Treatment of vena cava inferior leiomyosarcoma with cavotomy and right renal vein neoimplantation / Vena cava inferior leiomyosarcoma kezelése cavotomiával és a jobb vena renalis neoimplantatiójával

Leiomyosacroma of the inferior vena cava is an extremely rare malignancy originating from the tunica media of the venous wall. Its symptoms and radiomorphological signs do not always lead to an accurate diagnosis. Histological sampling can be dangerous due to its location. Therefore the diagnosis is often a challenge for clinicians. Its treatment is primarily surgical, supplemented by radiotherapy and chemotherapy applied together or in monotherapy. In our case, an asymptomatic 74-year-old female patient was diagnosed with a tumor of the inferior caval vein located just above the right renal vein and involving the right adrenal gland. As serum cortisol, epinephrine, norepinephrine, adrenocorticotropic hormone (ACTH), total and free testosterone, dehydroepiandrosterone sulfate (DHEA-S), sex hormone binding globulin (SHBG) was at normal level, the tumor showed no hormone secretion. Primary surgical resection was planned. Preoperative biopsy was not performed due to its high risk of complications. During operation, tumor resection was performed by resection of the venous wall, removal of the right adrenal gland and neoimplantation of the right renal vein. For better exposure, cholecystectomy was also performed at the beginning of surgery. There were no postoperative complications. The patient received adjuvant radiation therapy. Thanks to the early diagnosis of the tumor and the complete resection, we significantly increased the patient's chances of total recovery. With the radiotherapy performed, we reduced the likelihood of tumor recurrence.

[Hypothalamic intravascular B-cell lymphoma in an immunocompetent patient]. / Linfoma B intravascular hipotalamico en una paciente inmunocompetente.

TITLE: Linfoma B intravascular hipotalamico en una paciente inmunocompetente.

Efficacy of pegylated lyposomal anthracyclines and of intra-arterial carboplatin and doxorubicin combined with local hyperthermia in a case of malignant endovascular papillary angioendothelioma.

Malignant vascular tumors are exceedingly rare in childhood. Generally, their prognosis is dependent from a microscopically complete surgical resection. We observed the case of a 4-year-old boy affected by malignant endovascular papillary angioendothelioma, a rare vascular tumor of intermediate malignancy, involving all his left leg and partially the pelvis. Its very large size hampered any surgical intervention. However, we could demonstrate high sensitivity of the tumor to lyposomal anthracyclines and the child was eventually cured by the intra-arterial administration of carboplatin and doxorubicin coupled with local hyperthermia. This experience probably represents the first step toward an effective treatment of malignant vascular tumors in infancy.

Intravascular large B-cell lymphoma involving hemangiomas: an unusual presentation of a rare neoplasm.

We report the clinicopathological features of two cases of intravascular large B-cell lymphoma involving cutaneous hemangiomas. The cases were identified from the consultation files of two of the authors. Both patients were women, 64 and 55 years of age, who presented with long-standing cutaneous hemangiomas of the posterior scalp and left shoulder, respectively. The lesions were brought to medical attention by an increase in size and change in color. Biopsies and immunohistochemical evaluation of the hemangiomas revealed extensive involvement by intravascular large B-cell lymphoma. The neoplastic cells were diffusely positive for CD20 in both cases and negative for CD3, pan-cytokeratin (AE1/AE3), epithelial membrane antigen, S-100, Factor VIII-related antigen, CD34 and CD31. Disease was limited to the hemangiomas in both patients. Treatment consisted of chemotherapy (both patients) and adjuvant radiation therapy (one patient). One patient had a recurrence of disease 33 months after initial diagnosis, leading to an autologous stem cell transplant. The other patient is without evidence of disease 27 months after initial diagnosis. Although this is a rare neoplasm, it is important to consider intravascular large B-cell lymphoma in the differential diagnosis of vascular lesions containing intravascular neoplastic cells.

Effective segmental chemoembolization of advanced hepatocellular carcinoma with tumor thrombus in the portal vein.

PURPOSE: Evaluate therapeutic results of transcatheter segmental hepatic artery chemo-oily-embolization (segmental TAE) against advanced hepatocellular carcinomas (HCC) accompanied by portal tumor thrombus (PTT). METHODS: Segmental TAE was performed in nine patients with hepatic cirrhosis and advanced HCCs accompanied by PTT. Four subsegmental portal branches were obstructed by PTT in three patients, and two were obstructed in the remaining six patients. TAE was performed into the targeted segmental hepatic artery followed by embolization with gelatin sponge particles. RESULTS: In one patient undergoing subsequent resection, a marked anticancer effect on the PTT was demonstrated histologically. In the eight other patients who did not undergo subsequent resection, the cumulative survival rates were 67% at 6 months, 44% at 1 year, and 22% at 2 years. No serious complications were noted. CONCLUSIONS: This therapeutic approach is thought to be a useful treatment for HCC with PTT, because it reinforces anticancer effects and can be performed more safely than conventional transcatheter arterial embolization.