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Duodenal Gist
Duodenal Gist
Ioannidis O.1, Iordanidis F.2, Fidanis T.3, Chatzopoulos S.1, Kotronis A.1, Paraskevas G.4, Konstantara A.1,
Papadimitriou N.1, Makrantonakis A.1, Kakoutis E.1
1
First Surgical Department, General Regional Hospital George Papanikolaou, Thessaloniki, Greece
2
Department of Pathology, General Regional Hospital George Papanikolaou, Thessaloniki, Greece
3
Department of Radiology, General Regional Hospital George Papanikolaou, Thessaloniki, Greece
4
Department of Anatomy, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece
Summary
Gastrointestinal stromal tumours (GISTs) are considered to derive from the interstitial cells of The authors declare they have no potential
Cajal or their precursors and are defined by their expression of c-kit protein (CD117) that is posi- conflicts of interest concerning drugs,
tive in 95% percent of cases. These are rare mesenchymatous tumours, while they represent the products, orservices used in the study.
most common mesenchymal tumours of the alimentary tract. The majority of GISTs develop in Autoi deklaruj, e v souvislosti s pedmtem
the stomach and small intestine and more rarely in the rectum, colon, esophagus and mesen- studie nemaj dn komern zjmy.
tery; only 35% of all GISTs are located in the duodenum. The presenting symptoms include The Editorial Board declares that the manuscript
early satiation, dysphagia, bloating, abdominal pain and gastrointestinal bleeding, either acute met the ICMJE uniform requirements for
or chronic. Surgery remains the mainstay of treatment for localized, non-metastatic, resectable biomedical papers.
GISTs. We present acase of duodenal gastrointestinal stromal tumour of the third portion of the Redakn rada potvrzuje, e rukopis prce
duodenum that presented with acute upper gastrointestinal bleeding treated with segmental splnil ICMJE kritria pro publikace zaslan do
duodenal resection. biomedicnskch asopis.
Key words Orestis Ioannidis, MD, MSC
angiography bleeding duodenum GIST
Alexandrou Mihailidi 13
54640 Thessaloniki
Souhrn Greece
Pedpokld se, e gastrointestinln stromln tumory (GIST) vznikaj zCajalovch interstici- e-mail: [email protected]
lnch bunk nebo jejich prekurzor ajsou definovny expres c-kit proteinu (CD117), kter
je pozitivn v95% ppad. Jedn se ovzcn mezenchymln ndory, kter vak jsou nejas-
Submitted/Obdreno: 16. 9. 2011
tjmi mezenchymlnmi ndory zavacho traktu. Vtinou se vyvjej valudku atenkm
Accepted/Pijato: 13. 10. 2011
stev, vzcnji pak vrektu, tlustm stev, jcnu amezenteriu, piem pouze 3-5 % vech GIST
postihuje duodenum. Mezi pznaky pat asn nasycen, dysfagie, nadmn, bolest bicha
akrvcen do zavacho traktu, bu akutn, nebo chronick. Hlavnm terapeutickm postupem
ulokalizovanho, nemetastazujcho aresekovatelnho GIST zstv chirurgick een. Popi-
sujeme ppad pacienta sgastrointestinlnm stromlnm tumorem tetho oddlu duodena,
kter se projevoval akutnm krvcenm do hornho zavacho traktu abyl len klnovitou re-
sekc duodena.
Klov slova
angiografie krvcen duodenum GIST
Introduction
Gastrointestinal stromal tumors (GISTs)
are rare mesenchymatous tumors [1]
but represent however the most com-
mon mesenchymal tumors of the ali-
mentary tract [2] and account for
0.13% of all gastrointestinal ma-
lignancies [3]. Their incidence is
1020/1,000,000/year and their preva-
lence is estimated at 129 per million [4].
There seems to be no predilection of ei-
ther gender [4,5]. GISTs usually present in
patients in their sixth and seventh deca-
des [3] with 75% of cases occurring in pa-
tients over the age of 50 [5]. They are con-
sidered to derive from the interstitial cells
of Cajal or their precursors [4] and are de-
fined by their expression of c-kit protein
(CD117), which is positive in 95% percent
of the cases [1]. Most cases develop in the
stomach and small intestine and more
rarely the rectum, colon, esophagus and
mesentery while only 35% of all GISTs
are localized in the duodenum[1,3,4,6].
