Mesenchymal tumors

间充质肿瘤
  • 文章类型: Case Reports
    尽管它们相对罕见,胃肠道间质瘤(GIST)是胃肠道(GI)中最常见的间质瘤。这里,我们描述了一例罕见的62岁高血压女性,表现为腹痛和明显的肿块,根据放射学发现,最初怀疑是胰腺假性囊肿。手术切除后的组织病理学(HPE)检查显示出源自胃的大的囊性病变。以恶性上皮样GIST为特征。根据这些发现并考虑到患者的症状,决定将病人提前邮寄,没有术前活检研究的开放式手术探查。考虑到鉴别诊断,冻结的科室设施处于待机状态。由于冷冻切片显示胃部GIST,决定进行胃大部切除术,其次是胃空肠吻合术(GJ)和空肠吻合术(JJ)。此外,通过非解剖楔形切除术处理了与肝左叶粘附的囊肿部分。免疫组织化学(IHC)分析显示分化簇117(CD117)阳性,分化簇34(CD34)阴性,Desmin,并发现胃肠道间质瘤1(DOG-1)。肿瘤表现出侵袭性特征,包括高有丝分裂活性,即,>5/10高功率场(hpf),出血区,并渗入肝实质.然后患者接受基于伊马替尼的辅助化疗,并保持严格的随访。
    Despite their relative rarity, gastrointestinal stromal tumors (GIST) are the most common type of mesenchymal tumor in the gastrointestinal (GI) tract. Here, we describe a rare case of a 62-year-old hypertensive female presenting with abdominal pain and a palpable mass, initially suspected to be a pancreatic pseudocyst based on radiological findings. Subsequent histopathological (HPE) examination following surgical resection revealed a large cystic lesion originating from the stomach, characterized as a malignant epithelioid GIST. Based on these findings and taking into consideration the symptomatology of the patient, the decision was made to post the patient for an upfront, open surgical exploration without pre-operative biopsy studies. Frozen section facilities were kept on standby considering the differential diagnosis. Since the frozen section revealed a gastric GIST, a decision was made to perform subtotal gastrectomy, followed by gastrojejunostomy (GJ) and jejunojejunostomy (JJ). In addition, the part of the cyst adherent to the left lobe of the liver was dealt with with a non-anatomical wedge resection. Immunohistochemical (IHC) analysis showed positivity for Cluster of Differentiation 117 (CD117) with negativity for Cluster of Differentiation 34 (CD34), Desmin, and Discovered On Gastrointestinal Stromal Tumors 1 (DOG-1). The tumor exhibited aggressive features, including high mitotic activity, i.e., >5/10 high power field (hpf), hemorrhagic areas, and infiltration into the liver parenchyma. The patient then received adjuvant imatinib-based chemotherapy and was maintained on strict follow-up.
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  • 文章类型: Case Reports
    孤立性纤维性肿瘤是罕见的间充质肿瘤,通常发生在胸膜。子宫颈孤立性纤维瘤并不常见。我们报告了第一例患者,该患者因阴道壁腺癌接受了全子宫切除术,并被发现在子宫颈旁子宫并发孤立性纤维瘤。
    一名51岁女性因接触性出血入院。妇科检查发现阴道壁上有3.0×1.0厘米的结节,阴道镜活检显示阴道壁腺癌。在推荐的分期检查之后,患者接受了全子宫切除术,双附件切除术,盆腔淋巴结清扫术,阴道壁切除术.手术期间,在左侧宫颈旁区的肿块中间发现约2×2cm的结节。随后的术后组织病理学检查证实宫颈孤立性纤维瘤伴阴道壁腺癌。患者住院后随访46个月,未观察到复发或远处转移。
    在极少数情况下,孤立性纤维瘤可在宫颈或阴道壁形成大肿块。在手术前和手术中,它们很容易被误诊为良性或恶性宫颈肿瘤。术后病理及免疫组化有助于诊断。大多数孤立性纤维性宫颈肿瘤是良性的,偶尔有低恶性潜能,手术治疗是可行和有效的。
    UNASSIGNED: Solitary fibrous tumors are rare mesenchymal tumors that typically occur in the pleura. Solitary fibrous tumors of the uterine cervix are uncommon. We report the first case of a patient who underwent total hysterectomy for vaginal wall adenocarcinoma and was found to have a concurrent solitary fibrous tumor in the paracervical-uterus.
