1. WelcomeTo Grand Session
Presenters:
Dr. K. M. Hossain, FCPS student, General Pediatrics
Dr. Renesha Islam, Resident, Year 4, Pediatric Hematology and Oncology
DEPARTMENT.
2. Particulars Of The Patient:
Name: Sadia
Age: 10 years
Sex: Female
Address: Kurigram
DOA: 15/12/2020
DOE : 15/12/2020
Informant: Mother and herself
3. Chief Complaints:
•Pain and swelling in the upper part of left leg for 3
months.
•Difficulty in standing or walking for 1 month.
4. History of Present Illness
According to the statement of Sadia and her
mother, she was reasonably well 3 months back.
Then she developed swelling in the upper part of
left leg which was increasing in size. She also
complaints of pain in the same region which was
aching in nature, initially intermittent then became
persistent same in intensity during day and night,
not significantly improved by taking analgesics.
5. She also complaints of difficulty in standing or
walking for one month. She also noticed a small
painless swelling in the left supra orbital region for
15 days. She had no history of trauma, fever,
cough, breathing difficulty, gradual pallor, bleeding
manifestation, and exposure to ionizing radiation,
or contact with TB patient.
6. With these complaints, she visited different
physicians and treated with oral medications
including antibiotics and analgesics.
As her condition was not significantly improved, she
was referred to BSMMU and initially admitted in
Department of Orthopedics and treated accordingly
along with an incisional biopsy done from upper part
of Left leg and referred to our department for further
evaluation and management.
7. History of past illness:
No significant past illness.
Developmental History:
Age-appropriate.
8. Immunization History:
She is immunized as per EPI schedule.
Family History:
She is 2nd issue of non-consanguineous parents.
No other family members have similar type of
illness.
9. Socio-economic History:
She belongs to a lower socioeconomic background.
Her father is a farmer and mother is a housewife.
Their average monthly income is 10,000 taka. They
live in tin-shed house, drink tube well water and
use sanitary latrine.
14. Lymph node : Not enlarged
Bony Tenderness: Absent
Examination of Eye: Normal.
Examination of Ear, Nose and Throat: Normal
Swelling in left supra orbital region measuring about 1.5x01
cm which is non tender, soft in consistency, not fixed with
superficial and underlying structure, readily moves with slight
finger pressure.
15. Vital Signs:
Temperature : 98°F
Pulse: 88 beats/min
Respiratory Rate: 18 breaths/min
Blood Pressure: 110/70 mm Hg (Both SBP & DBP
lies between 50th to 90thcentile)
19. Locomotor System Examination
Look
There was a swelling in the anterolateral aspect
of the upper part of Lt leg.
Scar mark present.
Hypo pigmented skin over scar mark.
Irregular surface.
No discharging sinus.
Muscle wasting present in both lt culf muscle
and thigh muscle.
20. Feel
The swelling is about 10cm × 09cm n diameter.
Firm to hard in consistency.
Indistinct margin
Temperature normal
Tenderness present (grade 3/4).
There was no regional lymphadenopathy.
Scar mark about 8 cm in length.
23. Muscle Upper limbs (Rt. & Lt.) Lower limbs (Rt. & Lt.)
Bulk Normal Normal in both lower limbs
except decrease in Lt calf and
thigh muscle
Tone Normal Normal
Power Normal Normal
Motor function
24. Lower limb
Right Left
Knee Normal Could not be elicited
Ankle Normal Normal
Planter response: Bilaterally Flexor
Sensory: Intact.
25. Alimentary System
Oral cavity- normal
Abdomen proper:
Inspection:
Abdomen not distended
Umbilicus: Centrally Placed , inverted
No engorged vein, no scar mark.
26. Palpation:
◦ Abdomen is soft, non-tender.
◦ Liver: Not palpable
◦ Spleen: Not palpable
◦ No abdominal mass
Percussion:
◦ Percussion note: Tympanic
◦ Shifting Dullness : Absent
Auscultation:
◦ Bowel sound : Present
30. Cardiovascular system Examination
Inspection:
◦ No visible pulsation.
Palpation:
◦ Apex Beat: Located in the Left 5th ICS , just medial to the
midclavicular Line.
◦ Thrill : Absent
◦ Left Parasternal Heave: Absent.
◦ Palpable P2 : Absent
32. Salient Feature:
Sadia, a 10 years old girl, 2nd issue of non consanguinous
parents, hailing from Kurigram got admitted with the
complaints of pain and swelling in the proximal part of left
leg for 3 months. She had also difficulty in standing or
walking for last 1 month. She also noticed a painless
swelling in left supra orbital ridge for last 15 days. She had
no history of trauma, fever, cough, breathing difficulty,
gradual pallor, bleeding manifestation, and exposure to
ionizing radiation, or contact with TB patient.
