 |
Case Report
An atypical localization of a Merkel cell carcinoma: A case report and review of the literature
1 Resident, Radiology Department, National Institute of Oncology, Rabat, Morocco
2 Specialist, Radiology Department, National Institute of Oncology, Rabat, Morocco
3 Professor and Chief of Department, Radiology Department, National Institute of Oncology, Rabat, Morocco
4 Assistant Professor, Radiology Department, National Institute of Oncology, Rabat, Morocco
Address correspondence to:
Iraqi Fatima Zahra
Agdal, Rabat,
Morocco
Message to Corresponding Author
Article ID: 101237Z01IZ2021
Access full text article on other devices
Access PDF of article on other devices
How to cite this article
Zahra IF, Hounayda J, Rachida L, Youssef O. An atypical localization of a Merkel cell carcinoma: A case report and review of the literature. Int J Case Rep Images 2021;12:101237Z01IZ2021.ABSTRACT
Introduction: Merkel cell carcinoma (MCC) is an aggressive skin tumor of neuroendocrine origin that derives from Merkel cells and occurs predominantly on the head or neck of elderly individuals at an average age of 69 years old. Its main risk factors are immunosuppression and ultraviolet exposure. The diagnosis is established on the histological aspect and completed by immunohistochemistry.
Case Report: We report the case of a 65-year-old female patient, with no particular antecedents, who had right axillary swelling. Thoracic computed tomography (CT) showed a large right axillary mass of irregular contours, lobulated, heterogeneously enhanced after CP injection with central hypodense zone of necrosis. The confrontation of the morphological aspect and the results of the immunohistochemistry carried out made it possible to retain the diagnosis of Merkel cell carcinoma.
Conclusion: Merkel cell carcinoma is a very aggressive cancer of the elderly. In case of doubt, a biopsy for histological confirmation should be performed. The therapy is usually based on a radical surgery followed by adjuvant radiotherapy.
Keywords: Axillary mass, Carcinoma, Histology, Merkel
Introduction
Merkel cell carcinoma (MCC) is an aggressive skin tumor of neuroendocrine origin [1]. This tumor, which occurs predominantly on the head or neck of elderly individuals [2], at an average age of 69 years old [3], is derived from Merkel cells [1]. These cells have been described by Friedrich Sigmund Merkel in 1875, as non-keratinocyte, non-dendritic epidermal cells, having a function of tactile receivers [1]. Its main risk factors are immunosuppression, ultraviolet exposure [4] and in particular, photochemotherapy called PUVA which combines psoralen with ultraviolet A (UVA) [5].
Merkel cell carcinoma clinically presents as a hard, erythematous and purplish nodule, of rapid growth, localized most often at the extremities [6].
The diagnosis is established on the histological aspect and completed by immunohistochemistry [7],[8]
It is an aggressive tumor with a high risk of lymphatic or visceral dissemination [4]. The most frequent sites of visceral metastasis are lungs, liver, and bone [9],[10]. Treatment of MCC includes surgery with reconstruction associated with radiotherapy and/or chemotherapy for advanced stages [11]. The locoregional recurrence rate is 13% for patients with negative sentinel lymph node biopsy [12],[13].
For Allen et al., the use of adjuvant chemotherapy does not provide any benefit in terms of survival, conversely, other authors have reported that it improves survival and reduces recurrence, particularly in patients with high risk of MCC [14].
Case Report
We report the case of a 65-year-old female patient, with no particular antecedents, who had right axillary swelling.
Clinically, this solid mass was erythematous, not painful, and rapidly increased in size since four months.
Thoracic CT showed a large right axillary mass of irregular contours, lobulated, heterogeneously enhanced after CP injection with central hypodense zone of necrosis (Figure 1). It was measuring: 134 × 104 mm. Topographically, laterally right, it invaded the subcutaneous plane by respecting the anterior cutaneous wall. Inside: It came into contact with the chest wall, adhering to the 2nd and 3rd right sides (without bone lysis) and to the intercostal muscles at the height of K1–K2 and K2–K3 (Figure 2). Forward: it adhered to the deep plane of the small and large right pectoral muscles; Back and below: it came into contact with the scapula and subscapularis muscle without a distinctly individualizable borderline; Above: it came into contact with the axillary artery which remained permeable (Figure 2).
