The many faces of neuroblastoma.

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solid, extracranial tumor in infants and children. it is the third most common malignant ..... (arrows). (B) A CT scan of the facial bones demonstrates the tumor.

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RadloGraphlcs Index terms: GENITOURINARY IMAGING #{149} Adrenal MUSCULOSKELETAL IMAGING #{149} General/Multiple sites PEDIATRIC IMAGING #{149} GnItourlnary #{149} Musculoskeietal RADIATION THERAPY #{149} General/Multiple Sites

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The many faces neuroblastoma

cumulative Index terms: Neurobiastoma Neoplasms In Infants and children

Ruppert Neela

David, Lamki,

Susan Fan, Edward

M.D.’

of

1

Farzin Eftekhari,

M.D.t

Ali Shirkhoda,

M.D.t

M.D.

Pajendra

M.D.*

B. Singleton,

Kumar,

John E. Madewell,

M.D.*

M.D.1 M.D.

Absfract: Neurobiastoma is a common tumor in childhood. It arises in the adrenal gland or In various extraadrenalprimaiysites of the sympathetic chain. Clinically, it maypresent as an abdominal mass or as dissemInated metastatlc disease. We studied 52 patients with neuroblastoma, and the typical and unusual radiographic features ofthe disease are presented.

THIS EXHIBIT WAS DISPLAYED AT THE 74TH SCIENTIFIC ASSEMBLY AND ANNUAL MEETING OF THE PADIOLOGICAL SOCIETY OF NORTH AMERICA. NOVEMBER 27-DECEMBER 2. 1988. CHICAGO. ILLINOIS. IT WAS RECOMMENDED BY THE GENERAL RADIOLOGY AND MAGNETIC RESONANCE IMAGING PANELS AND WAS ACCEPTED FOR PUBLICATION AFTER PEER REVIEW AND REVISION ON APRIL. 1989.

From

the

Departments

Introduction Neuroblastoma is the most common solid, extracranial tumor in infants and children. it is the third most common malignant neoplasm of childhood, after leukemia and brain tumors. It represents only 7% of all cases of childhood cancer, yet results in 15% of cancer deaths in children. Next to Wilms’ tumor, it is the second most common intraabdominal tumor in children. Neuroblastomas are of neural crest origin, and most cases arise in the adrenal gland. Less often, the tumor may arise in other extraadrenal sites along the sympathetic chain, particularly in the retropenitoneal region of the abdomen and the posterior mediastinum (Table I). It is a sarcoma of nervous system origin, composed chiefly of neuroblasts, but the tumor has an enigmatic clinical character, and its pathogenesis is unknown.

of

Radiology, Baylor College of Medicine (#{149}) and the Univensity of Texas, M.D. Anderson Hospital and Tumor Institute (t), Houston, Texas; The University of Texas Medical Branch, Galveston, Texas (t); and the Milton S. Hershey Medical Center. The Pennsylvania State University. Hershey, Pennsylvania (s). II Presently

TABLEI PrImary Sites of Neurobiostomo (%)

Site

Percentile

Abdomen Adrenal Glands

36%

Extroadrenal Bilateral

18%

Adrenals

7-0%

Posterior Mediastinum of the Thorax Neck

at RSJ Corpo-

5%

Pelvis

nation, 30680 Bainbnidge Road, Solon. OH, 44139. Address reprint requests to N. Lamki, M.D., Department of Radiology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030.

14% 5% 2% 10% 10%

Brain

Other Unknown #{149} Reference 8.

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Wilms’ tumor, rhabdomyosancoma or liven neoplasm. An adrenal hemorrhage should also be considered in the newborn. The radiologist should also be cognizant of the adrenal and extraadrenal primary locations of this tumor. This article illustrates many nadiologic featunes of neuroblastoma on plain radiography, computed tomography (CT), ultrasonography (US), radionuclide scintigraphy, magnetic resonance imaging (MRI) and angiography.

