Inflammatory myofibroblastic tumour of paranasal sinuses with fatal outcome: reactive lesion or tumour?

Abstract

Inflammatory myofibroblastic tumours (IMTs) are clinicopathologically distinctive but biologically controversial entities, which have been described in the lungs, abdomen, retroperitoneum, and extremities, but rarely affect the head and neck region. IMT usually follows a benign clinical course after radical excision, but invasive, locally recurrent, and metastatic forms of abdominal and mediastinal IMT have also been described. This report describes a case of IMT of the paranasal sinuses with a fatal outcome. A 22 year old woman was admitted to hospital as a result of epistaxis. Computed tomography scan and magnetic resonance imaging showed an expansive process in the paranasal sinuses, extending into the nasal cavity, orbita, and endocranium. The tumour progressed despite several surgical procedures. Radiotherapy, corticosteroids, and chemotherapy were unsuccessful, and the patient died four years after diagnosis, as a result of extensive intracranial spread of the tumour. This is the first known case of an IMT of the head and neck region with a fatal outcome. It shows that the aggressive behaviour of IMTs is not limited to abdominal and mediastinal locations, and supports recent observations that at least a subset of IMTs represents true neoplasia rather than reactive myofibroblastic proliferation.

Pulmonary inflammatory myofibroblastic tumor after Hodgkin’s lymphoma and application of PET imaging

“…However, there is also evidence that IMT maybe a low-grade neoplastic process as cytogenetic and molecular studies have shown chromosomal aberrations with resultant monoclonality in some subsets [1][2][3]. Clonal rearrangements of the short arm of chromosome 2p23, the site of the ALK receptor tyrosine kinase locus have been shown in up to 50% of soft tissue IMT particularly those occurring in the abdomen and lung in the first decade of life [5,6]. Donner et al [7] have shown progression of IMT to sarcoma in 7% of pulmonary occurrences.…”

Inflammatory Myofibroblastic Tumor: A Rapidly Growing Soft Tissue Mass in the Posterior Mandible

“…The WHO delineates three primary histological patterns of IMT-a ''myxoid/vascular pattern'', a ''compact spindle cell pattern'' with fascicular architecture and a distinctive infiltrate of plasma cells, and a ''hypocellular pattern'' with heavy collagenisation, bearing resemblance to fibrous scar tissue [3,9,11]. The present case exhibited a compact spindle cell pattern.…”

“…These lesions were traditionally considered a benign reactive process [12], possibly as an exaggerated immune response to Epstein-Barr virus, human herpes virus-8, trauma, surgery or a foreign body [3,6]. At least a fraction of IMTs appear to be neoplastic in nature, based on reports of recurrence [11], regional metastases [3], extensive local invasion leading to death in one case, and malignant transformation [11]. This concept has been further supported by evidence of aberrations in the anaplastic lymphoma tyrosine kinase (ALK) receptor locus on chromosome 2p23, reported in up to 50 % of IMTs [9].…”

Sinonasal inflammatory myofibroblastic pseudotumor (plasma cell granuloma)

“…[3] This condition is known to occur in several organs including lymph nodes, spleen, brain, spinal cord, larynx, thyroid gland, breast, pancreas, gastrointestinal (GI) tract and bladder, but occurs most frequently in the lungs and liver. [4] Only a few cases in the sinonasal cavities and sinuses have been reported [5] and IMPT involving the ear lobe and retroauricular region have also been reported. [6,7] These tumors present with a variety of symptoms depending on their localization.…”