Orbital invasion of a conjunctival atypical fibroxanthoma in a patient with systemic diffuse large B-cell lymphoma.

An 88-year-old male patient presented with a large mass on the left lateral bulbar conjunctiva. The tumor appeared two months after the resection of a conjunctival atypical fibroxanthoma (AFX) performed by a cornea specialist. Magnetic resonance imaging of the orbits showed deep orbital invasion along the lateral rectus muscle. The mass and the entire conjunctival sac were totally excised with lid-sparing orbital exenteration. Histopathological analysis confirmed that the mass was an extension of the AFX. Two weeks after surgery, large B-cell lymphoma was diagnosed in the oropharynx. Chemotherapy was initiated, and after seven months of follow-up, there was no recurrence of the AFX. The authors believe that this is the first report of orbital invasion by AFX.

Orbital invasion routes of non-melanoma skin cancers and survival outcomes.

PURPOSE/OBJECTIVES: Overall non-melanoma head and neck skin cancer has a good prognosis; however, rarely patients have an aggressive variant which results in orbital invasion via perineural spread or direct extension. Despite these consequences, there are limited published studies defining this clinical entity. The main objectives of the current study are to describe orbital invasion patterns of non-melanoma head and neck skin cancers and their impact on survival. METHODS: Retrospective case series from a tertiary-care, academic institution performed between 2004 and 2014. Demographic and tumour characteristics are reported as well as patterns of orbital invasion, types of treatments received, and survival outcomes. RESULTS: There were 17 consecutive patients with non-melanoma skin cancer and orbital invasion who met inclusion criteria. Average age at orbital invasion diagnosis was 70.8 years old. 76% were male. Mean follow-up time was 28.5 months. Of these patients, 71% had squamous cell carcinoma and 29% had basal cell carcinoma. Brow (41%) was the most common primary sub-site followed by cheek (23%) and temple (12%). 76% of patients had a history of prior treatment. The lateral orbital wall (41%) was the most common site of invasion, followed by the medial orbital wall (29%) and antero-superior invasion (23%). Age, histology, and location of orbital invasion were associated with disease-specific and overall survival. CONCLUSION: Orbital invasion for non-melanoma head and neck skin cancers creates a treatment dilemma and the patterns of invasion are described. In addition, the location of orbital invasion is associated with survival outcomes.

Preserved visual function with an orbital invasion of pituitary adenoma.

Pituitary adenomas rarely extend to the orbit through the optic foramen and superior orbital fissure. Compressive optic neuropathy could be caused by orbital invasion of the mass showing variable visual prognosis. We are reporting a patient with intact visual acuity after 3 years of a profound orbital invasion of pituitary adenoma.

The implications of orbital invasion in sinonasal tract malignancies.

In recent decades, the management of sinonasal tumors abutting the orbit has been widely discussed. A real guideline has yet to be proposed, as prospective randomized studies on this topic are very difficult to organize, given the relative rarity of this pathology, the wide spectrum of histologic patterns, and the different clinical behavior of tumors. Nevertheless, in recent years, a better assessment of tumor extension has been obtained thanks to the refinement of preoperative imaging tools and, therefore, more conservative approaches could be adopted, with no worsening of the oncological outcomes and, at the same time, with more attention given to the post-surgical quality of life. Currently, tumors that extend to the bony orbital walls with or without focal infiltration of the periorbit are amenable to orbital preservation. On the other hand, infiltration of extraocular muscles and neurovascular structures are an indication to orbital exenteration. The ideal surgical treatment in cases of limited involvement of orbital fat still remains a matter of debate. We report and discuss the recent English literature on this interesting topic.

Periocular Desmoplastic Malignant Melanoma and Orbital Invasion With Uncontrollable Disease: A Case Report.

Primary orbital melanoma and metastatic cutaneous melanoma of the orbit are extremely rare. Desmoplastic melanoma (DM) is an infrequent variant of melanoma that can extend from a superficial location into deep tissues by neurotropic mechanisms. A 78-year-old male was referred to us with a periocular mixed malignant melanoma (spindle cell melanoma with desmoplastic reaction) in his left lower eyelid with uncontrollable disease (orbital and inferior orbital rim invasion) despite treatment. The surgical technique consisted of an extended orbital exenteration, maxillectomy, and ethmoidectomy, with a 2 cm macroscopic surgical margin. We performed a delayed socket reconstruction with a temporalis muscle flap using a transorbital approach. The patient remained disease-free for 1.5 years with a good quality of life since exenteration surgery. At this time, he presented a recurrence in the area of the malar scar with a new orbital invasion, and finally, he died due to mediastinal, pleural, and pulmonary metastasis. The treatment of a cutaneous melanoma arising in the periocular region is a challenging reconstructive problem and it may compromise the globe and visual function.

