成人肾母细胞瘤1例报告附文献复习

目的：报告1例罕见的成人肾母细胞瘤（AWT）,结合文献探讨其诊断和治疗。方法：通过病例报告,结合国内、外文献的全面复习,对AWT的临床特点及诊治进行综合分析。结果：AWT临床表现主要为血尿、腰腹部疼痛和腰腹部肿块,应以手术加放、化疗为主要治疗手段。结论：AWT术前与其它肾脏恶性肿瘤难以鉴别,治疗上应尽可能手术切除肿瘤,术后根据病理分期进行辅助性放疗和化疗。     

Hodgkin's Disease with Primary Presentation in the Bone Marrow

A 35-year-old male patient presented with low back pain and a sclerotic lesion of the right sacroiliac joint. Nodular sclerosing Hodgkin's disease (NSHD) was diagnosed on oriented trephine biopsy from this lesion. There was no evidence of lymph node involvement at the time of diagnosis. Four months later pulmonary spread occurred. The clinical course was unusually aggressive, considering the fact that NSHD generally has a relatively favorable prognosis. The patient died 18 months after initial diagnosis with disseminated disease.     

累及骶髂关节原始神经外胚瘤的整块切除与骨盆环重建术一例报道并文献复习

骶骨和骨盆环处恶性肿瘤的保肢治疗具有相当的挑战性,该部位解剖毗邻关系复杂、存在重要脏器和神经血管等常使手术难以达到恶性骨肿瘤的外科边界。恶性肿瘤一旦侵及骶髂关节,手术过程中需要将肿瘤组织包括骶髂关节一并切除,同时重建骨盆环的完整性,必将增加手术的难度。     

Mapping of MAX to human chromosome 14 and mouse chromosome 12 by in situ hybridization.

The protein encoded by the MAX gene is a member of the class of basic region-helix-loop-helix-zipper proteins and has been demonstrated to associate with N-, L-, and c-Myc proteins both in vitro and in vivo. Heterodimers formed between c-Myc and Max proteins have been shown to possess sequence-specific DNA-binding activity. Here we report the mapping of the MAX gene to a single region on human chromosome 14 (bands q22-q24) and to mouse chromosome 12 (region D). Chromosome abnormalities linked to several neoplasms have been previously associated with this region on human chromosome 14. The mapping results also confirm a region of homology between human chromosome 14q22-24 and mouse chromosome 12 region D.     

Second cancers after medulloblastoma: population-based results from the United States and Sweden

Medulloblastoma, one of the most common central nervous system(CNS)tumors in children, requires aggressive multimodality therapy including surgery, radiation therapy, and occasionally chemotherapy. Given its intensive treatment regimen and improved survival during the past 20 years, it is likely that a cohort of survivors will result who may incur consequences of therapy, including a second cancer. We used population-based data from the United States and Sweden to estimate risks of second neo plasms in patients with histologically confirmed medulloblastoma (n = 1,262).Overall, there was a 5.4-fold excess of second neoplasms (95 percent confidence interval = 3.3-8.4) based on 20 observed and 3.7 expected cancers. The second cancers occurred eight to 432 months after initial diagnosis(median, 73 months) with significantly elevated ratios for all intervals examined except for less than one year after initial diagnosis. Significantly elevated risks were seen for cancers of the salivary glands, cervix uteri, brain and CNS, thyroid gland, and acute lymphoblastic leukemia. Of the 15second cancers with treatment data, seven occurred in the radiation field or within areas of scatter while two others may have been radiation-related. Although based on small numbers of second cancers, the results suggest that as survival increases, some patients with medulloblastoma will have an increased risk of a second cancer, particularly a radiation-related cancer. Thus, as survival improves, late-occurring consequences of diagnosis and treatment will need to be carefully assessed. Identification of patients hypersensitive to radiation therapy, such as those with Gorlin Syndrome, should also be attempted in order to reduce the sequelae from intensive radiation exposure. This revised version was published online in July 2006 with corrections to the Cover Date.     

