Faizan Mahwish, Manzoor Jaida, Saleem Muhammad, Anwar Saadia, Mehmood Qaiser, Hameed Ambreen, Ali Agha Shabbir
Department of Pediatric Hematology-Oncology, The Children's Hospital and ICH, Lahore.
Department of Pediatric Endocrinology, The Children's Hospital and ICH, Lahore.
J Coll Physicians Surg Pak. 2017 May;27(5):313-315.
Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.
副肿瘤综合征是由免疫系统对肿瘤的异常反应引发的罕见病症。副肿瘤综合征可能是癌症的首发或最突出表现。肾母细胞瘤是儿童最常见的肾脏恶性肿瘤，通常表现为腹部肿块。文献中曾描述过一些不寻常的表现，如获得性血管性血友病、肺栓塞导致的猝死以及库欣综合征。库欣综合征作为儿童恶性肾肿瘤的首发症状是一种非常罕见的情况。文献中很少有病例报告探讨术前化疗以及直接进行肾切除术的选择。我们报告一例因肾母细胞瘤导致的罕见副肿瘤性库欣综合征病例。基于术后体重、血压、血清促肾上腺皮质激素和血浆皮质醇水平逐渐下降，以及肾母细胞瘤的组织学确诊，确诊为由肾母细胞瘤导致的副肿瘤性库欣综合征。
