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Journal of the Royal Society of Medicine 1985
Topics: Hemangiopericytoma; Humans; Infant, Newborn; Male; Orbital Neoplasms
PubMed: 4093942
DOI: No ID Found -
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 -
Journal of Cancer Research and... 2011Diagnosis of orbital retinoblastoma traditionally carries a dismal prognosis. Although its incidence is less in the developed countries, it continues to contribute to an... (Review)
Review
Diagnosis of orbital retinoblastoma traditionally carries a dismal prognosis. Although its incidence is less in the developed countries, it continues to contribute to an epidemic of extraocular disease at diagnosis in the developing world. Orbital retinoblastoma encompasses a wide range of distinct clinical entities with varying tumor load. There are no standard treatment protocols as of now but the current preferred management is multimodal with a combination of initial high-dose chemotherapy, surgery, external beam radiotherapy and prolonged chemotherapy for 12 cycles. Though orbital retinoblastoma is a catastrophic event, rapid advances on many fronts, especially the genetic, makes the future appear brighter than what it is now. This review looks at all the new frontiers that are in store in the near as well as the distant future. Looking at the ever expanding horizons makes one believe of a definite hope that one day we will conquer this disease as we have conquered many others in the past.
Topics: Humans; Orbital Neoplasms; Retinal Neoplasms; Retinoblastoma
PubMed: 21546735
DOI: 10.4103/0973-1482.80429 -
World Journal of Surgical Oncology Mar 2013Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin's lymphoma. The majority of non-Hodgkin's lymphomas of the orbit are...
BACKGROUND
Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin's lymphoma. The majority of non-Hodgkin's lymphomas of the orbit are extranodal marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue type. Because of nonspecific clinical signs and symptoms, some diagnostic delay may occur. The purpose of the study was to evaluate the diagnostic approach in orbital lymphomas and to analyze their treatment outcome.
METHODS
In the period from 2005 to 2012, from a group of 135 patients with tumors of the orbit, we identified 11 patients diagnosed with orbital lymphoma. This patient cohort was reviewed retrospectively.
RESULTS
The patient group consisted of 11 patients (seven females, male males) with a median age of 57.7 years (range 42 to 88 years). Orbital swelling, pain and motility impairment were the leading clinical symptoms. Diagnosis was confirmed by surgical biopsy. Depending on the anatomic location of the tumor, a surgical biopsy was taken using a blepharoplasty incision, a lateral orbitotomy or a navigation-guided biopsy. The predominant histology was extranodal non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue type (82%). All patients underwent complete clinical staging. These were clinical stage IEA in seven patients, and stages IIEA (n = 2) and IIIEA (n = 2) in four patients . Patients in stage IEA were treated with radiation therapy alone, with radiation doses between 25 and 40 Gy, and patients with stage IIEA received systemic chemotherapy with bendamustin/rituximab. Those two patients diagnosed with diffuse large B-cell lymphoma and mantle cell lymphoma received systemic chemotherapy according to the R-CHOP protocol.
CONCLUSIONS
Owing to unspecific clinical symptoms, some diagnostic delay may occur in orbital lymphoma. If unspecific orbital symptoms are present, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis. Once diagnosis is established and staging is complete, radiation therapy is the recommended treatment for stage IEA patients. Systemic chemotherapy is indicated in selected stage IIEA patients and in patients with stage IIIEA disease.
Topics: Adult; Aged; Aged, 80 and over; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Lymphoma; Lymphoma, B-Cell, Marginal Zone; Lymphoma, Non-Hodgkin; Male; Middle Aged; Neoplasm Staging; Orbital Neoplasms; Prognosis; Retrospective Studies; Survival Rate; Tomography, X-Ray Computed
PubMed: 23506357
DOI: 10.1186/1477-7819-11-73 -
Indian Journal of Ophthalmology Apr 2014Fibrous histiocytomas (FHs) are mesenchymal tumors that may be benign or malignant. Ocular involvement by FHs is infrequent and primarily limited to the orbit. Rarely,...
Fibrous histiocytomas (FHs) are mesenchymal tumors that may be benign or malignant. Ocular involvement by FHs is infrequent and primarily limited to the orbit. Rarely, FHs can also involve the conjunctiva and perilimbal area. We report the case of a 38-year-old male with lid, conjunctival, and neck FHs. The diagnosis was confirmed by histopathology.
