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110
P. Zhang et al.
Genetic Testing Heterozygous mutation in exon 11 of KIT,
mutation type is c.1742_1743insCCCAACACAACTTCCTT
ATGATCACAA (p.H580_K581insNPTQLPYDH). Exons
9, 13, and 17 of KIT and exons 12 and 18 of PDGFRA were
wild type.
16.2.3 Prognosis
The patient recovered well and was discharged on the 4th
postoperative day. The patient’s condition was diagnosed as
double-focal gastric GIST, very low risk. According to the
Chinese Consensus on Diagnosis and Treatment of
Gastrointestinal Stromal Tumors (2013 edition), the tumors
were classied as very low risk, and no adjuvant imatinib
treatment was required after surgery. As of September 2019,
28-month follow-up, no signs of recurrence or metastasis
were found.
16.2.4 Experience ofDiagnosis andTherapy
16.2.4.1 Does Small Gastric GIST Need Surgery?
If Surgery Is Required, How toChoose
theSurgical Method?
Currently, surgery is the main treatment for GIST [6].
Surgical treatment is still the rst choice for localized stromal tumors with a diameter of >2cm originating from the
stomach. For small gastric GIST with diameter ≤2cm, the
indications for different surgical methods are still controversial. Some scholars believe that most small gastric GISTs
have a benign clinical process, which can be observed regularly and do not require radical surgery. Other scholars
believe that small gastric GIST may be malignant. Once discovered, early surgical treatment is recommended [7]. The
NCCN guidelines point out that gastric GIST with a diameter of <2cm, but in the presence of unregular borders, ulceration, strong echoes, heterogeneity, and other adverse factors,
resection should be considered; if there are no adverse factors, the patient can be regularly reviewed with ultrasound
endoscopy [8]. However, ESMO recommends surgical resection even for gastric GIST with a maximum diameter of
<2cm. Based on a retrospective study of 90 cases of gastric
GIST, one author believed that early detection and timely
intervention for gastric GIST not only has the advantages of
minimal trauma and swift recovery, but can also effectively
reduce the recurrence of GIST after surgery and improve
patient prognosis [9]. Early radical surgical intervention is an
ideal and reasonable treatment for small gastric
GIST. Through a retrospective study of 63 cases of gastric
GIST, Zhang Hongwei etal. from Xijing Digestive Disease
Hospital suggested that small gastric GIST have malignant
potential and recommended surgical resection following
GIST diagnosis [10].
The dispute between serosal surgery and mucosal surgery
in small GIST has been ongoing for a long time. The detection rate of small gastric GIST under endoscopy is gradually
increasing, and with the rapid development of endoscopic
treatment technology, endoscopists have been able to achieve
full-thickness resection of gastric wall masses, and some
gastric GIST can be directly removed endoscopically to
avoid surgery. However, the characteristics of GIST determine that it originates in the muscularis propria of the stomach, which makes GIST far more difcult to treat than
mucosal lesions. Insufcient resection may lead to tumor
damage and a high incidence of perforation. Severe bleeding
perforation may endanger the lives of patients. Therefore, the
Chinese Consensus on Diagnosis and Treatment of
Gastrointestinal Stromal Tumors (2017 Edition) did not recommend routine endoscopic treatment of GIST [11]. The
author believes that in practical clinical work, each unit
should appropriately match the endoscopy and endoscopy
forces according to the technical strength of specic departments and carry out dual-lens combined technology with the
goal of maximizing the clinical benet for patients. This
author believes that for the rational treatment of small gastric
GIST, the patient’s condition (including tumor size, site,
general growth pattern, and patient’s treatment willingness)
and the physician’s technology (including the hardware and
software capabilities of endoscopic and laparoscopic treatment) should be taken into consideration to make individual
decisions with the goal of maximizing the clinical benet of
patients.
16.2.4.2 How toAvoid Missed Diagnosis
andMisdiagnosis inClinical Practice?
GIST is usually single, and multiple GIST is relatively
rare. There is no typical clinical manifestation of GIST in
the early stage, and GISTs are mostly found accidentally
while investigating symptoms such as upper abdomen and
upper gastrointestinal bleeding or on gastroscopy or
abdominal CT examination during physical examination.
