Orbital Metastases: A Systematic Review of Clinical Characteristics, Management Strategies, and Treatment Outcomes

Background: Orbital metastases often lead to severe functional impairment. The role of resection, orbital exenteration, and complementary treatments is still debated. We systematically reviewed the literature on orbital metastases. Methods: PubMed, Scopus, Web-of-Science, and Cochrane were searched upon PRISMA guidelines to identify studies on orbital metastases. Clinical characteristics, management strategies, and survival were analyzed. Results: We included 262 studies comprising 873 patients. Median age was 59 years. The most frequent primary tumors were breast (36.3%), melanoma (10.1%), and prostate (8.5%) cancers, with median time interval of 12 months (range, 0–420). The most common symptoms were proptosis (52.3%) and relative-afferent-pupillary-defect (38.7%). Most metastases showed a diffuse location within the orbit (19%), with preferential infiltration of orbital soft tissues (40.2%). In 47 cases (5.4%), tumors extended intracranially. Incisional biopsy (63.7%) was preferred over fine-needle aspiration (10.2%), with partial resection (16.6%) preferred over complete (9.5%). Orbital exenteration was pursued in 26 patients (3%). A total of 305 patients (39.4%) received chemotherapy, and 506 (58%) received orbital radiotherapy. Post-treatment symptom improvement was significantly superior after resection (p = 0.005) and orbital radiotherapy (p = 0.032). Mean follow-up was 14.3 months, and median overall survival was 6 months. Fifteen cases (1.7%) demonstrated recurrence with median local control of six months. Overall survival was statistically increased in patients with breast cancer (p < 0.001) and in patients undergoing resection (p = 0.024) but was not correlated with orbital location (p = 0.174), intracranial extension (p = 0.073), biopsy approach (p = 0.344), extent-of-resection (p = 0.429), or orbital exenteration (p = 0.153). Conclusions: Orbital metastases severely impair patient quality of life. Surgical resection safely provides symptom and survival benefit compared to biopsy, while orbital radiotherapy significantly improves symptoms compared to not receiving radiotherapy.

Orbital metastasis from neuroendocrine tumour: Case report and literature review

Orbital metastases from neuroendocrine tumour are rare entities. An incidental finding of orbital metastasis originating from neuroendocrine tumour is presented in the context of a comprehensive review of all case reports and series published to date. Demographics, clinical features, diagnostic work-up, treatment and prognostic data from the published literature are discussed. Ninety-five patients with orbital metastases from neuroendocrine tumours have been reported so far. Average age at presentation is 63.8 years (range 25–86), with no significant gender predominance. Gastrointestinal tract is the most common site of primary neuroendocrine tumours (62–85%). Typical presentation includes proptosis (80–85%) and diplopia (27–62%) in patients with known primary neuroendocrine tumour elsewhere. Histological subtype and presence of metastases seem to be important prognostic factors. Incidental finding of neuroendocrine tumour mestastases in asymptomatic patients with unknown primary disease is uncommon. In such cases, orbital biopsy and structural and functional imaging are essential to establish a diagnosis and stage the disease.

Proptosis and a Dermal Lesion as the Presenting Sign of Lung Adenocarcinoma

The purpose is to describe a clinical case of orbital metastases as the presentation sign of the primary tumor, and the importance of a multidisciplinary diagnosis. A 70-year-old man attended the Ophthalmology Department referring ocular pain. Mild proptosis and a dermal lesion in the neck were noticed. Biopsy of the dermal lesion and systemic work-up were compatible with lung adenocarcinoma with metastatic dissemination. After one cycle of palliative chemotherapy, patient’s medical condition worsened, and he eventually died. Although rare, orbital symptoms can be the initial clinical presentation preceding the diagnosis of the primary silent lung neoplasm. Ophthalmologist has an important role in diagnoses of metastatic orbital cancer. Involvement of the multidisciplinary team is important for diagnosis and treatment.

