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Background: This study aimed to assess the impact that the quality of primary and subsequent surgeries has on the survival of patients with para-testicular rhabdomyosarcoma (PTRMS)., Methods: Patients with localized (IRS I-III) and metastatic (IRS IV) PTRMS were enrolled in the two Cooperative Weichteilsarkom Studiengruppe (CWS) trials (CWS-96, CWS-2002P) and the Soft Tissue Sarcoma Registry (SoTiSaR)., Results: Among 196 patients (median age, 8.4 years), 106 (54.1%) had primary complete resection. Image-defined lymph node (LN) disease was detected in 21 (11.5%) patients in the localized cohort and 12 (92.3%) patients in the metastatic cohort. The 5-year event-free survival (EFS) and overall survival (OS) were respectively 87.3% and 94.0% for the patients with localized PTRMS and 46.2% and 42.2% for the patients with metastatic PTRMS. Protocol violations during the primary surgery (PV-PS) were observed in 70 (42%) of the IRS I-III patients. This resulted in higher rates of R1/R2 resections (n = 53 [76%] vs n = 20 [21%]; p < 0.001) with a need for pretreatment re-excision (PRE) (n = 50 [83%] vs n = 10 [17%]; p < 0.001) compared with the patients undergoing correct primary surgery. Protocol violations during PRE occurred for 13 (20%) patients. Although PV-PS did not influence the 5-year EFS or OS in the localized PTRMS cohort, the unadjusted log-rank test showed that R status after PRE is a prognostic factor for 5-year OS (R1 vs R0 [81.8% vs 97.6%]; p = 0.02)., Conclusions: The quality of surgical local control in PTRMS is unsatisfactory. Emphasis should be placed on evaluating the resection status after PRE in further clinical trials., (© 2024. The Author(s).)

Soft-tissue sarcomas represent a cohort of rare and heterogeneous malignant tumors that could affect various body parts, with a higher incidence in the lower extremity. When these tumors are surgically removed, both the superficial and deep lymphatic pathways could also be damaged and might require immediate reconstruction to prevent lymphatic complications. In the present report, we describe a case of a patient affected by a high-grade (G3) spindle cell pleomorphic rhabdomyosarcoma of the upper medial thigh. A 22 × 20 cm mass was removed with exposure of the deep femoral vessels and the great saphenous vein. After intraoperative indocyanine green lymphography, it was determined that the superficial lymphatic vessels were intact, but the deep lymphatic system was unavoidably damaged. As a reconstructive procedure, we performed a pedicled SCIP-based vascularized lymphatic vessel transfer and vascularized lymph node transfer to restore the deep lymphatic system and dead space obliteration. The procedure was successful, and no signs of lymphatic impairment were observed during the two-year follow-up period. We believe that this novel approach might be helpful in cases of large and profound defects that involve the deep lymphatic system. The combination of these two techniques could help restore deep lymph drainage, minimizing the risk of superficial system overload and lymphatic dysfunction. No other cases have been described so far employing the same approach. Considering the obtained results, this procedure might be worth further investigation., (© 2024 Wiley Periodicals LLC.)

Approximately one third of children with rhabdomyosarcoma relapse or have refractory disease. Treatment approaches include a combination of systemic therapies and local therapies, directed at tumour site(s). This review was conducted to evaluate the effectiveness and safety of the combination of surgery and brachytherapy as local therapy for treating children and young people with relapsed/refractory rhabdomyosarcoma. This review identified studies based on a previous systematic review looking at the treatments for children and young people under 18 years old with relapsed/refractory rhabdomyosarcoma. Studies conducted after 2000 were included. Survival outcomes, relapse rates, adverse events and functional outcomes were extracted. From 16,965 records identified in the baseline systematic review, 205 included the words 'AMORE' or 'brachytherapy', and were screened for eligibility in this substudy. Thirteen studies met the inclusion criteria for Local-REFoRMS, including over 55 relapsed and refractory rhabdomyosarcoma patients. Most studies were retrospective cohort studies conducted within Europe. Most patients had embryonal disease within the head and neck or bladder/prostate regions, and received local therapy for first relapse. Approximately one quarter of patients relapsed following surgery and brachytherapy, with local relapses occurring more than metastatic relapse. Adverse events and functional outcomes were infrequently reported, but related to the site of surgery and brachytherapy. Study quality was limited by inconsistent reporting and potential selection bias. Outcomes following surgery and brachytherapy for a selected group of relapsed and refractory rhabdomyosarcoma show reasonable benefits, but reporting was often unclear and based on small sample sizes., (© 2024 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Key Points: Surgery in adults with head and neck rhabdomyosarcoma does not improve survival rates. Surgery should be performed deliberately and focused on the timing of combined treatment modality. Adult head and neck rhabdomyosarcoma benefits from salvage surgery following chemoradiotherapy., (© 2023 ARS‐AAOA, LLC.)

Rhabdomyosarcoma (RMS) with TFCP2 rearrangement has been identified recently. This entity has a distinctive clinicopathologic features: a rapidly aggressive clinical course, a preference for the craniofacial bones, a spindle and epithelioid histomorphology, and positive immunohistochemistry for epithelial markers, ALK, and myogenic markers. RMS with TFCP2 rearrangement is rare and may be misdiagnosed as other spindle cell tumors. Here, we report a case of this entity arising in the mandible, which was initially diagnosed as ossifying fibroma in primary tumor in another hospital. A 26-year-old man presented with a recurred mass in the mandible for 1 month after the operation of mandibular tumor. The first excisional specimen was initially diagnosed as ossifying fibroma in another hospital. Histopathologic examination revealed the tumor with a hybrid spindle cell and epithelioid cytomorphology, spindle cells and spindle-to-epithelioid cells with eosinophilic and rich cytoplasm, with high-grade features, prominent nucleoli and some atypical mitosis. Immunohistochemical analysis revealed positivity for desmin, MYOD1, pan-keratin, ALK (5A4), ALK (D5F3). Based on the morphology and immunophenotype, molecular studies were performed, which revealed a FUS::TFCP2 fusion transcript, confirming the diagnosis of Rhabdomyosarcoma with FUS::TFCP2 fusion. Making a correct diagnosis is primarily dependent on awareness by the pathologist of this rare subtype of RMS and careful histopathological evaluation, supported by immunohistochemical and molecular analysis, to avoid potential diagnostic pitfalls., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

