Merkel Cell Carcinoma Of Eyelid Research Articles

Merkel cell carcinoma and the eye

Merkel cell carcinoma (MCC) is a rare cutaneous neuroendocrine tumor with a poor five-year survival rate. Yearly cases have risen nearly 350% since the early 1980s, and these are predicted to increase as the overall US population ages. MCC of the eyelid is uncommon and can be misdiagnosed as other benign inflammatory and neoplastic eyelid disorders. Although MCC of the head and neck is often more aggressive than it is at other sites, eyelid MCC shows a lower disease-specific mortality rate. A biopsy is essential for accurate diagnosis, including an immunohistochemical panel of CK20 and TTF-1, although other markers may be necessary. Staging can be assessed clinically through physical examination findings and imaging and/or pathologically with sentinel lymph node biopsy or fine-needle aspiration. Pathologic staging more accurately predicts the prognosis. Eyelid MCC treatments include Mohs micrographic surgery to allow for complete clearance and adequate reconstruction of lost tissue, followed by adjuvant radiotherapy. In advanced disease, immunotherapies are preferred over traditional chemotherapy and are a subject of ongoing research.

An Effective Primary Treatment Using Radiotherapy in Patients with Eyelid Merkel Cell Carcinoma.

Merkel cell carcinoma (MCC) is a rare type of neuroendocrine tumor. Palpebral localization represents 2.5% of MCCs. Surgery is not always possible due to the localization or comorbidities of elderly patients. We hypothesized that radiotherapy (RT) alone could be a curative treatment in patients contraindicated for oncological surgery. We performed a retrospective monocentric study of patients with localized eyelid MCC treated with curative intent using curative radiotherapy. Overall, 11 patients with histologically confirmed eyelid MCC were treated with curative radiotherapy. The median age was 77 years old (range: 53-94). Curative RT was decided mainly due to difficult localization and significant co-morbidities. The median lesion dose was 57 Gy (range: 47-70). Most patients had adjuvant lymph nodes irradiation with a median dose of 50 Gy (n = 9; 82%). The median follow-up was 62 months (6-152 months). None of the seven deaths were MCC-related. None of our patients relapsed during follow-up. Side effects related to radiotherapy were mild (no grade ≥ 2) and rare (n = 3, 21%). Our data suggest that curative radiotherapy is an effective and safe treatment for Merkel cell carcinoma of the eyelid and periocular region. Radiotherapy alone allows limiting the aesthetic and functional sequelae in elderly and comorbid patients who are contraindicated for oncological surgery.

Prevalence of Merkel Cell Polyomavirus in Primary Eyelid Merkel Cell Carcinomas and Association With Clinicopathological Features

Merkel cell polyomavirus (MCPyV) infection is a known to be a critical risk factor for the development of Merkel cell carcinoma (MCC). Various reports on cutaneous MCC have shown that the differences in clinicohistopathological characteristics depend on the presence of MCPyV, but the situation in eyelid MCC is unknown. This study aimed to assess the prevalence of MCPyV in patients with eyelid MCC and examine the clinicohistopathological characteristics of MCPyV-associated eyelid MCC. Retrospective observational case series with laboratory investigations. Ten patients treated for eyelid MCC were included. Histopathological characteristics were examined by immunohistochemical staining using 12 antibodies. MCPyV infection was evaluated by PCR using primer sets targeting large T antigens of the MCPyV genome and by immunohistochemical staining using CM2B4 and Ab3 monoclonal antibodies. The MCPyV viral load was also quantified by PCR using 3 primer sets. All patients (4 males and 6 females) were Japanese with mean age of 79 (range: 63 to 87) years. One patient died due to distant metastasis 8 months after surgery for MCC. Immunohistochemical studies showed typical MCC findings in all cases, including CK20 and neuroendocrine marker positivity. PCR and immunohistochemistry with CM2B4 and Ab3 detected MCPyV antigen in all tumors. Quantitative PCR using sT, LT4, and TAg primers yielded 0.94, 1.72, and 1.05 copies per cell, respectively. Clinical and histopathological characteristics of 10 patients with eyelid MCC were elucidated. MCPyV infection was detected in all eyelids. These results provide insight for understanding the tumorigenesis of eyelid MCC.

