eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pathology

Skin Cancer: Merkel Cell Carcinoma: Treatment

Author: Michael J Pitman, MD, Assistant Professor, New York Medical College; Director, The Voice and Swallowing Institute; Director, Division of Laryngology, Department of Otolaryngology, New York Eye and Ear Infirmary
Coauthor(s): James M Pearson, MD, Staff Physician, Department of Otolaryngology - Head and Neck Surgery, New York Eye and Ear Infirmary; James D Williams, MD, FACS, Consulting Staff, Department of Ear, Nose and Throat, Granville Medical Center
Contributor Information and Disclosures

Updated: Nov 6, 2008

Treatment

Surgical Therapy

For patients with operable disease, most agree that surgery is the treatment of choice. As with most tumors, treatment is based on the stage. The Yiengpruksawan system is used here.5

Stage I

Aggressive wide local excision is the treatment of choice for the primary tumor. A 2- to 3-cm margin is recommended because it is thought to decrease the risk of recurrence. All margins should be confirmed with frozen sections.

The role of elective neck dissection in the absence of clinically positive nodes is controversial. Because of the low incidence of Merkel cell carcinoma (MCC), most reports are based on retrospective or anecdotal studies. Lymph node metastases develop in approximately 55% of patients; therefore, some authors recommend prophylactic neck dissection in all patients. Others recommend neck dissection after the tumor reaches 2 cm in diameter.

Silva et al (1984) recommend neck dissection for tumors with 10 or more mitoses per high-power field, for cases of lymphatic invasion, or for tumors composed of small cells.6 Cotlar et al (1986) recommend lymphadenectomy for tumors present longer than 6 weeks.7 Tumors in the midline present the problem of bilateral drainage. Goepfert et al (1984) and Hitchcock et al (1988) recommend that patients with such lesions undergo a bilateral neck dissection.8,9

Recent studies have been conducted to investigate the efficacy of sentinel node biopsy to determine whether neck dissection is necessary. These studies have been small, but their results indicate that a negative sentinel node may obviate the need for neck dissection.

When one contemplates neck dissection, realize that no indications are universally accepted and that the effect of regional lymphadenopathy on overall survival is uncertain.

Merkel cell carcinoma (MCC) is a radiosensitive tumor, and radiotherapy is currently used as an adjuvant. Most clinical studies demonstrate better local control rates with adjuvant radiotherapy after surgery. Numerous authors have recommended postexcision irradiation of the primary site and primary areas of lymph node drainage. Adjuvant radiotherapy is routinely given by some practitioners, whereas other practitioners only give it to patients considered to be high risk, which is inconsistently defined as demonstrating any of the following: (1) primary tumor >1.5 cm; (2) positive margins; (3) margins <2 mm; (4) evidence of lymphatic, vascular, or perineural invasion; or (5) regional lymph node involvement.

The recommended dosing schedule is 45-50 Gy for 5 weeks; this is increased to 56-65 Gy for tumors with positive margins. These doses are similar to those used to treat squamous cell carcinoma. Therapy for recurrent localized disease or extensive unresectable stage I disease is similar to that given for stage II disease because lymphadenopathy is more likely to occur in this situation than in primary disease.

Stage II

A widely accepted practice is for patients with regional node metastases or local or regional recurrence to undergo excision of the primary lesion and lymph node dissection. Adjuvant radiation therapy to the primary site and regional nodes is generally recommended in addition to neck dissection.

Chemotherapy is generally not currently advocated for stage II disease.

Stage III

The development of distant metastases portends a poor prognosis, with a mean life expectancy of 5 months. Many types of chemotherapeutic agents have been used with brief success in treating stage III disease, with no increase in the survival rate. Because of the morphologic and immunohistochemical similarities of Merkel cell carcinoma (MCC) to small cell lung cancer, these regimens have been used as treatment models.

Pharmacologic agents most commonly used are doxorubicin and cyclophosphamide. Other agents are cisplatin, vincristine, etoposide, methotrexate, bleomycin, and 5-fluorouracil. However, reports of these treatments to date have consisted of small studies and anecdotal evidence. Merkel cell carcinomas (MCCs) often respond to chemotherapy; however, as with small cell carcinoma, remission is brief. No chemotherapeutic protocol has notably increased survival rates.

The role of radiation therapy in disseminated disease is to achieve palliation.

Follow-up

After treatment, monitor patients closely. Recommended follow-up is every month for 6 months, every 3 months for the next 2 years, and every 6 months thereafter.

