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Published in: European Archives of Oto-Rhino-Laryngology 8/2013

01-08-2013 | Review Article

Merkel cell carcinoma: a systematic review of ENT presentations

Authors: Federico Maria Gioacchini, Valentina Postacchini, Oriana Simonetti, Annamaria Offidani, Giuseppe Magliulo, Massimo Re

Published in: European Archives of Oto-Rhino-Laryngology | Issue 8/2013

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Abstract

The aim of this study was to perform a systematic literature review of Merkel cell carcinomas (MCCs) originating exclusively in the ear, nose and throat (ENT) district. An appropriate string was run on PubMed to retrieve articles dealing with ENT presentations of MCC. A double cross-check was performed on citations and full-text articles found using the selected inclusion and exclusion criteria. In total, 43 articles were finally included in the study, describing 51 cases of MCC involving the ENT region: 22/51 (43 %) cases involving the ear; 20/51 (39 %) cases involving the mucosal sites; 9/51 (18 %) cases involving other ENT regions. Patients with mucosal site involvement showed a higher mortality rate from the disease (45 %) in comparison with the other two groups, especially when compared with those patients having primary involvement of the ear (22 %). The ENT specialist should suspect and consider MCC, especially in elderly patients presenting with a suspicious lesion of the auricular pavilion, so as to avoid misdiagnosis and delayed treatments.
Literature
1.
go back to reference Prabhu S, Smitha RS, Punnya VA (2010) Merkel cell carcinoma of the alveolar mucosa in a young adult: a rare case report. Br J Oral Maxillofac Surg 48:48–50PubMedCrossRef Prabhu S, Smitha RS, Punnya VA (2010) Merkel cell carcinoma of the alveolar mucosa in a young adult: a rare case report. Br J Oral Maxillofac Surg 48:48–50PubMedCrossRef
2.
go back to reference Chiarelli TG, Grant-Kels JM, Sporn JR, Rezuke WN, Whalen JD (2000) Unusual presentation of a Merkel cell carcinoma. J Am Acad Dermatol 42(2 Pt 2):366–370PubMedCrossRef Chiarelli TG, Grant-Kels JM, Sporn JR, Rezuke WN, Whalen JD (2000) Unusual presentation of a Merkel cell carcinoma. J Am Acad Dermatol 42(2 Pt 2):366–370PubMedCrossRef
3.
go back to reference Huber GF, Khalil M, Falck V, Matthews TW, Dort JC (2008) Merkel cell carcinoma with solitary parotid metastasis: diagnostic dilemma in the absence of a primary site. J Otolaryngol Head Neck Surg 37(1):E19–E21PubMed Huber GF, Khalil M, Falck V, Matthews TW, Dort JC (2008) Merkel cell carcinoma with solitary parotid metastasis: diagnostic dilemma in the absence of a primary site. J Otolaryngol Head Neck Surg 37(1):E19–E21PubMed
4.
go back to reference Nazarian Y, Shalmon B, Horowitz Z, Bedrin L, Pfeffer MR, Talmi YP (2007) Merkel cell carcinoma of unknown primary site. J Laryngol Otol 121(4):e1PubMedCrossRef Nazarian Y, Shalmon B, Horowitz Z, Bedrin L, Pfeffer MR, Talmi YP (2007) Merkel cell carcinoma of unknown primary site. J Laryngol Otol 121(4):e1PubMedCrossRef
5.
go back to reference Virtaniemi J, Hirvikoski P, Pukkila M, Kumpulainen E, Johansson R, Kosma VM (2000) Merkel cell carcinoma of the auricle. Eur Arch Otorhinolaryngol 257(10):558–560PubMedCrossRef Virtaniemi J, Hirvikoski P, Pukkila M, Kumpulainen E, Johansson R, Kosma VM (2000) Merkel cell carcinoma of the auricle. Eur Arch Otorhinolaryngol 257(10):558–560PubMedCrossRef
6.
go back to reference Santos Gorjón P, Morales Martín AC, Blanco Pérez P, Gómez González JL, del Pozo de Dios JC, Romo Melgar A (2011) Merkel cell carcinoma: a presentation of 5 cases and a review of the literature. Acta Otorrinolaringol Esp 62(4):306–310PubMedCrossRef Santos Gorjón P, Morales Martín AC, Blanco Pérez P, Gómez González JL, del Pozo de Dios JC, Romo Melgar A (2011) Merkel cell carcinoma: a presentation of 5 cases and a review of the literature. Acta Otorrinolaringol Esp 62(4):306–310PubMedCrossRef
7.
