This absolutely will help anyone who reads this powerpoint!

1. UGIB in children

2. CASE PRESENTATION
Moderator;Dr .Degefu D.(pediatrician)
Presenter;Dr.Mesfin(R2)
Dr.Eyob(R1)
Dr.Jemal(MI)
April 2023G.C

3. Outline of presentation
• Patient history
• Scientific background
• Management
• Refferance

4. Patient history
Identification
Name: Bereket Abayneh
Age:1year and 6 months
Sex:M
Date of Admition:2/8/2015E.C
MRN= 014836/15

5. c/c: bloody vomiting of 2days duration
HPI: This a1year and 6 months old male toddler was
relatively healthy 2days back at w/c time he started to
experience bloody vomiting which is non projectile non
billious coffee ground appearance two times per day each
estimated to be around half ACC for two days and
passage of black tarry loose stool of two times per day
and crampy Abdominal pain.

6. Cont…
• Associated with this he has hx.of LGIF, intermittent
non whooping non barking type of cough for 1wk
duration but no fast breathing.
• In addition to this he has hx of itching type of skin rash
for 1month duration which come and go.

7. Cont…
• His mother noticed Abdominal mass on his left side while she was
washing him at his 2wks of age. Since then the mass gradually increased
to attain current size
• For the above compliants,he visited our hospital 4 months back and he
was diagnosed with ?HMS 2º repeated malarial attack+R/o
Hematologic malignancy and he was transfused with 1unit of cross
matched blood and discharged with iron sulfate 100mg po TID with
Chloroquine 50mg po weekly base. he was treated several times for
repeated malaria attack

8. Cont…
• He was born from a 35yrs. old Para 6 mother at home with
DOL of 6 hrs and intrapartum ROM.But his mother didn’t have
ANC followup
• He was exposed to sunlight starting from 1month of his age and
started complimentary feeding at his 6th month with caw milk 1
cup three times per day and feeds with separate plate
• Currently he is on family diet with breast feeding.

9. Cont…
• He completed vaccination for his age according to EPI schedule.
• He can walk independently by him self and speak jargon words.
Other wise:
 No hx of use of medications like aspirin.NSAIDs
 No hx of feeding difficulty and excessive crying episodes
 No hx of river water contact
No hx of yellowish discoloration of the eye.
No hx of weight loss, night sweating
No hx of chronic cough/contact with chronic cougher.

10. Cont…
 No hx of hospital admission on his neonatal age and no hx of umbilical
catheterization
 No hx of abdominal surgery
 No hx of easy bruising, gum bleeding
 No hx of body swelling
 No hx of stridor, diffulty of swallowing, hoarsness of voice
 No family hx of chronic illness, or known bleeding disorder and
hypercoagulable state

11. P/E
GA:ASL
V/S: BP- PR- 148 RR-28 T⁰-36.8 SPO2-
96%
Anthropometry :
wt -11kg WFL- b/n -1and -2
lengh-85cm WFA-b/n -1 and -2
MUAC- 14cm HFA –b/n 0 and -1 Not
affected
HC-47cm

12. HEENT : No eye lid drop
Slightly pale conjunctiva and NIS
No nasal polyps,no injury and bleeding in nares
LGS : no palpable LN on accessible LN area.
Chest : clear resonant and good air entry bilaterally
CVS : S1 AND S2 Well heard
-ejection systolic murmur beast heard at LLSB and Apex
Abdomen : full abdomen w/c move with respiration symmetrically.
no visible distended Abd.veins,
Spleen palpated 8cm BLCM
no hepatomegaly and palpable mass appreciated
no signs of fluid collection

13. Cont…
GUS :WFMEG
No undescended testis,
Testis are symmetric bilaterally with normal
consistency
Intigumentary: palmar pallor, no rash,no vascular
malformations
MSS : no edema, bone tenderness
CNS : conscious and alert
pupils are normal size and reactive to light bilaterally
ASS’t :UGIB 2º to Variceal hemorrhage 2º to non
cirrhotic portal HTN 2º to portal vein thrombosis 2º
??Lymphoma + Severe Anemia 2º to ABL + IDA +
CAP + Portal cholangiopathy

