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Which size is the best cutoff for primary small gastric gastrointestinal stromal tumor?

ABSTRACT: Background:The biological behavior of primary small gastric gastrointestinal stromal tumor (gGIST) is indolent. The cutoff size categorizing small gGIST continues to be controversial. To date, there is no consensus regarding whether it should be 1 cm, 2 cm, or another size. We aimed to find a new cutoff size. Methods:Retrospective clinicopathological and prognosis data of patients with small gGIST from January 1998 to January 2015 were collected among five medical centers in southern China. Tumor size was divided into two groups: <1 cm (Mirco group) and 1-2 cm (Small group). We compared the clinicopathological index and prognosis between these two groups and identified a new cutoff size to define small gGIST. Results:During this 18-year period, there were 276 patients with primary small gGIST treated at these five medical centers. The range of tumor size was 0.2-2.0 cm. The median tumor size was 1.0 cm. The range of the mitotic count was 0-70/50 high power fields (HPFs) with counts ?5/50 HPFs in 259 patients (93.8%), 5< counts ?10/50 HPFs in 7 patients (2.5%), and counts >10/50 HPFs in 10 patients (3.6%). The median follow-up time was 38 months (3-156 months). The 5-year overall survival rate was 98.7% in the entire group. Using Pearson correlation analysis, there was a positive correlation between the mitotic count and tumor size as a continuous variable (r=0.164, P=0.006). There were 137 patients in the Micro group and 139 cases in the Small group. In the Micro group, mitotic counts were ?5/50 HPFs in 134 patients, 5< counts ?10/50 HPFs in 0 patients, and counts >10/50 HPFs in 3 patients; mitotic counts in the Small group were counts ?5/50 HPFs in 125 patients, 5< counts ?10/50 HPFs in 7 patients, >10/50 HPFs in 7 patients. There was a statistically significant difference between these two groups (P=0.002); the Small group had more intermediate/high-risk cases. Using the receiver operating characteristic curve (ROC curve), we observed that 1.15 cm was the new cutoff size to separate low-risk cases and intermediate/high-risk cases (AUC =0.707, P=0.004, sensitivity =0.824, 1-specificity =0.429). Conclusions:Primary small gGIST has a good prognosis; gGIST <1 cm can be regarded as benign tumors that only requires endoscopic ultrasonography (EUS) follow-up. The proportion of potential intermediate/high-risk disease is high for patients with 1-2 cm gGIST. These patients should be treated with caution and the tumors should be resected if necessary. These results indicate that 1.15 cm may be the new cutoff size to separate small gGIST from large gGIST, but further studies are needed for verification.

SUBMITTER: Feng X

PROVIDER: S-EPMC7212102 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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Which size is the best cutoff for primary small gastric gastrointestinal stromal tumor?

Feng Xingyu X Yang Zifeng Z Zhang Peng P Chen Tao T Qiu Haibo H Zhou Zhiwei Z Li Guoxin G Tao Kaixiong K Wang Hui H Li Yong Y

Journal of gastrointestinal oncology 20200401 2

<h4>Background</h4>The biological behavior of primary small gastric gastrointestinal stromal tumor (gGIST) is indolent. The cutoff size categorizing small gGIST continues to be controversial. To date, there is no consensus regarding whether it should be 1 cm, 2 cm, or another size. We aimed to find a new cutoff size.<h4>Methods</h4>Retrospective clinicopathological and prognosis data of patients with small gGIST from January 1998 to January 2015 were collected among five medical centers in south ...[more]

PMID: 32399280

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Project description:BACKGROUND:Gastrointestinal stromal tumor (GIST) is the most common primary mesenchymal neoplasm of the gastrointestinal tract. Mutations of KIT and platelet-derived growth factor receptor alpha have been well characterized in GISTs. Patients with KIT mutations are generally sensitive to treatment with tyrosine kinase inhibitors. However, some patients with GIST, while initially sensitive to TKIs, gain resistance in later stages of treatment. Heterologous rhabdomyomsarcomatous dedifferentiation of advanced GISTs after long-term imatinib mesylate (IM) therapy has been reported. In these cases, the underlying molecular mechanism of tumor progression and transformation is unclear. CASE PRESENTATION:We report one such patient with rhabdomyosarcomatous dedifferentiation of a GIST without metastatic disease after brief 3-month therapy with IM. The tumor was composed of two distinct phenotypes, a CD117 negative region with rhabdomyosarcomatous differentiation directly adjacent to a CD117 positive classic GIST region. Molecular analysis identified the activating KIT exon 11 mutation in both regions, indicating a common origin for both phenotypes. Additionally, the dedifferentiated component contained two synonymous variants in platelet-derived growth factor receptor alpha and KIT. The increased number of synonymous variants in the rhabdomyosarcomatous region may reflect increased genetic instability of this tumor that may have resulted in the loss of CD117 expression in the dedifferentiated component. CONCLUSION:This study adds to the growing consensus that rhabdomyosarcomatous GIST progresses from a common GIST primary tumor. The role of IM in this progression is uncertain; however short duration of IM treatment in this study supports the hypothesis that rhabdomyosarcomatous GIST progression is not a consequence of IM therapy. Furthermore, we provide additional information supporting the observation that CD117 negative rhabdomyosarcomatous transformation maintains the activating KIT variant without KIT expression.

