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Inflammatory Myofibroblastic Tumour of the Skull Base

DOI: 10.1155/2013/103646

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Abstract:

Inflammatory myofibroblastic tumors (IMTs) are rare benign clinical and pathological entities. IMTs have been described in the lungs, abdomen, retroperitoneum, and extremities but rarely in the head and neck region. A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of the right sphenoid and clivus. MR imaging showed a large tumor of the skull base which was invading the sella turcica, right cavernous sinus, and sphenoidal sinus. A biopsy was performed and revealed an IMT. Corticosteroids were given for 3 months but were inefficient. In the framework of our pluridisciplinary consultation, fractionated conformal radiotherapy (FRT) was indicated at a low dose; 20?Gy in 10 fractions of 2?Gy over 12 days were delivered. Clinical response was complete 3 months after FRT. Radiological response was subtotal 6 months after FRT. Two years later, the patient is well. 1. Introduction Inflammatory myofibroblastic tumors (IMTs), also called inflammatory pseudo tumors or plasma cell granuloma, have been defined in the classification of soft tissue tumors as a lesion composed of myofibroblasts with inflammatory infiltrate [1]. This tumor is now recognized as a neoplastic mass that usually has an uneventful clinical course after radical resection. However, aggressive cases showing invasive, locally recurrent, multiple, and metastatic forms have also been reported [2]. This tumor has been primarily described in the soft tissues and viscera of children and young adults [3], with equal incidence in male and female patients [4]. IMT rarely affects the head and neck region. Despite an apparently benign morphological nature, they have been reported to have locally aggressive growth. Complete surgical excision has proved to be an effective treatment, with some recurrences occurring after surgery. Here, we report a rare case of a large IMT of the skull base causing headache, exophthalmia, and 6th nerve palsy, which could not be operated without heavy morbidity and which resolved after low-dose fractionated radiotherapy. 2. Case Report A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of right sphenoid and clivus (Figure 1). MR imaging showed a large tumor of the skull base that was invading the sella turcica, right cavernous sinus, and sphenoidal sinus (Figure 1). A biopsy was performed and revealed a dense proliferation of spindle

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