Mesenchymal chondrosarcoma of the orbit: A case report with 5 years of follow-up

Mesenchymal chondrosarcoma is a rare orbital tumor. Several case reports of this rare tumor have been published in the literature but only 6 cases have documented a follow up of 5 years or more. We report a case of 28 year-old female who presented with left orbital mass. Computed Tomography (CT) revealed a lobulated mass in the superior extraconal space with dense intralesional calcification. Patient underwent complete resection of the mass and histopathology was suggestive of mesenchymal chondrosarcoma. He was given adjuvant radiotherapy and there was no recurrence or metastasis at 5 years of follow-up. The case highlights that a complete resection with adjuvant radiotherapy in cases of orbital mesenchymal chondrosarcoma offers excellent prognosis.     

Acute Dacryocystitis Causing Orbital Cellulitis and Abscess

Purpose: Acute dacryocystitis commonly presents as preseptal cellulitis as lacrimal sac lies anterior to the orbital septum and hence infection localizes in the preseptal space. The purpose of this study is to describe presentation and management of cases of acute dacryocystitis presenting as orbital cellilitis and abscess secondary to acute dacryocystitis. Method: This retrospective, non-comparative, interventional case series included 6 patients presenting with orbital cellulitis and abscess secondary to acute dacryocystitis. All patients underwent drainage of abscess under general anesthesia. External dcaryocystorhinostomy was advised as the definitive treatment for all pateints. Result: Abscess was localized in the extraconal space in 4 patients and in the intraconal space in 2 patients. Patients with intraconal abscess had visual loss on presentation while vision could be preserved in patients with abscess in extraconal space. Both patients with intraconal abscess had history of acute dacryocystitis. Conclusion: Acute dacryocystitis is an ophthalmic emergency that can cause orbital cellulitis with abscess formation, and even vision loss, if left untreated.     

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma: comparison of long-term outcome between radiotherapy and chemotherapy

AIM: To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), and to compare the treatment outcome between postoperative radiotherapy (RT) and chemotherapy in a retrospective analysis nearly 20y. METHODS: A retrospective cohort study of 56 patients with orbital NRSTS were reviewed, 34 of whom received postoperative RT, and 22 received postoperative chemotherapy. The clinicopathological features, local recurrence, metastases, and survival data were recorded. Survival analysis was performed using the Kaplan-Meier method. RESULTS: During follow-up (111.8mo, ranged 8-233mo) for 56 patients, 19 patients of them developed local recurrence, and 7 patients developed distant metastases. Fifteen patients died during follow-up period. Overall survival rates considering the whole study group was 78.57% at 5y, and 72.16% at 10y after the initial diagnosis. Compared with chemotherapy, RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy, 0.263, 95% confidence interval (CI), 0.095-0.728, P=0.0015]; with lower risk of distant metastasis (hazard ratio for RT vs chemotherapy, 0.073, 95%CI, 0.015-0.364, P=0.0014); and with lower risk of death from disease (hazard ratio for RT vs chemotherapy, 0.066, 95%CI, 0.022-0.200, P<0.0001). The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group. CONCLUSION: In patients with orbital NRSTS, postoperative RT provides better control of local recurrence, distant metastasis, and death from disease than chemotherapy. RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.     

Relationship between dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and paranasal sinusitis

