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200
Fig. 29.3 H&E staining of gastric tissue
L. Tu et al.
Fig. 29.4 H&E staining of brain tissue
Immunohistochemistry CD117 (+), CD34 (−), DOG-1
(−).
Genetic Testing No mutations in the exons 9, 11, 13 and 17
of KIT, synonymous mutation in exon 12 of PDGFRA, no
mutations in exon 18 of PDGFRA.
29.1.4 Prognosis
Postoperative CT scan of the head on April 20 showed that
the intracranial metastases were stable, and the edema and
bleeding had been absorbed. A third PET-CT was performed
on April 29, and it was found that the tumor had reduced in
29 Gastrointestinal Stromal Tumors withBrain Metastases
201
size, the metabolism of the intracranial lesion was signicantly reduced, the bleeding in the lesion was largely
absorbed, and no new bleeding was observed. A repeat CT
scan on May 8 revealed that the intracranial metastases had
shrunk and the hematoma had absorbed further since the previous scan. The patient developed systemic edema and renal
insufciency during subsequent therapy, so the dose of sunitinib was reduced to 25 mg/d, but the symptoms did not
improve signicantly. Regorafenib treatment was initiated at
160mg/d on July 25. A PET-CT following the withdrawal of
regoranib on August 13 indicated that the primary lesion
was progressing. Sunitinib treatment was restarted on August
18, and the patient relapsed with systemic edema and renal
insufciency. Sunitinib was discontinued on September 1,
and the edema was relieved. A CT scan on September 2
revealed a new lesion on the brain. The patient died from
cachexia on October 29.
29.1.5 Experience ofDiagnosis andTherapy
Brain metastasis of GIST is very rare. There are only a
few reports of such cases at present, but once brain metastases occur, the disease is often critical, and the prognosis
is poor [1, 2]. Twenty days after the discovery of intracrania metastases and craniotomy, the patient was again
found to have multiple brain metastases with hemorrhage,
which showed rapid progression of the disease. In a case
report by Doniel etal., it was mentioned that the average
survival time of patients who died from GIST brain metastasis was 14.3months, and the survival time of a patient
who received only surgical treatment was just 7 months
[3].
This patient’s disease developed rapidly and increasing
the dose of imatinib did not control the disease effectively,
which may be related to the genetic type of the tumor.
According to the results of postoperative genetic testing,
the tumor could be judged as wild-type GIST. Wild-type
GIST can be roughly divided into succinate dehydrogenase
(SDH)-decient GIST and non-SDH-decient GIST [4].
This patient was not tested for SDH, so it is not possible to
determine the subtype. The targets of TKIs currently used
for treating GIST are those with KIT and PDGFRA mutations, while wild-type GIST does not carry genetic mutations, resulting in tumors being insensitive to traditional
TKIs [5]. According to statistics, the effective rate of imatinib against wild-type GIST is 14%, while the overall
effective rate against GIST is as high as 60–80%. Therefore,
it is not difcult to understand why the patient’s disease
could not be effectively controlled with preliminary
treatment.
The blood-brain barrier permeability of imatinib is only
10%, so its therapeutic effect for brain metastases is poor.
Sunitinib, another TKI, has a blood-brain barrier permeability of 40% and has effective anti-angiogenesis and antitumor activities [6]. Sunitinib currently has approval for use
in the treatment of advanced renal cell carcinoma, imatinib
refractory GIST, and advanced pancreatic neuroendocrine
tumors [7]. In this case, the disease could not be controlled
with an increasing dose of imatinib after brain metastasis.
However, the lesions shrank signicantly after sunitinib
treatment, conrming the therapeutic effect of sunitinib.
However, attention should also be paid to the adverse effects
associated with sunitinib. Common adverse effects include
hand-foot syndrome, stomatitis, and other skin toxicities. In
severe cases, cerebral hemorrhage may occur, especially in
patients with hypertension [8]. In this case, the patient developed systemic edema and renal insufciency after using
sunitinib, and the edema was relieved after withdrawal of the
drug. Therefore, although sunitinib is effective in the treatment of brain metastatic GIST, further exploration on how to
balance safety and efcacy is warranted.
