Ossifying fibroma of the lateral orbital wall in an adult

Orbital ossifying fibroma is an extremely rare condition, especially in adults. This rare tumor mostly develops at the medial wall of the orbit. We report a histopathologically confirmed ossifying fibroma involving the lateral orbital wall in an adult. This case suggests that orbital ossifying fibroma should be considered in the differential diagnosis of orbital bony lesions in adults and that unusual presentations may occur.     

Review of Lateral Orbital Wall Ossifying Fibroma

Significant histological overlap exists between fibro-osseous lesions and diagnosis is made on a clinicopathological basis. Ossifying fibroma is a benign fibro-osseous neoplasm of the jaw and craniofacial complex that has generated a degree of controversy regarding diagnosis and classification, especially with respect to the psammomatoid variant. Orbital lesions mainly arise from the paranasal sinuses affecting the medial or inferior orbital wall. Lateral orbital wall ossifying fibroma is, therefore, a rare condition with only a single previous case report. We present a second case of lateral orbital wall ossifying fibroma and a review of the associated literature.     

Review of lateral orbital wall ossifying fibroma

Significant histological overlap exists between fibro-osseous lesions and diagnosis is made on a clinicopathological basis. Ossifying fibroma is a benign fibro-osseous neoplasm of the jaw and craniofacial complex that has generated a degree of controversy regarding diagnosis and classification, especially with respect to the psammomatoid variant. Orbital lesions mainly arise from the paranasal sinuses affecting the medial or inferior orbital wall. Lateral orbital wall ossifying fibroma is, therefore, a rare condition with only a single previous case report. We present a second case of lateral orbital wall ossifying fibroma and a review of the associated literature.     

Psammomatoid ossifying fibroma of the orbit.

Psammomatoid ossifying fibroma of the orbit is a distinctive solitary fibro-osseous lesion of the orbital bones that histologically has characteristic small, round structures resembling psammoma bodies within a benign spindle cell stroma. We describe a 19-year-old woman with this uncommon orbital tumour.     

Ossifying fibroma: a rare cause of orbital inflammation

PURPOSE: To describe the clinical and radiologic features of 4 cases of ossifying fibroma affecting the orbit and to review the literature on orbital involvement by the tumor. METHODS: Small case series. RESULTS: Four patients (3 children and 1 adult) with ossifying fibromas invading the orbit were examined. Two of the 3 children were examined for ossifying fibromas on the orbital roof. One had the psammomatoid form of the disease and the other the trabecular variant. Despite striking differences in the histologic pattern and in the radiologic appearance of the lesions, both children displayed a significant degree of orbital inflammation mimicking orbital cellulitis. The third child and the adult patient had the orbit involved by trabecular ossifying fibromas invading the orbital floor. The tumor of the adult clearly originated in the maxilla, filled the maxillary sinus, and eroded the orbital floor. The tumor of the third child occupied the maxillary, ethmoid, and sphenoid sinuses. In both cases, the clinical presentation was painless eye dystopia and proptosis. CONCLUSIONS: Regardless of the histologic pattern (trabecular or psammomatoid), ossifying fibromas can induce a substantial degree of orbital inflammation in children and must be included in the differential diagnosis of acute orbital inflammation during childhood.     

Massive juvenile ossifying fibroma of maxillary sinus with orbital involvement.

A 14-year-old black girl had painless, progressive proptosis and upward displacement of the left eye for approximately six months. Computed tomography showed a massive tumour involving the maxillary sinus with extension into the sphenoid sinus, ethmoid sinus, and orbit. The mass was excised surgically and proved histopathologically to be a juvenile ossifying fibroma. The clinical and histopathological features of this uncommon orbital tumour are discussed and its place in the spectrum of fibro-osseous tumours of the orbital region is considered.     

Psammomatoid ossifying fibroma of the ethmoid

We present a 16 year-old girl who suffered since one year of a painless slow growing mass on the left medial orbital rim causing globe displacement. CT-scan and MRI of the orbit with T1 and T2 weighted images showed the presence of a large mucocoele in the frontal sinus. This occurred secondary to the obstruction of the sinonasal tract by a bony tumour. Histopathology showed a lesion consisting of fibrous tissue and ossicles or psammomatoid bodies. The diagnosis of a psammomatoid ossifying fibroma (POF) was made.     

Subperiosteal fibroma of the orbit

Fibromas are benign, well-circumscribed tumors that are characterized as spindle-cell lesions with interlacing fibrous stroma. Here, we describe the clinical presentation, management, and outcome of a patient with an orbital fibroma. To our knowledge, this is the second case report of a biopsy-proven subperiosteal orbital fibroma to date.     

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。     

Aneurysmal bone cyst secondary to ossifying fibroma in the orbit

A 28-year-old man presented with proptosis and medial displacement of the right eye, intermittent pain, and diplopia of 1 month's duration. Radiologic examination demonstrated a mass in the right lateral orbit resembling an aneurysmal bone cyst (ABC). Histopathologic evaluation of the surgically excised lesion via orbitotomy confirmed the radiologic diagnosis. Two months later, the patient presented again with a mass at the same location as the previous one, with a major component that was solid. The recurrent lesion was excised via frontal craniotomy. Histopathologic examination of the surgical specimen led to a diagnosis of ABC secondary to trabecular ossifying fibroma. During the follow up of 13 months, the lesion did not recur. To the best of the authors' knowledge, this is the first case of ABC secondary to ossifying fibroma in the orbit. ABC can dominate the radiologic appearance of the lesion and veil the primary neoplasm.     

