Clinical analysis of 5 adult laryngeal rhabdomyosarcoma / 临床耳鼻咽喉头颈外科杂志

Objective:To explore the clinical manifestations,the type of pathology, treatment and prognosis of laryngeal rhabdomyosarcoma, and to enhance the understanding of the clinical characteristics of the disease, while improving the diagnosis rateand reducing the misdiagnosis rate, in order to explore effective diagnosis and treatment methods. Methods:A retrospective analysis was conducted on the clinical data of 5 cases of laryngeal rhabdomyosarcoma treated in the First Affiliated Hospital of Zhengzhou University from May 2015 to May 2021. Results:All 5 cases of laryngeal rhabdomyosarcoma were misdiagnosed in the early stage. with tumors mostly occurring in the vocal cords and appearing as smooth mass. The clinical symptoms were mostly hoarseness. According to pathological classification, three cases were embryonic type, one case was polymorphic type, and one case was spindle type.Three patients died due to tumor recurrence, one patient had multiple systemic metastases, and another patient who underwent surgical resection in the early stage and supplemented with postoperative radiotherapy and chemotherapyhas been followed up to date without recurrence. Conclusion:Laryngeal rhabdomyosarcoma has low incidence rate, high malignancy degree and poor prognosis. It is easy to be misdiagnosed as a benign mass. Extensive surgical resection combined with radiotherapy and chemotherapy should be performed as soon as possible after diagnosis.

Clinical analysis of testicular rhabdomyosarcoma / 北京大学学报(医学版)

Testicular rhabdomyosarcoma is relatively rare in testicular tumors, but the age of patient is relatively young and the degree of malignancy is high. Therefore, this article introduces 4 cases of testicular rhabdomyosarcoma who were admitted to Peking University Third Hospital from May 1994 to February 2019, and reviews the literature to improve the diagnosis and treatment of this disease. The average age of the 4 patients was 17.5 years (14-21 years), the average hospital stay was 22.0 d (17-31 d), and the average body mass index was 19.6 kg/m2 (14.7-25.8 kg/m2). All the patients underwent routine preoperative blood and urine routine, biochemical tests, as well as serum tumor markers. Preoperative examinations also included chest radiograph, electrocardiogram, ultrasound of the scrotum and groin, and abdominal enhanced CT. Lung CT or other examinations were performed if necessary. The median serum human chorionic gonadotropin (HCG) of the 4 patients was 0.20 IU/L (0.06-0.86 IU/L) (all normal), and the median serum alpha-fetoprotein (AFP) was 1.03 g/L (0.65-1.66 g/L) (all normal). The average maximum diameter of the tumor was 10.0 cm (4.5-15.0 cm). Testicular rhabdomyosarcoma was mainly diagnosed by pathology. The main treatment was radical orchiectomy combined with retroperitoneal lymph node dissection, with or without postoperative adjuvant chemotherapy. The clinical manifestations of the patients with testicular rhabdomyosarcoma had no specific characteristics, but most patients were young at onset with mainly painless masses in the testicles, which were already large when they were found. Patients with testicular rhabdomyosarcoma have a poor prognosis, most of whom recur within two years. Because of the small number of cases of testicular rhabdomyosarcoma, there is no standard treatment currently. It is recommended that patients with testicular rhabdomyosarcoma undergo radical testicular resection combined with retroperitoneal lymph node dissection. Retroperitoneal lymph node metastasis is an important prognostic factor, and patients with postoperative adjuvant chemotherapy can still survive for a longer time. If local recurrence or limited metastasis is found after operation, local resection and salvage radiotherapy are feasible.

Sarcomas de partes blandas en pacientes menores de 2 años. Serie de casos / Soft tissue sarcomas in infants. Case series

Los sarcomas de partes blandas en menores de 2 años son infrecuentes. Durante 2007-2017, 445 pacientes con sarcomas de partes blandas fueron tratados en nuestra Institución; 6 (el 0,5 %) eran menores de 2 años. Se analizaron los resultados clínicos y oncológicos en este grupo. La edad media de diagnóstico fue 15 meses. Cuatro eran varones y 2, mujeres. El seguimiento promedio fue 29 meses. El fibrosarcoma (n = 4) fue la variedad más frecuente. Cinco fueron tratados con cirugía de conservación del miembro; al restante se le realizó amputación. Todos realizaron tratamiento adyuvante con quimioterapia. La supervivencia a 24 meses fue del 100 %. Dos pacientes presentaron recidiva local; ambos casos, antes de los 24 meses.El tratamiento quirúrgico asociado a la quimioterapia impresiona ser la mejor opción terapéutica. La proporción de recurrencia local es alta para este grupo de pacientes luego de la cirugía de conservación del miembro.

