Orbital Malignancies Research Articles

An Unusual Presentation of Alveolar Type of Rhabdomyosarcoma of Orbit

Orbital rhabdomyosarcoma is the commonest primary orbital malignancy in children. A case of alveolar type of orbital rhabdomyosarcoma with unusual presentation in a three and half years male child presented with painless nodular swelling in the left lower lid of one and half years duration and gradual protrusion of the left eyeball. Examination of the patient revealed a mass in the left lower lid region extending to other parts of orbit and lymphnode metastasis in the neck region along with non axial proptosis, rest of the systemic examination being normal. Patient underwent incisional biopsy of the mass and excisional biopsy of the lymphnodes which revealed alveolar type of primary orbital rhabdomyosarcoma with lymphnode metastasis.

A prospective comparison of fine-needle aspiration cytopathology and histopathology in the diagnosis of orbital mass lesions

Aim:To assess the diagnostic value of the orbital fine needle aspiration biopsy (FNAB) with an in vitro technique, eliminating the sampling error.Design:Prospective, non-randomised, interventional case series.Methods:Sixty-eight patients were studied prospectively...

Ophthalmic lymphoma: epidemiology and pathogenesis

With a lifetime risk of 1% and 700 new cases per year, Non-Hodgkin lymphoma (NHL) is the seventh most frequent type of cancer in Denmark. The incidence of NHL has increased considerably in Western countries over the last decades; consequently, NHL is an increasing clinical problem. Ophthalmic lymphoma, (lymphoma localized in the ocular region, i.e. eyelid, conjunctiva, lacrimal sac, lacrimal gland, orbit, or intraocularly) is relatively uncommon, accounting for 5%-10% of all extranodal lymphomas. It is, however, the most common orbital malignancy. The purpose of this thesis was to review specimens from all Danish patients with a diagnosis of ophthalmic lymphoma during the period 1980-2005, in order to determine the distribution of lymphoma subtypes, and the incidence- and time trends in incidence for ophthalmic lymphoma. Furthermore, an extended analysis of the most frequent subtype, extranodal marginal zone lymphoma (MALT lymphoma), was done to analyse clinical factors and cytogenetic changes with influence on prognosis. A total of 228 Danish patients with a biopsy-reviewed verified diagnosis of ocular adnexal-, orbital-, or intraocular lymphoma were identified. We found that more than 50% of orbital- and ocular adnexal lymphomas were of the MALT lymphoma subtype, whereas diffuse large B-cell lymphoma (DLBCL) predominated intraocularly (Sjo et al. 2008a). Furthermore, lymphoma arising in the lacrimal sac was surprisingly predominantly DLBCL (Sjo et al. 2006). Incidence rates were highly dependent on patient age. There was an increase in incidence rates for the whole population from 1980 to 2005, corresponding to an annual average increase of 3.4% (Sjo et al. 2008a). MALT lymphoma arising in the ocular region was found in 116 patients (Sjo et al. 2008b). One third of patients had a relapse or progression of disease after initial therapy and relapses were frequently found at extra-ocular sites. Overall survival, however, was not significantly poorer for patients with relapse. Furthermore, we found that the frequency of translocations involving the MALT1- and IGH-gene loci is low in ocular region MALT lymphoma (2 of 42, 5%), but may predict increased risk of relapse (Sjo et al. 2008b). In conclusion the incidence of ophthalmic lymphoma is increasing at a high rate in Denmark. Ophthalmic lymphoma consists primarily of MALT lymphoma. The molecular pathogenesis of MALT lymphoma arising in the ocular region rarely involves translocations in the MALT1- and IGH-gene loci.

Therapiekonzepte und Behandlungsergebnisse maligner Orbitatumoren im Kindesalter

Each year approximately 1,800 children are diagnosed with cancer in Germany. The orbit is a very rare manifestation site for malignancies in childhood. The most common primary malignant orbital tumour is the rhabdomyosarcoma. Secondary orbital tumours are generally metastases of neuroblastomas or haematological diseases. The five-year survival rate of children with rhabdomyosarcomas has increased to 90 % in the past 30 years due to innovations in radiation and chemotherapy. A paediatric oncological interdisciplinary approach is taken in the diagnostics and therapy for these rare tumours and is based on therapy optimisation studies that include polychemotherapy, radiation therapy, and surgical treatment. We discuss the current multimodal therapy strategies and results, as well as the role of the ophthalmologist, in the medical care of young patients with malignant orbital tumours, with examples of individual courses of rhabdomyosarcoma, non-Hodgkin's lymphoma, and metastases. Orbital rhabdomyosarcoma is one of the few life-threatening diseases often first seen by an ophthalmologist. The ophthalmologist's prompt diagnosis and initiation of therapy therefore strongly influences the patient's chance of survival. The role of the ophthalmologist in the medical care of children with orbital malignancies includes a timely clinical diagnosis with histological confirmation, close monitoring of visual functions, the management of ocular complications, as well as long-term follow-up care to detect later therapeutic side effects and/or complications. The treatment of children with suspected tumours is best performed in paediatric oncological centres with access to all necessary specialties in order to ensure rapid diagnosis and therapy.

