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Merkel Cell Carcinoma: Prognosis and Management

Merkel Cell Carcinoma: Prognosis and Management Abstract • Seventy patients with Merkel cell carcinoma were treated at Memorial Sloan-Kettering Cancer Center between 1969 and 1989. The overall estimated 5-year survival rate was 64%. Factors predictive of improved survival included head and neck site and negative lymph nodes at presentation. Local recurrence was seen in 18 patients (26%) and did not correlate with patient-, tumor-, or treatment-related variables. Nine patients with local recurrence (50%) were free of disease following aggressive reoperation. Regional nodes were involved at some point during the course of the disease in forty-six patients (66%). Regional lymph node involvement was apparent within 2 years of diagnosis in 40 (87%) of 46 patients in whom it occurred. Systemic disease was nearly uniformly preceded by the appearance of nodal metastases and was uniformly fatal regardless of subsequent therapy. This suggests an orderly "cascade" pattern of spread for this tumor, in which elective regional lymph node dissection may be justified. Our recommendations for treatment include a wide excision of the primary tumor and either elective or early therapeutic regional node dissection. The role of adjuvant radiotherapy or chemotherapy remains unproven. (Arch Surg. 1991;126:1514-1519) References 1. Toker C. Trabecular carcinoma of the skin . Arch Dermatol . 1972;105:107-110.Crossref 2. Tang CK, Toker C. Trabecular carcinoma of the skin: an ultrastructural study . Cancer . 1978;42:2311-2321.Crossref 3. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations . J Am Stat Assoc . 1958;53:457-481.Crossref 4. Cox DR, Oakes D. Analysis of Survival Data . London, England: Chapman & Hall; 1984. 5. De Wolf-Peeter C, Marien K, Mebis J, Desmet V. A cutaneous APUDoma or Merkel cell tumor? a morphologically recognizable tumor with a biological and histological malignant aspect in contrast with its clinical behaviour . Cancer . 1980;46:1810-1816.Crossref 6. Szadowska A, Wozniak L, Lasota J, Giryn I, Mirecka B, Wolska H. Neuroendocrine (Merkel cell) carcinoma of the skin: a clinico-morphological study of 13 cases . Histopathology . 1989;15:483-493.Crossref 7. Goepfert H, Remmler D, Silva EG, Wheeler B. Merkel cell carcinoma (endocrine carcinoma of the skin) of the head and neck . Arch Otolaryngol Head Neck Surg . 1984;110:707-712.Crossref 8. Raaf JH, Urmacher C, Knapper WK, Shiu MH, Cheng EWK. Trabecular (Merkel cell) carcinoma of the skin: treatment of primary, recurrent, and metastatic disease . Cancer . 1986;57:178-182.Crossref 9. Pilotti S, Rilke F, Bartoli C, Grisotti A. Clinicopathologic correlations of cutaneous neuroendocrine Merkel cell carcinoma . J Clin Oncol . 1988;6:1863-1873. 10. Hitchcock CL, Bland KI, Lahey RG, Franzini D, Harris B, Copeland EM. Neuroendocrine (Merkel cell) carcinoma of the skin: its natural history, diagnosis, and treatment . Ann Surg . 1988;207:201-207.Crossref 11. Tang CK, Toker C. Trabecular carcinoma of the skin: further clinicopathologic and ultrastructural study . Mt Sinai J Med . 1979;46:516-523. 12. Sibley RK, Rosai J, Foucar E, Dehner LP, Bosi G. Neuroendocrine (Merkel cell) carcinoma of the skin: a histologic and ultrastructural study of two cases . Am J Surg Pathol . 1980;4:211-221.Crossref 13. Warner TFCS, Uno H, Hafez GR, et al. Merkel cells and Merkel cell tumors: ultrastructural, immunocytochemistry and review of the literature . Cancer . 1983;52:238-245.Crossref 14. Silva EG, Mackay B, Goepfert H, Burgess MA, Fields RS. Endocrine carcinoma of the skin (Merkel cell carcinoma) . Pathol Annu . 1984;19:1-30. 15. Wick MR, Goellner JR, Scheithauer BW, Thomas JR, Sanchez NP, Schroeter AL: Primary neuroendocrine carcinomas of the skin (Merkel cell tumors): a clinical, histologic, and ultrastructural study of thirteen cases . Am J Clin Pathol . 1983;79:6-13. 16. Gould V, Moll R, Moll I, Lee I, Franke W. Biology of disease: neuroendocrine (Merkel) cells of the skin: hyperplasias, dysplasias, and neoplasms . Lab Invest . 1985;52:334-353. 17. Shaw JHF, Rumbull E. Merkel cell tumor: clinical behavior and treatment . Br J Surg . 1991;78:138-142.Crossref 18. Balch CM. The role of elective lymph node dissection in melanoma: rationale, results, and controversies . J Clin Oncol . 1988;6:163-172. 19. Schaefer GD, Erlandson RA, Urmacher C. A cutaneous neoplasm in the skin of the abdomen that was excised and was followed by the development of bilateral axillary masses . Am J Dermatopathol . 1982;4:549-554.Crossref 20. Feun LG, Savaraj N, Legha SS, Silva EG, Benjamin RS, Burgess MA. Chemotherapy for metastatic Merkel cell carcinoma: review of the M.D. Anderson Hospital's experience . Cancer . 1988;62:683-685.Crossref 21. Wynne CJ, Kearsley JH. Merkel cell tumor: a chemosensitive skin cancer . Cancer . 1988;62:28-31.Crossref 22. Marks ME, Kim RY, Salter MM. Radiotherapy as an adjunct in the management of Merkel cell carcinoma . Cancer . 1990;65:60-64.Crossref 23. Knox SJ, Kapp DS. Hyperthermia and radiation therapy in the treatment of recurrent Merkel cell tumors . Cancer . 1988;62:1479-1486.Crossref 24. Pacella J, Ashby M, Ainslie J, Minty C. The role of radiotherapy in the management of primary cutaneous neuroendocrine tumors (Merkel cell or trabecular carcinoma): experience at the Peter MacCallum Cancer Institute (Melbourne, Australia) . Int J Radiat Oncol Biol Phys . 1988;14:1077-1084.Crossref 25. Taxy JB, Ettinger DS, Wharam MD. Primary small cell carcinoma of the skin . Cancer . 1980;46:2308-2311.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