AIM: To determine the clinical features of the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and its relationship with paranasal sinusitis. METHODS: A retrospective analysis of 46 patients who received surgical treatment at the Department of Ophthalmology, Beijing Tongren Hospital, Capital Medical University for the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor from October 2010 to December 2012. Each patient underwent magnetic resonance imaging (MRI) of the orbits and the 4 paranasal sinuses. Disease status and the level of serum immunoglobulin G4 (IgG4) was measured before and 6mo after surgery. RESULTS: The initial clinical feature of the idiopathic dacryoadenitis type of orbital inflammatory pseudotumor was redness or swelling of the eyelids. Masses were palpated in the area of the lacrimal gland in some patients. Of the 46 patients, 16 also suffered from sinusitis (34.8%), with 14 cases of ethmoid sinusitis, 8 cases of maxillary sinusitis, 9 cases of sphenoid sinusitis, and 8 cases of frontal sinusitis. Of the 16 patients with sinusitis, 4 patients had a medical history of rhinitis (range: 10mo to 15y previously), 10 patients had occasional nasal congestion, and 2 patients had no nasal congestion. Thirteen of the 46 patients had elevated serum IgG4 levels. Nine of these 13 patients had MRI signs of sinusitis. All patients (n=46) received oral glucocorticoid treatment for approximately 3mo after surgery. No sign of recurrence was found in the orbital MRI 6mo after surgery. Of the 16 patients with sinusitis, 9 cases of elevated serum IgG4 levels improved after treatment with decreased serum IgG4 level and 7 cases of normal serum IgG4 levels remained unchanged. CONCLUSION: Some patients with the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor may also suffer from paranasal sinusitis. The incidence of paranasal sinusitis was much higher in patients with IgG4-elevated dacryoadenitis subtype orbital inflammatory pseudotumor than in those with normal IgG4 levels. Dacryoadenitis subtype orbital inflammatory pseudotumor and paranasal sinusitis may both the clinical manifestations of IgG4-related disease involved in different locations.     

Primary orbital schwannomas.

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.     

Ophthalmic manifestations of multiple myeloma

PURPOSE: To describe ophthalmic manifestations in a series of patients with multiple myeloma (MM) and review the literature. DESIGN: Retrospective case series. METHODS: Review of all cases with ophthalmic manifestations of MM seen at the Department of Ophthalmology, Royal Adelaide Hospital, between 1987 and 2002. RESULTS: Of all patients with MM who were managed at the Royal Adelaide Hospital in the study period, only 8 were referred to the Ophthalmology Department with ophthalmic manifestations. Five males and 3 females with a mean age of 61.25 years (range 42-78) who exhibited ophthalmic manifestations were studied. Six patients (75%) had known MM at the time of their ophthalmic evaluation. Four patients (50%) had neuro-ophthalmic symptoms resulting in diplopia or visual disturbances. Three patients (37.5%) presented with orbital involvement and 1 (12.5%) with hyperviscosity retinopathy. Five patients (62.5%) died within 2 months of their ophthalmic presentation. CONCLUSION: Ophthalmic manifestations of MM are uncommon and diverse. They may appear at the initial presentation of the disease or occur late in the disease process. Awareness of the possible manifestations may lead to an earlier diagnosis and have a positive influence on the disease course.     

Orbital Compartment Syndrome: The Ophthalmic Surgical Emergency

Orbital compartment syndrome is an uncommon, ophthalmic surgical emergency characterized by an acute rise in orbital pressure. When intraorbital tension rises, damage to ocular and other intraorbital structures, including irreversible blindness, may occur if not promptly treated. The diagnosis of orbital compartment syndrome is completely clinical and early recognition and emergent orbital decompression (even prior to imaging) is essential in preventing permanent vision loss. Lateral canthotomy and inferior cantholysis remain the mainstays of management. More extensive incision of the orbital septum and orbital bony decompression may be necessary in unresponsive cases. This review discusses the various etiologies and mechanisms resulting in orbital compartment syndrome, clinical features, imaging findings, treatment, and prognosis.     

Association of HLA antigen BW35 with severe Graves' ophthalmopathy

Human leukocyte antigens (HLA) in patients with Graves' disease were investigated according to the ophthalmic classification system of the American Thyroid Association. All prior HLA studies of Graves' disease have disregarded the patients' specific ophthalmic manifestations. Examination of 18 A and 34 B loci antigens disclosed an increased frequency (corrected P = 0.002, relative risk = 13.1) of HLA-BW35 in patients improving with oral corticosteroids who have severe extraocular muscle and orbital inflammation (class 4-5) when compared to a geographically and racially matched control population. No statistically significant associations were found when patients without ophthalmic manifestations or with other categories of Graves' ophthalmopathy were compared to controls. The association of severe Graves' ophthalmopathy with HLA-BW35 may provide immunologic evidence to explain both the unpredictable association of the orbital and thyroid disturbances of Graves' disease as well as the unpredictable response of the orbital inflammation to oral corticosteroids.     

