Department of Histopathology, Royal Marsden Hospital, SW3 6JJ London, UK.
Hum Pathol. 2013 Feb;44(2):289-93. doi: 10.1016/j.humpath.2012.08.008. Epub 2012 Nov 27.
Angiomatoid fibrous histiocytoma is a rare soft tissue tumor of intermediate biologic potential, predominantly arising in superficial extremities of children and young adults. Less than 5% metastasize. We describe a case of angiomatoid fibrous histiocytoma in the scalp of an 8-year-old boy, which metastasized to a postauricular lymph node 3 years after primary tumor excision. Histologically, primary and metastasis comprised nodules of bland ovoid cells, with primary additionally showing hemorrhagic cavities, fibrous capsule, and lymphoplasmacytic inflammation. Both strongly expressed desmin, with focal epithelial membrane antigen. Reverse transcription-polymerase chain reaction showed EWSR1-CREB1 fusion transcripts in both primary and metastasis. This is, to our knowledge, the first description of genetically proven metastatic angiomatoid fibrous histiocytoma. Angiomatoid fibrous histiocytoma can mimic both benign and malignant lesions, and although most behave indolently, it is important to recognize their metastatic potential.
血管外皮细胞瘤样纤维组织细胞瘤是一种罕见的软组织肿瘤，具有中等生物学潜能，主要发生在儿童和年轻成人的四肢浅表部位。不到 5%的病例发生转移。我们描述了一例发生在 8 岁男孩头皮的血管外皮细胞瘤样纤维组织细胞瘤，该患者在原发性肿瘤切除 3 年后转移至耳后淋巴结。组织学上，原发性和转移性肿瘤均由形态温和的卵圆形细胞结节组成，原发性肿瘤还显示出出血腔、纤维囊和淋巴浆细胞炎症。两种肿瘤均强烈表达结蛋白，局灶性上皮膜抗原阳性。逆转录-聚合酶链反应显示原发性和转移性肿瘤中均存在 EWSR1-CREB1 融合转录本。据我们所知，这是首例遗传证实的转移性血管外皮细胞瘤样纤维组织细胞瘤的描述。血管外皮细胞瘤样纤维组织细胞瘤可以模拟良性和恶性病变，尽管大多数表现为惰性，但认识到其转移潜能很重要。
