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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

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Abstract

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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References

  1. Otake S, Goto T. Stereotactic radiotherapy for oligometastasis. Cancers (Basel). 2019;11:133.

    Article  CAS  Google Scholar 

  2. Gomez DR, Blumenschein GR, Lee JJ, Hernandez M, Ye R, Camidge DR, et al. Local consolidative therapy versus maintenance therapy or observation for patients with oligometastatic non-small-cell lung cancer without progression after first-line systemic therapy: a multicentre, randomised, controlled, phase 2 study. Lancet Oncol. 2016;17:1672–82.

    Article  CAS  Google Scholar 

  3. Palma DA, Olson R, Harrow S, Gaede S, Louie AV, Haasbeek C, et al. Stereotactic ablative radiotherapy versus standard of care palliative treatment in patients with oligometastatic cancers (SABR-COMET): a randomised, phase 2, open-label trial. Lancet. 2019;393:2051–8.

    Article  Google Scholar 

  4. Ost P, Reynders D, Decaestecker K, Fonteyne V, Lumen N, De Bruycker A, et al. Surveillance or metastasis-directed therapy for oligometastatic prostate cancer recurrence: a prospective, randomized, multicenter phase II trial. J Clin Oncol. 2018;36:446–53.

    Article  CAS  Google Scholar 

  5. Chernock RD, Duncavage EJ. Proceedings of the NASHNP Companion Meeting, March 18th, 2018, Vancouver, BC, Canada: salivary neuroendocrine carcinoma-an overview of a rare disease with an emphasis on determining tumor origin. Head Neck Pathol. 2018;12:13–211.

    Article  Google Scholar 

  6. Kraemer BB, Mackay B, Batsakis JG. Small cell carcinomas of the parotid gland. A clinicopathologic study of three cases. Cancer. 1983;52:2115–21.

    Article  CAS  Google Scholar 

  7. Gnepp DR, Wick MR. Small cell carcinoma of the major salivary glands. An immunohistochemical study. Cancer. 1990;66:185–92.

    Article  CAS  Google Scholar 

  8. Fornelli A, Eusebi V, Pasquinelli G, Quattrone P, Rosai J. Merkel cell carcinoma of the parotid gland associated with Warthin tumour: report of two cases. Histopathology. 2001;39:342–6.

    Article  CAS  Google Scholar 

  9. Nagao T, Gaffey TA, Olsen KD, Serizawa H, Lewis JE. Small cell carcinoma of the major salivary glands: clinicopathologic study with emphasis on cytokeratin 20 immunoreactivity and clinical outcome. Am J Surg Pathol. 2004;28:762–70.

    Article  Google Scholar 

  10. Jorcano S, Casado A, Berenguer J, Arenas M, Rovirosa A, Colomo L. Primary neuroendocrine small cell undifferentiated carcinoma of the parotid gland. Clin Transl Oncol. 2008;10:303–6.

    Article  CAS  Google Scholar 

  11. Ghaderi M, Coury J, Oxenberg J, Spector H. Primary Merkel cell carcinoma of the parotid gland. Ear Nose Throat J. 2010;89:E24–27.

    Article  Google Scholar 

  12. Mulder DC, Rosenberg AJWP, Storm-Bogaard PW, Koole R. Spontaneous regression of advanced Merkel-cell-like small cell carcinoma of the parotid gland. British Journal of Oral and Maxillofacial Surgery. 2010;48:199–200.

    Article  CAS  Google Scholar 

  13. Chernock RD, Duncavage EJ, Gnepp DR, El-Mofty SK, Lewis JS. Absence of Merkel cell polyomavirus in primary parotid high-grade neuroendocrine carcinomas regardless of cytokeratin 20 immunophenotype. Am J Surg Pathol. 2011;35:1806–11.

    Article  Google Scholar 

  14. de Biase D, Ragazzi M, Asioli S, Eusebi V. Extracutaneous Merkel cell carcinomas harbor polyomavirus DNA. Hum Pathol. 2012;43:980–5.

    Article  CAS  Google Scholar 

  15. Kanazawa T, Fukushima N, Tanaka H, Shiba J, Nishino H, Mineta H, et al. Parotid small cell carcinoma presenting with long-term survival after surgery alone: a case report. J Med Case Rep. 2012;6:431.

    Article  Google Scholar 

  16. Fisher CA, Harms PW, McHugh JB, Edwards PC, Siddiqui J, Palanisamy N, et al. Small cell carcinoma in the parotid harboring Merkel cell polyomavirus. Oral Surg Oral Med Oral Pathol Oral Radiol. 2014;118:703–12.

    Article  Google Scholar 

  17. Knopf A, Bas M, Hofauer B, Mansour N, Stark T. Clinicopathological characteristics of head and neck Merkel cell carcinomas. Head & Neck. 2017;39:92–7.

    Article  Google Scholar 

  18. Bizzarro T, Buda R, Ricci M, Bernardi L. Cytological diagnosis of a rare case of primary Merkel cell carcinoma of the parotid gland. Cytopathology. 2017;28:552–4.

    Article  CAS  Google Scholar 

  19. Giroulet F, Tabotta F, Pomoni A, Prior J. Primary parotid Merkel cell carcinoma: a first imagery and treatment response assessment by 18F-FDG PET. BMJ Case Reports. 2019;12.

  20. Engels EA, Frisch M, Goedert JJ, Biggar RJ, Miller RW. Merkel cell carcinoma and HIV infection. Lancet. 2002;359:497–8.