Multidisciplinary Management of Adult Orbital Rhabdomyosarcoma*

ABSTRACT

We report the case of a 52-year-old man who presented with a 10-day history of right eye and eyelid inflammation and intermittent diplopia following blunt trauma to the right eyebrow.

The CT and MRI scans revealed an extraconal soft tissue mass on the orbital floor with maxillary and ethmoid sinus wall destruction, which on orbital biopsy was proven to be an Alveolar Rhabdomyosarcoma. The patient had a central retinal vein occlusion due to mass effect that resulted in total visual loss at 2 months. He was referred to oncologists who treated him according to the paediatric RMS protocol and is still in remission at 2-year follow-up. Rhabdomyosarcoma is a rare tumour in adults which requires multi-disciplinary management. This highlights the necessity of considering rhabdomyosarcoma in the differential diagnosis of orbital tumours in any age group.     

Primary orbital leiomyoma and leiomyosarcoma

A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.     

A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult

PURPOSE: A case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult. METHODS: A 48-year-old male patient presented at our clinic complaining of proptosis that had persisted for 2 weeks in his left eye. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle. Endoscopic intranasal biopsy revealed alveolar RMS. Conservative debulking and orbital wall decompression were performed. RESULTS: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS. Since ipsilateral cervical and spinal metastasis was detected, systemic treatment was administered simultaneously. CONCLUSIONS: Although rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors. Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.     

Atypical Rosai-Dorfman Disease with Lacrimal Gland Involvement

Abstract

Purpose: Rosai-Dorfman disease is a rare, benign, idiopathic histocytic proliferative disorder that typically presents in young adults with painless cervical lymphadenopathy. Here we report an atypical case of Rosai-Dorfman disease involving orbit tissue and lacrimal gland, unilaterally. Case: A 69-year-old Asian women developed a painless palpable mass with local edema over the left upper eyelid over several months. Computed tomography (CT) showed an orbital mass with homogenous soft tissue density over the left lacrimal gland and superior orbital area. The patient underwent complete excision of the orbital tumor. Observations: The histopathology revealed diffuse and nodular infiltrations of S-100 positive histiocytes, plasma cells and lymphocytes. Emperipolesis (lymphocytophagocytosis) was also noted. These findings were consistent with Rosai-Dorfman disease. Chest CT revealed hilar lymphadenopathy. Three months after excision of the orbital mass, the patient developed lymphadenopathy in the extremities that resolved spontaneously over a few weeks. There were no complications or recurrence without systemic treatment after the complete excision. Conclusions: Orbital Rosai-Dorfman disease is a rare disorder, especially in Asia. Though there is no consensus on therapeutic choices, including corticosteroids, chemotherapy, radiation therapy, and surgical excision, the complete surgical excision performed in this case was without complication and had a favorable outcome.     

Solitary fibrous tumor of the orbit: a clinicopathologic study of six cases with review of the literature

Solitary fibrous tumor of the orbit is a rare spindle cell neoplasm. There are 42 cases of solitary fibrous tumor of the orbit available in the literature. We present six more cases of orbital solitary fibrous tumors, which presented to our institute between 1999 and 2001. We highlight the need for clinical recognition of these tumors as a distinct entity and inclusion of this tumor in the etiological differential diagnosis of well-circumscribed orbital lesions presenting as unilateral proptosis in both children and in adults. The diagnosis may be suspected based on radiological features supported by histopathologic and immunohistochemical study. The strong CD34 immunoreactivity of this tumor supports its diagnosis. Complete surgical resection is the most important prognostic factor of this tumor.     

甲状腺相关性眼病内镜微创减压术的历史与展望

甲状腺相关性眼病是成人最常见的眼眶疾病,眶减压手术是其重要的治疗手段。随着内窥镜技术的发展,内窥镜下眶减压手术逐渐成为其首选的手术方案。现将重点介绍内窥镜技术在眼眶内侧壁、外侧壁以及下壁减压中的应用进展,并介绍其手术效果以及并发症的预防与处理。同时介绍内窥镜下经筛径路眶减压手术在甲状腺相关性眼病视神经病变中的手术进展。     

泪道孤立性纤维瘤特征及治疗

孤立性纤维瘤是一种罕见的起源于成纤维细胞间充质的肿瘤。最早报道于胸膜，近年来对胸膜外部位的孤立性纤维瘤病例报道越来越多，有报道该肿瘤几乎可发生于所有部位。泪道系统的结构功能有其特殊性，且目前关于泪道系统的孤立性纤维瘤报道仍然较少，故本文总结现有泪道孤立性纤维瘤的报道，以分析泪道这一疾病的临床特征和治疗方法。     

Pyogenic Granuloma Presenting as an Orbital Mass

Pyogenic granuloma (PG) of the eyelid and orbit is typically associated with trauma or surgery. We report a rare case of an orbital intraconal PG arising de novo in association with an orbital artery.     

Solitary extramedullary plasmacytoma of the maxillary antrum and orbit presenting as acute bacterial orbital cellulitis.

Orbital involvement by plasma cell tumours is rare. Orbital tumours do not generally present as an acute orbital inflammatory disease in adults, though tumours such as rhabdomyosarcoma may cause clinical signs similar to an acute orbital cellulitis in children. We describe a patient with bacterial orbital cellulitis and sinusitis who was found to have an extra-medullary plasmacytoma of the maxillary antrum and orbit and coexisting testicular seminoma.