29.2 Expert Comments
ZhigangJie
While GIST metastasis to the liver and peritoneum is
common, metastasis outside the abdomen is rare, and brain
metastasis is very rare. At present, there are only a few
reports of such cases. In the early stage of brain metastasis,
in addition to symptoms of the digestive system, the symptoms of the nervous system, such as signs of intracranial
hypertension, are often not obvious. Once there are nervous
system symptoms, it indicates that the tumor focus is large,
compressing nerves and blood vessels, presents with
symptoms such as intracranial hypertension, and the disease
develops rapidly. At this time, craniotomy may be required
to remove the tumor. In terms of drug treatment, it is difcult for the rst-line treatment drug, imatinib, to pass
through the blood-brain barrier. Therefore, the efcacy of
imatinib in brain metastasis is insufcient. Sunitinib,
another TKI, has a blood-brain barrier permeability of 40%.
In one study, sunitinib was given to patients with brain
metastasis. The results showed that the response lasted for
more than 4 weeks, indicating that sunitinib has a certain
therapeutic effect in controlling GIST brain metastasis.
However, due to the low incidence rate and the small number of cases, there is no systematic study on epidemiology,
clinical, and therapeutic results. The specic treatment plan
after GIST brain metastasis still needs more in-depth clini-
202
L. Tu et al.
cal exploration. At present, it is considered that the application of the second-line drug sunitinib is an appropriate
treatment option.
References
1. Takeuchi H, Koike H, Fujita T, Tsujino H, Iwamoto Y. Sunitinib
treatment for multiple brain metastases from jejunal gastrointestinal stromal tumor: case report. Neurol Med Chir (Tokyo).
2014;54(8):664–9. https://doi.org/10.2176/nmc.cr2012- 0426.
2. Prablek M, Srinivasan VM, Srivatsan A, Holdener S, Oneissi M, Heck
KA, Jalali A, Mandel J, Viswanathan A, Patel AJ.Gastrointestinal
stromal tumor with intracranial metastasis: case presentation and
systematic review of literature. BMC Cancer. 2019;19(1):1119.
https://doi.org/10.1186/s12885- 019- 6316- 7.
3. Drazin D, Spitler K, Jeswani S, Shirzadi A, Bannykh S, Patil
C. Multiple intracranial metastases from a gastric gastrointestinal stromal tumor. J Clin Neurosci. 2013;20(3):471–3. https://doi.
org/10.1016/j.jocn.2012.02.037.
4. Wada R, Arai H, Kure S, Peng WX, Naito Z. “Wild type” GIST:
clinicopathological features and clinical practice. Pathol Int.
2016;66(8):431–7. https://doi.org/10.1111/pin.12431.
5. Klug LR, Khosroyani HM, Kent JD, Heinrich MC.New treatment
strategies for advanced-stage gastrointestinal stromal tumours.
Nat Rev Clin Oncol. 2022;19(5):328–41. https://doi.org/10.1038/
s41571- 022- 00606- 4.
6. Soria JC, Blay JY, Spano JP, Pivot X, Coscas Y, Khayat
D.Added value of molecular targeted agents in oncology. Ann
Oncol. 2011;22(8):1703–16. https://doi.org/10.1093/annonc/
mdq675.
7. Bose D, Meric-Bernstam F, Hofstetter W, Reardon DA, Flaherty
KT, Ellis LM. Vascular endothelial growth factor targeted therapy in the perioperative setting: implications for patient care.
Lancet Oncol. 2010;11(4):373–82. https://doi.org/10.1016/
S1470- 2045(09)70341- 9.
8. Je Y, Schutz FA, Choueiri TK.Risk of bleeding with vascular endothelial growth factor receptor tyrosine-kinase inhibitors sunitinib
and sorafenib: a systematic review and meta-analysis of clinical
trials. Lancet Oncol. 2009;10(10):967–74. https://doi.org/10.1016/
S1470- 2045(09)70222- 0.
Gastrointestinal Stromal Tumors
withGiant Liver Metastases
LinxiYang, TianlongLing, andKaixiongTao
30
Keywords
Gastrointestinal stromal tumor · ALPPS · Liver metastases · Imatinib
30.1 Case 40 A49-Year-Old Woman
withAdvanced GIST Who Underwent
ALPPS forGiant Liver Metastases
LinxiYang and TianlongLing
30.1.1 Introduction
Targeted therapy is the main method for the treatment of recurrent and metastatic GIST.The value of surgery in the treatment
of recurrent and metastatic GIST is still controversial, but a
growing body of evidence-based science shows that cytoreductive surgery during the response to targeted drug therapy or
localized progression may help to prolong patient survival.
