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Luo J, Huang B, Li G, Jiang Y. Perianal embryonal rhabdomyosarcoma: A case report. Oncol Lett 2024; 28:487. [PMID: 39185492 PMCID: PMC11342414 DOI: 10.3892/ol.2024.14620] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2024] [Accepted: 07/17/2024] [Indexed: 08/27/2024] Open
Abstract
Perianal embryonal rhabdomyosarcoma (ERMS) is a rare disease with a poor prognosis. There are few reported cases of this disease, and specific clinical manifestations are lacking; therefore, making an early diagnosis before surgery is challenging. In November 2014, a 30-year-old man was admitted to Xiaoshan Affiliated Hospital of Wenzhou Medical University due to severe left perianal pain. Ultrasonography revealed a multilocular perianal abscess, and an emergency perianal abscess incision and drainage were performed. However, pathology combined with immunohistochemistry confirmed an ERMS. The patient did not receive postoperative radiotherapy or chemotherapy and died of multiple metastases and multiple organ failure 6 months later. Perianal ERMS is highly malignant and rare, and can easily be misdiagnosed as a perianal abscess. Clinicians must enhance their knowledge and improve preoperative diagnostic tests to prevent misdiagnoses.
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Affiliation(s)
- Jingyong Luo
- Department of Anorectal Surgery, Xiaoshan Affiliated Hospital of Wenzhou Medical University, Hangzhou, Zhejiang 311200, P.R. China
| | - Bin Huang
- Department of Pathology, Xiaoshan Affiliated Hospital of Wenzhou Medical University, Hangzhou, Zhejiang 311200, P.R. China
| | - Guofeng Li
- Department of Anorectal Surgery, Xiaoshan Affiliated Hospital of Wenzhou Medical University, Hangzhou, Zhejiang 311200, P.R. China
| | - Yiqian Jiang
- Department of Radiotherapy, Xiaoshan Affiliated Hospital of Wenzhou Medical University, Hangzhou, Zhejiang 311200, P.R. China
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Peng X, Xiong X, Li Y, Li C, Wang Z, Wu Y, Su M, Weng W, Huang K, Zhou D, Fang J. Local Treatment of Children Suffering From Parameningeal Rhabdomyosarcoma: A Retrospective Single-Center Study From China. Cancer Control 2024; 31:10732748241240655. [PMID: 38514935 PMCID: PMC10958813 DOI: 10.1177/10732748241240655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2023] [Revised: 01/27/2023] [Accepted: 02/22/2024] [Indexed: 03/23/2024] Open
Abstract
BACKGROUND Treatment for parameningeal rhabdomyosarcoma (PM-RMS) has been a challenge since local control is difficult. The goal of this study was to analyse the impact of different local treatment approaches on childhood PM-RMS patients and help dispel the doubt that whether secondary radical surgery (SRS) should be encouraged in the management of PM-RMS. METHODS A total of 17 children with PM-RMS who received unified systemic chemotherapy and individualized local therapy such as radiotherapy (RT) and/or SRS were included in this retrospective study. The overall survival (OS) and event free survival (EFS) were compared between groups adopting different local strategies. RESULTS The 3-year OS and EFS of our PM-RMS patients was 75.5% and 56.5% respectively. The OS and EFS of patients who received SRS were both significantly lower than that of the non-SRS group (3-year OS: 50.0% vs 90.0%, P = .031; 3-year EFS: 33.3% vs 60.6%, P = .020). The OS and EFS of the patients who received RT was higher than that of the patients of the non-RT group (3-year OS: 85.6% vs 0%, P = .001; 3-year EFS: 64.0% vs 0%, P = .011). CONCLUSION This study illustrates that SRS was associated with poor prognosis of PM-RMS and should not be routinely performed. Optimized RT strategies along with more intensive chemotherapy may be alternative options to improve the survival of patients with PM-RMS. Multi-center, large sample and prospective studies are needed to further validate these findings.
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Affiliation(s)
- Xiaomin Peng
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Xilin Xiong
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Yang Li
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Chunmou Li
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Zhixuan Wang
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Yu Wu
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Mingwei Su
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Wenjun Weng
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Ke Huang
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Dunhua Zhou
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Jianpei Fang
- Pediatric Hematology and Oncology, Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
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Polites SF, Rhee DS, Routh JC, Lautz TB, Rodeberg DA, Dasgupta R. Critical elements of pediatric rhabdomyosarcoma surgery. Semin Pediatr Surg 2023; 32:151341. [PMID: 38042091 DOI: 10.1016/j.sempedsurg.2023.151341] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/04/2023]
Abstract
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.
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Affiliation(s)
- Stephanie F Polites
- Division of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA
| | - Daniel S Rhee
- Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Jonathan C Routh
- Department of Urology, Duke University School of Medicine, Durham, NC, USA
| | - Timothy B Lautz
- Division of Pediatric Surgery, Ann & Robert H Lurie Children's Hospital of Chicago, Northwestern University, Chicago, IL, USA
| | - David A Rodeberg
- Department of Surgery, Department of Pediatric Surgery, University of Kentucky, Lexington, KY, USA
| | - Roshni Dasgupta
- Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Medical Center, University of Cincinnati, Cincinnati, OH, USA.
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Sharma K, Goswami A. Childhood Malignancies-Clinico-demographic Profile of Patients Attending the Department of Otorhinolaryngology at a Tertiary Care Centre in North-East India. Indian J Otolaryngol Head Neck Surg 2023; 75:2006-2016. [PMID: 37636808 PMCID: PMC10447731 DOI: 10.1007/s12070-023-03779-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2022] [Accepted: 04/04/2023] [Indexed: 08/29/2023] Open
Abstract
INTRODUCTION Malignancies in children are different from those found in adults and are a significant cause of childhood mortality.They have varied clinical presentation depending on site and type of disease.It is essential to recognize the early signs and symptoms of malignancies in childhood, especially those involving head and neck region, so as to reduce childhood mortality and morbidity. MATERIALS A total of 2384 children were admitted over a period of 7 years. Out of these, 1004 fulfilled the inclusion criteria and were chosen for further evaluation.They were thoroughly evaluated by undertaking a detailed history and clinical examination.Whenever required, additional investigations were performed.After carrying out the necessary investigations, the cases were accordingly managed. Data was evaluated using proper statistical tools. RESULTS Out of 1004 cases fulfilling the inclusion criteria, 42 turned out to be malignant, with a male-to-female ratio of 1:1.2. Malignancies in children were more common in the age group of 11-18 years, followed by 1-5 years,6-10 years and 0-1 years,with rates of 59.5%, 21.4%, 16.7% and 2.4% respectively. A wide variety of tumour types were recorded,e.g.,Hodgkin's lymphoma,non-Hodgkin's lymphoma,acute leukemia,papillary carcinoma thyroid, nasopharyngeal carcinoma,Langerhans cell histiocytosis,rhabdomyosarcoma, olfactory neuroblastoma and salivary gland neoplasm. CONCLUSION Incidence of head and neck tumors in pediatric age group was found to be 1.76% with lymphoma being the most frequent.Commonest age of presentation was above 10 years. There was an overall female predominance with a male:female ratio of 1:1.2. Awareness of a potential malignancy and careful follow-up of children with suspicious head and neck cancers is mandatory for early diagnosis and prompt treatment.