33. For these complaints she visited some local registered
physicians and treated with several oral analgesics and
antibiotics. As there was no significant improvement, she
got admitted in Orthopedics department of BSMMU initially
and treated accordingly along with incisional biopsy done
from proximal part of left leg. After receiving histopathology
report she was referred to Pediatric Hematology and
Oncology Department for further evaluation and
management.
34. Salient Feature (contd):
On examination, patient was ill-looking, mildly pale, vitals
were within normal limit, anthropometrically well thriving.
There is a swelling measuring about (10cm X 09 cm) on
upper part of left leg which was firm to hard in consistency,
indistinct margin, temperature normal, tenderness present
(grade ¾), with no overlying skin ulceration or discharging
sinus. Movement of knee joint restricted.
Another swelling measuring about (1.5x 01 cm) on left supra
orbital ridge which was non tender, soft in consistency, not
fixed with superficial and underlying deep structures, readily
moves with slight finger pressure.
Examination of other systems revealed normal findings.
38. Osteosarcoma:
Points in Favor:
Site
Age
Swelling
Pain not controlled with analgesic
Movement of knee joint restricted.
39. Ewing sarcoma:
Points in Favor:
Site
Pain not responsive to traditional analgesics
Mass gradually increasing in size which is firm to hard in
consistency
Restriction of movement.
40. Osteomyelitis
Points in Favor: Points against:
pain
swelling
No characteristic fever
No discharging sinus
No history of trauma
41. Metastatic Neuroblastoma
Points in Favor: Points against:
Pain and swelling in
upper part of left leg
Age
Swelling in the left supra
orbital ridge
No characteristic clinical
features like irritability,
paraneoplastic syndrome
etc.
49. Histopathology report: (02/11/20)
Specimen: Bone tissue from upper tibia (left)
Microscopic appearance:
Sections show a malignant neoplasm composed of nests of
small round cells with marked hyperchromasia and moderate
pleomorphism. Large area of necrosis, dead bone, blood clot
and inflammatory cell infiltrates are seen.
Dx: Features are suggestive of Ewing sarcoma
50. Immuno-histochemistry : (28/11/20)
Specimen: Bone tissue from upper tibia (lt)
Immunohistochemistry result:
CD56: Positive in Tumor cells
Synaptophysin: Positive in tumour cells
Chromogranin-A: Negative in tumour cells
CD99: Negative in tumour cells
51. Vimentin: Negative in tumour cells
LCA: Negative in tumour cells
TdT: Negative in tumour cells
NSE: Negative in tumour cells
Dx: Compatible with Neuroblastoma (?Metastatic)
Further workup is recommended.
53. Bone Marrow Report
Smear and stain: Good
Spicule: Adequate
Overall cellularity: Normocellular
Myeloid : Erytroid Ratio: Normal
Erythropoiesis: normally and active
Granulopoiesis: Active and orderly
Megakaryocytes: Present
Dx: Uninvolved bone marrow
54. USG of Whole Abdomen: 01/12/2020
Impression:
Normal Study
55. Chest X ray (A/P view) :
Impression:
Normal study
59. Incisional biopsy from Lt supra orbital ridge:
Specimen: Soft tissue mass from left supra orbital ridge.
Microscopic appearance:
Sections show skeletal muscle, fibro fatty tissue, nerve bundle and
thick walled blood vessels.
No malignancy is seen.
Impression: Lipoma.
62. Treatment
Counselling.
Supportive treatment:
◦ Diet – Neutropenic.
◦ Hydration
◦ Nystatin oral drop
◦ Chlorohexidine mouth wash
◦ Acriflavin hip bath.
◦ For Pain management: According to adapted WHO analgesic ladder
for cancer pain.
63. Specific treatment
Systemic: Neoadjuvent Chemotherapy and
Adjuvent Chemotherapy
Local: Surgery and Radiotherapy
Here alternating VDC/IE started from
28/12/2020 as Neoadjuvant therapy.
64. Follow up on 28/12/2020
Subjective Objective Assessment/ Plan Intervention
Reduced pain
then earlier
Ill looking
Mildly pale
RR-20/min
Pulse- 88 /min
BP-110/70 mm of Hg
P/A/E- No organomegaly
Locomotor system : Movment of
knee(Lt) restricted
Bowel and bladder habit –Normal
Patient condition
is stable
Start
chemotherapy
65. Follow up on 02/01/2021
Subjective Objective Assessment/ Plan Intervention
No new
complaints
Well alert
Moderately pale
RR-18/min
Pulse- 80 /min
BP-115/70 mm of Hg
P/A/E- No organomegaly
Locomotor system : Movment of
knee(Lt) restricted
Bowel and bladder habit –Normal
CBC –
Hb- 7.1 gm/dl
WBC-7.2x10^9/L
PLT- 289x10^9/L
Neutrophil-
87.1%
Lymphocyte-
11.5%
PRBC 1 unit
transfused and
discharge with
advice .
