[Metastasis in the periocular region]. / Metastase in der periokulären Region.

Orbital and periocular metastatic tumors used to be considered very rare; however, with the constant updating of drugs and detection methods for cancer treatment, new chemotherapies and radiation treatments are being used. The life expectancy of cancer patients has become longer and periocular metastases are becoming easier to detect. Our knowledge of this rare disease of metastases also needs to be updated. This article reviews the incidence, symptomatic presentation, clinical features, diagnostic approaches and current treatment of metastatic tumors of the orbit and ocular adnexa in these patients.

Unusual Metastasis to Eyelid from Extraocular Merkel Cell Carcinoma.

Merkel cell carcinoma (MCC) is an unusual skin tumor that has a significant rate of distant and local metastases. It is known that primary MCC of the eyelid usually occurs at the upper eyelid. Here we report an unusual case of MCC metastasis to the eyelid. A 63-year-old male was diagnosed with MCC three years earlier after initially presenting with a mass in his right thigh. After histopathological diagnosis, the patient received medical therapy. During treatment, he developed multiple distant metastases and a firm, purple, vascularized lesion on the upper eyelid. We confirmed the lesion was an eyelid metastasis of MCC by histopathological examination and imaging methods. This case shows that extraocular MCC can metastasize to the eyelids, particularly the upper eyelid, where primary periocular MCC usually appears.

Malignancies with a tendency to metastasize to the eyelid or ocular structures.

Metastatic tumors to the eye and eyelid are generally seen in patients with disseminated metastases in the setting of advanced disease. Occasionally, they can present as the first sign of occult malignancy. The choroid is the most common site of intraocular metastases secondary to its dense vascular supply. Similar to the eye, metastatic tumors to the eyelid can present with a variety of clinical findings and are most often seen in patients with a known history of cancer. The most common skin malignancy that can spread to ocular structures is cutaneous melanoma, whereas the most common noncutaneous malignancy is breast cancer followed by lung cancer. In pediatric patients, metastatic disease to the eye is rare and can be seen in neuroblastoma and Ewing sarcoma. The overall prognosis of metastatic lesions involving the eye and eyelid is typically poor, with a mean survival of months. Ophthalmologists play an important role in the diagnosis of metastatic disease of the eye and eyelid; therefore, it is imperative for patients to undergo a complete ophthalmic examination and systemic workup if they have new-onset vision changes and a known history of cancer. Early diagnosis and management with systemic and local therapies can maximize quality of life and preserve vision.

Uveal Melanoma Metastasis to the Contralateral Eye Structures: A Retrospective Comparative Analysis of 13 Consecutive Patients.

PURPOSE: To report uveal melanoma (UM) metastasis to the contralateral ocular and periocular structures. DESIGN: Retrospective study. PARTICIPANTS: Thirteen patients with UM metastasis to the contralateral ocular and periocular structures were included. METHODS: Clinical records were reviewed retrospectively. MAIN OUTCOME MEASURES: The development and time to onset of contralateral ocular and periocular metastasis, systemic metastasis, and death. RESULTS: Of the 13 000 treated UM patients, 13 patients were diagnosed with UM metastasis to the contralateral ocular and periocular structures. Mean patient age at primary UM diagnosis was 60 years (median, 60 years; range, 37-87 years). The primary uveal melanoma was in the choroid (n = 11) or ciliary body (n = 2) and was treated with brachytherapy (n = 11), proton beam radiotherapy (n = 1), or enucleation (n = 1). Systemic metastasis developed in 11 patients (85%) at a mean of 66 months (median, 34 months; range, 12-216 months) after diagnosis of the primary UM. All 11 patients (100%) showed liver metastasis and 8 patients (62%) also showed extrahepatic metastasis. The sites of metastasis to the contralateral ocular or periocular structures included the choroid in 4 patients (31%), the orbit in 7 patients (54%), and the eyelid in 2 patients (15%). One patient with eyelid metastasis demonstrated concurrent conjunctival nodule. Mean time to diagnosis of contralateral ocular or periocular metastasis was 94 months (median, 48 months; range, 9-375 months). Contralateral choroidal metastasis was multifocal in 3 of 4 patients (75%). Of 7 patients with orbital metastasis, 5 showed extraocular muscle involvement with restricted ocular motility. Treatment for contralateral choroidal metastasis included brachytherapy (n = 2), transpupillary thermotherapy (n = 1), and observation (n = 1). Treatment for contralateral periocular (orbit or eyelid) metastasis was excision (n = 5), external beam radiotherapy (n = 2), and observation (n = 2). Of 13 patients, death was documented in 11 patients at a mean of 17 months (median, 9 months; range, 3-54 months) as a result of systemic UM metastasis (n = 10) or unrelated cause (n = 1). CONCLUSIONS: Metastasis resulting from UM to the contralateral ocular and periocular structures is rare and generally occurs in patients with disseminated metastasis. Orbital tissue is the most common site of involvement, and these patients have short life expectancy.

