hepatic angiomyolipoma

肝血管平滑肌脂肪瘤
  • 文章类型: Case Reports
    肝血管平滑肌脂肪瘤(HAML)是一种罕见的脂肪组织肿瘤,平滑肌细胞,和血管。另一方面,炎症性乳腺癌(IBC)是一种罕见且严重的乳腺癌,进展迅速,表现为乳房炎症。HAML和IBC在同一患者中共存是非常不寻常的。在本研究中,我们描述了一例63岁的也门女性患者,该患者被诊断为局部晚期左乳腺癌,并出现左乳房和腋窝疼痛.用于分期的计算机断层扫描(CT)扫描显示偶然的大肝脏肿块,最终被发现是HAML。该患者在完成新辅助治疗后接受了改良的根治性乳房切除术,随后对该病变进行了保留实质的肝切除术;随访至今。由于重叠的临床和放射学特征,在IBC存在下诊断HAML可能会带来挑战。合并HAML和IBC的患者的治疗决策需要多学科方法;手术切除,栓塞,靶向治疗,和全身化疗可以根据疾病的程度和个体患者的因素考虑。最后,还对相关文献进行了简要回顾。
    Hepatic angiomyolipoma (HAML) is a rare tumor comprising adipose tissue, smooth muscle cells, and blood vessels. On the other hand, inflammatory breast cancer (IBC) is a rare and severe form of breast cancer that progresses quickly and presents as breast inflammation. It is incredibly unusual for HAML and IBC to coexist in the same patient. In the present study, we describe a case of a 63-year-old Yemeni female patient diagnosed with locally advanced left breast cancer presented with pain at the left breast and axilla. A computed tomography (CT) scan for staging showed an incidental large hepatic mass, which was eventually discovered to be HAML. The patient underwent a modified radical mastectomy after completing her neoadjuvant treatment and later underwent parenchyma-sparing liver resection of that lesion; follow-up has continued till now. The diagnosis of HAML in the presence of IBC can pose challenges due to overlapping clinical and radiological features. Treatment decisions for patients with coexisting HAML and IBC require a multidisciplinary approach; surgical resection, embolization, targeted therapies, and systemic chemotherapy may be considered based on the extent of the disease and individual patient factors. Lastly, a brief review of the related literature was also carried out.
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  • 文章类型: Case Reports
    肝血管平滑肌脂肪瘤(HAML)是一种罕见的由血管组成的肝脏肿瘤,平滑肌,和脂肪细胞。HAML发生在广泛的年龄范围内,症状包括腹部不适,腹胀,和减肥。由于影像表现各异,诊断具有挑战性。但组织病理学检查辅以免疫组织化学分析,特别是使用HMB-45,是确定的。
    方法:一名33岁男子,有两年右上腹腹痛史,偶尔使用镇痛药缓解,但在过去的一个半月中恶化。考试显示出一种柔和,腹部不扩张,肝脏明显。实验室测试,包括病毒标志物和肿瘤标志物均正常。对比增强CT显示,肝脏III段有明确的椭圆形肿块,并伴有异质性增强,从而暂时诊断为HAML。患者接受了成功的整块切除术,没有术中或术后并发症。
    对于有症状的病例或不确定的活检,建议进行手术切除。由于复发的可能性和与其他恶性肿瘤的关联,需要严格的随访。
    结论:HAML可表现为长期的非特异性腹部症状。CT成像有助于诊断脂肪组织丰富的病例。整块肿瘤切除在治疗症状表现方面被证明是安全有效的。
    UNASSIGNED: Hepatic angiomyolipoma (HAML) is a rare liver tumor composed of blood vessels, smooth muscle, and fat cells. HAML occurs across a wide age range, with symptoms including abdominal discomfort, bloating, and weight loss. Diagnosis is challenging due to varied imaging appearances, but histopathological examination supplemented by immunohistochemical analysis, particularly using HMB-45, is definitive.
    METHODS: A 33-year-old man presented with a two-year history of right upper quadrant abdominal pain, occasionally relieved with analgesics but worsening over the past month and a half. Examinations revealed a soft, non-distended abdomen with a palpable liver. Laboratory tests, including viral markers and tumor markers were normal. Contrast-enhanced CT revealed a well-defined oval mass in liver segment III with heterogeneous enhancement leading to provisional diagnosis of HAML. The patient underwent a successful en bloc excision with no intraoperative or postoperative complications.
    UNASSIGNED: Surgical resection is recommended for symptomatic cases or inconclusive biopsies, with stringent follow-up necessary due to the potential for recurrence and association with other malignancies.
