Multiple cavernous hemangiomas of the orbit

 Purpose: Cavernous hemangioma of the orbit is often unilateral and solitary. Multiple cavernous hemangiomas of the orbit are extremely rare. The authors reported four patients who had more than two cavernous hemangiomas in one orbit. Methods:Case reports.The clinical features, imaging findings, differential  diagnosis and surgical management werepresented. Results: We found 4 cases of multiple cavernous hemangiomas of the orbit out of 214 records of all patients with orbital cavernous hemangiomas who were referred to us from Jan 1,1986 to Dec 31,2000. Two patients were female and two were male. The mean age was 46.5 years, ranging from 37.0 to 57.0 years. The left orbits were affected in all patients, with two, three, eight and ten tumors respectively. The most common complaint was a painless, gradually progressive proptosis. The characteristic CT imaging features were multiple well circumscribed, round or ovoid masses that were sharply delineated from the surrounding tissue. B-scan ultrasonography showed round-shaped orbital masses with high internal reflectivity. The therapeutic outcomes of lateral or anterior orbitotomy were excellent. Conclusion: Although rare, cavernous hemangioma should be considered in the differential diagnosis of patients with multiple orbital mass lesions.     

以自发性出血为特征的眼眶静脉畸形临床分析

目的 探讨眼眶静脉畸形伴自发性出血的临床、影像学特征、诊断和治疗原则.方法 对15例单眼眼眶静脉畸形伴自发性出血的病例进行回顾性分析.结果 所有患者中,除1例Valsalva试验出现眼球突出度增加外,余均无扩张性病变临床征象.15例患者均行眼眶CT检查,6例误诊为肿瘤,其中5例均经MRI明确为出血.7例行保守治疗,出血吸收后,影像学随访发现扩张性静脉畸形.8例行手术治疗,2例病理提示为混合型静脉淋巴管瘤,6例为静脉性血管畸形,术后6例视力获得提高.结论 与全身静脉系统联系较少的眼眶静脉畸形,临床上常无特征性表现,一旦发生眶内出血易误诊为肿瘤.对不明原因的急性眼球突出伴眼眶胀痛,眼眶MRI常可发现出血性病变,无明显视力下降时可予保守治疗,待出血吸收后影像学随访检查常可发现扩张畸形的血管病变.视力显著下降的患者,应行手术干预,多可获得视力恢复.

Abstract：

Objective To approach the clinical and image features,diagnosis and treatment of orbital venous malformations(OVMs)presenting with spontaneous hemorrhage.Methods A retrospective review of 15 patients with OVMS presenting with spontaneous hemorrhage.Results None of all the patients showed clinically distensibility except 1 observed worsening proptosis with Valsalva maneuver.CT scan were done in all patients.6 were referred with a suspicion of orbital tumor,5 of them were identified orbital hemorrhage by typical findings on MRI.7 patients were treated conservatively.After resolution of the hemorrhage,they evidenced a distensible OVMs in a follow-up imaging study.8 patients were treated by surgical excision and were all examined histopathologically.2 of them were confirmed venous-lymphatic malformations,6 were confirmed OVMs.Visual acuity was improved in 6 patients.Conclusions OVMs with small venous connection may be not clinically evident.Spontaneous hemorrhage develops in this lesion can simulate orbital tumors.In cases that show acute exophthalmos and ocular pain,MRI should be used to verify the hemorrhage.It is better to wait,with conservative treatment when one without visual deterioration,until the hemorrhage is resolved and then performed a follow-up CT for the diagnosis.Surgical intervention should be confirmed to those with visual deterioratinn.Most of them can get visual recovery.     

