Arteriographic Manifestations of Ganglioneuromas 1
Diagnostic Radiology
N. Reed Dunnick, M.D. and Ronald A. Castelllno, M.D. The angiographic findings in four cases of ganglioneuroma are described. In all four cases a large vascular mass was demonstrated angiographically. The vessels supplying the tumors varied, depending on the location of the mass. Stretching and displacement of adjacent opacified vessels helped define the extent of the tumor. Abundant neovascularity was present with serpiginous vessels tending to cluster in nodular areas, with adjacent avascular areas. INDEX TERMS:
Ganglioneuroma. Neuroblastoma
Radiology 115:323-328, May 1975
ANGlIONEUROMAS are rare, benign tumors arising from sympathetic ganglia, and are usually found in the neck, posterior mediastinum or retroperitoneal space. Since these tumors frequently produce no symptoms, they are often discovered as an incidental finding on radiographs taken for other reasons and at autopsy. The radiographic findings are those of a soft-tissue mass, often ovoid in shape, which at times contains mottled calcifications. They may attain sufficient size to displace adjacent organs, and occasionally may present as a "dumbbell tumor" with neuroforaminal widening and spinal cord compression (5). There is little information regarding the angiographic manifestations of the ganglioneuroma. Bunn and King described a 51-year-Old man with a ganglioneuroma in the neck which was " almost avascular" on carotid arteriography (3). Antonie described a 45-year-old woman with a ganglioneuroma arising from the inferior pole of the kidney which was "avascular" on aortography (1). Among 20 cases of adrenal tumors, Rossi included a single case of a ganglioneuroma in a 20-year-old woman studied by aortography which showed small arteries which were "sparse, irregular, and displaced" (9). Nebesar et at. studied two retroperitoneal ganglioneuromas, one of which in a 7-year-old boy was described as showing "irregular tumor vessels" on selective lumbar arteriography (8). Tassy and Huguet presented findings of a hypervascular ganglioneuroma on a celiac arteriogram in a 17-year~0Id adolescent (10). This report describes the angiographic findings in four patients studied at Stanford Medical Center who had ganglioneuromas which were subsequently removed at surgery.
G
CASE REPORTS CASE I:
1
A 23-year-old white woman was found to have a large
irregularly calcified mass in the right upper quadrant on an upper gastrointestinal series which was obtained for intermittent midepigastric burning pain. She was otherwise asymptomatic with no specific metabolic or urologic complaints. The physical examination, routine laboratory values, and endocrinologic evaluation were all normal. An excretory urogram (Fig. 1, A) demonstrated intrinsically normal kidneys with inferior displacement of the right kidney by the calcified suprarenal mass. Abdominal aortography with selective right renal and middle adrenal arteriography disclosed a vascular mass which was supplied by branches of the right middle (Fig. 1, B) and right inferior (Fig. 1, C and D) adrenal arteries. There was abundant neovascularity clustered throughout the mass, with large avascular or hypovascular components. No arteriovenous shunting or contrast puddling was clearly identified. The right renal vein was markedly stretched over this mass. A small blush was interpreted as representing a laterally displaced adrenal gland. At surgery a 14-cm mass adherent to the renal vessels was found, and right nephrectomy and adrenalectomy were required (Fig. 1, E). The mass appeared encapsulated and on cut section was light tan in color, firm, and contained multiple small foci of calcification. Microscopy showed histologic features characteristic of a ganglioneuroma, with a predominance of Schwann cells. Multiple ganglion cells appeared singly and in clusters, and most were mature in appearance. Microscopy of the kidney and adrenal gland was normal. CASE II: A 2Y2-year-old white girl presented with a one-year history of bloodless diarrhea and failure to thrive. She was in the tenth percentile for height and weight. Physical examination was otherwise unremarkable as were routine laboratory studies and endocrinologic evaluation. An ovoid homogeneous right paravertebral mass was noted adjacent to the right lower thoracic spine on an upper gastrointestinal series. An excretory urogram was normal. A midthoracic and abdominal aortogram showed numerous neovascular vessels located within the mass, which arose from the ninth through the twelfth intercostal arteries (Fig. 2). The presence or absence of arteriovenous shunting or contrast puddling could not be evaluated with confidence on the basis of the aortogram alone. At surgery, a 5 X 8-cm mass was found in the right lower thoracic paravertebral area. The mass was firm in consistency and tan in
From the Division of Diagnostic Radiology, Stanford University Medical Center, Stanford, Calif. Accepted for publication in October 1974.