It is easier to diagnose large GIST using abdominal CT or
gastroscopy, but tumors less than 1cm, and sometimes in
the presence of poor gastric lling, are difcult to identify
using abdominal CT, similarly, it is difcult to nd tumors
growing out of the cavity by gastroscopy. Based on this,
we should not trust the imaging examination completely,
but should be alert to the possibility of multiple gastric
GIST, and fully explore the gastric wall during surgery to
avoid missing small tumors, resulting in poor prognosis
for patients.
16 Multiple Gastrointestinal Stromal Tumors
111
16.3 Expert Comments
JunZhang
Multiple primary sporadic GISTs are rare, the locations and
clinical manifestations are not signicantly different to those
of single GIST, and tumors often appear in the stomach and
small intestine [12]. Often, additional lesions are found when
a patient presents with symptoms caused by a larger GIST or
during intraoperative exploration. GIST has strong heterogeneity. There are different reports and opinions about the differences in clinicopathological features between primary
multiple GIST at home and abroad. However, existing results
tend to suggest that primary multiple GIST originates from
different Cajal cell subsets in the gastrointestinal stroma,
leading to the diversity of clinicopathological features. The
treatment of primary multiple GIST is also based on complete surgical resection. The current risk assessment methods
for postoperative GIST are mainly modied NIH classication and AFIP standards, but these methods are all aimed at
single GIST.Although retrospective studies have shown that
the efcacy of adjuvant therapy for patients with multiple
GIST is similar to that of single GIST after using the abovementioned evaluation methods, there may be some limitations in the evaluation of patients with complex multiple
primary GIST by applying the current criteria. How to accurately judge the risk of postoperative recurrence in such
patients and formulate a reasonable adjuvant treatment plan
needs to attract the attention of clinicians and be investigated
in corresponding research.
References
1. Gasparotto D, Rossi S, Bearzi I, Doglioni C, Marzotto A, Hornick
JL, Grizzo A, Sartor C, Mandolesi A, Sciot R, Debiec-Rychter M,
Dei Tos AP, Maestro R.Multiple primary sporadic gastrointestinal
stromal tumors in the adult: an underestimated entity. Clin Cancer
Res. 2008;14(18):5715–21. https://doi.org/10.1158/1078- 0432.
CCR- 08- 0622.
2. Li FP, Fletcher JA, Heinrich MC, Garber JE, Sallan SE, CurielLewandrowski C, Duensing A, van de Rijn M, Schnipper LE,
Demetri GD. Familial gastrointestinal stromal tumor syndrome:
phenotypic and molecular features in a kindred. J Clin Oncol.
2005;23(12):2735–43. https://doi.org/10.1200/JCO.2005.06.009.
3. Andersson J, Sihto H, Meis-Kindblom JM, Joensuu H, Nupponen
N, Kindblom LG.NF1-associated gastrointestinal stromal tumors
have unique clinical, phenotypic, and genotypic characteristics.
Am J Surg Pathol. 2005;29(9):1170–6. https://doi.org/10.1097/01.
pas.0000159775.77912.15.
4. Joensuu H.Risk stratication of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008;39(10):1411–9. https://
doi.org/10.1016/j.humpath.2008.06.025.
5. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol.
2006;23(2):70–83. https://doi.org/10.1053/j.semdp.2006.09.001.
6. Nishida T, Yoshinaga S, Takahashi T, Naito Y. Recent progress
and challenges in the diagnosis and treatment of gastrointestinal
stromal tumors. Cancers (Basel). 2021;13(13):3158. https://doi.
org/10.3390/cancers13133158.
7. Wang M, Xue A, Yuan W, Gao X, Fu M, Fang Y, Wang L, Shu P, Li
H, Hou Y, Shen K, Sun Y, Qin X.Clinicopathological features and
prognosis of small gastric gastrointestinal stromal tumors (GISTs).
J Gastrointest Surg. 2019;23(11):2136–43. https://doi.org/10.1007/
s11605- 018- 04070- 3.
8. von Mehren M, Randall RL, Benjamin RS, Boles S, Bui MM,
Ganjoo KN, George S, Gonzalez RJ, Heslin MJ, Kane JM, Keedy V,
Kim E, Koon H, Mayerson J, McCarter M, McGarry SV, Meyer C,
Morris ZS, O’Donnell RJ, Pappo AS, Paz IB, Petersen IA, Pfeifer
JD, Riedel RF, Ruo B, Schuetze S, Tap WD, Wayne JD, Bergman
MA, Scavone JL.Soft tissue sarcoma, version 2.2018, NCCN clinical practice guidelines in oncology. J Natl Compr Cancer Netw.
