FINAL EVALUATION

DEPARTMENT OF PEDIATRICS AND CHILD HEALTH FACULTY OF MEDICINE

DIPONEGORO UNIVERSITY/ DR KARIADI GENERAL HOSPITAL SEMARANG
2022
Name of participant : Mahafendy Suryamanika Tukan, MD
Date of examination : November 25th, 2022
I. PATIENT IDENTITY
Name : ZPA Father’s name : Mr. HA
Age : 7 years 11 months Age : 41 years old
nd
Date of birth : December 2 , 2014 Occupation : Entrepreneur
Gender : Male Education : Bachelor degree
Address : Semarang Mother’s name : Mrs. A
MR/Reg number : C682XXX/123XXXX Age : 31 years old
Date of admission : November 13th, 2022 Occupation : Entrepreneur
Room : Pediatric ward Education : Senior high school
II. HISTORY TAKING
Allo-anamnesis was performed on the 2nd day of admission (November 14th, 04.00 PM) at the
pediatric ward to the parents and data was obtained from the medical record.
1. History of present illness
Chief complaint: to undergo rhabdomyosarcoma 4th week chemotherapy
History of Illness
A 7 years 11 months old male patient had been diagnosed with rhabdomyosarcoma for the past
14 months. He had already undergone 2 times of excision tumor surgery and finished one cycle
of rhabdomyosarcoma chemotherapy for 24 weeks at June 4th, 2022. However, the tumor was
reappeared and then he underwent the 2nd cycle of rhabdomyosarcoma chemotherapy since
September 28th, 2022.
Three months before admission (August 2022), the patient’s mother said there was a small
tumor at his upper right chest, the size was about 1 cm, soft consistency, mobile, no redness or
pain. There was no complaint of the eyes, no nasal discharge, headache, vomiting, joint pain,
abdominal pain, or any complaints of urination and defecation. He was referred to plastic
surgeon and the tumor was not cheloid from the past surgery. Then he was referred to pediatric
surgeon and programmed to do surgery.
Two months before admission (September 28th 2022), the patient had wide tumor excision
and partial sternotomy. Pathology examination showed that the tumor was alveolar
rhabdomyosarcoma. Then he was planned to undergo 2nd cycle of high-risk rhabdomyosarcoma
chemotherapy.
A week before admission, the patient was planned to undergo the 4th week chemotherapy for
high-risk rhabdomyosarcoma. He had no fever, nausea, or vomiting. He had diarrhea 3-4 times
per day after the previous chemotherapy, but the diarrhea improved. He was active and had good
appetite.
On first day of admission, the patient was active. He had no fever, nausea, vomiting, or cough.
He had good appetite. There were no complaints of urination and defecation. Then, he was
planned to undergo the 4th week chemotherapy for high-risk rhabdomyosarcoma the following
day.
On second day of admission, he had 4th week chemotherapy of rhabdomyosarcoma. He had no
fever, nausea, vomiting, or cough. He had good appetite. There were no complaints of urination
and defecation. During the chemotherapy, the patient had no fever, nausea, or vomiting.
On third day of admission, he had nausea and vomiting 2 times after chemotherapy. However,
the complaint improved then. He still had good appetite and there were no complaints of
defecation or urination. He was planned for outpatient care.
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2. Past medical history
- One year 4 months before admission (July 2021), the patient’s mother found a small round
mass at his upper right chest, the size was about 3 cm, hard consistency, fixed, no redness,
and felt no pain. There was no complaint of the eyes, no nasal discharge, headache, vomiting,
joint pain, abdominal pain, or any complaints of urination and defecation. The patient went
to the clinic and was said to be lipoma. Then he was referred to the surgeon at private hospital
and was said to be lymph node infection. He was given cefixime for 5 days, but the mass’s
size was not improved.
- One year 3 months before admission (August 2021), the mass’s size enlarged, the size was
about 5 cm, hard consistency, no redness, and no tenderness. The patient had neck ultrasound
examination and the result was hemangioma with differential diagnosis soft tissue tumor.
Then he underwent thoracic MSCT and the result showed that there was teratoma dd/
rhabdomyosarcoma extended to thoracic cavity. He was referred to Kariadi Hospital for
further treatment.
- One year 2 months before admission (September 2021), the patient underwent the wide
excision tumor surgery and biopsy examination.
- One year 1 month before admission, the biopsy result showed that he had
rhabdomyosarcoma. Then he underwent the 24 weeks of chemotherapy for 9 months
(October 6th 2021 to June 4th 2022) without Actinomycin regiments due to its unavailability.
During the chemotherapy, the patient felt pain at bones and joints. He also had difficulty to
walk and sometimes fall. He was consulted to medical rehabilitation and got hydrotherapy
treatment for a month. Then he got better.
- There was no history of bleeding or pale.