30.1.2 Case Background
The patient, a 49-year-old woman, presented to local hospital
due to abdominal pain. The enhanced abdominal CT scan
showed a space-occupying lesion in the lesser curvature of the
stomach (possible GIST). There were multiple spaceoccupying lesions in the liver, and metastasis was considered.
L.Yang · T.Ling (*)
Department of Gastrointestinal Surgery, Renji Hospital, Shanghai
Jiaotong University School of Medicine, Shanghai, China
K.Tao
Department of Gastrointestinal Surgery, Union Hospital, Tongji
Medical College, Huazhong University of Science and Technology
University, Wuhan, China
e-mail: kaixiongtao@hust.edu.cn
The patient underwent needle biopsy of lesions in the right
lobe of the liver. The biopsy showed GIST with less tissue,
and the mitotic count could not be evaluated. Immunohistochemistry test results showed: CD117 (+), CD34 (+), DOG-1
(+), SMA (−), desmin (−), S100 (+), Ki67 (LI: 10%). Genetic
testing was not performed due to insufcient tissue volume.
The patient started imatinib treatment. The dose was 400mg/d.
The patient was regularly followed up until after 34months,
the latest abdominal enhanced CT scan showed that the maximum diameter of the primary gastric tumor increased from
2.8 to 3.0 cm; the largest metastatic tumor in the right lobe of
liver increased from 6.1 to 7.2 cm; the number of metastases
in the right lobe of the liver also increased. Considering the
tumor progression and possible imatinib drug resistance, imatinib was discontinued and sunitinib 50mg/d treatment was
initiated. An enhanced abdominal CT 6months later showed
that the largest diameter of the primary gastric tumor had
increased to 4.2cm, and the largest metastatic tumor in the
right lobe of the liver had increased to 12.2 cm. The patient
was admitted to hospital for further treatment.
30.1.2.1 Past History andFamily History
The patient was previously healthy and reported no history
of drug allergies. The patient’s parents were alive, and there
was no similar medical history in the family.
30.1.2.2 Physical Examination
The patient’s vital signs were stable, and the skin and mucous
membranes were not yellowish or pale. The abdomen was
at, and no gastrointestinal or peristaltic wave was observed.
The abdomen was soft, with right upper quadrant tenderness,
and no palpable mass in the abdomen. The bowel sounds
were normal.
30.1.2.3 Auxiliary Examination
Abdomen CT The space-occupying side of the lesser curvature of the stomach (GIST possible) was larger than before;
there were multiple liver-occupying masses, considering the
© People’s Medical Publishing House, PR of China 2024
K. Tao, H. Cao (eds.), Clinical Management of Gastrointestinal Stromal Tumor, https://doi.org/10.1007/978-981-99-9392-5_30
203
204
Fig. 30.1 CT showed obvious tumor progression
L. Yang et al.
ization) (Fig. 30.2). The patient recovered well after the
operation. Two weeks after the operation, the CT scan
showed that the multiple nodules in the liver were a similar
size to the previous scan. The FLR increased to 412mL, and
the FLR/SLV was 37%. The patient underwent right hepatic
trilobectomy on May 17, 2021. The omentum and liver sections were densely adhered, and the right liver was ischemic
and necrotic, with dark green effusion in the necrotic lesions;
the left liver displayed compensatory hyperplasia (Fig.30.3).
30.1.3.3 Postoperative Pathology andGenetic
Testing
Pathological Diagnosis Gastric GIST,
4.5cm×4cm×3cm in size, mitotic count 30/50 HPF.Liver
GIST, 19cm×16cm×10cm in size, mitotic count 40/50
HPF.
metastasis, and some lesions progressed signicantly compared with the previous (Fig.30.1).