2018;16(5):536–63. https://doi.org/10.6004/jnccn.2018.0025.
9. Zhao Q, Li Y, Yang P, Zang Y, Fan L, Tan B, Tian Y.Evaluation for
therapeutic measures to small gastric stromal tumor: a retrospective
study of 90 cases. Curr Probl Cancer. 2018;42(1):107–14. https://
doi.org/10.1016/j.currproblcancer.2018.01.012.
10. Yang J, Feng F, Li M, Sun L, Hong L, Cai L, Wang W, Xu G, Zhang
H.Surgical resection should be taken into consideration for the treatment of small gastric gastrointestinal stromal tumors. World J Surg
Oncol. 2013;11(1):273. https://doi.org/10.1186/1477- 7819- 11- 273.
11. 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.
12. Li K, Tjhoi W, Shou C, Yang W, Zhang Q, Liu X, Yu J. Multiple
gastrointestinal stromal tumors: analysis of clinicopathologic
characteristics and prognosis of 20 patients. Cancer Manag Res.
2019;11:7031–8. https://doi.org/10.2147/CMAR.S197560.
Hereditary Gastrointestinal Stromal Tumors
XiaoqiLi, LinxiYang, andGangZhao
17
Keywords
Gastrointestinal stromal tumors · Germline mutation
Targeted therapy
17.1 Case 24 A49-Year-Old Woman
withFamilial Hereditary GIST
XiaoqiLi and LinxiYang
17.1.1 Introduction
GIST is predominantly driven by gain-of-function mutations
of either KIT or PDGFRA genes, with the majority of these
mutations being somatic. However, a minority of GIST cases
exhibit germline mutations of these genes. This kind of GIST
often presents with some characteristic clinical manifestations and needs clinicians’ attention.
17.1.2 Case Background
A 49-year-old woman presented to the doctor with abdominal
pain. Gastroscopy and CT scan showed a 2.7 cm × 3.4 cm
mass occupied the descending part of the duodenum.
Neuroendocrine tumors could not be excluded. She underwent
duodenal tumor resection on May 26, 2016. Postoperative
pathology showed GIST, with a tumor size of
3.5 cm × 3 cm × 3 cm, without mitoses. Genetic testing
revealed a mutation in exon 13 of KIT, 654 AAA>GAA (heterozygosity). The patient did not receive adjuvant therapy and
was followed up with regular CT scans. 1 year after surgery,
follow-up CT scans showed multiple enhancement lesions in
X. Li · L. Yang (*) · G. Zhao
Department of Gastrointestinal Surgery, Renji Hospital, Shanghai
Jiaotong University School of Medicine, Shanghai, China
the horizontal and initial segments of the jejunum with a maximum diameter of 1.8 cm, considered to be possible
GIST. Repeat CT scans during the follow-up period showed
that the lesions were all similar in size. A CT scan in April
2019 showed that the multiple masses in the duodenum and
jejunum had increased in size, with a maximum diameter of
2.5 cm. The patient was referred to our hospital for further
treatment. After inquiring about the relevant medical history,
we learned that the patient’s mother had undergone surgery at
a local hospital for GIST in June 2018. A GIST was found on
the greater curvature of the stomach with multiple disseminated nodules in the bowel wall. Genetic testing also revealed
a mutation in exon 13 of KIT, 654 AAA>GAA (heterozygosity). Her mother received imatinib targeted therapy after surgery and had stable disease.
17.1.2.1 Past History andFamily History
The patient was previously healthy and reported no drug
allergies. The patient’s parents were alive and the patient’s
mother also suffered from GIST.
17.1.2.2 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 pattern or peristaltic wave was
observed. The abdomen was soft, with no palpable masses.
The bowel sounds were normal.
17.1.2.3 Auxiliary Examination
Abdominal CT The small intestinal wall in the abdominal
cavity was thickened, and the multiple masses in the duodenum and jejunum were larger than before, with a maximum
diameter of 2.5cm. The ndings were consistent with multiple GIST (Fig.17.1).
17.1.2.4 Preliminary Diagnosis
Postoperative recurrence and metastasis of duodenal GIST,
possible hereditary GIST.
