Extra-Adrenal and Unexpected: A Rare Case of Primary Retroperitoneal Paraganglioma
https://doi.org/10.15605/jafes.041.S1
- Author:
Raja Nurul Azafirah Raja Amir Shah
1
;
Masliza Hanuni Mohd Ali
1
;
Wan Mohd Hafez Wan Hamzah
1
;
Nor Hisham M
2
Author Information
1. Endocrinology Unit, Medical Department, Hospital Sultanah Nur Zahirah
2. Surgical Department, Hospital Sultanah Nur Zahirah
- Publication Type:Journal Article
- MeSH:
Paraganglioma
- From:
Journal of the ASEAN Federation of Endocrine Societies
2026;41(S1):66-
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Paragangliomas are rare neuroendocrine tumors arising
from extra-adrenal chromaffin cells, with an estimated
incidence of 2–8 cases per million per year. These tumors
originate from neural crest–derived cells of the sympathetic
and parasympathetic paraganglia and may secrete
catecholamines, resulting in malignant hypertension or
symptoms such as headache, palpitations, and diaphoresis.
They can occur anywhere along the paravertebral and
para-aortic regions from the skull base to the pelvic floor.
Case:We report a case of a 14-year-old male with no known
premorbid conditions who presented with presyncope
and a 1-month history of headache. On examination, he
had severe hypertension (242/167 mmHg), tachycardia
(127 bpm), and grade IV hypertensive retinopathy.
Investigations showed preserved renal function with
markedly elevated 24-hour urinary metanephrines
(normetanephrine 90.75 µmol/L). Computed tomography
revealed a lobulated, heterogeneously enhancing mass
measuring 5.0 × 6.1 × 5.4 cm along the left margin of the
abdominal aorta at the infrarenal level, suggestive of an
extra-adrenal lesion. Gallium-68 PET scan demonstrated
a somatostatin receptor–avid left peritoneal mass. The
patient underwent exploratory laparotomy and tumor
excision, complicated intraoperatively by blood pressure
lability requiring nitroprusside and inotropic support.
Postoperatively, he improved significantly and was able to
wean off all antihypertensive medications. Histopathology
confirmed left retroperitoneal paraganglioma.
Conclusion:Primary peritoneal paraganglioma is a rare but important
cause of secondary hypertension, especially in young
patients presenting with hypertensive emergency.
High index of suspicion is essential for early diagnosis.
Management requires a multidisciplinary approach
with careful preoperative optimization to minimize perioperative complications. Surgical resection remains the
definitive treatment and, as demonstrated in this case, can
result in marked clinical improvement with resolution
of hypertension.
- Full text:202608031647236365EP_A086.pdf