Orbital metastasis from breast carcinoma presenting as neurotrophic keratitis

CASE REPORT: This is a case report of an 81-year-old woman with previous breast cancer. The patient presented with a history of unilateral painless red eye and displayed on examination a nodule in the superior orbital rim, proptosis, and neurotrophic keratitis. Clinical and radiographic examinations were followed by biopsy of the orbital mass. Computed tomography displayed an extraconal mass adjacent to the left superior orbital rim extending to the soft tissues. Light microscopy findings suggested the diagnosis of orbital metastatic breast carcinoma that was confirmed by immunohistochemistry. The histopathologic diagnosis was metastatic adenocarcinoma of the breast to the left orbit. COMMENTS: This study emphasizes that orbital metastasis must be considered in patients with ocular complaints and neoplastic disease thought to be stable.     

非特异性炎症致眶尖综合征临床特征分析

目的 总结眶尖非特异炎症导致眶尖综合征患者的临床特征。设计 回顾性病例系列。研究对象 2016年6月至2019年5月北京同仁医院神经眼科及北京市普仁医院眼科眼眶非特异性炎症致眶尖综合征15例（16眼）。方法 回顾患者的临床症状、体征、化验及影像学检查、激素治疗效果及病情转归。主要指标 临床表现、眼眶及头颅CT、MRI表现。结果 15例中男性8例,平均年龄（53±16）岁。14/15例单眼发病。病灶均局限于眶尖区,7例同时累及海绵窦。首先出现患侧眼眶疼痛,相继出现眶尖综合征表现。眼眶MRI均显示眶尖部条片状不规则实性病灶,呈等T1WI、等T2WI信号,均匀强化。经全身或眼球局部糖皮质激素治疗后14眼（87.5%）眼痛完全消失,13眼（81.3%）上睑下垂改善,11眼（68.8%）眼球运动及复视症状改善。3个月后复查眼眶MRI病灶均缩小。仅一半患者治疗后视力提高,其余8眼（50.0%）视力无改善。随访过程中2例（12.5%）病变复发。结论 局限于眶尖的非特异炎症可导致严重的眶尖综合征。早期无眼红肿等炎症表现,眼球突出亦不明显,表现为眶周疼痛、眼球运动障碍,继而视力进行性损伤,易被误诊。临床表现和神经影像学是诊断主要依据。糖皮质激素治疗能有效改善眼球运动障碍和疼痛,但视力恢复困难,且病变有复发可能。（眼科,2021,30： 56-61）     

Orbital sarcoidosis manifesting with enophthalmos

PURPOSE: To describe a case of orbital sarcoidosis manifesting with enophthalmos. METHODS: A 60-year-old woman presented with right enophthalmos. She had a palpable mass in her anterior orbit inferiorly. She underwent a complete ophthalmic evaluation, magnetic resonance imaging, and histopathologic examination. RESULTS: A magnetic resonance imaging scan disclosed an orbital, extraconal mass, with displacement of the orbital floor. A biopsy specimen of the mass demonstrated a granulomatous inflammation without caesation necrosis. Workup revealed hilar lymphadenopathy, pleural nodule, and splenomegaly. The patient was treated with corticosteroids, with subsequent involution of the mass and disappearance of enophthalmos. CONCLUSION: Although orbital sarcoidosis usually manifests with proptosis or palpebral swelling, enophthalmos should be considered as ophthalmic manifestation of sarcoidosis.     