© 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_17
113
114
Fig. 17.1 CT from April 2019 demonstrated multiple masses in the duodenum and jejunum
X. Li et al.
17.1.3 Therapy
17.1.3.1 Case Analysis
The patient had low-risk duodenal GIST and received
radical surgery 3 years ago and with multiple mass-occupying lesions on the small intestinal wall in the abdominal
cavity. The possibility of recurrence and metastasis of the
original tumor was considered. Given that her mother also
suffered from GIST and the mother and daughter had the
same genetic mutation, the possibility of familial hereditary multiple GIST could not be ruled out. Surgical exploration to clarify the pathology, and peripheral blood
genetic testing on the immediate family members were
recommended.
17.1.3.2 Treatment
The patient underwent laparotomy, duodenal tumor resection and small bowel tumors resection on April 14, 2019.
We found that the omentum below the original incision in
her abdominal cavity was widely adhered to the abdominal
wall, and there were dozens of tumors of various sizes in
the duodenum and jejunum. The largest tumor was approximately 3cm in diameter and the smallest one was approximately 2 mm in diameter. The two largest tumors were
located at Treitz’s ligament and the jejunum, 50 cm from
the Treitz’s ligament, respectively (Fig.17.2). We removed
all tumors larger than 2mm which were visible and tried
not to damage the intestinal mucosa. Liquid biopsy of
peripheral blood by next-generation sequencing was performed on the patient, her mother and their immediate
family.
17.1.3.3 Postoperative Pathology andGenetic
Testing
Pathological Diagnosis Duodenal GIST (2cm×1.5cm×
1.5 cm), mitotic count <5/50 HPF. Four small intestinal
GIST (2 cm × 1.5 cm × 1 cm; 0.3 cm; 0.5 cm; 0.5 cm),
mitotic count <5/50 HPF.
Immunohistochemistry CD117 (+), DOG-1 (+), CD34
(+), S-100 (−), Ki-67 (LI: 5%), SDHB (+).
Genetic Testing Heterozygous mutations in exon 13 of
KIT, 654 AAA> GAA, the encoded amino acid was converted from lysine to glutamic acid. Exons 9, 11, and 17 of
KIT and exons 12 and 18 of PDGFRA were all wild type.
Liquid biopsy Heterozygous mutation in exon 13 of KIT,
654 AAA > GAA, was found in the patient and her mother.
No other family members had this mutation (Fig.17.3).
17.1.4 Prognosis
The patient recovered well after the operation, and imatinib
400mg/d treatment was started 2weeks after discharge. As
of follow-up in December 2021, all lesions in the abdominal
cavity of the patient and her mother were stable (Fig.17.4).
17.1.5 Experience ofDiagnosis andTherapy
Multiple GIST caused by germline KIT mutation is an
extremely rare autosomal dominant disorder. So far, more
than 30 families involving mutations at 8, 11, 13 and other
KIT sites have been reported [1–5]. In addition to intra-
abdominal multiple GIST, families with germline KIT
mutations may present with a variety of clinical symptoms,
including hyperpigmentation, urticaria pigmentosa, and dysphagia [6]. In this case, the patient and her mother did not
have any skin symptoms or swallowing problems. Like other
sporadic mutant GIST, imatinib is also effective for heredi-
17 Hereditary Gastrointestinal Stromal Tumors
Fig. 17.2 Intraoperative ndings
115
tary GIST.In the reported cases, the tumors progressed relatively slowly, and imatinib was effective for a long time.
Some patients have disease control for nearly 10years with
imatinib therapy [4, 5].
In this case, both the patient and her mother had multiple
intra-abdominal GIST, and the disease control time was
more than 3 years. For follow-up treatment of hereditary
advanced GIST, it is still recommended to formulate the
treatment plan according to the specic gene mutation site.
17.2 Expert Comments
GangZhao
GIST with germline KIT mutation only accounts for a very
small number of GIST, but it often has characteristics different from sporadic GIST, such as multiple primary lesions,
primary mutation in exon 13 of KIT, and inert biological
behavior. This also suggests that we need to consider the
possibility of hereditary GIST when we encounter patients
with atypical features. Genetic counseling and screening are
very important for GIST with germline mutations [7]. The
popularization of next-generation sequencing technology
greatly improves the accuracy and accessibility of genetic
screening [8], which will contribute to accurate treatment for
patients with relevant pathogenic gene mutations.
Fig. 17.3 Family tree
Fig. 17.4 CT 3 years after surgery
References
1. Halpern AL, Torphy RJ, McCarter MD, Sciotto CG, Glode LM,
Robinson WA.A familial germline mutation in KIT associated with
achalasia, mastocytosis and gastrointestinal stromal tumors shows
response to kinase inhibitors. Cancer Genet. 2019;233–234:1–6.
https://doi.org/10.1016/j.cancergen.2019.02.001.