The role of induction chemotherapy for orbital invasion in sinonasal malignancies: A systematic review.

BACKGROUND: Sinonasal malignancies (SNMs) frequently present with orbital invasion. Orbital exenteration (OE) can lead to significant morbidity. Induction chemotherapy (IC) is a promising treatment alternative that may allow for orbit preserving (OP) treatments without compromising patient survival. This systematic review was conducted to synthesize the published data on SNM patients with orbital invasion who underwent IC, including tumor response, orbital outcomes, and survival. METHODS: The study protocol was designed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Databases Embase, Cochrane, Medline, and Scopus, from inception to July 17, 2023, were searched. RESULTS: Nineteen studies were included, encompassing 305 SNM patients with orbital invasion treated with IC. Fourteen studies reported an overall IC response rate (positive response defined as complete or partial tumor volume reduction) of 77.2%. Among included studies, OE rates after IC ranged from 0 to 40%. Three studies reported a high rate of posttreatment functional orbital preservation (89.8-96.0%). Five studies specifically reported that 62.5% (60 out of 96) of patients were downgraded from planned OE to OP treatment following IC. Three studies reported a significant overall survival (OS) improvement in IC responders versus IC nonresponders. Following IC, 5-year OS ranged from 44.2 to 55.5%. Patients with olfactory neuroblastoma demonstrated the highest IC response rate and lowest OE rate (100 and 0%, respectively) versus those with sinonasal undifferentiated carcinomas (68.4 and 0%) or squamous cell carcinomas (76.7 and 16%). CONCLUSIONS: For select patients, IC may allow for OP in locally advanced SNMs with orbital involvement.

Long-term outcomes in advanced anterior skull base malignancy: a single quaternary institution experience.

BACKGROUND: Advanced skull base malignancies are a heterogenous subset of head and neck cancers, and management is often complex. In recent times, there has been a paradigm shift in surgical technique and the advent of novel systemic options. Our goal was to analyse the long-term outcomes of a single quaternary head and neck and skull base service. METHODS: A retrospective review of 127 patients with advanced anterior skull base malignancies that were treated at our institution between 1999 and 2015 was performed. Multiple variables were investigated to assess their significance on 5 and 10-year outcomes. RESULTS: The mean age was 60.9 (± 12.6 SD). Sixty-four percent were males and 36% were females. Ninety percent of patients had T4 disease. Median survival time was 133 months. The 5-year overall survival (OS) was 66.2%, disease-specific survival (DSS) was 74.7%, and recurrence-free survival (RFS) was 65.0%. The 10-year OS was 55.1%, DSS was 72.1%, and RFS was 53.4%. Histological type and margin status significantly affected OS & DSS. CONCLUSION: Surgical management of advanced skull base tumours has evolved over the last few decades at our institution with acceptable survival outcomes and complication rates. Histological diagnosis and margin status are the main predictors of survival. The addition of neoadjuvant systemic agents in current trials may improve outcomes.

Skull Metastasis With Orbital Invasion as a Primary Manifestation of Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) is a common worldwide cancer with a poor prognosis despite treatment advancements. Patients typically exhibit signs and symptoms pertaining to the liver. Extrahepatic metastasis of HCC is documented to be as low as 5% of cases. Bone metastasis ranks third following lungs and regional lymph nodes. The typical locations for bone metastasis include the vertebral column, pelvis, femora, and ribs, with skull metastasis, being reported in less than 1.6% of cases. Herein, we describe a case of HCC presenting with skull metastases and orbital invasion as the initial manifestation.

Outcomes in surgical management of sinonasal malignancy-A single comprehensive cancer center experience.

