Abstract | November 18, 2023
Acute Abdominal Pain Leading to Paraganglioma Diagnosis
Learning Objectives
- Identify a rare, neuroendocrine tumor in a patient with acute abdominal pain.
- Identify the initial diagnostic workup for a paraganglioma.
- Discuss the importance of outpatient hematology/oncology and surgery follow up on patients diagnosed with paraganglioma.
Introduction:
Paragangliomas are rare neuroendocrine tumors derived from extra-adrenal chromaffin cells of autonomic ganglia with the ability to secrete catecholamines. Parasympathetic paragangliomas often present in head and neck while sympathetic paragangliomas are often seen from the base of skull to bladder. When arising outside of head and neck, they often secrete catecholamines. The combined annual incidence of paraganglioma/ pheochromocytomas is 0.8 per 100,000 person years. The majority are benign, but malignancy is possible. Treatment may vary but generally involves complete resection.
Case presentation:
64-year-old male without significant medical history presented with 3-day history of right lower quadrant abdominal pain radiating to right groin. The pain was unassociated with additional symptoms and was unrelated to heavy lifting. There was no relief with medications and lying prone exacerbated the pain. The patient related his discomfort to a hernia he was diagnosed with years ago. On physical exam, he was noted to be hypertensive at 163/95 and found to have tenderness with deep palpation of the right lower quadrant. There were no palpable masses in the abdomen. Laboratory studies were unremarkable. CT scan of the abdomen revealed right hydronephrosis and a 5.1 x 4.5 cm retroperitoneal calcified mass at the aortic bifurcation. Oncology was consulted as imaging was concerning for a primary tumor. Testicular metastasis was considered, however scrotal ultrasound was unremarkable. Due to location of the mass, a diagnostic laparoscopy was required with subsequent core needle biopsy.
Final diagnosis:
Core needle biopsy of the retroperitoneal mass revealed sclerotic stroma with encased epithelioid cells and foamy cytoplasm. On immunohistochemistry, the atypical epithelioid cells stained positively for synaptophysin and surrounding cells were positive for S100. The pathology report suggested a paraganglioma. Catecholamine testing was not performed inpatient.
Management/Follow-up:
The patient was discharged home and provided close follow-up with Hematology/Oncology for additional diagnostic imaging to rule out metastases, necessary biochemical testing, and consideration of genetic testing. The patient was scheduled follow up with surgery for complete resection.
References and Resources
- Jacques W. M. Lenders and others, Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology & Metabolism, Volume 99, Issue 6, 1 June 2014, Pages 1915–1942, https://doi.org/10.1210/jc.2014-1498
- Hartmut P. H. Neumann , Charis Eng, The Approach to the Patient with Paraganglioma, The Journal of Clinical Endocrinology & Metabolism, Volume 94, Issue 8, 1 August 2009, Pages 2677–2683, https://doi.org/10.1210/jc.2009-0496
- Chrisoulidou, A., Kaltsas, G., Ilias, I., & Grossman, A. B. (2007). The diagnosis and management of malignant phaeochromocytoma and paraganglioma, Endocrine-Related Cancer Endocr Relat Cancer, 14(3), 569-585. Retrieved Jul 4, 2023, from https://doi.org/10.1677/ERC-07-0074