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Affiliation(s)
- Kalpana Sharma
- Dept. of Otorhinolaryngology, Gauhati Medical College, Guwahati, Assam 781032 India
| | - Abhilasha Goswami
- Dept. of Otorhinolaryngology, Gauhati Medical College, Guwahati, Assam 781032 India
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Feng S, Yang Y, Yu Z, Xiong Q, Bi Y. Survival and Analysis of Prognostic Factors for Bladder Malignancies in Children and Adolescents: A Population-based Study. Urology 2023; 177:156-161. [PMID: 37085053 DOI: 10.1016/j.urology.2023.04.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2022] [Revised: 04/04/2023] [Accepted: 04/10/2023] [Indexed: 04/23/2023]
Abstract
OBJECTIVE To explore the clinicopathological features and prognosis of pediatric patients with malignant bladder tumors in a population-based cohort. METHODS The database Surveillance, Epidemiology, and End Results was used to evaluate all pediatric patients diagnosed with malignant bladder tumors between 1975 and 2018. The log-rank test was used to compare survival curves. Kaplan-Meier estimations were used to create survival curves based on various parameters. The Cox proportional hazards model was utilized to determine the factors that were independently related to mortality. RESULTS A total of 263 children and adolescents with bladder malignancies were assessed. Papillary urothelial neoplasms of low malignant potential were the most frequent histologic subtype (35.1%), while embryonal rhabdomyosarcoma was more common during the first decade of life. Survival rates varied significantly by age at diagnosis, with older patients showing better outcomes. When compared to other subtypes, papillary urothelial neoplasms of low malignant potential had the highest overall survival rates (3- and 5-year were 99.2% and 98.3%, respectively). Multivariate analysis of the entire cohort showed that Surveillance, Epidemiology, and End Results stage and surgery were significant independent predictors of progression to disease-specific death in this model. CONCLUSION Bladder malignancies are rare in children and adolescents. The prognosis for them varies. The localized stage was independently associated with superior survival and surgery could extend survival time.
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Affiliation(s)
- Shaoguang Feng
- Department of Urology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China; Department of Pediatric Surgery, Hangzhou Children's Hospital, Hangzhou, Zhejiang, China
| | - Yicheng Yang
- Department of Urology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China
| | - Zhechen Yu
- Department of Urology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China
| | - Qianwei Xiong
- Department of Urology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China
| | - Yunli Bi
- Department of Urology, Children's Hospital of Soochow University, Suzhou, Jiangsu, China.
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Xu P, Chen C, Chen B, Bi E, Du W, Jiang N, Liu Z, Lan H, Cao M, Liu Y, Huang J, Shen H, Liu C, Liu C, Xu A. Long-term Follow-up of Detaenial Sigmoid Neobladder Reconstruction for Paediatric Patients with Bladder and Prostate Rhabdomyosarcoma: Technique and Results from a Single High-volume Centre. Eur Urol 2022; 82:543-550. [PMID: 36050131 DOI: 10.1016/j.eururo.2022.08.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2022] [Revised: 07/21/2022] [Accepted: 08/14/2022] [Indexed: 11/28/2022]
Abstract
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.
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Affiliation(s)
- Peng Xu
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Chunxiao Chen
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Binshen Chen
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Enguang Bi
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China; Department of Biochemistry and Molecular Biology, School of Basic Medical Science, Guangdong Provincial Key Laboratory of Single Cell Technology and Application, Southern Medical University, Guangzhou, China
| | - Wei Du
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Ning Jiang
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Zhe Liu
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Hekui Lan
- Department of Paediatrics, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Manming Cao
- Department of Oncology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Yazhen Liu
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Jingwen Huang
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Haiyan Shen
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Cunrong Liu
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Chunxiao Liu
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China.
| | - Abai Xu
- Department of Urology, Zhujiang Hospital, Southern Medical University, Guangzhou, China.
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Alaraifi AK, Alsalamah RK, Alsalem AA, Khan AL, Elkrim M. Adult Sinonasal Rhabdomyosarcoma With Spinal Metastasis: A Case Report and Review of the Literature. Cureus 2022; 14:e25886. [PMID: 35844319 PMCID: PMC9278485 DOI: 10.7759/cureus.25886] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/12/2022] [Indexed: 12/04/2022] Open
Abstract
Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor mainly seen in the pediatric population. Here, we describe a case of an aggressive sinonasal RMS with distant metastasis in an adult patient. A 51-year-old male presented to the otolaryngology clinic with a unilateral painless neck mass and nasal obstruction. A flexible transnasal endoscope showed a huge fungating mass obstructing more than 80% of the right nasal cavity. A contrasted computed tomography (CT) scan of the paranasal sinuses showed an enhancing soft-tissue density mass involving the right nasal cavity. A biopsy revealed RMS, an embryonal variant. The patient responded well to chemoradiotherapy but later developed spinal metastasis and cord compression. He was admitted for palliative care but died due to cardiopulmonary arrest 10 months after diagnosis. A high index of clinical suspicion for malignancy is required in adult patients with unilateral nasal symptoms.
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Peterson JM, Chemello F, Calore F. Editorial: MicroRNAs and Muscle Cell Death in Cancer. Front Genet 2022; 13:892136. [PMID: 35646081 PMCID: PMC9136531 DOI: 10.3389/fgene.2022.892136] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2022] [Accepted: 03/28/2022] [Indexed: 12/03/2022] Open
Affiliation(s)
- Jennifer M. Peterson
- School of Exercise and rehabilitation Sciences, University of Toledo, Toledo, OH, United States
| | - Francesco Chemello
- Department of Molecular Biology, University of Texas Southwestern Medical Center, Dallas, TX, United States
| | - Federica Calore
- Department of Cancer Biology and Genetics, The Ohio State University, Columbus, OH, United States
- *Correspondence: Federica Calore,
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Lu JJ, Chen MB, Gao XJ, Zhang Y, Liu YY, Yong Y, Li P. Gross perianal embryonal rhabdomyosarcoma with severe multiple bone metastases throughout the body: a case report. J Int Med Res 2022; 50:3000605221087050. [PMID: 35317622 PMCID: PMC8949711 DOI: 10.1177/03000605221087050] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
In adults, embryonal rhabdomyosarcoma (ERMS) is rare and has a poor prognosis. Giant perianal ERMS with severe multiple bone metastases at initial diagnosis has not been reported and lacks effective treatment options. This current case report describes a 31-year-old female patient that presented with a large lump on the right side of the anus. ERMS was diagnosed, accompanied by multiple bone metastases throughout the body and severe thrombocytopenia. She had an extremely low platelet count at initial diagnosis, making systemic chemotherapy inappropriate. Genetic testing did not help identify effective targeted drugs. A multi-target tyrosine kinase inhibitor, anlotinib, was selected to control the tumours combined with local radiotherapy to relieve pain. The lump became smaller and this reduction was maintained for 5 months. At 7 months after the diagnosis, the patient died of thrombocytopenia. This current case may provide supportive evidence for a potential treatment for patients with advanced ERMS, especially those not suitable for chemotherapy or surgery.
