GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm.

Primary Biatrial Cardiac Rhabdomyosarcoma.

Primary malignant neoplasms of the heart are rare. Cardiac rhabdomyosarcoma is the second most common primary sarcoma. We report a rare case of a 49-year-old woman with a huge biatrial cardiac rhabdomyosarcoma treated by performing surgical resection followed by salvage chemotherapy for local recurrence. Cardiac sarcoma that occupy both atria are extremely rare. Although the prognosis of cardiac rhabdomyosarcoma is dismal, surgical resection should be recommended as a first line therapy to clarify the diagnosis and to relieve symptoms associated with the tumor.

Primary biatrial cardiac Rhabdomyosarcoma

Abstract Primary malignant neoplasms of the heart are rare. Cardiac rhabdomyosarcoma is the second most common primary sarcoma. We report a rare case of a 49-year-old woman with a huge biatrial cardiac rhabdomyosarcoma treated by performing surgical resection followed by salvage chemotherapy for local recurrence. Cardiac sarcoma that occupy both atria are extremely rare. Although the prognosis of cardiac rhabdomyosarcoma is dismal, surgical resection should be recommended as a first line therapy to clarify the diagnosis and to relieve symptoms associated with the tumor.

Congenital Penile Rhabdomyosarcoma on FDG PET/CT.

Primary penile malignancy is uncommon. Congenital primary penile rhabdomyosarcoma diagnosed in an infant is extremely rare. We present the FDG PET/CT findings in a 1-month-old boy with penile mass. The images showed hypermetabolic lesion at the base of the penis and left inguinal lymph node. Pathological examination demonstrated rhabdomyosarcoma.

Malignant fibrous histiocytoma in a patient presenting with urinary system symptoms

Summary Malignant fibrous histiocytoma is a rare tumor. It is most commonly seen in individuals between the fifth and seventh decades of life, in extremities, and less frequently in the retroperitoneum. Although its etiology is not clearly known, radiotherapy, chemical agents, previous history of surgery, trauma and fracture, and Hodgkin lymphoma have been blamed. Leiomyosarcoma, liposarcoma and rhabdomyosarcoma should be taken into account in differential diagnosis. It is seen on computed tomography as a mass lesion with irregular borders and density similar to that of the surrounding muscle tissue. Necrotic and hemorrhagic components in the mass are characterized as heterogeneous low density areas. Fluid-fluid levels can be detected by computed tomography and magnetic resonance imaging.

Radiologic Response Assessment in Pediatric Soft Tissue Sarcoma: Computed-Assisted Volume Evaluation.

OBJECTIVES: To compare 3 methods of dimensional assessment, with particular attention to a new software assisted method of volume calculation, in soft tissue sarcoma, and to investigate the interobserver agreement and the intermethod agreement in chemotherapy response classification and resultant clinical repercussions. STUDY DESIGN: We studied 34 pediatric patients with nonmetastatic soft tissue sarcoma who had undergone only diagnostic biopsy. Tumor size was measured both at diagnosis and after induction chemotherapy by 3 observers and using 3 measurement methods: maximum axis (1 diameter), estimated volume (3 diameters), and computed volume (software-assisted volume calculation). We used overall concordance correlation coefficient and Bland-Altman statistical methods to assess interobserver agreement and overall concordance correlation coefficient and the κ Cohen coefficient to assess intermethod agreement. RESULTS: According to overall concordance correlation coefficient, the interobserver agreement was very high for each method, with a slight superiority of the software assisted method; this agreement was not confirmed in Bland-Altman plots for maximum axis and estimated volume methods. According to kappa coefficients, the intermethod agreement in chemotherapy response evaluation was poor. CONCLUSIONS: Computed volume was the most accurate method in soft tissue sarcoma tumor size assessment. One- and 3-dimensional methods are not concordant in chemotherapy response classification. In particular, the maximum axis method underestimates chemotherapy response and can lead to switching the chemotherapy regimen erroneously.

Malignant fibrous histiocytoma in a patient presenting with urinary system symptoms.

