).
  • [ISSN] 0035-1768
  • [Journal-full-title] Revue de stomatologie et de chirurgie maxillo-faciale
  • [ISO-abbreviation] Rev Stomatol Chir Maxillofac
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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72. Schultheis B, Kummer G, Tannapfel A, Strumberg D: Successful sequential antiangiogenic therapy for alveolar soft part sarcoma - a case report. Int J Clin Pharmacol Ther; 2010 Jul;48(7):468-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Successful sequential antiangiogenic therapy for alveolar soft part sarcoma - a case report.
  • [MeSH-major] Angiogenesis Inhibitors / administration & dosage. Sarcoma, Alveolar Soft Part / drug therapy

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  • (PMID = 20557847.001).
  • [ISSN] 0946-1965
  • [Journal-full-title] International journal of clinical pharmacology and therapeutics
  • [ISO-abbreviation] Int J Clin Pharmacol Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors
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73. Kasashima S, Minato H, Kobayashi M, Ueda Y, Oda Y, Hashimoto S, Inoue M: Alveolar soft part sarcoma of the endometrium with expression of CD10 and hormone receptors. APMIS; 2007 Jul;115(7):861-5
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  • [Title] Alveolar soft part sarcoma of the endometrium with expression of CD10 and hormone receptors.
  • Alveolar soft part sarcoma (ASPS) is a rare tumor of uncertain histogenesis, mainly localized in the extremities.
  • ASPS originating in the uterine corpus is quite rare; only eight such cases have been reported in the literature.
  • We here present another case of ASPS found in the endometrium in a 50-year-old woman.
  • Metastatic malignant tumor, including ASPS from other organs, was excluded by physical examination and imaging modalities.
  • The tumor showed characteristic histological features of ASPS: alveolar architecture with fibrovascular septa and abundant eosinophilic granular cytoplasm with periodic acid-Schiff-positive crystalline material.
  • Diffuse nuclear immunoreactivity for TFE3, a marker recently reported to be specific for ASPS, further supported the diagnosis of ASPS.
  • [MeSH-major] Endometrial Neoplasms / diagnosis. Neprilysin / analysis. Receptors, Estrogen / analysis. Receptors, Progesterone / analysis. Sarcoma, Alveolar Soft Part / diagnosis

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  • (PMID = 17614855.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Receptors, Estrogen; 0 / Receptors, Progesterone; 0 / TFE3 protein, human; EC 3.4.24.11 / Neprilysin
  • [Number-of-references] 15
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74. Campbell B, Seymour JF, Wheeler G, Sexton M: Alveolar soft-part sarcoma: a cardiac metastasis as a rare site of relapse. Am J Clin Oncol; 2006 Aug;29(4):422-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma: a cardiac metastasis as a rare site of relapse.
  • [MeSH-major] Heart Neoplasms / secondary. Sarcoma, Alveolar Soft Part / secondary. Soft Tissue Neoplasms / pathology

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  • (PMID = 16891875.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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75. Min KW, Na W, Oh YH, Park YW, Park MH: Fine needle aspiration cytology of alveolar soft part sarcoma of the cheek. Cytopathology; 2010 Jun;21(3):205-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Fine needle aspiration cytology of alveolar soft part sarcoma of the cheek.
  • [MeSH-major] Cheek / pathology. Sarcoma, Alveolar Soft Part / pathology

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  • (PMID = 19832880.001).
  • [ISSN] 1365-2303
  • [Journal-full-title] Cytopathology : official journal of the British Society for Clinical Cytology
  • [ISO-abbreviation] Cytopathology
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
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76. Kumar M, Patne S, Vishwanath A, Hasan Z: Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy. Indian J Cancer; 2010 Apr-Jun;47(2):234-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Sarcoma, Alveolar Soft Part / drug therapy. Sarcoma, Alveolar Soft Part / surgery. Tongue Neoplasms / drug therapy. Tongue Neoplasms / surgery