Minute gastric carcinoid tumor with regional lymph node metastasis

We report a patient with a minute gastric carcinoid tumor with lymph node metastasis, and a small gastric cancer. A 50-year-old man having a diagnosis of an elevated lesion on the anterior wall of the gastric body, detected by a series of upper gastrointestinal examinations, was referred to the Cancer Institute Hospital. Careful upper fluoroscopy disclosed a small superficial depressed lesion with converging folds and a superficial elevated lesion covered with nonspecific gastric mucosa. With a final preoperative diagnosis of depressed early cancer and minute carcinoid tumor of the stomach, made by upper gastrointestinal examinations including biopsy, the patient underwent segmental gastrectomy and perigastric lymph node dissection. Histological examination of the resected specimen revealed a lymph node metastasis from a gastric carcinoid tumor of 5-mm diameter, in addition to an early gastric cancer of poorly differentiated adenocarcinoma. Small gastric carcinoid tumors have been regarded as being benign neoplasms biologically. However, the case we present suggests that attention should be paid to the possibility of metastasis at the time of treatment for a minute sporadic gastric carcinoid tumor. We therefore discuss the malignant potential of these tumors, mainly from the viewpoint of histopathological classification, to gain understanding so that the patients can be treated adequately.     

合并膝关节急性疼痛的强直性脊柱炎早期临床特点分析

【摘要】目的了解以膝关节急性肿痛为首发症状的强直性脊柱炎（ankylosingspondylitis,AS）早期病情特征,以提高诊治水平。方法对2009年12月至2012年12月,我院经治的21例合并膝关节急性肿痛强直性脊柱炎患者的早期临床特点、影像学及实验室检查结果进行回顾性分析。结果年龄17—45岁。21例首发症状以患侧膝关节肿痛为主。膝关节腔积液黄白色、浑浊,涂片镜检白细胞4＋,膝关节滑膜组织为炎性肉芽组织。血沉、C反应蛋白、纤维蛋白原均高于正常,18例HLA—B27阳性。磁共振提示膝关节滑膜增生、关节腔内大量积液。16例骶髂关节及脊柱x线片显示,均无明显异常改变。结论强直性脊柱炎发病隐匿,往往以膝关节急性肿痛为首发症状,误诊率及漏诊率高。加强年轻患者早期筛查、需仔细询问病史、详细查体、密切观察病情变化,对强直性脊柱炎早期诊治有价值。     

Ultrasound localization of the ovaries for radiation-induced ovarian ablation

The case notes and simulator films were reviewed from 70 sequential patients who received pelvic irradiation to induce an early menopause as part of their treatment for breast cancer at the Staffordshire Oncology Centre. These patients underwent ultrasound localization of the ovaries immediately prior to simulation. Altogether, 128 ovaries were plotted on a diagrammatic representation of a gynaecoid pelvis to represent their position in both craniocaudal and lateral dimensions in relation to the true bony pelvis.The craniocaudal ovarian position varied from 2.5 cm above the lower aspect of the sacroiliac joint to 2.0 cm above the symphysis pubis. Three (4.6%) right sided ovaries were within I cm medial to the right lateral side wall, with none lying lateral to the wall. Seventeen (26%) left sided ovaries were lying within 1 cm of the left pelvic side wall, with four of these lying outside.The limits of the pelvic fields used were from the top of the sacroiliac joint to the bottom of the symphysis pubis. Sixty-one (88%) upper borders were on or above the lower sacroiliac joint. Twenty six (38%) and 49 (71%) fields were outside the right and left pelvic side walls respectively. This would suggest that field sizes were larger than standard; however, 87% were smaller than 150 cm² (assuming a 10 × 15 cm field as standard). Only one patient failed to respond to treatment. This was thought to be due to underdosing rather than a geographical miss. This patient was successfully retreated.The authors advocate the use of ultrasound localization prior to planning an irradiation menopause, to ensure that the ovaries are encompassed in the pelvic field, thus preventing a geographical miss and reducing field sizes.     

Cytogenetic abnormalities in natural killer cell lymphoma/leukaemia--is there a consistent pattern?

