liposarcoma

脂肪肉瘤
  • 文章类型: Case Reports
    精索恶性肿瘤是一种罕见的形式,精索脂肪肉瘤甚至是一种罕见的疾病。脂肪肉瘤通常是缓慢进展的,非招标,由于与筋膜室相符,形状可变的界限良好。我们报告一例65岁男性,阴囊有两个月的最初招标和后来的非招标肿块,在睾丸上方。超声检查显示右侧睾丸上极有6x3x3厘米的右侧腹股沟中段肿块,右侧睾丸周围有少量液体。右腹股沟肿块的细针抽吸细胞学(FNAC)显示梭形细胞肿瘤。该患者接受了右腹股沟根治性睾丸切除术,并局部广泛切除了精索起源的肉瘤。最终组织病理学证实去分化脂肪肉瘤。没有提供辅助治疗,对患者进行了监测。随访10个月以上未发现局部复发,区域或非区域淋巴结,或全身转移。
    Spermatic cord malignancies are a scarce modality and liposarcoma of spermatic cord is even a rarer condition encountered. Liposarcoma is usually a slowly progressive, non-tender, well circumscribed mass of variable shapes owing to conformity to fascial compartments. We are reporting a case of 65-year-old male, with a two-month history of initially tender and later non-tender mass in the scrotum, above the testis. Ultrasonography showed a right mid inguinal mass measuring 6x3x3 cm at the superior pole of the right testis and small fluid around the right testis. Fine needle aspiration cytology (FNAC) of the right inguinal mass revealed a spindle cell neoplasm. The patient underwent right inguinal radical orchiectomy with local wide excision of the sarcoma of the spermatic cord origin. Final histopathology confirmed dedifferentiated liposarcoma. No adjuvant treatment was offered and the patient was put on surveillance. Follow-up of more than 10 months has not revealed any local recurrence, regional or non-regional lymph nodes, or systemic metastasis.
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  • 文章类型: Journal Article
    脂肪肉瘤(LPS)是一种罕见的软组织肉瘤,从脂肪细胞的分化发展,通常发生在下肢和腹膜后间隙。根据其组织学形态和分子变化,LPS可分为各种亚型,每个都表现出不同的生物学行为。治疗期间,特别是在腹膜后产生的LPS,初次手术的范围和质量至关重要.治疗策略必须针对LPS的特定类型进行调整。在过去的几十年里,LPS的治疗经历了许多进步,随着新的治疗方法,如靶向药物和免疫疗法不断涌现。本文综述了其生物学特性,分子改变,以及各种LPS亚型的手术和药物治疗,目的是增强临床医生的理解并强调个体化精准治疗的重要性。随着对LPS生物学特性和分子改变的深入了解,未来的治疗趋势可能更侧重于制定个性化治疗计划,以更好地解决各种类型的LPS.
    Liposarcoma (LPS) is a rare soft tissue sarcoma that develops from the differentiation of fat cells, typically occurring in the lower extremities and retroperitoneal space. Depending on its histological morphology and molecular changes, LPS can be divided into various subtypes, each exhibiting distinct biological behaviors. During treatment, especially for LPS arising in the retroperitoneum, the extent and quality of the initial surgery are critically important. Treatment strategies must be tailored to the specific type of LPS. Over the past few decades, the treatment of LPS has undergone numerous advancements, with new therapeutic approaches such as targeted drugs and immunotherapies continually emerging. This paper reviews the biological characteristics, molecular alterations, as well as surgical and pharmacological treatments of various LPS subtypes, with the aim of enhancing clinicians\' understanding and emphasizing the importance of individualized precision therapy. With a deeper understanding of the biological characteristics and molecular alterations of LPS, future treatment trends are likely to focus more on developing personalized treatment plans to better address the various types of LPS.
