Endocrine Abstracts

Introduction: Paragangliomas are chromaffin cell tumors that arise from neuroectodermal cells. Multiple paragangliomas in the same patient are highly uncommon. These tumors have a variable clinical picture, and a very heterogeneous evolution. The pulmonary localization is one of the most frequent, representing 20 to 30% of all neuroendocrine tumors. The silent or aggressive character causes problems in diagnosis, management and prognosis.

Case report: We report a case of 46-year-old women, with no significant past medical, family and psychosocial history, admitted for management of two left mediastinal and retroperitoneal lesions, revealed by deep asthenia, anorexia, weight loss and recurrent abdominal pain. The clinical examination reveals an anorexic patient presenting profuse sweating, palpitations, nausea, vomiting and flush syndrome without clinical signs of carcinoid syndrome or catecholamine’s tumor hypersecretion. Screening test for hypertension revealed correct blood pressure (100/56 mmHg). Biological exploration confirmed the non-secreting nature of these masses, with a negative level of plasma catecholamines and 5 HIAA. Contrast-enhanced CT of the abdomen revealed a left mediastino-pulmonary lesion measuring 105*94 mm in contact with the thoracic aorta, the left subclavian artery, and the left common carotid artery which are permeable, invading the left upper pulmonary lobe. Another similar avidly enhancing necrotic solid lesion was seen in the infrarenal latero-aortic retroperitoneal measuring 53*29 mm. CT-guided transthoracic needle biopsy was done. The anatomopathological and immunohistochemical study concluded to a moderately differentiated neuroendocrine paraganglioma tumor, positive for chromogranin A, synaptophysin andSOX10; with a Ki67% at 5%. The MIBG scintigraphy confirmed the neuroendocrine nature of the tumors and does not reveal any secondary distant location. Surgical approach is rejected by our RCP due to the intime vascular relationships, and a therapeutic MIBG scintigraphy is proposed for the patient.

Conclusion: An associated non-functional thoracic and retroperitoneal paraganglioma is a very rare clinical situation. Often asymptomatic, their prognosis is dependent to local evolution and the presence of distant metastases. Management must be multidisciplinary, and surgical excision remains the standard treatment.