Mast-Cell Sarcoma

肥大细胞肉瘤
  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    我们报告了一名47岁男性的18F-FDGPET/CT扫描,诊断为弥漫性肥大细胞肉瘤伴淋巴结,骨头,肝脏,脾,脾和肺部受累。这个有趣的图像应该提醒同事们考虑肥大细胞肉瘤作为一个罕见的鉴别诊断的患者,各种器官和淋巴结中的强烈高代谢病变。
    UNASSIGNED: We report an 18 F-FDG PET/CT scan of a 47-year-old man diagnosed with diffuse mast cell sarcoma with lymph node, bone, liver, spleen, and lung involvement. This interesting image should remind colleagues to consider mast cell sarcoma as a rare differential diagnosis in patients with multiple, intensely hypermetabolic lesions in various organs and lymph nodes.
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  • 文章类型: Journal Article
    背景:淋巴结清扫术在肥大细胞瘤(MCT)中的治疗作用和预后相关性历来在区域而非前哨淋巴结中进行评估。
    目的:更新放射性药物引导下肿瘤切除和切除正常大小前哨淋巴结(SLN)后MCT犬的组织学结节(HN)类型与临床结果的相关性。
    方法:94只经组织学证实的初治MCT犬(71只皮肤,如果没有:远处转移,则包括22个皮下和1个结膜MCT),淋巴结肿大,并发混合皮肤,和皮下MCT。
    方法:这是一项单一队列研究。检索肿瘤特征,并根据Weishaar系统对SLN进行分类。MCT相关事件的发生率(本地,节点,远处复发),从头MCT或其他肿瘤和死亡(MCT相关和非MCT相关),被记录下来。比较了HN类别之间的发生率曲线。
    结果:27只狗有HN0、19只HN1、37只HN2和11只HN3SLN。13人(2个HN0、4个HN2和7个HN3)接受了辅助化疗。Kiupel高级,SLN和淋巴中心数量的增加与较高的HN等级相关.五只狗因MCT相关原因死亡:1只低度(HN0)和1只皮下(HN3)局部复发,2只高级别的具有远处复发(HN3-HN0),并且1只狗从从头皮下MCT发展疾病进展。没有淋巴结复发。14只狗发展了从头MCT。
    结论:低等级/低风险MCT伴无法触及且大小正常的SLN具有独立于HN的良好结局。结果应严格考虑与放射性药物标记物引导的术前和术中成功检测SLN相关。
    BACKGROUND: The therapeutic role and prognostic relevance of lymphadenectomy in mast cell tumor (MCT) has historically been evaluated on regional rather than sentinel lymph nodes.
    OBJECTIVE: To update information about the association of histological nodal (HN) classes with clinical outcome in dogs with MCT after tumor excision and extirpation of normal-sized sentinel nodes (SLN) guided by radiopharmaceutical.
    METHODS: Ninety-four dogs with histologically-confirmed treatment-naïve MCT (71 cutaneous, 22 subcutaneous and 1 conjunctival MCT) were included if without: distant metastases, lymphadenomegaly, concurrent mixed cutaneous, and subcutaneous MCT.
    METHODS: This was a monoistitutional cohort study. Tumors characteristics were retrieved and SLNs were classified according to Weishaar\'s system. Incidence of MCT-related events (local, nodal, distant relapse), de novo MCT or other tumors and death (MCT-related and non-MCT-related), were recorded. Incidence curves were compared among the HN classes.
    RESULTS: Twenty-seven dogs had HN0, 19 HN1, 37 HN2, and 11 HN3 SLN. Thirteen (2 HN0, 4 HN2, and 7 HN3) received adjuvant chemotherapies. Kiupel high grade, increasing number of SLN and lymphocentrums were associated with higher HN classes. Five dogs died for MCT-related causes: 1 low-grade (HN0) and 1 subcutaneous (HN3) had a local relapse, 2 high-grade had distant relapse (HN3-HN0) and 1 dog developed disease progression from a de novo subcutaneous MCT. No nodal relapse was registered. Fourteen dogs developed de novo MCTs.
