IntroductionPheochromocytomatosis, defined as the implantation of pheochromocytoma
cells to the intraoperatively opened surfaces during surgical manipulation, is an
infrequent complication of surgical intervention of pheochromocytomas. Only a handful
of pheochromocytomatosis cases have been reported since the first case was described
in 2001.Case reportIn 2011, a 33-year-old male patient presented with episodic palpitations
and hypertensive surges triggered by physical activity. Imaging revealed a left adrenal
tumor, which showed intense radiopharmaceutical uptake on 131I-metaiodobenzylguanidine
([131I]MIBG) scintigraphy. Urinary analysis of metanephrines confirmed pheochromocytoma,
and laparoscopic left-sided adrenalectomy was performed. Owing to the large tumor
size, intraoperative fragmentation was necessary for removal. The patient remained
asymptomatic for five years. In 2016, recurrent paroxysmal symptoms prompted imaging,
revealing a lesion at the left renal hilum. During the reoperation in 2017, multiple
peritoneal tumor deposits were observed and later confirmed histologically. Over the
following years, the patient received conservative, symptomatic treatment with tolerable
paroxysmal symptoms. In 2023, worsening symptoms led to the decision to commence three
cycles of ([131I]MIBG) therapy, followed by alleviation of symptoms, and a decrease
in biochemical parameters.DiscussionAn extensive literature search for publications
from the past 25 years identified 22 pheochromocytomatosis cases whose details were
also summarized and analyzed. This condition appears to have a longer recurrence-free
survival compared to patients’ cohorts with metastatic pheochromocytomas. Pheochromocytomatosis
is usually characterized by a prolonged asymptomatic postsurgical interval, emphasizing
the need for long-term follow-up with close biochemical and radiological surveillance.
Treatment strategies parallel those used for advanced/metastatic pheochromocytomas.