BACKGROUND Sinonasal rhabdomyosarcoma (RMS) is a rare malignancy in children and adolescents. It is aggressive and locally invasive, and can require local postoperative radiotherapy. This report presents the case of a 16-year-old girl with a sinonasal-cutaneous fistula following excision and radiotherapy for rhabdomyosarcoma, which required reconstructive surgery using an expanded forehead flap. CASE REPORT We report the case of a16-year-old girl who was referred to our clinic with sinonasal-cutaneous fistula. Prior to presentation at our department, she presented with bilateral intermittent nasal congestion 3 years ago. At a local hospital, orbital computed tomography and nasal endoscopic biopsy revealed an embryonal rhabdomyosarcoma (ERMS). One month later, skull base tumor resection, nasal cavity and sinus tumor resection, and low-temperature plasma ablation were performed at a local hospital. Two weeks after the operation, the patient received intensity-modulated radiation therapy for a total of 50 Gy. Chemotherapy started 15 days after radiotherapy, using a vincristine, dactinomycin, and cyclophosphamide (VAC) regimen. Approximately 1 month later, an ulcer appeared at the nasal root and the lesion gradually expanded. The patient was referred to our hospital due to the defect. Firstly, a tissue expander was implanted at the forehead for 7 months. Then, the skin around the defect was trimmed and forehead flap was separated to repair the lining and external skin. The flap survived well 1-year after the operation. CONCLUSIONS This report highlights the challenges of post-radiation reconstructive surgery and describes how an expanded forehead flap can achieve an acceptable cosmetic outcome in a patient with a sinonasal-cutaneous fistula.

Background: Recently, the infiltration of a subpopulation of cells represented by mononucleated cells extracted from peripheral blood [Peripheral Blood-Mononuclear Cells (PB-MNCs)] is becoming a useful technique for medical and surgical regenerative procedures. Due to the angiogenetic and regenerative properties of PB-MNCs, the infiltration of these cells is, in our opinion, a new option indicated in the treatment of pathologies characterized by tissue dystrophy, loss of vascularization, and non-healing wounds., Case Presentation: A 25-year-old active smoker patient was diagnosed with Rhabdomyosarcoma of the anterior tibial muscle of his left leg and treated with neoadjuvant chemo- and radiotherapy (RT). After the tumor excision, the patient developed wound dehiscence with bone exposure and a perilesional radiation-induced chronic dermatitis characterized by skin dyschromia and hair thinning along the treated area. The patient underwent surgical debridement and reconstruction with autologous skin grafts and dermal substitutes, with poor outcomes due to graft failure. The patient was subsequently treated with surgical debridement and coverage with a reverse sural fascia-cutaneous flap. After 13 days, wound dehiscence was observed, and reconstruction of the dehiscent areas was performed with a split-thickness autologous skin graft with no success. After wound debridement, a new split-thickness skin graft was performed, and a concentrate of autologous PB-MNCs was injected in the flap and perilesional skin. After 14 days, graft take was reached, and improvements in perilesional tissue tropism were noted. At 2 months follow-up, the patient appeared completely healed., Conclusions: In our opinion, the use of PB-MNCs to treat conditions characterized by tissue dystrophy, which require neoangiogenesis and cell regeneration, can be a useful and unconsidered technique that could be utilized to improve tissue tropism. Furthermore, prospective trials are necessary to validate our observations.

Objectives: To investigate the clinicopathological characteristics and prognosis of patients with primary sarcoma of the uterine cervix., Methods: We identified all patients with primary cervical sarcomas treated at our institution from 2002 to 2020 and analyzed the clinicopathological characteristics and prognosis., Results: 34 patients were identified, 7 (20.6%) patients had leiomyosarcoma, 6 (17.6%) had carcinosarcoma, 5 (14.7%) had Ewing sarcoma, 4 (11.8%) had rhabdomyosarcoma, 4 (11.8%) had undifferentiated sarcoma, 2 (5.9%) had adenosarcoma, 2 (5.9%) had endometrial stromal sarcoma, 1 (2.9%) had dermatofibrosarcoma protuberans, 1 (2.9%) had alveolar soft tissue sarcoma and 2 (5.9%) had sarcoma not otherwise specified. The median age of the whole patients was 43.5 years (range, 13-63). The median age of patients with Ewing sarcoma or rhabdomyosarcoma was 22 years (range, 13-39) and 17 years (range, 13-36 years), respectively. The distribution by stage was: stage I in 21 (61.8%) patients, stage II in 4 (11.8%), stage III in 6 (17.6%) and stage IV in 3 (8.8%). Overall, 30 patients (88.2%) received surgical treatment. The median follow-up was 33.3 months (range 3.6-187.3 months). 11 patients died within 2 years after diagnosis, most of them were patients with carcinosarcoma or undifferentiated sarcoma (45.5%, 5/11). In the entire cohort, 2- and 5-year OS were 67.2% and 56.9%, respectively. 5-year OS was 25.0% for undifferentiated sarcoma, 50.0% for rhabdomyosarcoma, 50.0% for carcinosarcoma, 53.3% for Ewing sarcoma, 57.1% for leiomyosarcoma., Conclusion: Cervical sarcomas are rare neoplasms with multiple histological subtypes and follow an aggressive course. Prognosis may be associated with tumor histology and stage., (© 2024. The Author(s).)

