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Erschienen in: Journal of Cancer Research and Clinical Oncology 6/2019

Open Access 28.03.2019 | Original Article – Clinical Oncology

Gastrointestinal stromal tumors (GISTs) with remarkable cystic change: a specific subtype of GISTs with relatively indolent behaviors and favorable prognoses

verfasst von: Anwei Xue, Wei Yuan, Xiaodong Gao, Yong Fang, Ping Shu, Chen Xu, He Li, Yifang Xu, Qi Song, Yingyong Hou, Kuntang Shen

Erschienen in: Journal of Cancer Research and Clinical Oncology | Ausgabe 6/2019

Abstract

Purpose

Gastrointestinal stromal tumors (GISTs) are typically solid neoplasms with small cystic change detected occasionally but in rare instances may present predominantly as cystic lesions. The histopathologic features and prognoses of cystic GISTs (cGISTs) are poorly understood.

Methods

We herein reviewed 20 cGISTs resected or consulted in our institution from January 1, 2003 to December 31, 2014.

Results

Of the 20 patients included, the mean age was 61 years and the male-to-female ratio was 9:11. The original locations were the stomach (n = 10, 50%), the small intestine (n = 9, 45%) and the omentum (n = 1, 5%). Indistinct diagnosis or misdiagnosis was established in 15 cases based only on preoperative radiology. Grossly, the cystic component made up the bulk of masses and was filled by dark bloody fluid and necrotic debris in 18 cases. Microscopically, cyst wall was composed of neoplastic spindle (n = 14, 70%)/epithelioid cells (n = 6, 30%) and collagenous fiber, with necrotic debris and granulation tissue lining on the inner surface. cGISTs resembled their solid counterparts in terms of morphology and immunohistology but demonstrated fewer malignant parameters. c-kit or PDGFRα mutations were detected in eleven cases with the remaining being wild type for these two mutations. Although classified as intermediate or high (3 and 17, respectively) risk of recurrence according to modified National Institute of Health criterion, most patients with cGISTs experienced long-term recurrence-free survival without adjuvant imatinib.

Conclusions

Cystic GISTs is a relatively indolent subset of GISTs with favorable prognoses and adjuvant imatinib should be a prudent consideration.
Hinweise
Anwei Xue and Wei Yuan contributed equally to this work.

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Introduction

Gastrointestinal stromal tumors (GISTs) represent the most common mesenchymal tumors arising from the digestive tract, with an estimated annual incidence of 10–20 per million individuals in western countries (Corless 2014). The majority of GISTs are located in the stomach (60–70%), small intestine (25–35%) and duodenum (5%) (Miettinen and Lasota 2006). Immunohistochemical and ultramicrostructural findings indicate that GISTs may originate from the intestinal cells of Cajal, which serve as pacemakers for peristaltic contractions (Kindblom et al. 1998). About 85–95% of GISTs harbor activating mutations in c-kit or PDGFRα that drives the pathogenesis and progression of the disease (Fletcher et al. 2002; Heinrich et al. 2003). Imatinib mesylate, which targets KIT and PDGFRα, has emerged as an effective therapeutic alternative for advanced GISTs while surgical resection remains the mainstay treatment for resectable ones (von Mehren et al. 2018).
GISTs are typically solid, sometimes with small cystic area developed, but rarely manifest as predominant cystic neoplasms. To date, reports of this uncommon form of GISTs have comprised mostly case reports focusing mainly on its clinical and radiographic features (Hamza et al. 2016; Okano et al. 2015; Shaikh et al. 2015; Sun et al. 2016; Takahashi et al. 2010; Wang et al. 2017; Zhu et al. 2014). There is very limited information in the literature relating to the pathologic features and prognoses of GISTs undergoing extensive cystic change. To better elucidate the characteristics of such lesions, we herein undertook this study of the clinical and morphologic features, the prognoses, and the mutational status of 20 affected patients.