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Affiliation(s)
- Jing-Jing Lu
- Department of Oncology, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
| | - Min-Bin Chen
- Department of Oncology, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
| | - Xiao-Jiao Gao
- Department of Pathology, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
| | - Yan Zhang
- Department of Oncology, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
| | - Yuan-Yuan Liu
- Clinical Research and Laboratory Centre, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
| | - Yang Yong
- Department of Gastrointestinal Surgery, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
| | - Ping Li
- Department of Oncology, Affiliated Kunshan Hospital of Jiangsu University, Kunshan, Jiangsu Province, China
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Mizuno Y, Shimojima N, Makimoto A, Yokokawa Y, Miyaguni K, Tsukizaki A, Hashimoto M, Ishikawa M, Ishihama H, Tomita H, Shimotakahara A, Yuza Y, Matsuoka K, Kawakubo H, Hirobe S. Primary alveolar rhabdomyosarcoma of the diaphragm requiring proximal gastrectomy. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102206] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
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Mulita F, Parchas N, Germanos S, Papadoulas S, Maroulis I. Case Report of a Local Recurrence of Spindle Cell Embryonal Rhabdomyosarcoma. Med Arch 2020; 74:240-242. [PMID: 32801444 PMCID: PMC7406004 DOI: 10.5455/medarh.2020.74.240-242] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/22/2020] [Accepted: 05/12/2020] [Indexed: 02/05/2023] Open
Abstract
INTRODUCTION Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents. Spindle cell RMS is a rare variant of embryonal RMS that has a predilection for young males. AIM We are presenting here a case of a local recurrence of an embryonal variant of the spindle cell RMS in a 19-year-old male. CASE REPORT In this report it is described the study of patient with local recurrence of spindle cell embryonal RMS of the left testis after left orchiectomy and adjuvant chemotherapy. Computed tomography of the abdomen was used to evaluate the tumor. The recurrent mass was about 7,5cm and the patient was operated and discharged after 6 days in a good condition. Six months after the operation the patient had a new recurrence of RMS in the left retroperitoneal space. CONCLUSION RMS is a malignant tumor of mesenchymal origin that is treated by a combination of surgery, chemotherapy, and radiation. However, up to one-third of patients experience recurrence.
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Affiliation(s)
- Francesk Mulita
- Department of General Surgery, General University Hospital of Patras, Achaia, Greece
| | - Nikolaos Parchas
- Department of Orthopedics, General University Hospital of Patras, Achaia, Greece
| | - Stylianos Germanos
- Department of General Surgery, General University Hospital of Patras, Achaia, Greece
| | - Spyros Papadoulas
- Department of Vascular Surgery, General University Hospital of Patras, Achaia, Greece
| | - Ioannis Maroulis
- Department of General Surgery, General University Hospital of Patras, Achaia, Greece
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Abstract
Urothelial pediatric neoplasms are relatively rare. Papillary urothelial neoplasms of low malignant potential (PUNLMPs) and rhabdomyosarcoma (RMS) are the most common bladder malignancies in the pediatric population. Clinical presentation encompasses macroscopic hematuria or lower urinary tract symptoms (or both) or is detected incidentally at imaging. Tumors arising from the bladder can originate from any of its four histological layers (urothelium, lamina propria, detrusor, and adventitia) and are divided into tumors that have an epithelial origin (arising from the urothelium) and those that have a non-epithelial origin (mesenchymal neoplasms). RMS is the most common malignant tumor of the urinary bladder in children younger than 10 years. Deriving from the embryonic mesenchymal cell, the histopathologic subtypes of RMS are embryonal RMS (>90%) and alveolar histology (<10%). Pre-treatment imaging should be carried out by computed tomography (CT) or at present is more likely with magnetic resonance imaging of the pelvis. Chest CT and bone scintigraphy are used to screen for metastases. In selected cases, a positron emission tomography scan may be recommended to evaluate suspicious lesions. The current prognostic classification considers age, histologic subtype, tumor site, size, and extent (nodal or distant metastases). Staging is based on pre-operative findings, group is based on intra-operative findings and pathology, and risk stratification is derived from both stage and group data. Pre-operative chemotherapy is the most common first-line intervention for bladder/prostate RMS, before surgery or radiation therapy. Collaborative groups such as the Soft Tissue Sarcoma Committee of the Children’s Oncology Group and the European Pediatric Soft Tissue Sarcoma Study Group endorse this therapy. PUNLMPs are generally solitary, small (1–2 cm), non-invasive lesions that do not metastasize. Therapy is usually limited to a transurethral resection of the bladder tumor. About 35% are recurrent and around 10% of them increase in size if they are not treated.
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Affiliation(s)
- Roberto Iglesias Lopes
- Pediatric Urology Unit, Division of Urology, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, 05402-000, Brazil
| | - Marcos Figueiredo Mello
- Pediatric Urology Unit, Division of Urology, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, 05402-000, Brazil
| | - Armando J Lorenzo
- Division of Urology, The Hospital for Sick Children and Department of Surgery, University of Toronto, Toronto, ON, Canada
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Zhu XL, Su WW, Tang JL, Yao LD, Lu LJ, Sun XJ. Primary alveolar rhabdomyosarcoma of retrorectal-presacral space in an adult patient: A case report of an uncommon tumor with rare presentation. Medicine (Baltimore) 2019; 98:e13416. [PMID: 30855432 PMCID: PMC6417633 DOI: 10.1097/md.0000000000013416] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
RATIONALE Rhabdomyosarcoma (RMS) has known as a highly malignant soft tissue sarcoma, representing 5% to 10% of all solid tumors in childhood. Alveolar rhabdomyosarcoma (ARMS) of the retrorectal-presacral space is an extremely rare lesion for adult, no study has been reported in the English literature. PATIENT CONCERNS A 51-year-old male presented with abdominal pain for 1 month, significantly worse when having a bowel movement. DIAGNOSIS Computed tomography (CT) and magnetic resonance imaging (MRI) of the pelvis showed a solid-cystic, enhancing lesion of dimension located in retrorectal-presacral space. The surgical specimen was reported as ARMS after pathological evaluation. INTERVENTIONS The tumor was complete surgical resection, and after surgery, the patient was treated with combination chemotherapy. OUTCOMES At 23 months follow up, the patient was asymptomatic with no evidence of metastases or local recurrence. LESSONS Improvements in imaging in addition to early surgical intervention and chemotherapy treatment are crucial to improve survival chances against RMS.
