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BRIEF REPORT Cystoscopy Assisted Transvesical Biopsy of Prostatic Rhabdomyosarcoma
Abstract
The treatment of prostatic rhabdomyosarcoma (RMS) depends on tumour stratification based on site and histology. An increasing range of cytogenetic, molecular, and immunohistochemistry studies are required. This is difficult to achieve using standard cystoscopic biopsies alone. We present a 5-year-old male, diagnosed with a pro-static RMS. He underwent cystoscopy to confirm the diagnosis and at the same time tissue was obtained for histology using laparoscopic graspers via a STEP TM Port inserted percutaneously into the apex of his bladder. Histology and cytogenetics confirmed an embryonal botryoid RMS for which he received chemotherapy followed by a radical prostatectomy for residual disease. Pediatr Blood Cancer. 2010;55:583–585.
... Despite having vast experience with tumor resections in adults, MIS procedures are not prevalent in pediatric population. Early reports, have confined to lymph node dissection or diagnostic laparoscopy and tumor biopsy [7,11,12]. In 1994, Tan et al. described one of the earliest reports of laparoscopy assisted cystoprostatectomy for a recurrent bladder RMS [13]. ...
Bladder rhabdomyosarcoma (RMS) in children, at presentation are often large, situated in close proximity to vital structures. This makes the surgical resection challenging. Hence, chemoradiotherapy is often used first. For those tumors which are amenable for resection, laparoscopy can serve as a better alternative. A 5-year-old boy presented with lump in the abdomen. MRI revealed a solid tumor of size 4.8 × 4.3 × 5.3 cms arising from dome of bladder. Tru-cut biopsy of the tumor showed features of RMS, after which VAC (Vincristine, Actinomycin-D, Cyclophosphamide) chemotherapy was started. By the end of two cycles of chemotherapy, patient required hospital admission multiple times due to chemotherapy toxicity. Review imaging of the tumor showed >60% reduction in size. Laparoscopic bladder preserving partial cystectomy was done. Post-operatively patient was discharged after six days. During follow-up, patient had no urinary symptoms. The resected specimen had tumor cells at inferior margin. Adjuvant chemotherapy course was completed. Two years after the surgery, patient remains free of recurrence. Laparoscopy serves as a good alternative to open surgery for bladder RMS surgeries. Laparoscopic bladder preserving surgery can be done with good access, limiting the operative morbidity. Patients can benefit from smaller scars and speedy post-operative recovery.
Rhabdomyosarcoma (RMS) is the most common soft tissue tumor in children, and 15% to 20% arise from the genitourinary tract. Multicenter collaborative studies have improved survival substantially, and in addition to excellent oncologic control, current treatment focuses on organ preservation and minimization of late treatment effects. The multiple modalities needed to treat RMS dictate that treating physicians must be familiar with the disease as well as the goals and possible sequelae of treatment with chemotherapy, radiotherapy, and surgery. This article discusses the current standards of care for bladder and prostate RMS.
To evaluate transrectal ultrasound as a means of diagnosis and of monitoring patients with rhabdomyosarcoma involving the prostate.
Serial transrectal ultrasonography was utilized to evaluate prostatic rhabdomyosarcoma in three patients.
Unlike prostatic adenocarcinoma and transitional cell carcinoma involving the prostate, which are predominantly hypoechoic, the echogenicity of rhabdomyosarcoma is similar to that of the normal prostate. Transrectal ultrasound provided a simple means of monitoring prostate size and sampling tissue in these patients.
Transrectal ultrasound imaging can be useful in both diagnosis and evaluation of treatment response as well as provide easy access for biopsies in patients with rhabdomyosarcoma involving the prostate.
Minimally invasive surgery (MIS) is an ideal way to obtain biopsy specimens in children with cancer. We examined the safety, reliability and outcome of decisions made based on tissue obtained using MIS.
Fifty-nine oncology patients underwent 62 MIS procedures between January 1994 and July 1998. Complications, biopsy results, and outcomes were reviewed.
The study population comprised 32 boys and 27 girls, with an average age of 8.8 years. There were 47 thoracoscopic and 15 laparoscopic operations. Laparoscopic procedures included initial biopsy, determination of resectability, and second-look exam. Thoracoscopic procedures included 40 lung biopsies and seven biopsies/resections of mediastinal masses. Diagnostic accuracy was 100% in all cases. No patient was found retrospectively to have been inadequately treated based on decisions made from tissue obtained by MIS.
MIS is a safe and accurate means of obtaining tissue in pediatric oncology patients. Treatment decisions can be made accurately and with confidence using these techniques.
We describe a novel technique of laparoscopic transvesical cross-trigonal Cohen anti-reflux ureteroneocystostomy.
