A RARE CASE OF DESMOPLASTIC SMALL ROUND CELL.pptx

8/12/2019 A RARE CASE OF DESMOPLASTIC SMALL ROUND CELL.pptx

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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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A RARE CASE OF DESMOPLASTIC SMALL ROUND CELL.pptx