OS 217: Systemic Diseases – Oncology Module
Plenary 01: Pediatric Oncology Cases
Plenary 01: Pediatric Oncology Case 2
C. Villegas, MD; M.Y. Lesaca-Medina, MD; M. Calaguas, MD; P. Fajardo, MD
TOPIC OUTLINE
I.
II.
III.
IV.
V.
VI.
VII.
September 18, 2014
( - ) polyuria, polyphagia, polydipsia,
tremor
( - ) dizziness, headache,
lightheadedness
( - ) arthritis, sprain/strain
( - ) pruritis, bruising, cyanosis,
pallor, rashes, clubbing
ENDOCRINE
History
Physical Examination
Differential Diagnoses
Pathophysiology
Work-up
Management
Comments from Panelists
OS 217 1
NEUROLOGIC
MSK
SKIN
E. PAST MEDICAL HISTORY
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(-) previous hospitalizations and infections
Allergy to excessive hamburgers at 8 months old
Allergic reaction to diclofenac (NSAID) given to her in one medical
mission last May due to persistent leg pain
o Presented with difficulty of breathing and skin manifestations
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(-) Cancer
(+) Hypertension (mother)
(+) Arthritis (grandfather)
(-) DM
(-) TB
(-) Kidney disease
(-) Brain disease
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
Born via CS at a hospital in Camarines Norte
2nd of 5 siblings
(-) fetomaternal complications
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At par with age
Currently, 9th grade student enrolled at a home study program –
prior to this, patient was a regular high school student at
Dasmarinas National High School with average grades


Not breastfed and given formula instead
Diet consists mostly of vegetables and meat with rice
2 MONTHS PTA (JULY 2014)

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Complete immunization administered by family paediatrician (EPI:
BCG, Hep B, DPT, Measels, OPV)
Unrecalled if given booster shots
Credits to Block 6 for presenting the case and providing us with their PPT
presentation. 
I. HISTORY
●
A.GENERAL DATA
J.M., 14 years old Female
○ Student (Grade 9)
○ Roman Catholic
○ Originally from Camarines Sur but resides in Dasmarinas, Cavite.
B. CHIEF COMPLAINT

Persistent pain on distal left thigh
C. HISTORY OF PRESENT ILLNESS
G. BIRTH AND MATERNAL HISTORY
4 MONTHS PTA (MAY 2014)
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Experienced weakness on the left leg after jogging (not induced by
trauma) described as “nangangalay, namimintig, iika-ika”
Experienced body malaise the next day
On the last week of may also started experiening pain on distal left
thigh (graded 7/10) described as “makirot”. Pain was non-radiating
and lasted 15 minutes per episode. It was sudden in onset and often
awakened the patient at night
No associated back pain, joint pain, and numbness
A palpable mass (2 inches) on the distal left thigh was reported.
The mass was pulsating, warm, and non-erythematous.
Tried hilot, vicks, and Salonpas but these did not relieve the pain
Consultation at UMC Hospital in Dasmarinas
o Labs done
o Given tramadol and paracetamol (325 mg every 8 hours) to
relieve pain. Pain persisted but is relieved by medications
Went to PGH for further tests: labs, CT scan, MRI
Patient was not able to walk after the biopsy procedure due to the
rapidly increasing mass.
Consulted at the PGH Orthopedics Department every week.
