Peripheral palisading with tumor-stroma retraction
• Trichoblastoma
Tumor lobule surrounded by fibroblastic stroma; not thick basement membrane
Classic Cylindroma Cylindroma is typically a dermal-based, well-circumscribed but unencapsulated tumor without connections to the overlying dermis.
Superficial Portion of Cylindroma This low-power image of cylindroma demonstrates the jigsaw puzzle arrangement of the tumor lobules filling the dermis.
Cylindroma With Hyalinized Membranes The tumor lobules are surrounded by a dense, eosinophilic basement membrane and show intratumoral hyalinized globules .
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
a.k.a., familial cylindromatosis or turban tumor syndrome
Autosomal dominant
Predilection for women
Eccrine spiradenomas and trichoepitheliomas often present
CLINICAL ISSUES
Epidemiology
• Incidence
Common adnexal tumor of sweat glands
• Age
Usually early adulthood
• Sex
Marked female predominance (F:M = 9:1)
Site
• 90% present on head and neck; 60% on scalp
Presentation
• Dermal mass
Treatment
• Surgical approaches
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
• Usually located in upper dermis
• No attachments to epidermis
• Circumscribed but not encapsulated
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and show intratumoral hyalinized globules 
.
