key: cord-0974682-a037ymxv authors: Ginting, Jeremy Thompson; Sigumonrong, Yacobda title: Abol-Enein pouch modification after radical cystectomy in bladder rhabdomyosarcoma in 5 years old child during COVID-19 pandemic: A case report date: 2021-12-20 journal: Int J Surg Case Rep DOI: 10.1016/j.ijscr.2021.106701 sha: e11de68920b1cde217840a68c3e71ea1549c23d7 doc_id: 974682 cord_uid: a037ymxv OBJECTIVE: To discuss the consideration of performing radical cystectomy for rhabdomyosarcoma in children during the COVID-19 pandemic in the urology department of Adam Malik Hospital, Medan. INTRODUCTION: Rhabdomyosarcoma is a rare malignancy that develops from primitive mesenchymal stem cells. The gold standard for the treatment of rhabdomyosarcoma is radical cystectomy. However, during the COVID-19 pandemic, radical cystectomy becomes a concern due to the risk of virus transmission. This article reported a patient who underwent radical cystectomy during the COVID-19 pandemic. CASE PRESENTATION: A five-year-old female child was admitted to the hospital with chief complaints of bloody urine 1 month before admission. A month before hospital admission, the patient had pain during urination and was treated with radical cystectomy with Abol-Enein pouch modification. RESULT: After the surgery, the patient was able to urinate without any disturbance. Hematuria was not found. There was no complaint related to stricture of the ureter after the surgery. CONCLUSION: In our center, radical cystectomy could be performed in child with bladder rhabdomyosarcoma during COVID-19 pandemic. The procedure is considered a high priority therapy in most regions during COVID-19 pandemic. Abol-Enein technique after radical cystectomy resulted in improvement of symptom with no reported complication in our case. The tumor develops from primitive mesenchymal cells which should undergo differentiation into skeletal muscle. 1 The distribution of cases is high during first decade and adolescence. Rhabodmyosarcoma is diagnosed in 4,5 cases/ million children/ adolescents every year. 2 Urogenitary system rhabdomyosarcoma was found in 15 -20% of all RMS. 3 The age distribution of RMS follows bimodal distribution with the first peak occurs in the age of 0-5 years and the second smaller peak occurs in adolescence. There are two major subtypes of RMS which are embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS). ERMS is diagnosed earlier before 10 years of age with peak incidence between 0 -4 years of age (42%) while ARMS is diagnosed equally in 0 -19 years of age. 4 Between the year of 1975 and 2005, rhabdomyosarcoma were diagnosed in 987 children aged 0-19 years. The majority of subtypes found were ERMS (57%) and ARMS (23%). Other less common subtypes found were embryonal sarcoma (2%), pleomorphic (1,5%), mixed type (1,4%), and spindle cell (0,6%). 4 Radical cystectomy is a gold standard for therapy of rhabdomyosarcoma. The timing of the procedure determined the prognosis and survival of patients with rhabdomyosarcoma. 5 According to Sharma, radical cystectomy in high risk cancer is a procedure not to be cancelled during COVID-19 pandemic while radical cystectomy in low risk cancer is optional or secondary to cancellation. 6 BAUS guidelines reported that low risk patient needing radical cystectomy could be in secondary cancellation while high risk patient is last to be cancelled. 7 During COVID-19 pandemic, radical cystectomy had been performed in several center in several countries such as Turkey and Europe. Following radical cystectomy, bladder substitution is conducted. Abol-Enein and Ghoneim performed an implantation of ureters into a detubularized bowel segment reservoir. The ureters were placed between the layer of tubularizing folds so that the serous-lined extramural tunnel was formed. The intraluminal pressure prevent reflux. The procedure could be performed for normal size ureter and grossly dilated and thick-walled ureter. Besides, the alternative method for neobladder creation was the I-pouch. However, the using of the procedure in a pediatric population has not been reported. 8 This article report a female patient, 5 years old with rhabdomyosarcoma of the bladder who underwent radical cystectomy. This case report was written according to SCARE guideline. 9 J o u r n a l P r e -p r o o f Journal Pre-proof A female child 5 years of age admitted to the hospital with chief complaint of blood in urine 1 month before admission. There was loss of weight up to 5 kg in the last 1 month. The patient was diagnosed with bladder rhabdomyosarcoma T3N3M0. On the laboratory examination, the hemoglobin was 10,6 g/dL, leukocytes were 9300 cells/µL and thrombocytes was 628.000 cells/µL. Serum ureum was 32 mg/dL and serum creatinine was 0.42 mg/dl. Serum electrolytes were in normal limit. Serum albumin was 3.0 mg/dl. The production of conduit was 11000cc/24 hours. The patient lived with her parents. Her father was a heavy smoker and her parents often used pesticide in the garden near their house. The patient underwent radical cystectomy with Abol-Enein pouch modification. The ileal conduit was performed after radical cystectomy. Figure 1 -4 demonstrated the procedure of radical cystectomy with Abol-Enein pouch modification. After the surgery, the pain improved. There was no macroscopic and microscopic hematuria reported. No stenosis of the ureters was found. Figure 5 and 6 showed the postoperative abdominal physical and radiological examination. The patient was stable and discharged with no complication. 1 month after the surgery, the patient came to the hospital for follow-up examination and no complication was reported. Rhabdomyosarcoma is the majority of soft-tissue sarcoma found among children. It accounts for 5% of all childhood cancers. 1 The finding of rhabdomyosarcoma in children is rare. 1 It represents about 3% of all childhood cancers. Rhabdomyosarcoma mostly occurs in children and teens. 10 Most rhabdomyosarcoma (87%) occurs in the patients younger than 15 years. 11 The incidence was slightly higher in male compared with female. 10 The rhabdomyosarcoma was classified into two main types which are embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma (ARMS). 10 Genitourinary rhabdomyosarcoma represents approximately 25% of all rhabdomyosarcoma. 