2. Ke H, Kazi JU, Zhao H, Sun J.Germline mutations of KIT in gas-
trointestinal stromal tumor (GIST) and mastocytosis. Cell Biosci.
2016;6:55. https://doi.org/10.1186/s13578- 016- 0120- 8.
3. Meir M, Maurus K, Kuper J, Hankir M, Wardelmann E, Rosenwald
A, Germer CT, Wiegering A. The novel KIT exon 11 germline
mutation K558N is associated with gastrointestinal stromal tumor,
mastocytosis, and seminoma development. Genes Chromosomes
Cancer. 2021;60(12):827–32. https://doi.org/10.1002/gcc.22988.
4. Bachet JB, Landi B, Laurent-Puig P, Italiano A, Le Cesne A,
Lévy P, Safar V, Duffaud F, Blay JY, Emile JF. Diagnosis, prog-
nosis and treatment of patients with gastrointestinal stromal tumor
(GIST) and germline mutation of KIT exon 13. Eur J Cancer.
2013;49(11):2531–41. https://doi.org/10.1016/j.ejca.2013.04.005.
5. Engin G, Eraslan S, Kayserili H, Kapran Y, Akman H, Akyuz A,
Aykan NF. Imatinib response of gastrointestinal stromal tumor
patients with germline mutation on KIT exon 13: a family report.
World J Radiol. 2017;9(9):365–70. https://doi.org/10.4329/wjr.
v9.i9.365.
6. Li FP, Fletcher JA, Heinrich MC, Garber JE, Sallan SE, Curiel-
Lewandrowski C, Duensing A, van de Rijn M, Schnipper LE,
116
X. Li et al.
Demetri GD. Familial gastrointestinal stromal tumor syndrome:
phenotypic and molecular features in a kindred. J Clin Oncol.
2005;23(12):2735–43. https://doi.org/10.1200/JCO.2005.06.009.
7. Kuroda N, Tanida N, Hirota S, Daum O, Hes O, Michal M, Lee
GH.Familial gastrointestinal stromal tumor with germ line mutation of the juxtamembrane domain of the KIT gene observed in
relatively young women. Ann Diagn Pathol. 2011;15(5):358–61.
https://doi.org/10.1016/j.anndiagpath.2010.05.003.
8. Vanden Bempt I, Vander Borght S, Sciot R, Spans L, Claerhout
S, Brems H, Lehnert S, Dehaspe L, Fransis S, Neuville B, Topal
B, Schöffski P, Legius E, Debiec-Rychter M. Comprehensive
targeted next-generation sequencing approach in the molecular diagnosis of gastrointestinal stromal tumor. Genes
Chromosomes Cancer. 2021;60(4):239–49. https://doi.
org/10.1002/gcc.22923.
Part IV
Gastrointestinal Stromal Tumors
with Other Malignant Tumors
Intermediate-Risk Gastrointestinal
Stromal Tumor withAdvanced Gastric
Cancer
TianlongLing, HaiboQiu, andLepingLi
18
Keywords
Gastrointestinal stromal tumors · Gastric cancer
Intestinal cancer · Adjuvant therapy · Imatinib
18.1 Case 25 An80-Year-Old Man
withSynchronous GIST andGastric
Cancer
TianlongLing and HaiboQiu
18.1.1 Introduction
GIST is a kind of unique mesenchymal tumor which displays
positive CD117. It is rare for a GIST to occur concurrently
with another type of gastrointestinal (GI) tumor. There is
minimal knowledge on the causes. Some scholars believe the
etiology for GIST with another GI tumor is long-term exposure to unknown pathogens. In these conditions, both mesenchymal and epithelial cells of patients with GIST are subject
to carcinogenic transformation, leading to the development
of synchronous tumors. Some other scholars propose that the
two malignant tumors have the same mutated tumor suppressor genes. For patients with dual tumors, it is necessary to
comply to the principle of individualized treatment, carry out
multidisciplinary team (MDT) discussion, and develop suitable treatment regimens. On the other hand, the presence of
synchronous tumors may increase the risk of metastasis.
Thus, it is necessary to arrange close follow-up for patient
with dual tumors.