Upper eyelid Merkel cell carcinoma treated with neoadjuvant chemotherapy and surgical excision.

Merkel cell carcinoma is a rare cutaneous carcinoma, featured by an aggressive clinical course and a mortality rate of 28% at 2 years. A 71-year-old female was affected by a 4.1-cm-wide locally advanced Merkel cell carcinoma of the upper eyelid, previously misdiagnosed as chalazion, with involvement of the extraocular muscles. Although the tumor showed a macroscopic spontaneous regression in size after the incisional biopsy, the mass was treated with neoadjuvant chemotherapy and surgical excision. Good functional and aesthetic result with preservation of the eyeball and absence of tumor recurrence were achieved at 3-year follow-up. In our experience, the combination of the inflammatory cascade due to the incisional biopsy and neoadjuvant chemotherapy led to the regression of a locally advanced large Merkel cell carcinoma of the eyelid.

A case report of eyelid Merkel cell carcinoma occurring under treatment with nivolumab for a lung adenocarcinoma

BackgroundMerkel cell carcinoma (MCC) is a rare neuroendocrine malignancy of the skin characterized by high aggressiveness. Four main factors are implicated in its development: immunosuppression, ultraviolet radiation, age and the Merkel cell polyomavirus (MCPyV). In recent years, immune checkpoint inhibitors have shown clinical activity in MCC treatment.Case presentationWe report the case of an 82-year-old man with a lung adenocarcinoma diagnosis, who underwent immunotherapy with nivolumab as second-line treatment. Seven months after the diagnosis of lung cancer during the nivolumab treatment, the patient developed an eyelid MCC, initially misdiagnosed as a chalazion. A palliative radiotherapy was performed with clinical benefit. After a total of seven cycles of nivolumab, computed tomography showed a lung and cerebral disease progression. In addition, clinical conditions worsened leading to the patient’s death 13 months after the initial lung cancer diagnosis.ConclusionsCases of co-occurrence of MCC and non-small cell lung cancer (NSCLC) have rarely been reported. Interestingly, common risk factors may be postulated for both cancers. Considering the rarity of this adverse event, its short-term temporal relation with the administration of the drug, which makes a relation improbable, and the coexistence of other risk factors, which may provide plausible explanations, it is possible to conclude according to the WHO Adverse Reaction Terminology that a causal relation between the occurrence of this serious adverse event and the exposure to the drug is unlikely. However, the case deserves to be reported in the literature.

Eyelid Merkel cell carcinoma in a patient treated with golimumab

ABSTRACTPurpose: 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: when reconstruction becomes a challenge.

Merkel cell carcinoma of the upper eyelid: when reconstruction becomes a challenge.

Merkel Cell Carcinoma of Eyelid: A Rare Clinical Presentation and Diagnostic Challenge

The purpose of this paper is to report a case of rare localised (limited to eyelid) malignant eyelid tumor in a 70 year old Indian male. Histopathology confirmed it to be a Merkel cell carcinoma. Surgical excision and eyelid reconstruction was done successfully. No local or systemic recurrence has been noted at a follow up of 2 years. The well defined nature of a highly malignant tumor, its localised presentation and management is being described in following report.

Sentinel Lymph Node Biopsy for Eyelid and Conjunctival Tumors

To describe current indications, methods, and outcomes of sentinel lymph node (SLN) biopsy for eyelid and conjunctival tumors. Review of experience to date and relevant articles published in PubMed in English. The use of SLN biopsy for conjunctival and eyelid tumors has evolved greatly in the past decade, and positive SLNs have been reported for conjunctival and eyelid melanoma, eyelid Merkel cell carcinoma, eyelid sebaceous carcinoma, and eyelid squamous cell carcinoma. Current indications for SLN biopsy of eyelid and conjunctival malignancies are the presence of conjunctival melanomas ≥2 mm in histologic thickness and/or histologic ulceration; cutaneous eyelid melanomas ≥1 mm thick, those with >1 mitotic figures per high-power field, and/or those with histologic ulceration; sebaceous carcinomas ≥10 mm in width; and Merkel cell carcinomas of any size. The frequency of false-negative biopsy results seems to be decreasing as more experience is gained with the technical nuances of the procedure and with the complex lymphatic drainage of the head and neck region. Given the emerging data published on feasibility and reported cases of microscopically positive SLNs identified in patients with otherwise normal examination of the regional lymph nodes and normal imaging studies, it seems appropriate to continue to further evaluate SLN biopsy for selected ocular tumors in future prospective studies. SLN biopsy is feasible for eyelid and conjunctival tumors, and continued use of the procedure is recommended. Future multi-institutional trials are needed to expand on currently available data, fine-tune patient selection criteria, and elucidate the relationships between SLN status and patient survival and tumor recurrence.

