Neuroendocrine Carcinoma Case Study

Neuroendocrine carcinoma of tongue : a case report with review of literature

INTRODUCTION

Neuroendocrine carcinomas (NECs) are rare in the oral cavity, especially in the tongue. There is ambiguity with respect to the classification and treatment modality of these tumors. To the best of our knowledge, only 10 cases regarding to the NECs in tongue have been reported in English literature. We present a case of Neuroendocrine tumor of the tongue and the related literature was reviewed with the purpose of providing certain reference for the diagnosis and treatment of such rare entities.

CASE PRESENTATION

A 38 year old Chinese man initially presented to our department with one month history of a non-tender, firm lump and odynophagia in the left

In WHO classification (2005), the laryngeal NECs are divided into 5 types: typical carcinoma, atypical carcinoma, small cell carcinoma, combined cell carcinoma and paraganglioma. However, along with the emerging NECs in the head and neck, the limitations of this classification are arising gradually. In some cases, NEC coexist with another carcinoma , or other carcinomas show neuroendocrine features, but there is no definition to distinguish between combined carcinoma, so someone in these cases could not be diagnosed as NECs according to 2005 classification, even if they still have an aggressive behavior and poor prognosis[13]. Therefore, the head and neck pathology community has reached the consensus that this classification does not adapt to the clinicopathological spectrum of NECs in the head and neck. A new classification of NECs proposed by Xu et al[14] is represented extensively, in which head and neck NECs are comprised of 5 groups, grade1, grade2, grade3 (large cell type), grade3 (small cell type), and combined neuroendocrine carcinoma with non-neuroendocrine carcinoma. In present case, it is supposed to be classified as a NEC combined with squamous cell carcinoma (SCC) due to the squamous differentiation in

But due to the paucity of reported cases, there is a definitive lack of standard protocol for the management of this type of tumor. Surgery is still the mainstream in the treatment of NECs, and the radical surgery with a wide excision margin has been advocated since the aggressive nature of this rare tumor. It has been reported that in a lingual NEC case, a surgical margin of 20 mm resulted in microscopic clearance of only 11.7mm at mucosal margin and 7mm deep margin.[7] It has been documented that surgery notably improves overall survival over other single modality treatments in NECs[18]. However, in of head and neck region, radical surgery is frequently not feasible due to poor general condition of patient, already existed disseminated disease , or some other reason.. In such cases, therapeutic radiotherapy and chemotherapy are recommended . Owing to the poorly differentiated status, most of the lingual NECs cases are sensitive to radiation treatment. Among the eleven cases, only three patients did not undergo radiotherapy, and the overwhelming majority of received cases demonstrated an excellent initial response with complete or partial resolution of the tumor. Nonetheless, the complication commonly appears with radiation should not be neglected, such as mucositis, necessitating a PEG tube, and neutropenia. Review articles of NECs in head and neck