Merkel cell carcinoma of the eyelid: A review

Merkel cell carcinoma (MCC) is a rare, aggressive tumor of both epithelial and neuroendocrine origin, which carries a mortality rate of up to 40%. MCC tumors typically present as painless, expanding nodules on the sun-exposed skin areas of older, white patients. Eyelid and periocular tumors comprise approximately 2.5% of all cases of MCC and may be mistaken for chalazia or basal cell carcinomas. Immunosuppression is a significant risk factor, particularly in solid-organ–transplant recipients, patients with chronic lymphocytic leukemia, and patients with HIV. Sentinel lymph node biopsy is often used for accurate staging of head and neck MCC. Treatment includes wide local excision, commonly with the addition of radiotherapy for improved locoregional disease control. Historically, adjuvant chemotherapy had been reserved for metastatic disease, but immunotherapy and targeted chemotherapies are currently being investigated for use in primary disease. The clinical characteristics of all available published cases of eyelid MCC are summarized in this article.     

A case series of Merkel cell carcinoma of the eyelid: a rare entity often misdiagnosed

Merkel cell carcinoma (MCC) is one of the rarest eyelid tumors, with high mortality rate due to lymphatic and metastatic spread. We hereby report six cases of patients with histological diagnosis of MCC referred to our Orbit Unit between 2012 and 2018, focusing on diagnosis, treatment, and subsequent follow up. All patients underwent surgical excision and systemic work-up. Both MCC TNM and eyelid MCC TNM were used to stage lesions. MCC of the eyelid is usually misdiagnosed as benign or other malignant lesions. A prompt examination and a wide local excision are mandatory. A close follow-up of these patients is advised due to high recurrence rate and lymphatic spread.     

Unusual Merkel Cell Carcinoma of the Eyelid

Merkel cell carcinoma of the eyelid is a rare tumor with less than 100 reported cases worldwide. We describe an unusual presentation of Merkel cell carcinoma of the eyelid in a 60 year old Asian male. He presented with multiple left lower lid conjunctival nodules, intense conjunctival erythema, as well as ipsilateral cervical lymphadenopathy. An incisional biopsy diagnosed him with Merkel cell carcinoma with a PET scan showing distant metastatic disease. He was then treated with chemotherapy. The combination of a presentation of conjunctival nodules and erythema, location in the lower eyelid and the conjunctiva, the presence of metastatic disease on diagnosis as well as an unusual immunohistochemical profile make this an unusual case.     

Orbital invasiveness of Merkel cell tumor of the eyelid]

A new case of neuroendocrine carcinoma (Merkel cell tumor) of the eyelid is reported. Local recurrence, spread to the orbit, led to exenteration and death of the patient. This trabecular carcinoma can be identified by histology, immuno-histochemistry and electron structural microscopy.     

Merkel cell carcinoma of the eyelid

The clinical and pathological features of two Merkel cell carcinomas of the eyelid skin are reported. Both tumours occurred in the upper lid in elderly women and enlarged rapidly to form smooth, round nodules which projected from the lid surface anterior to the grey line. In one case the primary excision biopsy was followed by a wider excision in 1982, and the patient has been free from recurrence or metastases in the intervening period. In the second more recent case, the tumour was inadequately excised in 1984 and the lid was treated by radiotherapy: this patient is at present free from recurrence. Histological examination of each tumour revealed an identical morphology — characterised by the presence of uniform polyhedral cells with multiple nucleoli lying in nests and lobules within the dermis: mitotic figures were prominent. Immunohistochemical studies showed the cells to react positively with antisera against neurone-specific enolase, epithelial membrane antigen and met-enkephalin. Electron microscopy revealed only rare neuroendocrine granules in one tumour, and numerous granules in the other. Intranuclear rodlets were not identified and there was paucity of cell membrane attachment modifications.     

Merkel cell turnour occurring simultaneously in the upper and lower eyelids

Purpose: To highlight an unusual presentation of two Merkel cell turnours in the same patient.
Result We report a case of two Merkel cell turnours occurring simultaneously in the upper and lower eyelids of an elderly woman, a unique occurrence. Wide local resection and reconstruction resulted in microscopic clearance of the tumours. Lymphatic spread was detected, a common feature. She had previously undergone excision of a similar lesion from the lower eyelid.
Conclusion: Merkel cell tumour is a rare turnour of the elderly that often locally recurs and metastasises early. Treatment consists of wide local excision with or without adjuvant radiotherapy. Juxtaposing lesions on the eyelids have not been previously reported. We postulate that this patient showed local recurrence and a new tumour of the upper eyelid simultaneously.     