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References (28)

Publisher
American Medical Association
Copyright
Copyright © 1991 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1991.01410360088014
Publisher site
See Article on Publisher Site

Abstract

Abstract • Seventy patients with Merkel cell carcinoma were treated at Memorial Sloan-Kettering Cancer Center between 1969 and 1989. The overall estimated 5-year survival rate was 64%. Factors predictive of improved survival included head and neck site and negative lymph nodes at presentation. Local recurrence was seen in 18 patients (26%) and did not correlate with patient-, tumor-, or treatment-related variables. Nine patients with local recurrence (50%) were free of disease following aggressive reoperation. Regional nodes were involved at some point during the course of the disease in forty-six patients (66%). Regional lymph node involvement was apparent within 2 years of diagnosis in 40 (87%) of 46 patients in whom it occurred. Systemic disease was nearly uniformly preceded by the appearance of nodal metastases and was uniformly fatal regardless of subsequent therapy. This suggests an orderly "cascade" pattern of spread for this tumor, in which elective regional lymph node dissection may be justified. Our recommendations for treatment include a wide excision of the primary tumor and either elective or early therapeutic regional node dissection. The role of adjuvant radiotherapy or chemotherapy remains unproven. (Arch Surg. 1991;126:1514-1519) References 1. Toker C. Trabecular carcinoma of the skin . Arch Dermatol . 1972;105:107-110.Crossref 2. Tang CK, Toker C. Trabecular carcinoma of the skin: an ultrastructural study . Cancer . 1978;42:2311-2321.Crossref 3. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations . J Am Stat Assoc . 1958;53:457-481.Crossref 4. Cox DR, Oakes D. Analysis of Survival Data . London, England: Chapman & Hall; 1984. 5. De Wolf-Peeter C, Marien K, Mebis J, Desmet V. A cutaneous APUDoma or Merkel cell tumor? a morphologically recognizable tumor with a biological and histological malignant aspect in contrast with its clinical behaviour . Cancer . 1980;46:1810-1816.Crossref 6. Szadowska A, Wozniak L, Lasota J, Giryn I, Mirecka B, Wolska H. Neuroendocrine (Merkel cell) carcinoma of the skin: a clinico-morphological study of 13 cases . Histopathology . 1989;15:483-493.Crossref 7. Goepfert H, Remmler D, Silva EG, Wheeler B. Merkel cell carcinoma (endocrine carcinoma of the skin) of the head and neck . Arch Otolaryngol Head Neck Surg . 1984;110:707-712.Crossref 8. Raaf JH, Urmacher C, Knapper WK, Shiu MH, Cheng EWK. Trabecular (Merkel cell) carcinoma of the skin: treatment of primary, recurrent, and metastatic disease . Cancer . 1986;57:178-182.Crossref 9. Pilotti S, Rilke F, Bartoli C, Grisotti A. Clinicopathologic correlations of cutaneous neuroendocrine Merkel cell carcinoma . J Clin Oncol . 1988;6:1863-1873. 