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Journal of the College of Physicians... May 2021Orbital teratoma is a rare tumour that is composed of tissues derived from all three germ cell layers. It presents, in otherwise, healthy neonates with unilateral...
Orbital teratoma is a rare tumour that is composed of tissues derived from all three germ cell layers. It presents, in otherwise, healthy neonates with unilateral proptosis that progresses rapidly, threatening vision through optic nerve compromise or exposure keratopathy. Globe conservation may be difficult too in advanced cases. Mature teratomas are benign, but the immature ones can sometimes become malignant. We report a case of a six-month female infant who presented with mature orbital teratoma since birth. Vision could not be salvaged; but following complete surgical excision, good cosmesis was achieved. Key Words: Mature teratoma, Orbit, Congenital tumour, Surger.
Topics: Dermoid Cyst; Exophthalmos; Female; Humans; Infant; Infant, Newborn; Orbit; Orbital Neoplasms; Teratoma
PubMed: 34027878
DOI: 10.29271/jcpsp.2021.05.596 -
BMJ Case Reports Jun 2022Rhabdomyosarcoma is the most common soft-tissue sarcoma in paediatric patients and may arise as a primary orbital neoplasm. Imaging studies show a unilateral solitary...
Rhabdomyosarcoma is the most common soft-tissue sarcoma in paediatric patients and may arise as a primary orbital neoplasm. Imaging studies show a unilateral solitary orbital lesion. With larger tumours, erosion of the adjacent orbital walls is a common accompanying bony change. We present an unusual case of rhabdomyosarcoma in a preschool girl with proptosis and temporal bossing. Imaging studies showed a homogeneous, well-delineated, extraconal mass of the right orbit with hyperostosis of the adjacent sphenoid bone. After excision of the mass and debulking of the sphenoid bone, histopathology showed embryonal rhabdomyosarcoma with no malignant infiltration of the adjacent bone. This is the first reported case of hyperostosis associated with rhabdomyosarcoma. Periosteal reaction is the proposed underlying mechanism.
Topics: Child; Child, Preschool; Female; Humans; Hyperostosis; Orbit; Orbital Neoplasms; Rhabdomyosarcoma; Rhabdomyosarcoma, Embryonal; Tomography, X-Ray Computed
PubMed: 35725289
DOI: 10.1136/bcr-2021-245466 -
Journal of Nepal Health Research Council Jun 2022Orbital tumors have rare incidence, still they play a significant role in terms of morbidity and mortality. Orbital tumors may be primary, secondary or metastatic. These...
BACKGROUND
Orbital tumors have rare incidence, still they play a significant role in terms of morbidity and mortality. Orbital tumors may be primary, secondary or metastatic. These consist of benign and malignant lesions with extreme variations in pediatric and adult groups. These lesions can have acute or chronic onset, slow to rapid progression with or without bony destructions leading to vision loss, deformity and sometimes death.
METHODS
This retrospective cross-sectional study was carried out in the Department of Ophthalmic Pathology and Laboratory Medicine in Biratnagar Eye Hospital. Fifty-one patients who underwent histopathological evaluation for their orbital lesions from June 2018 to December 2019 were included in the study.
RESULTS
Orbital tumor and tumor like lesions comprised 27 cases (52.94%) in adults and 24 (47.06%) in paediatrics. Histopathologically, the most common pediatric benign tumor was dermoid cyst and malignant was secondary to orbital extension of retinoblastoma. Also, the most common adult benign orbital tumor was cavernous hemangioma and malignant was non Hodgkins lymphoma. The clinico-pathological accuracy for diagnosis was 68.63%. Association between age groups and nature of orbital lesions and between clinical and histopathological diagnosis was found to be statistically significant (p<0.05).
CONCLUSIONS
Orbital tumors and tumor like lesions are uncommonly encountered. These masses showed significant variation in incidence in children versus adults. Combined efforts by different specialties help in early and prompt management of the orbital tumors.
