gms | German Medical Science

We report the case of a 56 year old patient who presented in our clinic with a dysphagia persisting for four months and multiple cervical swellings. Clinically an ulcerated tumor was seen in the area of the tongue base and in the sonography several enlarged lymph nodes were noted on both sides. Routine staging was otherwise unremarkable. The histological examination of a sample taken during a panendoscopy revealed a small cell neuroendocrine carcinoma. Since this tumor is very rare in the specific location, a lymph node was removed in a second operation, confirming the above mentioned histological diagnosis. To exclude a metastatic primary tumor in another location, a PET-CT and a bronchoscopy was performed. Multiple bone metastasis were found, a primary tumor could not be located. The patient received 3 cycles of chemotherapy according to the CEV scheme. A restaging was performed, which showed that the primary tumor and the cervical metastases had regressed.

Discussion: Small cell neuroendocrine carcinoma is found predominantly in the lungs. Here, it accounts for 25 to 30 percent of cancers. Small cell primary tumors found in extrapulmonary locations are very rare. They are found in descending order in the following head and neck locations: larynx, salivary glands, paranasal sinuses, tonsils, oral cavity, hypopharynx. Their estimated incidence is 1%. The localization in the base of the tongue is extremely rare. So far in the literature only 3 cases of primary neuroendocrine carcinoma in the tongue base have been described.