A right subclavicular adenopathy was also highlighted (Figure 3).
A biopsy of the skin lesion was performed. The confrontation of the morphological aspect and the results of the immunohistochemistry (Anti-CK20 antibodies +, anti-synaptophysine +, anti-chromogranin +, anti-CK AE1/AE3 +) made it possible to retain the diagnosis of Merkel cell carcinoma. It was negative for anti-TTF1, anti HMB45, and anti-Melan A.
Discussion
The estimated incidence of MCC is 0.23 per 100,000 people in a white, predominantly male population (2.3 men for each woman) [15]. Following sun exposure, immune deficiency caused by human immunodeficiency virus (HIV) and chronic leukemia are the main causes of this aggressive tumor [16],[17]. Recent studies suggest that polyomavirus may play a role in its pathogenesis [18],[19].
Clinical presentation of MCC is a single dome-shaped nodule, red or purplish, painless, rapidly increasing in size. It must be differentiated from basal cell carcinoma, melanoma, or superficial pyogenic granuloma with telangiectasia [11].
Histologically, differential diagnosis occurs with other small-cell tumors, such as small cell lung cancer. Immunohistochemically, MCC typically expresses CK20 (cytokeratine 20), and NFP (neurofilament protein) while it is negative for TTF-1 (thyroid transcription factors), CK7 (cytokeratin 7), and LCA (leukocyte transcription factor) [20].
The therapy depends on the stage of the tumor but it is usually based on a radical surgery followed by adjuvant radiotherapy to the site of the primary tumor and regional lymph nodes [3]. The surgical operations that are used for this carcinoma are wide local excision or Mohs micrographic surgery which is attractive for tumors on the head or neck [21],[22].
Chemotherapy is practiced in advanced stages using chemotherapeutic agents like Carboplatin, cisplatin, etoposide, cyclophosphamide, 5-fluorouracil, vincristine, and doxorubicin [21].
Conclusion
Merkel cell carcinoma (MCC) is a very aggressive cancer that is rapidly growing in elderly patients. It should be considered for any non-melanocytic lesion rapidly growing, especially on exposed skin UV rays in immunosuppressed people. In case of doubt, a biopsy for histological confirmation should be performed without hesitation.
REFERENCES
1.
Feldmeyer L, Gaide O. Merkel cell carcinoma: (r)evolution. [Article in French]. Rev Med Suisse 2013;9(380):723–9.
[Pubmed]
2.
Nardin C, Locatelli F, Puzenat E. Intérêt du lanréotide dans le traitement du carcinome de Merckel métastatique. JDP 2015;PO43:S522.
3.
Slovacek L. Merckel cell carcinoma of the skin treated with somatostatin analogue and mTOR inhibitor exhausted after primary surgery, adjuvant radiotherapy and palliative chemotherapy. Bratisl Lek Listy 2014;115(10):663–5. [CrossRef]
[Pubmed]
4.
Girard C. Guillot B. Carcinome à cellules de Merckel: Prise en charge actuelle. Annales de Dermatologie et de Vénérologie 2010;137(5):402–7. [CrossRef]
5.
Lunder EJ, Stern RS. Merkel-cell carcinomas in patients treated with methoxsalen and ultraviolet A radiation. N Engl J Med 1998;339(17):1247–8. [CrossRef]
[Pubmed]
6.
Heath M, Jaimes N, Lemos B, et al. Clinical characteristics of Merckel cell carcinoma at diagnosis in 195 patients: The AEIOU features. J Am Acad Dermatol 2008;58(3):375–81. [CrossRef]
[Pubmed]
7.