Magnetic Resonance Imaging and DWI Features of Orbital Rhabdomyosarcoma

 Purpose: To describe the magnetic resonance imaging (MRI) features of orbital rhabdomyosarcoma (RMS). Methods: Thirty-nine patients with histopathologically confirmed orbital RMS were retrospectively reviewed. All patients underwent orbital conventional MRI, including axial, sagittal, and coronal T1-weighted, T2-weighted, and postcontrast T1-weighted sequences. The location, shape, margin, and MRI signal of the 39 lesions were reviewed. DWI in 15 patients and susceptibility weighted imaging (SWI) in 2 patients were also analyzed. Results: Orbital MRI was available in 39 patients and revealed a soft tissue mass in the orbital region in all cases. Of the 39 patients, the primary tumor sites were limited to the orbital proper in 31 cases, while 28 cases had extraocular muscle invasion and 8 cases had extraorbital invasion. All lesions were unilateral. Thirty-three cases were well-defined soft tissue masses and 6 cases appeared as less well-defined soft-tissue masses. Thirty-four cases showed homogeneous isointense or slightly hypointense signals on T1-weighted imaging (T1WI) and hyperintense signal on T2-weighted imaging (T2WI) compared with extraocular muscles. Five cases had heterogeneous signals with focal areas of increased signal on T1WI or decreased signal on T2WI, including 1 case with hypointense signal on SWI. The mean apparent diffusion coefficient (ADC) value of the viable part of tumors was (0.925±0.09)×10-3 mm2/s. All cases showed moderate to marked enhancement after contrast administration. Conclusion:Several MRI features—including homogeneous isointense or slightly hypointense signal on T1WI and slightly hyperintense signal on T2WI, relative low ADC values, and moderate to marked enhancement, extraocular muscles invasion, and extraorbital extension—are helpful in the diagnosis of orbital RMS.     

Ocular Trauma as the First Presentation of Langerhans Cell Histiocytosis

Purpose： Ocular trauma occurs disproportionately in children on an annual basis. However, because of this frequency, oth- er diagnoses, such as orbital neoplasms, can easily be ignored. Methods：We report on a two-year-old boy who presented with a dark purple, irregularly shaped lesion on his lower left eyelid. The patient had suffered injuries twice to that area. Results： Axial computed tomography images demonstrated an ill-defined and inhomogeneous soft tissue mass in the lateral and posterior region of the left orbit, with bony destruction and absorption of the adjacent orbital wall. Magnetic reso- nance imaging of the orbit showed a lesion involving the greater sphenoidal wing, the supraorbital wall, and the frontal area. Enlarged nuchal and inguinal lymph nodes were detected by sonographic examination. Histopathological examination of a surgical biopsy showed an accumulation of characteristic Langerhans cells. The diagnosis of Langerhans cell histiocy- tosis was confirmed by immunohistochemical examinations positive for CDla and SP-100. Conclusion： Orbital Langerhans cell histiocytosis is rarely en- countered in ophthalmic practice, so the ophthalmologist needs to be familiar with its presentation and work-up, and has to be aware of possible OLCH diagnosis when a child （or even an adult） presents with prolonged and persistent eyelid edema, even with a history of ocular trauma.     

Multimodal therapy in the management of primary orbital mesenchymal chondrosarcoma

AIM:To evaluate the ophthalmic manifestations,radiographic features,and prognosis of Chinese patients with primary orbital mesenchymal chondrosarcoma(MCS).METHODS:The study included 6 cases with primary orbital MCS treated at Tianjin Eye Hospital from January 2009 to December 2019.Patitent ophthalmic manifestations,radiographic features,diagnosis,pathology,therapeutic regimens,and prognosis were retrospectively reviewed.RESULTS:Six patitents with primary orbital MCS were identified.The mean age at the first visit was 33y(range,25-42y).All six patients displayed manifestations of exophthalmos,diplopia,limitation of eye displacement,upper eyelid oedema,decreased visual acuity and ptosis.The mean disease history and range were 5 and 2-8mo,respectively.The tumors were located in the superonasal extraconal compartment(2/6,33.3%),intraconal compartment(2/6,33.3%),and bitemporal extraconal compartment(2/6,33.3%),respectively.Radiographic features were a well-defined,orbital mass with calcification and ossification on computed tomography(CT),and marked heterogenous enhancement on dynamic magnetic resonance imaging(MRI).Five patients were treated with tumor resection and one patient received orbital exenteration.Five patients in the cohort received postoperative radiation therapy,two patients received chemotherapy,and one patient did not receive postoperative adjuvant therapy because he refused.The histopathologic classification revealed a tumour composed of a mixture of mature chondroid tissue surrounded by small,round,and undifferentiated mesenchymal cells.Immunohistochemistry revealed Bcl-2,vimetin,CD99,and S-100 were expressed were expressed.After surgeries,two patients have developed a local recurrence.The median recurrence time of 58 mo(52-64 mo).One patient had distant recurrence included the lungs occurred 52mo after the initial surgery.CONCLUSION:The possibilty of orbital MCS need to be considered when a painless,slowly growing orbital mass with calcification and ossification.From our experience,trimodality treatment of radiation therapy,chemotherapy and surgery maybe the best option.Orbital MCS has a high tendency for late recurrence,regular long-term follow-up after complete excision is mandatory.     

