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 Table of Contents  
LETTER TO THE EDITOR
Year : 2012  |  Volume : 8  |  Issue : 3  |  Page : 460-461

Merkel cell carcinoma of the Auricle: A treacherous skin tumor


1 Department of Surgical Oncology, Cancer Institute (WIA), 36, Sardar Patel Rd, Adyar,Chennai - 600020, India
2 Department of Pathology, Cancer Institute (WIA), 36, Sardar Patel Rd, Adyar,Chennai - 600020, India

Date of Web Publication17-Nov-2012

Correspondence Address:
Arvind Krishnamurthy
Department of Surgical Oncology,Cancer Institute (WIA), 36, Sardar Patel Rd,Adyar, Chennai - 600020
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.103537

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How to cite this article:
Krishnamurthy A, Majhi U. Merkel cell carcinoma of the Auricle: A treacherous skin tumor. J Can Res Ther 2012;8:460-1

How to cite this URL:
Krishnamurthy A, Majhi U. Merkel cell carcinoma of the Auricle: A treacherous skin tumor. J Can Res Ther [serial online] 2012 [cited 2019 Nov 21];8:460-1. Available from: http://www.cancerjournal.net/text.asp?2012/8/3/460/103537

Sir,

Merkel cell carcinoma (MCC) is a rare neuroendocrine skin cancer that has a high propensity for local recurrence (25-30%) and nodal metastases (52-59%). [1] More than one-third of the patients with MCC die of the cancer, making it twice as lethal as melanomas. The incidence of MCC has been reported to be increasing due to the advancing age, sun exposure and due to immune suppression. [2],[3],[4] MCC is classically known to affect elderly men (mean age: 60-80 years) and is extremely rare in the younger age groups. We discuss the natural history and the management challenges of this unusual tumor in a young immunocompetent male.

A 17-year-old male was referred to our institution for a recurrent reddish nodule of his left auricle of 3 months duration. This was supposedly his second local recurrence in as many years, following prior local excisions. He also gave a history of receiving external beam radiotherapy following his second excision. On examination apart from the auricular nodule, there was a vague induration in the left parotid region along the healed scars of the previous excisions. The facial nerve function was preserved and there was insignificant cervical lymphadenopathy. A review of the histology of the excision done for the first recurrence, a year back favored the diagnosis of MCC. Immunohistochemistry revealed tumor cell positivity to keratin, EMA, Bcl 2, Bcl 6, NSE, CD56, CD57 and occasional positivity to CK7. 40% of the tumor cells showed strong nuclear positivity to Ki 67. [Figure 1]a-d The tumor cells were negative for TTF 1, LCA, CD3 and CD20. A PET-CT showed an ill-defined soft tissue density of the periauricular skin with increased metabolic activity in the 3.5 × 2 × 1.9 cm nodule and no other uptake elsewhere [Figure 2]a and b. The patient was taken for a radical surgery which entailed a lateral temporal bone excision and an elective neck dissection. The resultant defect was bridged by an anterolateral thigh microvascular flap [Figure 3]a- d. The patient is disease free for a year following surgery.
Figure 1: a. H and E X 40 Tumor composed of atypical round cells in clusters and diffuse sheets. The cells are round to oval with vesicular, cleaved nuclei and scanty cytoplasm. Mitosis was seen in 0-1/hpf and
with areas of necrosis. b. IHC X 100 revealed tumor cell positivity to keratin, c. IHC X 100 revealed tumor cell positivity to CD57, d. IHC X 100 40% of the tumor cells showed strong nuclear positivity to Ki 67


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Figure 2: PET- CT showing an ill-defined soft tissue density in the external auditory canal extending into the infratemporal fossa, measuring 3.5 × 2.0 × 1.9 cm showing increased metabolic activity, a. Sagittal PET-CT image, b. Axial PET-CT image

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Figure 3: (a) Pre-operative clinical photograph showing the tumor in the left auricle, (b) Specimen photograph, (c) Intra operative defect following lateral temporal bone resection and neck dissection, (d) Post operative clinical photograph following anterolateral thigh microvascular free flap reconstruction

