Mazzucchelli Manuel, Angelico Giuseppe, Salvatorelli Lucia, Vecchio Giada Maria, Romano Chiara, Vigneri Paolo, Mariani Maria Paola, Ferrara Gerardo, Magro Gaetano Giuseppe
Department of Medical, Surgical Sciences and Advanced Technologies "G.F. Ingrassia", Anatomic Pathology, University of Catania, Catania, Italy.
Department of Medicine and Surgery, Kore University of Enna, Enna, Italy.
Pathologica. 2025 Feb;117(1):39-44. doi: 10.32074/1591-951X-964.
Lipoblastoma-like tumor is a rare mesenchymal neoplasm, typically arising in the vulvar region of young women. Although it is considered a benign tumor, rare local recurrences and exceptionally distant metastases have been reported. Histological examination reveals a well-circumscribed tumor with lobulated pattern, composed of a mixture of mature adipocytes, spindle cells and lipoblasts set in abundant myxoid stroma with numerous thin-walled capillary-like vessels. Due to the rarity of this neoplasm and its morphological resemblance with other benign and malignant lipomatous tumors, the diagnosis of is often challenging. Herein, we present a case occurring in the inguinal region of a 28-year-old woman. Histological examination showed a mixture of mature adipocytes, bland-looking spindle cells with fibrillary cytoplasm, and numerous univacuolated lipoblasts set in a prominent myxoid matrix containing numerous thin-walled branching vessels. Immunohistochemically, neoplastic cells showed diffuse immunostaining for CD34 and negativity for α-smooth muscle actin, desmin, Rb1, MDM2 and STAT6. The main differential diagnoses included myxoid liposarcoma, spindle cell lipoma and cellular angiofibroma. FISH was negative for ; moreover, no evidence of regional gain or loss of was identified by FISH. Based on morphological, immunohistochemical and cytogenetic/molecular findings, a final diagnosis of " of the inguinal region was rendered.
脂肪母细胞瘤样肿瘤是一种罕见的间叶性肿瘤,通常发生于年轻女性的外阴区域。尽管它被认为是一种良性肿瘤,但已有罕见的局部复发和极少见的远处转移的报道。组织学检查显示肿瘤边界清晰,呈分叶状,由成熟脂肪细胞、梭形细胞和成脂母细胞混合组成,间质丰富呈黏液样,有许多薄壁毛细血管样血管。由于这种肿瘤罕见且其形态与其他良性和恶性脂肪瘤性肿瘤相似,其诊断往往具有挑战性。在此,我们报告一例发生于一名28岁女性腹股沟区的病例。组织学检查显示有成熟脂肪细胞、胞质呈纤维状的外观温和的梭形细胞以及许多单泡状成脂母细胞混合存在,位于含有许多薄壁分支血管的显著黏液样基质中。免疫组化方面,肿瘤细胞CD34呈弥漫性免疫染色,α-平滑肌肌动蛋白、结蛋白、Rb1、MDM2和STAT6呈阴性。主要鉴别诊断包括黏液样脂肪肉瘤、梭形细胞脂肪瘤和细胞性血管纤维瘤。荧光原位杂交检测结果为阴性;此外,荧光原位杂交未发现区域内该基因的扩增或缺失证据。基于形态学、免疫组化及细胞遗传学/分子学检查结果,最终诊断为腹股沟区“ ”。 (原文此处有缺失信息未完整给出具体诊断名称)