Summary
The Merkel cell carcinoma occurs primarily in the skin of the head and neck, and develops in the dermis with a trabecular growth pattern. Immunohistochemistry reveals positive staining for neuron-specific enolase, neurofilaments, cytokeratin and chromogranin A. Electron microscopically, the tumor cells contain dense-core granules, spinous cytoplasmic processes, desmosomes, zonulae adherentes and paranuclear filament aggregates besides frequent mitoses, focal necroses and lymphocyte and plasma cell infiltrates. The Merkel cell carcinoma is often co-existent with other malignancies such as squamous cell carcinoma or, as in the present study, with Bowen's disease. The definite diagnosis of the Merkel cell carcinoma can be effected only by electron microscopic examination of the tumor.
Similar content being viewed by others
References
Boysen M, Wetteland P, Hovig T, Brandtzaeg P (1989) Neuroendocrine carcinoma of the lip (Merkel cell tumour) examined by electron microscopy and immunohistochemistry. J Laryngol Otol 103:519–523
Breathnach AS (1971) Embryology of human skin. J Invest Dermatol 57:133–143
Ghadially FN (1988) Ultrastructural pathology of the cell and matrix, 3rd edn. Butterworths, London, p 413
Goepfert H, Remmler D, Silva E, Wheeler B (1984) Merkel cell carcinoma (endocrine carcinoma of the skin) of the head and neck. Arch Otolaryngol 110:707–712
Gomez LG, DiMaio S, Silva EG, Mackay B (1983) Association between neuroendocrine (Merkel cell) carcinoma and squamous carcinoma of the skin. Am J Surg Pathol 7:171–177
Gould VE, Moll R, Moll I, Lee I, Franke WW (1985) Neuroendocrine (Merkel) cells of the skin: hyperplasias, dysplasias, and neoplasms. Lab Invest 52:334–353
Groh V, Tani M, Harter A, Wolff K, Stingl G (1986) Leu-3/T4 expression on epidermal Langerhans cells in normal and diseased skin. J Invest Dermatol 86:115–120
Groh V, Gadner H, Radaszkiewicz T, Rappersberger K, Konrad K, Wolff K, Stingl G (1988) The phenotypic spectrum of histiocytosis X. J Invest Dermatol 90:441–447
Hanke CW, Conner AC, Temofeew RK, Lingeman RE (1989) Merkel cell carcinoma. Arch Dermatol 125:1096–1100
Hanna GS, Ali MH, Akosa AB, Maher EJ (1988) Merkel-cell carcinoma of the pina. J Laryngol Otol 102:608–611
Hartschuh W, Weihe E, Egner U (1989) Chromogranin A in the mammalian Merkel cell: cellular and subcellular distribution. J Invest Dermatol 93:641–648
Hashimoto K (1972) The ultrastructure of the skin of embryos. X. Merkel tactile cells in the finger and nail. J Anat 11:99–120
Heenan PJ, Cole JM, Spagnolo DV (1990) Primary cutaneous neuroendocrine carcinoma (Merkel cell tumor). An adnexal epithelial neoplasm. Am J Dermatopathol 12:7–16
Hitchcock CL, Bland KI, Laney RG, Franzini D, Harris B, Copeland EM (1988) Neuroendocrine (Merkel cell) carcinoma of the skin: its natural history, diagnosis, and treatment. Ann Surg 207:201–207
Hoefler H, Kerl H, Rauch HJ, Denk H (1984) New immunocytochemical observations with diagnostic significance in cutaneous neuroendocrine carcinoma. Am J Dermatopathol 6:525–530
Knox SJ, Kapp DS (1988) Hyperthermia and radiation therapy in the treatment of recurrent Merkel cell tumors. Cancer 62:1479–1486
Leff EL, Brooks JSJ, Trojanowski JQ (1985) Expression of neurofilament and neuron-specific enolase in small cell tumors of skin using immunohistochemistry. Cancer 56:625–631
