Myoepithelioma of the soft tissue: A systematic review of clinical reports

BackgroundMyoepithelioma of the soft tissue (MES) is a rare neoplasm, and available literature includes few studies with small sample sizes, thus limiting the comprehension of this disease. Hence, a systematic review was performed to summarize the available information on MES patients evaluated for surgical treatment.MethodsThe MEDLINE/PubMed, EMBASE and SCOPUS databases were reviewed for eligible studies in January 2019. Inclusion criteria were: patients with MES; indication for wide resection or amputation; observational studies; articles published from 1997 to 2018; English language; reporting of outcome measures such as overall survival (OS), disease-free survival (DFS), recurrence, metastases. Studies not including humans were excluded. Quality appraisal was performed using the Methodological Index for Non-Randomized Studies (MINORS) tool. A narrative synthesis of included studies was conducted, a formal meta-analysis being unfeasible.ResultsOverall, 10 eligible studies including 233 MES patients were identified. The most frequent tumor sites were the limbs and trunk. Most patients underwent surgical treatment, with R0 resection margin rates ranging from 24% to 78%. During follow-up, local recurrence rates ranged from 17% to 50, and distant metastasis rates varied 8%–48%. At 5 years, OS rates ranged from 8% to 94% and DFS rates from 11% to 40%. The average MINORS score was 9.1 (range: 6–11).ConclusionsSurgical resection was often sub-optimal. The long-term prognosis was poor, with a non-negligible rate of disease recurrence and metastasis. On average, the quality of available information is moderate. The centralization of patient information in large international registries is warranted to provide a better understanding of MES biology and ultimately improve patient outcomes.     

Preoperative evaluation of tumor ploidy in endometrial carcinoma: An accurate tool to identify patients at risk for extrauterine disease and recurrence.

BACKGROUND: Tumor ploidy is a strong prognostic factor in patients with endometrial carcinoma, but generally is evaluated only after surgery. The availability of a simple and reliable method to determine tumor ploidy before any treatment is initiated could be helpful in the selection of patients at high risk for advanced primary disease and subsequent recurrence, with several possible benefits. The objectives of the current study were: 1) to test the accuracy of flow cytometric determination of tumor ploidy from preoperative outpatient endometrial biopsies compared with standard postoperative evaluation from the surgical specimen and 2) to correlate this preoperative parameter with the local recurrence and extrauterine tumor spread. METHODS: Tumor ploidy from both preoperative biopsy material and the macroscopic surgical specimens was evaluated prospectively in 50 consecutive patients with endometrial carcinoma. DNA analyses were performed in a blind fashion. Patients were followed for a median of 26 months (range, 16-46 months). RESULTS: In 9 of 50 cases (18%) an aneuploid tumor was found by the standard postoperative analysis. All 9 aneuploid tumors (100%) also were identified correctly by the preoperative test on biopsy material. Occult extrauterine tumor spread was found in 10 patients (20%). The incidence rate of aneuploidy among these tumors was 50% compared with 10% in surgical International Federation of Gynecology and Obstetrics Stage I tumors (P = 0.01). The recurrence rate was 55.5% (5 of 9 tumors) in the aneuploid group and 2.4% (1 of 41 tumors) in the diploid group (P < 0.001). The disease free survival rates of patients with diploid and aneuploid tumors were 97.5% and 44.4%, respectively (P < 0.0001). CONCLUSIONS: Preoperative tumor ploidy determination based on outpatient endometrial biopsy is as accurate as the standard postoperative evaluation in patients with endometrial carcinoma. Tumor aneuploidy confirms the usefulness of this method in selecting patients at risk for occult extrauterine tumor diffusion and recurrence.     

19例结直肠胃肠道间质瘤的外科治疗

目的:探讨结直肠的胃肠道间质瘤(gastrointestinal stromal tumors,GIST)外科治疗效果及其影响因素.方法:对我院1990年1月至2004年3月首次治疗的19例结直肠GIST临床资料和病理切片(含免疫组织化学检查)重新复核并加以随访,分析手术切除的效果以及影响手术疗效的因素.结果:手术者中位生存时间为60.0个月,术后1、3、5年生存率分别为100%、79.3%和69.4%.非扩大切除术者(即肿瘤局部切除和肿瘤及所在器官切除)与扩大切除术者比较,差异有显著性(P=0.001).完全切除术患者的生存率与肿瘤大小、病理类型、核分裂和复发转移有关;但多因素COX回归分析显示,术后生存率仅与肿瘤大小、核分裂和复发转移相关(P＜0.05).结论:结直肠GIST仍以外科治疗为主,原则上行局部完全切除即可.     

