eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pathology

Skin Cancer: Merkel Cell Carcinoma: Follow-up

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

Outcome and Prognosis

Merkel cell carcinoma (MCC) is a deadly disease with a poor outlook for survival. Local recurrence occurs in 44% of patients; multiple local recurrences occur in 15%. These recurrences usually happen within 5 months after the primary lesion is treated. About 15% of patients have palpable nodes at the time of diagnosis. Lymph node metastases eventually develop in 55% of patients, and distant metastases develop in 34%. Most metastases occur before the eighth month after diagnosis.

The areas where metastases are most likely to occur are the liver, bone, brain, and lung. The presence of distant metastases is the only factor that is consistently predictive of the outcome. The mean time to death after the discovery of distant metastases is 5 months. Mortality rates for patients with distant metastases are 75-100%. In patients without distant metastases, mortality rates are 4%.

Future and Controversies

Future directions for the treatment of Merkel cell carcinoma (MCC) that are still under investigation include Mohs surgery for excision of the primary lesion, lymphoscintigraphy, intraoperative mapping of lymph nodes, biopsy of sentinel lymph nodes to treat and stage occult neck disease, as well as to further define the role of chemotherapy.

O'Connor et al (1997) compared the efficacy of Mohs surgery to that of wide local excision.10 Following up 86 patients, they determined that Mohs surgery fared well compared with the standard treatment of wide local excision.

When weighing the choice between wide local excision and Mohs surgery, one must consider the advantage of Mohs surgery because it allows for histologic control of margins of the tumor, minimizing the extent of excision. In the head and neck, conservation of tissue is imperative to preserve vital structures. Considering this information, many authors now advocate Mohs excision in lieu of wide local excision.

In Merkel cell carcinoma (MCC), the prognosis and the treatment of the disease is largely based on the presence or absence of metastases. To facilitate appropriate treatment and staging, the clinician must determine the state of the regional lymphatics in the clinically negative neck. At present, prophylactic neck dissection is advocated, but the procedure entails morbidity. In addition, because drainage patterns in the head and neck are notoriously ambiguous and difficult to predict, which lymph node basins should be dissected is often unclear.

For the treatment of melanomas of the head and neck, preoperative lymphoscintigraphy and intraoperative lymphatic mapping have been used to successfully identify draining lymph node basins and localize the sentinel lymph node for biopsy. Preliminary studies have demonstrated that, in Merkel cell carcinoma (MCC), the status of the sentinel node is predictive of the status of the remaining lymph node basin.

Recent work in this area by Schmalbach et al (2005) supports these preliminary findings.11 Regional failure in the setting of negative findings on sentinel lymph node biopsy was observed in 1 (13%) of 8 patients. This rate of regional recurrence compares favorably with mean rates of regional recurrence reported in the literature. If data from large studies confirm these findings, sentinel lymph node biopsy may provide an accurate and less morbid alternative to neck dissection for the treatment and staging of regional occult neck disease in Merkel cell carcinoma (MCC).

However, elective lymph node dissection may still be warranted in Merkel cell carcinoma (MCC) patients with head and neck primaries because of the complex and unpredictable nature of lymphatic drainage patterns in these areas. The best conclusion at this point is that sentinal node biopsy may allow for more selective lymphadenectomy and may help determine the need for elective radiotherapy to the proper nodal basins.

The role of adjuvant chemotherapy remains unresolved. Palliative chemotherapy is often used for unresectable or recurrent disease. No prospective randomized, case-controlled phase III studies exist to clearly define the role of chemotherapy in the treatment of Merkel cell carcinoma (MCC). Recently, the National Institutes of Health approved a phase II trial of imatinib mesylate (Gleevac) in treating metastatic or unresectable Merkel cell carcinoma (MCC).

 


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.