Large Paraganglioma of the Abdominal Cavity: A Case Report and Review of the Literature

Abstract

Background: Paragangliomas are rare tumors that derive from cells of the autonomic nervous system. They are usually located in the neck, i.e. arising from the glomus caroticum or glomus jugulare, but may also be located in the mediastinum and abdominal cavity arising from other ganglia. Paraganglioma located in the adrenal gland are called pheochromocytoma. Case Report: We report a case of an oligosymptomatic 50-year-old man presenting with a large intraabdominal tumor mass measuring 24 × 22 × 12 cm. Core needle biopsy revealed a tumor of mesenchymal origin with no clear-line differentiation, so the highly vascularized tumor was resected after embolization of the tumor vessels. Histology revealed epithelioid cells with expression of CD68 and CD10 but no expression of Pan-CK, CD30, or CD45. Ki67 staining was 20%. Lymphangiosis and angioinvasion were demonstrated. Differential diagnosis included histiocytic sarcoma and c-kit-negative gastrointestinal stromal tumor; the final diagnosis was paraganglioma. The 6-month follow-up showed no evidence of recurrence. Conclusions: Paraganglioma is a rare disease and should be considered in the differential diagnosis of abdominal masses. To our knowledge, this report is of the largest paraganglioma that has been described in the literature so far. Nomenclature, pathogenesis, and treatment options are discussed.

Nonfunctioning symptomatic paraganglioma: Is there an optimal follow-up for patients with extra-adrenal benign paragangliomas

“…Size is variable and >20 cm paragangliomas have been reported [4]. According to the study by Laird et al , size on its own is not correlated in a statistically significant way with malignancy in both paragangliomas and pheochromocytomas, but other factors such as invasion to adjacent organs and distant metastases are considered diagnostic for malignancy.…”

Multiple Retroperitoneal Paragangliomas: An Uncommon Entity

“…Paragangliomas are rare neuroendocrine neoplasms derived from neuroectodermal cells of the autonomous nervous system. 1 Also christened as extra-adrenal pheochromocytoma, they may occur anywhere from the skull base to the pelvic floor along the distribution of the paraganglia. Paragangliomas when retroperitoneal may pose diagnostic difficulty clinicoradiologically, especially when not associated with symptoms due to hormonal imbalance.…”