Rare Orbital Tumor Research Articles

Fibrolipoma of the orbit in a child

A 10-year-old girl presented to us with painless swelling of the left upper eyelid. She had a relatively ill-defined, non-tender mass in the superior orbit. Imaging revealed a relatively well defined hyperdense lesion in the anterior and superior orbit with no bony changes. The lesion was excised and a biopsy revealed a fibrolipoma. Pediatric orbital fibrolipomas are very rare orbital tumors and should considered in the differential diagnosis of an orbital space occupying lesion.

MR Imaging Characteristics of Solitary Fibrous Tumors of the Orbit

PurposeSolitary fibrous tumor (SFT) of the orbit is a rare tumor that was first described in 1994. We aimed to investigate its imaging characteristics that may facilitate the differential diagnosis between SFT and other types of orbital tumors.Material and MethodsMagnetic resonance imaging (MRI) data of patients with immunohistochemically confirmed orbital SFT from 2002 to 2022 at a tertiary care center were retrospectively analyzed. Tumor location, size, morphological characteristics, and contrast enhancement features were evaluated.ResultsOf the 18 eligible patients 10 were female (56%) with a mean age of 52 years. Most of the SFTs were oval-shaped (67%) with a sharp margin (83%). The most frequent locations were the laterocranial quadrant (44%), the extraconal space (67%) and the dorsal half of the orbit (67%). A flow void phenomenon was observed in nearly all cases (94%). On the T1-weighted imaging, tumor signal intensity (SI) was significantly lower than that of the retrobulbar fat and appeared predominantly equivalent (82%) to the temporomesial brain cortex, while on T2-weighted imaging its SI remained equivalent (50%) or slightly hyperintense to that of brain cortex. More than half of the lesions showed a homogeneous contrast enhancement pattern with a median SI increase of 2.2-fold compared to baseline precontrast imaging.ConclusionThe SFT represents a rare orbital tumor with several characteristic imaging features. It was mostly oval-shaped with a sharp margin and frequently localized in the extraconal space and dorsal half of the orbit. Flow voids indicating hypervascularization were the most common findings.

Endoscopic Endonasal Approach to Orbital Malignant Peripheral Nerve Sheath Tumor: A Minimally Invasive Method for Rare Orbital Tumor—Case Report and Review of Literature

AbstractMalignant peripheral nerve sheath tumors (MPNSTs) are uncommon tumors that rarely occur in the orbit. Malignant orbital tumors are always a challenge to remove completely. We present the case of a 42-year man with painful movement of the left eye and restriction of adduction of the left eye causing double vision. Magnetic resonance imaging (MRI) of the brain/orbit revealed intraconal tumor located inferomedial to the optic nerve. The imaging features were suggestive of schwannoma or cavernoma. Gross total resection of the tumor was done with an endoscopic endonasal intraconal approach. The histopathology revealed an MPNST. The patient received adjuvant radiotherapy and chemotherapy. His left eye adduction recovered completely and he had no local recurrence or systemic metastasis on follow-up evaluation.

Atypical lymphoid proliferation of the orbit.

