眼眶纤维组织细胞瘤临床分析

目的 探讨眼眶纤维组织细胞瘤的临床诊断及治疗方法.方法 对1989年1月至2009年1月收治的21例经病理证实的眼眶纤维组织细胞瘤患者的临床特征及影像学特点进行分析.结果 21例(15例初发,6例复发)均有不同程度的眼眶占位性病变体征;16例眼球突出移位;16例有视力下降;15例有眼球运动障碍;15例可触及眶缘肿块;9例结膜充血水肿;9例出现复视;6例眼睑肿胀.B超显示占位性病变,良性者,边界清,类圆形或椭圆形,内回声少,声衰减显著;恶性者,形状不规则、边界不清,内回声中等或不均匀,声衰减显著,不可压缩.彩色多普勒超声显示肿物内有丰富或中等量血流信号,呈动脉血流频谱.CT显示眶内呈椭圆形或不规则形、边清均质的软组织密度影,或伴有骨破坏,可强化.MRI显示肿物内T1WI呈中或低信号,T2WI呈中或高信号,可强化.所有患者均行手术治疗,6例患者行眶内容剜除术;15例患者行局部肿瘤切除术,其中4例术后随访中复发再次手术.术后病理,21例患者中良性纤维组织细胞瘤10例,中间型2例,恶性者9例.结论 影像学检查只能提供定位诊断,最终仍需病理确诊.治疗以手术切除为主,常复发,可辅以放疗或化疗.

Abstract：

Objective To explore the clinical diagnosis and management approach of the orbital fibrous histiocytomas. Methods The clinical and imaging features of 21 patients from Second Hospital of Tianjin Medical University were collected from January 1989 to January 1999. They were all diagnosed for fibrous histiocytoma by histopathology. Results There were orbital occupying lesion in all 21 patients, but 16 patients demonstrated the exophthalmos and displacement, 16 patients with decreased vision, 15 patients with the ocular movement disturbance, 15 patients with the mass of orbital margin. 9 patients with the diplopia. 9 patients with the conjunctival congestion and edema and 6 patients with the eyelid swelling. Type-B ultrasound showed space occupying lesions. Benign tumors demonstrated the well-defined and nearly round mass, the moderate or few inner echo and the obvious attenuation of sound. But malignant tumors showed the ill-defined and irregular mass. Color doppler flowing image (CDFI) discovered that there was abundant or moderate blood flow, accompanied with arterial frequency spectrum. Computed tomography (CT) revealed an oval-shape or irregular, well-defined or ill-defined and homogeneous soft tissue mass in the orbital cavity, sometimes with bone destruction. The masses were enhanced. Magnetic resonance imaging (MRI) demonstrated a intermediate or low signal on T1 weight imaging, but a intermediate or high signal on T2 weight imaging in the tumor. All the patients were managed by surgery. Six patients were performed by orbital exenteration and 15 patients were performed only surgical resection. But 4 patients recurred postoperatively and underwent another surgery. There were 10 benign tumors, 2 locally aggressive tumors and 9 malignant tumors postoperatively. Conclusions Surgery is the mainstay of therapy. Imaging examination may provide the level diagnosis, but the final diagnosis is made by histopathology. The tumors often recur and adjuvant therapy is utilized.     

Cemento-ossifying fibroma of the orbit.

Cemento-ossifying fibroma presents with ophthalmic symptoms and signs rarely. We report the clinical and pathological findings in a case of cemento-ossifying fibroma of the right maxilla with extension into the orbital floor causing intermittent vertical diplopia, proptosis, and upward displacement of the globe. Compression of the nasolacrimal duct produced epiphora early in the course of the disease. Fibrous dysplasia has often been diagnosed in other cases of benign monostotic fibro-osseous conditions. Ossifying fibroma is easily confused with fibrous dysplasia. The histopathological difference between the two lesions is described.     

