Management of patient with right upper quadrant pain Desmoplastic small round cell tumor (DSRCT)

1. M. Kamil
DP
Management of patient with right upper quadrant pain

2. Case
◦ Identifying information: Patient is 18-year-old Malay male, single.
◦ Chief complaint: “Abdominal pain for the past 1 month, nausea and vomiting for 2 weeks and
fever for 1 day”
◦ History of Presenting illness:
◦ Patient with underlying chronic gastritis, present with stomach pain for the past 1 month,
vomiting for 2 weeks and fever for 1 day. The symptom started when he is lying flat at rest and
suddenly develop abdominal pain. The abdominal pain is at the right upper quadrant and
getting worst and happen every day for the past 1 month. The pain is colicky in nature and worst
when he lying flat, and after eating and disturb his sleeping which he complaint that he did not
get enough sleep during the course of the pain. The pain radiates to his left shoulder especially
when he takes a deep breath and sometimes to the back. After 2 weeks, the pain is associated
with jaundice, nausea and vomiting. He also need to bend forward to walk during the pain
episode. He rated the pain as 8 to 9 during the attack on the pain scale of 0 to 10. At rest he
rated the pain as 4/10 in the pain scale.

3. Case: continue
◦ The jaundice develops for about 3 days when he has severe pain right before he was admitted in
the medicine ward. His skin color turns yellowish. But he has normal color stools, no steatorrhea,
no foul smelling stools, and normal color urine.
◦ The vomiting started after 3 weeks of abdominal pain. he usually vomits every time he eat and
drinks and become worst especially when he eats spicy foods. He vomits food particles and
there is no hematemesis.
◦ He has no history of recent travel, jungle trekking and swimming.
◦ This is the first episode that he experienced in his life.
◦ No history of previous jaundice
◦ No history of blood transfusion
◦ No history of sexual contact
◦ No tattoo

4. Case: Past medical history
◦ Childhood illness: Asthma
◦ Adulthood illness: Gastritis
◦ Hospitalization: Once. Last 2 weeks. Suspected gall bladder swelling. Admitted in the Kajang
Hospital for 1 day
◦ Allergy: Sea food. Reaction: develop itchiness and rash whole body usually for 2 days then
subsides
◦ Vaccination: Full
◦ Surgery: No previous history of surgery
◦ Trauma: No recent trauma

5. Case: Home medication
Medication Dose taken Duration
T. omeprazole 20mg 40mg OD
T. mefenamic acid 250mg 500mg PRN (pro re nata)
◦ Complementary/ Alternative medicine: SNE capsule for gastritis only for 3 days last 3 weeks,
then he stops taking the capsule.

6. Case: Significant family history
◦ Maternal Grandfather: Deceased at age 83 due to tumor in the ear.
◦ Mother: Diabetes mellitus, hypertension, hyperlipidemia. Undergo hysterectomy at age 48-year-
old due to suspected tumor.
◦ His mother’s siblings:
◦ One has breast cancer and was diagnosed at age 60-year-old. Patient unsure about her age.
◦ One died due to colon cancer at age 60+ year old and was diagnosed at age 60-year-old.

7. Case: Significant social history
◦ Patient live in HLS with his family.
◦ He is single and not married.
◦ 3 pack years of smoking history. Since 17 year old.
◦ He does not drink alcohol and does not take any illicit drug.
◦ He eats less vegetable because he does not like vegetable.
◦ He works as the customer service at UTC P since 1 month ago and does not perform regular
exercise since then.