Orbital Hyperostosis Following Exenteration

To report the development of unilateral, diffuse hyperostosis of orbital bones in 3 postexenteration patients. Three advanced cases of periorbital and orbital malignancies are presented, and their clinical, radiologic, and histopathologic features are reviewed. Postexenteration CT showed thickened orbital walls in all 3 cases. The histopathology revealed necrotic destruction of the trabeculae mixed with randomly distributed chronic inflammatory cells and fibrosis and inflamed overlying granulation tissue. The delayed healing of the granulation tissue during secondary intention in these cases is quite likely the cause of chronic osteitis of the underlying orbital bone that, in turn, led to postoperative diffuse hyperostosis.

Endoscopic power-assisted orbital exenteration: A novel technique

Orbital exenteration has traditionally been performed via an open approach for orbital malignancies. The open technique results in significant cosmetic deformity, provides limited exposure to the orbital apex, and leaves an exposed cavity requiring spontaneous granulation or flaps for coverage. The advent of minimally invasive techniques has allowed for consideration of orbital exenteration by using the endoscope as the primary surgical modality. This article describes the author's technique for endoscopic power-assisted orbital exenteration (EPOE).

Orbito-ocular lesions in Lagos

we present a 10year retrospective clinicopathologic study of 135 cases of orbito-ocular lesions. The aim is to document pattern of orbito-ocular lesions, with their site, age and sex distribution. The materials consisted of slides, paraffin embedded tissue blocks and histology request forms of all orbito-ocular samples received at the Morbid Anatomy Department of Lagos University Teaching Hospital Idi-Araba Lagos between 1994 and 2003. A total of 135 samples from 75(56%) males and 60(44%) females were analysed. The conjunctival, intraocular, orbital and eye lid lesions accounted for 27(20.1%), 75(55.5%), 18(13.4%) and 15(11%) cases respectively. The ages ranged from 20 days to 79 years with a mean age of 35 years. Sixty nine cases (51.1%) of all orbito-ocular lesions were seen in children of age 15 years and below. Squamous cell carcinoma was the commonest malignant conjunctival lesion (4 out of the 5 cases) occurring in adults with a mean age of 45 years. Retinoblastoma accounted for 46(85%) of all orbito-ocular malignancies with the peak in the 1-5year age group accounting for 37(80 %). Panophthalmitis, endophthalmitis and phthisis bulbi were common non neoplastic lesions requiring enucleation. Pseudotumour and rhabdomyosarcoma accounted for 5(27.8 %) and 4(22.2 %) cases respectively of all orbital lesions. Retinoblastoma remains the commonest ocular malignancy while rhabdomyosarcoma was the commonest orbital malignancy with both occurring in childhood. Inflammatory lesions unfortunately are important orbito-ocular lesions requiring surgery in our environment.

Ocular melanoma presenting as a case of panophthalmitis in a 78-year-old Nigerian woman (masquerade syndrome): an uncommon finding in Benin City, Nigeria

Theodore states that the term 'masquerade syndrome' first appeared in the ophthalmic literature in 1967 to describe a conjunctival carcinoma that presented as chronic conjunctivitis. Since then, the masquerade syndrome label has been applied to a group of disorders that mimic ocular inflammatory disease. Although some benign conditions can be considered masquerade syndromes, most often the term refers to malignant entities. Tsai and O'Brien also report that ocular oncology, the subspecialty that concerns itself with ocular and orbital malignancies, is by nature a field of uncommon disorders. A discussion of the masquerade syndrome therefore becomes a study in uncommon presentations of uncommon diseases. Nevertheless, the subject deserves wide attention because, in many cases, the diseases that masquerade are not only vision-threatening but potentially fatal. Significant morbidity and mortality can be averted by early recognition and diagnosis of the masquerade. In this article, we present a case of a 78-year-old Nigerian woman who had an ocular melanoma that presented as a panophthalmitis, a rare case of masquerade syndrome in our environment.