14. Workup
CBC
WBC=4.5*103, 12.84*103, 4*103
Gra%= 54%, 62.2%, 37.5%
Lymph% = 35.5%, 29.3% ,51.4%
RBC=3.8*103 , 3.21*103 ,
Hgb= 8.2g/dl , 6.1g/dl, 5.6g/dl
Hct=26.5% , 20.7%, 17%
MCV=69.8fl , 64.6fl , 65.5fl
Platelet=173k/ul , 159k/ul , 126k/ul
BG with Rh= A+ve ESR=44
 BF=no H/P/Seen
 S/E=No O/P seen few RBCseen
 HBsAG,HCV-Ab and VDRL=-ve
 AST=122,ALP=191

15. Cont…
Cre=0.09mg/dl
Serum Abumin=2.7g/dl
INR=1.32.PT=15.8.APTT=32.49
Stoo H.pylori Ag =Negative
Bilirubin(TBI= 0.8mg/dl, DBI=0.2mg/dl)
Serum electrolyte Na-137.K-4.1.Cl-106
P.morphology:Leukemoid reaction + Anemia
No blast or dysplasia seen
No feature of hematologic malignancy seen.

16. Imaging
CXR:bilateral para hilar hazziness
Index :Pneumonia
Abd.U/S:Spleen is enlarged measuring 10.8cm and has
homogenous parenchymal echo
• There is increased echogenicity along the portal vein
and the main portal vein is not well visualized
• There is a collateral formation seen at the pancreatic
head region and along portal vein
• The caudate lobe of the liver is prominent

17. Cont…
Conclusion:
1.Spleenomegally
2.Peri portal fibrosis with irregular narrow portal vein
and collateral formation secondary likely chronic portal
vein thrombosis
Doppler U/s:
Spleenomegally + Ascitis 2ᵒ to CLD with Portal HTN +
chronic portal vein Thrombosis

18. Cont…
Abd and chest CT scan with Contrast: there is multiple mesentric and
intra abdominal hypodence node(14mm)
INDEX: Spleenomegally +Intra abdominal LAP+ supramediastinal mass
with vascular enhancement
DDX:1.TB
2.NHL

19. Pending
Upper GI Endoscopy
Bone marrow biopsy
Biopsy from portal vein thrombus

20. Management
• Kept npo for 24 hrs
• Omeprazole 1mg/kg…10mg iv bid
• Mf
• Ceftriaxone 75mg/kg 413 mg BID
• Propranol 0.2mg/kg 2.2 mg po BID

21. Definition
• UGI bleeding arises from proximal to the ligament of Treitz in the
distal duodenum

22. EPIDEMIOLOGY
• As much as 20 percent of all episodes of gastrointestinal (GI) bleeding
in children come from an UGI source
• Commonly in critically ill pediatric patients who were not receiving
prophylactic therapy to prevent UGI bleeding
• Independent risk factors for bleeding included a high Pediatric Risk
Mortality score, coagulopathy, pneumonia, and multiple trauma

23. Etiology
Neonates
Swallowed maternal blood*…distinguished from fetal blood by Apt-
Downey test.
Vitamin K deficient bleeding (hemorrhagic disease of the newborn)
Stress gastritis or ulcers are associated with critical illness
Congenital anomalies including intestinal duplications or vascular
anomalies
Coagulopathy(eg, associated with infection,liver failure,congenital)
Milk protein intolerance…food protein induced proctocolitis

24. Infants, children and adolescents
 Mallory-Weiss syndrome…after forceful retching
Esophageal or GI foreign body
Esophagitis:GERD,eosinophilic esophagitis,caustic ingestion
Peptic ulcers and gastritis…critical illness,h.pylori infection,NSAIDs
Pill esophagitis…doxycycline,NSAIDs…ass with odynophagia
Bleeding from esophageal varices: due to portal hypertension
caused by Cirrhosis, Portal vein thrombosis, Hepatic vein obstruction
(Budd-Chiari syndrome)…etc

25. Ddx
Swallowed maternal blood
Epistaxis
Substances that resemble blood…Red food colorings and dyes
Medical child abuse…Munchausen syndrome by proxy

