Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine tumor that almost always presents as a cutaneous lesion in the sun-exposed areas on the bodies of elderly white males. Metastasis to lymph nodes in the presence or absence of a known primary site and occurrence of these tumors in non-sun-exposed sites have also been described; however, an incidence of recurrent disease arising in the palatine tonsil in the absence of any detectable primary lesion has never been reported in the literature. In this report, we discuss a case of a 72-year-old female who was found to have a single axillary lymph node, which was resected and proved to be positive for MCC of unknown primary (MCCUP). Since there was no evidence of additional disease, the patient elected not to pursue adjuvant therapies. Six and a half months later, she presented with a complaint of dysphagia and a right-sided exophytic tonsillar mass. Tonsillectomy revealed MCC with no detectable primary cutaneous lesion. She received adjuvant therapy with avelumab and demonstrated a complete response after one year of bi-weekly treatments. Seven months following cessation of adjuvant treatments, surveillance positron emission tomography (PET) revealed enlarged retroperitoneal, pretracheal, periaortic, and left axillary lymph nodes concerning for recurrence. She elected to forgo additional biopsies and restarted avelumab the following month. She continues to be followed up on a monthly basis.
Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a newly defined, rare malignant tumor of the nasal cavity. The clinical course and response to treatment remain uncertain. The purpose of this study is to report a new case of SNRCLA and review the literature to determine clinical characteristics, treatment options, and outcomes. A 26-year-old male presented with headache, epistaxis, and nasal obstruction. Physical examination revealed a tumor involving bilateral ethmoid sinuses and MRI revealed extension through the cribriform plate. Surgical excision with endonasal and a bifrontal craniotomy was performed followed by adjuvant radiotherapy (RT). After RT, the patient had persistent disease requiring salvage surgery. There are few previously reported cases of SNRCLA. A literature review yielded 14 previously reported cases with convincing diagnostic evidence of SNRCLA. Common presenting symptoms were epistaxis and nasal obstruction. Surgical excision was the primary treatment in fourteen cases, nine received RT, and none received chemotherapy. However, three cases had persistent or recurrent disease. Surgical excision is the mainstay of treatment for SNRCLA and adjuvant RT has been used in some patients with varying outcomes. The tumor is low grade with no reported cases of metastases or death. The best practice for treatment is yet to be determined.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.