Intravascular papillary endothelial hyperplasia of the spleen in a child: a case report.

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH) is a vascular tumor characterized by the proliferation of endothelial cells with papillary formation. It is a rare benign condition affecting the head and neck. Currently, no cases of IPEH of the spleen have been reported. Here, we report a case of IPEH of the spleen in a child and discuss its clinical manifestations, imaging features, and surgical treatment. CASE PRESENTATION: A 5-year-old female presented with a 4-month-old tumor in the left upper abdomen, abdominal pain, and constipation. She underwent radiography, barium enema, US, and MRI. A solid space-occupying mass was found in the left abdominal cavity on preoperative imaging, and it was diagnosed as angiosarcoma. The lesion was surgically resected. Histopathological analysis was consistent with IPEH. CONCLUSION: Clinicians should consider the possibility of IPEH in patients presenting with tumors in the spleen, which is curable by surgical resection. Malignant vascular tumors must be excluded in the differential diagnosis of IPEH to prevent misdiagnosis and inappropriate overtreatment.

Integration of different criteria for borderline resectable pancreatic cancer using classification tree analysis: the use of radiological tumour-vascular interface in correlation with surgical and pathological outcomes.

AIM: To integrate various criteria for borderline resectable pancreatic cancer (BRPC) based on radiological parameters using classification tree analysis. MATERIALS AND METHODS: The institutional review board approved this retrospective study and waived the requirement for informed consent. Two hundred and thirty-five tumour-vein interfaces and 67 tumour-artery interfaces in 245 patients with surgically confirmed pancreatic ductal adenocarcinoma who underwent both preoperative computed tomography (CT) and magnetic resonance imaging (MRI) were assessed by two independent readers. Radiological parameters for evaluation of the tumour-vascular interface were boundary, length of interface, degree of circumferential interface, and contour deformity of affected vessels. Classification tree analysis was performed to determine parameters associated with vascular invasion using pathological and surgical results as the reference standard. RESULTS: In the classification tree analysis for the tumour-vein interface, contour deformity and degree of circumferential interface were the first and second determining factors, respectively, for both surgical and pathological vascular invasion. For the tumour-artery interface, boundary and degree of circumferential interface were the first and second determining factors for surgical invasion, while contour deformity and length of interface were the first and second determining factors for pathological invasion. The BRPC group of modified criteria arbitrarily formed based on the results had similar surgical (74.1-81.6%) and pathological (54.3-63.3%) venous invasion compared to that of the National Comprehensive Cancer Network (NCCN) criteria, and the lowest surgical (33.3%) and pathological (6.7%) arterial invasion compared with those in previously established criteria for BRPC (43.3-55.6% and 22.2-26.1%, respectively). CONCLUSION: Various criteria for BRPCs were integrated using classification tree analysis, and a modified criterion for BRPC, which provides satisfactory results, was established.

Challenging diagnostic and therapeutic modalities for leiomyosarcoma of inferior vena cava.

Leiomyosarcoma of the inferior vena cava (IVCL) is a rare malignant tumour originating from the smooth muscle cells of the media with intra- or extra-luminal growth. The type of the lesion is further divided into three levels in relation to hepatic and renal veins respectively. The aim of this review was to evaluate the results of surgical treatment of IVCL with special reference to the extent of its histological spread and to analyse the recent literature in order to provide an update on the current concepts of diagnostic and therapeutic management of this entity. IVCL's patients may present with non-specific complaints such as dyspnoea, malaise, weight loss, nausea, vomiting, fever and abdominal pain. Haematogenous metastasis is frequent. At a later stage, IVCL may also spread through lymphatic. Multiple diagnostic imaging techniques have been proposed for accurate preoperative diagnosis, including Doppler ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI), individually or in combination with cavography echocardiography or CT-guided biopsy. Despite recent research on the therapeutic strategies against IVCL, surgical resection appears the only potentially curative approach. Unfortunately, a mere minority of patients is eligible to undergo surgical intervention. In addition, surgical removal of IVCL does not necessarily guarantee patient's long-term survival. Alternative therapies, such as radio- and chemo-therapy often proved insufficient. Debate continues regarding the optimal management of the IVC after tumour resection, with primary repair, ligation and IVC reconstruction all have been utilized with varying success.

Vein involvement during pancreaticoduodenectomy: is there a need for redefinition of "borderline resectable disease"?

INTRODUCTION: Current National Comprehensive Cancer Network guidelines recommend neoadjuvant therapy for borderline resectable pancreatic adenocarcinoma to increase the likelihood of achieving R0 resection. A consensus has not been reached on the degree of venous involvement that constitutes borderline resectability. This study compares the outcome of patients who underwent pancreaticoduodenectomy with or without vein resection without neoadjuvant therapy. METHODS: A multi-institutional database of patients who underwent pancreaticoduodenectomy was reviewed. Patients who required vein resection due to gross vein involvement by tumor were compared to those without evidence of vein involvement. RESULTS: Of 492 patients undergoing pancreaticoduodenectomy, 70 (14 %) had vein resection and 422 (86 %) did not. There was no difference in R0 resection (66 vs. 75 %, p = NS). On multivariate analysis, vein involvement was not predictive of disease-free or overall survival. CONCLUSION: This is the largest modern series examining patients with or without isolated vein involvement by pancreas cancer, none of whom received neoadjuvant therapy. Oncological outcome was not different between the two groups. These data suggest that up-front surgical resection is an appropriate option and call into question the inclusion of isolated vein involvement in the definition of "borderline resectable disease."

