PP 51. Retroperitoneal Paraganglioma Presenting Management Dilemmas in a Teenager

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S. Anwar, S. Mehmood, M. Siraj Ali, F. Siddiqui, N. Khurshaidi

18:46 - 18:52h at Lanchid Room

Categories: Poster Session , Surgical Oncology

Session: Poster (P4) - Surgical Oncology

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Background
Paragangliomas are rare tumours arising from extra-adrenal neuroendocrine cells. Most paragangliomas occur in head and neck region and occurrence at other sites including retroperitoneal area is relatively uncommon. Although a familial predisposition has been described, it remains a rare clinical consideration in healthy teenagers. We report a case of retroperitoneal paraganglioma in a teenager and discuss management dilemmas associated with this condition.

Material and Methods
A 17-year-old boy presented with one-day-history of severe left upper quadrant pain and a palpable mass. A CT scan revealed a 10x8.5 cm mass in the retroperitoneal area, suggestive of sarcoma. The patient underwent resection of tumour along with involved segment of colon. The histological examination confirmed paraganglioma. A 131I- Metaiodobenzylguanidine (MIBG) scan excluded distant metastasis.

Result
Approximately half of abdominal paragangliomas are malignant and once suspected, certain management considerations are pivotal. Urinary and plasma catecholamines along with CT, MRI, and MIBG scanning should be organised to diagnose, localise and characterise these tumours. Surgical excision is the treatment of choice for primary and recurrent tumours. Other treatment options include preoperative debulking with radiotherapy, chemotherapy or ablation with high dose I-131 MIBG. Unfortunately, no histological criteria for malignancy exist. These tumours are considered malignant if metastasis or recurrence occurs, requiring a lifelong follow-up.

Conclusion
Paragangliomas are rare tumours, and extensive biochemical and radiological investigations are usually required prior to surgical intervention. Inexistence of histological criteria for malignancy necessitates a lifelong surveillance in these patients.