30.1.3 Therapy
30.1.3.1 Case Analysis
A MDT discussion was held in our center to discuss the
patient’s condition. After joint discussion and evaluation by
the departments of oncology, liver surgery, gastrointestinal
surgery, and imaging, it was considered that the metastases
in the right lobe of the patient’s liver and the primary gastric
tumor had progressed, although the other intrahepatic metastases were still stable. Surgical resection could be considered, and genetic mutation information could be obtained at
the same time. The patient had no underlying liver disease.
The future liver remnant (FLR) of the patient was estimated
to be about 253mL based on the enhanced abdominal CT,
the standard liver volume (SLV) was about 1100mL and the
FLR/SLV was 23%. Therefore, associating liver partition
and portal vein ligation for staged hepatectomy (ALPPS)
could be considered. First, the primary gastric tumor was
resected. The right portal vein and the liver parenchyma were
ligated, and the liver parenchyma was severed. The patient
was evaluated weekly after the operation. After the FLR/SLV
was more than 30%, right hepatic trilobectomy was
performed.
30.1.3.2 Treatment
The patient underwent partial gastrectomy and special
hepatic segmentectomy (the rst step of ALPPS) on April 29,
2021. A lobulated exophytic tumor 4 cm in diameter was
located in the middle of the lesser curvature of the stomach,
with abundant blood supply. The right liver was occupied by
a huge tumor with a diameter of about 15cm. There were
several metastatic nodules in the left liver, ranging in diameter from 1 to 3cm, showing post-treatment changes (hyalin-
Immunohistochemistry CD117 (+), DOG-1 (+), CD34
(+), S-100 (−), Ki-67 (Li: 40%), SDHB (+).
Genetic Testing Heterozygous mutations in exon 11 of
KIT, 553–558 deletion, cause the deletion of the encoded
amino acids tyrosine, glutamic acid, valine, glutamine, tryptophan, and lysine. Exons 9, 13 and 17 of KIT and exons 12
and 18 of PDGFRA were all wild type.
30.1.4 Prognosis
The patient recovered well after the operation and resumed
imatinib 400mg/d treatment after discharge. As of December
2021 follow-up, there were no new metastasis in the abdominal cavity or liver (Fig.30.4).
30.1.5 Experience ofDiagnosis andTherapy
30.1.5.1 The Role ofCytoreductive Surgery
inDelaying Tumor Progression After
Secondary Resistance toTKIs
Previous studies have demonstrated that R0/R1 cytoreduction in patients who have not progressed on targeted drug
therapy can prolong PFS in patients with advanced GIST [1].
At the same time, in some patients with advanced GIST with
focal progression, complete resection of imatinib-resistant
lesions can also lead to longer disease control time [2–5]. At
present, it is believed that for the evaluation of resectable
focal recurrence and metastasis and liver metastasis of recurrent and metastatic GIST, surgical resection combined with
targeted drug therapy can bring survival benets to patients
with advanced GIST, and the 2021 Chinese Society of
Clinical Oncology GIST diagnosis and treatment guidelines
also recommend cytoreductive surgery as a grade II suggestion [6].
30 Gastrointestinal Stromal Tumors withGiant Liver Metastases
Fig. 30.2 Isolated liver
tumor and gastric tumor
specimens in the rst step of
ALPPS
Fig. 30.3 Intraoperative liver
in the second step of ALPPS
and isolated liver tumor
specimens
205
Fig. 30.4 CT results 2 weeks and 6 months after surgery
30.1.5.2 Treatment Options forAdvanced GIST
withLiver Metastases
Patients with advanced GIST and liver metastases are not
uncommon. In addition to drug therapy, the treatment modes
for patients with liver metastases include RFA, TACE, and
surgery. Surgical resection is usually reserved for patients
with resectable metastases and adequate residual liver volume. Surgery was previously considered inappropriate for
patients who would have insufcient remaining liver vol-
ume. Since ALPPS was rst reported in 2007, it has been
mostly used to treat large liver lesions with a high risk of
post-resection liver failure due to insufcient residual liver
volume. It is mainly used to treat liver metastases from
colorectal cancer, hepatocellular carcinoma, and hilar cholangiocarcinoma, and fewer than ten cases of ALPPS have
been reported for liver metastases from GIST [7, 8]. Among
the three patients with GIST who received ALPPS with follow- up, two patients had no metastasis at 17 months and
206
L. Yang et al.
37 months after surgery, and one patient had recurrence
6months after surgery [9–11].
