Preoperative transarterial embolization of a recurrent orbital solitary fibrous tumor with significant hypervascularity: a case report.

BACKGROUND: Orbital solitary fibrous tumors (SFTs) are rare neoplasms. Recurrent, hypervascular, malignant variations of orbital SFTs have recently been noted and can present a surgical challenge. CASE PRESENTATION: We describe a case of a 53-year-old Chinese woman with a history of a resected orbital SFT. She presented with proptosis, limited eyeball movement, and visual loss in the right eye, suggestive of a recurrent SFT. Ocular examination with multimodal imaging revealed a large, nonpulsatile, noncompressible, hypervascular mass behind the eyeball. The patient underwent preoperative transarterial embolization of the main blood supply to the tumor in order to control intraoperative blood loss, followed by ocular enucleation to optimize exposure and enable complete resection of the tumor. Embolization of the right ophthalmic artery and the distal branch of the right internal maxillary artery caused an immediate, substantial reduction of vascular flow, which allowed us to enucleate the eyeball and resect the tumor with minimal blood loss and no complications. CONCLUSIONS: Our case is so far the first Chinese case of successful preoperative embolization of the main blood supply to a large, recurrent, hypervascular orbital SFT. This case also described a different surgical approach to achieve total removal of an orbital SFT without osteotomy.

Primary orbital yolk sac tumor: report of a case and review of literature.

Germ cell tumor can affect extragonadal sites. Teratoma is a well-recognized extragonadal tumor in the orbit. Primary yolk sac tumor (YST) or endodermal sinus tumor of orbit is rare and only few cases have been reported in the literature. Its clinical presentation may mimic many common pediatric orbital conditions, and delay in diagnosis affects ocular morbidity and mortality. In the past orbital YST has been treated with multimodal therapy including surgery, systemic chemotherapy, and radiotherapy. Herein we describe a case of primary orbital YST and reviewed the literature for similar cases. The review aims to describe the clinical presentation, imaging features, histopathological characteristics, and management of orbital YST.

Mesenchymal chondrosarcoma of the orbit: an unusual site for a rare tumour.

Mesenchymal chondrosarcoma is a rare tumour with orbital involvement being an exceptional occurrence. We present a case of a 22-year old man with such disease, together with details of his management. A brief literature review of this uncommon tumour was also enclosed.

[Sphenoorbital meningiomas: results in long-term treatment]. / Sphenoorbitale Meningeome: Ergebnisse der Langzeitbehandlung.

BACKGROUND: The purpose of this study was to present the findings on growth patterns in a large surgical series of sphenoorbital meningiomas. PATIENTS AND METHODS: A retrospective analysis was performed on 77 patients (61 f) harboring sphenoorbital meningiomas, who underwent surgery between 1991 and 2009. The standard surgical approach consisted of pterional craniotomy and extradural resection of any infiltrated bone. The intradural and orbital tumor was resected, with complete reconstruction of the dura while the lateral bone was partially reconstructed. The follow-up period ranged from 6 to 130 months (mean: 57.9 months). RESULTS: Total macroscopic resection was achieved in 42 patients. Fifty-eight meningiomas extended to the orbital roof and/or lateral orbital wall, 42 involved the extraconal space, and 11 the intraconal space. Sixty-three tumors involved the superior orbital fissure, 54 the optic canal, and 24 the inferior orbital fissure. Seventeen tumors infiltrated the cavernous sinus and 37 involved the anterior clinoid process. The rate of minor morbidity was 14.3% (slight deficits) and the rate of major morbidity was 4% (significant deficits). Subtotal resections were performed on 35 patients because there was intraorbital tumor (n=8); tumor in the cavernous sinus (n=12); tumor invading the superior orbital fissure (n=12); and tumor of the skull base (n=3). Nine patients underwent postoperative three-dimensional conformal radiotherapy, which resulted in stable tumor volume at follow-up in eight patients. Tumor recurrence was identified in ten patients (12.9%) postoperatively (range of follow-up: 10-47 months). CONCLUSION: The goal of surgery is complete tumor removal without morbidity. Exact analysis of tumor growth and possible involvement of pertinent structures are mandatory in planning the procedure.

Preoperative embolisation of orbital solitary fibrous tumour.

