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Soft Tissue Pathology 

Pathology of Granular Cell Tumour      

Dr Sampurna Roy MD             

                                                                                                                      

 

 

The granular cell tumor was first described by Abrikossoff in 1926 ( also known as Abrikosoff’s tumours).

This first report was of a granular cell tumor of the tongue.

According to some authors this tumour is probably of Schwann cell origin.

Most granular cell tumours are benign, yet malignant forms have been sporadically reported. Only 10 to 15 % of granular cell tumours are multicentric.

Age: Usually occurs in middle aged adults (40-50 years)

Site: It is most common in the tongue, followed by the anterior and posterior chest wall and the upper limbs.

The tumour also occur on the head and neck region and in the larynx, stomach, vulva and anogenital region.

 [ Visit related post: Gingival Granular Cell Tumour of Newborn (Congenital Epulis) ]
           
Clinical presentation: The lesion is common in blacks and presents as a solitary yellow-tan nodule in the dermis or subcutis and less frequently in the submucosa, smooth muscle or striated muscle.

Microscopic features:  

Granular cell tumour is a nonencapsulated tumour with a poorly defined  margin.

The tumour is arranged in large sheets or in nests and may extend into the subcutis.

It is composed of large polygonal cells with coarse eosinophilic cytoplasm and central hyperchromatic nucleus and occasional eosinophilic droplets.

The cells are PAS positive and diastase resistant.

Nests of tumour cells may surround dermal appendages and may be present within the epineurium of nerve fibres.

Elastosis is common in the backround stroma. 

Some cases are associated with marked acanthosis or pseudoepitheliomatous hyperplasia of the overlying squamous epithelium.

 

Immunohistochemistry:  The tumour cells show  positivity with S100 protein, neuron-specific enolase, vimentin, PGP9.5  and melanoma-associated antigen (NK1/C-3). 

The pan-macrophage marker, CD68 (Kp1) reacts strongly with granular cell tumour.

Features indicating malignancy:

Tumour size more than 5  cms, presence of vascular invasion, necrosis and rapid growth.

Other features include brisk mitotic activity(>2/10 high-power fields), pleomorphism, presence of spindling of cells and angiogenesis.

Three or more of these features are required for a malignant diagnosis. 

If fewer than 3 are present, the tumour is considered to be “atypical granular cell tumor” and typically has a good prognosis if fully excised.

If metastatic lesions are found in bone, lymph nodes, peripheral nerves, peritoneal cavity or lungs - the prognosis is usually very poor.

Further reading:

Granular cell tumor: a review of the pathology

Malignant granular cell tumor: a clinicopathologic analysis of 10 cases with review of literature. 

Malignant granular cell tumor of soft tissues: a study of two new cases.

Malignant granular cell tumor of soft tissue: diagnostic criteria

Cellular heterogeneity of granular cell tumours: a clue to their nature?

Malignant granular cell tumor of soft tissue: diagnostic criteria and clinico pathologic correlation.

A Malignant Granular Cell Tumor Excised with Mohs

Granular cell tumor presenting as a tongue nodule

Granular cell tumors.

Immunoreactivity of S100 protein, alpha-1-antitrypsin, and CD68 in adult and congenital granular cell tumors.

Expression of calretinin and the alpha-subunit of inhibin in granular cell tumors.

Expression of PGP 9.5 in granular cell nerve sheath tumors: an immunohistochemical  study of six cases.

Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation.

CD68 and factor XIIIa expressions in granular-cell tumor of the skin.

 

Variants:

1) Gingival Granular Cell Tumour of Newborn (Congenital Epulis)

2) Primitive Polypoid Granular Cell Tumour: 

Syn: "Dermal non-neural granular cell tumor" ; "Primitive nonneural granular cell tumors of skin"

Primitive nonneural granular cell tumor of the skin was first described by LeBoit et al in 1991 as "primitive polypoid granular cell tumor".

The tumours are composed of elongated  spindle-shaped to polygonal or round cells with prominent granular cell change.

The tumour nuclei may show mild focal atypia to moderate atypia.

There is brisk mitotic activity. The cells do not stain for S100 protein.

These tumours are different from classic granular cell tumor by lacking neural differentiation.

 

 

Further reading:

Desmoplastic primitive nonneural granular cell tumor of the skin.

Primitive polypoid granular cell tumor.

Polypoid S-100-negative granular cell tumor of the oral cavity: a case report and review  of literature.

Primitive nonneural granular cell tumor (so-called atypical polypoid granular cell tumor). Report of 2 cases with immunohistochemical and ultrastructural correlation.

Primitive nonneural granular cell tumors of skin: clinicopathologic analysis of 13 cases.

Dermal non-neural granular cell tumour (so-called primitive polypoid granular cell tumour): a distinctive entity further delineated in a clinicopathological study of 11 cases.

Primitive polypoid granular-cell tumor and other cutaneous granular-cell neoplasms of apparent nonneural origin. 

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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