Solitary myofibromatosis of the skull

We present a case of solitary infantile myofibromatosis of the skull in a 3-month-old boy. A right parietal subcutaneous lump was found at birth, and it increased in size over the ensuing 3 months. Surgery was performed, and a diagnosis of myofibromatosis was confirmed histopathologically. Solitary myofibromatosis of the skull is extremely rare. The radiographical, CT, and MR appearances, as well as histopathological findings, are described in this article. Received: 30 December 1998; Revised: 21 April 1999; Accepted: 11 May 1999     

Atypical imaging findings of infantile hemangioendothelioma: a case report

Infantile hemangioendothelioma is the third most common hepatic tumor in children, and the most common benign vascular tumor of the liver in infancy. On computed tomography (CT) and magnetic resonance imaging (MRI), typical enhancement patterns similar to those seen in adult hemangiomas may help establish the diagnosis. However, atypical enhancement patterns may also occur. In this paper, we report a rare form of solitary infantile hemangioendothelioma that showed a rim-like calcification and rim-like enhancement on CT and MRI.     

Solitary infantile myofibromatosis of the skull.

We describe the CT and plain-film appearance of solitary infantile myofibromatosis of the skull in a 5-year-old boy. This benign lesion closely resembles histiocytosis X on imaging studies.     

Imaging findings in seven cases of congenital infantile myofibromatosis with cerebral, spinal, or head and neck involvement

Introduction

Congenital infantile myofibromatosis (IM) is a rare mesenchymal disease, presenting with tumors in the skin, muscle, viscera, bone, and subcutaneous tissue. It can present as (a) a solitary form with subcutaneous, erythematous nodules, (b) a multicentric form with subcutaneous, muscle, and/or bony lesions, and (c) a multicentric form with visceral involvement. Cerebral or spinal involvement in myofibromatosis has been reported rarely.

Methods

We report seven cases of histology-proven infantile myofibromatosis with brain, spine, and/or head and neck involvement.

Results

In three patients with multiple subcutaneous nodules, a multicentric form of IM with visceral involvement was diagnosed. In three patients, a multicentric form without visceral involvement was found. Two patients had brain involvement, and four patients had vertebral body involvement.

Conclusion

In a newborn presenting with intraparenchymal brain lesions, epidural spinal masses, and/or vertebra plana or lytic lesions of the calvarium and spine, infantile myofibromatosis should be considered as a possible differential diagnosis. The presence of subcutaneous or muscular nodules facilitates the diagnosis.     

Miofibromatosis infantil. Un caso estudiado con ecografía y resonancia magnética de cuerpo entero

Infantile myofibromatosis, despite being considered a rare condition, is the most common fibrous tumour in infancy. It is characterised by the presence of benign fibroblastic-myofibroblastic lesions. It usually occurs in children under two years-old, but it can appear at any age. The solitary form (myofibromas) may affect the skin, subcutaneous cellular tissue, muscle or bone. In the multi-centred form (myofibromatosis), there may also be visceral lesions. The lesions usually regress spontaneously in one or two years, with the prognosis being excellent in these cases. However, when there is visceral involvement, the prognosis is poor and treatment with chemotherapy is indicated. Lung involvement is more associated with a poor prognosis. Although the definitive diagnosis is by histopathology, diagnostic imaging tests are essential for characterising the lesions, establishing the extent of the disease, assessing visceral involvement, and following up the progression of the lesions.     

Aggressively recurrent infantile myofibroma of the axilla and shoulder girdle

Infantile myofibroma is the most common fibrous tumor of infancy, typically affecting neonates and children under 2 years of age. Though the multicentric variant portends a grave prognosis, solitary lesions have an excellent prognosis and frequently undergo spontaneous regression. Surgical excision of solitary lesions is usually curative. In this report, we describe a pediatric patient with an unusually aggressive solitary myofibroma of the axilla who ultimately required a forequarter amputation as a lifesaving measure following multiple tumor recurrences and progressive tumor growth. The clinical course, radiographic findings, histology, and management rationale are presented.     

CT and MR imaging of benign hepatic and biliary tumors.