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Affiliation(s)
| | | | - Jin-Long Tang
- Department of Pathology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
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Wang C, Qu J, Yan S, Gao Q, Hao S, Zhou D. PFK15, a PFKFB3 antagonist, inhibits autophagy and proliferation in rhabdomyosarcoma cells. Int J Mol Med 2018; 42:359-367. [PMID: 29620138 PMCID: PMC5979828 DOI: 10.3892/ijmm.2018.3599] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2017] [Accepted: 03/23/2018] [Indexed: 11/15/2022] Open
Abstract
Due to the high-level of metastatic and relapsed rates, rhabdomyosarcoma (RD) patients have a poor prognosis, and novel treatment strategies are required. Thereby, the present study evaluated the efficacy of PFK15, a PFKFB3 inhibitor, in RD cells to explore its potential underlying mechanism on the regulation of autophagy and proliferation in these cells. The effects of PFK15 on cell viability loss and cell death in different treatment groups, were evaluated by MTS assay, colony growth assay and immunoblotting, respectively. In addition, the autophagy levels were detected by electron microscopy, fluorescence microscopy and immunoblotting following PFK15 treatment, and the autophagic flux was analyzed with the addition of chloroquine diphosphate salt or by monitoring the level of p62. PFK15 was observed to evidently decrease the viability of RD cells, inhibit the colony growth and cause abnormal nuclear morphology. Furthermore, PFK15 inhibited the autophagic flux and cell proliferation, as well as induced apoptotic cell death in RD cells through downregulation of the adenosine monophosphate‑activated protein kinase (AMPK) signaling pathway. An AMPK agonist rescued the inhibited cell proliferation and autophagy induced by PFK15. In conclusion, PFK15 inhibits autophagy and cell proliferation via downregulating the AMPK signaling pathway in RD cells.
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Affiliation(s)
- Chunhui Wang
- Department of Orthopedics, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, Shandong 250021
| | - Jianghong Qu
- Department of Gynecology, Zhangqiu People's Hospital, Jinan, Shandong 250200
| | - Siyuan Yan
- State Key Laboratory of Mycology, Institute of Microbiology, Chinese Academy of Sciences, Beijing 100101
| | - Quan Gao
- State Key Laboratory of Mycology, Institute of Microbiology, Chinese Academy of Sciences, Beijing 100101
| | - Sibin Hao
- Department of Orthopedics, Zhangqiu People's Hospital, Jinan, Shandong 250200, P.R. China
| | - Dongsheng Zhou
- Department of Orthopedics, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, Shandong 250021
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15
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Häußler SM, Stromberger C, Olze H, Seifert G, Knopke S, Böttcher A. Head and neck rhabdomyosarcoma in children: a 20-year retrospective study at a tertiary referral center. J Cancer Res Clin Oncol 2018; 144:371-379. [PMID: 29143871 DOI: 10.1007/s00432-017-2544-x] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2017] [Accepted: 11/07/2017] [Indexed: 11/26/2022]
Abstract
OBJECTIVE The purpose of this study was to assess rhabdomyosarcomas (RMS) of the head and neck in pediatric patients with regard to clinical presentation, treatment, and survival. METHODS Data were retrospectively obtained from patient charts with regard to RMS of the head and neck diagnosed between 1996 and 2016 at a tertiary referral center. Clinical course, treatment modalities, and side effects were analyzed. Survival analysis was carried out using the Kaplan-Meier method. RESULTS Twenty-eight patients (17 male, 11 female) with a mean age at diagnosis of 6.8 ± 5.0 years have been included. Fourteen patients (50%) presented with painless swelling in the head and neck region and nine patients (32.1%) presented with cranial nerve deficit at initial diagnosis. The location of the majority of rhabdomyosarcomas was orbital (N = 9; 32.1%), followed by parapharyngeal (n = 4; 14.2%) and sinonasal (n = 3; 10.7%). All patients (n = 28; 100%) received polychemotherapy, additional adjuvant radiation therapy was carried out in 24 patients (85.7%), and surgery was performed in 12 cases (42.9%). The 5-year overall survival was 91.3%, and median progression-free survival was 46 ± 67.4 months. Common side effects resulting from chemotherapy and radiation therapy included neutropenia, mucositis, nausea and vomiting, and radiodermatitis, long-term side effects included microsomia and facial mutilation. CONCLUSION Multimodality treatment of rhabdomyosarcomas of the head and neck in pediatric patients may lead to a 5-year overall survival of up to 91.3%.
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Affiliation(s)
- Sophia Marie Häußler
- Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Augustenbuger Platz 1, 13353, Berlin, Germany.
| | - Carmen Stromberger
- Department of Radiation Oncology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany
| | - Heidi Olze
- Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Augustenbuger Platz 1, 13353, Berlin, Germany
- Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Charité Mitte, Berlin, Germany
| | - Georg Seifert
- Department of Pediatrics, Division of Oncology and Hematology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Berlin, Germany
| | - Steffen Knopke
- Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Virchow-Klinikum, Augustenbuger Platz 1, 13353, Berlin, Germany
| | - Arne Böttcher
- Department of Otorhinolaryngology, Head and Neck Surgery and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
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Abstract
Pediatric sarcomas are a heterogeneous group of tumors accounting for approximately 10% of childhood solid tumors. Treatment is focused on multimodality therapy, which has improved the prognosis over the past two decades. Current regimens focus on decreasing treatment for low-risk patients to decrease the long-term side effects while maximizing therapy for patients with metastatic disease to improve survival. Pediatric sarcomas can be divided into soft tissue sarcomas and osseous tumors. Soft tissue sarcomas are further delineated into rhabdomyosarcomas, which affect young children and nonrhabdomyosarcomas, which are most common in adolescents. The most common bone sarcomas are osteosarcomas and Ewing's sarcoma.
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17
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Mixed Botryoid and Spindle Cell Bladder Rhabdomyosarcoma: An Outstanding Pediatric Case. Case Rep Pediatr 2017; 2017:8397826. [PMID: 28116202 PMCID: PMC5237453 DOI: 10.1155/2017/8397826] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2016] [Accepted: 11/24/2016] [Indexed: 11/21/2022] Open
Abstract
We report a case of a 3-year-old North African child, initially assessed for nonspecific urinary symptoms such as haematuria and burning urination. The ultrasound evaluation showed a vegetating mass occupying the lumen with weak vascular signs at the Colour-Doppler evaluation. An explorative cystoscopy was performed and it revealed a nonbleeding lesion, white in colour, pedunculated, projecting into the lumen, and associated with a brown satellite formation. Histological examination showed a mixed Botryoid and Spindle Cell Rhabdomyosarcoma. This mixed histology has not been described before and no statistical data are reported in literature so far. Despite the Embryonal Rhabdomyosarcoma variant being the most common, the association characterized by two histological Rhabdomyosarcoma subtypes such as Botryoid and Spindle Cell is rarely observed and it is important to get an accurate histological diagnosis in order to immediately start the correct treatment protocol.
