The small cell carcinoma neuroendocrine type of the larynx—Case report

Introduction Extrapulmonary small cell carcinoma neuroendocrine type (ESCCNET) is a rare tumor (2.5%-4.0% of all small cell carcinomas), and during the past 30 years, only 160 cases of primary small cell carcinoma of the larynx have been reported worldwide. Aim To report a rare case of small cell carcinoma of the larynx, in a 54-year-old man, including diagnostic work-up, and treatment with chemotherapy (CHT) and radiotherapy (RT), compromised by the patient's nonadherence, and to spread awareness of the need of further research, focused on new therapeutic strategies, in patients with ESCCNET. Case study The patient underwent induction CHT based on cisplatin and etoposide. Partial remission was obtained after the 3rd cycle of CHT, confirmed by the CT scan. The patient refused further CHT. He underwent RT. The residual larynx tumor and elective nodal volume were irradiated. A follow-up laryngological examination and control CT scan, performed two months after the completion of RT, showed a complete remission. Eight months later brain CT revealed multiple metastases. Palliative cranial irradiation and CHT were applied. The patient died in October 2011. Results and discussion ESCCNET, an unusual neoplasm, accounting for 0.5% of all laryngeal carcinomas, is most commonly located in the supraglottic region. In contrast to squamous cell carcinoma of the larynx, a single modality therapy is not advised for ESCCNET, because these tumors are biologically aggressive, and at least 73% of patients have distant metastases. Therefore, RT and CHT in concomitant or sequential fashion offer the best chance of survival. However, in the presented case, the recommended therapy was not fully implemented, due to the patient's noncompliance. Conclusions This report underscores the need for thorough, systemic diagnostic work-up, and emphasizes that the combination of RT and CHT remains the treatment of choice for ESCCNET (while surgery is reserved for persistent and recurrent disease). In addition, it points out that the new therapeutic strategies to improve survival in ESCCNET are needed, such as a prophylactic cranial irradiation that could be explored in future clinical trials.