Dermal cylindroma

The dermal cylindroma ( Spiegler 's tumor) is a rare benign tumor of the skin appendages, which is usually observed in the range of head or neck. Distinction is a sporadic, mostly occurring in the form of individual tumors in the middle to late age and a rare familial autosomal dominant hereditary form in which multiple tumors often manifest themselves already in early adulthood (family cylindromatosis ). Cylindromas can rarely occur in the form of large, areally confluent tumors on the forehead and hairy scalp (so-called turban tumor). The tumor tissue shows both features of an apocrine as well as eccrine differentiation of a, so that the starting cell is a primitive sweat gland progenitor cell is discussed, which still has the ability to develop in the direction of both lines of differentiation. Associations with other skin appendage tumors such as Spiradenomen or Trichoepitheliomen ( Brooke- Spiegler syndrome) are described. Malignant degeneration of Zylindromen is occupied in individual cases.

Epidemiology

The dermal cylindroma is considered a rare tumor entity, with reliable statistical data are available on the frequency, however, so far. Women are significantly more likely than men ( 6-9: 1). The peak age is usually in the mid to later life, but with manifest hereditary forms often already in early adulthood. Regional or ethnic frequency differences have not been reported.

Etiology

Cylindromas arise mostly sporadic, neither the causes nor triggering risk factors are known. Genetic analyzes were able to identify in the case of sporadic mutations cylindromas on and in vicinity of the CYLD gene locus (chromosome 16q12 - q13 ). The familial form is also associated with mutations in the CYLD gene, whose precise function is currently unknown.

Pathology

Cylindromas appear macroscopically as a few millimeters to several centimeters in size, firm knots of pink, red or sometimes blue in color and rubbery consistency. Preferred locations of the tumors are head and neck, particularly in the context of the familial form sometimes a manifestation in the area of the trunk and extremities is observed.

Histology shows a dermal tumor with no direct relationship to the epidermis, which is composed of numerous oval and polygonal cell nests in puzzle - like arrangement. Umschieden these cell nests of wide, in the PAS staining clearly prominent hyaline membranes mainly composed of collagen type IV and thus show a similarity to basement membranes. The cell nests show a characteristic structure in a peripheral palisade- like layered cell sheet basophilic and in the center of large, rather pale stained cellular elements. Both outside and within the tumor cell islands are found sometimes small tubular lumina. Malignant cylindromas differ from the benign form by a significant cellular and nuclear pleomorphism.

Clinic

Depending on the size and extent of the lesion is the therapeutic approach to a diagnostic sampling (biopsy) go ahead with subsequent histologic examination by a pathologist. Treatment of choice in the treatment of solitary cylindromas is surgical resection. Small cylindromas may be removed by using carbon dioxide laser. Multiple cylindromas sometimes require extensive plastic surgery, with a more -step procedure may be useful.

Forecast

Patients with multiple Zylindromen require clinical follow-up, because they have a tendency to form another cylindromas. In the case of rare, previously described only in a few cases, malignant transformation often leads to the formation of organ metastases with a poorer prognosis. A rare, described both in benign as malignant Zylindromen complication is the erosion of the skull bone to tumor invasion of the cranial cavity.

Weblink

• NS Scheinfeld, A Mokashi, JT Celebi, AR Oppenheim: cylindroma. March 13, 2008; emedicine.medscape.com