Abstract
INTRODUCTION: Primary mucinous carcinoma of the skin is a very rare malignant tumor arising from sweat glands, with just over 200 cases reported in the literature. Typically, a low-grade neoplasm with low metastatic potential yet a high recurrence rate, it most commonly occurs in the head and periorbital region and predominantly affects patients over 60 years. PRESENTATION OF CASE: A 72-year-old man with no medical or surgical history presented with a month-long history of coccygeal pain. Physical examination revealed a swelling in the coccygeal region with multiple fistulous openings discharging gelatinous fluid. The initial clinical diagnosis was that of an infected pilonidal cyst. The patient underwent surgical excision. Histological analysis and immunohistochemistry confirmed the diagnosis of mucinous carcinoma. A comprehensive workup including endoscopy, colonoscopy, and computed tomography ruled out other primary sites, thereby confirming a diagnosis of primary cutaneous mucinous carcinoma of the intergluteal cleft. DISCUSSION: This case underlines the diagnostic challenges of mucinous carcinoma in atypical locations, where clinical features may mimic benign conditions. Differentiation from secondary lesions is crucial, given the significant implications for prognosis and treatment. CONCLUSION: Primary cutaneous mucinous carcinoma of the intergluteal cleft is exceptionally rare. Complete surgical excision with clear margins and vigilant follow-up are imperative to manage the high risk of local recurrence. This report significantly broadens the spectrum of primary mucinous carcinoma presentations and underscores the need for comprehensive evaluation.