亞東醫院嗜鉻細胞瘤危機的手術治療方式和預後報告

謝尚儒¹、陳國鋅²、鐘旭東¹、兪錫全¹

¹亞東紀念醫院 外科部 一般外科；²亞東紀念醫院 外科部 泌尿科

The Surgical Intervention and Outcome of Pheochromocytoma Crisis:

A Case Series from One Single Tertiary Medical Center

Shang-Ju Hsieh², Kuo-Hsin Chen¹, Shiu- Dong Chung², Shyi-Chun Yii²

Division of Urology¹ Department of Surgery, Far Eastern Memorial Hospital, New Taipei City Division of General surgery², Department of Surgery, Far Eastern Memorial Hospital, New Taipei City

Purpose: Pheochromocytomas are neuroendocrine tumors arising from chromaffin cells of the adrenal medulla. This distinct tumors result in extremely high blood pressure in addition to other adrenergic symptoms, including the classic triad of headache, palpitations, and diaphoresis. Pheochromocytoma crisis is an unusual presentation with subsequent involvement of the cardiovascular, pulmonary, neurological, gastrointestinal, renal, hepatic, and metabolic systems. Once the tumor ruptures, sudden excessive catecholamine release from the tumor can be life-threatening. Surgical resection is the only curable treatment for pheochromocytoma. We wish to analyze the surgical outcomes for those who experiencing pheochromocytoma crisis at presentation.

Materials and Methods: In this study, we reviewed the database of pheochromocytoma treated with surgical intervention in the past 7 years from a single tertiary medical center. The patients who were confirmed with the diagnosis of pheochromocytoma based on the pathology report from 2015 to 2021 would be enrolled into the study, and those who met the criteria of at least one organ failure before the surgery were reviewed systemically.

Results: Among the total seventeen patients who were diagnosis with pheochromocytoma during 2015 to 2021, only 3 patients experienced pheochromocytoma crisis at the presentation. All of the 3 patients were female and the median age was 57 years old. The average tumor size was 10 cm and all of the tumors were cystic pheochromocytomas. All of the three patients were sent to emergency department due to circulatory crisis leading to acute respiratory failure. One of the patients underwent the emergent laparotomy for tumor excision to cease tumor bleeding, but unfortunately expired with in twenty-four hours postoperatively. The rest of two patients underwent the laparoscopic adrenalectomy after the medical resuscitation and adequate alpha blockage with doxazocin. No perioperative or postoperative complications occurred on the two survive patients and both of them recovered well. The longest follow up period was three years with no evidence of metastasis or local recurrence.

Conclusions: Pheochromocytoma should be suspected in patients presenting with circulatory failure accompanied by a retroperitoneal lesion. Pheochromocytoma crisis caused by such neoplasms can be life-threatening if the diagnosis is delayed or left untreated. Although open surgery allows for a higher chance of R0 resection and the appropriate timing of surgery for pheochromocytoma crisis remains unclear, our cases suggest that laparoscopic adrenalectomy for huge pheochromocytoma in critically ill patients is safe and effective when performed by experienced and skilled surgeons.