Background

Cylindromas are rare benign adnexal tumors that arise primarily on the scalp. They occur at any age but usually appear in early adulthood favoring females. There are 2 distinct clinical forms: a sporadic solitary form, and a multiple form, which is dominantly inherited. The lesions are pink-red, nodular, firm, and usually painless, and vary from several millimeters to more than 6 cm in diameter. The tumors grow slowly in size and number throughout life. OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
AGE RANGE-MEDIAN	Early adulthood
SEX (M:F)	Females favored

 

PATHOGENESIS	CHARACTERIZATION
Adnexal cell of uncertain origin	Am J Dermatopathol. 1995;17:271-280 By electron microscopy, the majority of the tumor cells appear indeterminate, or show partial eccrine or apocrine diffentiation Suggests an adnexal progenitor cell as a possible source

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
BREAST
Cylindroma (Dermal Analog Tumor) of the Breast A Comparison With Cylindroma of the Skin and Adenoid Cystic Carcinoma of the Breast Jorge Albores-Saavedra, MD, etal.	Am J Clin Pathol 2005;123:866-873 Abstract quote We compared 4 breast cylindromas with 50 dermal cylindromas and 8 adenoid cystic breast carcinomas. Except for a modest increase in the number of eccrine ducts and reactive Langerhans cells in dermal cylindromas, breast and dermal cylindromas showed identical histologic and immunohistochemical features. Both were characterized by epithelial islands containing central basaloid cells and peripheral myoepithelial cells surrounded by a thickened, continuous, periodic acid–Schiff–positive basement membrane that was immunoreactive for collagen IV. Clusters of sebaceous cells and a few eccrine ducts are described in breast cylindromas. Cytokeratin 7 labeled predominantly the central basaloid cells, and smooth muscle actin stained peripheral myoepithelial cells in breast and dermal cylindromas. Eccrine ducts were highlighted by epithelial membrane antigen and carcinoembryonic antigen. S-100 protein and CD1a showed a variable number of dendritic Langerhans cells. Cylindromas of the breast and skin did not express cytokeratin 20, gross cystic disease fluid protein 15, or estrogen or progesterone receptor. Breast cylindroma might be confused with the solid variant of adenoid cystic carcinoma, especially in needle core biopsy specimens, because they share nodular and trabecular patterns, basaloid cells, myoepithelial cells, eccrine ducts, and hyaline globules of basement membrane material. However, adenoid cystic carcinoma displays an infiltrative growth pattern, cytologic atypia, and mitotic figures and lacks the continuous, thickened basement membrane.
TURBAN TUMORS	Autosomal dominant disorder with tumors covering the entire scalp

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Basaloid cells in the dermis that were arranged in a lobular pattern with a few ducts Cellular islands were usually surrounded by a thick hyaline sheath Occasionally, the same hyaline substance was seen in the lobules as small intercellular droplets No perineural or vascular invasion
VARIANTS
Malignant transformation	Cancer. 1993;72:1618-1623 Features that are suggestive of malignancy include disturbance of the jigsaw pattern, loss of hyaline sheaths, an infiltrative pattern, large cell predominance, nuclear pleomorphism and anaplasia, and frequent mitoses
Carcinosarcoma arising in a patient with multiple cylindromas. De Francesco V, Frattasio A, Pillon B, Stinco G, Scott CA, Trotter D, Patrone P. Institute of Dermatology, Department of Clinical and Experimental Pathology and Medicine, University School of Medicine, Udine, Italy.	Am J Dermatopathol. 2005 Feb;27(1):21-6. Abstract quote   Familial cylindromatosis (Brooke-Spiegler syndrome) is a rare autosomal dominant inherited disease characterized by the development of adnexal tumors, mostly cylindromas, but also trichoepitheliomas and spiradenomas. Malignant tumors may occur, usually with the features of a cylindrocarcinoma. The authors describe the case of a 75-year-old woman with the Brooke-Spiegler syndrome who presented with multiple nodules of the scalp, face, and trunk. In 1997 she underwent surgical excision of the entire forehead and scalp with skin grafting. Histologic examination revealed multiple cylindromas, some with areas of spiradenoma and one with an extensive adenomatous component; some trichoepitheliomas were also evident. In 2002, a nodule of the trunk suddenly increased in size and became painful. The lesion was excised and histologic and immunohistochemical evaluation revealed a malignant cutaneous biphasic tumor extending into the subcutis, consisting of a major portion with the features of an adnexal carcinoma and of a minor one of atypical spindle cells. Biphasic malignant skin tumors are rare and only a limited number have been described, none in association with the Brooke-Spiegler syndrome. The authors discuss the morphogenesis of the folliculosebaceous-apocrine unit from which the tumors in this syndrome derive, and the pivotal role of mesenchymal cells in determining the process. Since the Brooke-Spiegler syndrome is characterized by a germline mutation in the CYLD oncosuppressor gene, a biphasic tumor in this setting may represent a true carcinosarcoma.
Solitary Cylindroma (Dermal Analog Tumor) of the Breast A Previously Undescribed Neoplasm at This Site S. Tunc Gokaslan, M.D.; Brian Carlile, M.D; M. Dudak, M.D.; Jorge Albores–Saavedra, M.D. From the Department of Pathology, Division of Anatomic Pathology (S.T.G., B.C., J.A.-S.), and Department of Surgery (M.D.), The University of Texas Southwestern Medical Center, Dallas, Texas, U.S.A.	Am J Surg Pathol 2001;25:823-826 Abstract quote The authors report a previously undescribed small, well-demarcated breast tumor similar to a dermal cylindroma in a 63-year-old woman. The tumor was an incidental finding in a lumpectomy specimen for infiltrating lobular carcinoma. The cylindroma was surrounded by normal-appearing breast parenchyma and had the typical ``jigsaw'' pattern of epithelial basaloid islands. The islands showed focal squamous and myoepithelial differentiation. A notable number of reactive dendritic Langerhans cells permeated the epithelial cell islands, a feature considered to be characteristic of dermal cylindroma. There was also ductal differentiation. Thick bands of hyaline periodic acid–Schiff (PAS) stain and collagen IV-positive basement membrane material bordered the cell islands, and PAS–collagen IV-positive hyaline globules were seen within the cell islands. There was no nuclear pleomorphism or mitotic figures. The cylindroma did not express gross cystic disease fluid protein 15, carcinoembryonic antigen, estrogen and progesterone receptors, or cytokeratin 20 (CK20). There was diffuse and strong immunoreactivity to CK AE1/AE3, and focal reactivity for CK7 and smooth muscle actin. Cylindroma of the breast should be distinguished from adenoid cystic carcinoma and basal cell carcinoma. Although clearly epithelial, the exact histogenesis and cell phenotype of this unusual dermal type cylindroma of the breast are unknown.

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Treatment	Total excision When the lesions are too extensive and diffuse for a complete resection, surgery is primarily for cosmetic purposes Patients should be followed up for any possible recurrence or for malignant transformation

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Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated June 7, 2005

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