Radomiosarcoma paratesticular: a propósito de un caso / Paratesticular phabdomyosarcoma: a case report

OBJETIVO: Presentar un caso de radomiosarcoma paratesticular y revisión de la literatura. MÉTODOS: Describimos el caso de un varón de 16 años remitido a nuestro servicio por masa paratesticular izquierda de crecimiento progresivo, con imagen ecográfica de tumoración paratesticular heterogénea con flujo Doppler en su interior. RESULTADOS: Se realizó orquiectomía izquierda, con diagnóstico de rabdomiosarcoma. El paciente rechazó el tratamiento quimioterápico adyuvante. Tras 13 meses libre de enfermedad, reingresó por dolor cólico izquierdo detectándose en ecografía y TC una masa retroperitoneal paraaórtica izquierda que condicionaba uréterohidronefrosis grado III, y metástasis pulmonares. A pesar de instaurarse quimioterapia de rescate, no respondió presentando rápida progresión de la masa abdominal con importante deterioro general que no permitió el abordaje quirúrgico, siendo éxitus a las pocas semanas. CONCLUSIÓN: Los sarcomas paratesticulares son tumores infrecuentes y de mal pronóstico(AU)

OBJECTIVE: To report a case of paratesticular rhadomyosarcoma and to perform a bibliographic review. METHODS: We report the case of a 16-year-old male referred to our Department because of a left paratesticular hard tumor with progressive growth. Ultrasound examination showed a paratesticular heterogeneous mass with Internal flow on Doppler. RESULTS: The patient underwent left inguinal orchiectomy, with pathological diagnosis of rhabdomyosarcoma. He refused adjuvant chemotherapy. After being disease-free for 13 months, he presented with left colic pain. Ultrasound and CT examinations showed a left paraaortic retroperitoneal mass causing grade III ureterohydronephrosis, and lung metastases. Despite rescue chemotherapy treatment, there was no response and the abdominal mass progressed. A surgical approach was not possible since patient showed a rapid clinical worsening leading to his death a few weeks later. CONCLUSIONS: Paratesticular sarcomas are very uncommon tumors with poor prognosis(AU)

Lipoleiomiosarcoma en región paratesticular. Estudio de dos casos y revisión de la literatura / Lipoleiomyosarcoma in the paratesticular region. Presentation of two cases and a review of the literature

El lipoleiomiosarcoma corresponde a un liposarcoma bien diferenciado que muestra focalmente diferenciación hacia tejido muscular liso maduro con atipia. La localización característica es el retroperitoneo, aunque se han descrito casos en otras localizaciones, siendo raros en la región inguinal-paratesticular. El comportamiento de las lesiones es similar al liposarcoma bien diferenciado, siendo clave en el pronóstico de la lesión la localización y, en relación a esta, la posibilidad o no de extirpación quirúrgica completa. Presentamos dos casos de localización paratesticular y revisamos la literatura(AU)

Lipoleiomyosarcoma is a well differentiated liposarcoma with focal differentiation into mature smooth muscle with atypia. These tumours usually occur in the retroperitonum, although they have been reported in other locations. The inguinal paratesticular region is a rare site for these neoplasms. Their behaviour is similar to that of well differentiated liposarcomas. Their location and the possibility of complete surgical extirpation are key prognostic factors. Two cases of lipoleiomyosarcoma occurring in the paratesticular region are presented together with a review of the literature(AU)

[Acute renal failure as complication of neoplastic disease in children]. / Ostra niewydolnosc nerek jako powiklanie choroby nowotworowej u dzieci.

Three cases of acute renal failure (ARF) requiring renal replacement therapy (RRT) in the course of neoplastic disease were presented. 7.5-yr-old girl admitted with postrenal failure during palliative radiotherapy had metastases in retroperitoneal space. Improvement followed percutaneous placement of nephrostomy catheters. 16-yr-old boy with acute myeloid leukemia was effectively treated with hemodialysis for prerenal and renal ARF mediated by vasomotor, infectious and toxic factors. In 11-yr-old boy ARF was the first clinical presentation of non-Hodgkin's lymphoma. Chemotherapy brought restoration of renal function. As a conclusion we emphasize complex etiology of ARF in such patients as well as the necessity of early introduction of RRT and thorough diagnosis and proper management of the causes of impaired renal function.

Liomiossarcoma de esofago: relato de um caso e breve revisao de literatura / Leyomiosarcoma of the esophagus: a case report and a brief literature review

O Liomiossarcoma de Esofago e uma neoplasia rara, com 45 casos descritos na literatura inglesa ate 1986. E descrito um caso e feita breve revisao bibliografica. A molestia acomete ambos os sexos em igual proporcao. Os achados clinicos e radiologicos simulam outras doencas do mediastino ou do esofago. O tratamento de escolha e a cirurgia. Pacientes do sexo feminino e portadores de lesao polipoide parecem ter melhor prognostico

Paraneoplastic syndromes with soft-tissue sarcoma: a report of two unusual cases.

Paraneoplastic syndromes secondary to mesodermal tumors are relatively uncommon. In this report, we describe two unusual cases associated with soft-tissue sarcoma: a 69-year-old male who had a normochromic anemia, without apparent etiology, that resolved promptly after surgical resection of the primary tumor; and a 22-year-old female with hypercalcemia without evidence of bony destruction.

Mesenchymal tumors associated with hypoglycemia: case report and review of the literature.

Hypoglycemia secondary to malignant tumors is rare. Mesenchymal tumors of nonpancreatic origin are the most common tumors associated with the hypoglycemia syndrome, and the clinical features of 115 reported cases are reviewed. The major anatomic distributions of the tumors are thoracic (30%) abdominal (65%), and uncommon locations (less than 5%). Approximately 50% of the tumors were resectable (59 patients), and in 60% the surgical procedure was curative. In the remaining 40% local recurrence predominated related to site of tumor and presence of contiguous organ invasion. The application of multimodality adjuvant therapy for hypoglycemia associated mesenchymal tumors should be based on an understanding of the natural history of the tumor.

Budd Chiari syndrome from myosarcoma of right atrium.

A 50-year-old man presented with abdominal pain followed by marked liver and renal dysfunction. Although liver scan appearances were not diagnostic, at laparotomy a tense, enlarged liver was found with thrombosis of the inferior vena cava. Despite full supportive measures the patient died within a month of laparotomy. Subsequent necropsy confirmed the diagnosis of Budd Chiari syndrome and revealed the primary cause to be a myosarcoma of the right atrium, an occurrence hitherto unreported.