Retroperitoneal gastrointestinal stromal tumor: A case report and literature review.
Ontology highlight
ABSTRACT:
SUBMITTER: Nguyen TTMH
PROVIDER: S-EPMC7415774 | biostudies-literature | 2020 Oct
REPOSITORIES: biostudies-literature
Publications
Retroperitoneal gastrointestinal stromal tumor: A case report and literature review.
Nguyen Thai Thi My Hanh TTMH Dang Cong Thuan CT Pham Anh Vu AV Nguyen Thanh Thao TT
Radiology case reports 20200807 10
Retroperitoneal gastrointestinal tumor is the rarest subtype among 3 subtypes of extragastrointestinal tumors, which are uncommon stromal tumors. We herein report a case of a 55-year-old man with retroperitoneal gastrointestinal tumor detected by magnetic resonance imaging and confirmed by postoperative histology and immunohistochemistry. ...[more]
Similar Datasets
Project description:INTRODUCTION:Synchronous occurrence of different types of neoplasms is not very frequent, representing around 6% of all cases of cancer. Usually there is a lack of information on how to treat these patients, especially when both types of cancers are also uncommon. No cases of synchronous gallbladder adenocarcinoma and gastric gastrointestinal stromal tumor have been published before. PRESENTATION OF CASE:We present the case, management and follow-up, of a 66-year-old female with incidental diagnosis of a pT2NxMx gallbladder adenocarcinoma after elective cholecystectomy that latter, during staging, was also diagnosed with GIST. Total gastrectomy, wedge resection of the liver and lymphadenectomy were performed due to the new findings. Adjuvant chemotherapy for 36 months was indicated. After 16 months of the treatment she has no signs of recurrence. DISCUSSION:Gastrointestinal stromal tumors (GISTs) had a turnaround in the end of the 20th century after the introduction tyrosine-kinase inhibitor to the adjuvant treatment and now the trend is to extend it up to 36 months in selected patients. Gallbladder adenocarcinoma is an uncommon cancer but the incidental diagnosis is increasing with the popularity of laparoscopic cholecystectomy and, thus, specific management should be offered for these patients, what frequently includes a complementary surgery. Although, GISTs may be associated with another synchronous tumor in 20% of the cases, the simultaneous occurrence with gallbladder cancer is incredibly rare. CONCLUSION:Simultaneous occurrence of gastric GIST and gallbladder adenocarcinoma has not been reported before and, thus, any information about it may help in the management of those patients.
| S-EPMC6393670 | biostudies-literature
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.
| S-EPMC10964733 | biostudies-literature
Project description:BackgroundMost of gastrointestinal stromal tumors (GISTs) are driven by mutations in the KIT/PDGFRA genes and can benefit from TKIs treatment. However, a small subset of GIST (10%-15%) are called "wild-type" GISTs due to the lack of these mutations. Theoretically, they would not benefit from TKIs treatment and may even develop resistance. Therefore, this unexpected response may challenge inherent perceptions. Herein, we present a case of giant wild-type GIST exhibiting an unexpected response to imatinib(IM), followed by laparoscopic surgical resection. Subsequently, potential underlying mechanisms are discussed.Case descriptionThis case describes a 57-year-old man who presented with abdominal pain for two weeks. CT revealed a massive lesion near the splenic hilum along the greater curvature of the stomach, concurrently involving the splenic hilar vessels and surrounding lymph nodes. Ultrasound-guided fine needle aspiration biopsy confirmed it is a mesenchymal spindle cell tumor,GIST. Due to the enormous volume and local invasion, neoadjuvant chemotherapy was initially considered. After 6 months of IM 400 mg/d, CT imaging revealed marked changes in tumor heterogeneity and a significant reduction in volume. Subsequently, laparoscopic surgical resection was performed. Postoperative pathological examination, immunohistochemistry, and genetic testing collectively confirmed it is a wild-type GIST.The patient recovered well and was discharged on the 6th day after surgery, with continued oral IM(400 mg/d) after discharge. No recurrence was observed during follow-up until the publication of this report.ConclusionThis unexpected response suggests that wild-type GISTs may benefit from TKIs treatment, and the potential mechanisms warrant further investigation. Additionally, true wild-type GIST may not be discerned due to current limitations of Next-Generation Sequencing(NGS). Therefore, for advanced/high-risk GIST, additional genetic analysis can be performed after negative NGS results.
