Primary Nodal Merkel Cell Carcinoma: Rare Presentation
Yıl 2023,
Cilt: 45 Sayı: 4, 610 - 614, 20.07.2023
Emel Tekin
,
Alaattin Özen
,
Melek Gürbüz
Öz
Merkel cell carcinoma (MCC) is a very rare high-grade neuroendocrine carcinoma that is frequently located in the skin. Although most cases are associated with Merkel cell polyomavirus, there are also cases in which the virus cannot be detected. The purpose of presenting the case of MCC with primary nodal involvement is due to its rare presentation. A 59-year-old male patient presented to the hospital with painless mass in the neck. Imaging analyses revealed necrotic lymphadenopathies on the neck, measuring up to 4.5 cm in size. After an explorative surgery with preliminary diagnoses of lymphoma and squamous cell carcinoma , samples taken from the excised mass showed high-grade neuroendocrine carcinoma morphology. In addition to neuroendocrine markers, CK7 and CK20 positivity were observed in the immunohistochemical tests performed. The patient was diagnosed with primary nodal MCC due to the negativity of the markers performed to exclude other malignancies and the absence of a different focus on PET imaging analysis outside the lymph node. The differential diagnosis of MCC should be kept broad due to the histopathological morphology of small round cell malignant tumors. In high-grade neuroendocrine carcinomas, the possibility of MCC should be ruled out especially if CK20 positivity is present. Although is has been reported that primary nodal MCC cases have a better clinical course compared to nodal metastatic cases with a known primary, there are limited case series studies on this topic. A multisystem approach is recommended for proper clinical management.
Kaynakça
- 1. Elder DE, Massi D, Scolyer RA, Willemze R. WHO classification of skin tumours: International Agency for Research on Cancer; 2018.
- 2. Tang CK, Toker C. Trabecular carcinoma of the skin. An ultrastructural study. Cancer. 1978;42:2311-21.
- 3. Sauer C, Haugg A, Chteinberg E, Rennspiess D, Winnepenninckx V, Speel E-J, et al. Reviewing the current evidence supporting early B-cells as the cellular origin of Merkel cell carcinoma. Critical reviews in oncology/hematology. 2017;116:99-105.
- 4. Kotteas E, Pavlidis N. Neuroendocrine Merkel cell nodal carcinoma of unknown primary site: management and outcomes of a rare entity. Critical Reviews in Oncology/Hematology. 2015;94:116-21.
- 5. Hui JYC, Prathibha S, White M, Praska CE, LaRocca CJ, Ankeny J, et al. Oncologic outcomes of patients with lymph node–positive Merkel cell carcinoma with unknown primary: A retrospective cohort study. Journal of the American Academy of Dermatology. 2023;88:465-7.
- 6. Lebbe C, Becker JC, Grob J-J, Malvehy J, Del Marmol V, Pehamberger H, et al. Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline. European Journal of Cancer. 2015;51:2396-403.
- 7. Gauci M-L, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, et al. Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline–Update 2022. European Journal of Cancer. 2022;171:203-31.
- 8. Amin MB, Edge SB, Greene FL, Byrd DR, Brookland RK, Washington MK, et al. AJCC cancer staging manual: Springer; 2017.
- 9. Lemos B, Nghiem P. Merkel cell carcinoma: more deaths but still no pathway to blame. Journal of Investigative Dermatology. 2007;127:2100-3.
- 10. Houben R, Shuda M, Weinkam R, Schrama D, Feng H, Chang Y, et al. Merkel cell polyomavirus-infected Merkel cell carcinoma cells require expression of viral T antigens. Journal of virology. 2010;84:7064-72.
- 11. Harms PW, Vats P, Verhaegen ME, Robinson DR, Wu Y-M, Dhanasekaran SM, et al. The distinctive mutational spectra of polyomavirus-negative Merkel cell carcinoma. Cancer research. 2015;75:3720-7.
- 12. Harms KL, Healy MA, Nghiem P, Sober AJ, Johnson TM, Bichakjian CK, et al. Analysis of prognostic factors from 9387 Merkel cell carcinoma cases forms the basis for the new 8th edition AJCC staging system. Annals of surgical oncology. 2016;23:3564-71.
- 13. Fazio N, Maisonneuve P, Spada F, Gervaso L, Cella CA, Pozzari M, et al. Nodal Merkel Cell Carcinoma with Unknown Primary Site and No Distant Metastasis: A Single-Center Series. Cancers. 2022;14:4777.
- 14. Harms PW, Harms KL, Moore PS, DeCaprio JA, Nghiem P, Wong MK, et al. The biology and treatment of Merkel cell carcinoma: current understanding and research priorities. Nature reviews Clinical oncology. 2018;15:763-76.
