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.     

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.     

Merkel cell tumor of the eyelids: review of the literature and report of 2 patients

BACKGROUND: The Markel cell carcinoma is a rare malignant skin tumor. The tumor was first described in 1972 by Toker and he named it trabecular carcinoma. We had the opportunity to treat two patients with a Merkel cell carcinoma of the upper and lower eyelid and reviewed the literature using the "Medline" database concerning Merkel cell carcinomas of the ocular adnexa. HISTORY AND SIGNS: A 76-year-old female patient was referred to our hospital because of an inflammatory tumor of the left upper eyelid present for two months. A 91-year-old male patient noticed for four weeks a painless itching lesion at the left lower eyelid. The remaining ophthalmologic examination in these patients was unremarkable. THERAPY AND OUTCOME: Both tumors were excised. Histological and immunohistochemical examination verified a Merkel cell carcinoma in both cases. The 76-year-old female patient exhibited no recurrent tumor after a follow-up of 18 months. The 91-year-old male patient had a recurrent tumor inferior to the temporal lower eyelid 7 months after tumor excision, however, lymph node metastasis is not present as of yet. The patient underwent radiation therapy with cobalt of the left orbit with a total dose of 60 Gy. CONCLUSION: Merkel cell carcinomas can occur everywhere on the skin; the eyelids are affected in 10% of all cases. Best histochemical markers are cytokeratin 20 and neurospecific enolase. A review of the literature revealed 31 patients with Merkel cell carcinomas involving the eyelids. Female patients were more often affected than male patients. The upper eyelid was more frequently involved than the lower eyelid. Recurrent disease is frequent.     

Evaluation of treatment and prognosis of Merkel cell carcinoma of the eyelid in Japan

PURPOSE: To evaluate retrospectively the management and prognosis of Merkel cell carcinoma of the eyelid in Japanese patients. SUBJECTS AND METHODS: Cases diagnosed as Merkel cell carcinoma of the eyelid from January 1993 to February 2005 in 111 institutions in Japan were included in this retrospective study. Management and prognosis were evaluated. RESULTS: The total number of cases enrolled was 21(5 male and 16 female patients). Excision of the tumor was carried out in 18 cases. Two cases were treated with either irradiation or local injection of interferon after biopsy of the tumor. After initial treatment, there were recurrences in 3 cases; local recurrence in one case and nodal metastasis in two cases. No patient died because of Merkel cell carcinoma of the eyelid. CONCLUSIONS: Excision with wide surgical margins with irradiation is recommended as the first choice of treatment for Merkel cell carcinoma of the eyelid. Because the number of patients was only 21 and the duration of observation was short, further investigation is necessary to determine the optimal management and more accurate prognosis for Merkel cell carcinoma.     

A merkel cell carcinoma of the lacrimal gland

Merkel cell carcinoma is a rare neuroendocrine carcinoma that most often arises in sun-exposed skin. Primary Merkel cell carcinoma of the lacrimal gland is exceedingly rare, with only one prior case reported in the literature. The authors present a 49-year-old woman with a 6-month history of painless swelling of the left upper eyelid. MRI showed a lacrimal gland mass. Surgical excision was performed, and histologic evaluation revealed a Merkel cell carcinoma. Virology testing was positive for Merkel cell polyomavirus. The patient was treated with local radiation following surgical excision. She has had no recurrence after 1 year of follow up.     

Eyelid Basal cell carcinoma arising on the site of a congenital port wine hemangioma

 Purpose: Only one previous case of eyelid basal cell carcinoma arising in a facial port wine stain without previous local radiotherapy has been reported. We now report a second case. Methods: A 42-year-old female patient with eyelid basal cell carcinoma developing within a facial port wine stain underwent incisional biopsy, surgical excision and repair. Results: The patient had a mass at the inner canthus of the left eye for two years. She had a left facial congenital port wine hemangioma involving the left eyelid, for which no topical treatment had been given. Clinical examination disclosed a 1.5×1.2 cm ulcerated skin mass with irregular borders in the medial canthal region involving the medial aspect of both upper and lower left eyelids. Incisional biopsy revealed basal cell carcinoma. She underwent surgical excision by Mohs’ technique and subsequent reconstructive eyelid surgery. The wound healed well postoperatively. At 2 years of follow up the patient showed no recurrence. Conclusion: Patients with congenital facial port wine stain may develop basal cell carcinoma, and should be regularly monitored.     

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.     

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.     