Approximately 90% of neunoblastomas are manifested clinically in the first eight years of life, and roughly 50% of the patients are under two years of age at the onset of the lesion. Infrequently, the tumor may occur in olden children, adolescents and young adults. Both sexes are affected equally. The estimated number of new cases in the United States was approximately 500, in 1988. The differential diagnosis of a solid abdominal mass in a child includes neunoblastoma, Clinical

Presentation

The clinical presentation of neunobiastoma depends on the site of the primary lesion or 10cation of its metastatic spread. A palpable abdominal mass is present in 54% of patients, along with abdominal distention. The tumor may also appear as a lower abdominal or pelvic mass arising from the organ of Zuckerkandl (a cluster of paraganglia near the aortic bifuncation) on from the sympathetic chain in the netnopenitoneum. Pain and fever occur in approximately one-third of patients. Remote effects of this cancer include the opsoclonusmyoclonus paraneoplastic syndrome and ecchymosis. Opsoclonus is a sign of cenebellar disease in which the patient has spontaneous, conjugate, and chaotic eye movements, particulanly when voluntary eye movement is attempted. There Is often associated myoclonus of the trunk and extremities, with cerebellar ataxia. This complex may be detected in onefourth of patients with neuroblastoma. Another paraneoplastic complex consists of intractable watery diarrhea with hypokalemia and achlonhydnia. It occurs in up to 9% of patients, and is caused by increased amounts of a vasoactive intestinal polypeptide (VIP) secreted by the tumor. There is in almost all cases an increase in the levels of catecholamines and their byproducts. Vanillylmandelic acid (VMA), homovanillic acid (HVA) and cystathionine are found in the urine in oven 90% of patients, and can

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serve as clinical biological markers. Up to 30% of the patients may be hypertensive because of the excessive secretion of these hormones. Both norepinephnine and epinephnine produce profound additional metabolic effects including increased lipolysis, elevated oxygen consumption, a rise in body temperature, and hyperglycemia. Neurobiastoma may occasionally be present at birth, in either the localized or disseminated form. Neuroblastoma may rarely appear to be a familial tumor (0.2% annually), and it may be associated with aganglionosis of the bowel or congenital heart disease. The tumor has even been found in premature stillborn infants. In up to 60% of patients, a metastasis is the first manifestation of the disease to be necognized. The most common metastatic sites are the skeleton, regional lymph nodes and liven (Table II), Bone marrow involvement is noted histologically in roughly 50% of patients subjected to biopsy. The presence of a primary adrenal neunoblastoma with extensive skeletal metastases, particularly in the skull with proptosis and bone pain, has been referred to as Hutchinson’s syndrome. Pepper’s syndrome represents massive hepatomegaly caused by metastatic neuroblastoma and a primary adrenal tumor. When there are multiple metastatic skin lesions in the neonate, it is referred to as the “blueberry muffin” syndrome.

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TABLEII Frequent Sites of Neurobiastoma Metastasis Site

Percentage

of Patients

3

60%

Skeletal Lymph

.5

.0 .

Nodes

(Regional)

0

42%

Liver Intracranial

15%

Pulmonary

10%

0 3

14%

Pathology The tumor may be a barely discernible nodule or a large mass. Grossly, the tumor is round, irregular and lobulated, usually weighing 50 to 150 grams on more. It is soft and fniable on palpation and can easily fall apart on sectioning. The cut surfaces show dark areas of hemorrhage and necrosis (Figure IA). Calcification is frequently found within the tumor, Microscopic studies reveal small round cells

(slightly larger than lymphocytes) of neural crest origin, with scant cytoplasm (20 microns). The nuclei are oval or spherical in shape, with conspicuous nucleoli (Figure IB). High power microscopic sections show Homer-Wright nosettes consisting of one or two layers of neuroblasts surrounding a central zone of tangled neurofibnillary processes (Figure I C).

Figure 1A The cut surface of a neuroblastoma shows a lobulated appearance. Note the areas of hemorrhage (anrows) and necrosis (arrowheads).

