Poncet–Spiegler's cylindroma

Poncet–Spiegler's cylindroma is a rare type of skin tumor that predominantly affects the scalp and face. It is characterized by the development of nodular lesions that can be solitary or multiple. When multiple, these tumors can coalesce to form a larger mass, often described as resembling a "turban," hence the alternative name "turban tumor." Poncet–Spiegler's cylindroma is considered a benign condition, but it can cause significant disfigurement and discomfort to affected individuals.

Etiology and Pathogenesis

The exact cause of Poncet–Spiegler's cylindroma is not fully understood, but it is believed to have a genetic component. Mutations in the CYLD gene, located on chromosome 16q, have been identified in familial cases of the condition. This gene is thought to act as a tumor suppressor, and its mutation leads to uncontrolled cell growth. Poncet–Spiegler's cylindroma can occur sporadically or as part of an inherited syndrome known as Brooke–Spiegler syndrome, which is characterized by the development of multiple skin adnexal tumors including cylindromas, trichoepitheliomas, and spiradenomas.

Clinical Features

Poncet–Spiegler's cylindromas typically present as slow-growing, painless, pink to red nodules on the scalp and face. The size of the nodules can vary significantly, and when numerous, they can merge to form a large, lobulated mass. Although these tumors are benign, their location and potential for significant growth can lead to psychological distress and physical discomfort, particularly when located on the scalp, where they can interfere with hair grooming.

Diagnosis

The diagnosis of Poncet–Spiegler's cylindroma is primarily based on clinical examination and histopathological analysis of the tumor. Biopsy of the lesion reveals characteristic features such as islands of basaloid cells surrounded by a dense hyaline sheath, which is diagnostic of cylindroma.

Treatment

Treatment options for Poncet–Spiegler's cylindroma depend on the size, number, and location of the tumors. Small, solitary lesions can be surgically excised with good cosmetic results. For multiple lesions, especially those forming a "turban tumor," more extensive surgical procedures may be required, and reconstructive surgery may be necessary to address the cosmetic deformities resulting from tumor removal. In some cases, laser therapy and radiotherapy have been used as alternative treatment modalities, but their efficacy varies.

Prognosis

The prognosis for individuals with Poncet–Spiegler's cylindroma is generally good, as these tumors are benign and have a low risk of malignant transformation. However, the potential for recurrence after surgical removal is a concern, and regular follow-up is recommended to monitor for new or recurring lesions.

Poncet–Spiegler's cylindroma Resources
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