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PubMed2004

Decreased Hrad6B expression in lung cancer.

Human homologues of yeast Rad 6 (Hrad6B) encode ubiquitin-conjugating enzymes and complement the DNA repair and UV mutagenesis defects of Saccharomyces cerevisiae rad6 mutant. There is a larger subgroup with reduced DNA repair capacity that is likely to be at increased cancer risk. The authors investigated Hrad6B expression in lung cancer. An attempt was made to determine the influence of Hrad6B expression on clinicopathological features for patients with lung cancer who had undergone surgery. Expression of Hrad6B messenger RNA was evaluated by quantitative reverse transcription-polymerase chain reaction (RT-PCR) in 110 lung carcinomas and adjacent histological non-malignant lung samples from patients for whom follow-up data were available using LightCycler. The Hrad6B/glyceraldehydes-3-phosphate dehydrogenase (GAPDH) mRNA expression was significantly decreased in the lung cancer tissue (4.078+/-5.674) as compared with the non-malignant lung tissue (10.495+/-12.976, p<0.001). There was no relationship between Hrad6B/GAPDH expression and age, clinical stages, T-status, N-status, and pathological subtypes in lung cancer tissues. Hrad6B/GAPDH mRNA levels in males (3.521+/-4.280) and in females (6.420+/-8.167) were significantly different (p=0.0443) in lung cancer tissues, but not in non-malignant lung tissues. Heavy smokers had a slight non-significant tendency (p=0.0857) towards lower Hrad6B/GAPDH mRNA levels (3.453+/-4.743) in their lung cancer tissues as compared with light or non-smokers. Thus decreased Hrad6B mRNA expression might be a biomarker for decreased DNA repair capacity and the dysfunction of Hrad6B might play a role in the tobacco-related oncogenesis of lung cancer.

Aged↗

PubMed2003

Successful treatment of aggressive HIV-associated non-Hodgkin's lymphoma with combination chemotherapy, biotherapy with rituximab and HAART: presentation of a therapeutic option.

The incidence of non-Hodgkin's lymphoma (NHL) in individuals infected with human immunodeficiency virus (HIV) is more than 60 times higher than in matched controls. In the vast majority of cases aggressive pathological subtypes and advanced stages prevail, extranodal sites are involved and systemic symptoms are present. The prognosis of HIV-NHL remains poor and the optimal therapeutic approach has yet to be defined. We report a 48-year-old Ethiopian woman with advanced-stage HIV infection, who developed diffuse large cell, immunoblastic type B-cell NHL and was treated with a modified CHOP-like chemotherapy combined with Rituximab and supported with growth factor. Highly active antiretroviral therapy (HAART) and opportunistic infections prophylaxis were administered concomitantly. The patient completed 6 cycles of therapy and currently, 76 weeks after diagnosis, is in complete clinical remission. Despite the fact that there was a transient decrease in the CD4-positive cell number and a 1.5 log increase in plasma viral load there were no opportunistic infections, nor was life-threatening toxicity seen. Rituximab seems a well-tolerable and advantageous adjunct to chemotherapy and HAART in the treatment of aggressive HIV-associated NHL andshould be investigated in large trials in the future.

Antibodies, Monoclonal↗

PubMed2003

Anaplastic large cell lymphoma--a rare disorder in southern Taiwan.

Anaplastic large cell lymphoma (ALCL) is a subgroup of non-Hodgkin's lymphomas with large lymphoma cells expressing CD30 antigen. This entity has rarely been reported in Taiwan. We performed a retrospective clinicopathologic study in a medical center in southern Taiwan during a 13-year period and identified 13 cases. There were 10 males and 3 females with a median age of 49 years old. Seven presented with pure nodal disease and 5 had bony involvement. The staging results were stage I (5 patients), II (1), III (1), and IV (4). The pathologic subtypes were common variant (10), lymphohistiocytic variant (2), and small cell variant (1). Eleven tumors were of T-cell lineage; 2, null-cell. Immunohistochemically, 5 tumors (38.5%) expressed cytotoxic markers, T-cell intracellular antigen-1 and/or granzyme B. Two tumors (15.4%) expressed anaplastic lymphoma kinase (ALK). Long-term follow-up information was available in 8 patients. The 2 patients with ALK-expressing tumors (37 and 49 years old) were free of disease for 61 and 54 months, respectively. The other 6 patients were either died of disease (5 patients) or experienced relapse with progressive disease (1). In conclusion, we reported the largest series of ALCL in Taiwan. We confirmed ALK-expressing ALCL carries favorable prognosis and ALK-negative ALCL has similar poor prognosis as non-anaplastic T-cell lymphoma. As compared to the previous reports from the West, our ALK positive rate was lower and the age of our ALK-positive patients was older. A larger national or multi-institutional study is needed for further characterization of ALCL in Taiwan.

