Angiomatoid Fibrous Histiocytoma in a Child with Hereditary Spherocytosis: A Rare Co-occurrence.

INTRODUCTION: Angiomatoid fibrous histiocytoma (AFH) is a rare soft-tissue tumor of low malignant potential, and its radiological appearance is often non-specific, mimicking benign cysts, hematomas, or infections, necessitating histopathological and immunohistochemical confirmation. While hematological malignancies have been reported in Hereditary Spherocytosis (HS), no association with AFH has been documented. We present the first case of AFH involving the shoulder soft tissue in a post-splenectomy adolescent with HS, highlighting diagnostic challenges and a possible link between chronic hematologic stress and mesenchymal tumor development.

CASE REPORT: A child in his early adolescence presented with a 2-month history of right shoulder pain and swelling, which was progressively increasing in size, firm, painful, and associated with fever. His total leukocyte count was 15,500/mm3, and C-reactive protein was 152 mg/L. He had a history of multiple blood transfusions since the age of four. Eight months prior, he was diagnosed with HS and underwent total splenectomy for massive splenomegaly. Magnetic resonance imaging revealed a soft-tissue lesion posterior to the glenoid, abutting the spine of the scapula. A bone scan ruled out an infectious etiology. Ultrasound-guided fine needle aspiration cytology showed no organisms and was negative for malignant cells. Surgical excision of the mass was performed, and histopathology confirmed the diagnosis of AFH.

CONCLUSION: This case highlights the importance of considering AFH in the differential diagnosis of soft-tissue tumors in children with hematologic disorders.