Cylindroma Clinical Presentation

Updated: Nov 08, 2019
  • Author: Angela (Angel) M Crotty, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

The solitary form usually begins in middle age or later as a slow-growing, rubbery nodule exhibiting no symptoms.

The dominantly inherited, multiple variety appears shortly after puberty as numerous, rounded nodules of various sizes ranging from several millimeters to larger than 6 cm. Lesions grow slowly, and additional lesions develop over time.

Loss of CYLD can be linked with development of salivary gland cancers. [11, 32]

Brooke-Spiegler syndrome (BSS) associated with unilateral hearing loss has been reported. [33] BSS manifesting with pegged teeth has also been reported. [34]

 

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Physical Examination

Except for BSS, pertinent findings are largely limited to the skin. Histologically similar tumors have been found in the breast, parotid glands, salivary glands, lacrimal gland of the eye, Bartholin glands, the brain, lungs, and kidneys. An interesting case of cylindroma was reported on the breast in 2013. [35, 36]

Skin lesions associated with cylindromas

Solitary lesions are firm, rubbery nodules with pink, red, or sometimes blue coloring that range in size from a few millimeters to several centimeters. Larger solitary growths should not be necessarily ruled out though, because one 7-cm stalked cylindroma with a mushroomlike shape has been observed. [37]

The multiple form has numerous masses of pink, red, or blue nodules, sometimes resembling bunches of grapes or small tomatoes (sometimes called a tomato tumor). One case study of a patient with BSS has reported a plaque with flatter cylindroma rather nodular appearance. [38]

Dermal cylindromas are noted, some which can be in the deep dermis. [39]

Skin distribution of cylindromas

The solitary form is typically found on the head and neck. The multiple form most commonly occurs on the head and neck but can also be seen on the trunk and the extremities. [38]

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Complications

A cylindroma occasionally erodes through the skull, after the mass has caused thinning of the neighboring bone. [40]

Anal and rectal spiradenocylindroma-like basaloid carcinomas have been reported, with CYLD gene mutations defined. [41]

Scattered reports exist of malignant transformation of isolated cylindromas, so this must be a consideration for regular follow-up for patients with cylindromas. [40, 42, 43] This includes possible transformation into basal cell carcinoma. [2]

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