3. Family medical history and pedigree I

The patient’s great grandfather had lung cancer, II
his grandfather had lymphoma and his father has III
obesity.
IV
Figure 1. Pedigree
4. Perinatal history
The patient was born 38 weeks of gestation from a G1P0A0, 24-years-old mother. The mother
had routine prenatal care at obstetrician more than 4 times and took regular vitamin daily. No
history of fever, rash, edema, hypertension, no tobacco smoke exposure or alcohol consumption
during pregnancy. The patient was born by caesarian section due to narrow hip. He was a
vigorous baby with birth weight 3700 grams, birth length 49 cm, and no data for head
circumference. No history of breathing difficulty, no cyanosis, no jaundice.

5. Feeding history
He was exclusively breastfed until 6 months. He started complementary feeding at 6 months and
gradually increased to solid food. Now, he patient has regular meals 3 times daily with various
menu consist of rice, eggs, chicken, fish, meat, vegetables and fruits. He rarely drinks milk. He
often eats junk food, chocolate and ice cream. For the last three days before admission, he ate 3
times daily. He ate a portion of rice with chicken, a portion of rice with egg and vegetables and
a portion of rice with fish. No history of food allergy.
Conclusion: Exclusive breastfeeding, with excessive quantity and quality of food intake.

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6. History of immunization:
He received Hepatitis B at birth. BCG and polio 1 at 1 month old, DPT/HB/HIB 1 and polio 2 at
2 months old, DPT/HB/HIB 2 and Polio 3 at 3 months old, DPT/HB/HIB 3 and polio 4 at 4
months old, MR vaccine at 9 months old and 18 months old. He also received Td vaccine at 6
years old.
Conclusion: complete basic immunization according to health ministry’s program, complete
booster immunization.

7. Growth and development history:

Growth: Boy, 7 years 11 months old, birth-weight 3700 grams (P90), birth-length 49 cm (P50).
Body weight for the past 3 months were 38 kg, 37,8 kg, and 37,8 kg. Present weight is 38 kg,
present height is 125 cm, BMI 24.3 kg/m2. Weight for age is >P97, height for age is P25-P50, BMI
for age is >P97.
Conclusion: Cross-sectional: Obese, normal stature. Longitudinal: Flat of Growth.
Developmental: PedsQL3.0 cancer module scale: pain and hurt: 87.5, nausea: 80, procedural
anxiety: 33, treatment anxiety: 83, worry: 83, cognitive problems: 100, received physical
appearance: 100, communication: 100. Pediatric Symptom Checklist 17 Items (PSC-17): 4,
Strength and Difficulties Questionnaire; Total difficulties score: 6 (Emotional symptoms scale:
2, Conduct problems scale: 0, Hyperactivity score: 0, Peer problems score: 3), Prosocial
behavioral score: 10. Parenting style: democratic. Conclusion: normal developmental
milestones, appropriate for age, problem with procedural anxiety.

8. History of patient’s basic needs

Caring: The patient lives with parents, grandmother, aunt and a little brother in a permanent
house. His mother and father have good interaction with the patient at home. He had complete
immunization fulfilled by primary health care.
Loving: He is the first and expected child from first marriage of both parents. He lives with his
parents and brother, raises with full support.
Stimulating: He gets proper stimulation from both parents. He gets along well with his peers.
Conclusion: fulfilled basic needs.

9. Socio-economic condition of the family:

His father and mother are catering entrepreneur. Monthly income is around IDR 5.000.000.
Medical and hospital costs are covered by the national health insurance (class III on BPJS). This
family is categorized as a middle-class family according to the criteria of the Central Bureau of
Statistics.
The patient currently lives in a 233m2 house with 5 bedrooms, 3 bathrooms, and 1 kitchen. His
house had good ventilation and light. The distance between his house and primary health care is
around 1 km.
Conclusion: middle socio-economic status

