Paratesticular Rhabdomyosarcoma Research Articles

Paratesticular Rhabdomyosarcoma: About 3 Cases and Literature Review

Paratesticular rhabdomyosarcoma is a rare and highly aggressive embryonal tumor in infancy and childhood. The tumor is intrascrotal, localized in the spermatic cord, the epididymis, or in the tunica vaginalis. Multimodality treatment should involve surgery, radiotherapy and chemotherapy, which are indicated according to risk groups. Prognosis is excellent for localized tumors, survival rates exceed 90%. We report three cases of embryonal paratesticular rhabdomyosarcoma treated in our institution.

Robotic retroperitoneal lymph node dissection for paratesticular rhabdomyosarcoma in adolescents: a case series

Robotic retroperitoneal lymph node dissection for paratesticular rhabdomyosarcoma in adolescents: a case series

Treatment results of Para-Testicular Rhabdomyosarcoma (PT-RMS) using radiation as an alternative to retro-peritoneal nodal dissection: A single Institution experience.

Para-testicular Rhabdomyosarcoma (PT-RMS) has a favorable treatment outcome adopting multidisciplinary management; resection, namely high inguinal orchiectomy ± retro-peritoneal lymph node dissection (RPLND) followed by standard or intensive chemotherapy ± adjuvant radiation therapy. This is a retrospective study including all patients with pathologically proven PT-RMS, presented to the National Cancer Institute, Cairo University, during the period from 2005 to 2020. Endpoints included overall survival, disease free survival and patterns of failure of different treatment modalities. Forty one patients were identified. Median age in our cohort was 15 years (range: 2-54 years). After a median follow up of 26 months (range, 3-75 months) ,two and five years OS were 100% and 91.7% respectively and median survival was not reached. Patients who underwent retro-peritoneal nodal dissection had a 5-year DFS rate of 100% versus 73% for those who received radiation to para-aortic nodes (p = 0.185). Limitations include retrospective nature and deviation from COG protocol. This study shows promising results suggesting that less aggressive local treatment modalities including radiation to para-aortic chain could be an option in PT-RMS, given the excellent results of this subtype. However further validation in a prospective study is warranted.

Primary paratesticular embryonal rhabdomyosarcoma - An unusual presentation.

Paratesticular embryonal rhabdomyosarcoma (RMS) is a very rare and aggressive mesenchymal tumor. It is usually seen in children and adolescents presenting as a painless intrascrotal mass, localized in the paratesticular region. Hereby, we report two cases of paratesticular embryonal RMS in adults. One case was clinically suspected to be a testicular abscess, whereas the other presented with testicular swelling and lung metastasis. Localized forms have a good prognosis, whereas tumors presenting with metastases show a poor outcome. A treatment based on surgery and chemotherapy yields good results. Sperm cryopreservation and endocrine follow-up improve the overall survival and quality of life of these patients.

Indocyanine green fluorescence-guided lymphadenectomy with single site retroperitoneoscopy in children

Retroperitoneoscopic lymphadenectomy is an established surgical approach in adult urology, but rarely described in pediatric population. We develop retroperitoneoscopic surgical oncology in children, combining new technology innovations in pediatric surgery such as single site port retroperitoneoscopic in supine position and indocyanine green (ICG). The video describes a step-by-step approach from the ICG injection technique to the lymph-node retroperitoneoscopic harvesting. The video highlights anatomical landmarks and ICG intraoperative lymph nodes findings. Four consecutive surgical procedures were performed in children with paratesticular rhabdomyosarcoma who required staging template retroperitoneal lymph node dissection (RPLND) for staging. All patients were discharged the same day without 30-days postoperative complications. Retroperitoneoscopic approach with single port and indocyanine guided lymphatic mapping for template retroperitoneal lymph node dissection (RPLND) is a feasible minimally invasive procedure in children. Combining different technology innovations allows an effective lymph node harvesting with the possibility to offer an enhanced recovery after surgery in pediatric oncology population.

Paratesticular rhabdomyosarcoma: a rare case report from Syria.

In this paper, the authors report a case of a 6-year-old child who presented to the clinic with a painless mass in the right side of the scrotum. The mass was misdiagnosed and had evolved rapidly over 2 weeks. The mass measured 16×32mm on ultrasound, and therefore, an orchiectomy was performed. The histological examination of the excised tissue confirmed the diagnosis of paratesticular RMS. Paratesticular RMS mainly presented as a painless mass in the scrotum. It was a very metastatic lesion that required an immediate management. However, a lot of cases of paratesticular RMS misdiagnosed at first time, which worsens the overall prognosis. Eventually, paratesticular RMS should be always taken into consideration when a scrotal mass is suspected. Due to its extremely serious metastatic potential, this condition requires early diagnosis and management. The treatment is currently well codified combining surgery, chemotherapy, and radiotherapy.