10. Hitchcock CL, Bland KI, Lahey RG, Franzini D, Harris B, Copeland EM. Neuroendocrine (Merkel cell) carcinoma of the skin: its natural history, diagnosis, and treatment . Ann Surg . 1988;207:201-207.Crossref 11. Tang CK, Toker C. Trabecular carcinoma of the skin: further clinicopathologic and ultrastructural study . Mt Sinai J Med . 1979;46:516-523. 12. Sibley RK, Rosai J, Foucar E, Dehner LP, Bosi G. Neuroendocrine (Merkel cell) carcinoma of the skin: a histologic and ultrastructural study of two cases . Am J Surg Pathol . 1980;4:211-221.Crossref 13. Warner TFCS, Uno H, Hafez GR, et al. Merkel cells and Merkel cell tumors: ultrastructural, immunocytochemistry and review of the literature . Cancer . 1983;52:238-245.Crossref 14. Silva EG, Mackay B, Goepfert H, Burgess MA, Fields RS. Endocrine carcinoma of the skin (Merkel cell carcinoma) . Pathol Annu . 1984;19:1-30. 15. Wick MR, Goellner JR, Scheithauer BW, Thomas JR, Sanchez NP, Schroeter AL: Primary neuroendocrine carcinomas of the skin (Merkel cell tumors): a clinical, histologic, and ultrastructural study of thirteen cases . Am J Clin Pathol . 1983;79:6-13. 16. Gould V, Moll R, Moll I, Lee I, Franke W. Biology of disease: neuroendocrine (Merkel) cells of the skin: hyperplasias, dysplasias, and neoplasms . Lab Invest . 1985;52:334-353. 17. Shaw JHF, Rumbull E. Merkel cell tumor: clinical behavior and treatment . Br J Surg . 1991;78:138-142.Crossref 18. Balch CM. The role of elective lymph node dissection in melanoma: rationale, results, and controversies . J Clin Oncol . 1988;6:163-172. 19. Schaefer GD, Erlandson RA, Urmacher C. A cutaneous neoplasm in the skin of the abdomen that was excised and was followed by the development of bilateral axillary masses . Am J Dermatopathol . 1982;4:549-554.Crossref 20. Feun LG, Savaraj N, Legha SS, Silva EG, Benjamin RS, Burgess MA. Chemotherapy for metastatic Merkel cell carcinoma: review of the M.D. Anderson Hospital's experience . Cancer . 1988;62:683-685.Crossref 21. Wynne CJ, Kearsley JH. Merkel cell tumor: a chemosensitive skin cancer . Cancer . 1988;62:28-31.Crossref 22. Marks ME, Kim RY, Salter MM. Radiotherapy as an adjunct in the management of Merkel cell carcinoma . Cancer . 1990;65:60-64.Crossref 23. Knox SJ, Kapp DS. Hyperthermia and radiation therapy in the treatment of recurrent Merkel cell tumors . Cancer . 1988;62:1479-1486.Crossref 24. Pacella J, Ashby M, Ainslie J, Minty C. The role of radiotherapy in the management of primary cutaneous neuroendocrine tumors (Merkel cell or trabecular carcinoma): experience at the Peter MacCallum Cancer Institute (Melbourne, Australia) . Int J Radiat Oncol Biol Phys . 1988;14:1077-1084.Crossref 25. Taxy JB, Ettinger DS, Wharam MD. Primary small cell carcinoma of the skin . Cancer . 1980;46:2308-2311.Crossref

Journal

Archives of SurgeryAmerican Medical Association

Published: Dec 1, 1991

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