Chromosome 10 abnormality predicts prognosis of neuroblastoma patients with bone marrow metastasis

Background Neuroblastoma (NB) is the most common extracranial solid tumor in children. It is known for high heterogeneity and concealed onset. In recent years, the mechanism of its occurrence and development has been gradually revealed. The purpose of this study is to summarize the clinical characteristics of children with NB and abnormal chromosome 10, and to investigate the relationship between the number and structure of chromosome 10 abnormalities and NB prognosis. Methods Chromosome G-banding was used at the time of diagnosis to evaluate the genetics of chromosomes in patients with NB and track their clinical characteristics and prognosis. All participants were diagnosed with NB in the Medical Oncology Department of the Beijing Children’s Hospital from May 2015 to December 2018 and were followed up with for at least 1 year. Results Of all 150 patients with bone marrow metastases, 42 were clearly diagnosed with chromosomal abnormalities. Thirteen patients showed abnormalities in chromosome 10, and chromosome 10 was the most commonly missing chromosome. These 13 patients had higher LDH and lower OS and EFS than children with chromosomal abnormalities who did not have an abnormality in chromosome 10. Eight patients had both MYCN amplification and 1p36 deletion. Two patients had optic nerve damage and no vision, and one patient had left supraorbital metastases 5 months after treatment. Conclusions The results indicated that chromosome 10 might be a new prognostic marker for NB. MYCN amplification and 1p36 deletion may be related to chromosome 10 abnormalities in NB. Additionally, NB patients with abnormal chromosome 10 were prone to orbital metastases.

Orbital metastasis from hepatocellular carcinoma revealed by sudden exophthalmos

Orbital metastases represent a rare condition and only 33 cases of metastases from hepatocellular carcinoma (HCC), usually revealed by slowly progressive exophthalmos, have been described in the literature. A 62-year-old man affected by HCC reported a sudden left exophthalmos and amaurosis. A cranial CT scan revealed pathological tissue occupying the superior portion of the orbit. During endoscopic decompression of the optic nerve, an active venous intraorbital haemorrhage was noted. Following angiography, permanent occlusion was performed on a neoforming circulus fed by ramification of the anterior deep temporal artery, anterior ramification of the superficial temporal artery and sphenoidal ramification of the medial meningeal artery. A percutaneous incisional orbital biopsy showed features of metastatic foci of HCC. This is the first description of orbital metastasis from HCC revealed by a sudden exophthalmos.

Adjuvant Intravitreal Bevacizumab Injection for Choroidal and Orbital Metastases of Refractory Invasive Ductal Carcinoma of the Breast

The efficacy of combined intravitreal bevacizumab injection with systemic chemotherapy, palliative radiotherapy, and hormonal therapy to treat choroidal and orbital metastases is not known. Herein, we report the case of a 48-year-old woman with systemic chemotherapy-resistant choroidal and orbital metastases of the left eye originating from a stage IV invasive ductal carcinoma of the left breast. We describe the addition of a single intravitreal injection of bevacizumab in addition to treatment with systemic chemotherapy, hormonal therapy, and palliative radiotherapy. The patient’s outcome at 6-month follow-up was favorable, as the metastatic lesion reduced in size and visual acuity improved. Combined treatment with intravitreal bevacizumab injection, systemic chemotherapy, palliative radiotherapy, and hormonal therapy can resolve ocular metastatic lesions originating from breast cancers.

Unilateral Orbital Metastasis as the Unique Symptom in the Onset of Breast Cancer in a Postmenopausal Woman: Case Report and Review of the Literature