Index of Suspicion

Index of Suspicion

Therapeutic management of ocular adnexal MALT lymphoma

Non-Hodgkin lymphomas constitute 50% of all orbital malignancies. Mucosa-associatedlymphoid tissue (MALT)-type lymphoma is the most common histology category in thisanatomic region. Ocular adnexal lymphoma of MALT-type is an indolent and rarely lethalmalignancy that can often be managed with observation alone. Occasionally, lymphomatouslesions determine the symptoms that condition a patient’s quality of life, andso require an immediate antineoplastic treatment. Several strategies are available, butreliable indications supported by prospective trials do not exist. Some therapeuticstrategies are associated with substantial side effects and require a well-balancedtherapeutic decision, which should take into account several variables related to thepatient, the lymphoma, and the treatment itself. In this review, the authors analyzerelated literature and propose therapeutic guidelines for the management of ocular adnexallymphoma of MALT-type.

Orbital rhabdomyosarcoma: A case series

Orbital rhabdomyosarcoma is the most common orbital malignancy of childhood with the common presentation of rapidly evolving unilateral proptosis. We studied six patients who were diagnosed and treated for rhabdomyosarcoma between January 1999 and June 2004. The age of the patients ranged from 4 to 29 years. Four patients presented with acute onset proptosis associated with signs of inflammation, mimicking orbital cellulitis. One patient presented with lid mass. Another patient presented with a soft, blind eye that was pushed superotemporally by a large inflammed, vascularised mass. Embryonal rhabdomyosarcoma was the commonest histopathological type in our series found in five patients. One patient was completely cured with chemotherapy alone whereas two patients were treated with a combination of chemotherapy and radiotherapy. Three patients in our series required exenteration.

Preliminary Study of Positron Emission Tomography in the Detection and Management of Orbital Malignancy

To determine the clinical efficacy of positron emission tomography (PET) in the treatment of patients with orbital malignancy. Prospective, consecutive case series to assess the ability of PET to (1) identify lesions previously demonstrated on CT or MRI; (2) characterize the metabolic activity of these lesions; and (3) determine the presence of metabolically active metastases elsewhere in the body. Eighteen patients with suspected orbital malignancies, ages 32 to 78 years, underwent PET in addition to CT and/or MRI. Sixteen of 18 also underwent orbital biopsy. Histopathologic diagnosis included lymphoma (55%), carcinoma (22%), melanoma (11%), sarcoma (5.5%), and lymphoid hyperplasia (5.5%). All orbital lesions were seen on conventional imaging. Overall, 61% of orbital lesions were demonstrated on PET, including 55% of tumors that were lymphoproliferative and 100% (n = 5) of other malignancies. PET was useful in ruling out tumor recurrence after exenteration in 2 patients. In this small clinical series, PET proved effective in demonstrating nonlymphoproliferative orbital malignancy. This series is too small to study any correlation between histopathologic aggressiveness and PET findings in orbital lymphoma.

The orbits in cancer imaging

Primary malignant lesions in the orbit are relatively uncommon. However, the orbits are frequently involved in haematogeneous metastasis or by direct extension from malignancies originating from the adjacent nasal cavity or paranasal sinuses. This paper focuses on the more commonly encountered primary orbital malignancies and the mapping of tumour spread into the orbits.

A case of primary botryoid conjunctival rhabdomyosarcoma

Rhabdomyosarcoma is the most common childhood primary orbital malignancy, but the occurrence of this tumour in the conjunctiva is rare. A 14-year-old girl presented with a visible case of conjunctival mass in the left eye. The year before, the patient was referred to another hospital for a biopsy of a conjunctival mesenchymal tumour in the same site, which was periodically checked after the excision. We intervened by partially removing the conjunctival mass. The histopathology revealed a botryoid rhabdomyosarcoma. The patient underwent chemotherapy and fractionated radiotherapy. The follow-up after three years revealed that the patient remained clinically tumor-free, with no ocular complication from the radiotherapy. It is evident that by not excising the tumour in its entirety it is therefore possible to preserve the underlying rectus muscles and not interfere with ocular motility. Subsequent radiotherapy and chemotherapy helped to destroy any residual tumour. Alternatively, surgical excision of the mass alone could lead to recurrence of the tumour after some months or years.

Orbital exenteration: One size does not fit all

To evaluate the clinical indications for orbital exenteration in a tertiary referral center and to compare clinicopathologic correlation and cosmetic outcome with previously reported data. Retrospective, nonrandomized, consecutive case series. Review of Electronic Medical Record system, Orbital Clinic, Jules Stein Eye Institute, between January 1999 and December 2003. main outcome measures: Surgery type, clear margins histologically, survival, and wearing an eye patch. Thirty-four patients (mean age 67 years) underwent orbital exenteration; mean follow-up 1.2 +/- 1.5 years (6 months to 6 years). Diagnosis included orbital, ocular, and adnexal malignancies, with squamous and basal cell carcinoma being the most common. Twenty-one patients (62%) underwent total or extended orbital exenteration, and 13 patients (38%) underwent subtotal exenteration including tissue reconstruction. Clear surgical margins were obtained in 23 cases (68%), whereas positive margins were left in 11 cases (32%). Many of the patients preferred an eye patch to cover the surgical region regardless of surgical reconstruction. Only 4 patients (11.8%) who underwent subtotal exenteration with orbital prosthesis did not use a patch. During follow-up period 3 patients expired, only 1 of which was tumor-related. Clinical indications for orbital exenteration remain similar over the last four decades with a higher prevalence of squamous cell carcinoma in our institute. Orbital exenteration is considered curative in cases of basal or squamous cell carcinoma but not in cases of malignant infiltrative processes such as adenoid cystic carcinoma of the lacrimal gland. Patients are likely to wear an eye patch regardless of any attempt at surgical reconstruction.

