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
    背景:黏液性脂肪肉瘤黏液样脂肪肉瘤是一种来源于血管周围未分化基质细胞的恶性黏液样软组织肿瘤,下体腔和肌肉间隙,由前脂肪细胞分化为成熟细胞的不同阶段的细胞组成。在极少数情况下,它可能会从脂肪瘤恶性肿瘤改变。主要表现为无痛肿块,相对缓慢的增长,课程可以持续几十年,脂肪肉瘤在人群中的患病率为14%至18%,主要是成年人,男性患病率高于女性,但不重要。主要的好头发部分是大腿,有粘液性,高分化型,去分化类型,多态类型。临床诊断困难,早期没有明显的症状,所以诊断要结合B超,MRI,CT,以及其他辅助考试。金标准是病理检查。2023年12月,我们部门收治了一名腹部黏液性肿块患者。报告如下。
    方法:脂肪肉瘤会转移吗?手术后需要化疗吗?将来会复发吗?手术后的生存期是多少?
    方法:粘液性脂肪肉瘤。
    方法:手术切除肉瘤。
    结果:结节样本为33*28*13厘米,有完整的胶囊,灰色和黄色部分,质地细腻,软,灰色,红色,灰色,和黄色的粘液结节在某些地区,结节最大直径为21cm。免疫组化为:CD34(+),CDK4(+),CK(-),Desmin(弱+),Ki67(指数5%),MDM2(-),p16(弱+),S-100P(+),波形蛋白(+),BCL-2(+)。他还被送到北京协和医院病理科与陆朝晖教授会诊,其咨询意见与黏液脂肪肉瘤一致。
    结论:腹膜后脂肪肉瘤是一种常见的腹膜后肿瘤,但在临床实践中相对罕见;总体发病率较低,主要表现为腹痛和腹胀,腹胀,病程长;对放疗和化疗不敏感,并应密切随访CT检查以了解复发和转移。
    BACKGROUND: Mucinous liposarcoma myxoid liposarcoma is a malignant mucoid soft tissue tumor derived from undifferentiated stromal cells in perivascular, subbody cavity and intermuscular space, and composed of cells at different stages of differentiation from preadipocytes to mature cells. In rare cases, it may change from lipoma malignancy. The main manifestations is painless mass, relatively slow growth, the course can last decades, the prevalence of liposarcoma in the population is 14% to 18%, mainly in adults, male prevalence is higher than women, but not significant. The main good hair part is the thigh, have mucinous sex, high differentiation type, dedifferentiation type, polymorphic type. Clinical diagnosis is difficult, and there are no obvious symptoms in the early stage, so the diagnosis should be combined with B ultrasound, MRI, CT, and other auxiliary examinations. The gold standard is pathological examination. In December 2023, our department admitted a patient with a mucinous abdominal mass. The report is as follows.
    METHODS: Does liposarcoma metastasize? Is any chemotherapy required after surgery? Will it ever relapse in the future? What is the survival period after surgery?
    METHODS: Mucinous liposarcoma.
    METHODS: Surgical resection of the sarcoma.
    RESULTS: The nodule sample was 33 * 28 * 13 cm, with complete capsule, gray and yellow sections, fine texture, soft, gray, red, grayish, and yellow mucoid nodules in some areas, and the maximum diameter of the nodules was 21cm. Immunohistochemistry was: CD34 (+), CDK 4 (+), CK (-), Desmin (weak +), Ki67 (index 5%), MDM 2 (-), p16 (weak +), S-100P (+), Vimentin (+), BCL-2 (+). He was also sent to the Department of Pathology of Peking Union Medical College Hospital for consultation with Professor Lu Zhaohui, whose consultation opinion was in line with myxoliposarcoma.