Objective: Lymphoproliferative disorders are a group of lesions characterized by abnormal proliferation of lymphocytes. In the orbit, they can occur in the ocular adnexae. These neoplasms have defined clinical and pathologic characteristics and account for more than 20% of all orbital tumors. Several types of lymphoproliferative lesions have been described in the orbit. One example is lymphoid hyperplasia, which commonly involves the lacrimal gland. A benign lesion like lymphoid hyperplasia will show a general normal archetype of the tissues-involved lacrimal gland. We expect a polyclonal group of cells with more or less normal architecture of a follicle. On the other hand, lymphoma will show less organized arrangement of cells, and we expect them to be of monoclonal lineage.Methods: This is a case report of a 55-year-old Filipino female who came in for blurring of vision of both eyes. During her assessment, there was an incidental finding of bilateral upper eyelid swelling, and a 30x15 mm palpable firm mass under the right superior orbital rim and a 30x10 mm mass under the left were noted. The right globe was displaced inferiorly, but no proptosis was seen on exophthalmometry. On plain CT scan, we noted a homogenous mass with molding or contouring around the orbital structures. On coronal view, we noted homogenous masses that mold around the globe and recti, and this also confirmed on axial cuts. A section biopsy was done via anterior orbitotomy.Results: Our patient’s histopath had features of both. On scanning magnification, we noted a very cellular round cell tumor. The round cell lesion seemed to be reminiscent of a germinal center of a lymph node. Around it we noticed the glandular structures, which were expected, since this specimen was from the lacrimal gland. A closer view of the lesion on high power showed these lymphocytes within a germinal center. These are large macrophages that actively phagocytose apoptotic lymphoid cells in germinal centers. We expect to see a lot of them in benign hyperplasia, but only a few will be present in malignancies. In summary, our patient had both benign and malignant features, resulting in a histopath result of atypical lymphoid proliferation.Conclusion: Atypical lymphoid proliferation is a rare orbital tumor with benign and malignant features. There is no standard protocol for treatment, and proper multi-specialty coordination is important. External beam radiation therapy with linear accelerator (LINAC) appears to be an effective treatment, with no recurrence in our patient after 5 months.

Primary Lacrimal Gland Oncocytoma Associated With Hypervascularity: A Case Report With Imaging and Immunohistochemical Characterization

Oncocytoma is a rare benign neoplasm with few cases reported to involve the orbit. When affecting the orbit, oncocytomas have very slow growth and often evade early diagnosis. The diagnostic evaluation of these orbital tumors is not fully understood. The authors present a case of a benign oncocytoma arising in the lacrimal gland associated with significant vascularity and intraoperative bleeding. The authors provide immunohistochemical analysis of this rare orbital tumor and insights in surgical planning.

Clinical analysis of orbital ectopic meningiomas

Objective: To investigate the clinical features, imaging features, diagnoses and therapeutic regimens of orbital ectopic meningiomas. Methods: A retrospective study. All the clinical data of 10 patients (10 eyes) with orbital ectopic meningiomas were analyzed retrospectively from August 1999 to October 2016. They included imaging data such as color Doppler ultrasound, CT and MRI, and information of diagnosis, pathology, therapeutic regimen and prognosis. Results: The age of orbital ectopic meningiomas was 7 to 68 years old. There were 4 males and 6 females. The clinical manifestations were mainly with swollen eyelid (8 cases), exophthalmos (7 cases), visual impairment (2 cases) and accidentally found a mass in the orbit with physical examination(2 cases). The tumor was located in (5 cases) or outside (5 cases) the muscular funnel of the orbit. The imaging features of CT included the irregular shape, unclear border, and extraocular muscular adhesions (10 cases). T1 weighted image of MRI showed low and medium signals and T2 weighted image showed medium and high signals(8 cases). There was no definite diagnosis before surgery. Ten patients were treated with surgery, and it was confirmed intraoperatively that the tumors were not adjacent to the optic nerve and orbital periosteum. The pathological diagnoses were mostly epithelial meningiomas (9 cases). There were no significant changes in visual acuity, but temporary eye movement disorders occurred postoperatively (10 cases). The patients were followed for 6 months to 6 years. Two cases underwent recurrent at 3 months and 2 years after operation, and received treatment of γ-knife radiation therapy. The tumor reduced after γ-knife radiotherapy, and had no change in 2 years and 6 years, respectively. Conclusions: As a kind of rare orbital tumors, it is difficult to diagnose orbital ectopic meningiomas accurately. The clinical features and imaging findings can help to detect the disease, but lacking the characteristics of optic nerve sheath meningiomas and periosteal meningiomas. Surgical resection could achieve a good prognosis without visual impairment. If the tumor recurs after pathological diagnosis, γ-knife conformal radiation therapy may still be effective for tumor recurrence. (Chin J Ophthalmol, 2018, 54: 665-670).

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

ABSTRACTMesenchymal 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.