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  • (PMID = 20448401.001).
  • [ISSN] 1998-4774
  • [Journal-full-title] Indian journal of cancer
  • [ISO-abbreviation] Indian J Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] India
  • [Chemical-registry-number] 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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77. Lin YY, Hsieh TC, Kao CH, Wang CH, Wu YC, Yen KY, Sun SS: Bone scintigraphic images of a patient with unusual metastatic alveolar soft-part sarcoma. Clin Nucl Med; 2009 Nov;34(11):806-7
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  • [Title] Bone scintigraphic images of a patient with unusual metastatic alveolar soft-part sarcoma.
  • [MeSH-major] Bone Neoplasms / radionuclide imaging. Bone Neoplasms / secondary. Sarcoma, Alveolar Soft Part / pathology. Sarcoma, Alveolar Soft Part / radionuclide imaging. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / radionuclide imaging

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  • (PMID = 19851182.001).
  • [ISSN] 1536-0229
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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78. BaniHani MN, Al Manasra AR: Spontaneous regression in alveolar soft part sarcoma: case report and literature review. World J Surg Oncol; 2009;7:53
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  • [Title] Spontaneous regression in alveolar soft part sarcoma: case report and literature review.
  • BACKGROUND: Sarcomas are a type of malignant tumors that arise from connective tissue.
  • Pathological diagnosis for all lesions was Alveolar soft part sarcoma.
  • CONCLUSION: ASPS is a rare type of sarcomas that affect primarily the lower limbs.
  • [MeSH-major] Neoplasm Regression, Spontaneous. Sarcoma, Alveolar Soft Part / pathology

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  • (PMID = 19515237.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2703639
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79. Roma AA, Yang B, Senior ME, Goldblum JR: TFE3 immunoreactivity in alveolar soft part sarcoma of the uterine cervix: case report. Int J Gynecol Pathol; 2005 Apr;24(2):131-5
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  • [Title] TFE3 immunoreactivity in alveolar soft part sarcoma of the uterine cervix: case report.
  • SUMMARY: : Alveolar soft part sarcoma (ASPS) is an uncommon neoplasm that has only rarely been reported in the female genital tract.
  • We report a case of incidentally discovered ASPS in the uterine cervix of a 39-year-old woman that exhibited immunoreactivity for TFE3, a recently described marker of ASPS.
  • Strong nuclear immunoreactivity for TFE3 in tumors characterized by a chromosomal translocation involving the TFE3 gene has been reported in ASPS and a subset of pediatric renal cell carcinomas.
  • To the best of our knowledge, this is the first report of ASPS of the female genital tract with immunoreactivity for TFE3.
  • [MeSH-major] Biomarkers, Tumor / analysis. DNA-Binding Proteins / metabolism. Sarcoma, Alveolar Soft Part / pathology. Transcription Factors / metabolism. Uterine Neoplasms / pathology

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  • (PMID = 15782069.001).
  • [ISSN] 0277-1691
  • [Journal-full-title] International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists
  • [ISO-abbreviation] Int. J. Gynecol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / TFE3 protein, human; 0 / Transcription Factors
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80. Azizi AA, Haberler C, Czech T, Gupper A, Prayer D, Breitschopf H, Acker T, Slavc I: Vascular-endothelial-growth-factor (VEGF) expression and possible response to angiogenesis inhibitor bevacizumab in metastatic alveolar soft part sarcoma. Lancet Oncol; 2006 Jun;7(6):521-3
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  • [Title] Vascular-endothelial-growth-factor (VEGF) expression and possible response to angiogenesis inhibitor bevacizumab in metastatic alveolar soft part sarcoma.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Antibodies, Monoclonal / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / secondary. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Muscle Neoplasms / drug therapy. Psoas Muscles. Sarcoma, Alveolar Soft Part / drug therapy. Vascular Endothelial Growth Factor A / metabolism

