• Users Online: 167
  • Print this page
  • Email this page


 
  Table of Contents  
CONCISE COMMUNICATION
Year : 2021  |  Volume : 12  |  Issue : 1  |  Page : 156-158  

Ulcerative variant of merkel cell carcinoma in an immunocompetent individual: An unusual presentation


1 Department of Dermatology, Base Hospital Delhi Cantt, New Delhi, India
2 Department of Dermatology, Military Hospital Kirkee, Pune, Maharashtra, India
3 Department of Pathology, Command Hospital, Pune, Maharashtra, India
4 Department of Nuclear Medicine, Command Hospital, Pune, Maharashtra, India
5 Department of Dermatology, Military Hospital, Ramgarh, Jharkhand, India

Date of Submission26-Mar-2020
Date of Decision23-Jul-2020
Date of Acceptance12-Sep-2020
Date of Web Publication16-Jan-2021

Correspondence Address:
Anwita Sinha
Department of Dermatology, Military Hospital Kirkee, Pune, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_167_20

Rights and Permissions

How to cite this article:
Singh GK, Sinha A, Mishra PS, Jain A, Beniwal NS. Ulcerative variant of merkel cell carcinoma in an immunocompetent individual: An unusual presentation. Indian Dermatol Online J 2021;12:156-8

How to cite this URL:
Singh GK, Sinha A, Mishra PS, Jain A, Beniwal NS. Ulcerative variant of merkel cell carcinoma in an immunocompetent individual: An unusual presentation. Indian Dermatol Online J [serial online] 2021 [cited 2021 Feb 26];12:156-8. Available from: https://www.idoj.in/text.asp?2021/12/1/156/307158



Sir,

Merkel cell carcinoma (MCC) also called as cutaneous neuroendocrine carcinoma generally presents as rapidly growing solitary firm red to violaceous nodule with a smooth shiny surface with the involvement of photo exposed areas of face and neck in 50% cases, extremities in 40% of cases, and trunk and mucosa in less than 10% of cases.[1] Ulceration is rarely observed in advanced cases. Risk factors for MCC include advanced age with mean age of diagnosis at 70 years, UV radiation, immunosuppressed states like organ transplant recipients, HIV infection, or malignancies.[2] Merkel cell polyoma virus (MCPyV) has been recently postulated in the pathogenesis of MCC with MCPyV found to be clonally integrated in the genome of MCC tumor cells in 80% cases.[3] It is an aggressive tumor with poor prognosis, with a 5-year mortality of 40% and shows a tendency for local recurrence, lymph node involvement, and distant metastasis.[4] MCC is a very rare skin cancer with an incidence of 0.44/1,00,000 in US[3] and has been rarely reported in Indian population.[4] We report a case of MCC in an elderly male with two unusual clinical features–arising on photo protected site on lower back as an ulcerated growth mimicking squamous cell carcinoma and developing de novo without any underlying immunosuppression.

An 85-year-old male patient presented with asymptomatic gradually progressive growth over the lower back of 4 years duration, rapidly progressing since 4 months with ulceration. There was no history of weight loss or any other systemic complaints. Before presenting to our center patient had consulted a few other clinicians; however, the lesion was not subjected to histopathology examination. Examination revealed the involvement of left lower back in the form of solitary fungating growth with ulceration measuring 5 cm × 4 cm × 3.5 cm. Few sub-centimetric, discrete, hyperpigmented to erythematous satellite plaques were present in the vicinity of growth [Figure 1]a and [Figure 1]b. Patient was investigated with the differentials of squamous cell carcinoma, amelanotic melanoma, cutaneous lymphoma, and chronic granulomatous diseases like cutaneous leishmaniasis. Hematological and biochemical investigations were normal. Enzyme-linked immunosorbent assay for HIV was negative.
Figure 1: (a) Fungating growth with ulceration. (b) Hyperpigmented to erythematous satellite plaques present in the vicinity of growth

