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

Abstract

Background: Inflammatory myofibroblastic tumour is a rare neoplasm with a potential to behave in a malignant manner. It can occur anywhere in the body, however involvement of the head, especially the para-nasal sinuses is rare.

Case presentation: A 33-year-old South Asian male presented with coryzal symptoms including a persistent cough with an asymmetrical swelling of the left side of the face. Imaging revealed a mass lesion involving the para-nasal sinuses eroding into the orbit. Histology and the clinical picture were compatible with inflammatory myofibroblastic tumour. As curative excision of the tumour was not feasible, medical management was offered. Despite early features of remission to glucocorticoids, tapering resulted in recurrence. Hence combination therapy with glucocorticoids and methotrexate was commenced with dramatic reduction of tumour burden and the patient has been in remission to date.

Conclusion: Inflammatory myofibroblastic tumour has the potential to behave in a malignant manner. Medical management with chemotherapy, glucocorticoids and non-steroidal anti-inflammatory drugs though effective, do not have a uniform response pattern. Surgically unresectable inflammatory myofibroblastic tumour above neck should be treated aggressively with combination regimens. Combination of prednisolone with methotrexate has been shown to have good outcome.

Figure 1

Magnetic resonance images demonstrate the initial appearance and response to glucocorticoid mono-therapy. Top row - Imaging of the head and face revealed abnormal thickening of temporalis. Asymmetry and loss of normal facial planes were seen with infiltrates noted in the left infra temporal fossa. Inflammatory changes are also seen in the ethmoid sinus , left maxillary sinus with muco-periosteal thickening and with non-uniform thickening and sclerosis of sinus wall with erosion postero-laterally. Extension of tumour was noted in the left orbit antero-laterally with lacrimal gland involvement. Bottom row- Imaging demonstrates the initial significant response to steroid monotherapy which prompted rapid tailing off.

Figure 2

Histological appearance and immunohistochemistry staining of the tumour. A- Spindle cells and inflammatory cells mixed with collagen (arrow) (Haematoxylin and Eosin 40 x10), B- Spindle cells with a mixed population of inflammatory cells composed of lymphocytes, histiocytes, eosinophils and plasma cells in a collagenous background (Haematoxylin and Eosin 100 x10), C- Vimentin- Diffuse, strong positivity, D- Cluster of difference 34- Spindle cells negative , E- Leukocyte common antigen - Patchy positivity highlighting the lymphoid cells; other cells negative , F- Anaplastic lymphoma kinase 1- Negative.

Figure 3

Magnetic resonance images show response with combination therapy of methotrexate and prednisolone. Top row - Imaging reveals the recurrence of tumour in the left temporal region and infra temporal fossa with involvement of the sinuses [left maxillary antrum projecting into the middle meatus of the nose, sphenoidal and ethmoid sinus] and involvement of the recti muscles of the left orbit. Bottom row- Imaging demonstrates absence of tumour in the temporal region and orbit and clearance within the middle meatus. Though reduced, persistence of residual tumour within the left maxillary antrum is seen.