Rare neoplasm

  • 文章类型: Case Reports
    阑尾印戒细胞癌是一种极为罕见的肿瘤,仅占阑尾癌的4%。这是一种罕见的腹痛原因,可以模拟急性阑尾炎。该病例报告了一名77岁的女性,她因主诉右下腹腹痛而出现在急诊室。经过探究性腹腔镜检查和组织病理学研究,患者被发现患有腹膜癌和阑尾印戒细胞癌。由于其侵袭性,该诊断不幸具有相对较差的预后。这项研究讨论了病因,患病率,临床发现,和治疗腹痛的罕见原因。本报告阐明了早期发现和治疗阑尾印戒细胞癌的重要性。
    Appendiceal signet ring cell carcinoma is an exceedingly rare neoplasm which makes up only 4% of carcinomas of the appendix. It is a rare cause of abdominal pain which can mimic acute appendicitis. This case reports a 77-year-old female who presented to the emergency room with a complaint of right lower quadrant abdominal pain. After exploratory laparoscopy and histopathological studies, the patient was found to have peritoneal carcinomatosis and appendiceal signet ring cell carcinoma. This diagnosis unfortunately carries a relatively poor prognosis due to its aggressive nature. This study discusses the etiology, prevalence, clinical findings, and treatment of a rare cause of abdominal pain. This report sheds light on the importance of early detection and treatment of appendiceal signet ring cell carcinoma.
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  • 文章类型: Case Reports
    胶体肺腺癌是临床实践中很少遇到的肿瘤。这种罕见肿瘤的诊断过程因其频率低和对其特定分子影像学特征的了解有限而变得复杂。我们报告了一名65岁的男性,他被诊断患有肺胶体粘液性囊腺癌。进行氟18-氟脱氧葡萄糖(18F-FDG)正电子发射断层扫描/计算机断层扫描(PET/CT)进行初步评估。扫描显示原发性肿瘤部位有轻度的18F-FDG表达,一些非18F-FDG-高活性纵隔和食管旁淋巴结表现出可疑的形态学特征。由于持续的心房颤动,最初的组织病理学确认原发肿瘤具有风险感,提示在进行之前必须进行心脏病学评估。相反,进行了镓-68标记的成纤维细胞激活蛋白抑制剂(68Ga-FAPI)PET/CT,在胶体粘液组织学中,期望这在恶性潜能方面比18F-FDGPET更具信息。扫描显示在原发肿瘤部位有中等的68Ga-FAPI表达,但在可疑淋巴结有不显著的68Ga-FAPI表达。随后,纵隔淋巴结(左主动脉旁)经支气管内超声(EUS)活检显示良性结果.患者接受同步放化疗治疗。该病例强调了68Ga-FAPIPET/CT在罕见癌症的特定病例中的重要作用。特别是当组织活检的侵入性检测是不可行的。
    Colloid pulmonary adenocarcinoma represents a seldom encountered neoplasm in clinical practice. The diagnostic process for this rare neoplasm is complicated by its infrequency and the limited understanding of its specific molecular imaging characteristics. We report a 65-year-old male who was diagnosed with pulmonary colloid mucinous cystadenocarcinoma. Fluorine 18-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) was conducted for initial evaluation. The scan showed mild 18F-FDG expression at the primary tumor site, and several non-18F-FDG-avid mediastinal and paraesophageal lymph nodes exhibited suspicious morphologic features. Owing to the ongoing atrial fibrillation, initial histopathological confirmation of the primary tumor mass carries a sense of risk, prompting the imperative for cardiological assessment before proceeding. Instead, Gallium-68-labeled fibroblast activation protein inhibitor (68Ga-FAPI) PET/CT was performed, expecting this to be more informative in terms of malignancy potential than 18F-FDG PET in colloid mucinous histology. A scan revealed moderate 68Ga-FAPI expression at the primary tumor site but unremarkable 68Ga-FAPI expression at the questionable lymph node. Subsequently, a biopsy from a mediastinal node (left para-aortic) lymph node via endobronchial ultrasound (EUS) showed benign findings. The patient was treated with concurrent chemoradiation. This case underscores the vital role that 68Ga-FAPI PET/CT can play in specific cases of rare cancers, especially when invasive testing for tissue biopsy is not feasible.