Impact of hospital procedure volume on surgical operation and long-term outcomes in high-risk curatively resected rectal cancer: findings from the Intergroup 0114 Study.

PURPOSE: Prior studies have demonstrated superior outcomes after a curative surgical resection of rectal cancer at hospitals where the volume of such surgeries is high. However, because these studies often lack detailed information on tumor and treatment characteristics as well as cancer recurrence, the true nature of this relation remains uncertain. PATIENTS AND METHODS: We studied a nested cohort of 1,330 patients with stage II and stage III rectal cancer participating in a multicenter, adjuvant chemoradiotherapy trial. We analyzed differences in rates of sphincter-preserving operations, overall survival, and cancer recurrence by hospital surgical volume. RESULTS: We observed a significant difference in the rates of abdominoperineal resections across tertiles of hospital procedure volume (46.3% for patients resected at low-volume, 41.3% at medium-volume, and 31.8% at high-volume hospitals; P <.0001), even after adjustment for tumor distance from the anal verge. However, this higher rate of sphincter-sparing operations at high-volume centers was not accompanied by any increase in recurrence rates. Hospital surgical volume did not predict overall, disease-free, recurrence-free, or local recurrence-free survival. However, among patients who did not complete the planned adjuvant chemoradiotherapy (270 patients), those who underwent surgery at low-volume hospitals had a significant increase in cancer recurrence (adjusted hazard ratio, 1.94; 95% CI, 1.01 to 3.72; P =.04 for the trend) and a nonsignificant trend toward increased overall mortality (P =.08) and local recurrence (P =.10). In contrast, no significant volume-outcome relation was noted among patients who did complete postoperative therapy. CONCLUSION: Using prospectively recorded data, we found that hospital surgical volume had no significant effect on rectal cancer recurrence or survival when patients completed standard adjuvant therapy. Sphincter-preserving surgery was more commonly performed at high-volume centers.     

Impact of surgical and pathologic variables in rectal cancer: a United States community and cooperative group report.

PURPOSE: Substantial and successful effort has been focused on decreasing the risk of local failure after rectal cancer surgery through the use of adjuvant therapies. Our study examined data from studies conducted by United States cooperative groups to investigate the impact of surgical and pathologic variables in rectal cancer outcomes. PATIENTS AND METHODS: Surgical and pathologic reports from 673 patients with stage II/III rectal cancer enrolled onto three adjuvant clinical trials were reviewed for tumor and surgical variables. Additional information on individual institutions and operating surgeon was collected. Variables were tested for association with 5-year local recurrence and survival after adjustment for adjuvant treatments and other important prognostic factors. RESULTS: Five-year local recurrence and survival rates were 16% and 59%, respectively. Surgeons treating more than 10 study cases had lower local recurrence rates than those treating < or = 10 (11% v 17%, P =.02). Free radial margins also correlated with local recurrence (P =.01). Type of surgery, distal margins, and tumor radial spread were not significant. Tumor adherence to adjacent structures predicted local recurrence (35% v 14%, P <.001) and survival (30% v 63%, P <.001), regardless of en bloc resection. Although T and N classification predicted survival (P <.001), only N classification correlated with local recurrence. The number and percentage of positive nodes correlated with survival, but only the percentage independently predicted local recurrence. Several pathologic and surgical variables were reported suboptimally. CONCLUSION: Moderate variability in outcomes among surgeons was detected in this high-risk population. Efforts to improve surgical results will require changes in reporting practices to allow for more accurate assessment of the quality of surgery.     

