ALK negative inflammatory myofibroblastic tumor of the orbit:A masquerading entity

Abstract

Inflammatory myofibroblastic tumor is a biologically distinct neoplasm of intermediate grade, which can affect every possible tissue of the human body. It is a ‘masquerading tumor’ as the presenting complaints vary with the affected site. Occurrence of this tumor as an orbital mass is rare and is challenging for both the clinician as well as the pathologist, due to a varied number of lesions sharing a similar picture clinically and histologically. We discuss a rare case of inflammatory myofibroblastic tumor presenting as an orbital mass and the importance of immunohistochemistry in arriving at the diagnosis, which helps dictate the treatment and prognosis of the patient.

Inflammatory myofibroblastic tumor of the lacrimal gland: case report of an exceptional location

“…Grossly, the tumor is well-circumscribed, lobulated or multinodular and may be firm or fleshy with a tan-white cut surface [ 3 , 9 , 10 ].…”

Inoperable inflammatory myofibroblastic tumour of the para-nasal sinuses and orbit with recurrence responding to methotrexate and prednisolone: a case report

“…Steroid refractory IMT requiring alternate agents is well documented [ 13 ]. Paradoxically emergence of IMT whilst on immunosuppression following kidney transplant has been reported putting into perspective the basis of immunosuppressive treatment in the management of IMT [ 11 ]. The definitive role of glucocorticoid therapy in IMT is not clearly understood but a therapeutic trial should be considered in appropriate cases and continued into remission if clinical and radiological response is apparent.…”

“…Laboratory studies revealed persistently elevated inflammatory markers with the lowest documented C - reactive protein level of 96 mg/dL and erythrocyte sedimentation rate of 132 mm in the first hour. Whole blood analysis demonstrated normal red cell indices (haemoglobin of 11.2 g/dL) with mildly elevated white cell count of 13.48 × 10 9 /L [ 4 - 11 ] which was predominantly neutrophilic - 67.8%. Blood film did not yield any additional information.…”

Ocular Inflammatory Myofibroblastic Tumor in the Left Eye with Phthisis Right Eye: A Rare Occurrence in a Child

“…Our patient presented with a mass arising from perilimbal area. The other reported cases presented with a subconjunctival mass inducing diplopia [ 1 ], a mass inferior to inferior rectus muscle with multiple neuropathy [ 5 ], painful exophthalmos [ 6 ], painless gradual diminution of vision for 2 months [ 9 ], supraorbital mass with loss of vision and phthisis for 2 years [ 10 ], and rapid progressive painless proptosis [ 8 ]. The case that was most similar to ours was an eight-month-old infant, who presented with a 5-week history of painless progressive swelling in the left upper eyelid [ 7 ].…”

“…Multiple cranial nerve involvement (II, V1, V2, V3, and X) was only seen in the case of a fifty-year-old adult patient [ 5 ], while the CT of another case showed an intraconal mass of left orbit that was not well differentiated by the lacrimal gland [ 6 ]. The MRI orbit of one case showed a smooth lobulated mass involving the superior and lateral aspects of the right orbit with involvement of the greater wing of sphenoid bone [ 9 ]. Bone erosion was not seen in any of these cases.…”