go back to reference Yom SS, Rosenthal DI, El-Naggar AK, Kies MS, Hessel AC (2006) Merkel cell carcinoma of the tongue and head and neck oral mucosal sites. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 101(6):761–768PubMedCrossRef Yom SS, Rosenthal DI, El-Naggar AK, Kies MS, Hessel AC (2006) Merkel cell carcinoma of the tongue and head and neck oral mucosal sites. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 101(6):761–768PubMedCrossRef
8.
go back to reference Litofsky NS, Smith TW, Megerian CA (1998) Merkel cell carcinoma of the external auditory canal invading the intracranial compartment. Am J Otolaryngol 19(5):330–334PubMedCrossRef Litofsky NS, Smith TW, Megerian CA (1998) Merkel cell carcinoma of the external auditory canal invading the intracranial compartment. Am J Otolaryngol 19(5):330–334PubMedCrossRef
9.
go back to reference Edge SB, Byrd DR, Compton CC et al. (eds) (2010) Merkel cell carcinoma. In: AJCC cancer staging manual, 7th edn. Springer, New York, pp 315–323 Edge SB, Byrd DR, Compton CC et al. (eds) (2010) Merkel cell carcinoma. In: AJCC cancer staging manual, 7th edn. Springer, New York, pp 315–323
10.
go back to reference Ghaderi M, Coury J, Oxenberg J, Spector H (2010) Primary Merkel cell carcinoma of the parotid gland. Ear Nose Throat J 89(7):E24–E27PubMed Ghaderi M, Coury J, Oxenberg J, Spector H (2010) Primary Merkel cell carcinoma of the parotid gland. Ear Nose Throat J 89(7):E24–E27PubMed
11.
go back to reference Fornelli A, Eusebi V, Pasquinelli G, Quattrone P, Rosai J (2001) Merkel cell carcinoma of the parotid gland associated with Warthin tumour: report of two cases. Histopathology 39(4):342–346PubMedCrossRef Fornelli A, Eusebi V, Pasquinelli G, Quattrone P, Rosai J (2001) Merkel cell carcinoma of the parotid gland associated with Warthin tumour: report of two cases. Histopathology 39(4):342–346PubMedCrossRef
12.
go back to reference Yang GC, Schneck MJ, Hayden RE, Gupta PK (1994) Merkel cell tumor-like neuroendocrine carcinoma associated with the submandibular gland. Report of a case with cytologic, immunohistochemical, electron microscopic and flow cytometric studies. Acta Cytol 38(5):742–746PubMed Yang GC, Schneck MJ, Hayden RE, Gupta PK (1994) Merkel cell tumor-like neuroendocrine carcinoma associated with the submandibular gland. Report of a case with cytologic, immunohistochemical, electron microscopic and flow cytometric studies. Acta Cytol 38(5):742–746PubMed
13.
go back to reference Straka JA, Straka MB (1997) A review of Merkel cell carcinoma with emphasis on lymph node disease in the absence of a primary site. Am J Otolaryngol 18(1):55–65PubMedCrossRef Straka JA, Straka MB (1997) A review of Merkel cell carcinoma with emphasis on lymph node disease in the absence of a primary site. Am J Otolaryngol 18(1):55–65PubMedCrossRef
14.
go back to reference Nguyen BD (2002) Positron emission tomographic imaging of Merkel cell carcinoma. Clin Nucl Med 27(12):922–923PubMedCrossRef Nguyen BD (2002) Positron emission tomographic imaging of Merkel cell carcinoma. Clin Nucl Med 27(12):922–923PubMedCrossRef
15.
go back to reference Petković M, Krstulja M, Radic J, Zamolo G, Muhvić D, Lovasic I, Kujundzic M, Franko A (2008) Merkel cell carcinoma arising in the ear canal. Int J Surg Pathol 16(3):337–340PubMedCrossRef Petković M, Krstulja M, Radic J, Zamolo G, Muhvić D, Lovasic I, Kujundzic M, Franko A (2008) Merkel cell carcinoma arising in the ear canal. Int J Surg Pathol 16(3):337–340PubMedCrossRef
16.
go back to reference Palma S, Cavazzini L, Bovo R, Padovani D, Bugli AM, Borrelli M, Martini A (2007) Merkel cell tumour of the external ear. Report of a case. Auris Nasus Larynx 34(2):229–232PubMedCrossRef Palma S, Cavazzini L, Bovo R, Padovani D, Bugli AM, Borrelli M, Martini A (2007) Merkel cell tumour of the external ear. Report of a case. Auris Nasus Larynx 34(2):229–232PubMedCrossRef
17.
