Abstract

Angiomatoid fibrous histiocytoma (AFH) is a rare, slow-growing soft tissue tumor with an intermediate biologic potential and uncertain line of differentiation, and minimal metastatic potential. AFH may mimic both the clinical, histological, and radiological findings of several tumors; therefore, it is frequently misdiagnosed. Three cases of AFH were included in this study. A six-year-old male and two females with an age of 12 and 17 years are presented. The primary locations were in the right shoulder (case 1), left medial proximal thigh (case 2), and left lateral knee (case 3). Two cases (cases 2&3) were presented with a painful mass. In the three patients, the masses were firm, mobile, and not attached to the skin. Magnetic resonance imaging (MRI) was done, illustrating unspecific findings to reach a diagnosis. Needle biopsies were performed in all patient, and the diagnosis of AFH was reached. Allthree patients underwent wide surgical excision of the tumor. Patients were followed up routinely every three to four months with imaging studies to rule out recurrence and metastasis, for a period of 15 months (case 1), 26 months (case 2), and 19 months (case 3), which all resulted negatively.

Highlights

  • Angiomatoid fibrous histiocytoma (AFH) is a rare, slow-growing soft tissue tumor with an intermediate biologic potential and uncertain line of differentiation, and minimal metastatic potential [1,2,3]

  • AFH is presenting as a painless mass in the cutis or subcutis, some patients could present with a paraneoplastic effect like fever, malaise, weight loss, or anemia, and it is usually located in the extremities [10]; this disagrees with what was found in case 2&3, who presented with a painful mass in the medial proximal thigh and lateral knee

  • Reaching the correct diagnosis of AFH comes with a great value; most presentations are atypical, and as a result, it is frequently misdiagnosed, either for more aggressive tumors like myxoid liposarcoma, metastatic melanoma, Ewing’s sarcoma, myxofibrosarcoma, and synovial sarcoma, or benign tumors like hematoma, hemangioma, lipoma, nerve sheath tumor, and vascular anomalies and other wide differentials depending on the age group [6]

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Summary

Introduction

Angiomatoid fibrous histiocytoma (AFH) is a rare, slow-growing soft tissue tumor with an intermediate biologic potential and uncertain line of differentiation, and minimal metastatic potential [1,2,3]. It shows minimal if any enhancement post-gadolinium administration. The second patient (case 2) showed oval shape soft tissue mass with lobulated margin, cystic degeneration with multiple areas of fluid-fluid levels suggestive of intra lesion hemorrhagic components, hypo-intense signal intensity in T1, and heterogeneous hyper-intense signal intensity in T2. The histopathological reports came as spindle cell/epithelioid neoplasm with cystic changes and hemorrhage in keeping with AFH. For the last case, immunohistochemistry showed tumor cells are positive with anti-EMA and being negative for SMA, S100 protein, AE1/AE3, CD34, and desmin. Positive for EWSR1(22q12) gene rearrangement in 100 % of nuclei (45 cells) This result, along with the morphologic and immunohistochemical features, supported the diagnosis of AFH.

Discussion
Conclusions
Disclosures
Enzinger FM
Findings
14. Fisher C

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