Solitary fibrous tumours (SFT) is an encompassing terminology comprising of tumours with proliferating CD34 positive specialised fibroblasts. Orbital SFTs are rare slowly progressive highly vascular neoplasms. Complete surgical excision is considered the mainstay treatment. Incomplete resection is a known risk factor for recurrence and malignant transformation. Recently preoperative embolisation of SFT has shown promising results in reducing the vascularity of these tumours rendering them amenable to complete surgical excision. Less than 10 cases of embolisation of orbital solitary fibrous tumours have been described in literature. Our patient underwent an attempted surgical excision elsewhere with significant intraoperative haemorrhage which precluded its complete excision. Herein, we report successful outcome in a case of hypervascular orbital SFT managed with preoperative embolisation, surgical resection and adjuvant radiotherapy along with a review of relevant literature.

Therapy and outcome of orbital primitive neuroectodermal tumor.

Primary orbital primitive neuroectodermal tumor (PNET) is rare with no reported series. We report six cases of orbital PNET treated at a tertiary care oncology center in northern India from 2003 to 2008. None of them had distant metastases. All were treated with neoadjuvant chemotherapy followed by exenteration in two, radiotherapy and adjuvant chemotherapy in five cases. Three out of six achieved complete remission at end of therapy with globe salvage in three and vision in two cases. Chemoradiotherapy may help us to avoid mutilating surgery in large or locally advanced tumors, allowing preservation of vision or the globe.

[A variety of orbital lymphangioma treatments: one case treated with orbital decompression therapy and the other case with intralesional injection of OK-432 therapy].

BACKGROUND: Orbital lymphangioma can result in ocular emergencies due to acute enlargement, leading to painful proptosis, compressive optic neuropathy, exposure keratopathy, restrictive ocular motility and increased intraocular pressure. Treatment methods and clinical courses of two cases with orbital lymphangioma are reported, one with orbital decompression therapy and the other with intralesional injection of OK-432 therapy. CASE 1: An 8-year-old girl had multilocular cystic orbital intracornal lymphangioma. Emergency operation was deemed necessary because the patient presented with the painful proptosis, the compressive optic neuropathy, and increased intraocular pressure due to acute enlargement, we put the orbital inferior wall was decompressed by inserting an intact inframedial orbital strut. The proptosis vanished completely and visual acuity loss improved. CASE 2: A 2-year-old girl had multilocular cystic orbital intra and extracornal lymphangioma. She had severe proptosis and exposure keratopathy. The extracornal cystic lesion was treated with an intralesional injection of OK-432. On the 7th day after injection, the patient presented with painful severe proptosis. Aspiration of the fluid in the cystic lesion was performed, and the drain was preserved. After the treatment, both the severe proptosis and the exposure keratopathy improved. CONCLUSIONS: Inferior wall decompression was performed on case 1 while keeping the inframedial orbital strut. Although the eye movement disorder was low at the time, the medial and lateral wall decompression improved. In case 2, the extracornal cystic lesion was treated with an intralesional injection of OK-432 Judging from the results of that clinical course, the injection of OK-432 to the intracornal lesion could prove dangerous to the visual performance. Although OK-432 is predictably-effective against lymphangioma, it should not perform be applied without careful consideration.

More than meets the eye.

We report an extremely rare case of a hybrid tumour of the maxillary sinus. A 51-year-old man presented with a 6-week history of nasal congestion and epiphora. Radiological imaging demonstrated a maxillary sinus tumour, with extensive local invasion. Surgical excision included maxillectomy, left eye exenteration and free flap closure. Histology of the excised specimen showed a rare hybrid tumour containing adenoid cystic carcinoma, salivary duct carcinoma, epithelial-myoepithelial carcinoma and basal cell adenoma. Hybrid tumours are very rare tumour entities which are composed of at least two distinct tumour types. Each tumour entity conforms with a defined tumour type. The tumour entities of a hybrid tumour are not separated but have an identical origin within a definite topographical area. Diagnosis and appropriate management requires high index of suspicion, pathological endeavour to look for a more aggressive accompanying tumour and adequate oncological treatment according to the highest grade of tumour.

Sclerosing therapy for orbital lymphangioma using sodium tetradecyl sulfate.