    UNASSIGNED: A 51-year-old woman was admitted to our hospital due to contact bleeding. A gynecological examination revealed nodules of 3.0 × 1.0 cm on the vaginal wall, and a colposcopy with biopsy revealed adenocarcinoma of the vaginal wall. After the recommended staging examinations, the patient underwent total hysterectomy, double adnexectomy, pelvic lymph node dissection, and vaginal wall resection. During surgery, a nodule measuring approximately 2 × 2 cm was found in the middle of the mass in the left paracervical region. Subsequent postoperative histopathological examination confirmed an solitary fibrous tumor of the uterine cervix with adenocarcinoma of the vaginal wall. The patient was followed up for 46 months after hospitalization, and no recurrence or distant metastases were observed.
    UNASSIGNED: In rare cases, solitary fibrous tumors may form large masses in the cervical or vaginal wall. They can easily be misdiagnosed as benign or malignant cervical tumors before and during surgery. Postoperative pathology and immunohistochemistry are helpful for diagnosis. Most solitary fibrous cervical tumors are benign, occasionally with low malignant potential, and surgical treatment is feasible and effective.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GIST)是胃肠道(GI)最常见的间质瘤,通常起源于Cajal的间质细胞。临床表现根据其大小和形状而变化,但很少表现为可触及的腹部肿块。胰腺假性囊肿是慢性胰腺炎的常见并发症,其特征是由纤维和肉芽组织的非上皮化壁包围的液体聚集。患者可能会出现非特异性症状,如腹痛,恶心,和呕吐,他们通常有急性胰腺炎病史。小假性囊肿常自发消退,但较大的往往会出现症状,并可能导致并发症。在同一患者中很少发现胃的GIST和胰腺的假性囊肿。我们介绍了一名72岁男性的巨大GIST和胰腺假性囊肿的独特病例,该男性正在经历腹痛和腹胀。影像学显示一个源自胃后壁的巨大病变,类似于假性囊肿,与胰腺体相邻的明显囊性病变。在手术探查期间,发现了两种病理的复杂相互作用,需要全面的切除方法。成功的结果突出了在这种罕见情况下仔细评估和个性化管理的重要性。
    Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal (GI) tract, typically originating from the interstitial cells of Cajal. The clinical presentations are variable according to their size and shape but rarely present as a palpable abdominal mass. Pancreatic pseudocysts are common complications of chronic pancreatitis characterized by fluid collections surrounded by a non-epithelialized wall of fibrous and granulation tissue. Patients may present with non-specific symptoms like abdominal pain, nausea, and vomiting and they generally have a history of acute pancreatitis. Small pseudocysts often resolve spontaneously, but larger ones often become symptomatic and may lead to complications. It is rare to find both a GIST of the stomach and a pseudocyst of the pancreas in the same patient. We present a unique case of a giant GIST and a pancreatic pseudocyst in a 72-year-old male who was experiencing abdominal pain and distension. Imaging revealed a massive lesion originating from the posterior gastric wall, which resembled a pseudocyst, along with a distinct cystic lesion adjacent to the pancreatic body. During surgical exploration, a complex interplay of both pathologies was discovered, requiring a comprehensive resection approach. The successful outcome highlights the importance of careful evaluation and personalized management in such rare cases.
