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Pathology of Cylindroma               

Dr Sampurna Roy MD

 
Path Quiz Case 94: Case history and images:

Dermatopathology Case 15 

Diagnosis: Cylindroma

Skin Appendage Tumour

     

http://www.   histopathology-india.net/SAClass.htm

March  20142009
Benign sweat gland tumours

Cysts:

Hydrocystoma -Eccrine/Apocrine

Hamartomas:

Eccrine/Apocrine naevus

Eccrine Angiomatous Hamartoma

Porokeratotic eccrine ostial naevus

Benign Neoplasms:

Syringocystadenoma Papilliferum

Hidradenoma Papilliferum

Nipple Adenoma

Syringoma

Spiradenoma

Cylindroma

Chondroid  Syringoma (benign mixed tumour)

Poroma

Hidradenoma

Apocrine adenoma

Papillary eccrine adenoma

Syringofibroadenoma

Malignant sweat  gland tumour 

Low grade:

Microcystic adnexal carcinoma

Eccrine epithelioma

Adenoid cystic carcinoma

Mucinous carcinoma

Primary extramammary Pagetís disease

Intermediate grade:

Porocarcinoma

Digital papillary adenocarcinoma

High grade:

Hidradenocarcinoma

Apocrine adenocarcinoma

Eccrine ductal carcinoma

Spiradenocarcinoma

Malignant cylindroma

Cylindroma of the External Ear

Histology of the Normal Skin

Glossary 

Gross examination of  the skin specimen

Reporting of biopsies taken for Inflammatory Skin Diseases

Lichenoid (Interface)Tissue Reaction Pattern

Psoriasiform Reaction Pattern

Vesiculobullous Reaction Pattern

Spongiotic Reaction Pattern

Vascular Diseases: Various forms of Vasculitis

Lichen planus-like  lesions

Lichen Nitidus

Bullous Pemphigoid

Dermatitis Herpetiformis

Hailey-Hailey Disease 

Granulomatous Reaction Pattern of the Skin

Granuloma Annulare

Necrobiosis Lipoidica

Necrobiotic Xanthogranuloma   

Rheumatoid Nodule

Lupus Vulgaris

Cutaneous Sarcoidosis

Melkersson Rosenthal Syndrome

Annular Elastolytic Giant Cell Granuloma

Skin lesion in Crohn's Disease

Blastomycosis-like pyoderma

Foreign body granuloma

Rosacea

Interstitial  Granulomatous  Dermatitis

Interstitial  Granulomatous Drug Reaction

                  

Cylindroma is a benign adnexal tumour.

The tumour showed apocrine and trichoepitheliomatous differentiation which indicated complex hair follicle (folliculo-sebaceous-apocrine). According to recent studies immunohistochemical examination has shown myoepithelial, apocrine, eccrine, ductal, and secretory features in both cylindromas and spiradenomas.

Brooke-Spiegler syndrome represents an autosomal dominant disease characterized by the occurrence of multiple cylindromas, trichoepitheliomas and (sporadically) spiroadenomas.

Clinical presentation: 

Clinically, the tumour usually presents as a solitary, slow growing rubbery lesion.

The multiple form is autosomal dominantly inherited on cyld1 gene on chromosome 16.

This form is also known as 'turban tumour' and may be associated with spiradenoma.

Some of these lesions are painful.

This condition is associated with trichoepitheliomas and milia.

Site:  Usually located on the scalp, head and neck or trunk.

Microscopic features: 

 

 

Histologically cylindroma is a circumscribed, non-encapsulated , dermal tumour without attachment to the epidermis.

The lesion is composed of numerous oval and polygonal nests arranged in an interlocking 'jigsaw-like' pattern.

There are two cell types, the peripheral cells are small and basophilic and central cells are larger and pale stained.

Small ductal lumina may be present.

Thick PAS positive hyaline bands surround tumour islands.

Hyaline droplets may be present within the nests.

Differential Diagnosis:

Spiradenoma - Lack of lymphoid tissue in cylindroma and presence of variable number of dendritic Langerhans cells in the tumour. These cells are CD1a positive.

