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1.
Clear cell sarcoma of the kidney (CCSK) is uncommon pediatric renal tumor and can present a significant therapeutic challenge in those patients whose tumors spread beyond the kidney. Thus, identifying potential novel targets for treatment may be clinically important. Clear cell sarcoma of the kidney is characterized by a unique vascular pattern, in which nests of tumor cells are separated by regularly-spaced, fine fibrovascular septa. This distinctive histopathology raises the possibility that understanding the factors which drive angiogenesis in CCSK tumors may suggest new therapeutic targets. Here, we describe a case of CCSK and present immunohistochemical studies of its vasculature.  相似文献   

2.
IntroductionIntimal sarcoma is an extremely rare group of undifferentiated pleomorphic sarcoma arising from the intimal layer of vessels accounting for only 1% of all sarcomas, intimal sarcoma of large veins are even less common.Cases presentationWe present two cases of intima sarcoma, one originated form the basilar vein and the other from the cephalic vein, the first one was treated with surgery and postoperative chemotherapy followed by Radiotherapy (RT), the second case was treated with isolated limb perfusion followed by marginal resection and RT. Both patients progressed to the lungs in a short time, the first case was treated with metastasectomy of the lung and is without evidence of disease 7 months after surgery; the second case treated with isolated limb perfusion has stable disease.DiscussionIntimal sarcoma are very aggressive tumors, with a high metastatic potential, the two patients progressed to lung in a short time (2 months) after local treatment. Both cases exhibit good response to chemotherapy and metastasectomy with a disease – free period of 7 months.ConclusionWe propose that given the aggressive behavior of these tumors, they should be treated with chemoradiotherapy postoperative, either by systemic chemotherapy or isolated limb perfusion for the limp sparing surgery in this histology.  相似文献   

3.
We have performed pulmonary metastasectomy on 97 patients with various malignant tumors. We analyzed the outcome of surgical treatment and prognostic factors. Survival of patients undergoing metastasectomy was significantly different according to the type of malignant tumor. The patients with carcinomas had a good survival when they had 3 years or longer disease free interval (DFI) and no other lesion except the lungs. Those with sarcomas were good survivors, who had a solitary pulmonary lesion, 1 years or longer DFI, and had no symptom originating from pulmonary metastases. Survival of patients with germ cell tumors was significantly superior to other 2 types, and factors such as an existence of extra-pulmonary metastatic lesions, respiratory symptoms and a length of DFI had no impact on the chance of their survival. These results suggest that we should decide the indication for metastasectomy on patients according to their types of malignant tumor.  相似文献   

4.
Soft tissue sarcomas in children are rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or a non-RMS soft tissue sarcoma (NRSTS). RMS is more common in children 14 years old and younger and NRSTS in adolescents and young adults. Infants get NRSTS, but their tumors constitute a distinctive set of histologies. Surgery is a major therapeutic modality and radiation plays a role. RMS is treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the NRSTS that are high grade or unresectable. This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas.  相似文献   

5.

Introduction

Due to the scrotum’s multiple layers of different tissues, scrotal cancer can present with several unique histologies. Historically, outcome arising from these different sources has been historically aggregated together. However, it remains unclear whether survival differs by histology of scrotal cancer.

Methods

We queried the seventeen registries of the Surveillance, Epidemiology, and End Results database for patients diagnosed with primary scrotal cancer from 1973 to 2006. Patients were initially grouped by the following histologies: basal cell carcinoma, Extramammary Paget’s Disease (EMPD), sarcoma, melanoma, squamous cell carcinoma, and adnexal skin tumors. For some analyses, the former three histologies were reclassified as Low-Risk scrotal cancer and the latter three histologies as High-Risk scrotal cancer. Kaplan–Meier survival analyses were conducted to assess the impact of histology on overall survival (OS).

Results

The cohort consisted of 766 patients. Median (95% CI) OSs by histologies were basal cell carcinoma—143 (116–180), EMPD—165 (139–190), sarcoma—180 (141–219), melanoma—136 (70–203), squamous cell carcinoma—115 (97–133), and adnexal skin tumors—114 (55–174). Patients with Low-Risk scrotal cancer experienced a median (95% CI) OS of 166 (145–188) months, while patients with High-Risk scrotal cancer experienced a median (95% CI) OS of 118 (101–135) months.