Soft tissue sarcomas in children under 2 years of age are infrequent. During 2007-2017, a total of 445 patients diagnosed with soft tissue sarcomas were treated at our institution, 6 (0.5 %) were under 2 years. We analysed clinical and oncologic outcomes in this select group. The mean age of diagnosis was 15 months. Four patients were male and 2 female. The mean follow-up time was 29 months. Fibrosarcoma (n = 4) was the most frequent diagnosis. Five patients were treated with limb salvage surgery, and the remaining one had to undergo amputation. All patients received adjuvant treatment with chemotherapy. The 24-month survival rate was 100 %. Two patients presented a local recurrence before 24-months follow-up. Surgical treatment associated with chemotherapy seems to be the best therapeutic option. Local recurrence rate after limb salvage surgery is high for this group of patients

Clinical, pathological and therapeutic findings on lingual rhabdomyosarcoma in a dog - case report / Achados clínicos, patológicos e terapêuticos do rabdomiossarcoma em cão - relato de caso

The biological behaviour of the tumours vary according to the species in which they occur, its location within the cavity, clinical stage and histopathological nature. Lingual neoplasms are generally uncommon in companion animals. Rhabdomyosarcomas are malignant, solid, aggressive formations with high metastatic potential. The clinical signs are variable and a definitive diagnosis can only be reached through histopathological analysis of biopsy and necropsy specimen. In some cases, immunohistochemical study may be needed to confirm the diagnosis. This paper aims to highlight important points about this uncommon condition in dogs, using a case report of lingual rhabdomyosarcoma, which showed no evidence of metastasis after diagnosis, nor of local recurrence after surgical excision with a wide safety margin. It was concluded that early diagnosis, the correct interpretation of the complementary tests and the appropriate therapeutic approach contributed to improving the quality of life and survival of the patient in question.(AU)

O comportamento biológico dos tumores varia de acordo com a espécie animal, a localização na cavidade, a fase clínica e a natureza histopatológica. As neoplasias orais são geralmente incomuns em animais de companhia. Os rabdomiossarcomas são formações malignas, sólidas e agressivas, com alto potencial metastático. Os sinais clínicos são variáveis e o diagnóstico definitivo só pode ser alcançado por meio da análise histopatológica do material colhido. Em alguns casos, o estudo imuno-histoquímico pode ser necessário para confirmar o diagnóstico. Este trabalho tem como objetivo destacar pontos importantes sobre essa condição incomum em cães, utilizando um relato de caso de rabdomiossarcoma lingual, no qual não se evidenciaram metástases após o diagnóstico, nem recorrência local após a excisão cirúrgica com ampla margem de segurança. Concluiu-se que o diagnóstico precoce, a correta interpretação dos exames complementares e a abordagem terapêutica adequada contribuíram para melhorar a qualidade de vida e a sobrevida do paciente em questão.(AU)

Advanced Orofacial Rhabdomyosarcoma: A Retrospective Study of 31 Cases

Abstract Introduction Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma encountered in childhood and adolescence. Early diagnosis of pediatric cases is critical to improving outcomes, especially when socioeconomic status and geographical access to specialist services can reduce opportunities for early cancer detection and treatment. Objective The objective of this study is to determine factors that can delay referral and treatment in specialist pediatric oncology center upon our population specificities. Methods This retrospective study involved 31 children between 2003 and 2013. Children affected by histologically confirmed RMS occurring as a primary lesion in the orofacial area were included. Results Themedian age was 8 4.22 years (range: 3months - 15 years). The male to female ratio was 1.8:1. Most of the patients had advanced stage disease at presentation (81.7% group had 3-4 pretreatment staging) with parameningeal involvement in 80.6% of the cases. The 2-year event-free survival rate was 17.7 7.8% for all the patients. Delay of admission to our unit and abandonment of treatment seem to be important factors for the dismal prognosis. Conclusion Patient's location, socioeconomic status and health care coverage have had an impact on longer delays in seeking care and on follow-up. More studies are needed for implementation of a better management practices and a better supportive care upon specificities of our population.