MRI: revealed L distal femoral metaphyseal solid mass
suggestive of parosteal osteosarcoma, Ewing sarcoma, primitive
neuroectodermal tumor, or lymphoma)
3 DAYS PTA (SEPTEMBER 12 2014)

Experienced intermittent febrile episodes of unrecalled
temperature (4x in 3 days)
o Leg pain was persistent and was not associated with febrile
episodes
In the interim
 Experienced difficulty of breathing
 Noted weight loss (1 peso coin → 25 centavo coin)
September 15, 2014
 Admitted to Cancer Institute at PGH for chemotherapy
(-)Synaptophysin
(-)Chromogranin
 bone marrow scan result is still pending
D. REVIEW OF SYSTEMS
HEENT
RESPIRATORY
CARDIOVASCULAR
GIT
GU
YNA – ANGELI - ANTON
F. FAMILY HISTORY
( - ) blurring of vision, ringing of ears,
ear pain, eye redness
( - ) wheezing, cough, hemoptysis
( - ) chest pain, ortopnea, edema,
easy fatiguability
( - ) abdominal pain, jaundice,
diarrhea, bloody stools, nausea,
vomiting, loss of appetite
( - ) intermittency, hematuria,
nocturia, urgency, inocontinence,
pain in urination
H. DEVELOPMENTAL HISTORY
I. NUTRITIONAL HISTORY
J. IMMUNIZATION HISTORY
K. MENSTRUAL HISTORY
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Menarche at 11 y/o (Grade 6)
Regular menstruation with moderate flow (2 pads per day) lasting for
7 days
LMNP 1st week of September
L. PERSONAL AND SOCIAL HISTORY
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The patient is 2nd of 5 siblings (all girls).
Father is a truck driver and mother is a housewife
(-) Alcohol, smoking, illicit drug use
PE classes in school were her only exercise routine before onset of
symptoms
Living conditions in the province: houses are far apart, no known
exposure to environmental toxins and chemicals
II. PHYSICAL EXAMINATION
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BP 90/60 right arm supine, HR 123 bpm, RR 26 breaths/min
Temp 36.9°C, Ht: 139.7 cm, Wt: 43 kg, BMI: 22.0 (within normal
limits in growth chart)
Skin: (-) jaundice, (-) pallor, (-) cyanosis, pink nailbeds, (-) clubbing,
CRT < 2 secs
HEENT: pink conjunctiva, anicteric sclerae, pink oral mucosa, (-)
visible neck masses or distended veins, (-) CLAD
Observable mass on left leg around the distal thigh/knee area.
(+) tenderness, (-) erythema, (-) discharge, unable to fully extend
L leg, superficial vessels/striae
Page 1 / 7
Plenary 01: Pediatric Oncology Cases
OS 217
III. DIFFERENTIAL DIAGNOSES
Rule In
Osteosarcoma
Rule Out
Weakness and pain
on left leg
No history of injury
Palpable mass along
metaphysis of distal
left thigh
Limitation of motion
Figure 1. Mass on Distal Knee
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Intermittent fever
23 cm span (half circumference)
39 cm base circumference
6 cm elevation
8 cm longest diameter
Body malaise
Weight loss
CARDIO-RESPIRATORY
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Inspection: (-) chest wall deformities, adynamic precordium, (-) chest
retractions, (-) alar flaring, (-) use of accessory muscles, but with
noted tachypnea (RR 26 breaths/min)
Palpation: (-) tenderness or masses on the chest, (-) heaves or
thrills, point of maximal impulse at 5th ICS MCL, equal and
symmetrical tactile fremitus, symmetrical chest expansion
Auscultation: tachycardic (HR 123 bpm) with regular rhythm, clear
distinct heart sounds, (-) murmurs, apex beat at 5th ICS MCL, (-)
abnormal breath sounds
Ewing Sarcoma
Most
common
primary
malignant
bone
tumor
in
children
and
adolescents
Pain on left leg with
nighttime awakening
Palpable mass at
distal left thigh
Characteristic location
(diaphysis of long bones
and flat bones)
Age (usually <10 y/o)
Limitation of motion
ABDOMINAL
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Inspection: Abdomen does not appear distended. (-) flank bulges, (-)
masses or organs visible, (-) scars, (-) striae, (-) dilated veins.
Umbilicus is midline, neither depressed nor everted.
Palpation: (-) abdominal tenderness on light palpation, (-) abdominal
tenderness on deep palpation, (-) masses, liver edge not palpated
Auscultation: normoactive bowel sounds (13/min/quadrant)
Percussion: liver span = 7 cm
Splenic Percussion Sign and CVA Tenderness were not assessed.
