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Abdominal Mass
Abdominal Mass
3-1
infantile hypertrophic pyloric stenosis 3-6
3 2
90
( 3-1) 4
(hydronephrosis) multicystic dysplastic kidney
(intestinal
duplication), meconium pseudocyst
(Hirschsprungs disease) choledochal cyst
(hydrop gallbladder)
sacrococcygeal teratoma
neuroblastoma, Wilms tumor teratoma
neuroblastoma Wilms tumor 20
teratoma
(hematocolpos)
( 3-1)
3-1
0-1
Retroperitoneum
Renal
(posterior urethral valve)
pylorus infantile hypertrophic pyloric stenosis hepatoblastoma
sacrococcygeal teratoma choledochal
cyst
pancreatic pseudocyst
(pressure symptoms)
(urinary retention) sacrococcygeal teratoma
( 3-2)
opsoclonus-myoclonus
catecholamine metabolite ganglioneuroma ganglioneuroblastoma
secretory diarrhea vasoactive intestinal peptide
neuroblastoma5 Cushings corticosteroid
adrenocortical carcinoma
paraneoplastic syndrome
(systemic symptoms)
(metastatic
symptoms) neuroblastoma blueburry
muffin syndrome
hepatocellular carcinoma
(retroperitoneum)
(intraperitoneum) (anterior
abdomen) (pelvis)
(bimanual palpation)
sacrococcygeal teratoma
neuroblastoma Wilms tumor
(peritoneal cavity)
4 quadrants 9
(A)
(B )
3-2
BeckwidthWiedemann6,7
11p15.5
macrosomia, umbilical hernia or
embryonal tumor:
loss of
omphalocele, hemihyperplasia,
hepatoblastoma, Wilms tumor
imprinting visceromegaly, neonatal hypoglycemia
Denys-Drash8
WT1
WAGR association 9
WT1
Familial adenomatous
APC
intestinal polyposis
Wilms tumor
Colon cancer
circum-oral melanosis,
colon cancer, gastric cancer,
mucocutaneous lesions at palms
breast cancer and pancreatic
and soles, harmartomatous polyposis cancer
Turner syndrome
X
short stature, lymphadema,
monosomy broad chest, low hair-line,
low-set ears and web neck
gonadoblastoma, meningioma,
childhood brain tumor10
Kleinefelter syndrome
47XXY
Neurofibromatosis
type 1
NF1
neurofibroma, neurofibrosarcoma,
optic nerve tumor
von Hippel-Lindau
disease9
VHL
no specific dermatological or
developmental defects. Diagnosis
is usually made by index tumors:
cerebellar hemangioblastoma, retinal
angioma, pheochromocytoma and
renal cell cancer
Tuberous sclerosis12,13
TSC1
and
TSC2
quadrant
hepatocellular carcinoma
(Caf Ole spots) neurofibromatosis
( 3-3) ( 3-4)
Peutz-Jeghers
(hermaphrodite)
seminoma
testicular
3-3
Caf au lait spot neurofibromatosis
5
15
6
10
2,4,14
1.
2.
3.
4.
5.
6.