Adjuvant hepatic arterial infusion chemotherapy after hepatic resection of hepatocellular carcinoma with macroscopic vascular invasion.

BACKGROUND: The prognosis of hepatocellular carcinoma (HCC) with macroscopic vascular invasion is extremely poor even after hepatic resection. We aimed to clarify the efficacy of adjuvant hepatic arterial infusion chemotherapy (HAI) for HCC with vascular invasion. METHODS: A total of 73 HCC patients with macroscopic vascular invasion were divided into two groups: 38 with hepatectomy with HAI (HAI group) and 35 with hepatectomy alone (non-HAI group). From 1997 to 2007, HAI was performed via an implanted injection port. The treatment comprised three courses of weekly infusion of HAI, which comprised cisplatin (10 mg daily on days 1-5) followed by 5-fluorouracil (5-FU; 250 mg daily on days 1-5) infusion. From 2007, cisplatin (60 mg/m(2)), 5-FU (600 mg/m(2)), and a mixture of mitomycin C (3 mg/m(2)) and degradable starch microspheres were administered for two courses. RESULTS: Overall, 92 % of patients completed adjuvant HAI. In the HAI and non-HAI groups, the 5-year disease-free survival (DFS) rates were 33.1 % and 11.8 %, respectively (p = 0.029), and the 5-year overall survival (OS) rates were 46.7 % and 32.7 %, respectively (p = 0.318). Among the patients with Vp3/4 or Vv3 (n = 32) in the HAI group, the 3-year DFS and OS rates were 33.7 % and 56.8 %, respectively (p = 0.049). Those in the non-HAI group were 8.3 % and 12.0 %, respectively (p = 0.023). Cox proportional multivariate analysis for DFS revealed that HAI was an independent favorable prognostic factor in all 73 patients (hazard ratio 0.536; p = 0.029). CONCLUSIONS: Adjuvant HAI for HCC patients with vascular invasion might reduce the risk of recurrence.

Primitive retroperitoneal tumors. Vascular involvement--a major prognostic factor.

Primitive retroperitoneal tumors, although very rare, arouse an increased interest, because of the poor prognosis, unsatisfactory surgical and complementary therapy results. Up to now, the very low number of cases has impeded the acquisition of a unitary view of these tumors, a unanimously accepted algorithm of diagnostic and treatment being absent. Randomized trials regarding the effects of different therapies have not been possible. The main factor that can fundamentally increase the survival of these patients is radical resection, some authors even recommending compartmental surgery. We found no significant statistical difference between the survival rates of the patients with different types of non-radical interventions, that shoud be therefore, as much as possible, avoided. Our study evidences that vascular involvement is the main limiting factor in achieving radicality. The involvement of large retroperitoneal vessels makes often impossible a radical intervention, usually because of the lack of an adequate material and human endowment for ample vascular resections followed by laborious reconstructions. That is why, in our study, vascular involvement was associated with a decreased survival rate for operated patients. Therefore, we underline the necessity both of a solid material base and of establishing multidisciplinary surgical teams for adequate vascular interventions in oncologic general surgery.

Adjuvant arterial infusion chemotherapy after resection of hepatocellular carcinoma with portal thrombosis: a pilot study.

BACKGROUND/PURPOSE: The prognosis of hepatocellular carcinoma (HCC) with tumor thrombosis of the main trunk or major branches of the portal vein (mPVTT) is extremely poor, even if it is curatively resected. Uncontrollable multiple metastases to the residual liver are often observed within several months after the operation. We report here the results of a pilot study, showing the efficacy of adjuvant arterial infusion chemotherapy after the resection of HCC with mPVTT. METHODS: Twelve patients had curative resection of HCC with mPVTT. Six of the patients were treated by the arterial infusion of a chemotherapeutic agent via a subcutaneously implanted injection port after curative resection of HCC with mPVTT. The initial course consisted of the daily administration of cisplatin (CDDP) and continuous infusion of 5-fluorouracil (5-FU). This was followed by the weekly or biweekly administration of CDDP and subsequent infusion of 5-FU until the cumulative dose of 5-FU reached 15 g. RESULTS: The median overall survival time was 58.0 months with adjuvant chemotherapy and 8.0 months without adjuvant chemotherapy. The median disease-free interval was 15.0 months with adjuvant chemotherapy and 4.0 months without adjuvant chemotherapy. Adverse reactions were tolerable nausea and loss of appetite. CONCLUSIONS: This chemotherapeutic regimen achieved favorable results and may be useful as adjuvant chemotherapy in treating patients after curative resection of HCC with mPVTT.

Hyperaldosteronism in a cat with metastasised adrenocortical tumour.

In a 12-year-old male shorthaired cat with attacks of hypokalaemic muscular weakness in spite of oral potassium supplementation, highly elevated plasma aldosterone concentrations in combination with low plasma renin activity pointed to primary hyperaldosteronism. Ultrasonography and computed tomography revealed a large left-sided adrenal tumour growing into the phrenicoabdominal vein and the caudal vena cava. The tumour and its intravascular extension were surgically removed, but the subsequent stenosis of the caudal vena cava caused congestion and renal failure. At autopsy pulmonary micrometastases of the aldosteronoma were found.

Ukrain treatment in a patient with metastatic renal cell carcinoma extending to the vena cava inferior. Case report.

A 52-year-old man with renal cell carcinoma was treated with surgery and chemotherapy (vinblastine). Ukrain was administered after tumor progression to the vena cava inferior and appearance of liver metastasis. The drug induced a complete remission, which has lasted 32 months since the first therapy course.