401 CT of Neuroblastomas and Ganglioneuromas in Children Edward A. Armstrong 1 , 2 Computed tomography (CT) has made a profound impact on the diagnosis and Derek C. F. Harwood-Nash 1 treatment of neuroblastomas and ganglioneuromas. The size, location, calcification, Charles R. Ritz 1 composition, and contiguous spread of the tumors has been well demonstrated by CT. CT is essential for their staging, subsequent treatment, and follow-up. Seventy-seven Sylvester H. Chuang 1 children were reviewed, 67 with neuroblastoma and 10 with ganglioneuroma seen Holger.Pettersson 1 3 between 1976 and 1980. Fifty-eight had one or more body CT scans, 22 had metriza­ David J. Martin mide myelography and/ or CT metrizamide myelography, and three patients had cranial CT. Intraspinal extension of tumor occurred in 11 instances, several requiring decom­ pressive surgery. A workup plan for optimal use of CT and CT metrizamide myelography was developed from this experience. Neuroblastoma and ganglioneuroma are among the most common neoplasms of infancy and early childhood, exceeded only in frequency by Wilms tumor of the kidney. Staging serves to identify tumor subgroups and clinical prognosis. Several factors indicate a favorable prognosis including: onset of the tumor before 1 year of age [1-7], tumors arising from the chest or posterior mediastinum [8-11], or a paravertebral location with extradural extension into th e spi nal canal [1-3, 12-15]. Ganglioneuroblastoma and benign ganglioneuroma have a more favorable prognosis. Spontaneous regression from the more malignant to the more benign forms may occur [7]. Our purpose was to develop an imaging protocol in which CT played a central role in determining the location and overall geography of the tumor. A retrospec­ tive study of the accuracy of CT in demonstrating th e size, location, calcifi cation, This article appears in the July / August 1982 composition, and contiguous and metastati c spread of th e tumor as well as issue of AJNR and the September 1982 issue of AJR. intraspinal extension provides th e basis for this report of its effectiveness. Received July 6, 1981 ; accepted after revision January 26, 1982. Materials and Methods Presented at the annual meeting of the Sociely for Pediatric Radiology, San Francisco, March From th e medical records of th e Hospital for Sick Children, Toronto, Th e Tumor Registry, 1981, and the annual meeting of the European Society of Pediatric Radiology, Oslo, April 1981 . and th e computer retrieval system of th e Di vision of Special Procedures, we fou nd 77 patients with either neuroblastoma or ganglioneuroma between 1976 and 1980. All tumors I Department of Radiology, Di vision of Special Procedures and Neuroradiology, Hospital for Sick were hi stologicall y verified in our Department of Pathology. Ch ildren, 555 University Av e., Toronto, Ontario, Body CT and metrizamide myelography were first used together at the Hospital for Sick M5G 1 X8, Canada. Address reprints to D. C. F. Children in 1976. Initially, the Ohio Nuclear Delta-50, and later the General El ectric 8800 Harwood-Nash. CT-T scanners were used. One or more CT scans, including either th e chest and abdomen 2 Present address: University of Texas South­ or cranium, were obtained in 58 of th e 77 patients and CT metrizamide myelog rams in 18. western Medical School, Children's Medical Cen­ Thirty-two were boys and 26 were girls. Seven patients had ganglioneuromas first pre­ ter, Dallas, TX 75235. senting at a mean age of 7 years (range, 4-14.5 years). Fifty-one pati ents had neuroblas­ 3 Department of Radiology, Division of Ultra­ tomas with a mean age of onset of 2.7 years (range, 1 day to 16 years). Of th e 11 patients sound, Hospital for Sick Children, Toronto, On­ tari o, M5G 1 X8, Canada. with intraspinal extension of th e tumor, on ly six had neurolog ic symptoms suggesting involvement of the spinal cord or lu mbar nerve roots. The cli nical findin gs included pain , AJNR 3:401-406, Juty / August 1982 0195-6108/ 82/ 0304-0401 $00.00 scoli osis, paraparesis, or paraplegia. © American Roentgen Ray Society Pl ain radiog raphy, excretory urography, conventional tomography, radionudide imag in g, 402 ARM STRONG ET AL. AJNR:3, July/ August 1982 A B c Fig . 1 .-Three pati ents. A, Paravertebral mass with amorphous calcifica­ punctate calcifications. Displacement of thoracic dural sac (arrow ) indicates ti on displaci ng kidney. B, Primary th oracic neuroblastoma with discrete intraspinal tumor extension. C, Rim calcification. Th oracic neuroblastoma. A B c Fig. 2. -A, Typical plain radiog raphic findings of metastatic neuroblas­ toma to skull. Striated new bone formati on, sutural separation (white arrow), and sclerosis of sphenoid and orbital roof (black arrow). B, Hair-on-end appearance of inner table in response to dural metastasis well shown by CT. C, Enhancing extradural mass in left middle cranial fossa and intraorbital mass with resultant proptosis of left eye. Metastati c invasion of sphenoid bone (arrow). D, Low-density round metastatic deposits in liver. o AJNR:3, July/ August 1982 CT OF NEUROBLASTOMA AND GANGLIONEUROMA 403 Fig. 3. -A, Ganglioneuroma erodes transverse process (white arrow) and widens intervertebral foramina. Exten­ sion of primary chest ganglioneuroma into neck. Slight but definite indentati on on left side of cervical dural sac (black arrow) indicates also intraspinal exten­ sion. B, Ganglioneu roma (arrow) ex­ tended higher into soft ti ssues of neck than was clinically suspected. A B sonography, and angiography were th e oth er meth ods used in localization. Radionuclide bone scintigraph y helped locali ze bone metastases. All radiographic and scintigraphic films obtained were restudied by at least two radiologists. In th e few in stances where th e origin al film reports differed from surgical or pathologic reports, new inter­ pretation of the studies by an unbiased observer was made. Results Computed Tomography Calcification. Standard radiography demonstrated calci­ fication in 10% of chest lesions and 25% of abdominal lesions. Incidences of less than 5% of chest lesions and about 25% of abdominal lesions were previously reported [2 , 6 , 9, 10, 15-16]. CT demonstrated calcification in 25% of the 18 chest lesions and 43% of the 39 abdominal lesions in our patients; calcification was seen by CT in 40% of the neuroblastomas and 20% of the ganglioneuromas. Calcifi­ cation of the intraspinal component of neuroblastoma was seen in only one case. The CT pattern of calcification more often appeared as amorphous (cloud like) calcification (fig. 1 A) and less com­ monly, discrete punctate calcifications (fig. 1 B) or rim cal­ cifications (fig. 1 C) . Skull and Orbits. Recent reports [17 -22], show the use­ Fig. 4.-True coronal CT scan. Left paravertebral calcified mass (white fu lness of cranial computed tomography in the evaluation of arrow) just below diaphragm was prim ary abdominal neuroblastoma. In feri or metastatic neuroblastoma to the skull and orbits. Three of to this is contrast-filled small bowel and th ere is also contrast-filled small bowel (black arrow) in pelvis. Thi s projection makes determination of radia­ our patients had cranial CT because of proptosis, orbital tion port possible. swelling, ecchymosis, and/ or a soft-tissue mass over the cranium. CT showed extradural involvement and the intraor­ bital mass of the metastatic neuroblastoma (figs. 2A and extension into the neck. CT demonstrated displacement of 2B). Special CT reconstruction for bone (General El ectric the carotid artery in one case and extension into th e extra­ Review Program) enhanced the display of bone metastases dural space through the neural foramen in a second case (fig. 2C). (fig . 3). Neck. Neuroblastoma or ganglioneuroma uncommonly Chest. Tumor involved the chest in 18 patients scanned presents as a mass in the neck. In two patients, a chest with CT. Primary tumor, contiguous spread from an abdom­ radiograph revealed a larger posterior mediastinal mass with inal primary, and, in the case of th e neuroblastoma, metas- 404 ARMSTRONG ET AL. AJNR:3, July / August 1982 tases along the paravertebral sympathetic chain can all reconstruction or direct coronal CT imaging [23] (fig. 4) occur in the chest. Rarely, parenchymal and subpleural were used in treatment planning and determining radiation nodules or pericardial metastases have occurred. portals. Abdominal CT also documented the presence of Abdomen. Thirty-three neuroblastomas and six gangli­ liver metastases (fig. 20). oneuromas were abdominal. Twenty-nine of these were paravertebral and 10 were adrenal. Sagittal and coronal Metrizamide Myelography, Intraspinal Tumor Ex tension, and CT Metrizamide Myelography With the availability of metrizamide, the advent of body TABLE 1: Spinal Canal Studies computed tomography, and computer software capable of Tumor Type increasing bone resolution, CT metrizamide myelography Totat has become exceptionally useful in evaluating intraspinal Neuroblas- Ganglioneu- toma roma and paraspinal neoplasms in infants and chil dren [24]. Early No. patients 67 10 77 in the experience with metrizamide, both conventional mye­ CT 50 8 58 lography using water-soluble contrast material and CT scan­ CT metrizamide myelography only 6 3 9 ning after the conventional examination were performed. Metrizamide myelography only 5 3 8 This has been referred to as secondary or postmyelogram Metrizamide myelography and CT metrizamide myelography 7 2 9 CT metrizamide myelography. In cases with specific clinical Total metrizamide studies 18 8 26 indications, a primary CT metrizamide myelography proce­ dure for evaluating the presence of intraspinal extension of the tumor has been developed. A small er volume and more dilute concentration (170 mg / dl metrizamide) has been Fig . 5. -Conventional metriza­ used and better definition of the subarachnoid space, cord, mide myelogram. Complete extra­ cauda equina, and nerve roots has been obtained with fewer dural block at T1 2-L 1 (large arrow).