(radiologic
intervention)
3-4
(circumoral melanosis)
Peutz-Jeghers
11
neuroblastoma Wilms tumor
teratoma
plain x-rays 2,4 neuroblastoma
50-65 teratoma 50 Wilms tumor 3-15
5 2
retroperitoneal mass
()
(chest x-rays)
(ultrasonography)
12
Wilms tumor inferior vena cava15
color Doppler
(gold standard)
1
( 3-6)
CT scan resectability
13
neuroblastoma
3-6 CT scan 4
() ()
hepatoblastoma
enhancement
CT scan with contrast ()
14
, )
approach IVP
Wilms tumor neuroblastoma
IVP
IVP
CT scans
IVP
VCUG
posterior urethral valve
upper GI study
duodenum GI follow-through Barium enema
hypertrophic pyloric stenosis, lymphoma
barium enema
(pneumatic reduction)
diagnostic angiography, inferior venacavography
(radioactive substance)
99mTc diethylenetrianminepentaacetic acid scan (DTPA scan)
(ureteropelvic junction
15
obstruction)
4-6 (glomerular filtration rate)
17 99mTc methylene diphosphonate (MDP scan, bone scan)
osteoblast 131I metaiodobenzyl guanide
scan (MIBG scan) pre-synaptic sympathetic analog
neuroblastoma pheochromocytoma
5
Tumor marker
urinary vanillylmandellic acid (VMA) homovanillic acid (HVA)
neuroblastoma 70-90 17 ( 1)
alphafetoprotein (AFP) 90 hepatoblastoma
50 hepatocellular carcinoma18 AFP
(liver
regeneration)
1
1 tumor marker
Catecholamine metabolites:
Catecholamine dopamine, norepinephrine
epinephrine
Norepinephrine dopamine dopaminergic noradrenergic neurons sympathetic chromaffin cells
epinephrine norepinephrine adrenal medulla
metabolism catecholamine homovanillic acid (HVA)
vanillylmandelic acid (VMA)
(screening)
sympathetic adrenal medulla neuroblastoma pheochromocytoma
catecholamine excretion function
24 creatinine
HVA VMA 35 20 mg/g
16
1 ()
creatinine 0.50-6.55 mg/g creatinine
HVA urine VMA
tricyclic antidepressant
Ikeda H, Matsunaga T, Tsuchida Y. Hepatic tumors. in Voute PA, Barret A, Steven MCG,
Caron HN (eds) Cancer in Children: clinical management 5th edition, London, Oxford University
Press, 2005
17
(fine needle biopsy or core needle biopsy) ( 2)
(laparoscopic biopsy) ( 3-7)
RNA ultrafreezer
-80
neuroblastoma
urine VMA20,21
Neuroblastoma
Rhabdomyosarcoma
Immunohistochemistry
t(X;1)
1p deletions,
double minutes,
homogeneous
staining regions
(non specific)
t(11;22), t(21;22)
t(1;13), t(2;13)
Cytogenetics
PRCC;TFE3
MYCN amplification
(also seen in some
alveolar RMS)
FLI-1;EWS or
ERG;EWS
PAX3;FHKHR or
PAX3;FKHR
Molecular genetics
18
19
2
(Core needle biopsy)
11-16 G
(biopsy gun)
(imaging guidance)
( 2 x 0.16 )
(heterogeneity) teratoma
(soft tissue or bone sarcoma)
(mitotic figure)
2.
(tissue plane)
20
2 ()
3.
4% paraformaldehyde
4.
(orientation)
Rosenberg SA. Principles of surgical oncology. in Cancer: Principle and practice of oncology,
7th ed. Philadelphia. JB Lippincort 2005
(multidisciplinary tumor
conference)
tumor conference
21
1. Taylor LA, Ross AJ. Abdominal mass. In: Walker WA, Durie PR, Hamilton JR, et al. Pediatric
gastrointestinal diseases: Pathology, Diagnosis, Management, 2nd edn. WB Saunders, 1996:
227-240.
2. Felson B, Jacobsen HG. A modern approach to the abdominal mass in children. Semin Roentgenol
1988;23:147-231.
3. Melicow MM, Uson AC. Palpable abdominal masses in infants and children: A report bases on
a review of 653 cases. J Urol 1959;81:705-710.
4. Stevenson RJ. Abdominal masses. Surg Clin North Am 1985;65:1481-1504.
5. Caron HN, Pearson ADJ. Neuroblastoma in Voute PA, Barret A, Steven MCG, Caron HN (eds)
Cancer in Children: clinical management 5th edition, London, Oxford University Press, 2005.
6. Weksberg R, Shuman C, Smith AC. Beckwith-Wiedemann syndrome. Am J Med Genet 2005;137:
12-23.
7. Smith AC, Choufani S, Ferreira JC, Weksberg R. Growth regulation, imprinted genes, and
chromosome 11p15.5. Pediatr Res 2007;61:43R-47R.
8. Mueller RF. The Denys-Drash syndrome. J Med Genet 1994;31:471-477.
9. Nakashima Y, Inayama Y, Kato Y, Sakai N, Kanno H, Aoki I, Yao M. Pathological and molecular
biological aspects of the renal epithelial neoplasms, up-to-date. Pathol Int 2004;54:377-386.
10. Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA; UK Clinical Cytogenetics Group.
Cancer incidence in women with Turner syndrome in Great Britain: a national cohort study.
Lancet Oncol. 2008;9:239-246.
