Right Retroperitoneoscopic Adrenalectomy for Phaeochromocytoma Following Partial Hepatectomy for MEN 2B Associated Medullary Thyroid Cancer.
Dr P Touska, Mr V Constantinides, Dr S Robinson, Dr J Cox, Mr F Palazzo
Multiple endocrine neoplasia (MEN) 2B is a rare syndrome caused by mutations in the RET proto-oncogene on chromosome 10. It is characterised by early-onset medullary thyroid carcinoma (MTC) and a distinctive phenotype (including oro-facial neuromata, prominent lips and a Marfanoid habitus). Approximately 50% of MEN 2B sufferers develop phaeochromocytoma with almost 80% being bilateral. These tumours have been linked to 64% of deaths in patients with MEN 2. In the past, adrenalectomy has been performed open via a laparotomy, via a posterior incision or laparoscopically. The laparoscopic approach is generally favoured as it reduces post-operative morbidity and inpatient stay. However, in recent years, the retroperitoneoscopic approach has gained prominence with potential benefits including reduced risk of visceral injury, less pain and easier access in patients who have undergone previous abdominal surgery. Furthermore, it enables bilateral adrenalectomy without moving the patient.
We report the case of a 31-year-old Caucasian female index case of MEN 2B with a metachronous second phaeochromocytoma treated by a right retroperitoneoscopic adrenalectomy. Her past medical history included a total thyroidectomy with second stage neck dissection, partial hepatectomy for metastatic MTC and a left laparoscopic adrenalectomy for phaeochromocytoma. Pre-operatively, the absence of recurrent MTC was confirmed by a low serum calcitonin level. Following successful oral and intravenous alpha blockade, a right retroperitoneoscopic adrenalectomy was performed. Recovery was swift and uneventful and the patient was discharged the following day.
The retroperitoneoscopic approach was particularly valuable in this case, given the likely presence of intra-abdominal adhesions and consequent risk of injury to intra-peritoneal viscera. Furthermore, the patient reported greater satisfaction in terms of post-operative pain and return to functionality compared to the previous transperitoneal laparoscopic approach. In conclusion, retroperitoneal adrenalectomy represents an excellent means of managing adrenal tumours when previous surgery such as liver resection has been performed.