A case of Merkel cell carcinoma of the eyelid

An 84-year-old man with Merkel cell carcinoma in the right upper-eyelid is reported. The patient presented with a rapidly growing, reddish-purple, solid tumor with a relatively well-defined margin in the skin of the lid. Increased neuron specific enolase content in the blood returned to normal several months after resection of the tumor and 70 Gy postoperative cobalt radiation. Histopathologic examination of the resected specimen revealed that the tumor was limited by intact epidermis and occupied by moderate-sized, round malignant cells with marked mitoses and scant cytoplasm. The neoplastic cells were positively stained by labeled antibodies for neuron specific enolase, keratin and epithelial membrane antigen. Electron microscopic examination showed abundant perinuclear microfilaments and dense core granules.     

Eyelid Merkel cell carcinoma in a patient treated with golimumab

Purpose: To describe a clinical case of biopsy-proven Merkel cell carcinoma of the eyelid following golimumab therapy for rheumatoid arthritis (RA).

Methods: Interventional case report.

Results: A 73-year-old woman with a history of chronic RA presented with a right upper eyelid mass. She had been treated with golimumab (tumor necrosis factor (TNF) inhibitors) injection therapy for the past 6 months. A biopsy showed findings suggestive of a Merkel cell carcinoma of the eyelid.

Conclusions: Merkel cell carcinoma may be associated with anti-TNF treatment and should be included in the differential diagnosis of an eyelid tumor in patients treated with TNF inhibitors.     

Merkel cell carcinoma of the upper eyelid

Case report of a rare eyelid tumor in an 83-year-old woman. The excised lesion was classified as a neuroendocrine Merkel cell carcinoma on the basis of histologic, ultrastructural, and immunohistochemical examination. Detection of neurosecretory granules by electron microscopy and the characteristic cytokeratin and neurofilament pattern supported the diagnosis. The patient developed a local recurrence, which was successfully removed. There is no evidence of regional or distant metastases.     

Immunohistochemical studies of Merkel cell carcinoma of the eyelid.

Merkel cell carcinoma of the eyelid is a rare malignant tumor. Immunohistochemical studies can be helpful in establishing the diagnosis of this tumor. We encountered a case of Merkel cell carcinoma of the eyelid and conducted an immunohistochemical analysis for clarification of its cell properties. The patient in this study was a 78-year-old man who noted a small mass on his right upper eyelid, which was subsequently removed. However, the lesion recurred and progressively enlarged. The results of a biopsy indicated the possibility of a highly malignant tumor. The lesion was removed by orbital exenteration. Merkel cell carcinoma was finally diagnosed by using light and electron microscopy. In immunohistochemical studies, the tumor cells showed both neuron-specific enolase and cytokeratin. Most of the cells were also labeled with antibodies against the protein gene product 9.5, endocrine granule constituent and chromogranin A. However, no neuropeptides were labeled. The properties of the tumor cells appeared virtually the same as those of normal human Merkel cells.     

Management and prognosis of Merkel cell carcinoma of the eyelid

OBJECTIVE: To evaluate the clinical presentation, treatment, and long-term follow-up of eyelid Merkel cell carcinoma. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: Fourteen patients with primary eyelid Merkel cell carcinoma. METHODS: Cases of Merkel cell carcinoma for which long-term follow-up was available were solicited from members of the American Society of Ophthalmic Plastic and Reconstructive Surgery through an on-line e-mail/news group. MAIN OUTCOME MEASURES: Follow-up period, treatment history, presence and type of recurrence, and mortality. RESULTS: Average follow-up was 33.4 months. Of the 14 cases identified, only 2 patients (14%) received prophylactic therapy beyond wide surgical excision. Three patients (21%) had recurrences, none of whom initially received prophylactic therapy (i.e., radiation therapy, lymph node dissection, and/or chemotherapy) beyond wide surgical excision. One patient (7%) died of metastatic Merkel cell carcinoma. CONCLUSIONS: Merkel cell carcinoma is a rare skin malignancy that occasionally affects the eyelid, with the potential for regional and distant metastasis. Consideration should be given to the use of prophylactic adjunctive therapies beyond wide surgical excision while simultaneously considering the morbidity of these therapies.     

