Primary Alveolar Soft Part Sarcoma of Chest Wall: A Case Report and Review of the Literature

Farhad Zamani, MD; Mosadegh Jabbari, MD; Seyed Maysam Alimohamadi, MD; Ramin Shakeri, MD; Zohreh Rostami, MD; Behnoush Abedi, MD; Mahshid Hormazdi, MD; Reza Malekzadeh, MD

Disclosures

July 05, 2006

Introduction

Alveolar soft part sarcoma (ASPS), also called alveolar soft-tissue sarcoma (ASTS), is a rare malignant soft-tissue neoplasm, which was first described by Christopherson and colleagues^[1] in 1952. Its prevalence is less than 1% of all primary soft-tissue sarcomas.^[2] The histogenesis of ASPS is not clear; however, a myogenic phenotype and skeletal muscle differentiation are favored.^[3,4] It is more common in adolescents and young adults, especially in those who are 15-35 years old; occurs more commonly in women; and is rarely seen in children.^[5]

ASPS most commonly originates from the deep tissues of the proximal parts of the extremities, especially the lower limbs,^[5] and shows right-sided laterality, as described by Fassbender.^[6] In children the primary site is in the head and neck. It usually appears as a painless mass in the thigh or leg, but it may initially appear from other sites of the body. Regardless of its indolent growth pattern, the ultimate prognosis remains poor because of early metastatic spread to the lungs, bone, brain, and lymph nodes in order of frequency.^[7]

In many cases, the first manifestations are due to distant metastases. Surgery remains the mainstay of therapy.^[8] Little is known about other therapies, ie, chemotherapy, radiation therapy, and biologic therapy, and the data are still inconclusive.

Readers are encouraged to respond to George Lundberg, MD, Editor of MedGenMed, for the editor's eyes only or for possible publication via email: glundberg@medscape.net

References

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