Morphology and Beyond - A Rare Case of Metastatic Succinate Dehydrogenase-Deficient Paraganglioma of the Vulva.
Sayali Prakash Kshirsagar, Ashwini Ratnakar, Manasi A Gosavi
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Case in brief
A 35-year-old woman with a history of pheochromocytoma presented with a vulvar mass. Histology showed nests and trabeculae of neuroendocrine-appearing cells in a Zellballen pattern, resembling other nested tumors. The cells expressed synaptophysin, chromogranin, and CD56, but not cytokeratin, Melan-A, or S100; SDHB expression was lost, and the Ki-67 index was approximately 60%. Correlation with clinical and radiological findings supported a diagnosis of metastatic paraganglioma. The SDHB loss raised concern for an underlying hereditary syndrome, and genetic counseling was recommended.
What made this case unusual
The vulvar site is exceptionally uncommon, and the Zellballen morphology can mimic other tumors. In this case, the prior pheochromocytoma and immunohistochemical profile helped establish metastatic paraganglioma.
Diagnostic / clinical pearl
For a vulvar tumor with nested neuroendocrine morphology, integrate immunohistochemistry with clinical and radiological findings; SDHB loss may also raise the possibility of an underlying hereditary syndrome.