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       Path Quiz Case-84: Diagnosis-    

                    Microcystic Adnexal Carcinoma

       Dr  Sampurna Roy  MD

 

 Path Quiz Case 84: Case history and images:

     DermPath-India

     Site created by

 Dr  Sampurna Roy MD

          

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

January 2010   

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 Paget’s disease

Intermediate grade:

Porocarcinoma

Digital papillary adenocarcinoma

High grade:

Hidradenocarcinoma

Apocrine adenocarcinoma

Eccrine ductal carcinoma

Spiradenocarcinoma

Malignant cylindroma

Sebaceous tumours

Fordyce's Spots

Steatocystoma

Nevus Sebaceous

Folliculosebaceous Cystic Hamartoma

Sebaceous Hyperplasia

Sebaceoma

Sebaceous Adenoma

Sebaceous Carcinoma

Lichen planus-like lesions

Lichen Nitidus

Bullous Pemphigoid

Hailey-Hailey Disease 

Granuloma Annulare

Necrobiosis Lipoidica

Necrobiotic Xanthogranuloma   

Rheumatoid Nodule

Foreign body granuloma

Cutaneous Pseudolymphoma

Drug related cutaneous lesions

Epidermal tumours:

Epidermal Naevus ; Inflammatory linear verrucous epidermal nevus

Prurigo Nodularis

Acanthomas  

Clear cell acanthoma

Large cell acanthoma

Warty Dyskeratoma

Seborrheic Keratosis

Verruca vulgaris

Keratoacanthoma

Actinic Keratosis

Bowen's disease

Basal Cell Carcinoma

Squamous Cell Carcinoma

Verruciform Xanthoma

Cutaneous Infections and Infestations

   1 : Bacterial, Rickettsial and Chlamydial Infections

   2 : Spirochetal Infections

   3 : Mycoses and algal Infections

   4 : Protozoal Infections

   5 : Helminth Infections

   6 : Viral Infections

                   

Syn:  Pilomatricoma ; Calcifying epithelioma of Malherbe

Pilomatrixoma is a very common benign adnexal tumour with matrical differentiation.
Both ultrastructurally and histochemically these are similar to hair matrix.

Dermatopathology Case No 23 (Images)

Most cases are associated with beta-catenin gene mutations.

Clinical presentation:   Solitary and rarely multiple rubbery nodules (usually 5mm to 2cm in diameter). The tumour usually occurs in the first two decades of life.  Often diagnosed as epidermoid/pilar cysts.  May be associated with Gardner's syndrome or myotonic dystrophy- (multiple lesions).

Site: Located on head, neck or forehead .

Microscopic features:

Image Link1 ; Image Link2 ; Image Link3 ; Image Link4 .

DermAtlas-clinical image link (JHU).

Well circumscribed lobular sometimes cystic tumours ; Composed of proliferating basaloid (resemble matrical cells of the follicular bulb) and eosinophilic shadow keratinised cells (represent attempted cortical and inner root sheath differentiation) ; Dystrophic calcification and giant cell reaction to keratin and shadow cells  ;  sometimes osseous metaplasia;  cystic pilomatrixoma shows more basaloid cells ;  solid lesions are usually devoid of basaloid cells and there are more shadow cells, calcification and giant cell reaction.

Proliferating Pilomatrixoma:

A distinctive proliferative variant of pilomatrixoma ; 

This is a relatively large lesion in the dermis which sometimes extend to the  subcutis ;

Composed of lobular proliferation of basaloid cells in association with adjacent focal areas containing eosinophilic, cornified material with shadow cells ;  in some cases there are relatively large areas of shadow cells whereas, in others there are only small foci of shadows cells ; basaloid cells  show variable nuclear atypia and mitotic figures ;

Architectural pattern of the neoplasms is different from that of a large fully developed pilomatrixoma  with a cystic character and basaloid cells predominantly aligned at the periphery. 

Differential diagnosis: Matricoma, basal-cell carcinoma with matrical differentiation, and matrical carcinoma (pilomatrical carcinoma).