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  • 文章类型: Case Reports
    我们介绍了一个巨大的孤立性纤维瘤(SFT)的病例报告,并回顾了文献,并讨论了其生物学特征和诊断。一名43岁的男子因腹痛和腹胀来到我们的急诊科,经历了两天的演变。对比增强计算机断层扫描(CT)显示,位于胰头的界限良好的半固体肿块(12cmx10cmx12cm)。经内镜超声引导下经十二指肠肿瘤活检细针穿刺获得的组织学诊断为增生的短梭形细胞,提示低度的间充质瘤形成。我们进行了Whipple手术技术。切除肿瘤的组织病理学研究证实,组织中的梭形细胞增殖,在10个高功率场(HPF)中观察到一个有丝分裂图。CD34和STAT-6的免疫染色呈阳性。组织学诊断为恶性胰腺SFT。在手术后的六个月里,病人没有反复发作的疾病。术前诊断困难,需要全面的证据,包括临床,免疫组织化学,和组织学特征。由于目前没有公认的最佳做法,我们建议全手术切除和仔细的临床监测。
    We present a case report of a giant solitary fibrous tumor (SFT) with a review of the literature and discuss its biological features and diagnosis. A 43-year-old man presented to our emergency department with abdominal pain and distension with an evolution of two days. Contrast-enhanced computed tomography (CT) showed a large, well-circumscribed semisolid mass (12 cm x 10 cm x 12 cm) localized in the pancreatic head. The histological diagnosis obtained by endoscopic ultrasound-guided trans-duodenal tumor biopsy with fine-needle aspiration showed proliferating short spindle-shaped cells, suggesting a mesenchymal neoplasia of low grade. We proceeded to a Whipple surgical technique. The histopathological study of the resected tumor confirmed proliferating spindle-shaped cells in the tissue, and one mitotic figure was observed in 10 high-power fields (HPFs). Immunostaining was positive for CD34 and STAT-6. The histological diagnosis was a malignant pancreatic SFT. In the six months posterior to the surgical procedure, the patient has been free of recurrent disease. Preoperative diagnosis is difficult and requires comprehensive evidence including clinical, immunohistochemistry, and histological features. Since there are currently no recognized best practices, we advise total surgical excision and careful clinical monitoring.
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  • 文章类型: Case Reports
    纤维瘤,也被称为侵袭性纤维瘤病,代表一种罕见的成纤维细胞增殖形式。这些肿瘤可能出现在整个身体的任何肌肉筋膜结构中。由于几个独特的特征,它们被归类为良性的:组织学上,它们表现出规则的有丝分裂活性,并且没有转移潜力。计算机断层扫描(CT)仍然是精确诊断的最终方式,强烈建议手术切除。此帐户详细介绍了位于31岁女性患者前腹壁的硬纤维状肿瘤的表现,该患者明显缺乏任何先前的手术干预措施。手术干预需要切除肿瘤并随后使用聚丙烯网片重建腹壁。术后,病人在三天后从医疗机构获释,没有经历术后并发症。随后是六个月的间隔,没有任何不良事件。
    Desmoid tumors, also referred to as aggressive fibromatosis, represent an uncommon form of fibroblastic proliferation. These neoplasms may arise within any musculoaponeurotic structure throughout the body. They are classified as benign due to several distinctive features: histologically, they exhibit regular mitotic activity and are devoid of metastatic potential. Computed tomography (CT) remains the definitive modality for precise diagnosis, and surgical excision is strongly advised. This account details the manifestation of a desmoid tumor located in the anterior abdominal wall of a 31-year-old female patient who notably lacks any prior surgical interventions. The surgical intervention entailed the excision of the neoplasm and subsequent reconstruction of the abdominal wall utilizing a polypropylene mesh. Postoperatively, the patient was released from the medical facility after a period of three days, having experienced no post-surgical complications. This was followed by a six-month interval free of any adverse events.
