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Pathology of Median Raphe Cyst

Dr Sampurna Roy MD 2022

Median raphe cyst is a very rare, benign congenital lesion occurring mainly on the ventral aspect of the penis, but can develop anywhere in the midline between the external urethral meatus and anus.

It is most common near the glans penis.

They are thought to arise as a result of defective embryological closure of the median raphe, but some may result from the anomalous outgrowth of the entodermal urethral lining (urethroid cyst).

Median raphe cyst may be formed as a result of local trauma or secondary infection.

Gross presentation:

These cysts are usually less than 1 cm in diameter.

The contents are usually clear, but they may be turbid if there is abundant mucous glands in the wall.

Histopathology:

Median raphe cysts are usually located in the dermis.

These lesions do not connect with the overlying surface epithelium.

The cysts are lined by pseudostratified columnar epithelium.

In some areas they may be quite attenuated.

Occasionally, mucous glands are present in the wall, but ciliated cells are rare.

Pigmented cysts, resulting from the presence of melanocytes, have been reported.

In cysts situated near the meatus, the lining is usually stratified squamous epithelium.

Dermatopathology Case 108

Median raphe cyst. Report of two cases and literature review.

Median raphe cysts of the perineum in children.

Ciliated median raphe cyst of perineum presenting as perianal polyp: a case report with immunohistochemical study, review of literature, and pathogenesis.

Immunohistochemical characterization of a median raphe cyst of the penis.