BACKGROUND: Gastrointestinal stromal tumours (GIST) usually present with non-specific gastrointestinal symptoms such as abdominal mass, pain, anorexia and bowel obstruction. METHODS: We report a case of a 42 year old male who presented with a solitary intracranial space occupying lesion which was established as a metastasis from a mesenteric tumour. RESULTS: The patient was initially treated as a metastatic sarcoma, but a lack of response to chemotherapy prompted testing for CD117 which returned positive. A diagnosis of mesenteric GIST presenting as solitary brain metastasis was made, and the patient was treated with imatinib. CONCLUSION: We recommend that all sarcomas with either an intraabdominal or unknown origin be routinely tested for CD117 to rule out GIST

Anup K Das

AT van Oosterom

B Hughes

BJ Brooks

GD Demetri

GJC Burkill

Goura K Rath

Gowthaman Gunabushanam

K Akiyama

LG Kindblom

M Miettinen

M Shabahang

Monica Malik

Pramod K Julka

RP DeMatteo

Shikha Goyal

Tarun Puri

TS Emory

YK Cheon

English

PubMed

Crossref

Mesenteric gastrointestinal stromal tumour presenting as intracranial space occupying lesion

Abstract Background Gastrointestinal stromal tumours (GIST) usually present with non-specific gastrointestinal symptoms such as abdominal mass, pain, anorexia and bowel obstruction. Methods We report a case of a 42 year old male who presented with a solitary intracranial space occupying lesion which was established as a metastasis from a mesenteric tumour. Results The patient was initially treated as a metastatic sarcoma, but a lack of response to chemotherapy prompted testing for CD117 which returned positive. A diagnosis of mesenteric GIST presenting as solitary brain metastasis was made, and the patient was treated with imatinib. Conclusion We recommend that all sarcomas with either an intraabdominal or unknown origin be routinely tested for CD117 to rule out GIST.</p