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hC Figures 1B & C (B) A photomicrograph shows multiple small oval and round cells typical of a neuroblastoma. (C) This photomicrograph shows a Homer-Wright rosette within the tumor (arrow). A typical rosette has one or two layers of neuroblasts surrounding a central zone of tangled neunofibniliary processes. (Courtesy of Dr. Rolando Estrada, Houston, Texas)

A. Primary I. INTRAABDOMINAL

Neurobiastoma If the kidney displacement is inferolateral, a “drooping lily” sign will be seen. The tumor may cause ureteral obstruction and hydronephrosis (Figure 6). Sonography is the screening examination of choice for pediatric abdominal masses. On songraphy, the mass is usually hyperechoic (Figure 7). Acoustic shadows, which represent calcification within the tumor, may be seen behind the mass (Figure 8). lntraabdominal metastases may also be detected during the initial screening procedure with sonography.

NEUROBLASTOMA

Up to 75% of neuroblastomas occur in the abdomen, and 50% of these tumors occur in the adrenal gland. Plain radiognaphs usually show an abdominal mass, with bowel gas displacement. Elevation of the ipsilateral diaphragm may also be evident. Approximately 10% of the adrenal tumors are bilateral, About 60% of these tumors show calcifications on radiographs (Figures 2, 3 and 4). An excretory urognam will usually demonstrate displacement of the adjacent kidney (Figures 2 and 5).

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Figure 2 (A) This IVP shows a ganglioneuroblastoma1 arising in the right adrenal, with peripheral curvilinear calcifications along its inferior aspect. (B) CT shows the calcified tumor arising in the right adrenal.

3B Figure 3 (A) A large, heavily calcified ganglioneuroma arising in the right adrenal is seen in this abdominal radiograph. Note that the large mass crosses the midline and displaces bowel loops. (B) A radionuclide bone scan with Tc-99m MDP reveals uptake of the tracer in the calcified portion of the tumor (arrows).

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Figure 4 (A) A left adrenal neuroblastoma shows the presence of coalescent amorphous calcifications. (B) A selective left adrenal arteriogram reveals fine tumor vessels, which are stretched and displaced. Note the areas of hypervasculanity within the mass.



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Figure 5 (A) A left extraadrenal, retropenitoneal neunoblastoma is causing widening of the left psoas shadow and lateral displacement of the pnoximal left ureter. Bowel loops are also displaced. (B) An aortognam shows the mass to be hypovasculan. Note the contnalateral displacement of the abdominal aorta and the stretching of arteries on the left.

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(A)Th IVP shows a large neuroblastoma which arose in the organ of Zukerkandl. Note that the unnary bladder is displaced to the right, and the uretens are compressed and laterally deviated, resulting in bilateral hydnonephrosis. (B) A venacavognam

shows marked elevation and displacement of the left common iliac vein with tethering of the right cornmon iliac vein. Collateral venous channels are present in the pelvis and abdomen (arrows).

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Figure 7 Sonography shows a ganglioneuroma that is seen as a hypoechoic mass, M, anterior to the right kidney, K.

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Sonography shows a neuroblastoma as a solid mass in the right adrenal. Note the acoustic shadowing produced by calcifications within the tumor.

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CT and MRI are complementary imaging methods in the diagnosis and staging of the tumon. These techniques facilitate delineation of the tumor, and can detect intraabdominal metastases (Figures 9 and 10). CT is the imaging mode of choice for staging neuroblastoma in all sites, and is of further aid in the detection of calcification within the tumor, which may not be apparent on plain radiographs (Figure I I). Intraspinal extension from paraspinal tumors occurs in approximately 15% of patients, and can be better seen on CT and MR1 than on plain radiographs (Figure 12). The advantages of MRI relative to CT indude the ability to produce images in multiple planes, the elimination of ionizing radiation and intravenous contrast agent, and the improvement in tissue contrast resolution. Encasement of the abdominal vessels can also be appreciated. Multidirectional imaging is of benefit for preoperative surgical and radiation therapy planning. The role of angiography in the diagnosis and staging of neuroblastoma has been limited since the advent of CT and MRI, but may be useful in difficult cases (Figures 4 and 10). Neuroblastoma is a relatively hypovascular tumor, and the common angiographic findings mainly consist of stretching and displacement of vessels, which drape around the mass.