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18
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Ka S, Gnangnon F, Dieng MM, Diouf D, Thiam J, Gaye PM, Dem A. Embryonal rhabdomyosarcoma of the perineum in an adult: a case report. J Med Case Rep 2016; 10:353. [PMID: 27998313 PMCID: PMC5175321 DOI: 10.1186/s13256-016-1166-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2015] [Accepted: 12/01/2016] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND We report the case of an adult patient with embryonal rhabdomyosarcoma of the perineum admitted to our practice at Joliot Curie Institute in Dakar. It is a rare tumor at this age and has a bad prognosis at this localization. CASE PRESENTATION We describe the case of a 22-year-old African man admitted for a perineal mass that had evolved over 6 months. He complained of tenesmus, obstinate constipation, and dysuria. A clinical examination revealed perineal swelling spread over his anus, scrotum, penis, testicles, and inguinal lymph nodes. A perineal ultrasound and computed tomography scan showed a large mass driving his testicles forward with regional lymph node metastases. An ultrasound-guided biopsy showed embryonal rhabdomyosarcoma on histology and immunohistochemistry, with strong positivity of neural cell adhesion molecule and myogenin while results for cytokeratin AE1/AE3, cluster of differentiation 45, synaptophysin, and chromogranin were negative. Our patient was classified T2N1M1. Outcome was quickly marked by occlusive syndrome and colostomy. Our patient did not opt for chemotherapy and died after 6 months of follow-up. CONCLUSIONS The embryonic RMS of the adult is a rare disease. Despite the sensitivity to chemotherapy and surgery. Localization to perineum remains poor prognosis.
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Affiliation(s)
- Sidy Ka
- Joliot Curie Institute, Dakar, Senegal.
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19
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Rabdomiosarcoma infantil. RADIOLOGIA 2016; 58:481-490. [DOI: 10.1016/j.rx.2016.09.003] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2016] [Revised: 08/20/2016] [Accepted: 09/14/2016] [Indexed: 12/31/2022]
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20
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Radzikowska J, Kukwa W, Kukwa A, Czarnecka AM, Kawecki M, Lian F, Szczylik C, Krzeski A. Management of pediatric head and neck rhabdomyosarcoma: A case-series of 36 patients. Oncol Lett 2016; 12:3555-3562. [PMID: 27900036 PMCID: PMC5104052 DOI: 10.3892/ol.2016.5072] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2015] [Accepted: 05/19/2016] [Indexed: 01/14/2023] Open
Abstract
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric population. In 35% of cases, RMS develops in the head and neck (H&N) region, and only combined therapy is recognized as a curative treatment. However, recent advances in skull base and reconstructive surgery, along with microsurgery and endoscopic surgery, have strengthened the role of surgery as an important part of RMS treatment. In the present study, 36 pediatric RMS cases (24 males and 12 females) were analyzed after surgical treatment. The average age at diagnosis was 7 years. In total, 67% of tumors were localized in the parameningeal region. Alveolar RMS was the most common histopathological type. A total of 16 patients were treated due to disease recurrence or a previous non-radical surgical procedure, while 19 cases had inductive chemotherapy and/or radiotherapy preceding surgical treatment due to locally advanced disease. In 1 case, only diagnostic biopsy was performed. It is recommended that the management of H&N RMS is interdisciplinary from the beginning. Extensive surgical dissection in the H&N region for RMS may result in severe cosmetic defects and functional impairment; thus, these risks should be considered during treatment planning, and the surgical approach should be based on the individual characteristics of each patient.
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Affiliation(s)
- Joanna Radzikowska
- Department of Otorhinolaryngology, Faculty of Medicine and Dentistry, Medical University of Warsaw, Warsaw 00-739, Poland
| | - Wojciech Kukwa
- Department of Otorhinolaryngology, Faculty of Medicine and Dentistry, Medical University of Warsaw, Warsaw 00-739, Poland
| | - Andrzej Kukwa
- Department of Otolaryngology and Head and Neck Disease, University of Varmia and Mazury, Olsztyn 10-082, Poland
| | - Anna M. Czarnecka
- Department of Oncology and Molecular Oncology Laboratory, Military Institute of Medicine,Warsaw 04-141, Poland
| | - Maciej Kawecki
- Department of Oncology and Molecular Oncology Laboratory, Military Institute of Medicine,Warsaw 04-141, Poland
| | - Fei Lian
- Department of Urology, Emory University School of Medicine, Atlanta, GA 30322, USA
| | - Cezary Szczylik
- Department of Oncology and Molecular Oncology Laboratory, Military Institute of Medicine,Warsaw 04-141, Poland
| | - Antoni Krzeski
- Department of Otorhinolaryngology, Faculty of Medicine and Dentistry, Medical University of Warsaw, Warsaw 00-739, Poland
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21
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[Rhabdomyosarcoma of adult genital tract: A short review]. ACTA ACUST UNITED AC 2016; 45:821-826. [PMID: 27212613 DOI: 10.1016/j.jgyn.2016.03.011] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2016] [Revised: 03/22/2016] [Accepted: 03/22/2016] [Indexed: 11/24/2022]
Abstract
Rhabdomyosarcoma, most common soft tissue tumor in children, represent 8% of solid tumors in children. Conversely, in adults, this histology is very rare and no consensual recommendation is supported. If gynecological localization is one of the most frequent in children, it is a minority in adults. The management of this type of tumor is based on treatment multimodality combining surgery, chemotherapy, radiotherapy and brachytherapy. This pathological separate entity differs from other sarcomas by its greater sensitivity to chemotherapy and radiotherapy. The aim of this study is to conduct a general review of diagnostic and treatment of genital tract rhabdomyosarcoma in adults, and to report pathological characteristics of this type of tumor.
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22
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Ruiz-Mesa C, Goldberg JM, Coronado Munoz AJ, Dumont SN, Trent JC. Rhabdomyosarcoma in adults: new perspectives on therapy. Curr Treat Options Oncol 2016; 16:27. [PMID: 25975442 DOI: 10.1007/s11864-015-0342-8] [Citation(s) in RCA: 43] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
OPINION STATEMENT Rhabdomyosarcoma (RMS) is well known as a pediatric disease. Most of the knowledge, like biology, genetics, and treatments of this disease, comes from studies done in that age group. The two subtypes of RMS, embryonic RMS and alveolar RMS, that affect mainly the pediatric population are well described in the literature and that has had an impact on the improvement in overall survival during the past 20 years. RMS in the adult population has a low incidence, therefor the study of RMS in this group is challenging. Pleomorphic RMS is the subtype that mainly affects adults and its biology and genetics are not yet completely understood and described. The risk factors for this tumor and the differences among adults and children is also poorly understood. The treatments for adults that have RMS are not standardized having an impact on the overall survival. Pleomorphic RMS has, compared to other adult sarcomas, poor overall survival. Adult patients with RMS have poor prognosis. The standardization of treatments for the adult population is necessary as maybe new treatments for this specific group. There are new treatment options that are being studied mostly in pediatrics and young adults. Immunotherapy is currently proposed as an important treatment possibility including different techniques like vaccination, antigen-mediated therapy, and immune checkpoints. Even if we have a better understanding of RMS, there are still unanswered questions. The improvements seen in the pediatric population are encouraging, but there is still the need to enhance better therapies for adults with RMS.