The total course of the patient’s disease was nearly 4years,
she received rst- and second-line drug treatments, and disease progression was observed during both treatments. The
rst-line drug, imatinib, brought nearly 3 years of disease
control, and the second-line drug sunitinib only provided PFS
for less than 6 months. According to previous studies, if
patients receive third-line regorafenib therapy, gastric primary tumors and liver metastases may continue to progress.
Fortunately, the patient’s metastatic lesions were limited to
the liver, and the left liver lesions were all stable. The progressive lesions were limited to the right half of the liver, and
there was no metastatic lesion in the abdominal cavity, which
just met the requirements for ALPPS.The patient achieved
disease control for half a year, and all the liver metastases
which were previously well controlled by imatinib did not
progressed. No new lesions had appeared. The left liver had
satisfactory compensatory hyperplasia, with no abnormal
liver function and good quality of life.
30.2 Expert Comments
KaixiongTao
This case introduces the experience of surgical resection
of a localized progressive lesion during targeted drug therapy. The 2-staged hepatectomy used in this case is suitable
for liver lesions with large volume. The advantage is that the
volume and function of residual liver can be ensured and the
risk of liver failure can be reduced. However, this technology
is difcult and is not suitable for cases with other metastatic
lesions in the residual liver or the abdominal cavity. The
MDT cooperation for diagnosis and treatment is necessary to
guarantee success for such surgery.
References
1. Bauer S, Rutkowski P, Hohenberger P, Miceli R, Fumagalli
E, Siedlecki JA, Nguyen BP, Kerst M, Fiore M, Nyckowski P,
Hoiczyk M, Cats A, Casali PG, Treckmann J, van Coevorden F,
Gronchi A.Long-term follow-up of patients with GIST undergo-
ing metastasectomy in the era of imatinib– analysis of prognostic
factors (EORTC-STBSG collaborative study). Eur J Surg Oncol.
2014;40(4):412–9. https://doi.org/10.1016/j.ejso.2013.12.020.
2. Park SJ, Ryu MH, Ryoo BY, Park YS, Sohn BS, Kim HJ, Kim CW,
Kim KH, Yu CS, Yook JH, Kim BS, Kang YK.The role of surgical resection following imatinib treatment in patients with recurrent
or metastatic gastrointestinal stromal tumors: results of propensity
score analyses. Ann Surg Oncol. 2014;21(13):4211–7. https://doi.
org/10.1245/s10434- 014- 3866- 4.
3. Raut CP, Posner M, Desai J, Morgan JA, George S, Zahrieh D,
Fletcher CD, Demetri GD, Bertagnolli MM.Surgical management
of advanced gastrointestinal stromal tumors after treatment with
targeted systemic therapy using kinase inhibitors. J Clin Oncol.
2006;24(15):2325–31. https://doi.org/10.1200/JCO.2005.05.3439.
4. Yeh CN, Chen TW, Tseng JH, Liu YY, Wang SY, Tsai CY, Chiang
KC, Hwang TL, Jan YY, Chen MF.Surgical management in metastatic gastrointestinal stromal tumor (GIST) patients after imatinib
mesylate treatment. J Surg Oncol. 2010;102(6):599–603. https://
doi.org/10.1002/jso.21630.
5. Fairweather M, Balachandran VP, Li GZ, Bertagnolli
MM, Antonescu C, Tap W, Singer S, DeMatteo RP, Raut
CP. Cytoreductive surgery for metastatic gastrointestinal stromal
tumors treated with tyrosine kinase inhibitors: a 2-institutional
analysis. Ann Surg. 2018;268(2):296–302. https://doi.org/10.1097/
SLA.0000000000002281.
6. Li J, Ye Y, Wang J, Zhang B, Qin S, Shi Y, He Y, Liang X, Liu X,
Zhou Y, Wu X, Zhang X, Wang M, Gao Z, Lin T, Cao H, Shen
L, Chinese Society of Clinical Oncology Csco Expert Committee
on Gastrointestinal Stromal Tumor. Chinese consensus guidelines
for diagnosis and management of gastrointestinal stromal tumor.
Chin J Cancer Res. 2017;29(4):281–93. https://doi.org/10.21147/j.
issn.1000- 9604.2017.04.01.