眼眶转移性肿瘤的诊治分析

目的 了解眼眶转移性肿瘤的疾病种类,总结临床表现及诊治过程.方法 回顾性系列病例研究.回顾武警总医院眼眶病研究所经病理学确诊的眼眶转移性肿瘤患者30例,总结其流行病学特征、临床表现及影像学征象.结果 常见眼眶转移性肿瘤的前3位分别是:乳腺癌、肺癌和肝癌.男性以肝癌和肺癌多见,女性以乳腺癌和肺癌多见.男女比例1∶1.儿童和中老年是两个好发年龄段.50%患者以眼部表现首诊.乳腺癌病程最长,转移性母细胞瘤病程最短.该类疾病可累及单眼或双眼,常见症状是疼痛和复视,常见体征包括眼球突出、运动障碍、眶压升高等.CT和MRI等影像学检查显示,不同眼眶转移性肿瘤的累及部位和形态有一定规律,可分为4种类型:(1)骨膜下的以眶骨壁为中心的软组织肿物,伴骨破坏;(2)眶前部软组织肿物,包绕眼球,呈铸造样;(3)肌锥内软组织肿物,可侵犯眶尖;(4)孤立的软组织肿物,边界清晰.60%的随访者预后较差.结论 我国眼眶转移性肿瘤的原发疾病种类不同于西方国家,掌握其临床表现和诊断方法有助于缓解眼部症状,延长生命.(中华眼科杂志,2008,44:687-690)     

Spontaneous orbital hematoma in an adult. A case report

Spontaneous orbital hematoma is uncommon, especially in adults. It produces proptosis which progress rapidly and is often associated with ocular movement disturbance. The Authors report a case of spontaneous orbital hematoma in a 45-year-old woman, who had had no previous disease and had experienced sudden onset diplopia associated with moderate proptosis and pain in her right orbit. The diagnosis of orbital hematoma was made by computed tomographic scan. Further investigations revealed arterial hypertension. After a needle aspiration of this hematoma, proptosis resolved, ocular movement began to improve as well as visual acuity. Orbital hematoma revealing arterial hypertension is exceptional. It could be associated with ocular and orbital complications that lead the patient to consult an ophthalmologist. The diagnosis is based essentially on radiology. The other causes of orbital process, inflammation, tumor or vascular malformation, should be considered in the differential diagnosis. Therapeutic management could require surgical evacuation when visual function is compromised (compressive optic neuropathy).     

Non-specific orbital inflammation: Current understanding and unmet needs

Non-specific orbital inflammation (NSOI) is a noninfectious inflammatory condition of the orbit. Although it is generally considered the most common diagnosis derived from an orbital biopsy, it is a diagnosis of exclusion, meaning that the diagnosis requires exclusion of a systemic process or another identifiable etiology of orbital inflammation. The clinical diagnosis of NSOI is ill-defined, but it is typically characterized by acute orbital signs and symptoms, including pain, proptosis, periorbital edema, chemosis, diplopia, and less commonly visual disturbance. NSOI poses a diagnostic and therapeutic challenge: The clinical presentations and histological findings are heterogeneous, and there are no specific diagnostic criteria or treatment guidelines. The etiology and pathogenesis of NSOI are poorly understood. Here we recapitulate our current clinical understanding of NSOI, with an emphasis on the most recent findings on clinical characteristics, imaging findings, and treatment outcomes. Furthermore, gene expression profiling of NSOI and its implications are presented and discussed.     

Clinical manifestations of orbital mass lesions at the Jules Stein Eye Institute, 1999-2003.

BACKGROUND AND OBJECTIVE: To describe the clinical manifestations, diagnoses, treatments, and outcomes of orbital mass lesions at a tertiary care referral center. PATIENTS AND METHODS: All cases of newly diagnosed or referred orbital tumors at the Jules Stein Eye Institute from 1999 to 2003 were reviewed retrospectively. Demographic and clinical data were extracted from the electronic oculoplastics registry of the Division of Orbital and Ophthalmic Plastic Surgery. RESULTS: Three hundred sixty-nine cases of orbital mass lesions were evaluated (167 males and 202 females; mean age = 48 years). The most common presenting symptoms were mass/proptosis, pain, swelling, inflammation, and diplopia. The most common categories of diagnosis were cystic or structural lesions, benign tumors, inflammatory processes, neuronal processes, and fibrous processes. Increasing age was associated with an increased incidence of primary and metastatic malignant tumors. Half of all cases required surgical intervention consisting of excision, debulking, or exenteration; 20% to 30% of cases were managed conservatively. CONCLUSIONS: The differential diagnosis of orbital mass lesions differs across age groups. No clinical sign or symptom is specific for the underlying diagnosis and the biological behavior of the abnormal process may be misleading. Therefore, a careful diagnostic approach that considers the benefit of imaging studies must be undertaken. Almost 50% of these mass lesions can be managed with nonsurgical intervention.     