Therapieresistente Oberlidschwellung im Kindesalter

Swelling of the upper eyelid in childhood is caused by a variety of diseases and is very often generated by inflammation but orbital tumors should always be considered in the differential diagnostics. We report about a 4-year-old girl with a drug-resistant swelling of the upper eyelid and ptosis of the right eye. This case report demonstrates the route from initial clinical examination to diagnosis and additionally reviews the current status of therapeutic options. After magnetic resonance imaging (MRI) and diagnostic excision, Langerhans cell histiocytosis (LCH) could be histologically proven. Visual acuity and levator muscle function improved from 0.5 to 1.25 and from 2mm to 12mm, respectively, by amblyopic prophylaxis and immunosuppressive therapy. Persistent and drug-resistant swelling of the upper eyelid in childhood is also strongly suspicious for tumors. The suspicion of rare orbital tumors in children can be frequently substantiated by MRI. Biopsy and histological diagnosis are essential to plan adequate treatment and to estimate the prognosis. Particularly in Langerhans cell histiocytosis the methods of choice for over 10 years are specific immunochemical procedures (detection of protein s100 and CD1a).

Orbital hemangiopericytoma in an Asian population

Hemangiopericytoma is a very rare orbital tumor. The purpose of this study was to report the clinical and histopathological features of six cases of orbital hemangiopericytoma in an Asian population. Clinical and histopathological features were reviewed in six patients who were histopathologically confirmed as having primary orbital hemangiopericytoma in National Taiwan University Hospital between May 2001 and December 2010. Among the six cases who were diagnosed as having primary orbital hemangiopericytoma, all lesions were reported as vascular tumors and featured branching "staghorn appearance" vessels. All patients, including one male and five females, presented with progressive proptosis and some associated symptoms such as extraocular motility limitation with diplopia, displacement of the globe, afferent pupillary defect, congested vessels of conjunctiva, or decreased visual acuity. On computed tomography, the orbital tumors tended to manifest as circumscribed masses with homogeneous medium-to-high enhancement with contrast studies. All six patients received surgical treatments, and four of them had additional radiotherapy. Three patients had recurrence after surgeries, and one of them had multiple metastases to lung and liver. All patients were still alive after a follow-up period of 5-10 years. Orbital hemangiopericytoma has malignant potential, which may lead to local recurrence and/or metastasis. Histopathological findings alone are insufficient to predict the behavior of this tumor. Therefore, both clinical and histopathological findings are important to evaluate the treatment outcomes. Total excision accompanied with radiotherapy is suggested and long-term follow-up is required.

Orbital Paraganglioma

The authors report a case of orbital paraganglioma--an extremely rare orbital tumor. A 31-year-old woman presented a well-defined localized mass in the superotemporal extraconal aspect of the left orbit. Lateral orbitotomy of the left eye for excisional biopsy was performed, but the tumor could not be excised completely because of bleeding and involvement of critical structure. Histopathology and immunohistochemical analysis established the diagnosis. Gamma knife surgery was used as an alternative modality and resulted in stabilization of the tumor and preservation of her vision. We report a rare case of orbital paraganglioma, successfully managed with radiosurgery after incomplete excision.

Fractionated Stereotactic Radiation Therapy for Orbital Optic Nerve Sheath Meningioma – a Single Institution Experience and a Short Review of the Literature

Optic nerve sheath meningioma (ONSM) is a rare orbital tumor that generally induces a slow progressive visual loss in affected patients. Radiotherapy (RT) has currently become the first choice to treat ONSM. In this study our experience in ONSM treatment with fractionated stereotactic radiotherapy (FSRT) is reported. Five patients with diagnosis of orbital ONSM were treated between April 2007 and December 2009 at the Radiation Oncology department of our institution. All patients underwent history and physical, and ophthalmic examinations. Orbital MRI was performed before and 6 weeks after treatment; thereafter every 6 months for the first 2 years. By previous stereotactic localization of the target, RT was delivered with 28 daily fraction of 1.8 Gy by multiple non coplanar arcs dynamically conformed by a micro multileaf-collimator. At diagnosis, in all 5 patients, visual acuity limitations of different degrees were found, while exophthalmos was present in 2, diplopy in 2, orbital pain in 1, and proptosis in 1. In all patients pre-treatment MRI showed an orbital mass involving the optic nerve. After radiotherapy, previous symptoms improved in all patients. However, after RT the MRI consistently showed a stationary status compared to the MRI before RT. At a median follow up of 26 months (range 9-37) all patients had a subjective and/or objective better visual performance than before RT without any evidence of disease progression. No late side effects were recorded. Accordingly to the current literature, our experience confirms the efficacy and the safety of FSRT in patients with orbital ONSM.