11. Plon SE, Malkin D. Childhood cancer and hereditary. in Pizzo PA, Poplack DG (eds) Principles and
practice of pediatric oncology 5th edition, Philadelphia, Lippincott William & Wilkins, 2006.
12. Narayanan V. Tuberous sclerosis complex: genetics to pathogenesis. Pediatr Neurol 2003;29:
404-409.
13. Curatolo P, Bombardieri R, Jozwiak S. Tuberous sclerosis. Lancet 2008;372:657-668.
14. Pizzo PA, Hays DM, Horowitz ME, et al. Solid tumors of childhood. in: Devita VT, Hellman S,
Rosenberg SA. Cancer: Principle and practice of oncology, 7th ed. Philadelphia. JB Lippincort 2005.
15. Ritchey ML, Kelalis PP, Breslow N, et al. Intracaval and atrail involvement with neuroblastoma:
review of National Wilms Tumor Study 3. J Urol 1988;140:1113-1118.
16. Ditchfield MR, De Campo JF, Waters KD, et al. Wilms tumor: a rational use of preoperative imaging.
Med Pediatr Oncol 1995;24:93-96.
17. Keating MA, Mclaughlin KP. Kidney. in Oldham KT, Colombini PM, Foglia RP (eds) Surgery of
infants and children: scientific principles and practice, Philadelphia. Lippincort-Raven 1997.
18. Stocker JT. Hepatic tumors in children. Clin Liver Dis 2001;5:259-281.
19. Pappo AS, Parham DM, Rao BN, et al. Soft tissue sarcoma in children. Semin Surg Oncol 1999;16:
121-143.
20. Gitlow SE, Dziedzic LB, Strauss L, et al. Diagnosis of neuroblastoma by qualitative and quantitative
determination of catecholamine metabolites in urine. Cancer 1970;25:1377-1383.
22
21. Broder GN, Pritchard J, Berthold F, et al. Revision of the international criteria for neuroblastoma
diagnosis, staging and response to treatment. J Clin Oncol 1993;11:1466-1477.
22. Sangkhathat S, Domdee K, Chiengkriwate P, Patrapinyokul S. Diagnostic laparoscopy prevent
unnecessary laparotomy in selected cases of pediatric intraabdominal mass. Proceeding of the
annual scientific meeting of the Royal College of Surgeon of Thailand. 2005.
(concept)
2
85
1 150
2 93.0 (A)3
(A)
( 1-4 ) 8.5
( 5-15 )4 .. 2546 - 2550
0-14 547 5 ( 1-1)
carcinoma
retinoblastoma neuroblastoma
Wilms tumor (nephroblastoma) (rhabdomyosarcoma) epithelial tumor germ cell tumor
3,6 ( 1-1)
0-4
5-9
10-14
15-19
..2546
..2547
..2548
..2549
..2550
237
202
181
242
198
169
187
276
199
172
192
270
163
168
185
248
179
179
196
229
(: )
184.75
172
190
255.75
1-1
. .1987-1996
134 9 .. 2001-2003
166 10
104 80 11
5 9
acute lymphoblastic
leukemia (ALL) zA astrocytoma
(lymphoma) non-Hodgkin lymphoma (NHL)
Hodgkin disease
Neuroblastoma Wilms tumor (nephroblastoma) embryonal tumors
Wilms tumor
neuroblastoma11
retinoblastoma
hepatoblastoma hepatocellular carcinoma sarcoma
Great
Britain
(19871996)
Males Females Both
genders
I. Leukemias
35.5
27.5
43.1
II. Lymphomas
10.5
5.5
11.8
III. CNS and miscellaneous intracranial 15.2
13.5
31.3
and intraspinal neoplasms
IV. Neuroblastoma
13.3
9.1
9.6
V. Retinoblastoma
2.4
2.2
4.6
VI. Renal tumors
2.9
2.1
8.1
VII. Hepatic tumors
2.5
1.9
1.3
VIII. Malignant bone tumors
4.1
3.5
5.0
IX. Soft tissue and other extraosseous
5.1
3.8
9.7
sarcomas
X. Germ cell tumors, trophoblastic
5.2
5
4.4
tumors and neoplasms of gonads
XI. Carcinomas and other malignant
2.6
2.1
4.1
epithelial neoplasms
XII. Other and unspecified malignant
4.3
3.8
0.9
neoplasms
IXII 103.7
80.1
133.8 140.9
* 0-19 years, ** Ho-Chi-Minh City
Japan
(1993-2001)
US
(2001-2003)*
Vietnam
(1995-1997)**
Thailand
(2003)
8.4
3.2
6.1
2.1
10.0
12.9
13.1
11.8
0.6
157.1
12.0
3.9
9.3
1.5
5.6
9.6
4.8
2.7
0.6
174.3
93.3
1.0
22.1
8.1
8.2
2.8
7.1
1.7
7.8
10.8
83.3
4.8
2.6
3.7
2.0
9.1
3.1
1.5
0.9
4.4
86.9
5.3
3.6
6.5
2.6
4.1
2.0
0.8
1.2
7.1
72.1
0.2
1.2
2.7
4.3
5.6
2.7
2.2
2.8
4.7
0.0
0.7
1.8
5.1
2.7
2.6
2.6
1.9
2.4
Europe
(19931997)
Wilms tumor
9-11
aged-standardized
incidence rate
(Thai Pediatric Oncology Group)
20 12
( 1-2)
Wilms tumor
( 1-3)
1.
