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Atypical carcinoid (moderately differentiated neuroendocrine carcinoma) of the uvula.


Carcinoid (neuroendocrine) tumors of the head and neck rarely occur outside the larynx and, until now, none has been reported in the oral cavity. We describe what we believe is the first reported case of an atypical carcinoid tumor, or any other type of neuroendocrine carcinoma, of the uvula.


The terms carcinoid and neuroendocrine carcinoma are frequently used to describe tumors of the peripheral APUD (amine precursor uptake and decarboxylation) system. Neuroendocrine carcinomas are subclassified as well differentiated, moderately differentiated, and poorly differentiated. (1) In the more surgically oriented literature, authors use the time-honored terminology to classify them as carcinoids, atypical carcinoids, and small-cell tumors or oat-cell tumors, respectively. (Because most otolaryngologists are more familiar with the older terminology, it will be used primarily in this report.)

Carcinoids are rare in the head and neck. When they do occur there, they are most common in the larynx. Since the first report of laryngeal involvement in 1969, (2) more than 500 cases have been described in the literature. To the best of our knowledge, no case of an atypical carcinoid or any other type of neuroendocrine carcinoma of the oral cavity has been previously reported in the literature. In this article, we report a case of atypical carcinoid of the uvula.

Case report

A 57-year-old man sought evaluation for a feeling that something had been stuck in his throat for the previous month. He continually tried to clear his throat, but he experienced no relief. He reported no dysphagia or odynophagia. His history was significant for exposure to Agent Orange during the Vietnam War.

Examination revealed a bosselated, nonulcerated, 2-cm mass of the uvula. A marked cervical adenopathy was present bilaterally. Findings on the remainder of the examination were normal. Biopsy of the mass was reported as a poorly differentiated carcinoma of unknown type, possibly neuroendocrine. A resection of the soft palate was performed along with a radical neck dissection on the right and a modified neck dissection on the left. The surgical margin of the palatal dissection was negative for tumor.

On gross examination, the tumor was a firm, 2-cm mass partially covered with a pink-tan mucosa. It had a firm, homogeneous cut surface with no areas of necrosis. Microscopic examination revealed tumor cells that contained large hyperchromatic nuclei with scant cytoplasm. The mitotic rate was high. The tumor cells were arranged in vague organoid clusters and cords. Some areas of tumor displayed single-cell necrosis, while areas of confluent necrosis were seen elsewhere (figure).

Immunohistologic staining for pan-keratin (AE1/ AE2), epithelial membrane antigen, and synaptophysin were positive, which supported a diagnosis of neuroendocrine carcinoma. Stains for S-100, CD45, and chromogranin were negative. Metastatic tumor was found in four lymph nodes in the right neck dissection and in six nodes on the left.

Four months later, a tumor was noted in the patient's nasopharynx; the soft palate remained tumor-free. The patient was treated with radiation and chemotherapy, but he did not respond. Two months later, he developed multiple subcutaneous nodules and metastases to the liver and lung. He died 3 months later, 9 months following his surgery.



For typical carcinoids (well-differentiated neuroendocrine carcinomas) of the head and neck, wide local excision without neck dissection is the treatment of choice. Radiation and chemotherapy, alone or in combination, are not effective.

Atypical carcinoids (moderately differentiated neuroendocrine carcinomas) are the most common of the three classifications of tumor. They are aggressive malignancies that metastasize to the cervical lymph nodes. They can also spread to the liver, lungs, and skin. These tumors do not respond to radiation or chemotherapy, so the treatment of choice is surgical excision of the primary tumor and neck dissection.

Small-cell tumors (poorly differentiated neuroendocrine carcinomas) are also highly malignant. They can be treated with radiation and chemotherapy, but survival is poor.

To the best of our knowledge, no case of atypical carcinoid or any other type of neuroendocrine carcinoma has been reported in the uvula, soft palate, or anywhere else in the oral cavity. Cases of extralaryngeal neuroendocrine carcinoma have been reported in the maxillary sinus and nasopharynx. (3,4) Since carcinoids can occur in any tissue derived from the primitive gut, these tumors probably represented the most forward position of the cells of origin in the primitive foregut. The uvula being the site of the primitive buccopharyngeal membrane would therefore be derived from the same precursor? Neuroendocrine tumors would not be expected to occur in tissue derived from the primitive stomodeum.

The case of our patient demonstrates the clinical course and histologic appearance of an atypical carcinoid. Aggressive surgery, even with what appeared to be adequate surgical margins, was not successful. Chemo- and radiotherapy were of no value. Treatment of neuroendocrine tumors in rare locations in the head and neck is dependent on the histologic diagnosis, as is treatment of tumors of the larynx. (6)


(1.) Mills SE. Neuroectodermal neoplasms of the head and neck with emphasis on neuroendocrine carcinomas. Mod Pathol 2002;15(3): 264-78.

(2.) Goldman NC, Hood CI, Singleton GT. Carcinoid of the larynx. Arch Otolaryngol 1969:90(1):64-7.

(3.) Kanamalla US, Kesava PP, McGuff HS. Imaging of nonlaryngeal neuroendocrine carcinoma. AJNR Am J Neuroradiol 2000;21 (4):775-8.

(4.) Feng Y, Xu G, Liu B, et al. Four cases of primary neuroendocrine carcinoma in ear, nose, pharynx and larynx. Lin Chuang Er Bi Van Hou Ke Za Zhi 2003;17(9):549-50.

(5.) Davis J. Embryology of the Head and Neck in Relation to the Practice of Otolaryngology: A Manual Prepared for the Use of Graduates in Medicine. Rochester, Minn.: American Academy of Ophthalmology and Otolaryngology; 1965:24.

(6.) Gripp FM, Risse EK, Leverstein H, et al. Neuroendocrine neoplasms of the larynx. Importance of the correct diagnosis and differences between atypical carcinoid tumors and small-cell neuroendocrine carcinoma. Eur Arch Otorhinolaryngol 1995;252(5):280-6.

Nelson C. Goldman, MD; Robert E. Barnes Jr., MD

From the Division of Oral & Maxillofacial Surgery, Department of Surgery, University of Florida College of Medicine, Jacksonville (Dr. Goldman); and the Department of Pathology, Memorial Hospital of Jacksonville (Dr. Barnes).

Corresponding author: Nelson C. Goldman, MD, Division of Oral & Maxillofacial Surgery, UF College of Medicine, 653 W. 8th St., Jacksonville, FL 32209. Email:
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Author:Goldman, Nelson C.; Barnes, Robert E., Jr.
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Feb 1, 2012
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