Chai Louis, Ciullo Sean, Prasad Rajeev
St. Christopher's Hospital for Children, Department of Pediatric Surgery, Philadelphia, PA, 19134, United States; Drexel University College of Medicine, Hahnemann University Hospital, Department of General Surgery, Philadelphia, PA, 19102, United States.
St. Christopher's Hospital for Children, Department of Pediatric Surgery, Philadelphia, PA, 19134, United States; Drexel University College of Medicine, Hahnemann University Hospital, Department of General Surgery, Philadelphia, PA, 19102, United States.
Int J Surg Case Rep. 2019;61:263-266. doi: 10.1016/j.ijscr.2019.07.028. Epub 2019 Jul 19.
Ganglioneuromas (GN) are rare, benign tumors derived from neural crest cells. They are in the same family of neuroblastic tumors that includes the intermediate ganglioneuroblastoma (GNB) and the malignant neuroblastoma (NB), each of which carries a different prognosis based on tumor histopathology. GNs are generally asymptomatic and usually found incidentally when the tumor becomes palpable or has grown large enough to exert mass effect on adjacent structures. Unlike their malignant counterparts, GNs are rarely hormonally active and usually do not exhibit systemic metabolic activity. We present a case of an adolescent female with a pelvic tumor that was found to be a purely dopamine-secreting GN. Resection resulted in sudden dopamine withdrawal and unexpected severe emotional lability post-operatively.
A 16-year-old female presented with a history of increasingly irregular menses over the past year and was found to have an 8-centimeter pelvic tumor. Subsequent work up revealed the mass to be solely dopamine secreting. The tumor was excised without preoperative hormonal blockade. Post-operatively, the patient developed severe emotional lability and symptoms of depression, likely related to the acute withdrawal of circulating dopamine.
Ganglioneuromas are rarely metabolically active. However, a preoperative endocrine workup should be done to rule out other more commonly hormonally active tumors such as neuroblastomas, pheochromocytomas, and paragangliomas. If isolated dopamine secretion is found, hormonal blockade is not required preoperatively and operative manipulation and removal should be considered safe. However, one should anticipate potential emotional and psychiatric issues post-operatively due to the acute withdrawal of circulating dopamine.
神经节神经瘤（GN）是一种罕见的源自神经嵴细胞的良性肿瘤。它们与神经母细胞瘤家族中的其他肿瘤同属一类，该家族包括中间型神经节神经母细胞瘤（GNB）和恶性神经母细胞瘤（NB），根据肿瘤组织病理学，每种肿瘤的预后都不同。GN通常无症状，通常在肿瘤可触及或生长到足以对相邻结构产生占位效应时偶然发现。与恶性同类肿瘤不同，GN很少具有激素活性，通常也不表现出全身代谢活性。我们报告一例青少年女性盆腔肿瘤病例，该肿瘤被发现是一种单纯分泌多巴胺的GN。切除术后导致多巴胺突然戒断，并出现意外的严重情绪不稳定。
一名16岁女性，有过去一年月经越来越不规律的病史，被发现有一个8厘米的盆腔肿瘤。随后的检查显示该肿块仅分泌多巴胺。肿瘤在未进行术前激素阻断的情况下被切除。术后，患者出现严重的情绪不稳定和抑郁症状，可能与循环多巴胺的急性戒断有关。
神经节神经瘤很少有代谢活性。然而，术前应进行内分泌检查以排除其他更常见的具有激素活性的肿瘤，如神经母细胞瘤、嗜铬细胞瘤和副神经节瘤。如果发现孤立的多巴胺分泌，术前不需要进行激素阻断，手术操作和切除应被认为是安全的。然而，由于循环多巴胺的急性戒断，应预料到术后可能出现的情绪和精神问题。