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  • 文章类型: Case Reports
    脂肪肉瘤是一种起源于脂肪细胞的罕见软组织肿瘤。脂肪肉瘤的确切原因尚不清楚,症状因肿瘤的位置而异。一名49岁的男子出现在急诊室,抱怨上腹部疼痛辐射到背部和右上象限。横断面成像显示上腹部大肿块,最初被认为是十二指肠引起的胃肠道间质瘤(GIST)。患者接受了整块切除,并计划进行辅助化疗。随后,检查多个组织样本,最终诊断为去分化脂肪肉瘤。患者最终出现多次复发,并接受了再切除手术和三种不同的化疗方案。鉴于这种疾病的稀有性,没有标准化的治疗计划,强调需要更多的病例报告/系列和试验,以扩大我们对这种疾病的理解。
    Liposarcoma is a rare soft-tissue neoplasm originating from adipocytes. The exact cause of liposarcoma is unknown and symptoms vary depending on the tumor\'s location. A 49-year-old man presented to the emergency room complaining of epigastric pain radiating to the back and right upper quadrant. Cross-sectional imaging revealed a large upper abdominal mass that was thought to be a gastrointestinal stromal tumor (GIST) arising from the duodenum at first. The patient underwent en-bloc resection of the mass and was planned for adjuvant chemotherapy. Subsequently, multiple tissue samples were examined, leading to the final diagnosis of de-differentiated liposarcoma. The patient eventually developed multiple recurrences and was subjected to re-resection surgeries and three different chemotherapy regimens. Given the rarity of the disease, no standardized therapy plan is available, highlighting the need for more case reports/series and trials to broaden our understanding of this disease.
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  • 文章类型: Case Reports
    去分化原发性纵隔脂肪肉瘤占所有脂肪肉瘤病理的不到1%。我们报道了一名82岁男性患有进行性吞咽困难的病例,呼吸急促,和发音障碍,为期2个月。胸部CT增强扫描显示,巨大的后纵隔不均匀增强肿块延伸到颈部后部软组织,邻接双侧颈动脉,并使气管和食道移位。为我们的患者选择的治疗是手术切除,然后进行辅助放射治疗,解决了患者的症状。通过诊断获得的见解,管理,我们的病人的治疗可以用来处理这种类型的罕见肿瘤。
    De-differentiated primary mediastinal liposarcomas account for less than 1% of all liposarcoma pathology. We report the case of an 82-year-old male who was suffering from progressive dysphagia, shortness of breath, and dysphonia for a period of 2 months. A CT scan of the chest with contrast revealed a large heterogeneously enhancing posterior mediastinal mass extending into the posterior soft tissues of the neck, abutting bilateral carotid arteries, and displacing the trachea and esophagus. Treatment chosen for our patient was surgical resection followed by adjuvant radiation therapy which resolved the patient\'s presenting symptoms. The insights gained through the diagnosis, management, and treatment of our patient can be utilized to approach this type of rare neoplasm.
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    文章类型: Journal Article
    BACKGROUND: conventional parosteal osteosarcoma is an uncommon malignant bone tumor, comprising 4% of all osteosarcomas. Although rare, parosteal osteosarcoma is the most common type of osteosarcoma of the bone surface. We present the clinical, histological and imaging characteristics of a rare histologic variant of a parosteal osteosarcoma, review the literature and emphasize the importance of radio-pathological correlation as well as the interpretation of a representative biopsy in order to obtain the correct diagnosis.
    METHODS: a 36-year old woman began her condition one year prior to admission to the hospital with increased volume in the left knee and pain. Image studies showed a juxtacortical heterogeneous tumor localized on the posterior surface of the distal femoral metaphysis. An incisional biopsy was performed, with the diagnosis of a Parosteal Osteosarcoma and a wide surgical resection was undertaken. According to the findings of the surgical specimen, the diagnosis of a Parosteal Osteosarcoma with low grade chondrosarcoma and liposarcoma components was made. The knowledge of this rare parosteal osteosarcoma variant can lead the orthopedic oncologists to avoid overlooking the adipose component and provide adequate surgical margins.
    CONCLUSIONS: we present the clinical, histological and imaging characteristics of a Parosteal Osteosarcoma with low grade liposarcoma and chondrosarcoma components.
    UNASSIGNED: el osteosarcoma parosteal convencional es un tumor óseo maligno poco común, que comprende el 4% de todos los osteosarcomas. Aunque es poco común, el osteosarcoma parosteal es el tipo más común de osteosarcoma de la superficie ósea. Presentamos las características clínicas, histológicas y de imagen de una variante histológica rara de un osteosarcoma parosteal, revisamos la literatura y enfatizamos la importancia de la correlación radio-patológica, así como la interpretación de una biopsia representativa para obtener el diagnóstico correcto.
