endothelioma cutis; epithelioma cyindromatosus nevus; Spiegler tumors
HistoryThis section has been translated automatically.
Ancell, 1842; Spiegler, 1899
DefinitionThis section has been translated automatically.
A tumor disease occurring as a solitary disease or inherited as familial multiple cylindromatosis with benign, eccrine (and apocrine) differentiated adnexal tumors in the head area, which originate from poorly differentiated, pilo-glandular structures of the skin appendages.
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EtiopathogenesisThis section has been translated automatically.
Autosomal-dominantly inherited mutations of the gene CYLD1, which is mapped on gene locus 16q12-13. Consecutively, disorders of the cytoskeleton-associated protein glycine (CAP-GLY) occur.
ManifestationThis section has been translated automatically.
Predominantly occurring in childhood, but also in adolescents and adults.
LocalizationThis section has been translated automatically.
Mainly capillitium, neck, neck, face, rarely trunk and extremities.
Clinical featuresThis section has been translated automatically.
- Solitary or multiple, 0,5 to 2,0 cm large, skin-coloured or light red, bulging, protuberant tumours with a smooth, shiny surface. Rare tendency to ulceration of the surface.
- The Brooke-Spiegler syndrome must be distinguished from the solitary cylindromes. In this case multiple cylindromes may occur in combination with other adnexal tumors. The cylinder tumours are unusually large (3-5 cm), bulging, protuberant, flesh-coloured tumours (so-called turbo-tumours), most of which are localised on the head and which can overgrow the entire capillitium. Ulcerations are possible. Clinically important: Tumours of the salivary glands and possibly the kidneys may also be present.
HistologyThis section has been translated automatically.
Sharply defined, roundish or elongated basaloid cell complexes that fill the entire dermis and the adjacent areas of the subcutis. The cell nests are surrounded by a broad PAS-positive hyaline membrane. Drop-shaped, hyalinized material also between the epithelial nests. The cell nests consist of marginal basaloid elements in palisade position and central larger cells with lighter, somewhat irregular nuclei. Tubular structures possible.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Possibly excision, larger excisions require plastic surgery.
Progression/forecastThis section has been translated automatically.
Favorable prognosis. Rarely malignant degeneration is recognizable (mainly by a sudden growth spurt, ulceration or bleeding).
LiteratureThis section has been translated automatically.
- Regalado JJ et al (2003) A novel epidermal nevus syndrome with congenital cylindromatous turban tumor. J Cutan pathogen 30: 586-590
- Saunders H et al (2003) Pedigree of multiple benign adnexal tumours of Brooke-Spiegler type. Australas J Dermatol 44: 144-148
- Durani BK et al (2001) Malignant transformation of multiple dermal cylindromas. Br J Dermatol 145: 653-656
- Leonard N et al (2001) Loss of heterozygosity at cylindromatosis gene locus, CYLD, in sporadic skin adnexal tumours. J Clin Pathol 54: 689-692
- Schittkowski M et al (2002) Nasolacrimal duct obstruction caused by a cylindroma. Report of an atypical tumor manifestation and review of the literature. Clin Monatsbl Ophthalmology 2196: 602-608
Incoming links (16)Adenoma sebaceum; Brooke-spiegler syndrome; Cyld; Endothelioma cutis; Endotheliomatosis; Epithelioma cyindromatosus nevus; Hyperplasia angiolymphoids with eosinophilia; Multiple Trichoepithelioma ; Sebaceous epithelioma; Spiegler tumors; ... Show all
Outgoing links (6)Atheroma; Basal cell carcinoma (overview); Brooke-spiegler syndrome; Cytoskeleton; Excision; Neurofibromatosis (overview);
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