pleomorphic

多形性
  • 文章类型: Case Reports
    未分化多形性肉瘤(UPS)是一种罕见的间充质起源的恶性肿瘤。分化差的肿瘤细胞,可能采取巨细胞的形式,组织细胞,或者纺锤形细胞,构成肉瘤的UPS变体。如果软组织肿瘤增大并变成恶性,他们可能会成为一个问题。通过几次检查可以诊断出肉瘤,比如体检,MRI,CT扫描,或超声波。活检可产生有关肉瘤等级和亚型的信息,并且是明确诊断所必需的。化疗,放射治疗,广泛切缘切除是骨癌的标准治疗方法。UPS经常出现在50至70岁的人群中。然而,在这里,我们报告一名40岁的男性被诊断为UPS。我们的目标是讨论我们的案例与其他案例相比有多独特,以及在这种情况下可用的诊断和治疗替代方案。
    The undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumor of mesenchymal origin. Poorly differentiated tumor cells, which might take the form of giant cells, histiocytes, or spindle-shaped cells, make up the UPS variant of sarcomas. If soft tissue tumors enlarge and turn malignant, they may become an issue. Sarcoma is diagnosed by several tests, such as a physical examination, MRI, CT scan, or ultrasound. A biopsy yields information regarding the grade and subtype of the sarcoma and is required for a clear diagnosis. Chemotherapy, radiation therapy, and broad-margin excision are the standard treatments for cancers of the bone. UPS often appears in people between 50 and 70 years old. Yet, here we report a 40-year-old male diagnosed with UPS. Our goal is to discuss how unique our case is in comparison to others, as well as the available diagnostic and therapeutic alternatives in such cases.
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  • 文章类型: Case Reports
    背景:绝经后妇女阴道横纹肌肉瘤(RMS)是一种极为罕见的恶性肿瘤,最初被描述为起源于原始间充质细胞的一组独特的软组织肉瘤。1970年首次在绝经后妇女中报道,迄今为止报道的绝经后患者不到50例。
    方法:2023年10月11日,一名68岁的经产女性入院,主诉为包块导致阴道脱垂伴排尿不完全,持续4个月。阴道肿块大约是鸽子蛋的大小;躺下后,阴道肿块缩回。进行了完全切除,根据病理和免疫组织化学染色特征诊断为阴道多形性RMS。患者目前正在接受化疗。本研究还回顾了临床,组织学,阴道RMS的免疫组织化学特征和最新治疗建议。任何异常的阴道肿块应及时通过盆腔检查和适当的影像学检查。目前阴道RMS的初始治疗是活检和初级化疗。
    结论:当计划对阴道RMS进行手术时,应考虑保留器官的方法。
    BACKGROUND: Rhabdomyosarcoma (RMS) of the vagina in postmenopausal women is an extremely rare malignant tumor that was originally described as a unique group of soft tissue sarcomas originating from primitive mesenchymal cells. It was first reported in postmenopausal women in 1970, and fewer than 50 postmenopausal patients have been reported to date.
    METHODS: A 68-year-old multiparous female was admitted to the hospital on October 11, 2023, with the chief complaint of a mass causing vaginal prolapse with incomplete urination that had persisted for 4 months. The vaginal mass was approximately the size of a pigeon egg; after lying down, the vaginal mass retracted. Complete resection was performed, and vaginal pleomorphic RMS was diagnosed based on pathology and immunohistochemical staining features. The patient is currently undergoing chemotherapy. The present study also reviewed the clinical, histological, and immunohistochemical features and latest treatment recommendations for vaginal RMS. Any abnormal vaginal mass should be promptly investigated through pelvic examination and appropriate imaging. The current initial treatment for vaginal RMS is biopsy and primary chemotherapy.
    CONCLUSIONS: When surgery is planned for vaginal RMS, an organ-preserving approach should be considered.