Adolescent↗

PubMed2001

Children with difficult asthma: a practical approach.

Many open studies investigating the effects of innovative treatments for steroid-dependent asthma demonstrate some benefit. This is also true of the majority of placebo arms in placebo-controlled trials. This suggests that children with difficult asthma benefit from the high level of input that is typically provided in clinical trials, with or without additional medication. Such intensive management of patients, with the emphasis on establishing the diagnosis, improving adherence, and identifying provoking factors, is the key to optimizing asthma control for these children. For patients with genuinely severe asthma, despite high doses of conventional treatment, a greater understanding of the pathological basis of persistent symptoms is needed. Identification of different pathological subtypes of severe asthma should allow for more rational prescribing of asthma therapy, as well as the design of further trials of potential steroid-sparing treatments.

Administration, Inhalation↗

PubMed2003

Prognostic factors for patients with advanced stage serous borderline tumours of the ovary.

BACKGROUND: The aim of this study was to determine the prognostic factors for patients with advanced stage, low malignant potential ovarian tumour (LMPOT). PATIENTS AND METHODS: A retrospective review of 80 patients with serous LMPOT and peritoneal implants treated at or referred to our institution was carried out. RESULTS: Sixty-five patients had non-invasive implants. Fifteen patients had invasive implants. Twenty-nine patients had stage II and 51 patients had stage III disease. Three patients died of evolutive invasive disease and four of complications of treatment. The only prognostic factor of progression to 'evolutive invasive disease' is the pathologic subtype of peritoneal implants. The 5-year rates of evolutive invasive disease in patients with non-invasive implants and invasive implants were 2% and 31%, respectively (P <0.002). CONCLUSIONS: In this series, the only prognostic factor for patients with advanced stage borderline tumour is the type of peritoneal implant. More patients died of the treatment's complications than of the disease itself. The patients' prognosis with non-invasive implants seems to be excellent, and conservative management could be discussed in younger patients.

Adolescent↗

PubMed2004

The palliative benefits of MVP (mitomycin C, vinblastine and cisplatin) chemotherapy in patients with malignant mesothelioma.

BACKGROUND: With the rising incidence of malignant mesothelioma (MM), it is important to optimise treatment to control symptoms, maintain quality of life and, if possible, prolong life. We have analysed prospectively collected data to evaluate a frequently used palliative chemotherapy regimen. PATIENTS AND METHODS: Between October 1986 and May 2002 all patients with inoperable pleural mesothelioma were considered for treatment with MVP (mitomycin C 8 mg/m2 every 6 weeks, vinblastine 6 mg/m2 every 3 weeks and cisplatin 50 mg/m2 every 3 weeks) chemotherapy. Symptoms were assessed by physician assessment at baseline and after each cycle of chemotherapy. RESULTS: One hundred and fifty patients were treated with MVP for mesothelioma. Forty-three per cent had a performance status (PS) 2 or worse. The response rate was 15.3%, with 68.6% having stable disease. Sixty-nine per cent reported an improvement in symptoms; in particular there were good responses for pain (71%), cough (62%) and dyspnoea (50%). The most common grade 3/4 toxicity was neutropenia (22%). Median overall survival was 7 months, with 1-year survival 31% and 2-year survival 11%. Median survival for patients with PS 0/1 was 10 months, and was 6 months for patients with PS 2/3. Poor prognostic factors in univariate analysis included poor PS, weight loss, mixed or sarcomatoid histology, low haemoglobin and high white blood cell count. Excluding pathological subtype, the prognostic significance of poor PS and weight loss were retained in multivariate analysis. CONCLUSIONS: Palliation of symptoms in MM is achievable with current cisplatin-based treatments.