The group of putative natural killer cell lymphomas, also known as NK/T cell lymphomas (nasal and nasal-type) has been characterized only in recent years. Whilst a good amount of clinical information has been gathered on this group of uncommon lymphoid neoplasms, there is little information on the cytogenetic or molecular alterations. A review of the literature shows that chromosomal abnormalities are indeed commonly found in these tumours, and aberrations involving chromosome 6q are most frequent. Other non-random abnormalities include +X, i(1q), i(7q), +8, del(13q), del(17p), i(17q), and 11q23 rearrangement. It appears that deletions of the chromosome 6 at around q21-23 region is the commonest recurrent chromosomal abnormality, and fluorescence in situ hybridisation studies have confirmed the occurrence of deletions at 6q22-23 in the CD3- CD56+ tumour cells. Search for the involved genes located in this chromosome region can potentially shed light on the molecular pathogenesis of the natural killer cell neoplasms.     

以外周关节炎为首发的青少年强直陛脊柱炎的诊断特点

目的探讨以外周关节炎为首发的青少年强直性脊柱炎（AS）的诊断特点,避免误诊误治。方法回顾性分析我院2003年1月～2008年1月诊治的38例以外周大关节为首发症状的青少年强直性脊柱炎的临床特点。结果38例9～18岁的以外周关节炎为首发的青少年AS男性患者,外周关节炎首发部位分别为膝关节18例,踝关节8例,髋关节7例,肘关节3例,足跟末端跟腱2例。骶髂关节X线拍片或CT检查,单侧Ⅰ级者18例,双侧Ⅰ级者9例,单侧Ⅱ级者6例,双侧Ⅱ级者4例,Ⅲ级者1例,其中Ⅰ级和Ⅱ级单侧者共33例（86．8％）,双侧Ⅱ级以上者共5例（13．2％）。所有患者HLA—B27阳性。结论青少年AS的临床特点是以外周关节炎／滑膜炎为首发主要症状,缺乏典型的影像学表现,早期诊断主要根据病史、体征和实验室检查,X线检查一般不作为主要依据。     

Correlation of serum interleukin-2 levels, soluble interleukin-2 receptors and T lymphocyte subsets in cancer patients

An interleukin-2 (IL-2) in vitro reduced production has been observed in most metastatic cancer patients. At present, however, there are no data on blood IL-2 levels in vivo, because of the too low sensitivity of previous biological and enzyme immunoassay methods. The recent development of a sensitive RIA method allowed us to start a preliminary investigation of IL-2 production in basal conditions in human solid tumors. The study included 42 cancer patients. Breast and lung cancer were the two commonest neoplasms. Serum levels of IL-2 and soluble IL-2 receptors (SIL-2R), and CD4/CD8 ratio were measured in each patient. The control group consisted of 58 healthy subjects. Mean serum levels of IL-2 were significantly lower in metastatic patients (n = 23) than in those without metastases (n = 19). Patients with low CD4/CD8 ratio (n = 16) had significantly lower mean values of IL-2 than those with normal ratio (n = 26). Finally, mean IL-2 concentrations were significantly lower in patients with elevated levels of SIL-2R than in those with normal values. These results would suggest that metastatic dissemination is associated with a decreased IL-2 production in vivo, and that reduced IL-2 production is more frequent in patients with low CD4/CD8 ratio.     

