rare case

1. Malignant GIST of Duodenum
A Case report
By
Dr E Aravind

2. Back ground
• Primary malignant tumors of the duodenum
represent 0.3% of all gastro-intestinal tract
tumors.
• Upto 50% of these tumors are malignant
• Primary malignant tumors of the duodenum
must be differentiated from malignant tumors
of the ampulla, pancreas and common bile
duct

3. • The most frequent tumor of the duodenum is
adenocarcinoma .
• Other primary tumors are lymphomas,
leiomyosarcomas, carcinoid tumors,
gastrinomas, stromal tumors
• The tumor can be located in any part of the
duodenum but the most frequent location is
the second part.

4. • Gastrointestinal stromal tumors (GISTs)
represent the most common tumor of
mesenchymal origin arising in the
gastrointestinal tract
• Gastrointestinal stromal tumors (GISTs) arising
in the duodenum represent a rare entity.
• Owing to the complex anatomy of the
duodeno-pancreatic region, these tumors are
often challenging in diagnosis

5. Pathologic features
• GISTs in the duodenum do not differ from other
GISTs in histopathologically and in
immunohistochemical reaction.
• Most of them express CD-117 (c-kit) and CD-34
• The mitotic count has been found to be lower
in duodenal GISTs, with a median count <5/50
HPF

6. Case Report
• Name - Narayanamurtynaidu
• Age – 52
• Sex – Male
• Occupation - Farmer
• Address - Ramchandrapuram

7. • History
– Pain Abdomen Rt side of abdomen since 4 months
severe twisting type associated with high grade
fever which subsided on medication.
– Malena since 1 month
– No h/o jaundice
– No h/o similar complaint in past
– No h/o Major surgeries in past
– No h/o endoscopic procedures
– Known smoker
– Known Diabetic, Hypertensive

8. • General Examination
– Patient is consious coherent and co operative
– Patient is anemic
– No Icterus/ Clubbing/ Cyanosis/ Odema/
Generalised Lymphadenopathy
– Pt is well hydrated
– Well built and Well Nourished
– BMI- 28.4
– Kornofsky Score - 90

9. • Examination of Abdomen
• Inspection
– Abdomen flat
– Flanks normal
– Umbilicus midline normal
– No Scars
– No visible lumps
– No Visible Peristalsis
– No Engorged veins
– Hernial Sites normal
– Scrotum normal
– Lt Supraclavicular fossa empty
– Renal angles normal
– Spine normal

10. • Palpation
– Abdomen soft
– No Guarding or Rigidity
– No Palpable lumps
– No Hepatomegaly
– No Spleenomegaly
– Both testis in scrotum Lt Supraclavicular fossa
empty

11. • Percussion
– Upper border of liver at 6th intercostal space in
midclavicular line
– No free fluid
• Ausculation
– Normal Bowel Sounds heard
• Per Rectal Examination – NAD
• Other systems - NAD

12. • Provisional Diagnosis
• Liver abscess

13. Investigations
• Ultrasound Abdomen
– Multiple Liver Abcess
– Well defined hypoechoic lesion in arotocaval
region with communiction with adjacent bowel - ?
Bowel mass

14. • CECT Abdomen
– Exophytic soft tissue density lesion arising from
antero-lateral wall of 2nd part of duodenum - ?
GIST
– Non enhancing lesions in liver – Abscess
– Minimal B/L pleural Effusion

15. • UGIE
– Small hiatus hernia
– Severe diffuse gastrits
– Ulcerated growth in 2nd part of duodeneum
• ? Periampullary Carcinoma
• ? GIST
– Biopsy
• Well Differentiated Adenocarcinoma

16. Other Investigation
• Hb % - 7.7%
• RBS – 85mg/dl
• Bl Urea – 25mg/dl
• Sr Creatine – 0.5mg/dl
• ECG
• Xray Chest
• HIV - NR
• HbsAg - NR
• BGT – B+ve

17. • LFT’s
– Bilurubin – 0.8 mg/dl
– SGOT – 42 IU/L
– SGPT – 49 IU/L
– ALP – 125 IU/L
– Total proteins – 8.3 mg/dl
• Albumin – 4.5 mg/dl
• Globulin – 3.8 mg/dl

18. • Sr Electrolytes
– Na+ - 140 mmol/L
– K+ - 4.6 mmol/L
– CL- - 106 mmol/L
• Coagulation profile
– PT – 17.2 sec
– APTT – 31.8 sec
– INR – 0.98
• 2D Echo
• PFT