    UNASSIGNED: mujer de 36 años inició su cuadro un año antes de su ingreso al hospital con aumento de volumen en rodilla izquierda y dolor. Los estudios de imagen mostraron una tumoración heterogénea yuxtacortical localizada en la superficie posterior de la metáfisis femoral distal. Se realizó biopsia incisional, con diagnóstico de osteosarcoma parosteal y se realizó resección quirúrgica amplia. De acuerdo con los hallazgos de la pieza quirúrgica se realizó el diagnóstico de osteosarcoma parosteal con componentes de condrosarcoma y liposarcoma de bajo grado. El conocimiento de esta rara variante de osteosarcoma parosteal puede llevar a los ortopedistas oncólogos a considerar otros componentes y proporcionar márgenes quirúrgicos adecuados.
    UNASSIGNED: presentamos las características clínicas, histológicas y de imagen de un osteosarcoma parosteal con componentes de liposarcoma y condrosarcoma de bajo grado.
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  • 文章类型: Case Reports
    胃脂肪肉瘤(GL)极为罕见,根治性手术已成为常规治疗方法,即使是小肿瘤。腹腔镜楔形切除术已在世界范围内被报道用于胃的上皮下肿瘤。
    患者是一名无症状的63岁男性,表现为胃上皮下肿瘤。食管胃十二指肠镜检查显示,位于幽门上方胃窦后壁的3厘米溃疡软性肿瘤。进行了两次术前活检,结果为恶性肿瘤阴性。动态计算机断层扫描显示35×35mm明确的幽门肿块和脂肪密度。尽管肿瘤的位置很困难,进行功能保留手术.手术是通过带有四个套管针的腹腔镜方法开始的。大网膜解剖后,较大的曲率和胃的后壁暴露。在胃窦前壁进行了胃造口术。由于很难确定肿瘤的位置,进行了小型剖腹手术。在评估幽门和部分参数后,通过胃造口术取出肿瘤,并用线性吻合器切除。5天后患者出院,无并发症。组织学诊断为分化良好的脂肪肉瘤。切除边缘清晰。肿瘤细胞的MDM2检测为阴性。无辅助治疗。病人还活着,没有复发。
    尽管它很少,胃脂肪肉瘤在黏膜下肿瘤的鉴别诊断中值得重视.主要的诊断方法是组织学,手术是常规治疗方法,但尚未达成共识。即使位置靠近幽门,微创楔形切除术也可能是合适的治疗方法。需要多中心研究才能在这种病理的管理中获得更好的结果。
    UNASSIGNED: Gastric liposarcoma (GL) is extremely rare and radical surgery has been the conventional treatment, even in small tumors. Laparoscopic wedge resection has been reported worldwide for subepithelial tumors of the stomach.
    UNASSIGNED: The patient was an asymptomatic 63-year-old man presenting with a subepithelial gastric tumor. The esophagogastroduodenoscopy showed a 3 cm ulcerated soft tumor located in the posterior wall of the antrum just above the pylorus. Two preoperative biopsies were performed with a negative result for malignant neoplasm. Dynamic computerized tomography revealed 35 × 35 mm well-defined pyloric mass with fat density. Despite the difficult location of the tumor, function-preserving surgery was performed. Surgery was initiated by a laparoscopic approach with four trocars. After the dissection of the greater omentum, the greater curvature and the posterior wall of the stomach were exposed. A gastrostomy was performed in the anterior wall of the antrum. Due to the difficulty in identifying the tumor location, a mini-laparotomy was conducted. After assessing the pylorus and section parameters, the tumor was extracted by gastrostomy and resected with a linear stapler. The patient was discharged after five days with no complications. The histological diagnosis was a well-differentiated liposarcoma. Resection margins were clear. The tumor cells tested negative for MDM2. No adjuvant therapy was indicated. The patient is alive without recurrence.