Background: Pleomorphic rhabdomyosarcoma is a rare sarcoma in adults. The clinical characteristics, outcomes and prognostic factors associated with pleomorphic rhabdomyosarcoma remain unclear., Methods: We retrospectively analyzed data from the Bone and Soft Tissue Tumor Registry of Japan, and enrolled patients with pleomorphic rhabdomyosarcoma. Disease-specific overall survival, local recurrence-free survival and distant metastasis-free survival were estimated using the Kaplan-Meier method; Cox regression model was used to identify prognostic factors., Results: In total, 182 patients with pleomorphic rhabdomyosarcoma were included. Median age was 63 (range 20-95) years. The lower extremity (48%) was the most frequent tumor origin site, while head and neck were rare (4%). A total of 43 patients (24%) had distant or regional nodal metastases at first presentation. In all cases, the 2-year and 5-year survival rates were 66.3% and 54.1%, respectively. Distant metastasis was a significant poor prognostic factor (Hazard ratio 6.65; 95% confidence intervals, 3.00-14.75, P < 0.0001), with median survival of such patients being 9.4 (95% confidence intervals: 5.3-12.2) months. In 134 localized cases, the 2-year and 5-year survival rates were 91.5% and 68.3%, respectively. Large tumor size and older age were associated with poorer prognosis. Through data from localized and locally curative cases extracted and adjusted by propensity score matching, we found that perioperative chemotherapy did not improve disease-specific overall survival, distant metastasis-free survival or local recurrence-free survival., Conclusions: Clinical characteristics and outcomes of pleomorphic rhabdomyosarcoma are similar to those of other high-grade soft tissue sarcomas. Pleomorphic rhabdomyosarcoma may be less chemosensitive, and a strategy other than the standard cytotoxic chemotherapy is required to improve its prognosis., (© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

The authors present treatment of rhabdomyosarcoma of the gluteal region with secondary lesion of the lung and chest wall. Features of chest wall defect closure are analyzed.

Robotic assisted (RA) retroperitoneal lymph node dissection (RPLND) has grown in popularity as it offers decreased morbidity and faster recovery compared to the open technique. Proponents of open surgery raised concerns about the oncological fidelity of the RA approach for testicular tumors where complete resection is needed. In boys > 10 years with paratesticular rhabdomyosarcoma (RMS), RPLND is indicated for staging purposes only. In this population, the RA technique should provide its benefits without concerns for oncological compromise. We present an analysis of RA-RPLND for boys with paratesticular RMS. We queried our institution's prospectively collected database of pediatric robotic cases for patients undergoing RA-RPLND post-radical orchiectomy for paratesticular mass, confirmed by pathology as RMS. Demographic, surgical, follow-up, and oncological outcomes were evaluated between 2017 and 2023. Five patients underwent RA-RPLND for paratesticular RMS. The median age was 16.1 years (15-17), with median OR time of 456 min (357-508). No conversions to open occurred. Inpatient median total opioid use was 1.8 (0.4-2.7) morphine equivalent/kg. The median lymph node yield was 27 (8-44) and post-op length of stay was 3 days (2-5). The median time to initiating adjuvant chemotherapy was 10.5 days (7-13). One patient had complications: pneumothorax attributed to central line placement and chyle leak that resolved in 1 week with dietary restriction. Our series demonstrates the feasibility, safety, and efficacy of the RA approach for RPLND in pediatric patients with paratesticular RMS. This is the most extensive case series currently in the literature and the only one exclusively done for paratesticular RMS., (© 2023. The Author(s), under exclusive licence to Springer-Verlag London Ltd., part of Springer Nature.)

Background: 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., Patients and Methods: 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., Results: 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., Conclusions: 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.

Malignant melanoma is well-known for phenotypic plasticity, and rare cases of divergent differentiation have been described. This case report is of a tumour diagnosed as 'rhabdomyosarcoma' on the face of a man in his 80s. However, given the recent excision of an ulcerated melanoma (Breslow thickness 5.8 mm) from the same site, the more likely diagnosis would be recurrent melanoma with rhabdomyosarcomatous differentiation. This highlights a rare form of divergent differentiation and a potential diagnostic pitfall., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children, requires multimodal therapy which is determined by risk group stratification. Local control may be achieved by surgical resection, radiation, or both. Resection may occur upfront or following induction chemotherapy as a delayed primary excision. An R1 resection may allow a reduction in radiation exposure; however, debulking is not indicated nor is excision of residual masses at the end of therapy. Regional lymph node assessment is an important component of surgical care, as positive nodal basins require radiation. Depending on the tumor site and biology, sentinel lymph node biopsy vs biopsy of clinically or radiographically concerning nodes is indicated. Therapeutic lymph node dissection is never indicated. Familiarity with site-specific oncologic principles for RMS and participation in a multidisciplinary team including Pediatric Oncology and Radiation Oncology are necessary components of surgical care to ensure optimal outcomes., (Copyright © 2023. Published by Elsevier Inc.)