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  • [ErratumIn] Lancet Oncol. 2006 Jul;7(7):533
  • (PMID = 16750504.001).
  • [ISSN] 1470-2045
  • [Journal-full-title] The Lancet. Oncology
  • [ISO-abbreviation] Lancet Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / RNA, Messenger; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; 2S9ZZM9Q9V / Bevacizumab
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81. Tasaki I, Kuratomi H, Sakai T: Alveolar soft part sarcoma in the region of the axilla: a case report. J Plast Reconstr Aesthet Surg; 2009 Oct;62(10):e409-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft part sarcoma in the region of the axilla: a case report.
  • [MeSH-major] Muscle Neoplasms / diagnosis. Sarcoma, Alveolar Soft Part / diagnosis

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  • (PMID = 19195950.001).
  • [ISSN] 1878-0539
  • [Journal-full-title] Journal of plastic, reconstructive & aesthetic surgery : JPRAS
  • [ISO-abbreviation] J Plast Reconstr Aesthet Surg
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Netherlands
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82. Stockwin LH, Vistica DT, Kenney S, Schrump DS, Butcher DO, Raffeld M, Shoemaker RH: Gene expression profiling of alveolar soft-part sarcoma (ASPS). BMC Cancer; 2009 Jan 15;9:22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gene expression profiling of alveolar soft-part sarcoma (ASPS).
  • BACKGROUND: Alveolar soft-part sarcoma (ASPS) is an extremely rare, highly vascular soft tissue sarcoma affecting predominantly adolescents and young adults.
  • METHODS: For seven patients with confirmed primary or metastatic ASPS, RNA samples were isolated immediately following surgery, reverse transcribed to cDNA and each sample hybridized to duplicate high-density human U133 plus 2.0 microarrays.
  • A subset of the most interesting genes was then validated using quantitative RT-PCR and immunohistochemistry.
  • A number of muscle-restricted transcripts (ITGB1BP3/MIBP, MYF5, MYF6 and TRIM63) were also identified, strengthening the case for a muscle cell progenitor as the origin of disease.
  • CONCLUSION: Results from this first comprehensive study of ASPS gene expression identifies several targets involved in angiogenesis, metastasis and myogenic differentiation.

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  • (PMID = 19146682.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CO / N01-CO-12400
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Validation Studies
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2635365
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83. Lazar AJ, Lahat G, Myers SE, Smith KD, Zou C, Wang WL, Lopez-Terrada D, Lev D: Validation of potential therapeutic targets in alveolar soft part sarcoma: an immunohistochemical study utilizing tissue microarray. Histopathology; 2009 Dec;55(6):750-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Validation of potential therapeutic targets in alveolar soft part sarcoma: an immunohistochemical study utilizing tissue microarray.
  • AIMS: The molecular signature of alveolar soft part sarcoma (ASPS) is a specific der(17)t(X;17)(p11.2;q25) translocation, resulting in a chimeric transcription factor (ASPSCR1-TFE3).
  • When this disease is no longer amenable to surgical curative intervention, uniformly efficacious therapies are lacking.
  • The aim of this study was to evaluate the expression of potential molecular therapeutic targets in a cohort of ASPS tumour samples.
  • METHODS AND RESULTS: Immunohistochemical analysis for hepatocyte growth factor, c-Met, phosphorylated c-Met, phosphorylated AKT, phosphorylated MEK, epidermal growth factor receptor (EGFR), vascular endothelial growth factor (VEGF), p53 and vimentin was performed on an ASPS tissue microarray, yielding complete data from 26 tumours.
  • CONCLUSIONS: There is a crucial need for better anti-ASPS therapies.
  • [MeSH-major] Neoplasm Proteins / metabolism. Sarcoma, Alveolar Soft Part / metabolism. Soft Tissue Neoplasms / metabolism
  • [MeSH-minor] Humans. Immunohistochemistry. Tissue Array Analysis


84. Rakheja D, Kapur P, Tomlinson GE, Margraf LR: Pediatric renal cell carcinomas with Xp11.2 rearrangements are immunoreactive for hMLH1 and hMSH2 proteins. Pediatr Dev Pathol; 2005 Nov-Dec;8(6):615-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Alveolar soft part sarcoma and pediatric renal cell carcinoma share a similar chromosomal abnormality, t(X;17)(p11.2;q25).
  • Recently, it has been suggested that the inactivation of DNA mismatch repair genes hMLH1 and hMSH2 may play an additional role in the pathogenesis of alveolar soft part sarcoma.