    CONCLUSIONS: HAML may present with prolonged nonspecific abdominal symptoms. CT imaging aids in diagnosing cases with abundant fatty tissue. En bloc tumor excision proves safe and effective in treating symptomatic presentations.
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  • 文章类型: Case Reports
    肝血管平滑肌脂肪瘤是一种罕见的,可能是癌性的间质瘤,由三个部分组成:血管,平滑肌细胞,和脂肪组织。在本文中,我们报道了一例36岁的男性患者,他患有巨大的肝血管平滑肌脂肪瘤并自发性破裂和出血。患者因突发性上腹痛3小时入院。在肝脏的左侧和尾状叶发现了一个巨大的肿瘤,以及肝脏和骨盆周围的大量血液收集。血红蛋白,肝功能检查结果,血清肿瘤标志物水平均在正常范围内。为了防止出血,进行了紧急血管造影和栓塞。在血管造影期间,发现肿瘤由左肝动脉供应,内部血液供应非常丰富。在一周后的腹腔镜探查中,发现一个直径约11厘米的巨大左肝肿块从肝脏表面凸出并在那里破裂。破裂强烈地粘附在胃的较小曲率上。患者行腹腔镜左半肝切除术和尾状叶切除术,肿瘤标本是棕色的,与周围正常肝实质有明确的界限,肿瘤内有大量坏死性病变。组织病理学结果证实肿块为肝血管平滑肌脂肪瘤,切缘阴性。免疫组织化学染色显示肿瘤阳性的甲基溴-45。经过13个月的随访,患者无肿瘤复发或转移。
    Hepatic angiomyolipoma is a rare and possibly cancerous mesenchymal tumor that consists of three components: blood vessels, smooth muscle cells, and adipose tissue. In this paper, we reported a case of a 36-year-old man who had a giant hepatic angiomyolipoma with spontaneous rupture and hemorrhage. The patient was admitted to our hospital with sudden upper abdominal pain for 3 h. A giant tumor was found in the left and caudate lobes of the liver, as well as significant blood collection around the liver and in the pelvis. Hemoglobin, liver function test results, and serum tumor maker levels were all within normal ranges. To prevent bleeding, emergency angiography and embolization were performed. During angiography, it was discovered that the tumor was supplied by the left hepatic artery and had a very rich internal blood supply. A massive left hepatic mass of about 11 cm in diameter was found bulging from the surface of the liver and rupturing there during laparoscopic exploration a week later. The rupture was strongly adhered to the smaller curvature of the stomach. The patient underwent laparoscopic left hemihepatectomy and caudate lobectomy, and the tumor specimen was brown, with clear boundaries with the surrounding normal liver parenchyma, and there were a large number of necrotic lesions inside the tumor. Histopathological results confirmed the mass as hepatic angiomyolipoma with negative resection margins. Immunohistochemical staining indicated that the tumor had positive homatropine methylbromide-45. After 13 months of follow-up, no tumor recurrence or metastasis occurred in the patient.
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  • 文章类型: Case Reports
    背景:本文检查了原发性肝血管肉瘤(PHA)和脂肪贫乏的血管平滑肌脂肪瘤(AML),两种罕见的血管癌.在这些情况下的临床决策通常由病理报告和成像技术来辅助。不常见的血管内皮恶性肿瘤包括PHA。采用对比增强MR和对比增强计算机断层扫描(CT)成像技术时,另一个不应忽视的诊断是脂肪贫乏的AML,肝脏罕见的血管瘤之一。在这两种情况下,活检是诊断的主要手段。
    方法:在我们的文章中,除了PHA的诊断,脂肪贫乏的AML,另一种罕见的肝脏血管瘤,提到了。在案件中,一名50岁的女性VHL综合征患者因右上腹疼痛等非特异性病变入院,减肥,和恶心。腹部超声检查(US)显示低回声异质性病变,偶尔有模糊的轮廓。在计算机断层扫描中,在第4段观察到为高密度结节性病变。磁共振成像(MRI)显示病变不含脂肪。关于已知的VHL综合征病史,我们首先评估了AML的可能性。于是,采集组织病理学样本,诊断为脂肪含量为5%的贫脂AML.
