Article type : Case Report
Correspondace : Tirath Patel
Institute: American University of Antigua
Department: Medicine
City: Antigua and Barbuda
Contact: +91-8128250661
Email:
Tirathp611@gmail.com
Conflict of interest: None
Declaration: None
Funding: None
Abstract : This case report describes a 45-year-old man with a
history of episodic headache, palpitations, and sweating who was
diagnosed with pheochromocytoma. The diagnosis was confirmed by elevated
levels of plasma catecholamines and metanephrines and imaging studies
that revealed a 3 cm mass in the right adrenal gland with evidence of
local invasion into surrounding tissues. The patient underwent a
laparoscopic right adrenalectomy and was discharged on the third
postoperative day with normal blood pressure. Histopathological
examination confirmed the diagnosis of pheochromocytoma with evidence of
capsular and vascular invasion, but no malignancy markers were
identified. The patient was followed up for six months postoperatively
with resolution of symptoms and no evidence of tumor recurrence on
imaging. This case report highlights the importance of early diagnosis,
appropriate management, and follow-up for pheochromocytoma.
Introduction : Pheochromocytoma is a rare tumor arising from the
chromaffin cells in the adrenal medulla that produces catecholamines
(epinephrine and norepinephrine) in an uncontrolled and excessive
manner. It accounts for less than 0.2% of all diagnosed hypertension
cases. This tumor can occur sporadically or as part of an inherited
syndrome like Multiple Endocrine Neoplasia (MEN) or von Hippel-Lindau
(VHL) disease. Pheochromocytoma can present with a wide range of
symptoms including episodic headache, sweating, palpitations, anxiety,
and hypertension. The diagnosis can be challenging due to the
nonspecific nature of the symptoms, and hence, imaging and biochemical
testing play a crucial role. Surgical resection is the mainstay of
treatment.
Case Presentation : A 45-year-old man presented with a history
of episodic headache, palpitations, and sweating for the past 6 months.
He also reported feeling anxious and irritable during these episodes. He
denied any history of hypertension or other chronic medical conditions.
There was no family history of pheochromocytoma or other endocrine
disorders. Physical examination was unremarkable except for an elevated
blood pressure of 170/100 mmHg. Laboratory investigations revealed
elevated levels of plasma catecholamines (epinephrine: 550 pg/mL,
norepinephrine: 700 pg/mL) and metanephrines (metanephrine: 1800 pg/mL,
normetanephrine: 1200 pg/mL), confirming the diagnosis of
pheochromocytoma. Further investigations were performed to determine the
location of the tumor. Abdominal computed tomography (CT) scan showed a
3 cm mass in the right adrenal gland with evidence of local invasion
into the surrounding tissues. Magnetic resonance imaging (MRI) of the
brain was normal. Genetic testing for MEN and VHL was negative. The
patient was started on alpha-blockers (phenoxybenzamine) to control his
blood pressure and prevent intraoperative hypertensive crisis. He
underwent a laparoscopic right adrenalectomy. Intraoperative findings
confirmed the presence of a pheochromocytoma with local invasion into
the adjacent tissues. The tumor was removed completely, and the
postoperative period was uneventful. The patient was discharged on the
third postoperative day with normal blood pressure. Histopathological
examination confirmed the diagnosis of pheochromocytoma. The tumor
measured 3.5 cm in diameter and showed evidence of capsular and vascular
invasion. The mitotic rate was low, and Ki-67 index was 5%. The tumor
was negative for malignancy markers. The patient was followed up in the
clinic for six months postoperatively. He reported resolution of his
symptoms, and his blood pressure was normal on follow-up visits. Repeat
imaging did not show any evidence of tumor recurrence.
Discussion : Pheochromocytoma is a rare tumor that can present
with a wide range of symptoms, making the diagnosis challenging.
Biochemical testing for plasma catecholamines and metanephrines is the
cornerstone of diagnosis. Imaging studies like CT and MRI can localize
the tumor and determine its extent of invasion. Alpha-blockers are used
to control hypertension and prevent intraoperative hypertensive crises
during surgical resection. The prognosis of pheochromocytoma is
generally good with surgical resection being the mainstay of treatment.
However, the risk of recurrence and metastasis is higher in patients
with malignant pheochromocytoma.
Conclusion : In conclusion, this case report highlights the diagnostic and therapeutic challenges associated with pheochromocytoma, a rare tumor arising from the chromaffin cells in the adrenal medulla. The diagnosis of pheochromocytoma requires a high index of suspicion, given its nonspecific symptoms. The biochemical testing for plasma catecholamines and metanephrines and imaging studies like CT and MRI play a crucial role in the diagnosis and management of this tumor. Surgical resection is the mainstay of treatment, with alpha-blockers being used to control hypertension and prevent intraoperative hypertensive crises. The prognosis of pheochromocytoma is generally good with surgical resection, although the risk of recurrence and metastasis is higher in patients with malignant pheochromocytoma. This case report underscores the importance of early diagnosis, appropriate management, and follow-up for pheochromocytoma to ensure the best possible outcomes for patients.
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