IOA

hOB Figure 10 (A) A CT section of the pelvis shows a ganglioneuroblastoma that arises in the pelvis and is seen as a dumbbell shaped mass, M. Note the intraspinal extension with bone destruction, and also the displacement of the bladder, B. (B) A selective left hypogastnic arteniogram performed from a night fernoral approach shows subtle hypervasculanity, with no definite neovasculanity. The vessels are encased, stretched and displaced by the mass. Figure 9 An MR image (T2 weighted) shows a neunoblastoma arising in the left adrenal as a large mass with diffusely increased signal intensity.

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lhB Figure 1 1 (A) This chest radiograph (PA view) shows a rnediastinal ganglioneuroma that extends into the left hernithorax. Note the posterior left nib erosions and asymmetric intercostal spaces. (B) A thonacic CT scan depicts small intnatumonal calcifications within the tumor that are not demonstrable on the plain radiograph.

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12C Figure 12 (A&B) Chest radiographs (PA and lateral views) show a primary neuroblastorna arising in the left posterior mediastinurn. Note the mediastinal shift to the right and anteriorly. The left posterior ribs are eroded by the tumor. (C) A CT scan reveals that the soft tissue mass, M, is causing bone erosion, with vertebral foraminal encroachment and widening. Note spinal canal invasion, Ml, and cord, C, displacement. (D) A sagittal MR image (Ti -weighted) confirms the presence of intra- and paraspinal extensions of the tumor.

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II. INTRATHORACIC

NEUROBLASTOMA

III. INTRACRANIAL

faces

of neuroblastoma

NEUROBLASTOMA

Primary intracranial neuroblastomas occur in 2% of patients. Most commonly, the tumor arises in the olfactory nerve (esthesioneuroblastoma), cerebellum or cerebrum, On radiographs, bone destruction may be seen (Figures 13 and 14). CT and MRI reveal the presence of an intracranial, space occupying, soft tissue mass. Peripheral tumors may be associated with either lytic or sclerotic bone changes in the skull (Figures 13 and 14). The tumors that arise in the posterior fossa may cause hydrocephalus.

Primary intrathoracic neuroblastomas comprise 14% of all neurobiastomas. Initially, the patients may complain of chest pain, and cough because of airway obstruction. The chest radiograph will demonstrate a mass, and there may be extrinsic compression and displacement of the main bronchus in the case of a large tumor. Thoracic neunoblastomas usually occur in the posterior mediastinum, where they arise from the paravertebral sympathetic chain. Rib erosion (Figures 1 1 and 12) and intraspinal extension may occur (Figure 12). Loss of vertebral height with widening of the neural foramina may be present. Large masses may cause mediastinal shift (Figure 12). A metastatic neck mass may result in Homer’s syndrome and a superior vena caval syndrome.

Figure 13 (A) A plain radiograph shows a neuroblastorna arising in the anterior cranial fossa and causing erosion of the medial wall of the right orbit (arrows). (B) A CT scan of the facial bones demonstrates the tumor involving the night orbit with destruction of sphenoid and ethmoid bones.

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h4A Figure 14 (A) This lateral view of the skull shows a primary infracranial neuroblastoma of the olfactory nerve (esthesioneuroblastoma). Note the destruction of the pituitary fossa. (Courtesy of Dr. Bao-Shan Jing, Houston, Texas) (B) A CT scan shows destruction

B. Secondary I. SKELETAL

of the greater wing of the sphenoid and of the ethmoid bone as the tumor extends into the right middIe cranial fossa. The right orbit is also involved. Note the pnoptosis on the right.