Adult↗

PubMed2005

Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience.

BACKGROUND: Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct clinico-pathological subtype of diffuse large B-cell lymphoma (DLBCL). The optimal treatment is unknown, with some studies suggesting a superior outcome with dose-intensive chemotherapy regimens, and the role of radiotherapy remains ill-defined. PATIENTS AND METHODS: The British Columbia Cancer Agency lymphoma database was searched and records reviewed to identify those patients presenting with a prominent mediastinal mass and considered to be PMBCL based on the current REAL/WHO classifications. Patients were treated based on era-specific BCCA guidelines (1980-1992 MACOPB/VACOPB; 1992-2001 CHOP-type; 2001-present CHOP-R). Beginning in January 1998 involved-field radiotherapy was recommended to be routinely administered following chemotherapy. Prior to this, use of radiotherapy was individualized in advanced disease. RESULTS: In total, 153 patients with newly diagnosed PMBCL were identified between 28 July 1980 and 30 June 2003. The median age was 37 years (range 13-82) and the majority had stage I/II (74%), bulky mediastinal disease (75%). Overall (OS) and progression-free (PFS) survival at 5 years for the entire cohort were 75% and 69%, respectively. In direct comparison with a cohort of patients with DLBCL (n = 1273), OS (P = 10(-4)) and PFS (P = 0.0001) favored PMBCL. The age-adjusted International Prognostic Index (aaIPI) was not predictive of survival (P = 0.18). Five-year OS in patients < 65 years old treated with MACOPB/VACOPB, CHOP-R and CHOP-type was 87%, 81% and 71% respectively (P = 0.048). In pair-wise survival comparisons, only MACOPB/VACOPB and CHOP-type treated patients were significantly different (P = 0.016). In Cox multiple regression analysis, poor performance status remained the only predictor of survival, with treatment received demonstrating a trend to worse outcome for patients treated with CHOP-type regimens (P = 0.09). In an intention-to-treat analysis comparing the era before radiotherapy was routinely administered with after, there was no significant difference in 5-year PFS (74% versus 62%; P = 0.09) or OS (78% versus 69%; P = 0.14). CONCLUSIONS: In this single institution, population-based retrospective study, we found that PMBCL patients have excellent survival rates and a distinct plateau is observed in PFS, in striking comparison to DLBCL. The aaIPI was not predictive of survival in this population, suggesting that other prognostic models may be better suited for risk stratification. Dose-intensified chemotherapy with MACOPB or VACOPB demonstrated a trend to superior outcome over CHOP-type chemotherapy. However, further randomized studies are needed and the impact of rituximab on these comparisons must be considered. Finally, the routine addition of radiotherapy does not improve survival.

Adult↗

PubMed2001

Expression of the protein gene product 9.5, PGP9.5, is correlated with T-status in non-small cell lung cancer.

BACKGROUND: In a tumor, increased deubiquitination of cyclins by a protein gene product gene, PGP9.5, could contribute to the uncontrolled growth of somatic cells that is a hallmark of cancer. We hypothesized that PGP9.5 may be expressed in tumors of patients with non-small cell lung cancer (NSCLC). METHODS: Expression of PGP9.5 messenger RNA was evaluated by reverse transcription polymerase chain reaction (RT-PCR) in 95 non-small cell lung carcinomas and adjacent histological normal lung samples. The data were analyzed with reference to clinicopathological factors. RESULTS: PGP9.5 transcripts were detected in 18 (12.8%) of the tumor samples, although some of paired normal lung samples showed very weak expression. There was no relationship between PGP9.5 gene expression and age, gender, N-status or pathological subtype. PGP9.5 gene was preferentially expressed in T3/T4 NSCLC (12/41, 29.3%) compared with T1/T2 NSCLC (6/54, 11.1%) (p = 0.0482). CONCLUSIONS: Although the PGP9.5 gene was not expressed in a majority of NSCLC tumors, we suggest that PGP9.5 may correlate with tumor invasion or progression of NSCLC.