Optical coherence tomography enhanced depth imaging of chorioretinal folds in patients with orbital tumors

AIM: To characterize spectral-domain optical coherence tomography (SD-OCT) features of chorioretinal folds in orbital mass imaged using enhanced depth imaging (EDI). METHODS: Prospective observational case-control study was conducted in 20 eyes of 20 patients, the uninvolved eye served as a control. All the patients underwent clinical fundus photography, computed tomography, EDI SD-OCT imaging before and after surgery. Two patients with cavernous hemangiomas underwent intratumoral injection of bleomycin A5; the remaining patients underwent tumor excision. Patients were followed 1 to 14mo following surgery (average follow up, 5.8mo). RESULTS: Visual acuity prior to surgery ranged from 20/20 to 20/200. Following surgery, 5 patients’ visual acuity remained unchanged while the remaining 15 patients had a mean letter improvement of 10 (range 4 to 26 letters). Photoreceptor inner/outer segment defects were found in 10 of 15 patients prior to surgery. Following surgical excision, photoreceptor inner/outer segment defects fully resolved in 8 of these 10 patients. CONCLUSION: Persistence of photoreceptor inner/outer segment defects caused by compression of the globe by an orbital mass can be associated with reduced visual prognosis. Our findings suggest that photoreceptor inner/outer segment defects on EDI SD-OCT could be an indicator for immediate surgical excision of an orbital mass causing choroidal compression.     

Updates in Orbital Tumors

Orbital anatomy, the clinical features of orbital tumors, the recent development of the diagnosis and management of orbital tumors were described. The incidence of orbital tumors in Dr. Cipto Mangunkusumo Hospital in the past years were introduced. The principle of management of orbital tumors and their prognosis were discussed.     

Clinicopathological analysis of 39 patients with corneal tumor

 Purpose:Corneal tumors are rare in clinical practice. There was a paucity of data on the incidence of various corneal tumors, their clinical and pathological features and surgical management, especially on large patient samples.  Methods:The medical records of 39 patients with corneal tumors referred to Zhongshan Ophthalmic Center, Sun Yat-sen University, China from Jan 1,1996 to Dec 31,2002 were reviewed retrospectively. Results: Of the 39 cases with corneal tumors, 31 were males and 8 were females. The right eye was affected in 24 cases and the left one in 15 cases. The mean age at diagnosis was 45.7 years, ranged from 3 to 88 years. Squamous cell carcinoma was the most common tumor in the cornea (18 cases, 46.15%), followed by pigmented naevus (12 cases,30.77 %), papillary epithelioma (3 cases, 7.69%), melanoma (1 case, 2.56%), oncosis hyperplasia（1 case, 2.56%）, inverting papilloma （1 case, 2.56%）, primary acquired melanosis（1 case, 2.56%）,amyloid degeneration（1 case, 2.56%）,inflammatory pseudotumor（1 case, 2.56%）. They arose most commonly in the limbal region. All tumors were unilaterally involved. Squamous cell carcinoma of the cornea usually appears as a whitish, rough, irregular lesion or a telangiectatic, gelatinous epibulbar mass. Nevus in the cornea generally becomes clinically apparent in the first or second decade of life and the lesion may enlarge or more deeply pigmented afterwards. The corneal tumors were completely excised microsurgically in 22 cases. Six cases were treated with surgical resection combined with amniotic membrane graft. Three cases with surgical excision plus transfer of conjunctival flap. Two cases with surgical excision plus lamellar keratoplasty. Two cases with surgical excision plus cryosurgery. Two cases with orbital exenteration. One case with enuleation. One case with incision biopsy and observation. Conclusion: Squamous cell carcinoma and nevus, the most common corneal tumors, accounted for 76.92% of all cases. The therapeutic outcomes depended upon early pathologic diagnosis and early surgical management.     