Malignant fibrous histiocytoma is a rare tumor. It is most commonly seen in individuals between the fifth and seventh decades of life, in extremities, and less frequently in the retroperitoneum. Although its etiology is not clearly known, radiotherapy, chemical agents, previous history of surgery, trauma and fracture, and Hodgkin lymphoma have been blamed. Leiomyosarcoma, liposarcoma and rhabdomyosarcoma should be taken into account in differential diagnosis. It is seen on computed tomography as a mass lesion with irregular borders and density similar to that of the surrounding muscle tissue. Necrotic and hemorrhagic components in the mass are characterized as heterogeneous low density areas. Fluid-fluid levels can be detected by computed tomography and magnetic resonance imaging.

Ultrasonografía en la patología vesical, no malformativa ni litiásica: experiencia de un hospital pediátrico / Sonographic diagnosis of tumoral and pseudotumoral bladder diseases in children

A fin de demostrar la utilidad del US en la patología vesical, presentando la experiencia en un hospital pediátrico de alta complejidad se evaluaron retrospectivamente 17 pacientes seleccionados de nuestra base de datos en Epi Info (1994-1998), que consultaron por polaquiuría, disuria, tenesmo, piuria, hematuria o hipertensión arterial. La ecografía se realizó con vejiga llena y vacía. Concluimos que las patologías vesicales pueden ser detectadas y seguidas evolutivamente mediante ultrasonidos y que su diagnóstico etiológico dependerá de la clínica y la anatomía patológica, cuando sea necesario (AU)

Rabdomiosarcoma de epididimo / Epididymal Rhabdomyosarcoma

Se presenta un tumor mesenquimatoso maligno originado en las estructuras paratesticulares, que corresponde a un radiomiosarcoma de epidismo. Se destaca su baja incidencia en la literatura mundial y su manejo terapeútico multidisciplinario (cirugia, radioterapia, quimioterapia). Fue intervenido en el Servicio de Urología de Antártida Hospital Privado, en el año 1993 y se presenta su evaluación y seguimiento hasta la fecha(AU)

Masas pelvianas en pediatría / Pelvic masses in childhood

La utilidad de los métodos de diagnósticos por imagen en la evaluación de masas pelvianas benignas o malignas fue estudiada en 26 niños comprendidos entre las edades de recien nacidos y quince años de edad. Se analizaron las ventajas y desventajas de cada método para determinar la localización de las lesiones, su contenido y extensión así como información adicional para su diagnóstico, estadificación y tratamiento. De las 26 masas estudiadas, 16 (61,5 por ciento) correspondieron a las lesiones neoplásicas, siendo 7 (26,92 por ciento) benignas y 9 (34,61 por ciento) malignas. Las 10 masas restantes fueron de tipo no neoplásico (38,5 por ciento). El diagnóstico final fue establecido por cirugía con confirmación histopatológica en el 100 por ciento de los casos. La localización de las lesiones fue correctamente evaluada en todos los casos (100 por ciento) (AU)

Ventricular tumors: surgical options.

Primary cardiac tumors of the ventricles are very uncommon. During an 8-year period only, nine such patients were encountered: the tumor was primarily in the right ventricle in six, in the left ventricle in two and biventricular in one. There were seven children and two adults in this series. Histologically, the neoplasm was benign in all patients. The tumor was obstructive in six patients, five of whom underwent surgery (complete resection in three, partial excision in one and a Fontan-Kreutzer procedure in one); one neonate died before an operation could be performed. In addition, one patient with an epicardial tumor underwent complete resection. There were no early or late deaths among the six patients treated surgically. The tumor was small and non-obstructive in two patients, who were not operated on: one died suddenly, and the other was followed without intervention. In summary, complete or partial excision of ventricular neoplasms can usually be accomplished with good long-term results. If resection is not possible, effective palliation may be achieved with a Fontan-type operation, excluding the right ventricle from the circulation.

[Rhabdomyosarcoma: unusual aspects]. / Rabdomiossarcoma: aspectos inusitados.

The authors report a case of embryonal rhabdomyosarcoma with several interesting aspects which make it difficult to diagnose.