Adult↗

PubMed2002

Beta IGH3, a TGF-beta inducible gene, is overexpressed in lung cancer.

BACKGROUND: Transforming growth factor-beta (TGF-beta) is capable of affecting the proliferation of many cell types. beta IGH3, TGF-beta inducible gene, was originally isolated from lung adenocarcinoma cell line (A549). We have hypothesized that beta IGH3 mRNA levels could be predictors of the development and invasion of lung cancer. METHODS: The study included 71 lung cancer cases. The beta IGH3 mRNA levels were quantified by real time reverse transcription polymerase chain reaction (RT-PCR) using LightCycler. RESULTS: The beta IGH3 mRNA levels were elevated in tumor tissues from lung cancer (1.058 +/- 1.212) compared with non-malignant lung tissues (0.304 +/- 0.159) (p = 0.0001). No significant difference in beta IGH3 mRNA levels was found among gender, age, pathological subtype and lymph node metastasis. The beta IGH3 mRNA levels were elevated in tumor tissues from T4 lung cancer (1.425 +/- 1.470) compared with those from T1 lung cancer (0.702 +/- 0.655) (p = 0.05) and T2 lung cancer (0.736 +/- 0.734) (p = 0.044). CONCLUSION: Using the LightCycler RT-PCR assay, the determination of beta IGH3 mRNA level might provide a potential marker for the aggressiveness of lung cancer. However, further studies and a longer follow-up are needed to confirm the impact of beta IGH3 on the biological behavior of the tumor.

Adult↗

PubMed2004

The role of radiotherapy for thymic carcinoma.

OBJECTIVE: The aim of this study is to evaluate retrospectively the role of radiotherapy for thymic carcinoma. METHODS: Between 1973 and 1998, 14 patients with thymic carcinoma were treated at Gunma Prefectural Cancer Center. Two patients who had hematogenous metastasis were excluded from this study, therefore 12 patients were analyzed. The Masaoka staging system was used; four patients were diagnosed with stage III disease and eight patients with stage IV disease. The pathological subtype according to the World Health Organization histological criteria for thymic tumors was squamous cell carcinoma (low-grade histology) in six cases and undifferentiated carcinoma (high-grade histology) in six. Ten patients underwent thoracotomy, and two patients underwent excisional biopsy without thoracotomy. Ten patients (83%) received radiotherapy as a curative intent, and the median dose was 60 Gy. Systemic chemotherapy was administered to four patients (33%), and the majority (75%) of the regimens contained cisplatin. RESULTS: The 3-year overall survival rate was 25%. Histological subtype (low-grade versus high-grade), surgical resection (complete versus incomplete), radiotherapy and chemotherapy were evaluated as prognostic factors in a univariate analysis. Low-grade histology and complete resection were good prognostic factors, although these were not statistically significant. Patients who received radiotherapy had a better outcome than those who did not. The major sites of recurrence were the pleura and pericardium. Recurrence within the radiation field was observed in one of seven patients in whom failure patterns could be evaluated. CONCLUSION: Complete resection is mandatory if possible. Radiotherapy plays an important role in treating thymic carcinoma in terms of reducing local recurrence and prolonging survival time. Establishment of an innovative treatment protocol that includes chemotherapy is necessary to control intrathoracic relapse and distant metastasis.

Adult↗

PubMed1996

Combination chemotherapy for advanced bilharzial bladder carcinoma.

