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Parotid Small Cell Carcinoma With Long-Term Survival

Parotid Small Cell Carcinoma With Long-Term Survival

Abstract and Introduction

Abstract


Introduction Primary involvement of the salivary glands in small cell carcinoma is rare, and has one of the worst prognoses of salivary gland neoplasms. However, it has been reported that some cases have a favorable outcome, although the prognostic factors are still under consideration. Multidisciplinary therapy was usually required to achieve long-term survival. Recently, a resemblance of some small cell carcinomas of the salivary gland to cutaneous Merkel cell carcinoma was suggested; the latter have the potential for spontaneous regression, which is related to a favorable clinical outcome.

Case presentation We present a locoregional advanced parotid small cell carcinoma with multiple lymph node metastases in an 87-year-old Asian woman. The tumor was controlled by surgery alone, and nine-year disease-free survival was achieved without any adjunctive therapy. To the best of our knowledge, this is the longest reported follow-up of head and neck small cell carcinoma.

Conclusion We believe this to be the first case of small cell carcinoma with involvement of the salivary glands reported in the literature with a good outcome after surgery alone without any adjunctive therapy.

Introduction


Primary involvement of the salivary glands in small cell carcinoma (SmCC) is rare, and tumors in the salivary glands account for less than 1% of all carcinomas of the parotid gland and 3.5% of all malignant tumors of minor salivary glands. This tumor has one of the worst prognoses of salivary gland neoplasms. The prognosis for patients with SmCC of the salivary glands has been reported to be more favorable than for those with SmCC of other sites. However, there is no doubt that parotid SmCC is a high-grade malignancy that should be treated aggressively. Surgery, adjunctive radiation therapy and/or chemotherapy have been performed in most cases.

SmCCs of the salivary gland are classified into neuroendocrine types and ductal carcinomas. The neuroendocrine type can be classified further into Merkel-cell-like SmCC and pulmonary variants, based on cytokeratin 20 immunoreactivity with a dot-like staining pattern. Recently, the resemblance of Merkel-cell-like SmCCs of salivary gland to cutaneous Merkel cell carcinoma has been suggested, and some of them have spontaneous regression potential related to a favorable clinical outcome. In this article, we report a locoregional advanced parotid SmCC that had an unusual clinical course. The tumor was controlled by surgery alone, and nine-year disease-free survival was achieved without any adjunctive therapy. We also studied the expression of an oncogene or tumor suppressor gene, and demonstrated the expression of mammary serine protease inhibitor (Maspin), which is an important tumor suppressor gene of salivary gland carcinomas.



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