Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort.

Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.

Renal tumor and trauma: a pitfall for conservative (correction of conversative) management.

PURPOSE: Conservative management has been largely used for renal trauma. Although this approach is safe and highly recommended, it can hide a pre-existing unknown condition, such as tumors or urinary malformations. A high index of suspicion is needed for early recognition of these conditions. We present four cases treated at the Pediatric Oncology Institute - Federal University of São Paulo, which have been initially treated conservatively for renal trauma. MATERIALS AND METHODS: We reviewed all 218 renal cases of renal tumors treated at our institution in a 22-year period, searching for associated trauma events. RESULTS: Four cases of renal tumors were initially treated conservatively for blunt renal trauma of low energy mechanism. Patients ' ages ranged from 7 to 12 years old. Two patients had no previous symptoms, one patient had hematuria and another had an abdominal mass. Computerized Axial Tomography (CT) of the abdomen revealed disparate magnitude of the renal bleeding to the low energy mechanism of trauma. All patients underwent surgical treatment. Kidney specimens showed Wilms tumor in three cases and renal carcinoma in one. CONCLUSIONS: The association between renal tumors and trauma should be suspected when renal trauma hemorrhage on abdominal CT scan does not match the low energy mechanism of blunt abdominal trauma. The key for a successful diagnosis of renal tumor or congenital malformations is the high index of suspicion for these conditions.

Totally implantable catheter embolism: two related cases / Embolia de cateter totalmente implantável: relato de dois casos

CONTEXT AND OBJECTIVE: Long-term totally implantable catheters (e.g. Port-a-Cath®) are frequently used for long-term venous access in children with cancer. The use of this type of catheter is associated with complications such as infection, extrusion, extravasation and thrombosis. Embolism of catheter fragments is a rare complication, but has potential for morbidity. The aim here was to report on two cases in which embolism of fragments of a long-term totally implantable catheter occurred. DESIGN AND SETTING: Case series study at Hospital do Servidor Público Estadual, São Paulo. METHODS: Retrospective review of catheter embolism in oncological pediatric patients with long-term totally implantable catheters. RESULTS: The first patient was a 3-year-old girl diagnosed with stage IV Wilms' tumor. Treatment was started with the introduction of a totally implantable catheter through the subclavian vein. At the time of removal, it was realized that the catheter had fractured inside the heart. An endovascular procedure was necessary to remove the fragment. The second case was a boy diagnosed with stage II Wilms' tumor at the age of two years. At the time of removal, it was noticed that the catheter had disconnected from the reservoir and an endovascular procedure was also necessary to remove the embolized catheter. CONCLUSION: Embolism of fragments of totally implantable catheters is a rare complication that needs to be recognized even in asymptomatic patients.

CONTEXTO E OBJETIVO: Os cateteres de longa permanência totalmente implantáveis (por exemplo, Port-a-Cath®) são muito utilizados para acesso venoso de longa duração em crianças com câncer. O uso deste tipo de cateter está associado a algumas complicações como infecção, extrusão, extravasamento e trombose. A embolia de fragmento de cateter é complicação rara, mas com potencial morbidade. O objetivo do estudo foi relatar dois casos em que houve embolia de fragmento de cateter de longa permanência. TIPO DE ESTUDO E LOCAL: Série de casos do Hospital do Servidor Público estadual, São Paulo. MÉTODOS: Estudo retrospectivo das embolias de cateter de longa permanência em crianças. RESULTADOS: No primeiro caso, descreve-se uma menina com diagnóstico de tumor de Wilms estádio IV aos três anos de idade. Iniciou tratamento com implante de cateter de longa permanência em veia subclávia. Três anos após diagnóstico, foi programada a retirada do cateter por término de tratamento. No momento da retirada, houve fratura intracardíaca do cateter. Houve necessidade de retirada do fragmento intracardíaco com hemodinâmica. O segundo caso tratava-se de um menino com diagnóstico de tumor de Wilms estádio II aos dois anos de idade. Teve o cateter implantado no início do tratamento. No momento da retirada por fim de tratamento, o cateter já havia desconectado e embolizado. O fragmento intracardíaco foi retirado também por procedimento de hemodinâmica. CONCLUSÃO: A embolia de fragmentos de cateter em cateteres de longa permanência é uma complicação rara, mas que necessita ser reconhecida, mesmo em pacientes assintomáticos.