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  • (PMID = 16328670.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / Biomarkers, Tumor; 0 / Carrier Proteins; 0 / MLH1 protein, human; 0 / MLH2 protein, human; 0 / Neoplasm Proteins; 0 / Nuclear Proteins
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85. Stacy GS, Nair L: Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation. Clin Radiol; 2007 Oct;62(10):950-8
MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation.
  • The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma.
  • These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult.
  • [MeSH-major] Forearm. Leg. Magnetic Resonance Imaging. Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Diagnosis, Differential. Female. Humans. Male

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  • [CommentIn] Clin Radiol. 2007 Oct;62(10):959-60 [17765460.001]
  • (PMID = 17765459.001).
  • [ISSN] 0009-9260
  • [Journal-full-title] Clinical radiology
  • [ISO-abbreviation] Clin Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 20
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86. Fisher C: Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations. Virchows Arch; 2010 Feb;456(2):153-66
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations.
  • Many soft tissue sarcoma subtypes have consistent chromosomal translocations with novel fusion genes, which result in disordered cellular function.
  • This paper reviews the molecular pathology and related morphological and clinical features of sarcomas with non-EWS translocations.
  • These include synovial sarcoma, alveolar rhabdomyosarcoma, alveolar soft part sarcoma, dermatofibrosarcoma protuberans, low-grade fibromyxoid sarcoma, infantile fibrosarcoma and inflammatory myofibroblastic tumour.
  • [MeSH-major] Sarcoma / genetics. Translocation, Genetic / genetics

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  • (PMID = 19396640.001).
  • [ISSN] 1432-2307
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 196
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87. Park JH, Kang CH, Kim CH, Chae IJ, Park JH: Highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis. World J Surg Oncol; 2010;8:84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis.
  • PURPOSE: To evaluate the characteristics of highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis.
  • MATERIALS AND METHODS: The clinical and radiological characteristics of 18 cases of highly malignant soft tissue sarcomas of the extremity with a delayed diagnosis were determined.
  • Seven cases of synovial sarcoma, three cases each of alveolar soft part sarcoma and malignant fibrous histiocytoma, two cases each of highly malignant leiomyosarcoma and myxofibrosarcoma, and one case of clear cell sarcoma were enrolled.
  • Times from tumor detection to diagnosis ranged from 1 to 3 years in most cases; three of the seven synovial sarcoma cases took more than 10 years to diagnose.
  • Of the seven cases of synovial sarcoma, five cases of small, superficial located masses were simply excised without a pre-surgical biopsy.
  • Three cases of alveolar soft part sarcoma showed characteristic T1- and T2-weighted high signal intensities with signal voids in MR images.
  • In addition, one synovial sarcoma patient and one alveolar soft part sarcoma patient showed evidence of calcification on plain radiographs.
  • CONCLUSIONS: Contrary to general expectations, some soft tissue tumors that grow slowly are painless, and those that occur in superficial limbs may be highly malignant.
  • Thus, even when a slow growing, painless superficial mass is encountered in a limb, physicians should keep the possibility of highly malignant soft tissue sarcoma in mind.
  • [MeSH-major] Delayed Diagnosis. Forearm. Leg. Magnetic Resonance Imaging / methods. Neoplasm Staging / methods. Sarcoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Time Factors. Young Adult

