Pediatr Radiol (1992) 22:134-135
Pediatric
Radiology 9 Springer-Verlag 1992
Congenital primary cerebral angiosarcoma: CT, US, and MR findings I. R. Kirk, R. Dominguez, and M. Castillo The University of Texas Health Science Center at Houston, Department of Radiology, Houston, Texas, USA Received: 14 October 1991; accepted: 10 January 1992
Abstract. Congenital primary intracranial angiosarcoma (CAS) is an exceptionally rare tumor [1-6]. To our knowledge the imaging features of intracranial angiosarcomas have only been briefly mentioned in the neuropathologic literature [4-6]. To our knowledge, only one case of CAS has been reported [3]. We present a case of a pathologically proven CAS found in a neonate.
Case report A twelve-day-old female, product of an uneventful 38-week gestation twin pregnancy, presented with a rapidly enlarging head, progressive irritability, and increasingly frequent poor feedings followed by vomiting. On physical examination, the head circumference was 39.5 cm, (> 95 % for age). The forehead veins were prominent, the anterior fontanelle was tense, and the sutures were split to palpation. Right proptosis was also present. Cranial sonography revealed a predominantly echogenic mass in the right temporal region, midline shift to the left, and hydrocephalus (Fig. 1). Computed tomography (CT) without contrast administration demonstrated an hyperdense mass which contained small loci of calcifications (Fig. 2 a). Following iodinated contrast administration, the mass showed peripheral marked enhancement and a central hypodensity (Fig.2b). Magnetic resonance (MR) imaging showed the bulk of the mass to be isointense to gray matter on T1 weighted images (Fig. 3 a). Central areas of hyperintensity were suggestive of subacute hemorrhage. The mass was demarcated from normal brain by a low signal intensity rim. On T2 weighted images the mass was heterogenous and mainly of low signal intensity (Fig.3b). No gadolinium was administered. Hydrocephalus was present. At surgery, the extraxial tumor was adherent to the dura and separated from the brain by a fibrous capsule. Electron microscopy was performed and the diagnosis of CAS was made. Follow-up MR, 26 months after surgery, showed the patient to be free of disease. At the time of this report, she has only a mild developmental delay.
Discussion Primary congenital tumors of the central nervous system are rare. CAS is an exceptionally rare t u m o r and its occurrence m a y be considered a curiosity [1, 3-6]. To our knowledge, only one case of CAS has been reported [3].
CAS's are so unusual that their incidence cannot be reliably established. Overall, angiosarcomas comprise only 1-2 % of all primary intracranial tumors [1]. Only fourteen cases of true primary CNS angiosarcoma have been reported [3-9]. The parietal lobe is the most frequent site [3]. Meningeal origin is less frequent but occurs m o r e often in cases of primary spinal cord angiosarcomas [6, 8]. There is no age predominance and males are m o r e often affected [3]. Pathologically, primary CNS angiosarcomas are malignant neoplasias of vascular endothelium that may arise from mesenchymal elements of the brain and meninges [3, 6, 7]. Occasionally, they may be mistaken for gliosarcomas and other highly vascular cerebral sarcomas. Histologically, these tumors are comprised of vascular channels lined by malignant endothelial cells and contain a diffuse reticulin fiber network in a concentric perivascular pattern [3, 6, 7]. Electron microscopy d e a r l y demonstrates the ultrastructural features of the malignant endothelium and often reveals Weibel-Palade bodies. (rod shaped cytoplasm bodies typical of angiosarcomas) [3, 6, 7]. Clotting Factor V I I I related antigen and the U E A - I (Ulex Europaeus Agglutinin I) are other immunohistochemical techniques specific for primary CNS angiosarcoma [3, 7]. The imaging characteristics of the primary CNS angiosarcomas have only been briefly described and are nonspecific [4, 5]. On CT they may contain areas of hemorrhage and one case showed homogenous contrast enchancement [5]. In one case, M R Showed a mass containing areas of new and old h e m o r r h a g e as well as surrounding e d e m a [4]. In our patient the t u m o r was extraxial and our preoperative differential diagnosis was mainly that of a congenital meningioma. However, the presence of intratumoral hemorrhage was considered atypical for meningioma. Even though, intracranial angiosarcomas are malignant, our patient had a good outcome and was free of disease m o r e than two years after the diagnosis was made. The excellent outcome in this patient is probably related to the extraxial location of the mass which allowed complete surgical removal. CAS is an extremely rare t u m o r
