NUT Carcinoma Arising from the Parotid Gland: A Case Report and Review of the Literature
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CASE REPORTS
NUT Carcinoma Arising from the Parotid Gland: A Case Report and Review of the Literature Wei‑Ning Saik1 · Philip Da Forno2 · Khin Thway3 · Syed Ali Khurram1,3 Received: 14 September 2020 / Accepted: 17 November 2020 © The Author(s) 2020
Abstract NUT carcinoma is an aggressive carcinoma with an overall poor survival outcome. The mediastinum and head and neck area, especially the sinonasal region, are among the common sites of disease. Histopathological diagnosis of NUT carcinoma is often very challenging due to its overlapping features with other poorly differentiated carcinomas. We report a case of NUT carcinoma arising from the parotid gland of a young female patient. Primary NUT carcinoma of salivary gland is very rare, with only 15 such cases reported in the literature to date. Our case highlights the diagnostic challenges associated with such lesions. Keywords NUT carcinoma · BRD4-NUT · Parotid gland · Salivary gland · Poorly differentiated carcinoma · Head and neck
Introduction
Case History
NUT carcinoma is a rare, poorly differentiated carcinoma characterised by a genomic rearrangement of the nuclear protein of testis (NUT) gene, with BRD4-NUT being the most common fusion variant. It generally arises from the mediastinum, but numerous cases involving the head and neck, mainly from the sinonasal region, have been reported [1–5]. NUT carcinoma has a poor prognosis, with a mean survival of less than 12 months from diagnosis. People of all ages are equally affected, with no gender predilection [6–9]. Patients normally present with advanced lesions, typically of a rapidly expanding mass at the site involved [6]. There are no effective treatment regimens available to date.
A 34-year-old 6-week post-partum female with no significant medical history presented with a short-term history of a left parotid lump. Magnetic resonance imaging (MRI) and positron emission tomography (PET) imaging identified a solitary well-defined, 38 mm lesion within the left parotid gland (Fig. 1). The initial core biopsy was suggestive of a high-grade neuroendocrine carcinoma. Following this, the patient underwent a radical parotidectomy and ipsilateral selective neck dissection. The histology of the left parotidectomy was reported locally as a high-grade salivary gland neoplasm, possibly high-grade mucoepidermoid carcinoma. There was evidence of perineural invasion as well as metastatic deposits in two lymph nodes. The patient underwent adjuvant radiotherapy; however, a new lump appeared one month later, at the left parotidectomy surgical site. MRI and Computerized tomography (CT) imaging demonstrated a recurrent lesion in the left parotid region, accompanied by widespread metastatic disease in the spine and skull base (Fig. 1). Following this recurrence, the pathology was reviewed at a tertiary specialist oral and maxillofacial pathology department. Histology showed a multilobular and widely infiltrative neoplasm largely with a ‘small round blue cell’ morphology, exhibiting largely cohesive sheets of undifferentiated
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