Sirenomelia is a rare congenital malformation, characterised by abnormal development of caudal part of body with variable degree of fusion of lower limbs. VACTERL is an acronym used for a group of sporadic non-random birth defects involving multiple organ systems, namely vertebral (V), anal (A), cardiac (C), tracheoesophageal (TE), renal (R) and limb (L) defects. Combination of both the anomalies is very rarely reported in literature. Survival is extremely rare and early prenatal diagnosis may allow for termination of pregnancy. Here we present a case of sirenomelia phenotype, with a complete spectrum of autopsy findings, suggestive of VACTERL association.
Purpose/Objective(s): To compare quality of life (QOL) between two weeks intermediate course (IC) and long course (LC) preoperative radiation therapy for rectal cancer. Materials/Methods: Fourty seven patients with T3 rectal cancer were randomized to two weeks IC and LC preoperative radiation therapy in this prospective study. Initially, pelvic MRI, PET / CT in the treatment position were performed all patient. IC consisted of radiation therapy 30 Gy, 3 Gy/ fraction, with concurrent chronomodulated capecitabine 825 mg/m2/BID/daily. LC was 50.4 Gy, 1.8 Gy/fraction, with concurrent chronomodulated capecitabine 825 mg/m2/BID/Daily (based on Brunch Study). In both of arms surgery was performed in 8 to 12 weeks after chemoradiotherapy (CRT). Toxicity was evaluated according to the Radiation Therapy Oncology Group scoring system. QOL was measured using the EORTC QLQ-CR29. Assessments were performed at baseline and 3, 6 months after CRT in patients who had not relapsed. Evaluated response of therapy with PET/CT before surgery. Results: On acute side effect evaluation; skin (100% vs 68%, p:0,002), and haematological (28% vs 0%, p:0,007) have been seen more on LC CRT patients. On QOL; anal and urinary incontinence, body image, dysuria, urinary frequency and sexual desire; there hasn't been seen any important differences. In IC CRT patients erection (p:0,01) and dyspareunia (p:0,06) problems have been detected more than LC CRT patients. For both arms pathological complete response rates were detected 18% (4/22 vs 4/22 pts) and Sphincter-sparing surgery were similar for both arms (%86,3). Conclusion: LC and IC CRT in the local advanced rectum cancer treatment, has ensured similar results on quality of life evaluation, pathological complete response rates. In terms of acute side affects IC CRT was better than LC CRT. On these results IC CRT seems to be able to take the place of LC CRT.
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