Introduction. Pleomorphic rhabdomyosarcoma (RMS) is an aggressive and rare malignant neoplasm with a poor prognosis. As its name suggests, this tumor exhibits extensive pleomorphism with features of skeletal muscle differentiation. Due to its rarity, its diagnosis is often a clinical and pathological challenge. Since only small case series and a few scattered case reports exist in the literature, the impact of different demographic features, tumor site, and/or treatment modality on patient outcomes has yet to be extensively studied. Methods. We report a case of a pleomorphic RMS presenting atypically as an abdominal wall mass. We have also analyzed the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) database to determine the factors affecting the outcome of this neoplasm. Moreover, we present a review and summary of pleomorphic RMS cases arising from the abdominal wall reported in the English language literature. Results. We found two hundred and forty-two cases of pleomorphic RMS in the SEER database. The majority of the patients were diagnosed after the age of 40, with the age of diagnosis showing a unimodal distribution. The majority of the patients were Caucasian (82%) and male (59%). Age of diagnosis, tumor stage, and surgical management significantly affected the patients' outcome, while patients' ethnicity, sex, or tumor site did not affect the outcome. We only found five previously reported cases of pleomorphic RMS arising from the abdominal wall. Conclusions. Pleomorphic RMS arising from the abdominal wall is extremely rare. Our data sheds light on the factors affecting the outcome of pleomorphic RMS. We have also discussed the challenges involving the histopathological diagnosis of this rare neoplasm and how to best approach this task.

Purpose: In pediatric bladder/prostate-rhabdomyosarcoma, the rate of bladder preservation after neoadjuvant chemotherapy is high, with an excellent oncological outcome. Information about functional urological long-term outcomes is rare., Methods: Data of all patients who had undergone bladder-preserving surgery with or without brachytherapy at our institution between 2009 and 2020 were analyzed retrospectively. Detailed urological function was assessed focusing on age-related continence, bladder capacity and urodynamic findings., Results: We identified 40 patients, median age at surgery of 27 months (range 9-191), and 32 patients additionally received postoperative high-dose-rate brachytherapy. The median follow-up was 32.5 months (range 6-125). The bladder capacity increased from median 66.7% (21.1-180) of expected bladder capacity related to age 3 months after surgery to 87.4% (58.1-181.8) 9 months after surgery. In the group of aged > 6-year-old, continence was 94% (83% with brachytherapy, 100% without brachytherapy). Erectile function was normal in 92% (90% with brachytherapy, 100% without brachytherapy). Bladder capacity was more than 65% expected bladder capacity related to age in 70% (60% with brachytherapy, 86% without brachytherapy). 65% of all patients need neither anticholinergic drugs nor low-dose antibiotics (63% with brachytherapy, 71% without brachytherapy)., Conclusions: Bladder preservation with good functional outcome can be achieved in localized bladder/prostate-rhabdomyosarcoma. In selected cases, supportive brachytherapy additionally contributes to an improvement in the oncological outcome with calculable risks for bladder and erectile function. Careful urological aftercare should be a fixed priority after oncological follow-ups., (© 2022. The Author(s).)

Purpose: Head and neck rhabdomyosarcoma (HNRMS) is a rare but aggressive malignant neoplasm. Given the young patient age and critical anatomy of the head and neck, performing surgery on the primary tumor still remains debatable. This study aimed to evaluate the impact of the non-surgery-based treatment versus surgery-based treatment on patients with nonmetastatic HNRMS., Methods: Patients diagnosed with nonmetastatic HNRMS between 2004 and 2015 from the Surveillance, Epidemiology, and End Results (SEER) database were enrolled in our study. Inverse probability treatment weighting (IPTW) method was employed to balance confounding factors between surgery and non-surgery groups. Kaplan-Meier methods and COX regression analyses were used to analyze survival outcomes of overall survival (OS) and cancer-specific survival (CSS). Prognostic nomogram was established to predict survival., Results: A total of 260 eligible patients were extracted from the SEER database. Kaplan-Meier survival curves revealed that there was no significant difference in OS and CSS between the surgery and non-surgery groups both before and after IPTW (p > 0.05). Cox regression analyses and IPTW-adjusted Cox regression analyses for both OS and CSS showed similar survival between the two groups. Prognostic factors were explored and a nomogram for patients in the surgery group was constructed. Risk stratification based on the nomogram indicated that patients in surgery-high-risk group did not benefit from primary surgery. While those in surgery-low-risk group had an equal survival outcome to those in non-surgery group., Conclusions: Our study revealed that compared to patients receiving surgery, those not receiving surgery had similar survival outcomes for nonmetastatic HNRMS. Our established nomogram may serve as a practical tool for individual prognostic evaluations., (© 2023. The Author(s), under exclusive licence to Federación de Sociedades Españolas de Oncología (FESEO).)

Purpose: Multimodal therapy has improved survival in genitourinary rhabdomyosarcoma, a rare pediatric cancer. However, little is reported regarding postoperative complications and long-term urinary and sexual function and quality of life., Materials and Methods: We reviewed records from 1970-2018 to identify patients with genitourinary rhabdomyosarcoma of the bladder, prostate, pelvis, vagina, and uterus. We assessed modes of therapy, and if surgical, the type of resection, reconstruction, and reoperation. Primary outcomes included urinary continence, urinary tract infection occurrence, and stone formation. We also surveyed patients older than 18 years for urinary and sexual function., Results: Fifty-one patients were identified for the post-treatment outcomes cohort. All received chemotherapy, 46 (90.2%) underwent surgery, and 34 (67%) received radiation. Twenty-nine patients (56.9%) received trimodal therapy, 17 (33.3%) received chemotherapy/surgery, and 5 (9.8%) received chemotherapy/radiation. Twenty-six had up-front radical surgery (with staged continence mechanism creation); these patients had higher rates of continence, similar rates of urinary tract infection, and higher rates of stone formation compared to those who were organ-spared. A third (4/12) of organ-spared patients underwent additional corrective surgery. Thirty patients with genitourinary rhabdomyosarcoma were surveyed and 14 responded to questionnaires. Overall, urinary complaints were mild, but both male and female respondents reported significant sexual dysfunction., Conclusions: Organ-sparing treatment was more likely to predispose patients to high rates of additional reconstructive surgery due to compromised urological function. In survey results, both men and women reported poor sexual function, but the majority of patients remained satisfied with their urinary function.

