MyMedR

Fulltext Metastatic malignant schwannoma of orbit: a case report

Jaais F, Sivanesan S

Med J Malaysia, 1986 Dec;41(4):356-60.

A case of metastatic malignant schwannoma of the right orbit is presented here. Although the patient had disseminated disease, it was the eye problems which were striking and most symptomatic.
The orbital lesion was at first thought to be the primary tumour until a history of an excised right wrist mass was probed into and the histology traced.
This report is written as malignant schwannoma is an uncommon tumour. The difficulty in diagnosing malignant schwannoma is mentioned. The importance of diagnosis of an orbital metastatic lesion is also pointed out as this is often misdiagnosed.

Matched MeSH terms: Orbital Neoplasms/pathology; Orbital Neoplasms/secondary*

Fulltext Orbital myxoma--an unusual cause of inferoorbital mass

Ting SL, Koay CL, Ngo CT, Chieng LL, Chua CN

Med J Malaysia, 2010 Sep;65(3):224-6.

PMID: 21939174

To describe a case of isolated infraorbital mass which had been present for the past 9 years in a young woman. Despite the size, the mass was successfully excised in total.

Matched MeSH terms: Orbital Neoplasms/pathology; Orbital Neoplasms/radiography; Orbital Neoplasms/surgery*

Infantile malignant hemangiopericytoma of the orbit

Arshad AR, Normala B

Ophthalmic Plast Reconstr Surg, 2008 3 22;24(2):147-8.

PMID: 18356724 DOI: 10.1097/IOP.0b013e31816746b4

A 5-month-old girl presented with a soft-tissue mass over the lateral aspect of her right eyebrow. Surgical resection revealed a diagnosis of infantile malignant hemangiopericytoma. The mass recurred after excision and reconstruction. The patient died 4 months later with brain and lung metastasis.

Matched MeSH terms: Orbital Neoplasms/pathology*; Orbital Neoplasms/therapy

Benign chondroid syringoma of the orbit: an atypical location

Yap JF, Madatang A, Hanafi H

Orbit, 2020 Apr;39(2):135-138.

PMID: 31023118 DOI: 10.1080/01676830.2019.1604766

A 31-year-old male with no known medical illness presented with painless left eye protrusion for the past 2 years. Radiographic features were of an extraconal superior orbital mass with no invasion of adjacent structures. The patient underwent an excisional biopsy of the left superior extraconal mass a week later. The histopathological examination revealed a well-circumscribed lobulated mass with chondroid and myxoid stroma enveloping benign bland-appearing epithelial and myoepithelial cells. These pathological features were consistent with benign chondroid syringoma of the orbit. The patient had an uneventful recovery with no active ocular complaint post-operatively.Despite chondroid syringoma of the orbit being a rare tumour, it should be considered in the differential diagnosis of periorbital masses. Excision of the tumour remains the treatment of choice with histopathological examination as the gold standard of diagnosis. Regular follow-up is warranted in view of possible local recurrence or metastasis.

Matched MeSH terms: Orbital Neoplasms/pathology; Orbital Neoplasms/surgery*

Fulltext Adult embryonal rhabdomyosarcoma of the ethmoid: a rare entity

Rohaizam J, Doris EJ, Tang PI, Lee SC, Uchang J

Med J Malaysia, 2010 Jun;65(2):160-1.

PMID: 23756807 MyJurnal

Embryonal rhabdomyosarcoma is an exceedingly rare tumor in adult. We report an embryonal rhabdomyosarcoma of the ethmoid in a 59-year-old Iban lady who presented with proptosis and complete ptosis of her left eye for two months. Imaging investigations showed left ethmoidal and left orbital soft tissue mass with extradura and dura involvement. The patient was planned for chemotherapy. Unfortunately, in such an advanced disease, she succumbed before treatment.

Matched MeSH terms: Orbital Neoplasms

Fulltext A Rare Case of Aggressive, Huge Primary Orbital Lymphoma with Intracranial Extension and Bone Invasion

Sriram PR

Asian J Neurosurg, 2017 11 9;12(4):766-768.

PMID: 29114307 DOI: 10.4103/1793-5482.185055

Primary orbital lymphoma is a rare entity with only 1% of extranodal lymphomas. They usually present to ophthalmologist and surgical reserved for biopsy or tissue diagnosis. We present a patient who was referred to neurosurgery for a rapid growing orbital lymphoma. It grows from a small nodule in the eyelid to a huge, aggressive, disfiguring lesion invading bone and dura with intracranial extension within 3 months. The patient was treated with total surgical excision followed by systemic chemotherapy.

