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Review
. 2018 Mar;12(1):75-81.
doi: 10.1007/s12105-017-0828-8. Epub 2017 Jun 8.

Multiple Desmoplastic Cellular Neurothekeomas in Child: Report of the First Oral Case and Review of the Literature

Tatiana Fernandes Araújo Almeida  1 , Flaviana Dornela Verli  2 , Cássio Roberto Rocha Dos Santos  1 , Saulo Gabriel Moreira Falci  1 , Luciana Yamamoto Almeida  3 , Lana Kei Yamamoto Almeida  4 , Ana Terezinha Marques Mesquita  5 , Jorge Esquiche León  6
Affiliations
Review

Multiple Desmoplastic Cellular Neurothekeomas in Child: Report of the First Oral Case and Review of the Literature

Tatiana Fernandes Araújo Almeida et al. Head Neck Pathol. 2018 Mar.

Abstract

Cellular neurothekeomas (CNs) are distinctive benign tumors of uncertain histogenesis, with predilection for the skin of the head and neck region. We describe the first case of multiple desmoplastic CNs (DCNs) affecting the oral cavity in a 9-year-old girl. Histopathologic evaluation showed a proliferation of spindle and epithelioid cells, forming nests and bundles, supported by exuberant fibrous stroma, as well as scattered multinucleated floret-like giant cells. The tumor cells were immunopositive for vimentin, CD63, CD56, whereas AE1/AE3, S100, CD34, α-SMA, GFAP, EMA, CD57 and NSE were negative. Ki-67 was <2%. Multiple DCNs should be considered in the differential diagnosis of oral nodular lesions.

Keywords: Cellular neurothekeoma; Child; Desmoplastic cellular neurothekeoma; Immunohistochemistry; Oral cavity.

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Figures

Fig. 1
Fig. 1
Clinical features of the desmoplastic cellular neurothekeoma affecting the buccal mucosa and upper lip of the left side in a child patient
Fig. 2
Fig. 2
a Diffuse proliferation of spindle cells supported by exuberant connective tissue stroma (H&E, ×10). Notice sparse multinucleated floret-like giant cells (inset, ×40). b The tumor cells permeate bundles of skeletal muscle and adipose tissue (H&E ×10). c In high-power view, visualize epithelioid cells with granular eosinophilic cytoplasm forming nests adjacent to neurovascular bundle (H&E, ×40). d The cytoarchitectural organization and desmoplastic stroma are highlighted by Masson’s Trichrome (×40)
Fig. 3
Fig. 3
Immunohistochemical analysis of desmoplastic cellular neurothekeoma. a The tumor cells were positive for vimentin and, b CD63 (NKI-C3). c S100 (nerves serve as a positive internal control), d CD34 (endothelial cells serve as a positive internal control) and e α-SMA (smooth muscle cells serve as a positive internal control), were negative. f The Ki-67 labelling index was <2% (IHQ, ×40). Notice the cytoarchitectural organization of the tumor cells (nests and bundles) highlighted in all photomicrographs
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