Pituitary gland metastases are very rare. Most patients with pituitary gland metastases are asymptomatic; therefore, most cases of this disease are diagnosed during autopsies. Moreover, the four most common primary tumors that metastasize to the pituitary gland are breast, lung, thyroid, and renal carcinomas. We present a very rare case of pituitary metastasis of spindle cell rhabdomyosarcoma (RMS). Our patient presented with headache, visual disorder, panhypopituitarism, and diabetes insipidus. Due to tumor expansion, resection was not possible, so diagnosis was confirmed by biopsy, and chemotherapy and irradiation were administered. Our patient showed widespread spindle cell RMS, which harbors a mutation of myogenic differentiation 1 (MYOD1) and is associated with a poor prognosis. Even high-risk patients can show a remission after chemotherapy and irradiation. In the cases with indistinct lesions in the sella region, pituitary metastasis should always be considered., Competing Interests: None declared., (Thieme. All rights reserved.)

Objective: The clinical evaluation and management of an adult with head and neck rhabdomyosarcoma is explored to delineate the diagnostic challenge posed by soft-tissue sarcomas bordering scar tissue., Case Report: A 59 year old female presents with persistent, evolving paresthesia and burning in the right posterior neck, which was found to be in close proximity to a well-healed rhytidectomy scar. Serial biopsies were non-diagnostic. Six months after initial presentation, rhabdomyosarcoma was diagnosed subsequent to histopathological and immunohistochemistry analysis. A wide local excision with posterolateral neck dissection was performed., Conclusion: A high index of suspicion for soft-tissue sarcoma should be maintained for patients with persistent soft-tissue lesions, especially in areas of scarred tissue, who present with new-onset neurological symptoms in the context of nondiagnostic biopsies.

Background: Uterine embryonal rhabdomyosarcoma (uERMS) in adult women is a very rare malignant entity. The study aim was to report a case of adult uERMS and to discuss the implications of histopathological diagnosis on the treatment and prognosis., Case Presentation: We present here the clinicopathological features of a uERMS case in an adult woman. The study has been approved by the institutional Ethics Committee and an informed consent has been obtained (IJB∕CE3005). A 45-year-old woman presented to her gynecologist with intermenstrual bleedings and polypoid cervical mass (initially interpreted as benign polyp). A second biopsy was sent to our Department of Pathology at the Jules Bordet Institute, Brussels, Belgium for revision and was reinterpreted as botryoid-type uERMS. The patient underwent a total hysterectomy. The final pathology confirms a 3 cm cervical ERMS, and a simple surveillance was decided by our multidisciplinary team. Six months later, pelvic magnetic resonance imaging control showed a recurrence in the right pelvic lymph nodes. Multi-drug chemotherapy and radiotherapy were done before surgical resection. Pathological examination of the resected pelvic mass confirmed uERMS recurrence of 60 mm, with large zones of necrosis and the presence of cartilaginous structures. The patient is free of disease 60 months after diagnosis., Conclusions: Adult uERMS is rare and the pathological examination is the main element for diagnosis and treatment. It is often confused with other benign entities, at least at the time of diagnosis. ERMS should be included in the differential diagnosis of cervical and uterine polyp of adult women. Long-term survival is possible with a multimodal therapy approach.

Introduction: Paratesticular 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 Report: We 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 mm 2 . 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., Conclusion: Rhabdomyosarcoma 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., (© 2022. The Author(s).)

Background: 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., Methods: A total of 113 patients aged 0-18 years 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 24 weeks or ifosfamide plus VA in the European protocols and VA for 48 weeks or VA plus cyclophosphamide in the COG protocols., Results: 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 97.5 months, 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 >5 cm) significantly influenced overall survival: 96.2% (88.6%-98.8%) vs. 80.6% (59.5%-91.4%), respectively (p = .01)., Conclusions: 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 24 weeks may be considered in children with tumors <5 cm., (© 2022 American Cancer Society.)