Llombart B, Monteagudo C, López-Guerrero JA, et al. Clinicopathological and immunohistochemical analysis of 20 cases of Merkel cell carcinoma in search of prognostic markers. Histopathology 2005;46(6):622–34. [CrossRef]
[Pubmed]
8.
Bobos M, Hytiroglou P, Kostopoulos I, Karkevelas G, Papadimitriou CS. Immunohistochemical distinction between Merckel cell carcinoma and small cell carcinoma of the lung. Am J Dermatopathol 2006;28(2):99–104. [CrossRef]
[Pubmed]
9.
Wynne CJ, Kearsley JH. Merkel cell tumor. A chemosensitive skin cancer. Cancer 1988;62(1):28–31.
[Pubmed]
10.
Shack RB, Barton RM, DeLozier J, Rees RS, Lynch JB. Is aggressive surgical management justified in the treatment of Merkel cell carcinoma? Plast Reconstr Surg 1994;94(7):970–5.
[Pubmed]
11.
Miranda S, Gbaguidi X, Carvalho P, Chassagne P. Merckel cell carcinoma: The impact of multidisciplinary management. J Nutr Health Aging 2013;17(2):196–8. [CrossRef]
[Pubmed]
12.
Fields RC, Busam KJ, Chou JF, et al. Recurrence after complete resection and selective use of adjuvant therapy for stage I through III Merkel cell carcinoma. Cancer 2012;118(13):3311–20. [CrossRef]
[Pubmed]
13.
Fields RC, Busam KJ, Chou JF, et al. Recurrence and survival in patients undergoing sentinel lymph node biopsy for Merkel cell carcinoma: Analysis of 153 patients from a single institution. Ann Surg Oncol 2011;18(9):2529–37. [CrossRef]
[Pubmed]
14.
Gillenwater AM, Hessel AC, Morrison WH, et al. Merkel cell carcinoma of the head and neck: Effect of surgical excision and radiation on recurrence and survival. Arch Otolaryngol Head Neck Surg 2001;127(2):149–54. [CrossRef]
[Pubmed]
15.
Grim M, Halata Z. Developmental origin of avian Merkel cells. Anat Embryol (Berl) 2000;202(5):401–10.
[Pubmed]
16.
Miller RW, Rabkin CS. Merkel cell carcinoma and melanoma: Etiological similarities and differences. Cancer Epidemiol Biomarkers Prev 1999;8(2):153–8.
[Pubmed]
17.
Penn I, First MR. Merkel's cell carcinoma in organ recipients: Report of 41 cases. Transplantation 1999;68(11):1717–21.
[Pubmed]
18.
Rockville Merkel Cell Carcinoma Group. Merkel cell carcinoma: Recent progress and current priorities on etiology, pathogenesis, and clinical management. J Clin Oncol 2009;27(24):4021–6. [CrossRef]
[Pubmed]
19.
Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science 2008;319(5866):1096–100. [CrossRef]
[Pubmed]
20.
Bichakjian CK, Lowe L, Lao CD, et al. Merkel cell carcinoma: Critical review with guidelines for multidisciplinary management. Cancer 2007;110(1):1–12. [CrossRef]
[Pubmed]
21.
Ramahi E, Choi J, Fuller CD, Eng TY. Merkel cell carcinoma. Am J Clin Oncol 2013;36(3):299–309. [CrossRef]
[Pubmed]
22.
Su C, Bai HX, Christensen S. Relative survival analysis in patients with stage I-II Merkel cell carcinoma treated with Mohs micrographic surgery or wide local excision. J Am Acad Dermatol 2018;S0190-9622(18):30816–8. [CrossRef]
[Pubmed]
SUPPORTING INFORMATION
Author Contributions
Iraqi Fatima Zahra - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Jerguigue Hounayda - Conception of the work, Design of the work, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Latib Rachida - Conception of the work, Design of the work, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Omor Youssef - Conception of the work, Design of the work, Acquisition of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Iraqi Fatima Zahra et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
