Giant gastrointestinal stromal tumor with predominantly cystic changes: a case report and literature review

Wang, Liying; Liu, Libin; Liu, Zhaohui; Tian, Yufei; Zhe, Liu

doi:10.1186/s12957-017-1285-2

Cited by 18 publications

(19 citation statements)

References 12 publications

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“…GISTs typically form a regular, soft, solid mass, but they may also rarely present as cyst‐predominant lesions. GISTs with large cystic changes tend to grow exophytically from the gastrointestinal tract wall, and in some cases can be over 10 cm in diameter . It appears that cystic changes are more frequently observed in high‐grade GISTs, possibly due to the poor blood supply to the aggressively growing tumor.…”

Section: Discussionmentioning

confidence: 99%

“…GISTs with large cystic changes tend to grow exophytically from the gastrointestinal tract wall, and in some cases can be over 10 cm in diameter. [14][15][16] It appears that cystic changes are more frequently observed in high-grade GISTs, possibly due to the poor blood supply to the aggressively growing tumor. It was also reported that liquefactive necrosis, scattered empty spaces with lymphoid infiltration and loose hyaline material, can be observed in nearly half of GIST tumors, but without prognostic significance.…”

Section: Discussionmentioning

confidence: 99%

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Epithelioid variant of gastrointestinal stromal tumor harboring PDGFRA mutation and MLH1 gene alteration: A case report

Kobayashi

Inaguma

Kato

et al. 2019

Pathology International

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Gastrointestinal stromal tumors (GISTs) are the most important and common mesenchymal tumors of the gastrointestinal tract, especially in the stomach. GISTs are usually driven by activating mutations in either KIT or PDGFRA genes. It is known that activating gene mutations predicts, to a certain extent, not only the morphology of the tumor cells but also a response to treatment with tyrosine kinase inhibitors. Here, we present a case of an epithelioid variant of GIST harboring PDGFRA and MLH1 gene alterations in the stomach of a 55‐year‐old Japanese woman. The tumor of 98 mm with multiple cysts showed exophytic growth from the gastric fundus. Histopathologically, it consisted of scattered medium‐sized epithelioid tumor cells in a loose myxoid background. Based on c‐kit and DOG‐1 immunoreactivity and a PDGFRA mutation (p.Trp559_Arg560del), the tumor was diagnosed as an epithelioid variant GIST. Interestingly, it had a gene alteration (p.Met524Ile) in the MLH1 gene of unknown pathogenicity. It was assigned to Group 3a (low risk for malignant behavior). After surgery, the patient has been on imatinib therapy and disease‐free for 10 months.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Epithelioid variant of gastrointestinal stromal tumor harboring PDGFRA mutation and MLH1 gene alteration: A case report

Kobayashi

Inaguma

Kato

et al. 2019

Pathology International

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“…Although GIST is the most common mesenchymal tumor of the gastrointestinal tract, its diagnosis can be challenging [1]. Most atypical presentations are diagnosed only on histopathological basis after surgical resection [5]. The atypical presentation of GIST in our patient, with complete cystic degeneration, is no exception for the same although the diagnosis was made intraoperatively.…”

Section: Discussionmentioning

confidence: 67%

Atypical Presentation of Gastrointestinal Stromal Tumor as Multiple Intra-Abdominal Cysts: A Case Report

Gurram

Gnanasekaran

Midha

et al. 2020

Cureus

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Gastrointestinal stromal tumors (GISTs) are the most common nonepithelial solid neoplasms involving the alimentary tract. We report a case of cystic GIST with multiple cystic metastases. A 61-year-old man presented with upper abdominal pain for two months. Further evaluation revealed a large intra-abdominal cyst in the lesser sac and another cyst over the segment VII of the liver on imaging. Multiple intra-abdominal hydatidoses were suspected based on the imaging and its endemic nature in the geographical area. However, the hydatid serology was normal. In view of hemorrhagic cyst fluid, an intraoperative frozen biopsy of the cyst wall was done, which revealed features suspicious of a mesenchymal tumor. Sleeve gastrectomy with enbloc excision of the gastric cyst, excision of the hepatic cyst, and complete excision of multiple other intra-abdominal cysts were performed considering GIST as a possibility. Histology revealed a clear cell variant of GIST. Gastric GISTs primarily presenting as multiple intraabdominal cysts and of clear cell histological variants had never been reported in the literature. The patient was started on imatinib, and he has shown no evidence of recurrence after 12 months of follow-up. A high index of suspicion, intraoperative frozen section, meticulous surgery, and immunohistochemistry are all crucial for the effective management of atypical cases. GIST may be considered as a part of differential diagnosis in clinical scenarios with multiple intraabdominal cysts, especially in the equivocal setting.

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“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

Xue

Yuan

Gao

et al. 2019

J Cancer Res Clin Oncol

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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.

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Giant gastrointestinal stromal tumor with predominantly cystic changes: a case report and literature review

Cited by 18 publications

References 12 publications

Epithelioid variant of gastrointestinal stromal tumor harboring PDGFRA mutation and MLH1 gene alteration: A case report

Epithelioid variant of gastrointestinal stromal tumor harboring PDGFRA mutation and MLH1 gene alteration: A case report

Atypical Presentation of Gastrointestinal Stromal Tumor as Multiple Intra-Abdominal Cysts: A Case Report

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

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