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Diagnosis
of Path Case19: Granular cell tumour |
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February
2009
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Granular cell tumour is a benign tumour of disputed histogenesis. Some authors support a Schwann cell origin. 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 & anogenital region. Visit: Granular Cell Tumour of Bronchus 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.
Microscopical features:
Image 1
;
Image2
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Image3
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Image4.
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. Variants: Gingival granular cell tumour of newborn (congenital epulis): Congenital gingival granular cell tumours are rare benign tumours of uncertain histogenesis. Site: Alveolar ridge of the maxilla in newborns . Sex: Striking predilection for female infants suggests the presence of an endogenous hormonal stimulus in utero. Microscopic features: Image 1 ; Image2 ; Image3 ; Image4 Like adult granular cell tumour this lesion is composed of polygonal cells with abundant granular cytoplasm and small eccentric nuclei with occasional small nucleoli. There is a prominent vascular stroma with perivascular lymphocytes and histiocytes. Entrapped non-neoplastic odontogenic epithelium may be present in some cases. There is no pseudoepitheliomatous hyperplasia of the overlying squamous mucosa and no nerve bundles are seen within these lesions. The tumour is negative when immunostained with S100 protein, in contrast to adult granular cell tumours. Differential diagnosis: Teratoma, leiomyoma, congenital dermoid cyst, congenital fibrosarcoma, and congenital lipoma.
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