Click here to view


Skin biopsy for histopathology showed ulcerated tissue infiltrated by nests and trabeculae of round blue cells on low power, with high nucleocytoplasmic ratio, scant cytoplasm, dispersed powdery chromatin, inconspicuous nucleoli, and brisk mitotic activity seen on higher power [Figure 2]a, [Figure 2]b, [Figure 2]c. Immunohistochemistry was done to differentiate small round blue cell tumors of skin which showed positive CK20 [Figure 3]a, synaptophysin [Figure 3]b, chromogranin [Figure 3]c, CD56, EMA, and CD117 along with negative p40, CK7, vimentin, LCA, CD3, CD20, BEREP4, and CD138. MIB1 labelling index was high both in tumor cells and underlying squamous epithelium. Lympho-vascular invasion was seen at tumor margins. Final diagnosis was given as MCC with carcinoma in situ changes in lining squamous epithelium. Diagnosis of in situ carcinoma changes in the underlying squamous epithelium was made on the basis of histopathological features of high-grade dysplasia along with findings of high MIB-1 index with no unequivocal invasion by dysplastic squamous cells.
Figure 2: (a) Haematoxylin and eosin stain-5×: Ulcerated tissue infiltrated by nests and trabeculae of round blue cells. (b) Haematoxylin and eosin stain-10×: Ulcerated tissue infiltrated by nests and trabeculae of round blue cells. (c) Haematoxylin and eosin stain-20× High nucleocytoplasmic ratio, scant cytoplasm, inconspicuous nucleoli, and brisk mitotic activity

Click here to view
Figure 3: (a) Tumor cells positive for cytokeratin 20. (b) Tumor cells positive for synaptophysin. (c) Tumor cells positive for chromogranin

Click here to view


Positron Emission Tomography-Computed Tomography (PET-CT) scan for tumor and metastasis revealed FDG avid exophytic lesion in posterior abdominal wall, FDG avid nodular lesions in abdomen abutting left internal oblique muscle, and subcutaneous nodular lesions in Rt inguinal region suggesting metastasis [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. Patient was managed with wide local excision of tumor with 1 cm margin. Gross section of the excised specimen revealed an encapsulated nodular ulcerative tumor measuring 5 cm × 4 cm × 3.5 cm. Cut surface was gray pink, soft with areas of punctate hemorrhages and necrosis. Patient was started on radiotherapy and was offered chemotherapy which he declined, and subsequently succumbed to his illness within 2 months of diagnosis of the disease.
Figure 4: (a) FDG avid exophytic lesion in posterior abdominal wall. (b) FDG avid nodular lesions in abdomen abutting left internal oblique muscle. (c) FDG avid subcutaneous nodular lesions in right inguinal region. (d) MIP images in coronal section showing metastatic metabolically active right inguinal lymph node

Click here to view


The first description of MCC was given by Toker who called it Trabecular carcinoma of the skin.[5] MCC has a varied clinical presentation although rapid growth of an asymptomatic red violaceous papule or nodule is classically noted. In the absence of underlying immunodeficiency syndromes and diseases, age-related decline of immune function and cumulative doses of UV rays are implicated as risk factors.[6] In our reported case, advanced age of 85 years was a contributing factor, but the photo protected site of lower back is a rare site for origin of MCC.

Clinical differentials of squamous cell carcinoma, cutaneous lymphoma, and amelanotic melanoma were given initially owing to the atypical ulcerative and fungating morphology and slow growth of tumor for 4 years. Histopathology and immunohistochemistry are of paramount importance in clinching the diagnosis as was seen in our case. Three histopathological patterns have been described: (1) Trabecular type with connective tissue separating interconnecting cellular trabeculae, (2) Intermediate cell type, which is commonest, with solid nests and trabeculae at periphery, and (3) Small cell type consisting of sheets of small cells with a diffusely infiltrative pattern.[3] In the present case, the predominant pattern was of sheets of small round cells. The tumor cells in MCC are rounded, monomorphic, small to medium sized with scanty cytoplasm, round nuclei with inconspicuous nucleoli (blue cells). The nuclei have finely granular chromatin and may show the typical salt and pepper appearance.[7]

MCC is histologically indistinguishable from metastasis of small cell lung cancer, hematological malignancy, and other skin tumors with neuroendocrine differentiation, thereby highlighting the importance of immunohistochemistry. CK20 positivity, in a distinctive peri-nuclear dot-like pattern, is strongly specific staining 80%–90% of MCC. The dense core granules of MCC show immunoreactivity for neuroendocrine markers, chromogranin, synaptophysin, and neuron-specific enolase.[8] IHC for MCPyV antigen is also specific, however, could not be done in our case due to nonavailability. Histological features associated with poor outcome include tumor size (≥5 mm), extension into the subcutaneous tissue, diffuse growth pattern, absence of an intratumoural T-cell infiltrate, and high mitotic rate.[9]

MCC is a very aggressive tumor and, hence, the need for early diagnosis and staging. The most frequent sites of metastasis are skin, lymphatics, and liver although potentially any organ including CNS can be affected. PET-CT scans have been widely used in MCC for detecting locoregional nodal and distant metastatic disease and staging work up. It is a noninvasive imaging technique that has the potential to detect occult lesions bigger than 5–8 mm in minimal diameter that are not detectable by other imaging techniques.[10] As PET-CT scanning is a whole-body scan, it helped us in delineating the metastatic metabolically active lesions and lymph nodes, as well as the high metabolic activity of primary lesion suggesting its aggressiveness.