BACKGROUND: Carcinoma of the bilharzial bladder, the most common cancer in Egyptian patients has been, until recently, largely treated by surgery. We have studied the activity of a series of single agents in phase II trials and identified a number of active agents. Here we report the results of a trial in which therapeutic combinations of the most active agents were administered in alternating cycles to patients who had never received chemotherapy. PATIENTS AND METHODS: The study included 30 patients with histologically proven inoperable (20), recurrent (5, 2 of whom subsequently developed metastases), or metastatic disease (5). There were 27 males and 3 females, with a median age of 48.5 years (range 29-65 years). Fourteen patients had squamous cell carcinoma, 12 had transitional cell carcinoma, 2 had adenocarcinoma, and the remaining 2 had undifferentiated carcinoma. Chemotherapy consisted of epidoxorubicin (120 mg/sqm i.v. d1) and vincristine (1.4 mg/sqm i.v., days 1 and 8) alternating with etoposide (100 mg/sqm i.v. infusion over 1 hour, days 1 to 5) and ifosfamide (1800 mg/sqm i.v. infusion over 2 hours, days 1 to 5). Mesna was given as a uroprotector at 40% of the ifosfamide dose at 0, 4, and 8 hours after the ifosfamide infusion. Courses were repeated every 3-4 weeks. RESULTS: Among the 22 evaluable patients, 8 (36.5%) had a partial and one (4.5%), a complete response, giving a response rate of 46%. Three more patients had responses that were less than a partial remission, and 6 patients showed disease stabilisation on chemotherapy. Toxicities were tolerable and consisted mainly of myelosuppression. Results were further analysed in relation to pathologic subtype, disease status at the start of chemotherapy, and the delivered dose intensity. No relationship was found between any of these parameters and response to therapy. CONCLUSION: Advanced bilharzial bladder cancer is relatively sensitive to combination chemotherapy, but complete remission and prolonged survival is rare in this subgroup of patients with advanced disease. Further studies will be needed to determine the relative efficacy of single agents and drug combinations.

Adult↗

PubMed1996

Factors influencing the distribution of metastases and survival in metastatic breast carcinoma.

A total of 370 patients with metastatic breast, carcinoma who had been followed at Hacettepe Oncology Department between 1980 and 1991 were retrospectively analyzed for the factors influencing the distribution of metastases and survival. Median age was 47 years. Radical or modified radical mastectomies were performed in 199 (53.8%). Infiltrative ductal carcinoma was the most common pathologic subtype (69.4%). In 191 patients who were evaluated for estrogen receptor (ER) status, 101 (52.9%) were positive and 90 (47.1%) were negative. The distribution of first metastases did not differ between the soft tissue, bone, and visceral sites. The second, third, and fourth metastases were more common in visceral sites (p < 0.05). ER and menopausal status did not affect distribution. Mortality rate was significantly lower in the group having the first metastasis to the bone (p < 0.05). Of interest, first metastases were predominantly found in visceral sites in patients having radical or modified radical mastectomies (p < 0.05). Response to therapy, presence of initial metastases, axillary status, and age were the important factors influencing the overall survival in univariate analysis, whereas response to therapy, ER status, age, and presence of initial metastases were the important factors according to the multivariate analysis.

Adult↗

PubMed1991

Radiotherapy in breast cancer.

This review focuses on four aspects of radiotherapy in the treatment of breast cancer. The most controversial area is the treatment of ductal carcinoma in situ. Recent studies exploring breast-conserving surgery with and without radiation are outlined. Although no consensus exists on the recent studies, the emergence of ductal carcinoma in situ as a cancer with both significant pathologic subtypes and significant differences in clinical presentation is clear. Concerning the role of radiation in invasive breast cancer, all reports indicate continuation of the earlier observed success. Predictors for recurrence, the diagnosis of recurrence, and the relationship of an apparent isolated local recurrence with the eventual development of distant metastatic disease are reviewed. The outcome of treatment with breast-conserving surgery in radiation in unusual situations is also presented, including patients with silicone prostheses, those presenting with an axillary mass and occult breast disease, and those with multiple primary tumors in the same breast. The clinical evidence continues to support radiation delivered after mastectomy in patients who are at high risk for locoregional recurrence. Systemic adjuvant chemotherapy alone does not appear to prevent many of these recurrences. With the improved local control following treatment with both adjuvant systemic therapy and comprehensive postoperative radiation, preliminary reports are also documenting advantages in terms of disease-free survival.

Breast Neoplasms↗

PubMed2001

Abnormal haemorheology, endothelial function and thrombogenesis in relation to hypertension in acute (ictus < 12 h) stroke patients: the West Birmingham Stroke Project.

