Open Access
Alveolar soft part sarcoma of the right calf
Author(s) -
Bin Wang,
Huanhuan Wang,
Jinlong Wei,
Limei Qu,
Lingbin Meng,
Ying Xin,
Xin Jiang
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000018952
Subject(s) - alveolar soft part sarcoma , medicine , soft tissue , histogenesis , immunohistochemistry , pathology , pathological , sarcoma , soft tissue sarcoma
Abstract Rationale: Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue neoplasm with controversial histogenesis. ASPS accounts for 0.5% to 1% of all soft tissue sarcomas. Because of its rarity, ASPS is easily misdiagnosed, increasing the risk of incorrect treatment. Patient concerns: A 6-year-old female patient presented with a history of a 2.0 × 2.5 × 3.0-cm mass in the deep soft tissues of her right lower extremity. Diagnoses: Histopathological features indicated the diagnosis of ASPS. Microscopically, a diffuse arrangement of tumor cells or pseudoalveolar architectures separated by thin and well-vascularized fibrous septa were observed. Immunohistochemical staining of the tumor cells indicated positivity for transcription factor E3, myogenic determination factor 1, and periodic acid–Schiff–diastase (PAS-D) and showed a Ki-67 proliferating index of approximately 20%. Interventions: The patient underwent enlarged resection of the tumor and was treated with radiotherapy. Outcomes: During the 3-year follow-up, the patient has remained in good condition, with no symptom recurrence, distant metastatic spread, or significant toxicity during or after treatment. The patient remains under regular surveillance. Lessons: Its low incidence, lack of characteristic clinical manifestations, and atypical location often lead to ASPS misdiagnosis and subsequent incorrect treatment. Nuclear expression of transcription factor E3 is of diagnostic value for ASPS. At present, there is no consensus on the treatment for ASPS. In-depth pathological analysis is needed to better understand the characteristics of this tumor.