Primary (spontaneous) endometriosis of the umbilical ring

Endometriosis is currently regarded as one of the most prevalent pathologies of the female reproductive system, exerting a significant impact on women’s quality of life and reproductive health, and frequently serving as a major cause of female infertility. Despite extensive research, the etiology of the disease remains incompletely understood. Primary umbilical ring endometriosis represents an exceptionally rare variant of extragenital endometriosis, with an estimated prevalence of approximately 0.5–1% in the female population. The clinical presentation typically includes a painful nodular mass in the umbilical region and cyclic pain syndrome correlated with menstrual phases. Due to its rarity, this pathology may present diagnostic challenges and lead to delays in management. Furthermore, its manifestations may mimic other umbilical masses, thereby necessitating thorough differential diagnosis. Histopathological examination remains the gold standard for definitive diagnosis, allowing for reliable identification of endometrial tissue. The treatment of choice is surgical excision, with the extent of resection determined by individual patient characteristics, medical history, clinical findings, and future reproductive plans. In cases where surgical intervention is contraindicated or not feasible, medical management may be employed to control symptoms, most commonly continuous progestin therapy. Herein, we present a clinical case of a rare coexistence of primary umbilical ring endometriosis with uterine endometriosis (adenomyosis) in a reproductive-age patient, underscoring the diagnostic complexity of this condition and highlighting the importance of a personalized approach to its management.

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