Orbital Invasion Research Articles

Plaque radiotherapy for selected orbital malignancies: preliminary observations: the 2002 Montgomery Lecture, part 2.

To describe the principles and preliminary results of plaque brachytherapy for selected orbital malignancies. A custom-designed Iodine-125 plaque, designed to deliver a target dose of 50 Gray, was placed surgically in the region from which an orbital malignancy was partially resected. The mean dose to the target area was 50 Gray. The initial and follow-up patient data were reviewed. Of the 8 patients, the diagnosis was adenoid cystic carcinoma of the lacrimal gland (4 cases), orbital invasion by basal cell carcinoma (2), orbital extension of conjunctival melanoma (1), and metastatic carcinoma (1). Of the 4 with adenoid cystic carcinoma, there was microscopic residual tumor after excision and orbital exenteration was considered. Three have tumor control with follow-up of 1, 3, and 6 years. One patient required exenteration for recurrence separate from the field of brachytherapy and is free of tumor after 10 years. All 4 patients are alive and well with tumor control. Of the 2 patients with orbital extension of basal cell carcinoma, tumor control without recurrence has been achieved in both after 2 years. The patient with orbital metastasis responded to plaque radiotherapy, with no orbital recurrence, but died of systemic metastasis. The patient with orbital melanoma had local orbital recurrence separate from the area of irradiation and is currently being treated for systemic metastasis. Based on preliminary observations, plaque radiotherapy appears to be a reasonable alternative to exenteration and external irradiation for selected orbital malignancies.

Computed tomographic findings in patients with invasive fungal sinusitis.

To determine the radiographic findings of computed tomographic (CT) imaging most suggestive of invasive fungal sinusitis (IFS) in an immunocompromised patient population. A retrospective review of patients with a diagnosis of IFS reached with CT and confirmed by histopathologic evaluation. An academic tertiary care hospital. Twenty-three immunocompromised patients with confirmed IFS and preoperative CT imaging. Controls were 10 patients with acute myelocytic leukemia and CT evidence of sinusitis but no history of IFS. The CT scans were reviewed to identify factors predictive of invasive fungal disease. Parameters evaluated were nasal cavity and sinus soft tissue thickening, the presence of air-fluid levels, bone erosion, extrasinus extension, and unilateral or bilateral nasal cavity and sinus involvement. The CT findings included severe soft tissue edema of the nasal cavity mucosa (turbinates, lateral nasal wall and floor, and septum) in 21 of the 23 patients, sinus mucoperiosteal thickening in 21, bone erosion in 8, orbital invasion in 6, facial soft tissue swelling in 5, and retroantral fat pad thickening in 2. Two patients had air-fluid levels. No patients had intracranial involvement. Unilateral involvement was found in 21 patients, and bilateral involvement in 2. Review of the control group revealed only mild soft tissue edema of the nasal cavity in 2 (P<.001), unilateral involvement in 2 (P<.001), and evidence of bone erosion or extrasinus soft tissue involvement in none. Most patients do not have classic CT findings of bone erosion or extrasinus extension in the early course of IFS. We found that severe unilateral thickening of the nasal cavity mucosa was the most consistent finding on CT suggestive of underlying IFS, occurring much more frequently in immunocompromised patients with IFS than without IFS. Even though severe nasal cavity soft tissue thickening is much more common in IFS, this is a nonspecific finding that can be seen, to a lesser degree, in all forms of rhinosinusitis. Therefore, the clinician cannot rely solely on CT imaging and must maintain a high index of suspicion when evaluating immunocompromised patients to establish a prompt diagnosis. Early nasal endoscopy with biopsy and initiation of appropriate therapy are necessary to improve prognosis.