Topics: Adult; Biopsy; Diagnosis, Differential; Histiocytoma, Benign Fibrous; Humans; Male; Ophthalmologic Surgical Procedures; Orbit; Orbital Neoplasms
PubMed: 24817747
DOI: 10.4103/0301-4738.132104 -
The British Journal of Ophthalmology Mar 1982Seven histologically proved cases of primary orbital schwannoma have been seen at the University of British Columbia Orbital Clinic between September 1976 and December...
Seven histologically proved cases of primary orbital schwannoma have been seen at the University of British Columbia Orbital Clinic between September 1976 and December 1980. We describe here their varied clinical presentations, preoperative investigations, operative findings, and appearances on light and electron microscopy. Although no single feature is pathognomonic, a multiplicity of clinical, radiographic, and surgical features point to this lesion. Of preoperative investigations the computed tomography scan was the most helpful, especially in localising the lesion. Of the 7, 4 were intraconal and 3 were extraconal. The surgical approach was dictated by tumour site and included anterior, lateral, and panoramic orbitotomies. At surgery the nerve of origin of 4 of the tumours was identified. All tumours were excised totally or subtotally. There has been no recurrence to date.
Topics: Adult; Female; Humans; Male; Microscopy, Electron; Middle Aged; Neurilemmoma; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 7066273
DOI: 10.1136/bjo.66.3.194 -
F1000Research 2023Introduction Orbital lipoma is an extremely rare tumor, representing less than 1% of all orbital tumors. We review the literature and describe the presentation, the...
Introduction Orbital lipoma is an extremely rare tumor, representing less than 1% of all orbital tumors. We review the literature and describe the presentation, the differential diagnosis and the management of this tumor. Case report We report the case of a 63-year-old patient who was referred for a diplopia with recent hemi-cranial headache. Physical examination showed no exophthalmos nor decrease in visual acuity. The patient complained of diplopia on elevation and oculomotricity examination showed limited elevation of the right eye. The Hess Lancaster test was in favor of a limited course of the right inferior rectus muscle. Magnetic resonance imaging revealed a fusiform tissue process in the right inferior rectus muscle with a fatty signal. A complete excision of the tumor was performed by a trasncunjonctival approach. Cytopathological examination was consistent with a pleomorphic lipoma. The postoperative period was uneventful. The definitive histopathologic diagnosis was a lipoma. The postoperative Magnetic resonance imaging showed the complete disappearance of the lesion. With 3 years of follow up, there is no sign of recurrence or ocular motility trouble. Lipomas are rare tumors in the orbit. The clinic is variable depending on the size and the site. The clinical diagnosis is difficult to make. Only histology allows the final diagnosis.
Topics: Humans; Middle Aged; Orbital Neoplasms; Magnetic Resonance Imaging; Lipoma; Male
PubMed: 38726301
DOI: 10.12688/f1000research.130056.2 -
Anales Del Sistema Sanitario de Navarra 2008Orbital metastases are a defined subgroup within ocular affection secondary to the distant spread of breast cancer. We review the published experience on the incidence... (Review)
Review
Orbital metastases are a defined subgroup within ocular affection secondary to the distant spread of breast cancer. We review the published experience on the incidence of orbit extension from this type of tumour, with reference made to our experience as medical oncologists, together with the most common clinical features and the relevant aspects for imaging and histopathological diagnoses. The therapy for orbital metastases from breast cancer is included within the systemic therapy required by the distant spread of the disease, with some clinical benefits obtained from hormone therapy, chemotherapy and monoclonal antibodies. Palliative radiation and surgery may also play an important role in providing care to these patients. Although there are some published cases with long-term survival, the prognosis for these patients is poor, and new advances in knowledge and therapy are needed for this complication due to breast cancer.
Topics: Breast Neoplasms; Female; Humans; Orbital Neoplasms
PubMed: 19169301
DOI: No ID Found -
JAMA Ophthalmology Apr 2020No previous studies to date have validated the American Joint Committee on Cancer (AJCC) 8th edition of the TNM classification for orbital sarcoma.
IMPORTANCE
No previous studies to date have validated the American Joint Committee on Cancer (AJCC) 8th edition of the TNM classification for orbital sarcoma.
OBJECTIVES
To determine the prognostic performance of the most recent TNM classification for orbital sarcoma and to identify other prognostic factors for local recurrence, lymph node metastasis, distant metastasis, and death due to disease.
DESIGN, SETTING, AND PARTICIPANTS
This single-center retrospective cohort study included 73 consecutive patients treated for orbital sarcoma from March 1, 2003, through June 30, 2018. Data were analyzed from November 1 to December 31, 2018.