    Article  Google Scholar 

  21. Behera M, Ragin C, Kim S, Pillai RN, Chen Z, Steuer CE, et al. Trends, predictors, and impact of systemic chemotherapy in small cell lung cancer patients between 1985 and 2005. Cancer. 2016;122:50–60.

    Article  CAS  Google Scholar 

  22. Liang E, Brower JV, Rice SR, Buehler DG, Saha S, Kimple RJ. Merkel Cell Carcinoma Analysis of Outcomes: A 30-Year Experience. PLoS One. 2015;10.

  23. Becker JC, Stang A, DeCaprio JA, Cerroni L, Lebbé C, Veness M, et al. Merkel cell carcinoma. Nat Rev Dis Primers. 2017;3:17077.

    Article  Google Scholar 

  24. Nghiem PT, Bhatia S, Lipson EJ, Kudchadkar RR, Miller NJ, Annamalai L, et al. PD-1 Blockade with Pembrolizumab in Advanced Merkel-Cell Carcinoma. N Engl J Med. 2016;374:2542–52.

    Article  CAS  Google Scholar 

  25. Kaufman HL, Russell J, Hamid O, Bhatia S, Terheyden P, D’Angelo SP, et al. Avelumab in patients with chemotherapy-refractory metastatic Merkel cell carcinoma: a multicentre, single-group, open-label, phase 2 trial. Lancet Oncol. 2016;17:1374–85.

    Article  CAS  Google Scholar 

  26. Kaufman HL, Russell JS, Hamid O, Bhatia S, Terheyden P, D’Angelo SP, et al. Updated efficacy of avelumab in patients with previously treated metastatic Merkel cell carcinoma after ≥1 year of follow-up: JAVELIN Merkel 200, a phase 2 clinical trial. J Immunother Cancer. 2018;6:7.

    Article  Google Scholar 

  27. Ruers T, Punt C, Van Coevorden F, Pierie JPEN, Borel-Rinkes I, Ledermann JA, et al. Radiofrequency ablation combined with systemic treatment versus systemic treatment alone in patients with non-resectable colorectal liver metastases: a randomized EORTC Intergroup phase II study (EORTC 40004). Ann Oncol. 2012;23:2619–26.

    Article  CAS  Google Scholar 

  28. Andrews DW, Scott CB, Sperduto PW, Flanders AE, Gaspar LE, Schell MC, et al. Whole brain radiation therapy with or without stereotactic radiosurgery boost for patients with one to three brain metastases: phase III results of the RTOG 9508 randomised trial. Lancet. 2004;363:1665–72.

    Article  Google Scholar 

  29. Aoyama H, Shirato H, Tago M, Nakagawa K, Toyoda T, Hatano K, et al. Stereotactic radiosurgery plus whole-brain radiation therapy vs stereotactic radiosurgery alone for treatment of brain metastases: A randomized controlled trial. JAMA. 2006;295:2483–91.

    Article  CAS  Google Scholar 

  30. Mazzola R, Corradini S, Gregucci F, Figlia V, Fiorentino A, Alongi F. Role of Radiosurgery/Stereotactic Radiotherapy in Oligometastatic Disease: Brain Oligometastases. Front Oncol. 2019;9.

  31. Harary M, Kavouridis VK, Thakuria M, Smith TR. Predictors of survival in neurometastatic Merkel cell carcinoma. Eur J Cancer. 2018;101:152–9.

    Article  Google Scholar 

  32. Iyengar P, Wardak Z, Gerber DE, Tumati V, Ahn C, Hughes RS, et al. Consolidative Radiotherapy for Limited Metastatic Non-Small-Cell Lung Cancer: A Phase 2 Randomized Clinical Trial. JAMA Oncol. 2018;4:e173501.

    Article  Google Scholar 

  33. Palma DA, Olson R, Harrow S, Correa RJM, Schneiders F, Haasbeek CJA, et al. Stereotactic ablative radiotherapy for the comprehensive treatment of 4–10 oligometastatic tumors (SABR-COMET-10): Study protocol for a randomized phase III trial. BMC Cancer. 2019;19:816.

    Article  Google Scholar 

  34. Ahmadi Moghaddam P, Cornejo KM, Hutchinson L, Tomaszewicz K, Dresser K, Deng A, et al. Complete Spontaneous Regression of Merkel Cell Carcinoma After Biopsy: A Case Report and Review of the Literature. Am J Dermatopathol. 2016;38:e154–8.

    Article  Google Scholar 

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Authors and Affiliations

  1. University of British Columbia Faculty of Medicine, Vancouver, BC, Canada

    Sympascho Young

  2. Department of Radiation Oncology, BC Cancer, Vancouver, BC, Canada

    Justin Oh & Eric Tran

  3. Department of Pathology, BC Cancer, Vancouver, BC, Canada

    Hussam Bukhari & Tony Ng

  4. Department of Medical Oncology, BC Cancer, Vancouver, BC, Canada

    Nichole Chau

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  1. Sympascho Young
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Young, S., Oh, J., Bukhari, H. et al. Primary Parotid Merkel Type Small Cell Neuroendocrine Carcinoma with Oligometastasis to the Brain and Adrenal Gland: Case Report and Review of Literature. Head and Neck Pathol 15, 311–318 (2021). https://doi.org/10.1007/s12105-020-01164-w

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