    结论:结论:我们的病例报告中的PHA和我们诊所中的脂肪贫乏的AML是两种罕见的肝血管恶性肿瘤,发病率相当。重要的成像技术,如对比增强US(CEUS),CECT,在这两种情况下,CEMRI都给我们带来了巨大的优势。然而,活检用于提供最终诊断。
    BACKGROUND: This article examines primary hepatic angiosarcoma (PHA) and fat-poor angiomyolipoma (AML), two uncommon vascular cancers. Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques. Uncommon malignant tumors of the vascular endothelium include PHA. Another diagnosis that should not be overlooked when employing contrast-enhanced MR and contrast-enhanced computed tomography (CT) imaging techniques is fat-poor AML, one of the uncommon vascular tumors of the liver. In both conditions, biopsy is the primary means of diagnosis.
    METHODS: In our article, besides the diagnosis of PHA, fat-poor AML, one of the other rare vascular tumors of the liver, is mentioned. In the case, a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain, weight loss, and nausea. Abdominal ultrasonography (US) revealed a hypoechoic heterogeneous lesion with occasional faint contours. In computed tomography, it was observed as a hyperdense nodular lesion in segment 4. Magnetic resonance imaging (MRI) revealed that the lesion did not contain fat. In connection with the known history of VHL Syndrome, we first evaluated the possibility of AML. Thereupon, a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5% fat content.
    CONCLUSIONS: In conclusion, PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences. Important imaging techniques like contrast-enhanced US (CEUS), CECT, and CEMRI give us substantial advantages in both cases. However, a biopsy is used to provide the final diagnosis.
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  • 文章类型: Case Reports
    血管平滑肌脂肪瘤,血管周围上皮样细胞瘤,很容易识别为肾脏中的良性肿瘤。然而,当发生在肾外部位时,它可以模拟恶性肿瘤(癌症细胞病理。2017;125:257)。病理学家必须意识到这种病变的经典形态特征,它在外地的陷阱,以及需要进行免疫组织化学以建立正确的诊断(WorldJGastroenterol。2000;6:608)。我们报告了一例伴有髓外造血的血管平滑肌脂肪瘤,表现为巨大的肝脏肿块,通过内窥镜超声引导的细针穿刺细胞学诊断。我们的案例说明了经典的细胞学发现,这些发现对于区分肝血管平滑肌脂肪瘤(HAML)和该器官的差异(例如肝细胞癌(HCC)和局灶性结节增生)至关重要。简要回顾了HAML和HCC之间的重要特征,并进行了比较。
    Angiomyolipoma, a perivascular epithelioid cell tumour, is easily identifiable as a benign tumour in the kidneys. However, when occurring in extrarenal sites it can mimic malignancy (Cancer Cytopathol. 2017;125:257). Pathologists must be aware of the classical morphological features of this lesion, its pitfalls in extrarenal sites, and the need for immunohistochemistry in order to establish the correct diagnosis (World J Gastroenterol. 2000;6:608). We report a case of angiomyolipoma with extramedullary hematopoiesis presenting as a large hepatic mass, diagnosed by cytology through endoscopic ultrasound guided fine needle aspiration. Our case exemplifies the classic cytological findings that are important in the differentiation between hepatic angiomyolipoma (HAML) and differentials in this organ such as hepatocellular carcinoma (HCC) and focal nodular hyperplasia. A brief literature review and comparison of significant features between HAML and HCC are presented.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    背景:肝血管平滑肌脂肪瘤(HAML)是一种罕见的间叶性肝肿瘤,属于血管周围上皮样细胞肿瘤(PEComas)家族。它通常由血管组成,平滑肌,脂肪细胞,并且显示对HMB-45的强免疫反应性。
    一名57岁的妇女因肝脏病变不清被转诊到我们医院。细针活检显示怀疑有髓外造血的肝血管平滑肌脂肪瘤。术前成像显示,左肝叶II和III段的肿瘤直径为17厘米。对左叶II和III段进行了肺叶切除术。获得肝血管平滑肌脂肪瘤的病理诊断。
    结论:HAML中组织成分优势的变化阻碍了仅基于影像学的诊断。HAML最有希望的证据是脂肪瘤的组织学鉴定,肌瘤,血管瘤组织联合HMB-45免疫组化阳性。虽然肿瘤被认为是良性的,一些病例被描述为恶性行为。如果出现症状,应考虑手术切除,不确定的活检,或后续增长。其他手术适应症可能包括侵袭性模式,如血管侵犯,p53免疫反应性,或快速增殖的肿瘤细胞。
    结论:HAML是一种罕见的肝脏肿瘤。有症状的患者,不确定的诊断,或肿瘤生长,应根据肿瘤标准进行手术切除。
    BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours (PEComas). It is typically composed of blood vessels, smooth muscle, and adipose cells, and shows strong immunoreactivity for HMB-45.