Soft tissue sarcomas of the buttock: A systematic review and meta-analysis

BackgroundSoft tissue sarcomas of the buttock (BSTS) are historically associated with a poor prognosis. The literature includes retrospective studies with small sample size. It is difficult to estimate the rate of local recurrence (LR), distant metastasis (DM) and overall survival (OS). A systematic review and meta-analysis was deployed to summarize the available information on BSTS patients that underwent surgical treatment.MethodsThe PubMed, EMBASE, Scopus and Google Scholar databases were reviewed for eligible studies following PRISMA guidelines. Inclusion criteria: (1) primary BSTS confirmed by pathological biopsy (2) indication for surgical treatment (3) reporting either the rate of LR, DM, or OS at 5-year (4) articles published up to December 2021 (5) english language. The Methodological Index for Non-Randomized Studies (MINORS) was applied for the quality appraisal.ResultsSix eligible studies with 216 BSTS patients were identified. Most patients underwent surgical resection, associated with radiation therapy. The most represented histological types were liposarcoma (n = 43, 19,9%), rhabdomyosarcoma (n = 27, 12.5%), and undifferentiated pleomorphic sarcoma (n = 23, 10.6%). High-grade tumor rates ranged from 37% to 88.2%, marginal resection rates from 20.3% to 50%, LR rates from 0% to 62.5%, DM rates from 37.5% to 62.5%. The pooled 5-year OS from 1941 to 2002 was 41% (IC95%: 33%–49%). The average MINORS score was 9.3 (range: 6 to 11).ConclusionsSurgical resection of BSTS had on average dissatisfactory outcomes, and the 5-year overall survival was poor, with high rates of local recurrence and distant metastasis. An effort to collect new data with a higher level of evidence is warranted.     

Primary malignant retroperitoneal tumors: analysis of a single institutional experience.

AIMS: In order to achieve complete resection in the surgical management of retroperitoneal tumors, it is crucial to know the tumor's anatomical location relative to neighboring organs. METHODS: Forty-nine patients with primary malignant retroperitoneal tumors were divided by tumor location into two groups [upper abdomen (group 1) or lower abdomen (group 2)], and clinicopathological features, tumor recurrence, and patient survival were assessed. RESULTS: No significant differences in preoperative clinical characteristics existed between two groups, and liposarcoma was the most frequently observed tumor type. The difference in the rates of complete resection between the two groups was not statistically significant (75.9% for group 1 and 85% for group 2). En-bloc combined resection was performed in 52% and 30% of patients in groups 1 and 2, respectively. The local recurrence rate in group 2 (31.3%) was higher than that in group 1 (9.5%), despite the fact that the differences in rates of complete resection and distant recurrence rates (14.3% in group 1 and 12.5% in group 2) between the two groups were not statistically significant. The overall 5-year survival rates were 67.9% for group 1 and 43.2% for group 2 (p=0.038). The 5-year survival rate of patients with tumors smaller than 10 cm was 78.4%, while that of patients with tumors larger than 10 cm was 38.1% (p=0.017). The 5-year survival rate after complete excision was 61%, whereas that after incomplete resection or biopsy only was 40.0% (p<0.0001). CONCLUSIONS: An upper abdominal tumor location is a positive prognostic factor even if small tumor size (<10 cm) and complete resection of the tumor are still more important factors to improve outcome in patients with malignant primary retroperitoneal tumors. Because complete resection was shown to be the most important prognostic factor, an aggressive and careful surgical approach is recommended for the treatment of such tumors.     

Parathyroid Carcinoma: Update and Guidelines for Management

Parathyroid carcinoma is one of the rarest known malignancies that may occur sporadically or as a part of a genetic syndrome. It accounts for approximately 1% of patients with primary hyperparathyroidism. The majority (90%) of parathyroid cancer tumors are hormonally functional and hypersecrete parathyroid hormone (PTH). Thus, most patients exhibit strong symptomatology of hypercalcemia at presentation. Sometimes, it can be difficult to diagnose parathyroid cancer preoperatively due to clinical features shared with benign causes of hyperparathyroidism. Imaging techniques such as neck ultrasound and 99mTc sestamibi scan can help localize disease, but they are not useful in the assessment of malignancy potential. Fine needle aspiration (FNA) prior to initial operation is not recommended due to technical difficulty in differentiating benign and malignant disease on cytology specimens and the possible associated risk of tumor seeding from the needle track. Complete surgical resection with microscopically negative margins is the recommended treatment and offers the best chance of cure. Persistent or recurrent disease occurs in more than 50% of patients with parathyroid carcinoma. Surgical resection is also the primary mode of therapy for recurrence since it can offer significant palliation for the metabolic derangement caused by hyperparathyroidism and allows hypercalcemia to become more medically manageable. However, reoperation is rarely curative and eventual relapse is likely. Chemotherapy and external beam radiation treatments have been generally ineffective in the treatment of parathyroid carcinoma. Typically, these patients require repeated operations that predispose them to accumulated surgical risks with each intervention. In inoperable cases, few palliative treatment options exist, although treatment with calcimimetics can effectively control hypercalcemia in some patients. Most patients ultimately succumb to complications of hypercalcemia rather than from tumor burden or infiltration.     