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Affiliation(s)
- Catalina Ruiz-Mesa
- Department of Pediatrics, University of Miami/Holtz Children's Hospital at Jackson Health System, 1611 NW 12th Ave, Holtz Hospital 6th floor, Miami, FL, 33136, USA,
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23
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24
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Sangkhathat S. Current management of pediatric soft tissue sarcomas. World J Clin Pediatr 2015; 4:94-105. [PMID: 26566481 PMCID: PMC4637813 DOI: 10.5409/wjcp.v4.i4.94] [Citation(s) in RCA: 36] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/29/2015] [Revised: 07/21/2015] [Accepted: 09/29/2015] [Indexed: 02/06/2023] Open
Abstract
Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma (RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.
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25
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Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. Case Rep Pediatr 2014; 2014:792925. [PMID: 25405050 PMCID: PMC4227373 DOI: 10.1155/2014/792925] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2014] [Revised: 10/07/2014] [Accepted: 10/10/2014] [Indexed: 01/28/2023] Open
Abstract
Pediatric soft tissue sarcomas account for approximately 10% of all pediatric malignancies. Malignant ectomesenchymoma is rare biphasic sarcomas consisting of both mesenchymal and neuroectodermal elements. Approximately 64 cases have been reported in the literature and are believed to arise from pluripotent embryologic migratory neural crest cells. We report a 4-year-old boy who initially presented with a pelvic mass and inguinal lymphadenopathy at 6 months of age. Inguinal lymph node biopsy revealed a distinct biphasic tumor with microscopic and immunophenotypic characteristics diagnostic for both alveolar rhabdomyosarcoma and poorly differentiated neuroblastoma. The patient received national protocol chemotherapy against rhabdomyosarcoma with good response and presented with a cerebellar mass 21 months later. The metastatic tumor revealed sheets of primitive tumor cells and diagnostic areas of rhabdomyosarcoma and neuroblastoma were identified only by immunohistochemistry. Cytogenetic analysis of metastatic tumor demonstrated complex karyotype with multiple chromosomal deletions and duplications. The patient received national protocol chemotherapy against neuroblastoma and adjuvant radiotherapy after surgical resection of the cerebellar tumor with good response. He is currently off from any treatment for 18 months with no evidence of tumor recurrence or metastasis.
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26
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Muranjan M, Karande S, Parikh S, Sankhe S. A mistaken identity: rhabdomyosarcoma of the middle ear cleft misdiagnosed as chronic suppurative otitis media with temporal lobe abscess. BMJ Case Rep 2014; 2014:bcr-2014-206615. [PMID: 25240007 DOI: 10.1136/bcr-2014-206615] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/12/2023] Open
Abstract
A 5-year-old girl presented with a 3-month history of left side facial palsy, followed sequentially by purulent ear discharge, complete external ophthalmoplaegia and blurred vision. On clinical examination she was febrile with left-sided conductive hearing loss. She was clinically diagnosed to have chronic suppurative otitis media of the unsafe type with petrous apicitis, middle cranial fossa abscess and cavernous sinus involvement. Preliminary CT scan findings were reported as a large left temporal lobe abscess and left otitis media with cholesteatoma. MRI of the brain obtained later corroborated the abnormalities detected on the CT scan. Ten days after admission, a mass was seen protruding from the external auditory canal. A biopsy of the mass was obtained and sent for histopathological examination. Meanwhile, review of the MRI suggested an aggressive neoplasm such as sarcoma/rhabdomyosarcoma. Histopathology clinched the final diagnosis of an anaplastic type of embryonal rhabdomyosarcoma of the middle ear cleft.
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Affiliation(s)
- Mamta Muranjan
- Department of Pediatrics, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, India
| | - Sunil Karande
- Department of Pediatrics, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, India
| | - Shefali Parikh
- Department of Pediatrics, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, India
| | - Shilpa Sankhe
- Department of Radiology, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, India
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27
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Ultrasound of the paediatric urogenital tract. Eur J Radiol 2014; 83:1538-48. [DOI: 10.1016/j.ejrad.2014.04.001] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/25/2014] [Accepted: 04/07/2014] [Indexed: 02/04/2023]
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28
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Sepúlveda I, Spencer ML, Cabezas C, Platino MO, Schorwer M, Ortega P, Ulloa D. Orbito-ethmoidal rhabdomyosarcoma in an adult patient: a case report and review of the literature. Case Rep Oncol 2014; 7:513-21. [PMID: 25232320 PMCID: PMC4164072 DOI: 10.1159/000365547] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023] Open
Abstract
We report a patient who presented to the ENT service complaining of nasal obstruction, exophthalmos, edema and ipsilateral facial congestion. Imaging studies revealed an aggressive noncalcified solid mass centered in the left nasoethmoidal region and heterogeneous avid enhancement following contrast media injection. Subsequently, a biopsy confirmed the presence of solid alveolar rhabdomyosarcoma. The patient was treated with chemoradiation therapy for 7 weeks. Due to the advanced stage of the disease, the patient was enrolled in a palliative care and pain control program.
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Affiliation(s)
- Ilson Sepúlveda
- Otolaryngology - Head and Neck Surgery Service, USA ; Faculty of Dentistry, Finis Terrae University, Santiago, Chile, USA
| | - M Loreto Spencer
- Pathology Department, General Hospital of Concepción, USA ; Faculty of Dentistry, Finis Terrae University, Santiago, Chile, USA
| | - Claudia Cabezas
- Pathology Department, General Hospital of Concepción, USA ; Católica de la Santísima Concepción University, USA
| | - Maria Olga Platino
- Health Science Center at Houston, University of Texas, Houston, Tex., USA
| | - Max Schorwer
- Oncology Service, General Hospital of Concepción, USA
| | - Pablo Ortega
- Otolaryngology - Head and Neck Surgery Service, USA
| | - David Ulloa
- School of Medicine, San Sebastián University, Concepción, USA
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29
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Egas-Bejar D, Huh WW. Rhabdomyosarcoma in adolescent and young adult patients: current perspectives. ADOLESCENT HEALTH MEDICINE AND THERAPEUTICS 2014; 5:115-25. [PMID: 24966711 PMCID: PMC4069040 DOI: 10.2147/ahmt.s44582] [Citation(s) in RCA: 74] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
Rhabdomyosarcoma (RMS), a malignant tumor of mesenchymal origin, is the third most common extracranial malignant solid tumor in children and adolescents. However, in adults, RMS represents <1% of all solid tumor malignancies. The embryonal and alveolar histologic variants are more commonly seen in pediatric patients, while the pleomorphic variant is rare in children and seen more often in adults. Advances in the research of the embryonal and alveolar variants have improved our understanding of certain genes and biologic pathways that are involved in RMS, but much less is known for the other variants. Multimodality therapy that includes surgery and chemotherapy with or without radiation therapy is the mainstay of treatment for RMS. Improvements in the risk stratification of the pediatric patients based on presurgical (primary tumor site, tumor size, regional lymph node involvement, presence of metastasis) and postsurgical parameters (completeness of resection or presence of residual disease or metastasis) has allowed for the treatment assignment of patients in different studies and therapeutic trials, leading to increases in 5-year survival from 25%–70% over the past 40 years. However, for adult patients, in great part due to rarity of the disease and the lack of consensus on optimal treatment, clinical outcome is still poor. Many factors have been implicated for the differing outcomes between pediatric RMS versus adult RMS, such as the lack of standardized treatment protocols for adult RMS patients and the increased prevalence of advanced presentations. Now that there are increased numbers of survivors, we can appreciate the sequelae from therapy in these patients, such as bone growth abnormalities, endocrinopathies, and infertility. Improvements in risk stratification have led to clinical trials using lower doses of chemotherapy or radiation therapy with the intention of decreasing the incidence of side effects without compromising survival outcome.
