title: Primary small cell neuroendocrine carcinoma of the tonsil: a case report and review of the literature.
abstract: Small cell neuroendocrine carcinoma (NEC) that originates in the tonsil is extremely rare and carries a poor prognosis. Only a few cases of this tumor have been reported so far and the standard treatment protocol remains uncertain. Here we describe a 74-year-old woman presented with throat pain for about 2 months. Computed tomography (CT) scan revealed a 3.4×1.8 cm tumor with moderate enhancement in the left tonsil and a 1.3×1.0 cm neck mass in left level II. A biopsy of the tonsillar mass was performed and histologic examination revealed small round to oval tumor cells were arranged in cords or nests, containing hyperchromatic nuclei and scant cytoplasm. Mitotic figures were readily identified. Immunohistochemical staining showed that tumor cells were strongly positive for CD56, focally positive for PCK and negative for LCA. A diagnosis of primary small cell NEC of the left tonsil was obtained. The patient was treated by six cycles of cisplatin combined with etoposide and the masses showed initial complete response. But recurrence in the left neck was found 9 months after initial diagnosis and the patient refused any further treatment. With a review of the literature, the nomenclature, clinicopathological characteristics and treatment modalities of this rare tumor are discussed.
authors: Department of Otolaryngology-Head and Neck Surgery, West China Hospital of Sichuan University Chengdu, China.
authors: Hai-Yang
authors: HY
authors: Wang
authors: Department of Otolaryngology-Head and Neck Surgery, West China Hospital of Sichuan University Chengdu, China.
authors: Jian
authors: J
authors: Zou
authors: Department of Otolaryngology-Head and Neck Surgery, West China Hospital of Sichuan University Chengdu, China.
authors: Guang-Yao
authors: GY
authors: Zhou
authors: Department of Pathology, West China Hospital of Sichuan University Chengdu, China.
authors: Jia-Qi
authors: JQ
authors: Yan
authors: Department of Otolaryngology-Head and Neck Surgery, West China Hospital of Sichuan University Chengdu, China.
authors: Shi-Xi
authors: SX
authors: Liu
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D018278:Carcinoma, Neuroendocrine / Q000737:chemistry / Q000188:drug therapy / Q000473:pathology*; D018288:Carcinoma, Small Cell / Q000737:chemistry / Q000188:drug therapy / Q000473:pathology*; D002945:Cisplatin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D008940:Mitotic Index; D009364:Neoplasm Recurrence, Local; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014067:Tonsillar Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000473:pathology*; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D014408:Biomarkers, Tumor; D005047:Etoposide; D002945:Cisplatin
keywords: Neuroendocrine carcinoma; immunohistochemistry; oropharynx; small cell carcinoma; tonsil