The orbit represents an unusual metastases site for patients diagnosed with cancer, however, breast cancer is the main cause of metastases at this level. These orbital metastases were discovered in patients with a history of breast cancer as unique or synchronous lesions. We present a rare case of a unique retroocular metastasis as the first initial symptom of a tubulo-lobular mammary carcinoma in a postmenopausal woman. A 57-year-old patient complains of diplopia, diminishing visual acuity, orbital tenderness, slight exophthalmia and ptosis of the left eyelid, with insidious onset. Clinical examination and subsequent investigations revealed a left breast cancer cT2 cN1 pM1 stage IV. Breast conserving surgery was performed on the left breast. Pathological examination with immunohistochemistry staining established the complete diagnostic: pT2pN3aM1 Stage IV breast cancer, luminal B subtype. After two years from the initial breast cancer diagnosis, the patient was diagnosed by the psychiatrist with a depressive disorder and was treated accordingly. Orbital metastases are usually discovered in known breast cancer patients and they are found in the context of a multi-system end-stage disease. Most reports cite that up to 25% of the total orbital metastases cases are discovered before the diagnosis of the primary tumor, as our case did. MRI is the gold standard for evaluating orbital tumors. The ILC histological subtype metastasizes in the orbitals more frequently than invasive ductal carcinoma. The prognosis of patients with orbital metastases is poor. The median survival after diagnosis of orbital metastases from a breast cancer primary is ranging from 22 to 31 months. Overall survival of our patient was 56 months, longer than the median survival reported in literature. Orbital metastases must be taken into account when patients accuse ophthalmologic symptoms even in the absence of a personal history of cancer. Objective examination of every patient that incriminates these types of symptoms is essential, and breast palpation must be made in every clinical setting. Orbital biopsy is necessary for the confirmation of the diagnosis and for an adequate treatment. Although recommendations for management of orbital metastases are controversial, it appears that multidisciplinary treatment of both metastases and primary cancer improves overall survival.

Metastatic Orbital Tumor From Breast Ductal Carcinoma With Neuroendocrine Differentiation Initially Presenting as Ocular Symptoms: A Case Report and Literature Review

BackgroundOrbital metastases from cancers of various organs can arise via the hematogenous route, and many originate from breast, prostate, and lung cancers. Such metastatic orbital tumors may be diagnosed before the primary tumor. We have encountered a case of breast ductal carcinoma with neuroendocrine differentiation that metastasized to the orbit and responded to chemotherapy, with improvement in visual function.Case PresentationA woman in her fifties visited our ophthalmology department with a chief complaint of foreign body sensation and exophthalmos in her right eye. An elastic soft mass was palpated from the lateral orbit to the temporal region. A systemic examination revealed breast cancer and a metastatic orbital tumor. Excisional biopsy of the breast revealed a diagnosis of invasive ductal carcinoma with neuroendocrine differentiation, and immunohistochemical examination was negative for cytokeratin 7, making the case unusual. Chemotherapy was remarkably effective, and the tumor size decreased, resulting in improvement of visual function. Her general condition and quality of life are still good at present. We searched the PubMed English language literature focusing on metastatic orbital tumors from breast cancer in which ocular symptoms had been the initial presenting sign. No previous reports have documented neuroendocrine differentiation or cytokeratin 7 expression in isolated orbital metastases from breast cancer. Although it is not possible to be certain from this case alone, we speculated that some such cases might involve cytokeratin 7-negative invasive breast cancer with neuroendocrine differentiation.ConclusionWe have described our experience of a very rare case of cytokeratin 7 negative breast ductal carcinoma with neuroendocrine differentiation that metastasized to the orbit and formed a solitary giant tumor initially manifesting as ocular symptoms.

EGFR Mutated Lung Adenocarcinoma with Secondary Glaucoma as Early Manifestation: A Case Report

Background: Orbital metastases are rare in pulmonary adenocarcinoma and can be manifested as glaucoma.Case: A 64-year-old male patient complained of swelling, redness, painful and protruded left eye. Physical and radiological examination revealed mass in the right lung, retrobulbar intraconal mass of the left oculi, and metastases in the ribs, liver, brain, and vertebrae. Result of bronchial mucosal biopsy showed adenocarcinoma. Epidermal growth factor receptor (EGFR) mutation examinations detected exon 19 deletions, therefore Gefitinib was given. Left orbital exenteration was performed and obtained a biopsy result of adenocarcinoma metastases.Conclusion: Orbital metastasis occurs by hematogenous pathway due to direct access of systemic circulation to left orbital blood flow. Glaucoma as a symptom of orbital metastases can appear before primary tumor symptoms, because lung cancer often does not show specific symptoms until advanced stage. Definitive treatment consists of targeted therapy and surgical management for metastases.