Prognostic significance of Fas expression in retinoblastoma

Purpose: The loss of Fas is believed to enhance metastatic spread in diverse human tumors. We studied the expression of Fas by immunohistochemistry in retinoblastomas and correlated the results clinicopathologically. Methods: Archival specimens of 76 retinoblastomas were included in this study. The tumors were divided into three groups: Group A (n = 21) comprising tumors with no invasion; Group B (n = 46) tumors with invasion of choroid (diffuse), optic nerve (post-laminar), and orbit; and Group C (n = 9) tumors with both invasion and metastasis. Sections were immunostained with monoclonal antibody CD95 (APO-1/Fas) and the immunoreactivity was assessed. Results: Negative immunoreactivity of Fas was observed in Group B and Group C tumors (p < 0.001). Poorly differentiated retinoblastomas showed a decreased Fas expression (p = 0.006). Conclusions: Decreased expression of Fas is seen in aggressive tumors and tumors that are poorly differentiated. This preliminary observation deserves further investigation, which may then shed more light on the immune escape mechanisms of this tumor and thus enable novel therapeutic strategies.

CT assessment of bone marrow transplant patients with rhinocerebral aspergillosis

Purpose: Invasive rhinocerebral aspergillosis is a hazardous complication of bone marrow transplantation. A study was conducted to describe the computed tomography (CT) features of this disease. Materials and Methods: Charts and CT scans of bone marrow transplant patients with invasive rhinocerebral aspergillosis were reviewed. The diagnosis was confirmed by otolaryngologic examination and biopsy of suspected lesions. Sinus CT scans were conducted without contrast material. They were repeated with contrast if intracranial or intraorbital extension was suspected. CT scans were correlated with physical examination, history of disease, and histology. Results: Of 1,013 bone marrow transplantations carried out over a 10-year period, 21 patients (2.07%) developed invasive rhinocerebral aspergillosis. In most cases the infection was unilateral. Air-fluid levels and calcifications were rarely encountered. Mucosal thickening, soft tissue hyperdense masses in the nasal cavity and/or sinuses, and clear (uninvolved) ethmoid cells amid diseased cells, were significant CT features that characterized invasive fungal infection. Opacification of the sinus represented extensive disease with tissue necrosis. When bone destruction was encountered, fulminant infection was already present, usually with significant orbital and/or brain invasion. Conclusions: When correlated with clinical findings and histologic results, the above CT features can help identify early infection so that appropriate medical and surgical treatment can be instituted. (Am J Otolaryngol 2002;23:328-331. Copyright 2002, Elsevier Science (USA). All rights reserved.)

Sinonasal undifferentiated carcinoma and nasopharyngeal-type undifferentiated carcinoma: two clinically, biologically, and histopathologically distinct entities.

Sinonasal undifferentiated carcinoma (SNUC) is a rare aggressive neoplasm arising in the nasal cavity and paranasal sinuses. Primary sinonasal nasopharyngeal-type undifferentiated carcinoma (PSNPC) is an even rarer tumor that has not been adequately reported. Both tumors have been reported to be associated with Epstein-Barr virus (EBV). We studied the clinicopathologic features and EBV status of 36 SNUC and 13 PSNPC patients from Taiwan, an EBV endemic area. The median age of SNUC patients was 53 years (range 20-76 years), with a male/female ratio of approximately 2:1. Five patients had histories of previous nasopharyngeal carcinoma treated with irradiation 6-26 years earlier. The most common locations were nasal cavity and ethmoid sinus. Orbital and intracranial invasion and distant metastasis were frequent findings. The median survival was 10 months. All 36 tumors were negative for EBER-1 by in situ hybridization. The median age of PSNPC patients was 58 years (range 36-75 years), with a male/female ratio of approximately 2:1. The most common location is nasal cavity. Eight patients achieved disease-free survival. Eight tumors had the morphology of lymphoepithelioma, whereas significant inflammatory infiltrate was not detected in the other five tumors. All 13 tumors were positive for EBER-1 by in situ hybridization. Because of the difference in the relation with EBV, prognosis, and response to radiotherapy, SNUC and PSNPC should be considered as two entirely different entities. The most important criteria for PSNPC are vesicular nuclei, syncytial pattern, spindle cells, and absence of necrosis.