323
dk
Fig. 1. CASE I. A. Excretory urogram shows an intrinsically normal right kidney inferiorly displaced by a large calcified suprarenal mass. B. Selective right middle adrenal arteriogram demonstrates a large cluster of neovascular vessels (closed arrows) without encasement, arteriovenous shunting or laking. Large portions of the mass are hypovascular, with stretching of adjacent branches (open arrows). C and D. Selective right renal arteriogram, arterial and venous phases, demonstrates a prominent inferior adrenal artery (open arrow) stretched around the mass and giving rise to a single cluster of neovascularity (closed arrow). The renal vein is bowed by the mass, and the adrenal gland (curved arrow) is displaced laterally. E. Photograph of the removed ganglioneuroma, which has been transected, and adherent right kidney.
324
Diagnostic Radiology
Fig. 2. CASE II. Abdominal aortogram, early arterial (left), late arterial (middle), and venous (right) phases. The mass (closed arrows) is supplied by branches from the ninth through twelfth intercostal arteries. Localized areas of neovascularity are seen (open arrows).
Fig. 3. CASE III. Abdominal aortogram, early arterial (left) and late arterial (right) phases. The large suprarenal mass is supplied predominantly by branches of the right inferior adrenal artery (closed arrows) arising from the renal artery. An accessory hepatic artery (open arrows) arises from the superior mesenteric artery. Several focal areas of neovascularity (arrows) are interspersed in an otherwise avascular mass.
325
326
N. REED DUNNICK AND RONALD A. CASTELLINO
May 1975
Fig. 4. CASE IV. A. Selective left middle adrenal arteriogram, early arterial (left), late arterial (middle) and venous (left) phases. There is abundant neovascularity with at least one cluster of more pronounced neovascular vessels (open arrows). There is opacification of a large draining periureteric vein (closed arrow). B. Photograph of resected ganglioneuroma, which has been sectioned, and left kidney. The tissue between the ganglioneuroma and kidney was neuroblastoma.
color. Microscopy demonstrated a definite capsule surrounding the tumor which consisted of ganglion cells interspersed among interlacing Schwann cell fascicles. Moderate pleomorphism of the ganglion cells was noted, although no mitoses were seen. Small foci of calcification were identified. The diagnosis was that of a benign ganglioneuroma. Postoperatively the patient had no further episodes of diarrhea, and increased in both stature and weight. CASE III: A 3 Y2-year-6Id white girl presented with a one-year history of profuse sweating and facial erythema. At the time of admission blood pressure was 200/170; during hospitalization she experienced paroxysms of drenching sweat, marked erythema, and blood pressure elevations to 240/180 from a baseline of 80/40. Laboratory studies revealed elevated urinary catecholamines, both epinephrine and norepinephrine, as high as 1042 and 520 micrograms per 24 hours, respectively. Excretory urography was not performed. Abdominal aortography disclosed a large mass in the right suprarenal region with inferior displacement of the right kidney and right renal artery (Fig. 3). The
mass was supplied predominantly by enlarged inferior adrenal artery branches arising from the right renal artery. Scattered focal collections of neovascular vessels were identified within the mass, with other areas devoid of any vascularity. Adjacent arteries, particularly of the overlying liver, were stretched by the mass. At surgery an 8 X 10-cm encapsulated mass in the right suprarenal region was removed. On cut section the tumor was firm and tan in color. Microscopy demonstrated a homogeneous mass of ganglion cells, interspersed with glial cells, neurofibrosis, and glialfibrosis interpreted as representing a benign ganglioneuroma. No pheochromocytes were found on multiple sections to suggest a pheochromocytoma. Following surgery the patient had no further episodes of periodic hypertension, sweating or erythema, and the previously elevated urinary catecholamines returned to normal levels. CASE IV: A 10-year-old white boy had a one-year history of unexplained abdominal pain. The pain became more severe and was associated with diarrhea, a 23-pound (10.4 kg) weight loss, and a
Vol. 115
ARTERIOGRAPHIC MANIFESTATIONS OF GANGLIONEUROMAS