Lever WF (1990) Histopathology of the skin, 7th edn. Lippincott, Philadelphia, p 549
Marks ME, Kim RY, Salter MM (1990) Radiotherapy as an adjunct in the management of Merkel cell carcinoma. Cancer 65:60–64
Meland NB, Jackson IT (1986) Merkel cell tumor: diagnosis, prognosis and management. Plast Reconstruct Surg 77:632–638
Merkel F (1875) Tastzellen und Tastkörperchen bei den Haustieren und beim Menschen. Arch Mikr Anat 11:636–652
Merot Y, Margolis RJ, Dahl D, Saurat JH, Mihm MC (1986) Coexpression of neurofilament and keratin proteins in cutaneous neuroendocrine carcinoma cells. J Invest Dermatol 86:74–77
Moll I, Moll R, Franke WW (1986) Formation of epidermal and dermal Merkel cells during fetal skin development. J Invest Dermatol 87:779–787
Nelson EL, Houghton DC (1990) Concurrent spindle cell peripheral pulmonary carcinoid tumor and Merkel cell tumor of the skin. Arch Pathol Lab Med 114:420–423
Raaf JH, Urmacher C, Knapper WK, Shin MH, Cheng EWK (1986) Trabecular (Merkel cell) carcinoma of the skin. Treatment of primary, recurrent, and metastatic disease. Cancer 57:178–182
Rocamora A, Badia N, Vives R, Carrillo R, Ulloa J, Ledo A (1987) Epidermotropic primary neuroendocrine (Merkel cell) carcinoma of the skin with Pautrier-like microabscesses. J Am Acad Dermatol 16:1163–1168
Schenk P (1975) Melanocyten, Langerhanssche und Merkelsche Zellen in oralen Epithelien. Acta Otolaryngol (Stockh) 80:301–311
Sibley RK, Dahl D (1985) Primary neuroendocrine (Merkel cell?) carcinoma of the skin. II. An immunocytochemical study of 21 cases. Am J Surg Pathol 9:109–116
Sibley RK, Rosai J, Foucar E, Dehner LP, Bosl G (1980) Neuroendocrine (Merkel cell) carcinoma of the skin. Am J Surg Pathol 4:211–221
Sibley RK, Dehner LP, Rosai J (1985) Primary neuroendocrine (Merkel cell?) carcinoma of the skin. A clinicopathologic and ultrastructural study of 43 cases. Am J Surg Pathol 9:95–108
Sidhu GS, Feiner H, Flotte TJ, Mullins JD, Schaefler K, Schultenover SJ (1980) Merkel cell neoplasms. Histology, electron microscopy, biology, and histogenesis. Am J Dermatopathol 2:101–119
Silva EG, MacKay B, Goepfert H, Burgess MA, Fields RS (1984) Endocrine carcinoma of the skin (Merkel cell carcinoma). Pathol Ann 19:1–30
Tang CK, Toker C (1978) Trabecular carcinoma of the skin. An ultrastructural study. Cancer 42:2311–2321
Toker C (1972) Trabecular carcinoma of the skin. Arch Dermatol 105:107–110
Warner TFCS, Uno H, Hafez GR, Burgess J, Bolles C, Lloyd RV, Oka M (1983) Merkel cells and Merkel cell tumors. Cancer 52:238–245
Weidauer H, Altmannsberger HM (1987) Das neuroendokrine Karzinom (Merkelzelltumor) im Kopf-Hals-Bereich. Strahlenther Onkol 163:529–533
Winkelmann RK (1977) The Merkel cell system and a comparison between it and the neurosecretory or APUD cell system. J Invest Dermatol 69:41–46
Author information
Authors and Affiliations
Additional information
Dedicated to Prof. K. Burian on the occasion of his 70th birthday
Rights and permissions
About this article
Cite this article
Schenk, P., Konrad, K. Merkel cell carcinoma of the head and neck associated with Bowen's disease. Eur Arch Otorhinolaryngol 248, 436–441 (1991). https://doi.org/10.1007/BF00627629
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00627629