Albumin-bilirubin (ALBI) grade-based nomogram to predict tumor recurrence in patients with hepatocellular carcinoma

BackgroundTumor recurrence after curative resection is common in hepatocellular carcinoma (HCC), but large-scale long-term prediction on an individual basis has seldom been reported. We aimed to construct an albumin-bilirubin (ALBI) grade-based nomogram to predict tumor recurrence in patients with HCC undergoing surgical resection.MethodsA total 1038 patients with newly diagnosed HCC undergoing curative resection between 2002 and 2016 were enrolled. Baseline characteristics, tumor status and severity of liver functional reserve were collected. The Cox proportional hazards model was used to predict tumor recurrence and construct the nomogram. The performance of the nomogram was evaluated by the discrimination and calibration tests.ResultsAfter a mean follow up time of 30 months, 510 (49%) patients developed tumor recurrence. The cumulative recurrence-free survival at 1, 3, 5, and 10 years were 79%, 51%, 38% and 26%, respectively. In the Cox multivariate model, ALBI grade 2–3, multiple tumors, tumor size equal or large than 2 cm, serum ɑ-fetoprotein level equal or greater than 20 ng/ml and total tumor volume equal or larger than 227 cm³ were independent risk factors associated with tumor recurrence. A nomogram was constructed based on these five variables. Internal validation with 10,380 bootstrapped sample sets had a good concordance of 0.607 (95% of confidence interval: 0.587–0.627). The calibration plots for 1-, 3- and 5-year recurrence-free survival well matched the idealized 45-degree line.ConclusionsALBI is a feasible marker for tumor recurrence. This easy-to-use ALBI grade-based nomogram may predict tumor recurrence for individual HCC patient undergoing surgical resection.     

Breast-conserving surgery following radiation therapy of 50 Gy in stages I and II carcinoma of the breast: the experience at one institute in Japan

This study evaluated the results of breast-conserving therapy experienced in one institute. A total of 217 breasts in 215 patients with clinical stages I and II breast cancer undergoing breast-conserving therapy between August 1991 and December 1999 were included. The actuarial 5-year local recurrence-free survival, disease-free survival and overall survival rates were 97.4%, 88.5% and 92.5%, respectively. Microscopic margins were negative in 176 (81.1%) of the total breasts and positive in 41 (18.9%). There were no differences in the age at operation, tumor size, clinical stage, lymph node status, estrogen receptor status, or distance from tumor to nipple between patients with positive surgical margins and with negative surgical margins. During the follow-up period, local recurrence occurred in seven patients. Local recurrence was significantly less frequent in patients with surgical margin-negative tumors than those with surgical margin-positive tumors, and also in ER-positive tumors than ER-negative or ER-unknown tumors. There was no difference in overall survival between patients with and without breast-recurrence. Using multivariate analysis, the surgical margin was determined to be an independent predictive factor for local recurrence in the conserved breast. Nodal status and ER status were independent prognostic factors, but local recurrence did not have an independent negative impact on survival. We conclude that microscopic surgical margin is the most important factor associated with local recurrence in the conserved breast among patients treated with breast-conserving surgery and radiation therapy of 50 Gy. Local recurrence however, has no adverse effect on survival of patients.     

Tumoren der Nebenschilddrüse

Context

Benign and rarely malignant tumors of the parathyroid glands cause primary hyperparathyroidism (pHPT) which is the third most prevalent endocrine disease after type 2 diabetes mellitus and hyperthyroidism.

Materials and methods

A systematic literature search was performed in Pubmed, MEDLINE, current guidelines and by manual searching. Relevant publications from the past 5 years were analyzed and results were summarized in a structured review.

Results

In 80–90?% of cases pHPT results from a single parathyroid adenoma, in 10–20?% of cases multiple adenomas or diffuse hyperplasia of all parathyroid glands are found, in particular in familial tumor syndromes. Parathyroid carcinomas are found in less than 1?% of cases. Symptoms of pHPT are characterized by direct parathyroid hormone (PTH) effects in target organs and later by hypercalcemia-associated organ complications. Diagnostic procedures include laboratory tests (e.g. calcium, phosphate and PTH) and imaging procedures, e.g. ultrasound and methoxyisobutylisonitrile (sestamibi) scintigraphy for localization of the tumor. The goal of therapeutic interventions in pHPT and parathyroid tumors is normalization of the serum calcium concentrations with alleviation of any pHPT-associated symptoms and to cure the tumor. Symptomatic patients and carcinomas should be surgically treated by parathyroidectomy in specialized centres. In 95?% of patients with adenomas these can be cured by a primary surgical intervention. In parathyroid carcinoma patients survival is approximately 80?% after 5 years and 70?% after 10 years. In asymptomatic patients with pHPT a non-surgical approach may be justified. When the perioperative risk is high or in nonoperable carcinoma patients, treatment with calcimimetic drugs (cinacalcet) represents a highly effective drug therapy for normalization of serum calcium levels.

