Pheochromocytomas in pediatric Surgery: A Case Report

Robin Perdana Saputra, Andi Dwihantoro

Pediatric Surgery Division, Department of Surgery, Faculty of Medicine Universitas Gadjah
Mada/Dr. Sardjito Hospital, Yogyakarta 55281, Indonesia

ABSTRACT

Introduction
Pheochromocytomas (PHEO) and paragangliomas (PGL) are neural crest-derived
tumors that can arise from the sympathetic and parasympathetic paraganglia from the cranial
base to the pelvis. PHEO specifically refers to tumors that secrete catecholamines originating
in the adrenal medulla, whereas PGLs are at an extra-adrenal location and can secrete
catecholamines actively (functional PGL) or inactive (non-functional PGL). PHEO and PGL
are rare lesions in pediatrics. The overall incidence of PHEO and PGL is <0.3 cases per
million per year with 10-20% of these cases diagnosed during childhood. These tumors are
slightly more common in male children and diagnosed at a mean age of 11 years.

Case presentation
An eleven year old boy, 3 months before admission was complaining of right
abdominal pain with hypertension accompanied by vomiting, headache and sweating.
Doppler ultrasound found bilateral diffuse parenchymal renal disease. One month later the
complaint got worse. The patient underwent a contrast abdominal MSCT and the result
showed a mass in dextra suprarenal leading to Pheochromocytoma. The patient received
terazosin 1x5 mg, Mini aspirin 1x80 mg, Bisoprolol 2x5 mg, Candesartan 1x4mg as
therapies. The mass was not palpable from the physical examination. There were periods of
hypotension and hypertension during treatment. Later the patient was planned for an
excisional biopsy.

Results
The patient was planned for a right total adrenalectomy laparotomy and it was
successful. The patient is placed in the jackknife position on the Surgical table. A cross
section placed just below the tip of the 12th rib to enter the retroperitoneal space. Digital
palpation is used to expand the space. Continuous intraoperative blood pressure monitoring
via Intra-arterial catheters is very important and assists the anesthesiologist in managing
blood pressure fluctuation. An important principle of tumor resection is minimal
manipulation of the tumor to prevent the release of catecholamines that can cause
hemodynamic instability and the risk of tumor rupture.
After the procedure, the patient was admitted to the intensive care unit ward, and his
condition is stable. We evaluate vital signs and risk of postoperative hypotension or recurrent
hypertension.

Conclusion
Preoperative management anticipates complications of intravascular instability and
fluctuations in blood pressure that may occur during manipulation or removal of a
hormonally active tumor. The goals of perioperative antihypertensive management are
twofold (2): (1) avoiding fluctuations in blood pressure during induction of anesthesia and
surgical manipulation and (2) preventing postoperative hypotension due to an immediate
decrease in catecholamine load following surgical removal of the tumor. Laparotomy is
performed in patients with large or difficult-to-reach tumors. Transperitoneal approach or
retroperitoneal surgery is recommended, although it will depend on the location, size of the
tumor, and surgeon preference. During the initial 48 hours after surgery, close monitoring in
the intensive care unit is required because hemodynamic instability can occur at any time.