Background Perianal/perineal rhabdomyosarcomas (PRMS) are easily misdiagnosed soft tissue tumours with a poor prognosis. This study was designed to analyze the clinical, diagnostic, pathological and prognostic features of PRMS, and to explore currently available therapeutic modalities. Methods Clinical data of PRMS patients admitted to the Sixth Affiliated Hospital and the Cancer Center of Sun Yat-sen University and from related Chinese literature published from 1987 to 2018 were collected and analyzed. The Chi-square test was used to evaluate the differences between each group. The Kaplan–Meier methods were applied to estimate and compare survival rates. Results A total of 35 patients were included in this study; 20 identified within related Chinese literatures and 15 from our center admitted during the period of 1997–2019. Out of these cases, 34 presented with perianal masses and the remaining one manifested as an inguinal mass. Moreover, 20 patients complained of pain and 16 of them were misdiagnosed as perianal abscesses, in which the presence of pain contributed to the misdiagnosis (p < 0.05). The average time interval between symptom onset and pathological diagnosis was 3.1 months. Next, 13 cases were classified into IRS group III/IV and 20 cases into stages 3/4. Additionally, 14 and 9 cases received the pathological diagnoses of embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma, respectively. Regarding the patients’ survival rates, five patients survived for more than 2 years, and three of them survived for more than 5 years. The overall 2 years and 5 years survival rates were 32% and 24%, respectively. The symptom of pain and misdiagnosis both contributed to the poor prognosis in these patients (p < 0.05). MRI showed that the PRMS were closely related to external anal sphincter in 10 cases. Conclusion PRMS are easily misdiagnosed lesions, which often leads to an unfavourable outcome in affected patients. Patients with painful perianal masses should be evaluated to exclude PRMS. MRI revealed that PRMS are closely related to the external anal sphincter. Multidisciplinary management is recommended in the treatment of PRMS.
BackgroundPerianal/perineal rhabdomyosarcoma (PRMS) is rare, easy to be misdiagnosed and has poor prognosis. This study was designed to analyze the clinical, diagnostic, pathological and prognostic features of PRMS, and to explore its therapeutic modality.MethodsClinical data of PRMS patients in the Sixth Affiliated Hospital and the Cancer Center of Sun Yat-sen University and related Chinese literatures published between 1987 and 2018 were described and analyzed. Chi-square test was used to test the differences between groups. The Kaplan-Meier methods were used to estimate and compare survival rates.Results35 patients, 20 identified in the Chinese literatures and 15 of our center from 1997 to 2019, were included in this study. 34 cases presented as perianal mass and one presented as inguinal mass. 20 patients complained about pain and 16 cases were misdiagnosed as perianal abscess, in which pain was found related to misdiagnosis (p<0.05). The average time from symptoms to pathological diagnosis was 3.1 months. 13 cases were categorized into IRS group III/IV and 20 cases into stages 3/4. 14 Cases were diagnosed as embryonal rhabdomyosarcoma pathologically and 9 as alveolar rhabdomyosarcoma. Five patients survived for more than two years, three of them survived for more than five years. The overall survival rates of two years and five years were 32% and 24% respectively, in which misdiagnosis contributes to poor prognosis (p<0.05). MRI showed that PRMS was closely related to external anal sphincter in 10 cases. ConclusionPRMS is easy to be misdiagnosed, which could lead to worse outcome. Patients with painful perianal mass should be evaluated to exclude PRMS. MRI shows PRMS is closely related to external anal sphincter. Comprehensive therapy including radical operation and chemotherapy is recommended.
Purpose: Perianal/perineal rhabdomyosarcoma (PRMS) is rare, easy to be misdiagnosed and has poor prognosis. This study was designed to analyze the clinical, diagnostic, pathological and prognostic features of PRMS, and to explore its therapeutic modality.Methods: Clinical data of PRMS patients in the Sixth Affiliated Hospital and the Cancer Center of Sun Yat-sen University and related Chinese literatures published between 1987 and 2018 were described and analyzed. Chi-square test was used to test the differences between groups. The Kaplan-Meier methods were used to estimate and compare survival rates.Results: 35 patients, 20 identified in the Chinese literatures and 15 of our center from 1997 to 2019, were included in this study. 34 cases presented as perianal mass and one presented as inguinal mass. 20 patients complained about pain and 16 cases were misdiagnosed as perianal abscess, in which pain was found related to misdiagnosis (p<0.05). The average time from symptoms to pathological diagnosis was 3.1 months. 13 cases were categorized into IRS group III/IV and 20 cases into stages 3/4. 14 Cases were diagnosed as embryonal rhabdomyosarcoma pathologically and 9 as alveolar rhabdomyosarcoma. Five patients survived for more than two years, three of them survived for more than five years. The overall survival rates of two years and five years were 32% and 24% respectively, in which misdiagnosis contributes to poor prognosis (p<0.05). MRI showed that PRMS was closely related to external anal sphincter in 10 cases. Conclusion: PRMS is easy to be misdiagnosed, which could lead to worse outcome. Patients with painful perianal mass should be evaluated to exclude PRMS. MRI shows PRMS is closely related to external anal sphincter. Comprehensive therapy including radical operation and chemotherapy is recommended.
BackgroundNeoadjuvant Chemoradiotherapy (nCRT) is a widely accepted regimen for patients with locally advanced rectal cancer (LARC). This compared the long-term prognosis and postoperative quality of life (QoL) between patients with low-lying LARC receiving nCRT and primary surgery.MethodPatients underwent nCRT or primary surgery for low-lying LARC between 2010 and 2016 were identified. Five-year local recurrence (LR) and disease free survival (DFS) were compared between groups. Quality of life (QoL) of patients who were disease-free was investigated using European Organization for Research and Treatment of Cancer QoL questionnaire core-30 (EORTC QLQ-C30) and QLQ-Colorectal Cancer module (CR29).ResultsA total of 304 patients were included in this study. Differences in 5-year LR and DFS between groups showed no statistical significance. In terms of QoL, apart from less stoma care problem, nCRT patients showed unsatisfactory social function and worse symptoms including diarrhoea, financial difficulties, buttock pain, fecal incontinence, embarrassment and impotence compared with primary surgery group. Intergroup analysis indicated that the QoL of patients receiving nCRT with preserved sphincter was relatively inferior compared with other subgroups, as reflected in higher symptom scores including financial difficulties and those related to low anterior resection syndrome (LARS) such as diarrhea, stool frequency, flatulence and fecal incontinence.ConclusionFor patients with low-lying LARC, nCRT has no advantage in terms of 5-year survival and QoL. NCRT with sphincter preserving surgery should be conducted meticulously considering its limited benefits for patients.
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