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Retno Ayu Adhisy, MD
Consultant:
Susanto Nugroho, MD Pediatrician Consultant
A RARE CASE OF DESMOPLASTIC SMALL
ROUND CELL TUMOR IN A 6-YEAR-OLD BOY
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ABBREVIATIONS
DSRCT : Desmoplastic Small Round Cell Tumor
CT scan : Computerised Tomography
FNAB : Fine Needle Aspiration Biopsy
PNET : Primary Neuroectodermal Tumor
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INTRODUCTION
DSRCT is a rare and distinct small round cell tumor ofchildhood
It has a marked predilection for young adult males and usually
presents with diffuse abdominal or pelvic peritoneal tumor
masses with minimal organ involvement
Despite intensive multimodal therapy, the prognosis is still
poor and there is no general agreement for the treatment of
this malign diseaseChang F, 2006
Akhtar M, Iqbal A, Mourad W, Ali A, 1999
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INTRODUCTION...
The purpose of this case report is to present a
rare case of DSRCT in a 6 year old boy
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CASE REPORT
5
N, 6-year-old boy Emergency Room Saiful Anwar GeneralHospital
Complaints :
Pallor
High fever
A mass in his forehead
Headaches
Decreasing body weight
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CASE REPORT...Physical examinations :
Pale conjunctiva
Strabismus
A mass in the frontal region
with approximately 6 cm,hard consistency, firm, no
pain and no signs of
inflamation
Hepatospleenomegali
Undernourished
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SGPT : 55 U/L
SGOT : 48 U/L
Na : 127 mmol/l
K : 4.46 mmol/l
Cl : 95 mmol/l
SI : 67
TIBC :184
Hb : 7.6 g/dl
Lekosit : 4000/mm3
Tromb. : 111.000/mm3
Hct : 22.7 %
Albumin : 3.12 g/dl
CRP : 27.36 mg/dL
Initial laboratory findings
PH : 7.537
PCO2 : 22.4
PO2 : 112.9
HCO3 : 23.1
O2sat : 98.6
BE :1.7
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CASE REPORT...
Lateral skull X-ray revealed a soft tissue mass in the frontal regio
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CASE REPORT...
Initial diagnosis :
Soft tissue tumor : Rhabdomyosarcoma DD
Fibrosarcoma
Liposarcoma
Hypochromic Microcytic anemia due to chronic infection
Undernourished
WDx : Head CT scan, FNAB
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CASE REPORT...
Head CT scan found a suspected multiple malignant bone primary
tumor, both sides of the mid line extending to intracranial on the rightfrontal lobe accompanied by proptosis of right bulbus occuli andcerebral edema
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CASE REPORT...
FNAB I : Small cell with dense distribution, round nucleus,
uniform, hyperchromatic, thin cytoplasm and rosette like cells.
Malignant small round cell tumor caused by blastoma
differential diagnosed with embryonic rhabdomyosarcoma or
PNET
Day 13
FNAB II : Dense fibrotic tissue with group of cohesive smallround malignant cell, hyperchromatic nucleus
Desmoplastic Small Round cell Tumor
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CASE REPORT...
Diagnosis
Desmoplastic Small Round Cell Tumor Day 14Chemotherapy:
Vincristin 1 mg/m2iv day 1 and 5
Cyclophosphamide 500 mg/m
2
iv day 1Doxorubicyn 40 mg/m2/day at day 15
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CASE REPORT...
Day 20 :
Profuse vomiting
GCS 345
Complete blood count : Hb 7.8 g/dl, hematocrites 22.1%, total
leucocyte count 1600 /ul, platelet count 38.000 /ul and absolute
neutrofil count (ANC) was 800
Electrolite serum level : Na : 116 meq/l, potassium 3.65 meq/l,
chloride 93 mol/l, calsium 8 mg/dL
Diagnosis :
Profuse vomiting some dehydration and decreased conciousness
due to a metastatic process or encephalopathy
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CASE REPORT...