Benign hepatic and biliary tumors can present a difficult diagnostic challenge. Spiral computed tomography (CT) and magnetic resonance (MR) imaging are useful in the detection and characterization of these tumors. Imaging characteristics of lesions such as hepatic cyst, hemangioma, focal nodular hyperplasia (FNH), and hepatic adenoma are well known. Hepatic cysts demonstrate water attenuation at CT, are isointense relative to water at MR imaging, and do not enhance after intravenous administration of contrast material. Hemangiomas demonstrate characteristic nodular peripheral enhancement on early-phase images with subsequent fill-in centrally at both modalities. FNH classically demonstrates intense early enhancement with washout on delayed images. Although hepatic adenoma can also demonstrate intense early enhancement, it has a tendency to bleed and thus often appears more heterogeneous than FNH due to hemorrhage. Benign hepatic tumors that are less well described in the imaging literature include hepatic lipoma or angiomyolipoma, infantile hemangioendothelioma, and mesenchymal hamartoma. Hepatic lipoma has fat attenuation at CT, is isointense relative to fat at MR imaging, and does not enhance after intravenous administration of contrast material. Hepatic angiomyolipomas contain a variable amount of soft tissue in addition to fat and may therefore demonstrate enhancement at both modalities. The CT and MR imaging appearances of infantile hemangioma are similar to those of adult hemangioma. Infantile hemangioendothelioma occurs in infants under 6 months of age and is typically a larger lesion. Mesenchymal hamartoma also occurs in children, and its imaging appearance depends on the presence of stromal elements and the protein content of the cyst fluid. Familiarity with these imaging features can help distinguish particular disease entities.     

Myofibroma of the upper arm in a 52-year-old woman

Myofibromas are mesenchymal tumors that most often present in infancy. There are sporadic cases in adults throughout the literature. Myofibroma will demonstrate a variable appearance on CT and ultrasound, but tends to have a fairly consistent appearance on MR imaging. This report describes the MR and sonographic findings as well as the pathologic correlation of a myofibroma in the arm of a middle-aged woman. The patient was well, without evidence of recurrence 6 months following resection.     

Solitary infantile myofibromatosis involving the clavicle

A rare case of solitary infantile myofibromatosis of bone is reported in the right clavicle of a 15-year-old boy. A radiograph demonstrated an osteolytic lesion with a sharp margin and a sclerotic rim. CT revealed a circumscribed lesion with slight expansion of the cortex. On MRI the lesion appeared isointense to muscle on T1-weighted images, bright on T2-weighted images, and showed marked gadolinium enhancement. The patient was well, without evidence of recurrence or metastasis, 4 years and 5 months following resection. Received: 11 August 1998 Revision requested: 5 October 1998, 18 March 1999 Revision received: 15 March 1999, 23 April 1999 Accepted: 28 April 1999     

Infantile refsum disease: case report

Infantile Refsum disease is a rare inborn error of phytanic acid metabolism. It is inherited in an autosomal recessive manner and frequently causes signs and symptoms in the neonate period. The only source of phytanic acid in humans is exogenous, from diet. We report the MR imaging findings in two cases of infantile Refsum disease and note the MR imaging changes that occurred over time because of further progression of the disease. The initial diagnosis in both patients was made on basis of history, clinical findings, and biochemical studies.     

Intracranial solitary fibrous tumors: imaging findings in 6 consecutive patients

BACKGROUND AND PURPOSE: Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal neoplasms originating in the meninges. The aim of this study was to describe the CT, MR imaging, and angiographic features of the solitary fibrous tumor and to identify imaging characteristics. MATERIALS AND METHODS: We retrospectively reviewed CT, MR, and angiographic findings in 6 cases of ISFT. We evaluated the size, shape, and location of the tumor; the internal content and margin of the lesion; the pattern of enhancement; and the change of the adjacent structures. Density on noncontrast CT scans, signal intensity on MR images, and angiographic features were also documented. RESULTS: Each lesion appeared as a discrete extra-axial mass (size, 3-7 cm; mean, 5 cm). Five lesions were entirely solid, and 1 had peritumoral cyst. All 5 of the noncontrast CT scans showed hyperattenuated masses, and the tumors exhibited marked heterogeneous enhancement. No lesion contained calcification, and 2 cases showed bone invasions. On the MR images, 4 lesions showed mixed signal intensity on T2-weighted imaging. All of the lesions revealed marked heterogeneous enhancement. All of the tumors had thickening of the meninges adjacent to the tumor. Angiography showed delayed tumor blushing in all, and 3 of them had dysplastic dilation of the tumor vessels. CONCLUSION: Although there are no pathognomonic imaging findings, some imaging features, such as the "black-and-white mixed" pattern on T2-weighted images and marked heterogeneous enhancement, might be helpful in the diagnosis of intracranial solitary fibrous tumor.     