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Affiliation(s)
- Daniela Egas-Bejar
- Division of Pediatrics, The Children's Cancer Hospital, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Winston W Huh
- Division of Pediatrics, The Children's Cancer Hospital, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
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30
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Terwisscha van Scheltinga CEJ, Spronk P, van Rosmalen J, Wijnen MHWA, Heij HA, van Baren R, Merks JHM, van Noesel MM, Wijnen RMH. Diagnosis and treatment of lymph node metastases in pediatric rhabdomyosarcoma in the Netherlands: a retrospective analysis. J Pediatr Surg 2014; 49:416-9. [PMID: 24650468 DOI: 10.1016/j.jpedsurg.2013.08.002] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2013] [Revised: 07/09/2013] [Accepted: 08/05/2013] [Indexed: 12/16/2022]
Abstract
BACKGROUND In pediatric rhabdomyosarcoma (RMS), evaluation of lymph node involvement (N1) is an important staging aspect, but difficult to assess. The aim of our study was to evaluate the assessment of lymph node infiltration and impact on outcome in N1 RMS patients. METHODS We identified 277 non-metastatic RMS patients diagnosed and treated between 1990 and 2008. Patients with recorded N1 disease were evaluated for their diagnostic procedures and outcome. RESULTS In 13.7% N1 status was reported. In 19 of 34 N1 patients, lymph node biopsies were performed for histologically confirmation. Different treatment modalities were used to treat lymph node metastases. In total 23 of 31 patients received local treatment of the node (11/23 RT, 4/23 surgery, and 8/23 both). All patients received chemotherapy. Lymph node relapse occurred in 7 of 31 patients who were treated with one or two modalities. Only 1 (14%) of 8 patients treated with three modalities relapsed. In N0 patients 10 (4.2%) of 239 had a regional lymph node relapse, and 9 of 10 died. CONCLUSION Lymph node metastases are an essential part of staging. Node positivity contributes to relapse of disease. Nodal relapse is also associated with a high mortality rate. These data imply that nodal assessment needs to be optimal and standardized for improved staging.
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Affiliation(s)
| | - Pauline Spronk
- Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands
| | | | - Marc H W A Wijnen
- Department of Pediatric Surgery, UMCN, Nijmegen and AMC, Amsterdam, the Netherlands; Department of Pediatric Surgery, AMC, Amsterdam, the Netherlands
| | - Hugo A Heij
- Department of Pediatric Surgery, UMCN, Nijmegen and AMC, Amsterdam, the Netherlands
| | | | | | - Max M van Noesel
- Department of Pediatric Oncology/Hematology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.
| | - Rene M H Wijnen
- Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands
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Dénes FT, Duarte RJ, Cristófani LM, Lopes RI. Pediatric genitourinary oncology. Front Pediatr 2013; 1:48. [PMID: 24400293 PMCID: PMC3864259 DOI: 10.3389/fped.2013.00048] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2013] [Accepted: 12/02/2013] [Indexed: 02/03/2023] Open
Abstract
Tumors of the kidney, bladder, prostate, testis, and adrenal represent a large part of the adult urologic practice, but are relatively infrequent in children. The natural history and management of these tumors in the pediatric age is different from that of the adults. As result of the successful work of several clinical trial groups in recent decades, there has been a significant improvement in their cure rates. The aim of this article is to review their most significant clinical aspects, as well as to present an update in their management.
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Affiliation(s)
- Francisco Tibor Dénes
- Uropediatric Unit, Division of Urology, Hospital das Clínicas, University of São Paulo , São Paulo , Brazil
| | - Ricardo Jordão Duarte
- Uropediatric Unit, Division of Urology, Hospital das Clínicas, University of São Paulo , São Paulo , Brazil
| | - Lílian Maria Cristófani
- Pediatric Onco-Hematology Unit, Department of Pediatrics, Hospital das Clínicas, University of São Paulo , São Paulo , Brazil
| | - Roberto Iglesias Lopes
- Uropediatric Unit, Division of Urology, Hospital das Clínicas, University of São Paulo , São Paulo , Brazil
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Stein R, Frees S, Schröder A, Russo A, Gutjahr P, Faber J, Thüroff JW. Radical surgery and different types of urinary diversion in patients with rhabdomyosarcoma of bladder or prostate--a single institution experience. J Pediatr Urol 2013; 9:932-9. [PMID: 23385048 DOI: 10.1016/j.jpurol.2013.01.008] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/09/2012] [Accepted: 01/03/2013] [Indexed: 12/11/2022]
Abstract
PURPOSE In a retrospective study we analyzed the outcome of patients treated for rhabdomyosarcoma (RMS) of the bladder/prostate with special attention to radical surgery. METHODS In 25 patients with genitourinary RMS (15 bladder/10 prostate) the median age at diagnosis was 4 years [1-18], and 8 patients had a stage II RMS, 12 stage III and 5 stage IV. In 19/25 (12 bladder/7 prostate), radical surgery and urinary diversion were performed. Urinary diversion comprised 2 continent anal diversions, 11 continent cutaneous diversions, 4 colon conduits and 2 urethral diversions (2 + 3 years of age). In the younger child with urethral diversion, a cutaneous appendix stoma was additionally constructed in case of inability to void spontaneously. RESULTS 4/19 patients who underwent radical surgery died of metastatic RMS; 1 patient with neurofibromatosis died of a secondary tumor. After median follow-up of 132 months (14-420), 14 patients currently have no evidence of disease. 8/14 patients who survived developed 17 complications requiring operative revision. All patients with a continent diversion are continent. The patients with orthotopic bladder substitution are continent day & night and void spontaneously. CONCLUSION For RMS confined to the bladder or bladder neck, radical cystoprostatectomy and orthotopic bladder substitution are an option. Urethral diversion using the ileocecal segment (Mainz-pouch I) offers the advantage of utilizing the appendix as an additional continent cutaneous stoma, which enables parents to evacuate residual urine in young boys, until able to empty the pouch completely themselves. For all other patients with vital tumor after primary chemotherapy, cutaneous urinary diversion is an option. Long-term complication rates in this complex group of patients are acceptable.