    CONCLUSIONS: Low grade/low-risk MCT with nonpalpable and normal sized SLN have a favorable outcome independently from the HN. Result should be considered strictly related to the successful SLN detection guided pre- and intraoperative by radiopharmaceutical markers.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    肥大细胞肉瘤(MCS)是一种极为罕见的肥大细胞增多症,其特征是侵袭性恶性肥大细胞生长和转移潜能。MCS的诊断非常具有挑战性,因为它具有明显的形态学变异和与其他肿瘤的显著免疫表型重叠。在这项研究中,我们对我们系列的10例MCS进行了广泛的研究,回顾了文献中的另外24例,为了更好地阐明MCS的临床病理和分子特征。从我们10例的分析来看,MCS同样涉及男性和女性,中位年龄为54.5岁(范围1-63)。骨头是最常见的受累部位,如9/10的案例所示。两名患者先前有生殖细胞肿瘤(纵隔生殖细胞肿瘤和卵巢无性细胞瘤),9例患者中有1例同时出现系统性肥大细胞增生症.6/7的患者血清类胰蛋白酶水平升高,3/9的患者有肥大细胞活化症状。形态学上,肿瘤细胞通常较大,多形性,常见的反应性嗜酸性粒细胞.通过免疫组织化学染色,MCS一致表达CD43(8/8),CD117(10/10),和肥大细胞类胰蛋白酶(10/10),以及CD13(3/3)和CD33(10/10),CD2(1/9)的阳性变量,CD25(4/9),CD30(5/8),和CD68(5/9)。值得注意的是,在我们的研究中,在9例病例中未检测到KITD816V,尽管两例有其他KIT基因突变。八分之七的患者接受了有或没有放疗的化疗。然而,反应很差,8例患者中有4例在5个月的中位随访间隔内死亡.一起来看,目前尚无用于MCS的标准化治疗方案,预后很糟糕.因此,进一步研究和表征这种罕见的实体至关重要,希望提高诊断准确性并提供更有效的诊断,靶向治疗。
    Mast cell sarcoma (MCS) is an exceedingly rare form of mastocytosis characterized by invasive malignant mast cell growth and metastatic potential. Diagnosis of MCS is very challenging due to its marked morphologic variations and significant immunophenotypic overlap with other neoplasms. In this study, we undertook an extensive study of 10 cases of MCS from our series, with review of additional 24 cases from the literature, to better clarify the clinicopathologic and molecular features of MCS. From the analyses of our 10 cases, MCS equally involved males and females with a median age of 54.5 years (range 1-63). The bone was the most common site of involvement, as noted in 9/10 of cases. Two patients had prior germ cell tumors (mediastinal germ cell tumor and ovarian dysgerminoma), and concurrent systemic mastocytosis was noted in one of nine patients. Serum tryptase levels were elevated in 6/7 of patients, and 3/9 of patients had mast cell activation symptoms. Morphologically, the tumor cells were typically large and pleomorphic with frequent reactive eosinophils. By immunohistochemical staining, MCS consistently expressed CD43 (8/8), CD117 (10/10), and mast cell tryptase (10/10), as well as CD13 (3/3) and CD33 (10/10), with variable positivity of CD2 (1/9), CD25 (4/9), CD30 (5/8), and CD68 (5/9). Notably, KIT D816V was not detected in nine cases in our study, although two cases had other mutations of KIT gene. Seven out of eight patients received chemotherapy with or without radiotherapy. However, the response was poor, and four out of eight patients died within a median follow-up interval of five months. Taken together, there are no standardized therapeutic regimens available for MCS at this time, and the prognosis is dismal. Therefore, it is critical to further investigate and characterize this rare entity, with the hope of improving diagnostic accuracy and providing more effective, targeted therapies.
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  • 文章类型: Case Reports
    我们介绍了一例成年男性,其皮肤孤立性肥大细胞肿瘤具有异常的核多态性和有丝分裂活性。肿瘤被切除了,在2年内复发,4年后再次切除,诊断后6年以上未复发。从发病开始,通过Ki67染色,肿瘤显示出进行性细胞核异型性和高有丝分裂和增殖率。在肿瘤和骨髓中未发现KIT突变。血清类胰蛋白酶水平,骨髓穿刺液和环钻活检均正常。尽管皮肤肿瘤的组织形态学与肥大细胞肉瘤一致,没有全身进展的临床行为与这种诊断相反.肿瘤最终被认为是非典型肥大细胞瘤,肥大细胞肉瘤的边缘.目前,患者正在密切随访中,仍处于完全缓解状态。
    We present a case of an adult male with a solitary mast cell tumor of the skin with unusual nuclear pleomorphism and mitotic activity. The tumor was excised, recurred within 2 years, was reexcised after 4 years and did not recur >6 years after diagnosis. The tumor showed progressive cytonuclear atypia and a high mitotic and proliferation rate by Ki67-staining from the onset. No KIT mutations were identified in the tumor and bone marrow. Serum tryptase levels and a bone marrow aspirate and trephine biopsy were normal. Although the histomorphology of the skin tumor was consistent with mast cell sarcoma, the clinical behavior without systemic progression argued against this diagnosis. The tumor was finally considered as atypical mastocytoma, borderline to mast cell sarcoma. Currently, the patient is in close follow-up and still in complete remission.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    Strong to moderate vascular endothelial growth factor (VEGF) expression may be a negative prognostic factor in canine mast cell tumors (MCTs). This study set out to determine the prognostic value of combined analysis of VEGF-A immunoreactivity, clinical presentation, patient staging, and tumor histologic grade in canine MCTs. In this study, intense VEGF staining was significantly associated with decreased survival (P = .025). Immunohistochemical expression of VEGF is not routinely employed as a prognostic factor in canine MCT workup. However, results of this study support the inclusion of this marker in the MCT prognostic panel. Investigation of VEGF expression may assist in the development of anti-VEGF drugs.
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