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  • 文章类型: Case Reports
    乳腺原发性肉瘤是极为罕见且异质的恶性肿瘤;它们应与叶状肿瘤区分开。它们的特点是复发率高,快速增长和积极粗糙。我们介绍了一例41岁的巴勒斯坦女性,她反复出现乳房肿块。活检显示原发性肉瘤,影像学证实腋窝淋巴结转移。患者接受了六个周期的异环磷酰胺-阿霉素方案,导致完全的病理反应。她接受了左侧改良根治术,然后进行放射治疗,并再进行了六个周期的辅助手术。原发性乳腺肉瘤是罕见的肿瘤,需要多学科讨论来指导治疗。这些肿瘤的方法是化疗,然后在可手术时进行手术切除,除了通过放疗和辅助化疗进行局部控制。
    Primary sarcomas of the breast are extremely rare and heterogenous malignancies; they should be differentiated from phyllodes tumors. They are characterized by their high rate of recurrence, rapid growth and aggressive coarse. We present a case of a 41-year-old Palestinian female who presented with a recurrent breast mass. Biopsy showed primary sarcoma and imaging confirmed metastasis to axillary lymph nodes. The patient received six cycles of ifosfamide-adriamycin protocol resulting in complete pathological response. She underwent left sided modified radical mastectomy followed by radiation and six more cycles of the same protocol as adjuvant. Primary breast sarcomas are rare neoplasms that require multidisciplinary discussion to guide treatment. The approach to these tumors is chemotherapy followed by surgical resection when operable, in addition to local control via radiotherapy and adjuvant chemotherapy.
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  • 文章类型: Case Reports
    炎性肌纤维母细胞瘤(IMT)是来源于间充质细胞的罕见梭形细胞肿瘤。原发性泌尿生殖系统IMT与各种恶性梭形细胞肉瘤具有相同的形态学和分子特征,这带来了诊断挑战。我们介绍了一名50岁女性的病例,该女性被转诊以评估血尿和非特异性泌尿症状,并被发现有源自膀胱的肿块,累及子宫颈和右卵巢。经尿道膀胱肿瘤切除术(TURBT)和免疫组织化学分析显示IMT。据我们所知,这是记录的首例原发性泌尿生殖系统IMT伴宫颈和卵巢受累的病例.
    Inflammatory myofibroblastic tumors (IMT) are rare spindle cell neoplasms derived from mesenchymal cells. Primary genitourinary IMTs share morphological and molecular features with various malignant spindle cell sarcomas, which introduces a diagnostic challenge. We present the case of a 50-year-old female who was referred for evaluation of hematuria and nonspecific urinary symptoms and was found to have a mass originating from the urinary bladder that involved the cervix and right ovary. Transurethral resection of bladder tumor (TURBT) and immunohistochemical analysis revealed an IMT. To our knowledge, this is the first documented case of primary genitourinary IMT with cervical and ovarian involvement.
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  • 文章类型: Case Reports
    中枢神经系统肉瘤是罕见的间质起源肿瘤。血管肉瘤是一种罕见的亚型,预后不良,对治疗方法尚无共识。
    本文介绍了一名50岁的巴勒斯坦女性患者的案例,该患者有治疗过的乳腺癌病史,该患者因强直阵挛性癫痫发作而被送往急诊室。脑计算机断层扫描显示,通过开颅手术和大体肿瘤切除术治疗了脑占位性病变。组织病理学显示上皮样脑血管肉瘤。患者接受替莫唑胺同步放化疗治疗,导致肿瘤消退以及症状和主诉。
    原发性脑上皮样血管肉瘤是一种病因不明的高度恶性肿瘤。患者的乳房病史造成了额外的挑战,因为必须排除有转移的复发。血液产品降解的迹象是一个重要的放射学特征。根据现有文献,大体肿瘤切除后同步放化疗提供了最佳的方法和结果.
    脑血管肉瘤是一种罕见的疾病,由于现有文献的匮乏,其治疗方法具有挑战性。应纳入占位性病变的鉴别诊断,尤其是那些有放疗史的人。免疫组织化学是诊断的关键。与单独的任何一种选择相比,手术切除后同步放化疗与更长的无病生存期相关。
    UNASSIGNED: Central nervous system sarcomas are rare tumors of mesenchymal origin. Angiosarcomas are an even rarer subtype with poor prognosis and no consensus regarding therapeutic approach.
    UNASSIGNED: This article presents the case of a 50-year-old Palestinian female patient with a history of treated breast cancer who presented to the emergency room with a tonic-clonic seizure. Brain computed tomography showed a cerebral space-occupying lesion managed with craniotomy and gross tumor resection. Histopathology revealed an epithelioid cerebral angiosarcoma. The patient was treated with concurrent chemoradiotherapy with temozolomide resulting in the resolution of the tumor as well as symptoms and complaints.