III. PHYSICAL AND SUPPORTING EXAMINATION

1. Physical Examination
Performed on November 14th 2022, 04.30 PM, 2nd day of admission in the pediatric ward.
The patient was alert and active. HR 92 bpm, regular pulse, adequate volume and pressure, BP
100/60 mmHg (P50), RR 20 rpm, temperature 36.7oC, oxygen saturation 99% room air. His
current body weight was 38 kg, body height was 125 cm, BSA 1.14 m2. Eyes examination
revealed no pale conjunctiva, no icteric sclera, and no palpebral edema. There was no dental
caries, no gingival hypertrophy, and his pharynx not hyperemic, T1-T1 tonsil. There was no
lymphadenopathy, but there was acanthosis on neck. His heart and lungs were normal. There
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was surgical scar on his chest in form of vertical line approximately 10 cm long. Abdominal
examinations revealed that there were tympani, no shifting dullness in his flank, no abdominal
striae, the liver was just palpable and there was no spleen enlargement, no lymphadenopathy
inguinal was found. There was no sign of hemorrhage, no epitaxies, gum bleeding, petechiae,
nor melena. On genitalia, there was no phimosis, no erythema on the orifice external urethrae,
no buried penis and no scrotal edema. Tanner stage; genitalia stage 2, pubic hair stage 2.
Conclusion: physical examination within normal limit with surgical scar on the chest.

2. Supportive Investigation
Blood examination (November 7th 2022): Hemoglobin 11.1 g/dl, hematocrit 34.1 %, MCH
26.1 pg, MCV 80 fL, MCHC 32.8 g/dL, leucocyte 4400/mm3, platelet 395000/mm3
Conclusion: blood examination within normal
Rontgen of Thorax (August 31st 2022): No cardiomegaly, No infiltrate nor nodule on
pulmonary, there was lobulated opacity on paravertebral sinistra et dextra, suggesting soft
tissue mass. (Figure 5)
Mass Biopsy (September 9th 2021): Malignant round cell tumor on hemithorax dextra,
differential diagnosis: Rhabdomyosarcoma, malign lymphoma.
Mass Biopsy (September 29th 2022): Alveolar rhabdomyosarcoma on costae region to
sternum.
Thoracal MSCT with contrast (August 2nd 2021): solid cystic mass at superior anterior
dextral mediastinum extended to proximal and lateral dextral sternum region, the latero-lateral
size was 4 cm x antero-posterior size 5 cm x cranio-caudal size 4.2 cm, with enhancement,
suggesting teratoma dd/ rhabdomyosarcoma; no bones destruction, no paraaorta and
paratracheal lymph nodes enlargement. (Figure 6)

IV. SUMMARY
A 7-year-11-months-old boy with a body weight of 38 kg and length of 125 cm was diagnosed
with rhabdomyosarcoma, the child was continuing his 4th weeks chemotherapy. He already
had undergone 2 times of tumor excision surgery and finished one cycle of rhabdomyosarcoma
chemotherapy for 24 weeks at June 4th, 2022. However, the tumor reappeared and then he
underwent the 2nd cycle of high-risk rhabdomyosarcoma chemotherapy since September 28 th,
2022. He had no fever, nausea, vomiting, or cough. He had good appetite. There were no
complaints of urination and defecation. On third day of admission, the day after chemotherapy,
he had nausea and vomiting 2 times. However, the complaint gradually improved. The physical
examination had acanthosis on his neck and blood examination within normal limit. Laboratory
examination revealed hemoglobin 11.1 g/dl, hematocrit 34.1 %, MCH 26.1 pg, MCV 80 fL,
MCHC 32.8 g/dL, leucocyte 4400/mm3, platelet 395000/mm3. Rontgen of the chest on
November 15th 2022 showed no cardiomegaly, no infiltrate nor nodule on pulmonary, there
was lobulated opacity on paravertebral sinistra et dextra, suggesting soft tissue mass. While
th
mass biopsy on September 29 2022 resulted as alveolar rhabdomyosarcoma at costae region
to sternum. He was diagnosed with rhabdomyosarcoma. The child was admitted to the pediatric
ward and programed for chemotherapy with high-risk rhabdomyosarcoma protocol.

V. DIFFERENTIAL DIAGNOSIS
1. Rhabdomyosarcoma
2. Obesity dd/ idiopathic dd/ Endogen

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VI. WORKING DIAGNOSIS
1. Main diagnosis : Rhabdomyosarcoma (C49.9)
2. Comorbid :-
3. Complication : Vincristine-Induced Peripheral Neuropathy (G62.0)
4. Growth status :
- Cross sectional : Obese, normal stature (E66.9)
- Longitudinal : Flat of Growth
5. Developmental status : Normal developmental status and had procedural anxiety problem
6. Immunization status : Complete basic and booster immunization.
7. Socioeconomic status : Middle socioeconomic status