Topics: Adult; Child; Cross-Sectional Studies; Hospitals; Humans; Nepal; Orbital Neoplasms; Retrospective Studies
PubMed: 35945849
DOI: 10.33314/jnhrc.v20i01.3727 -
International Ophthalmology Jun 2023Orbital lymphoma is one of the most common adult orbital malignancies, accounting for approximately 10% of all orbital tumors. This study aimed to analyze the effects of...
OBJECTIVES
Orbital lymphoma is one of the most common adult orbital malignancies, accounting for approximately 10% of all orbital tumors. This study aimed to analyze the effects of surgical resection and orbital iodine-125 brachytherapy implantation for orbital lymphoma.
PATIENTS AND METHODS
This was a retrospective study. Clinical data of 10 patients were collected from October 2016 to November 2018 and followed up to March 2022. Patients underwent the primary surgery for maximal safe removal of the tumor. After a pathologic diagnosis of a primary orbital lymphoma was established, iodine-125 seed tubes were designed based on the tumor size and invasion range, and direct vision was placed into the nasolacrimal canal or/and under the orbital periosteum around the resection cavity during the secondary surgery. Then, follow-up data, including the general situation, ocular condition, and tumor recurrence, were recorded.
RESULTS
Of the 10 patients, the pathologic diagnoses included extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (6 cases), small lymphocytic lymphoma (1 case), mantle cell lymphoma (2 cases), and diffuse large B-cell lymphoma (1 case). The number of seeds implanted ranged from 16 to 40. The follow-up period ranged between 40 and 65 months. All patients in this study were alive and well had tumors that were completely controlled. No tumor recurrences or metastases occurred. Three patients had dry eye syndrome and two patients had abnormal facial sensation. No patient had radiodermatitis involving the skin around the eye, and no patient had radiation-related ophthalmopathy.
CONCLUSIONS
Based on preliminary observations, iodine-125 brachytherapy implantation appeared to be a reasonable alternative to external irradiation for orbital lymphoma.
Topics: Adult; Humans; Orbital Neoplasms; Brachytherapy; Retrospective Studies; Neoplasm Recurrence, Local; Lymphoma, B-Cell, Marginal Zone
PubMed: 36906873
DOI: 10.1007/s10792-022-02594-x -
Survey of Ophthalmology 2015Orbital lymphaticovenous malformations consist of abnormal vascular channels lined by endothelial cells with a spectrum from venous to lymphatic characteristics. They... (Review)
Review
Orbital lymphaticovenous malformations consist of abnormal vascular channels lined by endothelial cells with a spectrum from venous to lymphatic characteristics. They may be venous-dominant or lymphatic-dominant. These lesions continue to present management challenges. Total excision or obliteration is not always achievable, recrudescence is common, and interventions carry a risk of damaging normal structures. Patients likely benefit most from a multidisciplinary approach, including both surgical and nonsurgical (e.g., sclerosants and liquid polymers) therapeutic modalities. Targeted biologic therapy would be ideal; nevertheless, this goal is complicated by the heterogeneous venous-lymphatic and stromal characteristics of these lesions. Ideally, antiangiogenic agents targeting both lymphatic and blood vascular endothelial cells will be developed to treat these lesions and reduce their regrowth. Further studies are warranted to enhance our understanding of these orbital lesions with regard to their angiogenic (proliferative) activities and profiles of marker expression, with a goal to produce effective medical therapies.
Topics: Cerebral Veins; Humans; Lymphangioma; Lymphatic Vessels; Magnetic Resonance Imaging; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 26077629
DOI: 10.1016/j.survophthal.2015.03.001 -
Asia-Pacific Journal of Ophthalmology... 2015Lymphoproliferative diseases of the orbit account for majority of orbital tumors. The pathologies range from reactive lymphoid hyperplasia to specific IgG4-related... (Review)
Review
Lymphoproliferative diseases of the orbit account for majority of orbital tumors. The pathologies range from reactive lymphoid hyperplasia to specific IgG4-related inflammation to malignant lymphomas. This review summarizes current concepts regarding pathology, clinical presentation, diagnosis, staging, and treatment strategies of major orbital lymphoproliferative diseases based on updated and relevant bibliography.