- 15. Vandeven N, Lewis CW, Makarov V, Riaz N, Paulson KG, Hippe D, et al. Merkel Cell Carcinoma Patients Presenting Without a Primary Lesion Have Elevated Markers of Immunity, Higher Tumor Mutation Burden, and Improved SurvivalMCC Unknown Primary: Enhanced Immunity and Improved Survival. Clinical Cancer Research. 2018;24:963-71.
- 16. Moshiri AS, Doumani R, Yelistratova L, Blom A, Lachance K, Shinohara MM, et al. Polyomavirus-negative Merkel cell carcinoma: a more aggressive subtype based on analysis of 282 cases using multimodal tumor virus detection. Journal of Investigative Dermatology. 2017;137:819-27.
- 17. Le MD, O'Steen LH, Cassarino DS. A rare case of CK20/CK7 double negative Merkel cell carcinoma. The American Journal of Dermatopathology. 2017;39:208-11.
- 18. Calder KB, Coplowitz S, Schlauder S, Morgan MB. A case series and immunophenotypic analysis of CK20−/CK7+ primary neuroendocrine carcinoma of the skin. Journal of cutaneous pathology. 2007;34:918-23.
- 19. Pasternak S, Carter MD, Ly TY, Doucette S, Walsh NM. Immunohistochemical profiles of different subsets of Merkel cell carcinoma. Human Pathology. 2018;82:232-8.
- 20. Jensen K, Kohler S, Rouse RV. Cytokeratin staining in Merkel cell carcinoma: an immunohistochemical study of cytokeratins 5/6, 7, 17, and 20. Applied Immunohistochemistry & Molecular Morphology. 2000;8:310-5.
- 21. Bobos M, Hytiroglou P, Kostopoulos I, Karkavelas G, Papadimitriou CS. Immunohistochemical distinction between Merkel cell carcinoma and small cell carcinoma of the lung. The American Journal of Dermatopathology. 2006;28:99-104.
- 22. Walsh NM, Cerroni L. Merkel cell carcinoma: A review. Journal of Cutaneous Pathology. 2021;48:411-21.
Primer Nodal Merkel Hücreli Karsinom: Nadir Prezentasyon
Yıl 2023,
Cilt: 45 Sayı: 4, 610 - 614, 20.07.2023
Emel Tekin
,
Alaattin Özen
,
Melek Gürbüz
Öz
Merkel hücreli karsinom (MHK) sıklıkla deri yerleşimli oldukça nadir görülen yüksek dereceli nöroendokrin karsinomdur. Olguların çoğu Merkel cell polyomavirus ile ilişkilendirilmekle birlikte virusun saptanamadığı olgular da mevcuttur. Bu yazıda oldukça nadir bir prezentasyon olması nedeni ile primeri bulunamayan nodal MHK tanılı olgunun sunulması amaçlandı. 59 yaşında erkek hasta boynunda ağrısız ele gelen kitle varlığı nedeni ile hastaneye başvurdu. Görüntüleme analizlerinde boyunda 4,5 cm’e ulaşan lenfadenopati ile uyumlu nekrotik kitleler saptandı. Lenfoma ve skuamöz hücreli karsinom ön tanıları ile yapılan eksploratif cerrahi sonucunda gönderilen kitleden alınan örnekler yüksek dereceli nöroendokrin karsinom morfolojisinde idi. Yapılan immunhistokimyasal tetkiklerde nöroendokrin belirteçlerin yanısıra CK7 ve CK20 pozitifliği eşlik etmekte idi. Diğer maligniteleri dışlamak amacıyla yapılan belirteçlerin negatifliği ve PET görüntüleme analizinde lenf nodu dışında farklı bir odak saptanmaması nedeni ile olgu primer nodal MHK tanısı aldı. MHK’un histopatolojik olarak küçük yuvarlak hücreli malign tümör morfolojisi nedeni ile ayırıcı tanısı oldukça geniş tutulmalıdır. Yüksek dereceli nöroendokrin karsinomlarda CK20 pozitifliği durumunda MHK olasılığı mutlaka ekarte edilmelidir. Primer nodal MHK olgularının, primeri bilinen nodal metastatik olgulara göre daha iyi klinik seyir gösterdiği bildirilse de bu konuda küçük olgu serili çalışmalar mevcuttur. Doğru klinik yönetim için multisistemik bir yaklaşım önerilmektedir.
Kaynakça
- 1. Elder DE, Massi D, Scolyer RA, Willemze R. WHO classification of skin tumours: International Agency for Research on Cancer; 2018.
- 2. Tang CK, Toker C. Trabecular carcinoma of the skin. An ultrastructural study. Cancer. 1978;42:2311-21.
