The incidence of mesenchymal tumours in the salivary gland range from 0.3% to 1%. 1 Adamantinoma-like Ewing sarcoma (ALES) is an unusual variant of Ewing sarcoma with more incidences in the head and neck of young patients, but scarce in the salivary gland. 2 The closest differential is sialoblastoma, also known synonymously as congenital basal cell adenoma/embryoma, another rare tumour of the salivary gland that almost exclusively arises from the parotid gland 3 in infants. ALES exhibits distinct is this meant to be epithelial? morphology and nesting pattern and shows translocation involving EWSR and FLI1, 4,5 like conventional Ewing sarcoma. ALES has a relatively higher incidence in the head and neck region of about 5% with unique morphology recapitulating epithelial differentiation with the formation of squamous pearls, peripheral palisading and a diffuse cytokeratin as well as p40 positivity. 2,4 This tumour encompasses cells with basaloid morphology reminiscent of salivary anlage structures 6 and has an array of morphological patterns with characteristic immunohistochemistry. There are rare reports available on cytomorphological features of ALES. 7 We report an unusual case of ALES of the parotid gland in a 12-year-old, masquerading as sialoblastoma. | C A S E REP ORT | Clinical featuresA 12-year-old girl presented to the clinic with complaints of preauricular swelling for 2 months. Clinical examination revealed a 4 × 3 cm firm, non-tender swelling with well-defined borders, and restricted mobility. Haematological and biochemical investigations were mostly within normal limits. Fine needle aspiration (FNA) was performed with a radiological possibility of pleomorphic adenoma. | MicroscopyAspirate smears were highly cellular with nests and sheets of tumour cells demonstrating peritheliomatous pattern at places. These cells showed epithelioid morphology with moderate atypia, round to ovoid nuclei, finely granular chromatin, and occasional prominent nucleoli. Vague rosetting was also appreciated focally ( Figure 1A-E). As per the Milan System, the morphology fitted in category VI, malignant tumour with epithelioid morphology. Certain unique morphological features, such as a nesting of small round blue cells with epithelioid morphology posed a diagnostic challenge in categorising the lesion. Therefore, on FNA cytology, two possible differentials were suggested; sialoblastoma and Ewing sarcoma/primitive neuroectodermal tumour. Subsequently, superficial parotidectomy with right cervical lymph node excision was done. The specimen was well encapsulated; no macroscopic extracapsular extension was visualised. Cut section revealed a whitish, relatively circumscribed tumour that was replacing the entire salivary gland with patchy areas of congestion reaching up to the capsule ( Figure 1F). Macroscopically, the tumour had replaced the whole of the salivary gland with no visible healthy salivary gland tissue. Microscopic sections, however, displayed rim of normal salivary gland tissue at the periphery, with an ill circumscr...
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