Background. Merkel cell carcinoma of the head and neck is a rare and aggressive malignant tumour. Both the dermatological and surgical colleagues should be aware of this entity as lesions usually present on sun exposed areas of the skin such as the head and neck. Main Observation and Treatment. A 69-year-old male originally presented to the maxillofacial surgery department with a growing lesion on the left eyebrow. Histological analysis confirmed Merkel cell carcinoma and consequently surgical excision was carried out. A follow-up PET/CT scan 2 years later demonstrated a hotspot in the left parotid gland. Fine needle aspiration and cytology revealed Merkel cell carcinoma. A subtotal parotidectomy left side with ipsilateral selective neck dissection levels I to III was carried out. Conclusions. Potential secondary Merkel cell carcinoma in the head and neck region should be taken into account when planning short- and long-term follow up for previously diagnosed patients. This followup should involve both dermatological and surgical colleagues. 1. Introduction Merkel cell carcinoma (MCC) is a rare and aggressive malignant tumour of neuroendocrine origin, with incidence being reported as low as 0.44/100？000 cases a year [1]. Clinical diagnosis is difficult due to a nonspecific appearance. Often the lesion presents as a nonindurated and slightly erythematous nodule. The most common location of the lesion is on sun exposed areas of the skin, with UVB radiation posing an increased risk [2]. Epidemiological studies reveal further risk in immunocompromised patients and Caucasian patients older than 50 [3]. Furthermore 48% of lesions are diagnosed in the head and neck region, with 61% of patients being male [4]. Heath et al. [3] use a favourable acronym when looking at typical clinical features at presentation: AEIOU, asymptomatic, enlarging rapidly, immunosuppression, older age, and UV exposed site. MCC has a poor 5-year prognosis, with a 75% survival for local disease and 50% for regional [5]. The prognosis is even poorer for: (1) male patients, (2) primaries of T2 size and extension, (3) nodal involvement, (4) and if metastatic disease is present [4]. The frequency of both local and regional spread of disease is high, with up to 21% of cases developing distant metastatic lesions [6]. Common sites of metastasis have been described as lymph nodes, mediastinum, lung, liver, and bone [7]. The mortality rate of MCC is twice that of melanoma [8]. Even with this aggressive nature of the disease and high risk of mortality, the awareness of MCC is poor amongst
References
[1]
N. C. Hodgson, “Merkel cell carcinoma: changing incidence trends,” Journal of Surgical Oncology, vol. 89, no. 1, pp. 1–4, 2005.
[2]
R. W. Miller and C. S. Rabkin, “Merkel cell carcinoma and melanoma: etiological similarities and differences,” Cancer Epidemiology Biomarkers and Prevention, vol. 8, no. 2, pp. 153–158, 1999.
[3]
M. Heath, N. Jaimes, B. Lemos et al., “Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU features,” Journal of the American Academy of Dermatology, vol. 58, no. 3, pp. 375–381, 2008.
[4]
V. A. Smith, E. R. Camp, and E. J. Lentsch, “Merkel cell carcinoma: identification of prognostic factors unique to tumors located in the head and neck based on analysis of SEER data,” The Laryngoscope, vol. 122, no. 6, pp. 1283–1290, 2012.
[5]
B. A. Reichgelt and O. Visser, “Epidemiology and survival of Merkel cell carcinoma in the Netherlands. A population-based study of 808 cases in 1993–2007,” European Journal of Cancer, vol. 47, no. 4, pp. 579–585, 2011.
[6]
P. J. Allen, W. B. Bowne, D. P. Jaques, M. F. Brennan, K. Busam, and D. G. Coit, “Merkel cell carcinoma: prognosis and treatment of patients from a single institution,” Journal of Clinical Oncology, vol. 23, no. 10, pp. 2300–2309, 2005.
[7]
H. Sihto, H. Kukko, V. Koljonen, R. Sankila, T. B？hling, and H. Joensuu, “Clinical factors associated with merkel cell polyomavirus infection in merkel cell carcinoma,” Journal of the National Cancer Institute, vol. 101, no. 13, pp. 938–945, 2009.
[8]
F. Q. Zhan, V. S. Packianathan, and N. C. Zeitouni, “Merkel cell carcinoma: a review of current advances,” Journal of the National Comprehensive Cancer Network, vol. 7, no. 3, pp. 333–339, 2009.
[9]
C. Andres, U. Puchta, and M. J. Flaig, “Detection of merkel cell polyomavirus DNA in atypical fibroxanthoma in correlation to clinical features,” American Journal of Dermatopathology, vol. 32, no. 8, pp. 799–803, 2010.
[10]
E. Voog, P. Biron, J. P. Martin, and J. Y. Blay, “Chemotherapy for patients with locally advanced or metastatic Merkel cell carcinoma,” Cancer, vol. 85, no. 12, pp. 2589–2595, 1999.
[11]
P. T. H. Tai, E. Yu, E. Winquist et al., “Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases,” Journal of Clinical Oncology, vol. 18, no. 12, pp. 2493–2499, 2000.
[12]
T. Jouary, C. Leyral, B. Dreno et al., “Adjuvant prophylactic regional radiotherapy versus observation in stage I merkel cell carcinoma: a multicentric prospective randomized study,” Annals of Oncology, vol. 23, no. 4, pp. 1074–1080, 2012.
[13]
S. G. Gupta, L. C. Wang, P. F. Pe？as, M. Gellenthin, S. J. Lee, and P. Nghiem, “Sentinel lymph node biopsy for evaluation and treatment of patients with Merkel cell carcinoma: the Dana-Farber experience and meta-analysis of the literature,” Archives of Dermatology, vol. 142, no. 6, pp. 685–690, 2006.
[14]
A. E. Brissett, K. D. Olsen, J. L. Kasperbauer et al., “Merkel cell carcinoma of the head and neck: a retrospective case series,” Head and Neck, vol. 24, no. 11, pp. 982–988, 2002.