Materials and methods

Study approval was obtained from the institutional review board at Zhongshan Hospital, Fudan University. Surgical pathology database and consultation files of our hospital were queried for GISTs with cystic change from January 1, 2003 to December 31, 2014. The diagnosis of “cystic gastrointestinal stromal tumors (cGISTs)” was established if the proportion of cystic component was larger than 75% and cyst wall was relatively regular by corresponding gross reports and/or preoperative radiology reports. A total of 20 cases were retrieved, 10 from the surgical pathology database and 10 from the consultation files. Patient variables included age, sex, symptoms on presentation, preoperative radiology and medical history.
Gross pathology reports were assessed for location, size, whether unilocular or multilocular, cystic fluid and septa thickness of the tumor. Hematoxylin and eosin-stained slides or corresponding scanned photographs were reviewed by two experienced pathologists for cellular type (spindle, epithelioid or mixed), cellularity, nuclear atypia, mitotic activity (number of mitoses per 50 high power fields). Lymph node metastasis, vascular, fat, nerve or mucosal infiltration, mitoses ≥ 10/50 HPF, muscularis propria infiltration, coagulative necrosis, perivascular pattern and severe nuclear atypia were considered as malignant biological behaviors and evaluated in all cases (Hou et al. 2009a, b).
Immunohistochemistry of CD117, desmin and CD34 were performed in all cases using formalin-fixed paraffin-embedded tissue sectioned at 4 µm while S-100, SMA and DOG-1 immunostain were available for analysis in a varying number of cases. Selected mutation hotspots in c-kit exons 9, 11, 13 and 17 as well as PDGFRα exons 12, 14 and 18 were examined using polymerase chain reaction (PCR) and Sanger sequencing.
To compare the prognostic factors and outcomes between solid and cystic GISTs, our surgical pathology database was searched for solid GISTs of comparable external size and resected during the same period. Finally, 200 counterparts were identified and clinicopathological characteristics were compared using Chi square test or non-parametric test. Recurrence-free survival (RFS) was defined as the period between surgical resection and radiologic evidence of recurrence. Survival curves were computed by Kaplan–Meier product limit method with intergroup difference compared by log-rank test. All the tests were two-sided and statistical significance was defined as a P value < 0.05.

Results

Clinical features

Clinical and follow-up data are summarized in Table 1. Of the 20 patients included in this study, 9 were males and 11 females, with a mean age of 61 years (range 31–73 years) at diagnosis. Clinical presentations were known in 16 patients: 5 patients presented with abdominal pain or discomfort, 4 had gastrointestinal bleeding and 2 presented with abdominal mass. The remaining 5 patients were discovered incidentally by physical or imaging examinations for other reasons. The original location of cGISTs were the stomach in ten patients, the jejunum or ileum in seven, the duodenum in two and the omentum in one. Preoperative computed tomography (CT) or magnetic resonance imaging (MRI) reports were available for all patients but did not provide robust evidence for differential diagnosis with other cystic lesions. cGISTs usually demonstrated as an exophytic, well-defined, low-density mass with peripheral enhancement on contrast imaging (Fig. 1a, b). Endoscopic ultrasound (EUS) was performed only in 1 patient and showed a hypoechoic structure arising from the fourth layer of the stomach.
Table 1
Clinical and follow-up information of 20 cases of cGIST
Case
Age (years)/sex
Clinical presentation
Radiologic findings
Location
Treatment
Follow-up (months)
Status
1
66M
NA
Abdominal occupying lesion
Small intestine
Laparotomy
60
ANED
2
71F
Gastrointestinal bleeding
Abdominal occupying lesion
Stomach
Laparotomy
143
ANED
3
55F
Abdominal discomfort
Occupying lesion between liver and stomach
Stomach
Laparotomy
103
ANED
4
42M
NA
Abdominal occupying lesion
Small intestine
Laparotomy
128
ANED
5
50F
Gastrointestinal bleeding
Occupying lesion in the head of pancreas: GISTs?
Duodenum
Laparotomy
128
ANED
6
71F
Incidental findings during examination for cystic disease of kidney
Cystic lesion in the tail of pancreas
Stomach
Laparotomy
123
ANED
7
35F
Abdominal mass
Ovarian cyst
Small intestine
Laparotomy+
Imatinib
39
ANED
8
73F
Incidental finding during examination for appendix mucinous adenocarcinoma
Abdominal malignant tumor: GISTs?
Small intestine
Laparotomy
4
DUD
9
31F
Gastrointestinal bleeding
Abdominal occupying lesion
Stomach
Laparotomy
75
ANED
10
72F
Abdominal mass
Cystic lesion between liver and stomach: GISTs
Stomach
Laparotomy
79
ANED
11
61F
Incidental finding during physical examination
Abdominal cystic lesion: GISTs?
Stomach
Laparotomy
36
ANED
12
62M
Abdominal pain
Abdominal cystic lesion: GISTs?
Stomach
Laparotomy
63
ANED
13
58M
Abdominal discomfort
Abdominal occupying lesion
Stomach
Laparotomy
51
ANED
14
47M
Gastrointestinal bleeding
Abdominal occupying lesion
Duodenum
Laparotomy
59
AWD
15
61F
NA
Abdominal occupying lesion
Stomach
Laparotomy
36
ANED
16
68M
Abdominal discomfort
Abdominal occupying lesion
Stomach
Laparotomy + imatinib
45
ANED
17
57M
Abdominal pain
Diverticulum or GISTs
Small intestine
Laparotomy
19
ANED
18
61F
Incidental finding during physical examination
Ovarian chocolate cyst
Omentum
Laparotomy
30
ANED
19
45M
NA
Abdominal occupying lesion
Small intestine
Laparotomy
66
ANED
20
64M
Incidental finding during physical examination
Pelvic occupying lesion
Small intestine
Laparotomy + imatinib
92
ANED
ANED alive with no evidence of disease, DUD died of unrelated disease, AWD alive with disease