Ocular Dermoid in Patient With Basal Cell Nevus Syndrome.

A 47-year-old women presented for Mohs Micrographic Surgery for a biopsy proven basal cell carcinoma involving the right nasal ala. The patient had a history of basal cell nevus syndrome (BCNS) and previous history of multiple basal cell carcinomas.

A unique case of eyelid metastasis from chondroid chordoma.

A unique case of eyelid metastasis from nasopharyngeal chondroid chordoma in a 63-year-old woman was reported. Chordomas are rare tumors of the bone deriving from remnants of the embryonic notochord. Histologically, the tumor showed lobulated structure and concludes two types of cells: liquid drop cell and small round/cubic cell. Immunohistochemically, AE1/AE3, epithelial membrane antigene (EMA), and S100 showed a uniform and strong positivity. It has a great capacity for recurrence and malignant transformation, despite their slow-growing nature. The most common sites of metastases are liver, lungs, and bones. The eyelid metastasis from chordoma is an extremely rare finding, which may suggest a poor prognosis for the patient. Its significant clinicopathological characteristic could prompt us to take it into consideration when assessing the patient's prognosis.

Eyelids metastases from uveal melanoma: clinical and histopathologic features of two cases and literature review.

PURPOSE: To report the clinical and histopathologic features of two cases of eyelids metastases from uveal melanoma diagnosed in a metachronous and synchronous fashion. METHODS: Monocentric retrospective case series of histopathologically proven eyelids metastases from uveal melanoma at our institution. RESULTS: Two patients were presented to our hospital for upper eyelids pigmented and firm lesions. Patient 1 had an history of left uveal melanoma treated conservatively with proton beam therapy 5 years earlier. Examination revealed bilateral upper eyelids lesions. Patient 2 had no malignancy history but was incidentally diagnosed with a cerebral nodule few months earlier. Examination revealed a right upper eyelid nodule and a previously unknown right uveal melanoma. Excisional biopsy was performed for both patients. Pathological assessment allowed the presence of melanoma cells. The lack of BAP1 nuclear expression on immunohistochemistry as well as the absence of cutaneous or mucosal melanoma were consistent with an uveal origin. Diffuse metastatic spread was noted for both patients. Systemic therapies were prescribed. Patient 1 died from metastatic spread (62 months and 4 months after uveal melanoma diagnosis and eyelids metastases removal, respectively) whereas patient 2 was still alive (14 months follow up). CONCLUSIONS: Eyelids metastases from uveal melanoma is an exceptional finding. Excisional biopsy and pathological assessment are of main importance to confirm the diagnosis and to identify genetic mutations for further targeted therapies. Currently, prognosis remains poor.

Prognostic factors for local recurrence, metastasis and survival for sebaceous carcinoma of the eyelid: observations in 100 patients.

BACKGROUND/AIMS: To validate the predictive value of the American Joint Committee on Cancer (AJCC) 8th-edition classification for local recurrence, metastasis and survival in patients with eyelid sebaceous carcinoma. METHODS: We performed a retrospective review of 100 consecutive patients with eyelid sebaceous carcinoma. Eyelid carcinomas were staged according to the AJCC 7th-edition and 8th-edition criteria. Associations between T and N categories and disease-related outcomes including local recurrence, lymph node metastasis, distant metastasis and survival were evaluated. RESULTS: 60 women and 40 men had a median age of 67 years (range, 41-94 years). The proportions of patients who experienced local recurrence, lymph node metastasis, distant metastasis and death from disease were 6%, 21%, 7% and 6%, respectively. Two-year and 5-year disease-specific survival (DSS) rates were 93.8% and 92.0%, respectively. There were significant correlations between (1) T2c or worse category and lymph node metastasis (p=0.04) and distant metastasis (p=0.01), (2) T3b or worse category and local recurrence (p=0.01) and death from disease (p=0.01) and (3) N1 category at presentation and distant metastasis (p<0.01) and death from disease (p<0.01). The AJCC 8th-edition classification showed a better homogeneity of the T-category distribution (p<0.01) and a slightly higher discrimination ability for lymph node metastasis (C=0.734 vs C=0.728) than the 7th-edition. CONCLUSIONS: T and N categories per AJCC 8th-edition classification are predictive of local recurrence, metastasis and DSS outcomes for eyelid sebaceous carcinoma. Surgeons should perform strict surveillance testing for nodal and systemic metastases in patients with T2c or worse T category and/or N1 disease at presentation.

Primary mucinous adenocarcinoma of the eyelid: A case report and literature review.

Primary mucinous adenocarcinoma (PMA) of the eyelid is an exceptionally rare clinical entity. Often, it mimics with benign lesions on clinical examination and with metastatic mucinous adenocarcinoma on histological examinations. We report a case of PMA in a 60-year- old male patient who came with a slow-growing, painless swelling near the lower lid of the left eye. Excisional biopsy from the mass revealed a mucinous adenocarcinoma. To differentiate it from a metastatic mucinous adenocarcinoma, a wide range of immunohistochemistry panel was run. The tumor cells showed strong positivity for cytokeratin7, cytokeratin5/6, P63, estrogen receptor, progesterone receptor and negativity for cytokeratin20. Moreover, extensive metastatic work-up did not show any primary malignancy elsewhere, hence a final diagnosis of PMA was made. We believe that, this is the second reported case from the Middle East and the first in the Madinah region of Saudi Arabia.