Intermittent fever
Body malaise
Weight loss
Second most
common primary
malignant bone
tumor in children
and adolescents
MUSCULOSKELETAL
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
Upper Extremities:
o Muscles were symmetrical and without any observable atrophy,
hypertrophy, involuntary movements, fasciculations, joint
contractures or postural tremors.
o Muscle Tone and Strength: (-) spasticity/rigidity, Grade of 5/5 for
shoulder abduction, elbow flexion and extension, wrist flexion
and extension. 4/5 for finger extensors and finger abductors
of both hands, 4/5 for finger flexor of left arm. 5/5 for the
other hand and finger muscles.
o Light touch Sensation: intact and symmetric (R to L, proximal to
distal)
o Pain Sensation: intact and symmetric (R to L, proximal to distal).
But not able to differentiate dull from sharp in C6 of right arm.
o Position sense: intact for both left and right.
Lower Extremities:
o Not tested because of painful mass in left leg.
o (+) L knee/distal thigh swollen mass spanning 23 cm, (-)
erythema, (+) tenderness, (-) discharge, unable to extend L,
superficial vessels/striae
o Light touch Sensation: intact and symmetric (R to L, proximal to
distal)
o Pain Sensation: intact on right and left sides (left side was not
tested but patient reported pain from the mass)
o Position sense: intact for both.
NEURO: CRANIAL NERVES
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CN I: Not tested but no complaints on olfaction.
CN II: Visual acuity OD 20/20 -2, OS 20/20 -3. (-) visual field cuts
CN II and III: Pupils symmetric in size. Consensual and direct
reflexes were intact.
CN III, IV, VI: Full range of motion for EOMs, good accommodation,
(-) nystagmus
CN V: Intact and symmetrical pain and light touch sensation for
ophthalmic, maxillary, and mandibular nerves. Strength grading of
5/5 for muscles of mastication.
CN VII: Intact facial nerve, (-) asymmetry, (-) spasms, (-) facial
weakness, taste sensation not tested
CN VIII: Rinne BC>AC both ears. (-) lateralization in Weber.
CN IX and X: Able to phonate. (-) dysphagia. Gag reflex not tested.
CN XI: Strength grading 5/5 and symmetrical SCM and trapezius
muscles.
CN XII: Tongue is midline, (-) atrophies, (-) fasciculations. Grade 5/5
YNA – ANGELI - ANTON
Osteomyelitis
Pain on left leg
No draining sinus tract
Palpable warm mass
at distal left thigh
No fever at onset of
symptoms
Intermittent fever
4 months duration
Characteristic
location (metaphysis
of long bones)
Tachycardia,
tachypnea
Benign Bone
Tumors
Pain on left leg
(osteochondroma,
osteoblastoma, osteoid
osteoma, fibroma etc.)
Palpable mass
distal left thigh
Limitation of motion
at
Intermittent fever
Body malaise
Characteristic
location
(metaphysis of long
bone e.g., distal
femur, proximal
humerus, proximal
tibia)
Weight loss
One of the most
common
benign
tumors in children
Soft Tissue Tumors
(rhabdomyosarcoma,
non-rhabdomyosarcoma)
Pain on left leg
Palpable mass
distal left thigh
at
Page 2 / 7
Plenary 01: Pediatric Oncology Cases
Most
common
pediatric soft tissue
sarcoma (usually <
20 y/o)
Lymphoma
Weakness and pain
on left leg
OS 217
V. WORK UP
Diagnostic Pivot: Bone pain with or without swelling
No CLAD
No hepatomegaly
Bone cyst
(e.g. aneurismal bone
cyst)
Weight loss
Weakness and pain
on left leg
Palpable mass
distal left thigh
at
Limitation
motion
of
No associated neurologic
symptoms
or
spinal
involvement
Reactive lesion of
bone seen in <20 y/o
Characteristic
location (femur, tibia,
spine)
Histiocytosis
Progressively
growing,
expansile
lesion
Pain on left leg
(e.g. Eosinophilic
granuloma)
Characteristic
(skull)
location
Age group (5-10 yrs.;
mean age of 5.5 yrs.)