Paciente joven con carcinoma de merkel palpebral: microcirugía de mohs versus exenteración

We present the case of a 47-year-old female suffering from progeria who developed an eyelid Merkel cell carcinoma (MCC) following cyclosporine treatment for a corneal transplant. She underwent excision of the lesion by Mohs micrographic surgery (MMS) and received adjuvant treatment with radiotherapy. Later she had a recurrent tumour for which a wide excision with orbital exenteration was performed. The patient was fit and well at the 24-month follow-up. MCC is an aggressive tumour that has a larger incidence in elderly people, women and immunosuppressed patients. Our approach with the patient must be global, avoiding risk factors in predisposed subjects. MMS seems unsuitable for the treatment of MCC; if tumour recurrence occurs a wide resection should be performed (Arch Soc Esp Oftalmol 2009; 84: 581-584).

Merkel Cell Carcinoma of the Eyelid: Review of the Literature and Report of Patients with Merkel Cell Carcinoma Showing Spontaneous Regression

To evaluate the clinical and histopathologic characteristics of Merkel cell carcinoma (MCC) of the eyelid. Retrospective case series and literature review. Three consecutive patients with MCC of the eyelid who were referred to the Ocular Oncology Unit of Leiden University Medical Center, Netherlands. Clinical records and histopathologic material of patients with eyelid MCC were reviewed. The clinical presentation and treatment were evaluated. Clinical and histopathologic description of eyelid MCC, with histologic proof of spontaneous regression of the tumor. Three patients with MCC of the eyelid were included. Diagnosis was made by pathologic investigation and immunohistochemistry (S100, cytokeratin 20, epithelial membrane antigen, chromogranin). Two of the patients showed histologically proven complete spontaneous regression after nonradical excision of the tumor. After local excision, none of the MCCs demonstrated local recurrence, without regional or distant metastases. Mean clinical follow-up was 50 months. Nonocular MCC is known to recur in 66% of patients and to be lethal in almost 33%. Merkel cell carcinoma of the eyelid is a rare malignancy that can not be recognized clinically. Clinical differential diagnosis must be made with a chalazion, and histopathologic differential diagnosis must be made with small cell carcinomas. Close follow-up of these patients is advised because of the potential high recurrence rate and lymphatic spread. The immunologic phenomenon of spontaneous regression points out the importance of the immune system in this disease.

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.

Experience with sentinel lymph node biopsy for eyelid and conjunctival malignancies

7549 Background: To describe our experience with sentinel lymph node (SLN) biopsy in patients with eyelid and conjunctival melanoma, Merkel cell carcinoma of eyelid, and sebaceous cell carcinoma of the eyelid. Methods: 20 patients with eyelid or conjunctival malignancies mentioned above were treated at The University of Texas M. D. Anderson Cancer Center between May 2002 and July 2003, had clinically and radiographically negative regional lymph nodes, and underwent preoperative lymphoscintigraphy using an injection of technetium Tc-99m (0.3 mCi) in 0.2 mL volume. Intraoperative mapping was performed with the same volume of technetium Tc-99m along with an injection of isosulfan blue dye. Results: 6 patients had conjunctival melanoma, 6 had eyelid sebaceous cell carcinoma, 6 had eyelid melanoma, and 2 had eyelid Merkel cell carcinoma. SLN(s) were identified in all patients. Intraoperatively, no SLN(s) were blue. 1 patient with conjunctival melanoma and 1 patient with Merkel cell carcinoma had a histological...

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.