Background: Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma. Approximately 15-20% of RMS cases arise from the bladder and prostate (B/P). The optimal treatment strategy for B/P RMS remains unclear., Objective: To retrospectively evaluate the applicability of our procedure performed to treat paediatric patients with B/P RMS., Design, Setting, and Participants: This is a retrospective analysis from a single tertiary referral hospital. From August 2003 to March 2021, 62 children pathologically diagnosed with B/P RMS underwent radical cystectomy and orthotopic detaenial sigmoid neobladder reconstruction in our centre., Surgical Procedure: Surgical procedures included laparoscopic radical cystectomy and detaenial sigmoid neobladder reconstruction, which is demonstrated in the accompanying video., Measurements: Demographic, clinical, and follow-up data were collected. Perioperative and long-term oncological and functional outcomes were reported. A logistic regression analysis was also performed., Results and Limitations: All surgeries, including three intracorporeal laparoscopic surgeries, were completed successfully. Of the 62 patients, 54 were alive without evidence of disease recurrence or metastasis at the last follow-up. Five of the 14 >12-yr-old boys reported that they experienced erections. Two female patients >12 yr old reported that they menstruated. However, this was a retrospective study conducted at a single centre with limited surgeon experience., Conclusions: Our results confirmed the safety and feasibility of primary orthotopic sigmoid neobladder reconstruction after radical cystectomy for paediatric patients with B/P RMS. Good outcomes in terms of oncological control and functional recovery were achieved. The high histocompatibility and tissue adaptability of children are inspiring., Patient Summary: We describe our stepwise technique of radical cystectomy and detaenial sigmoid neobladder reconstruction for paediatric patients with bladder and prostate rhabdomyosarcoma. With this technique, we were able to achieve good functional recovery without compromising cancer control and significantly increasing complications., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: To present our experience in a single pediatric urology practice over a 10-year period with bladder tumors in the pediatric population in an effort to add to the relatively small amount of existing data. We hope to expand the community's knowledge of presentations, management and natural history of pediatric bladder tumors., Methods: We retrospectively queried our electronic medical records for International Classification of Diseases, Tenth Revision (ICD-10) and Current Procedural Terminology (CPT) codes relevant for bladder tumors. Patients with underlying bladder pathology, such as neurogenic bladder, history of bladder exstrophy, and history of bladder augmentation, were excluded., Results: We identified 30 patients with bladder tumors from 2011 to 2021. There were 21 males and 9 females. Age at diagnosis ranged from 16 months to 19 years. Tumors identified were: 11 of various inflammatory subtypes; 4 papillomas; 4 rhabdomyosarcomas; 3 papillary urothelial neoplasms of low malignant potential and 8 of other types. Treatment included transurethral resection of bladder tumor, chemoradiation and laparoscopic partial cystectomy. Twenty nine patients had disease limited to the bladder and 1 had disease outside the bladder. Follow-up ranged from 2 weeks to 13 years (median 19 months). All patients had no evidence of disease at most recent follow-up., Conclusion: Pediatric bladder tumors range from aggressive rhabdomyosarcomas to more benign urothelial lesions. Fortunately, the latter type of tumor is the more prevalent lesion. Knowledge of the treatment options and natural history of these tumors will hopefully be of benefit to clinicians and parents alike., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: CWS/RMS-96 was an international multicenter trial with randomization between two therapy arms of the standard four-drug therapy (vincristine, ifosfamide, adriamycin, dactinomycin [VAIA]) versus an intensified six-drug regimen (carboplatin, epirubicin, vincristine, dactinomycin, ifosfamide, and etoposide [CEVAIE]) for high-risk rhabdomyosarcoma (RMS), extraskeletal Ewing sarcoma (EES), and undifferentiated sarcoma (UDS) in children, adolescents, and young adults aiming to improve their survival. Intensified chemotherapy with CEVAIE did not improve outcome., Methods: Patients younger than 21 years with a previously untreated localized HR-RMS, EES, and UDS were enrolled from Cooperative Weichteilsarkom Studiengruppe (CWS) centers in Germany, Austria, Poland, Switzerland, and from Italian Soft Tissue Sarcoma Committee (STSC) centers. Randomization (1:1) to receive either 9 × 21 days cycles of VAIA or CEVAIE was performed separately in CWS and STSC. Hyperfractionated accelerated radiotherapy (32-44.8 Gy) was added at week 9-12 according to histology and response to chemotherapy. A secondary microscopically complete nonmutilating resection was performed if possible. Primary endpoints were response to chemotherapy, event-free (EFS) and overall survival (OS)., Results: Five hundred fifty-seven patients (HR-RMS: n = 416, EES and UDS: n = 141) underwent randomization: VAIA (n = 273) or CEVAIE (n = 284). Radiotherapy was given to 70% of patients in both groups. A secondary resection was performed in 47% and 48% patients, respectively. The 5-year EFS and OS for the VAIA and CEVAIE treatment arms were 59.8% and 60.8% (p = .89), and 74.2% and 68.3% (p = .16), respectively. No differences in response, toxicity, or second malignancies emerged in the two groups., Conclusion: The use of an intensified regimen failed to show a significant improvement in tumor response and outcome of patients with localized HR-RMS, EES, and UDS., (© 2022 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Background: Conservative surgery (CS) brachytherapy (BT) techniques for local therapy in bladder-prostate rhabdomyosarcoma (BP-RMS) seek to retain organ function. We report bladder function after high-dose rate (HDR) BT combined with targeted CS for any vesical component of BP-RMS., Procedure: Prospective cohort of all BP-RMS patients between 2014 and 2019 receiving HDR-BT (iridium-192, 27.5 Gy in five fractions) with/without percutaneous endoscopic polypectomy (PEP) or partial cystectomy (PC). Functional assessment included frequency-volume chart, voided volumes, post-void residual, flow studies, continence status and ultrasound scanning; abnormalities triggered video urodynamics., Results: Thirteen patients (10 male), aged 9 months to 4 years (median 23 months), presented with localised fusion-negative embryonal BP-RMS measuring 23-140 mm (median 43 mm) in cranio-caudal extent. After induction chemotherapy, local treatment consisted of PC+BT in three, PEP+BT in four and BT alone in six. At a median 3.5 years (range 21 months to 7 years) follow-up, all were alive without relapse. At a median age of 6 years (4-9 years), the median bladder capacity was 86% (47%-144%) of that expected for age, including 75% (74%-114%) after PC. Radiation dose to the bladder was associated with urinary urgency, but not bladder capacity or nocturnal enuresis. Complications occurred in two: one urethral stricture and one vesical decompensation in a patient with pre-existing high-grade vesico-ureteric reflux (VUR). The remaining patients were dry by day; five with anticholinergic medication for urinary urgency. Three patients are enuretic., Conclusions: Day-time dryness at a median 3.5 years after CS-HDR-BT was achieved in 92%, with 85% voiding urethrally, and 62% attaining day-and-night continence aged 4-9 years. We report reduced open surgery with minimally invasive percutaneous surgery, with HDR-BT or BT alone being suitable for many., (© 2022 Wiley Periodicals LLC.)