Orbital solitary fibrous tumor: A report of two cases

Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm that can be found in the orbit. Here, we report two cases affected by orbital SFT. Both patients were female, aged 52 years and 59 years, respectively, and had experienced a painless unilateral orbital lesion. Computed tomography (CT) imaging revealed a well-circumscribed and contrast-enhanced soft tissue mass simultaneously. The tumors were located in the laterotemporal extraconal space of the right orbit and the inferior portion of the left orbit, respectively. Both patients underwent complete resection of their tumors. The histological findings showed alternating hypercellular and hypocellular areas composed of bland spindle cells with a fibrous stroma. The strong immunoreactivity for CD34 supported the diagnosis of orbital SFT. There was no recurrence at the 2-year and 3-year follow-up visits, respectively. SFTs should be considered in the differential diagnosis of an orbital tumor. The combination of a CT scan, histologic findings, and immunohistochemical staining will provide an accurate diagnosis. En bloc excision of the tumor is the mainstay of treatment in order to avoid recurrence.     

Acute proptosis as first symptom of a lung carcinoma

PURPOSE/METHOD: We present the case of a 57-year-old patient who was visited in our ophthalmology department complaining of acute right ocular proptosis, fever and malaise. The clinical, ophthalmic and radiologic examination showed the presence of an extraconal orbital tumor as the cause of this exophthalmos. RESULTS/CONCLUSION: Further complementary studies revealed a small cell carcinoma of the lung metastatic to the right orbit. We report the excellent response to chemotherapy both of the primary lung neoplasm and the orbital metastasis.     

Orbital schwannoma: correlation of magnetic resonance imaging and pathologic findings

Purpose To report the correlation between magnetic resonance imaging (MRI) findings and histopathology of orbital schwannomas.Methods This study is a retrospective noncomparative interventional case series of three consecutive patients with orbital schwannoma. After preoperative MRI, all patients underwent orbitotomy and tumor removal. Histopathologic and immunopathologic studies of the excised tumors were performed. The main outcome measures were MRI features, histopathology, and their relationship to the cohesiveness of the tumor.Results The patients ages were 56 years (patient 1), 43 years (patient 2), and 18 (patient 3) years. MRI demonstrated that the tumors were well-circumscribed, intraconal in patient 1, superior extraconal in patient 2 and inferotemporal extraconal in patient 3. The tumor in patient 1 had a partly cavitary appearance, while the tumors in patients 2 and 3 had a solid appearance on MRI. The tumor had heterogeneous signal intensity in patients 1 and 2 and homogeneous signal intensity in patient 3 on T2-weighted images. All patients underwent orbitotomy and tumor removal. In patients 1 and 2, the tumor was gelatinous and fragmented during removal. The fragments were completely excised in patient 1 and an incomplete piecemeal tumor excision was made in patient 2. In patient 3, complete excision without fragmentation was performed. Pathologic examination revealed that the tumor was of Antoni B pattern in patient 1, mixed Antoni A and B patterns in patient 2, and marked Antoni A pattern in patient 3. At a mean follow-up of 23.7 months (range: 22–26 months) after surgery, all patients retained preoperative visual acuity. Patient 2 developed recurrence at 21 months follow-up and underwent Krönlein orbitotomy for excision of the recurrent tumor.Conclusions MRI is useful in depicting both the anatomic location and internal consistency of orbital tumors. Schwannomas with MRI evidence of cavitary change or heterogeneous signal intensity on T2-weighted images were found to have marked Antoni B pattern on histopathology, imparting a friable structure and fragmented excision.An erratum to this article can be found at     

眼眶病常用影像学检查设备操作指南(2024)