palpable left upper quadrant mass. Endocrinologic work-up revealed elevated urinary vanillylmandelic acid. Excretory urography demonstrated a large left upper quadrant mass which contained fine calcifications and displaced the left kidney inferiorly. At surgery a large lobulated retroperitoneal tumor arose from the region of the left adrenal gland, extended to the left hemidiaphragm, and surrounded several major intra-abdominal vessels as well as the duodenum, and was considered nonresectable. Biopsy showed a neuroblastoma. Upon transfer to Stanford University Hospital, further laboratory studies revealed elevated urinary catecholamines and metanephrine, as well as vanillylmandelic acid. No evidence of metastasis was found. Arteriography was performed to delineate the extent of tumor for radiotherapy treatment planning. The left suprarenal mass displaced the left kidney and renal artery inferiorly, the splenic artery superiorly, and the celiac and superior mesenteric arteries to the right. The mass, which was supplied by a hypertrophied left middle adrenal artery. showed abundant neovascularity with nodular or focal collections of neovascularity more pronounced in some areas (Fig. 4, A). Large draining veins were seen but no arteriovenous shunting or contrast puddling was identified. Following 1400 rads to the tumor and chemotherapy, including vincristine, cyclophosphamide and adriamycin, the abdominal mass decreased in size. An attempt to surgically remove the tumor revealed a firm, predominantly left-sided yellowish mass adjacent to the left kidney, surrounding the aorta and inferior vena cava, and in close proximity to the right kidney. The bulk of the tumor as well as the left kidney and adrenal were removed, although residual tumor was left near the right kidney (Fig. 4, B). Careful histologic review of the tumor revealed that the bulk of the mass consisted of well-differentiated and unusually well-delineated ganglioneuroma, but a small area adjacent to the kidney had typical Homer-Wright rosettes characteristic of a more primitive neuroblastoma. Calcification was seen extensively throughout the tumor. Both the adrenal gland and kidney were normal.
327
Diagnostic Radiology
Fig. 5. Selective right renal arteriogram demonstrating arterial encasement (arrow) in a 5-year-old white girl with metastatic neuroblastoma confirmed at surgery.
DISCUSSION
Ganglioneuromas are more common in females than males and sixty per cent of patients are under the age of twenty (1, 3, 4, 11). Although patients are usually asymptomatic, chest pain, abdominal distension, ptosis and altered gait have been reported, presumably due to pressure (6). Additionally, hormonally active benign ganglioneuromas may cause diarrhea or episodic hypertension, sweating and flushing suggestive of a pheochromocytoma (4, 6). In a review of 417 cases of ganglioneuroma, 43 % were found in the mediastinum, 320/0 in the abdomen, and 8 % in the neck (3). Other sites included the mesentery, alimentary tract, genitourinary system, nasopharynx, tongue, leg and skin. Intratumoral calcification is common but is not helpful in determining malignancy (6). Deformities of adjacent bones due to erosion by the tumor mass and displacement of soft tissues are frequent radiographic findings. Histologically ganglioneuromas are composed of Schwann cells arranged in coarse interlacing fascicles. Mature ganglion cells may be found in compact clusters or arranged in an irregular fashion. Less mature ganglioneuromas and ganglioneuroblastomas show prominent vacuoles in the fascicles. Many cases have been reported of presumed maturation of neuroblastomas
into benign ganglioneuromas (2). All benign tumors must be carefully examined for less mature areas with sections from all parts of the mass in order not to miss a more malignant area. In each of our four cases a large vascular mass was demonstrated on arteriography. The vessels supplying the tumors were variable and dependent on the location of the mass. Stretching and displacement of adjacent opacified vessels were helpful in defining the extent of the tumor. Abundant neovascularity was present in each case. The neovascularity was not typical of that seen in the usual vascular malignant tumor (e.g., hypernephroma, hepatoma) in that no sudden changes in arterial caliber, arteriovenous shunting, contrast laking or puddling, or fine, bizarre vessels were seen. Instead, the appearance was more like extremely serpiginous and meandering vessels, tending to cluster in nodular areas, with adjacent avascular areas. Although in two of our cases the ganglioneuroma encircled major arteries to such an extent that they could not be dissected free, arterial encasement was not noted on the angiogram. Arterial encasement, however, may be seen on angiograms of neuroblastomas (Fig. 5) (7). The presence of the more typical appearance of neovascularity, as well as its more diffuse distri-