Conclusions

Tumors of the parathyroid glands are largely benign adenomas and rarely carcinomas which can be cured in most cases by primary surgical intervention. This intervention also cures the concomitantly occurring pHPT. For non-surgical treatment strategies, calcimimetic agents are available to lower serum calcium concentrations.     

Prognostic factors for long term results of the treatment of patients with malignant submandibular gland tumors.

BACKGROUND: Concerning malignant submandibular gland tumors, a rare disease entity, there are few recent reports of long term treatment results and of prognostic factors modifying these results. METHODS: The clinical data of 43 patients with malignant submandibular gland tumors who were treated at the Netherlands Cancer Institute between 1973 and 1994 were reviewed to evaluate treatment results and to control described prognostic factors. The median follow-up for patients alive at the end of follow-up was 143 months. Only univariate analyses were performed. RESULTS: The crude 5- and 10-year survival rates were 50% and 36%, respectively; the 5- and 10-year disease specific survival (DSS) rates were 61% and 51%, respectively; and the 5- and 10-year recurrence free percentages were 57% and 52%, respectively. Factors predicting crude survival were age at diagnosis (P = 0.0006), International Union Against Cancer/American Joint Committee on Cancer (UICC/ AJCC) TNM classification and its regrouping into tumor stage (P = 0.001), and clinical skin invasion (P = 0.005). In surgically treated patients, soft tissue invasion (P = 0.005), metastatic lymph nodes (P = 0.006), and perineural growth (P = 0.01) were prognostic for survival. Factors predicting DSS were the UICC/AJCC TNM classification and regrouping into tumor stage (P = 0.002). In surgical patients, perineural growth (P = 0.0008) conferred a lower DSS. Factors predicting tumor recurrence were the UICC/AJCC TNM classification and its regrouping into tumor stage (P = 0.009). In surgical patients, perineural growth (P = 0.003) predicts tumor recurrence. CONCLUSIONS: Of patients with submandibular gland carcinoma treated according to a stable treatment protocol in a European tertiary referral center, 52% were tumor free 10 years later. Patients exhibiting the described adverse prognostic factors are likely to benefit from added radiotherapy.     

Evaluation of short-course radiotherapy followed by neoadjuvant bevacizumab,capecitabine, and oxaliplatin and subsequent radical surgical treatment in primary stage IV rectal cancer

BackgroundTo evaluate the efficacy and tolerability of preoperative short-course radiotherapy followed by capecitabine and oxaliplatin treatment in combination with bevacizumab and subsequent radical surgical treatment of all tumor sites in patients with stage IV rectal cancer.Patients and methodsAdults with primary metastasized rectal cancer were enrolled. They received radiotherapy (5 × 5 Gy) followed by bevacizumab (7.5 mg/kg, day 1) and oxaliplatin (130 mg/m², day 1) intravenously and capecitabine (1000 mg/m² twice daily orally, days 1–14) for up to six cycles. Surgery was carried out 6–8 weeks after the last bevacizumab dose. The percentage of radical surgical treatment, 2-year survival and recurrence rates, and treatment-related toxicity was evaluated.ResultsOf 50 included patients, 42 (84%) had liver metastases, 5 (10%) lung metastases, and 3 (6%) both liver and lung metastases. Radical surgical treatment was possible in 36 (72%) patients. The 2-year overall survival rate was 80% [95% confidence interval (CI) 66.3%–90.0%]. The 2-year recurrence rate was 64% (95% CI 49.8%–84.5%). Toxic effects were tolerable. No treatment-related deaths occurred.ConclusionsRadical surgical treatment of all tumor sites carried out after short-course radiotherapy, and bevacizumab–capecitabine–oxaliplatin combination therapy is a feasible and potentially curative approach in primary metastasized rectal cancer.     