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  • 文章类型: Review
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  • 文章类型: Review
    硬化性纤维瘤(SF)是一种罕见的皮肤纤维瘤,偶发或与Cowden综合征(CS)相关。我们报告了一例已知CS且头皮上有孤立性病变的患者。组织学检查显示界限清楚的病变,硬化真皮和螺旋胶原蛋白模式,多核巨细胞,和树突状梭形细胞。未注意到核异型或有丝分裂图。巨细胞对Melan-A呈阴性,SOX-10EMA,SOX-10和因子XIIIa。这些发现与巨细胞胶原瘤(GCC)一致。尽管可能与SF重叠,GCC尚未与CS相关联。这使我们的案例独特,并建议GCC应包括在CS相关皮肤病变的范围内。SF的诊断可能导致先前未诊断的CS的识别;因此,GCC,即使作为孤立性病变存在,可能表明需要进一步检查和筛查CS。
    Sclerotic fibroma (SF) is a rare subset of dermal fibromas that occurs sporadically or in association with Cowden syndrome (CS). We report a case of a patient with known CS and a solitary lesion on the scalp. Histologic examination demonstrated a well-circumscribed lesion with sclerotic dermis and a whorled collagen pattern, multinucleated giant cells, and dendritic spindle cells. Nuclear atypia or mitotic figures were not noted. The giant cells were negative for Melan-A, SOX-10, EMA, SOX-10, and factor XIIIa. These findings are consistent with a giant cell collagenoma (GCC). Despite possible overlap with SF, GCC has not been associated with CS. This makes our case unique and suggests that GCC should be included in the spectrum of CS-associated cutaneous lesions. The diagnosis of SF may lead to the identification of previously undiagnosed CS; accordingly, GCC, even when present as a solitary lesion, may indicate the need for further work-up and screening for CS.
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  • 文章类型: Case Reports
    多形性腺瘤主要影响腮腺(85%),颌下腺(5%),和小唾液腺(5%)。它们通常被称为良性混合肿瘤,因为它们由两种上皮细胞组成,它们是来自身体表面的细胞,和肌上皮细胞,存在于腺体内部以帮助分泌。一名88岁的男性溃疡10天,上颚肿胀3至4个月大,到达OPD。在检查中,在硬腭和软腭的交汇处,有一个单一的,轮廓分明,留在正中一半的小唾液腺的结节状外生性发育(大多数多形性腺瘤是单侧的)。为了进一步确认诊断,细针穿刺细胞学,免疫组织化学试验,和放射诊断,即,X光片,进行的检查显示根尖周脓肿和腭肿胀,与脓液无关。手术切除肿胀并拔除根端。要求患者严格戒烟。术后,患者被给予适当的药物治疗。
    Pleomorphic adenoma affects mostly the parotid gland (85%), submandibular glands (5%), and the minor salivary glands (5%). They are usually referred to as benign mixed tumors because they are composed of both epithelial cells, which are cells from the body\'s surface, and myoepithelial cells, present inside glands to aid in secretion. An 88-year-old male who had an ulcer for 10 days and a three to four-month-old swelling on his palate arrived in the OPD. On examination, at the intersection of the hard and soft palates, there is a single, well-delineated, nodular exophytic development of small salivary glands left to the median half (most of the pleomorphic adenomas are unilateral). For further and confirmatory diagnosis, fine needle aspiration cytology, immunohistochemistry tests, and radiodiagnosis, i.e., X-rays, were performed which revealed periapical abscess and swelling on palate unassociated with pus discharge. Surgical removal of the swelling and extraction of the root stump was done. The patient was asked to stop smoking strictly. Post-operatively, the patient was given suitable medications.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    原发性肝肉瘤是极其罕见的恶性肿瘤之一,占所有原发性恶性肿瘤的不到0.1%。
    一位已知的代偿性肝硬化HCV60岁女性女士MELD评分8分,有控制糖尿病和高血压病史,出现在我们的肝胆诊所,经历了慢性右肩软骨疼痛,持续3个月。O/E有右侧软骨病和上腹部压痛。进行骨盆腹部超声检查,然后进行PETCT检查,发现较大的外生性局灶性病变,约8×7×6cm,FDG摄取最大SUV达到12.4。术前US引导真切活检显示小卵圆形细胞,局灶性纺锤体,肿瘤细胞显示高度染色的多形核,几乎没有细胞质,肿瘤细胞被Desmin和Myogenin染色呈阳性反应,诊断为多形性横纹肌肉瘤。患者计划接受新辅助化疗,然后选择性右半肝切除术。尽管在肿瘤切除过程中遇到了许多挑战,因为肿瘤附着在隔膜的下表面,切除肿瘤的安全边缘为1cm,冷冻组织病理学检查未发现任何恶性细胞。除伤口感染外,患者术后病程顺利,术后第六天出院。
    结论:经皮肝穿刺活检在肝脏横纹肌肉瘤的诊断中起重要作用。新辅助化疗解决了肿瘤的行为,与早期手术干预一起可导致良好的结局并减少复发.