Primary Orbital Liposarcoma in Li-Fraumeni Cancer Family Syndrome: A Case Report

The aim of this study was to describe a case of primary orbital liposarcoma in Li-Fraumeni syndrome. In July 1998 a 20-year-old woman with a histological diagnosis of orbital myxoid liposarcoma underwent surgical treatment in our department. Since the patient's family pedigree met the clinical criteria for the diagnosis of LFS, molecular analysis was performed, which resulted in a molecular profile consistent with Li-Fraumeni syndrome. The patient underwent orbital exenteration extended to the upper eyelid; surgical reconstructive steps were performed to permit placement of an orbital prosthesis. Two years after primary surgery the patient underwent a quadrantectomy with lymphadenectomy of the right axilla because of the presence of a nodule of 1.5 cm in diameter in the upper-lateral quadrant of the right breast. One year after the last surgery, the patient is disease free. The diagnosis of an orbital malignancy in a young patient with a family history of cancer should suggest the presence of an underlying genetic disorder like LFS; with molecular analysis we can now determine the genetic disorder and the exact location of the mutation, and also obtain important prognostic data using specific cellular markers. More prognostic information increases the chances of adequate personalized treatment.

Orbital rhabdomyosarcoma.

Although rhabdomyosarcoma (RMS) is a rare tumor among the entire group of mesenchymal malignancies, it is a relatively common lesion and significant challenge for the ocular oncologist in terms of its diagnosis and management. A comprehensive literature search of articles published over the past 30 years in PubMed was conducted. Orbital RMS usually presents as a space-occupying lesion in the orbit during the first decade and may mimic other neoplastic or inflammatory masses. The tumor has predilection for the superior nasal quadrant of the orbit. The clinical manifestations depend on the location of the tumor within the orbit and its rate of growth. The common histopathologic types are embryonal and alveolar varieties. CT and MR imaging are important in the evaluation of this tumor. Particular attention should be placed on the bone invasion and extension of the tumor into the intracranial cavity and paranasal sinuses. Treatment usually consists of a combination of chemotherapy and radiation therapy following excisional biopsy. Survival of orbital RMS has improved due to advances in chemotherapy and radiotherapy. Posttreatment complications, including side effects of radiotherapy and secondary orbital malignancies, as well as visual dysfunction, occur more often and present new challenges due to improved long-term survival.

The surgeon's role in pediatric orbital malignancies: an oncologist's perspective.

The surgeon's role in pediatric orbital malignancies: an oncologist's perspective.

Downgaze “hang-up” of the upper eyelid in patients with adult-onset ptosis: An important sign of possible orbital malignancy

Purpose To highlight, in patients with adult-onset ptosis, the importance of upper eyelid hang-up during downgaze as a clinical sign of serious orbital disease. Design Retrospective, observational case series. Patients and methods Retrospective review of the presenting clinical symptoms and signs for a series of seven patients, some referred for treatment of adult-onset ptosis, with hang-up of the upper eyelid on down-gaze. Results All patients had symptoms of upper eyelid ptosis at presentation, starting at ages ranging from 29 to 81 years (mean, 64 years; median, 70 years). All patients displayed marked hang-up of the affected upper lid on downgaze, and the levator excursion was reduced (range, 2–13 mm; mean, 9 mm) compared with the asymptomatic side. All patients with downgaze hang-up had associated orbital malignancy, but they otherwise had only minimal or no underlying orbital disease. Exophthalmometry varied between subtle (1 mm) relative enophthalmos in two cases, to mild relative proptosis (3/7 cases; range, 2–3 mm). Ipsilateral up gaze was reduced in 4 of 7 patients (57%). Conclusion In contrast to patients with adult-onset blepharoptosis (resulting from dehiscence of the levator muscle aponeurosis), adults presenting with ptosis resulting from malignant infiltration of the upper orbital tissues may display hang-up of the upper eyelid in downgaze. This important sign should alert the clinician to the possibility of serious underlying orbital disease.

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.