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  • (PMID = 20863406.001).
  • [ISSN] 1477-7819
  • [Journal-full-title] World journal of surgical oncology
  • [ISO-abbreviation] World J Surg Oncol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
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88. Fadare O: Uncommon sarcomas of the uterine cervix: a review of selected entities. Diagn Pathol; 2006;1:30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Uncommon sarcomas of the uterine cervix: a review of selected entities.
  • Sarcomas constitute less than 1% of all cervical malignancies.
  • With over 150 reported cases, rhabdomyosarcomas represent the most commonly reported sarcoma at this location.
  • In this report, a select group of the more uncommon sarcomas of the uterine cervix are reviewed, including all previously reported examples of leiomyosarcoma, liposarcoma, alveolar soft part sarcoma, Ewing sarcoma/primitive neuroectodermal tumor, undifferentiated endocervical sarcoma, and malignant peripheral nerve sheath tumor (MPNST).

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  • (PMID = 16981999.001).
  • [ISSN] 1746-1596
  • [Journal-full-title] Diagnostic pathology
  • [ISO-abbreviation] Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1584249
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89. Dezanzo P, Lifschitz-Mercer B, Czernobilsky B, Rosai J: Alveolar soft-part sarcoma of paranasal sinuses. Int J Surg Pathol; 2010 Feb;18(1):66-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar soft-part sarcoma of paranasal sinuses.
  • [MeSH-major] Paranasal Sinus Neoplasms / pathology. Sarcoma, Alveolar Soft Part / pathology
  • [MeSH-minor] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / metabolism. Biomarkers, Tumor / metabolism. Diagnosis, Differential. Female. Humans. Paraganglioma / diagnosis. Periodic Acid-Schiff Reaction. Perivascular Epithelioid Cell Neoplasms / diagnosis. Young Adult

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  • (PMID = 19926641.001).
  • [ISSN] 1940-2465
  • [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 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Biomarkers, Tumor; 0 / TFE3 protein, human
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90. Huang HJ, Jiang CH, Chen SJ: [Alveolar soft part sarcoma of the tongue root: a case report]. Hua Xi Kou Qiang Yi Xue Za Zhi; 2008 Aug;26(4):454-6
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  • [Title] [Alveolar soft part sarcoma of the tongue root: a case report].
  • Alveolar soft part sarcoma is a rare, aggressive malignancy of uncertain histological origin with a propensity for vascular invasion and distant metastasis.
  • The case presented involves a 31-year-old woman with alveolar soft part sarcoma in the tongue root.
  • The clinical features, pathogenesis, diagnosis and treatment were discussed.
  • [MeSH-major] Sarcoma, Alveolar Soft Part. Tongue Neoplasms

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  • (PMID = 18780514.001).
  • [ISSN] 1000-1182
  • [Journal-full-title] Hua xi kou qiang yi xue za zhi = Huaxi kouqiang yixue zazhi = West China journal of stomatology
  • [ISO-abbreviation] Hua Xi Kou Qiang Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
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91. Strunk T, Bastian PJ, Ellinger J, Biermann K, Müller SC: [Aggressive course of a malignant alveolar soft tissue sarcoma]. Urologe A; 2007 Oct;46(10):1422-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Aggressive course of a malignant alveolar soft tissue sarcoma].
  • Alveolar soft part sarcoma is a rare soft tissue tumor.
  • Most patients who are affected by this sarcoma are between 15 and 35 years old.
  • Publications concerning this sarcoma subform are rare and the best therapeutic procedure is not yet clear.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Neoplasms, Second Primary / diagnosis. Sarcoma, Alveolar Soft Part / diagnosis
  • [MeSH-minor] Aged. Capillaries / pathology. Diagnosis, Differential. Disease Progression. Fatal Outcome. Humans. Lymph Node Excision. Lymph Nodes / pathology. Lymphatic Metastasis / pathology. Lymphoma, Non-Hodgkin / pathology. Male. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / radiotherapy. Neoplasm Recurrence, Local / surgery. Radiotherapy, Adjuvant. Retreatment. Testicular Neoplasms / pathology. Tomography, X-Ray Computed

