The dose–volume interaction in adult supratentorial low-grade glioma: higher radiation dose is beneficial among patients with partial resection

Purpose To evaluate the hypothesis that adults with partially resected (PR<50% resection) supratentorial low-grade glioma (LGG) benefit from higher doses of radiation. Methods Patients receiving post-operative radiation for WHO grade I–II LGG at the University of Western Ontario between 1979 and 2001 were studied. Patient characteristics evaluated included: age, gender, symptom duration >30 days, seizures at presentation, Karnofsky performance status (KPS) <70, astrocytoma pathology (AS), and radiation dose. A Cox proportional hazard regression model was constructed to test the influence of radiation dose. Results One hundred and seven patients were analyzed. Patients who had PR were not significantly different from those with STR (subtotal/total resection) in terms of patient characteristics. Median survival (MST) of PR patients who received ≤50 Gy was 16.5 months while those who received >50 Gy had a MST of 109.2 months. The interaction of radiation dose and extent of resection was tested after controlling for other patient factors by Cox regression model. The interaction was highly significant for both OS and PFS (P=0.013 and P=0.003, respectively). This model remained significant after excluding six patients receiving doses <42 Gy (OS, P=0.024, and PFS, P=0.001). Conclusions The outcome for patients with LGG is dependent on extent of tumor resection and radiation dose. Patients with PR should be considered for higher radiation dose schedules (>50 Gy). Future trials on therapeutic strategies for LGG should consider stratification of patients by extent of tumor resection. Our data suggests that one dose does not fit all. An erratum to this article can be found at     

Pathogenesis of histologic variations of appendiceal mucinous neoplasms

BackgroundIn order for peritoneal metastases from a primary appendiceal mucinous neoplasm to occur, the wall of the appendix must perforate to allow mucus with tumor cells access to the peritoneal spaces. With progression the peritoneal metastases show a broad spectrum of tumor biology varying from indolent to aggressive activity.MethodsThe histopathology of peritoneal tumor masses was determined from the clinical material resected at the time of cytoreductive surgery (CRS). All groups of patients were treated by a uniform strategy that involved complete CRS and perioperative intraperitoneal chemotherapy. Overall survival was determined.ResultsFrom a database of 685 patients, four histologic subtypes were identified and long-term survival determined. Four hundred and fifty patients (66.0%) had low-grade appendiceal mucinous neoplasm (LAMN), 37 patients (5.4%) had mucinous appendiceal adenocarcinoma of intermediate subtype (MACA-Int), 159 patients (23.2%) had mucinous appendiceal adenocarcinoma (MACA), and 39 patients (5.4%) had a mucinous appendiceal adenocarcinoma with positive lymph nodes (MACA-LN). The mean survival of the four groups was 24.5, 14.8, 11.2 and 7.4 years, respectively (p < 0.0001). These four subtypes of mucinous appendiceal neoplasms were shown to have distinct survival estimates.ConclusionsThe estimated survival of these four histologic subtypes in patients having a complete CRS plus HIPEC is of value to the oncologist managing these patients. A mutations and perforations hypothesis was offered in an attempt to explain the broad spectrum of mucinous appendiceal neoplasms that exist. Inclusion of MACA-Int and MACA-LN as standalone subtypes was thought to be necessary.     

北方食管癌高发区食管和食管-胃交界部低级别内瘤变队列随访

目的:食管和贲门上皮不典型增生是癌前病变,分为轻度,中度和重度三个级别.2000年WHO建议用上皮内瘤变取代不典型增生,将轻度和中度归为低级别内瘤变,并将贲门腺癌称为食管-胃交界腺癌.本文探讨食管癌高发区食管和食管-胃交界部低级别上皮内瘤变癌变危险性和时间演变规律,为食管癌二级预防提供科学数据.方法:选择磁县2个乡于2001年10月～2002年10月,开展内镜碘染色队列筛查,干预乡0～85岁总人口数为22 016例,其中40～69岁共计6 596例,男性3 257例,女性3 339例,除去禁忌症、外出打工、拒绝参加者外,实际内镜筛查3 506例,筛查率53.2%.病理以WHO诊断为标准,将食管鳞状上皮轻度和中度不典型增生及食管-胃交界部轻度不典型增生划为低级别内瘤变人群,共诊断616例;内镜筛查无癌前病变和非癌诊断的作为正常人群共2478例.2008年6～9月对该队列人群进行随访核实.其中有174例失访,随访率95.0%.结果:低级别内瘤变人群和内镜病理正常人群随访分别为3 970.7人·年和16 120.0人·年,低级别内瘤变人群癌变率251.7/10万人·年,正常人群68.2/10万人·年;中位癌变时间分别是38个月和47个月.与正常人群比较,LIN癌变相对危险度RR=3.69(1.57～8.69),P=0.001.结论:低级别内瘤变人群是一组高危险人群,每年内镜复查是必要的.     

Low-grade fibromyxoid sarcoma of the hand: a case report

We report a case of low-grade fibromyxoid sarcoma with giant collagen rosettes in the hand of a 21-year-old female. This is a clinically and radiological benign appearing tumor that has a high rate of recurrence and metastasis.     