A 10, an 11 and a 32-year-old patient with symptomatic unilateral vesicoureteral reflux underwent laparoscopic cross-trigonal ureteral reimplantation. Two 5 mm. balloon tip ports were suprapubically inserted into the bladder. Using a transurethral resectoscope with a Collins knife a 4 to 5 cm. cross-trigonal submucosal trough was created from the refluxing ureteral orifice to the contralateral side of the bladder. The refluxing ureteral orifice and intramural ureter were completely mobilized intravesically, advanced transtrigonally and secured to the detrusor muscle at the apex of the trough with 3 deep interrupted sutures. The elevated mucosal flaps of the trough were suture approximated over the ureter to create a submucosal tunnel. All suturing was performed by freehand laparoscopic technique.
Operative time was between 2.5 and 4.5 hours and blood loss was 10 to 50 cc. Adequate submucosal trough creation, ureteral extravesical mobilization and intravesical advancement, and bladder mucosal flap reapproximation were done to create a submucosal tunnel in all cases. Satisfactory transtrigonal anchoring of the neoureteral orifice to the detrusor muscle and mucosa was achieved with 3 stitches. Hospital stay was 2, 2 and 1 days in the 3 cases, and the Foley catheter remained in place for 3, 1 and 1 week, respectively. At 6 months reflux had resolved in 2 patients, while in 1 grade II reflux persisted, which was improved from grade IV preoperatively. All patients have remained infection-free without antibiotics.
Laparoscopic transvesical cross-trigonal antireflux ureteral reimplantation is technically feasible. Intravesical laparoscopic suturing is possible. Potential advantages include a decreased hospital stay, decreased narcotic requirement and better cosmesis. Further experience is necessary to refine the technical nuances and evaluate outcomes compared to the open technique.
To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968.
From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5-18) had RMS of bladder/prostate or vagina. Twenty of the patients received primary polychemotherapy (vincristine, actinomycin D, cyclophosphamide, adriamycin, and more recently including etoposide and ifosfamide), two had primary surgery and seven had additional radiotherapy. Fourteen patients had radical cystoprostatectomy, with continent cutaneous urinary diversion with an ileocaecal pouch in seven, in one each a transverse colonic pouch, orthotopic ileocaecal bladder substitution, a rectal reservoir and rectosigmoid pouch and a colonic conduit diversion in two patients.
After a mean (range) follow-up of 8.6 (1.0-26) years, 17 patients had no evidence of disease. Five patients presenting initially with advanced tumour stages died from progressive RMS. Two patients with a continent urinary diversion required ureteric reimplantation for stenosis. In two patients severe bladder contraction after radiotherapy required bladder augmentation.
Primary chemotherapy followed by radical surgery of RMS of the prostate and/or bladder allows complete tumour resection in most cases, and yields excellent cure rates.
The aim of this study was to evaluate the advantages and complications of endosurgical procedures for benign and malignant pediatric solid tumors. Endosurgical techniques of biopsy and excision were used for diagnosis and treatment of solid tumors, respectively. Since July 1997, a total of 24 biopsies and 24 excisions have been performed laparoscopically for neuroblastoma ( n=24), ovarian solid tumors ( n=10) and other tumors. Seventeen biopsies and six excisions were performed for abdominal neuroblastoma, while ten excisions were performed for ovarian tumor. In these patients, the length of the operation, blood loss, time to start postoperative feeding, time to start postoperative chemotherapy and length of hospital stay were evaluated and compared to the those of the open surgery group. Furthermore, intra- and postoperative complications were analyzed in all patients of both groups. The length of the hospital stay and time to start postoperative feeding were significantly shorter in the group of patients who underwent endosurgical procedures for either abdominal neuroblastoma or ovarian tumor when compared to the open procedure group. The time to start postoperative chemotherapy was shorter only in the abdominal neuroblastoma group. The procedure for two patients undergoing endosurgical tumor excision had to be converted to open surgery due to large tumor size. Two weeks after thoracoscopic excision of a dumb bell-type neurofibroma, one patient underwent open repair of the dura mater because of leakage of cerebrospinal fluid. There were no port-site recurrences in any tumor types. Endosurgical procedures for solid tumors are effective and minimally invasive. However, better indicators are needed for their implementation in order to prevent complications and subsequent conversions to open procedures.
Age and androgens are key determinants of benign prostate hyperplasia, but the mechanisms remain unclear. We examine the relationship between androgens and total, central, and peripheral prostate volume with a focus on early life factors.
We conducted a cross-sectional observational study of 406 community-dwelling Australian men aged 20-82 yr old without known prostate disease. Prostate zonal (total, central, and peripheral) volumes were measured by planimetric transrectal ultrasound. Participants completed questionnaires, underwent physical examination, and provided blood samples to measure total, free, and bioavailable testosterone, dihydrotestosterone, estradiol, SHBG, LH, FSH, and prostate-specific antigen.
Prostate zonal volumes were positively associated with age, prostate-specific antigen, early onset of puberty, current height, body surface area, lean body mass, hip and waist circumference as well as recalled height and weight during puberty and adolescence but not current weight, fat mass, or body mass index. Stepwise multivariate regression modeling indicated that age and height were the only independent predictors of prostate zonal volumes. When adjusted for age and sampling time of day, the negative correlations of age-adjusted prostate zonal volumes with current blood total, free, and bioavailable testosterone and the positive correlation with blood SHBG were no longer significant.