8. Case: Review of systems
◦ Constitutional: Lethargic, loss of appetite, weight loss: 10 kg for 3 weeks since he has abdominal
pain and vomiting. Occasionally insomnia due to pain. BMI: 21.3 (normal). No night sweat. Diet
changes: he could not eat spicy food since the onset of symptoms because he will develop
abdominal pain and vomiting.
◦ HEENT, respiratory, CVS, GU, peripheral vascular: Not significant
◦ GI: As per HPI

9. Vital signs: Normal
On admission Day 4
Blood Pressure 122/74 mmHg 125/72 mmHg
Heart rate 80 bpm 72 bpm
Respiratory rate 19/ min 20 / min
SpO2 98% 100%
FSG 5.3 mmol/L

10. PHYSICAL EXAM (Gastrointestinal system)
- Physical examination was done on day 4 hospitalization.
◦ General inspection: Normal build. Patient was not in respiratory distress.
◦ HANDS:
◦ Inspection of both of the hands: There was no cyanosis, slightly pallor, no jaundice, no palmar erythema
(CLD), no spider nevi, no telangiectasia. No tattoo. No scratch marks. No leukonychia, no koilonychia, no
needle track. No tobacco staining, no deputyrene contracture.
◦ Palpation: Both hands were warm, capillary refill < 2 seconds, no sweat, and normal skin turgor, no
clubbing (IBD, malabsorption, cirrhosis, GI lymphoma), no asterixis (hepatic encephalopathy).
◦ ARMS
◦ Branchial pulse: Good volume
◦ No tattoo, no telangiectasia, no spider nevi

11. PHYSICAL EXAM (Gastrointestinal system)
- Physical examination was done on day 4 hospitalization.
◦ EYE:
◦ Sclera: White
◦ Conjunctiva: Slight pallor
◦ NECK
◦ Lymph nodes were not palpable. No tenderness. No bilateral parotid swelling.
◦ MOUTH
◦ No central cyanosis
◦ Good dentition
◦ No angular stomatitis
◦ No mouth ulcer

12. PHYSICAL EXAM (Gastrointestinal system)
- Physical examination was done on day 4 hospitalization.
◦ ABDOMEN
◦ Inspection: Abdomen was flat, flank was not full, umbilicus was inverted, no scar, no caput medusa, no
obvious mass, and normal movement with respiration. The hyper pigmented skin was appreciated at the
lower quadrant bilaterally due to fungal infection (on topical fungal medication: Miconazole cream).
◦ Palpation:
◦ Guarding.
◦ Tenderness on the RUQ
◦ The mass was appreciated at right upper quadrant and extend to the half of right lower quadrant. The mass was liver.
◦ The spleen was not able to be appreciated by palpation.
◦ Ballot kidney: Kidneys were not able to be appreciated
◦ No ankle edema.

13. PHYSICAL EXAM (Gastrointestinal system)
- Physical examination was done on day 4 hospitalization.
◦ ABDOMEN
◦ Percussion:
◦ Liver span: 20 cm.
◦ Urinary bladder: Not full.
◦ Others:
◦ No pyoderma gangrenosum, no lower limb edema, no shifting dullness. No
sacral edema.
◦ Auscultation:
◦ Bowel sound was present, no renal and no iliac bruits.

14. Problem lists
◦ Underlying chronic gastritis
◦ RUQ pain for the past 1 month
◦ Nausea and vomiting for 2 weeks
◦ Fever for 1 day
◦ History of jaundice prior to admission
◦ Pain was associated with jaundice, nausea and vomiting
◦ Weight loss 10 kg over 3 weeks
◦ Loss of appetite
◦ Lethargy
◦ RUQ pain with guarding
◦ Hepatomegaly

15. Ddx
◦ Liver
◦ Primary liver cancer
◦ Liver metastasis – From chronic gastritis
◦ Hepatitis – HBV, HCV, HIV
◦ Hepatic abscess – Bacterial
◦ Gall bladder
◦ Cholecystitis

16. Investigations
◦ CBC
◦ RP
◦ LFT – Liver enzymes
◦ Ca, Mg, PO4
◦ Blood culture and sensitivity
◦ Viral markers –(HBV, HCV, HIV)
◦ Serum amylase
◦ Tumor markers:
◦ AFP
◦ CA19-9
◦ CEA

17. CBC
Date 31/12/15 01/01/16
Normal Values
On admission
WBC 12.1↑ 11.8 ↑ 4.00-10.00 109/L
HGB 13.3 12.3 13.0-17.0 g/dL
Platelets 371 336 150.0-410.0 109/L
Hct 39 37 0.400-0.500 L/L
MCV 83 83 83.0-101.0 fL