    UNASSIGNED: Primary cerebral epithelioid angiosarcoma is a highly malignant tumor of an unknown etiology. The patient history of breast created an additional challenge, as recurrence with metastasis had to be excluded. Signs of blood product degradation are an important radiological feature. According to existing literature, gross tumor resection followed by concurrent chemoradiotherapy offers the best approach and outcome.
    UNASSIGNED: Cerebral angiosarcoma is a rare disease with a challenging therapeutic approach due to the scarcity of available literature. It should be included in the differential diagnosis of space-occupying lesions, especially in those with a history of exposure to radiotherapy. Immunohistochemistry is key for diagnosis. Surgical resection followed by concurrent chemoradiotherapy is associated with a longer disease-free survival when compared to either option alone.
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  • 文章类型: Journal Article
    背景:阴道中肾腺癌(MA)是一种罕见的肿瘤,起源于女性生殖道中肾残留物(Wolffian)。这是一种肿瘤,没有关于其诊断的重要证据,治疗,随访和预后。
    方法:在Scopus进行了文献的系统研究,PubMed/MEDLINE,ScienceDirect和Cochrane图书馆,包括观察性前瞻性和回顾性研究,病例系列和病例报告。我们收集了与诊断和治疗方案相关的研究数据,评估了以下方面:研究设计,人口,治疗类型,手术并发症发生率和生育结局。我们进一步纳入了一例腹腔镜治疗MA的病例报告。
    结果:文献中有13例阴道MA,包括我们的病例报告.诊断时的中位年龄为52岁;大多数患者报告阴道出血为症状(38%);超声检查,其次是磁共振和CT扫描是最常用的诊断工具.在54%的案例中,进行了手术活检,92%的患者接受了开放入路或阴道切除术的前期手术,除了一例完全通过微创手术治疗。大多数患者(68%)接受了化疗或放疗或其组合的辅助治疗。平均随访期为6年。
    结论:尽管这种癌症罕见且位置怪异,经过多学科评估,微创方法似乎是可行的。根据这种肿瘤的稀有性,任何未来的病例和后续数据都必须在文献中报告,以扩大相关知识。
    BACKGROUND: Mesonephric adenocarcinoma (MA) of the vagina is a rare tumor that arises from mesonephric remnants (Wolffian) in the female genital tract. It is a neoplasm with no significant evidence about its diagnosis, treatment, follow-up and prognosis.
    METHODS: Systematic research of the literature was conducted in Scopus, PubMed/MEDLINE, ScienceDirect and the Cochrane Library, including observational prospective and retrospective studies, case series and case reports. We collected data regarding studies related to diagnosis and treatment options evaluating the following aspects: study design, population, treatment type, rate of surgical complications and fertility outcome. We further included a case report of laparoscopic management of MA with pictorial assays.
    RESULTS: Thirteen cases of MA of the vagina are available in the literature, including our case report. The median age at diagnosis was 52 years old; the majority of patients reported vaginal bleeding as a symptom (38%); and ultrasound, followed by a magnetic resonance and CT scan were the diagnostic tools most used. In 54% of the cases, a surgical biopsy was performed, and 92% of the patients underwent upfront surgery with an open access or vaginal resection except one case fully managed by minimally invasive surgery. Most of the patients (68%) received adjuvant treatment with chemotherapy or radiotherapy or a combination of them. The mean follow-up period was 6 years.
    CONCLUSIONS: Despite the rarity of this cancer and bizarre location, a minimally invasive approach seems feasible after multidisciplinary evaluation. According to the rarity of this tumor, any future case and follow-up data must be reported in the literature in order to enlarge the knowledge about it.