    UNASSIGNED: Despite its rarity, gastric liposarcoma should be respected for differential diagnosis in submucosal tumors. The main diagnostic method is histological, and surgery is the conventional treatment without yet having a consensus. Minimally invasive wedge resection might be a suitable treatment even if the location is close to the pylorus. Multicenter studies are required to obtain better results in the management of this pathology.
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  • 文章类型: Journal Article
    腹膜后肉瘤(RPS)的术前活检可实现适当的多学科治疗计划。对1990年至2022年6月的文献进行了系统回顾,使用人口,干预,比较和结局模型,以评估术前活检与未活检的局部复发和总生存期。在筛选的3192项研究中,纳入5项回顾性队列研究.三次报告活检针道播种,只有一项研究报告活检部位复发2%。两个发现局部复发没有显着差异,一个发现在没有进行活检的人中5年局部复发率更高。三项研究报告了总生存率,包括一个倾向匹配的,没有显示总生存期的差异。总之,RPS术前芯针活检与局部复发或不良生存结局无关.
    Preoperative biopsy for retroperitoneal sarcoma (RPS) enables appropriate multidisciplinary treatment planning. A systematic review of literature from 1990 to June 2022 was conducted using the population, intervention, comparison and outcome model to evaluate the local recurrence and overall survival of preoperative biopsy compared to those that had not. Of 3192 studies screened, five retrospective cohort studies were identified. Three reported on biopsy needle tract seeding, with only one study reporting biopsy site recurrence of 2 %. Two found no significant difference in local recurrence and one found higher 5-year local recurrence rates in those who had not been biopsied. Three studies reported overall survival, including one with propensity matching, did not show a difference in overall survival. In conclusion, preoperative core needle biopsy of RPS is not associated with increased local recurrence or adverse survival outcomes.
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  • 文章类型: Review
    原发性肝脂肪肉瘤是一种极其罕见的源自脂肪细胞的恶性肿瘤,是间质肿瘤组的一部分。我们介绍了一名43岁的西班牙裔男性患者,患有多形性肝脂肪肉瘤且没有MDM2基因扩增。手术后两年六个月,患者无症状。本病例是该实体的第一份报告,其p16,p53,S100,波形蛋白的免疫组织化学检测呈阳性,并且没有MDM2基因扩增。
    Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification.
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  • 文章类型: Review
    背景:腹膜后分化脂肪肉瘤(RPDDL)是一种罕见的恶性肿瘤,由于在腹膜后腔中具有足够的空间并且在疾病的早期阶段缺乏临床表现,因此通常多年未被发现。外科手术通常作为治疗的首选。然而,手术后容易局部复发,导致不良预后。我们的目的是从新病例中吸取有益的教训,为疾病的管理提供一些经验。
    方法:我们描述了一名55岁的男性患者,他因3周的左腰持续隐痛而入院。在体格检查中触诊了左上腹部的大肿块。此外,影像学检查显示肿块直径约21厘米,一些邻近的重要器官被侵入,这给完成手术切除带来了很大的挑战。
    方法:术后病理证实肿块为RPDDL,浸润胰腺等周围重要结构,脾,脾左肾上腺,左肾,和有肿瘤栓子的脉管系统。
    方法:我们的多学科团队进行了肿块的手术切除。患者术后1个月接受化疗。
    结果:化疗的效果似乎不令人满意。手术后约2个月考虑肿瘤的局部多灶性复发。最后,他放弃了任何治疗,死于这种疾病。
    结论:定期体检和超声筛查可以尽早发现疾病,特别是对于60-70岁的高危人群,应该推广。不完全切除,血管浸润,中断术后治疗可能导致不良预后。因此,我们认为这种疾病的患者可能受益于完整的手术切除和不间断的辅助治疗.
    BACKGROUND: Retroperitoneal dedifferentiated liposarcoma (RPDDL) is an uncommon malignancy, which often remains undetected for many years due to having adequate space in the retroperitoneal cavity and lacking clinical manifestations in the early stage of the disease. Surgical procedure is usually used as the first choice for treatment. However, it is prone to local recurrence after the operation, resulting in an unfavorable prognosis. Our aim is to draw useful lessons from the new case and provide some experience for management of the disease.
