Pheochromocytoma in multiple endocrine neoplasia type 2: a prospective study

OBJECTIVE: The aim of this prospective study is to update our knowledge of the chronology of pheochromocytoma occurrence in multiple endocrine neoplasia type 2 (MEN 2), and to better manage MEN 2 patients after the genetic diagnosis. DESIGN: Eighty-seven non-index gene carrier MEN 2 patients were included in this prospective study: 84 patients with MEN 2A (from 52 families) and 3 with MEN 2B (from 3 families). METHODS: Medullary thyroid carcinoma (MTC) was diagnosed by measuring plasma calcitonin in basal conditions or after pentagastrin stimulation. The search for pheochromocytoma consisted of clinical evaluation, 24 h determination of urinary catecholamines and adrenal imaging. The mean age at genetic diagnosis of MEN 2 was 14.0+/-7.0 years, the mean duration for the follow-up was 7.6+/-2.8 years. RESULTS: All 87 patients had a MTC detected at the same time as the genetic diagnosis was made. Urinary catecholamine measurements led to the diagnosis of pheochromocytoma and a combination of imaging techniques enabled the correct localization of both unilateral or bilateral adrenal involvement. Pheochromocytoma was detected simultaneously with MTC in only seven patients, and seven others were detected throughout the follow-up. Of the 14 patients with pheochromocytoma, 11 had bilateral involvement: nine were initially bilateral and two became so during follow-up. CONCLUSION: This study demonstrates that in MEN 2, MTC is the lesion which appears earliest. Pheochromocytoma develops later during the evolution of the disease, and necessitates regular clinical and biological monitoring throughout follow-up. Determination of urinary and/or plasma catecholamines and metanephrines should be performed to detect pheochromocytoma. Imaging techniques lead to the detection of both unilateral and bilateral pheochromocytoma, thus making video-assisted laparoscopic adrenalectomy possible.     

超短波透热化疗与单纯化疗对晚期恶性肿瘤疗效比较

本文报告手术或放疗后复发和/或转移的晚期恶性肿瘤26例,随机分为两组.一组用两台超短波同步交叉透热合并化疗(热化组),有效率84.6％,生存时间20.9±4.5个月.另一组单纯化疗(单化组),有效率53.8％,生存时间8.3±3.8个月.两组疗效比较有显著性差异.采用超短波交叉透热合并化疗,方法简单、安全、经济、无痛苦,易于推广.     

Improved survival outcome for hepatoblastoma based on an optimal chemotherapeutic regimen--a report from the study group for pediatric solid malignant tumors in the Kyushu area

Background/purpose

The survival outcome for patients with hepatoblastoma normally depends on the resectability of the tumor. In Japan, the pre and/or postoperative chemotherapy protocol using a combination of cisplatin (CDDP) and tetrahydropyranyl-Adriamycin (THP-ADR) has been the standard treatment since 1991. This study aims to assess exactly what influence the establishment of this chemotherapy protocol has had on both the tumor resectability and the outcome of patients with hepatoblastoma.

Methods

From 1982 to 1997, 60 patients with hepatoblatoma were treated in the Kyushu area, Japan. Based on the pretreatment extent of disease (PRETEXT), the outcome and tumor resectability were compared between group A (1982 to 1990, n = 27, PRETEXT I:5, II:8, III:6, IV:8) and group B (1991 to 1997, n = 33, PRETEXT I:9, II:9, III:5, IV:10).

Results

The 5-year survival rates (group A and group B) were 33% and 73% for all cases (P < .01), 100% and 89% for PRETEXT I, 38% and 89% for II (P < .05), 17% and 80% for III (P < .01), and 0% and 40% for IV (P < .01), respectively. The 5-year survival rates for patients with metastases were 0% for group A (n = 5) and 57% for group B (n = 7; P < .01). The rates of a complete resection of primary tumor were 48% for group A and 67% for group B. In particular, a significant difference was found regarding the complete resection rate between groups A and B in the patients with PRETEXT III (17% for group A and 80% for group B; P < .01). In the patients with an incomplete tumor resection (14 for group A, 11 for group B), the 5-year survival rates were 0% for group A and 45% for group B (P < .01).

Conclusions

The optimal chemotherapeutic regimen of CDDP and THP-ADR was thus found to greatly contribute to the improved survival rate of hepatoblastoma patients. Preoperative chemotherapy resulted in an increased resectability of the tumor, whereas postoperative chemotherapy played an important role in the increased cure rate of cases with either an incomplete tumor resection or metastasis. However, refractory cases with PRETEXT IV or metastasis may still require the development of an even more effective treatment modality, including the use of blood stem cell transplantation.     