328
N. REED DUNNICK AND RONALD A. CASTELLINO
bution within the mass, and the presence of arterial encasement might serve to separate neuroblastoma from ganglioneuroma, since the latter may show a more serpiginous, meandering neovascular pattern which is irregularly clustered in the mass without arterial encasement. Nevertheless, at present we do not believe it is possible to distinguish with confidence the benign ganglioneuroma from the malignant neuroblastoma on the basis of the arteriographic findings. REFERENCES 1. Antonie TE: Ganglioneuroma presenting as an abdominal mass: report of a case. Aust NZ J Surg 42:71-74, Aug 1972 2. Aterman K, Schueller EF: Maturation of neuroblastoma to ganglioneuroma. Am J Dis Child 120:217-222, Sep 1970 3. Bunn ND Jr, King AB: Cervical ganglioneuroma. A case report and review of the literature. Guthrie Clin Bull 30:5-14, Jan 1961 4. Carpenter WB, Kernohan JW: Retroperitoneal ganglioneuromas and neurofibromas. A clinicopathological study. Cancer 16: 788-797, Jun 1963
May 1975
5. Fagan CJ, Swischuk LE: Dumbbell neuroblastoma or ganglioneuroma of the spinal canal. Am J Roentgenol 120:453-460, Feb 1974 6. Hamilton JP, Koop CE: Ganglioneuromas in children. Surg Gynecol Obstet 121:803-812, Oct 1965 7. McDonald P, Harwood-Nash DCF: Arterial stenoses in neuroblastoma. Am J Roentgenol 112: 167-169, May 1971 8. Nebesar RA, Fleischli DJ, Pollard JJ, et al: Arteriography in infants and children, with emphasis on the Seldinger technique and abdominal diseases. Am J RoentgenoI106:81-91, May 1969 9. Rossi P: Arteriography in adrenal tumors. Br J Radiol 41: 81-98, Feb 1968 10. Tassy R, Huguet JF: L'arteriographie dans les tumeurs sur-: renaliennes chez I'enfant. J Radiol Electrol Mad Nucl 55:173-176, 1974 11. Stout AP: Ganglioneuroma of the sympathetic nervous system. Surg Gynecol Dbstet 84:101-110, Jan 1947
Ronald A. Castellino, M.D. Department of Radiology Stanford University Medical Center Stanford, Calif. 94305
Diagnostic Radiology
N. Reed Dunnick, M.D. and Ronald A. Castelllno, M.D. The angiographic findings in four cases of ganglioneuroma are described. In all four cases a large vascular mass was demonstrated angiographically. The vessels supplying the tumors varied, depending on the location of the mass. Stretching and displacement of adjacent opacified vessels helped define the extent of the tumor. Abundant neovascularity was present with serpiginous vessels tending to cluster in nodular areas, with adjacent avascular areas. INDEX TERMS:
Ganglioneuroma. Neuroblastoma
Radiology 115:323-328, May 1975
ANGlIONEUROMAS are rare, benign tumors arising from sympathetic ganglia, and are usually found in the neck, posterior mediastinum or retroperitoneal space. Since these tumors frequently produce no symptoms, they are often discovered as an incidental finding on radiographs taken for other reasons and at autopsy. The radiographic findings are those of a soft-tissue mass, often ovoid in shape, which at times contains mottled calcifications. They may attain sufficient size to displace adjacent organs, and occasionally may present as a "dumbbell tumor" with neuroforaminal widening and spinal cord compression (5). There is little information regarding the angiographic manifestations of the ganglioneuroma. Bunn and King described a 51-year-Old man with a ganglioneuroma in the neck which was " almost avascular" on carotid arteriography (3). Antonie described a 45-year-old woman with a ganglioneuroma arising from the inferior pole of the kidney which was "avascular" on aortography (1). Among 20 cases of adrenal tumors, Rossi included a single case of a ganglioneuroma in a 20-year-old woman studied by aortography which showed small arteries which were "sparse, irregular, and displaced" (9). Nebesar et at. studied two retroperitoneal ganglioneuromas, one of which in a 7-year-old boy was described as showing "irregular tumor vessels" on selective lumbar arteriography (8). Tassy and Huguet presented findings of a hypervascular ganglioneuroma on a celiac arteriogram in a 17-year~0Id adolescent (10). This report describes the angiographic findings in four patients studied at Stanford Medical Center who had ganglioneuromas which were subsequently removed at surgery.