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  • 文章类型: Journal Article
    本章中介绍的肿瘤有些非常罕见,由于有关其起源细胞的信息匮乏,他们的管理仍在争论中,行为,和生物学。治疗选择仍然有限,但我们相信,在不久的将来,通过发现驱动肿瘤生长的遗传和生物学机制,我们将能够提供新的靶向治疗,应该是手术的侧翼,放射治疗,和实际使用的化学治疗剂。本章的目的是强调这些肿瘤的最重要的已知特征,提供识别疾病的机会,然后为患者提供最佳的治疗机会。第5次WHO分类中枢神经系统通过进一步推进分子诊断在中枢神经系统肿瘤分类中的作用而具有实质性变化。但仍然植根于其他既定的肿瘤表征方法,包括组织学和免疫组织化学,而且很可能,许多肿瘤的类别会发生变化。这里,总结了每种肿瘤最重要的特征,重点是遗传机制和分子途径,他们的组织病理学足迹,症状和体征,放射学特征,治疗方法,和预后以及随访方案。还提供了示意性分类以提供对病理学的更好理解。
    The presented tumors in this chapter are somewhat very rare, and their management is still debated due to the scarcity of information about their cell of origin, behavior, and biology. Treatment options are still limited, but we are confident that in the near future by discovering the genetic and biological mechanisms that drive tumor growth we will be able to offer new target therapies that should be flanked by surgery, radiotherapy, and chemotherapeutic agents actually in use. The purpose of this chapter is to highlight the most important known characteristics of these tumors offering the chance to recognize the disease and then offer the best opportunity for treatment to patients. The 5th WHO Classification Central Nervous System features substantial changes by moving further to advance the role of molecular diagnostics in CNS tumor classification, but remaining rooted in other established approaches to tumor characterization, including histology and immunohistochemistry, and probably, the category of many tumors will change. Here, the most important characteristics of each neoplasm are summarized focusing on genetic mechanisms and molecular pathways, their histopathologic footprints, signs and symptoms, radiologic features, therapeutic approaches, and prognosis as well as follow-up protocols. Schematic classifications are also presented to offer a better understanding of the pathology.
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  • 文章类型: Case Reports
    Splenic rupture secondary to blunt trauma is a common condition. Non-traumatic, also known as spontaneous or pathological splenic rupture is an uncommon, but potentially life-threatening condition. Spontaneous splenic rupture caused by a primary splenic tumor is rare. In this case study, we present a special, benign tumor causing splenic rupture. Our 78-year-old female patient was hospitalized due to left shoulder pain and chest discomfort. Her blood pressure was low, the laboratory tests showed anemia, and the chest CT scan involving also the upper abdomen raised the suspicion of a splenic rupture. During the emergency splenectomy, there was a large amount of blood in the abdominal cavity. Macroscopic pathological examination of the removed spleen showed multifocal cystic lesions that led to splenic rupture. Immunhistochemical analyses revealed a littoral cell angioma. Littoral cell angioma is a rare, benign vascular tumor of the spleen, which is thought to originate from the red pulp sinuses lined with littoral cells. The aim of our report is to describe an unusual cause of sudden splenic rupture without traumatic history, the histologically benign littoral cell angioma that has not been published in Hungary. Orv Hetil. 2023; 164(10): 393-397.
    A traumás lépruptura gyakori sérülés. A nem traumás, más néven spontán vagy patológiás lépruptura viszont ritka, de potenciálisan életveszélyes állapotot okozó betegség. A spontán lépruptura primer lépdaganat miatt történő kialakulása ritkaság. Tanulmányunk egy léprepedést okozó, különleges, jóindulatú daganat esetét mutatja be. 78 éves nőbetegünk bal vállfájdalom és mellkasi diszkomfort miatt került kórházba. Vérnyomása alacsony volt; a laborvizsgálatok anaemiát mutattak ki, a felhasi területet is leképező mellkas-CT lépruptura gyanúját vetette fel. A sürgősséggel elvégzett splenectomia során a hasüregben nagy mennyiségű vér volt. Az eltávolított lép patológiai vizsgálata makroszkóposan multifokális cysticus elváltozást talált, mely a léprepedéshez vezetett. Mikroszkóposan az immunhisztokémiai vizsgálatok dongasejtes angiomát igazoltak. A dongasejtes angioma egy lépben keletkező ritka, jóindulatú érdaganat, amelyről úgy gondolják, hogy a dongasejtekkel bélelt vörös pulpasinusokból származik. Közleményünk célja, hogy ismertesse a traumás előzmény nélkül, hirtelen bekövetkezett léprepedés egy szokatlan okát, a Magyarországon még nem publikált jóindulatú lépmegbetegedést, a dongasejtes angiomát. Orv Hetil. 2023; 164(10): 393–397.