Project description:Gastrointestinal stromal tumors (GIST) are rare mesenchymal smooth muscle sarcomas that can arise anywhere within the gastrointestinal tract. Sporadic mutations within the tyrosine kinase receptors of the interstitial cells of Cajal have been identified as the key molecular step in GIST carcinogenesis. Although many patients are asymptomatic, the most common associated symptoms include: abdominal pain, dyspepsia, gastric outlet obstruction, and anorexia. Rarely, GIST can perforate causing life-threatening hemoperitoneum. Most are ultimately diagnosed on cross-sectional imaging studies (i.e., computed tomography and/or magnetic resonance imaging in combination with upper endoscopy. Endoscopic ultrasonographic localization of these tumors within the smooth muscle layer and acquisition of neoplastic spindle cells harboring mutations in the c-KIT gene is pathognomonic. Curative treatment requires a complete gross resection of the tumor. Both open and minimally invasive operations have been shown to reduce recurrence rates and improve long-term survival. While there is considerable debate over whether GIST can be benign neoplasms, we believe that all GIST have malignant potential, but vary in their propensity to recur after resection and metastasize to distant organ sites. Prognostic factors include location, size (i.e., > 5 cm), grade (> 5-10 mitoses per 50 high power fields and specific mutational events that are still being defined. Adjuvant therapy with tyrosine kinase inhibitors, such as imatinib mesylate, has been shown to reduce the risk of recurrence after one year of therapy. Treatment of locally-advanced or borderline resectable gastric GIST with neoadjuvant imatinib has been shown to induce regression in a minority of patients and stabilization in the majority of cases. This treatment strategy potentially reduces the need for more extensive surgical resections and increases the number of patients eligible for curative therapy. The modern surgical treatment of gastric GIST combines the novel use of targeted therapy and aggressive minimally invasive surgical procedures to provide effective treatment for this lethal, but rare gastrointestinal malignancy.

Project description:Splenosis pertains to the phenomenon wherein a segment of the spleen undergoes detachment and becomes embedded in other anatomical regions subsequent to traumatic rupture or therapeutic resection, and then progressively establishing blood circulation to foster the regeneration of splenic tissue. Existing literature posits that splenosis predominantly manifests within the confines of the abdominal and pelvic cavities. The objective of the current study was to present an uncommon case involving the occurrence of splenosis within the gastric myometrium, thereby contributing to the current knowledge regarding splenosis. A 16-year-old female sought medical assistance owing to recurrent abdominal pain persisting for a duration of six months, and had a history of splenectomy two years prior. Gastroscopy, endoscopic ultrasound and computed tomography (CT) examination collectively identified a lesion in the submucosal prominence of the fundus of the stomach. Initial considerations based on imaging examinations leaned towards a gastrointestinal stromal tumor. Consequently, an endoscopic resection was undertaken. Remarkably, the pathological findings and histochemistry concurred with the alterations associated with ectopic spleen implantation, leading to a stable postoperative course. In conclusion, splenosis denotes the implantation of a segment of the spleen into extraneous anatomical sites, attributable to traumatic rupture or therapeutic resection. The preoperative diagnosis of splenosis can pose a challenge, potentially culminating in unnecessary radical clinical interventions. Therefore, the acquisition of a comprehensive medical history, with a particular focus on surgical and trauma events, emerges as pivotal for an accurate diagnosis. In light of novel diagnostic modalities, the non-invasive technology of nuclear medicine can efficaciously visualize ectopic splenic tissue, thereby averting superfluous surgical procedures. It is both feasible and imperative to implement individualized treatment strategies for patients afflicted with splenosis.

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Which size is the best cutoff for primary small gastric gastrointestinal stromal tumor?

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Which size is the best cutoff for primary small gastric gastrointestinal stromal tumor?

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