VII. PROBLEMS
1. Problems on diagnostic: PET scan
2. Problems on management: Side effects of chemotherapy, medication compliance
regarding the relatively long-term chemotherapy, chemotherapy regimen availability,
nutritional management, procedural anxiety management.
3. Problems on prevention: Prevention of delayed chemotherapy, side effect of
chemotherapy, infection, metabolic syndrome caused of obesity.
4. Problems on monitoring: Monitor treatment response, side effects of chemotherapy, sign
of recurrent mass, metastases, weight loss, quality of life, tolerance of nutritional
interventions.
5. Problems on prognosis: acceptance of the chemotherapy, drugs (chemotherapy)
resistance, compliance of chemotherapy, recurrent mass, metastases.
6. Problems on family psychology: The psychological burden of the family is related to the
current state of the patient and the prognosis.

VIII. MANAGEMENT PLAN

1. Emergency management: -
2. Diagnostic investigation: Total cholesterol, triglyceride, HDL cholesterol, LDL
cholesterol, uric acid, liver function test, renal function test.
3. Medical therapy: Chemotherapy with High-risk Rhabdomyosarcoma 4st week consists of
vincristine 1.5mg/m2, irinotecan 50 mg/m2 intravenously.
4. Non-medical therapy: nutritional management, physiotherapy.
5. Pediatric nutrition care:
Boy, 7 years- 11 months, body weight: 38 kg, ideal body weight: 26 kg, height: 125 cm.
Nutritional management with 5 steps of pediatric nutrition care (PNC):
1) Assessment: Obese, normal stature
2) Nutritional requirements:
- Current body weight 38 kg, ideal body weight 25 kg, height 125 cm, height age 7 year-
6 months
- Fluid requirement = Holiday Segar =1860 ml/day
- Calories requirement (RDA 80 kcal/kg/day) = (80 kcal x 25 kg) = 2000 kcal/day
- Proteins requirement (RDA 1 grams/kg/day) = 1grams x 25 kg = 25 grams/day
3) Route of administration: oral
4) Formula:
- 3x1 portion rice with modification diet (green food, fiber 12 gr/day), 250 ml skim milk,
2 times sliced fruit snack, mineral water between main course and snack with total
calories 1900 kcal/day (90% RDA), protein 63 grams/day (250% RDA), and fat 40
grams/day (160% RDA)
- Nutrition fulfillment percentage: Diet of 2000 kcal/day (carbohydrate 50%, protein 20%,
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 unsaturated fat 20%, saturated fat 10%)
6. Monitoring: acceptability, tolerance, affectivity (body weight)
7. Medical Care: Preventing infection with universal precautions, personal hygiene (oral
hygiene, hand hygiene, anal hygiene), providing information on patient’s illness and
conditions, positive pressure isolation room for immunocompromised patient
8. Monitoring plan: Monitoring on general conditions, vital signs, diet acceptability,
tolerance and affectivity, signs recurrent mass, metastases and medication adverse effects.
Evaluation of PedsQL for 3-6 months later.
9. Information and education plan: Providing information about the disease and diagnostic
procedures, prognosis, lifestyle changes to prevent carcinogenic agent and nutritional
problems, and side effects of chemotherapy.

IX. PROGNOSIS
Quo ad vitam : dubia ad malam
Quo ad sanam : dubia ad malam
Quo ad fungsionam : dubia ad malam

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X. FOLLOW UP

Nov 14th (D1) AD 2 Nov 15th (D2) AD 3 Nov 16th (D3) Nov 17th (D4) Nov 18 -20th (D5-7)
at Hematology Clinic
No fever, nausea, vomiting, or No fever, nausea (+), vomiting No fever, nausea, vomiting, or
cough. He had good appetite. 4x a day. There were no No fever, nausea, vomiting, or No fever, nausea, vomiting, or cough. He had good appetite.
There were no complaints of complaints of urination and cough. He had good appetite. cough. He had good appetite. There were no complaints of
urination and defecation. defecation. There were no complaints of There were no complaints of urination and defecation.
urination and defecation. urination and defecation.
Physical and laboratory Physical and laboratory Physical examination in normal
examination in normal limit examination in normal limit Physical examination in normal Physical examination in normal limit
limit limit BW: 37.8 kg

Assessment Assessment Assessment

Assessment Assessment
1. Rhabdomyosarcoma on 4th 1. Rhabdomyosarcoma post 4th 1. Rhabdomyosarcoma post 4th
1. Rhabdomyosarcoma post 4th 1. Rhabdomyosarcoma post 4th
week chemotherapy week chemotherapy week chemotherapy
week chemotherapy week chemotherapy
2. VIPN 2. VIPN 2. VIPN
2. VIPN 2. VIPN
3. Obese, normal stature 3. Obese, normal stature 3. Obese, normal stature
3. Obese, normal stature 3. Obese, normal stature