Management of MCC is dependent on tumor staging at presentation, with curative intent for locoregional disease and palliative intent for distant disease. Optimal management of MCC includes surgical excision of primary tumor with wide margins, and adjuvant radiotherapy, chemotherapy, immunotherapy, and molecular-targeted therapy in the case of metastasis.[11] Our patient was managed with wide local excision of the tumor with the histopathological margins being free of tumor and was started on radiotherapy for subcutaneous and intraabdominal metastasis. Patient was offered chemotherapy which he declined, and succumbed to his illness within two months of diagnosis of MCC.

Our case highlights an unusual variant of MCC presenting as an insidious onset ulcerated growth over lower back in an elderly male without any underlying immunosuppression. A high index of suspicion is of paramount importance on the part of the treating dermatologist, for prompt and early diagnosis of MCC to arrest the disease in its incipient stages.

Acknowledgement

Nagendra Singh Beniwal-Department of Dermatology, Military Hospital, Ramgarh, Jharkhand.

Isha Sharma-Department of Pathology, Armed Forces Medical College, Pune.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Cirillo F, Vismarra M, Cafaro I, Martinotti M. Merkel cell carcinoma: A retrospective study on 48 cases and review of literature. J Oncol 2012;2012:749030.  Back to cited text no. 1
    
2.
Smith PD, Patterson JW. Merkel cell carcinoma (neuroendocrine carcinoma of the skin). Am J Clin Pathol2001;115:S68-78.  Back to cited text no. 2
    
3.
Schadendorf D, Lebbé C, Zur Hausen A, Avril M-F, Hariharan S, Bharmal M, et al. Merkel cell carcinoma: Epidemiology, prognosis, therapy and unmet medical needs. Eur J Cancer. 2017;71:53-69.  Back to cited text no. 3
    
4.
Gaopande VL, Joshi AR, Khandeparkar SG, Deshmukh SD. Merkel cell carcinoma of the abdominal wall. Indian Dermatol. Online J 2015;6:269-73.  Back to cited text no. 4
    
5.
Khader A, George S, Balakrishnan S, Aravindan KP. Spontaneous regression in an ulcerated CK7 positive Merkel cell carcinoma. Indian J Dermatol Venereol Leprol 2015;81:170-3.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Schrama D, Becker J. Merkel cell carcinoma–pathogenesis, clinical aspects and treatment. J Eur Acad Dermatol 2011;25:1121-9.  Back to cited text no. 6
    
7.
Bichakjian CK, Lowe L, Lao CD, Sandler HM, Bradford CR, Johnson TM, et al. Merkel cell carcinoma: Critical review with guidelines for multidisciplinary management. J.Cancer 2007;110:1-12.  Back to cited text no. 7
    
8.
Hanly AJ, Elgart GW, Jorda M, Smith J, Nadji M. Analysis of thyroid transcription factor-1 and cytokeratin 20 separates Merkel cell carcinoma from small cell carcinoma of lung. J Cutan Pathol 2000;27:118-20.  Back to cited text no. 8
    
9.
Becker C, Schrama D. Merkel cell carcinoma. In: Griffith C, Barker J, Bleiker T, editors. Rook's Textbook of Dermatology. 9th ed. Wiley Blackwell; 2016. p. 145.3.  Back to cited text no. 9
    
10.
Enzenhofer E, Ubl P, Czerny C, Erovic BM. Imaging in patients with merkel cell carcinoma. J Skin Cancer 2013;2013:973123.  Back to cited text no. 10
    
11.
Rockville Merkel Cell Carcinoma Group. Merkel cell carcinoma: Recent progress and current priorities on etiology, pathogenesis, and clinical management. J Clin Oncol 2009;27:4021-6.  Back to cited text no. 11
    


    Figures

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



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    References
    Article Figures

 Article Access Statistics
    Viewed138    
    Printed0    
    Emailed0    
    PDF Downloaded13    
    Comments [Add]    

Recommend this journal