More on Skin Cancer: Merkel Cell Carcinoma

Overview: Skin Cancer: Merkel Cell Carcinoma
Workup: Skin Cancer: Merkel Cell Carcinoma
Treatment: Skin Cancer: Merkel Cell Carcinoma
Follow-up: Skin Cancer: Merkel Cell Carcinoma
Multimedia: Skin Cancer: Merkel Cell Carcinoma
References
[ CLOSE WINDOW ]

References

  1. Toker C. Trabecular carcinoma of the skin. Arch Dermatol. Jan 1972;105(1):107-10. [Medline].

  2. Hodgson NC. Merkel cell carcinoma: changing incidence trends. J Surg Oncol. Jan 1 2005;89(1):1-4. [Medline].

  3. Yao M, Smith RB, Hoffman HT, et al. Merkel cell carcinoma: two case reports focusing on the role of fluorodeoxyglucose positron emission tomography imaging in staging and surveillance. Am J Clin Oncol. Apr 2005;28(2):205-10. [Medline].

  4. Gould VE, Moll R, Moll I, et al. Neuroendocrine (Merkel) cells of the skin: hyperplasias, dysplasias, and neoplasms. Lab Invest. Apr 1985;52(4):334-53. [Medline].

  5. Yiengpruksawan A, Coit DG, Thaler HT, et al. Merkel cell carcinoma. Prognosis and management. Arch Surg. Dec 1991;126(12):1514-9. [Medline].

  6. Silva EG, Mackay B, Goepfert H, et al. Endocrine carcinoma of the skin (Merkel cell carcinoma). Pathol Annu. 1984;19 Pt 2:1-30. [Medline].

  7. Cotlar AM, Gates JO, Gibbs FA Jr. Merkel cell carcinoma: combined surgery and radiation therapy. Am Surg. Mar 1986;52(3):159-64. [Medline].

  8. Goepfert H, Remmler D, Silva E, et al. Merkel cell carcinoma (endocrine carcinoma of the skin) of the head and neck. Arch Otolaryngol. Nov 1984;110(11):707-12. [Medline].

  9. Hitchcock CL, Bland KI, Laney RG 3d, et al. Neuroendocrine (Merkel cell) carcinoma of the skin. Its natural history, diagnosis, and treatment. Ann Surg. Feb 1988;207(2):201-7. [Medline].

  10. O'Connor WJ, Roenigk RK, Brodland DG. Merkel cell carcinoma. Comparison of Mohs micrographic surgery and wide excision in eighty-six patients. Dermatol Surg. Oct 1997;23(10):929-33. [Medline].

  11. Schmalbach CE, Lowe L, Teknos TN, et al. Reliability of sentinel lymph node biopsy for regional staging of head and neck Merkel cell carcinoma. Arch Otolaryngol Head Neck Surg. Jul 2005;131(7):610-4. [Medline].

  12. Agelli M, Clegg LX. Epidemiology of primary Merkel cell carcinoma in the United States. J Am Acad Dermatol. Nov 2003;49(5):832-41. [Medline].

  13. Ames SE, Krag DN, Brady MS. Radiolocalization of the sentinel lymph node in Merkel cell carcinoma: a clinical analysis of seven cases. J Surg Oncol. Apr 1998;67(4):251-4. [Medline].

  14. Boyer JD, Zitelli JA, Brodland DG, et al. Local control of primary Merkel cell carcinoma: review of 45 cases treated with Mohs micrographic surgery with and without adjuvant radiation. J Am Acad Dermatol. Dec 2002;47(6):885-92. [Medline].

  15. Crown J, Lipzstein R, Cohen S, et al. Chemotherapy of metastatic Merkel cell cancer. Cancer Invest. 1991;9(2):129-32. [Medline].

  16. Ecker HA Jr, Abt AB, Graham WP 3rd, et al. Trabecular or Merkel-cell carcinoma of the skin. Plast Reconstr Surg. Oct 1982;70(4):485-9. [Medline].

  17. Eng TY, Boersma MG, Fuller CD, et al. A comprehensive review of the treatment of Merkel cell carcinoma. Am J Clin Oncol. Dec 2007;30(6):624-36. [Medline].

  18. Fenig E, Lurie H, Klein B, et al. The treatment of advanced Merkel cell carcinoma. A multimodality chemotherapy and radiation therapy treatment approach. J Dermatol Surg Oncol. Sep 1993;19(9):860-4. [Medline].