Diagnostic Value of the Post-Processing Technique of Multi-Slice Spiral CT in Orbital Cyst Diseases

Purpose:To analyze the features of CT imaging of orbital cystic disease. Methods:.A total of 30 patients were pathologically confirmed with orbital cystic disease between January 2007 and March 2012. Prior to operation, the participants underwent CT scans with image processing by maximum intensity projection (MIP) and volume rendering (VR). Preoperative CT diagnostic outcomes were compared with postoperative pathological findings. Results:.The patients presented with round,.oval and dumbbell shaped masses. Fourteen cases of dermoid cyst and 8 cases of epidermoid cyst showed heterogeneous density. Among the dermoid cyst patients, 6 cases had demixing phenomenon, 3 cases had lipid drift and 5 cases showed mild or moderate intensity enhancement in the cyst wall. No intensity enhancement was observed in the epidermoid cyst patients. Five cases had high intensity due to hemorrhage and 3 patients presented with adjacent sclerotin arc compression;.5 cases of myxoid cyst had homogenous density and no intensity enhancement was found when iohexol was injected..Three patients with atheromatous cyst had heterogeneous density,.with CT value ranging from -36Hu to 191Hu. Floss was noted centrally in 1 case and mild to moderate intensity enhancement was observed in the cyst wall with iohexol injection. Conclusion:.Multi-slice CT is useful in the diagnosis of orbital cystic disease. Multi-slice CT combined with image processing can be helpful in displaying the location and size of masses, and revealing the relationship between masses and surrounding boney tissue, providing an objective basis for surgical planning when combined with 3D-CT imaging.     

眼外蔓延的脉络膜黑色素瘤的临床观察

目的 总结眼外蔓延的脉络膜黑色素瘤的诊断方法和治疗经验.方法 回顾性系列病例研究.收集12例经病理学确诊的眼外蔓延期脉络膜黑色素瘤患者临床资料,总结病史特点、临床表现及影像学征象、治疗与随访结果.结果 4例既往有误诊为青光眼的病史;4例既往确诊者,其中3例接受肿瘤局部切除术.常见体征包括:视力下降,眼球突出,上睑下垂,眼球运动受限,结膜充血,眼压或眶压升高等,特异性体征为巩膜表面隆起性肿物.眼B超检查6例,典型表现为眼球壁结构中断,眼内、外肿瘤连续,眼外肿瘤呈低回声,或呈中、低回声混杂.CT检查6例,表现为肌锥内团块状肿瘤与球内肿瘤相连续,边清,均质,包绕眼球或视神经生长.所有患者行MRI T1和T2加权像检查,球内、外的肿瘤有4种类型的信号特征,肿瘤在T1加权像呈高信号,T2加权像呈低信号为典型表现.球外肿瘤的最大直径超过4 mm者有9例.MRI检查可显示球外多灶蔓延及远处转移,且有助于显示微小的球外蔓延灶,最小直径3 mm.11例患者行眶内容物剜除术,1例行眼球及球外瘤体摘除术.病理学检查显示肿瘤以上皮细胞型最为多见,有3种向眼外蔓延的途径.有随访记录者8例,术后联合化疗、放疗或干扰素治疗,随访1～5年,6例无眼部复发,全身情况良好,1例死亡,1例转移.结论 脉络膜黑色素瘤发展至眼外蔓延期的主要原因可能是延误治疗,临床缺少特异表现,确诊多需结合多种影像学检查.手术联合辅助治疗有助于减少局部复发和全身转移.

