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December 1991, Vol 126, No. 12 >
ARTICLE | December 1991

Merkel Cell Carcinoma: Title and subTitle BreakPrognosis and Management FREE

Anusak Yiengpruksawan, MD; Daniel G. Coit, MD; Howard T. Thaler, PhD; Carlos Urmacher, MD; William K. Knapper, MD
[+] Author Affiliations

Accepted for publication October 5, 1991.

Presented at the 44th Annual Cancer Symposium of the Society of Surgical Oncology, Orlando, Fla, March 26, 1991.

Reprint requests to Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10021 (Dr Coit).


Arch Surg. 1991;126(12):1514-1519. doi:10.1001/archsurg.1991.01410360088014
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• 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.
2 +
Tang CK, Toker C.  Trabecular carcinoma of the skin: an ultrastructural study . Cancer . 1978;;42:2311-2321.
3 +
Kaplan EL, Meier P.  Nonparametric estimation from incomplete observations . J Am Stat Assoc . 1958;;53:457-481.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
21 +
Wynne CJ, Kearsley JH.  Merkel cell tumor: a chemosensitive skin cancer . Cancer . 1988;;62:28-31.
22 +
Marks ME, Kim RY, Salter MM.  Radiotherapy as an adjunct in the management of Merkel cell carcinoma . Cancer . 1990;;65:60-64.
23 +
Knox SJ, Kapp DS.  Hyperthermia and radiation therapy in the treatment of recurrent Merkel cell tumors . Cancer . 1988;;62:1479-1486.
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.
25 +
Taxy JB, Ettinger DS, Wharam MD.  Primary small cell carcinoma of the skin . Cancer . 1980;;46:2308-2311.

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1 +
Toker C.  Trabecular carcinoma of the skin . Arch Dermatol . 1972;;105:107-110.
2 +
Tang CK, Toker C.  Trabecular carcinoma of the skin: an ultrastructural study . Cancer . 1978;;42:2311-2321.
3 +
Kaplan EL, Meier P.  Nonparametric estimation from incomplete observations . J Am Stat Assoc . 1958;;53:457-481.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
21 +
Wynne CJ, Kearsley JH.  Merkel cell tumor: a chemosensitive skin cancer . Cancer . 1988;;62:28-31.
22 +
Marks ME, Kim RY, Salter MM.  Radiotherapy as an adjunct in the management of Merkel cell carcinoma . Cancer . 1990;;65:60-64.
23 +
Knox SJ, Kapp DS.  Hyperthermia and radiation therapy in the treatment of recurrent Merkel cell tumors . Cancer . 1988;;62:1479-1486.
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.
25 +
Taxy JB, Ettinger DS, Wharam MD.  Primary small cell carcinoma of the skin . Cancer . 1980;;46:2308-2311.

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