G
CASE REPORTS CASE I:
1
A 23-year-old white woman was found to have a large
irregularly calcified mass in the right upper quadrant on an upper gastrointestinal series which was obtained for intermittent midepigastric burning pain. She was otherwise asymptomatic with no specific metabolic or urologic complaints. The physical examination, routine laboratory values, and endocrinologic evaluation were all normal. An excretory urogram (Fig. 1, A) demonstrated intrinsically normal kidneys with inferior displacement of the right kidney by the calcified suprarenal mass. Abdominal aortography with selective right renal and middle adrenal arteriography disclosed a vascular mass which was supplied by branches of the right middle (Fig. 1, B) and right inferior (Fig. 1, C and D) adrenal arteries. There was abundant neovascularity clustered throughout the mass, with large avascular or hypovascular components. No arteriovenous shunting or contrast puddling was clearly identified. The right renal vein was markedly stretched over this mass. A small blush was interpreted as representing a laterally displaced adrenal gland. At surgery a 14-cm mass adherent to the renal vessels was found, and right nephrectomy and adrenalectomy were required (Fig. 1, E). The mass appeared encapsulated and on cut section was light tan in color, firm, and contained multiple small foci of calcification. Microscopy showed histologic features characteristic of a ganglioneuroma, with a predominance of Schwann cells. Multiple ganglion cells appeared singly and in clusters, and most were mature in appearance. Microscopy of the kidney and adrenal gland was normal. CASE II: A 2Y2-year-old white girl presented with a one-year history of bloodless diarrhea and failure to thrive. She was in the tenth percentile for height and weight. Physical examination was otherwise unremarkable as were routine laboratory studies and endocrinologic evaluation. An ovoid homogeneous right paravertebral mass was noted adjacent to the right lower thoracic spine on an upper gastrointestinal series. An excretory urogram was normal. A midthoracic and abdominal aortogram showed numerous neovascular vessels located within the mass, which arose from the ninth through the twelfth intercostal arteries (Fig. 2). The presence or absence of arteriovenous shunting or contrast puddling could not be evaluated with confidence on the basis of the aortogram alone. At surgery, a 5 X 8-cm mass was found in the right lower thoracic paravertebral area. The mass was firm in consistency and tan in
From the Division of Diagnostic Radiology, Stanford University Medical Center, Stanford, Calif. Accepted for publication in October 1974.
323
dk
Fig. 1. CASE I. A. Excretory urogram shows an intrinsically normal right kidney inferiorly displaced by a large calcified suprarenal mass. B. Selective right middle adrenal arteriogram demonstrates a large cluster of neovascular vessels (closed arrows) without encasement, arteriovenous shunting or laking. Large portions of the mass are hypovascular, with stretching of adjacent branches (open arrows). C and D. Selective right renal arteriogram, arterial and venous phases, demonstrates a prominent inferior adrenal artery (open arrow) stretched around the mass and giving rise to a single cluster of neovascularity (closed arrow). The renal vein is bowed by the mass, and the adrenal gland (curved arrow) is displaced laterally. E. Photograph of the removed ganglioneuroma, which has been transected, and adherent right kidney.
324
Diagnostic Radiology
Fig. 2. CASE II. Abdominal aortogram, early arterial (left), late arterial (middle), and venous (right) phases. The mass (closed arrows) is supplied by branches from the ninth through twelfth intercostal arteries. Localized areas of neovascularity are seen (open arrows).
Fig. 3. CASE III. Abdominal aortogram, early arterial (left) and late arterial (right) phases. The large suprarenal mass is supplied predominantly by branches of the right inferior adrenal artery (closed arrows) arising from the renal artery. An accessory hepatic artery (open arrows) arises from the superior mesenteric artery. Several focal areas of neovascularity (arrows) are interspersed in an otherwise avascular mass.
