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CASE REPORT
Year : 2019  |  Volume : 22  |  Issue : 2  |  Page : 102-104

Merkel cell carcinoma: A case report


1 Department of Surgery, Aminu Kano Teaching Hospital/Bayero University, Kano, Nigeria
2 Department of Histopathology, Aminu Kano Teaching Hospital/Bayero University, Kano, Nigeria
3 Department of Paediatrics, Aminu Kano Teaching Hospital/Bayero University, Kano, Nigeria

Date of Submission01-Jun-2017
Date of Acceptance31-Jan-2018
Date of Web Publication20-Jun-2019

Correspondence Address:
Dr. Ismail Inuwa Mohammed
Department of Surgery, Aminu Kano Teaching Hospital, Kano
Nigeria
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DOI: 10.4103/smj.smj_42_17

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  Abstract 


Merkel cell carcinoma (MCC) is a rare type of skin cancer which often appears on sun-exposed areas. It is a fairly common tumor among the Caucasians but rarely occurs among Africans. Therefore, we report the case of a 25-year-old African woman who presented initially with painless swelling on the left upper limb which progressively increased in size and became painful with associated ipsilateral supraclavicular lymph node enlargement and lymphedema. She had excisional biopsy and histology which revealed MCC. The patient was subsequently referred for chemotherapy.

Keywords: Chemoradiation, Merkel cell, Merkel cell carcinoma, skin cancer


How to cite this article:
Mohammed II, Umar AB, Ahmad J, Aliyu I, Oyebanji T, Abdurrahman S. Merkel cell carcinoma: A case report. Sahel Med J 2019;22:102-4

How to cite this URL:
Mohammed II, Umar AB, Ahmad J, Aliyu I, Oyebanji T, Abdurrahman S. Merkel cell carcinoma: A case report. Sahel Med J [serial online] 2019 [cited 2019 Jul 17];22:102-4. Available from: http://www.smjonline.org/text.asp?2019/22/2/102/260838




  Introduction Top


Merkel cell carcinoma (MCC) is a rare type of skin cancer that usually appears as a fleshy red nodule often on the head, neck, and face (which are sun-exposed areas). It is also called neuroendocrine carcinoma of the skin, and it is usually seen in patients with weak immune system or a history of longtime sun exposure.[1],[2] It is highly aggressive skin cancer caused by Merkel cell polyomavirus which was discovered by a German Scientist at the University of Pittsburg in 2008.[3] Freidrich Sigmund first described the Merkel cell as mechanoreceptors in animals in 1875;[4] however, Toker[5] in 1972 was the first to describe MCC,[4] and since then, over 600 cases have been reported in the literature.[4] An annual incidence rate of 0.2–0.45 cases per 100,000 of the population has been reported which makes MCC 100 times rarer than melanoma.[4] However, recent evidence has suggested that the incidence may be increasing as was witnessed by the surveillance epidemiology and end result database.[4] There are no reliable data in Africa on MCC; and in our center, only three cases have so far been diagnosed over a 15-year period. Merkel cells are found in the top layer of the skin which is close to the nerve endings that receive touch sensation.[6] MCC usually presents as a single painless lump on sun-exposed skin;[4] we therefore present the case of a 25-year-old woman with clinical and histologic diagnosis of MCC.


  Case Report Top


A 25-year-old homemaker in a rural setting in Kano presented to their primary health center with complaint of a 6-month history of lump on the left hand; this lump was initially tiny and painless progressively increasing in size, and 2 months into the illness there was associated left upper limb swelling with associated limb pain, and tenderness. Though there was no chest complaint, fever, or significant weight loss. However, there was swelling of the ipsilateral axillary and supraclavicular lymph node [Figure 1]; the supraclavicular lymph node measured 20 cm × 30 cm while the nonpitting lymphedema involved the whole left upper limb [Figure 2]. The lump was on the dorsum of the hand close to the wrist joint measuring 20 cm × 40 cm [Figure 2]. She had normal chest and cervical X-rays; chest computerized tomography (CT) scan was not done due to financial constraint. She had excisional biopsy of the lump and the histology revealed MCC [Figure 3] and [Figure 4]. Immunohistochemistry revealed leukocyte common antigen negative, CK 20 negative, CD 117 negative, and chromogranin A negative, which exclude lymphoid, gastrointestinal stromal, and neuroendocrine tumors. The patient was subsequently referred to a tertiary center for chemoradiation therapy. She had only a single course of a scheduled four-course cisplatin-based chemotherapy (cisplatin 60 mg/m2, etoposide 100 mg/m2). However, the patient died after the single course of chemotherapy.
Figure 1: Swelling of the ipsilateral supraclavicular lymph node

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Figure 2: lymphedema involving the whole left upper limb

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Figure 3: Sheets of small tumor cells exhibiting perivascular cuffing (H and E, ×40)

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Figure 4: Tumor cells with pleomorphic hyperchromatic nuclei and scanty-to-moderate cytoplasm with frequent abnormal mitosis (H and E, ×100)

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  Discussion Top


Evaluation of patients with such rare skin cancer in a rural setting is quite challenging. Furthermore, the index case presented with an atypical feature of a skin lump.[7] The initial presentation was a painless lump and the absence of significant weight loss despite an advanced disease stage defies the typical[1] distinguishing clinical features of MCC as was described by Pearson and Meyers;[4] this can result in delayed diagnosis; however, the exact origin of the tumor in the index case was not clear until after the histology was done. MCC is more common in the elderly;[4] however, the index case was only 25 years old, which made it a unique presentation. MCC involving the extremities has also been reported by Kadiri et al.;[8] however, their case involved a 58-year-old Black woman with MCC affecting the right elbow with associated ipsilateral axillary lymph node involvement as was also witnessed in our index case.

