Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment, and Outcome in a Series of 33 Patients at a Single Institution
Purchase on Springer.com
$39.95 / €34.95 / £29.95*
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.
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.
Demographics, tumor sizes, sites and extent of disease, treatments provided, progression-free survival, and overall survival were evaluated.
A total of 33 patients were identified. Preoperative imaging misdiagnosed a vascular malformation in three cases (9%), delaying treatment. The most common location of primary tumor was the thigh. The median diameter of the mass was 9 cm (range, 2–15 cm). The tumor was deeply located in most cases (78.7%). A R0 resection was obtained in 27 cases. Adjuvant radiotherapy was delivered in 12 cases, in 6 cases in association with chemotherapy; preoperative systemic chemotherapy was delivered in 4 cases, in the adjuvant setting in another 3 cases. Twenty-one (63.6%) of 33 patients exhibited metastases either at presentation (10 patients, 30.3%) or later. Metastatic sites included lymph nodes, lung, bone, and liver. Median overall follow-up was 72 months. Overall survival was 68.7% at 5 years and 53.4% at 10 years. Metastectomies were performed in 33% of metastatic cases (7 of 21 patients).
Prognosis of ASPS is basically related to the characteristics of the disease and the quality of surgery. Overall, the occurrence of distant metastases is quite common, with a typical indolent course. New agents are eagerly needed to complement surgery to eradicate this disease.
- Christopherson WM, Foote FWJ, Steward FW. Alveolar soft-part sarcoma: structurally characteristic tumors of uncertainly histogenesis. Cancer. 1952;5:100. CrossRef
- Ekfors TO, Kalimo H, Rantakokko V, Latvala M, Parvinen M. Alveolar soft part sarcoma: a report of two cases with some histochemical and ultrastructural observations. Cancer. 1979;43:1672. CrossRef
- Lieberman PH, Brennan MF, Kimmel M, et al. Alveolar soft-part sarcoma. A clinico-pathologic study of a half a century. Cancer. 1989;63:1. CrossRef
- Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW, Childers EL. Lingual alveolar soft part sarcoma. 14 cases: novel clinical and morphological observations. Histopathology. 2004;45:526. CrossRef
- Kim HS, Lee HK, Weon YC, Kim HJ. Alveolar soft-part sarcoma of the head and neck: clinical and imaging features in five cases. Am J Neuroradiol. 2005;26:1331.
- Suh JS, Cho J, Lee SH, et all. Alveolar soft part sarcoma: MR and angiographic findings. Skeletal Radiol. 2000;29:680–9. CrossRef
- Evans HL. Alveolar soft part sarcoma. A study of 13 typical examples and one with a histologically atypical component. Cancer. 1985;55:912–7. CrossRef
- Temple HT, Scully SP, O’Keefe RJ, Rosenthal DI, Maukin HJ. Clinical presentation of alveolar soft-part sarcoma. Clin Orthop Relat Res. 1994;(300):213–8.
- Ordonez NG. Alveolar soft part sarcoma: a review and update. Adv Anat Pathol. 1999;6:125–39. CrossRef
- Pappo AS, Parham DM, Cain A, et al. Alveolar soft part sarcoma in children and adolescents: clinical features and outcome of 11 patients. Med Pediatr Oncol. 1996;26:81–4. CrossRef
- Portera CA, Ho V, Patel SR, et al. Alveolar soft part sarcoma. Clinical course and patterns of metastases in 70 pts treated at a single institution. Cancer. 2001;91:585–91. CrossRef
- Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol. 1996;14:1679–89.
- Coindre JM, Terrier P, Bui NB, et al. Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study on 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol. 1996;14:869–77.
- Stojadinovic A, Leung DH, Hoos A, et al. Analysis of the prognostic significance of microscopic margins in 2,084 localized primary adult soft tissue sarcomas. Ann Surg. 2002;235:424–34. CrossRef
- Gronchi A, Casali PG, Mariani L, et al. Status of surgical margins and prognosis in adult soft tissue sarcomas of the extremities: a series of patients treated at a single institution J Clin Oncol. 2005;23:96–104. CrossRef
- Gronchi A, Lo Vullo S, Colombo C, et al. Extremity soft tissue sarcoma in a series of patients treated at a single institution: local control directly impacts survival. Ann Surg. 2010;251:512–7. CrossRef
- Kuriyama K, Todo S, Hibi S, Morimoto A, Imashuku S. Alveolar soft part sarcoma with lung metastases: response to interferon alpha-2a? Med Pediatr Oncol. 2001;37:482–3. CrossRef
- Roozendaal KJ, de Valk B, ten Velden JJ, van der Woude HJ, Kroon BB. Alveolar soft-part sarcoma responding ti interferon alpha-2b. Br J Cancer. 2003;89:243–5. CrossRef
- Stacchiotti S, Tamborini E, Marrari A, et al. Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res. 2009;15:1096–104. CrossRef
- Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment, and Outcome in a Series of 33 Patients at a Single Institution
Annals of Surgical Oncology
Volume 17, Issue 12 , pp 3229-3233
- Cover Date
- Print ISSN
- Online ISSN
- Additional Links
- Industry Sectors
- Author Affiliations
- 1. Department of Surgery, Istituto Nazionale Tumori, Milan, Italy
- 2. Department of Pathology, Istituto Nazionale Tumori, Milan, Italy
- 3. Department of Cancer Medicine, Istituto Nazionale Tumori, Milan, Italy