Endocrine Abstracts

Introduction: Paragangliomas (PGL) are rare neuroendocrine tumors of the chromaffin cells of the sympathic and parasympathic ganglia. They are responsible for catecholaminergic hypersecretion syndrome. Clinical presentation and malignant potential are variable and closely linked to the genetic profile.

Clinical Case 1: Fifty three year old patient, diagnosed with diabetes and high blood pression for 4 years, she reported a Menard’s Triad evolving for 5 years. Abdominal CT scan noted a mass in the right adrenal lodge, spontaneous density (40 HU), measuring approximately 51×31×35 mm, completed by Urinary Methoxyled Derivatives (UMD) of 24 h, elevated to 30 times normal for metanephrine (MN). A right adrenalectomy performed but with no clear clinicobiological improvement. UMD controle were positive to 9 times normal for MN. MIBG scintigraphy revealed a retroperitoneal mass over the right kidney measuring 30*29 mm, associated with an 8.7 mm hepatorenal lymph node, suggesting local invasion. Surgical revision was performed, with histological evidence of a paraganglioma. An octreoscan performed after the 2nd surgery revealed the persistence of a 21×17 mm right retroperitoneal mass. After a multidisciplinary staff meeting, the diagnosis was a locally advanced abdominal paraganglioma; the decision was a 3rd surgery.

Clinical Case 2: Forty eight year old patient, followed for multiple breast, ovarian and S2-S3 radicular cysts, abdominal imaging performed in the face of chronic right lumbar pain, revealed a heterogeneous right retrocavous mass measuring 39×28×25.6 mm. She reported a chronic Menard’s Triad. Urinary Methoxyled Derivatives (UMD) of 24 h, elevated to 6 times normal for normetanephrine (NMN). A surgical removal of the mass was done with histological evidence of paraganglioma PASS score 2.

Discussion: The frequency of advanced PGLs in certain genetic diseases varies from 1% to 90%. SDH mutations account for 30% of patients with mtastatic PGL. ¹. Retroperitoneal localization is rare. Certain PGL can be hereditary, particularly in the case of VHL syndrome. Surgery is potentially curative, but tumor dissemination limits the chances of curative resection (2). Other treatment modalities include radiopharmaceutical techniques, chemotherapy, radiotherapy and experimental therapies. Targeted radiotherapy with 131 I MIBG is an option for systemic treatment (3,4).

Conclusion: Surgery remains the cornerstone in locally advanced forms, supplemented by chemotherapy depending on progression, with a response rate of around 40-50%, despite the limitation represented by the scarcity of retrospective studies, the lack of impact on overall survival and the associated toxicity profile (5).

Key words: paraganglioma, genetic profil, surgery.