Secondary Merkel Cell Carcinoma Manifested in the Parotid

Hindawi Publishing Corporation Case Reports in Dermatological Medicine Volume 2013, Article ID 960140, 4 pages http://dx.doi.org/10.1155/2013/960140 Case Report Secondary Merkel Cell Carcinoma Manifested in the Parotid M. Basati, K. Kassam, and A. Messiha Northwick Park Hospital, Watford Road, Harrow HA1 3UJ, UK Correspondence should be addressed to M. Basati; Received 2 October 2013; Accepted 23 October 2013 Academic Editors: B. Giomi and M. Viglione Copyright © 2013 M. Basati et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 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 practitioners. The following case report aims to increase awareness and stimulate debate and education within units. 2. Case Presentation A 69-year-old male originally presented to the oral and maxillofacial department in 2010 with a gradually increasing lump in the left eyebrow region of four weeks duration. A diagnosis of localised MCC was made following wide local excision. There was total eradication of residual disease. The patient was under regular follow-up scans and in 2012 a PET/CT revealed a 1.3 cm left intraparotid node of uncertain significance. Medical history to note was noninsulin dependent diabetes, hypertension, and atrial fibrillation. The patient had good social support, living with wife and extended family. 3. Investigations The patient underwent an urgent FDGPET and fine needle aspirate (FNA) ultrasound. The FNA ultrasound together with cytopathology (positive for CAM5.2 and CK20) was 2 Case Reports in Dermatological Medicine Figure 1: Ultrasound of parotid/neck region. Within the right parotid a 15 mm hypoechoic lesion was seen. Initial differentials: salivary gland tumour and abnormal possibly necrotic intraparotid node. suggestive of MCC in the parotid (Figure 1). Immunocytochemistry was performed for confirmation. The results of the investigations were discussed at both skin and head and neck multidisciplinary team (MDT) meeting with colleagues, and it was decided that the most appropriate management was a subtotal parotidectomy left side with ipsilateral selective neck dissection levels I to III. 4. Differential Diagnosis The following is a differential diagnosis for MCC, and the list is by no means exhaustive [9]: (i) basal cell carcinoma, (ii) small cell melanoma, (iii) lymphoma, (iv) blue round cell tumours, (v) metastatic small lung carcinoma. 5. Treatment An extra oral incision from the left preauricular region to the left neck was made and raised. Anterior, superior, and posterior flaps were developed in aid to carry out a selective neck dissection I–III with identification and preservation of the following anatomical structures: Figure 2: Careful dissection revealing the branches of the facial nerve which were preserved. The facial nerve passes through the parotid gland after emerging from the stylomastoid foramen. The arrow points to the point of division into five branches: temporal, zygomatic, buccal, marginal mandibular, and cervical. the branches of the facial nerve, in particular the marginal mandibular branch. The marginal mandibular branch of the facial nerve provides motor innervations to the muscles of the lower lip and the chin. Damage to this nerve can leave the patient with a drooping lip and chin on that side (Figure 2). The specimen was removed with further deep harvest of salivary tissue on islands (Figure 3). Drains were placed and closure was carried out in layers. 6. Outcome and Followup Histopathology revealed a well-sampled parotid gland with no metastatic tumour within salivary parenchyma. One intraparotid lymph node was almost completely replaced by a poorly differentiated tumour (20 mm in diameter) in keeping with metastatic MCC. There was no extracapsular spread. A final diagnosis of metastatic MCC left parotid was made. The facial nerve was spared and immediately after surgery the patient had good facial expression and no signs of neurological deficit. At a short-term follow-up appointment the patient had no shoulder weakness and only slight weakness of his lower lip. As this weakness was not present immediately after surgery it is likely this is a temporary paresis due to post-op oedema. The patient is under regular followup and at present free of disease. (i) (...truncated)