Malignant Cylindroma:

Cylindromas occasionally undergo malignant transformation.

Malignant cylindromas are characterised by islands of cells displaying marked nuclear pleomorphism.

There is increase in mitosis and many abnormal forms are identified.

The tumour shows invasion into the surrounding tissue and loss of the delicate hyaline sheath.

                            

Further reading:

Identification of a large rearrangement in CYLD as a cause of familial cylindromatosis.

Expression of p53 and TP53 mutational analysis in malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma, sporadic or associated with Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma and eccrine spiradenoma.

Blaschkoid distribution of cylindromas in a germline CYLD mutation carrier.

Malign cylindroma of the scalp with multiple cervical lymph node metastasis-A case report.

Cylindroma of head and neck: review of the literature and report of two rare cases.

Transition from cylindroma to spiradenoma in CYLD-defective tumours is associated with reduced DKK2 expression.

Brooke-Spiegler syndrome: report of two cases not associated with a mutation in the CYLD and PTCH tumor-suppressor genes.

Five novel germline function-impairing mutations of CYLD in Italian patients with multiple cylindromas.

Malignant cylindroma of the scalp arising in a setting of multiple cylindromatosis: a case report.

Defective Laminin 5 Processing in Cylindroma Cells.American Journal of Pathology. 2002;160:459-468.

Linkage and LOH studies in 19 cylindromatosis families show no evidence of genetic heterogeneity and refine the CYLD locus on chromosome 16q12-q13.Hum Genet. 2000;106(1):58-65.

Spiradenocylindromas of the skin: Tumors with morphological features of spiradenoma and cylindroma in the same lesion: Report of 12 cases.Pathology International, Volume 49, Number 5, 1999, pp. 419-425

Spiradenoma and Dermal Cylindroma: Comparative Immunohistochemical Analysis and Histogenetic Considerations . American Journal of Dermatopathology . 1997;19(2):154-161.

Cutaneous Adnexal Carcinoma Arising within a Solitary Cylindroma-Spiradenoma. American Journal of Dermatopathology.1996; 18(1):77-82, 

Familial cutaneous cylindromas: investigations in five generations of a family.J Am Acad Dermatol. 1995;33(2 Pt 1):199-206

Familial cylindromatosis (turban tumour syndrome) gene localised to chromosome 16q12-q13: evidence for its role as a tumour suppressor gene.Nat Genet. 1995;11(4):441-3.

Dermal cylindroma An immunohistochemical study of thirteen cases. Am J Dermatopathol .1995 ;17: 260-265.

Cutaneous cylindroma with malignant transformation. Cancer 1993 ; 72: 1618-1623.

Cylindroma expresses immunohistochemical markers linking it to eccrine coil. J Cutan Pathol 1993 ; 20: 40-43.

Malignant dermal cylindroma in a patient with multiple dermal cylindromas, trichoepitheliomas, and bilateral dermal analogue tumors of the parotid gland.Am J Dermatopathol. 1989;11(4):353-9.

Dermal eccrine cylindroma.Ann Otol Rhinol Laryngol.1989 ;98 (12 Pt 1):991-2.

Metamorphosis of dermal cylindroma: possible relation to malignant transformation. Case report of cutaneous cylindroma with direct intracranial invasion.J Am Acad Dermatol. 1985;12(1 Pt 2):188-95

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Consultant  Histopathologist (Kolkata - India)

 

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Tumours of the Hair Follicle

Hair Germ  Differentiation:

Trichoepithelioma 

Desmoplastic  Trichoepithelioma

Trichofolliculoma

Trichoblastoma

Cutaneous lymphadenoma

               
               
Infundibular differentiation:

Trichoadenoma

Dilated Pore of Winer

Pilar Sheath Acanthoma

Tumour of Follicular Infundibulum

Outer root sheath differentiation:
                    
Trichilemmoma

Trichilemmal Carcinoma

Proliferating Trichilemmal Cyst (Pilar Tumour)

Matrical differentiation:
                  
Pilomatrixoma and Pilomatrix Carcinoma

 

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