18. Liver function test
LFT
31/12/15 01/01/16 Normal values
Total Protein 78 75 60-83 g/L
Albumin 38↓ 35↓ 40-49 g/L
Total bilirubin 11 13 2-17 µmol/L
ALP (Alk. Phos) 145↑ 126↑ 5-119 U/L
ALT (Ala Ami.T) 92↑ 82↑ 5-41 U/L
AST(Asp Ami.T) 71↑ 10-40 U/L

19. Investigations
31/12/15 01/01/16 05/01/16 06/01/16 Normal value
Amylase 528 628 620 117 28-100
Tumor markers
Normal values
AFP 2.14 0-6
CA19-9 4.5 0-37
CEA 0.8 <5.0
PSA 0.47 <4.0

20. Investigations
◦ Renal profile – Normal
◦ Ca, Mg, PO4 - Normal
◦ Blood culture and sensitivity: Negative
◦ Viral markers for HBV, HCV, HIV: Non reactive
◦ Tumor markers: Negatives

21. Imaging
◦ CHEST X-RAY: was taken on the day of admission: Normal CXR
◦ ULTRASOUND OF HEPATOBILIARY SYSTEM
◦ Was taken on day 2 admission
◦ Impression: Multiple echogenic liver lesions. These are likely to suggest metastases. Need to determine primary lesion. Less likely diagnosis is
liver abscess. Suggest correlation with alpha fetoprotein and biohazard screening.
◦ CT- THORAX, ABDOMEN AND PELVIS
◦ Was taken on day 7 admission
◦ Impression:
◦ Duodenal mass with involvement of the pancreatic head and enlarged paraaortic, mesenteric and inguinal lymphadenopathy causing biliary
obstruction.
◦ Multiple diffuse liver lesions suggestive of liver metastasis.
◦ The duodenal mass could be GIST, duodenal carcinoma and lymphoma.
◦ Another differential includes pancreatic carcinoma however it is less likely.
◦ Splenomegaly.
◦ Bilateral pleural effusions.
◦ The solitary nodule in the right middle lobe is nonspecific, most likely infective in origin.

22. CT- THORAX, ABDOMEN AND PELVIS
Veerabadaran P, Gnanaprakasam, Jamila A. Case report: Rare intra-abdominal tumor in a young male
Multiple diffuse liver lesions
suggestive of liver metastasis.

23. ESOPHAGOGASTRODUODENOSCOPY (OGDS)
◦ ESOPHAGOGASTRODUODENOSCOPY (OGDS) was done
on day 11 hospitalization
◦ Findings: Duodenal ulcer
◦ Site: D1/D2; D2
◦ Size: 2-5 cm
◦ Appearance: Malignant
◦ Stigmata of recent hemorrhage: IIc Hematin-covered lesion
◦ Biopsy was performed for histopathologic examination
(HPE)

24. HPE result ◦ Microscopic:
◦ Section shows multiple fragments of small
intestinal mucosa which is ulcerated at areas.
The lamina propria is infiltrated by malignant
epithelial cells in clusters and sheets displaying
moderately sized round and pleomorphic
hyperchromatic nuclei with scanty amount of
eosinophilic cytoplasm. These tumor cells are
surrounded by desmoplastic stroma. Mitoses are
occasionally seen. The surrounding stroma is
infiltrated by neutrophils, lymphocytes and
plasma cells.
◦ Immunohistochemistry:
◦ These tumor cells are positive to Pan-CK, CK7,
MIC2, Desmin (dot-like positivity) and WT1. They
are negative to CK20, CD34, CD117, SMA,
Chromogranin, Synaptophysin, SMA, Fli-1 and
LCA.
◦ Diagnosis:
◦ Ulcer edge biopsy: Desmoplastic small round cell
tumor.
Veerabadaran P, Gnanaprakasam, Jamila A. Case report: Rare intra-abdominal tumor in a young male