go back to reference Skia B, Bibas A, Hickey SA, Ryley NG (1997) Merkel cell carcinoma of the pinna. J Laryngol Otol 111(12):1195–1198PubMedCrossRef Skia B, Bibas A, Hickey SA, Ryley NG (1997) Merkel cell carcinoma of the pinna. J Laryngol Otol 111(12):1195–1198PubMedCrossRef
18.
go back to reference Moayed S, Maldjianb C, Adam R, Bonakdarpour A (2000) Magnetic resonance imaging appearance of metastatic Merkel cell carcinoma to the sacrum and epidural space. Magn Reson Imaging 18(8):1039–1042PubMedCrossRef Moayed S, Maldjianb C, Adam R, Bonakdarpour A (2000) Magnetic resonance imaging appearance of metastatic Merkel cell carcinoma to the sacrum and epidural space. Magn Reson Imaging 18(8):1039–1042PubMedCrossRef
19.
go back to reference Pagella F, Semino L, Corno S, Colombo A, Benazzo M (2005) Merkel cell carcinoma of the auricle. Am J Otolaryngol 26(5):324–326PubMedCrossRef Pagella F, Semino L, Corno S, Colombo A, Benazzo M (2005) Merkel cell carcinoma of the auricle. Am J Otolaryngol 26(5):324–326PubMedCrossRef
20.
go back to reference Bellome J, Bays DR (1998) Merkel cell carcinoma of the ear: report of a case. J Oral Maxillofac Surg 56(8):984–988PubMedCrossRef Bellome J, Bays DR (1998) Merkel cell carcinoma of the ear: report of a case. J Oral Maxillofac Surg 56(8):984–988PubMedCrossRef
21.
go back to reference Cotlar AM, Gates JO, Gibbs FA Jr (1986) Merkel cell carcinoma: combined surgery and radiation therapy. Am Surg 52(3):159–164PubMed Cotlar AM, Gates JO, Gibbs FA Jr (1986) Merkel cell carcinoma: combined surgery and radiation therapy. Am Surg 52(3):159–164PubMed
22.
go back to reference Peréz JA, Poblete MT, Castillo P, Ramírez P, de Sousa U, Pérez A (1998) Merkel cell carcinoma. Report of 4 cases. Rev Med Chil 126(5):553–558PubMed Peréz JA, Poblete MT, Castillo P, Ramírez P, de Sousa U, Pérez A (1998) Merkel cell carcinoma. Report of 4 cases. Rev Med Chil 126(5):553–558PubMed
23.
go back to reference Al-Dousary S, Maqbool S, Zaid MA, al Rikabi A (1996) Merkel cell carcinoma of the auricle. J Otolaryngol 25(6):408–411PubMed Al-Dousary S, Maqbool S, Zaid MA, al Rikabi A (1996) Merkel cell carcinoma of the auricle. J Otolaryngol 25(6):408–411PubMed
24.
go back to reference Hanna GS, Ali MH, Akosa AB, Maher EJ (1988) Merkel-cell carcinoma of the pinna. J Laryngol Otol 102(7):608–611PubMedCrossRef Hanna GS, Ali MH, Akosa AB, Maher EJ (1988) Merkel-cell carcinoma of the pinna. J Laryngol Otol 102(7):608–611PubMedCrossRef
25.
go back to reference Dargallo G, Ramos M, Molina F, Redondo E (1991) Primary neuroendocrine carcinoma of the pavilion of the ear. Acta Otorrinolaringol Esp 42(6):429–432PubMed Dargallo G, Ramos M, Molina F, Redondo E (1991) Primary neuroendocrine carcinoma of the pavilion of the ear. Acta Otorrinolaringol Esp 42(6):429–432PubMed
26.
go back to reference Suntharalingam M, Rudoltz MS, Mendenhall WM, Parsons JT, Stringer SP, Million RR (1995) Radiotherapy for Merkel cell carcinoma of the skin of the head and neck. Head Neck 17(2):96–101PubMedCrossRef Suntharalingam M, Rudoltz MS, Mendenhall WM, Parsons JT, Stringer SP, Million RR (1995) Radiotherapy for Merkel cell carcinoma of the skin of the head and neck. Head Neck 17(2):96–101PubMedCrossRef
27.