Paratesticular rhabdomyosarcoma: A systematic review and analysis of patient characteristics and treatment outcome of 191 patients

Paratesticular rhabdomyosarcoma: A systematic review and analysis of patient characteristics and treatment outcome of 191 patients

Surgical resection of a massive residual retroperitoneal mass after chemotherapy for a paratesticular rhabdomyosarcoma: a case report

IntroductionParatesticular rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, accounting for only 7% of all rhabdomyosarcomas. It is mainly encountered in children and adolescents. The standard treatment consists of radical orchidectomy with negative surgical margins. However, chemotherapy is recommended to control retroperitoneal micrometastasis. The place of surgery for progressive retroperitoneal lymph node metastases remains controversial. We present a case of paratesticular rhabdomyosarcoma with progressive retroperitoneal lymph node metastases treated with surgery.Case reportWe report a case of a 17-year-old North African male with no particular medical history who presented with a left scrotal mass that had been evolving for several months. Beta-human chorionic gonadotropin, alpha-fetoprotein, and lactate dehydrogenase were normal. Scrotal ultrasonography revealed the presence of a 6 cm heterogeneous hypoechogenic tissular mass with cystic areas adherent to the left scrotal wall, which was thickened in some places and vascularized by color Doppler. It exerted a mass effect on the homolateral testicle, which was of average volume. The thoracic–abdominal–pelvic computed tomography scan showed the presence of suspicious paraaortic lymph nodes. The most voluminous one measured 16 × 23 mm2. A left orchidectomy was performed. The final pathology report revealed an 8 cm paratesticular rhabdomyosarcoma of the embryonic type that displaced the testicle without invading it. Without going beyond it, it infiltrated the epididymis, the rete testis, and the albuginea. The surgical margin at the level of the spermatic cord was free. The patient had adjuvant chemotherapy (ifosfamide, vincristine, and dactinomycin). The patient had a challenging paraaortic lymph node dissection since the mass enlaced the left ureter and renal vessels. On histological examination, the paraaortic lymph nodes were metastatic.ConclusionRhabdomyosarcoma is an aggressive malignancy with high metastatic potential. Therefore, only an accurate diagnosis and early treatment can ensure better survival. Surgery in expert hands seems to be a good option for progressive retroperitoneal nodes. However, further studies are needed to determine the place of surgery in this setting.

PARATESTICULARRHABDOMYOSARCOMA:A CASE REPORT AND REVIEW OF THE LITERATURE

Paratesticular Rhabdomyosarcoma (RMS) is a rare tumor. Treatment must be multimodal and based on surgery, chemotherapy and radiotherapy. We report an observation of a paratesticular RMS in a child and willdiscuss the diagnostic and therapeuticmodalities in the highlight of a review of the literature.

Patients with completely resected nongenitourinary low-risk embryonal rhabdomyosarcoma are candidates for reduced duration low-intensity chemotherapy.

The survival of patients with localized embryonal rhabdomyosarcoma (RMS) completely resected at diagnosis is greater than 90%. Most patients have paratesticular, uterine, or vaginal RMS, limiting specific analyses of RMS localized in other anatomic regions. This international study was conducted to define the outcome for completely resected embryonal RMS at sites other than paratesticular, uterine, or vaginal primary sites. A total of 113 patients aged 0-18years were identified who were enrolled from January 1995 to December 2016 in Children's Oncology Group (COG) (64 patients) and European protocols (49). Genitourinary nonbladder and prostate RMS were excluded. The recommended chemotherapy was vincristine and actinomycin-D (VA) for 24weeks or ifosfamide plus VA in the European protocols and VA for 48weeks or VA plus cyclophosphamide in the COG protocols. The most common primary sites were nonparameningeal head and neck (40.7%), other (23.9%), and extremities (20.4%). In the COG studies, 42% of patients received VA and 58% VA plus cyclophosphamide. In Europe, 53% received VA and 47% ifosfamide plus VA. With a median follow-up of 97.5months, the 5-year progression-free and overall survival was 80.0% (71.2%-86.4%) and 92.5% (85.6%-96.2%), respectively, without significant differences between chemotherapy regimens. Tumor size (< or >5cm) significantly influenced overall survival: 96.2% (88.6%-98.8%) vs. 80.6% (59.5%-91.4%), respectively (p = .01). Survival of patients with nonalveolar RMS completely resected at diagnosis is excellent among tumors arising from nonparatesticular, uterine, and vaginal sites, and patients may be treated successfully with low-intensity chemotherapy. To reduce the burden of treatment, VA for 24weeks may be considered in children with tumors <5cm.