Topics: Antineoplastic Agents; Autoimmune Diseases; Humans; Immunoglobulin G; Lymphoma; Lymphoproliferative Disorders; Neoplasm Staging; Orbital Neoplasms; Radiotherapy
PubMed: 26065355
DOI: 10.1097/APO.0000000000000102 -
Asia-Pacific Journal of Ophthalmology... 2017The aim of this review article is to provide an update of the current literature on orbital tumors. The authors conducted a PubMed literature search of English language... (Review)
Review
The aim of this review article is to provide an update of the current literature on orbital tumors. The authors conducted a PubMed literature search of English language articles published between January 2014 and December 2016 using the following search items: orbit, tumors, lacrimal gland, lymphoma, hemangioma, lymphangioma. The authors included reviews, original articles, case series, and case reports with relevant new information. There is new information about the clinical spectrum of orbital tumors, capillary hemangioma, cavernous hemangioma, lymphangioma, orbital venous malformation, lacrimal gland tumors, and orbital lymphoma. This review highlights the current understanding, practice, and guidelines in the diagnosis and management of common tumors of the orbit.
Topics: Global Health; Humans; Morbidity; Neoplasm Staging; Ophthalmology; Orbital Neoplasms; Periodicals as Topic
PubMed: 28399335
DOI: 10.22608/APO.201736 -
Ophthalmic Plastic and Reconstructive...To study the multidisciplinary management and survival outcomes of orbital metastasis (OM).
PURPOSE
To study the multidisciplinary management and survival outcomes of orbital metastasis (OM).
METHODS
All patients with a diagnosis of OM treated during 1999-2019 were included. Clinical data were retrospectively collected and analyzed.
RESULTS
The study included 118 patients, 71 females and 47 males, with a median age of 61 years. The most common primary tumor types were breast carcinoma (43 patients), melanoma (17), and lung (13), thyroid (7), renal cell (6), and neuroendocrine carcinoma (6). Ninety-six patients had a known history of cancer at OM diagnosis. The median time from diagnosis of primary cancer to OM was 31 months (range, 0-304). In 22 patients, OM was the first sign of cancer. In 47 patients, the orbit was the only site of metastasis. The most common presenting features were restricted by extraocular motility (77 patients) and proptosis (61). Eight patients had enophthalmos. OM was diagnosed based on clinical history and imaging studies in 81 patients and orbital biopsy in 37. One hundred nine patients were treated with chemotherapy and immunotherapy, 75 with radiation, and 21 with palliative surgical resection. Eighty-two patients died during follow up. The median overall survival (OS) time after diagnosis of OM was 17 months (95% CI: 12-28). OM from renal cell carcinoma was associated with the best and OM from thyroid cancer with the worst OS. Patients with breast cancer had longer median survival (28 months; 95% CI: 15-60) than patients with lung, melanoma, neuroendocrine, or thyroid cancer.
CONCLUSION
In this large series, breast cancer and melanoma were the most common causes of OM. Most patients had a known history of cancer at OM diagnosis and did not require orbital biopsy to confirm the diagnosis. Patients with renal cell carcinoma and breast carcinoma had the best prognosis after diagnosis of OM.
Topics: Breast Neoplasms; Female; Humans; Male; Melanoma; Middle Aged; Orbital Neoplasms; Prognosis; Retrospective Studies
PubMed: 33587417
DOI: 10.1097/IOP.0000000000001939 -
Archivos de La Sociedad Espanola de... Feb 2022Orbital metastases are an uncommon condition. They may be the clinical presentation of a previously unknown malignancy. Depending on the series, the rate of orbital...
BACKGROUND AND OBJECTIVE
Orbital metastases are an uncommon condition. They may be the clinical presentation of a previously unknown malignancy. Depending on the series, the rate of orbital metastasis as a first manifestation of a malignant tumour is 20%-42%. The clinical presentation and survival is presented in a series of 11 cases of orbital metastasis corresponding to 10 patients.
MATERIAL AND METHODS
Descriptive retrospective study of a series of 10 adult patients diagnosed with orbital metastasis from solid tumours during a 9-year period. Metastasis involving the orbit was included, and lymphomas and contiguity invasions from adjacent structures were excluded. A note was made on whether the clinical picture was the first sign of tumour onset. Signs and symptoms at clinical debut were registered, as were primary tumour location, distance seeding, orbital structures involved, and survival time since the diagnosis was established.