Idiopathic orbital inflammation with bone destruction and extension into the paranasal sinuses

Idiopathic orbital inflammation developed in the right orbit of a woman in her mid-thirties, causing tearing, photophobia, diplopia, altered depth perception, proptosis, and pain on eye movements. Computed tomography disclosed a mass involving the intraconal and extraconal nasal right orbit, extending to the orbital apex with anterior displacement of the globe, effacement of the medial rectus muscle, portions of the fat plane, and the superior oblique muscle, and bone destruction with extension of the mass through the orbital floor into the superior maxillary sinus and through the lamina papyracea into the ethmoid sinus. Orbital biopsy disclosed dense fibrous connective tissue with numerous lymphocytes and macrophages. Immunohistochemical stains supported a diagnosis of idiopathic inflammatory pseudotumor involving the orbit and sinus mucosa. Treatment with a prednisone taper and a retrobulbar injection of triamcinolone acetonide have relieved her symptoms and diminished her proptosis. This patient highlights the rare potential of idiopathic orbital inflammation to erode though bone into adjacent cranial structures.     

Malignant Lymphoproliferative Disorders Extending into the Orbit from the Paranasal Sinuses

Background/Aims: To describe the clinical features, imaging, histologic spectrum, treatment and prognosis of patients with malignant orbital lymphoproliferative disorders extending from the paranasal sinuses. Methods: Patients were culled from the University of British Columbia Orbit Clinic (1977–2004) and their clinical charts, imaging and pathology specimens reviewed. Results: The study included 6 patients with non-Hodgkin's lymphoma (NHL) and 5 multiple myeloma (MM). Orbital symptoms at presentation were periorbital swelling, diplopia, proptosis and pain. Major clinical findings were globe displacement, abnormal ocular movements, periorbital fullness, fifth nerve hypoesthesia and inflammatory signs. Imaging showed a soft tissue mass in one or more sinuses invading the orbit. Three NHL patients were still alive at last follow-up with one patient lost to follow-up. Two NHL patients and all MM died. Conclusion: Presenting symptoms of malignant orbital lymphoproliferative disorders extending from the paranasal sinuses include mass effect occasionally with infiltrative and inflammatory characteristics accompanied by symptoms of sinus disease. On imaging, a soft tissue mass in the sinuses and orbit with bone destruction is commonly seen. Biopsy is essential for conclusive diagnosis. Treatment should be initiated promptly as local symptoms are frequently relieved and survival is possible, especially in cases of NHL.     

Ophthalmic manifestations of esthesioneuroblastoma

Esthesioneuroblastoma, a tumor that arises from the olfactory sensory epithelium, often manifests with ocular and orbital signs and symptoms. A review of 38 cases of esthesioneuroblastoma at the Mayo Clinic revealed that ophthalmic signs or symptoms occurred in 28. Twenty patients had ophthalmic signs or symptoms at the time of presentation, and five of these had ocular symptoms as the primary complaint. Five patients presented to an ophthalmologist, but a nasal or paranasal sinus tumor was not suspected. Periorbital pain and excessive tearing were the most commonly reported symptoms. The most common ophthalmic sign was eyelid edema followed by proptosis, globe injection, and ptosis. Recognition of the signs and symptoms of neoplasms of the nose and paranasal sinuses and consideration of esthesioneuroblastoma in the differential diagnosis by clinicians and pathologists allow for prompt management of this tumor.     

