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Revista Española de Cirugía Oral y Maxilofacial

On-line version ISSN 2173-9161Print version ISSN 1130-0558

Abstract

MERINO DOMINGO, Francisco et al. Mammary analogue secretory carcinoma: presentation of 2 cases and a review of its diagnosis and treatment. Rev Esp Cirug Oral y Maxilofac [online]. 2018, vol.40, n.1, pp.15-21. ISSN 2173-9161.  https://dx.doi.org/10.1016/j.maxilo.2017.01.004.

Introduction:

Mammary analogue secretory carcinoma (MASC) is a neoplastic tumour of the salivary glands, characterised by having important histological and immuno-histochemical similarities with secretory breast carcinoma. Until its description in 2010, MASC was often diagnosed as carcinoma of acinar cells due to their morphological overlapping. As there is a lack of evidence in the scientific literature concerning the optimal treatment of these tumours, the aim of this article is to present 2 recently treated cases, and review the described literature about their diagnosis and treatment.

Material and methods:

The first case concerns a 41 year-old male with MASC of the minor salivary gland of the hard palate, and the other a 56 year-old patient with the same diagnosis at the level of the submandibular gland.

Results:

Surgical resection with lesion-free margins was performed in both cases. Clinical surveillance was decided for one case with free margins, while in the other one, it was decided to give adjuvant therapy with radiotherapy due to the narrow lesion free margin. Both patients are disease free and continue on clinical follow-up.

Discussion:

The literature describes the existence of a salivary glands tumour with common morphological characteristics between acinar cell carcinoma and breast carcinoma, and immunohistochemically characterised by being positive to vimentin and S-100 protein, but it was not known as MASC until its description in 2010.

The genetic alteration associated with MASC is the presence of a translocation t(12;15) (q25; Q13) in ETV6-NTRK3, making it a pathognomonic tumour marker. There is consensus in the surgical treatment of primary lesions, but there is no agreement as regards neck dissection. The value of post-surgical radiotherapy is difficult to assess by the few cases in the literature.

Conclusions:

MASC is a recently described salivary gland tumour characterised by ETV6 translocation. Due to its behaviour as a low grade malignant neoplasm, it is recommended that its treatment should be excision with surgical margins, but in some cases its behaviour can be aggressive.

Keywords : Carcinoma análogo secretor de mama; Translocación ETV6; MASC.

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