Eyelid reconstruction with Fricke's flap: report of two cases

Skin cancer is the most common neoplasm in human beings and among them, basal cell carcinoma is the most prevalent. This report shows two cases with this tumor, located in the lateral canthus, affecting both the superior and inferior eyelids. The first case was a female, 72 years old, with amaurosis of the right eye. One year ago, she noted a tiny papule at the left lateral canthus. The CT scan revealed that the tumor had compromised about 80% of the left superior eyelid, 70% of the inferior eyelid and showed superficial invasion of the lateral orbital wall. The histopathological test diagnosed basal cell carcinoma, with clear margins. The reconstruction of both eyelids was done with Fricke's flap. The second patient was a female, 68 years old, who had an accident with multiple scars at the left side of her face two years before. One year later, she noted a small lesion at the left side of the lateral canthus. The CT scan showed that the tumor had affected about 90% of the left superior eyelid, 65% of the inferior eyelid and showed superficial invasion of the lateral orbital wall. The histopathological test diagnosed basal cell carcinoma, with clear margins. The reconstruction of both eyelids was done with Fricke's flap. The authors believe that although this flap is not the first choice for a reconstructive procedure, it should not be forgotten because in some patients, it would be the only possible choice.     

Eyelid verrucous carcinoma

PURPOSE: To report a case of verrucous carcinoma (VC) that presented as an eyelid mass on the right eye. CASE REPORT: A 66-year-old man developed a slow-growing eyelid lesion on the lateral canthus of the right eye. The tumor was firm, well-defined, sessile, acuminate, and exuded foul-smelling fluid. RESULTS: Excisional biopsy revealed a VC. The patient remains asymptomatic. CONCLUSIONS: VCs are extremely rare eyelid tumors. This clinicopathological entity is a low-grade variant of squamous cell carcinoma that occurs mainly in the intraoral, genitogluteal, and plantar areas. A role of human papillomavirus (HPV) in these tumors has been recently identified. VCs characteristically show locally aggresive growth. This is the second case reported in the literature on the eyelid. VC should be considered in the differential diagnosis of eyelid tumors.     

Smart phone apps every ophthalmologist should know about

AIM: To evaluate the demographic features, location, and histopathologic results in primary eyelid tumors. METHODS: We retrospectively reviewed the records of patients with primary eyelid tumor diagnosed and treated between November 1997 and 2016 on our service. Results: Nine hundred and eleven lesions from 874 patients were included in this study. Four hundred and forty-six (51.0%) of the patients were females and 428 (49.0%) were males. The mean age was 51.0y (range: 3mo-94y). The lesions were located in the upper eyelid (418 cases, 45.9%), lower eyelid (378 cases, 41.5%), medial canthus (89 cases, 9.8%), and lateral canthus (26 cases, 2.9%). Four hundred and seventy (51.6%) lesions were located on the right side and 441 (48.4%) on the left. Of the eyelid lesions, 666 (73.1%) were benign, 230 (25.2%) malignant, and 15 (1.6%) premalignant. When the eyelid tumors were classified according to their tissue or cell of origin, 527 (57.8%) of the lesions were found to be epidermal, 171 (18.8%) adnexal, 131 (14.4%) inflammatory and infectious, and 65 (7.1%) stromal. The most common benign lesions were squamous cell papilloma (139 lesions, 15.2%), intradermal nevus (97 lesions, 10.6%), epidermoid inclusion cysts (78 lesions, 8.6%), seborrheic keratosis (60 lesions, 6.6%), and inflammatory masses (59 lesions, 6.5%). Basal cell carcinoma was the most common malignant tumor (191 lesions, 21.0%) followed by squamous cell carcinoma (16 lesions, 1.8%), sebaceous carcinoma (14 lesions, 1.5%), and malignant melanoma (5 lesions, 0.5%). CONCLUSION: In this study, 73.1% of eyelid lesions were benign and the remaining 26.9% were premalignant and malignant. Basal cell carcinoma was the most common tumor among all histopathological diagnosis followed by squamous papilloma.     

Eyelid merkel cell carcinoma: report of three cases

PURPOSE: To present three cases of Merkel cell carcinoma, a primary cutaneous neuroendocrine tumor that arises from Merkel cells, and to describe their clinical features, diagnosis, and management. METHODS: Case series. RESULTS: In only one case, the disease was clinically recognized. In the others, the diagnosis was confirmed by histology, and chalazion was initially suspected in one of them. Surgery was performed in all patients, and there was no evidence of metastatic disease. CONCLUSIONS: The rarity of eyelid Merkel cell carcinoma and the fact that it can simulate benign entities frequently lead to difficulties in diagnosis and a delay in the establishment of suitable therapy.     

Squamous cell carcinoma of the eyelids

AIM: To review the clinical features, management, and outcomes of surgical treatment of eyelid squamous cell carcinoma (SCC). METHODS: A retrospective review of all eyelid SCCs treated between 1992 and 2001. RESULTS: 51 cases were identified in 50 patients. Patient ages ranged from 26 to 93 years, with a mean age of 65 years. 33 patients were male and 17 were female. The lesion was found on the lower lid in 31 cases, upper lid in five cases, lateral canthus in six cases, and medial canthus in nine cases. Perineural invasion was found in four patients, and orbital invasion in three patients. Recurrence occurred in one patient. Treatment was by complete excision with histological confirmation of clear margins. Exenteration was required in three patients. No patients developed lymph node or distant metastases. One patient, who declined treatment, died as a result of the tumour. Mean follow up was 31.1 months. CONCLUSIONS: Eyelid SCC is a relatively uncommon, but potentially fatal disease. However, if detected early and treated adequately, the prognosis is generally excellent. Treatment by complete excision with histological confirmation of tumour clearance is recommended. Perineural spread is an adverse prognostic sign, which may require postoperative radiotherapy. Orbital invasion is a rare complication but, if recognised early, can be treated effectively with exenteration. Because presentation varies and histological examination is required for accurate diagnosis, any suspicious lesion occurring on the eyelids should be excised or biopsied. All patients with eyelid SCC should be advised of the risk of recurrent or new tumours and encouraged to attend lifelong follow up. Prevention remains of prime importance in minimising the morbidity and mortality of these lesions.     