go back to reference Yanguas I, Goday JJ, González-Güemes M, Oleaga JM, Lozano M, Soloeta R (1997) Spontaneous regression of Merkel cell carcinoma of the skin. Br J Dermatol 137(2):296–298PubMedCrossRef Yanguas I, Goday JJ, González-Güemes M, Oleaga JM, Lozano M, Soloeta R (1997) Spontaneous regression of Merkel cell carcinoma of the skin. Br J Dermatol 137(2):296–298PubMedCrossRef
28.
go back to reference Gangopadhyay K, Abuzeid MO (1999) Merkel cell carcinoma of the pinna. Otolaryngol Head Neck Surg 120(2):286PubMedCrossRef Gangopadhyay K, Abuzeid MO (1999) Merkel cell carcinoma of the pinna. Otolaryngol Head Neck Surg 120(2):286PubMedCrossRef
29.
go back to reference Sneshkoff NT, Freeman JH, Liming BJ (2010) Pathology quiz case 2. Merkel cell carcinoma (MCC) of the left auricle. Arch Otolaryngol Head Neck Surg 136(7):745, 747–748 Sneshkoff NT, Freeman JH, Liming BJ (2010) Pathology quiz case 2. Merkel cell carcinoma (MCC) of the left auricle. Arch Otolaryngol Head Neck Surg 136(7):745, 747–748
30.
go back to reference Karapantzos I, Tsaroucha A, Polychronidis A, Simopoulos C, Simasko N (2003) Merkel cell carcinoma: report of seven cases. ORL J Otorhinolaryngol Relat Spec 65(6):370–374PubMedCrossRef Karapantzos I, Tsaroucha A, Polychronidis A, Simopoulos C, Simasko N (2003) Merkel cell carcinoma: report of seven cases. ORL J Otorhinolaryngol Relat Spec 65(6):370–374PubMedCrossRef
31.
go back to reference Ecker HA Jr, Abt AB, Graham WP 3rd, Herceg S (1982) Trabecular or Merkel-cell carcinoma of the skin. Plast Reconstr Surg 70(4):485–489PubMedCrossRef Ecker HA Jr, Abt AB, Graham WP 3rd, Herceg S (1982) Trabecular or Merkel-cell carcinoma of the skin. Plast Reconstr Surg 70(4):485–489PubMedCrossRef
32.
go back to reference Matichard E, Descamps V, Grossin M, Genin R, Bouvet E, Crickx B (2002) Merkel cell carcinoma in a black human immunodeficiency virus-infected patient. Br J Dermatol 146(4):671–673PubMedCrossRef Matichard E, Descamps V, Grossin M, Genin R, Bouvet E, Crickx B (2002) Merkel cell carcinoma in a black human immunodeficiency virus-infected patient. Br J Dermatol 146(4):671–673PubMedCrossRef
33.
go back to reference Inoue T, Shimono M, Takano N, Saito C, Tanaka Y (1997) Merkel cell carcinoma of palatal mucosa in a young adult: immunohistochemical and ultrastructural features. Oral Oncol 33(3):226–229PubMedCrossRef Inoue T, Shimono M, Takano N, Saito C, Tanaka Y (1997) Merkel cell carcinoma of palatal mucosa in a young adult: immunohistochemical and ultrastructural features. Oral Oncol 33(3):226–229PubMedCrossRef
34.
go back to reference Orsini G, Fioroni M, Rubini C, Piattelli A (2000) Merkel cell carcinoma of the lip: report of a case. J Oral Maxillofac Surg 58(9):1044–1047PubMedCrossRef Orsini G, Fioroni M, Rubini C, Piattelli A (2000) Merkel cell carcinoma of the lip: report of a case. J Oral Maxillofac Surg 58(9):1044–1047PubMedCrossRef
35.
go back to reference Dolezal RF, Cohen M, Taxy JB (1988) Merkel tumor of the lower lip: a case report and updated review of the literature work performed at the University of Illinois. J Surg Oncol 37(2):123–127PubMedCrossRef Dolezal RF, Cohen M, Taxy JB (1988) Merkel tumor of the lower lip: a case report and updated review of the literature work performed at the University of Illinois. J Surg Oncol 37(2):123–127PubMedCrossRef
37.
go back to reference Drijkoningen M, de Wolf-Peeters C, van Limbergen E, Desmet V (1986) Merkel cell tumor of the skin: an immunohistochemical study. Hum Pathol 17(3):301–307PubMedCrossRef Drijkoningen M, de Wolf-Peeters C, van Limbergen E, Desmet V (1986) Merkel cell tumor of the skin: an immunohistochemical study. Hum Pathol 17(3):301–307PubMedCrossRef
38.