26. CLINICAL PRESENTATION
• Hematemesis and/or melena…in addition, Because of short intestinal
transit time, neonates and infants with UGI bleeding are more likely
than adults to present with hematochezia

27. CLINICAL ASSESSMENT
• Initial assessment and resuscitation …for those with hemodynamic
instability(shock,orthostatic hypotension)
• Endoscopy…after resuscitation or if there is brisk bleeding
• Detail history and p/e
• Lab and imaging diagnosis

28. History
• Characteristics of hematemesis and stool
• Accompanying symptoms:Epigastric pain or heartburn, Odynophagia
(pain with swallowing), poor feeding and irritability, Jaundice,
Epistaxis (recent or recurrent), Easy bruising or bleeding
• Underlying disease:Chronic liver disease, Bleeding disorder, Critical
illness
• Medications:Tetracyclines (eg, for acne) or NSAIDs, or
bisphosphonates,Beta adrenergic blockers
• Other history:Breast-fed infant, History of choking episode…etc

29. Physical exam finding
• Rapid assessment: tachycardia,delayed capillary refill,…shock
• Skin:Bruising, petechiae,Vascular malformations (eg, telangiectasias,
hemangiomas), Melanosis
• Nasopharynx:Blood or injury to the nares, Pharyngeal bleeding or
injury
• Abdomen:Hepatomegaly, hard liver edge, and/or splenomegaly

30. Non Cirrhotic Portal Hypertension
Portal hypertension
• defined as an elevation of portal pressure >10-12 mmHg or a
hepatic venous pressure gradient >4 mm Hg
• occurs when there is increased portal resistance or
increased blood flow through the portal system
Presence of portal hypertension in the absence of
cirrhosis

32. Etiology
Prehepatic
• Portal vein thrombosis*
• Splenic vein thrombosis
• Splanchnic arteriovenous fistula
• Splenomegaly (eg, from lymphoma, Gaucher's disease)

33. Intrahepatic
• Presinusoidal:
• Schistosomiasis,
• Primary sclerosing cholangitis,AV fistulas,
• Neoplastic occlusion of the intrahepatic portal vein(due
to eg.lymphoma,CLL )

34. • Sinusoidal:
• Schistosomiasis
• Acute hepatic injury
• Viral hepatitis
• Vinyl chloride toxicity
• Arsenic poisoning
• Postsinusoidal:
• Budd-Chiari syndrome,
• venooclussive disease

35. • Posthepatic
• IVC obstruction (eg, Budd-Chiari syndrome*)
• Cardiac disease (constrictive pericarditis, restrictive
cardiomyopathy)

36. schistosomiasis (bilharzia)
Encompasses the acute and chronic inflammatory disorders
caused by human infection with Schistosoma spp. parasites.
Schistosoma organisms are the trematodes, or flukes, that
parasitize the bloodstream
Five schistosome species
• Schistosoma haematobium, S. mansoni, S. japonicum, S.
intercalatum, and S. mekongi.
Disease common in older children and young adults
But it can occur in younger children who accompany family to
river water

37. Pathogenesis
The c/manifestations is due to immune mediated granulomatous
reaction to infective stage of parasite(cercariae)
Primary deposition sites
• Mesenteric vessels…for s.mansoni and s.japonicum
• Perivesical venous plexus…s.haematobium
Then can disseminate to liver,cns

38. Life cycle

39. Clinical manifestations
Acute manifestations
• schistosomal dermatitis or swimmer’s itch.
• snail fever or Katayama syndrome…after 4-8 weeks of contact
Chronic manifestations….major pathology
• urogenital schistosomiasis
• microscopic or macroscopic hematuria and/or pyuria..in acute
phase
• frequency, dysuria, and hematuria,thickened bladder wall
• chronic renal failure, secondary infections, and squamous
carcinoma of the bladder…in advanced stage
• intestinal schistosomiasis

40. Intestinal schistosomiasis
colicky abdominal pain and bloody diarrhea are the most common.
On the liver
• hepatosplenomegaly, portal hypertension, ascites, and
hematemesis…late syndrome
• Mechanism of injury in liver…periportal fibrosis…This leads to
occlusion of the portal veins, portal hypertension with splenomegaly,
portocaval shunting, and gastrointestinal varices
• the liver is firm and nodular. Hepatocellular liver function is not
impaired.
Lung
• pulmonary hypertension
Cns…Transverse myelitis,