Background: Outcome of children with bladder-prostate rhabdomyosarcoma (RMS) has improved with multimodal therapies, including surgery and/or radiotherapy for local treatment. Our aim was to report long-term urological complications after a conservative approach combining conservative surgery and brachytherapy., Patients and Methods: Eighty-six patients, free of disease, were retrospectively reviewed. Symptoms related to urinary tract obstruction, incontinence, infection, and lithiasis were reported and graded according to the Common Terminology Criteria for Adverse Events (CTCAE) classification. Only symptomatic patients underwent urodynamic studies. Risk factors for complications were analyzed., Results: There were 76 males and 10 females. The median follow-up was 6.3 years (18 months to 24 years). Complications occurred after a median follow-up of 5 years (0-21). Twenty-two patients (26%) had long-term urological complications. Urinary tract obstruction was found in 15 patients (17%) and urinary incontinence in 14 patients (16%). Recurrent urinary tract infection occurred in four patients and urinary lithiasis in four (5%). The underlying physiopathology included bladder dysfunction in 15 patients (17%), urethral stenosis in six (7%), and ureterovesical junction stenosis in five (6%). On univariate analysis, posterior bladder wall dissection (p = .001), bladder neck trigone dissection (p = .010), and partial prostatectomy (p = .023) were significantly associated with an increased risk of bladder dysfunction; on multivariate analysis, only age ≤2 years (p = .028) at operation and posterior bladder wall dissection (p = .006) were found to be significant., Conclusion: The conservative surgical approach combined with brachytherapy for bladder-prostate RMS leads to long-term urological complications in 26% of survivors. Optimizing brachytherapy doses for young children and establishing a clear and long-term follow-up protocol could help to reduce these complications., (© 2021 Wiley Periodicals LLC.)

Objective: Rhabdomyosarcoma is the most common soft tissue tumor in children, with average age of onset being 5 years, and approximately 70% cases diagnosed below 10 years of age. It accounts for 37% of primary head and neck malignancies in children. Chemotherapy with surgery, and radiation is selected as the primary treatment. We report a rare case of rhabdomyosarcoma in the temporal bone presenting with glossopharyngeal and vagus nerve paralysis as well as facial palsy., Case Report: The patient was a 6-year-old boy, and his initial symptom was dizziness followed by facial palsy and hoarseness. Although a severe type of otitis media was suspected in the first clinic, CT and MRI showed a temporal bone tumor with parameningeal extension. Biopsy with cortical mastoidectomy revealed an embryonal-type rhabdomyosarcoma. Pretreatment re-excision was abandoned because of parameningeal involvement. The tumor disappeared after a series of chemotherapy, however, meningeal dissemination occurred, and he eventually died even after an additional administration of anti-cancer agents and intensive modulated radiation therapy., Conclusion: In the case of facial palsy concomitant with other cranial nerve paralysis, care must be taken into neoplastic origin. Early image diagnosis may offer a chance of complete resection in addition to chemoradiotherapy.

Rhabdomyosarcoma is the most common soft-tissue sarcoma in paediatric patients and may arise as a primary orbital neoplasm. Imaging studies show a unilateral solitary orbital lesion. With larger tumours, erosion of the adjacent orbital walls is a common accompanying bony change. We present an unusual case of rhabdomyosarcoma in a preschool girl with proptosis and temporal bossing. Imaging studies showed a homogeneous, well-delineated, extraconal mass of the right orbit with hyperostosis of the adjacent sphenoid bone. After excision of the mass and debulking of the sphenoid bone, histopathology showed embryonal rhabdomyosarcoma with no malignant infiltration of the adjacent bone. This is the first reported case of hyperostosis associated with rhabdomyosarcoma. Periosteal reaction is the proposed underlying mechanism., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Paratesticular sarcoma is an aggressive malignant tumor of mesenchymal origin. The rhabdomyosarcoma is the most common among children. Rhabdomyosarcoma treatment consists of surgery, chemotherapy and radiotherapy. Prognosic depends on local recurrence and distant metastasis., Material and Methods: We present the case of a 16-year-old male, who in April 2016 underwent right radical orchiectomy surgery by testicular mass rapidly evolving, with pathological results indicating a paratesticular rhabdomyosarcoma. The extension study showed a precaval adenopathy suggestive of lymph node metastasis, therefore it was a high-grade rhabdomyosarcoma. There was an appropriate response after chemotherapy (Protocol EpSSG RMS2005) and we decided to perform a robotic lymphadenectomy., Results: We performed a transperitoneal approach with 8 mm trocar and 12 mm 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 240 min with a blood loss of 200 ml. 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., Conclusions: Robotic lymph node metastasis lymphadenectomy from paratesticular sarcomas is a feasible treatment with the advantage of minimally invasive surgery and acceptable morbidity., Competing Interests: Conflict of interest None., (Copyright © 2022. Published by Elsevier Ltd.)

