Inflammatory Myofibroblastic Tumour of the Skull Base

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 p...

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Main Authors: Jean-Philippe Maire, Sandrine Eimer, François San Galli, Valérie Franco-Vidal, Sigolène Galland-Girodet, Aymeri Huchet, Vincent Darrouzet
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Otolaryngology
Online Access:http://dx.doi.org/10.1155/2013/103646
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author Jean-Philippe Maire
Sandrine Eimer
François San Galli
Valérie Franco-Vidal
Sigolène Galland-Girodet
Aymeri Huchet
Vincent Darrouzet
author_facet Jean-Philippe Maire
Sandrine Eimer
François San Galli
Valérie Franco-Vidal
Sigolène Galland-Girodet
Aymeri Huchet
Vincent Darrouzet
author_sort Jean-Philippe Maire
collection DOAJ
description 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.
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series Case Reports in Otolaryngology
spelling doaj-art-5de554e710974574be8dd95fe84ce6c62025-08-20T02:06:03ZengWileyCase Reports in Otolaryngology2090-67652090-67732013-01-01201310.1155/2013/103646103646Inflammatory Myofibroblastic Tumour of the Skull BaseJean-Philippe Maire0Sandrine Eimer1François San Galli2Valérie Franco-Vidal3Sigolène Galland-Girodet4Aymeri Huchet5Vincent Darrouzet6Department of Radiation Oncology, Saint-André University Hospital, University Bordeaux Segalen, 33076 Bordeaux, FranceDepartment of Pathology, Pellegrin University Hospital, University Bordeaux Segalen, Bordeaux, FranceDepartment of Neurosurgery A, Pellegrin University Hospital, University Bordeaux Segalen, Bordeaux, FranceDepartment of Otolaryngology and Skull Base Surgery, Pellegrin University Hospital, University Bordeaux Segalen, Bordeaux, FranceDepartment of Radiation Oncology, Saint-André University Hospital, University Bordeaux Segalen, 33076 Bordeaux, FranceDepartment of Radiation Oncology, Saint-André University Hospital, University Bordeaux Segalen, 33076 Bordeaux, FranceDepartment of Otolaryngology and Skull Base Surgery, Pellegrin University Hospital, University Bordeaux Segalen, Bordeaux, FranceInflammatory 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.http://dx.doi.org/10.1155/2013/103646
spellingShingle Jean-Philippe Maire
Sandrine Eimer
François San Galli
Valérie Franco-Vidal
Sigolène Galland-Girodet
Aymeri Huchet
Vincent Darrouzet
Inflammatory Myofibroblastic Tumour of the Skull Base
Case Reports in Otolaryngology
title Inflammatory Myofibroblastic Tumour of the Skull Base
title_full Inflammatory Myofibroblastic Tumour of the Skull Base
title_fullStr Inflammatory Myofibroblastic Tumour of the Skull Base
title_full_unstemmed Inflammatory Myofibroblastic Tumour of the Skull Base
title_short Inflammatory Myofibroblastic Tumour of the Skull Base
title_sort inflammatory myofibroblastic tumour of the skull base
url http://dx.doi.org/10.1155/2013/103646
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