41. Diagnosis
Microscopic urine analysis…for detection of s.haematobium
Stool exam by Kato-Katz thick smear procedure
The unique schistosome antigens
• circulating anodic antigen (CAA) and
• circulating cathodic antigen (CCA) may also be detected in
urine and serum

43. Treatment
praziquantel (40 mg/ kg/day orally [PO] divided twice daily
[bid] for 1 day for schistosomiasis haematobia, mansoni, and
intercalatum; 60 mg/kg/day PO divided 3 times daily [tid] for 1
day for schistosomiasis japonica and mekongi).
• Children <5 yr old with S. mansoni may need up to 60
mg/kg/day PO tid for 1 day to achieve clearance. A 2nd
treatment 4-6 wk after the 1st course may help in eliminating
residual infection.

44. Idiopathic noncirrhotic portal hypertension
(nodular regenerative hyperplasia)
Definition
• It is characterized by portal hypertension in the absence of liver
cirrhosis, with histologic features of dense portal fibrosis, marked
phlebosclerosis, and dilated sinusoids.
Epidemiology
• Incidence declining in countries with improved socioeconomic
status
• encountered in patients who are socioeconomically disadvantaged

45. Etiologic pathophysiology
Chronic or recurrent infections…
Repeated episodes of umbilical sepsis, bacterial infections, and
diarrhea…lead to portal pyemia and pylephlebitis…vascular
endothelial injury, microthrombosis, sclerosis, and obstruction of
small- and medium-sized portal vein radicals.
Drugs and toxin:may induce fibrosis in the space of Disse…eg
azathioprine
HIV infection…due to drug s/e(didanosine) on microvasculature of
liver or direct effect of virus

46. Altered immune response…. Anti-DNA antibodies, severe primary
antibody deficiencies
Genetic predisposition mutation in DGUOK, a deoxyguanosine kinase
required for mitochondrial DNA replication
Hypercoagulability
Miscellaneous…role of endothelin-1, nitric oxide, and connective
tissue growth factor

47. Clinical mani
• Variceal bleeding…often relatively well tolerated due to preserved
liver function
• massive splenomegaly >10 cm…in > 95% of cases
• mild hepatomegaly (<4 cm below the right subcostal margin)…in 50%
• occasionally develop jaundice, ascites, or hepatic encephalopathy
• Anemia, leukopenia, and thrombocytopenia are common because of
hypersplenism.
• Liver biochemical tests are usually normal or nearly normal

48. Diagnosis
Diagnostic criteria:
• Presence of at least one of the clinical signs of portal
hypertension( eg v.bleeding,splenomegaly)
• Exclusion of cirrhosis on liver biopsy
• Exclusion of chronic liver disease that may cause either cirrhosis or
noncirrhotic portal hypertension
• Imaging showing patent portal and hepatic veins

49. Portal vein thrombosis
• Occlusion of the portal vein by thrombus (portal vein thrombosis
[PVT]) typically occurs in patients with cirrhosis and/or prothrombotic
disorders
• Chronic PVT develops in patients with acute PVT that does not resolve
(with or without treatment)
• Patients with chronic PVT develop collateral blood vessels that bring
blood in a hepatopetal manner around the area of obstruction,
known as cavernous transformation of the portal vein or portal
cavernoma

51. Cavernous transformation of portal vein

52. Etiology
In neonates,
• Umbilical infection (omphalitis)
• umbilical vein catheterization
• dehydration, and/or sepsis.
• TORCH infections…congenital CMV,toxoplasmosis
• Mechanism of CMV induced VTE is 1.formation of APS ab in response
to CMV infection 2.formation of anticardiolipin ab
• Developmental anomalies producing extrahepatic portal
hypertension include agenesis, atresia, stenosis, or a web of
the portal vein

53. In older children
• intraabdominal infection (appendicitis, peritonitis, pancreatitis),
• inflammatory bowel disease
• primary sclerosing cholangitis, or biliary infection
• hypercoagulable states, such as deficiencies of factor V Leiden,
protein C, or protein S
• Nephrotic syndrome,CHD,LUPUS
• Malignancy