Harm or benefit of hormonal treatment in metastatic low-grade endometrial stromal sarcoma: single center experience with 10 cases and review of the literature

Endometrial stromal sarcoma (ESS) is a rare disease with probably less than 700 new cases in the US or EU per year. ESS usually expresses steroidal receptors and is regarded to be hormone-sensitive. A higher risk in women receiving estrogen replacement therapy (ERT) or tamoxifen has been suspected, and remissions following treatment with progestins have been reported in case studies. Aromatase inhibitors represent an interesting new treatment option. Due to the rarity of the tumor, only few case series and no prospective studies are published. We therefore conducted a retrospective study to evaluate the influence of hormonal treatment to ESS. METHODS: Our institutional sarcoma data bank was screened for cases of ESS since 1999. All corresponding files and radiographs were reviewed retrospectively. RESULTS: Ten patients with low-grade ESS were identified. Diagnosis was established before or by hysterectomy in 6 patients, by local recurrence after previous hysterectomy for nonmalignant disease in 3 patients or by pulmonary metastases with no primary tumor found so far in 1 patient. 5/10 patients were on ERT and 3/10 on tamoxifen at the time of diagnosis of metastatic disease. Treatment strategies consisted of stopping ERT and tamoxifen, respectively, or initiation of the progestin MPA or letrozole. Three patients achieved stable disease after stopping ERT. 2/3 patients responded to MPA as first-line treatment (1 CR; 50+ months, 1 PR; 9 months). 4/5 patients responded to letrozole as first-line therapy (3 PR;3+, 9+ and 10+ months) or second-line treatment after MPA (1 PR; 37+ months). 9/10 patients are alive 33 to 255 months after hysterectomy. Survival since diagnosis of metastatic disease is 4 to 164 months. CONCLUSIONS: Patients with a previous history of low-grade ESS should not be treated with estrogens or tamoxifen. If nevertheless present, withdrawal of ERT or tamoxifen is strongly advised, resulting in disease stabilization in some cases. MPA and letrozole, in particular, are highly effective and lead to sustained disease control in most cases.     

Ovarian low-grade serous carcinoma: a comprehensive update

Ovarian low-grade serous ovarian carcinoma (OvLGSCa) comprises a minority within the heterogeneous group of ovarian carcinomas. Despite biological differences with their high-grade serous counterparts, current treatment guidelines do not distinguish between these two entities. OvLGSCas are characterized by an indolent clinical course. They usually develop from serous tumors of low malignant potential, although they can also arise de novo. When compared with patients with ovarian high grade serous carcinoma (OvHGSCa) patients with OvLGSCa are younger and have better survival outcomes. Current clinical and treatment data available for OvLGSCa come from retrospective studies, suggesting that optimal cytoreductive surgery remains the cornerstone in treatment, whereas chemotherapy has a limited role. Molecular studies have revealed the preponderance of the RAS-RAF-MAPK signaling pathway in the pathogenesis of OvLGSCa, thereby representing an attractive therapeutic target for patients affected by this disease. Improved clinical trial designs and international collaboration are required to optimally address the unmet medical treatment needs of patients affected by this disease.     

Primary low-grade B-cell lymphoma in mucosa-associated lymphoid tissue of the lung

A 67-year-old asymptomatic woman was referred to our department for treatment for a left lung tumor. The tumor was 40 mm in diameter and was present in the left lower lobe. A primary low-grade B-cell lymphoma in the mucosa-associated lymphoid tissue of the lung was suspected after a trans-bronchial lung tissue biopsy. Systemic screening revealed no other organ involvement, and the tumor was considered to be a primary lung tumor. A left lower lobectomy with hilar and mediastinal lymph node sampling was performed. Pathological examination revealed centrocyte-like cells that expressed CD20. These cells had invaded the bronchiolar epithelium to form lymphoepithelial lesions. The pathological diagnosis was confirmed to be primary low-grade B-cell lymphoma in the mucosa-associated lymphoid tissue of the lung. The origin of this tumor type in the lung is relatively rare.     

Fatigue in low-grade glioma

The aim of this study was to determine the prevalence and severity of fatigue in long-term survivors with a low-grade glioma (LGG), and to analyze the relationship between fatigue and demographic variables, disease duration, tumor characteristics, former tumor treatment modalities, antiepileptic drug (AED) use, self-reported concentration, motivation, and activity. Fifty-four patients with stable disease (age range, 25–73 years) who were diagnosed and treated more than 8 years ago were included in this study. Fatigue was analyzed with the Checklist Individual Strength (CIS). Thirty-nine percent of the LGG patients were severely fatigued, with older patients being most affected. Severe fatigue was associated with AED use, and with reduced self-reported concentration, motivation, and activity. No relation was found between fatigue and gender, histology, tumor laterality, disease duration, type of neurosurgical intervention and radiation treatment. Fatigue is a severe problem in a large proportion of long-term surviving LGG patients.