    CONCLUSIONS: Retroperitoneal liposarcoma is a common retroperitoneal tumor, but it is relatively rare in clinical practice; the overall morbidity is low, mainly manifested as abdominal pain and abdominal distension, abdominal distension, and a long course of disease; it is not sensitive to radiotherapy and chemotherapy, and should be closely follow up by CT examination to understand the recurrence and metastasis.
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  • 文章类型: Case Reports
    在脂肪肉瘤中,高分化脂肪肉瘤和去分化脂肪肉瘤是最常见的。这些肿瘤中的大多数在深腹膜后或四肢中发现。当在腹膜外发现时,这些脂肪源性肿瘤被称为非典型脂肪瘤(ALT)。浅表ALT特别罕见;因此,对他们的临床表现知之甚少,基因组状态,和管理。这里,我们介绍了一个54岁的男子的情况,在他的左上背部逐渐长大了两年多,偶然诊断为ALT。这个病人的ALT,然而,显示与MDM2和对照着丝粒12(CEP12)共扩增和阴性CD34和S100和RB1表达的高度多态性,与文献中描述的大多数其他ALT不同。该病例报告详细介绍了脂肪组织的诊断检查和组织病理学发现,并总结了不同的亚型。包括非典型梭形细胞/多形性脂肪瘤,多形性脂肪肉瘤,梭形细胞/多形性脂肪瘤,简要讨论管理。
    UNASSIGNED: Among liposarcomas, well-differentiated liposarcoma and dedifferentiated liposarcoma are the most common. The majority of these tumors are found in deep retroperitoneum or extremities. When found outside the retroperitoneum, these adipose-derived tumors are known as atypical lipomatous tumors (ALT). Superficial ALT are particularly rare; thus, little is known about their clinical presentation, genomic status, and management. Here, we present the case of a 54-year-old man with an intermittently bothersome, slowly growing mass on his left upper back for over 2 years, which was incidentally diagnosed as ALT. This patient\'s ALT, however, showed a profound degree of pleomorphism with MDM2 and control centromere 12 (CEP12) coamplification and negative CD34 and S100 and RB1 expression, unlike most other ALT described in the literature. This case report details the diagnostic workup and histopathological findings for adipose tumors and summarizes the different subtypes, including atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, and spindle cell/pleomorphic lipoma, with brief discussion on management.
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  • 文章类型: Case Reports
    怀孕期间的腹膜后脂肪肉瘤是罕见的,并提出了重大的诊断挑战。即使是经验丰富的专家。我们提供了一名27岁女性患者的病例报告,怀孕15周,他因巨大的腹膜后脂肪肉瘤入院。患者接受了肿瘤的手术切除。术后病理证实诊断为高分化脂肪肉瘤。虽然怀孕期间的脂肪肉瘤是罕见的和具有挑战性的诊断,CT或MRI在其检测中起着至关重要的作用。复发率取决于病理阶段,组织学分级,以及切除肿瘤的能力.
    Retroperitoneal liposarcoma during pregnancy is rare and poses significant diagnostic challenges, even for experienced specialists. We present a case report of a 27-year-old female patient, 15 weeks pregnant, who was admitted to the hospital due to a massive retroperitoneal liposarcoma. The patient underwent surgical resection of the tumor. Postoperative pathology confirmed a diagnosis of well-differentiated liposarcoma. Although liposarcoma during pregnancy is rare and challenging to diagnose, CT or MRI plays a crucial role in its detection. The recurrence rate depends on the pathological stage, histological grade, and ability to resect the tumor.
