Alveolar Soft Part Sarcoma of Uterine Corpus in a Young Female: A Case Report With Review of Literature

Abstract

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma, often occurs in adolescents and young adults with a particular predilection for the deep soft tissue of extremities. Occurrence of ASPS in the female genital tract is very uncommon and poses a significant diagnostic challenge. A case of ASPS of the uterine corpus is described in a young unmarried female, who presented to the out-patient clinic of our Institute with complaints of abnormal uterine bleeding for the past 9 mo. She was being treated with oral contraceptive pills, progesterone and tranexamic acid. Following radiological imaging and hysteroscopy, a therapeutic curettage of the lesion was done. The histopathologic features raised a differential diagnosis of a myriad of morphologic mimickers. The diagnosis was clinched by exclusion of mimickers by relevant immunohistochemical markers and strong nuclear expression of TFE3 on immunohistochemistry. The patient is on regular follow-up with oral contraceptives and antifibrinolytic medication. Despite being infrequent at this location, ASPS should be kept in differential diagnosis in young females presenting with abnormal uterine bleeding.

Advances in treatment of alveolar soft part sarcoma: an updated review

“…ASPS presents asymptomatically as a slow-growing tumour ( 4 , 11 ) and commonly arises in deep soft tissues of the extremities (61%), trunk (20%), head and neck (9%), and internal organs (8%) ( 2 , 10 ). Uncommon sites include bone ( 12 , 13 ), brain ( 14 , 15 ), orbit ( 16 ), tongue ( 17 ), lung ( 18 ), mediastinum ( 19 , 20 ), bladder ( 21 ), prostate ( 22 ), uterus ( 23 ) and vagina ( 24 ). Because of the lack of associated symptoms or functional impairment, patients with ASPS often present with metastatic disease ( 4 ).…”