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  • (PMID = 17641862.001).
  • [ISSN] 0340-2592
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
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92. Kebudi R, Ayan I, Görgün O, Ağaoğlu FY, Vural S, Darendeliler E: Brain metastasis in pediatric extracranial solid tumors: survey and literature review. J Neurooncol; 2005 Jan;71(1):43-8
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

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  • The diagnosis was sarcomas in 12 patients: 5 osteosarcomas, 4 Ewing's sarcoma family tumors, 1 rhabdomyosarcoma, 1 clear cell sarcoma of the soft tissue, 1 alveolar soft part sarcoma.
  • Four patients (25%) had brain metastasis at diagnosis.
  • Twelve (75%) developed brain metastasis during therapy or relapse at a median duration of 16 (1-70) months from initial diagnosis.
  • Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5.
  • Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis.
  • Although, the outcome for these patients is dismal in this series and in the literature; reports of long term survival in a few cases with Wilms' tumor, osteosarcoma and alveolar soft part sarcoma who had isolated brain metastasis, suggest that a subset of patients may benefit from therapy.
  • [MeSH-major] Brain Neoplasms / epidemiology. Brain Neoplasms / secondary. Sarcoma / epidemiology. Sarcoma / secondary

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  • (PMID = 15719274.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 43
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93. Fox BD, Patel A, Suki D, Rao G: Surgical management of metastatic sarcoma to the brain. J Neurosurg; 2009 Jan;110(1):181-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Surgical management of metastatic sarcoma to the brain.
  • OBJECT: Metastatic sarcoma to the brain is rare and represents a therapeutic challenge due to its relative resistance to radio- and chemotherapy.
  • The authors reviewed a series of patients with metastatic sarcoma to the brain treated surgically to determine outcomes and identify predictors of survival in these patients.
  • METHODS: A retrospective review of prospectively collected data was undertaken on patients undergoing surgery between 1993 and 2005 for metastatic sarcoma to the brain at The University of Texas, M.D.
  • RESULTS: During the study period, 62 patients underwent 84 operations for metastatic sarcoma to the brain.
  • In multivariate and univariate analysis, control of systemic disease, and sarcomas originating from bone, cartilage, or soft tissue were predictors of survival.
  • Patients with control of systemic disease had survival advantage when compared with those who did not.
  • In patients with alveolar soft-part sarcoma, there was a significantly increased survival advantage compared with all other histological subgroups.
  • CONCLUSIONS: The authors' results suggest that in selected patients, resection of metastatic sarcoma to the brain is associated with a relatively low risk of operative death and results in improvement in neurological function.
  • Patients with systemic control of their primary disease and certain histological subtypes (specifically alveolar soft-part sarcoma) have improved overall and progression-free survival.
  • [MeSH-major] Brain Neoplasms / secondary. Brain Neoplasms / surgery. Neurosurgical Procedures. Sarcoma / secondary. Sarcoma / surgery


94. Shaddix KK, Fakhre GP, Nields WW, Steers JL, Hewitt WR, Menke DM: Primary alveolar soft-part sarcoma of the liver: anomalous presentation of a rare disease. Am Surg; 2008 Jan;74(1):43-6
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  • [Title] Primary alveolar soft-part sarcoma of the liver: anomalous presentation of a rare disease.
  • Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant.
  • It comprises less than 1 per cent of all soft-tissue sarcomas.
  • Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are affected.
  • Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx.
  • Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver.
  • Despite surgical resection and numerous chemotherapeutic regimens, this patient had widespread metastasis and died approximately 2 years after the diagnosis was established.
  • [MeSH-major] Liver Neoplasms / diagnosis. Sarcoma, Alveolar Soft Part / diagnosis

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  • (PMID = 18274427.001).
  • [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
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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95. Armah HB, Parwani AV: Xp11.2 translocation renal cell carcinoma. Arch Pathol Lab Med; 2010 Jan;134(1):124-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • These include a distinctive RCC that bears a translocation with the identical chromosomal breakpoints (Xp11.2, 17q25) and identical resulting ASPL-TFE3 gene fusion as alveolar soft part sarcoma.
  • [MeSH-minor] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics. Diagnosis, Differential. Humans. Oncogene Proteins, Fusion / genetics. Prognosis

