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.

Sirolimus-induced regression of a large orbital lymphangioma.

Microcystic lymphatic malformations are difficult to treat surgically, especially when located in the orbital apex. Recently, pharmacologic inhibition of the mTOR pathway by sirolimus was reported as a safe and efficacious treatment option for lymphatic malformations (also known as lymphangiomas). We report the case of a young male patient in which a unilateral, retrobulbar lymphatic malformation regressed to a large extent under treatment with 1 mg sirolimus given orally twice a day over a period of six months.

Low-Grade Malignant Fibrous Histiocytoma Originating From the Medial Rectus Muscle.

PURPOSE: Fibrous histiocytomas (FHs) involving the orbit are uncommon. To our knowledge, here the authors report the first patient with an orbital low-grade malignant FH originating from the medial rectus muscle. METHODS: A clinical report relating clinical features as well as imaging and histopathologic findings is presented. RESULTS: A 26-year-old female presented with a left eye proptosis that had been present for 4 months. A small left exotropia was observed and ocular motility evaluation revealed a -3 adduction and -1 abduction of the left eye. B-mode ultrasonic scans demonstrated a 2.5 × 2.0 cm mass with medium internal echo and a clear boundary located in the nasal region of the left orbit. Magnetic resonance imaging (MRI) showed a 2.8 × 2.0 cm oval extraconal orbital mass, with isointensity on T1-weighted MRI and hyperintensity on T2-weighted MRI. An anterior orbitotomy was performed and the tumor was completely removed. The mass originated from the medial rectus muscle. Histopathologic evaluation confirmed a low-grade malignant FH. After surgery, the left exotropia was no longer present. Ocular motility revealed a -1 adduction and -1 abduction of the left eye. No recurrence was evident at 1 year after surgery. CONCLUSIONS: This rare patient demonstrates that FH may originate from the medial rectus muscle. With careful surgical dissection, a near normal function of the involved muscle can be restored.

Juvenile psammomatoid ossifying fibroma of the orbit and paranasal sinuses. A case report.

Juvenile psammomatoid ossifying fibroma (JPOF) is an uncommon benign fibro-osseous lesion predominantly arising in the paranasal sinuses and orbits of children and young adults. We report a case of JPOF involving the paranasal sinuses and orbit in a 15-year-old boy that presented due to progressive proptosis and downward displacement of the left eye. The lesion, first described as fibrous dysplasia (FD), was totally removed surgically, and then proved to be a JPOF, by histopathology. We discuss its differential diagnosis with other fibro-osseous lesions, histopathological features, and treatment options.

Endoscopic transnasal cryo-assisted removal of an orbital cavernous hemangioma: a technical note.

The cryoprobe device is commonly used by orbital surgeons for the extraction of intraorbital lesions. Cryoprobes provide a safe mechanism to manipulate fluid-filled tumors. Such lesions can present in locations in which intraoperative neurosurgical assistance is essential. The authors describe a technique whereby removal of an orbital hemangioma was facilitated by the aid of an endoscopic, transnasal cryoprobe while standard microsurgical dissection was performed concurrently via a transconjunctival approach.

[Neurothekeoma of the orbit]. / Przypadek neurothekeoma oczodolu.

AIM: The aim of this study was to present a rare benign tumour of neural origin located in the orbit. MATERIAL AND METHODS: Authors describe case of six-year-old boy with tumour of the right orbit, upper eyelid and right zygomatic fossa. Medical history revealed gradually increasing in size, painless tumour. Before admission to the hospital computed tomography was taken to assess the size, localization of the tumour and to plan the proper treatment. RESULTS: The patient was operated on using Krönlein approach. Tumour was removed in macroscopically radical limits without need to incise the muscles responsible for eye movement. Histopathological examination revealed: Neurothekeoma, classic type. During one year follow-up there was no recurrence. CONCLUSION: Neurothekeoma may occur in young boys in eyelid and orbit. A unique localization of neurothekeoma in the orbit, close to the lacrimal gland shows that this kind of histopatological tumours may infiltrate deeper tissues. Surgical treatment of neurothekeoma is the same as in other benign tumours.

Extended Endonasal Endoscopic Complete Resection of a Solitary Intraorbital Myofibroma: A Case Report and Literature Review.

BACKGROUND: Infantile myofibromatosis is a rare benign disease of mesenchymal origin. It occurs mostly in infants but can occur in children and adults. It presents in 2 forms: solitary and multicentric. The presence of an orbital component, whether as a solitary lesion or as part of the multicentric disease, is even rarer. Surgery is required when these tumors behave aggressively and grow rapidly or when they are large enough to cause compression symptoms. Several surgical approaches have been described to resect such lesions. CASE DESCRIPTION: We present a case of a solitary intraorbital myofibroma extending into the optic canal in a 6-year-old girl that was completely resected via an extended endonasal endoscopic approach. CONCLUSIONS: This case report highlights the advantages of the extended endonasal endoscopic approach in terms of intraoperative and postoperative factors.

Multiple cavernous hemangiomas in the orbit: A case report and review of the literature.

