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A Long-Standing Primary Vaginal Paraganglioma—Coexisting with Esophageal Carcinoma

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Sir, Paraganglioma is a rare neoplasm derived from the neural crest cells residing in the paraganglia of the autonomic nervous system. Primary vaginal paraganglioma is extremely rare. [1]. Herein, we… Click to show full abstract

Sir, Paraganglioma is a rare neoplasm derived from the neural crest cells residing in the paraganglia of the autonomic nervous system. Primary vaginal paraganglioma is extremely rare. [1]. Herein, we present a unique case of a vaginal paraganglioma which was radiologically interpreted as a metastatic nodule in a young female, which is a known case of esophageal carcinoma. A 28-year-old married female presented with dysphagia to solid foods was diagnosed as having keratinizing squamous cell carcinoma of the lower esophagus on biopsy 5 years back. She received neoadjuvant chemoradiotherapy (NACRT). Post-NACRT PET-CT revealed moderate to intense FDG (flurodeoxyglucose) uptake with decline in SUV max to 6.7 indicating a favorable response to chemotherapy. However, PET-CT revealed an intense FDG-avid (SUV max = 20.9) homogeneous soft tissue lesion measuring 2.1 × 2.1 cm in the left lateral vaginal wall with well-maintained fat planes with the adjacent urinary bladder wall (Fig. 1a). On pervaginum examination, a firm, well-defined mass measuring 2 × 3 cm was noted along the middle one-third of the left lateral vaginal wall, though she did not have any gynecological complaints. Fine-needle aspiration cytology (FNAC) from the vaginal mass did not reveal any malignant cells, and the cells were interpreted as histiocytes. Following this, she underwent trans-hiatal esophagectomy which confirmed the diagnosis of keratinizing squamous cell carcinoma. Her follow-up PET-CT scan post-surgery showed similar FDG uptake in the vaginal mass, without any increase in its size. However, the most recent PET-CT scan showed increase in the FDG uptake. Contrast-enhanced computed tomography (CECT) showed the vaginal lesion to be heterogeneously enhancing soft tissue mass measuring 3.6 × 2 cm with increased vascularity and a clinical possibility of metastatic tumor deposit was favored. USGguided FNAC from the mass revealed predominantly blood along with few histiocytic collections (Fig. 1b). Finally, the mass was surgically excised and the vaginal wall was reconstructed. Grossly, it measured 3 × 3 cm and had a firm and brown-colored cut-surface. On microscopic examination, the tumor exhibited a classical Zellballen pattern comprising of nests and islands of tumor cells with a prominent intervening fibrovascular stroma (Fig. 1c). The tumor cells were large and polygonal shaped with fine-stippled chromatin, inconspicuous nucleoli, and abundant granular eosinophilic to clear cytoplasm with well-defined cell membranes. Tumor cell nests were bordered by few spindle-shaped sustentacular cells (Fig. 1a). At the periphery, the blood vessels were evenmore prominent and hyalinized. No mitosis or necrosis was noted. On IHC, the tumor cells were strongly positive for chromogranin (Fig. 1d), synaptophysin (Fig. 1e), neuron-specific enolase (NSE), and CD 56. S-100 protein highlighted the sustentacular cells (Fig. 1f), and pan-cytokeratin was negative. Based on these morphological features, a diagnosis of primary vaginal paraganglioma was made. Retrospective review of FNAC smears revealed few singly scattered tumor cells with round nuclei and abundant granular cytoplasm consistent with * Rashi Garg [email protected]

Keywords: carcinoma; vaginal paraganglioma; mass; primary vaginal; paraganglioma; tumor

Journal Title: Indian Journal of Surgical Oncology
Year Published: 2017

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