7. Lang H, Baumgart J, Mittler J.Associated liver partition and portal vein ligation for staged hepatectomy (ALPPS) registry: what
have we learned? Gut Liver. 2020;14(6):699–706. https://doi.
org/10.5009/gnl19233.
8. Lai Q, Mennini G, Larghi Laureiro Z, Rossi M.Uncommon indications for associating liver partition and portal vein ligation for
staged hepatectomy: a systematic review. Hepatobiliary Surg Nutr.
2021;10(2):210–25. https://doi.org/10.21037/hbsn- 20- 355.
9. Liu J, Zhang C, Hong D, Shang M, Yao W, Chen Y.Percutaneous
microwave ablation liver partition and portal vein embolization for
planned hepatectomy due to large gastrointestinal stromal tumor
metastases: a case report. Medicine (Baltimore). 2017;96(42):e8271.
https://doi.org/10.1097/MD.0000000000008271.
10. Sanei B, Sheikhbahaei S, Sanei MH, Bahreini A, Jafari
HR.Associating liver partition and portal vein ligation for staged
hepatectomy: a surgical technique for liver resections. J Res Med
Sci. 2017;22:52. https://doi.org/10.4103/jrms.JRMS_829_16.
11. Troja A, Khatib-Chahidi K, El-Sourani N, Antolovic D, Raab
HR. ALPPS and similar resection procedures in treating extensive hepatic metastases: our own experiences and critical discussion. Int J Surg. 2014;12(9):1020–2. https://doi.org/10.1016/j.
ijsu.2014.07.006.
Part VII
Management of Misdiagnosed Gastrointestinal
Stromal Tumors
Small Intestine Gastrointestinal Stromal
Tumor Misdiagnosed asGynecological
Tumor
LinTu, YouweiKou, andJinboGao
31
Keywords
Gastrointestinal stromal tumor · Gynecological tumor
Secondary resistance
31.1 Case 41 A56-Year-Old Woman
withSmall Intestine GIST Misdiagnosed
asaGynecological Tumor
LinTu and YouweiKou
31.1.1 Introduction
Due to the relatively xed location of giant small intestinal
GIST, especially small intestine GIST in the pelvic cavity,
which move poorly and adhere to the uterus and appendages, it is difcult to distinguish from gynecological tumors
and is often misdiagnosed as ovarian tumors or uterine
broids. Incorrect diagnosis and treatment may lead to iatrogenic dissemination of tumors, thereby affecting patient
prognosis.
L.Tu
Department of Gastrointestinal Surgery, Renji Hospital, Shanghai
Jiaotong University School of Medicine, Shanghai, China
Y.Kou (*)
Department of Gastrointestinal Surgery, Shengjing Hospital of
China Medical University, Shenyang, China
e-mail: kouyw@sj-hospital.org
J.Gao
Department of Gastrointestinal Surgery, Union Hospital, Tongji
Medical College, Huazhong University of Science and Technology
University, Wuhan, China
31.1.2 Case Background
The patient, a 56-year-old woman, presented to the gynecology clinic of our hospital in June 2015. She reported that she
had frequent urination for the past year, up to 10–13 times a
day, with no abnormal vaginal bleeding or discharge, no urgent
urination or odynuria, and no abdominal pain or fever. She
developed distension and pain in the middle and lower abdomen 6 days previously. Ultrasonography was performed in
another hospital and showed a space-occupying lesion in the
right pelvic cavity, indicating an ovarian tumor. The patient
came to our hospital for further diagnosis and treatment.
31.1.2.1 Past History andFamily History
The patient had a 2-year history of hypertension, highest
blood pressure of 175/95mmHg, and took oral amlodipine,
1 tablet/day, which controlled the blood pressure at
120/85 mmHg. She reported no history of infectious diseases, such as hepatitis and tuberculosis, heart disease, diabetes, trauma, allergies to food and drug, or blood
transfusions.
