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Answers: 2009 Series -  July 7, 2009 Lecture 26 of 52  NEXT»

To see views enlarged, click on the individual pictures...

QOW070709_1A

This 82-year-old female was seen by her family practitioner with the lesion as seen in the picture above. The lesion was first noticed by the patient two months prior to presentation and had been growing steadily in size. She was otherwise fit and well, with no other medical problems.

* In this exercise, more than one answer can be correct for each question.

1. The differential diagnosis could include:

e -- all of the above

The differential diagnosis of an eyelid lesion such as the one pictured would include: cysts, chalazions, basal cell carcinomas, and merkel cell carcinoma (MCC) which is the lesion seen here.

MCC of the eyelid most commonly appears as a smooth, violaceous nodule with surface telangiectasiae, and usually without ulceration.1  There is often diagnostic confusion, probably because of its low incidence, and MCC can thus be confused with cysts, chalazions, and basal cell carcinomas.2  As a result, treatment is often delayed.


2.

If the diagnosis was merkel cell carcinoma, appropriate treatment would not include:

c -- intralesional mitomycin

All of the methods are appropriate forms of treatment for MCC except for intralesional mitomycin. The low incidence of this tumour, particularly on the eyelid, means that there are few data on optimal management. At present, the consensus seems to be that the primary tumour should be excised widely. However, it is controversial whether there should be subsequent prophylactic elective lymph node dissection, radiotherapy and/or chemotherapy.2  Wide surgical excision has been favoured as a means of treating the local site in eyelid MCCs and Mohs’ micrographic surgery, has not been widely used, but has been advocated as a suitable way of managing this tumour.3

QOW070709_1B
The picture above shows her eyelid contour after surgery.

3. Which of the following is/are true regarding merkel cell carcinoma:

a -- The most commonly affected area is the head and neck; c -- It is histologically a neuroendocrine neoplasm; and d -- It can lead to death.

Merkel cell carcinoma (MCC) of the skin is a neuroendocrine neoplasm that shares ultrastructural and immunohistochemical characteristics related to the normal merkel cell (shown below).

QOW070709_2A QOW070709_2B
Left; Haematoxylin and eosin stain of the merkel cell tumour.
Right; Immunostaining showing CD56 positivity of the merkel cell tumour.

It is a highly malignant skin cancer that occurs on sun-exposed areas. Consequently the most commonly affected area is the head and neck, accounting for 50-55% of MCCs.4  The tumour has a propensity for local, nodal and distant recurrence. It is thought that local recurrence occurs in about one-third of patients. Two-thirds have regional node involvement or recurrence, and up to one-half metastasize widely, resulting in death. Due to the risk of local recurrence or metastatic disease, these patients require careful and continued ophthlamic follow up.


References:

1. Hamilton J, Levine M, Lash R et al. Merkel cell carcinoma of the eyelid. Ophthal Surg 1993; 24(11): 764-769.

2. Peters BP, Meyer DR, Shields JA et al. Management and prognosis of merkel cell carcinoma. Ophthalmology 2001; 108:1575-1579.

3. 10. Boyer JD, Zitelli JA, Brodland DG et al. Local control of primary Merkel cell carcinoma: review of 45 cases treated with Mohs micrographic surgery with and without adjuvant radiation. J Am Acad Dermatol 2002; 47(6); 885-892.

4. Brisset AE, Olsen KD, Kasperbauer JL et al. Merkel cell carcinoma of the head and neck: a retrospective case series. Head & Neck 2002; 24: 982-988.



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