Therapy Therapy
IVFD D51/2 NS 10 ml/hour IVFD D51/2 NS 10 ml/hour
Monitoring of nutritional Programed for 5th week Monitoring of nutritional
acceptability High-risk Rhabdomyosarcoma acceptability
Intravenous: Intravenous: Chemotherapy
Vincristine 1.71 mg Ondansetron 6 mg
Irinotecan 57 mg Physiotherapy Physiotherapy
Physiotherapy
Ondansetron 6 mg Diet:
- 3x1 portion rice with
Diet: modification diet
- 3x1 portion rice with - 250 ml skim milk
modification diet - 2 times sliced fruit snack
- 250 ml skim milk per day - mineral water between main
- 2 times sliced fruit snack course and snack
- mineral water between main
course and snack Program:
- Outpatient care
- Preparing for 5th week
rhabdomyosarcoma
chemotherapy on November
21st 2022

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 XI. CASE ANALYSIS O
Short term P
Risk Factors
History of cancer in family
LoE3
- Chemotherapy effect T
• Neuropathy I
• Nausea M
Past medical history
History of mass at his upper right chest - Chemotherapy with High-risk • Vomiting A
History of rhabdomyosarcoma on Rhabdomyosarcoma 4th week • Weight loss L
chemotherapy consists of vincristine 1.5mg/m2 - Mass infiltration
History of numbness and pain after LoE1 Relapse Alveolar
LoE1 and irinotecan 50mg/m2 G
chemotherapy intravenously Long term R
Rhabdomyosarcoma
Vincristine Induced - Wide tumor excision and partial - Metastases O
Mass Biopsy sternotomy - 5 years event- free survival rates
Alveolar rhabdomyosarcoma on costae
LoE1 Peripheral Neuropathy W
- Symptomatic therapy (55%)
region to sternum. T
- Personal hygiene - Risk of relapse
H
Thoracal MSCT LoE1
Suggesting teratoma dd/ &
rhabdomyosarcoma; no bones
destruction, no paraaorta and - PedsQL at 3-6 months
paratracheal lymph nodes enlargement. Psychosocial support: - PSC17 and SDQ at 3 – 6 months D
- Cognitive Behavior Therapy E
PedsQL 3.0 Cancer Module LoE1 Problem with procedural LoE1 - Art therapy V
Procedural anxiety: 33 anxiety - Individual supportive E
L
LoE1 Quo ad vitam : dubia ad malam
O
Weight: 38 kg, height: 125 cm, Cross-sectional: Obese, Food rules
BMI: 24.3 kg/m2. WAZ >P97, LoE1
Increase activity Quo ad sanam : dubia ad malam P
normal stature. Quo ad fungsionam : dubia ad malam M
HAZ P25-P50, BMI for age is Behavior modification
>P97. Longitudinal: Flat of Growth Educate family to support food rules E
N
STIMULATION – LOVE – CARING – INTEGRATED PSYCHOSOCIAL SUPPORT T

