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Myths in the Diagnosis and Management of Orbital Tumors.

Gündüz K, Yanık Ö - Middle East Afr J Ophthalmol (2015 Oct-Dec)

Bottom Line: Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future.This will enable targeted treatments even for diseases with the same histopathologic diagnosis.Phenotypic variability within the same disease will be addressed using targeted treatments.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Ankara University Faculty of Medicine, Ankara, Turkey.

ABSTRACT
Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments.

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A 31-year-old male with a 5 years history of proptosis in the left eye. (a) Facial photograph shows left proptosis. (b) T1-weighted magnetic resonance imaging demonstrates a cavitary mass in the left orbit. (c) T2-weighted magnetic resonance imaging shows fluid-fluid levels in the tumor. (d) Gross photo of the excised tumor shows yellowish hemorrhagic tumor fragments. (e) Histopathologic examination demonstrates schwannoma consisting mostly of spindle shaped tumor cells with elongated nuclei forming bundles and cellular areas without obvious mitosis (Antoni A pattern) (H and E, ×400). (f) Immunohistochemically, the tumor cells diffusely stain positive with S-100 (S-100, ×100)
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Figure 3: A 31-year-old male with a 5 years history of proptosis in the left eye. (a) Facial photograph shows left proptosis. (b) T1-weighted magnetic resonance imaging demonstrates a cavitary mass in the left orbit. (c) T2-weighted magnetic resonance imaging shows fluid-fluid levels in the tumor. (d) Gross photo of the excised tumor shows yellowish hemorrhagic tumor fragments. (e) Histopathologic examination demonstrates schwannoma consisting mostly of spindle shaped tumor cells with elongated nuclei forming bundles and cellular areas without obvious mitosis (Antoni A pattern) (H and E, ×400). (f) Immunohistochemically, the tumor cells diffusely stain positive with S-100 (S-100, ×100)

Mentions: A 27-year-old male presented with a 5 years history of left proptosis, loss of visual acuity, and diplopia [Figure 3a]. The patient had been followed elsewhere with a presumptive diagnosis of orbital lymphangioma. Surgery was not performed for years due to concerns of intraoperative hemorrhage for years. Orbital MRI findings revealed a cavitary mass with fluid-fluid levels occupying almost the entire orbit [Figure 3b and c]. A detailed search demonstrated that tumors producing fluid-fluid levels include lymphangioma, hemangioma, aneurysmal bone cyst, chondroblastoma, osteoblastoma, intracranial schwannoma, peripheral nerve schwannoma, and extracranial neck and head schwannomas.8910111213 Nontumoral causes include fibrous dysplasia, tumoral calcinosis, and myositis ossificans.813 We thought that the lesion could be a lymphangioma as suspected, but it could also represent one of the rarer histopathologic entities listed above. We proceeded with an anterior orbitotomy and performed a piecemeal subtotal tumor excision [Figure 3d]. The lesion was yellow-white in color, had intrinsic vascularity, was unencapsulated and with a fragile structure during surgical dissection. Histopathology findings and immunohistopathology were consistent with schwannoma [Figure 3e and f]. This case illustrates that a high index of suspicion is required for imaging findings thought to represent the classic appearance of certain tumors.


Myths in the Diagnosis and Management of Orbital Tumors.

Gündüz K, Yanık Ö - Middle East Afr J Ophthalmol (2015 Oct-Dec)

A 31-year-old male with a 5 years history of proptosis in the left eye. (a) Facial photograph shows left proptosis. (b) T1-weighted magnetic resonance imaging demonstrates a cavitary mass in the left orbit. (c) T2-weighted magnetic resonance imaging shows fluid-fluid levels in the tumor. (d) Gross photo of the excised tumor shows yellowish hemorrhagic tumor fragments. (e) Histopathologic examination demonstrates schwannoma consisting mostly of spindle shaped tumor cells with elongated nuclei forming bundles and cellular areas without obvious mitosis (Antoni A pattern) (H and E, ×400). (f) Immunohistochemically, the tumor cells diffusely stain positive with S-100 (S-100, ×100)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4660525&req=5

Figure 3: A 31-year-old male with a 5 years history of proptosis in the left eye. (a) Facial photograph shows left proptosis. (b) T1-weighted magnetic resonance imaging demonstrates a cavitary mass in the left orbit. (c) T2-weighted magnetic resonance imaging shows fluid-fluid levels in the tumor. (d) Gross photo of the excised tumor shows yellowish hemorrhagic tumor fragments. (e) Histopathologic examination demonstrates schwannoma consisting mostly of spindle shaped tumor cells with elongated nuclei forming bundles and cellular areas without obvious mitosis (Antoni A pattern) (H and E, ×400). (f) Immunohistochemically, the tumor cells diffusely stain positive with S-100 (S-100, ×100)
Mentions: A 27-year-old male presented with a 5 years history of left proptosis, loss of visual acuity, and diplopia [Figure 3a]. The patient had been followed elsewhere with a presumptive diagnosis of orbital lymphangioma. Surgery was not performed for years due to concerns of intraoperative hemorrhage for years. Orbital MRI findings revealed a cavitary mass with fluid-fluid levels occupying almost the entire orbit [Figure 3b and c]. A detailed search demonstrated that tumors producing fluid-fluid levels include lymphangioma, hemangioma, aneurysmal bone cyst, chondroblastoma, osteoblastoma, intracranial schwannoma, peripheral nerve schwannoma, and extracranial neck and head schwannomas.8910111213 Nontumoral causes include fibrous dysplasia, tumoral calcinosis, and myositis ossificans.813 We thought that the lesion could be a lymphangioma as suspected, but it could also represent one of the rarer histopathologic entities listed above. We proceeded with an anterior orbitotomy and performed a piecemeal subtotal tumor excision [Figure 3d]. The lesion was yellow-white in color, had intrinsic vascularity, was unencapsulated and with a fragile structure during surgical dissection. Histopathology findings and immunohistopathology were consistent with schwannoma [Figure 3e and f]. This case illustrates that a high index of suspicion is required for imaging findings thought to represent the classic appearance of certain tumors.

Bottom Line: Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future.This will enable targeted treatments even for diseases with the same histopathologic diagnosis.Phenotypic variability within the same disease will be addressed using targeted treatments.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Ankara University Faculty of Medicine, Ankara, Turkey.

ABSTRACT
Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments.

Show MeSH
Related in: MedlinePlus