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[与甲状旁腺激素相关蛋白(PTH-rP)相关的高钙血症揭示恶性肝上皮样血管内皮瘤]

[Hypercalcemia related to PTH-rP revealing malignant hepatic epithelioid hemangioendothelioma].

作者信息

Boukhris Imène, Azzabi Samira, Kéchaou Ines, Chérif Eya, Kooli Chékib, Romdhane Khaled Ben, Omar Souheil, Khalfallah Narjes

机构信息

Service de médecine interne, Hôpital Charles Nicolle, Faculté de médecine, Université El Manar, Tunis, Tunisie.

Laboratoire d'anatomopathologie, Institut Salah Azaiez, Tunis, Tunisie.

出版信息

Ann Biol Clin (Paris). 2016 Jan-Feb;74(1):98-102. doi: 10.1684/abc.2015.1118.

Abstract

Hypercalcemia caused by tumor production of PTH-rp occurs most often in cases of squamous cell carcinoma of the lung, aerodigestive tract cancer, gynecological cancer and lymphoma. We report an exceptional case of PTH-rp related to a hepatic hemangioendothelioma. A 70 years-old male admitted for deterioration of the general state. The laboratory investigations revealed hypercalcemia, related to tumor production of PTH-rp. Imaging revealed tumoral hepatic lesions. Histopathological study and immunohistochemistry showed diffuse response for CD31 marker, CK20 (+) with CK7 (-) and hepatocyt antigen (-). The diagnosis of PTH-rp related to hepatic hemangioendothelioma was make. The patient died with recurrence of fatal hypercalcemia. Management of patients presenting with humoral hypercalcemia includes a vigorous search for tumor lesions. Elevated PTH-rp can be a bad prognostic factor. In front of tumoral liver lesions, a hepatic epithelioid hemangioendothelioma must be considered. Immunohistochemistry is necessary to make diagnosis.

摘要

由肿瘤产生甲状旁腺激素相关蛋白(PTH-rp)引起的高钙血症最常见于肺癌、气消化道癌、妇科癌症和淋巴瘤患者。我们报告了一例与肝血管内皮瘤相关的PTH-rp异常病例。一名70岁男性因全身状况恶化入院。实验室检查发现高钙血症,与肿瘤产生PTH-rp有关。影像学检查显示肝脏有肿瘤性病变。组织病理学研究和免疫组化显示CD31标记弥漫性反应,细胞角蛋白20(CK20)阳性、细胞角蛋白7(CK7)阴性且肝细胞抗原阴性。诊断为与肝血管内皮瘤相关的PTH-rp。患者因致命性高钙血症复发而死亡。对出现体液性高钙血症的患者进行管理包括积极寻找肿瘤病变。PTH-rp升高可能是不良预后因素。面对肝脏肿瘤性病变时,必须考虑肝上皮样血管内皮瘤。免疫组化对于做出诊断是必要的。

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