Primary Small Cell Carcinoma of the Hypopharynx: A Report of Two Cases and Review of Nine Additional Cases

Abstract

Objective Two patients with primary small cell carcinoma (SmCC) of the hypopharynx, an extremely rare site for the occurrence of SmCC, are reported and nine additional well-documented cases are reviewed. Methods Case report and review of the literature concerning primary SmCC of the hypopharynx. Results On the final analysis, we reviewed eleven cases of primary SmCC of the hypopharynx. The tumors contained mixed elements of SmCC and squamous cell carcinoma (SCC) in six (55%) of eleven patients. Out of eleven patients, two patients had distant metastasis at the initial presentation. Even though nine patients presented with locoregional disease, development of distant metastasis after treatment was seen in five patients (56%), whereas there was no report of treatment failure on the primary site. To achieve more than two-year survival, patients should have received more than 4 cycles of chemotherapy. Conclusion We report two cases of primary SmCC of the hypopharynx with a review of the literature. In more than half of the cases, combined carcinomas with SCC are seen. Because this tumor has a strong propensity for distant metastasis even in patients with clinically localized tumor, new powerful systemic agents should be explored.

<p>Primary poorly differentiated small cell type neuroendocrine carcinoma of the hypopharynx</p>

“…Lee et al19 reported the case of a patient with a Ki-67 mitotic index that approached 70% and made the diagnosis of LCNEC of the hypopharynx. Nakahira et al15 also reported two cases of primary small cell carcinoma of the hypopharynx with high Ki-67 positivity. This high Ki-67 expression is consistent with the current case.…”

“…This suggests that an organ preservation strategy with platinum-based chemotherapy and concurrent radiotherapy for the primary lesion is also reasonable for SmCC of the hypopharynx 15. However, in contrast to SmCC of the larynx, there is a higher risk of failed completion of chemoradiotherapy in patients whose deglutition has been already severely compromised by advanced hypopharyngeal tumors, and these patients might be candidates for radical surgery, if possible, instead of chemoradiotherapy 15. In the current case, we selected chemotherapy after the diagnosis of SmCCs.…”

“…They reported that patients with sinonasal primary tumors experienced improved survival with surgery, whereas those with nonsinonasal tumors had better survival with radiation therapy 14. In contrast, Nakahira et al15 successfully achieved local control in all patients, regardless of the therapeutic modality used for the primary tumor. This suggests that an organ preservation strategy with platinum-based chemotherapy and concurrent radiotherapy for the primary lesion is also reasonable for SmCC of the hypopharynx 15.…”

A Rare Case of Human Papillomavirus-associated High-grade Neuroendocrine Carcinoma of the Hypopharynx

“…SCC of the hypopharynx (SCCH) is extremely rare. There are only 11 reported cases of SCCH to date [2]. The first case was reported in 1980 [2].…”

“…There are only 11 reported cases of SCCH to date [2]. The first case was reported in 1980 [2]. This case report presents the twelfth case of SCCH.…”