IV. PATHOPHYSIOLOGY
Primary Working Impression: OSTEOSARCOMA on distal left femur
to rule out SOFT TISSUE MALIGNANCY
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•
•
Osteosarcoma is the most common primary bone malignancy, with a
high incidence in children and adolescents
Tumors most often arise in the long bones from osteoid-producing
neoplastic cells near the growth plates
The most common sites include:
o Femur (42%, most in the distal femur)
o Tibia (19%, most in proximal tibia)
o Humerus (10%, most in proximal humerus)
Interplay between genetic and environmental factors
Genetic cause largely unknown but research shows:
o Mutations involving p53 and Rb gene
o High level of chromosomal instability
 microsatellite instability (MIN)
 CpG island methylator phenotype (CIMP)
o Unstable telomere maintenance
 Females- shorter telomere length > inc. risk
o Lack of Sprouty2 protein expression
 Increased cell proliferation and migration
o Human P2X7 receptor expression
 P2X7RA and truncated P2XRB7 are powerful stimuli for
growth
o Increased calcium mobilization and enhanced NFATc1 activity
stimulate growth and metastasis
Certain genetic and acquired conditions (hereditary retinoblastoma,
Li-Fraumeni syndrome, Rothmund-Thomson syndrome) predispose
patients to development of osteosarcoma.
Known environmental risk factors
o Radiation exposure
o Methylcholanthrene
o Chromium salts
o Beryllium oxide
o Zinc beryllium silicate
o Aniline dyes
o Asbestos
YNA – ANGELI - ANTON
STAGING AND RISK ASSESSMENT
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Definitive diagnosis requires histological examination of tumor
material obtained by open biopsy performed by an orthopedic
oncologist
Primary tumor must be evaluated by plain radiographs (X-ray) in
two planes
MRI is the most useful to evaluate intramedullary and soft tissue
extension of osteosarcoma and its relation to vessels and nerves
Chest X-ray (10% negative) or CT scan to check for lung
metastases
o Cannonball-like lesions on CXR, nodules on CT scan
Radionuclide bone scan or skeletal survey (X-ray) to check for
bone metastases
PLAIN RADIOGRAPH
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Osteosarcoma
o Sclerotic destruction (less commonly lytic)
o “Sunburst pattern”
Page 3 / 7
Plenary 01: Pediatric Oncology Cases
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Ewing Sarcoma
o Primarily lytic
o Multi-laminar periosteal reaction
o “Onion-skinning”
OS 217
Taken 8/2/14 (UPMC)
 Fairly defined 10.6 x 8 x 6.7 mass arising from the medial and
posterior cortical aspects of the distal femoral metaphysis
associated with periosteal upliftment as well as abnormal
marrow signals.
o Suggestive of parosteal osteosarcoma, Ewing sarcoma, PNET,
or lymphoma
 Myositis, edema of the vastus medialis and biceps femoris muscles
 Abnormal intrasubstance signals in the popliteus tendon (tumor
infiltration or partial tear)
 The rest of the musculature, bones, subcutaneous fatty layer,
superficial veins, acetabulofemoral and tibiofemoral joints are
unremarkable.
CHEST CT-SCAN PLAIN
Taken 9/10/14 (Cavite)
 There are hazy and patchy densities seen in the superior
segment of the right lower lobe. There is subcentimeter nodular
density seen in the antero-basal segment of the left lower lobe
abutting the lateral pleura.
 No evident enlarged lymph nodes.
 No pleural effusion nor atelectasis noted.
 The heart and blood vessels are normal.
 The tracheo-bronchial tree is patent with no intraluminal lesions
evident.
 Additional sections of the liver and adrenal glands reveal no focal
lesion.
 The rest of the soft tissues and osseous structures are intact.