Conclusions

Survival of scrotal cancer depends on tumor histology. Classification of histologies into Low and High Risk can be clinically useful for counseling and clinical decisions.  相似文献   

6.
Conventional external beam radiation has proved its profit in pediatric tumors; but its complications have limited it in therapeutical approach. Intraoperative radiotherapy delivers a high single dose in residual tumor or high risk areas during surgery. In our center, during last two years, 7 patients have been candidates to surgery with intraoperative radiotherapy (the age range was between 5 months-17 years; mean 8.5 years). Two patients were excluded of our protocol because of their intraoperative stage. Patients tumors types were: neuroblastoma (n = 3; stage III and IV), soft tissue sarcomas (n = 1) and Ewing's sarcoma (n = 1). The radiation doses ranged from 500 cGyto-1200 cGy. Local control tumor was achieved in 4 patients and no-complications were present secondary to surgery or intraoperative radiotherapy. Intraoperative radiotherapy seems to be a feasible treatment which might promote local control in pediatric tumors with protection of normal tissues and could be an excellent complement in special cases.  相似文献   

7.
BackgroundThe role of local surgery in patients with metastatic soft tissue sarcoma (STS) remains unknown. The study aims to assess the clinical outcomes and impact of surgical resection on survival in patients with metastatic STS and elucidate the survival differences between synchronous and metachronous metastatic groups.MethodsAmong the 272 patients with STS treated between 2000 and 2018, 84 with synchronous or metachronous metastasis were included. Associations between overall survival and primary tumor resection and metastasectomy were assessed using multivariate Cox regression analyses to adjust for baseline differences between surgically and non-surgically treated patients. Propensity score matching was applied to compare synchronous and metachronous metastasis.ResultsAmong the 84 patients included, 69 (82%) and 41 (49%) underwent primary tumor resection and metastasectomy, respectively. The 2- and 5-year overall survivals of all patients after first detection of metastasis were 51.1% and 24.4%, respectively. Multivariate analysis showed that size <8 cm, grade <3, and number of metastases <4 were associated with longer overall survival. After adjusting for baseline demographic and tumor characteristics, primary tumor resection and metastasectomy still had favorable effects on survival. Tumor subtypes, grade, and number of metastases differed significantly between synchronous and metachronous groups. However, after adjusting for these valuables, both groups exhibited comparable survival.ConclusionsApproximately one fourth of the patients with metastatic STS survived for >5 years. Our results showed that surgical resection of primary tumors or metastatic lesions had favorable impact on survival even after adjusting for patient backgrounds, with comparable survival observed between those with synchronous and metachronous metastases.  相似文献   

8.

Purpose

In pediatric endoscopic surgery, a limited view and lack of tactile sensation restrict the surgeon's abilities. Moreover, in pediatric oncology, it is sometimes difficult to detect and resect tumors due to the adhesion and degeneration of tumors treated with multimodality therapies. We developed an augmented reality (AR) navigation system based on preoperative CT and MRI imaging for use in endoscopic surgery for pediatric tumors.

Methods

The patients preoperatively underwent either CT or MRI with body surface markers. We used an optical tracking system to register the reconstructed 3D images obtained from the CT and MRI data and body surface markers during surgery. AR visualization was superimposed with the 3D images projected onto captured live images. Six patients underwent surgery using this system.

Results

The median age of the patients was 3.5 years. Two of the six patients underwent laparoscopic surgery, two patients underwent thoracoscopic surgery, and two patients underwent laparotomy using this system. The indications for surgery were local recurrence of a Wilms tumor in one case, metastasis of rhabdomyosarcoma in one case, undifferentiated sarcoma in one case, bronchogenic cysts in two cases, and hepatoblastoma in one case. The average tumor size was 22.0 ± 14.2 mm. Four patients were treated with chemotherapy, three patients were treated with radiotherapy before surgery, and four patients underwent reoperation. All six tumors were detected using the AR navigation system and successfully resected without any complications.