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Affiliation(s)
- Raimund Stein
- Division of Pediatric Urology, Department of Urology, University of Mainz, Medical School, Mainz, Germany.
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Kojima Y, Hashimoto K, Ando M, Yonemori K, Hirakawa A, Kodaira M, Yunokawa M, Shimizu C, Tamura K, Katsumata N, Hosono A, Makimoto A, Fujiwara Y. Clinical outcomes of adult and childhood rhabdomyosarcoma treated with vincristine, d-actinomycin, and cyclophosphamide chemotherapy. J Cancer Res Clin Oncol 2012; 138:1249-57. [PMID: 22441933 DOI: 10.1007/s00432-012-1199-x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2012] [Accepted: 03/06/2012] [Indexed: 10/28/2022]
Abstract
BACKGROUND Outcomes in adult patients with rhabdomyosarcoma are poor, with a 5-year survival rate of approximately 30 %. The current study aimed to compare the clinical outcomes of adult and childhood rhabdomyosarcoma patients with local and metastatic disease and to examine the impact and timing of local therapy on metastasis. METHODS Clinicopathological features and patient outcomes were reviewed retrospectively for rhabdomyosarcoma patients receiving chemotherapy between 1981 and 2010 at our institution. Adults were defined as those aged 21 years or older. RESULTS Of the 98 patients identified, 36 were adults (median age, 29; range, 21-72) and 62 were children (median age, 11; range, 0.6-20). Median progression-free survival of localized and metastatic disease for children and adults was as follows: localized disease, 166.9 versus 22.4 months (p = 0.005), and metastatic disease, 13.3 versus 13.3 months (p = 0.949), respectively. Multivariate regression analysis revealed that older age (≥ 21 vs. <21) was a significant poor prognostic factor in localized disease. Conversely, age was not related to survival in metastatic disease. Receiving radiotherapy to the primary site was an independent factor indicating a better prognosis. An analysis of the optimal timing of local therapy was performed for 53 patients; however, its significance on survival could not be determined. CONCLUSIONS Age was a negative prognostic factor in rhabdomyosarcoma patients with localized disease, but it did not affect the survival in metastatic disease. For metastatic disease, although local therapies may be effective for survival, the timing of such therapies should be determined individually.
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Affiliation(s)
- Yuki Kojima
- Department of Medical Oncology and Breast Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan
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Abstract
Rhabdomyosarcoma (RMS) is a malignant childhood tumor of mesenchymal origin that currently has a greater than 70% overall 5-year survival. Multimodality treatment is determined by risk stratification according to pretreatment stage, postoperative group, histology, and site of the primary tumor. Pretreatment staging is dependent on primary tumor site, size, regional lymph node status, and presence of metastases. Unique to RMS is the concept of postoperative clinical grouping that assesses the completeness of disease resection and takes into account lymph node evaluation. At all tumor sites, the clinical grouping, and therefore completeness of resection, is an independent predictor of outcome. Overall, the prognosis for RMS is dependent on primary tumor site, patient age, completeness of resection, extent of disease, including the presence and number of metastatic sites and histology and biology of the tumor cells. Therefore, the surgeon plays a vital role in RMS by contributing to risk stratification for treatment, local control of the primary tumor, and outcome. The current state-of-the-art treatment is determined by treatment protocols developed by the Soft Tissue Sarcoma Committee of the children's Oncology Group.
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Affiliation(s)
- Roshni Dasgupta
- Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
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Ramírez-Peinado S, Alcázar-Limones F, Lagares-Tena L, El Mjiyad N, Caro-Maldonado A, Tirado OM, Muñoz-Pinedo C. 2-Deoxyglucose Induces Noxa-Dependent Apoptosis in Alveolar Rhabdomyosarcoma. Cancer Res 2011; 71:6796-806. [DOI: 10.1158/0008-5472.can-11-0759] [Citation(s) in RCA: 72] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Bolger JC, Walsh JC, Hughes RE, Eustace SJ, Harrington P. Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review. Foot Ankle Surg 2010; 16:e51-4. [PMID: 20654999 DOI: 10.1016/j.fas.2010.03.003] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2009] [Revised: 02/16/2010] [Accepted: 03/02/2010] [Indexed: 02/04/2023]
Abstract
We report a case of alveolar rhabdomyosarcoma arising between the fourth and fifth metatarsal. A 13-year-old boy presented to outpatients with a history of pain and swelling in the lateral aspect of his left forefoot. Plain radiographs and MRI showed a soft tissue mass displacing the fourth metatarsal. Percutaneous biopsy revealed an alveolar rhabdomyosarcoma. Staging scans showed advanced metastatic disease. The patient was treated with chemotherapy. This highly malignant lesion remains challenging to diagnose, and difficult to treat successfully.
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Affiliation(s)
- J C Bolger
- Department of Surgery, Beaumont University Hospital, Beaumont, Dublin 9, Ireland.
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Abstract
PURPOSE OF REVIEW Although head and neck masses represent a common entity in children, malignancy is uncommon. The otolaryngologist may be the first physician to see these children, and early recognition of malignancy is of obvious importance. This review aims to discuss the cause, diagnosis, investigation, treatment options, and prognosis for the most common head and neck malignancies of childhood. RECENT FINDINGS Over recent years, significant developments have been made in characterizing the epidemiologic, phenotypic, and genotypic variability of childhood head and neck cancers. Improved awareness of tumor biology is reflected by more sophisticated diagnostics, estimates of prognosis, and an increasing individualization of treatment regimens. SUMMARY The latest evidence for the diagnosis and management of childhood head and neck malignancy is summarized. The rarity of these tumors inevitably results in a paucity of high-level evidence to guide treatment. A combination of translational research from tumor biology studies, multicenter clinical trials, and smaller case series and case reports will continue to guide new advances in diagnosis and treatment.
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Nazarian-Mobin SS, Simms K, Urata MM, Tarczy-Hornoch K, Hammoudeh JA. Misleading presentation of an orbital lymphangioma. ACTA ACUST UNITED AC 2009; 109:e82-5. [PMID: 20031456 DOI: 10.1016/j.tripleo.2009.08.042] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2009] [Accepted: 08/31/2009] [Indexed: 11/26/2022]
Abstract
We describe a 2-year-old patient with an orbital lymphangioma who presented with massive proptosis. We highlight the importance of magnetic resonance imaging in evaluating such patients, because physical examination can be misleading. We also demonstrate the difficulties of managing recurrent hemorrhages when only subtotal excision is possible, as is frequently the case.
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Affiliation(s)
- Sheila S Nazarian-Mobin
- Division of Plastic Surgery, University of Southern California, Children's Hospital Los Angeles, Los Angeles, California 90027, USA.
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