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HDAC Overexpression in a NUT Midline Carcinoma of the Parotid Gland with Exceptional Survival: A Case Report

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Abstract

NUT midline carcinoma (NMC) is a recently described entity with a predilection for young individuals, characterised by a rearrangement of NUT, most commonly with BRD4. It usually involves midline structures, with a minority of cases presenting outside the midline axis. Given its dismal prognosis, new molecularly targeted therapies (eg, HDAC inhibitors) are gaining ground, but the HDAC expression pattern remains unknown. We describe the exceptional evolution of a NMC arising in the parotid gland. A 34-year-old male presented with a rapidly growing 35 mm left-parotid mass. Parotidectomy and lymphadenectomy were performed. The tumour invaded the surrounding soft tissue and lay adjacent to the surgical margin. No lymph node metastases were identified. Histology revealed blue nests of undifferentiated cells merging with foci of necrosis and occasional abrupt foci of keratinising squamous epithelium. FISH analysis confirmed a rearrangement of NUT, but not of BRD4. A diagnosis of NMC was rendered. Currently, after adjuvant chemoradiotherapy and 47 months after diagnosis, the patient is alive and well. The tumour was found to have increased immunoexpression of HDAC2, 4 and 6 and phospho-HDAC4/5/7. This case emphasises the importance of considering NMC in the differential diagnosis of poorly differentiated carcinomas of the head and neck region in young adults, even away from midline structures. As molecular targets hold the promise of successful therapy for the vast majority of NMC patients, the knowledge of their HDAC expression patterns will probably be relevant.

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Author notes

  1. Gonçalo Esteves and Joana Ferreira contributed equally to the work.

Authors and Affiliations

  1. Anatomic Pathology Department, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Rua Prof. Lima Basto, 1099-023, Lisboa, Portugal

    Gonçalo Esteves, Joana Ferreira & Ana Félix

  2. NOVA Medical School, Lisbon, Portugal

    Gonçalo Esteves, Joana Ferreira, Rita Afonso & Ana Félix

  3. Molecular Pathology Research Unit (UIPM), Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon, Portugal

    Carmo Martins

  4. Department of Surgery of the Head and Neck, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon, Portugal

    Carlos Zagalo

  5. Egas Moniz Interdisciplinary Investigation Centre (CiiEM), Lisbon, Portugal

    Carlos Zagalo

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  1. Gonçalo Esteves
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  2. Joana Ferreira
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Correspondence to Gonçalo Esteves.

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Esteves, G., Ferreira, J., Afonso, R. et al. HDAC Overexpression in a NUT Midline Carcinoma of the Parotid Gland with Exceptional Survival: A Case Report. Head and Neck Pathol 14, 1117–1122 (2020). https://doi.org/10.1007/s12105-020-01130-6

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  • DOI: https://doi.org/10.1007/s12105-020-01130-6

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