Clinical Findings Diagnosis & complication Management Prognosis

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XII. CASE DISCUSSION
Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin, the most
common form of soft tissue sarcoma accounting for 4.5% of all cases of childhood cancer. It
is the third most common extracranial solid tumor of childhood after Wilms tumor and
neuroblastoma. The incidence was six cases per 1,000,000 population per year, which accounts
for about 250 new cases in children each year. There is a bimodal distribution for the age at
presentation with a peak between 2-6 years and then again between 10-18 years of age (LoE
1).1 An observational study included 41 cases children with rhabdomyosarcoma showed that
the ratio of male and female was 1.43:1, average age was 6.79±0.56 years (median age was 6
years) and primary sites were in the nasopharynx and ethmoid sinus, eyelid and orbit, as well
as ear and check, which accounted for 29.26% (12/41), 56.09% (23/41), 17.65% (6/41)
respectively (LoE 3a)2, similar in this patient was male, diagnosed for RMS in 6 years 9 month
old, and primary site of the tumor was in his trunk.
WHO classified the RMS to 4 types with 2 common type embryonal RMS and alveolar
RMS and 2 less common type: pleomorphic and spindle cell/sclerosing RMS. Although the
majority of RMS is embryonal type, the alveolar type (ARMS) is the most studied molecularly.
Genetically, 80% of ARMS cases have been associated with a chromosomal translocation
between PAX3/PAX7 and FOXO1 genes. Multiple studies have proposed the integration of
the fusion status for risk stratification for treatment protocols. Unfortunately, with the limited
facility, the chromosomal status of our patient is unknown. (LoE 1)3
RMS typically presents as an asymptomatic mass found by the patient or the parents of
younger children. Rhabdomyosarcoma often presents with a visible or palpable mass, or as a
result of compression or invasion of adjacent structures. The most common sites are head and
neck (which includes the orbit, non-parameningeal and parameningeal areas; 36%),
genitourinary tract (23%) and extremities (20%). Risk factors of RMS has not been clearly
known.4 The known risk factors of RMS can be divided to risk factors from parents such as:
young and advanced parental age, parental exposure of recreational drugs use, prenatal
diagnostic radiation and various occupation exposures and risk factors from the children: such
as high or low birth weight, less breastfeeding time, exposure to chemicals. (LoE 1)5 Many
literatures showed association about RMS and Recklinghausen disease, Li-Fraumeni
syndrome, Costello, Noonan or Beckwith-Wiedemann syndromes suggests the role of genetic
factors. (LoE 1)4 A case control study resulted that family history of cancer was associated with
greater risk of RMS (OR 2.37, 95% CI 1.34-4.18) (LoE 3b)6
RMS diagnosis should base on the precise physical investigation, past medical history,
radiological imaging, histology, laboratory and molecular tests. Every painless, especially
tender and fixed mass that has been present for several weeks or has grown very rapidly should
be suspected for malignancy. When malignancy is suspected, radiological evaluation
(ultrasound, MSCT, MRI, PET scan) and biopsy should be performed as quickly as possible.
(LoE 1)7 In this patient, there was not any complain as a result of compression or invasion,
children only complain about mass in his trunk, there was not family history of another
syndrome. However, there was family history of cancer. Radiological examination and
excision or biopsy revealed Alveolar Rhabdomyosarcoma (ARMS).
Staging of RMS is determined by the site and size of the primary tumor, degree of tumor
invasion, nodal status, and the presence or absence of metastases and is based solely on the
preoperative workup of imaging and physical examination. Patients are divided into low,
intermediate, and high risk (LoE 1)1. A report from the Children’s Oncology Group in 38
patients with ARMS revealed that patients with low-risk embryonal rhabdomyosarcoma
(ERMS) treated with Vincristine-Actinomycin-Cyclophosphamide (VAC) chemotherapy (5-
year survival, 85%); intermediate-risk Stage 2/3, Group III ARMS (5-years survival, 55%)
(LoE 1b)8. A phase III investigation randomized newly diagnosed intermediate-risk patients to
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VAC versus VAC plus vincristine-irinotecan (VI) resulted that the 4-year event-free survival
(EFS) was 63% with VAC and 59% with VAC/VI (p 0.51), and 4-year overall survival was
73% for VAC and 72% for VAC/VI (p 0.80) had comparable outcomes (4-year EFS 65% vs.
68%), but VAC/VI was associated with fewer hospitalizations and because the seven courses
of irinotecan may be poorly tolerated due to gastrointestinal toxicity, VAC chemotherapy alone
may be reasonable in certain patients (LoE 1b) 9. At first diagnosed in 2021, according pre-
treatment clinical staging, patient was classified Stage 2 (T2bN1M0) clinical group III,
intermediate risk then the patient was programmed for excision and biopsy revealed alveolar
Rhabdomyosarcoma. Patient programmed chemotherapy with VAC regimen (Vincristine,
Actinomycin D, Cyclophosphamide) for 24 weeks but the patient did not get Actinomycin D
regimen due to the unavailability. Three months after last low risk chemotherapy regiments,
patient complained recurrent mass in his trunk and the biopsy revealed alveolar
rhabdomyosarcoma and programmed to high-risk chemotherapy regiment.