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  • 文章类型: Case Reports
    该病例报告介绍了一名42岁女性的临床细节,该女性以前没有医学问题,该女性表现为以黑素性粪便为特征的上消化道出血(UGIB)。初步检查显示轻度贫血,随后的内窥镜检查发现4厘米的粘膜下胃肿块显示近期出血指标。随后的手术病理证实2级高级别胃肠道间质瘤(GIST),复发风险增加。这种情况的意义在于强调在UGIB的鉴别诊断中考虑GIST的必要性。特别是在没有可识别的危险因素的中年人中,例如近期或慢性非甾体抗炎药(NSAID)的使用,消化性溃疡疾病,或报警症状。早期发现和及时手术干预在提高患者预后方面至关重要。虽然完全切除是治疗的基石,建议高危患者使用伊马替尼辅助治疗,以降低复发风险.
    This case report presents the clinical details of a 42-year-old female without previous medical issues who presented with upper gastrointestinal bleeding (UGIB) characterized by melanotic stools. Initial examination revealed mild anemia and subsequent endoscopy identified a 4 cm submucosal gastric mass displaying recent bleeding indicators. Subsequent surgical pathology confirmed a high-grade gastrointestinal stromal tumor (GIST) of grade 2 with a heightened risk of recurrence. The significance of this case lies in underscoring the necessity of considering GIST in the differential diagnosis of UGIB, particularly among middle-aged individuals with no identifiable risk factors such as recent or chronic non-steroidal anti-inflammatory drug (NSAID) use, peptic ulcer disease, or alarm symptoms. Early detection and prompt surgical intervention assume paramount importance in enhancing patient outcomes. While complete resection stands as the cornerstone of treatment, adjuvant imatinib therapy is recommended for high-risk patients to mitigate the risk of recurrence.
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  • 文章类型: Case Reports
    血管黏液瘤是一种罕见的间质瘤,发生在盆腔和会阴区域。膀胱血管黏液瘤诊断困难,因为它缺乏典型的体征和症状,依赖免疫化学。我们介绍了一名53岁患者在超声检查中偶然发现膀胱肿瘤的情况。经尿道完全切除后,病理报告导致血管粘液瘤诊断。6周后,继发性TURB,连同CT尿路图显示没有残余体积的证据。经尿道电切术是一种安全有效的治疗膀胱血管黏液瘤的方法。
    Angiomyxoma is a rare mesenchymal tumor arising in the pelvic and perineal regions. Diagnosis of urinary bladder angiomyxoma is difficult, as it lacks typical signs and symptoms, and relies on immunochemistry. We present the case of a 53 year old patient presenting with an incidental finding of bladder tumor during an ultrasound. After a complete transurethral resection was performed, the pathology report led to angiomyxoma diagnosis. After 6 weeks a secondary TURB, along with a CT urogram showed no evidence of residual volume. Transurethral resection seems to be a safe and effective treatment of urinary bladder angiomyxoma.
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  • 文章类型: Case Reports
    血管周围上皮样细胞肿瘤,最著名的是PEComas,是极不常见的间充质肿瘤PEComas的病因尚不确定,其临床表现通常是良性的。PEComas缺乏独特的症状表现;因此,这些肿瘤的诊断主要依靠病理检查。这些肿瘤对黑素细胞标记物具有非常独特的免疫反应性,这对它们的鉴定至关重要。由于这些肿瘤的罕见性和缺乏独特的疾病表现,我们讨论了一名56岁女性患者的影像学和病理结果的诊断相关性,该患者诊断为肺的右中叶PEComa.
    Perivascular epithelioid cell tumors, best known as PEComas, are extremely uncommon mesenchymal tumors The etiology of PEComas remains unestablished and its clinical presentation is usually benign. PEComas lack a distinctive symptomatic presentation; thus, the diagnosis of these tumors relies mainly on pathological examinations. These neoplasms have a very distinct immunoreactivity for melanocytic markers critical for their identification. Due to the rarity of these tumors and lack of a distinct disease presentation, we discuss the diagnostic relevance of imaging and pathologic findings in a 56-year-old woman diagnosed with a PEComa in the right middle lobe of the lung.