PURPOSE: To describe the results of intralesional injection of the sclerosing agent sodium tetradecyl sulfate in patients with orbital lymphangioma. METHODS: Four young patients with a history of orbital lymphangioma were treated on one or more occasions with percutaneous puncture and injection of sodium tetradecyl sulfate under computed tomography guidance. Resolution of the signs and symptoms, complications resulting from surgery, and recurrence of bleeding were studied. RESULTS: Three patients with a long-standing history of unilateral proptosis and one patient with progressive unilateral visual loss from multiple recurrent orbital hemorrhages were studied. Treatment ranged from one to three sodium tetradecyl sulfate injections. All patients showed improvement of their signs and symptoms after treatment. A decrease in the size of lesions was demonstrated by orbital imaging. Visual acuity and intraocular pressure remained unchanged. Complications included transient postoperative localized inflammation in all cases and transient ophthalmoparesis in one patient. Follow-up time ranged between 15 and 36 months, during which no recurrence of bleeding was observed. CONCLUSIONS: Our study suggests that intralesional injection of sodium tetradecyl sulfate under computed tomography guidance is an effective treatment for patients with orbital lymphangioma and is not associated with vision-threatening complications.

Sclerotherapy for Orbital Lymphangioma - Case Series and Literature Review.

Orbital lymphangioma is a lymphatic system lesion that commonly presents in childhood. Management of these lesions is complex. Sclerotherapy is a therapy used to treat and shrink lesions prior to or as an alternative to surgery. We present three cases of orbital lymphangioma that were treated with sclerotherapy. Case 1: A 6-month-old boy was presented in 2010 with right ptosis and proptosis. Magnetic resonance imaging (MRI) identified a lesion involving the right orbit and face. Case 2: A 3-year-old girl was presented in 2011 with intermittent right periorbital swelling and medial canthal bleeding. MRI identified a soft-tissue lesion in the right orbit, extending into the face. Case 3: A 3-year-old girl was presented in 2012 with vomiting, and painful right proptosis. MRI identified an intra-conal lesion in the right orbit with fluid filled levels. All three patients were treated with sclerotherapy (sodium tetradecylsulfate). Sclerotherapy is a promising treatment for orbital lymphangioma. Its use may prevent the need for, or minimise the amount of surgical management. Several sclerosants are now commonly used to treat these lesions.

Outcome of primary orbital lymphoma treated with induction chemotherapy followed by conformal radiotherapy.

PURPOSE: To analyze the clinical outcome of primary orbital lymphoma (POL) patients treated with a combined modality approach with local radiotherapy after induction chemotherapy. METHODOLOGY: We retrospectively retrieved demographic, treatment and outcome data of patients treated for POL from 2000 to 2010. The charts were reviewed and the data were tabulated in a predesigned pro-forma. RESULTS: 23 patients of POL were found evaluable. Median age was 55 years (range 24-70 years). Of 23 patients, 15 were male and 8 female, making the male:female ratio approximately 1.9:1. Patients were thoroughly evaluated and staged. All but one patient received multi agent chemotherapy. Radiotherapy was delivered for all cases. Radiation was delivered by 3DCRT technique. Median dose of radiation was 45Gy (range 20-45Gy). Median follow up was 26.8 months. None of the patients had any evidence of local failure or systemic progression. CONCLUSION: A combined modality therapy with a combination of CHOP/COP based chemotherapy and moderate dose of radiotherapy imparts excellent long term local and systemic disease control.

[Lymphoma of the ocular adnexa]. / Lymphome der okulären Adnexe.

BACKGROUND: Lymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics. AIM: The aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy. DIAGNOSTICS: Depending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory. THERAPY: External beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.

Embolization of neurosurgical lesions involving the ophthalmic artery.

OBJECTIVE: A number of anteriorly located cranial base and extracranial lesions receive their vascular supply wholly or in part from the ophthalmic artery, and embolization of the ophthalmic artery can be helpful in the management of these lesions, either as the primary treatment or as an adjunct to surgery. We present situations in which the embolization of lesions involving the ophthalmic artery was performed to effect a partial or total cure of the lesion. METHODS: Twelve patients underwent a total of 15 embolization attempts on lesions involving the ophthalmic artery. Four patients had arteriovenous malformations of the orbit, four had dural arteriovenous fistulae, two had orbital meningiomas, one had a planum sphenoidale meningioma, and one had a juvenile nasal angiofibroma. In each case, a Tracker No. 18 microcatheter (Target Therapeutics, Inc., Fremont, CA) was navigated into the ophthalmic artery using a steerable guidewire and digital road mapping. Embolic agents included polyvinyl alcohol particles ranging from 350 to 1500 microm in diameter, 2-mm platinum microcoils, and n-butyl-cyanoacrylate. In 12 of 15 cases, lidocaine and amytal provocation tests were conducted before any attempt at embolization to assess the role of the ophthalmic artery in vision. RESULTS: Embolization was successfully performed in the 14 situations in which it was attempted. Positive results of two lidocaine/amytal tests were noted. In one case, embolization was not attempted. In the other case, a larger caliber embolic agent (2-mm platinum coils) was used. A single transient decrease in visual acuity lasting 4 days was the only embolization-related complication. CONCLUSION: Proper case selection, judicious use of embolic agents, and use of provocative testing can result in safe embolization of lesions supplied by the ophthalmic artery.