ASSESSMENT

Angiography can assist in surgical planning and estimate response
of tumor to preoperative chemotherapy
o High-grade tumor = ↑ vascularity = ↑ contrast enhancement
o Enhancement referred to as “tumor blush”
o Disappearance of tumor vascularity after preoperative
chemotherapy correlates with good treatment response
See Appendix I for Radiologic Studies used in the Evaluation of
Osteosarcoma and Detection of Mets
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Alkaline phosphatase (30%) and lactate dehydrogenase (40%)
are elevated levels correlate with poor prognosis
Before interdisciplinary treatment, assessment of organ function
and general health must be performed including complete blood
count (CBC) with differential count, blood group typing, coagulation
profile, renal and liver function test as well as hepatitis and HIV
testing
Before chemotherapy, baseline assessment of cardiac and
auditory function including echocardiogram or radionuclide
ventriculography and audiogram
LABORATORY FINDINGS
See Appendix II for complete laboratory findings tables.
DIAGNOSIS
OSTEOSARCOMA (Stage IVa) on distal left
femur with PULMONARY METASTASIS
VI. MANAGEMENT
MRI
Determine:
 Intraosseous vs extraosseous tumor extension
 Neurovascular bundle identification
SURGERY
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Primary objective is to achieve long-term disease-free survival while
preserving limb function as much as possible
o Limb-sparing/limb-salvage operation
If limb-sparing resection cannot be performed amputation should be
considered
Multimodal treatment of high-grade osteosarcoma increases
disease-free survival probabilities from only 10-20% to >60%
CHEMOTHERAPY
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YNA – ANGELI - ANTON
Doxorubicin, cisplatin, high-dose methotrexate with leucovorin
rescue and ifosfamide
Standard protocol: Doxorubicin + cisplatin +/- methotrexate
Preoperative chemotherapy (Neo-adjuvant therapy)
o Induce tumor necrosis of primary tumor
o Treatment of micrometastasis
o Facilitates surgical resection with wide margins
o Prognostic factor
Page 4 / 7
Plenary 01: Pediatric Oncology Cases
OS 217
REHABILITATION
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Early prosthetic fitting and gait training
Patient information and counseling
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Monitor for local and systemic recurrence
o History and PE plus imaging of extremity and chest
 Every 3 months for the first two years
 At least every 6 months from the 2nd to 5th years
 Yearly after the five years
o Annual bone scan for the first two years
Long-term effects of chemotherapeutic agents
o Fatal: Cardiotoxicity from anthracycline
FOLLOW-UP
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PROGNOSIS
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Surgical resection alone is curative only for parosteal osteosarcoma
patients
Conventional osteosarcoma requires multi-agent chemotherapy
o 5-year survival of patients with nonmetastatic extremity
osteosarcoma is 65-75% with chemotherapy and surgery
o Up to 75% with nonmetastatic extremity osteosarcoma are cured
o Prognosis not favorable for pelvic tumors
20-30% with pulmonary metastases can be cured with aggressive
chemotherapy and lung nodule resection
Late isolated lung metastases may be cured with surgical resection
alone
Bone metastases and widespread lung metastases have poor
prognosis
VII. COMMENTS FROM PANELISTS
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From Dr. Villegas: I would amputate this patient for pain relief, this
would also decrease the tumor burden for chemotherapy despite
being stage IV, to improve the quality of life the remaining days of
the patient.
From Rad Onco: Proper treatment for this is still surgery. Radio
oncologists are always the last to be consulted if necessary, or in
cases where there are positive or close margins or the
osteosarcoma is not responsive to other modalities.
From Pedia Onco:
o Include in your PE the Visual Pain Assessment Scale
o Note that after the first day of chemo the patient will feel relief of
pain and there will be control of Angiogenesis
o You should probably also refer the patient and her family to
hospice care
o Try adding TB or TB joint to your differential diagnosis
 In TB joint you find mass, bone involvement, calcifications, soft
tissue swelling and cold joints (painless)
o What do you find on Xrays showing osteomyelitis?