Das Anup K

Goyal Shikha

Malik Monica

Gunabushanam Gowthaman

Puri Tarun

Julka Pramod K

Rath Goura K

Directory of Open Access Journals

World Journal of Surgical Oncology

Springer - Publisher Connector

BioMed Central
World Journal of Surgical Oncology
ssOpen AcceCase report
Mesenteric gastrointestinal stromal tumour presenting as 
intracranial space occupying lesion
Tarun Puri*1, Gowthaman Gunabushanam2, Monica Malik1, Shikha Goyal1, 
Anup K Das3, Pramod K Julka1 and Goura K Rath1
Address: 1Department of Radiotherapy and Oncology, All India Institute of Medical Sciences, New Delhi, India, 2Department of Radiodiagnosis, 
All India Institute of Medical Sciences, New Delhi, India and 3Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
Email: Tarun Puri* - tarunpuri90@hotmail.com; Gowthaman Gunabushanam - gowthaman7@gmail.com; 
Monica Malik - dr_monica_malik@hotmail.com; Shikha Goyal - shikha@india.com; Anup K Das - drmunmun01@hotmail.com; 
Pramod K Julka - drjulka@rediffmail.com; Goura K Rath - gkrath@rediffmail.com
* Corresponding author    
Abstract
Background: Gastrointestinal stromal tumours (GIST) usually present with non-specific
gastrointestinal symptoms such as abdominal mass, pain, anorexia and bowel obstruction.
Methods: We report a case of a 42 year old male who presented with a solitary intracranial space
occupying lesion which was established as a metastasis from a mesenteric tumour.
Results: The patient was initially treated as a metastatic sarcoma, but a lack of response to
chemotherapy prompted testing for CD117 which returned positive. A diagnosis of mesenteric
GIST presenting as solitary brain metastasis was made, and the patient was treated with imatinib.
Conclusion: We recommend that all sarcomas with either an intraabdominal or unknown origin
be routinely tested for CD117 to rule out GIST.
Background
Gastrointestinal stromal tumours (GIST) are thought to
arise from the interstitial cells of Cajal, the intestinal pace-
maker cells [1]. GIST commonly arises from the stomach
and small intestine and usually presents with non-specific
abdominal symptoms [2]. GIST mostly metastasizes
within the abdomen [3]. To our knowledge, GIST present-
ing with brain metastasis has not been reported in the lit-
erature. We report a case of a 42 year old male who
presented with a solitary intracranial space occupying
lesion which was subsequently established to be a metas-
tasis from a mesenteric GIST.
Case presentation
A 42-year-old male presented in January 2004 complain-
ing of headache and left-sided weakness of eight months
duration and vomiting of four months duration. There
was no history of trauma, seizures or loss of conscious-
ness. Computed Tomography (CT) scan of the brain (Fig-
ure 1) showed a 3.5 cm sized ring-enhancing mass in the
right parietal lobe with significant perilesional oedema.
Magnetic resonance imaging (MRI) of the head showed
the mass to be isointense on T1 and hypointense on T2
weighted images. The patient underwent right parietal
craniotomy with gross total excision of the tumour. His-
topathology of the operative specimen showed features of
undifferentiated sarcoma with areas of necrosis (Figure 2).
Published: 14 November 2006
World Journal of Surgical Oncology 2006, 4:78 doi:10.1186/1477-7819-4-78
Received: 24 June 2006
Accepted: 14 November 2006
This article is available from: http://www.wjso.com/content/4/1/78
© 2006 Puri et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 4
(page number not for citation purposes)
World Journal of Surgical Oncology 2006, 4:78 http://www.wjso.com/content/4/1/78The pathological differential diagnoses given were meta-
static sarcoma and primary intracranial sarcoma. Radio-
graphic skeletal survey, chest x-ray and ultrasonogram of
the abdomen were normal at this time. The patient was
treated with postoperative cranial radiotherapy to a dose
of 60 Gray with concomitant chemotherapy with carbopl-
atin at a dose of 150 mg weekly.
The patient remained asymptomatic till June 2004 when
he complained of abdominal fullness and pain. CT of the
abdomen showed a large mesenteric mass measuring 8 ×
6 cm in size. An ultrasound guided tru-cut biopsy from the
mesenteric mass revealed a malignant spindle cell tumour
with morphology similar to that of the brain tumour. The
mitotic index of the abdominal tumour was 10 per 50
HPF (high power fields). Immunohistochemistry was
positive for CD34, vimentin; and negative for GFAP (glial
fibrillary acidic protein) and SMA (smooth muscle actin).
Thereafter the patient was treated with six cycles of combi-
nation chemotherapy with ifosfamide 1.8 gm/m2 i.v. D1-
4 with mesna uroprotection and epirubicin 60 mg/m2 i.v.
in two divided doses, as 3-weekly cycles until November
2004. However, there was no apparent clinical response to chemotherapy, and abdomen CT (Figure 3) done at this
time showed a 10 × 8 cm sized heterogeneously enhanc-
ing mesenteric mass.
Given the failed response to chemotherapy, the pathology
specimen was retested for CD117/c-kit which turned out
to be positive. Thus, the patient was diagnosed as a pri-
mary mesenteric gastrointestinal stromal tumour present-
ing as solitary brain metastasis. The patient was then
treated with imatinib mesylate 600 mg oral daily. CT scan
Contrast enhanced CT (CECT) scan of the abdomen done in N vember 2004 after 6 cycles of ifosfamide and epirubicin combina io  chemotherapy showing a 10 × 8 cm size  heter-ogeneously en ancing mesenteric massFigu e 3
Contrast enhanced CT (CECT) scan of the abdomen done in 
November 2004 after 6 cycles of ifosfamide and epirubicin 
combination chemotherapy showing a 10 × 8 cm sized heter-
ogeneously enhancing mesenteric mass.
CT scan of the brain showing a 3.5 cm sized ring-enhancing mas  (arr w) in the right parietal lobe with sig ificant mass effect d compression of the right lateral ventricleFigure 1
CT scan of the brain showing a 3.5 cm sized ring-enhancing 
mass (arrow) in the right parietal lobe with significant mass 
effect and compression of the right lateral ventricle. There is 
significant peri-lesional oedema.
Section from operative specimen from right parietal lobe lesion shows features of undiff rentiated sarcoma with areas of necrosisFigure 2
Section from operative specimen from right parietal lobe 
lesion shows features of undifferentiated sarcoma with areas 
of necrosis. (Haematoxylin & Eosin, 400×)Page 2 of 4
(page number not for citation purposes)
World Journal of Surgical Oncology 2006, 4:78 http://www.wjso.com/content/4/1/78of the chest and abdomen after three months of therapy
showed a good response, evidenced by a cystic conversion
of tumour mass with no visible areas of enhancement
(Figure 4). The patient continued to remain on imatinib
therapy. However, after the initial response, CT scan of
abdomen done after another three months showed a focal
nodular area of enhancement (arrow) within the
mesenteric mass, suggestive of disease progression. (Fig-
ure 5) The patient subsequently developed cachexia and
died four months later.
Discussion
Gastrointestinal stromal tumours (GIST) are thought to
arise from the interstitial cells of Cajal, the intestinal pace-
maker cells [1]. GIST most commonly arises from the
stomach (60–70%) and small intestine (20–30%) with
less than 10% arising from the rest of gastrointestinal tract
(oesophagus, colon, rectum) or extraintestinal sites
(omentum, mesentery, retroperitoneum) [2-4].
The clinical presentation of patients with GIST can vary
depending on the tumour location, size and aggressive-
