Malignant GIST of Duodenum A Case report

By

Dr E Aravind

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 tumorsof the ampulla, pancreas and common bile duct

• 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.

• 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

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

Case Report

• Name - Narayanamurtynaidu

• Age – 52

• Sex – Male

• Occupation - Farmer

• Address - Ramchandrapuram

• 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

• 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

• 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

• Palpation

– Abdomen soft

– No Guarding or Rigidity

– No Palpable lumps

– No Hepatomegaly

– No Spleenomegaly

– Both testis in scrotum Lt Supraclavicular fossaempty

• 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

• Provisional Diagnosis

• Liver abscess

Investigations

• Ultrasound Abdomen

– Multiple Liver Abcess

– Well defined hypoechoic lesion in arotocavalregion with communiction with adjacent bowel - ? Bowel mass

• 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

• UGIE

– Small hiatus hernia

– Severe diffuse gastrits

– Ulcerated growth in 2nd part of duodeneum

• ? Periampullary Carcinoma

• ? GIST

– Biopsy

• Well Differentiated Adenocarcinoma

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

• 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

• 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

• 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

• Post operative period

– Un eventfull

– Sutures Removed on POD – 10

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

• 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 tumourinfiltration

– Omentum - Nil particular

Follow up

• Case was referred to Department of Radiotherapy, GGH, KKD for further management

• They referred the case to higher centre for chemotherapy

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

• The great majority of duodenal mesenchymaltumors 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

• 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

• 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

• 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

References1. 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 stromaltumour. 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