Matched MeSH terms: Orbital Neoplasms

Endoscopic transnasal approach to medial orbital lesions

Tan SH, Prepageran N

J Laryngol Otol, 2015 Sep;129(9):928-31.

PMID: 26193981 DOI: 10.1017/S0022215115001838

This case report presents our experience of endoscopic transnasal management of medial intra- and extraconal lesions.

Matched MeSH terms: Orbital Neoplasms/diagnosis; Orbital Neoplasms/secondary*; Orbital Neoplasms/surgery*

Benign orbital apex tumors treated with multisession gamma knife radiosurgery

Goh ASC, Kim YD, Woo KI, Lee JI

Ophthalmology, 2013 Mar;120(3):635-641.

PMID: 23149128 DOI: 10.1016/j.ophtha.2012.08.015

OBJECTIVE: The orbital apex is an important anatomic landmark that hosts numerous critical neurovascular structures. Tumor resection performed at this complex region poses a therapeutic challenge to orbital surgeons and often is associated with significant visual morbidity. This article reports the efficacy and safety of multisession gamma knife radiosurgery (GKRS) in benign, well-circumscribed tumors located at the orbital apex.

DESIGN: Retrospective interventional case series.

PARTICIPANTS: Five patients with visual disturbances resulting from a benign, well-circumscribed orbital apex tumor (3 cases of cavernous hemangioma and 2 cases of schwannoma).

METHODS: Each patient treated with GKRS with a total radiation dose of 20 Gy in 4 sessions (5 Gy in each session with an isodose line of 50%) delivered to the tumor margin.

MAIN OUTCOME MEASURES: Best-corrected visual acuity, visual field changes, orbital imaging, tumor growth control, and side effects of radiation.

RESULTS: All patients demonstrated improvement in visual acuity, pupillary responses, color vision, and visual field. Tumor shrinkage was observed in all patients and remained stable until the last follow-up. No adverse events were noted during or after the radiosurgery. None of the patients experienced any radiation-related ocular morbidity.

CONCLUSIONS: From this experience, multisession GKRS seems to be an effective management strategy to treat solitary, benign, well-circumscribed orbital apex tumors.

Matched MeSH terms: Orbital Neoplasms/pathology; Orbital Neoplasms/surgery*

Fulltext Secondary mucoepidermoid carcinoma of the orbit

Siuw CP, Tan SW, Abdul Wahid AB, Vasudevan S

Indian J Ophthalmol, 2016 Mar;64(3):238-41.

PMID: 27146939 DOI: 10.4103/0301-4738.181748

A 40-year-old man presented with right eye axial proptosis and ophthalmoplegia for 3 months. Imaging study showed a right intraconal mass with the erosion of the orbital floor. Incisional biopsy revealed mucoepidermoid carcinoma. Nasal endoscopy was normal and systemic tumor screening was negative for a primary source. The patient underwent right orbital exenteration, uncinectomy, nasal and maxillary mucosal biopsy. Malignant cells were found present in the mucosa of maxillary sinus roof and uncinate bone. The postoperative positron emission tomography scan showed residual active lesion in right orbital apex and maxilla but no primary lesion elsewhere. The patient subsequently underwent 35 cycles of postoperative radiotherapy. Primary mucoepidermoid carcinoma of the orbit is rare and typically arises from the lacrimal gland or sac. Those tumors not arising from lacrimal apparatus should be presumed metastatic in origin, and the thorough systemic survey should be undertaken in the search for the primary tumor.

Matched MeSH terms: Orbital Neoplasms/diagnosis*; Orbital Neoplasms/surgery

Orbital multiple myeloma: a diagnostic challenge

Tai E, Sim SK, Haron J, Wan Hitam WH

BMJ Case Rep, 2017 Aug 07;2017.

PMID: 28790098 DOI: 10.1136/bcr-2017-220895

Orbital involvement in multiple myeloma is unusual. We describe the case of an 85-year-old woman who presented with right eye proptosis, reduced visual acuity and diplopia. Computed tomography showed a lobulated, enhancing soft tissue mass arising from the right greater wing of the sphenoid with intraconal, lacrimal gland and ocular muscle involvement. Histopathology revealed predominantly atypical plasma cells in a background of reactive lymphocytes, with monoclonality towards kappa light chain protein, suggestive of multiple myeloma. This case illustrates the diagnostic imaging challenge of orbital multiple myeloma.