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  • (PMID = 20073616.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / TFE3 protein, human
  • [Number-of-references] 30
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96. Aulmann S, Longerich T, Schirmacher P, Mechtersheimer G, Penzel R: Detection of the ASPSCR1-TFE3 gene fusion in paraffin-embedded alveolar soft part sarcomas. Histopathology; 2007 Jun;50(7):881-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Detection of the ASPSCR1-TFE3 gene fusion in paraffin-embedded alveolar soft part sarcomas.
  • AIMS: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumour with unique morphology and a recurrent, non-reciprocal translocation der(17)t(X;17)(p11.2;q25) leading to the fusion of ASPSCR1 (also known as ASPL) to the transcription factor TFE3.
  • Although diagnosis is straightforward in classical cases, tumours with atypical morphological features may be difficult to classify solely on the basis of conventional histopathology.
  • The aim of this study was to analyse the chromosomal breakpoints in paraffin-embedded tissue.
  • METHODS AND RESULTS: Three male and two female ASPS patients including one case with uncommon histology were investigated by fluorescence in situ hybridization with split- and fusion-probes.
  • Hybridization results showed a t(X;17)(p11.2;q25) in all tumours with a duplication of the telomeric part of chromosome Xp.
  • CONCLUSIONS: Molecular confirmation of ASPSCR1-TFE3 gene fusion is applicable to routinely processed archival and diagnostic tumour samples and aids in the differential diagnosis of ASPS.
  • [MeSH-major] Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics. Neoplasm Proteins / genetics. Oncogene Fusion. Oncogene Proteins, Fusion / genetics. Sarcoma, Alveolar Soft Part / genetics. Soft Tissue Neoplasms / genetics

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  • (PMID = 17543078.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / ASPSCR1 protein, human; 0 / Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0 / DNA, Neoplasm; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion; 0 / TFE3 protein, human
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97. Schittenhelm J, Ebner FH, Harter P, Bornemann A: Symptomatic intraspinal oncocytic adrenocortical adenoma. Endocr Pathol; 2009;20(1):73-7
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  • We here present the case of a 44-year-old woman with a lesion in the cauda equina that fulfilled the radiologic criteria of schwannoma and caused clinical symptoms for 3 years.
  • The diagnosis of an oncocytic adrenal cortical adenoma was made.
  • These extraadrenal tumors are thought to arise from heterotopic adrenocortical tissue in the spinal cavity.
  • Immunohistochemistry excluded oncocytic paraganglioma, oncocytic meningioma, renal cell carcinoma, alveolar soft part sarcoma, and granular cell tumor.

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  • (PMID = 19039533.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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98. Skubitz KM, D'Adamo DR: Sarcoma. Mayo Clin Proc; 2007 Nov;82(11):1409-32
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  • [Title] Sarcoma.
  • Sarcomas comprise a heterogeneous group of mesenchymal neoplasms.
  • They can be grouped into 2 general categories, soft tissue sarcoma and primary bone sarcoma, which have different staging and treatment approaches.
  • This review includes a discussion of both soft tissue sarcomas (malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, aggressive fibromatosis or desmoid tumor, rhabdomyosarcoma, and primary alveolar soft-part sarcoma) and primary bone sarcomas (osteosarcoma, Ewing sarcoma, giant cell tumor, and chondrosarcoma).
  • The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery.
  • Subsequent treatment depends on the specific type of sarcoma.
  • Because sarcomas are relatively uncommon yet comprise a wide variety of different entities, evaluation by oncology teams who have expertise in the field is recommended.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma / therapy. Soft Tissue Neoplasms / therapy

