A typical carcinoid is extremely rare in the oral cavity. We here present a case of a typical carcinoid arising in the sublingual gland of a 62-year-old woman. The tumor was removed by primary excision with 10 mm surgical margins and submandibular dissection. Examination of the tumor showed medium-sized tumor cells that were positive for CD56 and chromogranin A, with no necrosis, and with a mitotic count less than 1/10 HPF. A pathological diagnosis of typical carcinoid was made from both morphological and immunological examinations. One year after excision surgery, there was no tumor recurrence or neck metastasis.
Neuroendocrine tumors (NETs) are heterogeneous, ranging from benign to highly malignant. In the larynx, the most frequent NETs are atypical carcinoids (53.7%), followed by neuroendocrine-type small cell carcinomas (27.6%), paragangliomas (12.1%), and typical carcinoids (6.6%) [
Most carcinoids occurring in the head and neck area are found in the larynx, followed by the middle ear [
A 62-year-old woman came to the Department of Oral and Maxillofacial Surgery, University of Tsukuba Hospital, with a clinical diagnosis of a sublingual gland tumor. She had noticed swelling in the sublingual gland, with no pain, for 9 months. She had a history of hypertension, cerebral infarction, and hyperlipidemia. Her face was symmetrical and there was no trismus. The regional lymph nodes were normal, and the submandibular gland was hard and swollen. Examination of the oral cavity showed a hard, elastic mass measuring 28 × 13 mm on the left side of the floor of her mouth. The mucosal surface was normal, with no ulceration (Figure
Intraoral examination. Examination of the oral cavity showed a 28 × 13 mm hard elastic mass in the left floor of the mouth. The mucosal surface was normal, with no ulceration.
Magnetic resonance (MR) images [short TI inversion recovery (STIR)] showed a well-defined tumor of the sublingual gland with a high-signal mass measuring 28 × 25 × 12 mm (Figure
MR images (STIR) showed a well-defined tumor of the sublingual gland with a high-signal mass measuring 28 × 25 × 12 mm.
FDG-PET. The sublingual mass measured 28 × 13 mm and had a max SUV of 10.4.
The carcinoid was removed by primary excision performed extraorally under general anesthesia, with 10 mm surgical margins and submandibular dissection with the level I lymph node and submandibular gland. The intraoral resected defect was partially sutured and covered with polyglycolic acid (PGA) sheets and fibrin glue. The postoperative course was uneventful. One year later, there was no tumor recurrence or neck metastasis.
Histological examination revealed that the resected tumor, which was 26 × 24 × 12 mm, was whitish in color and solid in consistency. The round cells were solid or arranged in cords, trabeculae, or nests, with hyalinized stroma with high vascularization. The tumor cells had eosinophilic cytoplasm. The nuclei were round and varied in size, and the rough chromatin and mitotic count was less than 1/10 HPF. There was no necrosis (Figure
HE staining with low power (a) and high power (b). The round cells were arranged as cords, solids, trabeculae, and nests with a highly vascularized, hyalinized stroma. There was no necrosis, and the mitotic count was <1/10 HPF.
Immunohistochemical staining. The staining was positive for CD56 (a) and slightly positive for chromogranin A (b).
Carcinoid tumors are the most common of the NETs. They are usually found in the gastrointestinal tract (55%) or the bronchopulmonary tract (10%) [
Typical carcinoids are extremely rare in the head and neck region [
Reported cases of oral typical carcinoid.
Reference | Age/gender | Site | Size (mm) | Treatment | Prognosis (M) |
---|---|---|---|---|---|
Coleman et al. [ | 46/F | Mandible | 40 × 38 × 50 | Excision | NED (24) |
Yang et al. [ | 46/F | Retromolar region | 15 × 20 | Excision | NED (11) |
Present case | 62/F | Floor of mouth | 28 × 25 × 12 | Excision | NED (12) |
NED: no evidence of disease.
Our case was diagnosed as a typical carcinoid based on our findings and the algorithm described by Mahomed [
According to the 2005 WHO classification of NETs of the larynx, atypical carcinoid tumors exhibit more mitotic and more atypical cells than do the typical carcinoids [
Previously reported NETs have been treated by tumor resection. Radiation and chemotherapy are ineffective against typical carcinoids, making surgery the treatment of choice. NETs should be resected as conservatively as possible while still removing the tumor completely. A neck dissection is not warranted. In the present case, the sublingual tumor extended deeply toward the border of the mandible. The excision was performed from the submandible extraorally with submandibular dissection. Moreover, the patient was in her sixth decade and was being treated surgically. The reported rate of metastases for a carcinoid of the larynx is 33%, and the 5-year survival rate is 48% [
The authors declare that there is no conflict of interests regarding the publication of this paper.