Dermal cylindroma

from Wikipedia, the free encyclopedia
Classification according to ICD-O-3
8200/0 Cylindroma of the skin
ICD-O-3 first revision online

The dermal cylindroma (Spiegler tumor) is a rare benign tumor of the skin appendages that is usually observed in the head or neck area. A distinction is made between a sporadic, in middle to late age, mostly occurring in the form of single tumors, and a rare familial, autosomal dominant hereditary form, in which multiple tumors often manifest themselves in early adulthood (familial cylindromatosis). Cylindromas can rarely also occur in the form of large, areally confluent tumors on the forehead and hairy scalp (so-called turban tumors). The tumor tissue shows features of both an apocrine and an eccrineDifferentiation, so that a primitive sweat gland precursor cell is discussed as the starting cell, which still has the ability to develop in the direction of both lines of differentiation. Associations with other skin tumors such as spiradenoma or trichoepithelioma ( Brooke-Spiegler syndrome ) are described. A malignant degeneration of cylindromas has been proven in individual cases.

Epidemiology

The dermal cylindroma is considered a rare tumor entity, although reliable statistical information on the frequency has so far been lacking. Women are affected significantly more often than men (6–9: 1). The age peak is usually in middle to later life, although hereditary forms often manifest in early adulthood. Regional or ethnic differences in frequency have not yet been reported.

etiology

Cylindromas usually develop sporadically, whereby neither the triggering causes nor risk factors are known. In the case of sporadic cylindromas, genetic analyzes identified mutations on and in the vicinity of the CYLD gene locus ( chromosome 16q12-q13). The familial form is also associated with mutations in the CYLD gene, the exact function of which is currently unknown.

pathology

Histological picture of a dermal cylindroma.

Cylindromas appear macroscopically as solid nodes a few millimeters to several centimeters in size, pink, red, or sometimes blue in color and rubbery in consistency. Preferred localizations of the tumors are the head and neck, with a manifestation in the area of ​​the trunk and extremities in particular in the context of the familial form.

Histologically, there is a dermal tumor with no direct relationship to the epidermis , which consists of numerous oval and polygonal cell nests in a puzzle-like arrangement. These cell nests are surrounded by broad hyaline membranes, clearly protruding in the PAS staining, which are mainly composed of type IV collagen and thus show a similarity to basement membranes . The cell nests show a characteristic structure in a peripheral, palisade-like layered layer of basophilic cells and in the center of large, rather pale cell elements. Small tubular lumens are sometimes found both outside and inside the tumor cell islands. Malignant cylindromas differ from the benign form in that they have considerable cell and nuclear polymorphism.

clinic

Depending on the size and extent of the findings, the therapeutic procedure is preceded by a diagnostic sampling ( biopsy ) followed by a tissue examination by the pathologist. The treatment of choice for the treatment of solitary cylindromas is surgical resection. Small cylindromas can be removed using a carbon dioxide laser. Multiple cylindromas sometimes require extensive plastic surgery, whereby a multi-stage approach can be useful.

forecast

Patients with multiple cylindromas require clinical follow-up as they show a tendency to develop further cylindromas. In the case of the rare malignant degeneration, which has so far only been described in a few cases, organ metastases often develop with a correspondingly poor prognosis. A rare complication that has been described in both benign and malignant cylindromas is the erosion of the skull bone and even tumor invasion of the skull cavity .

Web link

Individual evidence

  1. ↑ AL Gerretsen, SC van der Putte, W Deenstra, WA van Vloten: Cutaneous cylindroma with malignant transformation . In: Cancer , Sep 1 1993; 72 (5): 1618-23.
  2. ↑ PY Lin, SM Fatteh, KM Lloyd: Malignant transformation in a solitary dermal cylindroma . In: Arch Pathol Lab Med , Aug 1987; 111 (8): 765-7.
  3. ↑ a b H Kerl, C Garbe, L Cerroni, HH Wolff: Histopathology of the skin . Springer, 2003, ISBN 3-540-41901-2 .
  4. ↑ a b c d e f NS Scheinfeld, A Mokashi, JT Celebi, AR Oppenheim: Cylindroma (March 13, 2008); medscape.com
  5. ^ MF Stoner, ER Hobbs: Treatment of multiple dermal cylindromas with the carbon dioxide laser . In: Journal of Dermatologic Surgery and Oncology . 1988 Nov; 14 (11): 1263-7. PMID 2846665
  6. ^ L Wyld, S Bullen, FS Browning: Transcranial erosion of a benign dermal cylindroma . In: Ann Plast Surg . 1996 Feb; 36 (2): 194-6. PMID 8919387