Merkel cell carcinoma

Merkel cell carcinoma is a neuroendocrine tumor of the skin, originating from neuroendocrine cells. A case report of Merkel cell carcinoma, discovered in a 77-Year-old woman, was diagnosed and confirmed on a biopsy. Diagnostic and therapeutic orientations of this unusual but malignant tumor are described.     

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 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.     

Merkel cell carcinoma masquerading as a chalazion

CASE REPORT: A 62-year-old woman presented with a large rapidly growing violaceous mass initially diagnosed as a chalazion. Histopathologic examination disclosed Merkel cell carcinoma (MCC). Radiotherapy was not tolerated. Despite chemotherapy, she succumbed to widespread metastases 13 months later. COMMENTS: MCC must be included in the differential diagnosis of solitary eyelid nodules, requiring early and aggressive treatment.     

Presumed choroidal metastasis of Merkel cell carcinoma

Merkel cell carcinoma is a rare skin tumor of neural crest origin and is part of the amine precursor uptake and decarboxylase system. It typically occurs on the face of elderly people. Distant metastasis is almost uniformly fatal. Choroidal metastasis, to our knowledge, has not been described. We report a patient with Merkel cell carcinoma who had a synchronous solid choroidal tumor and a biopsy-proven brain metastasis. Our 56-year-old patient presented with a rapidly growing, violaceous preauricular skin tumor. Computed tomography of the head disclosed incidental brain and choroidal tumors. Light and electron microscopy of biopsy specimens of both the skin and the brain lesions showed Merkel cell carcinoma. Ophthalmoscopy, fluorescein angiography, and A and B echography revealed a solid choroidal mass. The brain and skin tumors responded well to irradiation. A radioactive episcleral plaque was applied subsequently to the choroidal tumor. All tumors regressed, and the patient was doing well 28 months later. To our knowledge this is the first case of presumed choroidal metastasis of Merkel cell carcinoma.     

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 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.     

Bilateral primary Merkel cell carcinoma of the upper lid misdiagnosed as Basal cell carcinoma

Merkel cell tumour is a rare primary malignant tumour of the skin that can affect the lids and periocular region. It has a high rate of recurrence and metastasis following surgical excision and an overall mortality rate of 20-33%. Clinically, it is difficult to distinguish from other malignancies and the diagnosis requires careful histological analysis and immunocytochemical staining. A case of Merkel cell tumour affecting the lids and originally misdiagnosed is presented. Correct diagnosis was made only after the appearance of a second Merkel cell tumour on the contralateral lid. The pathogenesis, clinical progression, histology and management of this difficult condition are reviewed.     

Metastatic squamous cell carcinoma of the conjunctiva

Metastasis from carcinoma of the conjunctiva is said to be rare. The authors have reviewed the clinical and histopathologic findings of squamous cell carcinoma of the conjunctiva in ten patients from Saudi Arabia in whom distant metastasis developed. There were six men and four women who ranged in age from 45 to 75 years (mean, 61 years). The initial site of metastasis included the parotid gland, submandibular and submaxillary glands, preauricular, cervical lymph nodes, lungs, and bone. Squamous cell carcinoma of the conjunctiva appears to follow a more aggressive course in Saudi Arabia than elsewhere, which appears to be related to delay in management.     

Merkel cell carcinoma of the eyebrow extending into the orbit

BACKGROUND: Merkel cell carcinoma is a rare and aggressive cutaneous neoplasm with high local recurrence and metastatic rates. PURPOSE: To highlight an unusual presentation of a Merkel cell carcinoma. CASE REPORT: We report a large Merkel cell tumor in an 85-year-old woman. It extended from the eyebrow into the upper nasal orbit posterior to the equator of the eye globe. Local resection resulted in microscopically complete removal of the tumor. During follow-up (20 months) no local recurrence was noticed but there was a metastasis in a lymphatic nodule. CONCLUSIONS: Merkel cell carcinoma often presents in the eyelids and periorbital region. However, extension from the eyebrow deep into the orbit is unusual.     

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 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.