Abstract：

Objective To evaluate the clinical features, diagnostic methods and treatment of extraocular extension of choroidal melanoma. Methods It was a retrospective case series study. The records of 12 consecutive cases with extraocular extension of choroidal melanoma confirmed by pathologic examination were analyzed with special attention to the case histories, clinical manifestations, imaging findings, treatment and follow up results. Results Four patients were misdiagnosed as glaucoma. Another 4 patients were confirmed the diagnosis of melanoma and 3 of them received the operations of transscleral local resection. The common clinical manifestations included: impaired vision, exophthalmos,blephroptosis, limited ocular motility, conjunctival hyperemia, and increased ocular or orbital pressure, etc.Characteristic sign was raised mass on the surface of sclera. All cases underwent orbital MRI preoperatively,6 underwent ocular B-scan echography, and 6 underwent orbital CT scanning to evaluate for extraocular extension of tumor. Typical ultrasonography revealed the discontinued ocular wall and an intraocular mass with a continuous hypoechoic extraocular mass. In some cases the extraocular mass showed hypoechoic with medium echo area. CT scan showed a well-defined homogeneous intraconal mass connecting with intraocular lesion in 6 cases, which could infiltrate eye or optic nerve. The MR signal features of intraocular tumors with extraocular extension showed 4 patterns in all patients. The typical pattern was the tumor showed hyperintensity on T1 and hypointensity on T2-weighted image. The maximum diameter of extraocular tumor was measured over 4 mm in 9 cases. MRI was useful for demonstrating multiple extraocular lesions, remote metastatic lesion or micro extraocular tumor, of which minimal diameter was 3 mm in our cases. Orbital exenteration was performed in 11 cases and ocular enucleation with excision of extraocular tumor was in 1 cases. Microscopic examination showed the epithelioid-cell-type tumors were the most common. The tumor extended out of the eye by three types of way in our cases. Follow-up ranged from 1 to 5 years in 8 cases,who underwent chemotherapy, radiotherapy or interferon. Six cases had no recurrences and general health.Death and metastasis was respectively recorded in one case. Conclusions Delayed treatment could induce the growth of choroidal melanoma to the stage of extraocular extension, which has few specific clinical manifestations. The combination of multiple imaging examinations was helpful for the correct diagnosis.Surgical operation with adjunctive therapies could partly reduce recurrence and metastasis of melanoma.     

原发性眼眶骨肿瘤或瘤样病变的临床病理分析

目的探讨原发性眼眶骨肿瘤或瘤样病变的临床改变、影像特征、治疗原则和组织病理学类型。方法回顾性分析近25年收检的20例原发性眼眶骨肿瘤或瘤样病变的病例资料。结果患者的主要临床表现为眼球突出和眶周肿块。X光检查或CT扫描证实眼眶骨壁占位病变，有多种影像特征。病理检查结果：良性肿瘤11例，其中骨瘤5例．血管瘤3例，骨母细胞瘤2例，纤维组织细胞瘤1例。恶性肿瘤2例，为骨肉瘤。中间性肿瘤1例，为侵袭性骨母细胞瘤。瘤样病变6例，包括骨纤维异常增生症4例，动脉瘤样骨囊肿和非骨化性纤维瘤各1例。随访3个月至9年，2例眼眶骨肉瘤和1例骨母细胞瘤复发。结论眼眶骨肿瘤或瘤样病变发病率低，种类繁多。CT是有效的检查手段，需通过组织病理学检查确诊，良性肿瘤或瘤样病变手术切除效果好。     

Orbital tumors in the older adult population

PURPOSE: To determine the types and frequency of orbital tumors in the older adult population. DESIGN: Retrospective consecutive case series. PARTICIPANTS: Two hundred consecutive patients aged 60 years or older with an orbital tumor managed at a tertiary referral center. MAIN OUTCOME MEASURES: The relative incidence and management of orbital tumors in patients aged 60 years or older. RESULTS: Of approximately 950 consecutive patients with orbital space-occupying lesions managed over a 25-year period on the Oncology Service, 200 patients (21%) were 60 years or older at the time of diagnosis. The orbital tumor was unilateral in 183 patients (92%) and bilateral in 17 patients (8%). The most common clinical features at presentation included mass in 51 patients (26%), proptosis in 36 (18%), and pain in 30 (15%). The mean duration of symptoms was 11 months before referral. The orbital tumor was malignant in 125 patients (63%) and benign in 75 (27%). The most common diagnoses were malignant lymphoma in 47 patients (24%), idiopathic orbital inflammation in 19 (10%), and cavernous hemangioma in 15 (8%). The diagnosis was made with clinical findings and imaging studies in 58 patients (29%) and additional histopathologic examination in 142 patients (71%). Of 217 tumors, the main tumor location was in the superior half of the orbit in 106 lesions (49%), in the anterior orbit in 100 (46%), and in the extraconal space in 135 (62%). Imaging studies showed a mass with well-defined margins in 90 tumors (41%) and ill-defined margins in 127 (59%). The tumors were solid in 203 cases (94%), cystic in 6 (3%), and mixed in 8 (3%). Over a mean of 31 months follow-up, 50 (25%) patients had orbital tumor-related systemic involvement (metastasis or lymphoma), and 150 (75%) remained free of systemic involvement. CONCLUSIONS: Orbital tumors in the senior adult population are malignant in 63% of cases. Malignant lymphoma is the most common tumor in this age group, accounting for 24% of cases. Overall, 25% of patients have systemic problems related to the orbital process develop, so systemic evaluation is warranted.     