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  • MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.
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  • (PMID = 17976362.001).
  • [ISSN] 0025-6196
  • [Journal-full-title] Mayo Clinic proceedings
  • [ISO-abbreviation] Mayo Clin. Proc.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 391
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99. Kayton ML: Pulmonary metastasectomy in pediatric patients. Thorac Surg Clin; 2006 May;16(2):167-83, vi
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  • Because many pediatric metastatic tumors are rare, surgeons have grouped together patients of different histologies for the generation and analysis of case series.
  • By examining tumor types individually, however, it is seen that certain histologies (adrenocortical carcinoma, alveolar soft part sarcoma, osteosarcoma) mandate surgical metastasectomy for patient survival.
  • Other pediatric tumors (Wilms tumor, Ewing's sarcoma) are radiation sensitive, and the application of metastasectomy is controversial.
  • [MeSH-minor] Biopsy. Child. Humans. Infant. Minimally Invasive Surgical Procedures. Neoplasms, Complex and Mixed / diagnosis. Neoplasms, Complex and Mixed / secondary. Neoplasms, Complex and Mixed / surgery. Neoplasms, Glandular and Epithelial / diagnosis. Neoplasms, Glandular and Epithelial / secondary. Neoplasms, Glandular and Epithelial / surgery. Pulmonary Surgical Procedures. Sarcoma / diagnosis. Sarcoma / secondary. Sarcoma / surgery

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  • (PMID = 16805206.001).
  • [ISSN] 1547-4127
  • [Journal-full-title] Thoracic surgery clinics
  • [ISO-abbreviation] Thorac Surg Clin
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 91
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100. Balgley BM, Guo T, Zhao K, Fang X, Tavassoli FA, Lee CS: Evaluation of archival time on shotgun proteomics of formalin-fixed and paraffin-embedded tissues. J Proteome Res; 2009 Feb;8(2):917-25
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • There is increasing acceptance of the critical importance of correlating the morphologic features of tissue with the data obtained from various molecular analytic techniques.
  • Access to archived formalin-fixed and paraffin-embedded (FFPE) tissue specimens via shotgun-based proteomic analyses may, therefore, open new avenues for both prospective and retrospective translational research.
  • Optimized protein extraction and digestion procedures for handling FFPE tissues are coupled with the capillary isotachophoresis-based proteome technology to evaluate the effects of length of storage period on archival tissue proteome analysis across 10 archived uterine mesenchymal tumor tissue blocks, including 9 uterine leiomyomas dating from 1990 to 2002 and a single case of alveolar soft part sarcoma (ASPS) from 1980.
  • Low abundance proteins may be more susceptible to the long-term storage as these proteins are more difficult to be retrieved and extracted as the tissue block ages in paraffin.
  • Despite using tissue blocks stored for as many as 28 years, high confidence and comparative proteome analysis between the leiomyomas and the sarcoma is achieved.
  • Though sharing over 1800 common proteins in a core set, a total of 80 proteins unique to the sarcoma are identified distinguishing the ASPS from the leiomyomas.
  • Vacuolar proton translocating ATPase 116 kDa subunit isoform a3, one of the unique proteins expressed in the ASPS, is further validated by immunohistochemistry (IHC).

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  • (PMID = 19128014.001).
  • [ISSN] 1535-3893
  • [Journal-full-title] Journal of proteome research
  • [ISO-abbreviation] J. Proteome Res.
  • [Language] ENG
  • [Grant] United States / NIGMS NIH HHS / GM / R01 GM073723; United States / NCI NIH HHS / CA / R42 CA122715; United States / NIGMS NIH HHS / GM / R01 GM073723-04; United States / NCI NIH HHS / CA / R41 CA122715; United States / NCI NIH HHS / CA / CA122715; United States / NIGMS NIH HHS / GM / GM073723; United States / NIGMS NIH HHS / GM / GM073723-04
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Peptides; 1HG84L3525 / Formaldehyde
  • [Other-IDs] NLM/ NIHMS91394; NLM/ PMC2664629
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