Pathologic findings

Pathologic findings are presented in Table 2. On macroscopic examination, cGISTs appeared as soft, well-circumscribed masses and ranged in size from 7 to 20 cm with a mean of 11.5 cm (Fig. 1c, d). Most of the neoplasms were unilocular (n = 15, 75%). On sectioning, the cut surface varied in color from gray/white to red/brown depending on the degree of hemorrhage. The cystic component made up the vast majority of the masses and was surrounded by neoplastic parenchyma variable in thickness (range 0.1–4.5 cm). Most of the cysts (14/16, 87.5%) were filled by dark bloody fluid and necrotic debris but one contained grey-green turbid, the other light-yellow fluid.
Table 2
Pathologic details of 20 cases of cGIST
Case
Size (cm)
Focality
Cystic contents
Sectioning
Cellularity
Cellular type
Mitosis (/50 HPF)
Cellular atypia
1
15
Unilocular
Dark bloody necrosis debris
NA
Moderate
Epithelioid
0
Mild–moderate
2
12
Unilocular
Dark brown fluid
Grey/white
Moderate
Epithelioid
3
Mild–moderate, partially severe
3
8
Multilocular
Light yellow clear fluid
Grey/red
Moderate
Epithelioid
2
Moderate–severe
4
8
Unilocular
Dark bloody necrotic debris
Grey/white
Moderate
Spindle
0
Mild–moderate
5
10
Unilocular
Dark brown fluid
Grey/white
Moderate
Spindle
1
Mild–moderate, partially severe
6
7
Unilocular
Dark red fluid
Grey/red
Moderate
Spindle
2
Mild–moderate
7
9
Unilocular
Dark bloody necrotic debris
Grey/brown
Moderate
Spindle
2
Mild–moderate
8
8
Unilocular
Grey–green turbid fluid
Grey/red
Moderate
Spindle
1
Mild–moderate
9
8.5
Unilocular
NA
NA
Moderate-Condense
Spindle
1
Moderate
10
16
Unilocular
Grey–red turbid fluid
Grey/white
Moderate
Epithelioid
0
Moderate–severe
11
12.5
Multilocular
NA
Grey/white
Moderate
Spindle
1
Mild–moderate
12
20
Multilocular
Dark red fluid
Grey/white
Moderate
Epithelioid
0
Moderate, partially severe
13
13.5
Unilocular
Brown fluid
Brown
Moderate
Spindle
0
Mild
14
12
Unilocular
Dark red necrotic debris
Grey/white
Moderate
Spindle
2
Mild–moderate
15
13
Unilocular
NA
NA
Moderate
Epithelioid
11
Moderate
16
15
Multilocular
Dark bloody necrotic debris
Grey/white
Moderate
Spindle
1
Mild–moderate
17
9
Unilocular
Dark red necrotic debris
Grey/brown
Moderate
Spindle
0
Moderate–severe
18
8
Unilocular
NA
NA
Moderate
Spindle
8
Moderate
19
14
Unilocular
Dark bloody necrotic debris
NA
Mild
Spindle
1
Moderate
20
13
Multilocular
Dark bloody serous fluid
Grey/red
Moderate
Spindle
0
Mild–moderate
Microscopically, cyst wall was composed of neoplastic spindle (n = 14, 70%)/epithelioid cells (n = 6, 30%) and collagenous fiber with necrotic debris and granulation tissue lining on the inner surface (Fig. 2a). The degree of cellularity varied in different cases and areas but was moderate in general. In rare cases were the cyst wall occupied by collagenous fiber with little cellular component (Fig. 2b). The nuclei were of mild to moderate atypia in 17 (85%) patients and mitotic count was fewer than 5/50 HPF in 18 (90%) patients. In addition, hemosiderin deposits and foamy histocytes were observed in several cases. Within the cysts, there were liquefactive necrosis, hemorrhage and myxoid changes but neoplastic cells were absent. Malignant biological parameters, such as severe nuclear atypia, mucosal infiltration and muscularis propria infiltration, were discovered in nine patients but none involved more than two parameters.