Histiocytoid variant of eccrine sweat gland carcinoma of the eyelid and orbit: report of five cases

ObjectiveTo study the clinicopathologic features of the histiocytoid variant of adenocarcinoma of the eccrine sweat gland of the eye and orbit. DesignRetrospective case series. ParticipantsFive patients undergoing orbital and eyelid biopsy as a diagnostic procedure. MethodsThe authors examined the clinical histories and pathologic findings of five patients with eccrine adenocarcinoma of the eyelid with orbital invasion. Main outcome measuresClinical and histopathologic examinations, including routine histopathology, immunohistochemistry, and electron microscopy studies. ResultsThe tumors presented as insidious, diffusely infiltrative, firm cutaneous masses in the periocular area that later infiltrated the orbit. Histopathologic examination revealed that the tumors infiltrated the dermis and were composed of cells with a histiocytic to signet ring appearance. Tumor cells exhibited intracellular mucin production. Immunohistochemical stains were positive in tumor cells for low and high molecular weight cytokeratins, carcinoembryonic antigen, and epithelial membrane antigen. Electron microscopic examination showed lumen formation and intracytoplasmic mucin in tumor cells. ConclusionsThe histiocytoid variant of adenocarcinoma of the eccrine sweat gland of the eyelid may present as an insidious tumor and diffusely invade the orbit. These cases may be confused with metastatic adenocarcinoma.

Clinicopathologic factors related to metastasis in retinoblastoma.

To investigate the orbital invasion and metastatic pattern in retinoblastoma, and to analyze the correlation with other pathologic and prognostic factors. Pathologic and admission records of 45 patients with a pathologically confirmed diagnosis of retinoblastoma during the past 20 years were reviewed. Clinical risk factors and pathologic findings were analyzed for association with tumor invasion/metastasis using chi-square, Fisher's exact, and Student's t tests. Clinical risk factors such as exophthalmos, cataract, and pseudohypopyon were significantly correlated with tumor invasion/metastasis. The pathologic finding of choroid involvement was highly predictive of tumor invasion/metastasis. Delayed enucleation (>3 months) also was significantly correlated with tumor invasion/metastasis. Tumor invasion/metastasis is strongly associated with various ocular, pathologic, and treatment outcome factors. Identification of these risk factors may help plan treatment regimens.

Ectopic chordoma with orbital invasion

PURPOSE: To report a rare ectopic chordoma within the orbital wall. METHODS: Case report. RESULTS: A 63-year-old woman developed swelling of the eyelid, tearing, blurred vision, and progressive proptosis RE of 1 month’s duration. Neuroimaging studies revealed an osteolytic mass with epicenter at the sphenozygomatic suture that eroded intracranially, invaded into the orbit, and compressed orbital soft tissues. Surgical debulking was done followed by radiation treatment. The pathologic findings of physaliphorous epithelial cells with multiple vacuoles containing mucin, prominent nuclei, and positive immunohistochemical staining for S-100, Vimentin, epithelial membrane antigen, and pancytokeratin were diagnostic for chordoma. CONCLUSION: Orbital wall ectopic localization of a chordoma distant from the clivus is a rare occurrence.

Ethmoidal sinus adenocarcinoma with orbital invasion.

To report a rare case of massive ethmoidal adenocarcinoma with orbital invasion but minimal ophthalmic symptoms on presentation. Case report of a 69-year-old, otherwise healthy, retired carpenter who was referred for treatment of bilateral senile cataract. A relative afferent pupillary defect and sectorial disc atrophy on ophthalmic examination led to further investigation and identification of an extensive ethmoidal neoplasm with orbital invasion. An incisional biopsy was performed and histopathologic examination revealed an adenocarcinoma of low-grade malignancy. Ethmoidal adenocarcinomas with orbital involvement may occasionally be relatively asymptomatic and masked by coexisting ocular disease.

Does the combination of radiotherapy and debulking surgery favor survival in paranasal sinus carcinoma?