Malignant testicular tumors in children: A single institution's 12-year experience.

Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management. This study aimed to create a holistic picture of the interprofessional team in the management of malignant testicular tumors.Seventeen patients had mixed germ cell tumors, 15 had pure yolk sac tumors, 2 had immature teratomas, 2 had teratocarcinomas, and 1 had a sex cord stromal tumor. Five lesions were diagnosed as nongerm cell tumors: 2 embryonal rhabdomyosarcomas, 2 lymphomas, and 1 acute myeloid leukemia. At initial presentation, retroperitoneal (n = 2), bone marrow (n =1), and mediastinal (n = 1) metastases were identified in 4 (10%) patients. The operative interventions performed included radical inguinal orchiectomy (n = 5), scrotal orchiectomy (n = 31), and testicular biopsy or testis-sparing enucleation of the tumor (n = 6). Postoperatively, 18 patients received either adjuvant chemotherapy (n = 14) or chemoradiation (n = 5). Five patients with mixed germ cell tumors (n = 2), group IV paratesticular rhabdomyosarcoma (n = 2), and acute myeloid leukemia with myeloid sarcoma (n =1) died of disease progression. Thirty-six patients remained alive and disease-free at the last visit.Malignant testicular tumors in children deserve proper diagnostic support from a therapeutic perspective. Any concern or suspicion of a testicular tumor warrants an inguinal approach to avoid scrotal violation.

Robotic retroperitoneal lymphadenectomy in paratesticular rhabdomyosarcoma

Paratesticular sarcoma is an aggressive malignanttumor of mesenchymal origin. The rhabdomyosarcoma is the most common among children.Rhabdomyosarcoma treatment consists ofsurgery, chemotherapy and radiotherapy. Prognosicdepends on local recurrence and distant metastasis. We present the case of a 16-year-old male, who inApril 2016 underwent right radical orchiectomy surgery by testicular mass rapidly evolving, with pathological results indicating a paratesticular rhabdomyosarcoma. The extension study showed aprecaval adenopathy suggestive of lymph nodemetastasis, therefore it was a high-grade rhabdomyosarcoma. There was an appropriate response after chemotherapy (Protocol EpSSG RMS2005) andwe decided to perform a robotic lymphadenectomy. We performed a transperitoneal approach with 8mm trocar and 12mm optica trocar. We accessed the retroperitoneal space through a latero-colic incision. Then we performed a craniocaudal lymph node dissection until the aortic bifurcation. The surgical time was 240min with a blood loss of 200ml. There were no complications. The patient was discharged on the fourth day after surgery. Pathology showed metastasis of rhabdomyosarcoma without capsular rupture. After two months, we placed the left testicle into inguinal canal prior to radiotherapy. Robotic lymph node metastasis lymphadenectomy from paratesticular sarcomas is a feasible treatment with the advantage of minimally invasive surgery and acceptable morbidity.

Paratesticular rhabdomyosarcoma in a 30 months old child at the Lagoon Mother and Child Teaching Hospital (CHUMEL) in Cotonou

INTRODUCTION: Paratesticular rhabdomyosarcoma is a rare and aggressive tumor. We report here a case in a 30 months old boy. OBSERVATION: The parents would have noticed a small, painless and rapidly evolving testicular mass in the six months old child. Not having health insurance, they went to the hospital only five months later. This was followed by a total ablation of the mass (without orchidectomy) and an anatomopathological investigation concluding to a pleiomorphic rhabdomyosarcoma. No chemotherapy was performed and the mass recurred two months later. This time, its ablation was followed by a series of non-adapted chemotherapy sessions with irregular follow-ups leading eight months later to the child’s admission at our department. He presented with a degraded general condition, associating an infectious and anemic syndrome, an important increase of the scrotal mass and the occurrence of a tumoral mass in the left iliac fossa. The tumoral nature of these masses was confirmed on abdominal ultrasound and scan. The tumor was classified stage II (AJCC Prognostic stages). The multidisciplinary medical staff indicated a total ablation of the two masses, followed by a new session of adapted chemotherapy, in the absence of radiotherapy means. The child died in the immediate surgery follow-ups due to cardiac arrest. CONCLUSION: In our context of insufficient technical facilities, only early detection and adapted imperative chemotherapy, would have enabled a durable remission in front of the paratesticular rhabdomyosarcoma.

