[Cylindrocarcinoma in a patient with Brooke-Spiegler syndrome].
BACKGROUND
Cutaneous cylindromas are benign adnexal tumors that may occur as a solitary lesion or in a multiple familiar pattern. Malignant change is rare. The association of multiple trichoepitheliomas and cylindromas, the so called Brooke-Spiegler Syndrome, is supposed to be inherited in an autosomal dominant manner.


METHODS
A 55 year old woman presented with multiple skin tumors on the scalp, as well as the nasolabial and periauricular area since the age of 20 years. Her daughter and her nephew were also affected. Tumors were surgically removed for cosmetic reasons and showed histological signs of benign cylindromas and trichoepitheliomas. 35 years after diagnosis patient presented with a large exulceration on the right occipital area with intracranial invasion. Staging revealed multiple metastases in both lungs. Patient died 1 month later.


RESULTS
Histological examination showed beside zones of massive necrosis multiple mitosis. The jigsaw puzzle pattern and the thick PAS positive basal layer, the striking characteristics of benign cylindromas, were lost. These histological findings and the clinical presentation led to the diagnosis of cylindrocarcinoma.


CONCLUSIONS
Cylindrocarcinoma is an aggressive tumor with tendency to a local destructive growth and metastases. As malignant transformation of a benign cylindroma occurs more often in the multiple form a close follow-up of patients with multiple cylindromas is necessary. A family study is indicated.
Völter
Christiane
C
Klinik und Poliklinik für Hals-, Nasen- und Ohrenkranke der Universität Würzburg, Germany. c.voelter@mail.uni-wuerzburg.de
Baier
G
G
Schwager
K
K
Müller
J G
JG
Rose
C
C
D003528:Carcinoma, Adenoid Cystic / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D062109:Dermatologic Surgical Procedures; D003937:Diagnosis, Differential; D004428:Ear Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery; D004431:Ear, External / Q000473:pathology / Q000601:surgery; D005153:Facial Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D008938:Mitosis / Q000502:physiology; D009336:Necrosis; D009364:Neoplasm Recurrence, Local / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D012535:Scalp / Q000601:surgery; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery