Skip to main content
Top
Published in: World Journal of Surgical Oncology 1/2008

Open Access 01-12-2008 | Case report

Merkel cell carcinoma of the upper extremity: Case report and an update

Authors: Michail Papamichail, Ioannis Nikolaidis, Nicolas Nikolaidis, Chryssoula Glava, Ioannis Lentzas, Konstantinos Marmagkiolis, Kriton Karassavsa, Michail Digalakis

Published in: World Journal of Surgical Oncology | Issue 1/2008

Login to get access

Abstract

Background

Merkel cell carcinoma is a rare but aggressive cutaneous primary small cell carcinoma. It is commonly seen in elderly affecting the head, neck, and extremities. Macroscopically may be difficult to distinguish MCC from other small cells neoplasms especially oat cell carcinoma of the lung.

Case presentation

It is presented a case report concerning a 72 years old male with a MMC on the dorsal aspect of the right wrist. The patient underwent a diagnostic excisional biopsy and after the histological confirmation of the diagnosis a second excision was performed to achieve free margins. No postoperative radiation or adjuvant chemotherapy was given and within 9 years follow up no recurrence was reported.

Conclusion

Although most cases present as localized disease treatment should be definitive due to high rates of local or systemic recurrence. Treatment includes excision of the lesion, lymphadenectomy, postoperative radiotherapy and chemotherapy depending on the stage of the disease. Even when locoregional control is achieved close surveillance is required due to high rates of relapse.
Appendix
Available only for authorised users
Literature
1.
go back to reference Toker C: Trabecular carcinoma of the skin. Arch Dermatol. 1972, 105: 107-110. 10.1001/archderm.105.1.107.CrossRefPubMed Toker C: Trabecular carcinoma of the skin. Arch Dermatol. 1972, 105: 107-110. 10.1001/archderm.105.1.107.CrossRefPubMed
2.
go back to reference Youker SR, Billingsley EM: Combined Merkel cell carcinoma and atypical fibroxanthoma. J Cutan Med Surg. 2005, 9: 6-9. 10.1007/s10227-005-0024-4.CrossRefPubMed Youker SR, Billingsley EM: Combined Merkel cell carcinoma and atypical fibroxanthoma. J Cutan Med Surg. 2005, 9: 6-9. 10.1007/s10227-005-0024-4.CrossRefPubMed
3.
go back to reference Schwartz RA, Lambert WC: The Merkel cell carcinoma: a 50-year retrospect. J Surg Oncol. 2005, 89: 1-4. 10.1002/jso.20165.CrossRef Schwartz RA, Lambert WC: The Merkel cell carcinoma: a 50-year retrospect. J Surg Oncol. 2005, 89: 1-4. 10.1002/jso.20165.CrossRef
4.
go back to reference McAfee WJ, Morris CG, Mendenhall CM, Werning JW, Mendenhall NP, Mendenhall WM: Merkel cell carcinoma: treatment and outcomes. Cancer. 2005, 15; 104: 1761-1764. 10.1002/cncr.21355.CrossRef McAfee WJ, Morris CG, Mendenhall CM, Werning JW, Mendenhall NP, Mendenhall WM: Merkel cell carcinoma: treatment and outcomes. Cancer. 2005, 15; 104: 1761-1764. 10.1002/cncr.21355.CrossRef
5.
go back to reference Payne MM, Rader AE, McCarthy DM, Rodgers WH: Merkel cell carcinoma in a malignant pleural effusion: case report. Cytojournal. 2004, 18; 1: 5-10.1186/1742-6413-1-5.CrossRef Payne MM, Rader AE, McCarthy DM, Rodgers WH: Merkel cell carcinoma in a malignant pleural effusion: case report. Cytojournal. 2004, 18; 1: 5-10.1186/1742-6413-1-5.CrossRef
7.
go back to reference Dinh V, Feun L, Elgart G, Savaraj N: Merkel cell carcinomas. Hematol Oncol Clin North Am. 2007, 21: 527-544. 10.1016/j.hoc.2007.04.008.CrossRefPubMed Dinh V, Feun L, Elgart G, Savaraj N: Merkel cell carcinomas. Hematol Oncol Clin North Am. 2007, 21: 527-544. 10.1016/j.hoc.2007.04.008.CrossRefPubMed
8.
go back to reference Pectasides D, Pectasides M, Economopoulos T: Merkel cell cancer of the skin. Ann Oncol. 2006, 24: 1489-1495. 10.1093/annonc/mdl050.CrossRef Pectasides D, Pectasides M, Economopoulos T: Merkel cell cancer of the skin. Ann Oncol. 2006, 24: 1489-1495. 10.1093/annonc/mdl050.CrossRef
9.
go back to reference Veness MJ: Merkel cell carcinoma (primary cutaneous neuroendocrine carcinoma): an overview on management. Australas J Dermatol. 2006, 47: 160-165. 10.1111/j.1440-0960.2006.00263.x.CrossRefPubMed Veness MJ: Merkel cell carcinoma (primary cutaneous neuroendocrine carcinoma): an overview on management. Australas J Dermatol. 2006, 47: 160-165. 10.1111/j.1440-0960.2006.00263.x.CrossRefPubMed
10.
go back to reference Boyse K, Foley EH, Bradley V, Scarborough D: Merkel cell carcinoma: a case report with treatment summary and updates. Cutis. 2004, 74: 350-356.PubMed Boyse K, Foley EH, Bradley V, Scarborough D: Merkel cell carcinoma: a case report with treatment summary and updates. Cutis. 2004, 74: 350-356.PubMed
11.
go back to reference Bichakjian CK, Lowe L, Lao CD, Sandler HM, Bradford CR, Johnson TM, Wong SL: Merkel cell carcinoma: critical review with guidelines for multidisciplinary management. Cancer. 2007, 110: 1-12. 10.1002/cncr.22765.CrossRefPubMed Bichakjian CK, Lowe L, Lao CD, Sandler HM, Bradford CR, Johnson TM, Wong SL: Merkel cell carcinoma: critical review with guidelines for multidisciplinary management. Cancer. 2007, 110: 1-12. 10.1002/cncr.22765.CrossRefPubMed
12.
go back to reference Rosai J: Rosai and Ackerman's Surgical Pathology. Mosby. 2004, 1: 177-179. 9 Rosai J: Rosai and Ackerman's Surgical Pathology. Mosby. 2004, 1: 177-179. 9
13.
go back to reference Bayrou O, Avril MF, Charpentier P, Caillou B, Guillaume JC, Prade M: Primary neuroendocrine carcinoma of the skin. Clinicopathologic study of 18 cases. J Am Acad Dermatol. 1991, 24: 198-207.CrossRefPubMed Bayrou O, Avril MF, Charpentier P, Caillou B, Guillaume JC, Prade M: Primary neuroendocrine carcinoma of the skin. Clinicopathologic study of 18 cases. J Am Acad Dermatol. 1991, 24: 198-207.CrossRefPubMed
14.
go back to reference Sidhu GS, Feiner TJ, Mullins JD, Schaefler K, Schultenhover SJ: Merkel cell Neoplasms. Histology, electron microscopy, biology and histogenesis. Am J Dermatopathol. 1980, 2: 101-119. 10.1097/00000372-198000220-00001.CrossRefPubMed Sidhu GS, Feiner TJ, Mullins JD, Schaefler K, Schultenhover SJ: Merkel cell Neoplasms. Histology, electron microscopy, biology and histogenesis. Am J Dermatopathol. 1980, 2: 101-119. 10.1097/00000372-198000220-00001.CrossRefPubMed
15.
go back to reference Walsh NM: Primary neuroendocrine (Merkel cell) carcinoma of the skin. Morphologic diversity and implications thereof. Hum Pathol. 2001, 32: 680-689. 10.1053/hupa.2001.25904.CrossRefPubMed Walsh NM: Primary neuroendocrine (Merkel cell) carcinoma of the skin. Morphologic diversity and implications thereof. Hum Pathol. 2001, 32: 680-689. 10.1053/hupa.2001.25904.CrossRefPubMed
16.
go back to reference Gaudin PB, Rosai J: Florid vascular proliferation associated with neural and neuroendocrine neoplasms: a diagnostic clue and potential pitfall. Am J Surg Pathol. 1995, 19: 642-652.CrossRefPubMed Gaudin PB, Rosai J: Florid vascular proliferation associated with neural and neuroendocrine neoplasms: a diagnostic clue and potential pitfall. Am J Surg Pathol. 1995, 19: 642-652.CrossRefPubMed
17.
go back to reference Scott MP, Helm KF: Cytokeratin 20: a marker for diagnosing Merkel cell carcinoma. Am J Dermatopathol. 1999, 21: 16-20. 10.1097/00000372-199902000-00003.CrossRefPubMed Scott MP, Helm KF: Cytokeratin 20: a marker for diagnosing Merkel cell carcinoma. Am J Dermatopathol. 1999, 21: 16-20. 10.1097/00000372-199902000-00003.CrossRefPubMed
18.
go back to reference Wick MR, Scheithauer BW, Kovacs K: Neuron-specific enolase in neuroendocrine tumours of the thymus bronchus and skin. Am J Clin Pathol. 1983, 79: 703-707.PubMed Wick MR, Scheithauer BW, Kovacs K: Neuron-specific enolase in neuroendocrine tumours of the thymus bronchus and skin. Am J Clin Pathol. 1983, 79: 703-707.PubMed
19.
go back to reference Brinkschmidt C, Stolze P, Fahrenkamp AG, Hundeiker M, Fisher-Colbrie R, Zelger B, Bocker W, Schmid KW: Immunohistochemical demonstration of cromogranin A, chromogranin B, and secretoneurin in Merkel cell carcinoma of the skin. An immunohistochemical study on 18 cases suggesting two types of Merkel cell carcinoma. Appl Immunohistochem. 1995, 3: 37-44. Brinkschmidt C, Stolze P, Fahrenkamp AG, Hundeiker M, Fisher-Colbrie R, Zelger B, Bocker W, Schmid KW: Immunohistochemical demonstration of cromogranin A, chromogranin B, and secretoneurin in Merkel cell carcinoma of the skin. An immunohistochemical study on 18 cases suggesting two types of Merkel cell carcinoma. Appl Immunohistochem. 1995, 3: 37-44.
20.
go back to reference Haneke E, Schulze HJ, Mahrle G: Immunohistochemical and immunoelectron microscopic demonstration of chromogranin A in formalin-fixed tissue of Merkel cell carcinoma. J Am Acad Dermatol. 1993, 28: 222-226.CrossRefPubMed Haneke E, Schulze HJ, Mahrle G: Immunohistochemical and immunoelectron microscopic demonstration of chromogranin A in formalin-fixed tissue of Merkel cell carcinoma. J Am Acad Dermatol. 1993, 28: 222-226.CrossRefPubMed
21.
go back to reference Layfield L, Ulich T, Liao S, Barr R, Cheng L, Lewin KL: Neuroendocrine carcinoma of the skin. An immunohistochemical study of tumour markers and neuroendocrine products. J Cutan Pathol. 1986, 13: 268-273. 10.1111/j.1600-0560.1986.tb01522.x.CrossRefPubMed Layfield L, Ulich T, Liao S, Barr R, Cheng L, Lewin KL: Neuroendocrine carcinoma of the skin. An immunohistochemical study of tumour markers and neuroendocrine products. J Cutan Pathol. 1986, 13: 268-273. 10.1111/j.1600-0560.1986.tb01522.x.CrossRefPubMed
22.
go back to reference Sibley RK, Dahl D: Primary neuroendocrine (Merkel cell?) carcinoma of the skin. II. An immunohistochemical study of 21 cases. Am J Surg Pathol. 1985, 9: 109-116. 10.1097/00000478-198502000-00005.CrossRefPubMed Sibley RK, Dahl D: Primary neuroendocrine (Merkel cell?) carcinoma of the skin. II. An immunohistochemical study of 21 cases. Am J Surg Pathol. 1985, 9: 109-116. 10.1097/00000478-198502000-00005.CrossRefPubMed
23.
go back to reference Silva EG, Ordóòez NG, Lechago J: Immunohistochemical studies in neuroendocrine carcinoma of the skin. Am J Clin Pathol. 1984, 81: 558-562.PubMed Silva EG, Ordóòez NG, Lechago J: Immunohistochemical studies in neuroendocrine carcinoma of the skin. Am J Clin Pathol. 1984, 81: 558-562.PubMed
24.
go back to reference Su LD, Fullen DR, Lowe L, Uherova P, Schnitzer B, Valder R: CD117 (KIT receptor) expression in Merkel cell carcinoma. Am J Dermatopathol. 2002, 24: 289-293. 10.1097/00000372-200208000-00001.CrossRefPubMed Su LD, Fullen DR, Lowe L, Uherova P, Schnitzer B, Valder R: CD117 (KIT receptor) expression in Merkel cell carcinoma. Am J Dermatopathol. 2002, 24: 289-293. 10.1097/00000372-200208000-00001.CrossRefPubMed
25.
go back to reference Byrd-Gloster AL, Khoor A, Glass LF, Messina JL, Whitsett JA, Livingstone SK, Cagle PT: Differential expression of Thyroid Trascription Factor – 1 in small cell lung carcinoma and Merkel cell tumor. Hum Pathol. 2000, 31: 58-62. 10.1016/S0046-8177(00)80199-9.CrossRefPubMed Byrd-Gloster AL, Khoor A, Glass LF, Messina JL, Whitsett JA, Livingstone SK, Cagle PT: Differential expression of Thyroid Trascription Factor – 1 in small cell lung carcinoma and Merkel cell tumor. Hum Pathol. 2000, 31: 58-62. 10.1016/S0046-8177(00)80199-9.CrossRefPubMed
26.
go back to reference Chan JK, Suster S, Wenig BM, Tsang WY, Chan JB, Lau AL: Cytokeratin 20 immunohistochemistry distinguishes Merkel cell (primary cutaneous neuroendocrine) carcinomas and salivary gland small cell carcinomas from small cell carcinomas of various sites. Am J Surg Pathol. 1997, 21: 226-234. 10.1097/00000478-199702000-00014.CrossRefPubMed Chan JK, Suster S, Wenig BM, Tsang WY, Chan JB, Lau AL: Cytokeratin 20 immunohistochemistry distinguishes Merkel cell (primary cutaneous neuroendocrine) carcinomas and salivary gland small cell carcinomas from small cell carcinomas of various sites. Am J Surg Pathol. 1997, 21: 226-234. 10.1097/00000478-199702000-00014.CrossRefPubMed
27.
go back to reference Cheuk W, Kwan MY, Suster S, Chan JK: Immunostaining for Thyroid Trascription Factor – 1 and cytokeratin 20 aids the distinction of small cell carcinoma from Merkel cell carcinoma, but not pulmonary from extrapulmonary small cell carcinomas. Arch Pathol Lab Med. 2001, 125: 228-231.PubMed Cheuk W, Kwan MY, Suster S, Chan JK: Immunostaining for Thyroid Trascription Factor – 1 and cytokeratin 20 aids the distinction of small cell carcinoma from Merkel cell carcinoma, but not pulmonary from extrapulmonary small cell carcinomas. Arch Pathol Lab Med. 2001, 125: 228-231.PubMed
28.
go back to reference Wick MR, Kaye VN, Sibley RK, Tyler R, Frizzera G: Primary neuroendocrine carcinoma and small cell malignant lymphoma of the skin. A discriminant immunohistochemical comparison. J Cutan Pathol. 1986, 13: 347-358. 10.1111/j.1600-0560.1986.tb00469.x.CrossRefPubMed Wick MR, Kaye VN, Sibley RK, Tyler R, Frizzera G: Primary neuroendocrine carcinoma and small cell malignant lymphoma of the skin. A discriminant immunohistochemical comparison. J Cutan Pathol. 1986, 13: 347-358. 10.1111/j.1600-0560.1986.tb00469.x.CrossRefPubMed
29.
go back to reference Rosai J: Rosai and Ackerman's Surgical Pathology. Mosby. 2004, 1: 50-9 Rosai J: Rosai and Ackerman's Surgical Pathology. Mosby. 2004, 1: 50-9
30.
go back to reference Cerroni L, Kerl H: Primary cutaneous neuroendocrine (Merkel cell) carcinoma in association with squamous- and basal-cell carcinoma. Am J Dermatopathol. 1998, 19: 610-613. 10.1097/00000372-199712000-00011.CrossRef Cerroni L, Kerl H: Primary cutaneous neuroendocrine (Merkel cell) carcinoma in association with squamous- and basal-cell carcinoma. Am J Dermatopathol. 1998, 19: 610-613. 10.1097/00000372-199712000-00011.CrossRef
31.
go back to reference Gomez LG, DiMaio S, Silva EG, Mackay B: Association between neuroendocrine (Merkel cell) carcinoma and squamous carcinoma of the skin. Am J Surg Pathol. 1983, 7: 171-177.CrossRefPubMed Gomez LG, DiMaio S, Silva EG, Mackay B: Association between neuroendocrine (Merkel cell) carcinoma and squamous carcinoma of the skin. Am J Surg Pathol. 1983, 7: 171-177.CrossRefPubMed
32.
go back to reference Gould E, Albores-Saavedra J, Dubner N, Smith W, Payne CM: Eccrine and squamous differentiation in Merkel cell carcinoma. An immunohistochemical study. Am J Surg Pathol. 1988, 12: 768-772.CrossRefPubMed Gould E, Albores-Saavedra J, Dubner N, Smith W, Payne CM: Eccrine and squamous differentiation in Merkel cell carcinoma. An immunohistochemical study. Am J Surg Pathol. 1988, 12: 768-772.CrossRefPubMed
33.
go back to reference Heenan PJ, Cole JM, Spagnolo DV: Primary cutaneous neuroendocrine carcinoma (Merkel cell tumour). Immunohistochemical and biochemical analyses. Virchows Arch [A]. 1985, 406: 339-350.CrossRef Heenan PJ, Cole JM, Spagnolo DV: Primary cutaneous neuroendocrine carcinoma (Merkel cell tumour). Immunohistochemical and biochemical analyses. Virchows Arch [A]. 1985, 406: 339-350.CrossRef
34.
go back to reference Eng TY, Boersma MG, Fuller CD, Cavanaugh SX, Valenzuela F, Herman TS: Treatment of merkel cell carcinoma. Am J Clin Oncol. 2004, 27 (5): 510-515. 10.1097/01.coc.0000135567.62750.f4.CrossRefPubMed Eng TY, Boersma MG, Fuller CD, Cavanaugh SX, Valenzuela F, Herman TS: Treatment of merkel cell carcinoma. Am J Clin Oncol. 2004, 27 (5): 510-515. 10.1097/01.coc.0000135567.62750.f4.CrossRefPubMed
35.