54. Malignancy
• Lymphoma: NHL
• Benign liver tumors:
• Idiopathic noncirrhotic portal hypertension (nodular
regenerative hyperplasia)
• Hepatic hemangioma
• Focal nodular hyperplasia
• Hepatic adenoma
• ALL,CLL

55. Diagnosis
Lab: CBC,BG rh,LFT,RFT,viral markers,coagulation profile, amylase and
lipase
Endoscopy
Abdominal ultrasound with doppler imaging
• demonstrates hyperechoic material within the portal vein that
may extend into the mesenteric or splenic veins
• dilation of the portal vein and its tributaries, and absence of flow
within the portal vein
• a mass of tortuous vessels at the porta hepatis or within the liver
• Velocity usually less than that in a normal portal vein

56. Abdominal CT
• reveals a network of intertwined, densely packed veins in the
hepatoduodenal ligament and porta hepatis
• communication between collateral vessels and intrahepatic portal
veins may be seen
Abdominal MRI
• portal vein occlusion as well as collateral veins around the porta
hepatis
• On MRI angiography as a filing defect
hypercoagulability workup

57. Complications
• Portal hypertension
• Portal cholangiopathy (also referred to as portal biliopathy)
• intestinal ischemia and infarction if there is extension of the clot into
the superior mesenteric vein

58. Clinical manifestations
Bleeding: hematemesis or melena from:
• Esophageal varices…most commonly
• Portal gastropathy,
• gastric antral ectasia, or
• stomal, intestinal, or anorectal varices
The age of first bleed is dependent on the underlying etiology of
portal hypertension
Can be precipitated by minor febrile, intercurrent
illness,NSAIDS,cough

59. Esophageal varices
Any child with hematemesis and splenomegaly should be
presumed to have esophageal variceal bleeding until proved
otherwise.
Children tend to tolerate variceal bleeding better due to
generally well compensated liver disease.mortality <1% after
initial bleed
Diagnosis:
• Endoscopy:large varices, red marks, and the presence of
gastric varices

60. Splenomegaly:
• the second most common finding in children with portal
hypertension
• may be related to increased venous congestion and splenic arterial
flow
• may be initially recognized on routine physical examination.
• With concurrent cytopenias.
• Most children with splenomegaly are asymptomatic.
Biliopathy; including pruritus, obstructive jaundice, cholecystitis, and
cholangitis

61. Ascites
• is the presenting sign of portal hypertension in 7–21% of
children.
• Ascites can develop at any time with cirrhosis or if there is
new onset portal vein obstruction
Hepatopulmonary syndrome (HPS) in ≥10% of patients with
portal hypertension…dyspnea, cyanosis, clubbing, and spider
nevi
Portopulmonary hypertension (PP-HTN)…most commonly
present with exertional dyspnea…due to pulmonary arteriopathy

62. Omphalitis
Definition: Omphalitis is an infection of the umbilicus and/or
surrounding tissues
• purulent discharge from the umbilical cord stump with
surrounding induration, erythema, and tenderness
• Umbilical stump bleeding
• Systemic signs, including lethargy, fever, irritability, and poor
feeding are suggestive of more severe infection or complication

63. Risk factors:
• low birth weight,
• prolonged labor,
• prolonged rupture of membranes or maternal infection
• nonsterile delivery,
• umbilical catheterization, and
• home birth

64. Causative etiology
• Staphylococcus aureus
• Streptococcus pyogenes,
• Gram-negative bacteria such as Escherichia coli, Klebsiella
pneumoniae, and Proteus mirabilis
• anaerobic bacteria such as Bacteroides fragilis, Clostridium
perfringens, and Clostridium tetani

65. Antibiotic rx
• antistaphylococcal penicillin and aminoglycoside agents
• Vancomycin if MRSA suspected
• Clindamycin or metronidazole…for anaerobic coverage

66. Complications
• Sepsis
• septic umbilical arteritis,
• portal vein thrombosis,
• liver abscess,
• peritonitis,
• intestinal gangrene,
• small bowel evisceration,
• necrotizing fasciitis, and death