发生于眶隔后眶骨、眶内及眶周组织的疾病或全身疾病侵犯眼眶者均属于眼眶病。眼眶病涉及的疾病类型复杂,临床表现多样,由于其病变处在眼眶及眶内而非眼球内,常规眼科光学检查对于疾病的诊断帮助有限,因此眼科影像检查技术如眼部B超、CT、MRI等成为眼眶病诊断和病情评估的最主要手段。规范眼眶病常用检查设备的操作及总结眼眶病变影像学特征,可以提高眼眶病诊疗准确性和及时性,有助于减少眼眶病的误诊和漏诊,对眼眶病的诊治具有重要意义。本指南总结了眼眶病的常用检查设备原理、操作流程及影像特征,为规范眼眶病的常用检查方法、提高基层医疗眼眶病诊断效能提供参考指南。     

Langerhans cell histiocytosis

The clinical manifestations of Langerhans cell histiocytosis have been recognized for more than a century. For most of that time, physicians have viewed the disease from different perspectives, interpreting portions of its clinical spectrum as if they were distinct and unrelated entities. More recently, Langerhans cell histiocytosis has been unified into a single concept, though the disease continues to defy traditional classification. By most accounts, Langerhans cell histiocytosis appears to be a morphologically benign proliferation of inflammatory cells that escapes regulatory control mechanisms. Studies from patients with all stages of the disease, however, document clonal proliferation of immune processing cells (i.e., Langerhans cells), suggesting a malignant disease process. The most common ophthalmic manifestation of Langerhans cell histiocytosis is a solitary lesion of orbital bone, which typically responds to minimally invasive therapy. The best management of solitary orbital Langerhans cell histiocytosis is debatable and has been complicated by its recent designation as a risk factor for central nervous system disease. This article summarizes recent developments in understanding the biology of Langerhans cell histiocytosis, reviews its ophthalmic manifestations, prognosis, and the controversy surrounding treatment of isolated orbital disease.     

Primary mesenchymal chondrosarcoma of the orbit: Histopathological report of 3 pediatric cases

Mesenchymal chondrosarcoma (MCS) is an unusual tumor mainly found in the skeleton. Around third of the cases occur in extra-skeletal sites with the orbit being the third most common site in these cases. In previous reviews of the orbital cases, it has been concluded that orbital MCS tends to occur in women in the second or third decades of life. However, 8 cases of orbital MCS have been reported so far in the pediatric age group (age less than 18?years-old) one of which has been considered congenital MCS in a 5-days old newborn girl. We describe 3 additional pediatric cases with primary orbital MCS and they were all males. Our cases presented with proptosis and calcific orbital masses on imaging studies. Histopathological examination of the excised masses shared the typical presence of undifferentiated mesenchymal cells and immature areas of cartilage. The diagnosis of MCS was further confirmed by immunohistochemical staining. Brief review of the literature in relation to this diagnosis in the orbit is also presented.     

Mesenchymal Chondrosarcoma of the Orbit

Purpose: To report a case of mesenchymal chondrosarcoma of the orbit and describe its clinical features, radiological findings and management.

Method: Interventional case report.

Result: A 50 year old man presented with right sided proptosis of 3 months duration. CT scan showed well circumscribed lobulated extraconal mass lesion in the inferotemporal qaudrant with specked calcification within. Patient underwent excisional biospsy with excision of mass lesion in toto. Histopathological examination was suggestive of mesenchymal chondrosarcoma of orbit. Post operatively patient was advised radiotherapy.

Conclusion: Orbital mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.     

Ophthalmic Manifestations of Metastatic Breast Cancer

Metastatic breast cancer is the most common primary tumor metastasizing to the ocular structures. An analysis of 30 patients demonstrated a wide spectrum of ophthalmic manifestations including cranial nerve involvement, brain involvement with papilledema, Horner's syndrome, and choroidal and orbital tumors. The mean age of patients presenting with an ophthalmic sign was 54 years and the mean interval from the diagnosis of breast cancer to the development of the ophthalmic sign was 4.9 years. The carcinoembryonic antigen (CEA) was useful in confirming the diagnosis of metastatic disease as it was elevated in 18 of the 22 patients in whom it was measured. The estrogen receptor assay, performed on metastatic tissue removed from the orbit, can indicate the sensitivity of the breast cancer to hormonal therapy.