Infantile myofibromatosis: review of imaging findings and emphasis on correlation between MRI and histopathological findings

BackgroundInfantile myofibromatosis (IM) is the most common fibrous tumor of infancy. MRI is considered the gold standard in IM evaluation. Very little has been published about IM with histopathology correlation in the pediatric age.PurposeDescribe imaging findings in IM and correlate MRI findings with histopathology.Material and methodsImaging findings of 17 patients with IM were retrospectively analyzed including CT, US and MRI. Signal characteristics on T1-, T2-weighted and STIR imaging, extent of T2-hyperintensity, degree & pattern of enhancement, diffusion restriction, location & margins, & involvement of adjacent structures were tabulated. Histopathology findings included cellularity, collagenization, myxoid changes, atypia, mitosis & microscopic invasion. Established grading scores were utilized.ResultsRelative to normal skeletal muscle, on T1-weighted imaging, 9 lesions had similar signal while the remaining had a mixture of iso & hypo intensity; whereas on T2-weighted and STIR imaging, all 12 lesions demonstrated a mixture of iso, hypo & hyperintensity. T2-hyperintensity was grade 2 in one, grade 3 in 8 & grade 4 in 3 lesions. Intensity of enhancement was grade 2 in one, grade 3 in 8 & grade 4 in 3 lesions. Enhancement was predominantly peripheral in all 12 lesions.Extent of T2-hyperintensity & degree of enhancement corresponded to variable grades on histopathology.CT and US showed nonspecific findings.ConclusionOn MRI, IM has a mixture of signal intensity with predominant hyperintense signal on T2W images. However various signal & enhancement features correlated poorly with specific histopathologic grades.     

Staging of malignant pleural mesothelioma: comparison of CT and MR imaging

OBJECTIVE: This article compares the accuracy of CT with that of MR imaging in staging of malignant pleural mesothelioma. SUBJECTS AND METHODS: Ninety-five patients were enrolled in a prospective staging protocol based on the International Mesothelioma Interest Group staging system. Sixty-five patients underwent CT and MR imaging and a surgical procedure (excluding percutaneous needle biopsy) to stage and resect the tumor. Receiver operating characteristic analyses were performed. CT and MR scans were interpreted independently by observers who were unaware of the results of the other imaging study; these imaging findings were compared with the results of surgery and pathologic examination. RESULTS: The areas under the receiver operating characteristic curves for eight of 10 features revealed by imaging showed no statistically significant differences between CT and MR imaging. However, MR imaging was superior to CT in revealing invasion of the diaphragm (A(z) = .55 for CT versus .82 for MR imaging) and in revealing invasion of endothoracic fascia or solitary resectable foci of chest wall invasion (A(z) = .46 for CT; A(z) = .69 for MR imaging). Several anatomic regions could not be evaluated because positive findings at surgery were rare. CONCLUSION: CT and MR imaging are of nearly equivalent diagnostic accuracy in staging malignant pleural mesothelioma. MR imaging is superior to CT in revealing solitary foci of chest wall invasion and endothoracic fascia involvement and in showing diaphragmatic muscle invasion; however, this advantage does not affect surgical treatment. For cost reasons, CT should be considered the standard diagnostic study before therapy.     

Canavan disease: CT and MR imaging of the brain

Canavan disease (spongy degeneration of the brain) is a rare lethal neurodegenerative disorder of infancy; fewer than 100 cases have been reported. We describe a series of nine patients with enzymatic defects and clinical features consistent with Canavan disease; in two patients the disease was proved by biopsy. All patients were examined with CT and seven with MR. In every instance, both CT and MR showed white-matter disease, sometimes sparing the external and internal capsules, the corpus callosum, and the deep cerebellar white matter. Atrophic changes were found in six patients; the changes were marked in one and slight in five. There was no obvious correlation between the severity of the white-matter disease and the clinical presentation--one patient with severe white-matter disease was clinically normal. The CT and MR findings in Canavan disease are nonspecific and somewhat nonuniform: its preautopsy diagnosis relies primarily on biochemical findings.     

Solitary hepatic infantile hemangioendothelioma: dynamic gadolinium-enhanced MR imaging findings

We report the MRI findings of a solitary hepatic infantile hemangioendothelioma (IHE) diagnosed in a 14-day-old girl. To the best of our knowledge, only one report has illustrated the dynamic gadolinium-enhanced MR imaging features of IHE previously. Compounding the rarity of presentation as a solitary mass, the gadolinium-enhanced MRI appearance in our case is unique, because the IHE showed an early rim-like pseudocapsular enhancement followed by progressive fill-in of the lesion on delayed imaging.     

Imaging of musculoskeletal fibromatosis.