Conclusions

The AR navigation system is very useful for detecting the tumor location during pediatric surgery, especially for endoscopic surgery.  相似文献   

9.
The use of VATS to perform diagnostic metastasectomy is uniformly accepted, but the role of therapeutic VATS metastasectomy remains controversial. We retrospectively compared the value of therapeutic metastasectomy by VATS with that by open thoracotomy in the management of pulmonary metastatic tumors. The forty-six patients who underwent a complete metastasectomy of pulmonary metastatic tumors were divided into groups: one group in which metastasectomy was performed by VATS (VATS group; 23 cases, 25 operations) and another group in which metastasectomy was performed by open thoracotomy (Thoracotomy group 23 cases, 26 operations). Here, complete metastasectomy means compete resection of all tumors, which were detected by preoperative high resolution computed tomography in both groups and also by intraoperative manipulation in the thoracotomy group, without positive lesions of the surgical margin. The groups were matched with respect to preoperative clinical characteristics (age, sex, primary lesion, disease-free interval) and histological findings (maximum tumor size, number of metastatic tumors, minimum distance between surgical margin and tumor edge). The cumulative survival one-year and three-year rates were 83.5 and 83.5% in the VATS group, and 89.5 and 77.5% in the thoracotomy group, respectively. The cumulative one-year and three-year non-recurrence rates were 72.3 and 68.0% in the VATS group and 83.9 and 63.8% in the thoracotomy group, respectively. There were no significant differences between the two groups. We conclude that pulmonary metastasectomy by VATS can be used safely not only as a diagnostic but also therapeutic tool if complete resection is done following diagnosis by preoperative high resolution computed tomography.  相似文献   

10.
The International Registry of Lung Metastases was established in 1991 to asses the long-term results of pulmonary metastasectomy. The Registry has accrued 5206 cases of lung metastasectomy, from 18 departments of thoracic surgery in Europe (n = 13), USA (n = 4) and Canada (n = 1). Of these patients 4572 (88%) underwent complete surgical resection. The primary tumor was epithelial in 2260 (43%), sarcoma in 2173 (42%), germ cell in 363 (7%), and melanoma in 328 (6%) patients. The disease-free interval was 0 to 11 months in 1729 (33%) cases, 12 to 35 months in 1857 (36%) and more than 36 months in 1620 (31%). Single metastases accounted for 2383 (46%) cases and multiple lesions for 2726 (52%). Mean follow up was 46 months. Analysis was performed by Kaplan-Meier estimates of survival, relative risk of death and multivariate Cox model. The actuarial survival after complete metastasectomy was 36% at 5 years, 26% at 10 years and 22% at 15 years (median 35 months); the corresponding values for incomplete resection were 13% at 5 years and 7% at 10 years (median 15 months). Among complete resections, the 5-year survival was 33% for patients with a disease free-interval of 0 to 11 months and 45% for those with a disease-free interval of more than 36 months; 43% for single lesions and 27 for four or more lesions. Multivariate analysis showed a better prognosis for patients with germ cell tumors, disease-free interval of 36 months and more and single metastases. These results confirm that lung metastasectomy is a safe and potentially curative procedure.  相似文献   

11.
Craniofacial resection for anterior skull base tumors   总被引:6,自引:0,他引:6  
We present the results of treatment of 30 patients with anterior skull base tumors operated on over an 11-year period. At the time of surgery, intracranial invasion was present in 10 patients. Histology revealed epithelial tumors in 18 patients, sarcoma in 6, esthesioneuroblastoma in 4, and 2 miscellaneous histologies. The overall median survival was 5 years and varied according to histology and grade of tumor. Currently malignancies involving the skull base can be successfully resected using a craniofacial approach, with minimum operative mortality. Limited intracranial invasion need not necessarily represent a major contraindication of this procedure if morbidity can be kept to a minimum.  相似文献   