Giant Cell Angiofibroma, A Variant of Solitary Fibrous Tumor, of the Orbit in a 16-Year-Old Girl

A 16-year-old girl presented with diplopia and gradual-onset, painless proptosis of the left eye. Orbital CT showed a well-circumscribed, enhancing, extraconal mass in the superior orbit, and the surgical excision was performed. Histopathology was interpreted as capillary hemangioma. Five years later, her symptoms recurred, and she was referred to the Oncology Service, Wills Eye Institute. Repeat orbital MRI showed a well-defined, extraconal mass with loculated areas of enhancement in the left orbit superonasally. Complete surgical excision was performed. Histopathologic examination showed benign, patternless spindle-cell proliferation with prominent intrinsic vascularity and multinucleated giant cells, consistent with giant cell angiofibroma, a variant of solitary fibrous tumor. There was intense immunoreactivity for CD34. After 20 months follow-up, there was no recurrence or development of metastasis. Giant cell angiofibroma, a variant of solitary fibrous tumor, is a rare orbital tumor that presents as a well-circumscribed, enhancing mass and can be found in children.

Phakomatous choristoma: A rare orbital tumor presenting as an eyelid mass with obstruction of the nasolacrimal duct

We describe a case of phakomatous choristoma presenting as a lower eyelid mass with nasolacrimal duct obstruction. The literature is reviewed and the unique aspects of the case are discussed.

Orbital solitary fibrous tumor: a clinicopathologic analysis

To study clinicopathologic features, histologic characteristics, differential diagnosis and the treatment of orbital solitary fibrous tumor (SFT). Clinical, radiographic and pathologic findings of 6 cases of SFT were retrospectively analyzed. Immunohistochemistry were performed on selected samples. Four patients were males and 2 were females. Patients age ranged from 19- to 57-years-old. The location of the tumor was in the muscle cone (case 1 and case 5), medial (case 3), lateral (case 4), superior (case 2) and inferolateral (case 6) portion of the orbit, respectively. The presenting symptom was proptosis in 3 cases and was mass of subconjunctival or orbit margin in other 3 cases. Image examination: SFT appeared as a round (case 6 showed irregular) and well-circumscribed parenchymatous mass that could be homogenously enhanced by contrast. Histologically, SFT displayed as a mass of spindle cells in an irregular arrangement Sometime, tumor cells could be storiform or sarciniform. Mitotic figures were infrequent and usually there were 0 to 3 mitotic figures per 10 high-power fields. Hyalinization and staghornform blood vessels were frequently observed. SFT was immunoreactive for markers such as Vim, CD34 and CD99. Two cases were recurred. SFT is a rare orbital tumor and could be confused with other types of orbital tumors. This tumor can be diagnosed by pathological and immunocytochemical studies, these characteristics can be used to differentiate it from other types of orbital tumors.

Solitary Fibrous Tumor of the Orbit: A Case Series

Purpose: Solitary fibrous tumor (SFT) occurs rarely in the orbit. We present four such cases, representing one of the largest case series reported to date of this rare orbital tumor. Method: The four patients ranged from ages 20 to 50, all of whom presented with lid swelling or bulging in the involved eye. All four patients underwent CT scan to confirm the diagnosis of an orbital mass, which was then excised. Results: The tumors of all four specimens contained spindle-shaped cells with bland nuclei and rare mitotic figures. They all stained positively with CD34 and vimentin. Resection margins were positive in two of the four cases. One of these cases demonstrated residual tumor on follow-up CT scan, which remained unchanged at one-year follow-up. No evidence of residual tumor was found in the other three cases, despite one having malignant pathology. Conclusion: SFT is a rare and generally benign tumor of the orbit. Immunohistochemical testing with CD34 is necessary to confirm the diagnosis. Although en bloc tumor resection is the definitive treatment, residual tumor may remain stable for some time. However, this must be followed closely if complete resection is not carried out.

