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specific lymph nodes are involved, by adjuvant radiation
therapy within 4 weeks. Adjuvant chemotherapy in
regional disease could be considered depending on clinical
judgment. In cases of disseminated disease, chemotherapy
represents first line therapy; the choice of the agents to be
taken based on clinical judgment and experience.
Here we present a case of MCC in an elderly man. The
patient is consented and agrees with this publication.
CASE REPORT
An 86-year-old man presented with a purple-violaceous
mass with vegetations in the left pre-auricular region,
extending to the maxilla and involving the parotid area
[Figure 1]. The lesion had been enlarging for more than Figure 2: Response to therapy after 1 month of treatment
three months. The patient had no history or evidence of
comorbidities, apart from gallbladder stones and allergy
to penicillin. An incisional biopsy was performed MCC.
The neoplasm involved dermis and hypodermis;
histology showed a dense infiltrate of small tumour
cells with hyperchromatic nuclei and lacking cytoplasm.
Immunohistochemistry was consistent with the diagnosis
of MCC.
The immune histochemical phenotype of the dermal-
located malignant cells was characterized by dot-like focal
positivity for Cytokeratin 20 (CK20+), diffuse positivity
for synaptophysin(+), cytokeratin AE1/AE3, CD99+, and
strong nuclear positivity for Ki-67 (+100%). There was
negative staining for chromogranin, CEA-, TTF1-, CD56-, Figure 3: Complete clinical response after 2 months
S100-, CD20-, CD79a-, CD3-, CD23-, CD5-, CD10-, and
Cyclin D1-. studies. Computed tomography (CT) scans showed no
involvement of local lymph-nodes or distant metastases.
The tumour was classified MCC, T2, locally advanced. Based on these clinical findings, the history, and on his
No other abnormalities were detected in the laboratory age, an oral chemotherapeutic treatment was proposed.
The patient started oral etoposide with the dosage schedule
of 50 mg/m per 10 days followed by 7 days rest.
2
After one month of treatment the tumour showed a
significant response [Figure 2]. There were side effects or
laboratory abnormalities.
By 2 months there was evidence of complete objective
response [Figure 3]. Considering the results, therapy was
held. Adjuvant radiation was then given.
DISCUSSION
First described as trabecular carcinoma in 1972 by
Toker, MCC represents an aggressive, primary
[6]
cutaneous carcinoma incorporating both epithelial and
neuroendocrine features. The diagnosis is made by clinical
evaluation and biopsy, although other small round cell
tumors may be considered. For this reason a complete
immunohistochemistry panel is needed for the correct
diagnosis. Cytokeratin 20 (CK-20), a marker of epithelial
origin, is a very sensitive marker for MCC since it is
[7]
positive in 89-100% of cases. Together with negativity
Figure 1: Merkel cell carcinoma at time of first evaluation of transcription factor 1 (TTF-1), it provides the greatest
Journal of Cancer Metastasis and Treatment ¦ Volume 2 ¦ August 17, 2016 ¦ 311