325
326
N. REED DUNNICK AND RONALD A. CASTELLINO
May 1975
Fig. 4. CASE IV. A. Selective left middle adrenal arteriogram, early arterial (left), late arterial (middle) and venous (left) phases. There is abundant neovascularity with at least one cluster of more pronounced neovascular vessels (open arrows). There is opacification of a large draining periureteric vein (closed arrow). B. Photograph of resected ganglioneuroma, which has been sectioned, and left kidney. The tissue between the ganglioneuroma and kidney was neuroblastoma.
color. Microscopy demonstrated a definite capsule surrounding the tumor which consisted of ganglion cells interspersed among interlacing Schwann cell fascicles. Moderate pleomorphism of the ganglion cells was noted, although no mitoses were seen. Small foci of calcification were identified. The diagnosis was that of a benign ganglioneuroma. Postoperatively the patient had no further episodes of diarrhea, and increased in both stature and weight. CASE III: A 3 Y2-year-6Id white girl presented with a one-year history of profuse sweating and facial erythema. At the time of admission blood pressure was 200/170; during hospitalization she experienced paroxysms of drenching sweat, marked erythema, and blood pressure elevations to 240/180 from a baseline of 80/40. Laboratory studies revealed elevated urinary catecholamines, both epinephrine and norepinephrine, as high as 1042 and 520 micrograms per 24 hours, respectively. Excretory urography was not performed. Abdominal aortography disclosed a large mass in the right suprarenal region with inferior displacement of the right kidney and right renal artery (Fig. 3). The
mass was supplied predominantly by enlarged inferior adrenal artery branches arising from the right renal artery. Scattered focal collections of neovascular vessels were identified within the mass, with other areas devoid of any vascularity. Adjacent arteries, particularly of the overlying liver, were stretched by the mass. At surgery an 8 X 10-cm encapsulated mass in the right suprarenal region was removed. On cut section the tumor was firm and tan in color. Microscopy demonstrated a homogeneous mass of ganglion cells, interspersed with glial cells, neurofibrosis, and glialfibrosis interpreted as representing a benign ganglioneuroma. No pheochromocytes were found on multiple sections to suggest a pheochromocytoma. Following surgery the patient had no further episodes of periodic hypertension, sweating or erythema, and the previously elevated urinary catecholamines returned to normal levels. CASE IV: A 10-year-old white boy had a one-year history of unexplained abdominal pain. The pain became more severe and was associated with diarrhea, a 23-pound (10.4 kg) weight loss, and a
Vol. 115
ARTERIOGRAPHIC MANIFESTATIONS OF GANGLIONEUROMAS
palpable left upper quadrant mass. Endocrinologic work-up revealed elevated urinary vanillylmandelic acid. Excretory urography demonstrated a large left upper quadrant mass which contained fine calcifications and displaced the left kidney inferiorly. At surgery a large lobulated retroperitoneal tumor arose from the region of the left adrenal gland, extended to the left hemidiaphragm, and surrounded several major intra-abdominal vessels as well as the duodenum, and was considered nonresectable. Biopsy showed a neuroblastoma. Upon transfer to Stanford University Hospital, further laboratory studies revealed elevated urinary catecholamines and metanephrine, as well as vanillylmandelic acid. No evidence of metastasis was found. Arteriography was performed to delineate the extent of tumor for radiotherapy treatment planning. The left suprarenal mass displaced the left kidney and renal artery inferiorly, the splenic artery superiorly, and the celiac and superior mesenteric arteries to the right. The mass, which was supplied by a hypertrophied left middle adrenal artery. showed abundant neovascularity with nodular or focal collections of neovascularity more pronounced in some areas (Fig. 4, A). Large draining veins were seen but no arteriovenous shunting or contrast puddling was identified. Following 1400 rads to the tumor and chemotherapy, including vincristine, cyclophosphamide and adriamycin, the abdominal mass decreased in size. An attempt to surgically remove the tumor revealed a firm, predominantly left-sided yellowish mass adjacent to the left kidney, surrounding the aorta and inferior vena cava, and in close proximity to the right kidney. The bulk of the tumor as well as the left kidney and adrenal were removed, although residual tumor was left near the right kidney (Fig. 4, B). Careful histologic review of the tumor revealed that the bulk of the mass consisted of well-differentiated and unusually well-delineated ganglioneuroma, but a small area adjacent to the kidney had typical Homer-Wright rosettes characteristic of a more primitive neuroblastoma. Calcification was seen extensively throughout the tumor. Both the adrenal gland and kidney were normal.
327
Diagnostic Radiology
Fig. 5. Selective right renal arteriogram demonstrating arterial encasement (arrow) in a 5-year-old white girl with metastatic neuroblastoma confirmed at surgery.