| S-EPMC10864548 | biostudies-literature
Project description:BackgroundGastrointestinal stromal tumor (GIST) can arise anyplace along the gastrointestinal (GI) tract. The uncommon tumor location in groin area is rarely reported.Case presentationWe herein reported a metastasized case presented as GI hemorrhage complicated with indirect hernia, and underwent tumor cytoreduction, herniorrhaphy and chemotherapy for jejunal GIST. The case was described consecutively based on the process of surgical management, with a good follow-up result. A literature review by searching similar case reports from two national medical databases was performed to summarize clinical features of such unusual presentation of GIST, which included hernia characteristics, short- and long-term outcomes of this disease. It showed GIST presenting as groin hernia was rarely reported and all available 11 cases suggested a primary tumor and required both tumor resection and hernia repair. The long-term results indicated 64.3% overall survival at 5 years after the incidental diagnosis.ConclusionsInguinal hernia is an extremely rare presentation of GIST, with limited case reports available in the literature. A radical involving tumor resection plus hernia repair is an optimal surgical approach for such uncommon condition. An adjuvant medication mounting on mutated KIT gene should be strictly followed for high risk cases.
| S-EPMC7931599 | biostudies-literature
Project description:Intussusception mostly occurs in childhood and is rare in adults. Although intussusception can occur in any part of the gastrointestinal tract, gastroduodenal intussusception caused by a gastric tumor is relatively uncommon in clinical practice. A PubMed search identified 24 published cases of gastroduodenal intussusception caused by gastric gastrointestinal stromal tumor (GIST); however, it is possible that we missed other cases not included in PubMed. Here we report a case of gastroduodenal intussusception caused by gastric GIST in an 85-year-old man. He came to the hospital because of recurrent black stools. Plain computed tomography (CT) scan indicated a mass in the gastric antrum, with slight enhancement in the arterial phase on enhanced CT scan. He was diagnosed with GIST. In addition, images indicated that the mass overlapped into the duodenum, and gastroduodenal intussusception was thus considered. Gastroscopy showed a huge mass in the gastric body. According to the gastroscopy and CT results, gastroduodenal intussusception caused by a gastric tumor was considered. The patient underwent complete surgical removal, which revealed a mass originating from the gastric antrum and overlapping into the duodenum. The postoperative pathological diagnosis was intermediate-risk gastric GIST. The patient was followed up for 4 months without tumor recurrence.
| S-EPMC9150259 | biostudies-literature
Project description:Malignant triton tumor (MTT) is a rare subtype of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. MTTs are solid tumors commonly located in the head, neck, extremities, and trunk. The presence of this tumor in an infant's retroperitoneum is extremely rare, and prompted the authors to report this case with reference to existing literature.
| S-EPMC7475308 | biostudies-literature
Project description:Background: Microcystic stromal tumor is a recently described subtype of ovarian tumor characterized by microcystic pattern and diffuse immunoreactivity for CD10, vimentin, and ?-catenin and negative for EMA. However, its diagnostic criterion and standard treatment remain unclear. Case presentation: We report a rare case of a left side microcystic stromal tumor with diameter about 7 cm in a 25-year-old female and summarize all cases of MCST reported in this study. The present patient underwent left ovarian tumor resection. Generally, the tumor was solid and cystic mixed. Immunohistochemically, the tumor was expressed CD10, WT1, cyclin D1 and vimentin, and nuclear immunoreactivity for ?-catenin but negative for ?-inhibin, calretinin, CK AE1/AE3, PLAP, SALL-4, CK7, P53, EMA, CD99, AFP, desmin, CgA, E-cadherin, and melanA. Conclusion: Unilateral ovary, solid-cystic, and a larger than 4-8 cm pelvic mass without serious abdominal pain are its clinical features. The immunophenotype of vimentin+/CD10+/WT-1+/?-catenin+(nuclei)/cyclin D1+ is supportive of diagnosis. For these patients, unilateral oophorectomy dissection could be selected.