BACKGROUND: Sinonasal malignancies are a complex and diverse group of tumors. Over the past five decades, treatment advances have changed the management paradigms for these tumors. Our aim was to analyze the outcomes of patients from a comprehensive cancer center. MATERIALS AND METHODS: We retrospectively assessed 400 patients with sinonasal malignancies treated with surgery at our center between 1973 and 2015. Multiple variables were reviewed to assess the influence on 5-year outcomes. RESULTS: The median age was 56 years (IQR 46.8-68). Two hundred and fifty-nine (65%) were males and 141 (35%) were females. Overall survival (OS) and disease-specific survival (DSS) improved in the last analyzed decade. Orbital invasion, advanced pT-classification and pN-classification, and melanoma histology were associated with poorer outcomes. CONCLUSION: Treatment outcomes for patients with sinonasal malignancy have improved over time. This is likely multifactorial with advances in surgical technique, adjuvant treatment, and patient selection. pT-classification, pN-classification, orbital invasion, and histology are predictive of survival.

Utility of the deep learning technique for the diagnosis of orbital invasion on CT in patients with a nasal or sinonasal tumor.

BACKGROUND: In nasal or sinonasal tumors, orbital invasion beyond periorbita by the tumor is one of the important criteria in the selection of the surgical procedure. We investigated the usefulness of the convolutional neural network (CNN)-based deep learning technique for the diagnosis of orbital invasion, using computed tomography (CT) images. METHODS: A total of 168 lesions with malignant nasal or sinonasal tumors were divided into a training dataset (n = 119) and a test dataset (n = 49). The final diagnosis (invasion-positive or -negative) was determined by experienced radiologists who carefully reviewed all of the CT images. In a CNN-based deep learning analysis, a slice of the square target region that included the orbital bone wall was extracted and fed into a deep-learning training session to create a diagnostic model using transfer learning with the Visual Geometry Group 16 (VGG16) model. The test dataset was subsequently tested in CNN-based diagnostic models and by two other radiologists who were not specialized in head and neck radiology. At approx. 2 months after the first reading session, two radiologists again reviewed all of the images in the test dataset, referring to the diagnoses provided by the trained CNN-based diagnostic model. RESULTS: The diagnostic accuracy was 0.92 by the CNN-based diagnostic models, whereas the diagnostic accuracies by the two radiologists at the first reading session were 0.49 and 0.45, respectively. In the second reading session by two radiologists (diagnosing with the assistance by the CNN-based diagnostic model), marked elevations of the diagnostic accuracy were observed (0.94 and 1.00, respectively). CONCLUSION: The CNN-based deep learning technique can be a useful support tool in assessing the presence of orbital invasion on CT images, especially for non-specialized radiologists.

Adenocarcinoma NOS arising from accessory lacrimal glands: A case report and a review of the literature.

Primary malignant epithelial tumours arising from accessory lacrimal glands (ALGs) are extremely rare, with only few cases reported in literature. They generally appear as gradually increasing masses of the upper or the lower eyelid. Only one case of primary adenocarcinoma or adenocarcinoma not otherwise specified (ACNOS) from ALGs has been reported in literature. Herein, we describe a case of ACNOS arising from ALGs with an atypical clinical presentation and review prior cases of ALGs epithelial malignancies reported in the literature. A 78-year-old man referred to our Ocular Oncology Unit for adjuvant therapy after the excision of a conjunctival tumour of the left eye with a histological diagnosis of squamous cell carcinoma. He underwent topical chemotherapy with MMC and during follow up he presented a multinodular iris mass in his left eye. The MRI of the orbit showed an ocular mass infiltrating orbital soft tissues of the inferior palpebral region with an involvement of the corresponding zygomatic cutis. We performed orbital exenteration and histological studies revealed an epithelial neoplasm with a solido-glandular growth pattern with lumens containing an eosinophilic material positive for PAS and PAS-D. The immunohistochemical findings confirmed the diagnosis of adenocarcinoma NOS from ALGs. Although ALGs epithelial malignancies are extremely uncommon, they should be considered in the differential diagnosis of ocular tumours. A vigilant approach towards these entities is required, since they can be clinically insidious and locally aggressive.

Nasopharyngeal carcinoma (NPC) in the ophthalmic practice: A serious neoplasm presenting initially to ophthalmologists.