Pilomatrix Carcinoma: Dermatopathology Case No 29 (Images)                                   

Pilomatrix carcinoma is a neoplasm of low-grade malignancy.

The architectural features are those of a malignant neoplasm - asymmetry and poor circumscription, presence of several markedly sized and variably shaped basaloid aggregations, and ulceration.

Composed of pleomorphic basaloid cells with prominent nucleoli and frequent atypical mitoses accompanied by central areas with keratotic material, shadow cells, and foci of necrosis.

Tumour nests are surrounded by a desmoplastic stroma ;

There is infiltration into the adjacent tissues.

Differential diagnosis:   Should be distinguished from the-  Conventional pilomatrixoma and its variant  proliferating pilomatrixoma ; Matricoma ;  Basal cell carcinoma with matrical differentiation.

Clinicians and pathologists  should be aware of the occurrence of pilomatrix carcinoma because of its potential for distant metastases. Malignant pilomatrixoma carries a high risk of metastases to the bones, lungs, and lymph nodes.

Surgical wide resection is the recommended treatment.  

[ Summary of findings of Pilomatrix carcinoma :  Larger in size, shows cytological pleomorphism , infiltrative growth pattern and potential to metastasize.]  
            

               

Abstracts:

Aggressive pilomatrixoma of the infra-auricular area: a case report.Auris Nasus Larynx. 2005 ;32(4):407-10. Epub 2005

Basal cell carcinoma with matrical differentiation in a transplant patient: a case report and review of the literature.J Cutan Pathol. 2005;32(6):445-8.

Pilomatrix carcinoma with lymph node metastases.J Cutan Pathol. 2004;31(4):330-5.

Pilomatricoma with a bullous appearance.J Cutan Pathol. 2004;31(8):558-60.

Bullous appearance of pilomatricoma.Dermatol Surg. 2003 ;29(10):1066-7.

Pilomatrix carcinoma: histologic and immunohistochemical features. Two studies. Ann Pathol. 2003 Feb;23(1):50-4.

Low frequency of beta-catenin gene mutations in pilomatricoma.Acta Derm Venereol. 2002;82(6):428-31.

Pilomatrix carcinoma with lymph node and pulmonary metastasis: report of a case arising on the knee. Am J Dermatopathol. 2002;24(2):139-43.

Pilomatrix carcinoma: a clinicopathologic study of six cases and review of the literature.Am J Dermatopathol. 2001;23(5) :394-401.

Pilomatrix carcinoma: case report and review of literature.Pathology. 2001 May;33(2):248-51.

Bullous pilomatricoma: a report of clinical and pathologic findings and review of dermal bullous disorders.J Cutan Med Surg. 2001;5(5):394-6.

Pilomatricoma associated with several hair follicles.Am J Dermatopathol. 1999;21(5):458-61.

Proliferating pilomatricoma. A histopathologic simulator of matrical carcinoma.J Cutan Pathol. 1997;24(4):228-34.

Morphological stages of pilomatricoma. Am J Dermatopathol. 1996;18 (4):333-8.

Multiple pilomatricoma. Apropos of a case. J Fr Ophtalmol. 1996;19(6-7):464-6.

 

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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)

Myxoid Tumours of Soft Tissue

Classification of Soft Tissue Tumour

Gross examination of soft tissue specimen          

A practical approach to histopathological reporting of soft tissue tumours

Grading of soft tissue tumours

Lipomatous tumours

Neural tumours

Myogenic tumours

Fibroblastic/Myofibroblastic tumours

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Fibrohistiocytic tumours

ChondroOsseous tumours

Soft TissueTumours of Uncertain Differentiation               

Notochordal Tumour - Chordoma

Extra-adrenal Paraganglioma

Gastrointestinal Stromal Tumour

Melanocytic tumours

Acquired Melanocytic Naevus

Ancient Naevus

Halo naevus

Balloon cell naevus

Mongolian Spots /Ota's naevus /Ito's naevus

Blue naevus-variants

Deep penetrating naevus  

Combined Naevus

Recurrent naevus

Spitz naevus

Dysplastic naevus

Congenital naevus


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