Microsecretory adenocarcinoma of the skin, a novel type of sweat gland carcinoma: Report of three additional cases

Abstract

Microsecretory adenocarcinoma (MSA) is a newly described salivary gland tumor harboring a characteristic balanced chromosomal translocation resulting in MEF2C:: SS18 gene fusion. Six primary cutaneous MSA cases have been recently described.We report three additional cases confirming the relevance of this recently identified entity of primary cutaneous adnexal tumor. Three patients aged 53-, 64-and 78-year-old were retrospectively diagnosed with MSA of the skin (MSAS) as consultation cases of the CARADERM (CAncers RAres DERMatologiques) national network.

Metachronous primary secretory carcinomas of the eyelid and the parotid gland

“…11 As PCSC exhibits ETV6-NTRK3 gene fusion and microsecretory adenocarcinoma MEF2C-SS18 gene fusion, molecular biology is very helpful in their differential diagnosis. 12 We did not find previously reported multicentric PCSC or SC of the salivary glands, although we found some rare multicentric mammary SC, 13 nor any underlying genetic predisposition.…”

“…Finally, the recently described cutaneous microsecretory adenocarcinoma, characterized by an MEF2C::SS18 fusion, presents microcystic morphology, basophilic secretions, and uniform cells that are embedded in a fibromyxoid stroma 11 . As PCSC exhibits ETV6–NTRK3 gene fusion and microsecretory adenocarcinoma MEF2C–SS18 gene fusion, molecular biology is very helpful in their differential diagnosis 12 …”

Microsecretory adenocarcinoma of the hard palate: a case report and literature review

“…MSA usually occurs in the oral cavity, especially in the minor salivary glands; however, identifying MSA in an extraoral location is not rare. The primary tumour location was in the skin, which expands the sites that can be affected by MSA, including the nose, chin, posterior scalp [ 11 ], external auditory canal [ 7 , 11 ], forearm, fourth finger of the hand and vertex [ 13 ]. These cutaneous tumours are identical to those described in the salivary glands, with unique histopathologic features and molecular underpinnings.…”

“…These cutaneous tumours are identical to those described in the salivary glands, with unique histopathologic features and molecular underpinnings. Bogiatzi et al [ 13 ] suggested that MSA of the skin (MSAS) is a skin homologue of the salivary gland. The MSA is composed of tubules, microcysts and cord structures with monotonous bland cells and sometimes presents with focal infiltration and perineural invasion.…”

“…et al [ 6 ] n = 24 M:11 F:13 17–83 Buccal Palate Retromolar trigone Parotid gland Angle of mandible 0.6–3.0 NED (1–216) Dibbern, M.E. et.al [ 7 ] n = 1 F 89 External auditory canal N.A N.A Jurmeister, P., et al [ 8 ] n = 1 M 61 Buccal N.A NED (144) He XJ, et al [ 9 ] n = 1 M 27 Buccal 2 NED (11) Gui, H., et al [ 10 ] n = 1 M 70 Palate 5.3 NED (20) Bishop, J.A., et al [ 11 ] n = 4 M:4 61–74 Nose Chin Posterior scalp External auditory canal 0.5 NED (4–6) An FX, et al [ 12 ] n = 1 M 48 Palate 1.5 N.A Bogiatzi, S. et al [ 13 ] n = 3 M:1 F:2 53...…”