Sinus Tumors Invading The Orbit

Forty-seven of 79 patients with sinus and paranasal tumors had clinical, radiographic or operative evidence of orbital involvement. Seventy percent of those patients with orbital extension had clinical or radiographic involvement of the orbit at the time of initial presentation. Common presenting signs and symptoms included proptosis, nasal obstruction or discharge, nasal mass, facial and/or eye pain, visual loss, facial and/or lid edema and diplopia.The most common tumor seen was squamous cell carcinoma. The maxillary sinus was the most frequent site of origin. Three patients were misdiagnosed as having sinusitis on initial evaluation. A diagnosis of sinusitis is tentative and should be reevaluated early with repeat roentgenographic studies and biopsy, especially in the presence of protracted facial and eye pain. When ordering CT scans, one must specifically request cuts of the base of the sinuses and skull as routine brain CT scans do not evaluate those regions.Two of four patients with intractable pain unrelieved by narcotics obtained pain relief with cisplatinum.     

Orbital invasion of frontal sinus lymphoma

Paranasal sinus lymphoma is an uncommon malignancy and is often difficult to diagnose. Early diagnosis is essential for effective treatment. The tumour generally has poor prognosis. Ophthalmological symptoms and signs occur early in the disease process due to the close proximity of the orbit to the paranasal sinuses. Common presenting features include eye pain, proptosis, visual loss and diplopia. We report a case of frontal sinus lymphoma that presented as a superior-nasal orbital mass in an 84 year old man. CT scan demonstrated a mass occupying the frontal sinuses, with destruction of the anterior bony wall of the frontal sinus and extending to the right orbit. Histology revealed diffuse large B cell non Hodgkin's lymphoma. The tumor was treated with radiotherapy and showed regression; however the patient died 9 months later. Sinus tumours are encountered by ophthalmologists and should be considered in patients presenting with an orbital mass.     

Australasian orbital and adnexal Wegener's granulomatosis

OBJECTIVE: To report a retrospective case series of 29 Australian and New Zealand patients with orbital and adnexal Wegener's granulomatosis (WG). DESIGN: Retrospective case series. PARTICIPANTS: Twenty-nine cases of orbital and adnexal WG were identified. METHODS: A number of oculoplastic surgeons and other clinicians in Australia and New Zealand was asked about their experience with orbital and adnexal WG. Clinical data regarding these cases were conveyed by means of a questionnaire. Cases of ophthalmic WG without features of orbital or adnexal disease were excluded. MAIN OUTCOME MEASURES: Data obtained from the questionnaire includes age, gender, limited or generalized disease, antineutrophil cytoplasmic antibody (ANCA) status, symptoms and signs: nasolacrimal obstruction, sinusitis, fistula/orbital bone erosion, orbital mass/proptosis, extraocular muscle/diplopia, visual acuity reduction caused by optic nerve compression, orbital pain, lid edema/erythema, biopsy status, and treatment status. RESULTS: Twenty-nine patients with orbital and adnexal WG were identified and described. Symptoms included awareness of an orbital mass, epiphora, orbital pain and diplopia. Signs included an orbital mass or proptosis (69%), nasolacrimal duct obstruction (52%), limited ocular rotations (52%), lid erythema and edema (31%), bony destruction (21%), and reduced visual acuity (17%). Two patients had a persistent nasolacrimocanthal fistula. Cytoplasmic pattern antineutrophil cytoplasmic antibodies (c-ANCA) were present in 52% of patients, and in 9 of 10 patients with generalized disease. However, c-ANCA was positive in only 32% (6 of 19) of patients with limited WG. Perinuclear pattern antineutrophil cytoplasmic antibodies (p-ANCA) was positive in 10% of cases. CONCLUSIONS: To diagnose and treat ophthalmic WG effectively, the clinician must be aware of its protean orbital and adnexal manifestations. WG may occur with or without systemic involvement, and c-ANCA was negative in approximately half our cases. Our cases also demonstrated two orbital fistulae, an observation previously believed to be rare.     

Relapsing polychondritis.