12.
The Authors present 9 cases of gastro-enteropancreatic neuro-endocrine biologically inactive tumors. In 5 cases the tumor site was appendicular. In 4 patients an appendectomy was performed, in one patient a right hemicolectomy and the patients after a period of 3-9 years are well and disease free. In a patient with a gastric carcinoid and a single hepatic metastasis a total gastrectomy with an hepatic metastasectomy were performed but the patient died 16 months thereafter. In a case localized to the right colon with a single hepatic metastasis a right hemicolectomy was performed with a metastasectomy but the patient died after 12 months. In a case localized to an ileal loop a segmental resection was performed followed by a medical therapy with octreotide and the patient is well and disease free after 3 years. In a case localized to the pancreas with widespread lymphatic metastasis it was performed a simple biliary diversion (coledocho-duodenostomy) followed by medical therapy with octreotide. Surprisingly after 4 years the patient is alive and a TC control shows a decrease of the pancreatic tumor and of the lympho glandular tumefactions.  相似文献   

13.
Prognosis of Lymph Node Metastasis in Soft Tissue Sarcoma   总被引:6,自引:0,他引:6  
Background: We defined the tumor characteristics and prognosis of patients with regional lymph node metastasis (RLNM) from soft tissue sarcoma.Methods: All patients with RLNM from soft tissue sarcoma were identified from the Royal Marsden Hospital Sarcoma Unit prospective database from January 1990.Results: A total of 73 (3.4%) of 2127 patients had RLNM. Fifty-seven patients (78.1%) had RLNM as the first site of spread, and 16 patients (21.9%) presented with RLNM and distant metastasis synchronously. The most common histologies were rhabdomyosarcoma, epithelioid sarcoma, and angiosarcoma. There were 9 T1 and 36 T2 tumors, and tumor size was not available in 28 patients. There were 6 grade 1, 16 grade 2, and 51 grade 3 tumors. Forty-two patients presented with RLNM along with the primary tumor. Of the remaining patients, the median time to development of RLNM from diagnosis of the primary tumor was 13.5 months (95% confidence interval [CI], 1–100 months). The 1-year survival for patients with isolated RLNM was 77.49% (95% CI, 62.99%–86.88%), compared with 36.27% (95% CI, 13.32%–60.04%) for patients who presented with RLNM and distant metastasis (P = .005). The 1-year survival for metachronous and synchronous RLNM was 94.44% (95% CI, 66.64%–99.20%) and 67.54% (95% CI, 47.89%–81.12%), respectively (P = .05).Conclusions: Lymph node metastasis is rare. Patients who present with isolated RLNM have an improved survival compared with patients who present with regional and distant metastasis at diagnosis. Synchronous RLNM with the primary tumor have a poorer outcome than metachronous RLNM in the absence of distant metastasis.  相似文献   

14.
The clinicopathologic features of 17 fibrous tumors of the pleura are presented. Eight were benign localized fibrous tumors; all of these were negative when stained with antibodies to keratin. Eight were diffuse malignant tumors that demonstrated intense immunohistochemical staining of the spindle cells with antibodies to keratin. One case presented as a histologically malignant spindle cell tumor that was initially localized but recurred subpleurally three times in 12 years. This spindle cell tumor was consistently negative when stained for keratin. We conclude that there are three types of fibrous tumors of the pleura: (a) a localized, histologically benign, keratin-negative spindle cell tumor that might be termed "fibroma," (b) a diffuse, histologically malignant, keratin-positive neoplasm that might appropriately be termed a "sarcomatoid mesothelioma," and (c) a histologically malignant, keratin-negative, spindle cell tumor with the potential to spread under the pleura that might appropriately be termed a "sarcoma." Immunohistochemistry proved to be useful in distinguishing the sarcomatoid mesothelioma from the sarcoma.  相似文献   

