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Tumor Discovery Inflammatory orbital pseudotumors

always be thorough to eliminate any plausible association ischemic type. In cases of resistance or recurrence, a repeat
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with systemic or other diseases. anatomopathological examination is warranted, as certain
Fine-needle aspiration biopsies are used in cases of forms may transition to lymphoma. 17
suspected metastases, although larger fragments are The therapeutic approaches for IDO are diverse and
preferred for lymphoma studies, limiting the applicability well-documented in published series. Steroids are the
of this method. Histology remains the gold standard in mainstay of treatment, offering an excellent response.
paraclinical explorations. However, its indications remain However, despite the success of low-dose radiation
limited to cases with progressive neurological deficits, lack therapy in managing recurrent myositis and optic nerve
of response to steroids, or persistent imaging abnormalities. decompression, documented recurrences underscore its
In Case 2, the ophthalmologists refrained from limitations. Calcineurin inhibitors such as cyclosporine or
performing an orbital tumor biopsy due to its close tacrolimus have demonstrated efficacy, with cyclosporine
relationship with the optic nerve. emerging as a viable option for corticosteroid-intolerant
diabetic patients. Antimetabolites (cyclophosphamide,
Furthermore, to the best of our knowledge,
the association between hemolytic anemia and an azathioprine, and methotrexate) have proven effective
in several published series, although requiring regular
inflammatory orbital pseudotumor has been described in biological monitoring to monitor side effects. Monoclonal
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only one case. The clinical and radiological response to
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corticosteroid therapy after 6 months was favorable. antibodies have also shown promise. Infliximab, when
used alone or in combination with methotrexate, yielded
In general, imaging modalities (PET scan and MRI) reveal favorable results. Rituximab, administered either
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different types of involvement, encompassing structures such intravenous weekly at a dosage of 375 mg/m or at 1000 mg
2
as lacrimal glands, oculomotor muscles, optic nerve, sclera, on days 0 and 15, has exhibited efficacy in refractory cases
episclera, Tenon’s capsule, uvea, and orbital fat. In addition, unresponsive to steroids, radiosurgery, or conventional
extensions into intracranial structures through the cavernous treatments, resulting in positive clinical responses.
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sinus and the middle cranial fossa represent the two most While tocilizumab has demonstrated effectiveness in
common locations. This distinction aids in categorizing the the treatment of systemic juvenile idiopathic arthritis
disease as localized or diffuse. Our three cases reflect the and rheumatoid arthritis, its use in IDO lacks published
different involvement of the orbital structures. evidence. 21
Mombaerts cases reflect the different ins used to
describe the different histological forms of IDO. The 4. Conclusion
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classic form is characterized by polymorphic inflammation Inflammatory diseases of the orbit are a highly
and a polymorphic infiltrate comprising lymphocytes, heterogeneous group requiring meticulous clinical
plasma cells, macrophages, eosinophils, and neutrophils. examination and thorough investigation to guide
Multinucleated giant cells with foreign bodies are therapeutic management. Knowledge of the
infrequently observed. physiopathology is paramount in effectively utilizing
The sclerosing form is characterized by extensive fibrosis the available therapeutic options. Despite the generally
and a paucity of inflammatory cells, while the granulomatous benign nature of the lesions, they pose a significant threat
form is characterized by the presence of histiocytes and to the functional prognosis of the orbit. This complexity
multinucleated cells, suggestive of a sarcoid reaction. The underscores the need for further experimental work
final form presents as small-vessel vasculitis. Classical and to refine global management protocols, particularly
sclerotic forms are the most prevalent. This classification considering the variability in histological lesions such as
holds prognostic significance, with classical forms generally sclerosis, fibrosis, inflammation, myositis, and granuloma.
exhibiting a better prognosis than sclerotic forms. However,
research by Swamy et al. demonstrated a recurrence rate of Acknowledgments
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0.3 per patient-year in classical forms versus 0.15 per patient- None.
year in sclerotic forms. In the classic form, T-lymphocytes
outnumber B-lymphocytes, with helper T-lymphocytes Funding
surpassing suppressor T-lymphocytes. The proportion of
15
fibrovascular stroma increases in both subacute and chronic None.
forms, leading to fibrosis formation that fixes muscle Conflict of interest
structures by destroying fatty tissue. The inflammatory
infiltrate is believed to stem from local fat necrosis of the The authors declare that they have no competing interest.

Volume 3 Issue 1 (2024) 5 https://doi.org/10.36922/td.1792