   |
|
CORRESPONDENCE |
|
| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 761-763 |
|
| Concurrent merkel cell carcinoma and bowen's disease in a young lady |
|
|
Meenakshi Swain1, Anuja Yadav1, Devyani Pendharkar1, Satyanath Patnaik2
1 From the Department of Histopathology, Apollo Hospitals, Hyderabad, Telangana, India
2 Patnaik's Skin Clinic, Hyderabad, Telangana, India
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Meenakshi Swain
From the Department of Histopathology, Apollo Hospitals, Hyderabad, Telangana
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_926_20
|
|
How to cite this article:
Swain M, Yadav A, Pendharkar D, Patnaik S. Concurrent merkel cell carcinoma and bowen's disease in a young lady. Indian J Dermatol 2022;67:761-3 |
How to cite this URL:
Swain M, Yadav A, Pendharkar D, Patnaik S. Concurrent merkel cell carcinoma and bowen's disease in a young lady. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:761-3. Available from: https://www.e-ijd.org/text.asp?2022/67/6/761/370356 |
Sir,
Merkel cell carcinoma (MCC) is a rare cutaneous malignant neuroendocrine carcinoma first described in 1972 by Toker.[1] It affects elderly males and has a predilection for the head and neck region. Other affected sites include the extremities and trunk with rare cases in the oral and genital mucosa. It is occasionally found coexisting with other diseases, such as squamous cell carcinoma, basal cell carcinoma, actinic keratosis, and miscellaneous adnexal tumours.[2] However, its association with Bowen's disease is rare and its presentation at a young age is unusual. Early and correct diagnosis is possible with immunohistochemical techniques.
A 32-year-old lady presented with a reddish ulcerated nodule on the dorsum of hand of 2 months duration. The lesion was excised and sent for histopathological evaluation [Figure 1].
Macroscopically, the lesion measured 1.2 cm in diameter with an overlying crust. Histological examination revealed a round cell tumour in the dermis. Brisk mitosis was seen, averaging 8–10/high power field. The overlying epidermis depicted areas of full thickness dysplasia with loss of polarity, moderate nuclear pleomorphism, and atypical mitosis. The basement membrane was intact. The differential diagnosis considered for the dermal tumour were lymphoma, neuroendocrine tumour, and small cell carcinoma [Figure 2]. | Figure 2: a - Tumour in the dermis composed of monomorphic small cells arranged in sheets (H and E stain; Low power magnification, 10X). b - Tumour showing monomorphic cells with hyperchromatic nuclei, scant cytoplasm, and mitoses. (H and E stain; High power magnification, 40X). c - Overlying epidermis with Bowen's disease. (H and E stain; medium and high power magnification, 20X). d - Overlying epidermis with Bowen's disease (H and E stain; medium and high power magnification, 40X)
Click here to view |
Immunohistochemically, the tumour in the dermis demonstrated diffuse positivity for neuron specific enolase (NSE) and perinuclear dot-like expression of cytokeratin 20 (CK20). It was negative for chromogranin, leucocyte common antigen (LCA), and thyroid transcription factor (TTF-1), thus confirming the diagnosis of MCC. Cytokeratin 5/6 (CK5/6) demonstrated cytoplasmic positivity in overlying squamous cells, dermal tumour cells being negative. This immunohistochemical profile confirmed a diagnosis of MCC with Bowen's disease, having excluded the other differential diagnosis [Figure 3]. | Figure 3: a - Dermal tumour demonstrated diffuse cytoplasmic granular positivity for NSE. (IHC; Medium power magnification, 20X). b - Dermal tumour depicting perinuclear dot-like expression of CK20. (IHC; High power magnification, 40X). c - CK 5/6 demonstrated cytoplasmic positivity in overlying squamous cells, dermal tumour is negative. (IHC; Medium power magnification, 20X). d- The tumour cells were negative for chromogranin, TTF-1, and LCA. (IHC; Low power magnification, 10X). (h and e: Haematoxylin and Eosin stain, IHC: Immunohistochemistry, NSE: Neuron specific enolase, TTF-1: Thyroid transcription factor 1, LCA: Leukocyte common antigen)
Click here to view |
Lymph node dissection performed later at a tertiary cancer centre depicted two lymph nodes out of 14 with metastases.
MCC is a rare aggressive cutaneous neuroendocrine carcinoma considered to arise from Merkel cells, since the cells contain electron-dense core granules, which is believed to be a feature of Merkel cells.[1],[3]
MCC is known to be associated with squamous cell carcinoma, basal cell carcinoma, actinic keratosis, miscellaneous adnexal tumours, and rarely Bowen's disease. Smith et al.[4] suggested that MCC may arise from a primitive pluripotent stem cell with a capacity to differentiate along different cell lines. On the other hand, Gomez et al.[5] suggested that there may be a possible common carcinogenic influence on different precursor cells of the skin that leads to the coexistence of different tumours.
