Large cell neuroendocrine carcinoma presenting with neck swelling in the submandibular gland: a case report

Abstract

IntroductionLarge cell neuroendocrine carcinoma in the salivary glands is rare. We report a second case of large cell neuroendocrine carcinoma of the submandibular gland diagnosed at autopsy, and a review of the literature.Case presentationA 68-year-old Japanese man was referred to our hospital for thorough investigation of swelling on the right side of his neck. Fine-needle aspiration cytology of the cervical mass suggested poorly differentiated metastatic carcinoma. The primary tumor could not be detected by several examinations. One month after admission, he died of cancer. An autopsy was performed, and it revealed a tumor of the right submandibular gland. The histopathological diagnosis was large cell neuroendocrine carcinoma of the submandibular gland.ConclusionTo the best of our knowledge, only eight cases of large cell neuroendocrine carcinoma in the salivary glands, including our case, have been reported. This report indicates total biopsy and immunohistochemistry are necessary for diagnosing large cell neuroendocrine carcinoma properly.

Neuroendocrine Neoplasms of the Head and Neck: Some Suggestions for the New WHO Classification of Head and Neck Tumors

“…In their review of 1,675 salivary gland tumors, 2 neoplasms were reclassified as LC-NEC. After these report, others have recognized rare LC-NEC in salivary gland highlighting the benefits of using a standard classification for neuroendocrine carcinomas [20,26].…”

“…Kasafuka et al [17] in a series of 8 mucosal LC-NEC reported that 7 of 8 (80 %) patients presented with regional lymph node metastases and 3 had died of disease after follow-up ranging from 15 to 90 months. Three of 4 (75 %) patients with well documented LC-NEC of the salivary glands have died of their disease [14,20,26]. necrosis''.…”

“…It is difficult to make conclusive statements regarding the clinical behavior of NEC grade 1 given their rarity and paucity of good prognostic data. Age at presentation has ranged from 23 to 71 years [7,23,26] with most patients being alive with no recurrent disease after follow-up periods ranging from 18 to 124 months [7,22,23,27].…”

Case Report of a Primary Parotid Large Cell Neuroendocrine Carcinoma

“…LCNEC account for less than 1% of all salivary gland tumors. Kawartani, et al [5] reported only eight such cases in a PubMed review of the last 40-years [5]. Most large cell neuroendocrine carcinomas contain the major salivary glands, and especially the parotid gland.…”

Large cell neuroendocrine carcinoma of the head and neck: a distinct clinicopathologic entity

“…the parotid gland is the second most common site [5,11,12,25]. lCNCs are increasingly being recognized in other sites and have been reported in sinonasal tract [9,26], oral cavity [27], oropharynx [8,28], hypopharynx [8], and submandibular gland [10,29]. Given the limitations of the current WHO classification, which does not make a distinction between moderately differentiated neuroendocrine carcinoma and lCNC, a reliable estimate of the true incidence of lCNC is not possible.…”