Treatment of rhabdomyosarcoma advances over the past several decades have
significantly improved the long-term survival outcomes for patients with localized disease, an
estimated 25–30% of these patients still suffer relapses; for patients with metastatic disease,
relapse rates approach 70%. A multivariate study analyzed the factors identified at first relapse
that most strongly associated with poor outcome were metastatic relapse (odds ratio [OR], 4.19;
95% CI, 2.0- 8.5), prior radiotherapy treatment (OR, 3.64; 95% CI, 2.1-6.4), initial tumor size
≥5 cm (OR, 2.53; 95% CI, 1.5-4.1), and time of relapse below 18 months from diagnosis (OR,
2.20; 95% CI, 1.3-3.6). Unfavorable primary disease site (p=0.012), nodal
involvement at diagnosis (p=0.015), alveolar histology, and previous three or six drug
chemotherapy were also independently associated with poor outcome (LoE 1b)10. Patient had
poor outcome after relapse, the risk factors are relapse time 12 months from diagnosis,
unfavorable primary site, nodal involvement at diagnose, and alveolar histology.
The prognosis of the RMS specifically ARMS is poor. Despite aggressive therapy, 5-
year survival rate for relapse ARMS with metastasis is 30%. The advance in molecular biology
revealed in depth analysis of genetic background in ARMS. The existence of fusion of PAX3/7
– FOX01 genes in in combination with TP53 mutations and CDK4 amplification in the tumor
cells had been described as predictor for poor prognosis. Unfortunately, our patient has limited
genetic information in order to accurately predict the prognosis. (LoE 1)11
The dose-limiting side effect of vincristine (VCR) is neurotoxicity, which may lead to
severe peripheral sensory and motor neuropathies affecting quality of life (QOL), treatment
delay, and vincristine substitution or discontinuation and most symptoms improved within 4
weeks after the last dose of VCR. A recent meta-analysis of more than 4000 chemotherapy-
treated patients found the prevalence of VIPN to be 68.1% (95% CI = 57.7–78.4) within the
first month of the end of chemotherapy, 60.0% (36.4–81.6) at 3 months, and 30.0% (6.4–53.5)
at 6 months or later (LoE 1a)12. One clinical trial demonstrated that the proportions of patients
experiencing paresthesia, numbness, and pain in the 0.67 mg/week low-dose group were 43%,
34%, and 14%, respectively and in the dose 1.33 mg/week group were up to 60%, 70%, and
62%, respectively (LoE 1b)13. The patient complains joints pain, numbness and paresthesia
during chemotherapy. He got 1.7mg/week dose of vincristine for last 24 weeks, after 4 weeks
of last chemotherapy, the symptoms improved. In this high-risk chemotherapy regiment, he got
vincristine again and complained about joint numbness after chemotherapy. The patient got
pyridoxine 1 tablet per 8 hours for its VIPN.
The patient's nutritional status was obese (BMI >P97) and normal stature. Obesity
occurs due to imbalance between energy intake and expenditure, resulting in excess energy that
is stored in the form of fat tissue. (LoE 1)14 The prevalence of obesity for children and
adolescents aged 2–19 years in 2017–2020 was 19.7% and affected about 14.7 million children
and adolescents. There was no data on obesity prevalence in RMS patients. (LoE 1)15
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 The etiologies of obesity are idiopathic and endogen. Idiopathic obesity is the most
common (>90%), characterized with tall stature (height/age >P50), a family history of obesity
without mental retardation, a normal bone age, and physical examination. Endogenous obesity
(10%) is distinguished by short stature (height/age P50), no history of obesity in the family
with mental retardation, delayed bone age, and abnormal physical examination. (LoE 1)15 In
this case, the etiology was idiopathic. He also has bad eating habits and limited physical
activity.
Obesity-related comorbidities can be categorized into medical and psychosocial
components. The medical consequences result in metabolic effects (the cardiovascular,
endocrine, gastrointestinal, and renal systems) and mechanical effects (the pulmonary, skeletal,
and central nervous systems). The major medical comorbidities in childhood obesity are
metabolic syndrome, sleep disorders, dyslipidemia, and hypertension. Psychological issues i.e.,
anxiety and depression, along with eating disorders affect the quality of life in children with
obesity. (LoE 1)16
Neuropathy is a common neurological consequence in obesity. Obesity increased the
level of free fatty acid and induced inflammatory mediator which directly cause injury to dorsal
root ganglia neurons, all sensory nerve ending of the peripheral nervous system, and the blood-
nerve barrier. It leads to decrease neurotrophic support and increase neurodegeneration. These
conditions are aggravated by the other medical consequences lead to metabolic syndrome. In
this case, the patient also complained neuropathy symptoms and pain problems. (LoE 1)17
Management for obesity was including dietary management, increasing the physical
activity, behavior modification, pharmacotherapy, and surgery. Dietary management for
obesity based on food rules. Food schedules are 3 times a day and snacks 2 times a day
(especially fresh fruit), water between food schedules and snacks, eat duration is 30 minutes.
Calories requirements based on the result between recommended dietary allowances according
to height age and ideal weight according to height. The food rules include reducing 200-500
calories per day with weight loss target 0,5 kg per week. Final weight target is 20% above the