  19. Feun LG, Savaraj N, Legha SS, et al. Chemotherapy for metastatic Merkel cell carcinoma. Review of the M.D. Anderson Hospital's experience. Cancer. Aug 15 1988;62(4):683-5. [Medline].

  20. Gomez LG, DiMaio S, Silva EG, et al. Association between neuroendocrine (Merkel cell) carcinoma and squamous carcinoma of the skin. Am J Surg Pathol. Mar 1983;7(2):171-7. [Medline].

  21. Haag ML, Glass LF, Fenske NA. Merkel cell carcinoma. Diagnosis and treatment. Dermatol Surg. Aug 1995;21(8):669-83. [Medline].

  22. Heenan PJ, Cole JM, Spagnolo DV. Primary cutaneous neuroendocrine carcinoma (Merkel cell tumor). An adnexal epithelial neoplasm. Am J Dermatopathol. Feb 1990;12(1):7-16. [Medline].

  23. Henness S, Vereecken P. Management of Merkel tumours: an evidence-based review. Curr Opin Oncol. May 2008;20(3):280-6. [Medline].

  24. Hill AD, Brady MS, Coit DG. Intraoperative lymphatic mapping and sentinel lymph node biopsy for Merkel cell carcinoma. Br J Surg. Apr 1999;86(4):518-21. [Medline].

  25. Koljonen VS. Merkel cell carcinoma. World J Surg Oncol. Feb 8 2006;4(1):7. [Medline].

  26. Kuhajda FP, Olson JL, Mann RB. Merkel cell (small cell) carcinoma of the skin: immunohistochemical and ultrastructural demonstration of distinctive perinuclear cytokeratin aggregates and a possible association with B cell neoplasms. Histochem J. May 1986;18(5):239-44. [Medline].

  27. Kwekkeboom DJ, Hoff AM, Lamberts SW, et al. Somatostatin analogue scintigraphy. A simple and sensitive method for the in vivo visualization of Merkel cell tumors and their metastases. Arch Dermatol. Jun 1992;128(6):818-21. [Medline].

  28. Lawenda BD, Thiringer JK, Foss RD, et al. Merkel cell carcinoma arising in the head and neck: optimizing therapy. Am J Clin Oncol. Feb 2001;24(1):35-42. [Medline].

  29. Mendenhall WM, Mendenhall CM, Mendenhall NP. Merkel cell carcinoma. Laryngoscope. May 2004;114(5):906-10. [Medline].

  30. Merkel F. Stzellen und Tastkorperchen bei den Haustieren und beim Menschen. Arkiv Mikroskopische Anatomie Entwicklungsmechanik. 1875;11:636-52.

  31. Messina JL, Reintgen DS, Cruse CW, et al. Selective lymphadenectomy in patients with Merkel cell (cutaneous neuroendocrine) carcinoma. Ann Surg Oncol. Jul-Aug 1997;4(5):389-95. [Medline].

  32. O'Brien CJ, Uren RF, Thompson JF, et al. Prediction of potential metastatic sites in cutaneous head and neck melanoma using lymphoscintigraphy. Am J Surg. Nov 1995;170(5):461-6. [Medline].

  33. O'Connor WJ, Brodland DG. Merkel cell carcinoma. Dermatol Surg. Mar 1996;22(3):262-7. [Medline].

  34. Pan D, Narayan D, Ariyan S. Merkel cell carcinoma: five case reports using sentinel lymph node biopsy and a review of 110 new cases. Plast Reconstr Surg. Oct 2002;110(5):1259-65. [Medline].

  35. Pilotti S, Rilke F, Bartoli C, et al. Clinicopathologic correlations of cutaneous neuroendocrine Merkel cell carcinoma. J Clin Oncol. Dec 1988;6(12):1863-73. [Medline].

  36. Pitale M, Sessions RB, Husain S. An analysis of prognostic factors in cutaneous neuroendocrine carcinoma. Laryngoscope. Mar 1992;102(3):244-9. [Medline].

  37. Rice RD Jr, Chonkich GD, Thompson KS, et al. Merkel cell tumor of the head and neck. Five new cases with literature review. Arch Otolaryngol Head Neck Surg. Jul 1993;119(7):782-6. [Medline].

  38. Sharma D, Flora G, Grunberg SM. Chemotherapy of metastatic Merkel cell carcinoma: case report and review of the literature. Am J Clin Oncol. Apr 1991;14(2):166-9. [Medline].

  39. Stein JM, Hrabovsky S, Schuller DE, et al. Mohs micrographic surgery and the otolaryngologist. Am J Otolaryngol. Nov-Dec 2004;25(6):385-93. [Medline].

