Diagnostic enigma in retroperitoneal paraganglioma--a case report

Paragangliomas and pleomorphic undifferentiated sarcoma/malignant fibrous histiocytomas are examples of uncommon tumours in retroperitoneum. Where malignant fibrous histiocytoma is to be treated aggressively to save the life, long-term follow-up after surgery is the basic management for paraganglioma. Hence an inappropriate diagnosis of malignant fibrous histiocytoma in place of a paraganglioma which in most cases behave as benign tumour, can lead to a significant complication related to postoperative therapy. Here in this case a patient of retroperitoneal tumour having discrepancies between pre-operative cytological and postoperative histopathological diagnosis is reported. After careful review of clinical, radiological and histopathological features, the appropriate diagnosis of paraganglioma was rendered and the patient was kept for long-term close follow-up instead of aggressive therapy. The appropriate clinicopathologic correlation of the case by a group of experienced oncologists and pathologists revealed the diagnostic enigma and offered the appropriate management which may otherwise lead to iatrogenic complications.