BACKGROUND: Relapsing polychondritis (RPC) is a rare, chronic, and potentially fatal multisystemic inflammatory disorder targeting cartilaginous structures. This disorder is frequently associated with rheumatoid arthritis, systemic vasculitis, connective tissue diseases, and/or hematologic disorders. RPC afflicts patients with recurrent and often progressive episodes of inflammation with the potential for destruction of the affected structures. Tissues involved include the ears, joints, nose, larynx, trachea, eyes, heart valves, kidneys,and skin. Ocular manifestations commonly include episcleritis, scleritis, conjunctivitis, iridocyclitis, chorioretinitis, and proptosis. Lid edema, orbital inflammation, muscle palsies, and corneal melting may also occur. CASE REPORT: An 83-year-old man previously diagnosed with RPC presented to our clinic with acute unilateral chemosis, conjunctivitis, lid edema, proptosis, and extraocular muscle restriction. After orbital cellulitis was ruled out, further evaluation revealed posterior scleritis with choroidal detachment OS. A course of oral indomethacin and topical antibiotic-steroid combination drops was implemented in the treatment of the ocular manifestations. The quick positive response to the anti-inflammatory agents confirmed the diagnosis of ocular complications secondary to RPC. DISCUSSION: The presenting ocular signs and symptoms of RPC often resemble other commonly encountered ocular conditions. It is important for the eye care practitioner to be familiar with the ocular manifestations of RPC because the eyes are sometimes the initial site of involvement and may be a marker of severity. Early diagnosis and intervention may significantly improve the patient's outcome. This case report with literature review will hopefully bring to light features of this disease which will help the eye care practitioner in the diagnosis and management of this condition.     

Bilateral optic nerve edema presenting as initial manifestation of thyroid eye disease

A 48-year-old smoker with a history of hyperthyroidism treated 10 years prior to presentation with radioactive iodine ablation of the thyroid gland presented to his ophthalmologist with a 2-week history of transient loss of vision in the right eye occurring for 1 to 2 hours each morning. He denied ocular pain, diplopia or change in the prominence of one or both eyes. Examination revealed 2 mm of relative proptosis on the right, bilateral temporal flare and lower lid retraction. There was minimal upper lid retraction and no evidence of lid lag. Ocular motility was full. Dilated fundoscopic examination revealed bilateral optic nerve edema, right more than left. CT of the orbit demonstrated enlargement of the extraocular muscles bilaterally with marked enlargement of the right medial rectus and left inferior rectus muscles resulting in crowding at the orbital apex bilaterally. Laboratory testing revealed the patient to be hyperthyroid. The patient was treated with high dose oral steroids followed by orbital radiation. Hyperthyroidism was managed by the patient’s primary care physician. Visual symptoms rapidly improved with oral steroids and orbital radiation. Optic nerve edema completely resolved. Repeat CT imaging demonstrated a reduction in the enlargement of the extraocular muscles with relief of bilateral optic nerve compression.     

Meningiomas of the anterior visual system

Meningiomas, whether primary in the orbit, optic canal or intracranial area, typically cause slowly progressive loss of vision covering months to years. They usually occur in white women of middle age. Tumors in the orbit or optic canal almost always affect vision unilaterally; intracranial tumors, while usually causing unilateral visual loss initially, eventually cause bilateral loss of vision, often with blindness in one eye. Depending upon the size and location of the tumor, the ocular signs and symptoms of meningiomas may include visual field abnormalities, optic atrophy, edema of the ipsilateral optic disc, papilledema, diplopia, and proptosis. The diagnosis of meningiomas in all locations has been greatly facilitated by recent advances in computer assisted tomography. However, tumors confined to the optic canal or its foramina are small when vision is first lost and therefore are still difficult to detect. Hypocycloidal polytomography may be useful; other neuroradiologic studies rarely are. Treatment is surgical. Based on a review of more than 3000 meningiomas reported in the literature, the frequency and characteristics of the signs and symptoms of meningiomas in each location are discussed, as are the anatomy, pathology, natural history, and probable mechanisms.     

Klinische und neuroradiologische Diagnostik bei Orbitatumoren

Exophthalmus is the leading sign of space-occupying lesions of the orbit. Patients may further present with lid swelling, impaired ocular motility and optic neuropathy including a relative afferent pupillary defect, compressive optic disc edema or optic atrophy. Orbital tumors can be classified into various categories depending on the etiology, as lymphoproliferative lesions (in particular non-Hodgkin's lymphoma as the most common malignant orbital tumor of adulthood), optic nerve and meningeal lesions, lacrimal gland lesions, secondary orbital tumors which extend to the orbit from neighboring structures and metastases. Slightly less common are vasculogenic and cystic lesions including cavernous hemangioma as the most common benign orbital tumor of adulthood and dermoid cysts as the most common benign orbital tumor of childhood. Rhabdomyosarcoma is the most common malignant orbital tumor of childhood but has a low total incidence. Orbital tumors might not only cause symptoms like pain, diplopia and loss of visual acuity but may also lead to esthetically disfiguring changes. Particular attention should be paid to underlying systemic diseases and generalized tumor diseases. This article illustrates the approach to a detailed clinical and neuroradiological assessment which is mandatory for the care of orbital tumor patients.     