  40. Straka JA, Straka MB. A review of Merkel cell carcinoma with emphasis on lymph node disease in the absence of a primary site. Am J Otolaryngol. Jan-Feb 1997;18(1):55-65. [Medline].

  41. Suarez C, Rodrigo JP, Ferlito A, et al. Merkel cell carcinoma of the head and neck. Oral Oncol. Sep 2004;40(8):773-9. [Medline].

  42. Tai P. Merkel cell cancer: update on biology and treatment. Curr Opin Oncol. Mar 2008;20(2):196-200. [Medline].

  43. Tennvall J, Biörklund A, Johansson L, et al. Merkel cell carcinoma: management of primary, recurrent and metastatic disease. A clinicopathological study of 17 patients. Eur J Surg Oncol. Feb 1989;15(1):1-9. [Medline].

  44. Tuneu A, Pujol RM, Moreno A, et al. Postirradiation Merkel cell carcinoma. J Am Acad Dermatol. Mar 1989;20(3):505-7. [Medline].

  45. Veness MJ, Palme CE, Morgan GJ. Merkel cell carcinoma: a review of management. Curr Opin Otolaryngol Head Neck Surg. Apr 2008;16(2):170-4. [Medline].

  46. Wanebo HJ, Harpole D, Teates CD. Radionuclide lymphoscintigraphy with technetium 99m antimony sulfide colloid to identify lymphatic drainage of cutaneous melanoma at ambiguous sites in the head and neck and trunk. Cancer. Mar 15 1985;55(6):1403-13. [Medline].

  47. Wasserberg N, Schachter J, Fenig E, et al. Applicability of the sentinel node technique to Merkel cell carcinoma. Dermatol Surg. Feb 2000;26(2):138-41. [Medline].

  48. Wells KE, Cruse CW, Daniels S, et al. The use of lymphoscintigraphy in melanoma of the head and neck. Plast Reconstr Surg. Apr 1994;93(4):757-61. [Medline].

  49. Wilder RB, Harari PM, Graham AR, et al. Merkel cell carcinoma. Improved locoregional control with postoperative radiation therapy. Cancer. Sep 1 1991;68(5):1004-8. [Medline].

  50. Zeitouni NC, Cheney RT, Delacure MD. Lymphoscintigraphy, sentinel lymph node biopsy, and Mohs micrographic surgery in the treatment of Merkel cell carcinoma. Dermatol Surg. Jan 2000;26(1):12-8. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

carcinoma, Merkel, Merkel cell carcinoma, Merkel cell carcinoma of the skin, MCC, trabecular carcinoma of the skin, neuroendocrine carcinoma of the skin, cutaneous neoplasm, ectodermal dysplasia, Bowen disease, squamous cell carcinoma, basal cell carcinoma, solar keratoses, lentigo maligna, B cell lymphoma, radiation exposure, keratoacanthoma, amelanotic melanoma, epidermal cysts, lymphoma, metastatic carcinoma of the skin, skin cancer, Merkel cell, skin tumor, metastatic oat cell carcinoma, lymphadenopathy, Mohs surgery, neck dissection, Merkel cell cancer

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Michael J Pitman, MD, Assistant Professor, New York Medical College; Director, The Voice and Swallowing Institute; Director, Division of Laryngology, Department of Otolaryngology, New York Eye and Ear Infirmary
Michael J Pitman, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, and Voice Foundation
Disclosure: Nothing to disclose.

Coauthor(s)

James M Pearson, MD, Staff Physician, Department of Otolaryngology - Head and Neck Surgery, New York Eye and Ear Infirmary
Disclosure: Nothing to disclose.

James D Williams, MD, FACS, Consulting Staff, Department of Ear, Nose and Throat, Granville Medical Center
James D Williams, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

Medical Editor

M Abraham Kuriakose, MD, DDS, FRCS, Chairman, Head and Neck Institute, Amrita Institute of Medical Sciences
M Abraham Kuriakose, MD, DDS, FRCS is a member of the following medical societies: American Association for Cancer Research, American Head and Neck Society, British Association of Oral and Maxillofacial Surgeons, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

M Sherif Said, MD, PhD, Assistant Professor of Pathology, Director of Head and Neck Pathology, Department of Pathology, University of Colorado Health Sciences Center
M Sherif Said, MD, PhD is a member of the following medical societies: American Medical Association, American Society for Clinical Pathologists, College of American Pathologists, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.