RATIONAL: Cavernous hemangiomas are one of the most common benign primary orbital lesions. These tumors are insidious in onset, slowly progressive and present more often in middle aged women. Multiple orbital cavernous hemangiomas are extremely rare, and only a few cases have been reported in the published literature. PATIENT CONCERNS: Here, we report the diagnosis and treatment of multiple cavernous hemangiomas in the right orbit of a female patient with impaired visual acuity and proptosis of the eye for more than 10 years. DIAGNOSIS: Magnetic resonance imaging of the orbit showed a giant and irregular soft mass filling the intraconal and extraconal space of the right orbit, compressing the right optic nerve. After tumor resection, histopathological examination confirmed the diagnosis of cavernous hemangioma. INTERVENTIONS: A lateral orbitotomy was performed and a total of 13 tumors were excised, with the largest tumor measuring approximately 2.5 × 3.0 cm. OUTCOMES: The visual acuity of the patient was preserved, with only a slightly dilated pupil of the right eye. The follow-up period was 6 months with no signs of recurrence. LESSONS: Multiple cavernous hemangiomas in the orbit is rare and should be excised surgically as soon as possible.

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.

Vulvar Melanoma with Isolated Metastasis to the Extraocular Muscles: Case Report and Brief Literature Review.

BACKGROUND/AIM: Orbital metastasis of systemic cancer is exceedingly rare. This is a case report of a patient treated for locally recurrent vulvar melanoma who later presented with unilateral proptosis and was found to have an isolated biopsy-proven extraocular muscle metastasis. PATIENTS AND METHODS: A 94-year-old female with locally recurrent vulvar melanoma presented with eye discomfort and blurry vision. Patient underwent histopathological, genetic, and imaging studies. RESULTS: All prior work-up, including brain MRI and PET/CT, was negative for disease elsewhere from local recurrence. Orbital MRI demonstrated a mass involving the extraocular muscle, and immunohistochemistry staining of biopsy was consistent with metastasis. The patient underwent radiation therapy and tolerated treatment well. CONCLUSION: This is the first reported case of vulvar melanoma with extraocular muscle metastasis. The absence of findings on imaging as part of the staging work-up underscores the importance of considering extraocular muscle (EOM) metastasis as a differential for patients with vulvar melanoma who present with proptosis.

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.

Extraconal cystic schwannoma mimicking an orbital dermoid cyst.

To our knowledge, we report the first case of an extraconal orbital cystic schwannoma in Brazilian literature. The tumor grew slowly and progressively and was associated with minimal eccentric proptosis and diplopia. The radiologic study (orbital computed tomography) revealed a rounded, encapsulated, and extraconal cystic mass in the superior medial region of the right orbit in the supraorbital and supratrochlear nerve topography. An anterior orbitotomy with full excision of the tumor was performed, and the histopathology examination revealed that the tumor was a schwannoma. After the surgery, the patient experienced total remission of his symptoms.

Unusual progression of pleomorphic adenoma of the lacrimal gland: case report.

A 72-year-old female complained of acute pain on left eye movement followed by progressive exophthalmos. Neuroimaging revealed a large well-demarcated lesion consisting of solid and cystic parts, as well as bone destruction and hemorrhage, within the left orbital cavity. The preoperative diagnosis was pleomorphic adenoma with or without malignant transformation, or cavernous angioma. En bloc excision including adjacent tissues was planned to resolve the progressive symptoms and to obtain a histological diagnosis. The transcranial route was chosen since tumor invasion to the cranial base was possible. The histological diagnosis was pleomorphic adenoma. Pathological and preoperative radiological examinations indicated that repeated intratumoral hemorrhage had caused the orbital bone destruction and acute orbital pain. Neoplasms should be differentiated from a wide spectrum of other possible pathologies. Accurate clinical diagnosis of neoplasm in the orbital cavity is important for correct therapeutic management. Malignancy is generally suspected if painful and progressive signs and symptoms are associated with an orbital mass lesion. The present case suggests that pleomorphic adenoma should also be considered in the differential diagnosis. The therapeutic strategy for lacrimal gland tumors remains controversial, so a flexible management approach is required.

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.

Frontal intraosseous cryptic hemangioma presenting with supraorbital neuralgia.

Primary intraosseous cranial hemangiomas are rare benign tumors comprising 0.2% of all osseous neoplasms. Symptomatic cranial cryptic hemangiomas are extremely rare. We report the case of a 43-year-old man with a cryptic hemangioma of the superior orbital rim. Radiological investigations revealed it to be an intraosseous cryptic mass which was totally excised and the supraorbital nerve was decompressed, relieving the patient of his symptoms. Histopathology showed features of an intraosseous hemangioma.

Ultrasound findings in a patient with primary orbital hemangiopericytoma.

A 20-year-old white male with a left superorbital mass and significant left proptosis was referred from his primary care physician. A and B scan ultrasound examination revealed a mass in the superonasal quadrant of the left orbit that demonstrated compression, encapsulation, internal vascularity and low internal reflectivity. Diagnosis of orbital hemangiopericytoma was confirmed histologically from frozen sections taken during surgical excision of the tumor.

[Intracranial and intraorbital meningioma ]. / Meningioame intraorbitare si intracraniene--tema de granita neurooftalmologica.

UNLABELLED: AIM OF THE ARTICLE: To discuss the simultaneous presence of intraorbitar and intracranian meningiomas, the origin and the link between these tumors. MATERIAL AND METHODS: We show 3 cases with intracranian and intraorbitar meningiomas. RESULTS: Therapeutically attitude and debates about anatomopathological results. CONCLUSIONS: Difficulty in determine the intracranian or intraorbitar origin of meningiomas.