MCC typically arises from sun-exposed areas of the face, neck, and arms in the form of single pink or a purple nodule which is painless. It rapidly grows, spreading to regional lymph nodes which were also witnessed in the index case; however, it later became painful in our patient. This may be attributed to associated lymphedema, which might have been inflamed.

Approximately 53% of MCC occur in the head-and-neck and 35% affect the extremities; however, 11%–15% present with lymph node involvement at the initial phase of illness, but 75%–83% will eventually develop regional nodal and distant metastasis during the illness.[4],[9] Although biopsy establishes the diagnosis, CT scan of the head-and-neck region as well as abdominal ultrasound is essential for efficient staging of the disease; furthermore, magnetic resonance imaging, positron emission tomography scan,[5] and sentinel lymph node biopsy are required for proper evaluation and treatment. Therefore, our inability to do a CT and other investigations was a limitation which arose due to financial constraint and nonavailability of such facilities in our rural setting. Although the immunohistochemistry reported CK20 negativity, this does not completely rule MCC because about 5% CK20 negativity has been reported in MCC cases; there is a suggestion that cases negative for CK20 may also be Merkel cell polyomavirus negative.[10] In early disease, wide surgical excision followed by chemoradiation therapy is the best option of treatment; however, late disease has poor prognosis; therefore, palliation with chemoradiation and possibly surgical debulking to reduce pressure symptoms and tumor bulk are possible options.[5],[6],[11] Common drugs used for MCC are cisplatin, carboplatin, etoposide, and topotecan.[12] Other factors that influence the prognosis are age, tumor site, recurrence, and the general well-being of the patient.


  Conclusion Top


MCC is a highly aggressive skin cancer occurring on sun-exposed areas; tissue biopsy is paramount in establishing the diagnosis because atypical presentations may occur as was observed in the index case.

Declaration of patient consent

We, the authors certify that we have obtained written informed patient consent on 23rd March 2017. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patients understand that her names and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. It was explained that failure to consent will not influence the patient's care.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
National Cancer Institute. Treatment of Merkel Cell Carcinoma; 2016. Available from: http://www.cancer.gov. [Last accessed on 2017 Jan 02].  Back to cited text no. 1
    
2.
Mayo Clinic Overview on Merkel Cell Carcinoma; 2015. Available from: http://www.mayoclinic.org. [Last accessed on 2016 Dec 21].  Back to cited text no. 2
    
3.
Allison G. Newly Discovered Virus Linked to Neuro-Endocrine Cancer of the Skin. Medscape; 2008. Available from: http://www.medscspe.com. [Last accessed on 2017 Jan 02].  Back to cited text no. 3
    
4.
Pearson JM, Meyers AD. Skin Cancer MCC; 2017. Available from: http://www.emedicine.medscape.com. [Last accessed on 2017 Jan 02].  Back to cited text no. 4
    
5.
Toker C. Trabecular carcinoma of the skin. Arch Dermatol 1972;105:107-10.  Back to cited text no. 5
    
6.
Merkel Cell Skin Cancer/Cancer Research UK; 2014. Available from: http://www.cancerresearchuk.org. [Last accessed on 2017 Jan 02].  Back to cited text no. 6
    
7.
O'Connor WJ, Brodland DG. Merkel cell carcinoma. Dermatol Surg 1996;22:262-7.  Back to cited text no. 7
    
8.
Kadiri S, Aissa A, Berhili S, Khmou M, Elmajjaoui S, Kebdani T, et al. Merkel cell carcinoma occurring in a black woman: A case report. J Med Case Rep 2017;11:25.  Back to cited text no. 8
    
9.
Suárez C, Rodrigo JP, Ferlito A, Devaney KO, Rinaldo A. Merkel cell carcinoma of the head and neck. Oral Oncol 2004;40:773-9.  Back to cited text no. 9
    
10.
Miner AG, Patel RM, Wilson DA, Procop GW, Minca EC, Fullen DR, et al. Cytokeratin 20-negative merkel cell carcinoma is infrequently associated with the merkel cell polyomavirus. Mod Pathol 2015;28:498-504.  Back to cited text no. 10
    
11.
Tuneu A, Pujol RM, Moreno A, Barnadas MA, de Moragas JM. Postirradiation merkel cell carcinoma. J Am Acad Dermatol 1989;20:505-7.  Back to cited text no. 11
    
12.
Crown J, Lipzstein R, Cohen S, Goldsmith M, Wisch N, Paciucci PA, et al. Chemotherapy of metastatic Merkel cell cancer. Cancer Invest 1991;9:129-32.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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