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  • 文章类型: Case Reports
    胰腺的脂肪性假性肥大(LPH)是一种罕见的疾病,其中胰腺实质被成熟的脂肪组织取代。这是一种特发性疾病,其诊断基于组织病理学分析。在这里,我们报道了一例50岁的男性患者,该患者的胰头有脂肪性肿块,在计算机断层扫描中进行了肾脏肿瘤的仔细检查。我们怀疑是脂肪肉瘤,并进行了剖腹手术。然而,组织学分析显示LPH。LPH的一些影像学发现可以进行无创诊断并有助于其临床方法。
    Lipomatous pseudohypertrophy of the pancreas (LPH) is a rare disease in which the pancreatic parenchyma is replaced with mature adipose tissue. It is an idiopathic condition whose diagnosis is made based on histopathological analyses. Herein, we report the case of a 50-year-old male patient with a lipomatous mass in the head of the pancreas on computed tomography for close examination of a renal tumor. We suspected liposarcoma, and laparotomy was performed. However, histological analyses revealed LPH. Several imaging findings of LPH can enable a noninvasive diagnosis and help its clinical approach.
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  • 文章类型: Case Reports
    源自软组织的肿瘤并不常见,在这些肿瘤中,脂肪肉瘤是最常见的。这些肿瘤长时间无症状,只有当它们达到一个重要的尺寸时才会暴露出来。在这种情况下,治疗困难,需要广泛的手术程序,可以切除几个相邻的结构,可能通过辅助放疗完成。尽管治疗成功,复发率仍然很高。我们报告了一个巨大的脂肪肉瘤的病例,需要进行涉及肿瘤切除的整体广泛切除,左肾,左肾上腺,和后腹壁的一部分。
    Tumors originating from soft tissues are uncommon, among these tumors, liposarcomas are the most frequent. These tumors remain asymptomatic for a long time, and only revealing themselves when they reach an important size. In such cases, treatment is difficult, requiring extensive surgery procedures that can excise several adjacent structures, potentially completed by adjuvant radiotherapy. Despite successful treatment, the recurrence rate remains very high. We report the case of a giant liposarcoma requiring a monobloc extensive resection involving the removal of the tumor, left kidney, left adrenal gland, and a portion of the posterior abdominal wall.
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  • 文章类型: Case Reports
    背景:多态变体是最不常见的,因此,最不为人所知的脂肪肉瘤亚型。它占所有脂肪肉瘤的<5%,并在成年后期发展。
    方法:我们报告了一例77岁男性患者,患者右侧髂窝和右侧腹股沟区疼痛。CT扫描显示盲肠水平有石质病变。进行了右半结肠切除术,病理报告为多形性脂肪肉瘤阳性。因此,他被转介接受辅助放疗。
    分子分析验证了脂肪肉瘤亚型的区别,尽管组织学亚型仍然是日常实践中可靠的预后参数,最近的证据表明,基因谱最终可能会影响个体患者的风险分层。
    结论:脂肪肉瘤的多形性亚型很少见,它们在盲肠中非常罕见,因此,目前建议由多学科团队对患者进行评估以做出治疗决定,因为这是一种非常攻击性行为的亚型,在开始任何类型的肿瘤治疗之前。
    BACKGROUND: The pleomorphic variant is the least common and consequently, the least known subtype of liposarcomas. It represents <5 % of all liposarcomas and develops during late adulthood.
    METHODS: We report the case of a 77-year-old male who presented with pain in the right iliac fossa and right inguinal region. A CT scan revealed a stony lesion at the level of the cecum. A right hemicolectomy was performed, and the pathology report was positive for pleomorphic liposarcoma. Consequently, he was referred for adjuvant radiotherapy.
    UNASSIGNED: Molecular analysis has validated the distinction of liposarcoma subtypes, although histological subtype remains the reliable prognostic parameter in daily practice, recent evidence suggests that genetic profiles may eventually influence risk stratification of individual patients.
    CONCLUSIONS: The pleomorphic subtype of liposarcomas is infrequent, and they are very rare in the cecum, so it is currently recommended that patients be evaluated by a multidisciplinary team to make a therapeutic decision as it is a subtype of very aggressive behavior, before initiating any type of oncologic treatment.
