Angiomatoid fibrous histiocytoma of the adrenal gland: A clinical and pathological observation of two cases

Background Angiomatoid fibrous histiocytoma (AFH) is a rare low-grade neoplasm, typically arising in pediatric/adolescent extremity subcutaneous tissues. Adrenal AFH is extremely uncommon, with limited reported cases. Elucidating clinicopathologic/molecular profiles of adrenal AFH is critical for accurate diagnosis and management. Case presentation Two adrenal AFH cases (18-year-old male, 50-year-old female) from Guangxi Medical University Second Hospital were retrospectively analyzed, focusing on clinical features, histopathology, immunohistochemistry (IHC), fluorescence in situ hybridization (FISH), and follow-up. Case 1: Classic AFH morphology with unique features: diffuse intratumoral lymphoid tissue (with follicles) instead of capsular lymphoid cuffs, and lymphangioma-like cystic spaces (lacking angiomatoid hemorrhage).Case 2: Cystic tumor misdiagnosed as "adrenal pseudocyst"; true parenchyma resided in the cyst wall, with nodular lymphoid aggregates (no lymphoid cuff) and erythrocyte/hemosiderin-filled vascular clefts. Marked hyaline fibrosis was noted.Molecular: Both cases showed EWSR1 break-apart (FISH+), negative for EWSR1::CREB1/ATF1 fusions. IHC revealed desmin positivity (both), ALK-D5F3 expression (Case 2). Conclusions Atypical-location AFH (e.g., adrenal) exhibits divergent pathologic phenotypes. Diagnosis requires integrated IHC/molecular analysis (e.g., EWSR1 status). Prognostic implications of genetic alterations (e.g., ALK expression) highlight the need for molecular profiling to guide therapy and prognosis.