Department of General Surgery, Duke Life Point Conemaugh
Memorial Medical Center, Johnstown, PA, USA
Corresponding Author:
Karleigh R. Curfman, MD, Department of General Surgery,
Conemaugh Memorial Medical Center, 1086 Franklin Street,
Johnstown, PA, USA.
Email: kcurfman@conemaugh.org
The American Surgeon
00(0) 1–4
© The Author(s) 2020
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DOI: 10.1177/0003134820945274
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Functionally Silent, Giant
Pheochromocytoma Presenting
With Varicocele
Karleigh R. Curfman, MD, Joseph A. Di Como, MD, Timothy R. Chung, DO, and
Russell D. Dumire, MD
Abstract
Adrenal incidentalomas are a known entity that have been increasing in commonality with the advent of improved
diagnostic and imaging techniques. There are a vast variety of causative pathologies to which this diagnosis can be
attributed. Some of these pathologies are more common than others, while many remain extremely rare. One of the dis-
tinct entities that is a unique cause of incidentalomas is the diagnosis presented herein: pheochromocytoma.
Pheochromocytomas are often an exceptional subset of incidentalomas that can certainly play a major role in treatment
and management plans. However, the exclusivity of a pheochromocytoma diagnosis alone is not where the uniqueness of
this case rests. The uniqueness in this patient is paramount to report due to 2 additional significant factors. The first was
that the patient was entirely asymptomatic aside from complaints related to scrotal swelling due to a varicocele, for which
this presentation of pheochromocytoma has been scarcely reported in the literature. Second, the patient had a pathology
proven diagnosis of pheochromocytoma, while lacking both symptoms and biochemical findings to support the diagnosis,
making it truly a silent pheochromocytoma.
Keywords
pheochromocytoma, incidentaloma, varicocele
SESC Annual Meeting Poster Paper
Pheochromocytoma is a rare neuroendocrine tumor of
the chromaffin cell origin often found in the adrenal
gland; more specifically, approximately 90% occur in
the abdomen, of which 90% of these occur within the
adrenal gland.
1
There is a reported estimated incidence
of pheochromocytoma at approximately 2-8 cases per 1
million people per year.
1
These tumors have been
known to secrete catecholamines and other active pep-
tides which can result in clinical symptoms.
1
Though
not witnessed in the case presented within this report,
the typical presenting symptoms can include hyperten-
sion, tachycardia, sweating, and headache.
1
Additional
symptoms that have been described are pallor, nausea,
abdominal pain, and panic attacks. Given that pheo-
chromocytomas are typically associated with hyperten-
sion, the estimated incidence of pheochromocytoma in
the hypertensive population is 0.2%.
1
Despite the evi-
dence of uniqueness of this pathology at baseline, func-
tionally silent pheochromocytomas, those presenting
without outward signs of existence, have been proven
to be even more unusual. With a reported incidence of
pheochromocytomas at 0.0008% in the general popula-
tion, silent pheochromocytomas will make up 8% of
those reported, equivalent to 0.000064% of the popula-
tion.
1
Recognition of the entity is often made via imag-
ing, typically computed tomography (CT), and then
confirmed with laboratory values, such as levels of
metanephrines, normetanephrines, or catecholamines.
1
In active pheochromocytomas, medical management
can play a significant role in treatment.
2
The aim of
medical treatment is to reduce the potentially lethal
abrupt changes in blood pressure that can occur with
anesthesia and surgical manipulation, as well as the
severe hypotension after surgical resection.
2
In silent