Most people probably have not heard of the term “pheochromocytomatosis.” Pheochromocytoma itself is already an odd word, let alone pheochromocytomatosis. The more I practice medicine, the more evident is to me that a disease’s presentation and natural history could be affected by medicine itself. Even 30 years ago, pheochromocytoma was still mostly suspected based on clinical symptoms; with the wide use of imaging, most cases of pheochromocytoma nowadays are found incidentally by imaging. Pheochromocytomatosis is likely a modern phenomenon associated with laparoscopic adrenalectomy.
A pheochromocytoma is surrounded by a fibrous capsule. During a surgical operation, the surgeon separates the pheochromocytoma from the normal tissue and organs and removes the tumor with the capsule intact, so that no tumor is left behind. That usually did not pose a problem in the era of open operation to remove the pheochromocytoma. During a laparoscopic operation, inexperience in surgical skills or accidents can result in rupture of the capsule and tumor cell spillage into the abdomen. The spilled tumor cells can form numerous tiny tumors that may grow to a few centimeters, which is called pheochromocytomatosis. It is not clear how often tumor capsule rupture occurs during adrenalectomy and how often pheochromocytomatosis occurs in those with tumor capsule rupture.
Once pheochromocytomatosis happens, it becomes an incurable problem. Pheochromocytomatosis, however, is indolent and may manifest itself years after the original operation. As pheochromocytomatosis is very rare, we are not sure how to best control it. Reoperation is usually done to remove visible gross tumors. Patients are then monitored. If the tumors grow again, MIBG radiotherapy or PRRT can be tried. If the tumors are still growing after the radiotherapies, somatostatin analogs and other agents may be used.
