Chondroid syringoma
represents the cutaneous counterpart of mixed tumor ("pleomorphic
adenoma") of salivary glands, therefore it is also termed 'mixed tumour of
the skin'. The cutaneous lesion has different behaviour in that it rarely
recurs even if inadequately excised.
It is generally accepted that there are both eccrine and apocrine variants
of mixed tumour of skin.
Age: Usually occurs in middle aged and elderly patients.
Common
sites: Head and neck region and rarely in the distal extremities.
Clinical presentation:
The tumour presents as a solitary, slow growing nodule (0.5 - 3cm in
diameter).
Microscopic features:
Well
circumscribed tumour located in the dermis and subcutaneous tissue ;
No epidermal connection with the overlying epidermis.
;
Tumour consists of epithelial component set in a chondroid, myxoid and
fibrous stroma ; There are epithelial nests, islands, ducts and
tubular structures ;
Tubules or lumina may
be- i) Large, multilayered, or complex structures ii) Small
relatively round tubules or simple tubules.
Focal areas of apocrine
secretion may be present ; In rare cases there are intracytoplasmic lumina of eccrine type; Some cases show
follicular and sebaceous differentiation (shadow cells, other elements
of hair follicles and sebocytes) ;
Other features
include: Keratinous cyst formation ;
Presence of eosinophilic globules (collagenous spherulosis);
Islands of squamous epithelium ; Areas of ossification.
Immunohistochemistry:
Epithelial cells are CEA and cytokeratin positive;
The
outer layer of ductal cells (myoepithelial cells) are Vimentin and S100
positive.
Hyaline cell-rich chondroid syringoma
:-
Histopathological features:
Lobulated neoplasm composed
of hyaline cells with plasmacytoid features showing ovoid nuclei, with
occasional invaginations, finely granular chromatin, and discrete
nucleoli; the cytoplasm is deeply eosinophilic with occasional
dot-shaped paranuclear hyaline inclusions ;
Hyaline
cells might possess an aberrant myoepithelial differentiation;
Immunohistochemistry: Hyaline cells are strongly and diffusely positive for S-100
protein, vimentin, pan and high molecular weight cytokeratins. Cells are
focally positive for GFAP, neuron-specific enolase, and cytokeratin 14.
Differential diagnosis:
Malignant
melanoma and extra-skeletal myxoid-chondrosarcoma.
Atypical mixed tumours:-
Borderline features of malignancy
characterized by an infiltrative margin, satellite tumour nodules, and
tumour necrosis
. These tumours do not metastasize.
Malignant
chondroid syringoma:- A few cases have been
reported; More common in women ; Occurs most often in trunk and extremities
; May metastasize to both
the regional and distant lymph nodes, causing the death of the patient.
In these cases, radiation therapy follows the surgical excision.
Microscopic features: Lobulated appearance ;
Composed of epithelial and mesenchyme-like component (myxomatous and
cartilaginous areas) ; Epithelial component predominates at the periphery of the tumour; Mesenchymal component is at the
center; Scattered mitoses ; Variable pleomorphism.
Immunohistochemistry: Positive staining for cytokeratin,
S-100 protein, neuron- specific enolase and glial fibrillary acidic
protein.
Visit:
Salivary gland-type mixed
tumours of the
Lung
;Pleomorphic
Adenoma of the External Ear
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