67. Mediastinal mass
Anatomy of mediastinum
The space between the lungs
Borders
• Superiorly…thoracic inlet
• Inferiorly…diaphragm
• Anteriorly…Sternum
• Posteriorly….spine
• Laterally…pleural space

68. Normal contents
Anterior mediastinum
• Thymus
• Loose ct
• Adipose tissue
• Lymphatic vvs and LNs
• Branches of internal thoracic
vvs
Middle mediastinum
• Heart
• Pericardium
• Lt an rt main bronchi
• Ascending aorta
• Pulm trunk
• SVC

69. Posterior mediastinum
• Symphatetic trunk
• Esophagus
• Vagus nerve
• Thoracic aorta
• Azygus vein
• Thoracic duct and lymphatics

70. Approach to mediastinal mass
• The most common site for chest mass in childhood
• Per etiology it can be congenital,infectious,benign and malignant
neoplasms
• 70 to 75% are malignant masses in childhood

71. Ddx of mediastinal mass
Anterior compartment
• Lymphoma…NHL
• Thymoma/Thymic
hyperplasia
• Germ cell tumors…eg
teratoma.
• Substernal goiter/Ectopic
thyroid tissue
• Hemangioma
• Parathyroid adenoma
• Lipoma/Liposarcoma
Middle compartment
• NHL
• Bronchogenic cyst(tracheal
duplication cyst)
• Tb lymphadenopathy
• Teratoma
• Plasma cell granuloma
• Rhabdomyosarcoma
• Cardiac rhabdomyoma

72. Posterior compartment
• Neuroblastoma
• Ganglioneuroblastoma
• Neurofibroma
• Leiomyosarcoma
• Enterogenous cyst
• Meningoceles
• Thoracic spine lesions (eg, Pott's disease)

73. c/presentation
Incidental finding on imaging,most of the time
Rapid progression of symptoms vs gradual onset
Mass effect
• Cough,stridor,dysphagia,hoarsness,chest pain,hemoptysis
• Upper extremity and facial swelling(svs)
• Hypotension(due to cardiac tamponade)
• Horner syndrome
Systemic symptoms
• B symptoms and paraneoplastic syndrome

74. Diagnosis
Lab
• Tumor markers: beta HCG,AFP,LDH
• Anticholinestrase receptor antibodies
• Urine catecholamines
Imaging
• CECT…shows tumor size,location,characteristics,attachment to
near by structures. It helps in further rx plan and response
assessment

75. • MRI
• PET-CT
• MR spine
• Scrotal u/s
Pathologic dx….FNAC,biopsy,BM aspiration
eg..for lymphoma dx

76. Non Hodgkin lymphoma
• NHL is the fifth most common diagnosis of pediatric cancer in children
under the age of 15 years
• The median age at diagnosis is approximately 10 years
• Rare in infants <1% and account for approximately 4, 14, 22, and 25
percent of neoplasms in children age 1 to 4, 5 to 9, 10 to 14, and 15 to 19
years, respectively.
• Predisposing factors
• Both congenital and acquired immunodeficiency syndromes
• Wiskott-Aldrich syndrome
• Viruses(HIV,EBV)
• Geneti s(bloom syndrome,ataxia-telangiectasia)

77. the most common subtypes of pediatric NHL are derived from B cell
progenitor
the most common subtypes
• lymphoblastic lymphoma (LBL),
• mature B-cell lymphoma, and
• anaplastic large cell lymphoma (ALCL;

78. Clinical manifestations
The clinical manifestations of childhood and adolescent NHL
depend primarily on pathologic subtype and sites of
involvement.
As oncologic emergency
• Superior or inferior vena cava syndrome
• Spinal cord compression
• Venous thromboembolic disease
• Hyperuricemia and tumor lysis syndrome
NHL commonly presents as enlarging, non-tender lymphadenopathy

79. CNS involvement occurs in 6 percent of pediatric NHL
B symptoms in minority of cases

80. DIAGNOSIS
pathologic evaluation of involved tissue
Subtypes of NHL are identified using histology
Lab and imaging
• BM aspiration
• CBC, Serum electrolytes, lactate dehydrogenase,uric acid
• Lp with cell count, and protein
• chest radiographs; and abdominal ultrasound

