Department of Pathology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, 350004, China.
Department of Pathology, Fujian Provincial Hospital, Fuzhou, 350001, China.
Med Mol Morphol. 2024 Dec;57(4):299-305. doi: 10.1007/s00795-024-00400-4. Epub 2024 Jul 30.
Angiomatoid fibrous histiocytoma (AFH) is a soft tissue tumor of uncertain differentiation. Although its prognosis is good, its diagnosis and differential diagnosis remain a challenge, particularly for tumors with an atypical morphology. We evaluated the clinicopathological characteristics of 14 AFH cases and examined the key factors in its diagnosis or differential diagnosis. The cohort comprised 6 men and 8 women aged 9-65 years (average age: 31.2 years). Most of the tumors (11/14, 79%) were located in soft tissues, whereas 3/14 (21%) were located in the lung (1 case) and brain (2 cases). Tumor cells were spindle-shaped to epithelioid, with a visible fibrous capsule (9/14, 64%), hemorrhagic gap (9/14, 64%), lymphocyte sleeve (7/14, 50%), necrosis (3/14, 21%), and infiltrative boundary (4/14, 29%). The tumors expressed desmin (10/14, 71%) and exhibited low levels of Ki-67. 13 cases (93%) displayed ESWSR1 gene rearrangement. At follow-up, 1 case (7%) experienced local tumor recurrence. AFH is a rare intermediate tumor. Its pathological diagnosis requires a comprehensive analysis of histological, immunophenotypic, and molecular genetic features to avoid misdiagnosis. Our study has further enriched the histological features of AFH, emphasizing the importance of differential diagnosis and providing a reference for clinical practice.
血管肌纤维母细胞瘤（AFH）是一种分化不确定的软组织肿瘤。尽管其预后良好，但诊断和鉴别诊断仍然具有挑战性，尤其是对于形态不典型的肿瘤。我们评估了 14 例 AFH 病例的临床病理特征，并研究了其诊断或鉴别诊断的关键因素。该队列包括 6 名男性和 8 名女性，年龄 9-65 岁（平均年龄：31.2 岁）。大多数肿瘤（11/14，79%）位于软组织中，而 3/14（21%）位于肺部（1 例）和脑部（2 例）。肿瘤细胞呈梭形至上皮样，可见纤维性包膜（9/14，64%）、出血性间隙（9/14，64%）、淋巴细胞袖套（7/14，50%）、坏死（3/14，21%）和浸润性边界（4/14，29%）。肿瘤表达结蛋白（10/14，71%），Ki-67 水平较低。13 例（93%）显示 ESWSR1 基因重排。随访时，1 例（7%）发生局部肿瘤复发。AFH 是一种罕见的中间性肿瘤。其病理诊断需要综合分析组织学、免疫表型和分子遗传学特征，以避免误诊。本研究进一步丰富了 AFH 的组织学特征，强调了鉴别诊断的重要性，并为临床实践提供了参考。