Merkel cell carcinoma: a distinct lesion of the eyelid

Merkel cell carcinoma (trabecular cell carcinoma) is a rare, distinct, primitive, neuroendocrine malignancy of the skin, usually affecting elderly patients. It develops from Merkel cells and nearly one out of every 10 Merkel cell carcinomas occurs in the eyelids and periocular region. The tumor manifests itself clinically as a bulging lesion near the lid margin, painless, reddish colored with teleangiectatic blood vessels on the surface. Histologically, the tumor can mimic malignant lymphoma, undifferentiated melanoma, sebaceous carcinoma or cutaneous metastases of pulmonary microcytoma. Immunohistochemical studies with antibodies to neuron-specific enolase, cytokeratins and neurosecretory granules are necessary to differentiate these tumors. One third of all Merkel cell carcinomas result in death. In the present paper, we present data on the clinical features, treatment and long-term follow-up of three patients.     

Merkel cell carcinoma of the eyelid: a case report

A 22-year-old woman who presented with a tumour of the upper eyelid with clinical, histological, immunohistochemical and ultrastructural features of a Merkel cell carcinoma is reported. This is a unique case of Merkel cell carcinoma in such a young patient. This neuroendocrine tumour is typically found in the elderly. It can grow rapidly, has the potential for local recurrence and early metastatic spread. The authors recommend wide resection of the primary site, which proved effective in this case.     

Mohs' micrographic surgery for Merkel cell carcinomas of the eyelid

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumour that may affect the eyelid. There is no consensus on the optimal treatment for eyelid MCC, and management remains controversial. Here we present our experience in a case of eyelid MCC treated by Mohs' micrographic surgery. Although this treatment has been advocated for general skin MCCs, there is very little experience with this technique in relation to eyelid MCCs. We suggest that Mohs' micrographic surgery may be an appropriate method of treating MCCs of the eyelid.     

Merkel cell carcinoma of the eyelid and periocular tissues.

Five patients had eyelid and periocular Merkel cell carcinoma. The tumor was located on the left lower eyelid in two patients, the left upper eyelid in one patient, the right upper eyelid in one patient, and was metastatic to the right outer canthus in one patient. The mean duration of symptoms was approximately four months. The diagnosis of Merkel cell carcinoma was not suspected clinically in any of the four primary eyelid cases, but was only established on histopathologic examination of biopsy specimens. Light microscopy disclosed carcinoma with small primitive cells in all five tumor biopsy specimens. Immunohistochemical studies showed neuron-specific enolase and keratin and transmission electron microscopy demonstrated neurosecretory granules typical for Merkel cell carcinoma. All five patients in this study were treated with wide surgical excision of the eyelid tumors with intraoperative frozen-section monitoring of the margins of resection. The left lower eyelid Merkel cell carcinoma spread to the preauricular lymph node in one patient. This patient subsequently died of metastatic Merkel cell carcinoma. One patient with metastatic right outer canthus Merkel cell carcinoma received radiotherapy (6,550 cGy). Eyelid Merkel cell carcinoma has the potential for recurrence and metastatic spread. We recommend lifetime follow-up for patients treated for eyelid Merkel cell carcinoma.     

Primary neuroendocrine carcinoma ("Merkel cell tumor") of the eyelid: a report of two cases

BACKGROUND. Merkel cell tumor is an uncommon, aggressive neoplasm of the skin, now regarded as a neuroendocrine carcinoma. Eyelids are among the sites where it can develop, often mimicking a benign process. The purpose of this report is to describe two new cases and to discuss briefly the problems of diagnosis and treatment. METHODS. Two patients with Merkel cell tumor of the eyelids are described. In both cases, the original clinical diagnosis was chalazion. Progressive growth of the lesion identified it as a tumor some 2-7 months after it was first noticed. The patients were treated by surgical excision of the tumor tissue, and only one by a course of local radiotherapy. The visual acuity was measured with Snellen fractions. The dose of local radiotherapy is given in cGy. RESULTS. Histopathological and immunohistochemical studies identified the lesion as a neuroendocrine carcinoma, consistent with Merkel cell tumor. The natural history was marked by aggressive behavior in one case, and by a delayed recurrence in the other, requiring different therapeutic approaches. CONCLUSION. Merkel cell carcinoma of the eyelid is a tumor that ophthalmogists should be aware of, as early diagnosis is a prerequisite for successful treatment. Rapid recurrence of any chalazion in a middle-aged or elderly patient should therefore prompt its histological examination to exclude the possibility of a malignant tumor.