Paratesticular Rhabdomyosarcoma in a 30 Months Old Child at the Lagoon Mother and Child Teaching Hospital (CHUMEL) in Cotonou

Introduction: Rhabdomyosarcoma is the most common soft tissue tumor in children. Modern multidisciplinary approaches make it possible to better characterize the different entities and to adapt the treatment accordingly. Paratesticular localization is rare and aggressive. We report here a case of paratesticular localization in a 30 months old boy. Observation: The parents would have noticed a small, painless and rapidly evolving testicular mass in the 6 months old child. Not having health insurance, they went to the hospital only 5 months later. This was followed by a total ablation of the mass (without orchidectomy) and a pleiomorphic rhabdomyosarcoma had been concluded. No chemotherapy was performed and the mass recurred 2 months later. This time, its ablation was followed by a series of non-adapted chemotherapy sessions with irregular follow-ups leading 8 months later to the child’s admission at our department. He presented with a degraded general condition, associating an infectious and anemic syndrome, an important increase of the scrotal mass and the occurrence of a tumoral mass in the left iliac fossa. The tumoral nature of these masses was confirmed on abdominal ultrasound and scan. The tumor was classified stage II (TNM: initial Tumors, Nodes, Metastasis). The multidisciplinary medical staff indicated a total ablation of the two masses, followed by a new session of adapted chemotherapy, in the absence of radiotherapy means. The child died in the immediate surgery follow-ups due to cardiac arrest. Conclusion: In our context of insufficient technical facilities, only early detection and adapted imperative chemotherapy, would have enabled a durable remission in front of the paratesticular rhabdomyosarcoma.

Management of an Alveolar paratesticular Rhabdomyosarcoma: A case report

Paratesticular Rhabdomyosarcoma is a rare mesenchymal tumor. The Alveolar variant is the one with the worse prognosis. It comprehend 20% of cases. The treatment is multimodal combining surgery, chemotherapy and radiotherapy. Depending on the extent of disease and the staging group, the approach of treatment is different. Here we report the case of an alveolar Paratesticular rhabdomyosarcoma and review the literature regarding this unusual clinical entity.

V03-12 ROBOTIC-ASSISTED LAPAROSCOPIC RPLND FOR PARATESTICULAR RHABDOMYOSARCOMA USING 5 MM INSTRUMENTS IN PEDIATRIC SETTING