Pelvic insufficiency fractures in patients with pelvic irradiation

BACKGROUND: Insufficiency fractures (IF) occur as a result of normal physiological stress on bones with deficient elastic resistance. Pelvic insufficiency fractures are a complication of osteoporosis due to postmenopausal status, high dose of corticosteroids, or local irradiation. They are important because differential diagnosis includes pelvic bone metastases. Diagnosis is based on both clinical manifestations and radiographic and scintigraphic findings. METHODS AND MATERIALS: We examined eight patients with pelvic cancer who had previously undergone external beam radiation therapy as part of their treatment. In the follow-up, they developed insufficiency fractures, and no factor other than pelvic irradiation was present. Diagnosis was confirmed by radionuclide bone scan followed by conventional radiography and computed tomography (CT) scan. RESULTS: The average onset of symptoms was 13.7 months after radiation therapy was completed. The initial symptom in all cases was pain. In all of the patients, the bone scan showed abnormalities. One to four increased uptake foci were observed, in the sacroiliac joint in all cases, and in the pubis in three cases. The initial diagnosis was bone metastases in five patients. CT scan showed fractures in all of the patients, in sacrum and pubis, both endostic and cortical. Treatment, consisting of nonsteroidal anti-inflammatory drugs and rest, led to symptomatic relief in all cases. CONCLUSION: Knowledge of pelvic insufficiency fractures is essential in order to rule out metastasic disease, and thus avoid inaccurate treatment. Although radionuclide bone scan is useful in early detection of pelvic IF, definitive diagnosis is provided by CT scan.     

Magnetic resonance and computed tomography imaging of a grade IV papillary endolymphatic sac tumour

The endolymphatic sac tumour (ELST) is an adenomatous neoplasm of the papillary pattern originating from the endolymphatic sac's epithelium. We describe the computed tomography and magnetic resonance imaging features of a rare grade IV tumour with extensive skull base, cerebello-pontine and nasopharyngeal spread as well as involvement of the left temporomandibular joint. Papillary ELST may easily be misinterpreted on histopathological and even on immunohistochemical examination with other papillary lesions. Thus the radiological imaging features and localization in conjunction with histopathological features and clinical presentation play a paramount role in making the correct diagnosis.     

关节内原发恶性肿瘤九例报告

目的：报告9例关节内原发恶性肿瘤病例,探讨合理的诊断方法及治疗方案。方法2001年8月至2011年7月共收治关节内原发恶性肿瘤的9例,其中男6例、女3例,年龄21～76岁、平均43.8岁。7例滑膜肉瘤（均为膝关节）,1例脂肪肉瘤（膝关节）,1例恶性弥漫性巨细胞瘤（髋关节）。回顾其临床表现、影像特点、诊断途径、手术方式,评价术后关节功能、并发症及转归。结果9例入院前均有关节疼痛,MRI典型表现为T1加权像低信号,T2加权像高信号或混杂信号伴明显强化,均接受手术治疗,术后平均随访27.5（12～60）个月,中位随访27个月,4例最终行截肢术,另5例保肢治疗后功能良好。结论关节内原发恶性肿瘤发病率极低;易误诊为良性病变。本组病例以膝关节滑膜肉瘤多见;术前需活检明确诊断,根据病理结果制订治疗方案（行新辅助化疗或术后放化疗）,患者能有良好的预后。根据MRI评估关节受累情况,需设计良好的外科切除边界;获得有效的肿瘤切除。     

Erosive Spondylo-Arthropathy and Tertiary Hyperparathyroidism

A patient on long term haemodialysis for analgesic nephropathy, with known teriary hyperparathyroidism developed a rapidly evolving, erosive, non-infective spondylo-arthropathy at two vertebral levels. In addition, erosions were noted adjacent to the right sacroiliac joint. Subsequent postmortem material from the affected vertebral levels demonstrated changes consistent with hyperparathryoidism alone and this is suggested as the possible aetiology underlying this recently recognised destructive spondyloarthropathy.     

Impact of the COVID-19 Pandemic on Colorectal Cancer Care in the Netherlands: A Population-based Study