ideal body weight, in this case was 30 kg. Modified nutritional diets are carbohydrate 50-60%,
fat 30%, protein 15-20% and high fiber diet. The physical activity should be gradually for
increase with moderate to vigorous activity for at least 60 minutes each day. Behavior and sleep
modification such as screen time < 1-2 hours/day, routine sleep pattern, and 11-14 hours of
sleep should be implemented. Pharmacotherapy might be considered in some cases, including
sibutramine (reducing appetite), orlistat (decreasing nutrient absorption, and leptin
recombinant. Bariatric surgery is indicated in failed after 6 months of therapy, super obese
(BMI≥40), reach bone maturity age (female ≥13 years old, male ≥15 years old), and presence
of complications. (LoE 1)14
This patient nutritional requirements were calories 80 kcal/kg/day, protein 1
gram/kg/day. He was given modification diet with 3 x 1 portion of rice, 250 ml skim milk, 2 x
1 portion of fruits with total calories 1900 kcal/day (90% RDA), protein 63 g/day (250% RDA)
and fat 40 g/day (160% RDA). His balanced diets are 50-60% carbohydrates, 15-20% protein,
and 20-30% fat. During the treatment, we monitored diet acceptability, feeding intolerance,
and body weight. Sedentary time should be reduced as possible, maximum screen time was 2
hours per day, minimizing time spent on gadgets. Other obesity managements include changing
eating habits by reducing sugary drinks and foods, fast food, and having an adequate fiber diet.
(LoE 1)14
There are three types of preventions for obesity. Primary prevention consists of food
rules, optimal physical activity, exclusive breastfeeding until 6 months and continue breastfed
until 12 months. Secondary prevention includes early adiposity rebound which should be
happens at 5-6 years old and comorbidities evaluation in tertiary prevention. (LoE 1)14
Obesity is a severe pathologic condition which causes both morphological and
11
functional disorders. It associated with a high morbidity and mortality. Childhood obesity has
also been found to affect school performance negatively. A research study concluded that
overweight and obese children were four times more likely to have bad academical
performance than normal weight peers. (LoE 1)16
Assessment of quality of life in childhood cancer is a special challenge for clinicians
and researchers because developmentally influenced cognitive ability and illness levels are so
varied. Quality of life for children and adolescent cancer patients is a personal, subjective
experience, influenced by the environment, and is most accurately assessed individually with
each patient serving as his own standard.18 A better understanding of these children and their
families while coping with this specific stressor could be valuable to healthcare professionals. 19
The psychological impairments of childhood cancer vary with age. It is known that some
children with cancer may develop problems with mood or anxiety because of the need for
frequent treatments and procedures (e.g. chemotherapy).18 It is known that when children get a
diagnosis of cancer, both parents/proxy respondents and medical staff might hide the
information, especially from younger patients. If the child/adolescent does not know the reason
for frequent hospital visits and medical treatment, anxiety levels might elevate.
Parents/caregiver’s self-efficacy might also influence child distress and cooperation during
procedures.20 This patient got anxious due to frequent treatments during his chemotherapy
programs. Although he can cope with his own anxiety so far, he still needs support to help him
against this condition.
Children and their caregivers can adjust themselves after the diagnosis of cancer to
promote psychosocial and emotional adjustment and reduce their distress to help them all cope
with the disease.21 The Standards of Psychosocial Care for Children with Cancer and their
Family recommend that “youth with cancer and their family members should have access to
psychosocial support and interventions throughout the cancer trajectory and access to
psychiatry as needed”. (LoE 1)22 These include psychoeducation, supportive individual
therapy, cognitive behavioral therapy, problem-solving therapy, social support groups, social
skills training, health promotion, bibliotherapy, art therapy, and mindfulness-based therapies.23
Verbal therapy or expressing one’s self through words can help pediatric cancer patients and
survivors find closure and foster personal growth. By approaching the illness experience
through a narrative lens, these verbal therapies encourage patients to share their deepest
thoughts and feelings about cancer and its broad impact on their lives. (LoE 1)24 This patient
has singing and storytelling skills using English. These skills can be used as verbal therapy for
him. He can share his thought during experiencing chemotherapy with his parents or friends,
so he can reduce his stress, cope with the disease, and get emotional support from his
surroundings.

12
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XIII. APPENDIX
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Figure 2. Growth Chart

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Figure 3. BMI Growth Chart

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Figure 4. Chest Rontgen September 22th 2021

Figure 5. Chest Rontgen August 31st 2022

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Figure 6. Thoracal MSCT with Contrast August 2nd 2021

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