467例Ⅱ期胸段食管鳞癌切除术后患者的生存分析

背景与目的:食管癌是我国常见恶性肿瘤之一,多数患者就诊时已是局部晚期。迄今为止,手术切除仍是其最主要的治疗方法。本研究旨在探讨影响Ⅱ期胸段食管鳞癌术后生存的因素。方法:回顾性分析中山大学肿瘤防治中心1990年1月至1998年12月收治的467例Ⅱ期胸段食管癌患者的临床资料,应用Kaplan-Meier法进行生存分析,组间比较用log-rank检验,多因素分析采用Cox模型。结果:全组总的1、3、5和10年生存率分别为87.1%、54.6%、43.0%和32.3%,ⅡA期和ⅡB期患者的5年生存率分别为51.0%和19.9%。81例(17.3%)术后复发,其中有70例(86.4%)在术后3年内复发。单因素分析表明患者的性别、肿瘤浸润深度、淋巴结转移、病理分期、淋巴结转移区域数、组织分化、切缘阳性和肿瘤复发均为预后影响因素。Cox回归分析显示性别、肿瘤浸润深度、病理分期、淋巴结转移区域数、切缘阳性和肿瘤复发是食管癌预后的独立影响因素。结论:影响Ⅱ期胸段食管癌患者术后生存的独立因素有性别、肿瘤浸润深度、病理分期、淋巴结转移区域数、切缘阳性和肿瘤复发。外科手术仍是ⅡA期患者的主要治疗方法,但对于ⅡB期患者应采取以手术为主的综合治疗。     

硬腭小涎腺癌的疗效评价和预后因素分析

背景与目的:硬腭小涎腺癌由于发病率低、长期生存率较高,使评价其治疗效果及预后因素较为困难。本研究目的是分析硬腭小涎腺癌的治疗效果及影响预后的因素。方法:对1964年～2001年在中山大学肿瘤防治中心住院治疗的96例原发于硬腭的小涎腺癌进行回顾性分析。应用Kaplan-Meier法计算累积生存率,各因素间比较用秩和对数检验(log-rank),多因素分析采用Cox模型。结果:本组96例患者总的5年、10年生存率分别为65.60%和47.90%。单纯手术治疗53例和手术加放疗35例的5年生存率分别为73.14%和66.58%,10年生存率分别为67.40%和46.60%。多因素分析结果表明:年龄≥50岁、肿瘤≥3cm、切缘阳性及肿瘤复发者预后差(P<0.05)。结论:手术治疗或以手术为主的综合治疗是硬腭小涎腺癌的首选治疗方法。年龄、肿瘤大小、切缘情况及复发情况为影响预后的独立因素。     

Trends in the incidence and treatment of parathyroid cancer in the United States

BACKGROUND: Parathyroid cancer is a rare cause of hyperparathyroidism. The objectives of this study were to determine the patterns of disease, treatment trends, and outcomes among patients with parathyroid cancer by using a population-based data source. METHODS: Surveillance, Epidemiology, and End Results (SEER) cancer registry data were used to identify patients who were diagnosed with parathyroid cancer from 1988 through 2003. To assess whether the incidence rate, treatment, tumor size, and cancer stage changed over time, the Cochrane-Armitage trend test was used, and Cox proportional-hazards modeling was used to identify the factors associated with an improved overall survival rate. RESULTS: From 1988 through 2003, 224 patients with parathyroid cancer were reported in the SEER data. Over that 16-year study period, the incidence of parathyroid cancer increased by 60% (1988-1991, 3.58 per 10,000,000 population; 2000-2003, 5.73 per 10,000,000 population). Most patients (96%) underwent surgery (parathyroidectomy, 78.6% of patients; en bloc resection, 12.5% of patients; other, 4.9% of patients). The rate of surgical treatment increased significantly during the study period. The 10-year all-cause mortality rate was 33.2%, and the 10-year cancer-related mortality rate was 12.4%. Patient age (P<.0001), sex (P=.0106), the presence of distant metastases at diagnosis (P=.0004), and the year of diagnosis (P=.0287) were associated significantly with the overall survival rate. Tumor size, lymph node status, and type of surgery were not associated significantly with the overall survival rate. CONCLUSIONS: Although parathyroid cancer is rare, the incidence increased significantly in the United States from 1988 through 2003. Young age, female gender, recent year of diagnosis, and absence of distant metastases were associated significantly with an improved survival rate.     

