Page 13 - Read Online

sensitivity and specificity to exclude small cell lung needed to rule out possible recurrence.
cancer, although N-specific enolase, synaptophysin, and
[8]
chromogranin-A represent markers of neuroendocrine Financial support and sponsorship
origin with possible positivity. In our case, pathology Nil.
and immune-histochemical markers, along with clinical
features confirmed the diagnosis. Conflicts of interest
There are no conflicts of interest.
MCC generally shows a malignant behaviour, with regional
lymph-nodes as well as distant metastasis being frequently REFERENCES
involved. Twenty-five percent of patients present with
lymphadenopathy and 5% with a distant metastasis. Skin, 1. Morrison KM, Miesegaes GR, Lumpkin EA, Maricich SM.
lung, nervous system, bone, and liver are the most frequent Mammalian Merkel cells are descended from the epidermal lineage.
Dev Biol 2009;336:76-83.
secondary locations. For this reason lymph-nodal 2. Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N,
[9]
examination should be performed. Additionally, PET/CT Schwartz AM, Henson DE. Merkel cell carcinoma demographics,
is often useful for complete staging. morphology, and survival based on 3870 cases: a population based
study. J Cutan Pathol 2010;37:20-7.
Surgery plays the key role for clinically localized MCC and 3. Lemos BD, Storer BE, Iyer JG, Phillips JL, Bichakjan CK, Fang LC,
a complete surgical excision with 2 cm safety margin, if Johnson TM, Liegeois-Kwon NJ, Otley CC, Paulson KG, Ross MI,
feasible, seems to be the best treatment approach. Adjuvant Yu SS, Zeitouni NC, Bryd DR, Sondak VK, Gershenwald JE, Sober
AJ, Nghiem P. Pathologic nodal evaluation improves prognostic
radiation with 50 Gy to the tumour bed and regional lymph- accuracy in Merkel cell carcinoma: analysis of 5823 cases as the
nodes is also recommended, especially for advanced local basis of the first consensus staging system. J AM Acad Dermatol
and regional disease. When surgery is not feasible, 2010;67:751-61.
[10]
radiation therapy alone, or combined with alternative 4. Becker JC, Houben R, Ugurel S, Trefzer U, Pfohler C, Schrama D.
therapy (e.g., chemotherapy) should be considered. MC polyomavirus is frequently present in Merkel cell carcinoma of
European patients. J Invest Dermatol 2009;129:248-50.
5. Heath M, Jaimes N, Lemos B, Mostaghimi A, Wang LC, Peñas
Presently, there is no first-line chemotherapy established PF, Nghiem P. Clinical characteristics of Merkel cell carcinoma at
for MCC, since no controlled randomized trials exist. Only diagnosis in 195 patients: the AEIOU features. J AM Acad Dermatol
retrospective case series and case reports are available. 2008;58:375-81.
6. Toker C. Tarbecular carcinoma of the skin. Arch Dermatol
The chemotherapy regimens used have combined 1972;105:107-10.
carboplatin or cisplatin with etoposide, cyclophosphamide 7. Scott MP, Helm KF. Cytokeratin 20: a marker for diagnosing
with vincristine, doxorubicin, bleomycin, or 5-fluorouracil. 8. Merkel cell carcinoma. Am J Dermatopathol 1999;21:16-20.
Hanly AJ, Elgart GW, Jorda M, Smith J, Nadji M. Analysis of
thyroid transcription factor-1 and cytokeratin 20 separates merkel
Despite a good initial response, early recurrences are the cell carcinoma from small cell carcinoma of lung. J Cutan Pathol
rule. In a retrospective analysis including a wide number 2000;27:118-20.
of patients, adjuvant chemotherapy was linked to a worse 9. Medina-Franco H, Urist MM, Fiveash J, Heslin MJ, Bland KI,
overall survival compared to patients who did not received Beenken SW. Multimodality treatment of Merkel cell carcinoma:
chemotherapy. [3] case series and literature review of 1024 cases. Ann Surg Oncol
2001;8:204-8.
10. Lebbe C, Becker JC, Grob JJ, Malvehy J, Del Marmol V,
In our patient, considering locally advanced disease, Pehamberger H, Peris K, Saiag P, Middleton MR, Bastholt L,
age, and patient’s history, we decided to start mono- Testori A, Stratigos A, Garbe C; European Dermatology Forum
chemotherapy with oral etoposide. (EDF), the European Association of Dermato-Oncology (EADO)
and the European Organization for Research and Treatment
Previously, one group achieved complete responses in 3 of Cancer (EORTC). Diagnosis and treatment of Merkel cell
out of 4 MCC patients treated with oral etoposide, two of carcinoma. European consensus-based interdisciplinary guideline.
whom had rather long remissions (16 and 36 months). [11] Eur J Cancer 2015;51:2396-403.
11. Schlaak M, Podewski T, Von Bartenwerffer W, Kreuzberg N,
Bangard C, Mauch C, Kurschat P. Induction of durable responses
Our patient achieved a complete objective response in by oral etoposide monochemotherapy in patients with metastatic
a short period of time. However, long term follow-up is Merkel cell carcinoma. Eur J Dermatol 2012;22:187-91.

312
Journal of Cancer Metastasis and Treatment ¦ Volume 2 ¦ August 17, 2016 ¦