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The Pan African Medical Journal 2022Masson tumor is a benign vascular lesion characterized by an intravascular papillary endothelial hyperplasia. Peri orbital locations are rare. We report a case of Masson... (Review)
Review
Masson tumor is a benign vascular lesion characterized by an intravascular papillary endothelial hyperplasia. Peri orbital locations are rare. We report a case of Masson tumor localized in the upper internal angle of the left orbit revealed by progressive ocular proptosis. Orbital computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a vascular mass in the left internal canthus mimicking an arterioveinous malformation. The patient underwent total removal of the lesion with a favorable postoperative follow up. Histological examination found an intravascular papillary endothelial hyperplasia without atypical features corresponding to Masson tumor. A thorough literature review of Masson tumor is presented with a discussion of clinical findings and management.
Topics: Humans; Hyperplasia; Magnetic Resonance Imaging; Orbital Neoplasms; Tomography, X-Ray Computed; Vascular Neoplasms
PubMed: 36160283
DOI: 10.11604/pamj.2022.42.147.29078 -
Journal of the Formosan Medical... Jun 2014Hemangiopericytoma is a very rare orbital tumor. The purpose of this study was to report the clinical and histopathological features of six cases of orbital...
BACKGROUND/PURPOSE
Hemangiopericytoma is a very rare orbital tumor. The purpose of this study was to report the clinical and histopathological features of six cases of orbital hemangiopericytoma in an Asian population.
METHODS
Clinical and histopathological features were reviewed in six patients who were histopathologically confirmed as having primary orbital hemangiopericytoma in National Taiwan University Hospital between May 2001 and December 2010.
RESULTS
Among the six cases who were diagnosed as having primary orbital hemangiopericytoma, all lesions were reported as vascular tumors and featured branching "staghorn appearance" vessels. All patients, including one male and five females, presented with progressive proptosis and some associated symptoms such as extraocular motility limitation with diplopia, displacement of the globe, afferent pupillary defect, congested vessels of conjunctiva, or decreased visual acuity. On computed tomography, the orbital tumors tended to manifest as circumscribed masses with homogeneous medium-to-high enhancement with contrast studies. All six patients received surgical treatments, and four of them had additional radiotherapy. Three patients had recurrence after surgeries, and one of them had multiple metastases to lung and liver. All patients were still alive after a follow-up period of 5-10 years.
CONCLUSION
Orbital hemangiopericytoma has malignant potential, which may lead to local recurrence and/or metastasis. Histopathological findings alone are insufficient to predict the behavior of this tumor. Therefore, both clinical and histopathological findings are important to evaluate the treatment outcomes. Total excision accompanied with radiotherapy is suggested and long-term follow-up is required.
Topics: Adult; Female; Hemangiopericytoma; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Orbital Neoplasms; Retrospective Studies
PubMed: 24820631
DOI: 10.1016/j.jfma.2012.08.004 -
Eye (London, England) Apr 2016To retrospectively assess the clinicopathological characteristics of orbital granular cell tumours (GCTs). (Review)
Review
OBJECTIVE
To retrospectively assess the clinicopathological characteristics of orbital granular cell tumours (GCTs).
METHODS
A non-comparative review of the clinical characteristics, imaging, histopathological features, management, and prognosis of five cases of benign GCT and one case of malignant GCT (MGCT) was conducted, along with a review of the English language literature.
RESULTS
Among the six cases, four tumours were adherent to the extraocular muscle (EOM), and three tumours to the optic nerve (ON). Morphologic examinations revealed polygonal cells containing periodic-acid-Schiff-positive eosinophilic granules. All tumours (100%) were positive for VIM and NSE, five (83.3%) tumours were positive for S-100, and three (50%) tumours were positive for CD68. The follow-up examination of the MGCT witnessed recurrence and brain metastasis despite several thorough resections, but the patient remained alive; the follow-up examination of the four benign GCTs that had received incomplete excision revealed recurrence in one patient and dramatic shrinkage of the residual tumour in another; there was no recurrence in the other two patients.