Purpose: To determine the contribution of debulking surgery on local control and survival in paranasal sinus tumors. As most patients present with locally advanced disease, the possibility of radical surgery is limited. Consequently, radiotherapy is often needed as monotherapy or as an adjunct to surgery. Methods and Materials: Between 1977 and 1996, 73 patients (50 male: 23 female) with a paranasal sinus carcinoma were treated. The histology distribution was as follows: squamous cell carcinoma, 55%; adenocarcinoma, 19%; adenoid cystic carcinoma, 11%; and undifferentiated carcinoma, 15%.The clinical T classification was (UICC/TNM 1997): T2 14%, T3 27%, and T4 59%. Pathological neck nodes were found in 11% of patients. Treatment consisted of surgery only in 3, chemotherapy only in 1, radiotherapy only in 18, both surgery and radiotherapy in 50 patients. One patient did not receive any treatment at all. Three patients had concurrent chemotherapy. Median follow-up was 66 months (range, 1–213 months). Results: Five-year local control (LC) was 65% with combination of radiotherapy and debulking surgery in comparison with 47% with radiotherapy alone, but this difference was not statistically significant ( p = 0.58). However, combination treatment gave significantly better 5-year overall survival (OS) (60% vs. 9%; p = 0.001) and 5-year disease-free survival (DFS) (53% vs. 6%; p < 0.0001). Cox-regression analysis showed that pathologic N status ( p = 0.04), palliative intention of treatment ( p = 0.018), clinical orbital invasion ( p = 0.003), and orbital wall invasion ( p = 0.003) were parameters significantly associated with poor local control. Total radiation dose of greater than 65 Gy ( p = 0.05) and treatment consisting of radiotherapy alone ( p = 0.002) were associated with worse overall survival; for disease-free survival clinical orbital invasion ( p = 0.0005), age of greater than 65 years ( p = 0.013) and pathologic T4 classification ( p = 0.002) were significant factors for an unfavorable outcome. In 19 of 73 patients, 26 serious (mainly ophthalmological) complications were reported; in the majority of these, the visual tract was (partly) included in the treatment fields because of tumor extension. To analyze on which basis patients were selected for the combination therapy, a logistic regression was performed, concluding that clinical T4 classification ( p = 0.05), radiological evidence of skull base invasion ( p = 0.005), age of greater than 65 years ( p = 0.026), radiological evidence of nasopharynx invasion ( p = 0.02), clinical suspicion of palate invasion ( p = 0.02), and radiological evidence of skin invasion ( p = 0.009) were associated with choosing radiotherapy alone. Conclusion: Debulking surgery of paranasal sinus malignancies followed by high-dose radiotherapy to the involved sites was associated with better survival and (although not statistically significant) local control. Patient selection, based on clinical and radiological impression of tumor extension, was the main factor explaining these favorable results. We favor this combination regimen because the surgery gives quick relief of complaints and, at the same time, offers an excellent histologically proven staging method, enabling radiotherapy to be adjusted to the involved sites, thereby decreasing the risk of complications. This can all be achieved with a very low orbital exenteration rate.

The clinical radiological and histological correlation of orbital assessment in malignant lesions of the maxillo-ethmoid complex

Twelve patients undergoing surgery between May 1995 and March 1999 for malignant tumours of the paranasal sinuses abutting or invading the orbital walls were studied for the need to remove orbital contents. Alt patients were evaluated clinically, radiologically (CT Scan) and per-operatively to delineate the extent of orbital invasion. Combined regimen (Surgery + Radiotherapy) were used in all patients. A biopsy from periorbita was taken when eye was spared. It was found that per-operative evaluation of the orbit is equally specific in comparision to clinical and CT Scan combined and eye may be preserved in selected patients with involvement of orbital periosteum provided there is no frank soft tissue mass in the orbit.

Perineural spread in head and neck skin cancer.

