Aggressive Fibromatosis of the Left Mesocolon Mimicking a Gastrointestinal Stromal Tumor: A Case Report
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SUBMITTER: Abu-Jeyyab M
PROVIDER: S-EPMC10601818 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
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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:IntroductionGastrointestinal tumor (GIT) is an uncommon gastrointestinal tumor most commonly arising in the stomach. Duodenum is an uncommon site accounting for only about 3-5% of cases.Case presentationIn this case report, we present a case of high-risk duodenal GIST and review its management strategies.DiscussionAn abdominal mass and gastrointestinal (GI) bleeding are its usual presentation, however it may be lost among the long list of differentials of an abdominal mass and GI bleeding, if a high index of suspicion is not maintained. Surgery, with or without tyrosine kinase inhibitors like imatinib mesylate, has been the cornerstone in management of GIST.ConclusionThis case underlined the importance of duodenal GIST as a cause of GI bleed and abdominal mass as well as shone light upon historical developments, current updates and managements of GISTs.
| S-EPMC9577536 | biostudies-literature
Project description:Rationale Aggressive fibromatosis is a rare and locally infiltrative monoclonal fibroblastic proliferation with lack of metastatic potential. We describe a rare case of intra-abdominal aggressive fibromatosis on young female with hyperemesis. Patient concerns A 23-year-old female was admitted with hyperemesis and loss of weight. Diagnoses According to imaging findings and immunohistology findings, a diagnosis of intra-abdominal aggressive fibromatosis was formulated. Outcomes After the surgery, no evidence of local recurrence was noted during the 6 months of follow-up. Lessons AF may explain why pregnant women may have severe hyperemesis.
| S-EPMC9989300 | biostudies-literature
Project description:BackgroundGastrointestinal stromal tumor (GIST) is the most common primary mesenchymal tumor. The most common metastasis sites are the liver and the surface of the peritoneum. In this study, we present a case of orbital GIST metastasis.Case presentationA 43-year-old woman who had a history of small intestinal stromal tumor 4 years ago suffered GIST metastasis to the left orbit. MRI confirmed the presence of a lacrimal gland lesion with isointense on T1 and hyperintense on T2 weighted images. Histopathology analysis of the tumor showed predominantly spindle or oval cells with nuclear pleomorphism and increased mitoses. The tumor also stained positive for c-KIT (CD117) upon immunochemistry, confirming the diagnosis.ConclusionsGISTs typically occur as sporadic solitary tumors, and their common metastasis sites are the liver and the surface of the peritoneum. Orbital involvement is extremely rare. The orbital GIST metastatic tumor has special imaging properties, as evidenced by histopathology, immunochemistry, and magnetic resonance imaging (MRI).
| S-EPMC6699248 | biostudies-literature
Project description: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.
| S-EPMC7415774 | biostudies-literature
Project description:IntroductionGastrointestinal Stromal Tumors (GIST) are rare mesenchymal neoplasm of gastrointestinal tract. Stomach is the most common site affected by GIST compared to other places in gastrointestinal track. The coexistence of GIST with another malignancy represents a rare phenomenon with few literature reported.Case presentationWe present here 65 years old patient with stomach GIST and synchronous pancreatic adenocarcionoma discovered during surgery for suspected pancreatic mucinious cystadenoma. Distal pancreaticosplenectomy with excision of GIST Tumor & wedge resection of stomach was done. Histopathological examination of resected specimens reported the margins are clear.DiscussionIn this article we discuss on the option of systemic therapy versus upfront surgery and their outcome benefit based on literature review.ConclusionThe coexistence of GIST with pancreatic adenocarcinoma is a rare condition. High clinical analysis needed during laparotomy for GIST to detect a synchronous tumor. In a case of GIST the surgeon should recognize the possibility of another tumor with different histological origin. Surgical excision is the mainstay of therapy and it has proven to be curative for our patient. . Due to its rare occurrence and limited literature further studies has to be done on GIST with other synchronous tumor to help the surgeon to manage the patient optimally.
| S-EPMC9142392 | biostudies-literature
Project description:BackgroundGastrointestinal 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 presentationWe 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.ConclusionThis 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.
| S-EPMC6743131 | biostudies-literature
Project description:We present an extremely rare report on gastric inlet obstruction due to giant abdominal aortic aneurysm. Despite the rare occurrence of this manifestation, it should be considered in the differential diagnosis of gastric inlet obstructions.
| S-EPMC8142391 | biostudies-literature
Project description:BACKGROUND:Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract and liver and peritoneum are the main sites of recurrence. Ovarian metastases from GIST are very rare. CASE PRESENTATION:A 50 years-old woman was found to have a pelvic mass on transvaginal ultrasound (TV-US) and computed tomography (CT)-scan, considered as a right ovarian mass. The patient underwent surgical abdominal exploration that showed an ileal mass, a normal right ovary and an irregular and vascularized surface of the left ovary. A segmental ileal resection and an ileal anastomosis were performed. Frozen section showed a GIST and surgery was completed with hysterectomy, bilateral salpingo-oophorectomy, pelvic peritonectomy, peritoneal washing and Burch procedure. The histological examination confirmed an ileal GIST with ovarian metastases, harboring in both sites of disease a KIT exon 11 deletion. CONCLUSIONS:Ovarian localizations, as far as rare, can be a clinical finding in case of ileal GIST patients, and both gynecologists, pathologists and medical oncologists should be able to recognize them.
| S-EPMC5857138 | biostudies-literature
Project description:BackgroundGastrointestinal stromal tumors rarely occur in children, but when they do, their biological behavior and histopathological patterns differ from those of adults.Case presentationA 13-year-old boy with a gastrointestinal stromal tumor was characterized by a rare genetic mutation. The patient complained of "fatigue with intermittent abdominal pain for 1 month". According to the preoperative imaging examination, gastroscopy, and gastroscopic biopsy, the patient was diagnosed with a gastric stromal tumor. Postoperative pathology showed that the tumor cells were fusiform and ovoid, and mitotic figures were easily seen. Immunohistochemistry revealed that the tumor was S-100(+), SOX10(-), CD34(+), SMA(partially+), DOG-1(+), CD117(+), KI-67 (positive for 20% + of the subjects and 40% + of the hotspots), and SDHB(-). Genetic tests showed missense mutations in ALK and TSC1. With surgical treatment, the tumor was completely removed. The patient recovered well and was discharged on the ninth day after the operation. He is currently under follow-up.ConclusionsIn this case involving a patient with a gastrointestinal stromal tumor, immunohistochemistry indicated that the tumor was an "SDH-deficient type", and gene detection showed no KIT or PDGFRA mutation but rare ALK and TSC1 mutations, which adds to the knowledge of the types of gene mutations in children with gastrointestinal stromal tumors.
| S-EPMC8059153 | biostudies-literature