The results of immunohistochemical and mutational analysis are summarized in Table 3. All cases demonstrated positive staining for CD117 (20/20) and negative staining for desmin (20/20) (Fig. 2c). DOG-1 was positive in 14/15 (93.3%) cases, CD34 was positive in 16/19 (84.2%) cases, and SMA was positive in 7/20 (35%) cases. Staining was also performed for several other antibodies, but results were not included because of the limited number of cases. Mutational analyses revealed c-kit or PDGFRα mutations in eleven cases with the remaining nine being wild type for these two mutations. Among all c-kit/PDGFRα mutants, c-kit exon 11 point substitution was the most common (4/11), followed by c-kit exon 11 deletions (2/11), combination of substitution and deletions in c-kit exon 11 (2/11), PDGFRα exon 18 D842V substitution (2/11) and c-kit exon 9 deletions (1/11).
Table 3
Immunohistochemical and mutational analysis of 20 case of cGIST
Case
Malignant parameters
Malignancy
NIH criterion
CD117
Desmin
DOG-1
CD34
SMA
S-100
Mutational status
1
No
Nonmalignant
High
Pos
Neg
NA
Pos
Pos
NA
None in c-kit or PDGFRα
2
Severe nuclear atypia
Low
High
Pos
Neg
Pos
Neg
Pos
Neg
None in c-kit or PDGFRα
3
Infiltration of muscularis propria and vessels
Low
Intermediate
Pos
Neg
Pos
Pos
Neg
Pos
None in c-kit or PDGFRα
4
No
Nonmalignant
High
Pos
Neg
Pos
Pos
Neg
NA
None in c-kit or PDGFRα
5
Severe nuclear atypia
Low
High
Pos
Neg
Pos
Pos
Pos
Pos
c-kit exon 11 Y568S, DEL569-573
6
No
Nonmalignant
Intermediate
Pos
Neg
Pos
Pos
Neg
Neg
c-kit exon 11 V559D
7
No
Nonmalignant
High
Pos
Neg
Pos
Pos
Pos
Neg
None in c-kit or PDGFRα
8
Muscularis propria infiltration
Low
High
Pos
Neg
Pos
Pos
Pos
Pos
c-kit exon 11 L576P
9
No
Nonmalignant
Intermediate
Pos
Neg
NA
Pos
Neg
Neg
c-kit exon 11 DEL559-561
10
Severe nuclear atypia
Low
High
Pos
Neg
Pos
Pos
Neg
NA
c-kit exon 11 V559D
11
No
Nonmalignant
High
Pos
Neg
Pos
Pos
Neg
NA
c-kit exon 11 DEL557-558
12
Severe nuclear atypia
Low
High
Pos
Neg
Pos
Pos
Neg
Neg
PDGFRα exon 18 D842V
13
No
Nonmalignant
High
Pos
Neg
Neg
Pos
Neg
NA
PDGFRα exon 18 D842V
14
Infiltration of mucosa and muscularis propria
Low
High
Pos
Neg
NA
Pos
Pos
Neg
c-kit exon 11 Y553D, DEL554-572
15
No
Nonmalignant
High
Pos
Neg
Pos
NA
Neg
NA
None in c-kit or PDGFRα
16
No
Nonmalignant
High
Pos
Neg
Pos
Pos
Neg
Neg
c-kit exon 11 V559D
17
Severe nuclear atypia
Low
High
Pos
Neg
Pos
Pos
Neg
Neg
c-kit exon 9 INS502-503
18
Severe nuclear atypia
Low
High
Pos
Neg
Pos
Pos
Neg
Neg
None in c-kit or PDGFRα
19
No
Nonmalignant
High
Pos
Neg
NA
Neg
Pos
NA
None in c-kit or PDGFRα
20
No
Nonmalignant
High
Pos
Neg
NA
Neg
Neg
Neg
None in c-kit or PDGFRα
DEL deletion, INS insertion