Neuroblastoma

METASTASES

metaphyseal line (Figure 20); (4) vertical linear radiolucent streaks in the metadiaphysis of a long bone (Figure 21); (5) a pathologic fractune (Figure 22); (6) multicentnic lytic lesions (Figure 23); (7) vertebral collapse (Figure 24); and (8) metastases to the cranium, often manifested as widening of the cranial suture lines because of subjacent dural metastatic lesions (Figure 25). With healing, the osteolytic lesion may become sclerotic (Figures 26 and 27). Metastatic bone lesions may mimic other round cell tumors, such as Ewing’s sarcoma, rhabdomyosarcoma, leukemia and lymphoma on radiographs.

Skeletal metastases occur in up to 60% of cases. Bone scintigraphy with Tc-99m MDP is useful as a survey method for the detection of metastatic bone lesions. The radionuclide tracer may also be taken up by the primary tumon (Figure 3). Early skeletal lesions may be missed when cortical destruction is limited, and a complementary radiographic bone sunvey is advocated to detect the subtle intramedullary changes of insidious disease. MRI can also detect early bone metastases. Radiographically, a skeletal metastasis may manifest itself as: (I) a peniosteal reaction (Figures 15 and I#{243}); (2) an osteolytic focus (Figures 17, 18 and 19); (3) a lucent horizontal

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This radiograph of the left knee (AP view) shows fine, “hair on end” peniosteal reaction in the distal femur (arrows).

Figure 17 A radiograph of the right shoulder reveals a small oval, lytic metastatic lesion in the proximal humerus (arrow).

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Figure 18 This radiograph of the right knee shows an osteolytic metastasis in the distal metaphysis that extends across the physis into the epiphysis of the right femur.

Figure 19 A radiograph of the right knee shows a moth eaten pattern of bone destruction in the proximal tibia.

Figure 20 A radiograph of the left elbow demonstrates permeative bone destruction and a lucent metaphyseal line (large arrow) in the proximal radius. Peniosteal reaction is present in the distal humerus (small arrow).

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Figure 22 A radiograph of the left knee (AP view) shows a permeative lesion with peniosteal reaction and an undisplaced pathologic fracture in the proximal tibia, TIEs radiograph

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Figure 23 This radiograph of the tous osteolytic skeletal the partial obstruction ten produced by nodal

pelvis shows ubiquimetastases. Note of the left distal uremefastases.

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Figure 24 This lateral view of the lumbar spine shows metastases causing marked vertebral collapse of L5.

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25B Figure 25 (A) This lateral skull radiograph shows widened cranial sutures caused by dunal metastases. (B) A followup radiograph, i 8 months later, shows progressive widening of the sutures despite treatment.

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radiograph (lateral view) shows mixed sclerotic and lytic bone lesions after nine months of systemic chemotherapy.

An AP view of the skull demonstrates an untneated mixed lytic and sclerotic metastasis in the frontopanietal region.

Thisskufl

II. INTRACRANIAL AND MAXILLOFACIAL METASTASES

ures 26 and 27). Bone sclerosis is often seen and probably represents a reactive process. Direct bone metastasis to the skull may produce a lytic lesion. 3. Dural metastatic involvement usually is seen as widening of the cranial sutures on plain radiographs (Figure 25). 4. CT and MR1 can easily detect most intracranial and craniofacial metastases (Figures 28 and 29). CT is particularly helpful for the detection of recurrent disease.

I . Intracranial metastasis is commonly seen as a space occupying lesion, and may be manifested clinically by cranial nerve deficits and bone destruction. 2. Metastases may occur in the duna and brain substance. When large, dural metastases may compress the brain and cause destruction of the bone of the overlying cranium (Fig-

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Figure 28 A CT scan of the skull shows a dural metastasis in the night panietal region, which extends into the adjacent bone.

III. INTRATHORACIC

Figure 29 A CT scan of the facial bones shows a neuroblastoma metastasizing to the intraconal area of the orbit and the ethmoid sinus on the right.