Matched MeSH terms: Orbital Neoplasms/diagnosis*; Orbital Neoplasms/pathology

Recurrence of multiple myeloma with soft tissue plasmacytoma presenting as unilateral proptosis

Pan SW, Wan Hitam WH, Mohd Noor RA, Bhavaraju VM

Orbit, 2011 Mar;30(2):105-7.

PMID: 21322793 DOI: 10.3109/01676830.2010.546553

To describe a rare case of soft tissue plasmacytoma of the orbit presenting with proptosis.

Matched MeSH terms: Orbital Neoplasms/diagnosis*; Orbital Neoplasms/radiotherapy

Orbitofacial dermatofibrosarcoma protuberans with intranasal extension

Sharudin SN, Tan SW, Mohamad NF, Vasudevan SK, Khairan H, Mun YC, et al.

Orbit, 2018 Jun;37(3):196-200.

PMID: 29058523 DOI: 10.1080/01676830.2017.1383474

A 25-year-old Chinese woman presented with recurrent painless swelling over the left medial canthus region for 3 months and intranasal mass for an indeterminate duration. Initial incision biopsy of the mass was reported as nodular fasciitis but the lesion recurred 3 weeks later. Intraoperative findings during repeat biopsy showed a mass extending from the deep dermal tissue into the anterior orbit and polyp-like nasal mass. Histopathology findings were that of dermatofibrosarcoma protuberans (DFSP). The mass recurred 4 months later without orbital or intranasal recurrence. Wide excision biopsy under frozen section guidance was attempted however; clear surgical margins could not be achieved despite extensive resection. She was subsequently referred for adjuvant radiotherapy. We report an exceptionally rare case of local recurrence of DFSP in an unusual anatomic location. This case was surgically challenging in achieving negative margins, and thus neoadjuvant therapy may improve overall outcome to prevent local relapse.

Matched MeSH terms: Orbital Neoplasms/pathology*; Orbital Neoplasms/radiotherapy

Extensive facial and orbital infantile hemangiomas associated with high intraocular pressure

Shatriah I, Norazizah MA, Wan-Hitam WH, Wong AR, Yunus R, Leo SW

Pediatr Dermatol, 2013 Jan-Feb;30(1):151-4.

PMID: 22329437 DOI: 10.1111/j.1525-1470.2011.01618.x

High intraocular pressure is a rare ophthalmic condition associated with infantile hemangiomas that involves the orbit, eyelid, or both. Here, we describe a patient with extensive facial and orbital infantile hemangiomas associated with high intraocular pressure in the affected eye. The prompt management of this challenging condition is essential.

Matched MeSH terms: Orbital Neoplasms/complications; Orbital Neoplasms/diagnosis*; Orbital Neoplasms/drug therapy

Fulltext Proptosis: a rare presentation of metastatic renal cell carcinoma

Ho CC, Krishna KK, Praveen S, Goh EH, Lee BC, Zulkifli MZ

Med J Malaysia, 2010 Sep;65(3):229-30.

PMID: 21939176

We present a case of a middle-aged man who was incidentally found to have right renal solid mass while investigating for his left eye proptosis. Computerised tomography (CT) scan confirmed the diagnosis of renal cell carcinoma and the tumour was successfully excised via open surgery. The histopathology examination revealed the 10x7x8 cm mass to be a clear cell type renal cell carcinoma. The rare presentation of this metastatic renal cell carcinoma, its diagnosis and management will be discussed.

Matched MeSH terms: Orbital Neoplasms/complications; Orbital Neoplasms/radiography; Orbital Neoplasms/secondary*

Orbital rhabdomyosarcoma in an HIV positive child

Khairy-Shamel ST, Shatriah I, Adil H, Zunaina E, Bakiah S, Rohaizan Y, et al.

Orbit, 2008;27(5):388-90.

PMID: 18836940 DOI: 10.1080/01676830802336629

We reported a case of orbital rhabdomyosarcoma with an intracranial extension in an HIV-infected child. It was an uncommon sarcoma in a retroviral-positive patient that resulted in a diagnostic and therapeutic dilemma. The child is currently asymptomatic following surgery, chemotherapy, and reinstitution of highly active retroviral therapy (HAART).

Matched MeSH terms: Orbital Neoplasms/pathology*; Orbital Neoplasms/radiography; Orbital Neoplasms/surgery

Development of the international orbital Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) staging system

El Rassi E, Adappa ND, Battaglia P, Castelnuovo P, Dallan I, Freitag SK, et al.

Int Forum Allergy Rhinol, 2019 07;9(7):804-812.