Rabdomiossarcoma de cabeça e pescoço: 24 casos e revisão da literatura / Rhabdomyosarcoma of the head and neck: 24 cases and literature review

O rabdomiossarcoma (RMS) é o tumor maligno de partes moles mais comum na infância, localizando-se principalmente na cabeça e pescoço. Apresenta comportamento clínico-biológico variado, devendo receber terapia individualizada. OBJETIVO: Descrever os dados de pacientes portadores de RMS de cabeça e pescoço diagnosticados e tratados em um hospital comparando-os aos da literatura. Forma de Estudo: Descritivo retrospectivo. MATERIAL E MÉTODO: Análise retrospectiva de dados de prontuários de 24 pacientes portadores de RMS de cabeça e pescoço diagnosticados e tratados em um hospital no período de 1994 a 2008. RESULTADOS: A média de idade foi de 7,79 anos. Quanto ao sexo, encontramos 54,17 por cento do sexo masculino e 45,83 por cento do sexo feminino. Todos os pacientes foram submetidos a quimioterapia (QT), sendo que 62,5 por cento destes também realizaram radioterapia (RT) e 16,67 por cento foram submetidos a cirurgia. Dos 24 pacientes, 8 (33,3 por cento) foram a óbito, 6 (25 por cento) encontravam-se livres de neoplasia e 2 ( 8,3 por cento) apresentaram recidiva do tumor. CONCLUSÃO: O RMS de cabeça e pescoço frequentemente se apresenta com sintomas inespecíficos. Terapia multimodal individualizada deve ser realizada, incluindo cirurgia, quimioterapia e radioterapia.

Rhabdomyosarcoma (RMS) is a malignant tumor of soft tissues, more common in childhood, mainly located in the head and neck. It presents varied clinical and biological behavior and requires individualized management. AIM: To describe information on patients with head and neck RMS diagnosed and treated in a hospital, and to compare them to results in the literature. Study design: Descriptive and retrospective. MATERIALS AND METHODS: A retrospective analysis of data from 24 patients with head and neck rhabdomyosarcoma diagnosed and treated in a hospital from 1994 to 2008. RESULTS: The mean age was 7.79 years. According to gender, 54.17 percent were males and 45.83 percent were female. All patients underwent chemotherapy (CT), 62.5 percent of them also underwent radiotherapy (RT) and 16.67 percent were submitted to surgery. Of the 24 patients, 8 (33.3 percent) died, 6 (25 percent) were found free of neoplasia and 2 (8.3 percent) experienced tumor recurrence. CONCLUSION: The RMS of the head and neck often presents with nonspecific symptoms. Individualized multimodal therapy should be performed for these patients, including surgery, chemotherapy and radiotherapy.

Paratesticular rhabdomyosarcoma

Paratesticular rhabdomyosarcoma occurs predominantly in children and adolescents. is a rare tumor and It's developped proximal to the tesicle. Clinical signs are like any intrascrotal tumor. Scrotal ultrasound, CT scan and tumor markers are necessary for the diagnostic and stadification of this cancer. Orchidectomy followed by chemotherapy and radiotherapy is the usual treatment. We report 3 cases of paratesticular rhabdomyosarcoma. We discuss the diagnosis and therapeutic attitudes, evolutionary aspects and prognostic factors of these tumors re also discussed

Rabdomiosarcoma de vía biliar en la infancia, su tratamiento con cirugía conservadora video-laparóscopica / Biliary rhabdomyosarcoma in children: treatment with video assisted laparoscopic surgery

La escasa frecuencia del rabdomiosarcoma del árbol biliar extrahepátco,ha impedido la estandarización de su tratamiento.Se presenta en este trabajo la metodología diagnóstica y terapéutica aplicada a una paciente de 3 años de edad,portadora de divha patología.Se pone énfasis es que es el primer paciente tratado por cirugía videolaparóscopica,exponiéndose los resultados de la misma.Se discuten los protocolos de tratamiento

Rabdomiossarcoma manifestando-se na boca / Rhabdomyosarcoma affecting the mouth

Rabdomiossarcoma é um tumor mesenquimal maligno de músculo esquelético que raramente afeta a cavidade oral. Microscopicamente apresenta vários padröes com células variando de fusiformes a arredondadas com núcleo excêntrico. A análise imuno-histoquímica mostra positividade para vimentina, desmina e actina muscular específica. Neste artigo, nós apresentamos um caso clínico de rabdomiossarcoma com um seguimento de sete anos

Rabdomiosarcoma de oído medio. Caso clínico / Embrionary rabdiomiosarcoma of the middle ear. Case report

Se reporta un caso de rabdomiosarcoma embrionario de oído medio con extensión a fosa media en un paciente femenino de 5 años de edad. Esta patología, aunque rara, se puede confundir clínicamente con una otitis media crónica. Es el segundo reporte en castellano y el primero en América Latina