GISTs are symptomatic in approxima-
tely two thirds of the patients, about one
fifth is found incidentally and about one Fig. 1. The abdominal CT scan demonstrated a well demarcated tumor measuring
tenth is discovered at autopsy[3,4]. The 6x 5.5 cm at the third part of the duodenum compressing and abutting forward the
presenting symptoms include early sati- duodenum.
ation, dysphagia, bloating, obstruction,
abdominal pain and gastrointestinal mia with a 19.1% Ht and 6.4 g/dl he- ableeding ulcer in the third part of the
bleeding, either acute or chronic [35]. moglobin, left-shifting leukocytosis duodenum.
We present acase of duodenal GIST of with a WBC count of 12,300 106/ml The abdominal computed tomogra-
the third portion of the duodenum that (75.7% neutrophils) and thrombocyto- phy (CT) scan demonstrated awell de-
presented with acute upper gastrointes- penia with aPLT count of 87,000/mm3. marcated tumor measuring 6 5.5 cm
tinal bleeding treated by segmental duo All other blood chemistry was within at the third part of the duodenum com-
denal resection. the normal range. The patient was trans- pressing and abutting forward the duo
fused with 4 units of blood. Emergency denum without though any intra-abdo-
Case Report gastroduodenal endoscopy revealed minal metastatic lesion (Fig. 1).
A 66 years old male presented to our
hospital with acute upper gastrointesti-
nal bleeding. The patient reported me-
lenas for two days and a hematemesis
recently. His medical history included
arterial hypertension and diabetes mel-
litus and he received valsartan 160 mg
once aday and metformin 425 mg twice
a day. Also the patient had iron defici-
ency anemia for about ayear for which
he received ferrous sulfate sesquihyd-
rate 80 mg daily.
Physical examination revealed apale
man with arterial pressure 108/52 mmHg
and heart rate of 112 beats/min. Rectal
examination confirmed the melena. La- Fig. 2. The angiography revealed an abnormal leash of vessels arising from the super-
boratory examination revealed ane- ior mesenteric artery.
Fig. 4. A. Histologically, the duodenal tumor composed of relatively uniform spindle cells of arranged in short fascicles or whorls.
B. CD117(KIT) staining in GIST show diffuse, strong, cytoplasmic positivity in the majority of tumor cells.
defined heterogeneous masses with We consider the tumor in the present gastrointestinal tract after segmental
a peripheral enhancing border of va- case to be of dual differentiation as it ex- duodenectomy have been described
riable thickness and central low atte pressed both myogenic and neurogenic including end to side duodenojejunos-
nuation [10]. Small tumors are usually markers (SMA and S-100 respectively). tomy with papiloplasty [14], rectocolic
depicted as sharply marginated, smooth, All GISTs have acertain potential for Roux-en-Y duodenojejunostomy [15],
homogenous masses with mode- malignancy [1] and the most important side to side duodenojejunostomy [16],
rate contrast enhancement while large prognostic factors are their size and their side to end duodenojejunostomy [6]
tumors show heterogeneous contrast mitotic count. The tumor in our case be- and an end to end duodenojejunos-
enhancement and tend to have mucosal longed to the intermediate risk group as tomy [17]. Wedge resection of the duo
ulceration, cavitation and central necro- the mitotic index was less than 5/50 HPF denum is generally indicated for small
sis [8,9]. In MRI studies the solid com- and the tumor size was more than 5 cm lesions less than 1 cm except of those le-
ponents of GISTs are usually low sig- but less than 10 cm. Also, recently other sions located within 2 cm from the am-
nal on T1-weighted images and high clinicopathological factors have been pulla of Vater. Segmental resection is in-
signal in T2-weighted images and en- shown to affect overall survival and di- dicated for large tumors, over 3 cm, on
hance following gadolinium administra- sease free survival including, tumor loca- the third and fourth portion of the duo
tion[9,10]. Angiography reveals ahyper- tion, male sex, R1 resection, tumor rap- denum, while Whipplesresection is in-
vascular mass with dense homogeneous ture and tumor grade [4,12]. dicated for periampularry GISTs and for
capillary staining, prominent feeding ar- Differential diagnosis of GISTs in- large tumors of the first and second por-
teries and enlarged draining veins [9]. clude mesenchymal neoplasms such as tion of the duodenum [7]. In the present
Positron emission tomography (PET) is gastrointestinal leiomyoma, leiomyo case we performed a segmental rese-
avaluable complementary tool for treat- sarcoma, schwannoma, submucosal ction of the third and fourth portion of
ment monitoring [4,9] and provides gastrointestinal lesions such as ectopic the duodenum. As all GISTs are virtually
functional information about the GIST. pancreas, carcinoid with a spindle cell associated with arisk of metastasis, fol-
Macroscopically, GISTs are often cove- morphology and epithelial gastrointesti- low-up is essential and CT imaging is re-
red by apseudocaspule and are usually nal neoplasms such as adenocarcinoma. commended every three to six months
fleshy and solid, grey-white in appearance Other tumors that need to be conside- for aminimum of five years [18].