You have accessJournal of UrologyPediatrics (V03)1 Sep 2021V03-12 ROBOTIC-ASSISTED LAPAROSCOPIC RPLND FOR PARATESTICULAR RHABDOMYOSARCOMA USING 5 MM INSTRUMENTS IN PEDIATRIC SETTING Angeline Johny, Vinaya Bhatia, Alan Hsieh, Jeffrey White, and Chester Koh Angeline JohnyAngeline Johny More articles by this author , Vinaya BhatiaVinaya Bhatia More articles by this author , Alan HsiehAlan Hsieh More articles by this author , Jeffrey WhiteJeffrey White More articles by this author , and Chester KohChester Koh More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000001991.12AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The primary objective of this video is to demonstrate that the robotic approach using 5 mm instruments for primary retroperitoneal lymph dissection (RPLND) in the pediatric patient population with paratesticular rhabdomyosarcoma (PT-RMS) is an effective and safe procedure with favorable oncologic outcomes. Patients greater than 10 years of age with PT-RMS are often under staged and under treated. Additionally, the incidence of micrometastases has been shown to be as high 40%. Micrometastases is not amenable to chemotherapy and as such, the current US recommendation is for patients who are ten years and older with PT-RMS to undergo an ipsilateral retroperitoneal lymph node dissection regardless of radiographic findings. Following initiation of these guidelines, studies have demonstrated that ipsilateral RPLNDs have improved overall survival to 86%. METHODS: In this video, two case presentations were discussed demonstrating both left and right unilateral RPLND templates. For our left template, the patient is an 11 year old male with unremarkable history who presented with one month history of enlarging left hemiscrotum and work-up notable for elevated LDH of 577. He underwent a left radical inguinal orchiectomy with final pathology demonstrating a spindle cell RMS variant with positive margins. For our right template, the patient is a 10 year old male with history of Wiskott-Aldrich Syndrome with associated thrombocytopenia who also presented with a large painless right scrotal mass. Work-up was notable for LDH of 1012. He underwent a right radical inguinal orchiectomy with final pathology demonstrating Embryonal RMS with spermatic cord, tunic, and epididymal. Both patients underwent a primary robotic RPLND using 5 mm instruments and tolerated the procedure well, with favorable immediate post operative and long term oncologic outcomes. RESULTS: To approach our template adequately, we planned the following port placement: an 8 mm camera port at the superior margin of the umbilicus, two 5 mm robotic ports in the midline, with one inferior to the xiphoid, and the second inferior to the umbilicus but superior to the bladder. The assistant port was placed in the right versus left lower quadrant depending on the laterality of the RPLND. This port placement permits visualization of the renal hilar vessels, the great vessels, the sympathetic nerve trunks, as well as the ureter and major pelvic vessels, and was modified from techniques already described by Cost et al. 2012. CONCLUSIONS: Robotic assisted RPLND enables urologists to easily and safely to apply the benefit of minimally invasive surgery with 5 mm instruments. The major advantages of the robotic approach include reduced morbidity and mortality with preserved oncologic outcomes, enhanced visualization, and heightened instrument precision. Barriers to wide spread utilization of this technique include steep learning curve, need for proctor to oversee procedure, as well as cost. Nonetheless, our video demonstrates that the advantages of using 5 mm robotic instrument ports during a primary RPLND in the pediatric setting are technically feasible, with enhanced clinical and oncologic outcomes. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e225-e226 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Angeline Johny More articles by this author Vinaya Bhatia More articles by this author Alan Hsieh More articles by this author Jeffrey White More articles by this author Chester Koh More articles by this author Expand All Advertisement Loading ...

Surgical management of paratesticular rhabdomyosarcoma: A consensus opinion from the Children's Oncology Group, European paediatric Soft tissue sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe.

The treatment of paratesticular rhabdomyosarcoma (PT-RMS) has varied over time and by cooperative group. The International Soft Tissue Sarcoma Database Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft tissue sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). The INSTRuCT surgical committee has been given charge of the development of internationally applicable consensus guidelines for the surgical treatment of rhabdomyosarcoma. This clinical consensus opinion document addresses accepted principles and areas of controversy, such as scrotal violation and retroperitoneal nodal evaluation, providing an evidence-based guideline for the surgical treatment for PT-RMS.

Paratesticular Embryonal Rhabdomyosarcoma with Lung Metastasis – A Rare Case Report

Paratesticular rhabdomyosarcomas (RMS) are very rare malignant tumors arising from the mesenchymal tissues of tunica, epididymis or spermatic cord. They present as painless hard masses in inguinoscrotal region, and large tumors can be mistaken for testicular tumors. They can spread to retroperitoneal lymph nodes or hematogenously metastasize to lung, bones and bone marrow. Here, we report a case of Embryonal RMS with spindle cell component presenting with painless scrotal mass and lung metastasis at initial diagnosis.

Long-term remission of metastatic pleomorphic paratesticular rhabdomyosarcoma with chemotherapy in an adolescent boy - A case report

AbstractRhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric age group. They are classified based on the histopathological characteristics. The prognosis of RMS depends on the site of occurrence and the histopathology. Although paratesticular site is considered as one of the favorable sites, pleomorphic histology, even at this favorable site, confers a poor prognosis. Treatment includes surgery chemotherapy and radiation in metastatic cases. Here, we describe a case of an adolescent boy with pleomorphic paratesticular RMS treated successfully with chemotherapy.

Paratesticular Embryonal Rhabdomyosarcoma on Ectopic Testis: A Case Report

Para testicular rhabdomyosarcoma is a rare malignant tumour, which usually presents as a painless mass in the scrotum or groin. A case of paratesticular embryonal rhabdomyosarcoma in a 16-year-old male, with a history of left testicular ectopia treated with orchiopexy, is being reported here who presented with chronic scrotal swelling. Paratesticular rhabdomyosarcoma is a rare non germ cell tumour of scrotal sac in children and young adult/teens which can invade testis at presentation. Embryonal variant is the most common type. 40% cases can have metastasis to retroperitoneal lymph node. Diagnosis can be done on high degree of clinical suspicion coupled with biopsy and immunohistochemistry. Multimodality approach of treatment is often beneficial for patients.