| S-EPMC7051939 | biostudies-literature
Project description:BACKGROUND: Retroperitoneal Schwannomas are uncommonly found in the retroperitoneum and few of them show malignant transformation and invasion. Local recurrence are common in malignant Schwannomas with very few reports of tumor distinct differentiation at recurrences. CASE PRESENTATION: We report here a rare case of retroperitoneal schwannoma with multiple origins from retroperitoneum and pelvic wall. Pathological examination confirmed the case as a schwannoma with malignant transformation. Radical dissection of the tumors along with the sacrifice of adjacent sigmoid colon and left kidney failed to provide a cure for this patient. Due to tumor recurrence, a second and a third surgery of radical excision were performed 6 months and 17 months later after the first surgery, respectively. Histopathologic analysis identified that the recurrent tumors were different from the original schwannoma because of their smooth muscle-like differentiation. CONCLUSION: Malignant schwannomas are uncommon sarcomas with a high incidence of local recurrence. Distinct immunohistochemical staining results of the tumors at recurrence indicate their potential of smooth-muscle like differentiation. Radical excision of the tumors may provide benefit for their local recurrences.
| S-EPMC2927596 | biostudies-literature
Project description:Gastrointestinal stromal tumors are the most common mesenchymal tumors of the digestive tract, most of which are sporadic, and familial GISTs with germline mutations are rarely seen. Here, we report a 26-year-old female with a germline p. W557R mutation in exon 11 of the KIT gene. The proband and her father and sister presented with multifocal GIST and pigmented nevi. All 3 patients underwent surgery and imatinib therapy. To date, only 49 kindreds with germline KIT mutations and 6 kindreds with germline PDGFRA mutations have been reported. Summarizing the reported kindreds, the majority of familial GISTs manifest as multiple primary GISTs complicated with special clinical manifestations, including cutaneous hyperpigmentation, dysphagia, mastocytosis, inflammatory fibrous polyps, and large hands. Familial GISTs are generally thought to exhibit TKI sensitivity similar to that of sporadic GISTs with the same mutation.
| S-EPMC10712720 | biostudies-literature
Project description:The dedifferentiation of the gastrointestinal stromal tumors (GISTs) has been reported in a small number of cases, usually under the pressure of the tyrosine kinase inhibitor (TKI) treatment. Herein, we described a de novo dedifferentiated GIST with the SDH deficiency in a 32-year-old Chinese woman. The tumor was located on the lesser curvature of the gastric antrum, measuring 4.1x9.1 cm2. Microscopically, the tumor was composed of 2 distinct morphological populations, mild epithelioid cells arranged in the multinodular growth pattern and hyperchromatic spindle cells arranged in the fascicular or sheet-like architecture. The two zones showed different immunophenotypes. The former proved to be an epithelioid GIST with the positive expression for C-KIT, DOG-1, and CD34, and the latter expressed the CKpan and P53, but negative for the C-KIT, DOG-1, and CD34. However, the SDHB staining was negative in both areas. Genetically, the next-generation sequencing (NGS) analysis showed the SDHC mutation (p.S48*) in both components and the MDM2 amplification was only in the spindle cell area. The lesion was diagnosed as the SDH-deficient GIST with the epithelial cell dedifferentiation. We proposed that the P53 associated gene alteration or other alternative escape mechanisms for the KIT-independent signaling pathways might play a role in the dedifferentiation.
| S-EPMC10540086 | biostudies-literature