Solitary Orbital Myofibroma: Clinical,Radiographic, and Histopathologic Findings. A Report of Two Cases

This describes a non-interventional case series of 2 patients, aged 7 and 9 years referred to Oculoplastic Unit, both for evaluation of a gradually enlarging, painless, mass of the cheek. CT scan of the first case revealed left orbital floor destruction from a well-defined intraosseous mass. The second was a round circumscribed orbital floor tumor without bone destruction. Histological diagnosis of myofibroma was rendered in both cases. Solitary myofibromas are rare in the orbit. Their rapid growth and bony destruction can mimic malignant tumors. Complete excision with close follow-up is the preferred treatment. Solitary myofibroma should be considered in the differential diagnoses of fibrous tumors with bone destruction in the orbit.     

Solitary orbital myofibroma: clinical, radiographic, and histopathologic findings. A report of two cases

This describes a non-interventional case series of 2 patients, aged 7 and 9 years referred to Oculoplastic Unit, both for evaluation of a gradually enlarging, painless, mass of the cheek. CT scan of the first case revealed left orbital floor destruction from a well-defined intraosseous mass. The second was a round circumscribed orbital floor tumor without bone destruction. Histological diagnosis of myofibroma was rendered in both cases. Solitary myofibromas are rare in the orbit. Their rapid growth and bony destruction can mimic malignant tumors. Complete excision with close follow-up is the preferred treatment. Solitary myofibroma should be considered in the differential diagnoses of fibrous tumors with bone destruction in the orbit.     

眼眶软组织肿瘤455例临床病理观察

目的：探讨眼眶软组织肿瘤临床病理特征。

方法：回顾性分析我院2003-11/2018-11眼眶软组织肿瘤患者455例的病例资料,观察其临床特点、病理分类和少见肿瘤的病理学特征。

结果：眼眶软组织肿瘤患者455例中,良性肿瘤421例(92.5%),居于前5位的依次为海绵状血管瘤258例、毛细血管瘤58例、神经纤维瘤16例、纤维瘤15例和神经鞘瘤14例; 中间型27例(5.9%),其中孤立性纤维性肿瘤(SFT)23例、低度恶性纤维组织细胞瘤2例、低度恶性黏液性神经纤维瘤2例; 恶性7例(1.5%),其中眼眶恶性SFT者2例,眼眶髓系肉瘤(MS)2例,黏液样脂肪肉瘤(MLS)、梭形细胞未分化肉瘤和外周原始神经外胚叶肿瘤(PNET)各1例。对部分病例进行免疫组化和分子检测,结果发现新抗体如STAT6和分子检测技术的应用能提高诊断准确性。

结论：眼眶软组织肿瘤中,良性肿瘤占大部分,脉管肿瘤中的海绵状血管瘤居于首位,不乏眼眶部位少见软组织肿瘤病理学类型如Erdheim-Chester病(ECD)、砂粒体性骨化性纤维瘤(POF)、平滑肌瘤和黏液瘤等; 中间型和恶性肿瘤少见,眼眶部位SFT、MLS、梭形细胞未分化肉瘤和MS等少见的病理学类型常诊断困难,容易漏诊误诊,新型免疫组化抗体和分子检测技术能提高诊断的准确率。     

Clinical applications of color Doppler imaging in the management of orbital lesions

PURPOSE: To present the clinical applications of color Doppler imaging (CDI) as an adjunctive study in the diagnosis and treatment of orbital lesions. METHODS: Retrospective noncomparative case series. Medical records of 17 patients with orbital lesions who underwent orbital CDI were reviewed, and their orbital CDI results, diagnosis, and treatment were studied. The main outcome measures were detection of abnormal orbital vascular flow and presence or absence of blood flow within orbital lesions by CDI. RESULTS: Absence of intralesional flow was associated with an orbital lesion considered benign. Tumors in which flow was present were more frequently malignant or had malignant potential. Reversal of flow in the superior ophthalmic vein was observed in patients with carotid-cavernous fistula and orbital varix. CONCLUSIONS: CDI is a useful adjunctive imaging study for evaluating the vascularity of orbital tumors. The absence or presence of intratumoral blood flow as demonstrated by CDI can help determine the nature of the orbital mass and can assist with surgical planning.     