 You find sequestrum, new bone formation and you may find a
characteristic bowing of the knees known as saber shin
o In bone tumors we usually see periosteal reaction wherein a new
layer of bone forms and codman triangle, which is the new
subperiosteal bone that is formed when a tumor raises the
periosteum from the bone; these signs however are not specific to
tumors
o Note that in this patient palliative care is being given, thus, only
60% of the treatment regimen is being given to prevent
neutropenic fever.
END OF TRANSCRIPTION
YNA – ANGELI - ANTON
Page 5 / 7
Plenary 01: Pediatric Oncology Cases
OS 217
APPENDIX I
APPENDIX II: COMPLETE LABORATORY FINDINGS
Taken 9/10/14 (Cavite)
Exam Name
CBC
WBC
Hemoglobin
Hematocrit
Platelet
Differential Count
Neutrophil
Lymphocyte
Monocyte
Eosinophil
Basophil
Creatinine
ALT
Total Bilirubin
Direct Bilirubin
Indirect Bilirubin
Albumin
Calcium
Magnesium
Sodium
Potassium
YNA – ANGELI - ANTON
Ref. Value
Result
4 - 11
115 - 148
0.38 - 0.44
150 - 400
15.6
109
0.32
580
0.55 - 0.65
0.25 - 0.35
0.03 - 0.06
0.02 - 0.04
0.00 - 0.01
0.69
0.21
0.06
0.01
0.00
3.4 - 20.5
0.0 - 8.6
3.4 - 11.9
3.5 - 5.1
0.66 - 1.07
136 - 145
53 - 97
0 - 55
35 - 50
2.23 - 2.5
52.4
123
13.5
9.7
3.8
33
2.23
0.86
138
3.2
Unit
x 109/L
g/L
Interpretation
High
Low
Low
High
High
Low
Low
umol/L
IU/L
umol/L
umol/L
umol/L
g/L
mmol/L
mmol/L
mmol/L
mmol/L
Low
High
High
Low
Page 6 / 7
Plenary 01: Pediatric Oncology Cases
OS 217
Taken: 9/15/14 (UP-PGH)
Exam Name
CBC
WBC
RBC
Hemoglobin
Hematocrit
MCV
MCH
MCHC
RDW
Platelet
Differential Count
Neutrophil
Lymphocyte
Monocyte
Eosinophil
Basophil
Creatinine
ALT
Total Bilirubin
Albumin
Calcium
Magnesium
Sodium
Potassium
Urinalysis
Color
Specific Gravity
Transparency
pH
Sugar
Protein
Microscopic
RBC
WBC
Epithelial Cells
Bacteria
Mucus Thread
Cast Crystals
Special
Bilirubin
Urobilinogen
Ketone
Leucocytes
Nitrite
Blood
YNA – ANGELI - ANTON
Ref. Value
Result
Unit
Interpretation
4.5 - 11
4.2 - 54
120 - 160
0.38 - 0.47
80 - 96
23 - 31
320 - 360
11 - 16
150 - 450
12.79
4.11
108
0.34
83.1
26.4
318
12.2
491
x 109/L
x 1012/L
g/L
High
Low
Low
Low
0.50 - 0.70
0.20 - 0.50
0.02 - 0.09
0.00 - 0.06
0.00 - 0.02
0.67
0.26
0.06
0.01
0.00
27 - 88
8 - 32
5.1 - 20.5
31 - 48
2.24 - 2.58
0.7 - 1
132 - 143
3.4 - 5.4
50
141
11.2
23
2.06
0.91
136
3.4
fL
pg
g/L
Low
Low
x 109/L
High
umol/L
IU/L
umol/L
g/L
mmol/L
mmol/L
mmol/L
mmol/L
High
Low
Low
Yellow orange
1.015
Hazy
7.0
+1
1-3
25 - 30
Few
+4
Occasional
-
/hpf
/hpf
/hpf
/hpf
/hpf
+1
+1
Trace
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