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  • 文章类型: Case Reports
    该病例报告介绍了一名51岁男性持续性吞咽困难的高分化食管脂肪肉瘤的成功内镜黏膜下剥离术(ESD)。最初的原因被诊断为从食管上括约肌延伸到食管中部的10厘米带蒂病变。与传统手术相比,选择了ESD,因为它的侵入性较小。该程序涉及精确的粘膜下注射和切除,并采用特殊技术来管理中央血管的出血。尽管由于病变的大小而存在提取挑战,口服成功取出。8.3×4.2×2.3cm标本的组织病理学检查显示了分化良好的脂肪肉瘤的特征,包括MDM2和CDK4阳性。随访显示没有复发,此后一直进行主动监测。该报告强调了ESD治疗重要食管肿瘤的多功能性,并为其作为微创替代方案的有效性提供了证据。
    This case report presents the successful endoscopic submucosal dissection (ESD) of a well-differentiated esophageal liposarcoma in a 51-year-old male with persistent dysphagia. The cause was initially diagnosed as a 10 cm pedunculated lesion extending from the upper esophageal sphincter to the mid-esophagus. An ESD was chosen over traditional surgery because it is less invasive. The procedure involved a precise submucosal injection and excision with special techniques to manage bleeding from a central vessel. Despite the extraction challenges owing to the size of the lesion, it was successfully removed orally. A histopathological examination of the 8.3×4.2×2.3 cm specimen revealed the characteristic features of a well-differentiated liposarcoma, including MDM2 and CDK4 positivity. The follow-up revealed no recurrence, and active surveillance has been performed since. This report highlights the versatility of ESD in treating significant esophageal tumors and provides evidence for its efficacy as a minimally invasive alternative.
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  • 文章类型: Case Reports
    副睾丸脂肪肉瘤由精索周围的脂肪组织形成,并覆盖睾丸和附睾。它是一种极其罕见的病理实体。我们报告了一名58岁的非洲男子的病例,该男子的肿瘤肿块是从右精索形成的。由于肿块的大小很大,右睾丸切除术和广泛切除肿瘤具有挑战性。手术标本的组织学检查有利于睾丸旁脂肪肉瘤。
    Para-testicular liposarcoma develops from the fatty tissue surrounding the spermatic cord and covers the testicle and epididymis. It is an extremely rare pathological entity. We report the case of a 58-year-old african man who presented with a tumor mass developed from the right spermatic cord. Right orchidectomy with wide excision of the tumor was challenging due to the significant size of the mass. The histological examination of the surgical specimen favored a paratesticular liposarcoma.
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  • 文章类型: Case Reports
    纵隔脂肪肉瘤免疫治疗后手术的可行性仍不确定。此外,目前仍缺乏对脂肪肉瘤的免疫治疗。我们报告了一例左纵隔脂肪肉瘤切除术后复发的病例。复发后,一个疗程的pembrolizumab加盐酸安洛替尼没有显示肿瘤缩小,基因检测显示CDK4扩增和PD-L1TPS<1%;因此,该计划更改为一个疗程的pembrolizumab加palbociclib,但是肿瘤仍然没有缩小。因此,进行了第二次肿瘤切除。此外,术后病理仍为高分化脂肪肉瘤.免疫治疗在脂肪肉瘤中的意义仍需进一步探讨。在没有手术禁忌症的情况下,二次手术可能是可行的。
    The feasibility of surgery after immunotherapy for mediastinal liposarcoma remains uncertain. Besides, the case of immunotherapy for liposarcoma is still lacking. We report a case of recurrence after resection of a left mediastinal liposarcoma. After recurrence, one course of pembrolizumab plus anlotinib hydrochloride showed no tumor shrinkage, and genetic testing showed CDK4 amplification and PD-L1 TPS <1%; therefore, the plan was changed to one course of pembrolizumab plus palbociclib, but the tumor still did not shrink. Thus, second tumor resection was performed. In addition, the postoperative pathology was still well-differentiated liposarcoma. The significance of immunotherapy in liposarcoma still needs to be further explored. In the absence of surgical contraindications, secondary surgery might be feasible.
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