15.
OBJECTIVE: To determine incidence trends and outcomes for pediatric patients with malignant breast disease. METHODS: The Surveillance, Epidemiology, and End Results registry was examined for all females 19 years of age and younger diagnosed with a malignant breast tumor between 1973 and 2004. RESULTS: A total of 75 patients with malignant breast tumors were identified. Overall, 14.5% of patients had in situ tumors, and 85.5% had invasive disease. Tumors were classified as being either carcinomas (n = 41, 54.7%) or sarcomas (n = 34, 45.3%). The majority of sarcomatous lesions were phyllodes tumors (n = 29, 85.5%), whereas most carcinomas were of a ductal etiology (n = 19, 46.3%). The age-adjusted incidence of all malignant pediatric breast tumors in 2003 was 0.08 cases per 100,000 people (0.03 carcinoma and 0.06 sarcoma cases per 100,000 people). In the carcinoma group, regionally advanced disease was present in 11 patients (26.8%), whereas only 3 patients (7.3%) presented with metastatic disease. All patients with sarcomatous tumors presented with localized disease. Adjuvant radiation therapy was administered in only 9.8% of carcinomas and 8.8% of sarcomas, and 85.4% of carcinoma patients and 97.1% of sarcoma patients underwent surgical resection for their primary disease. Subgroup analysis revealed 5- and 10-year survival rates of 89.6% for patients with sarcomatous tumors and 63.1% and 54.3% for carcinomas. CONCLUSIONS: Malignant pediatric breast malignancies remain relatively rare. The two most common histologies of breast neoplasms in children are malignant carcinomas followed by sarcomas. Although uncommon, malignant disease must be considered in the differential diagnosis of the pediatric patient with a breast mass.  相似文献   

16.
In a 34-year-old woman extensive intrapleural disease developed within 1 year of video-assisted thoracoscopic surgery (VATS) removal of two pulmonary metastases from a previously treated synovial sarcoma. She underwent a successful complete pleurectomy through a left thoracotomy to excise the pleural tumor and remains well 6 months later. This case highlights tumor dissemination as one of the pitfalls of VATS metastasectomy and raises concerns about using VATS in this situation.  相似文献   

17.
郝林  王涛  刘巍峰  金韬  牛晓辉 《中华骨科杂志》2012,32(11):1015-1019
 目的 探讨应用前方带股血管蒂的股四头肌肌皮瓣覆盖臀后部创面的半骨盆截肢术(前侧皮瓣半骨盆截肢术)的手术适应证、手术方法、患者预后和并发症情况。方法 自2009年4月至2010年10月,北京积水潭医院骨肿瘤科对10例患者采用前侧皮瓣半骨盆截肢术,男9例,女1例;年龄30~62岁,平均45岁。软骨肉瘤7例、上皮样肉瘤1例、多形性脂肪肉瘤1例、梭形细胞肉瘤1例。结果 9例患者获得随访,随访时间12~30个月,平均21.5个月。术后仅1例出现轻度皮缘坏死,无伤口感染者。局部复发3例(33.3%),2例软骨肉瘤、1例上皮样肉瘤。死亡3例(33.3%),包括复发的2例、另1例软组织脂肪肉瘤患者因肺转移死亡;1例复发患者带瘤存活;2例患者肺转移带瘤存活;余3例未见复发和转移。结论 前侧皮瓣半骨盆截肢术适用于:(1)臀后部软组织肿块侵及皮肤皮下或反应区到达皮下;(2)骨盆恶性肿瘤复发需行半骨盆截肢,原手术切口污染臀部软组织,无法使用常规后侧皮瓣;(3)臀部皮肤软组织因放疗等原因影响后侧皮瓣血运。此手术的必要条件是髂外血管到股血管通畅良好,可顺利游离且不在肿瘤反应区内。适应证明确的情况下,前侧皮瓣覆盖较常规后侧半骨盆截肢更便于操作和覆盖,便于获得更好的外科边界,术后并发症少。  相似文献   

18.
OBJECTIVES Pulmonary metastasectomy is firmly established in the multidisciplinary management of patients with advanced sarcomas. While the number of metastases, completeness of resection, disease-free interval and grading of the primary sarcoma are well established prognostic factors in metastatic surgery, histological parameters are not widely evaluated. The objective of the present study was to evaluate the prognostic impact of intrapulmonary growth patterns of sarcoma metastases. METHODS We retrospectively analysed the clinicopathological characteristics of 52 sarcoma patients who underwent surgical resection of lung metastases at our centre from January 2006 to January 2009. The histological growth characteristics of all 261 metastases have been categorized and published previously. 'Interstitial growth' was defined as a diffuse spread of the sarcoma cells into the alveolar septae at the transition of the metastasis to the normal lung tissue and was found to be prognostic. 'Pleural penetration' was defined as the infiltration and destruction of all visceral pleural layers by the tumour and was found to be a risk factor for local recurrence. RESULTS The median post-metastasectomy overall survival was 50.3 months, and the corresponding 5-year survival rate was 44.7%. Age >55 years at metastasectomy (P?=?0.02), the presence of interstitial growth (P?=?0.008), size of the largest metastasis >35?mm (P?=?0.023) and the presence of tumour recurrence at any site after metastasectomy (P?5?mm were found to be risk factors for local intrapulmonary recurrence. CONCLUSIONS Interstitial tumour growth, which is easily detected by light microscopy, can serve as a strong predictor of survival following pulmonary metastasectomy in sarcoma patients. Obvious pleural infiltration indicates the need for larger margins.  相似文献   

