Mullerian-Type Ciliated Cyst of the Thigh with PAX-8 and WT1 Positivity: A Case Report and Review of the Literature

Abstract

Mullerian-type ciliated cysts are uncommon lesions usually found in the lower extremities and perineal region of young females. They have however been reported in males and in other anatomic sites. The cyst lining is typically positive for estrogen receptor (ER), progesterone receptor (PR), PAX-8, and WT1 immunohistochemical stains. This staining pattern has led to the notion that these cysts are of Müllerian origin. The vast majority of cases are located in the dermis where the preferred nomenclature is cutaneous ciliated cyst (CCC). We report a case of Müllerian-type ciliated cyst in the thigh of a 16-year-old girl. Unlike most of the cases reported in the English literature, this cyst was not centered in the dermis. Only a few other cases of Müllerian-type ciliated cysts with no cutaneous connection have been reported. We propose the term ectopic Müllerian cyst for this rare subset of lesions that are not skin based as is the current case.

Benign paravertebral cyst of Hattori: A case report and review of literature

“…Extra genito-urinary tract or pelvic cysts of Mullerian origin are rare and have been reported mostly in the skin and the retroperitoneal cavity of female patients [4][5][6].…”

“…Following his first report, Hattori further undertook a study of the prevalence of estrogen and progesterone receptor expression in mediastinal cysts [3]. Extra genito-urinary tract or pelvic cysts of Mullerian origin are rare and have been reported mostly in the skin and the retroperitoneal cavity of female patients [4][5][6].…”

Posterior Mediastinal Paravertebral Müllerian Cyst (Cyst of Hattori): Literature Review

“…Paired box gene 8 (PAX-8); a gene found at 2p13, is associated with tumours of thyroid gland, kidney/upper urinary tract and Müllerian system [30,31] and is positive in COH [2,15,22,25,27]. Cancer antigen 125 (CA-125), a protein encoded by MUC16 gene, is used as a tumour marker with a 79% sensitive for ovarian cancer.…”

A Remarkably Rare Position of a Cutaneous Ciliated Cyst in a 16 Month-old Female

“…A cutaneous Mullerian cyst is an extremely rare, benign entity, frequently located on the lower extremities of young women after puberty, during the second and third decade of life ( 1 , 2 ). However, there are some published cases worldwide concerning men ( 2 , 3 ).…”

“…The entity was first described by Hess, in 1890 and the term Cutaneous ciliated cyst (CCC) was established by Farmer and Helwig, describing a unique type of cyst arising on the lower extremities of young women ( 1 ). CCCs, also known as cutaneous Müllerian cysts, are cystic lesions, mostly found on the lower extremities of females.…”