Rhabdomyosarcoma is a rare mesenchymal malignancy with four different morphological subtypes: alveolar, embryonal, pleomorphic and spindle cell/sclerosing. It is the most common soft tissue sarcoma of children and adolescents but occurs less commonly in adults. We describe a male patient in his 20s with sclerosing rhabdomyosarcoma of the left cheek who developed an isolated free flap donor site metastasis in the first instance but subsequently progressed with bilateral pulmonary metastases. Multidisciplinary team involvement in a sarcoma specialist centre is essential and collaboration between the pathologist, radiologist, head and neck surgeon, orthopaedic surgeon, radiation oncologist and medical oncologist were integral in providing optimal management in this patient. Furthermore, this case report highlights this phenomenon of implantation metastasis in a patient with rhabdomyosarcoma, and emphasises the importance of surgical barriers between the resection and reconstruction teams in an oncological case., Competing Interests: Competing interests: AH received consultancy fee from Bayer, Provectus and Q Biotics., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Extremity rhabdomyosarcoma differs from other soft tissue sarcomas, being highly sensitive to chemotherapy and radiotherapy and having a high rate of metastasis to lymph nodes. Therefore, the treatment modality differs from that of other soft tissue sarcomas. The purpose of this study was to conduct a longitudinal questionnaire survey of orthopedic oncologists in charge of surgical treatment for extremity rhabdomyosarcoma in Japan to determine whether the treatment modality chosen here is in line with the international and national treatment ones., Methods: Questionnaire surveys were conducted in 2012 and 2019 to orthopedic oncologists of Japanese Orthopaedic Association and Japanese Musculoskeletal Oncology Group., Results: Responses were obtained from 80 facilities and 76 facilities, respectively. Fewer than 50% of the facilities treated one or more patients a year in both years. Many facilities first performed diagnostic biopsy, but most did not perform pretreatment re-excision. The number of facilities that provided radiotherapy in addition to surgery increased significantly from 2012 to 2019 (P = 0.028), but it was still 21% in 2019. The number of facilities performing excision and lymph node dissection was 19% in both 2012 and 2019, which was a very low result without improvement. The departments responsible for follow-up have been changed to pediatrics and orthopedic oncology in tandem (P = 0.0004)., Conclusions: Radiotherapy and pathological evaluation of lymph nodes are important for improving the prognosis of patients with extremity rhabdomyosarcoma. It is necessary to continue and develop more efficient educational activities on the appropriate medical treatment modalities for extremity RMS., (© The Author(s) 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction: Primary rhabdomyosarcoma (RMS) of breast is an uncommon entity and axillary lymph node (ALN) involvement is exceedingly rare., Methods: Herein, we are reporting a case of RMS of breast with ALN metastasis in an adolescent girl. We searched Pubmed and Cochrane databases with keywords rhabdomyosarcoma and breast. All studies published in English language literature were included. Articles describing metastatic involvement of breast with RMS were excluded., Result: The initial search yielded a total of 8468 studies, out of which 03 were found to be duplicate. 8420 studies were excluded based on title and abstract as they did not fulfill inclusion criteria. Full text of the remaining 48 studies was screened. After full text screening, 26 case reports describing primary breast RMS were included. Overall 21% patients had axillary lymph node metastasis., Conclusion: Axillary staging should be considered in every patient undergoing surgery for breast RMS. However, it's impact on recurrence and survival could not be determined based on current review.

Procedure: The survival of children with rhabdomyosarcoma (RMS) has gradually improved as a result of the adoption of multidisciplinary treatments. Dedicated skills and facilities are indispensable and more readily available at reference centers. In this study, we examined the role of centers' experience (based on the number of patients treated) in their management of patients with RMS., Methods: We analyzed 342 patients with localized RMS enrolled in the European RMS 2005 protocol from October 2005 to December 2016 at 31 Italian centers that are part of the Soft Tissue Sarcoma Committee (STSC). We grouped the centers by the number of patients each one enrolled (Group 1: >40; Group 2: <40 and >10; and Group 3: <10), and compared a number of indicators to assess the appropriateness of patients' diagnostic workup and treatment and their survival., Results: Overall, 74.6% of patients were treated at 10 centers, and only three of them classifiable as high-volume centers. Only minor differences emerged between the three patient groups in terms of diagnostic investigations and treatment modalities. Survival was similar in the three groups. Approximately, one in four children treated at the centers in Groups 2 and 3 traveled to another center for surgery or radiotherapy., Conclusion: Patients treated at STSC centers with different amounts of experience had similar results in terms of survival. This is attributable to all centers in the network adhering to protocol recommendations and receiving the STSC's support on diagnostics and multidisciplinary treatments for RMS., (© 2021 Wiley Periodicals LLC.)

We report a case of primary breast rhabdomyosarcoma. A 16-year-old girl noticed tumor of her right breast and consulted a local clinic. From the result of core needle biopsy, breast sarcoma was suspected, so she attended our hospital. Breast ultrasonography showed a mosaic pattern tumor occupying the whole right breast. CT images revealed an axillary node metastasis and no distant organ metastasis. Immunohistochemical staining of the tumor yielded positive results for desmin, MyoD1, and myogenin. Based on reverse transcription polymerase chain reaction(RT-PCR), she was diagnosed as an alveolar rhabdomyosarcoma with PAX3-FKHR(FOXO1)fusion transcripts[t(2;13)(q35;q14)]. She underwent total mastectomy and dissection of axillary lymph nodes. After surgery, the whole-body magnetic resonance imaging(MRI) demonstrated metastases of sacrum and left foot, so she was under systemic chemotherapy.

Background: Our aim is to show whether the sentinel node procedure (SNP) is recommendable for pediatric patients with extremity rhabdomyosarcoma (RMS). Lymph node metastases are an important prognostic factor in pediatric patients with extremity RMS. Accurate nodal staging is necessary to treat the patient accordingly. An alternative to the current recommended lymph node sampling is the sentinel node procedure (SNP)., Methods: A systematic review was performed summarizing all published cases of SNP in addition to 13 cases from our hospital and 8 cases from two other hospitals that have not been published before., Results: For all patients (n = 55), at least one SLN was identified, but the SNP technique used was not uniform. The SNP changed the nodal classification of eight patients (17.0%) and had a false-negative rate of 10.5%., Conclusions: The SNP is recommendable for pediatric patients with extremity RMS. It can change lymph node status and can be used to sample patients in a more targeted way than nodal sampling alone. Therefore, we recommend use of the SNP in addition to clinical and radiological nodal assessment for pediatric patients with extremity RMS., (© 2021. The Author(s).)

A 63-year-old diabetic and hypertensive lady presented in New York Heart Association class III-IV dyspnea on exertion. Echocardiography showed a large mass attached to the anterior mitral leaflet and the base of the interatrial septum. After removal of the mass and excision of the anterior and posterior mitral leaflets, a bioprosthetic valve was deployed. The postoperative course was uneventful. Histopathology showed that the tumor was a high-grade rhabdomyosarcoma. Although it is a highly lethal tumor, surgical removal was indicated to relieve dyspnea, clarify the diagnosis, and improve short-term survival. Our patient survived for 8 months after surgical excision.