19. • Provisional Diagnosis
– ? GIST of 2nd part of Duodeneum
• Plan
– 2 points Blood transfusion preoperative to correct
anemia
– Reserve 4 points of cross matched blood for
surgery
– Surgery – Whiples procedure
• Operative Findings
– A 6 X 5 cms mass in 2nd part of Duodenum

23. • Post operative period
– Un eventfull
– Sutures Removed on POD – 10

24. Post operative biopsy of specimen
• Gross Appearance
– Received 23 cms long intestinal segment with
serosa showing 6X6X3.5 cm elanated nodular dark
brown to grey brown to to grey white mass
– C/S of intestine show loss of mucosal folds
corresponding to growth remaining normal
– Received omentum of 45X10X2 cms

25. • Microscopic Appearance
– Sections studied from 6X6X3.5 cms grey white firm
tumour of small intestine show the features of
“Malignant Stromal Tumour of Small Intestine”
possibly GIST “Gastrointestinal Stromal Tumour /
Leiomyosarcoma”
– Tumour is infiltrating the mucosa and into serosa, the
overlying mucosa show non specific inflamation with
focal ulceration.
– Both resected margins are free from tumour
infiltration
– Omentum - Nil particular

27. Follow up
• Case was referred to Department of
Radiotherapy, GGH, KKD for further
management
• They referred the case to higher centre for
chemotherapy

28. Discussion
• Gastrointestinal endoscopy remains the most
common diagnostic procedure in duodenal GISTs,
especially in patients with intramural growth or
mucosa ulceration and bleeding
• It allows forceps biopsy
• Endoscopic ultrasound (EUS) has been found to
be very helpful for esophago-gastro-duodenal
GISTs, with high sensitivity and specificity rates
• EUS-guided FNA cytology with
immunocytochemical evaluation(CD117 & CD34)
can diagnose GIST

29. • The great majority of duodenal mesenchymal
tumors are GISTs, which have a spectrum from
small indolent tumors to overt sarcomas. LMs
and LMSs are rare
• Metastases were in the abdominal cavity, liver,
and rarely in bones and lungs but never in
lymph nodes

30. • Treatment is complete surgical resection with
clear margins (R0 resection)
• There is no consensus on the optimal surgical
treatment for GISTs arising from the duodenum.
Operations which vary from tumour enucleation
(for extramural GISTs) to
pancreaticoduodenectomy for infiltrating or
larger tumors
• Limited resections (LR) can be performed in small
tumors not infiltrating the surrounding
structures, and when the papilla of Vater can be
preserved
• Should be done when R0 resection can be
possible

31. • Imatinib mesylate, a tyrosine kinase inhibitor,
plays a key role in the management of GISTs.
Its use in neoadjuvant therapy, adjuvant
therapy and in tumor recurrence
• In neoadjuvant setting for GISTs located in the
second portion of the duodenum, it can
beused for tumor downstaging in order to
perform a less extensive surgery with free
resection margins
• This requires precise preoperative diagnosis of
GIST which is not always easy to obtain

32. • The major limitation of Imatinib is the
development of tumor resistance, which is
related to the acquisition of additional c-kit
mutations
• Recently used drugs like receptor tyrosine
kinase inhibitor STI-571 used as effective
therapy for GISTs

33. References
1. Pierre-Louis Fagniez and Nelly Rotman Malignant tumors of the
duodenum, Surgical Treatment: Evidence-Based and Problem-
Oriented ,Service de Chirurgie Digestive, Hopital Henri-Mondor,
Créteil, France 2001
2. G. Cavallaro a, A. Polistena b, G. D’Ermo b, G. Pedullà b, G. De
Tomab, Duodenal gastrointestinal stromal tumors: Review on
clinical and surgical aspects, International Journal of Surgery 10
(2012) 463e465
3. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ,
et al. Diagnosisof gastrointestinal stromal tumors: a consensus
aproach. Hum Pathol 2002;33:459e65.
4. Rubin BP, Heinlich MC, Corless CL. Gastrointestinal stromal
tumour. Lancet 2007;369:1731e41.
5. Miettinen M, Kopczynski J, Makhlouf HR, Sarlomo-Rikala M,
Gyorffy H, Burke A, et al. Gastrointestinal stromal tumors,
intramural leiomyomas, and leiomyosarcomas in the duodenum: a
clinicopathologic, immunohistochemical, and molecular genetic
study of 167 cases. Am J Surg Pathol 2003;27:625e41