Singh Neha, Thakur Kishan Kumar, Moidu Shalikh, Anand Nidhi
Department of Radiodiagnosis & Imaging, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India.
Department of Endocrine Surgery, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, India.
Radiol Case Rep. 2024 Sep 23;19(12):6152-6156. doi: 10.1016/j.radcr.2024.08.144. eCollection 2024 Dec.
Pheochromocytoma is a rare neuroendocrine tumor arising from adrenal medulla. Patients usually show classic triad of headache, palpitations and diaphoresis along with persistent or paroxysmal hypertension. Majority of the tumors are solid or mixed solid and cystic. But purely cystic variant is extremely rare with few cases reports available in the published literature. We report a case of purely cystic pheochromocytoma in a male in 30s who presented to our hospital for unrelated symptom of recurrent burning micturition. He was evaluated for causes of recurrent UTI which revealed incidental right adrenal cyst. The patient's blood pressure was raised on clinical examination and imaging showed simple adrenal cyst without solid component or septations. Suspecting possibility of pheochromocytoma, biochemical analysis was done which revealed elevated 24-hour urinary metanephrine. The diagnosis of cystic pheochromocytoma was made. Adrenalectomy was performed showing cystic lesion in the right adrenal region. Histopathology and immunohistochemistry revealed pheochromocytoma with cystic degeneration. Patient's hypertension resolved during the follow up. In conclusion, purely cystic pheochromocytoma must be considered as differential for adrenal cystic lesions, especially when atypical features are present.
嗜铬细胞瘤是一种起源于肾上腺髓质的罕见神经内分泌肿瘤。患者通常表现出头痛、心悸和多汗的典型三联征,同时伴有持续性或阵发性高血压。大多数肿瘤为实性或实性与囊性混合。但纯囊性变异极为罕见,已发表的文献中仅有少数病例报告。我们报告一例30多岁男性的纯囊性嗜铬细胞瘤病例,该患者因反复尿痛这一无关症状前来我院就诊。对其复发性尿路感染的病因进行评估时发现了右肾上腺囊肿。临床检查时患者血压升高,影像学显示为单纯肾上腺囊肿,无实性成分或分隔。怀疑有嗜铬细胞瘤的可能,进行了生化分析,结果显示24小时尿间甲肾上腺素升高。诊断为囊性嗜铬细胞瘤。实施肾上腺切除术,显示右肾上腺区域有囊性病变。组织病理学和免疫组织化学检查显示为伴有囊性变的嗜铬细胞瘤。随访期间患者的高血压得到缓解。总之,纯囊性嗜铬细胞瘤必须被视为肾上腺囊性病变的鉴别诊断之一,尤其是当出现非典型特征时。