Planned treatment :
Rehydration
Correct the sodium level
Ondansetron 3x4 mg
Consult The Ophtalmology DepartmentPapil edema
Consult The Pediatric Neurology Department :
- Head CT scan
- Dexamethasone 3x2 mg
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CASE REPORT...
Day 30The patient health had improved.
The patient was discharged and the patients advise to
come for routine check-ups at the outpatient clinic for
chemotherapy
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DISCUSSION
Lumps and bumps on the head are common complaints inchildren
Differential diagnosis is broad and radiological evaluation is
often requested in a wide spectrum of congenital lesions and
acquired lesions
In this case, patient complaining of mass in his forehead, a
skull X-ray and head CT scan was performed to confirmthe diagnosis
Morron F, Morris M, Jones J, Hunter J, 2011
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DISCUSSION...
The skull X-ray found soft tissue tumor Initial diagnosis :
Soft tissue tumor : Rhabdomyosarcoma DD
FibrosarcomaLiposarcoma
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DISCUSSION...
Rhabdomyosarcoma The most common soft tissue tumor in children
Often cause a noticeable lump on childsbody
Frequently found in children under the age of 15 in thehead and neck region
In this case :
The skull X-ray indicating soft tissue tumor
There was a lump or mass in the forehead
The patient was a 6-year-old boy
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DISCUSSION...
Liposarcoma : Account for 5% all soft tissue sarcoma in childhood
The major site was lower extremities, retroperitoneal and
shoulder
Rarely occured in children less than 8 year old
Fibrosarcoma : Infantile formin children < 1 year old
Most common in arm and legs
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DISCUSSION...
The Head CT scan found a suspected malignant primarytumor of the bone, multiple at frontal bone extending to
intracranial on the right frontal lobe
FNAB I : Malignant small round cell tumor caused by blastoma
differential diagnosed with embryonic rhabdomyosarcoma orPNET
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DISCUSSION...
Malignant round cell tumors (MRCT) in children are a group of
neoplasms characterized by small, round, relativelyundifferentiated cells. Because of these features,
morphological diagnosis may be difficult
Several studies have demonstrated that the diagnosis of
MRCT can be made effectively by FNAB
In this case, the diagnosis was confirmed using FNAB result
Cohn S, 1999
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DISCUSSION...
DSRCT is a highly aggressive tumor that predominantly
affects young males
It tend to spread along the peritoneum and mesothelial-lined
Other body sites including paratesticular region, pleural
serosa, posterior cranial fossa, soft tissues and bone, ovary,parotid gland, and lungs
In this case, the patient was a 6-year-old boy with a solid
mass in the frontal region, the second FNAB result found a
DSRCT
Mihok N, Cha I, 2001
Saab R, Khouri JD, Davidoff AM, Navid F, 2007
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DISCUSSION...
DSRCT arranged as thin trabeculae, small to medium-sized, round to oval hyperchromatic nuclei and rosette likestructures. arranged in sheets and clusters surrounded bydense collagenous stroma
In this case :
First FNAB Relatively small cells with dense distribution,round nucleus, uniform, hyperchromatic, thin cytoplasm and
rosette like cell Second FNAB Dense fibrotic tissue with groups of
cohesive malignant cells, small round hyperchromatic nucleus
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DISCUSSION...
Anamnesis
Physical
Examination
Skull X-ray
CT scan
FNAB
Small round cell carcinoma
Ewings sarcoma/ PNET
Embryonic rhabdomyosarcoma
Desmoplastic Small
Round Cell Tumor
Histologicaly, DSRCT must be distinguished from other small round cell
tumors
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DISCUSSION...
Occurs primarily in the bone orsoft tissue.
Often found in the long bonesand can involve soft tissuearround the tumor.
2-3% childhood cancers
The second most common
malignant bone tumor inchildren
Age 10-20
Pain arround the site of tumor
EWINGS SARCOMA
In this case :
Head CT showing a primarily
bone tumor
Found at the frontal bone
6-year-old boy
No pain
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DISCUSSION...
The most common type ofrhabdomyosarcoma
frequently found in children
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