CONCLUSIONS
GCT should be considered in the differential diagnosis of orbital tumours, which may affect EOMs and ON. The natural course of GCT can include tumour progression, stability, or spontaneous regression. To avoid recurrence, complete resection is recommended for orbital GCT. To the best of our knowledge, primary orbital MGCT is reported for the first time.
Topics: Adult; Biomarkers, Tumor; Female; Granular Cell Tumor; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Neoplasms; Neoplasm Proteins; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Optic Nerve Neoplasms; Orbital Neoplasms; Prognosis; Retrospective Studies
PubMed: 26742863
DOI: 10.1038/eye.2015.268 -
BMC Ophthalmology Nov 2018To evaluate the ophthalmic manifestations and radiographic features of ectopic orbital meningioma to improve diagnostic accuracy.
BACKGROUND
To evaluate the ophthalmic manifestations and radiographic features of ectopic orbital meningioma to improve diagnostic accuracy.
METHODS
Patient data from patients admitted to our institution during a 217-month period from August 1999 to September 2017 were included. Patient ophthalmic manifestations, radiographic features (CT and MRI), diagnosis, pathology, therapeutic regimens, and prognosis were retrospectively reviewed.
RESULTS
Six patients with ectopic orbital meningioma were identified. The mean age at the first visit was 33.2 years (range, 7-56 years). All six patients displayed manifestations of exophthalmos, upper eyelid oedema, and motility impairment with a mean history of illness of 20.3 months (range 3-72 months). Optical lesions were located in the superonasal extraconal compartment (3/6, 50%), bitemporal extraconal compartment (1/6, 16.7%) and orbital intraconal compartment (2/6, 33%). Radiographic features were ill-defined, heterogeneous, enhancing soft tissue masses with extraocular muscular adhesion (6/6, 100%) and calcification (1/6, 16.7%), not adjacent to the optic nerve and not extending along the dura. Six cases were treated intraoperatively with complete surgical resection, indicating that all lesions were independent of the optic nerve and sphenoid ridge. The histopathologic classification was mostly of meningothelial cells (5/6, 83%). Immunohistochemistry revealed EMA and vimentin to have positive expression in all six cases, while two cases were calponin-positive and strongly expressed in the olfactory bulb. Postoperatively, lesions caused no visual impairment, and there were no cases of recurrence.
CONCLUSIONS
Ectopic orbital meningiomas are rare tumours that are not easily diagnosed without postoperative histopathology. This report highlights some of the distinguishing features of isolated orbital lesions, especially around the location of frontoethmoidal suture. Accompanying upper eyelid oedema and eye mobility restriction were observed to be dissimilar to other orbital tumours. In these cases, a diagnosis of ectopic orbital meningioma should be considered.
Topics: Adolescent; Adult; Biomarkers, Tumor; Child; Choristoma; Edema; Exophthalmos; Eyelid Diseases; Female; Humans; Immunoenzyme Techniques; Magnetic Resonance Imaging; Male; Meningeal Neoplasms; Meningioma; Middle Aged; Neoplasm Proteins; Orbital Neoplasms; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 30419957
DOI: 10.1186/s12886-018-0959-z -
Medicine Feb 2021Orbital schwannoma is a relatively rare orbital tumor, and calcification of the lesion is rarely found in the orbit. We report a case of orbital schwannoma which was...
RATIONALE
Orbital schwannoma is a relatively rare orbital tumor, and calcification of the lesion is rarely found in the orbit. We report a case of orbital schwannoma which was characterized by calcification in the orbital muscle cone, and was cured by intracapsular excision.
PATIENT CONCERNS
A 54-year-old female with a complaint of a mass in the left orbit during a magnetic resonance imaging examination and symptom of dizziness 6 months before, presented with painless exophthalmos and vision decline in the left eye.
DIAGNOSES
According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was orbital schwannoma, with calcification in the muscle cone.
INTERVENTIONS
The patient was treated by intracapsular excision of the left orbit. We removed the intracapsular mass and most part of the cyst wall in order to prevent orbital apex syndrome.