Radiation therapy for local control of eyelid sebaceous cell carcinoma: report of two cases and review of the literature

PURPOSE: Because of previous reports of increased rate of recurrence and mortality after radiation therapy, eyelid sebaceous cell carcinoma has been considered radioresistant. Recent reports of success with primary radiation therapy have been attributed to advancements in irradiating technology and technique. Two cases of eyelid sebaceous cell carcinoma successfully treated with radiation therapy are reported. The techniques used are compared with the techniques described in previous reports, and factors favoring successful treatment are reevaluated. METHODS: Case series and review of the literature. Two cases of eyelid sebaceous cell carcinoma that underwent radiation therapy are described. Both patients were offered, but refused, surgical excision. One patient received 69 Gy combined superficial and megavoltage x-ray irradiation to the left lower eyelid. The second patient received 59 Gy megavoltage electron beam irradiation to the right upper eyelid. A review of the literature was performed, and Fisher's exact test analysis was used to compare the results of all reported cases treated with < or =55 Gy with those treated with >55 Gy. RESULTS: In both cases, the tumor responded to radiation therapy. One patient died 39 months after treatment, of myocardial infarction. The second patient is without clinical evidence of tumor recurrence 46 months after treatment. Fisher's exact test showed an advantage to patients treated with >55 Gy radiation (p = 0.05). CONCLUSION: Radiation therapy with an appropriate delivery system is effective as a curative treatment for eyelid sebaceous cell carcinoma when >55 Gy of radiation dose is delivered. It should be considered for patients seeking an alternative to surgical excision.     

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.     

Reconstrucción de párpado inferior mediante injerto de pericráneo y colgajo de Mustardé

We report the case of a 68-year-old man with a left lower eyelid basal cell carcinoma with squamous differentiation. Resection was performed under frozen section technique and the defect (inner canthus, two thirds of the lower eyelid and 2.5 cm of the cheek) reconstructed 2 days after surgery in a single surgical operation: posterior lamella with pericranial graft and anterior lamella with Mustarde flap. After one year of follow-up, the patient has an adequate appearance, good eyelid support and position, vascularized tissue like the native eyelid and no tumor recurrence.     

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.     

An unusual presentation of merkel cell carcinoma of the eyelid

Merkel cell carcinoma is a rare primary neuroendocrine tumor occurring on any part of the body. It usually presents as a firm, nontender, violaceous, or purple nodule located on areas of the body that have been exposed to sunlight. A 68-year-old female presented with a rapidly growing lesion on the left lower eyelid from 6 weeks. Examination showed a 4 cm diameter, exophytic, ovoid skin lesion of the left lower lid. Wide local excision of this lesion was followed by adjuvant chemotherapy. Histological examination of therapeutic frozen section of the lesion and the presence of neuroendocrine marker and cytokeratin markers established the diagnosis of Merkel Cell carcinoma. The follow-up at 8 months was uneventful. Merkel cell carcinoma can have an unusual presentation of a large, exophytic, oval lesion resembling a basal cell carcinoma. Merkel cell carcinoma has predilection for rapid spread; hence, in a case of lid lesions, a suspicion for this diagnosis should be kept in mind.     

Periocular keratoacanthoma: clinical features,pathology, and management

PURPOSE: To review the clinical features and results of surgical treatment of keratoacanthoma of the eyelids. DESIGN: Retrospective, interventional case series. PARTICIPANTS: Ten patients. METHODS: Chart review of all eyelid keratoacanthomas treated between 1992 and 2001. MAIN OUTCOME MEASURES: Adequate excision, recurrence rate, and complications. RESULTS: Patient ages ranged from 27 to 78 years, with a mean age of 59 years. Six patients were male and four were female. The lesion was found on the lower lid in five patients, upper lid in two, medial canthus in two, and lateral canthus in one. The maximum diameter of the lesion varied from 2 to 25 mm, with a mean of 7.2 mm. All lesions were treated by surgical excision, with frozen-section control of margins in five cases. All lesions were excised completely with clear resection margins, and there were no cases of recurrence. The only complication was a minor wound infection in one patient. Mean follow-up was 34.5 months. CONCLUSIONS: Because of the aggressive nature and uncertain relationship to squamous cell carcinoma, we recommend excision of periocular keratoacanthoma. Surgical excision of eyelid keratoacanthoma provides good results and a very low risk of recurrence. Frozen-section control of margins should be used in selected cases to ensure complete excision.