PURPOSE: Nasopharyngeal carcinoma (NPC) is an aggressive malignant tumor that arises from the nasopharyngeal epithelial lining. Most patients with NPC present with a neck mass or cranial nerve palsy. It is infrequent for patients with NPC to present to an ophthalmologist initially with ophthalmic complaints and absence of prior diagnosis of NPC. We are reporting a series of six NPC cases that presented solely with ophthalmic complaints, to attract the attention of ophthalmologist to such a serious neoplasm. METHODS: A retrospective observational study of all consecutive patients diagnosed with NPC based on their initial ophthalmic presentation with no prior diagnosis of this neoplasm. Patients presented for the first time to two tertiary eye hospitals. Data were collected by the chart review for demographics, clinical presentation, radiological imaging, treatment regimen, and follow-up. Histopathological review of their tissue specimens was conducted by two pathologists. RESULTS: We had six patients who presented for the first time to our ophthalmic service complaining mostly of limitation of extraocular muscle motility with or without diplopia (4/6), orbital or facial pain (2/6), and disturbance in vision (2/6). The age ranged from 35 to 92 years with a mean of 58 years. A male predominance was observed with male-to-female ratio of 5:1. The cases are described along with their imaging and histopathological findings. Relevant literature review was also presented. Most of our patients (5/6) received combination of radiotherapy and chemotherapy. One patient refused treatment and 2 eventually passed away after 7 months. CONCLUSION: Ophthalmologists may play a major role in diagnosing such patients accurately and referring them for early management and better prognosis.

Corticosteroid Therapy in Optic Neuropathy Secondary to Nasopharyngeal Carcinoma.

INTRODUCTION: Nasopharyngeal carcinoma (NPC) is a tumor arising from the epithelial cells of the nasopharynx. NPC can spread and invade the base of skull, nasal cavity, paranasal sinuses, pterygopalatine fossa, and apex of the orbit. However, the involvement of the optic nerve in NPC is rare. The purpose of this case report is to report the efficacy of corticosteroid therapy in optic neuropathy secondary to NPC. CLINICAL CASE: A 56-year-old Chinese woman, an active smoker, presented with a hearing deficit, persistent tinnitus and nasal congestion. Examination and investigations revealed the presence of a mass in the nasopharynx. Tissue biopsy revealed nasopharyngeal carcinoma. However, the Epstein-Barr virus was not tested. She was counseled for chemotherapy, but refused and was subsequently lost to follow up. She presented one year later with right eye ptosis associated with progressive worsening of diplopia and blurring of vision. Examination revealed multiple (second, third, fourth and sixth) cranial nerve involvement. Systemic examination and investigations revealed cervical lymphadenopathy and liver metastasis. Repeated imaging showed that the mass had invaded the base of the skull, cavernous sinus and orbital apices. Pulse dosing of corticosteroid therapy was commenced, resulting in dramatic improvement of vision. CONCLUSION: Optic neuropathy may be the presenting sign of NPC. Corticosteroid therapy can offer immediate visual improvement.

Prognostic factors and outcomes of multimodality treatment in olfactory neuroblastoma.

OBJECTIVE: The clinical data on olfactory neuroblastomas (ONBs) are scarce owing to their rarity. This study aimed to assess the potential prognostic factors, outcomes, and optimal treatment strategies in patients with ONB. METHODS AND MATERIALS: The data of 217 patients with ONB between 1991 and 2019 were retrospectively reviewed. Long-term survival, potential prognostic factors, and outcomes with combined treatment strategies were analyzed. RESULTS: All patients received radiotherapy (RT); 185 patients underwent surgery, and 139 patients received chemotherapy. The 5-year overall survival (OS), progression-free survival (PFS), locoregional failure-free survival (LRFS), and distant metastasis-free survival (DMFS) of the entire cohort were 80.0%, 79.0%, 79.3%, and 80%, respectively. On univariate analyses, R0/R1 resection, early Kadish stage, negative lymph nodes, absence of orbital invasion, and administration of surgery with RT were found to be favorable factors. Conversely, combined sequential treatment with surgery, RT, and chemotherapy was not associated with survival. Multivariate analysis demonstrated lymph node status, orbital invasion, and the combination of surgery and RT to be independent prognostic factors. CONCLUSIONS: Patients with ONB, who had lymph node metastases, orbital invasion diseases, advanced Kadish stages, R2 resection margins, and received RT alone, had poor outcomes. Combined administration of surgery and RT may be a potentially useful strategy in patients with advanced Kadish stages; the role of chemotherapy in these stages requires further evaluation.