    METHODS: We describe a 55-year-old male patient who was admitted for a 3-week history of persistent dull ache of the left waist. A large mass of the left upper abdomen was palpated in physical examination. Moreover, the imaging examination revealed that the diameter of the mass was about 21 cm, and some adjacent vital organs were invaded, which brought great challenges to complete surgical resection.
    METHODS: The postoperative pathological results confirmed that the mass was RPDDL with invasion of the surrounding vital structures including pancreas, spleen, left adrenal gland, left kidney, and vasculature with tumor emboli.
    METHODS: Surgical resection of the mass was performed by our multidisciplinary team. The patient received chemotherapy 1 month after surgery.
    RESULTS: The effect of chemotherapy seemed to be unsatisfactory. Local multifocal recurrence of the tumor was considered about 2 months after surgery. Finally, he gave up any treatments and died of the disease.
    CONCLUSIONS: Regular physical examination and ultrasound screening may detect the disease as early as possible, especially for high-risk group aged 60 to 70, which should be popularized. Incomplete resection, vascular invasion, and interruption of postoperative treatment may lead to an unfavorable prognosis. Therefore, we think that patients with the disease may benefit from complete surgical resection and uninterrupted adjuvant therapy.
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  • 文章类型: Case Reports
    背景:睾丸旁脂肪肉瘤是一种罕见的泌尿生殖系统恶性肿瘤。这种恶性肿瘤占所有脂肪肉瘤的不到12%。已报道约200例睾丸旁脂肪肉瘤。巨大的睾丸旁脂肪肉瘤大小超过10厘米是罕见的,只有少数病例报告。由于这种疾病的罕见,没有关于其发病率的标准化指南,诊断,复发,和治疗。
    方法:一名73岁的男性3年前出现无痛的左阴囊肿块来医院就诊。病人做了左阴囊的超声检查,这证明左睾丸有大量的肿块和血管过度。腹肾盂CT显示实性囊肿肿块,尺寸±16,6×9,6×18,2厘米,分叶状,对比增强,没有转移性疾病的迹象。患者行根治性睾丸切除术,无任何并发症。组织病理学和免疫组织化学检查(波形蛋白,MDM2,danCDK4)显示高分化的脂肪肉瘤。
    根治性睾丸切除术是最好的治疗方法。辅助化疗和放疗的获益仍无定论。该患者在手术后随访了两年,未发现复发的肿块和转移。高分化型具有更好的预后,但如果不完全切除,则局部复发的发生率很高。结果表明,这种方法产生了极好的结果,没有任何复发。
    结论:巨大的睾丸旁脂肪肉瘤是一种可以通过根治性治疗的罕见疾病。长期随访对于观察这种恶性肿瘤的复发很重要。
    BACKGROUND: Paratesticular liposarcoma is a rare variant of genitourinary malignancy. This malignancy accounts for less than 12 % of all liposarcomas. Approximately 200 cases of paratesticular liposarcoma have been reported. Giant paratesticular liposarcoma sizing over 10 cm is rarer, with only a few reported cases. Due to the rarity of this disease, there are no standardized guidelines regarding its incidence, diagnostic, recurrence, and treatment.
    METHODS: A 73-year-old male came to the hospital with a painless left scrotal mass three years ago. The patient had an ultrasound examination of the left scrotal, which proved a solid mass and hypervascular on the left testicular. Abdominopelvic computed tomography (CT) showed a solid-cyst masses, size ±16,6 × 9,6 × 18,2 cm, lobulated, contrast enhancement with no sign of metastatic disease. The patient had radical orchiectomy without any complications. Histopathological and immunohistochemistry examination (Vimentin, MDM2, dan CDK4) showed well-differentiated liposarcoma.
    UNASSIGNED: Radical orchiectomy is the best curative therapy. Adjuvant chemotherapy and radiotherapy benefit is still inconclusive. The patient had followed up for two years after surgery found no recurrent mass and metastatic. The well-differentiated type has a better prognosis but has a high incidence of local recurrence if incompletely excised. The result showed that this approach produces excellent outcomes without any relapse.
    CONCLUSIONS: Giant Paratesticular Liposarcoma is a rare condition that can be managed by radical. Long-term follow-up is importance to observe the relapse of this malignancy.
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