The musculoskeletal fibromatoses comprise a wide range of lesions with a common histopathologic appearance. They can be divided into two major groups: superficial and deep. The superficial fibromatoses are typically small, slow-growing lesions and include palmar fibromatosis, plantar fibromatosis, juvenile aponeurotic fibroma, and infantile digital fibroma. The deep fibromatoses are commonly large, may grow rapidly, and are more aggressive. They include infantile myofibromatosis, fibromatosis colli, extraabdominal desmoid tumor, and aggressive infantile fibromatosis. Radiographs typically reveal a nonspecific soft-tissue mass, and calcification is common only in juvenile aponeurotic fibroma. Advanced imaging (ultrasonography, computed tomography, and magnetic resonance [MR] imaging) demonstrates lesion extent. Involvement of adjacent structures is common, reflecting the infiltrative growth pattern often seen in these lesions. MR imaging may show characteristic features of prominent low to intermediate signal intensity and bands of low signal intensity representing highly collagenized tissue. However, fibromatoses with less collagen and more cellularity may have nonspecific high signal intensity on T2-weighted images. Local recurrence is frequent after surgical resection due to the aggressive lesion growth. It is important for radiologists to recognize the imaging characteristics of musculoskeletal fibromatoses to help guide the often difficult and protracted therapy and management of these lesions.     

Infantile desmoid-type fibromatosis with involvement of temporal bone: case report

Infantile desmoid-type fibromatosis is a rare, benign, infiltratively growing tumor of the soft tissue. The histological classification is often confusing due to various patterns. Although surgical excision is the treatment of choice, local recurrence is high. Ultrasound, CT, and MRI cannot differentiate the different types of fibrous lesions, but are very essential, showing the localization and infiltrated tissues. We report an unusual case of desmoid-type fibromatosis destroying the temporal bone in a now-5-year-old boy. The literature contains only few reports of radiolological findings in patients with infantile fibromatosis. Correspondence to: T. Hagen     

CT and MR imaging characteristics of infantile hepatic hemangioendothelioma

Aim

This study aims to analyze computed tomography (CT) and magnetic resonance (MR) imaging features of infantile hepatic hemangioendotheliomas before and after treatment.

Materials and methods

CT and MR examinations of seven infants with biopsy proven hepatic hemangioendotheliomas were retrospectively analyzed. The distribution, number, size, imaging appearance, enhancement pattern and post-treatment changes of the tumors were evaluated.

Results

A total of 153 hepatic hemangioendotheliomas were detected on CT (111) and MR (42) imaging. In six infants, 109/111 (98.2%) tumors were hypodense and 2/111 (1.8%) lesions contained calcification on unenhanced CT. On MR imaging, all 42 lesions in one infant were heterogeneously T1-hypointense and T2-hyperintense compared to the normal liver parenchyma. Contrast-enhanced CT and MRI showed peripheral rim (51.6%), uniform (48.4%), fibrillary (33.3%), and nodular (28.8%) contrast enhancement in the hepatic arterial phase. Homogeneous (100%), rim (98.2%) and mixed enhancement patterns were noted in tumors <1.0 cm, >2.0 cm and 1.0-2.0 cm in diameter respectively in the hepatic arterial phase. In three patients who underwent steroid therapy, follow-up CT examination demonstrated tumor size reduction and increased intra-tumoral calcification in two patients.

Conclusion

Infantile hepatic hemangioendotheliomas show some typical imaging features and size-dependent pattern of contrast enhancement on CT and MR imaging, which allow accurate imaging diagnosis and post-treatment evaluation.     

Solitary hepatic metastasis: Comparison of dynamic contrast-enhanced CT and MR imaging with fat-suppressed T2-weighted,Breath-hold T1-weighted FLASH,and dynamic gadolinium-enhanced FLASH sequences

Twenty consecutive cancer patients with a solitary hepatic metastasis detected with dynamic contrast-material—enhanced computed tomography (CT) who were considered for hepatic resection underwent magnetic resonance (MR) imaging within 18 days after CT. Histologic confirmation was obtained in all lesions. CT depicted 20 solitary lesions. MR imaging showed a solitary lesion in 14 patients, two lesions in three patients, and more than two lesions in three patients, for a total of 37 lesions. Twenty-three lesions less than 2 cm in diameter were missed with CT, and six lesions less than 1.3 cm in diameter were missed with MR imaging. MR imaging was superior to CT in the detection of hepatic metastases on a patient-by-patient basis (P <.01). The results suggest that MR imaging is superior to dynamic contrast-enhanced CT for the detection of hepatic metastases.