Nomogram for Preoperative Estimation of Orbit Invasion Risk in Periocular Squamous Cell Carcinoma.

Importance: Orbital invasion occurs in some periocular squamous cell carcinoma (SCC), compromising surgical outcomes, and prognoses of patients. To date, however, there are no validation studies on the clinical features related to orbital invasion in patients with periocular SCC. Objective: To explore clinical features that may be associated with orbital invasion and build a model for predicting the risk of orbital invasion. Design, Setting, and Participants: In this retrospective mono-center case-control study, 90 patients with periocular SCC were treated at the Ninth People's Hospital Shanghai Jiao Tong University School of Medicine from January 2005 to August 2019. "Case" is defined as a SCC patient with orbit invasion prior to operation. "Exposure" is defined as the different sites of lesion. Main Outcomes and Measures: Clinical features, including "time to relapse after surgery," were collected. Multivariate logistic regression analysis was applied to identify the independent risk clinical features associated with orbital invasion, which was then incorporated into a nomogram. Results: Of the 90 patients included in this study, 33 patients (36.7%) had orbital invasion. 14 of the 33 orbit-invasive patients had local recurrence, while 11 of 57 orbit non-invasive patients had local recurrence, suggesting that orbital invasion is a risk factor for local recurrence. The multivariate binary logistic regression indicated that the lesions at the medial canthus [odds ratio (OR), 5.024, 95% CI, 1.409-17.912, P = 0.013], the age at diagnosis (10-years intervals; OR, 0.590, 95% CI, 0.412-0.844, P = 0.004), and bleeding in the lesion (OR, 3.480, 95% CI, 1.254-9.660, P = 0.017) were three preoperative clinical features significantly associated with orbital invasion. Conclusion: For periocular SCC, lesions at the medial canthus, the younger age of the patients at diagnosis, and bleeding in the lesion were the three main clinical features associated with orbital invasion. The risk score model for orbital invasion can act as a supportive tool for optimized clinical evaluation and treatment decisions.

Management of orbital invasion in sinonasal squamous cell carcinoma: 15 years' experience.

BACKGROUND: This study was intended to review our management strategy for sinonasal squamous cell carcinomas (SNSCCs) with orbital invasion and to explore the role of radiotherapy in orbital preservation. METHODS: We retrospectively analyzed 93 SNSCC patients with orbital invasion who underwent radiotherapy with or without surgery over the past 15 years. The degree of orbital invasion was classified into 3 grades. RESULTS: Eighty-eight patients presented with T4 tumors and 36 had grade III orbital invasion. Seventy-two patients received surgery plus radiation and 67 received platinum-based chemotherapy. The median follow-up for surviving patients was 60 months. Five-year overall survival (OS) for the whole group was 57.4%. The patients treated with surgery plus radiation had a 5-year survival rate of 62.2% and orbital preservation was feasible in 90.3% of cases. Twenty-one patients with SNSCCs that extended into the extraocular muscles or eye globe also underwent orbital preservation. Five-year locoregional relapse-free survival (LRFS) was 69.5% for patients treated with orbital preservation and 57.1% for those treated with orbital exenteration, indicating no statistical difference. Five-year survival, 5-year progression-free survival (PFS), and 5-year distant metastasis-free survival (DMFS) were similar between groups. Grade III orbital invasion was independently associated with shorter OS, LRFS, PFS, and DMFS. CONCLUSION: Orbital invasion in grade III was associated with the worst survival outcomes. Invasion of either the extraocular muscles or eye globe is not a contraindication for eye-sparing surgery. Preoperative chemoradiation continues to offer hope to patients with a strong desire to preserve their eyes.

Considerations in Orbital Exenteration.

Orbital exenteration (OE) is a radical operation associated with significant psychosocial disability and functional impairment. Indications for OE include primary tumors of the eye, oral cavity, paranasal sinuses, skin, and brain. Careful consideration regarding the likelihood of local control and cure is needed before proceeding with this operation. Multidisciplinary work-up should be performed before proceeding with surgery. The method of reconstruction after OE should be tailored to the defect and the postoperative needs of the patient. Appropriate follow-up and rehabilitation should be arranged for the patient.

