Deslarzes Philip, Djafarrian Reza, Matter Maurice, La Rosa Stefano, Gengler Carole, Beck-Popovic Maja, Zingg Tobias
Department of Visceral Surgery, Lausanne University Hospital, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.
Department of Pathology, Institute of Pathology, Lausanne University Hospital, Lausanne, Switzerland.
Front Pediatr. 2022 May 17;10:869518. doi: 10.3389/fped.2022.869518. eCollection 2022.
Neuroblastic neoplasms (NN) include ganglioneuromas (GN), ganglioneuroblastomas (GNB), and neuroblastomas (NB). They generally arise in childhood from primitive sympathetic ganglion cells. Their incidence in adults, especially among elderly, is extremely low.
This is the case of a 74-year-old woman with history of abdominal pain, weakness and night sweating since several months. Blood pressure was normal. CT-scan showed a 10 cm left adrenal mass, without other pathologic findings. An open left-sided adrenalectomy was performed. Recovery was uneventful with hospital length of stay of 8 days. Based on morphological, immunohistochemical, and molecular features the diagnosis was a nodular GNB. A positron emission tomography (PET) performed 6 weeks after the resection did not show any residual tumor or distant metastases. The patient was followed-up with annual clinical and radiological exams.
This case presentation, associated with a review of the literature, illustrates the importance to include NN in the preoperative differential diagnosis of adrenal tumors in adults and highlights the need for multidisciplinary patient work-up and management.
神经母细胞瘤性肿瘤(NN)包括神经节瘤(GN)、神经节神经母细胞瘤(GNB)和神经母细胞瘤(NB)。它们通常在儿童期起源于原始交感神经节细胞。其在成人中的发病率极低,尤其是在老年人中。
这是一名74岁女性的病例,她数月来一直有腹痛、乏力和盗汗的症状。血压正常。CT扫描显示左侧肾上腺有一个10厘米的肿块,无其他病理发现。实施了开放性左侧肾上腺切除术。恢复过程顺利,住院时间为8天。根据形态学、免疫组织化学和分子特征,诊断为结节性GNB。切除术后6周进行的正电子发射断层扫描(PET)未显示任何残留肿瘤或远处转移。对该患者进行了年度临床和影像学检查随访。
本病例报告结合文献复习,说明了在成人肾上腺肿瘤的术前鉴别诊断中纳入NN的重要性,并强调了对患者进行多学科检查和管理的必要性。