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Paraaortic Extra-Adrenal Paraganglioma: Challenging Robotic Resection
Andrei Nikiforchin1, Ekaterina Baron1, Jessica A Wernberg1
1Surgical Oncology, Marshfield Medical Center, Marshfield, WI, USA.
Annals of Surgical Oncology
|April 13, 2024
Summary
Hereditary paraganglioma-pheochromocytoma syndrome (HPPS) linked to SDHB mutations requires lifelong surveillance. Robotic resection is effective, but genetic testing and cascade screening are crucial for managing familial risks.
Area of Science:
- Endocrinology
- Surgical Oncology
- Genetics
Background:
- Germline mutations, particularly in the succinate dehydrogenase (SDH) B gene, are found in up to 41% of paragangliomas, increasing the risk of malignant and metastatic disease.
- Hereditary paraganglioma-pheochromocytoma syndrome (HPPS) necessitates genetic testing and counseling for patients and families.
- Awareness of HPPS is crucial for surgeons managing these tumors.
Observation:
- A 17-year-old female presented with symptoms of a catecholamine-secreting tumor, including hypertension, tachycardia, and diaphoresis.
- Imaging identified a paraaortic mass, confirmed as a paraganglioma with an SDHB gene mutation.
- The patient underwent successful robotic resection of the retroperitoneal tumor.
Findings:
- Pathology revealed a poorly differentiated paraganglioma with no lymph node metastasis.
- Post-surgery, the patient remained disease-free with normalized biochemical markers and no detectable circulating tumor DNA.
- Genetic screening identified asymptomatic SDHB mutation carriers within the patient's family.
Implications:
- Robotic-assisted surgery is a safe and effective option for retroperitoneal malignant paragangliomas.
- Management of HPPS requires a multidisciplinary approach, including genetic counseling, cascade testing, and lifelong surveillance.
- Early identification of SDHB mutations is critical for proactive management and risk assessment in affected families.