31.1.2.2 Physical Examination
The patient was conscious, with no yellowing of sclera, no
appearance of anemia, clear breath sounds on both lungs, and
a heart rate of 80 beats/min. The abdomen was soft, with slight
tenderness in the lower abdomen, and no rebound tenderness
or guarding. The liver and spleen were not palpable below the
costal margin, there were no bowel sounds, shifting dullness,
or edema in the lower limbs, and the patient had normal movement of the limbs. Gynecological examination revealed normal vulva development, smooth vagina, no mucosal
hyperemia, a small amount of secretion in the vagina, no peculiar smell. Cervical hypertrophy, center, anterior uterus, which
was shrinking, hard, a slightly irregular shape, and movable. A
12cm mass could be palpated in the right adnexal area, the
mass was hard, with poor mobility, and tenderness. There
were no obvious abnormalities in the left adnexa area.
© People’s Medical Publishing House, PR of China 2024
K. Tao, H. Cao (eds.), Clinical Management of Gastrointestinal Stromal Tumor, https://doi.org/10.1007/978-981-99-9392-5_31
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L. Tu et al.
31.1.2.3 Auxiliary Examination
Blood Routine WBC 8.38×109/L, RBC 4.0× 1012/L, Hb
119g/L, PLT 351×109/L↑, NEUT% 59.4%, LY% 32.6%.
Blood Biochemistry No abnormalities.
Tumor Marker CA125 21.85U/mL.
Gynecological Color Doppler Ultrasound The uterus was
about 3.7cm×3.5cm×3.2cm in size with uniform echo,
and the intima was 0.3cm in thickness. The left ovary was
unclear. A hypoechoic mass was seen in the right pelvic cavity, with a range of approximately 16.8 cm × 12.6 cm ×
11.4 cm, irregular shape, unclear borders, and no capsule.
Multiple irregular anechoic areas were seen inside of the
mass, the largest one was 3.3cm×2.8cm in size, the color
blood ow was rich, and the arteriovenous frequency spectrum could be explored. A free anechoic space was seen in
the pelvis, about 1.2cm in depth. Ultrasound ndings showed
postmenopausal uterus, right pelvic space occupying lesion
(Fig.31.1).
31.1.2.4 Preliminary Diagnosis
1. Space-occupying lesion of right pelvic cavity: possible
ovarian tumor
2. Hypertension, grade 2, Intermediate Risk
31.1.3.2 Treatment
After the patient was admitted to the hospital, active preoperative preparations were performed, and the patient underwent
laparotomy. During the operation, a solid mass of 16cm in
diameter was observed in the right pelvic cavity. The surfaces
of the liver, spleen, stomach, and diaphragm were smooth, and
the greater omentum was normal. The mass originated from
the small intestine and adhered to the appendix, colon, and
abdominal wall. An abdomen opener was used, the intestinal
tube was padded with a bowel guard, and, after loosening the
adhesions, the mass was gradually removed. A frozen sample
was sent for pathological analysis which revealed that the
tumor was a small intestinal GIST.Following the diagnosis, a
partial small bowel resection was performed.
31.1.3.3 Postoperative Pathology andGenetic
Testing
Postoperative Pathology Small intestinal GIST, mitotic
count 11/50 HPF, modied NIH recurrence risk classication: high risk.
Immunohistochemical Staining CD117 (+), CD34 (+),
DOG-1 (+), SMA (−), S-100 (−), Ki67 (Li: 10%).
Genetic Testing Mutation in exon 11 of KIT, Exons 9, 13 and
17 of KIT and exons 12 and 18 of PDGFRA were all wild type.
31.1.3 Therapy
31.1.3.1 Case Analysis
The patient was a middle-aged woman with abdominal distension for more than 1week. Gynecological color Doppler
ultrasound and CT of abdomen and pelvis revealed a huge
mass in the right pelvic cavity, which was considered to be a
gynecological tumor. In order to clarify the nature of the
mass and guide the subsequent diagnosis and treatment,
exploratory laparotomy was performed.
31.1.4 Prognosis
The postoperative genetic test revealed a mutation in exon 11
of KIT, and the patient was treated with imatinib 400mg/d
and was reexamined regularly after surgery. The tumor
recurred in situ 17 months postoperatively (Fig.31.2), suggesting tumor resistance. The patient chose surgical treatment
again after discussion with her family. Extensive adhesions
between the greater omentum, peritoneum, and small intestine were seen and were carefully separated during the opera-
Fig. 31.1 Preoperative CT results