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 . 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 . This tumor has been primarily described in the soft tissues and viscera of children and young adults , with equal incidence in male and female patients . 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
C. M. Coffin, J. Watterson, J. R. Priest, and L. P. Dehner, “Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunohistochemical study of 84 cases,” American Journal of Surgical Pathology, vol. 19, no. 8, pp. 859–872, 1995.
R. A. Morotti, M. D. Legman, N. Kerkar, B. R. Pawel, W. G. Sanger, and C. M. Coffin, “Pediatric inflammatory myofibroblastic tumor with late metastasis to the lung: case report and review of the literature,” Pediatric and Developmental Pathology, vol. 8, no. 2, pp. 224–229, 2005.
A. C. De Oliveira Ribeiro, V. M. Joshi, W. K. Funkhouser, and S. K. Mukherji, “Inflammatory myofibroblastic tumor involving the pterygopalatine fossa,” American Journal of Neuroradiology, vol. 22, no. 3, pp. 518–520, 2001.
D. K. Lee, Y. S. Cho, S. H. Hong, W. H. Chung, and Y. C. Ahn, “Inflammatory pseudotumor involving the skull base: response to steroid and radiation therapy,” Otolaryngology, vol. 135, no. 1, pp. 144–148, 2006.
Y. Sasagawa, T. Akai, S. Itou, and H. Iizuka, “Multiple intraosseous inflammatory myofibroblastic tumors presenting with an aggressive clinical course: case report,” Neurosurgery, vol. 69, no. 4, pp. E1010–E1015, 2011.
P. R. Olmos, J. M. Falko, G. L. Rea et al., “Fibrosing pseudotumor of the sella and parasellar area producing hypopituitarism and multiple cranial nerve palsies,” Neurosurgery, vol. 32, no. 6, pp. 1015–1021, 1993.
A. M. Buccoliero, A. Caldarella, M. Santucci et al., “Plasma cell granuloma—an enigmatic lesion: description of an extensive intracranial case and review of the literature,” Archives of Pathology & Laboratory Medicine, vol. 127, no. 4, pp. e220–e223, 2003.
V. Ganesan, J. P. Lin, W. K. Chong, F. J. Kirkham, and R. A. H. Surtees, “Painful and painless ophthalmoplegia with cavernous sinus pseudotumour,” Archives of Disease in Childhood, vol. 75, no. 3, pp. 239–241, 1996.
M. H. Han, J. G. Chi, M. S. Kim et al., “Fibrosing inflammatory pseudotumors involving the skull base: MR and CT manifestations with histopathologic comparison,” American Journal of Neuroradiology, vol. 17, no. 3, pp. 515–521, 1996.
S. R. Kodsi, B. R. Younge, J. A. Leavitt, R. J. Campbell, and B. W. Scheithauer, “Intracranial plasma cell granuloma presenting as an optic neuropathy,” Survey of Ophthalmology, vol. 38, no. 1, pp. 70–74, 1993.
M. K. Kasliwal, A. Suri, D. K. Gupta, V. Suri, A. Rishi, and B. S. Sharma, “Sphenoid wing inflammatory pseudotumor mimicking a clinoidal meningioma: case report and review of the literature,” Surgical Neurology, vol. 70, no. 5, pp. 509–513, 2008.
S. R. Cherukupally, L. A. Mankarious, W. Faquin, and M. J. Cunningham, “Pediatric non-orbital pseudotumor of the head and neck,” International Journal of Pediatric Otorhinolaryngology, vol. 67, no. 6, pp. 649–653, 2003.
S. L. A. Lawson, D. K. Azoumah, K. Lawson-Evi et al., “Imflammatory myofibroblastic tumour of nose and paranasal sinuses in a little girl of 7-year-old,” Archives de Pediatrie, vol. 17, no. 1, pp. 34–37, 2010.
A. Modarressi, G. Pietramaggiori, T. Bezzola et al., “A facial inflammatory myofibroblastic tumour in a 6-year-old girl: plastic surgery lessons from a rare case,” Journal of Cranio-Maxillofacial Surgery, vol. 39, no. 2, pp. 141–144, 2011.
J. G. Seol, L. A. Loevner, B. W. O'Malley, and M. S. Grady, “Inflammatory pseudotumor of the trigeminal nerve: a neoplastic mimic you do not want to miss,” American Journal of Neuroradiology, vol. 30, no. 10, pp. 1941–1943, 2009.
F. L. Ampil and F. S. Bahrassa, “Primary orbital lymphoma—pseudotumor (pseudolymphoma): case reports and review of radiotherapy literature,” Journal of Surgical Oncology, vol. 30, no. 2, pp. 91–95, 1985.
S. Al-Sarraj, J. Wasserberg, R. Bartlett, and L. R. Bridges, “Inflammatory pseudotumour of the central nervous system: clinicopathological study of one case and review of the literature,” British Journal of Neurosurgery, vol. 9, no. 1, pp. 57–66, 1995.