While the blood vessels are exposed to high pressures in hypertension, the main complications of hypertension (stroke and myocardial infarction) are paradoxically thrombotic rather than haemorrhagic. To investigate abnormalities of haemorheology (plasma viscosity, fibrinogen), endothelial dysfunction (von Willebrand factor), platelet activation (soluble P-selectin) and thrombogenesis (plasminogen activator inhibitor and fibrin D-dimer) in stroke and the effects of concurrent hypertension, we studied 86 consecutive patients (58 male, 28 female) aged < 75 years (mean age +/- SD, 64.2 +/- 9.2 years) with acute stroke (ictus < 12 h). Baseline blood tests on admission were compared with 46 'hospital controls' (patients with uncomplicated essential hypertension; mean age +/- SD, 65.9 +/- 3.8 years) and 24 healthy normotensive controls (mean age +/- SD, 65 +/- 14.0 years). Further comparisons were made between stroke patients with hypertension (systolic blood pressure > 160 mmHg and/or diastolic > 90 mmHg) on admission and those without hypertension. Mean plasma viscosity (one-way analysis of variance, P = 0.026) and fibrinogen levels (P = 0.016) were significantly higher in stroke patients and hospital controls, when compared with healthy controls. The von Willebrand factor, plasminogen activator inhibitor soluble P-selectin and fibrin D-dimer levels were highest in the acute stroke patients, intermediate in hospital controls and lowest in healthy controls (all P < or = 0.001). There were no significant differences in measured indices of haemorheology, endothelial dysfunction and thrombogenesis between the three stroke pathological subtypes (ischaemic/thrombotic, haemorrhagic or transient ischaemic attack). There were also no significant differences in the measured parameters for stroke patients with or without systolic blood pressure > 160 mmHg or diastolic blood pressures > 90 mmHg using clinical (manual) readings or mean daytime or night-time ambulatory blood pressure monitoring recordings. There were no statistically significant differences between the measured parameters on admission and at 3 months follow-up in 26 patients (all P = not significant). Plasma viscosity was significantly correlated with mean daytime systolic blood pressure (r = 0.314, P = 0.021) and mean night-time systolic blood pressure (r = 0.309, P = 0.025). This study of hypertension and haemostasis in acute stroke has demonstrated clear abnormalities of haemorheology, endothelial dysfunction, platelet activation and thrombogenesis, which do not appear to be affected by the height of the blood pressure or the presence of hypertension. This is despite the known hypercoagulable state found in hypertension and the relationship of haemostatic abnormalities to vascular complications.

Aged↗

PubMedDate not supplied

Castleman disease of the abdomen: imaging spectrum and clinicopathologic correlations.

PURPOSE: The purpose of this work was to define the imaging spectrum of Castleman disease of the abdomen and to correlate the results with clinicopathologic findings. METHOD: Seventeen patients (male/female 7:10; mean age 35.6 years) with pathologically proved Castleman disease in the abdomen were included in this study. Radiologic findings (CT, n =17; US, n =10; MR, n =1) were retrospectively reviewed by two readers and were correlated with clinical and pathologic findings. RESULTS: Subjects were divided into those with localized (n = 11) and disseminated (n = 6) disease. In localized disease, the pathologic subtypes were hyaline vascular type in eight and plasma cell type in three. Radiologic studies showed a single large mass in six and a single dominant mass with small satellite nodules in five. Central low attenuation was seen in two cases, and calcification was seen in three cases. Regional lymphadenopathy was found in five cases. In disseminated disease, there were three hyaline vascular types and three plasma cell types. Radiologic findings included diffuse lymphadenopathy (n = 6), hepatosplenomegaly (n = 5), ascites (n = 3), and thickening of the retroperitoneal fascia (n = 3). Disseminated disease revealed symptoms and abnormal laboratory findings (anemia, hypoalbuminemia, elevated erythrocyte sedimentation rate/C-reactive protein, etc.) more frequently than localized disease and showed poor prognosis. CONCLUSION: There are two distinctive types of radiologic manifestations in Castleman disease of the abdomen: localized and disseminated. The localized type usually shows single or multiple discrete masses, and the disseminated type frequently shows nonspecific organomegaly and lymphadenopathy.