Perineural spread (PNS) in the head and neck is an infrequent but aggressive manifestation of skin cancer. As such, it can provide access to the intracranial cavity. Squamous cell carcinoma is the most common histology with the facial and trigeminal nerves most often involved. Orbital invasion is an uncommon but devastating result of PNS located around the orbit, particularly the forehead. Diagnosis can be difficult and initial investigations are often unhelpful. Treatment should ideally be directed at preventing further spread before it develops. Adjuvant radiotherapy is often recommended. The disease may present at an advanced state within the orbit or parotid gland or even within the intracranial cavity. Clinicians need to be aware of the potential for PNS because a group of these patients will die from this potentially preventable and treatable form of metastatic skin cancer.

Mucoepidermoid Carcinoma of an Accessory Lacrimal Gland With Orbital Invasion

To report a case of mucoepidermoid carcinoma of an accessory lacrimal gland with orbital invasion. The clinical history and pathologic findings of a patient with a left upper eyelid lesion were reviewed. The patient was evaluated and found to have an epithelial tumor arising in an accessory lacrimal gland. Special stains showed mucin production by individual tumor cells. The tumor was classified as mucoepidermoid carcinoma. Mucoepidermoid carcinoma may arise in accessory lacrimal glands and invade the orbit.

Efficacité et danger de l'interféron alpha dans le traitement des hémangiomes graves du nourrisson

Alarming hemangiomas, due to their site or repercussions, require pharmacological treatment. Corticosteroid therapy is indicated by first intention. In the event of failure, interferon alpha is proposed. Case 1. A five-week-old infant was admitted to hospital for an extensive hemangioma of the left side of the face and neck with necrosis of the upper lip and ear. Prednisolone (2 mg/kg/day) by intravenous route brought about no improvement. Interferon alpha 2a (3 MU/m2/day of Referon by subcutaneous injection) enabled regression of lesions from the sixth month of treatment. After 11 months of treatment, the hemangioma had all but disappeared and interferon therapy was stopped. Repair surgery was planned at 24 months of age. Case 2. A one-month-old infant suffered from a hemangioma of the right side of the face with orbital invasion and risk of amblyopia. Prednisone (2 mg/kg/day) by oral route was ineffective. Interferon alpha 2a enabled regression of the hemangioma and the eye opened from the third month of treatment. Interferon therapy was stopped after 14 months. Initial repair surgery intervention was possible at two years of age. Spastic paraplegia was diagnosed at 18 months of age. The brain and medullar magnetic resonance imaging was normal. No etiology could explain the neurological attack. The possible toxic effect of interferon alpha is discussed. Interferon alpha is an effective treatment for hemangiomas. It significantly reduces spontaneous regression time. The uncertainty of long-term effects in infants with hemangiomas incites its indication to be limited to alarming corticosteroid-resistant forms and necessitates prolonged neurological surveillance.

Fronto-ethmoid mucocele: an unusual presentation

Mucoceles arising from the sinuses usually present with proptosis and displacement of the globe. Posterior orbital invasion can occasionally present with ophthalmoplegia or ptosis. This communication discusses an unusual presentation of a fronto-ethmoid mucocele presenting as a fistula in the upper lid. The diagnosis was based upon the computerised tomographic scan of the paranasal sinuses. The patient subsequently underwent surgical excision of the mass, which relieved his symptoms.

Concha bullosa mucocele with invasion of the orbit

Concha bullosa mucocele with invasion of the orbit

Squamous cell carcinoma of the sinonasal tract invading the orbit.