MAIN OUTCOMES AND MEASURES
T and N categories at presentation and disease-related outcomes, including local recurrence, lymph node metastasis, distant metastasis (DM), and death due to disease (DD).
RESULTS
The 73 participants included 43 men (59%), and the median age was 21 (range, 0-77) years. The common histologic types were rhabdomyosarcoma (RMS) (35 [48%]), solitary fibrous tumor/hemangiopericytoma (10 [14%]), and Ewing sarcoma (8 [11%]). The most common TNM designations were T2 N0 M0 (26 [36%]) and T4 N0 M0 (24 [33%]). T category was associated with the risk of all disease-related outcomes, including local recurrence (hazard ratio [HR] for T2 vs T4, 0.22 [95% CI, 0.06-0.81]; HR for T3 vs T4, 0.59 [95% CI, 0.13-2.65]; P = .03), lymph node metastasis by the last follow-up (T1, 1 [14%]; T2, 0; T3, 0; T4, 12 [35%]; P = .001), DM (HR for T2 vs T4, 0.29 [95% CI, 0.08-1.07]; P = .04), and DD (HR of T2 vs T4, 0.16 [95% CI, 0.04-0.73]; HR of T3 vs T4, 0.30 [95% CI, 0.04-2.34]; P = .02). Higher risk of DM and higher risk of DD were associated with disease category of at least T3 (HR for DM, 3.24 [95% CI, 0.89-11.72; P = .06]; HR for DD, 6.32 [95% CI, 1.43-27.95; P = .005]), N1 disease (HR for DM, 13.33 [95% CI, 4.07-43.65; P < .001]; HR for DD, 7.07 [95% CI, 2.45-20.44; P < .001]), tumor size larger than 3 cm (HR for DM, 2.72 [95% CI, 0.92-8.05; P = .06]; HR for DD, 5.79 [95% CI, 1.85-18.14; P < .001]), and age of patient with RMS younger than 1 year or 10 years or older (HR for DM, 6.85 [95% CI, 0.83-56.53; P = .04]; HR for DD, 7.03 [95% CI, 0.85-57.83; P = .04]). Higher risk of local recurrence was associated with disease category of at least T3 (HR for
3 cm, 0.27 [95% CI, 0.09-0.77]; P = .009). Higher risk of lymph node metastasis was associated with disease category of at least T3 (odds ratio [OR], 13.33 [95% CI, 1.77-602.30]; P = .004), alveolar RMS (OR, 9.98 [95% CI, 2.13-51.55]; P = .001), and age of patient with RMS younger than 1 year or 10 years or older (OR, 9.20 [95% CI, 1.01-458.29] P = .03). CONCLUSIONS AND RELEVANCE
In patients with orbital sarcoma, T and N categories at presentation (defined by the AJCC 8th edition classification) correlate with metastasis and survival. These findings appear to support consideration of strict surveillance testing for regional nodal and systemic metastases in patients with orbital sarcoma with disease category of at least T3 and/or N1 disease.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Metastasis; Neoplasm Recurrence, Local; Neoplasm Staging; Orbital Neoplasms; Prognosis; Proportional Hazards Models; Retrospective Studies; Sarcoma; Survival Rate; United States
PubMed: 32105303
DOI: 10.1001/jamaophthalmol.2020.0039 -
BMJ Case Reports Mar 2017Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic... (Review)
Review
Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic histological biphasic pattern. Radical resection with negative margins is the mainstay of treatment; role of adjuvant chemotherapy and radiotherapy is yet not well defined. We report a rare case of 18-year-old man who was diagnosed to have orbital MC. He presented with locally advanced disease with no vision in the affected eye. He underwent right orbital exenteration; a transcranial intradural approach was used to divide the optic nerve, and the temporalis muscle flap was utilised to fill the exenterated orbit. Though optic nerve involvement is rare in orbital MCs, a transcranial approach may be used effectively to avoid traction on optic chiasma and ensure margin-free resection in case of optic nerve involvement up to orbital apex. Unfortunately, prognosis remains dismal in MCs despite treatment.
Topics: Adolescent; Chondrosarcoma, Mesenchymal; Craniotomy; Humans; Male; Orbital Neoplasms; Radiotherapy, Adjuvant; Treatment Outcome
PubMed: 28258179
DOI: 10.1136/bcr-2016-218744