METASTASES

Metastatic lesions of neurobiastoma are often seen in the thorax. Pulmonary metastases may occur as nodular infiltrates (Figure 30). Bone metastases are often present in the rib cage and thonacic spine. Rib erosions may also occur as a result of direct involvement by a pnimary neuroblastoma arising in the thorax (Figures I I, 12 and 31). A nadionuclide bone scan may be required for the detection of skeletal lesions. Mediastinal and retrocrural lymphadenopathy is commonly present (Figure 32).

Figure 30 This chest radiograph shows left pulmonary metastases from an adrenal neunoblastoma.

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Figure 31 A chest radiograph static rib erosions in the left posterior any to a mediastinal ma. Note the right sis.

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Figure 32 An 1VP shows a left adrenal neuroblastoma displacing the left kidney infeniorly and laterally, producing the “drooping lily” sign. Note the nefrocrural mediastinal lymphadenopathy (black arrows) with calcification (arrowhead).

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METASTASES

33 and 34), bones (Figure 9), the mesentery (Figures 34 and 35), and pelvic organs (Figure 36). At times, calcification may be seen in intraabdominal metastatic lesions. After thenapy, CT and MRI are helpful in detecting recurrent disease,

Intnaabdominal metastases usually nepresent spread from an abdominal primary neuroblastoma. These metastases may involve vanous abdominal viscera, lymph nodes (Figures

Figure 34 This CT scan of the abdomen shows metastatic neunoblastoma with cystic degeneration within the tumon (large arrows), night kidney invasion (small annow) and retnopenitoneal metastases (arrowheads).

Figure 33 Sonography reveals netropenitoneal lymphadenopathy in a child with a left adrenal neunoblastoma. Note the anterior displacement of the aorta.

Figure 35 This MR image shows a right adrenal neuroblastoma (arrows), with mesentenic and retropenitoneal metastases, that was discovered at surgery. Note the night hydnonephrosis, the displaced abdominal vessels, and the mixed signal intensity in the night abdominal mesentery.

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Figure 36 (A) A contrast medium filled urinary bladder from an IVP shows extninsic compression and displacement because of a metastatic left pelvic mass, arising from a left adrenal neuroblastoma. (B) This transverse sonogram demonstrates a left pelvic mass representing metastatic lymph nodes on the left side of the urinary bladder. (B = bladden, M = mass)

Therapy The stage of the tumor will determine the mode of its therapy, but surgical extirpation is the most definitive mode of therapy, complemented with adjuvant chemotherapy and inradiation (Table III). Preoperative irradiation and chemotherapy are utilized to debulk the tumor and to “down stage” the disease in selected cases. Nonsungical therapy may also be help-

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ful in the relief of pain caused by bone metastases. Radioiodine labeled meta-iodobenzylguanidine (1311-MIBG) has been used for diagnosing, staging and treating Stage IV neuroblastoma in children, and it may gain greaten currency as more experience is achieved.

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TABLEIII

S ‘C

Anatomic Staging and Pro9nosis of Neuroblastoma

I-

Stage

Definition

Therapy

Prognosis

I

Tumor limited to primary organ

Surgery

II

Tumor extends in continuity to ip-

Surgery in combination with radi-

silateral structures, the homolateral regional lymph nodes,

80% Survival Rate

ation therapy or both.

60% Survival Rate

or chemotherapy

but does not cross the midline.

III

Tumor extends across the midline and may involve regional lymph nodes bilaterally.

V

IV-S

tion therapy motherapy.

or with

Stage I or II Tumor, with metastasis confined to one or more of

Variable Therapy

sites:

liver,

motherapy

and radiation

30% Survival Rate

che-

Combinations of surgery, che-

or bone

7% Survival Rate

thera-

py. 75%-87% Survival Rate with various forms of therapy or no therapy, particularly under 1 2

skin

marrow.

months

of age.

Reference 5.