The association of Bowen's disease with MCC is rare and the exact cause is unknown. According to several studies, arsenic is considered to induce the occurrence of MCC in addition to squamous cell carcinoma, basal cell carcinoma, and Bowen's disease.[6] This patient did not have any history of arsenic exposure, viral infection, or immunosuppression.
MCC involves the dermis and the subcutaneous tissue. The epidermis is rarely involved by the tumour. This case showed focal areas of Bowen's disease in the overlying epidermis. MCC can be confused histologically with lymphoma, melanoma, undifferentiated squamous cell carcinoma, eccrine sweat gland carcinoma, neuroblastoma, Ewing's sarcoma, leukemic infiltrates, and metastatic small cell carcinoma of the lung. Immunohistochemical stains are required for confirming the diagnosis. CK20 is the most useful immunohistochemical stain for MCC with a perinuclear dot-like staining pattern. Additionally, the tumour cells stain for neuron-specific enolase (NSE), chromogranin, synaptophysin, and neurofilaments. Absent staining for chromogranin, TTF-1, and LCA exclude metastatic small cell carcinoma and lymphoma.
Park et al.[7] in 2012 reported that MCC is concurrent with Bowen's disease in a 75-year-old female on the left mandibular angle. In 2013, Ishida M et al. reported two cases, an 86-year-old male and another 87-year-old female who presented with nodules on the chest and cheek, respectively.[8] There are recently reported cases of MCC concurrent with Bowen's disease mostly affecting older age groups with head and neck being common sites.[9]
The association of MCC with Bowen's disease is rare, mostly limited to single case reports.[10]
Diagnosis of MCC is important as it is an aggressive tumour. The preferred treatment is surgical excision with sentinel lymph node biopsy followed by lymph node dissection, if the latter is positive. Postoperative radiotherapy is also administered.
This patient had an axillary dissection because of a palpable lymph node. Two lymph nodes out of 14 showed metastatic deposits, hence the female patient received radiotherapy after which she is well and completely free of disease now, 7 years after the initial diagnosis.
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 | |  |
| 1. | Toker C. Trabecular Carcinoma of the Skin. Arch Dermatol 1972;105:107-10.  |
| 2. | Sirikanjanapong S, Melamed J, Patel RR. Intraepidermal and dermal Merkel cell carcinoma with squamous cell carcinoma in situ: A case report with review of literature. J Cutan Pathol 2010;37:881-5. |
| 3. | Freeman MBB, Holman DM, Qin J, Lunsford NB. Merkel cell carcinoma incidence, trends, and survival rates among adults aged ≥50 years from United States Cancer Statistics. J Am Acad Dermatol 2019;80:1154-6. |
| 4. | Smith KJ, Skelton HG 3 rd, Holland TT, Morgan AM, Lupton GP. Neuroendocrine (Merkel cell) carcinoma with an intraepidermal component. Am J Dermatopathol 1993;15:528-33. |
| 5. | Gomez LG, Silva EG, DiMaio S, Mackay B. Association between neuroendocrine (Merkel cell) carcinoma and squamous carcinoma of the skin. Am J Surg Pathol 1983;7:171-7. |
| 6. | Lien HC, Tsai TF, Yu Yun Lee, Hsiao CH. Merkel cell carcinoma and chronic arsenicism. J Am Acad Dermatol 1999;41:641-3. |
| 7. | Park HC, Kang HS, Park KT, Oh YH, Yu HJ, Kim JS. Merkel cell carcinoma concurrent with Bowen's disease. Ann Dermatol 2012;24:77-80. |
| 8. | Ishida M, Okabe H. Merkel cell carcinoma concurrent with Bowen's disease: Two cases, one with an unusual immunophenotype. J Cutan Pathol 2013;40:839-43. |
| 9. | Müller-Richter UDA, Gesierich A, Kübler AC, Hartmann S, Brands RC. Merkel cell carcinoma of the head and neck: Recommendations for diagnostics and treatment. Ann Surg Oncol 2017;24:3430-7. |
| 10. | Kiyohara T, Shijimaya T, Miyamoto M, Nagano N, Nakamaru S, Makimura K, et al. In-transit recurrence of Merkel cell carcinoma associated with Bowen's disease: The first reported case successfully treated by avelumab. J Dermatol 2019;46:440-3. |
[Figure 1], [Figure 2], [Figure 3] |
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 176 | | | Printed | 8 | | | Emailed | 0 | | | PDF Downloaded | 10 | | | Comments | [Add] | | |
|
|