Management of orbital invasion in esthesioneuroblastoma: 14 years' experience.

BACKGROUND: There is a scarcity of data about the prognostic value of orbital invasion in esthesioneuroblastoma (ENB), as well as about its management strategies. Indications for the preservation of orbital contents remain controversial, and the evaluation of orbital invasion has been ill defined. METHODS: This retrospective analysis contained 60 ENB patients with orbital invasion who underwent radiotherapy with or without surgery over the past 14 years. Orbital invasion was classified into three grades. RESULTS: There were 52 patients at stage C and 8 at stage D, according to Foote classifications. Grade I, grade II and grade III orbital invasion was detected in 12, 23, and 25 patients, respectively. The median follow-up was 57 months (IQR 32-95 months). Fourteen patients received radical radiotherapy, with a 5-year overall survival (OS) of 63.5%; 46 received surgery plus radiation, with a 5-year OS of 70.7%; and the difference was not statistically significant (p = 0.847). Orbital preservation was feasible in 100% of cases, including 18 cases that extended to extraocular muscles or the eye globe. Five-year locoregional relapse-free survival was 100% in patients with prophylactic elective neck irradiation (PENI) and 58.1% in patients without PENI (p = 0.004). Univariate analysis showed that grade II/III orbital invasion was associated with poorer OS and progression-free survival. Neck metastasis (with a Foote stage of D) was independently associated with shorter OS and distant metastasis-free survival in multivariate analysis. CONCLUSIONS: Our data suggested that primary radiotherapy achieved comparable survival to surgery plus radiotherapy in advanced ENB. Invasion of either the extraocular muscles or the eye globe is not a contraindication for eye-sparing surgery. Orbital invasion in grade II/III was significantly associated with adverse survival outcomes. Prophylactic radiotherapy to the neck with N0 significantly reduces the risk of regional recurrence.

A review of nasal, paranasal, and skull base tumors invading the orbit.

Tumors that invade the orbit are uncommon. The majority are meningiomas arising from the sphenoid ridge (66%). Others are bone and cartilage tumors arising from the surrounding bones of the orbit, pituitary adenomas, and epithelial tumors arising from the paranasal sinuses and nasal cavity. Meningiomas occur more often in women, whereas epithelial tumors have a predilection for men. Meningiomas and epithelial tumors typically present in the sixth decade of life, whereas bone tumors tend to affect individuals in their third decade of life. Patients often present with a combination of ophthalmological and otorhinolaryngological symptoms, including proptosis, pain, decreased visual acuity, restrictions in motility of the eye, epistaxis, and nasal obstruction. Sarcomas and benign bone and cartilage tumors arise from surrounding structures, whereas carcinomas usually arise from the paranasal sinuses. Surgery is the mainstay of treatment. Depending on the aggressiveness and histology of the tumor, surgery may be combined with radiation and chemotherapy. The prognosis is generally poor, but varies depending on histology and cell origin, size of the tumor, and degree of invasion. Meningiomas and benign bone tumors have the best prognoses. Sinonasal undifferentiated carcinomas, small-cell neuroendocrine carcinomas, osteosarcomas, and rhabdomyosarcomas have poorer prognoses.

Atypical pituitary adenoma with orbital invasion: Case report and review of the literature.

Pituitary adenoma invasion into the orbit is a rare phenomenon with only 22 cases, including the present case, in the literature. Our case is a 31-year-old man who presented with biopsy-proven atypical pituitary adenoma invading the right orbit after a prior resection. We compare his clinical course with previous cases and discuss clinical features, radiological features, management considerations, histologic features, and prognosis. Cases are organized by specific pituitary tumor type to aid in determining appropriate management. Early surgical intervention is the key, especially in the setting of pathologic features indicating aggressive tumor behavior or worsening visual function but is generally not indicated in prolactin-secreting adenomas that may respond to medical therapy. The role of radiation therapy is not fully established; however, it should be strongly considered in conjunction with or after surgery, especially in cases where complete resection is not achieved or histological and molecular analyses indicate a high likelihood of recurrence. More uniform and comprehensive data about management and outcomes are needed to determine the optimal treatment approach for this rare entity.