135
Fig. 1. Coronal ultrasound section through the anterior fontanelle shows a large mass (solid arrows) in the right temporal region. The mass is mostly hyperechoic but in its central aspect contains an area of low echos (open arrow). There is midline shift with displacement of the ventricular system towards the left side. The left lateral ventrical is dilated probably secondary to entrapment of the foramen of Monroe Fig.2. a Non-contrast axial CT section shows the mass to be mostly hyperdense. This finding is related to the presence of psammomatous calcifications. The center of the mass is of slightly lower density. Notice that there is widening of the subarachnoid space (arrow) anteriorly to the mass, reflecting its extraxial location. Again noted is midline shift and massive hydrocephalus, b Axial CT section at the same level as Fig. 2 a following administrationof contrast material. There is diffuse and intense enhancement of the mass. The central non-enchancinglow density, is more obvious
Fig.3. aNon-contrast axial MR T1 weighted image (700/20/2 [TR/TE/NEX] ) shows the bulk of the tumor to be of similar signal intensityto that of normal gray matter. There is a small rim of low signal intensity throughout the periphery of the mass which correlated with a fibrous capsule at surgery. The central area of high signal intensity (which corresponds to the low density seen on CT) represents subacute blood (methemoglobin). There is a small blood-CSF level (arrow) within the dependent portion of the markedly dilated left
lateral ventricle. This hemorrhage is most likely secondary to previous tapping of the ventricular system. The calcifications are not readily appreciated, b Axial T2 weighted MR image (2500/60/1) at the same level as Fig. 3 a. The mass contains multiple foci of very low signal intensitywhich could be related to multiple calcifications seen on CT and found in the surgical specimen. This study clearly demonstrates the peripheral zone of low signal intensity representing the tumor capsule. The central area remains of high signal intensity
that should b e c o n s i d e r e d in the differential diagnosis of c o n g e n i t a l b r a i n t u m o r s , especially if they c o n t a i n h e m o r rhagic loci.
6. Russell DA, Rubinstein JJ (1989) Pathology of tumors of the nervous system, Williams and Wilkins, Baltimore 7. Mena H, Garcia JH (1978) Primary brain sarcomas. Light and electron microscopic features. Cancer 42:1298--1307 8. Kristoferitsch W, Jellinger K (1986) Multifocal spinal angiosarcoma after chordotomy. Acta Neurochir 79:145-153 9. Zeigler JW (1975) Primary angiosarcoma of the brain: report of a case. J Am Osteopath Assoc 74:957-960
References 1. Fleming JFR (1985) Intracranial sarcomas neurosurgery. McGraw-Hill, New York 2. Enzinger JM (1988) Soft tissue tumors. Mosby, St Louis 3. Mena H, Ribas JL, Enzinger FM, Parisi JE (1991) Primary anglosarcoma of the central nervous system. J Neurosurg 75:73-76 4. Charman HR Lorenstein DH, Cho KG, DeArmond SJ, Wilson CB (1988) Primary cerebral angiosarcoma. Case report. J Neurosurg 68:806-810 5. Cookston M, Cotter GW (1991) Primary angiosarcoma of the brain. A case report. Southern Med J 84:517-520
R. Dominguez, MD Department of Radiology UTHSC-Houston 6431 Fannin Room 2.132 MSB Houston, TX 77030 USA
Pediatric
Radiology 9 Springer-Verlag 1992
Congenital primary cerebral angiosarcoma: CT, US, and MR findings I. R. Kirk, R. Dominguez, and M. Castillo The University of Texas Health Science Center at Houston, Department of Radiology, Houston, Texas, USA Received: 14 October 1991; accepted: 10 January 1992
Abstract. Congenital primary intracranial angiosarcoma (CAS) is an exceptionally rare tumor [1-6]. To our knowledge the imaging features of intracranial angiosarcomas have only been briefly mentioned in the neuropathologic literature [4-6]. To our knowledge, only one case of CAS has been reported [3]. We present a case of a pathologically proven CAS found in a neonate.