go back to reference 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(5):519–523PubMedCrossRef 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(5):519–523PubMedCrossRef
39.
go back to reference English KB, Hammond EH, Stayner N (1990) Primary culture of cells arising from a neuroendocrine skin carcinoma. Am J Dermatopathol 12(6):589–597PubMedCrossRef English KB, Hammond EH, Stayner N (1990) Primary culture of cells arising from a neuroendocrine skin carcinoma. Am J Dermatopathol 12(6):589–597PubMedCrossRef
40.
go back to reference Hayter JP, Jacques K, James KA (1991) Merkel cell tumour of the cheek. Br J Oral Maxillofac Surg 29(2):114–116PubMedCrossRef Hayter JP, Jacques K, James KA (1991) Merkel cell tumour of the cheek. Br J Oral Maxillofac Surg 29(2):114–116PubMedCrossRef
41.
go back to reference Longo F, Califano L, Mangone GM, Errico ME (1999) Neuroendocrine (Merkel cell) carcinoma of the oral mucosa: report of a case with immunohistochemical study and review of the literature. J Oral Pathol Med 28(2):88–91PubMedCrossRef Longo F, Califano L, Mangone GM, Errico ME (1999) Neuroendocrine (Merkel cell) carcinoma of the oral mucosa: report of a case with immunohistochemical study and review of the literature. J Oral Pathol Med 28(2):88–91PubMedCrossRef
42.
go back to reference Baker P, Alguacil-Garcia A (1999) Moderately differentiated neuroendocrine carcinoma in the floor of the mouth: a case report. J Oral Maxillofac Surg 57(9):1143–1147PubMedCrossRef Baker P, Alguacil-Garcia A (1999) Moderately differentiated neuroendocrine carcinoma in the floor of the mouth: a case report. J Oral Maxillofac Surg 57(9):1143–1147PubMedCrossRef
43.
go back to reference Azizi L, Marsot-Dupuch K, Bigel P, Mzali S, Meyer B, Tubiana JM (2001) Merkel cell carcinoma: a rare cause of hypervascular nasal tumor. AJNR Am J Neuroradiol 22(7):1389–1393PubMed Azizi L, Marsot-Dupuch K, Bigel P, Mzali S, Meyer B, Tubiana JM (2001) Merkel cell carcinoma: a rare cause of hypervascular nasal tumor. AJNR Am J Neuroradiol 22(7):1389–1393PubMed
44.
go back to reference Karkos PD, Sastry A, Hampal S, Al-Jafari M (2010) Spontaneous regression of Merkel cell carcinoma of the nose. Head Neck 32(3):411–414PubMed Karkos PD, Sastry A, Hampal S, Al-Jafari M (2010) Spontaneous regression of Merkel cell carcinoma of the nose. Head Neck 32(3):411–414PubMed
45.
go back to reference Snow SN, Larson PO, Hardy S, Bentz M, Madjar D, Landeck A, Oriba H, Olansky D (2001) Merkel cell carcinoma of the skin and mucosa: report of 12 cutaneous cases with 2 cases arising from the nasal mucosa. Dermatol Surg 27(2):165–170PubMedCrossRef Snow SN, Larson PO, Hardy S, Bentz M, Madjar D, Landeck A, Oriba H, Olansky D (2001) Merkel cell carcinoma of the skin and mucosa: report of 12 cutaneous cases with 2 cases arising from the nasal mucosa. Dermatol Surg 27(2):165–170PubMedCrossRef
46.
go back to reference Mulder DC, Rosenberg AJ, Storm-Bogaard PW, Koole R (2010) Spontaneous regression of advanced Merkel-cell-like small cell carcinoma of the parotid gland. Br J Oral Maxillofac Surg 48(3):199–200PubMedCrossRef Mulder DC, Rosenberg AJ, Storm-Bogaard PW, Koole R (2010) Spontaneous regression of advanced Merkel-cell-like small cell carcinoma of the parotid gland. Br J Oral Maxillofac Surg 48(3):199–200PubMedCrossRef
Metadata
Title
Merkel cell carcinoma: a systematic review of ENT presentations
Authors
Federico Maria Gioacchini
Valentina Postacchini
Oriana Simonetti
Annamaria Offidani
Giuseppe Magliulo
Massimo Re
Publication date
01-08-2013
Publisher
Springer Berlin Heidelberg
Published in
European Archives of Oto-Rhino-Laryngology / Issue 8/2013
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI
https://doi.org/10.1007/s00405-012-2283-1

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