    UNASSIGNED: Primary hepatic sarcomas are one of the extremely rare malignant tumors representing less than 0.1 % of all primary malignant neoplasms.
    UNASSIGNED: A known compensated cirrhotic HCV 60-year-old female lady MELD score 8 with a medical history of controlled diabetes and hypertension presented to our hepatobiliary clinic experiencing a chronic right hypochondrial pain that radiate to her right shoulder of 3 months duration. O/E there was right hypochondrial and epigastric tenderness. Pelvi-abdominal US was done then PET CT which revealed a large exophytic focal lesion measuring about 8 × 7 × 6 cm and achieving 12.4 SUV max on FDG uptake. Pre-operative US guided true-cut biopsy showed small oval rounded cells with focal spindling and the neoplastic cells showed hyperchromatic pleomorphic nuclei with little cytoplasm with a positive reaction of tumor cells stained with Desmin and Myogenin with a diagnosis of pleomorphic rhabdomyosarcoma. The patient was scheduled for neoadjuvant chemotherapy and then elective Right hemihepatectomy. Although many challenges were encountered during the resection of the tumor as the tumor was attached to the under surface of the diaphragm, the tumor was resected with a safety margin of 1 cm with frozen histopathological examination being negative for any malignant cells. The patient\'s postoperative course was uneventful apart from wound infection and was discharged on the postoperative sixth day.
    CONCLUSIONS: The percutaneous liver biopsy plays an important role in the diagnosis of liver\'s rhabdomyosarcomas. Neoadjuvant chemotherapy addresses the behavior of the tumor, together with early surgical intervention can lead to favorable outcomes and reduce the recurrence.
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  • 文章类型: Case Reports
    成人肾脏原发性横纹肌肉瘤(RMS)很少见,只有少数案例发表在文献中。它是与侵袭性和快速临床进展过程相关的间充质肿瘤。我们介绍了一名58岁女性的原发性肾RMS病例,该女性在过去一年中出现间歇性腹痛。计算机断层扫描(CT)扫描显示,中极有20×25×8cm的异质固体块延伸到右肾的下极。因此,患者接受了右肾输尿管根治性切除术。组织病理学检查和免疫组织化学研究证实了具有多形性成分的RMS的诊断。术后,患者出院,无任何并发症,并转诊至肿瘤科医生接受化疗。然而,随访2个月的CT扫描显示广泛的肝转移和局部复发.患者接受吉西他滨和多西他赛,但她的病情恶化了,5个月后她就去世了.原发性肾RMS在成人中很少见。此外,肝转移并不常见,也知之甚少。因此,我们描述了临床病理特征,包括我们病例的临床随访,关注疾病进展,治疗,和结果。
    Primary rhabdomyosarcoma (RMS) of the kidney in an adult is rare, with only a few cases published in the literature. It is a mesenchymal tumor associated with an aggressive and rapid clinical progression course. We present a case of primary renal RMS in a 58-year-old female who presented with intermittent abdominal pain in the past year. The computed tomography (CT) scan revealed a 20×25×8 cm heterogeneous solid mass in the middle pole extended to the lower pole of the right kidney. Therefore, the patient underwent a right radical nephroureterectomy. Histopathology examination and immunohistochemistry studies confirmed the diagnosis of RMS with pleomorphic components. Postoperatively, the patient was discharged without any complications and was referred to an oncologist for chemotherapy. However, a follow-up CT scan in 2 months showed widespread liver metastasis and local recurrence. The patient received Gemcitabine and Docetaxel, but her condition worsened, and she passed away 5 months later. Primary renal RMS is rare in adults. In addition, liver metastasis is uncommon and poorly understood. Hence, we describe the clinicopathologic characteristics, including clinical follow-up of our case, focusing on the disease progression, treatment, and outcome.
