Alveolar Soft Part Sarcoma in Japan: Multi-Institutional Study of 57 Patients from the Japanese Musculoskeletal Oncology Group

Abstract

Objective: The clinical features and the management of alveolar soft part sarcoma (ASPS) are not well known. The efficacy of chemotherapy for soft tissue sarcoma, including high-dose ifosfamide and cisplatin, has not been established yet. Some reports suggest ASPS may occur primarily in bone. Methods: We report on a series of 57 patients with ASPS over 20 years. Their ages ranged from 7 to 75 years (mean 25). Results: There were 37 females and 20 males. Thirteen lesions (23%) showed bone involvement at the primary site, and 6 of them were diagnosed as bone tumors at presentation. Thirty-seven patients had distant metastases at presentation. Tumor size, bone involvement at the primary site and the presence of metastases at presentation were prognostic indicators (p < 0.05). Marginal excision with radiotherapy or wide excision without radiotherapy achieved good local control. Chemotherapy was performed in 47 patients with different regimens. Two patients treated with intra-arterial chemotherapy regimens responded partially, but intravenous chemotherapy with high-dose ifosfamide or cisplatin failed. Conclusions: ASPS can present primarily as a bone tumor. No advantage of chemotherapy with high-dose ifosfamide or cisplatin could be demonstrated.

Alveolar Soft Part Sarcoma

“…16 Prognosis is largely dependent on the initial presentation (localized versus metastatic disease), tumor size, and age. 4,11,15,27 Patients with localized disease at presentation have a 71% 5-year survival rate, compared with 20% for patients with metastatic disease at time of diagnosis. 4 Patients who present with large tumors are most likely to have metastasis at the time of diagnosis.…”

Alveolar Soft Part Sarcoma located at the upper extremity: a case report and reviev of the literature

“…ASPS'da santral kohezyondan yoksun, poligonal yapıdaki tümöral hücre toplulukları psödoalveolar yapılar oluştururlar ve tümöral hücrelerde PAS pozitif diastaz rezistan intrasitoplazmik granüller/çubuk benzeri kristallerin görülmesi karakteristiktir (2,3) . Tüm yumuşak doku sarkomlarının %0,5-1'ini (4,5) , pediatrik olgularda yumuşak doku sarkomlarının %0, 8'ini oluştururlar (6,7) . Sıklıkla alt ekstremitelerde, baş-boyun bölgesinde yerleşimli olmakla birlikte; uterus, vajina, retroperiton, gastrointestinal sistem, mesane, meme, mide ve kemik gibi vücudun herhangi bir lokalizasyonunda gelişim gösterebilirler.…”

“…Sıklıkla alt ekstremitelerde, baş-boyun bölgesinde yerleşimli olmakla birlikte; uterus, vajina, retroperiton, gastrointestinal sistem, mesane, meme, mide ve kemik gibi vücudun herhangi bir lokalizasyonunda gelişim gösterebilirler. Genelde yumuşak kıvamlı, ağrısız, yavaş büyüme gösteren, yuvarlak-lobüle kitlesel lezyonlardır (2)(3)(4)(5)(6)(7)(8)(9) . Ultrastrüktürel olarak monocarboxylate transporter 1 ve CD147 içeren, intrasitoplazmik rhamboid çubuk şeklinde kristaller izlenir.…”

“…Ultrastrüktürel olarak monocarboxylate transporter 1 ve CD147 içeren, intrasitoplazmik rhamboid çubuk şeklinde kristaller izlenir. Moleküler sitogenetik çalışmalar ASPS'larda, X kromozomu ve 17 no.lu kromozom arasında dengesiz bir translokasyon t(X;17) (p11;25) ile karakterize edilir (8,9) . ASPS'nin tedavisinde lezyonun tam olarak cerrahi rezeksiyonu esastır.…”

“…ASPS'nin tedavisinde lezyonun tam olarak cerrahi rezeksiyonu esastır. Kemoterapi ve radyoterapi kombine kullanıl-makla birlikte etkinliği tartışmalıdır (4,8) .…”