ness. Typically, patients present with non-specific gas-
trointestinal symptoms such as abdominal mass, pain,
vomiting, anorexia and bowel obstruction. Rarely,
patients may present with acute haemorrhage into perito-
neal cavity from tumour rupture or cutaneous metastases
[5,6].
GIST mostly metastasizes within the abdomen. In one
series of 83 patients [7], the common sites of metastases
were to the liver (46%) and peritoneum (41%). Other
sites of metastases reported in this series include the retro-
peritoneum, lung, bone and abdominal scar. Involvement
of the central nervous system (CNS) by metastatic GIST is
extremely rare. A review of the literature reveals only three
reports of CNS involvement by GIST. In all cases, patients
had known metastatic disease elsewhere prior to the
development of CNS metastases. In one case [8], a 60-
year-old male with diagnosed metastatic GIST developed
sudden unilateral blindness that was found to be caused
by metastatic involvement of the cavernous sinus. In the
second case [9], a 47-year-old male who was being treated
with imatinib for metastatic GIST developed multiple cer-
ebral relapses even while systemic disease appeared to be
controlled. In the third case [10], a 75-year-old male with
multiple liver and peritoneal metastases developed a sin-
gle intracranial lesion. In the third patient, all lesions
including the intracranial lesion showed a good response
to imatinib therapy.
In the present case, the patient had an unusual presenta-
tion as an intracranial metastasis. The patient was initially
treated as a metastatic sarcoma from unknown primary
due to the absence of extracranial disease on radiological
workup. Even after the detection of the mesenteric pri-
mary, the patient was initially treated as a mesenteric sar-
coma as histopathology showed a sarcoma which was
positive for CD34 and vimentin antigen. The possibility
of GIST was suspected only after the patient failed to
respond to ifosfamide and epirubicin combination chem-
otherapy. As the immunohistochemisty returned positive
for CD117 at this stage, the patient was treated with imat-
inib [11,12]. A good initial treatment response was noted
with imatinib, with a reduction in tumour size and the
development of cystic changes in the tumour mass. How-
CECT of the abdomen done three months after that in Fig-ure 4 showing a f cal odular a a of en ancement (arrow) within the mesenteric mass, which is suggestiv  of disease p ogressionFigu  5
CECT of the abdomen done three months after that in Fig-
ure 4 showing a focal nodular area of enhancement (arrow) 
within the mesenteric mass, which is suggestive of disease 
progression. The patient died four months later.
CECT of the abdomen done after three months of imatinib therapy showing a good response, evid nced by a cystic con-version of the mesenteric mass wi  no enhancing are swithinFigure 4
CECT of the abdomen done after three months of imatinib 
therapy showing a good response, evidenced by a cystic con-
version of the mesenteric mass with no enhancing areas 
within.Page 3 of 4
(page number not for citation purposes)
World Journal of Surgical Oncology 2006, 4:78 http://www.wjso.com/content/4/1/78Publish with BioMed Central   and  every 
scientist can read your work free of charge
"BioMed Central will be the most significant development for 
disseminating the results of biomedical research in our lifetime."
Sir Paul Nurse, Cancer Research UK
Your research papers will be:
available free of charge to the entire biomedical community
peer reviewed and published immediately upon acceptance
cited in PubMed and archived on PubMed Central 
yours — you keep the copyright
Submit your manuscript here:
http://www.biomedcentral.com/info/publishing_adv.asp
BioMedcentral
ever, the patient subsequently developed progressive dis-
ease and died.
Conclusion
The present report describes a unique presentation of
GIST as intracranial metastasis. To the best of our knowl-
edge, this is the first report of its kind in the literature. The
true nature of disease remained obscure for a considerable
duration; this delay could be attributed, in part to the
atypical presentation and to the lack of routine testing for
CD117. We recommend that all cases of sarcomas with an
intraabdominal origin or unknown origin be routinely
tested for CD117 to detect GIST as these tumours usually
respond to imatinib.
Competing interests
The author(s) declare that they have no competing inter-
ests.
Authors' contributions
TP, GG, MM, SG, AKD, PKJ, GKR contributed significantly
to draft the manuscript. All authors read and approved the
final manuscript.
Acknowledgements
Consent was taken from the patient's relatives for publication of this 
report.
References
1. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindbhom JM: Gas-
trointestinal pacemaker cell tumour (GIPACT): Gastroin-
testinal stromal tumors show phenotypic characteristics of
the interstitial cells of Cajal.  Am J Pathol 1998, 152:1259-1269.
2. DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan
MF: Two hundred gastrointestinal stromal tumours: recur-
rence patterns and prognostic factors for survival.  Ann Surg
2000, 231:51-58.
3. Miettinen M, Lasota J: Gastrointestinal stromal tumours – defi-
nition, clinical, histological, immunohistochemical, and
molecular genetic features and differential diagnosis.  Vir-
chows Arch 2001, 438:1-12.
4. Emory TS, Sobin LH, Lukes L, Lee DH, O'Leary TJ: Prognosis of gas-
trointestinal smooth-muscle (stromal) tumours: depend-
ence on anatomic site.  Am J Surg Pathol 1999, 23:82-87.
5. Cheon YK, Jung IS, Cho YD, Kim JO, Lee JS, Lee MS, Kim JH, Hur KY,
Jim SY, Shim CS: A spontaneously ruptured gastric stromal
tumour with cystic degeneration presenting as hemoperito-
neum: a case report.  J Korean Med Sci 2003, 18:751-755.
6. Shabahang M, Livingstone AS: Cutaneous metastases from a gas-
trointestinal stromal tumour of the stomach: review of liter-
ature.  Dig Surg 2002, 19:64-65.
7. Burkill GJC, Badran M, Al-Muderis O, Meirion Thomas J, Judson IL,
Fisher C, Moskovic EC: Malignant gastrointestinal stromal
tumour: Distribution, imaging features and pattern of meta-
static spread.  Radiology 2003, 226:527-532.
8. Akiyama K, Numaga J, Kagaya F, Takazawa Y, Suzuki S, Koseki N, Kato
S, Kaburaki T, Kawashima H: Case of optic nerve involvement in
metastasis of a gastrointestinal stromal tumour.  Jpn J Ophthal-
mol 2004, 48:166-168.
9. Hughes B, Yip D, Goldstein D, Waring P, Beshay V, Chong G: Cere-
bral relapse of metastatic gastrointestinal stromal tumour
during treatment with imatinib mesylate: case report.  BMC
Cancer 2004, 4:74.
10. Brooks BJ, Bani JC, Fletcher CD, Demetri GD: Challenges in oncol-
ogy. Case 4. Response of metastatic gastrointestinal stromal
tumour including CNS involvement to imatinib mesylate
(STI-571).  J Clin Oncol 2002, 20:870-872.
11. van Oosterom AT, Judson I, Verweij J, Stroobants S, Donato di Paola
E, Dimitrijevic S, Martens M, Webb A, Sciot R, Van Glabbeke M, Sil-
berman S, Nielsen OS: Safety and efficacy of imatinib (STI 571)
in metastatic gastrointestinal stromal tumours: a phase I
study.  Lancet 2001, 358:1421-1423.
12. Demetri GD, von Mehren M, Blanke CD, Van den Abbeele AD, Eisen-
berg B, Roberts PJ, Heinrich MC, Tuveson DA, Singer S, Janicek M,
Fletcher JA, Silverman SG, Silberman SL, Capdeville R, Kiese B, Peng
B, Dimitrijevic S, Druker BJ, Corless C, Fletcher CD, Joensuu H: Effi-
cacy and safety of imatinib mesylate in advanced gastrointes-
tinal stromal tumours.  N Engl J Med 2002, 347:472-480.Page 4 of 4
(page number not for citation purposes)