- 3. Sauer C, Haugg A, Chteinberg E, Rennspiess D, Winnepenninckx V, Speel E-J, et al. Reviewing the current evidence supporting early B-cells as the cellular origin of Merkel cell carcinoma. Critical reviews in oncology/hematology. 2017;116:99-105.
- 4. Kotteas E, Pavlidis N. Neuroendocrine Merkel cell nodal carcinoma of unknown primary site: management and outcomes of a rare entity. Critical Reviews in Oncology/Hematology. 2015;94:116-21.
- 5. Hui JYC, Prathibha S, White M, Praska CE, LaRocca CJ, Ankeny J, et al. Oncologic outcomes of patients with lymph node–positive Merkel cell carcinoma with unknown primary: A retrospective cohort study. Journal of the American Academy of Dermatology. 2023;88:465-7.
- 6. Lebbe C, Becker JC, Grob J-J, Malvehy J, Del Marmol V, Pehamberger H, et al. Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline. European Journal of Cancer. 2015;51:2396-403.
- 7. Gauci M-L, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, et al. Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline–Update 2022. European Journal of Cancer. 2022;171:203-31.
- 8. Amin MB, Edge SB, Greene FL, Byrd DR, Brookland RK, Washington MK, et al. AJCC cancer staging manual: Springer; 2017.
- 9. Lemos B, Nghiem P. Merkel cell carcinoma: more deaths but still no pathway to blame. Journal of Investigative Dermatology. 2007;127:2100-3.
- 10. Houben R, Shuda M, Weinkam R, Schrama D, Feng H, Chang Y, et al. Merkel cell polyomavirus-infected Merkel cell carcinoma cells require expression of viral T antigens. Journal of virology. 2010;84:7064-72.
- 11. Harms PW, Vats P, Verhaegen ME, Robinson DR, Wu Y-M, Dhanasekaran SM, et al. The distinctive mutational spectra of polyomavirus-negative Merkel cell carcinoma. Cancer research. 2015;75:3720-7.
- 12. Harms KL, Healy MA, Nghiem P, Sober AJ, Johnson TM, Bichakjian CK, et al. Analysis of prognostic factors from 9387 Merkel cell carcinoma cases forms the basis for the new 8th edition AJCC staging system. Annals of surgical oncology. 2016;23:3564-71.
- 13. Fazio N, Maisonneuve P, Spada F, Gervaso L, Cella CA, Pozzari M, et al. Nodal Merkel Cell Carcinoma with Unknown Primary Site and No Distant Metastasis: A Single-Center Series. Cancers. 2022;14:4777.
- 14. Harms PW, Harms KL, Moore PS, DeCaprio JA, Nghiem P, Wong MK, et al. The biology and treatment of Merkel cell carcinoma: current understanding and research priorities. Nature reviews Clinical oncology. 2018;15:763-76.
- 15. Vandeven N, Lewis CW, Makarov V, Riaz N, Paulson KG, Hippe D, et al. Merkel Cell Carcinoma Patients Presenting Without a Primary Lesion Have Elevated Markers of Immunity, Higher Tumor Mutation Burden, and Improved SurvivalMCC Unknown Primary: Enhanced Immunity and Improved Survival. Clinical Cancer Research. 2018;24:963-71.
- 16. Moshiri AS, Doumani R, Yelistratova L, Blom A, Lachance K, Shinohara MM, et al. Polyomavirus-negative Merkel cell carcinoma: a more aggressive subtype based on analysis of 282 cases using multimodal tumor virus detection. Journal of Investigative Dermatology. 2017;137:819-27.
- 17. Le MD, O'Steen LH, Cassarino DS. A rare case of CK20/CK7 double negative Merkel cell carcinoma. The American Journal of Dermatopathology. 2017;39:208-11.
- 18. Calder KB, Coplowitz S, Schlauder S, Morgan MB. A case series and immunophenotypic analysis of CK20−/CK7+ primary neuroendocrine carcinoma of the skin. Journal of cutaneous pathology. 2007;34:918-23.
- 19. Pasternak S, Carter MD, Ly TY, Doucette S, Walsh NM. Immunohistochemical profiles of different subsets of Merkel cell carcinoma. Human Pathology. 2018;82:232-8.
- 20. Jensen K, Kohler S, Rouse RV. Cytokeratin staining in Merkel cell carcinoma: an immunohistochemical study of cytokeratins 5/6, 7, 17, and 20. Applied Immunohistochemistry & Molecular Morphology. 2000;8:310-5.
- 21. Bobos M, Hytiroglou P, Kostopoulos I, Karkavelas G, Papadimitriou CS. Immunohistochemical distinction between Merkel cell carcinoma and small cell carcinoma of the lung. The American Journal of Dermatopathology. 2006;28:99-104.
- 22. Walsh NM, Cerroni L. Merkel cell carcinoma: A review. Journal of Cutaneous Pathology. 2021;48:411-21.