Abstract
Background Pheochromocytoma is a tumour of the adrenal medulla, derived from catecholamine producing chromaffin cells. Malignant pheochromocytomas constitute 10–25% of all cases. These are difficult to diagnose microscopically. Therefore, malignant pheochromocytomas are diagnosed by the presence of local invasion or metastatic disease. Case presentation We present a case of malignant Pheochromocytoma in a 20-year-old woman from south India with classic symptoms whose urinary metanephrines levels were elevated. After controlling the blood pressure preoperatively and laparoscopic right-sided adrenalectomy was performed. The Postoperative period was uneventful. Histopathology proved to be malignant pheochromocytoma with a PASS score of 16/20 and immunohistochemical staining was positive. DOTATATE PET/CT showed no evidence of disease anywhere else in the body. Conclusion Malignant pheochromocytomas are rare tumor, so they pose a significant diagnostic and therapeutic challenge. Surgery is the mainstay of treatment. DOTATATE PET/CT helps in the localization of metastatic disease.
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