OUTCOMES
The diagnosis of schwannoma with calcification was confirmed finally through histological and immunohistochemical exam. The patient was followed up for 28 months and the orbital CT scan showed that there were no significant lesions found in the orbital muscle cone.
LESSONS
Understanding clinical, imaging diagnostic, and histopathological features of rare orbital schwannoma with calcification will facilitate timely diagnosis and treatment of this condition. The intracapsular excision can help in avoiding complications.
Topics: Calcinosis; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Neurilemmoma; Oculomotor Muscles; Orbital Neoplasms
PubMed: 33607842
DOI: 10.1097/MD.0000000000024801 -
Eye (London, England) Nov 2013Primary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved,...
PURPOSE
Primary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved, orbital involvement is extremely rare. We describe six patients with cranio-orbital IOH, the largest case series to date.
PATIENTS AND METHODS
Retrospective review of six patients with histologically confirmed primary IOH involving the orbit. Clinical characteristics, imaging features, approach to management, and histopathological findings are described.
RESULTS
Five patients were male with a median age of 56. Pain and diplopia were the most common presenting features. A characteristic 'honeycomb' pattern on CT imaging was demonstrated in three of the cases. Complete surgical excision was performed in all cases with presurgical embolisation carried out in one case. In all the cases, histological studies identified cavernous vascular spaces within the bony tissue. These channels were lined by single layer of cytologically normal endothelial cells.
DISCUSSION
IOCH of the cranio-orbital region is rare; in the absence of typical imaging features, the differential diagnosis includes chondroma, chondrosarcoma, bony metastasis, and lymphoma. Surgical excision may be necessary to exclude more sinister pathology. Intraoperative haemorrhage can be severe and may be reduced by preoperative embolisation.
Topics: Adult; Female; Hemangioma, Cavernous; Humans; Male; Middle Aged; Orbital Neoplasms; Retrospective Studies; Skull; Skull Neoplasms; Spine; Vascular Malformations
PubMed: 23989119
DOI: 10.1038/eye.2013.162 -
Journal of Epidemiology and Global... Mar 2019Orbital lesions vary in their classification, incidence, and presentation depending on the age and geographic distribution. Such lesions in the pediatric age group have...
Orbital lesions vary in their classification, incidence, and presentation depending on the age and geographic distribution. Such lesions in the pediatric age group have been studied extensively because of the possibility of faster progression of orbital involvement and the higher risk of morbidity in this age group in which vision is still developing. In Saudi Arabia, orbital lesions were studied over a 6-year period in the late 1980s, when retinoblastoma cases used to present late with orbital involvement. In this study, we revisited the same topic 20 years later aiming to find out the most recent prevalence of orbital lesions in a similar population of patients over a longer period (14 years) in the same eye center, and compare the current results to other reports worldwide. A total of 107 lesions from 106 patients were identified by tissue diagnosis, of which more than half of the lesions were benign cystic (being the most common), vasculogenic, and inflammatory in 63% [95% confidence interval (CI, 53.3-72.0)] of all biopsied lesions. Neoplasms accounted for 37% [95% CI (28.0-45.8)] with rhabdomyosarcoma being the most common, accounting for about one third of neoplasms, and no orbital cases of retinoblastoma were found. Our results demonstrated different distribution of orbital lesions in recent years reflecting the indirect effect of the improved health awareness and medical care in Saudi Arabia. This baseline demographic study is expected to be helpful for further clinical and prognostic studies with emphasis on pediatric orbital malignant lesions, their clinical presentation, management, and prognosis.