IntroductionThe COVID-19 pandemic disrupted health care services worldwide. In the Netherlands, the first confirmed COVID-19 infection was on February 27, 2020. We aimed to investigate the impact of the pandemic on colorectal cancer care in the Netherlands.MethodsColorectal cancer patients who were diagnosed in 25 hospitals in weeks 2 to 26 of the year 2020 were selected from the Netherlands Cancer Registry (NCR) and divided in 4 periods. The average number of patients treated per type of initial treatment was analyzed by the Mantel-Haenszel test adjusted for age. Median time between diagnosis and treatment and between (neo)adjuvant therapy and surgery were analyzed by the Mann Whitney test. Percentages of (acute) resection, stoma and (neo)adjuvant therapy were compared using the Chi-squared test.ResultsIn total, 1,653 patients were included. The patient population changed during the COVID-19 pandemic regarding higher stage and more clinical presentation with ileus at time of diagnosis. Slight changes were found regarding type of initial treatment. Median time between diagnosis and treatment decreased on average by 4.5 days during the pandemic. The proportion of colon cancer patients receiving a stoma significantly increased with 6.5% during the pandemic. No differences were found in resection rate and treatment with (neo)adjuvant therapy.ConclusionDespite the disruptive impact of the COVID-19 pandemic on global health care, the impact on colorectal cancer care in the Netherlands was limited.     

Changes in treatment patterns and their influence on long‐term survival in patients with stages I–III gastric cancer in The Netherlands

Studies investigating perioperative chemotherapy and/or radiotherapy changed the treatment of curable gastric cancer in The Netherlands. These changes were evaluated including their influence on survival. Data on patients diagnosed with gastric cancer from 1989 to 2009 were obtained from The Netherlands Cancer Registry. Changes over time in surgery and administration of perioperative chemotherapy, 30‐day mortality, 5‐year survival and adjusted relative excess risk (RER) of dying were analyzed with multivariable regression for cardia and noncardia cancer. In stages I and II disease, most patients underwent surgery. Since 2005, more patients are treated with (neo)adjuvant chemotherapy. Postoperative mortality ranged from 1% to 7% and 0.4% to 12.2% in cardia and noncardia cancer (<55 to 75+ years). Five‐year survival for cardia cancer and noncardia cancer stages I–III and X (unknown stage) was 33% and 50% (2005–2008). The RER of dying was associated with period of diagnosis, age, gender, region, stage, (neo)adjuvant chemotherapy in case of cardia cancer and type of gastric resection in case of noncardia cancer. Administration of (neo)adjuvant chemotherapy has increased. No improvement in long‐term survival could yet be seen, though it is still too early to expect an improvement in survival as a result of the use of chemotherapy.     

CT导向盆腔深部肿块穿刺切割活检术不同穿刺路径的可行性探讨

目的 探讨根据盆腔深部肿块位置不同,设计相应穿刺路径进行CT导向下经皮穿刺切割活检术(PINB)的可行性.方法 70例患者的72个盆腔新发肿块,根据肿块部位以及肿块与周围组织脏器的关系,以避开重要脏器、组织为原则,采取不同穿刺体位和路径,在CT导向下将穿刺活检针准确置入盆腔肿块区行切割活检,评价穿刺结果 和安伞性.结果 采用俯卧位经梨状肌、臀肌、后斜径路穿刺的肿块分别为27、16和5个,采用仰卧位经前或前侧径路、髂肌径路、直接经盆骨穿刺径路的穿刺肿块各8个.72个肿块中,恶性64个,其中腺癌30个,鳞癌19个,未能分型恶性肿瘤细胞5个,小细胞癌3个,恶性骨巨细胞瘤和肝细胞癌各2个,假阴性3个;良性8个,其中纤维组织6个,骨结核和卵巢囊肿各1个.PINB诊断敏感件为95.3%(61/64),特异性为100%(8/8),准确性为95.8%(69/72).22例经梨状肌穿刺的患者出现局部一过性针刺样疼痛,并向下肢放射,未行特殊处理.70例患者术后均未出现明显血肿、神经损伤、盆腔感染和肿瘤种植等并发症.结论 在CT导向下,根据盆腔区肿块位置及比邻关系,设计不同路径行PINB,不仪安全,且成功率和准确率较高.     

Gout‐mimicking sarcoma recurrence at a prosthesis bone interface remote from any joint

Gout is known to occur in a variety of organs but most commonly presents as an inflammatory arthropathy. A few reported cases have documented its occurrence in prosthetic neo‐joints or juxta‐articular to this. We present the first reported case at a bone prosthesis interface remote from any joint and mimicking sarcoma recurrence because of its unusual location.