[Results of therapy for orbital malignant lymphoma].

PURPOSE: To elucidate the clinical and histopathological features of orbital malignant lymphoma, and to develop a protocol for effective treatment. MATERIALS AND METHODS: The present study analyzed 18 cases of orbital lymphoma which were treated at Yamagata University Hospital over the last 14 years. The tentative strategy for choosing the treatment was to treat the cases of clinical stage I (Ann Arbor criteria) with curative intent and the cases of stage II-IV with palliative intent. RESULTS: Six patients had primary orbital lymphomas, 2 had secondary orbital lymphomas, and 10 had metastatic orbital lymphomas. Histopathologically, all the cases of primary lymphoma were of low-grade malignancy and all the cases of secondary lymphoma were of intermediate-grade malignancy. Of the metastatic lymphoma cases, 3 were of low-grade and 7 of intermediate-grade malignancy. The cases of primary lymphoma were Stage I and were treated by radiation or chemotherapy. The cases with secondary lymphoma were Stage II and were treated by both radiation and chemotherapy. The cases with metastatic lymphoma were Stage III or IV and were treated by chemotherapy and/or radiation. Control of orbital lymphoma was achieved in all 6 cases with primary lymphomas. Although the survival rate was 0% for secondary lymphomas and 50% for metastatic lymphomas, ocular complications were improved during the remainder of the patients' life. CONCLUSIONS: All the primary orbital lymphomas were treated effectively by radiation or chemotherapy. For secondary and metastatic orbital lymphoma, our strategy for treatment was beneficial by improving ocular complications and the quality of life.

[Sclerotization of orbital lymphangioma with OK-432]. / Sklerosierung orbitaler Lymphangiome mit OK-432.

Orbital lymphangiomas are mostly congenital, apparent vascular space-occupying lesions, which can lead to disfiguring swelling of the periorbital soft tissues, ocular motility disorders, optic nerve compression and keratopathy. The treatment is challenging because the disease is principally incurable. Lymphangiomatous tissue can be surgically partially reduced or treated by intralesional injection of various sclerosants. In this review we report the successful use of OK-432 for destruction of a macrocystic orbital lymphangioma.

[Periorbital liposarcoma in pediatric patient: a case report]. / Lipossarcoma periorbital em paciente pediátrico: relato de caso.

The purpose of this study is report a child with periorbital liposarcoma describing the clinical, epidemiological and therapeutic aspects. Six-months-old female baby with increasing tumor in the right fronto-zigomatic region wich was submitted to excision and the patologic and immunohistochemistry analisys observed typical findings of lipoblastoma. After that, there were three tumors relapse with the same diagnosis. One year after the last surgery there was a recurrence of the tumor but at this time the diagnosis was lipossarcoma and the patient was referred for additional treatment wilth radiotherapy and chemotherapy no new injuries so far Due to its rarity, liposarcoma usually does not enter the differencial diagnosis in the patients with orbital masses, however because of its local aggressiveess, it's vital the early identification and treatment to provide better therapeutic results and quality of life.

Combined surgical and sclerotherapy for an extensive venous-lymphatic orbital anomaly.

Combined venous-lymphatic anomalies (lymphangiomas) of the orbit are nonhereditary, congenital lesions. We report the case of a 6-year-old boy with an extensive right orbital venous-lymphatic anomaly and severe facial deformity who developed 3 intraconal hemorrhages over 3 months. Although the visual acuity was recovered in the first two surgical interventions, the last episode of bleeding resulted in permanent visual acuity loss. At that stage, adjunctive therapy with n-butyl-cyanoacrylate allowed for greater surgical excision. No further episodes of recurrent hemorrhage occurred and the exophthalmos was corrected, thereby improving his overall cosmesis.