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  • 文章类型: Journal Article
    简介:恶性孤立性胸膜纤维性肿瘤(mSFTP)是极其罕见的疾病(小于所有胸膜肿瘤的5%),具有不可预测的行为。手术仍然是这些肿瘤的护理标准;然而,估计患者预后和规划随访仍然具有挑战性.已经提出了几种风险分层模型,但是具有诊断和预后潜力的分类尚未完全标准化。这项研究的目的是分析mSFTP的临床病理数据,以调查其预后特征,并比较文献中提出的三种风险分层模型的性能。方法:对2000年至2019年在单个中心进行根治性手术切除的所有经证实的mSFTP病例进行观察性回顾性队列研究。人口统计,检查手术和病理资料。所有患者都使用三种预测模型进行风险分层:改良的Demicco,DePerrot和Tapias。分析总生存期(OS)和无病生存期(DFS)。结果:有21名男性和13名女性(中位年龄,67年,范围,23-83岁)。21例患者(62%)出现症状。中位随访时间为111个月(范围,6-258个月)。5年OS和DFS分别为81.2%和77.4%,分别。9名患者(26.5%)尝试复发。在单变量分析中,坏死的存在(p=0.019),核异型性(p=0.006),尺寸大于11.5cm(我们队列的中位值)(p=0.037)和复发/疾病进展(p=0.001)是OS恶化的独立预后因素.辅助治疗的施用是存活的保护性独立因素(p=0.001)。切除的根治性(p=0.005);肿瘤尺寸(p=0.013),存在坏死(p=0.041)、核异型性(p=0.007)和胸膜模式(p=0.011)是DFS恶化的独立预后因素。分析了三种风险分层模型,Tapias评分是预测mSFTP患者OS(p=0.002)和DFS(p=0.047)的最佳指标.结论:使用Tapias提出的风险分层模型,可以在手术时确定复发风险最高的患者,以建立更频繁的影像学监测和更长的随访时间.辅助治疗在mSFTP治疗中的作用尚未确定,但是对复发风险高的患者进行进一步分析,根据风险模型分层,随着生物分子面板可以定制未来的术后治疗。
    Introduction: Malignant solitary fibrous tumours of the pleura (mSFTP) are extremely rare diseases (<5% of all pleural neoplasms) with unpredictable behaviour. Surgery remains the standard of care for these tumours; however, estimating patient prognosis and planning follow-up remain challenging. Several risk stratification models have been proposed, but a classification with diagnostic and prognostic potential has not been well standardised yet. The aim of this study was to analyse the clinicopathological data of mSFTP to investigate their prognostic features and to compare the performance of three risk stratification models proposed in the literature. Methods: Observational retrospective cohort study on all proven cases of mSFTP surgically resected with radical intent between 2000 and 2019 in a single centre. Demographic, surgical and pathological data were examined. All patients were risk-stratified by using three prediction models: modified Demicco, De Perrot and Tapias. Overall survival (OS) and disease-free survival (DFS) were analysed. Results: There were 21 men and 13 women (median age, 67 years, range, 23-83 years). Twenty-one patients (62%) were symptomatic. The median follow-up was 111 months (range, 6-258 months). The 5-year OS and DFS were 81.2% and 77.4%, respectively. Nine patients (26.5%) experimented recurrences. At univariate analysis, the presence of necrosis (p = 0.019), nuclear atypia (p = 0.006), dimension greater than 11.5 cm (median value of our cohort) (p = 0.037) and relapse/disease progression (p = 0.001) were independent prognostic factor of worse OS. The administration of adjuvant treatment was a protective independent factor for survival (p = 0.001). Radicality of resection (p = 0.005); tumour dimension (p = 0.013), presence of necrosis (p = 0.041) and nuclear atypia (p = 0.007) and pleural pattern (p = 0.011) were independent prognostic factors of worse DFS. Analysing the three risk stratification models, the Tapias score was revealed as the best index to predict both OS (p = 0.002) and DFS (p = 0.047) in patients with mSFTP. Conclusions: Using the risk stratification model proposed by Tapias, patients with the highest risk of recurrence could be identified at the time of surgery to establish a more frequent imaging surveillance and longer follow-up. The role of adjuvant treatment in mSFTP therapy has not been established yet, but further analysis on patients with a high risk of recurrence, stratified according to risk models, along with biomolecular panels may tailor future post-surgical therapies.
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  • 文章类型: Case Reports
    系统性囊性血管瘤病(SCA)是一种罕见的疾病,通常涉及内脏器官,在其阴险过程中进行偶然检测。在射线照相术上,它可以表现为多发性囊性病变。在极少数情况下,它可以表现为混合病变(溶解性,硬化)就像我们的病人一样。该疾病比大多数累及骨骼的血管肿瘤具有更好的预后。我们提出了一种罕见的这种疾病,涉及多个器官,并表现出一种阴险的发作。
    Systemic cystic angiomatosis (SCA) is a rare disorder, usually involving the visceral organs with incidental detection during its insidious course. On radiography, it can present as multiple cystic lesions. In rare instances, it can present as mixed lesions (lytic, sclerotic) as was the case with our patient. The disease has a better prognosis than most vascular neoplasms involving the bones. We present a rare case of this disease, involving multiple organs, and presenting with an insidious onset.
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