Abdomen↗

PubMed2002

Familial medulloblastoma: case report of one family and review of the literature.

OBJECTIVE AND IMPORTANCE: Medulloblastoma is the most common malignant brain tumor and the most common malignant solid tumor in children. Most medulloblastomas are sporadic, but rare familial forms have been described. To the best of our knowledge, only 10 case reports of familial medulloblastoma have been published. A variety of candidate genes have been suggested to be involved in familial medulloblastomas. However, the exact pathogenesis and genetics involved in familial medulloblastoma remain unknown. CLINICAL PRESENTATION: We describe the presentation of medulloblastoma in two siblings (one of each sex) and their great-uncle. The three cases differ with regard to age at onset and pathological subtype of medulloblastoma. INTERVENTION OR TECHNIQUE: Immunostaining of tissue blocks for gene products involved in medulloblastoma differed in the two siblings for beta-catenin and was similar with staining for gli. CONCLUSION: This article is only the second report in the literature to address the genetics of familial medulloblastoma in the absence of characterized conditions such as Li-Fraumeni's cancer syndrome and basal cell nevus, Rubinstein-Taybi's, and Turcot's syndromes. The discrepancy in beta-catenin staining in the two siblings suggests that the two tumors differentiated through divergent pathways. We briefly summarize all published cases of familial medulloblastoma and review the literature on the genes involved in medulloblastoma formation.

Adult↗

PubMed1982

The reliability of noninvasive diagnostic procedures in children with brain tumors.

To determine the most reliable method of following the progress of disease in children with primary intracranial neoplasms, the noninvasive diagnostic procedures in 92 children were analyzed. Computerized tomographic (CT) scanning was found to be significantly superior to all other testing procedures in sensitivity, specificity, and overall accuracy. This superiority was seen regardless of tumor location or pathological subtype. When results in follow-up were compared to initial findings at diagnosis, the sensitivity of CT scanning was again significantly more reliable than the other procedures. Cerebrospinal fluid analysis was unreliable and added little information to that obtained from simultaneous CT and Radionuclide (RN) scanning. It is recommended that combined CT and RN scanning be used for management of intracranial neoplasms until a reliable method for detecting microscopic disease is available.

Adolescent↗

PubMed2005

Comparison of presentation and outcome for patients 18 to 40 and 60 to 70 years old with solid renal masses.

PURPOSE: We compared histological subtype, pathological features and outcome of patients with solid renal masses who were 18 to 40 years old vs patients who were 60 to 70 years old. MATERIALS AND METHODS: We conducted a retrospective review of the Mayo Clinic Nephrectomy Registry from 1970 to 2000, and identified 124 patients 18 to 40 years old and 1,067 patients 60 to 70 years old available for analysis. RESULTS: There was no significant difference in the incidence of benign solid renal masses between patients 18 to 40 years old and those 60 to 70 years old (13.7% vs 10.2%). Among patients with renal cell carcinoma (RCC), younger patients were more likely to have chromophobe RCC (13.1% vs 3.6%) and less likely to have clear cell RCC (70.1% vs 81.5%) than older patients. Among patients with clear cell RCC, younger patients were more likely to have stage pT2b or lower tumors (82.7% vs 69.9%) and a higher incidence of cystic clear cell RCC (10.7% vs 2.2%) than older patients. Younger patients had an improved cancer specific survival compared with older patients but this difference was not statistically significant (risk ratio 0.71, p =0.127). CONCLUSIONS: We found that patients 18 to 40 years old were more likely to have chromophobe and less likely to have clear cell RCC compared with patients 60 to 70 years old. We did not identify a higher incidence of papillary RCC in younger patients. Patients with clear cell RCC 18 to 40 years old had a higher incidence of low stage and cystic tumors compared with patients 60 to 70 years old, features which have been shown to have a favorable prognosis. These factors likely contributed to improved cancer specific survival for younger patients.

Adenocarcinoma, Clear Cell↗