Case report A twelve-day-old female, product of an uneventful 38-week gestation twin pregnancy, presented with a rapidly enlarging head, progressive irritability, and increasingly frequent poor feedings followed by vomiting. On physical examination, the head circumference was 39.5 cm, (> 95 % for age). The forehead veins were prominent, the anterior fontanelle was tense, and the sutures were split to palpation. Right proptosis was also present. Cranial sonography revealed a predominantly echogenic mass in the right temporal region, midline shift to the left, and hydrocephalus (Fig. 1). Computed tomography (CT) without contrast administration demonstrated an hyperdense mass which contained small loci of calcifications (Fig. 2 a). Following iodinated contrast administration, the mass showed peripheral marked enhancement and a central hypodensity (Fig.2b). Magnetic resonance (MR) imaging showed the bulk of the mass to be isointense to gray matter on T1 weighted images (Fig. 3 a). Central areas of hyperintensity were suggestive of subacute hemorrhage. The mass was demarcated from normal brain by a low signal intensity rim. On T2 weighted images the mass was heterogenous and mainly of low signal intensity (Fig.3b). No gadolinium was administered. Hydrocephalus was present. At surgery, the extraxial tumor was adherent to the dura and separated from the brain by a fibrous capsule. Electron microscopy was performed and the diagnosis of CAS was made. Follow-up MR, 26 months after surgery, showed the patient to be free of disease. At the time of this report, she has only a mild developmental delay.
Discussion Primary congenital tumors of the central nervous system are rare. CAS is an exceptionally rare t u m o r and its occurrence m a y be considered a curiosity [1, 3-6]. To our knowledge, only one case of CAS has been reported [3].
CAS's are so unusual that their incidence cannot be reliably established. Overall, angiosarcomas comprise only 1-2 % of all primary intracranial tumors [1]. Only fourteen cases of true primary CNS angiosarcoma have been reported [3-9]. The parietal lobe is the most frequent site [3]. Meningeal origin is less frequent but occurs m o r e often in cases of primary spinal cord angiosarcomas [6, 8]. There is no age predominance and males are m o r e often affected [3]. Pathologically, primary CNS angiosarcomas are malignant neoplasias of vascular endothelium that may arise from mesenchymal elements of the brain and meninges [3, 6, 7]. Occasionally, they may be mistaken for gliosarcomas and other highly vascular cerebral sarcomas. Histologically, these tumors are comprised of vascular channels lined by malignant endothelial cells and contain a diffuse reticulin fiber network in a concentric perivascular pattern [3, 6, 7]. Electron microscopy d e a r l y demonstrates the ultrastructural features of the malignant endothelium and often reveals Weibel-Palade bodies. (rod shaped cytoplasm bodies typical of angiosarcomas) [3, 6, 7]. Clotting Factor V I I I related antigen and the U E A - I (Ulex Europaeus Agglutinin I) are other immunohistochemical techniques specific for primary CNS angiosarcoma [3, 7]. The imaging characteristics of the primary CNS angiosarcomas have only been briefly described and are nonspecific [4, 5]. On CT they may contain areas of hemorrhage and one case showed homogenous contrast enchancement [5]. In one case, M R Showed a mass containing areas of new and old h e m o r r h a g e as well as surrounding e d e m a [4]. In our patient the t u m o r was extraxial and our preoperative differential diagnosis was mainly that of a congenital meningioma. However, the presence of intratumoral hemorrhage was considered atypical for meningioma. Even though, intracranial angiosarcomas are malignant, our patient had a good outcome and was free of disease m o r e than two years after the diagnosis was made. The excellent outcome in this patient is probably related to the extraxial location of the mass which allowed complete surgical removal. CAS is an extremely rare t u m o r