PMID: 30809970 DOI: 10.1002/alr.22316

BACKGROUND: Orbital cavernous hemangiomas (OCH) are the most common adult orbital tumor and represent an ideal index lesion for endonasal orbital tumor surgery. In order to standardize outcomes reporting, an anatomic-based staging system was developed.

METHODS: An international, multidisciplinary panel of 23 experts in orbital tumor surgery was formed. A modified Delphi method was used to develop the cavernous hemangioma exclusively endonasal resection (CHEER) staging system with a total of 2 rounds being completed.

RESULTS: Tumors medial to a plane along the long axis of the optic nerve may be considered amenable for an exclusively endonasal resection. In select cases, tumors may extend inferolaterally if the tumor remains below a plane from the contralateral naris through the long axis of the optic nerve (ie, plane of resectability [POR]). This definition reached consensus with 91.3% of panelists in agreement. Five stages were designed based on increasing technical resection difficulty and potential for morbidity. Stages were based on the relationship of the tumor to the extraocular muscles, the inferomedial muscular trunk of the ophthalmic artery (IMT), and orbital foramina. Staging by anatomic location also reached consensus with 87.0% of panelists in agreement. Size was not included in the staging system due to the lack of agreement on the contribution of size to resection difficulty.

CONCLUSION: Endoscopic orbital tumor surgery is a nascent field with a growing, yet heterogeneous, body of literature. The CHEER staging system is designed to facilitate international, high-quality, standardized studies establishing the safety, efficacy, and outcomes of endonasal resection of OCH.

Matched MeSH terms: Orbital Neoplasms

Fulltext Orbital rhabdomyosarcoma: a case series

Chen, C.Y., Nor Hidayah, A.B., Adil, H.

Malaysian Journal of Medicine and Health Sciences, 2013;9(2):69-72.

MyJurnal

In this paper, we report three cases of orbital rhabdomyosarcoma in different age groups with different histopathologic types. Case 1 is a 10-year old Malay boy who presented with painless proptosis of the left eye. Magnetic resonance imaging (MRI) showed soft tissue mass arising from the lateral rectus muscle. Tissue biopsy revealed rhabdomyosarcoma of embryonal type. Case 2 is a 32-year old Malay man who presented with a progressive left eye proptosis associated with pain and redness. Computed Tomography (CT) scan showed a retrobulbar mass extending medially in the left orbit. Meanwhile, biopsy showed rhabdomyosarcoma of pleomorphic type. Case 3 is a 67-year old Malay woman who presented with proptosis and fungating growth of left orbital region, epistaxis and nasal blockage. CT scan revealed an ill-defined mass filling up the left orbital cavity. Biopsy showed rhabdomyosarcoma of alveolar type. Rhabdomyosarcoma is the most common in childhood but it should be considered as a differential diagnosis of orbital tumours irrespective of age.

Matched MeSH terms: Orbital Neoplasms

Fulltext A Clinicopathologic Study of Seven Cases of Orbital Solitary Fibrous Tumours

Ting XW, Sothiraghagan S, W Md Kasim WM, Muhammed J

Cureus, 2020 May 24;12(5):e8259.

PMID: 32596077 DOI: 10.7759/cureus.8259

Objective To describe the patient demographics, clinical findings, investigations, surgical outcomes, and histopathological findings of seven cases of orbital solitary fibrous tumours. Method This was a retrospective review of seven cases of orbital solitary fibrous tumour, which were followed up in Hospital Serdang, a national oculoplastic centre, from years 2008-2017. Results This study included seven patients with ages between 21 and 35 years old; two were males and five were females. All seven patients presented with painless chronic unilateral proptosis. Radiological imaging of the orbit showed a localized contrast enhancing intraorbital mass. All patients underwent orbitotomy and excisional biopsy. Intraoperative findings showed a well-encapsulated and vascularized mass. Histological findings of spindle-shaped cells were noted. All cases had positive staining for cluster of differentiation (CD) 34, five were positive for CD 99, four were positive for B-cell lymphoma (BCL-2), and five patients had positive staining for S-100. Three of the patients did not have clear margins during the primary operation and subsequently had a recurrence within two years. Conclusion A solitary fibrous tumour is a rare mesenchymal tumour with a pleural origin. The orbit is the most common extrapleural site of the tumour and they are usually benign. Immunohistochemistry is important to differentiate it from other, more aggressive forms of orbital tumours. Regular follow-up is important to monitor for recurrence.

Matched MeSH terms: Orbital Neoplasms

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