Sabater Marco Vicente, Navarro Cerveró Lara, Sabater Abad Vicente Javier, Bagán Sebastián José Vicente
Servicio de Anatomía Patológica. Consorcio Hospital General Universitario de Valencia, Valencia, España.
Servicio de Anatomía Patológica. Consorcio Hospital General Universitario de Valencia, Valencia, España.
Rev Esp Patol. 2022 Jul-Sep;55(3):197-202. doi: 10.1016/j.patol.2020.06.005. Epub 2020 Jul 25.
The lipomatous variety solitary fibrous tumor is a soft tissue neoplasm composed of mature adipose tissue and hemangiopericytoma areas. A 53-year-old man consulted for facial asymmetry and maxillofacial magnetic resonance imaging showed a cystic lesion, 3 x 2 cm in size, in front of the anterior wall of the maxillary sinus. Histologically, there were dense spindle cells expressing CD34, CD99, Bcl-2, and STAT6, myxoid zones, hemangiopericytomatous blood vessels, and S100 positive adipocytes. NAB2/STAT6 gene fusion was revealed by RT-PCR. The main differential diagnosis was raised with the spindle cell lipoma and malignant variant of the lipomatous solitary fibrous tumor. STAT6 overexpression and NAB2/STAT6 gene fusion are specific for lipomatous solitary fibrous tumor and the presence of lipoblasts and atypical lipomatous tumor areas suggests malignancy. These tumors located in the head and neck region have a benign biological behavior.
脂肪型孤立性纤维瘤是一种由成熟脂肪组织和血管外皮细胞瘤区域组成的软组织肿瘤。一名53岁男性因面部不对称前来就诊，上颌面部磁共振成像显示上颌窦前壁前方有一个大小为3×2厘米的囊性病变。组织学上，有表达CD34、CD99、Bcl-2和STAT6的密集梭形细胞、黏液样区域、血管外皮细胞瘤样血管以及S100阳性脂肪细胞。通过逆转录聚合酶链反应发现了NAB2/STAT6基因融合。主要鉴别诊断与梭形细胞脂肪瘤和脂肪型孤立性纤维瘤的恶性变体有关。STAT6过表达和NAB2/STAT6基因融合是脂肪型孤立性纤维瘤的特异性表现，而脂母细胞和成脂肪细胞瘤区域的存在提示恶性。这些位于头颈部区域的肿瘤具有良性生物学行为。