18.1.2 Case Background
An 80-year-old man was admitted to our hospital in July
2014. He had been diagnosed with an abdominal mass following a routine screening test at a local hospital. He received
a series of examinations in our hospital. The abdominal CT
scan showed a mass at the gastric cardia close to the lesser
curvature. The mass appeared to be a GIST originating from
the gastric wall (Fig.18.1). A low-density lesion in liver segment II, likely to be a liver cyst was also observed.
Gastroscopy revealed an ulcerated mass between the cardia
and the fundus of the stomach (Fig. 18.2). The pathology
ndings identied a gastric adenocarcinoma with moderate
differentiation.
T.Ling
Department of Gastrointestinal Surgery, Renji Hospital, Shanghai
Jiaotong University School of Medicine, Shanghai, China
H.Qiu (*)
Department of Gastric Surgery, Sun Yat-sen University Cancer
Center, Guangzhou, China
e-mail: qiuhb@sysucc.org.cn
L.Li
Department of Gastrointestinal Surgery, Shandong Provincial
Hospital, Jinan, China
e-mail: Lileping@medmail.com.cn
© 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_18
Fig. 18.1 Abdominal CT
119
120
T. Ling et al.
Fig. 18.2 Gastroscopic ndings
18.1.2.1 Past History andFamily History
The patient was previously healthy and reported no history
of drug allergies. There was no similar medical history in the
family.
18.1.2.2 Physical Examination
The patient’s vital signs were stable, and the skin and sclera
were not yellowish or pale. The abdomen was at, and no
gastrointestinal or peristaltic waves were seen. The abdomen
was soft, with no tenderness, rebound pain, or palpable
abdominal masses and the bowel sounds were normal.
18.1.2.3 Auxiliary Examination
Gastroscopy An ulcerated mass between the cardia and the
fundus of the stomach.
Abdominal CT There was a mass in the gastric cardia,
close to the lesser curvature, which appeared to be a GIST
originating from the gastric wall. Additionally, there was a
low-density lesion in liver segment II, likely to be a liver
cyst.
Blood (Routine) White blood cells 24.5×109/L; hemoglo-
bin 92g/L; platelets 90.9×109/L.
Liver and Renal Function Normal.
Coagulation Test Normal.
Cardiac Ultrasound Unremarkable.
18 Intermediate-Risk Gastrointestinal Stromal Tumor withAdvanced Gastric Cancer
18.1.2.4 Preliminary Diagnosis
1. Type III adenocarcinoma of the esophagogastric junction
(AEG)
2. Leukocytosis with unknown cause
18.1.3 Therapy
18.1.3.1 Case Analysis
The patient was an elderly man. The diagnosis of type III
AEG was conrmed by gastroscopy, pathology ndings,
and CT scans. However, the cause of leukocytosis was
unknown. Thus, a bone marrow biopsy was performed, and
a hematology consultation was requested. The biopsy
revealed active bone marrow hyperplasia, in which the
granulosa system had slightly maturation and left shift. No
malignant tumor cells were found in the smear. As a result,
the hematologist ruled out chronic myeloid leukemia and
believed the AEG was the main cause of leukocytosis. The
subsequent MDT meeting, considering that both tumors
had no distant metastasis and were expected to be completely resected, concluded that surgical intervention
should be performed.
18.1.3.2 Treatment
Fig. 18.3 An ulcerative mass between the fundus and the cardia of the
stomach
The patient underwent total gastrectomy with D2 lymph
node dissection, esophagojejunal Roux-en-Y anastomosis,
and splenectomy on July 12, 2014. During the surgery, the
primary tumor was assessed and was found to be
7cm×6cm in size and located between the fundus and the
cardia of the stomach. The tumor had invaded the retroperitoneum and splenic hilus but not the pancreas, the colon or
the liver. Gross observation of the postoperative specimen
revealed an ulcerative mass, 4cm×3cm in size, between
the fundus and the cardia of the stomach (Fig. 18.3).
Another mass, 6cm × 7cm in size, was also identied at
right side of the cardia, not attached to the primary tumor
(Fig.18.4).
121
18.1.3.3 Postoperative Pathology
Pathological Diagnosis (Tumor 1) AEG, 3.5 cm ×
3cm×1cm, intestinal type, with poor to moderate differentiation. The tumor inltrated the supercial muscularis.
There was no evidence of vascular or neural invasion and
there were no tumor cells in the omentum majus or spleen.
Lymph node invasion: 1/45. The tumor was located at the
cardia of the stomach. The mass resembled a cauliower in
appearance, was greyish white in color and was rm.
Fig. 18.4 A mass at right side of the cardia not attached to the primary
tumor