    UNASSIGNED: A 57-year-old woman was referred to our hospital with an unclear liver lesion. A fine needle biopsy revealed a suspicion of hepatic angiomyolipoma with extramedullary haematopoiesis. Preoperative imaging revealed a tumour 17 cm in diameter in the left liver lobe segments II and III. A lobectomy of the left lobe segments II and III was performed. The pathological diagnosis of hepatic angiomyolipoma was obtained.
    CONCLUSIONS: Variations in the predominance of the tissue components in HAML impedes diagnosis based on imaging alone. The most promising evidence of HAML is the histological identification of lipomatous, myomatous, and angiomatous tissue combined with immunohistochemical positivity for HMB-45. Although the tumour is considered benign, some cases have been described with malignant behaviour. Surgical resection should be considered in case of symptoms, inconclusive biopsy, or growth in follow-up. Other surgical indications may include aggressive patterns such as vascular invasion, p53 immunoreactivity, or rapidly proliferating tumour cells.
    CONCLUSIONS: HAML is a rare liver tumour. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed according to oncological criteria.
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  • 文章类型: Case Reports
    El angiomiolipoma hepático es un tumor mesenquimatoso infrecuente perteneciente al grupo de los PEComas (Perivascular Epithelioid Cells), que cuenta con un potencial maligno indeterminado. Los síntomas clínicos son inespecíficos, siendo las características radiológicas principales la alta vascularización de la lesión y la presencia de tejido adiposo macroscópico. Presentamos un caso clínico de un angiomiolipoma hepático izquierdo diagnosticado por tomografía computada de manera incidental y asintomático en una paciente sin antecedentes de importancia que se sometió a hepatectomía lateral izquierda con resección completa del tumor, el cual fue negativo para malignidad y sin complicaciones.
    Hepatic angiomyolipoma is a mesenchymal tumor known as PEComas (Perivascular Epithelioid Cells) with unknown malignancy pattern. We present a case report of left hepatic angiomyolipoma diagnoses by computed tomography in an asymptomatic patient with no previous medical diseases. The tumor was complete resected during a left lateral hepatectomy with no complications and final histologic report was negative to cancer.
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  • 文章类型: Journal Article
    BACKGROUND: Hepatic angiomyolipoma is a rare tumour and is difficult to obtain the accurate diagnosis preoperatively because the imaging features are similar to hepatocellular carcinoma.
    METHODS: We present a case study of an 80-year old woman with a liver tumour measuring 6.2 cm × 6.0 cm. We were able to diagnose the tumour preoperatively as a rare hepatic myomatous angiomyolipoma based on the presence of early venous return evident on angiography and small low-intensity areas corresponding to fat within the tumour revealed by out-of-phase EOB-MRI. The tumour was removed by minimally invasive surgery and our preoperative diagnosis was confirmed by positive immunoreactivity for both angiomyolipoma-specific human melanoma black 45 and smooth muscle cell positivity for melanin.
    CONCLUSIONS: We consider that the information obtained in this case will be useful for preoperative diagnosis of other hepatic angiomyolipomas, thus facilitating more appropriate and less invasive surgery and improving the overall outcome.
    CONCLUSIONS: Hepatic myomatous angiomyolipoma is a rare tumour. We illustrated the two specific imaging features to diagnose it preoperatively.
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  • 文章类型: Case Reports
    OBJECTIVE: To compare the enhancement pattern of hepatic angiomyolipoma (HAML) on contrast enhanced ultrasound (CEUS) and magnetic resonance (MR).
    METHODS: The data of seven patients (females; age 28-52 years; mean, 42 years) with histologically proven HAMLs were retrospectively reviewed. All patients underwent CEUS and MR examination. The images were analyzed by two experienced doctors who blinded to the clinical and pathological information of cases.
    RESULTS: The mean diameter of the nodule was 5.7 cm (range: 3.2-10 cm). Histopathologic results revealed 4 nodules to be myomatous type and 3 nodules to be mixed type. All nodules showed hyperenhanced during arterial phase on both CEUS and MRI. During portal and delayed phase, washout was more showed on MRI (5/7, 71.4%) than on CEUS (2/7, 28.6%).
    CONCLUSIONS: There is discrepancy of enhancement pattern between CEUS and MRI. The quick wash-in and sustained hyperenhancement on CEUS may be helpful for the diagnosis of HAML.
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