Endocrine Abstracts

Introduction: Paragangliomas are neuroendocrine tumors that develop from the paraganglia of the sympathetic and parasympathetic nervous systems. They are benign in approximately 80% of cases and are usually localized. However, in rare situations, they can be malignant, which is defined by the presence of tumor cells in nearby tissues or at a distance (metastases). Most parasymphathetic paragangliomas are located in the skull base and along the vagus and glossopharyngeal nerves, while the majority of sympathetic paragangliomas are found in the belly. Paragangliomas found in the mesentery are extremely rare.

Observation: A 44-year-old man with no previous pathological history arrived with widespread abdominal pain extending to the left shoulder. An abdominal CT scan revealed an intra-abdominal tissue mass, paramedian, on the right below the pancreas, measuring 86/74 mm, with multiple secondary adenopathies, the largest of which was 26 mm. The tumor could not be removed (inoperable tumor), and a biopsy revealed a malignant mesenteric paraganglioma, which is why it was referred to us for care. The patient’s general condition was satisfactory, and he was normotensive with no other clinical symptoms suggesting multiple endocrine neoplasia type 2. In the 24-hour ambulatory blood pressure test, the blood pressure profile was normal, and the ophthalmological examination was normal. Hormonal evaluation: 24-hour plasma and urine methoxylates are normal; calcitonin levels and phosphocalcic assessment are also normal. Extension exploration: a cervico-thoraco-abdomino-pelvic CT scan showed a well-limited (40×10×40 mm) polylobed mesenteric intraperitoneal development with a necrotic segment VII liver nodule measuring 14×13 mm and no bone lesions. MIBG scintigraphy was negative. The patient was later sent to the Oncology Department for combined follow-up, and chemotherapy was advised as palliative treatment.

Conclusion: Non-secreting mesenteric paragangliomas with malignant potential are commonly detected inadvertently and have a poor prognosis.