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Primary Parotid Merkel Type Small Cell Neuroendocrine Carcinoma with Oligometastasis to the Brain and Adrenal Gland: Case Report and Review of Literature

Journal

HEAD & NECK PATHOLOGY
Volume 15, Issue 1, Pages 311-318

Publisher

SPRINGER
DOI: 10.1007/s12105-020-01164-w

Keywords

Merkel cell type; Merkel cell carcinoma; Small cell neuroendocrine carcinoma; Parotid gland; Oligometastasis; Brain metastasis; Stereotactic radiotherapy; Case report

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Oligometastatic disease is a hypothetical intermediate stage between localized and widespread metastatic cancer, where localized treatment may provide survival benefits. This case describes a patient with rare oligometastatic small cell neuroendocrine carcinoma Merkel type, treated with chemotherapy and radiotherapy, maintaining good functional status and low disease burden.
Oligometastatic disease is a hypothesized intermediate stage of disease between localized and widespread metastatic cancer. Localized treatment of oligometastatic lesions may offer survival advantages in addition to systemic treatment. In this case report, we describe a patient who presented with small cell neuroendocrine carcinoma Merkel type (SNECM) of the parotid gland which had metastasized to the brain and adrenal gland. He was treated with chemotherapy followed by stereotactic radiotherapy and volumetric modulated arc therapy for oligometastasis. He maintains good functional status with low burden of disease at 20-months after diagnosis. SNECM is a rare and aggressive parotid cancer with immunohistochemical and morphologic similarities to Merkel cell carcinoma (MCC). There are only 44 cases of parotid SNECM in the English literature. This is the first case to describe management of oligometastatic SNECM and we review literature on management of both SNECM and metastatic MCC.

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