Malignant fibrous histiocytoma of the conjunctiva

Fibrous histiocytomas are mesenchymal tumours composed of cells with fibroblastic to histiocytic differentiation. They can occur in any part of the body including the orbital tissues. To date, there are 18 cases of fibrous histiocytoma arising from the corneoscleral limbus reported in the literature. Eleven of these were classified as benign, and the rest were malignant fibrous histiocytomas. Benign fibrous histiocytomas have been reported in the orbit, eyelid, episclera and conjunctiva. Malignant fibrous histiocytoma has been well described in the orbit, but rarely as a primary conjunctival tumour. The rarity of the tumour makes its diagnosis and management a challenge. Herein, the clinicopathological features of a case of malignant fibrous histiocytoma are presented and its management with wide excision and cryotherapy followed by ocular reconstruction with amniotic membrane transplant is discussed.     

Frontoethmoidal mucocele presenting with ocular manifestations

Mucocele of the paranasal sinuses is a slowly expanding benign lesion developing when there is impeded physiological drainage of the mucous produced by the epithelial lining of the paranasal sinuses, at the sinus ostium, which is an opening that connects the sinus to the nasal cavity. Aetiologies of ostial occlusion include infection, allergy, trauma, previous surgery, benign neoplasm (osteoma or fibrous dysplasia), and malignant or metastatic tumours. Mucoceles commonly develop in the frontal sinus (70–80 per cent), followed by the ethmoid (25 per cent), frontoethmoidal (10–14 per cent), and maxillary (three per cent or less) sinuses. The most common manifestations in these cases are ocular oedema, proptosis (22–83 per cent), and diplopia (28 per cent). Due to these ocular signs and symptoms, the optometrist may be first in line managing paranasal sinus disease patients, reducing the risk of permanent damage. A case report and review of frontoethmoidal mucocele will be discussed in this report, to include the role of the optometrist in its management and treatment.     

鼻窦肿瘤的眼部表现分析

为了早期诊断和治疗以眼部症状就诊的鼻窦肿瘤患者，分析６０例鼻窦肿瘤的眼部症状，表现为眼球突出、眼球运动障碍、复视、视力下降或丧失、上睑下垂等。其中４２例首诊于眼科。所有病例均经ＣＴ扫描和病理病检查证实。４８例采用手术治疗，３２例眼部症状改善。结果提示鼻窦肿瘤易出现眼部症状的原因，主要是由于鼻窦与眶壁、鼻窦与视神经管之间密切的解剖学关系。ＣＴ扫描有助于该病的早期诊断。     

Late-onset presentation of orbital lymphangioma

PURPOSE: To report a group of patients with biopsy-proven orbital lymphangioma diagnosed late in life. METHODS: The clinical records and histopathologic findings of 3 patients with biopsy-proven orbital lymphangioma were reviewed. RESULTS: Three patients were identified who were diagnosed with orbital lymphangioma at the ages of 70, 80, and 82 years old. Each patient presented with different symptoms and signs including presence of a superficial mass, periorbital pain, and gradual painless proptosis, respectively. In one case, imaging revealed an isolated superficial mass, and in the other 2 cases it demonstrated the presence of a heterogeneous, cystic, retrobulbar mass. Pathologic findings in all cases included endothelium-lined channels with intervening fibrous septae infiltrated with aggregates of lymphocytes. Two of the three patients had complete excisions without recurrences and the third patient was followed after biopsy. CONCLUSIONS: Lymphangioma, one of the most common vascular lesions in childhood, should also be included in the differential diagnosis of orbital masses in adults, including elderly adults.