Treatment and follow-up

All patients underwent laparotomy for surgical resection of cGISTs and no severe complication occurred postoperatively. According to modified National Institute of Health (NIH) criterion, the risk of recurrence was estimated to be intermediate in 3 cases (15%) and high in 17 cases (85%). Based on biological behaviors proposed for evaluating GISTs by our institute, 11 patients were classified as nonmalignant and 9 as low degree of malignancy (Table 3). Adjuvant imatinib was carried out in 3 nonmalignant/high-risk patients (12, 30 and 39 months respectively) and no evidence of recurrence was observed during follow-up (45, 92 and 39 months respectively). For patients without adjuvant imatinib, local recurrence was detected in one (low degree of malignancy/high risk) after a median duration of 63 months (range 4–143 months). The patient underwent a second operation and postoperative imatinib therapy and was progression free after another follow-up of 35 months. One patient died of an unrelated cause 4 months after surgery, whereas all the other patients were alive till the end of follow-up (Table 1).

Comparison between cystic and solid GISTs

The clinicopathological characteristics of cystic GISTs in comparison with their solid counterparts are summarized in Table 4. There was no difference between the two groups in terms of age, sex, tumor location, cellular type, NIH criterion and adjuvant imatinib, except for mitotic index and numbers of biological parameters. Eighteen (90%) cGISTs had a mitotic rate of five or fewer per 50 HPF with a median of 1/50 HPF. The number of malignant biological parameters was none in 9 (45%) cGISTs and 1–2 in 11 (55%). In comparison, 85 of the 200 (42.5%) solid GISTs had mitoses more than 10/50 HPF and 27 (13.5%) had 6 to 10 mitoses per 50 HPF. Solid GISTs were more likely to manifest malignant biological behaviors, with more than 2 parameters in 81 (40.5%) cases and 1–2 parameters in 77 (38.5%) cases. Of 167 solid GISTs with mutational information, 103 (61.7%) harbored mutations in c-kit exon 11, 41 (24.5%) in c-kit exon 9, 1 (0.6%) in c-kit exon 13, 4 (2.4%) in PDGFRα exon 18 D842V and 18 (10.8%) in none. In addition, survival analysis showed that patients with solid GISTs had a significantly worse recurrence-free survival (5-year RFS = 66.1%) than patients with cGISTs (5-year RFS = 94.4%) (Fig. 3).
Table 4
Comparison of clinicopathological characteristics between cystic and solid GISTs
 