一种新的^3H—TdR掺入法抗癌药敏试验

作者对琼脂-液体双层抗癌药敏试验系统加以改进,建立了更为简便有效的试验系统。经对84例临床肺癌患者活标本测试,成功率达85%。将肺癌细胞对长春新碱(VCR),丝裂霉素(MMC),顺铂(DDP)和阿霉素(ADM)的敏感界限,以1/10血液峰值浓度下细胞存活率表示,并分别定为50%、40%、30%和30%时,测试结果与文献报告的肺癌临床单药化疗有效率近似。本法与干细胞法作平行试验测试10个肺癌细胞系对4种药物的敏感性,两法符合率为83%。测试4例肺癌活检标本,两法符合第为100%。作者认为本试验系统具有临床推广应用价值。     

Evidence of activity of Irinotecan in patients with advanced AIDS-related Kaposi's sarcoma

Fourteen HIV-infected patients with advanced Kaposi's sarcoma (KS) received Irinotecan 150 mg/m intravenously on days 1 and 10. All patients were relapsed/progressed during highly active antiretroviral therapy, administered as primary antineoplastic therapy. An objective response, all partial remissions, occurred in 75% of patients. Irinotecan was well tolerated, severe leukopenia occurred in only 33% of patients. In HIV-infected patients with advanced KS, irinotecan is active and well tolerated.     

Autologous hematopoietic stem cell transplantation for breast cancer in Europe: critical evaluation of data from the European Group for Blood and Marrow Transplantation (EBMT) Registry 1990-1999

The aim of this study was to identify trends in high-dose chemotherapy (HDC) with autologous hematopoietic stem cell transplantation (ASCT) and to assess survival in a large cohort of breast cancer (BC) patients receiving this therapy in Europe from 1990 to 1999. A total of 7471 patients who received HDC with ASCT between January 1, 1990 and December 31, 1999 were reported to the European Group for Blood and Marrow Transplantation Registry. Data required for demographics and survival analysis were available for 2679 patients with high-risk primary BC; 921 patients with inflammatory BC (IBC), and 2295 patients with metastatic disease. The main evaluation parameters were progression-free survival (PFS) and overall survival (OS). Between 1990 and 1998, autotransplants for BC increased 30-fold. Significant trends included use of blood-derived rather than marrow-derived stem cells, increment of reporting centers and decrease of mortality within 100 days from transplantation. The 5-year PFS and OS probabilities were 53 and 68% for high-risk disease and 42 and 53% for IBC, respectively. For metastatic disease 5-year PFS and OS probabilities in the whole cohort were 18 and 27%, respectively, while for women transplanted in complete remission the 5-year PFS was 29%. In conclusion, HDC with ASCT has been increasingly used until 1998 and the 100-day mortality rate has been constantly less than 2% from 1995 to date. The 5-year survival of high-risk BC is related to the number of axillary nodes involved at surgery. Outcome of patients with IBC is encouraging, suggesting the need for randomized trials. Patients with metastatic disease responding to pretransplant chemotherapy and harboring ER+ tumors have a better outcome.     

Late effects in 164 patients with rhabdomyosarcoma of the bladder/prostate region: a report from the international workshop

PURPOSE: We determined the late sequelae in children and adolescents with rhabdomyosarcoma of the bladder/prostate treated in the United States, Canada and selected Western European countries, primarily France, Germany, Italy and the United Kingdom, from 1979 to 1998. MATERIALS AND METHODS: We used a data collection form to record data from patient records available at the group statistical centers. RESULTS: A total of 164 patient charts had sufficient data available to be included in the study. Median patient age at diagnosis was 2.4 years. Median length of followup was approximately 8 years (range 3 to 24). Of the patients with available data 78 did not undergo cystectomy, 49 underwent partial cystectomy and 34 underwent complete cystectomy. Urinary continence was assessed at age 6 years or older in 62 patients who did not undergo cystectomy. Of these patients 43 (69%) were continent, 16 had nocturnal incontinence and 9 had diurnal incontinence. Of 44 patients who underwent partial cystectomy and had pertinent followup data 32 (73%) were continent, and 12 had nocturnal and/or diurnal incontinence. Only 11 patients underwent urodynamic investigation. Other nephrourological complications consisted of 3 or more urinary tract infections in 29 of 53 patients, abnormal renal function in 19 of 48 (tubulopathy 14, increased creatinine/blood urea nitrogen 5), chronic hematuria in 13 of 51 and hydronephrosis in 8 of 54 with available data. Vesicoureteral reflux, urinary stones and bowel problems were infrequent. CONCLUSIONS: Of the patients 48% had a relatively intact bladder after biopsy only. However, 31% of patients 6 years or older had some urinary incontinence, as did 27% of patients who had undergone partial cystectomy. In addition, 55% of all patients had 3 or more urinary tract infections, 40% had decreased renal function and 25% had chronic hematuria. Other complications were present in 15% or less of the patients with available data.