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  • 文章类型: Journal Article
    背景:胃肠道平滑肌肉瘤极为罕见。我们报告了一例空肠平滑肌肉瘤并在Treitz角度下发生肠肠套叠的病例,该病例通过腹腔镜切除术成功治疗,然后使用三角形吻合术进行体内重建。
    方法:一名54岁男子因疲劳和食欲不振被转诊至我院。血液检查显示贫血。肠镜检查和随后使用氨基三磺酸葡胺钠的肠造影显示,空肠中容易出血性肿瘤(直径10cm),刚好超出Treitz的角度。对比增强计算机断层扫描显示空肠空肠肠套叠。活检标本的组织病理学检查显示平滑肌肉瘤。进行腹腔镜切除肿瘤而不减少肠套叠。在本例中,近端肠的切除线非常靠近Treitz的韧带。使用三角形吻合术完成了体内空肠空肠吻合术,其中在Treitz韧带周围最小动员后,在近端和远端空肠的后壁之间进行吻合。病人的术后过程是顺利的,他在术后10天出院。术后2年内未观察到复发。
    结论:我们介绍了一个病例,其中成功进行了完全腹腔镜手术,治疗了位于Treitz角度的平滑肌肉瘤,并伴有空肠空肠肠套叠。
    BACKGROUND: A leiomyosarcoma of the gastrointestinal tract is extremely rare. We report a case of jejunal leiomyosarcoma with intestinal intussusception at the angle of Treitz that was successfully treated with laparoscopic resection followed by intracorporeal reconstruction using a delta-shaped anastomosis.
    METHODS: A 54-year-old man was referred to our hospital due to fatigue and loss of appetite. Blood tests showed anemia. Enteroscopy and subsequent enterography using meglumine sodium amidotrizoate showed easily hemorrhagic tumor (10 cm in diameter) in the jejunum just beyond the angle of Treitz. Contrast-enhanced computed tomography revealed jejunojejunal intussusception. Histopathological examination of a biopsy specimen revealed a leiomyosarcoma. Laparoscopic resection of the tumor without reduction of the intussusception was performed. The resected line of the proximal intestine was very close to the ligament of Treitz in the present case. Intracorporeal jejunojejunostomy was completed using a delta-shaped anastomosis, wherein anastomosis was performed between the posterior walls of the proximal and distal jejunums after minimal mobilization around the ligament of Treitz. The patient\'s postoperative course was uneventful, and he was discharged at 10 days postoperatively. No recurrence has been observed within 2 years after surgery.
    CONCLUSIONS: We present a case in which a totally laparoscopic surgery for leiomyosarcoma located at the angle of Treitz with jejunojejunal intussusception was performed successfully.
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  • 文章类型: Case Reports
    Retroperitoneal liposarcomas (RPL) are rare mesenchymal tumours with an annual incidence of 2.7 cases per million. Well-differentiated liposarcomas (WDLs) and dedifferentiated liposarcomas (DDLs) are the most common subtype. WDLs are widely known to be low-grade tumours that are less likely to metastasise and easily resected. In contrast, DDLs are high-grade aggressive metastatic tumours with mortality rates between 50% and 70%. We present an unusual case of a 47-year-old male with a background of hypertension presenting with cardiac-sounding chest pain. Initially managed as an acute coronary syndrome (ACS), he eventually underwent a CT scan which revealed a 20x18x17cm retroperitoneal complex mass with possible infiltrates to the posterior wall of the greater curvature of the stomach. Ultrasound-guided biopsy and subsequent histopathology analysis revealed DDL consistent with MDM2 amplification. This case highlights how RPL can present with diagnostic difficulties. Multidisciplinary input from haematology, surgery and specialist teams is vital to optimise patient management.
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