• extra-gastrointestinal stromal tumors
• imatinib
• long-term survival
• molecular-targeted therapeutic strategies
• primary hepatic GIST
• sunitinib

• National Natural Science Foundation of China

Primary hepatic stromal tumours are very rare and there are only sporadic reports in the literature. Due to the lack of specificity in their clinical manifestations and imaging features, these tumours are easily misdiagnosed. This current report presents a case of primary liver stromal tumour that was misdiagnosed as a liver cyst. The 72-year-old male patient was admitted to the hospital due to right upper abdomen fullness and discomfort for more than 2 weeks. Colour Doppler ultrasonography and enhanced computed tomography examinations revealed a cystic mass in the right lobe of the liver. The preoperative diagnosis was a liver cyst and the laparoscopic fenestration was performed. The pathological examination demonstrated that it was a primary hepatic stromal tumour. Gastroenteroscopy was performed postoperatively and no lesions were found in the gastrointestinal tract. Imatinib mesylate was given orally as the salvage therapy and a radical operation was planned at the patient's request. This current case serves as a reminder that clinicians should consider the possibility that it could be a primary hepatic stromal tumour rather than a hepatic cyst. A multidisciplinary team is necessary for the diagnosis and treatment of patients with a primary hepatic stromal tumour.

Primary extra-gastrointestinal stromal tumors (E-GIST) of the liver are rare. The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect. Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care. However, under specific circumstances, a cytoreductive approach may represent a therapeutic option. We describe herein the case of an 84-year-old woman who presented with a tender, protruding epigastric mass. Abdominal computed tomography scan revealed a large, heterogeneous mass located across segments III, IV, V, and VIII of the liver. The initial approach was transarterial embolization of the tumor, which elicited no appreciable response. Considering the large size and central location of the tumor and the advanced age of the patient, non-anatomic complete resection was indicated. Due to substantial intraoperative bleeding and hemodynamic instability, only a near-complete resection could be achieved. Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver. Considering the risk/benefit ratio for therapeutic options, debulking surgery may represent a strategy to control pain and prolong survival.

Here, we present a case report of a patient diagnosed with E-GIST primary of the liver, which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.

E-GIST primary of the liver is a rare conditional, the treatment is with systemic therapy and total resection surgery. However, a cytoreductive surgery will be necessary when a complete resection is no possible.

• Extra-gastrointestinal stromal tumor
• Primary gastrointestinal stromal tumor of the liver
• Cytoreductive surgery
• Debulking surgery
• Case report

Gastrointestinal stromal tumors (GIST), which is the most common mesenchymal tumor of the digestive tract, account for 1%-3% of gastrointestinal tumors. Primary stromal tumors outside the gastrointestinal tract are collectively referred to as extra GISTs, and stromal tumors in different regions often have different prognoses. A primary hepatic GIST is a rare tumor with an unknown origin, which may be related to interstitial Cajal-like cells. Although primary hepatic GIST has certain characteristics on imaging, it lacks specific symptoms and signs; thus, the final diagnosis depends on pathological and genetic evidence. This review summarizes all cases of primary hepatic GIST described in the literature and comprehensively analyzes the detailed clinical data of all patients. In terms of treatment, local resection alone or with adjuvant therapy was the prioritized choice to obtain better disease-free survival and longer survival time. For advanced unresectable cases, imatinib mesylate was applied as the first-line chemotherapy agent. Moreover, transcatheter arterial chemoembolization, radiofrequency ablation, and microwave ablation were shown to improve overall survival for selected patients. Liver transplantation was a final treatment option after resistance to chemotherapy developed.

• Extra gastrointestinal stromal tumor
• Treatment
• Liver transplant
• Review

• Antineoplastic Agents/therapeutic use
• Benzamides/therapeutic use
• Carcinoma, Hepatocellular
• Chemoembolization, Therapeutic
• Gastrointestinal Neoplasms/diagnosis
• Gastrointestinal Neoplasms/epidemiology
• Gastrointestinal Neoplasms/therapy
• Gastrointestinal Stromal Tumors/diagnosis
• Gastrointestinal Stromal Tumors/epidemiology
• Gastrointestinal Stromal Tumors/therapy
• Humans
• Liver Neoplasms/epidemiology
• Liver Neoplasms/therapy
• Piperazines/therapeutic use
• Prevalence
• Pyrimidines

• dogs
• life span
• liver tumor
• metastatic tumor
• primary tumor

• chemotherapy
• gastrointestinal stromal tumors
• gene mutation analysis
• pathology

• Abdominal Pain/diagnosis
• Adrenal Gland Neoplasms/diagnostic imaging
• Adrenal Gland Neoplasms/pathology
• Adrenal Gland Neoplasms/secondary
• Adrenal Gland Neoplasms/surgery
• Adrenalectomy/methods
• Fatal Outcome
• Female
• Gastrointestinal Stromal Tumors/diagnostic imaging
• Gastrointestinal Stromal Tumors/pathology
• Gastrointestinal Stromal Tumors/surgery
• Gastrointestinal Stromal Tumors/ultrastructure
• Hepatectomy/methods
• Humans
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/pathology
• Liver Neoplasms/surgery
• Middle Aged
• Neoplasm Invasiveness
• Thirst
• Tomography, X-Ray Computed/methods

Recurrence and metastasis are the most important factors affecting the quality of life and survival rate of patients with gastrointestinal stromal tumors (GISTs). Accurate preoperative determination of the malignant degree of GISTs and the development of a reasonable treatment plan can effectively reduce the recurrence rate. CT is currently considered the preferred imaging modality for initial assessment. Until now, there have only been a few studies investigating the relationship between CT features and recurrence of GISTs. However, the value of CT features in prognostic assessment is still unclear. In this study, we attempted to investigate the prognostic significance of CT features and the Ki67 index in GISTs.

We retrospectively analyzed the clinicopathological and imaging data for 151 patients with a histopathological diagnosis of GIST who had received contrast‐enhanced CT examination and surgical resection at XinHua Hospital from October 2008 to December 2015 or Sir Run Run Shaw Hospital in 2017. Then, we explored the correlation among CT features, the Ki67 index, and risk stratification of GISTs. The correlation among CT features, the Ki67 index, and risk stratification was mainly analyzed using the Spearman rank correlation.

The incidence of high‐risk disease or metastasis was clearly higher in the group with Ki67 > 5% than that in the group with Ki67 ≤ 5% (P < 0.001). The Ki67 index was positively correlated with risk stratification (r = 0.558) or mitotic index (r = 0.619). CT imaging features including size, contour, and margin of the tumor were associated with the Ki67 index (r = 0.332, 0.333, and 0.302, respectively). The multivariate logistic regression analysis revealed that the tumor size [P = 0.043 Exp (B) = 1.150] and the presence of ulceration [P = 0.011, Exp (B) = 3.669] were effective variables in distinguishing between the groups with Ki67 ≤ 5% and >5%. The presence of necrosis or cystic degeneration, tumor contour, tumor margin, and pattern of enhancement were associated with risk stratification (r = 0.530, 0.501, 0.419, and 0.447, respectively).

Our findings suggest that the Ki67 index is an effective complementation in predicting the prognosis of GISTs, and CT features including size, contour, and margin of the tumor, presence of necrosis or cystic degeneration, and pattern of enhancement provide evidence to support the importance of preoperative assessment.

• CT
• Ki67 index
• gastrointestinal stromal tumors
• the risk stratification

• Extra-gastrointestinal stromal tumors
• GIST
• Liver sarcoma
• Liver tumors