Desafios e importância da avaliação pré-operatória na doença de Peyronie / Challenges and importance of the preoperative evaluation in Peyronie's disease

A doença de Peyronie, notória desde 1743, segue sendo um grande desafio na prática médica, com prevalência relevante e grande impacto na vida sexual dos casais. O tratamento cirúrgico é a principal modalidade terapêutica capaz de restabelecer a vida sexual nos pacientes com doença de Peyronie significativa. A escolha do momento de implementação do tratamento cirúrgico, bem como a escolha da técnica a ser empregada, varia de acordo com três pontos centrais: a fase da doença, a deformidade apresentada e a função erétil. Estes pilares para a decisão terapêutica do paciente com doença de Peyronie possuem nuances, não sendo simples a caracterização destes fatores em muitos casos. Uma avaliação pré-operatória criteriosa, fundamental para a melhor escolha terapêutica, exige experiência e um conhecimento aprofundado sobre o tema. O objetivo do presente artigo é promover uma ampla discussão acerca de fatores primordiais da avaliação pré-operatória de pacientes com doença de Peyronie.

Malignant paraganglioma in children treated with embolization prior to surgical excision.

BACKGROUND: Paragangliomas (PGL) are rare tumors derived from neural crest cells, whose origins may vary along the chain of the sympathetic nervous system. Such tumors are often characterized by secretion of catecholamines, but sometimes they are biochemically inactive, which makes diagnosis often challenging. Malignant paraganglioma is defined by the presence of this tumor at sites where chromaffin cells are usually not found or by local invasion of the primary tumor. Recurrence, either regional or metastatic, usually occurs within 5 years of the initial complete resection but long-term recurrence is also described. Malignancy is often linked to a SDHB mutation. Preoperative embolization has been applied in the surgical management of PGLs with the objective to decrease intra-operative blood loss and surgery length without complications. CASE PRESENTATION: We report two cases of patients with abdominal or pelvic malignant PGLs who have been treated surgically at our center after preoperative embolization. Surgery was a very challenging procedure with multiple surgical teams involved and embolization did not prevent major blood loss and intraoperative complications. Patients required adjuvant treatment with either chemotherapy or radiotherapy. CONCLUSIONS: Many studies in the adult population have established recommendations for the diagnosis and therapeutic management of PGL, but few studies concern the pediatric population. Because malignant PGL is more important in the pediatric population, screening and early diagnosis of PGL is advisable in children with genetic predisposing. Surgical resection is the mainstay of treatment, but a multimodal approach is often required due to the complexity of cases.  The role of preoperative embolization is not established and in our experience it has provided little benefit and major complications.