19.
BACKGROUND: Salivary gland tumors represent 1% of head and neck tumors, with only 5% of these occurring in patients younger than 20 years. Mucoepidermoid carcinoma (MEC) is one of the most frequent salivary gland cancers among adults and children. METHODS: This survey was conducted among 34 French pediatric oncology departments. From 1980 to 2000, 18 cases were reported. RESULTS: Treatment included surgery or radiotherapy, or both. The 5-year survival rate was 93.7%. Eleven patients had been previously treated by radiotherapy and/or chemotherapy for a first malignant tumor, specifically, lymphoid leukemia (n = 4), lymphoma (n = 3), brain tumor (n = 2), sarcoma (n = 1), and retinoblastoma (n = 1). CONCLUSIONS: MEC is very rare in the pediatric age group. Treatment involves surgical removal of the tumor plus radiotherapy, according to histologic staging. MEC has a good prognosis in young patients. The survival rate does not differ in the subgroup of patients with MEC as a secondary tumor.  相似文献   

20.
The survivin:fas ratio in pediatric renal tumors   总被引:16,自引:0,他引:16  
BACKGROUND/PURPOSE: Apoptosis factors inducing or preventing cell death may govern the behavior of certain tumors. Fas is a pro-apoptotic receptor that induces cell death when bound by its ligand and is expressed at greater levels in pediatric renal tumors of good prognosis. Survivin is a novel inhibitor of apoptosis that is expressed in a cell cycle-dependent manner and is abundantly expressed in several tumors of unfavorable histology. This study evaluates the expression of survivin, as well as the prognostic value of the survivin:fas ratio in various types and stages of pediatric renal tumors. METHODS: Multiple apoptosis mRNA species were quantified by Rnase protection assay (RPA) in 32 pediatric renal tumors and adjacent normal kidney specimens before chemotherapy: Wilms' tumor (WT), n = 9; clear cell sarcoma (CCS), n = 4; rhabdoid tumor of the kidney (RTK), n = 5; mesoblastic nephroma (MN), n = 3 and normal kidney, n = 11. Western Blot and immunocytochemistry were used to confirm survivin protein expression in a selective specimen survey. Follow-up data were obtained on patient outcomes, and antiapoptotic to proapoptotic ratios were calculated and correlated with clinical recurrence of disease. RESULTS: Pediatric renal tumors express greater levels of both pro- and antiapoptotic factors than normal kidney. Survivin and fas appeared to be expressed differentially in the tumor specimens sampled. Five of 10 (50%) tumors that went on to recur expressed survivin, whereas survivin was present in only 2 of 11 (18%) nonrecurrent tumors. Conversely, only 2 of 10 (20%) tumors that recurred were fas positive, whereas 5 of 11 (45%) tumors that did not recur expressed fas. The mean survivin:fas ratio was significantly greater in the 10 tumors that went on to recur after treatment (4 RTK, 3 CCS, 3 WT), than in tumors not recurring (2.16+/-1.4 v 1.0+/-1.07; P =.01, Kruskal-Wallis test). The positive predictive value of tumor recurrence was 85.7% (CI: 42.1%, 99.6%) and the negative predictive value was 71.4% (CI: 41.9%, 91.6%) when a cutoff ratio of 1.6 was considered. CONCLUSIONS: The survivin:fas mRNA ratio is of prognostic value in its ability to predict recurrent disease in children undergoing treatment for pediatric renal tumors. In this series, a ratio of greater than 1.6 predicted recurrent disease with a high probability irrespective of clinical stage or pathologic type. Determining the survivin:fas ratio may guide treatment, follow-up and counseling of patients with pediatric renal tumors.  相似文献   

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