Primary hepatic gastrointestinal stromal tumor with right adrenal gland invasion

Abstract

Introduction: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors that mainly occur in the gastrointestinal tract. The GISTs that are sporadically reported in extra-gastrointestinal regions are named as extra-gastrointestinal stromal tumors (EGISTs). However, the primary EGISTs that originate from the liver are rare. Patient Concerns: A 64-year-old female presenting with right upper abdominal pain and thirsty for more than 20 days. Diagnosis: A diagnosis of a 15 × 14 × 7 cm liver mass located in the posterior right lobe of liver and spread to the right adrenal gland was confirmed. Pathological results showed that the tumor was mainly composed of epithelial cells and tested positive for CD117 and SDHB (succinate dehydrogenase complex iron sulfur subunit B). The gene mutational analyses for c-Kit and platelet-derived growth factor receptor alpha exons revealed negative results. Fluorescence in situ hybridization of murine double minute 2 produced negative fluorescence results which distinguished it from dedifferentiated liposarcomas. The postoperative gastroduodenal and colorectal endoscopy did not find any neoplastic lesions. To this end, the diagnosis of primary hepatic EGIST of wild type nature was confirmed. Interventions: The patient received right hepatectomy and adrenalectomy, no postoperative chemotherapy was administered. Outcomes: The patient died 11 months after surgery due to tumor metastasis. Conclusion: Primary hepatic EGIST is a rare and complicated disease of liver, a multidisciplinary team is necessary in diagnosis and treatment of primary hepatic EGIST.

Prevalence, diagnosis, and treatment of primary hepatic gastrointestinal stromal tumors

“…Although the incidence is rare, the possibility of GIST should be considered clinically for large space-occupying lesions of the liver. PHGIST is currently not associated with any specific tumor marker, except for a few cases that presented with slightly elevated but within normal range alpha-fetoprotein, CA19-9, and CA125 levels[ 11 ].…”

“…Second, on the fast-forward and fast-reverse phases, the arterial phase was significantly strengthened to a level higher than the normal liver parenchyma. Identification was challenging when equal or low density signals were similar to those for hepatocellular carcinoma, especially when the lesion had a false capsule[ 11 ]. In one case, an entirely cystic tumor was seen, which was difficult to distinguish from a hepatic cyst during the early stage.…”

“…For locally advanced tumors, after an evaluation is performed, obtaining R0 resection should be attempted followed by adjuvant treatment. In one case, a liver mass was located in the right posterior lobe of the liver, which spread to the right adrenal gland[ 11 ]. Because conventional methods could not be used for resection, external hepatectomy and autotransplantation were performed[ 37 ].…”

“…GISTs are classified into four levels according to their size, mitotic image, and location of the lesion using the National Institutes of Health (NIH) classification guideline, which corresponds to different prognoses and preferred treatment methods. According to previous reports, the risk classification and prognosis of EGISTs are significantly worse than those of gastrointestinal tumors[ 10 , 11 ].…”

Correlation analysis of multi-slice computed tomography (MSCT) findings, clinicopathological factors, and prognosis of gastric gastrointestinal stromal tumors

“…However, they are the most frequently diagnosed mesenchymal neoplasms involving the GI tract (1)(2)(3)(4). GISTs originate from mesenchymal pluripotent stem cells, which are programmed to differentiate into interstitial Cajal cells (5). To distinguish these tumors, the term "stromal tumors" was first introduced by Mazur et al (6) in 1983.…”