Totally implantable catheter embolism: two related cases.

CONTEXT AND OBJECTIVE: Long-term totally implantable catheters (e.g. Port-a-Cath) are frequently used for long-term venous access in children with cancer. The use of this type of catheter is associated with complications such as infection, extrusion, extravasation and thrombosis. Embolism of catheter fragments is a rare complication, but has potential for morbidity. The aim here was to report on two cases in which embolism of fragments of a long-term totally implantable catheter occurred. DESIGN AND SETTING: Case series study at Hospital do Servidor Público Estadual, São Paulo. METHODS: Retrospective review of catheter embolism in oncological pediatric patients with long-term totally implantable catheters. RESULTS: The first patient was a 3-year-old girl diagnosed with stage IV Wilms' tumor. Treatment was started with the introduction of a totally implantable catheter through the subclavian vein. At the time of removal, it was realized that the catheter had fractured inside the heart. An endovascular procedure was necessary to remove the fragment. The second case was a boy diagnosed with stage II Wilms' tumor at the age of two years. At the time of removal, it was noticed that the catheter had disconnected from the reservoir and an endovascular procedure was also necessary to remove the embolized catheter. CONCLUSION: Embolism of fragments of totally implantable catheters is a rare complication that needs to be recognized even in asymptomatic patients.

Gastric hemangioma in a 5-year-old boy.

We report a rare cause of gastrointestinal bleeding in a 5-year-old boy with hematemesis and melena for a month. Upper gastrointestinal endoscopy and an abdominal magnetic resonance image suggested a large hemangioma on the gastric fundus. The patient underwent an exploratory laparotomy with partial gastrectomy limited to the gastric fundus. An intraoperative endoscopy helped to ensure a complete resection of the tumor. Histopathology showed a cavernous hemangioma of the gastric fundus.

Cavectomy for the treatment of Wilms tumor with vascular extension.

PURPOSE: Vascular extension to the vena cava occurs in 4% of Wilms tumor cases and can reach the right atrium in up to 1%. When this happens the thrombus is usually not adherent to the vessel wall, and there is blood flow around it. Preoperative chemotherapy can cause thrombus regression and even resolution. If the thrombus persists after chemotherapy, surgery will be a challenge. On the other hand, if the thrombus invades the vessel wall, its removal may not be feasible. In this situation cavectomy is a good surgical strategy because it provides complete resection. The prerequisite for cavectomy is the absence of blood flow in the vena cava on preoperative Doppler ultrasonography. We report 3 cases of Wilms tumor with vena caval invasion in which cavectomy was performed, and discuss the principles, indications and operative technique. MATERIALS AND METHODS: A total of 171 patients with Wilms tumor were treated at our institution between 1984 and 2004. Of these patients 6 with intravascular extension of thrombus within the right atrium were treated with extracorporeal circulation, cardiac arrest and profound hypothermia, and 3 were treated with cavectomy. RESULTS: There were no instances of surgical complications or postoperative renal failure in our patients who underwent cavectomy. All remain well and free of disease. CONCLUSIONS: Cavectomy is a safe procedure for treating pediatric patients with Wilms tumor when there is extension and invasion of the vena cava wall without blood flow.

Unusual case of epigastric heteropagus twinning.

Asymmetrical conjoined twins or heteropagus twins are extremely rare. They are characterized by an incomplete component (parasite) that is normally smaller and dependent on the host (autosite). In cases of an epigastric heteropagus twin, the insertion occurs in the epigastrium. There are few reports of epigastric heteropagus twinning in the English-language literature. The authors report an extremely rare case of epigastric heteropagus twinning in which the parasite presented with head, thorax, and a rudimentary heart.