DISCUSSION
Ganglioneuromas are more common in females than males and sixty per cent of patients are under the age of twenty (1, 3, 4, 11). Although patients are usually asymptomatic, chest pain, abdominal distension, ptosis and altered gait have been reported, presumably due to pressure (6). Additionally, hormonally active benign ganglioneuromas may cause diarrhea or episodic hypertension, sweating and flushing suggestive of a pheochromocytoma (4, 6). In a review of 417 cases of ganglioneuroma, 43 % were found in the mediastinum, 320/0 in the abdomen, and 8 % in the neck (3). Other sites included the mesentery, alimentary tract, genitourinary system, nasopharynx, tongue, leg and skin. Intratumoral calcification is common but is not helpful in determining malignancy (6). Deformities of adjacent bones due to erosion by the tumor mass and displacement of soft tissues are frequent radiographic findings. Histologically ganglioneuromas are composed of Schwann cells arranged in coarse interlacing fascicles. Mature ganglion cells may be found in compact clusters or arranged in an irregular fashion. Less mature ganglioneuromas and ganglioneuroblastomas show prominent vacuoles in the fascicles. Many cases have been reported of presumed maturation of neuroblastomas
into benign ganglioneuromas (2). All benign tumors must be carefully examined for less mature areas with sections from all parts of the mass in order not to miss a more malignant area. In each of our four cases a large vascular mass was demonstrated on arteriography. The vessels supplying the tumors were variable and dependent on the location of the mass. Stretching and displacement of adjacent opacified vessels were helpful in defining the extent of the tumor. Abundant neovascularity was present in each case. The neovascularity was not typical of that seen in the usual vascular malignant tumor (e.g., hypernephroma, hepatoma) in that no sudden changes in arterial caliber, arteriovenous shunting, contrast laking or puddling, or fine, bizarre vessels were seen. Instead, the appearance was more like extremely serpiginous and meandering vessels, tending to cluster in nodular areas, with adjacent avascular areas. Although in two of our cases the ganglioneuroma encircled major arteries to such an extent that they could not be dissected free, arterial encasement was not noted on the angiogram. Arterial encasement, however, may be seen on angiograms of neuroblastomas (Fig. 5) (7). The presence of the more typical appearance of neovascularity, as well as its more diffuse distri-
328
N. REED DUNNICK AND RONALD A. CASTELLINO
bution within the mass, and the presence of arterial encasement might serve to separate neuroblastoma from ganglioneuroma, since the latter may show a more serpiginous, meandering neovascular pattern which is irregularly clustered in the mass without arterial encasement. Nevertheless, at present we do not believe it is possible to distinguish with confidence the benign ganglioneuroma from the malignant neuroblastoma on the basis of the arteriographic findings. REFERENCES 1. Antonie TE: Ganglioneuroma presenting as an abdominal mass: report of a case. Aust NZ J Surg 42:71-74, Aug 1972 2. Aterman K, Schueller EF: Maturation of neuroblastoma to ganglioneuroma. Am J Dis Child 120:217-222, Sep 1970 3. Bunn ND Jr, King AB: Cervical ganglioneuroma. A case report and review of the literature. Guthrie Clin Bull 30:5-14, Jan 1961 4. Carpenter WB, Kernohan JW: Retroperitoneal ganglioneuromas and neurofibromas. A clinicopathological study. Cancer 16: 788-797, Jun 1963
May 1975
5. Fagan CJ, Swischuk LE: Dumbbell neuroblastoma or ganglioneuroma of the spinal canal. Am J Roentgenol 120:453-460, Feb 1974 6. Hamilton JP, Koop CE: Ganglioneuromas in children. Surg Gynecol Obstet 121:803-812, Oct 1965 7. McDonald P, Harwood-Nash DCF: Arterial stenoses in neuroblastoma. Am J Roentgenol 112: 167-169, May 1971 8. Nebesar RA, Fleischli DJ, Pollard JJ, et al: Arteriography in infants and children, with emphasis on the Seldinger technique and abdominal diseases. Am J RoentgenoI106:81-91, May 1969 9. Rossi P: Arteriography in adrenal tumors. Br J Radiol 41: 81-98, Feb 1968 10. Tassy R, Huguet JF: L'arteriographie dans les tumeurs sur-: renaliennes chez I'enfant. J Radiol Electrol Mad Nucl 55:173-176, 1974 11. Stout AP: Ganglioneuroma of the sympathetic nervous system. Surg Gynecol Dbstet 84:101-110, Jan 1947
Ronald A. Castellino, M.D. Department of Radiology Stanford University Medical Center Stanford, Calif. 94305