MALT-Lymphom der Orbita

MALT lymphomas of the orbit are rare orbital tumors; the differential diagnosis needs to exclude inflammatory pseudotumors of the orbit, but also benign lymphoproliferations, pseudolymphomas, and other orbital neoplasms. After histological confirmation of the diagnosis staging is necessary, and - as long as the disease is localized exclusively in the orbit - radiation therapy should be started. The clinical picture of MALT lymphoma and its differential diagnosis and treatment are discussed with reference to an actual case.

Atypical lipoma: Well-differentiated liposarcoma of the orbit with dedifferentiated areas

Primary orbital liposarcoma is a very rare orbital tumor and dedifferentiation in the liposarcomas occurs rarely. Dedifferentiation is defined by development, in a well-differentiated tumor, of poorly differentiated, high-grade areas. The dedifferentiated liposarcomas (DDLS) have a better prognosis than pleomorphic sarcomas as a whole. The treatment of DDLS patients proves to be complicated due to the fact that the histopathological diagnosis usually becomes evident only after the surgery. In the present paper, the clinical presentation, histopathological features, radiological characteristics and clinical treatment of one case of well-differentiated liposarcoma with dedifferentiated areas, abruptly delimited from the well-differentiated adipocytic component, are reported.

Cytogenetic analysis of rare orbital tumors: further evidence for diagnostic implication

Cytogenetic analysis of rare orbital tumors: further evidence for diagnostic implication

Cytogenetic analysis of rare orbital tumors: further evidence for diagnostic implication

INTRODUCTION . The pathologic diagnosis, and hence the management, of rare tumors of the orbit can represent a challenge. An increasing number of tumors have been shown to display specific chromosomal abnormalities detectable by cytogenetic analysis. MATERIALS AND METHODS . Two examples of rare primary orbital tumors are presented in which the correct pathologic diagnosis was either established or confirmed by cytogenetic analysis. Small pieces of the tumor were obtained at the time of biopsy and placed immediately, in a sterile fashion, in fresh culture medium. The cells were Giemsa stained and processed for chromosome analysis. RESULTS . In the first case, a 12-year-old boy was diagnosed with a sino-orbital mass after sustaining minor trauma. On biopsy, the pathologic examination was at first consistent with a Burkitt-like lymphoma, until cytogenetic analysis showed a t(8;21)(q22;q22) translocation, specific for granulocytic sarcoma, which corresponds to the localized solid form of acute myeloblastic leukemia. Consequently, proper chemotherapy was re-oriented and remission was achieved. In the second case, a 2-year-old boy presented with a well-vascularized orbital tumor. The histopathologic diagnosis of alveolar soft-part sarcoma of the orbit was confirmed by the finding of a specific t(X;17)(p11;q25) translocation. After two unsuccessful courses of chemotherapy, wide excision surgery with primary reconstruction brought remission. CONCLUSIONS . Cytogenetic analysis should be considered in the diagnosis of any orbital tumor which is suspected of malignancy.

Metastatic Liposarcoma to the Orbit

To report a woman with blurred vision and proptosis who harbored an orbital liposarcoma that had metastasized from her abdomen. The patient underwent an orbital biopsy, followed by postoperative intravenous corticosteroids and radiation. Pathology showed a dedifferentiated liposarcoma. The patient's visual acuity improved from 20/200 to 20/50 after treatment. Physicians should suspect the presence of this rare orbital tumor in a person with a history of liposarcoma who has proptosis. Liposarcomas usually exhibit a higher grade of malignancy with recurrence, as shown in this patient.