RESULTS
One-half (50%) of the patients were women. Mean age at diagnosis was 60.9 years (range 42-82). In nine cases (90%), the metastasis was unilateral, while in the remaining one the involvement was bilateral. The most frequent primary tumour location was the breast (36% of the cases); followed by the bladder (27%), lung (18%), and ovary and cavum (9%). Seventy percent of the patients had a previously diagnosed neoplasm; in 3 cases the metastasis was the first malignancy manifestation. Most frequent symptoms were diplopia (60%), visual impairment (40%), and pain (30%). The most common signs were resistance to ocular retropulsion (60%), presence of a mass on orbital palpation, and ocular dystopia (50%), and bulbar hyperaemia and proptosis (40%). The most employed management modality was clinical observation (5 patients). In 3 patients radiotherapy was administered, combining chemotherapy plus hormonal therapy in one case, and orbital exenteration in another one. In 2 cases, chemotherapy was administered as an isolated regime. Median survival time was 4.8 months since the diagnosis. There was a statistically significant difference between the survival time in the observation group (median 2.5 months) and in the active treatment group (median 29.2 months), p=.034.
CONCLUSIONS
In the series presented, 27% of the cases established the clinical debut of the malignant neoplasm. The ophthalmologist plays an essential role when this condition is suspected, diagnosing it, and proposing its management together with the Oncology Service.
Topics: Adult; Aged; Aged, 80 and over; Exophthalmos; Female; Humans; Middle Aged; Orbit; Orbit Evisceration; Orbital Neoplasms; Retrospective Studies
PubMed: 35152953
DOI: 10.1016/j.oftale.2020.07.014 -
Maxillary Ameloblastoma with Orbital Involvement: An Institutional Experience and Literature Review.Ophthalmic Plastic and Reconstructive... 2016To describe 8 patients with orbital involvement by ameloblastoma and to review the literature on this topic. (Review)
Review
PURPOSE
To describe 8 patients with orbital involvement by ameloblastoma and to review the literature on this topic.
METHODS
The electronic medical records and pathology databases of the Hospital of the University of Pennsylvania were searched to identify all patients with histopathologically confirmed ameloblastoma diagnosed between 1990 and 2015. PubMed database was searched for all well-documented cases of maxillary ameloblastoma and ameloblastic carcinoma ex-ameloblastoma with orbital involvement published in the English literature. The information collected on the compiled 23 patients included age, sex, clinical presentation, imaging findings, management, tumor histopathologic features, and follow up.
RESULTS
Review of medical records identified 8 patients with orbital involvement by ameloblastoma. Literature search yielded 15 patients with well-documented orbital involvement by ameloblastoma. Most tumors occurred in men (19 of 23, M:F = 4-5:1) with an average age of 56 years. The overall rates of recurrence, visual compromise, death, and confirmed disease-related mortality were 70% (16/23), 26% (6/23), 39% (9/23), and 22% (5/23), respectively. The initial surgical approach correlated with prognosis. The rates of recurrence, orbital exenteration, and mortality in the cohort managed with conservative surgery or partial maxillectomy were 57% (8/14), 29% (4/14), and 50% (7/14), respectively. In contrast, the patients initially managed with a radical resection had substantially lower frequencies of tumor recurrence (2/7, 29%), exenteration (1/7, 14%), and death (1/7, 14%). Malignant transformation to ameloblastic carcinoma occurred in the setting of recurrent disease in 3 patients and in 1 patient with prolonged duration of symptoms, suggestive of a long-standing tumor.
CONCLUSIONS
Maxillary ameloblastoma can rarely involve the orbit, leading to significant ocular morbidity and occasional mortality. Prompt radical resection of the tumor has the potential to decrease the likelihood of recurrence and visual compromise, and can improve survival.
Topics: Ameloblastoma; Humans; Maxillary Neoplasms; Neoplasm Invasiveness; Orbit; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 26505234
DOI: 10.1097/IOP.0000000000000580