This study suggests that early and long-term androgen exposure may have long-acting effects on mature prostate zonal volumes, whereas relationships with current blood androgens and related hormones levels were mostly a result of confounding by age. Additional studies on the mechanism of androgen effects on late-life prostate diseases should consider lasting effects of early-life androgen exposure.
Benign prostatic hyperplasia is common in aging men, but rare in childhood. We report a case of juvenile prostatic hyperplasia in a 10-year-old boy, who visited our clinic for hematuria and voiding symptoms. Radiologic studies revealed a huge prostate protruding into the bladder. The prostate was 33 g. The prostate biopsy showed no evidence of cancer. At 13 years of age, he was seen again because of general weakness, fever, and voiding difficulty. Bilateral hydroureteronephrosis was found on radiologic studies, and his serum creatinine was elevated. The prostate was 55 g. Suprapubic prostatectomy was performed.
Embryonal rhabdomyosarcoma (ERMS) is the most common subtype of RMS that predominantly involves the genitourinary tract and the head and neck regions in children younger than 10 years of age. Cytogenetically, ERMS is most frequently hyperdiploid, with extra copies of chromosomes 2, 7, 8, 11, 12, 13, and 20. No consistent structural chromosomal alteration has been identified in ERMS. In contrast, a t(2;13)(q35;q14) or t(1;13)(q36;q14) corresponding to PAX3-FOXO1A (previously FKHR) and PAX7-FOXO1A gene fusions are considered tumor-specific anomalies for alveolar RMS (ARMS). Occasionally, a recurrent secondary structural rearrangement involving chromosomes 1 and 16 is seen in translocation-positive ARMS, a der(16)t(1;16) resulting in an imbalance of 1q and 16q material. Conventional cytogenetic analysis of an ERMS arising in the urinary bladder of a 22-month-old male child revealed this nonrandom secondary chromosomal aberration, der(16)(1;16)(q22;q24), in a hyperdiploid complement with extra copies of chromosomes 2, 7, 8, 10, 12, 13, 19, and 20. Subsequent analyses showed tumor cells to be negative for FOXO1A, PAX3, or PAX7 gene locus rearrangements (by fluorescence in situ hybridization) and also negative for PAX3-FOXO1A and PAX7-FOXO1A fusion transcripts (by reverse transcriptase-polymerase chain reaction). These results suggest that the unbalanced t(1;16) translocation may be seen in RMSs lacking a primary genetic rearrangement.
The use of minimally invasive surgery (MIS) in pediatric cancer is a matter of debate. The diagnostic and ablative roles of MIS were evaluated in a consecutive series of children with malignancies.
A prospective study, including all patients, who underwent abdominal and thoracic surgery for confirmed or highly suspected pediatric cancer was performed from September, 2000, to December, 2005. An interdisciplinary panel approved the indication for minimally invasive or conventional surgery.
At a single institution, 301 operations were performed on 276 children with cancer. A minimally invasive approach was attempted in 90 of these patients (30%) and successfully employed in 69 (77%) of the operations. However, 21 operations (23%) were converted to an open approach. Regarding the abdominal operations attempted laparoscopically, 41 abdominal operations for biopsy or staging purposes were attempted laparoscopically (53%), but 6 were converted. In all, 139 abdominal resections were performed and 24 were attempted laparoscopically. Ten of these (42%) were converted. In 34 thoracic operations requiring biopsy, thoracoscopy was attempted in 14 (41%) and was successful in all but 1 (93%). Fifty-one thoracic tumors were resected and the thoracoscopic approach was attempted in 11 (22%) and successful in 7 (14%). Conversions from a minimally invasive operation to an open procedure occurred mainly due to limited visibility. Three bleeding complications occurred with 1 patient requiring a blood transfusion. In addition, 1 small bowel injury occurred with immediate laparoscopic closure. There were no port site recurrences after a median of 39 months.
MIS is a reliable diagnostic tool for pediatric abdominal and thoracic malignancy. The role of ablative MIS in pediatric cancer remains limited.
Over the last 2 decades, minimally invasive surgery (MIS) has become a significant tool for the diagnosis and treatment of malignant disease in adults. Despite initial reports of port-site metastases and peritoneal spread following laparoscopic resection of colorectal cancer in the 1990s, MIS is now commonly used for many applications in adult surgical oncology, including biopsy and resection of malignant disease in the chest and abdominal cavities, mediastinal and retroperitoneal lymph node dissection, staging of abdominal, pelvic and thoracic malignancies, and management of therapeutic complications. The use of MIS techniques in children is growing with the availability of smaller instruments and equipment more suitable to the pediatric patient. Herein, we review the role of MIS in the diagnosis, staging and treatment of malignant disease in children. We will also evaluate MIS as it applies to the palliation of disease and the management of treatment complications in childhood cancer.
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