13
90 1
2.
1
3. hepatocellular carcinoma
B
14,15 age-standardized incidence rate hapatocellular
carcinoma 10 10 0.88
0.07 Kaposis sarcoma
human immunodeficiency
virus
4.
1
5. hepatoblastoma
(very low birth weight) 16,17
1,500 (relative risk)
hepatoblastoma 25-50 2,500 18,19
35.0
9.8
8.2
24.8
7.7
12.4
28.2
7.6
11.8
26.6
5.5
7.6
3.9
4.7
4.0
1.9
2.1
4.4
2.6
3.7
0.9
2.2
1.5
1.7
1.2
4.6
3.7
1.9
2.0
2.7
3.0
3.8
2.4
1.3
2.5
2.8
3.0
1.7
2.0
2.0
3.1
2.6
1.4
1.9
4.5
84.7
1.7
63.7
3.4
70.6
5.1
64.5
Beckwidth-Wiedemann hepatoblastoma Wilms tumor ( 3-2
3) Li-Fraumani adrenocortical carcinoma
neuroblastoma
20
acute leukemia 21
papillary leukemia 22
papillary
23
papillary thyroid carcinoma
9 24
(concept)
5 (5-year survival) 1990s 75
1960s 28% 90
leukemia lymphoma germ cell Wilms tumor
NWTS
(multidisciplinary care and centralization)
(protocol)
(centralization)29
(risk-based management)
27,30
Wilms tumor
(evidence based medicine)
immunohistochemistry,
mRNA
small round blue cell
(H&E)31
MYCN amplification
neuroblastoma32
(gene therapy)33 tumor vaccine
1 34,35
(supportive
and palliative cares)
36,37
recombinant human erythropoietin granulocyte colony-stimulating factor 38,39 cytoprotective
drug mesna platinum40
dexrazoxane anthracyclines41
42
43
pulmonary fibrosis,
alkylating agents44
10
fairly tale
45 Ribero
( 1-2)
( 1-3)
46
37
disease related guarantee
11
3-1 ( 1)
0 1 47 25-30
body
cavity
8 10
immunohistochemistry 47
1
12
Pediatric Oncology
Group International Study of Pediatric Oncology
(protocol)
48
outreach program St Jude Childrens Research Hospital
Twinning program
My Child Matters Program 47
80
49
50
(gross domestic product)51
1,51
1-4
13
Cancer Research UK
[http://info.cancerresearchuk.org/cancerstats/childhoodcancer/]
population based Thai Pediatric Oncology
Group [http://www.nci.go.th/]
[http://medinfo2.psu.ac.th/cancer/]
1-4
( 52)
Phase ()
(pilot project)
(twinning
program)
(center of excellence)
(satellite centers)
Regional program
National program
14
Footnotes
1.
2.
3.
4.
15
16
19.
20.
21.
22.
23.
24.
25.
26.
27.
28.
29.
30.
31.
32.
33.
34.
35.
36.
37.
38.
39.
40.
41.
42.
43.
44.
45.
46.
47.
48.
49.
50.
51.
52.
17
1280-1291.
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girls. Int J Cancer 2001;91:402-406.
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18