but may also present hemorrhage, central red in the differential diagnosis are lym- Imatinib is an ATP analogue that binds
cystic degeneration or necrosis[5,6,11]. phoma, local extension by a primary the intracellular portion of KIT and inhi-
Microscopically, GISTs are consisted retroperitoneal dedifferentiated lipo- bits signaling [5] and is considered the
by spindle cells (80%), epithelioid cells sarcoma, intra-abdominal fibromatosis first line of treatment for recurrent and
(2030%), or mixed spindle and epitheli- (desmoid tumors), peritoneal carcino- metastatic GISTs [18]. Neoadjuvant and
oid cells (10%) [4]. Epithelioid GISTs most matosis, benign and malignant vascular adjuvant imatinib is presently evaluated
commonly originate in the stomach[4,5] tumor, inflammatory fibroid polyp and under study conditions [5,6] and is not
Spindle cell GISTs are commonly arran- metastatic disease such as spindle cell advised for resectable non metastatic
ged in fascicles, while epithelioid lesions melanoma or carcinoma [9,13]. GISTs but neoadjuvant imatinib can be
may be arranged in nests or sheets [5]. Surgery remains the mainstay of treat- used when surgery would result in sig-
Immunohistochemistry reveals positivity ment for localized, non metastatic, re- nificant morbidity or loss of organ func-
for KIT (CD117) in 95% of cases, for vimen- sectable GISTs [4,6,7]. Complete enbloc tion[18]. Interim results of clinical trials
tin in nearly all GISTs, for CD34 in 6070% surgical resection of the tumor with ne- show that adjuvant imatinib improves
and for myogenic marker smooth gative surgical margins and avoidance overall survival and delays recurrence[4].
muscle actin (SMA) in 3040% [46]. of tumor rupture, which can lead to peri- In conclusion GISTs are considered to
GISTs are usually negative for neuroge- toneal spread should be the goal of sur- derive from the interstitial cells of Cajal
nic marker S-100 (95%) and desmin or ke- gery [1,4,6,7]. As GISTs metastasize ex- or their precursors and are defined by
ratin (9899%). Most GISTs posses activa- tremely rare in the locoregional lymph their expression of c-kit protein (CD117),
ting KIT or platelet derived growth factor nodes and lymph node recurrence is li- which is positive in 95% percent of the
receptor alpha (PDGFRA) mutations [5]. mited, lymphadenectomy is unneces- cases. The majority of cases (75%) occur
GISTs can be classified in four types based sary. For duodenal GISTs, the commo- in patients over the age of 50. They are
on phenotypical features: nest procedures are wedge resection, rare mesenchymatous tumors but re-
1. tumors that show differentiation to- segmental resection, pancreaticoduo present however the most common
ward smooth muscle cells, denectomy and pancreas sparing duo mesenchymal tumors of the alimentary
2. tumors that show differentiation to- denectomy but the optimal surgical tract. Most cases develop in the stomach
ward neural elements, treatment of duodenal GISTs still rema- and small intestine and more rarely the
3. tumors that show no differentiation ins unclear [2,7]. Reconstruction after rectum, colon, esophagus and mesen-
and segmental GISTs is very challenging tery while only 35% of all GISTs locali-
4. tumors that show dual differentia- and is not free of complications. Seve- zed in the duodenum. The presenting
tion[8]. ral techniques for reconstruction of the symptoms include early satiation, dys-
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