Small Cell Carcinoma of the Major Salivary Glands: Clinicopathologic Study With Emphasis on Cytokeratin 20 Immunoreactivity and Clinical Outcome

Division of Anatomic Pathology, Mayo Clinic, Rochester, MN 55905, USA.
American Journal of Surgical Pathology (Impact Factor: 5.15). 07/2004; 28(6):762-70. DOI: 10.1097/01.pas.0000126776.65815.48
Source: PubMed


Small cell carcinomas arising in salivary glands, extremely rare high-grade malignant tumors, are subclassified into neuroendocrine and ductal types. The neuroendocrine type may be segregated further into Merkel cell and pulmonary varieties according to cytokeratin 20 immunoreactivity. Whether subclassification of this tumor group has any biologic or clinical significance is not known. We examined 15 cases (11 men, 4 women; mean age, 66.5 years) of small cell carcinoma of major salivary glands from a single institution and analyzed their clinicopathologic profiles, including immunohistochemical features and prognostic factors. Three fourths of small cell carcinomas showed cytokeratin 20-positive immunostaining, often with a paranuclear dotlike pattern of reactivity. All tumors were immunoreactive for at least 2 of 6 neuroendocrine markers examined, and 6 tumors were also positive for neurofilament, with a paranuclear dotlike pattern. Postoperatively, 9 patients developed metastatic disease, and 10 patients died of disease 2 to 45 months (mean, 15.9 months) after diagnosis. By log-rank analysis, overall survival was reduced significantly for patients with a primary tumor larger than 3 cm in diameter (P = 0.032), negative immunostain reaction for cytokeratin 20 (P = 0.012), and decreased immunoreactivity for neuroendocrine markers (P = 0.034). These results indicate that small cell carcinoma of major salivary glands is a highly aggressive tumor, although the prognosis may be better than for extrasalivary neoplasms. Our data also suggest that most salivary gland small cell carcinomas exhibit neuroendocrine differentiation. Immunohistochemical expression of cytokeratin 20 can be used to classify salivary small cell carcinomas into Merkel cell and pulmonary types and may have prognostic significance.

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    • "The tumor is often observed in patients between 50 and 80 years old and is more frequently identified in males. The largest reported series of SCC of the major salivary glands (n=15) revealed a 73% male predominance (14). "
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    ABSTRACT: Small cell carcinoma (SCC) is a malignant epithelial tumor that predominantly arises in the lungs. Primary SCC of the parotid gland is rare and difficult to diagnose by analysis of frozen sections obtained during surgery. Due to the aggressive nature of SCC and the frequent occurrence of distant metastases, identification of the disease is important. The current study reports the case of a male patient who presented with a right parotid gland mass. The tumor was resected and evaluated by light microscopy and immunohistochemical analysis. Immunohistochemically, the tumor was positive for cytokeratin, epithelial membrane antigen, cluster of differentiation 117, synaptophysin and thyroid transcription factor-1, which indicated that the tumor was a SCC of the parotid gland. An extended resection of the right parotid gland mass and dissection of the facial nerve were performed. Following discharge from the hospital, the patient received radiation therapy postoperatively. The patient has remained disease free during five months of follow-up.
    Oncology letters 09/2014; 8(3):1275-1278. DOI:10.3892/ol.2014.2258 · 1.55 Impact Factor
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    • "SmCC can occur in any organ, although the vast majority occur in the lung. The prognosis for patients with SmCC of the salivary glands has been reported to be more favorable than for those with SmCC of the lung or larynx [1,2,4]. However, there is no doubt that parotid SmCC is a high-grade malignancy that should be treated aggressively. "
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    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.
    Journal of Medical Case Reports 12/2012; 6(1):431. DOI:10.1186/1752-1947-6-431
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    • "However, in a recent study on MCC, salivary gland cancer was considered as a second primary and a significant excess was found, though the authors discuss the difficulty in making this distinction [8]. In fact, the presence of salivary gland cancer and MCC could be indicative of: (1) MCC that developed in the skin and infiltrated the parotid gland; (2); MCC-like primary small cell carcinoma of the parotid gland, which is closely related to cutaneous MCC [23]; or (3) parotid metastases of MCC. In fact, we found a third death certificate with non-localized MCC mentioning parotid metastases of MCC. "
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    ABSTRACT: Few population-based epidemiological data are available on Merkel cell carcinoma (MCC), a rare lethal non-melanoma skin cancer. We analysed multiple-cause-of-death records to describe MCC mortality and trends and the association with other primary cancers. We reviewed all 6,713,059 death certificates in Italy (1995-2006) to identify those mentioning MCC. We evaluated the association with other primary cancers by calculating the ratio of observed to expected deaths, using a standardized mortality ratio (SMR)-like analysis. We also evaluated the geographic distribution of deaths. We identified 351 death certificates with the mention of MCC. The age-adjusted mortality was 0.031/100,000, with a significant trend of increase and a slight north-south gradient. There was a significant deficit for solid cancers (SMR = 0.15) and a non-significant excess for lymphohematopoietic malignancies (SMR = 1.62). There were significant excesses for chronic lymphocytic leukemia (SMR = 4.07) and Waldenström's macroglobulinemia (SMR = 27.2) and a non-significant excess for chronic myeloid leukemia (SMR = 5.12). The increase in MCC mortality reflects the incidence trend in the literature. The association with chronic lymphocytic leukemia confirms the importance of immunologic factors in MCC. Regarding Waldenström's macroglobulinemia, an association with MCC has never been reported.
    Cancer Causes and Control 08/2011; 22(11):1521-7. DOI:10.1007/s10552-011-9826-4 · 2.74 Impact Factor
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