Cystic (n = 20)
Solid (n = 200)
P
Age (years)
59.5 (66–73)
60 (19–84)
0.554
Sex
 Male
9
116
0.263
 Female
11
84
Location
 Stomach
10
117
0.462
 Non-stomach
10
83
Cellular type
 Spindle
14
175
0.071
 Non-spindle
6
25
Size (cm)
9 (7–20)
12 (7–20)
0.056
 7–9.9
8
102
0.601
 10–14.9
8
70
 ≥ 15
4
28
Mitotic Index (/50 HPF)
1 (0–11)
8 (1–210)
< 0.001*
 ≤ 5
18
88
0.0004*
 6–10
1
27
 > 10
1
85
NIH criterion
 Intermediate
3
32
1
 High
17
168
Predictive parameters of malignancy
0 (0–2)
2 (1–6)
< 0.001*
 0
11
42
< 0.001*
 1–2
9
77
 > 2
0
81
Mutational status
 c-kit
9
145
< 0.001*
 PDGFRα
2
4
 Neither
9
18
Adjuvant imatinib
 Yes
3
49
0.498
 No
17
151
*P < 0.05 was considered statistically significant

Discussion

Gastrointestinal stromal tumors typically appear as regular, soft, solid masses, varying greatly in size and distributed within and outside the gastrointestinal tract. They are usually well circumscribed and unencapsulated with an endophytic or exophytic growth pattern. Small cystic areas are frequently observed in GISTs with large size, but GISTs rarely manifest predominantly as cystic tumors. Cases of this uncommon form have been described but no retrospective study has been reported in the English literature (Hamza et al. 2016; Okano et al. 2015; Shaikh et al. 2015; Sun et al. 2016; Takahashi et al. 2010; Wang et al. 2017; Zhu et al. 2014). In addition, clinical follow-up information was not available in most cases. In the present study, a cohort of 20 GISTs with extensive cystic change, designated as cGISTs, were evaluated to characterize their clinicopathological features and determine their biological behaviors and prognoses.
Most of the cGISTs, both in our study and prior reports, demonstrated an exophytic growth pattern and lacked pathognomonic signs or symptoms until the late stage of the disease. CT or MRI played an important role in differential diagnosis, providing evidence for original locations while excluding inconsistent neoplasms, but was not potent enough to establish exact diagnosis preoperatively. Although suggested as a useful modality for diagnosing GISTs, EUS-guided FNA appears as a prudent choice for cGISTs due to the possibility of insufficient sample volume and fear of dissemination. For all these reasons, misdiagnoses were easily made preoperatively, which included duplication cysts, mucin-producing tumors, pancreatic pseudocysts, cystic lymphangioma, cystic degeneration of other solid neoplasms, etc. Therefore, successful diagnosis of GISTs necessitates further histological and immunohistochemical examinations. Of note, diagnosis of GISTs should be considered when cystic tumors of unknown origin are encountered in the abdomen.
Pathological analysis of cGISTs revealed similar morphologic and immunohistochemical features with solid ones except that cGISTs were less likely to demonstrate malignant biological behaviors. cGISTs usually compressed or dislocated rather than invaded abutting organs. On microscopy, the mitotic figures were fewer than 5/50 HPF in the majority and malignant parameters such as mucosal invasion and muscularis propria infiltration were less common compared to solid GISTs of similar size (Table 3). Prominent cystic change may be responsible for their indolent behaviors, leaving only a small proportion of viable tumor cells. Cystic change of GISTs takes place in the following situations: (a) primary GISTs with expansive growth pattern, in which cystic structure takes up a large proportion, (b) cystic change induced by rapid growth rate and subsequent necrosis in malignant GISTs, (c) metastatic lesions to liver and pancreas which is cystic in nature, (d) GISTs on treatment with imatinib (Bechtold et al. 2003). Different from cystic change caused by rapid tumor growth, cGISTs are characterized by a relatively even cyst wall and fewer parameters of malignancy. As for their low frequency of c-kit/PDGFRα mutations, lack of enough neoplastic cells may be an explanation, or it is a unique inherent feature associated with their development.