go back to reference Eng TY, Naguib M, Fuller CD, Jones WE, Herman TS: Treatment of recurrent Merkel cell carcinoma: an analysis of 46 cases. Am J Clin Oncol. 2004, 27 (6): 576-583. 10.1097/01.coc.0000135926.93116.c7.CrossRefPubMed Eng TY, Naguib M, Fuller CD, Jones WE, Herman TS: Treatment of recurrent Merkel cell carcinoma: an analysis of 46 cases. Am J Clin Oncol. 2004, 27 (6): 576-583. 10.1097/01.coc.0000135926.93116.c7.CrossRefPubMed
36.
go back to reference Senchenkov A, Barnes SA, Moran SL: Predictors of survival and recurrence in the surgical treatment of merkel cell carcinoma of the extremities. J Surg Oncol. 2007, 1;95 (3): 229-234. 10.1002/jso.20647.CrossRef Senchenkov A, Barnes SA, Moran SL: Predictors of survival and recurrence in the surgical treatment of merkel cell carcinoma of the extremities. J Surg Oncol. 2007, 1;95 (3): 229-234. 10.1002/jso.20647.CrossRef
37.
go back to reference Yamana N, Sueyama H, Hamada M: Cardiac metastasis from Merkel cell skin carcinoma. Int J Clin Oncol. 2004, 9 (3): 210-212. 10.1007/s10147-004-0396-2.CrossRefPubMed Yamana N, Sueyama H, Hamada M: Cardiac metastasis from Merkel cell skin carcinoma. Int J Clin Oncol. 2004, 9 (3): 210-212. 10.1007/s10147-004-0396-2.CrossRefPubMed
38.
go back to reference Mehrany K, Otley CC, Weenig RH, Phillips PK, Roenigk RK, Nguyen TH: A meta-analysis of the prognostic significance of sentinel lymph node status in Merkel cell carcinoma. Dermatol Surg. 2002, 28 (2): 113-117. 10.1046/j.1524-4725.2002.02901.x.PubMed Mehrany K, Otley CC, Weenig RH, Phillips PK, Roenigk RK, Nguyen TH: A meta-analysis of the prognostic significance of sentinel lymph node status in Merkel cell carcinoma. Dermatol Surg. 2002, 28 (2): 113-117. 10.1046/j.1524-4725.2002.02901.x.PubMed
39.
go back to reference Dancey AL, Rayatt SS, Soon C, Ilchshyn A, Brown I, Srivastava S: Merkel cell carcinoma: a report of 34 cases and literature review. J Plast Reconstr Aesthet Surg. 2006, 59 (12): 1294-1299. 10.1016/j.bjps.2006.03.044.CrossRefPubMed Dancey AL, Rayatt SS, Soon C, Ilchshyn A, Brown I, Srivastava S: Merkel cell carcinoma: a report of 34 cases and literature review. J Plast Reconstr Aesthet Surg. 2006, 59 (12): 1294-1299. 10.1016/j.bjps.2006.03.044.CrossRefPubMed
40.
go back to reference Allen PJ, Bowne WB, Jaques DP, Brennan MF, Busam K, Coit DG: Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005, 23 (10): 2300-2309. 10.1200/JCO.2005.02.329.CrossRefPubMed Allen PJ, Bowne WB, Jaques DP, Brennan MF, Busam K, Coit DG: Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005, 23 (10): 2300-2309. 10.1200/JCO.2005.02.329.CrossRefPubMed
Metadata
Title
Merkel cell carcinoma of the upper extremity: Case report and an update
Authors
Michail Papamichail
Ioannis Nikolaidis
Nicolas Nikolaidis
Chryssoula Glava
Ioannis Lentzas
Konstantinos Marmagkiolis
Kriton Karassavsa
Michail Digalakis
Publication date
01-12-2008
Publisher
BioMed Central
Published in
World Journal of Surgical Oncology / Issue 1/2008
Electronic ISSN: 1477-7819
DOI
https://doi.org/10.1186/1477-7819-6-32

Other articles of this Issue 1/2008

World Journal of Surgical Oncology 1/2008 Go to the issue