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MCC has traditionally been described as a skin cancer that lacks distinguishing clinical features. Heath et al. [5] in a study of 195 patients of MCC defined clinical features that may serve as clues in the diagnosis of MCC. They summarized the most significant features in an acronym: AEIOU (asymptomatic/lack of tenderness, expanding rapidly, immune suppression, older than 50 years, and ultraviolet ray exposure). Our patient had only two of the above criteria, he was very young with a slow growing recurrent swelling and there was no evidence of any immunosuppression. MCC in the HIV-positive patients occurs at a significantly younger age with a possible association with Merkel cell polyoma virus; [6] our patient did not have any underlying disease or predisposing risk factor.

The histogenesis of MCC is controversial. The possible cells of origin include the epidermal Merkel cell, a dermal Merkel cell equivalent or a neural-crest-derived cell of the amine precursor uptake. The diagnosis in most cases of MCC is made on the basis of histopathologic features aided by immunohistochemistry, the latter is essential to differentiate MCC from metastatic small cell lung carcinoma and other small round blue cell tumors. [7]

The treatment of the MCC is surgery, which normally consists of an excision with wide margins or micrographic surgery and preferably with adjuvant radiotherapy. [8],[9] Some studies have suggested that elective lymph node dissection decreases the chance of local recurrences and improves survival. The use of adjuvant chemotherapy has been shown to be toxic and with no survival benefit and is hence not routinely recommended. [10] Since immunological factors have been implicated with the occurrence of MCC, their manipulation may possibly offer additional therapeutic options in the future.

 
 > References Top

1.Medina-Franco H, Urist MM, Fiveash J, Heslin MJ, Bland KI, Beenken SW. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann Surg Oncol 2001;8:204- 8.  Back to cited text no. 1
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2.Boersma GA, van Raalte JA, Botke G, de Vogel PL, Marck KW. Merkel cell carcinoma: A treacherous skin tumor. Ned Tijdschr Geneeskd 1989;133:1933-5.  Back to cited text no. 2
    
3.Karapantzos I, Tsaroucha A, Polychronidis A, Simopoulos C, Simasko N. Merkel cell carcinoma: report of seven cases. ORL J Otorhinolaryngol Relat Spec 2003;65:370-4.  Back to cited text no. 3
    
4.Rockville Merkel Cell Carcinoma Group. Merkel cell carcinoma: recent progress and current priorities on etiology, pathogenesis, and clinical management. J Clin Oncol 2009;20:4021-5.  Back to cited text no. 4
    
5.Heath M, Jaimes N, Lemos B, Mostaghimi A, Wang LC, Peñas PF, et al. Clinical Characteristics of Merkel Cell Carcinoma at Diagnosis in 195 Patients: The AEIOU Features. J Am Acad Dermatol 2008;58:375- 81.  Back to cited text no. 5
    
6.Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science 2008;319:1096- 100.  Back to cited text no. 6
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7.Bobos M, Hytiroglou P, Kostopoulos I, Karkavelas G, Papadimitriou CS. Immunohistochemical distinction between merkel cell carcinoma and small cell carcinoma of the lung. Am J Dermatopathol 2006;28:99-104.  Back to cited text no. 7
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8.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;23:2300-9.  Back to cited text no. 8
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9.Lewis KG, Weinstock MA, Weaver AL, Otley CC. Adjuvant local irradiation for Merkel cell carcinoma. Arch Dermatol 2006;142:693- 700.  Back to cited text no. 9
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10.Poulsen MG, Rischin D, Porter I, Walpole E, Harvey J, Hamilton C, et al. Does chemotherapy improve survival in high-risk stage I and II Merkel cell carcinoma of the skin? Int J Radiat Oncol Biol Phys 2006;64:114-9.  Back to cited text no. 10
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    Figures

  [Figure 1], [Figure 2], [Figure 3]


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