Infantile myofibromatosis

Abstract

The clinical and pathologic features of 61 cases of congenital and infantile myofibromatosis are presented. The tumor affected almost exclusively infants and young children; 88% of cases occurred before the age of two years, and 60% were noted at birth or shortly thereafter. Solitary (45 cases) and multicentric (16 cases) forms of the tumor could be distinguished; the solitary form was more common in males (69%) and affected chiefly the soft tissues of the head-neck region and the trunk. The multicentric form, on the other hand, predominated in females (63%) and was found not only in soft tissues but also in bones and viscera. Fourteen of the 16 cases with multicentric lesions were present at birth, and four of them had evidence of visceral involvement. Among the 16 cases were two sets of siblings, both brother and sister. Grossly, nearly all of the tumors were well demarcated; they measured from 0.5 to 7 cm in greatest diameter. Microscopically, they formed well-circumscribed nodules consisting of short bundles of plump, spindle-shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. Necrosis or a hemangiopericytoma-like pattern was often found in the center of the tumor nodules. Intravascular growth was encountered in several instances, but this feature did not seem to affect the clinical behavior. Of the 43 patients with follow-up data (mean-follow up 5.1 years), 36 were alive and well. Of the solitary lesions, three recurred. Of the multicentric lesions, five of the 15 patients showed evidence of spontaneous regression of both soft tissue and bone lesions. Four died, three of extensive involvement of multiple viscera, especially the lung, and one of "crib-death."

Scientific poster session

“…NUNES 1 , P. VILELA 2 1 Hospital Garcia da Orta-Neuroradiology Department, Almada, PORTUGAL, 2 Hospital da Luz Imagiology Center, Lisbona, PORTUGAL PURPOSE Susceptibility weighted imaging (SWI) is a novel MRI technique that combines magnitude and phase information from a high spatial resolution 3D gradient-echo sequence. It is highly sensitive to the difference in magnetic susceptibility of various substances compared to their neighbouring backround tissue, showing excellent image contrast and depicting anatomic structures, such as small veins, in great detail.…”

“…Investigation should include Compute Tomography, MRI and an arterial angiography (CTA, MRA or Digital Subtraction Angiography). Hanyang University Guri Hospital, Radiology, Guri, SOUTH KOREA, 2 Hanyang University Hospital, Radiology, Seoul, SOUTH KOREA PURPOSE A ventriculoperitoneal (VP) shunt is the most common way of treatment for hydrocephalus. Cervicothoracic complications with VP shunts are rare, but we present extremely rare case of the distal migration of distal catheter of a VP shunt into heart, via internal jugular vein.…”

“…KUBAS 1 , J. Walecki 2 , E. Tarasow 1 , J. Kochanowicz 1 , J. Pniewski 2 1 Medical University of Bialystok, Bialystok, POLAND, 2 Polish Academy of Sciences Medical Research Center, Warsaw, POLAND PURPOSE To assess the role of 1H MRS in the detection of changes in cerebral metabolite levels in pyramidal tracts after cortical/ subcortical infarction and to compare metabolite alterations to clinical outcome (Barthel index, Scandinavian Stroke Scale). MATERIALS AND METHODS 21 patients who underwent supratentorial cortical/ subcortical infarction were studied, 1 month after the onset of clinical symptoms of ischemic stroke.…”

“…T Krings 1,2 , S Geibprasert 2,3 , P Lasjaunias 2 , V Pereira 2 1 Department of Neuroradiology, University Hospital Aachen, Aachen, GERMANY, 2 Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicêtre, Paris, FRANCE, 3 Department of Radiology, Ramathibodi Hospital, Mahidol University, Bangkok, THAILAND OBJECTIVE While it is generally accepted that Developmental Venous Anomalies (DVAs) are benign vascular malformations, over the past years, we have experienced symptomatic DVAs that, together with various reports describing complicated DVAs have fuelled our interest in reviewing how and when DVAs can become clinically significant. METHODS Charts and angiographic films of 17 patients with DVAs whose 18 vascular symptoms could be attributed to a DVA were selected from a neurovascular databank of our hospital.…”

“…RYU 1 , W Choi 2 , B Park 2 , D Yang 1 1 East-west Neomedical center, collage of medicine, kyunghee university, Seoul, SOUTH KOREA, 2 Kyunghee medical center, collage of medicine, kyunghee university, Seoul, SOUTH KOREA OBJECTIVE Biphasic contrast media injection would fulfill two contrary requirements of head and neck CT imaging: tumor conspicuity and vessel enhancement. The purpose of this study was to evaluate the value of biphasic injection of contrast material in head and neck CT. METHODS A pilot study was performed to generate and to compare time-density curves of monophasic injection (100 ml at 3 ml/sec) and biphasic injection (initially 50 ml at 3 ml/sec and an additional 50 ml administered 35 seconds after initiation of the first injection) of contrast material.…”