Natural

Course

of Neuroblastoma

Although neunoblastomas are malignant tumors, their clinical course, evolution, and response to therapy vary greatly. Currently, the physician’s ability to image neuroblastoma far outweighs his ability to treat the disease, panticularly in patients with metastases. The specific location of the metastases, the age of the patient, and the stage of the tumor on mitial recognition, are important prognostic factons. Neuroblastoma tends to be clinically less aggressive in infants under one year of age, and this group has a greater survival rate. Neunoblastomas arising in the upper abdomen have a less favorable prognosis than those arising in the thorax and pelvis. Also intriguing is

880

alone

Tumor metastasizes to remote areas of skeleton, soft tissues, distant lymph nodes and other organs.

the following

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the potential of a neuroblastoma for matunation to a well differentiated, clinically less aggressive, and indeed benign neoplasm, the ganglioneunoma. Such maturation to ganglioneunoma is reported to occur in 0.2% of patients with neunoblastoma. It may occur either “spontaneously” on following treatment (Figune 37). The underlying mechanism of the spontaneous maturation is still obscured. Mixed pathologic forms of the tumor, such as ganglioneuroblastomas, have also been neported (Figure 9). Secondary malignant neoplasms may occur in the previously irradiated sites in the survivors of neuroblastoma,

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37B Figure 37 Maturation of neunoblastoma to ganglioneunoma. (A) This chest radiograph shows a right neck mass, displacing the trachea to the left. (B) A followup chest radiograph taken eight years after chemotherapy, shows that the neck mass has markedly regressed in size. A biopsy revealed it to be a benign ganglioneunoma.

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Suggested 1. Daneman A. Adrenal neoplasms In children. Semin Roentgenol 1988; (23:205-215. 2. Lopez-Ibor B, Schwartz AD. Neuroblastoma. Pediatr Clin North Am 1985; 32:755-778. 3. Stark DD. Moss AA, Brasch RC. et al. Neuroblastoma: agnostic imaging and staging. Radiology 1983;

9. Evans AE. D’Angio GJ. Koop CE. Diagnosis and treatment of neuroblastoma. Pediatr Clin North Am 1976;

23:161-170. 10. Evans AE. Natural history of neuroblastoma. In: Evans AE. ed. Advances in neuroblastoma research. New York: Raven. 1980. 3-12. I I . Kumar R. David R. Sayle BA. et al. Adrenal scintigraphy. Semin Roentgenol 1988; 23:243-249. 12. Munkner T. 131I-meta-iodobenzylguanidine scintlgraphy of neuroblastomas. Semin NucI Med 1985; 15:154-

Di-

148:101-105.

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Figure 2 previously (Chicago: Year Book We wish to thank this manuscript.

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160. 13. Hadley GP, Rabe E. Scanning with iodine-131 MIBG in children with solid tumors: An initial appraisal. J NucI Med 1986; 27:620-626. 14. Harbert JC. Meta(131)iodobenzylguanidine therapy of malignant pheochromocytomas and other neuroendocrine lesions. In: Nuclear medicine therapy. New York: Thieme, 1987; 99-108. 15. Rubin E. Aponte GE. Farber JL. Pathology. Philadelphia: Lippincott. 1988.

terms: appeared in Radiologic Oncology Medical. 1988 pages 723 and 725). Mrs. Becky Baxter and the supporting

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4. Appelbaum FR. Intensive chemotherapy or chemoradiotherapy with autologous marrow support as treatment for patients with solid tumors. Hematol Oncol Clin North Am 1988; 2:345-352. 5. Finklestein JZ. Neuroblastoma: The challenge and frustration. Hematol Oncol Clin North Am 1987; 1:675-694. 6. Stout AP. Ganglioneuroma of the sympathetic nervous system. Surg Gynecol Obstet 1947; 84:101-110. 7. Jaffe N. Neuroblastoma: Review of the literature and an examination of factors contributing to its enigmatic character. Cancer Treat Rev 1976; 3:61-82. 8. Page D. DeLellis R. Hough A. Tumors of the adrenal. In: Atlas of tumor pathology. Washington. DC: Armed Forces Institute of Pathology, 1985.

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