胃肠间质瘤大小与预后

 目的 探讨胃肠间质瘤（GIST）原发肿瘤大小与预后的关系。方法 对该院1990年1月至2006年3月132例能完全切除的GIST患者的临床资料和病理切片（含免疫组织化学检查）重新复核并进行随访，分析原发肿瘤大小与预后的关系。结果 患者中位生存时间为66.0个月，术后2年和5年生存率分别为89.4 ％和70.9 ％。原发灶肿瘤完全切除术患者的生存率与其性别、肿瘤部位和大小、肿瘤性质、核分裂及复发转移有关；但多因素的Cox回归分析显示，术后生存率仅与肿瘤大小、肿瘤性质和复发转移相关（P＜0.05）。以2 cm为界比较生存率有统计学意义（P＜0.05），以5 cm为界比较生存率无统计学意义（P＞0.05）。结论 GIST仍以外科治疗为主，GIST原发灶大小是影响预后的独立高危因素，直径≥2 cm的患者应加强随诊。     

Malignant fibrous histiocytoma: a retrospective study of 109 cases

The purpose of this report is to assess the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues. Between 1975 and 1998, 109 patients diagnosed with MFH of the soft tissues, seen at King Faisal Specialist Hospital and Research Center, have been reviewed. Of the 109 patients, 75 were men and 34 were women. The median age at presentation was 48 years (range: 3-94). Seven patients (6%) had regional nodal disease and 10 other patients (9%) with distant metastases were excluded from survival analysis. The remaining 92 patients had localized disease and had surgery as the primary treatment modality with or without radiotherapy and/or chemotherapy. Extremities were the most common location (58%). Tumors less than 5 cm represented 32%, whereas 68% had tumors 5 cm or more. Low-grade tumors constituted 46%, and the remaining 54% were high grade. Thirty-seven percent of patients had positive surgical margins histologically after complete gross resection. The 5- and 10-year relapse-free survival (RFS) rates were 39% and 36%, respectively. Isolated local recurrence occurred in 20 patients (22%), isolated metastatic disease without local recurrence in 9 patients (10%), and combined local and metastatic disease occurred in 20 patients (22%). The overall 5- and 10-year overall survival (OS) rates were 50% and 43%, respectively. On multivariate analysis, tumor size and radiation dose were significant factors for RFS (p = 0.04 and 0.0005, respectively). In terms of OS, size, histologic grade, and surgical margins were significant factors on multivariate analysis (p = 0.001. 0.006, and 0.0001, respectively). Complete surgical resection at the time of primary tumor presentation is likely to afford the best chance for RFS and OS. Radiation therapy plays an important role, in combination with surgery for better local control, particularly in high-grade lesions, and in cases with positive surgical margins after wide complete gross excision. The role of adjuvant chemotherapy remains investigational.     

Defining the Optimal Treatment Strategy in Patients With Uterine Serous Carcinoma

AimsUterine serous carcinoma (USC) is an aggressive subtype of endometrial cancer with high rates of relapse and death. As adjuvant therapy might be beneficial in early-stage disease, the impact of standard complete surgical staging is questioned. Therefore, we wanted to explore the optimal treatment strategy for women diagnosed with USC.Materials and methodsA retrospective multicentre study of women diagnosed with primary USC in the UK and the Netherlands. Treatment strategy in relation to overall survival and progression-free survival was recorded and evaluated with Kaplan–Meier and Cox regression analysis. Furthermore, primary surgical staging and/or adjuvant treatment in relation to patterns of recurrence were evaluated.ResultsIn total, 272 women with a median age of 70 years were included. Most patients presented with International Federation of Gynecology and Obstetrics (FIGO) stage I disease (44%). Overall, 48% of patients developed recurrent disease, most (58%) with a distant component. Women treated with chemotherapy showed significantly better overall survival (hazard ratio 0.50, 95% confidence interval 0.31–0.81; P = 0.005) and progression-free survival (hazard ratio 0.48, 95% confidence interval 0.28–0.80; P = 0.04) in multivariable analysis. Furthermore, even in surgically staged women with FIGO stage IA disease, a high recurrence rate of 42% was seen.ConclusionWomen with USC who received adjuvant chemotherapy showed better survival rates compared with those who received other or no adjuvant treatment. The benefit of adjuvant chemotherapy was observed across all tumour stages, including surgically staged FIGO stage IA. These data question the role of surgical staging in the absence of macroscopic disease in USC.