Cylindroma

 Can have areas indistinguishable from cylindroma


image Usually encapsulated

image Tumor stroma has prominent vasculature


• Malignant cylindroma/cylindrocarcinoma

• Basal cell carcinoma
image Peripheral palisading with tumor-stroma retraction

• Trichoblastoma
image Tumor lobule surrounded by fibroblastic stroma; not thick basement membrane



image
Classic Cylindroma
Cylindroma is typically a dermal-based, well-circumscribed but unencapsulated tumor without connections to the overlying dermis.


image
Superficial Portion of Cylindroma
This low-power image of cylindroma demonstrates the jigsaw puzzle arrangement of the tumor lobules filling the dermis.

image
Cylindroma With Hyalinized Membranes
The tumor lobules are surrounded by a dense, eosinophilic basement membrane image and show intratumoral hyalinized globules image.

image
Cylindroma at High Magnification
This higher power image demonstrates how the tumor lobules fit together in an irregular jigsaw puzzle pattern. Note the prominent basement membranes surrounding the tumor lobules that are characteristic of cylindroma.


TERMINOLOGY


Synonyms




• Dermal cylindroma

• Turban tumor (clinical term for multiple tumors on head)


Definitions




• Benign, dermal-based adnexal tumor with distinctive jigsaw puzzle pattern

• May show apocrine differentiation in most cases (formerly considered eccrine)


ETIOLOGY/PATHOGENESIS


Loss of Heterozygosity of Tumor Suppressor Gene CYLD




• Mutation of CYLD found in both sporadic and familial cases


Sporadic or Familial




• Familial cases associated with Brooke-Spiegler syndrome
image a.k.a., familial cylindromatosis or turban tumor syndrome

image Autosomal dominant

image Predilection for women

image Eccrine spiradenomas and trichoepitheliomas often present


CLINICAL ISSUES


Epidemiology




• Incidence
image Common adnexal tumor of sweat glands

• Age
image Usually early adulthood

• Sex
image Marked female predominance (F:M = 9:1)


Site




• 90% present on head and neck; 60% on scalp


Presentation




• Dermal mass


Treatment




• Surgical approaches
image Complete excision is curative


Prognosis




• Benign; may recur if incompletely excised

• Rare transformation to malignant cylindroma (cylindrocarcinoma), usually in large, longstanding tumors


MICROSCOPIC


Histologic Features




Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cylindroma
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