81. Staging
Staging studies
• CT of neck, chest, abdomen, and pelvis with or without PET
• Bilateral iliac crest bone marrow aspiration and biopsy
• Lumbar puncture for examination of cerebrospinal fluid

82. staged according to the Murphy stage
Stage I a single tumor extranodal or nodal excluding the abdomen
and mediastinum
Stage II
• single extranodal area plus regional lymph nodes
• Two single extranodal tumors on the same side of the diaphragm
with or without regional lymph nodes
• Primary gastrointestinal tumor (completely resected) with or
without mesenteric lymph nodes

83. Stage III
• Primary intrathoracic (mediastinal, thymic, pleural) disease
• Two extranodal sites on opposite sides of the diaphragm
• Extensive primary intra-abdominal disease
• Two or more nodal areas on opposite sides of the diaphragm
• Any paraspinal or epidural tumors
Stage IV – Any of the above with involvement of the bone marrow,
central nervous system, or both

84. Neuroblastoma
• Neuroblastomas are embryonal cancers of the peripheral
sympathetic nervous system(ganglia)
• Known for heterogeneous clinical presentation and course
• The tumors undergo spontaneous regression to very aggressive
behaviour unresponsive to intensive multimodal therapy.

85. Epidemiology
• It is the third most common childhood cancer, after leukemia and
brain tumors
• is the most common solid extracranial tumor in children
• Occurs most often in the first 3 years of life
• the most commonly diagnosed malignancy in infants.
• The median age at diagnosis is 22 mon.

86. Pathology
• Neuroblastoma tumors classification:
neuroblastoma,ganglioneuroblastoma,ganglioneuroma.
• The degree of differentiation and stromal component of
neuroblastoma tumors can be predictive of outcome
• Neuroblastoma is the most aggressive with primarily
undifferentiated small round cells
• Ddx:rhabdomyosarcoma, Ewing sarcoma, and non-Hodgkin lymphoma
• Tumors consisting of mature and maturing schwannian stroma
with ganglion cells (ganglioneuroblastoma and
ganglioneuroma).

87. Pathogenesis

88. Clinical manifestations
The signs and symptoms of neuroblastoma reflect the tumor
site and extent of disease
Neuroblastoma may develop at any site of sympathetic nervous
system tissue
Approximately half of neuroblastoma tumors arise in the
adrenal glands, and most of the remainder originate in the
paraspinal sympathetic ganglia
Primary mediastinal neuroblastoma occurs in 11% to 26% of all
neuroblastomas and has favorable prognosis than that of
abdomen

89. Cont’d…
Local effect:
• spinal cord compression, bowel obstruction, and superior vena cava
syndrome.
Metastatic disease
• fever, irritability, failure to thrive, bone pain, cytopenias, bluish
subcutaneous nodules, orbital proptosis, and periorbital ecchymoses
Associated neurologic signs and symptoms.
• Horner syndrome, opsoclonus-myoclonus–ataxia
syndrome(paraneoplastic syndrome)

90. Catecholamines release
• increased sweating and hypertension, and may release
vasoactive intestinal peptide, causing a profound secretory
diarrhea
tumor lysis syndrome and disseminated intravascular
coagulation

91. Diagnosis
Usually discovered as a mass or multiple masses on plain R,CT,MRI
Confirmed by….without primary tumor biopsy:
• If small round blue tumor cells are observed in bone marrow
samples and VMA or HVA levels are elevated in the urine
Nb:Tumor markers:catecholamine metabolites homovanillic
acid (HVA) and vanillylmandelic acid (VMA)
Metastatic disease evaluation: CT or MRI of the chest and
abdomen, bone scans to detect cortical bone involvement,
Iodine-123 metaiodobenzylguanidine, PET combined with CT or
MRI

92. Staging
The International Neuroblastoma Risk Group (INRG)
Staging System (INSS)
Extent of locoregional disease is based on specific local image-
defined risk factors (IDRFs).
• L1 tumors (previously classified as INSS stage 1) are
localized and confined to 1 body compartment without any
IDRFs
• L2 tumors (previously classified as INSS stages 2 and 3)
refer to localized tumors with the presence of IDRFs