11 The development of rhabdomyosarcoma is sporadic which could be affected by several risk factors. Several cases of rhabdomyosarcoma had familial predisposition such as Li-Fraumeni syndrome and neurofibromatosis. There are still few studies exploring the relationship between several risk factors including pre-conceptional use of recreational drugs, prenatal X-ray exposure, maternal history of stillbirths, advanced maternal age at childbirth, high birthweight, parity, and the risk of rhabdomyosarcoma in children. 12 Grufferman S et al stated that fathers' cigarette smoking was associated with childhood rhabdomyosarcoma with relative J o u r n a l P r e -p r o o f risk of 3.9. 13 In this report, we found that the patient's father was a heavy smoker. This could be risk factor of childhood rhabdomyosarcoma in our patient. The common clinical presentation of bladder rhabdomyosarcoma was hematuria. In our patient we found gross hematuria. Priyadarashi also reported that microscopic hematuria was found in bladder rhabdomyosarcoma. 11 Other clinical manifestations that could present include straining during voiding, sense of incomplete voiding, weak stream urine, suprapubic tenderness, and dribbling of urine. 11 Our patient also had pain related to the tumor. Radical cystectomy remains the gold standard for treatment of bladder cancer. The timing of the procedure is essential since reduction in overall survival and in progression-free survival was observed following a 90-delay in conducting radical cystectomy. 5 hospitalization time of the patients was 11 days followed by discharged with full recovery. 12 Soytas M et al also performed radical cystectomy for 2 patients. 13 of rhabdomyosarcoma is cystectomy continued by reconstructive surgery. 15 According to survey of urologists in Europe, the radical cystectomy was included on the priority list higher than expected rates. The rate of radical cystectomy postponement was lower than expected rates. 7 Overall the priority rank of radical cystectomy in 6 regions (Africa, East/ South East Asia, Europe, North America, South America, West/ South West Asia) was 8 from the scale of 1 (lowest priority) to 10 (highest priority). Different region had different priority rank of radical cystectomy. Radical cystectomy was ranked 9 in Europe. In Africa, East/ South East Asia, North America, and South America, the procedure was ranked 8 while in the West/ South West Asia, the procedure was ranked 6. 7 following the procedure. 15 In our patient, we did not find stenosis of the ureters within the follow-up period of 1 month. Patients have a right to privacy. Patients' and volunteers' names, initials, or hospital numbers should not be used. Images of patients or volunteers should not be used unless the information is essential for scientific purposes and explicit permission has been given as part of the consent. If such consent is made subject to any conditions, the Editor in Chief must be made aware of all such conditions. Even where consent has been given, identifying details should be omitted if they are not essential. If identifying characteristics are altered to protect anonymity, such as in genetic pedigrees, authors should provide assurance that alterations do not distort scientific meaning and editors should so note. The patient and her parents have given their consent in order for us to publish this case Please specify the contribution of each author to the paper, e.g. study concept or design, data collection, data analysis or interpretation, writing the paper, others, who have contributed in other ways, should be listed as contributors. BJM carried out the data collection, analyzing the data and and drafted the manuscript. YS participated in the design of the study and helped to draft the manuscript. All authors have read and approved the manuscript 3. Hyperlink to your specific registration (must be publicly accessible and will be checked): Rhabdomyosarcoma in pediatric patients: The good, the bad and the unusual Cancer Incidence and Survival among Children and Adolescents. United States SEER Progr Current treatment of pediatric bladder and prostate rhabdomyosarcoma (bladder preserving vs. radical cystectomy) Trends in Childhood RMS Incidence and Survival in the US Determining the optimal time for radical cystectomy after neoadjuvant chemotherapy Urological surgery in the time of coronavirus pandemic Prioritising Urological Surgery in the COVID-19 Era: A Global Reflection on Guidelines Advances in bladder substitution and creation of neobladders in children The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines About Rhabdomyosarcoma What Is Rhabdomyosarcoma ? Cancer Facts Fig Rhabdomyosarcoma of the Urinary Bladder Association of Urology Guidelines Office Rapid Reaction Group: An Organisation-wide Collaborative Effort to Adapt the European Association of Urology Guidelines Recommendations to the Coronavirus Disease Analysis of patients undergoing urological intervention amid the COVID-19: experience from the pandemic hospital Adapting Management of Sarcomas in COVID-19: An Evidence-Based Review Reconstructive option after radical mutilating surgery in children with genitourinary rhabdomyosarcoma: When sparing the bladder is not an option Impact of the COVID-19 pandemic on the urologist's clinical practice in Brazil: A management guideline proposal for low-and middle-income countries during the crisis period A novel uretero-ileal reimplantation technique: The serous lined extramural tunnel. A preliminary report Serous lined extramural ileal valve: A new continent urinary outlet The authors would like to thank everyone who supported this study. Special thanks are given to Adam Malik General Hospital, Universitas Sumatera Utara and Universitas Indonesia which fully supported the authors during writing period. The following information is required for submission. Please note that failure to respond to these questions/statements will mean your submission will be returned. If you have nothing to declare in any of these categories, then this should be stated. Please state any conflicts of interest All authors must disclose any financial and personal relationships with other people or organisations that could inappropriately influence (bias) their work. 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