68. P r e s e n t e r s :
Dr. Renesha Islam
Resident (Year 4)
Pediatric Hematology and Oncology DEPARTMENT.
69.
70. o A malignant tumor of bone or soft
tissue.
o Second most common malignant
primary bone tumor of children &
young adults.
71. o American Pathologist.
o Served as Professor of Pathology
for 33 years at Cornell University
New York.
o in 1921, described Ewing sarcoma
as an undifferentiated tumor
involving the diaphysis of long
bones that is radiation sensitive
(in contrast to osteosarcoma).
72.
73. o Age : median age 15 years.
o Median time to diagnosis 3-9 months.
o Common site: Diaphysis of long bones.
74. o Exact etiology is unknown.
o Certain epidemiologic association studies have indicated
higher rates in children with a history of inguinal hernia
and in children of farm workers.
o 80% of patients are younger than 20 years at diagnosis.
o Low incidence in Black and Asian children.
o Not usually associated with familial cancer syndromes.
o The risk of ewing sarcoma has not been shown to
increase following radiation exposure.
SADIA
Age: 10 years
Father: Farmer
76. Intraosseous component:
-Firm gray white.
-Moist and glistening.
-May be almost liquid and may
resemble to pus.
Extraosseous comonent:
o Softer & more friable.
o Diffuse involvement of medullary
cavity is often obvious.
79. Commonly present with the following
symptoms:
• Pain at site of tumor (96%)
• Local swelling & or palpable mass (61%)
• Fever (21%)
• Pathologic fracture (16%).
• Constitutional symptoms – Weight loss,
malaise.
80. Extent and duration of
physical symptoms as well as
the associated physical
limitations.
• General: Temperature, Pallor, Weight.
• Local: Tender palpable mass. For
centrally located tumors palpable
mass may not be evident.
• Most commonly involved site :
Diaphysis of long bones.
Complete Blood count
Urinalysis
BUN, S. Creatinine, liver
enzymes, ALP, S. LDH
History taking
Physical findings
Lab investigations
SADIA
-Pain & swelling
-Tender
palpable mass
-LDH- High
89. Chest Xray
Bone marrow study (Bilateral)
Chest CT scan
USG of whole abdomen
CT scan of abdomen
Bone scan
FDG PET scan
Biopsy from other suspicious site.
SADIA
• Urinary VMA-
within normal
value
• Uninvolved bone
marrow
• Chest Xray, Chest
CT, USG & CT
abdomen- Normal
findings
• Histopathological
findings: left supra
orbital ridge- No
malignancy.
90. Standard risk
• Localized
disease
• <13.7 years
• Size: <8cm
• Primary site:
extremity
Intermediate
risk
• Non SR
localized
disease
High risk
• Metastatic
disease.
Sadia
101. In cases of patient presenting with limb pain,
swelling & restriction of movement, suspicion of bone
tumor must be excluded.
Proper clinical examination, imaging, histopathology
& immunohistochemistry are essential diagnostic
workup for a malignant bone tumor.
most common site of osteosarcoma is proximal tibia
Diffuse sclerosis and foci of lytic area with ant cortical destruction with periosteal reaction is noted in upper metadiaphyseal region of left tibia. Zone of transition is wide and indistinct.
Articular surfaces are smooth
Joint space is maintained
Mild soft tissue swelling is seen
Comment:
Features are suggestive of malignant bone tumor likely Osteosarcoma/ Ewing Sarcoma.
D/D : Osteomyelitis
Findings:
Heterogeneously enhancing hypo intense area involving epiphysis, metaphysis, partly diaphysis and involved surrounding anterior compartments of muscles and compressed posterior and lateral compartments.
Joint spaces are maintained
Rest of the superficial and deep vessels appear normal.
Impression:
Heterogeneously enhancing area involving tibia and ant compartment of muscles features suggest Ewing sarcoma however possibility of osteomyelitis could not be ruled out.
Note: the most common sites of distance metastases are lymph nodes, bone, bone marrow, liver, orbit and dura.
Liver is mildly enlarged in size (15.5 cm). No focal lession is noted.
Gall bladder is normal in size, wall is normal in thickness.
Spleen is normal in size.
Pancreas is normal in size, shape, position with uniform tissue attenuation.
Both kidnyes are normal in size, shape, position & axial orientation with normal excretion of contrast media. Pelvicalyceal systems of both kidneys are not dilated. Both uterus are not dilated.
Both adrenal glands are normal in appearance.
Urinary bladder reveals no abnormality.
Bowel loops also reveal no abnormality.
No ascites is noted.