25. Desmoplastic small round cell tumor (DSRCT)
◦ Soft tissue sarcoma. Tumor of gastrointestinal tract.
◦ An uncommon tumor that most typically presents with multifocal
abdominal masses.
◦ 90% in male. Which mean age 15 to 35.
◦ Aggressive tumor occurs in children and young adults.
◦ Pathogenesis: Chromosomal translocations
◦ Reciprocal chromosomal translocation, t(11;22)(p13;q12)
◦ that results in fusion of genes associated with Ewing sarcoma (EWS) and Wilms
tumor (WT1).
◦ Morphologically, the tumor is resemblance to Ewing sarcoma and related tumors.

26. Selected translocations associated with
sarcomas

27. Treatment
◦ Initial treatment on admission in the ward:
IV tramadol 50mg
IV maxalon 10mg
Miconazole cream
IV rocephin 2g (ceftriaxone)
IV hydrocortisone

28. Treatment for DSRCT
◦ Until 2011, less than 200 cases are reported in the world literature. Because of the rarity of this
disease, little is known about optimal treatment.
◦ Surgical excision is only recommended for non-metastatic disease with combination chemo-
radiotherapy as an adjunct.
◦ Chemotherapy, radiotherapy, and surgical approaches have not been standardized.
◦ Mainstay: Surgery along with chemotherapy.
◦ Tumors may remain surgically un-resectable.
◦ For advanced disease, symptom control is most important as these modalities impact survival
minimally and palliation of secondary symptoms.

29. One study
Patient were treated with high dose alkylator based regimen labeled as P6 Protocol.
10 patients previously untreated and 2 patients previously treated were assessed.
7 out of 10 untreated patients had partial response or very good response to the protocol which
included HD-CAV (high dose Cycloposphamide, Doxorubicin and Vincristine).
2 patients had no assessable disease after tumor resection at diagnosis.
After chemotherapy and surgery,
7 patients were in complete remission (5 of it remained event free)
2 were in partial remission.

30. Treating the complication
◦ One of the complication is gastric outlet obstruction.
◦ Thus, requires a multidisciplinary approach from various departments

31. Prognosis:
◦ Remains poor.
◦ Despite aggressive therapy: overall survival:-
◦ 3-year overall survival has been estimated at 44% and the
◦ 5-year survival rate remains around 15%.

32. References
◦ Arora VC, Price AP, Fleming S, et al. Characteristic imaging features of desmoplastic small round cell tumour. Pediatr Radiol 2013; 43:93.
◦ Robbins, S., Kumar, V., & Cotran, R. (2010). Robbins and Cotran pathologic basis of disease. (8th ed. / Vinay Kumar ... [et al.] ; with illustrations by James A. Perkins. ed.).
Philadelphia, Pa.: Saunders/Elsevier.
◦ 1) Lae ME, Roche PC, Jin L, Lloyd RV, Nascimento AG. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors. Am
J Surg Pathol. 2002 Jul;26(7):823-35.
◦ 2) Stuart-Buttle CE, Smart CJ, Pritchard S, Martin D, Welch IM. Desmoplastic small round cell tumour: a review of literature and treatment options. Surg Oncol. 2008
Aug;17(2):107-12.
◦ 3) Lee YS, Hsiao CH. Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients. J Formos Med Assoc. 2007
Oct;106(10):854-60.
◦ 4) Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F. Desmoplastic small round cell tumor in childhood: the St. Jude Children's Research Hospital experience. Pediatr Blood
Cancer. 2007 Sep;49(3):274-9.
◦ 5) Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005 Jan;
40(1):251-5.
◦ Hayes-Jordan, A., & Anderson, P. M. (2011). The diagnosis and management of desmoplastic small round cell tumor: a review. Current Opinion in Oncology, 23(4). Retrieved
from http://journals.lww.com/co-oncology/Fulltext/2011/07000/The_diagnosis_and_management_of_desmoplastic_small.13.aspx

33. THANK YOU