Case of optic nerve involvement in metastasis of a gastrointestinal stromal tumour.

Chong G: Cerebral relapse of metastatic gastrointestinal stromal tumour during treatment with imatinib mesylate: case report.

CS: A spontaneously ruptured gastric stromal tumour with cystic degeneration presenting as hemoperitoneum: a case report.

Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumours.

F i s h e r C , M o s k o v i c E C : Malignant gastrointestinal stromal tumour: Distribution, imaging features and pattern of metastatic spread. Radiology

Gastrointestinal stromal tumours – definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch

GD: Challenges in oncology. Case 4. Response of metastatic gastrointestinal stromal tumour including CNS involvement to imatinib mesylate (STI-571). J Clin Oncol

Livingstone AS: Cutaneous metastases from a gastrointestinal stromal tumour of the stomach: review of literature. Dig Surg

Meis-Kindbhom JM: Gastrointestinal pacemaker cell tumour (GIPACT): Gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal.

MF: Two hundred gastrointestinal stromal tumours: recurrence patterns and prognostic factors for survival. Ann Surg

O'Leary TJ: Prognosis of gastrointestinal smooth-muscle (stromal) tumours: dependence on anatomic site.

OS: Safety and efficacy of imatinib (STI 571) in metastatic gastrointestinal stromal tumours: a phase I study. Lancet

file:///data/core-remote/dit/data/Springer-OA/pdf/0e3/aHR0cDovL2xpbmsuc3ByaW5nZXIuY29tLzEwLjExODYvMTQ3Ny03ODE5LTQtNzgucGRm.pdf

Mesenteric gastrointestinal stromal tumour presenting as intracranial space occupying lesion

Abstract

Similar works

Full text

Available Versions

Crossref

Directory of Open Access Journals

Springer - Publisher Connector

Springer - Publisher Connector