93. • M (previously classified as INSS stage 4) Disseminated
tumors with metastases to bones, bone marrow, liver, distant
lymph nodes, and other organs
• Stage MS (previously stage 4S) refers to
neuroblastoma in children <18 mo old with
dissemination to liver, skin, or bone marrow without
bone involvement and with a primary tumor that
would otherwise be staged as L1 or L2

94. Management

95. Germ cell tumors(GCTs)
• Gonadal and extra gonadal GCTs
• Extra gonadal GCTs occur in the midline of the body:
• anterior mediastinum,
• retroperitoneum,
• the pineal and suprasellar region
• sacrococcygeal teratomas….in infants and young children
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96. • Primary mediastinal gct are rare in children
• If there…located in anterior mediastinum
• They are difficult to distinguish from testicular GCTs
• Thus, any patient who presents with mediastinal GCTs needs
exclusion of testicular and ovarian primary tumors.

97. Mediastinal GCTs
Include
• mature teratomas, immature teratomas, seminomas, and
nonseminomatous GCTs.
Mature teratomas
• Mature teratomas of the mediastinum tend to grow slowly
• diagnosed incidentally while they are still asymptomatic

98. Symptoms
• chest pain, cough, dyspnea, and bronchial obstruction and
postobstructive pneumonia)
• Erosion into an adjacent bronchus can rarely lead to
expectoration of hair (trichoptysis) or sebaceous debris
• Erosions into the pericardium, adjacent vascular structures, or
through the skin to form a draining fistula are rare but serious
complications

99. Diagnosis
• Plain chest radiography typically shows an anterior mediastinal
mass, with calcification and well-formed teeth or bone
• CT and MRI localizing lesions and determining the spatial
relationships with surrounding structures. characterize densities
within the lesion suggestive of fat, sebaceous material, or cystic
elements
Treatment
• surgical excision, and this is almost always curative
• through a median sternotomy or posterolateral thoracotomy

101. Immature teratoma
• Immature teratomas are characterized by mature elements from
all three germinal layers, which are mixed with immature tissue.
• Macroscopically, immature teratomas form cystic masses with
areas of hemorrhage and necrosis…in adults and is rare and
aggressive
• In children, immature mediastinal teratomas behave similarly to
mature teratomas (ie, as benign tumors) and should be completely
excised.

102. Mediastinal seminoma
• Mediastinal seminomas constitute approximately one-third of
malignant mediastinal GCTs and 2 to 4 percent of mediastinal
masses
• Mediastinal seminomas occur predominantly in men between the
ages of 20 and 40 yrs.
• In order for a GCT to be classified as a seminoma in men or a
dysgerminoma in women, no other histologic element of a GCT
can be present, and serum alpha-fetoprotein (AFP) must be
normal

103. c/f
• Chest pain – 39 percent
• Dyspnea – 29 percent
• Cough – 22 percent
• Weight loss – 19 percent
• Superior vena cava syndrome –
12 percent
• Fever – 6 percent
• Nausea – 6 percent
The majority of mediastinal
seminomas have metastasized
by the time they are detected,
most often to the lymph nodes
and less commonly to lungs,
bone, and/or liver
Treatment
• Seminomas are exquisitely
sensitive to both cisplatin-
based chemotherapy and RT,
regardless of location

104. Thymus
• The tumors are:Thymoma and Thymic cancer
• As an incidental finding identified on imaging in an asymptomatic
patient
• Because of local (thoracic) symptoms
• Due to symptoms from a paraneoplastic syndrome

105. Thymoma
• They are epithelial neoplasms containing variable number of lymphocytes
• Thymomas typically are localized to the thymic gland and surrounding
organs
• Thoracic manifestations: chest pain, shortness of breath, cough, phrenic
nerve palsy,SVC
• Paraneoplastic disorders
• Myasthenia gravis: diplopia, ptosis, dysphagia, weakness, and fatigue.
• In patients with thymoma and myasthenia gravis, thymectomy usually
results in an attenuation of the severity of myasthenia gravis, although
some symptoms persist in most patients

106. • Hypogammaglobulinemia and pure white blood cell aplasia:
Patients usually have recurrent infections, diarrhea, and
lymphadenopathy
• Thymectomy does not reliably lead to the return of normal
immunoglobulin levels
• Pure red cell aplasia
• Treatment