135
Fig. 1. Coronal ultrasound section through the anterior fontanelle shows a large mass (solid arrows) in the right temporal region. The mass is mostly hyperechoic but in its central aspect contains an area of low echos (open arrow). There is midline shift with displacement of the ventricular system towards the left side. The left lateral ventrical is dilated probably secondary to entrapment of the foramen of Monroe Fig.2. a Non-contrast axial CT section shows the mass to be mostly hyperdense. This finding is related to the presence of psammomatous calcifications. The center of the mass is of slightly lower density. Notice that there is widening of the subarachnoid space (arrow) anteriorly to the mass, reflecting its extraxial location. Again noted is midline shift and massive hydrocephalus, b Axial CT section at the same level as Fig. 2 a following administrationof contrast material. There is diffuse and intense enhancement of the mass. The central non-enchancinglow density, is more obvious
Fig.3. aNon-contrast axial MR T1 weighted image (700/20/2 [TR/TE/NEX] ) shows the bulk of the tumor to be of similar signal intensityto that of normal gray matter. There is a small rim of low signal intensity throughout the periphery of the mass which correlated with a fibrous capsule at surgery. The central area of high signal intensity (which corresponds to the low density seen on CT) represents subacute blood (methemoglobin). There is a small blood-CSF level (arrow) within the dependent portion of the markedly dilated left
lateral ventricle. This hemorrhage is most likely secondary to previous tapping of the ventricular system. The calcifications are not readily appreciated, b Axial T2 weighted MR image (2500/60/1) at the same level as Fig. 3 a. The mass contains multiple foci of very low signal intensitywhich could be related to multiple calcifications seen on CT and found in the surgical specimen. This study clearly demonstrates the peripheral zone of low signal intensity representing the tumor capsule. The central area remains of high signal intensity
that should b e c o n s i d e r e d in the differential diagnosis of c o n g e n i t a l b r a i n t u m o r s , especially if they c o n t a i n h e m o r rhagic loci.
6. Russell DA, Rubinstein JJ (1989) Pathology of tumors of the nervous system, Williams and Wilkins, Baltimore 7. Mena H, Garcia JH (1978) Primary brain sarcomas. Light and electron microscopic features. Cancer 42:1298--1307 8. Kristoferitsch W, Jellinger K (1986) Multifocal spinal angiosarcoma after chordotomy. Acta Neurochir 79:145-153 9. Zeigler JW (1975) Primary angiosarcoma of the brain: report of a case. J Am Osteopath Assoc 74:957-960
References 1. Fleming JFR (1985) Intracranial sarcomas neurosurgery. McGraw-Hill, New York 2. Enzinger JM (1988) Soft tissue tumors. Mosby, St Louis 3. Mena H, Ribas JL, Enzinger FM, Parisi JE (1991) Primary anglosarcoma of the central nervous system. J Neurosurg 75:73-76 4. Charman HR Lorenstein DH, Cho KG, DeArmond SJ, Wilson CB (1988) Primary cerebral angiosarcoma. Case report. J Neurosurg 68:806-810 5. Cookston M, Cotter GW (1991) Primary angiosarcoma of the brain. A case report. Southern Med J 84:517-520
R. Dominguez, MD Department of Radiology UTHSC-Houston 6431 Fannin Room 2.132 MSB Houston, TX 77030 USA
