Long-term outcomes for desmoid tumors treated with radiation therapy

PURPOSE: To evaluate long-term outcomes in patients with desmoid fibromatosis treated with radiation therapy (RT), with or without surgery. METHODS AND MATERIALS: Between 1965 and 2005, 115 patients with desmoid tumors were treated with RT at our institution. The median age was 29 years (range, 8-73 years). Of the patients, 41 (36%) received RT alone (median dose, 56 Gy) for gross disease, and 74 (64%) received combined-modality treatment (CMT) consisting of a combination of surgery and RT (median dose, 50.4 Gy). RESULTS: Median follow-up was 10.1 years. Local control (LC) rates at 5 and 10 years were 75% and 74%, respectively. On univariate analysis, LC was significantly influenced by tumor size (< or =5 cm vs. 5-10 cm vs. >10 cm) (p = 0.02) and age (< or = 30 vs. >30 years) (p = 0.02). There was no significant difference in LC for patients treated with RT alone for gross disease vs. CMT. For patients treated with CMT, only tumor size significantly influenced LC (p = 0.02). Patients with positive margins after surgery did not have poorer LC than those with negative margins (p = 0.38). Radiation-related complications occurred in 20 (17%) of patients and were associated with dose >56 Gy (p = 0.001), age < or =30 years (p = 0.009), and receipt of RT alone vs. CMT (p = 0.01). CONCLUSIONS: Desmoid tumors are effectively controlled with RT administered either as an adjuvant to surgery when resection margins are positive or alone for gross disease when surgical resection is not feasible. Doses >56 Gy may not be necessary to control gross disease and are associated with high rates of radiation-related complications.     

Duchenne muscular dystrophy--a molecular service.

In 1987 a carrier detection and prenatal diagnostic service for Duchenne muscular dystrophy using molecular technology was instituted at the Department of Human Genetics, University of Cape Town, to serve affected families in southern Africa. DNA samples from 100 affected male subjects and 350 of their relatives from a total of 110 families have been banked. To date restriction fragment length polymorphism (RFLP) analysis and deletion screening has been performed on the DNA of 60 male patients and 116 female relatives at risk of being carriers of the faulty gene. The DNA probes used were pERT 87-1 (MspI polymorphism) pERT 87-15, pXJ1.1, pXJ2.3 (TaqI, polymorphism), pXJ1.2 (BclI polymorphism), P20 (MspI and EcoRV polymorphism) and the cDNA probes. DNA deletions have been detected in 30 of the 60 affected boys and the carrier risks of 49 women have been determined by RFLP analyses. In those families where the risks were uncertain because the affected males had died, prenatal exclusion testing was offered to potential carriers. Two pregnancies were terminated when male fetuses were shown to be affected, since they had the same deletion as that observed in the proband.     

Correlates of aortic root size in a population-based screening by hand-held echocardiography: "The Heart in the City" Study

Our experience underscores the ability of hand-held echocardiography to identify the association between aortic root dilation and left ventricular mass in a population-based screening among normotensive and hypertensive participants of "The Heart in the City", a project designed to detect pre-clinical echocardiographic markers of heart failure by hand-held echocardiography.     

Development of an adaptive tsetse population management scheme for the Luke community, Ethiopia

Since 1996, tsetse (Glossina spp.) control operations, using odor-baited traps, have been carried out in the Luke area of Gurage zone, southwestern Ethiopia. Glossina morsitans submorsitans Newstead was identified as the dominant species in the area, but the presence of Glossina fuscipes Newstead and Glossina pallidipes Austen also was recorded. Here, we refer to the combined number of these three species and report the work undertaken from October 2002 to October 2004 to render the control system more efficient by reducing the number of traps used and maintaining the previously reached levels of tsetse occurrence and trypanosomiasis prevalence. This was done by the design and implementation of an adaptive tsetse population management system. It consists first of an efficient community-participatory monitoring scheme that allowed us to reduce the number of traps used from 216 to 127 (107 monitoring traps and 20 control traps). Geostatistical methods, including kriging and mapping, furthermore allowed identification and monitoring of the spatiotemporal dynamics of patches with increased fly densities, referred to as hot spots. To respond to hot spots, the Luke community was advised and assisted in control trap deployment. Adaptive management was shown to be more efficient than the previously used mass trapping system. In that context, trap numbers could be reduced substantially, at the same time maintaining previously achieved levels of tsetse occurrences and disease prevalence.     

CYP3A5 genotypes and risk of oesophageal cancer in two South African populations

CYP3A5 is the major cytochrome P450 enzyme in the oesophagus and metabolises many potentially carcinogenic compounds. The frequencies of CYP3A5 allelic variants, CYP3A5*2, *3, *6 and *7 which code for enzymes with severely decreased activities were compared between 241 oesophageal cancer patients and 272 controls in Black and Mixed Ancestry South Africans. A significantly higher frequency of CYP3A5*3 was observed in the controls compared to patients amongst the Mixed Ancestry group (P=0.025). Individuals homozygous for defective CYP3A5 had reduced risk of developing oesophageal cancer (P=0.032).     

Periosteal margin in soft-tissue sarcoma

BACKGROUND: Soft-tissue sarcomas frequently rest in contact with bone. The purpose of the study was to evaluate the risk of local recurrence for sarcomas adjacent to bone and to determine whether the periosteum provides an adequate margin of resection. METHODS: Fifty patients with soft-tissue sarcomas abutting bone were treated at a single institution between 1990 and 2004. All patients had high-grade, T2 (>5 cm), nonmetastatic disease in the lower extremity. Bone contact was verified by preoperative magnetic resonance imaging (MRI) and/or computed tomography (CT) scans. Forty-three of 50 patients received preoperative radiation with a mean dose of 50 Gy. In 11 cases a composite resection of bone and soft tissue was performed. In 39 cases the excision involved only soft tissue. RESULTS: True bone invasion was verified by histopathologic examination in 3 of 50 cases (6%). Local recurrence in the soft tissues developed in 8 of 50 (16%) patients. In no case did the recurrence involve destruction of cortical bone or erosion into bone. The recurrent tumor resided against the region of previous bone contact in 1 of 8 cases. There was no statistically significant difference in local recurrence between patients who had composite bone resection and patients who had soft-tissue resection only (P = .87). CONCLUSIONS: Relatively few sarcomas are able to penetrate cortical bone. Composite bone and soft-tissue resections are indicated primarily for frank bone invasion. In the absence of this, the periosteum is an adequate surgical margin for sarcomas treated with wide excision and radiation.