Metástasis palpebral como primera manifestación de un tumor renal / Eyelid metastasis as first presentation of renal cell carcinoma

Caso clínico: Un varón de 87 años desarrolló en el párpado superior un nódulo de crecimiento lento, no doloroso y bien definido. El tumor de 1 cm de diámetro estaba pigmentado y adherido a planos profundos. El estudio anatomopatológico fue compatible con un carcinoma de células renales. El estudio de extensión mostró una masa tumoral en cada riñón y múltiples metástasis pulmonares. Conclusión: El oftalmólogo puede jugar un papel importante en el diagnóstico del cáncer metastásico, cuando la enfermedad ocular está presente. Por tanto, cabe destacar la relevancia de realizar una biopsia en aquellas lesiones sospechosas y/o recurrentes del párpado (AU)

Case report: An 87-year-old male presented with a slow-growing, painless and well defined nodule in the upper eyelid. The tumour measured 1cm, and was pigmented and adhered to deep planes. The histopathology analysis was compatible with renal cell carcinoma. The extension study showed a tumour mass in each kidney, as well as multiple pulmonary metastases. Discussion: The ophthalmologist can play an important role in the diagnosis of metastatic cancer when eye disease is present. Therefore, the importance of a biopsy should be noted in those suspicious and/or recurrent lesions of the eyelid (AU)

Eyelid metastasis as first presentation of renal cell carcinoma. / Metástasis palpebral como primera manifestación de un tumor renal.

CASE REPORT: An 87-year-old male presented with a slow-growing, painless and well defined nodule in the upper eyelid. The tumour measured 1cm, and was pigmented and adhered to deep planes. The histopathology analysis was compatible with renal cell carcinoma. The extension study showed a tumour mass in each kidney, as well as multiple pulmonary metastases. DISCUSSION: The ophthalmologist can play an important role in the diagnosis of metastatic cancer when eye disease is present. Therefore, the importance of a biopsy should be noted in those suspicious and/or recurrent lesions of the eyelid.

Risk Factors for Local Recurrence or Metastasis of Eyelid Sebaceous Gland Carcinoma After Wide Excision With Paraffin Section Control.

PURPOSE: To identify risk factors for local recurrence and metastasis of sebaceous gland carcinoma (SGC) after wide excision with paraffin section control. DESIGN: Retrospective, observational case-control study. METHODS: Setting: Single institution. PATIENT POPULATION: Thirty-four patients with SGC who had undergone excision with 5-mm surgical margins and paraffin section pathologic analysis. Observational Procedures: The following were considered potential risk factors for local recurrence/metastasis of SGC: patient's sex, patient's age, initial diagnosis at other clinics, topical treatments at other clinics, interval between appearance of symptoms and referral to our institution, tumor location, tumor origin, tumor presentation pattern, T category, tumor size, presence/or absence of pagetoid spread, tumor differentiation, mitosis grade, growth pattern. Logistic regression analysis was performed to identify the actual risk factors. MAIN OUTCOME MEASURES: Risk factors for local recurrence or metastasis of SGC after wide excision with paraffin section control. RESULTS: Involvement of both upper and lower eyelids, topical treatments at other clinics, multicentric origin, diffuse pattern, stage T3a, large tumor size, and a nonlobular pattern significantly influenced local recurrence/metastasis (P < .050). Pagetoid spread tended to affect local recurrence/metastasis (P = .052). The other items did not significantly influence local recurrence/metastasis (P > .050). CONCLUSIONS: This study identified risk factors for local recurrence or metastasis of SGC after wide excision with paraffin section pathologic analysis. The results of this study will be helpful to surgeons when planning additional management, such as map biopsy or adjuvant treatment.

A very rare case of eyelid metastasis originating from lung adenocarcinoma.

PURPOSE: To report a very rare eyelid mass confirmed to be a metastasis from lung adenocarcinoma. METHODS: A 68-year-old man had a two-month history of a large nodule in the right upper eyelid. He was a known case of lung adenocarcinoma since 2 years without a history of systemic metastasis. He underwent further investigations including orbital imaging and biopsy of the lesion. RESULTS: Biopsy and immunohistochemical evaluations of the eyelid lesion revealed a moderate to poorly differentiated adenocarcinoma, a similar histology to the lung lesion. CONCLUSIONS: Metastatic involvement of the eyelids is rarely seen; specifically, those associated with lung adenocarcinoma as the source have been reported extremely rare. Eyelid metastases present with various and non-specific clinical features, and therefore biopsy of suspicious or recurrent lesions is highly recommended to rule out such malignant processes.