Cabibi Daniela, Pipitone Giulia, Porcasi Rossana, Ingrao Sabrina, Benza Ignazio, Porrello Calogero, Cajozzo Massimo, Giannone Antonino Giulio
Department of Sciences for the Promotion of Health and Mother and Child Care, Anatomic Pathology, University of Palermo, Palermo, Italy.
Anatomia Patologica, A.O.U. Policlinico 'P. Giaccone', Via del Vespro 129, 90127, Palermo, Italy.
Diagn Pathol. 2017 Aug 15;12(1):60. doi: 10.1186/s13000-017-0652-1.
Pleural angiosarcoma is a rare tumor that causes diffuse pleural thickening and effusion, mimicking mesothelioma. Immunohistochemistry is needed to highlight endothelial differentiation. We describe the first case of pleural angiosarcoma with lymphatic differentiation following radiometabolic therapy for thyroid carcinoma.
A 50-year-old man showed diffuse pleural thickening and effusion. Nine years earlier, he underwent thyroidectomy and radiometabolic therapy for thyroid carcinoma with lymph node metastases. Histologically, the tumor consisted of a solid proliferation of atypical epithelioid cells and anastomosed vascular spaces, lacking of red blood cells and containing Alcian blue positive material. The tumor showed positive immunostaining for Vimentin, CD31, CK7, D2-40, c-MYC, Ki67, focal positivity for PanCK, and negative immunostaining for Factor VIII, CD34, WT1, CK5/6, Calretinin, EMA, HBME-1, CEA, p63, EpCAM, Bcl-2, TTF1 and Thyroglobulin. CD99 showed a granular/paranuclear pattern of positivity. The histological and immunohistochemical features were consistent with "pleural angiosarcoma with lymphatic differentiation, epithelioid variant".
Epithelioid angiosarcoma with lymphatic differentiation is very rare and aggressive. Moreover, the positivity for c-MYC suggests the relationship with radiometabolic therapy. To our knowledge, this is the first case of pleural c-MYC-positive angiosarcoma with lymphatic differentiation reported in the literature and the first one arisen after radiometabolic therapy for thyroid carcinoma.
胸膜血管肉瘤是一种罕见肿瘤，可导致弥漫性胸膜增厚和胸腔积液，酷似间皮瘤。需要通过免疫组织化学来突出内皮分化。我们描述了首例甲状腺癌放射性核素治疗后发生的具有淋巴管分化的胸膜血管肉瘤病例。
一名50岁男性表现为弥漫性胸膜增厚和胸腔积液。9年前，他因甲状腺癌伴淋巴结转移接受了甲状腺切除术和放射性核素治疗。组织学上，肿瘤由非典型上皮样细胞的实性增生和相互吻合的血管腔构成，缺乏红细胞并含有阿尔辛蓝阳性物质。肿瘤波形蛋白、CD31、细胞角蛋白7、D2-40、c-MYC、Ki67免疫染色呈阳性，广谱细胞角蛋白局灶性阳性，而凝血因子VIII、CD34、WT1、细胞角蛋白5/6、钙视网膜蛋白、上皮膜抗原、HBME-1、癌胚抗原、p63、上皮细胞黏附分子、Bcl-2、甲状腺转录因子1和甲状腺球蛋白免疫染色呈阴性。CD99呈颗粒状/核旁阳性模式。组织学和免疫组化特征符合“具有淋巴管分化的上皮样胸膜血管肉瘤”。
具有淋巴管分化的上皮样血管肉瘤非常罕见且具有侵袭性。此外，c-MYC阳性提示与放射性核素治疗有关。据我们所知，这是文献中报道的首例具有淋巴管分化的胸膜c-MYC阳性血管肉瘤病例，也是首例甲状腺癌放射性核素治疗后发生的此类病例。
