Alveolar soft part sarcoma in children and adolescents: Clinical features and outcome of 11 patients

Pappo, Alberto S.; Parham, David M.; Cain, Alvida M.; Luo, Xiaolong; Bowman, Laura C.; Furman, Wayne L.; Rao, Bhaskar N.; Pratt, Charles B.

doi:10.1002/(sici)1096-911x(199602)26:2<81::aid-mpo2>3.0.co;2-q

Cited by 92 publications

(67 citation statements)

References 11 publications

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“…Data from the files of the Armed Forces Institute of Pathology (AFIP) between 1970 and 2003 showed that only 13 out of 266 cases of ASPS (approximately 5 %) occurred in the tongue [4,8]. However, in infants and children, 25 % of head and neck ASPS involve the tongue [14], this frequency increasing to 70 % when the orbits are excluded [15]. Table 1 summarizes all reported cases of intraoral ASPS in the pediatric population (0-18 years) [2,[4][5][6][7][8], totaling forty-one cases, including the present series.…”

Section: Casementioning

confidence: 99%

“…PAS positive diastase resistant granules may represent precursors to the rod shaped crystals. The cell surface and cytoplasm in the region of these crystals stains for MCT1 and CD147 [1,14,15]. MCT1, the monocarboxylate transporter 1, is found predominantly in cardiac and skeletal muscle, and catalyzes the rapid transport of monocarboxylates from the rough endoplasmic reticulum across plasma membranes.…”

Section: Casementioning

confidence: 99%

“…ASPS prognosis is based mainly on clinical features such as location of the primary lesion, size of the tumor, and age of patient [1,3,5,18]. ASPS can demonstrate an early metastasizing potential, sometimes prior to detection of the primary tumor, but can also metastasize decades after the initial diagnosis [1,[3][4][5][6][7][8][9][15][16][17][18][19][20]. Hence, complete local excision, adjuvant therapy if clinically indicated, and long-term patient follow-up are advised for all cases of ASPS.…”

Section: Casementioning

confidence: 99%

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Oral Alveolar Soft Part Sarcoma in Childhood and Adolescence: Report of Two Cases and Review of Literature

Argyris

Reed

López‐Terrada

et al. 2012

Head and Neck Pathol

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Alveolar soft part sarcoma (ASPS) constitutes a rare soft tissue malignant neoplasm comprising less than 1 % of all soft tissue sarcomas. ASPS demonstrates a strong predilection for adolescents and young adults, with a female predominance reported. The head and neck region is the most commonly affected region in pediatric patients with the tongue and orbit affected most commonly. Herein we present the clinical, radiographic, histopathologic, immunohistochemical and molecular features of two examples of ASPS affecting the oral cavity of 4 and 13 year-old boys, along with a focused review of the literature on intraoral ASPS in pediatric patients.

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Section: Casementioning

confidence: 99%

Section: Casementioning

confidence: 99%

Section: Casementioning

confidence: 99%

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Oral Alveolar Soft Part Sarcoma in Childhood and Adolescence: Report of Two Cases and Review of Literature

Argyris

Reed

López‐Terrada

et al. 2012

Head and Neck Pathol

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“…Alveolar soft part sarcoma is a rare slow-growing malignant tumor, accounting for 5% of pediatric nonrhabdomyosarcoma soft-tissue sarcomas (60). The exact histogenesis is still unknown, but the name is derived from the presence of nests of eosinophilic cells that contain abundant precrystalline cytoplasmic granules organized in a fashion resembling respiratory alveoli, separated by a rich vascular blood supply (61).…”

Section: Alveolar Soft Part Sarcomamentioning

confidence: 99%

“…Local recurrence and lymph node metastases are also seen (62). Treatment is surgical, with a better prognosis reported with smaller tumors, the presence of localized disease only, and a younger age at diagnosis (60,61).…”

Section: Amentioning

confidence: 99%

Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation

Laffan¹,

Ngan²,

Navarro³

2009

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Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: The St. Jude Children's Research Hospital experience

Pappo

Rao

Jenkins

et al. 1999

Med. Pediatr. Oncol.

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Background Because the natural history of pediatric patients with metastatic nonrhabdomyosarcomatous soft‐tissue sarcomas (NRSTS) had not been well described, we retrospectively reviewed our single‐institution experience with these tumors. Procedure We identified 26 patients with metastatic NRSTS who were treated at St. Jude Children's Research Hospital from December 1971 through July 1995. We evaluated the characteristics of each patient, including age, sex, primary site, stage, type of therapy received, and outcome. Sites of metastatic disease at diagnosis and relapse were noted. Results The median age of the 26 study patients at diagnosis was 14.8 years; 54% of patients were male and 69% were white. The most common histologies were synovial sarcoma, alveolar soft‐part sarcoma, and malignant fibrous histiocytoma. Most primary tumors (73% of cases) occurred in the trunk or extremities. Most patients presented with large (>5 cm), high‐grade lesions. All 26 patients received chemotherapy, and 8 responded to an ifosfamide‐ or cyclophosphamide‐doxorubicin–based regimen. Radiotherapy was administered to 15 patients, and 13 had a partial or complete resection of the primary tumor. Six patients underwent thoracotomy. The estimated 2‐year survival for the cohort was 34.6% ± 8.9%; the 2‐year progression‐free survival was 15.4% ± 6.3%. The lung was the most common site of failure. Conclusions Children with metastatic NRSTS have a poor outcome, which is similar to that in adults. More effective systemic chemotherapy is needed to facilitate complete surgical resection of primary and metastatic sites. Aggressive pulmonary metastatectomy can increase disease control. Med. Pediatr. Oncol. 33:76–82, 1999. © 1999 Wiley‐Liss, Inc.

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Alveolar soft part sarcoma in children and adolescents: Clinical features and outcome of 11 patients

Cited by 92 publications

References 11 publications

Oral Alveolar Soft Part Sarcoma in Childhood and Adolescence: Report of Two Cases and Review of Literature

Oral Alveolar Soft Part Sarcoma in Childhood and Adolescence: Report of Two Cases and Review of Literature

Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation

Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: The St. Jude Children's Research Hospital experience

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