Topics: Adolescent; Age Factors; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Orbital Diseases; Orbital Neoplasms; Retinal Neoplasms; Retinoblastoma; Rhabdomyosarcoma; Saudi Arabia; Tertiary Care Centers
PubMed: 30932383
DOI: 10.2991/jegh.k.181224.001 -
Medicine Aug 2023Microcystic adnexal carcinoma (MAC), a rare and low-grade malignant skin tumor, is characterized by a high rate of misdiagnosis and a preponderance for local recurrence,... (Review)
Review
Microcystic adnexal carcinoma (MAC), a rare and low-grade malignant skin tumor, is characterized by a high rate of misdiagnosis and a preponderance for local recurrence, but seldom seen nodal or distant metastasis. Although MAC typically occurs almost in the head and neck region, primary eyelid or orbital MAC is very rare. To explore the unique characteristics of the eyelid and orbital MAC, we reviewed the relevant literature. Based on its distinctive anatomical location and the aggressive behavior, eyelid or orbital MAC not only exhibit a high rate of misdiagnosis and local recurrence, but also lead to serious complications such as disfigurement after orbital exenteration, paranasal sinuses or intracranial invasion, even death. Misdiagnosis of MAC commonly result from its rarity and nonspecific clinical and histopathological presentation. To reduce or avoid misdiagnosis, it is important to increase awareness for MAC and obtain a full-thickness biopsy specimen in histopathological analysis. Due to its extensive invasive growth pattern, MAC has a high rate of local recurrence, so complete excision with clear margins and long-term follow-up of patients with MAC are necessary. About those serious complications of the eyelid and orbital MAC, early and accurate diagnosis, complete excision is very important. Moreover, an interprofessional team consisting of ophthalmologist, otolaryngologist, neurologist, dermatologist, pathologist, radiologist is needed to evaluate and treat this disease. In summary, increasing awareness, early and accurate diagnosis, complete excision, long-term follow-up, and a multidisciplinary team is crucial for management of the eyelid and orbital MAC.
Topics: Humans; Orbit; Orbital Neoplasms; Skin Neoplasms; Eyelids
PubMed: 37565854
DOI: 10.1097/MD.0000000000034709 -
BMC Cancer Aug 2019To report the clinical experience of eye sparing surgery (ESS) and adjuvant carbon-ion or proton radiotherapy (CIRT or PRT) for orbital malignancies.
BACKGROUND
To report the clinical experience of eye sparing surgery (ESS) and adjuvant carbon-ion or proton radiotherapy (CIRT or PRT) for orbital malignancies.
METHODS
An analysis of the retrospective data registry from the Shanghai Proton and Heavy Ion Center for patients with orbital tumors was conducted. The 2-year local progression-free, regional recurrence-free, distant metastasis-free, progression-free, and overall survival (LPFS, RRFS, DMFS, PFS, OS) rates as well as associated prognostic indicators were analyzed. Radiotherapy-induced acute and late toxicities were summarized.
RESULTS
Between 7/2014 to 5/2018, 22 patients with orbital malignancies of various pathologies received ESS followed by CIRT (18), PRT (1), or PRT + CIRT boost (3). With a median follow-up of 20.25 (range 3.8-38.8) months, the 2-year OS, PFS, LPFS, RRFS, and DMFS rates were 100, 57.9, 92.9, 93.3, and 72.8%, respectively. No acute severe (i.e., ≥grade 3) toxicity was observed. Two patients experienced severe visual impairment as late toxicities.
CONCLUSION
With few observed acute and late toxicities, particle radiotherapy following ESS provided effective local control with infrequent severe toxicities for patients with orbital malignancies.
Topics: Adolescent; Adult; Aged; Biopsy; Combined Modality Therapy; Female; Heavy Ion Radiotherapy; Humans; Male; Middle Aged; Multimodal Imaging; Orbital Neoplasms; Organ Sparing Treatments; Prognosis; Proton Therapy; Radiotherapy, Adjuvant; Survival Analysis; Symptom Assessment; Treatment Outcome; Young Adult
PubMed: 31387548
DOI: 10.1186/s12885-019-5964-y -
The Veterinary Clinics of North... Dec 1998This article reviews options in dealing with tumors of the cornea and conjunctiva, nictitating membrane neoplasms, and eyelid neoplasms. Orbital tumors, optic nerve and... (Review)
Review
This article reviews options in dealing with tumors of the cornea and conjunctiva, nictitating membrane neoplasms, and eyelid neoplasms. Orbital tumors, optic nerve and other intraocular tumors are also discussed.
Topics: Animals; Eye Neoplasms; Horse Diseases; Horses; Orbital Neoplasms
PubMed: 9891726
DOI: 10.1016/s0749-0739(17)30188-8