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  • 文章类型: Journal Article
    UNASSIGNED: Pleomorphic carcinoma of the lung is a rare malignant epithelial tumor. Due to its rarity, its clinicopathological characteristics are not clear, and there is no defined therapeutic path for this type of tumor.
    METHODS: We retrospectively analyzed the medical and pathological reports of 8 patients who underwent surgical resection for pleomorphic carcinoma between 2007 and 2010.
    UNASSIGNED: Eight patients were analyzed (7 males and 1 female, mean age 60). All patients underwent CT scans, and the average diameter of the nodules was 56 mm. Four patients were also investigated with FDG-PET with hypermetabolic activity in all four cases. In four patients, the carcinomatous component was adenocarcinoma (all with sarcomatoid component of spindle cell and giant cell carcinoma), although in two patients, it was squamous cell carcinoma (one with spindle cell and one with giant cell). In the two remaining patients, one showed a non-small cell carcinoma with giant cell carcinoma, and the other was a non-small cell carcinoma and squamous cell carcinoma with spindle and giant cell carcinoma. All cases were treated with surgical resection. Only two patients underwent neoadjuvant chemotherapy. At the time of data analysis, only one patient treated with neoadjuvant chemotherapy was alive.
    CONCLUSIONS: The prognosis for these patients with a diagnosis of pleomorphic carcinoma undergoing surgery is generally better than those not treated with surgical resection, however the survival remains poor. Although with low number of patients, our research would suggest to consider neoadjuvant chemotherapy an appropriate approach for improving the outcomes before surgery.
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  • 文章类型: Journal Article
    我们介绍一例自发性未分化/未分类肉瘤,以前称为恶性纤维组织细胞瘤(UPS/MFH)的多形性亚型,来自实验室老鼠的胰腺。剖腹手术后从6个月大的雌性实验室Wistar大鼠中切除肿块。它呈现出一个不规则形状的巨大的多叶团块,它来自胰腺,几乎占据了整个腹膜腔。在组织学上,肿瘤的特征是高度可变的形态学模式,频繁地从矩形区域过渡到多形区。广泛的免疫组织化学检查显示没有特定的分化线。免疫组织化学阳性仅观察到MIB-1(高Ki-67增殖指数),波形蛋白和CD68抗体。诊断与UPS/MFH兼容。据我们所知,本病例是首次报道来自实验室大鼠胰腺的自发性原发性UPS/MFH。
    We present a case of spontaneous undifferentiated/unclassified sarcoma, of a pleomorphic subtype formerly known as malignant fibrous histiocytoma (UPS/MFH), arising from the pancreas of a laboratory rat. The mass was excised after laparotomy from a 6-month-old female laboratory Wistar rat. It presented a giant multilobulated mass of irregular shape, which had arisen from the pancreas and occupied almost the entire peritoneal cavity. Histologically the tumor was characterized by a highly variable morphological pattern, with frequent transitions from storiform to pleomorphic areas. An extensive immunohistochemical examination revealed no specific lines of differentiation. Immunohistochemical positivity was observed only to MIB-1 (high Ki-67 proliferation index), vimentin and CD68 antibodies. The diagnosis was compatible with UPS/MFH. To the best of our knowledge, the present case is the first report of a spontaneous primary UPS/MFH arising from the pancreas of a laboratory rat.
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