To date, little is known about the cause of predominant cystic change in GISTs and we speculate that exophytic growth pattern with a small area of attachment may restrict blood supply to the tumor. Aggravated by occasional vascular obstruction and incapability of angiogenesis, congestion, hemorrhage, degeneration and liquefactive necrosis occur, resulting in remarkable cystic change. Alternatively, cystic development may be attributed to communication between tumor mass and gastrointestinal tract in certain cases. In three previously reported cases as well as one included in the present study, it is likely that ulceration of gastrointestinal mucosa allows entrance of enteric leakage into tumor mass and induces abscess formation subsequently.
Patients with cGISTs reported in the literature usually underwent surgical resection as the primary treatment but follow-up information was available in fewer than half. In our series, surgery was also the therapy of choice, with three patients receiving additional adjuvant imatinib. Recurrence was detected in one patient without adjuvant imatinib (low degree of malignancy/high risk) and brought under control by surgery and imatinb. In comparison, the 5-year RFS was 66.1% in patients with solid GISTs of similar size. We can say from our experience that surgery is safe and effective for patients with cGISTs. In view of lack of pathology and risk of rupture, preoperative administration of imatinib is not warranted. According to modified NIH criterion, the majority of cGISTs (85% in our series) was stratified as high risk of recurrence and necessitates adjuvant imatinib. However, to avoid excessive administration of imatinib, we recommend meticulous evaluation of malignant parameters prior to decision. For patients with cGISTs classified as nonmalignant, surgical resection alone may achieve long-term recurrence free survival; for patients with cGISTs classified as low degree of malignancy, adjuvant imatinib should be considered but its benefit might be counteracted by low incidence of recurrence.
Several limitations were implicit in our study. First, the cases included were from surgical pathology database and consultation files over a long period of time, resulting in incomplete clinical and pathological information. Second, it was a retrospective study with a limited sample size, which renders the conclusions provisional and warrants further investigations. Third, the magnitude of cystic change was assessed according to radiology reports and/or gross reports. A more qualitative determination of tumor cells may need to perform during radiologic and pathological examinations.
In conclusion, although similar to solid GISTs in terms of morphologic and immunohistochemical features, cGISTs should be considered as a specific subtype of GISTs with relatively indolent behaviors and favorable prognoses. Parameters of malignancy are more applicable than modified NIH criterion in determining recurrence risk and whether to administrate adjuvant imatinib. Future studies analyzing a larger cohort with more detailed information should help shed light on the pathogenesis and long-term survival for these neoplasms.

Acknowledgements

This study was supported by Shanghai Municipal Commission of Health and Family Planning, key developing discipline (NO. 2015ZB0201) and Shanghai Municipal Science and Technology Commission (14411970000).

Compliance with ethical standards

Conflict of interest

All the authors listed have approved the manuscript and no conflict of interest exists

Ethical approval

Study approval was obtained from the institutional review board at Zhongshan Hospital, Fudan University.
OpenAccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://​creativecommons.​org/​licenses/​by/​4.​0/​), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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Metadaten
Titel
Gastrointestinal stromal tumors (GISTs) with remarkable cystic change: a specific subtype of GISTs with relatively indolent behaviors and favorable prognoses
verfasst von
Anwei Xue
Wei Yuan
Xiaodong Gao
Yong Fang
Ping Shu
Chen Xu
He Li
Yifang Xu
Qi Song
Yingyong Hou
Kuntang Shen
Publikationsdatum
28.03.2019
Verlag
Springer Berlin Heidelberg
Erschienen in
Journal of Cancer Research and Clinical Oncology / Ausgabe 6/2019
Print ISSN: 0171-5216
Elektronische ISSN: 1432-1335
DOI
https://doi.org/10.1007/s00432-019-02853-y

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