The treatment of squamous cell carcinoma (SCC) of the sinonasal tract has evolved from routine exenteration of the orbital contents to sparing of the orbit when the SCC does not transgress the periorbita. Nonetheless, the influence of this change in treatment over the rate of local recurrence or survival has not been clearly elucidated. The objective was to ascertain whether orbital sparing surgery for the treatment of SCC of the paranasal sinuses influences the rate of local recurrence or survival. This is a retrospective study of patients presenting with SCC arising in the sinonasal tract, treated primarily at the University of Pittsburgh Medical Center from 1977 to 1990, including meta-analysis of the English literature regarding SCC of the sinonasal tract. Review of medical records regarding demographics, histology, extension of tumor, pathologic results, type of surgery, adjunctive therapy, and outcome. Articles for meta-analysis were identified by Medline search and cross-referencing. Fifty-eight patients with orbital invasion, including bone and/or soft tissue invasion, were included in the study. Patients presenting with invasion of the bony orbit without soft tissue invasion were treated with maxillectomy and/or ethmoidectomy, sparing the orbital contents. Patients presenting with invasion of the orbital bones and soft tissues were treated with ethmoidectomy or maxillectomy, including orbital exenteration. At 3 years' follow-up, 52% of the patients whose orbit was exenterated were alive and without evidence of disease, compared with 59% of the patients whose orbit was spared. This difference was not statistically significant. Similarly, the rate of local recurrence was not statistically significant (P > .05). A meta-analysis of the literature revealed similar results. Our data suggest that sparing of the soft tissues of the orbit when the periorbita has not been transgressed by SCC does not downgrade the rate of cure or local control.

Intraepithelial and invasive squamous cell carcinoma of the conjunctiva: analysis of 60 cases

AIMTo evaluate the clinical features, treatment results, and recurrence rates in patients with either intraepithelial or invasive squamous cell carcinoma of the conjunctiva.METHODSRetrospective analysis of 60 cases (22 conjunctival intraepithelial...

Paranasal sinus mycoses in north India.

Recognizing the high incidence of paranasal sinus mycoses in north India, we analysed retrospectively the clinical, mycological and management aspects of 178 patients with proven disease attending our institute. On the basis of clinical, radiological, histopathological and mycological findings, the patients could be categorized into those with allergic (8), non-invasive (92) and invasive (78) disease types. Bony erosion without mucosal invasion by fungi was seen in 16 patients with non-invasive disease. Young men from rural areas were the most commonly affected. Rhinorrhoea with nasal polyposis (45.8%) and proptosis (46.4%) was the most common presentation. Concurrent involvement of the maxillary and ethmoid sinuses was common in these patients, whereas isolated sphenoid and frontal sinuses were involved in the invasive variety only. Orbital and intracranial extensions were detected in 100% and 13.2%, respectively, of patients with the invasive type of disease. Aspergillus flavus (79.7%) was the most common isolate. Surgical debridement and sinus ventilation were adequate for the effective management of the non-invasive disease. However, adjuvant medical therapy was included in treatment of the semi-invasive and invasive varieties of the disease. Itraconazole was found to be most useful in prevention of recurrence in the invasive type. Mortality was highest (33.3%) among patients with zygomycotic infection. Invasive fungal granuloma with orbital and intra-cranial invasion is a distinct entity in terms of its clinical course and treatment compared with non-invasive fungal sinusitis, and it needs to be treated aggressively with surgical excision and postoperative itraconazole.

Studies of the anatomy and pathology of the orbit in carcinoma of the maxillary sinus and their impact on preservation of the eye in maxillectomy.

The periorbita has been traditionally regarded as the decisive layer whose involvement in carcinoma of maxillary sinus indicates orbital exenteration. Anatomic texts depict that the orbital fat rests on the periorbita. Our experience at surgical dissections has been different. Twenty-five patients underwent total maxillectomy for advanced (T3-T4) carcinoma of the maxillary sinus with postoperative radiotherapy during a 15-year period between 1981 and 1995. In 11 patients, despite radiologic evidence of orbital invasion, and in 5 patients with infiltration of the periorbita, the eye could be preserved without evidence of recurrence. In 6 patients who in addition underwent orbital exenteration, histopathologic evidence of tumor invasion of the orbit was present in only one surgical specimen. Clinicoanatomic studies have clearly shown the presence of a thin, distinct fascial layer which surrounds the periocular fat and separates it from the periorbita. Neither clinical examination nor imaging could predict orbital invasion with absolute accuracy. Histopathologic examination of the surgical specimens shows that tumor infiltration of the eye is rare. Periocular fat is surrounded by a thin fascial layer and is not in direct contact with the periorbita. Intraoperative decision making with respect to preservation of the eye is sometimes essential.