A rare case of giant multicystic solitary fibrous tumor of the orbit

Solitary fibrous tumor (SFT) is a rare spindle cell tumor of the orbit of mesenchymal origin. Though these tumors are mostly solid, partial or complete cystic changes can rarely occur. Only six such previous cases of cystic fibrous tumor of the orbit have been mentioned in the literature. We report a case of an elderly male who presented with a huge left sided medial orbital mass. Magnetic resonance imaging showed a predominant cystic orbital mass separated by septae and suggested a diagnosis of Hydatid cyst. The patient underwent complete excision of the mass and histopathology and immunohistochemistry were suggestive of cystic SFT. Cystic degeneration in SFT is extremely rare and can be a harbinger of malignancy, and pose risk of recurrence. Close follow up and monitoring is required for all such cases.     

Recurrent orbital solitary fibrous tumor in a 14-year-old girl

PURPOSE: To report a case of orbital solitary fibrous tumor in a pediatric patient. METHODS: Case report and review of the literature. RESULTS: A 14-year-old girl presented with a 5-month history of painless proptosis of the left eye. Magnetic resonance imaging revealed a well-circumscribed mass in the anterior superomedial left orbit. The lesion was excised, and histopathologic examination revealed a solitary fibrous tumor. The lesion recurred in the orbit 4 months postoperatively, and histologic examination of the new lesion was consistent with solitary fibrous tumor. CONCLUSION: Based on this case report of orbital solitary fibrous tumor in a pediatric patient, solitary fibrous tumor should be included in the differential diagnosis of pediatric orbital tumors.     

Orbital fibrous histiocytoma: case report and literature review.

BACKGROUND: Fibrous histiocytomas are a diverse group of soft tissue tumors classified histiologically as benign, locally aggressive, and malignant. These tumors are found throughout the body, but seem to have an affinity for the periorbital area. They account for one percent of all ocular masses and are the most common primary mesenchymal tumor of the orbit. Associated ocular signs and symptoms include decreased visual acuity, proptosis, diplopia, pain, restricted extraocular muscle movement, swelling of the eyelids, and conjunctiva, as well as disk edema. CASE REPORT: A case of a benign orbital fibrous histiocytoma is presented. The patient reported intermittent pain and occasional diplopia; severe edema of the right upper eyelid; and proptosis and inferior vertical displacement of the right globe were observed. Computed tomography revealed a well-defined mass that was subsequently surgically removed and histopathological results from the Armed-Forces Pathology Institute confirmed the diagnosis. CONCLUSIONS: Orbital fibrous histiocytomas are rare periocular tumors that can manifest multiple ocular signs and symptoms. Careful histologic examination is necessary for diagnosis, since these tumors have a wide range of morphology. Differential diagnoses include orbital masses with similar radiologic or histologic findings.     

正电子发射体层摄影-CT检查对眼眶眼球恶性肿瘤的诊断价值

目的 探讨正电子发射体层摄影-X线电子计算机体层摄影（positron emission tomography-computer tomography,PET-CT）检查对眼眶及眼球恶性肿瘤的诊断价值.方法 对眼眶及眼球恶性肿瘤33例（41眼）.病程1月～5年,行18氟-2-氟-2-脱氧-D-葡萄糖（18F-2-fluoro-2-deoxy-D-glucose,18F-FDG） PET-CT肿瘤代谢全身体层融合显像.结果 术后病理学证实眼眶眼球恶性肿瘤33例,其中淋巴瘤16例,脉络膜黑色素瘤7例,视网膜母细胞瘤4例,眼眶转移瘤6例.17例在眼眶或眼球局部或在其外的皮肤、淋巴结、骨骼、内脏等组织器官有18F-FDG高代谢灶,明确提示为眼眶或眼球恶性肿瘤或转移瘤,3例未能给予明确提示,结合临床进一步检查确诊.13例提示术后或化疗后改变,其中4例可见18F-FDG放射性浓集灶,提示恶性肿瘤术后残留、复发或转移,9例未见18F-FDG浓聚,提示治疗效果良好.结论 PET-CT肿瘤全身断层融合显像通过人体组织的分子功能及CT复合影像,对眼眶及眼球肿瘤的良恶性质鉴别有重要参考价值;对肿瘤术后有无残留、复发和疗效监测有重要临床意义.