Tumores a celulas pequeñas en niños y adolescentes: estudio imnohistoquimico en 28 casos / Annual reports 1994 / Small round blue cell tumors in children and young adults: an immunohistochemical study in 28 cases

El grupo de timores a "celulas pequeñas" denominado así debido a que histológicamente presentan en común un aspecto morfológico homogéneo, incluye una serie de tumores con características clínicas, pronósticas y terapéuticas diferentes, cuyo diagnóstico diferencial se establece por medio de técnicas especializadas como inmunohistoquímica, microscopía electrónica o biología molecular. Veintiocho biopsias de pacientes cuyas edades oscilan entre 1 año/8 meses y 18 años, con el diagnóstico histológico de "tumor a células pequeñas" fueron estudiadas por inmunohistoquímica para establecer el diagnóstico diferencial, mediante el uso de los siguientes anticuerpos monoclonales anti: antígeno de la membrana epitelial (EMA), antígeno leucocitario común CD45 (LCA), desmina, vimentina, proteína S-100, enolasa neuronal especifica (NSE), mioglobina, CD45RO (linfocitos T), CD20 (linfocitos B) y citoqueratina. Del total de muestras, 8 (29 por ciento) fueron rabdomiosarcomas, 7 (25 por ciento) linfomas no hodgkinianos, 7 (25 por ciento) tumores de origen neuroectodérmico, 3 (11 por ciento) neuroblastomas, 2 (7 por ciento) tumores de Ewing y 1 (3 por ciento) caso de tumor de origen neuroepitelial. Establecer el diagnóstico diferencial en los tumores a "células pequeñas" utilizando marcadores inmunológicos constituye un paso fundamental ya que permite definir la conducta terapéutica y a la vez establecer criterios pronósticos de acuerdo al tipo de tumor

Rhabdomyosarcoma mimicking Wilm's tumor

É apresentado um caso de rabdomiossarcoma pleomórfico em criança de seis anos envolvendo simultaneamente a musculatura da perna direita e o rim homolateral. O tumor renal distorcia o sistema pielocalicial e teve diagnóstico clínico de tumor de Wilms. Os autores discutem acêrca do possível sítio primário do tumor

Avanços em oncologia na última década e perspectivas futuras em tumores sólidos pediátricos / Advances in oncology on the last decada and future perspectives in pediatric solid tumors

Nos últimos anos o prognósticos das crianças com câncer melhorou sensivelmente. Isto foi devido a tratamentos padronizados por meio de grupos cooperativos. Atualmente säo conhecidos fatores prognósticos que permitem orientar o tratamento, reduzindo-os em casos de melhor prognóstico e intensificando-os nos casos de pior prognóstico. O objetivo atual näo é somente curar a criança mas curar com o menor efeito colateral

Rabdomiosarcoma orbitario con extensión a tejidos blandos extraorbitarios: presentación de un caso / Orbitary rhabdomyosarcoma invading extraorbitary soft tissues: report of a case

Se presenta el caso de un rabdomiosarcoma de órbita derecha con extensión a fosa temporal, en un niño de 4 años de edad. Se realizó diagnóstico histopatológico y fue enviado a otra institución donde radio y quimioterapia. La evolución después de un año ha sido favorable. Se discute la presentación clínica del rabdomiosarcoma y las medidas terapéuticas actuales

Rhabdomyosarcoma in Thai children.

Twenty-six cases of rhabdomyosarcoma diagnosed from a total of 845 Thai children with childhood malignancy who had been treated at the Department of Pediatrics Ramathibodi Hospital, from May 1970 to December 1982 were analyzed retrospectively. There were 16 boys and 10 girls, aged 3 months to 13 years old (mean age was 5.6 +/- 4.1 years old). The common type was the embryonal cell sarcoma (19 cases), 3 cases were undetermined, 2 cases of alveolar cell type and one each of the pleomorphic and undifferentiated cell type. Only 2 cases had stage II disease, 10 and 14 cases were stage III and IV respectively. The common locations were head and neck (10 cases), abdomen (8 cases including one in the uterus, scrotum and 3 in the urinary bladder), and in 7 cases in the extremities. One patient had small lesion at the buttock. The treatment consisted of surgery, radiation therapy, actinomycin, vincristine and cyclophosphamide. Eight cases were lost to follow-up, 10 cases were discharged in advanced stage after been treated for 6.2 +/- 4 months, 4 cases had no evidence of disease for 2 to 7.5 years, 4 cases died after 4 to 27 months of treatment. The poor result obtained was probably due to the late stages of the disease at diagnosis, and limitations in the use of effective medications.