Paraganglioma of the spermatic cord: A rare tumor with unique imaging findings and diagnostic challenges

Abstract

Pheochromocytomas and paragangliomas are rare endocrine neoplasms derived from neural crest cells, with spermatic cord paragangliomas being exceptionally uncommon. A 42-year-old man presented with a longstanding complaint of left scrotal enlargement. Initial imaging raised suspicion of testicular carcinoma, but contrast-enhanced computed tomography and magnetic resonance imaging revealed a well-circumscribed, hypervascular mass distinct from the testis. The tumor exhibited heterogeneous T2 signal intensity, characteristic of vascular lesions, a thick capsule, and early-phase peripheral contrast enhancement with delayed homogeneous filling. A solitary fibrous tumor was initially considered as a differential diagnosis. Surgical resection confirmed the tumor's origin in the spermatic cord. Histopathology revealed small, round neoplastic cells with a delicate sinusoidal vascular network, and immunohistochemical analysis was positive for chromogranin A and synaptophysin, confirming the diagnosis of paraganglioma, with its origin traced to the spermatic cord. The surgical margins were clear, and postoperative imaging showed no metastases. At 18 months follow-up, no recurrence was detected, and biochemical markers remained normal. This case highlights the diagnostic challenges of spermatic cord paragangliomas due to their rarity and imaging resemblance to other intra-scrotal neoplasms. Although preoperative diagnosis is crucial for appropriate management, almost all of the reported cases of spermatic cord paragangliomas have been diagnosed postoperatively. New imaging techniques, including ⁶⁸Ga-DOTATATE PET/CT, may change this situation. This report expands the limited literature on spermatic cord paragangliomas and underscores the importance of considering paraganglioma in the differential diagnosis of intra-scrotal masses.