Recurrent malignant peripheral nerve sheath tumor presenting as an asymptomatic intravenous thrombus extending to the heart: a case report

Background Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma mainly treated via surgical resection. Herein, we report a case of MPNST wherein a massive tumor thrombus extended to the major veins and heart. Case presentation A 39-year-old female with a history of neurofibromatosis type 1 developed MPNST from the right radial nerve. In addition to adjuvant chemotherapy, she underwent wide tumor resection and concomitant radial nerve resection, followed by postoperative radiotherapy. Histological evaluation revealed marked venous invasion. The 2-year follow-up CT revealed an asymptomatic recurrent tumor thrombus extending from the right subclavian vein to the heart. An urgent life-saving operation was performed to ligate the base of the right subclavian vein and remove the entire intravenous thrombus that extended to the right ventricle. The remaining tumor in the right subclavian vein increased in size 3 months after thrombectomy. After confirming the absence of any metastatic lesions, the patient underwent extended forequarter amputation to achieve surgical remission. One year later, a new metastasis to the right diaphragm was safely resected. The patient remains alive without any evidence of disease 2 years after the extended forequarter amputation. Conclusions In cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.

PUTH Grading System for Urinary Tumor With Supradiaphragmatic Tumor Thrombus: Different Surgical Techniques for Different Tumor Characteristics

PurposeTo explore the different treatment strategies for urinary tumors with Mayo IV thrombus.Materials and MethodsWe retrospectively analyzed the patients with Mayo IV thrombus in Peking University Third Hospital from January 2014 to April 2021. We used the Peking University Third Hospital (PUTH) grading system to classify urinary tumors with supradiaphragmatic thrombus. PUTH-A referred to the filled thrombus whose tip just reached above the diaphragm, or the thrombus entering the right atrium (< 2cm). PUTH-B referred to the filled thrombus entering the right atrium (> 2cm), or the thrombus invading the wall of the inferior pericardial vena cava. Detailed techniques were described for various scenarios. Clinicopathological data and perioperative outcomes were reported. Group difference statistical analysis was performed.ResultsA total of 26 cases of urinary tumors with supradiaphragmatic IVC thrombus (Mayo grade IV) underwent treatment were enrolled in this study. 19 patients in the PUTH-A group received the open approach without sternotomy and cardiopulmonary bypass. Seven patients in the PUTH-B group received open thoracotomy assisted by cardiopulmonary bypass. No intraoperative death occurred. After 56 months of follow-up, 46.2% (12 of 26) patients died of all causes. Estimated 1-year, 2-year, and 3-year overall survival were 72.0% (95% CI, 54.4%-89.6%), 58.2% (95% CI, 38.0%-78.4%), and 52.4% (95% CI, 31.2%-73.6%), respectively.ConclusionsWe introduced the PUTH grading system for the characteristics of urinary tumors with supradiaphragmatic tumor thrombus, and selected different surgical techniques according to different classifications. This grading system was relatively feasible and effective.

Gamma Knife Radiosurgery Versus Transcatheter Arterial Chemoembolization for Hepatocellular Carcinoma With Portal Vein Tumor Thrombus: a Propensity Score Matching Study

BackgroundThe optimal management for hepatocellular carcinoma (HCC) patients with portal vein tumor thrombus (PVTT) is unclear. This study aimed to investigate the efficacy of Gamma knife radiosurgery (GKR) versus transcatheter arterial chemoembolization (TACE) in HCC patients with PVTT. MethodsThis retrospective study included 572 HCC patients with PVTT (GKR, 207; TACE, 365). Propensity score matching (PSM) analysis identified 188 matched pairs of patients. The primary endpoint was overall survival (OS).ResultsBefore PSM, the GKR group demonstrated significantly median OS (mOS) than TACE group (17.4 vs. 8.0 months, p < 0.001). In the subgroup analysis, GKR was associated with significantly longer OS for patients with PVTT II-IV (17.5 vs. 8.2 months, p < 0.001; 19.3 vs. 7.4 months, p < 0.001; 14.5 vs. 6.6 months, p = 0.008, respectively) and comparable OS for patients with PVTT I. After PSM, the GKR group had also a longer OS (mOS) than TACE group (15.8 vs. 9.8 months, p < 0.001). In the subgroup analysis, the GKR group demonstrated superior mOS for patients with PVTT II-IV (all P < 0.05) and comparable OS for patients with PVTT I. ConclusionsGKR was associated better OS than TACE in HCC patients with PVTT, especially for patients with PVTT II-IV.

Clinical Experience and Management Strategy of Retroperitoneal Tumor With Venous Tumor Thrombus Involvement

Background: To report the surgical management, complications, and outcomes for patients with retroperitoneal tumor and venous thrombus Methods: We retrospectively analyzed 19 cases of retroperitoneal tumor with venous tumor thrombus from August 2015 to March 2021. According to the characteristics of surgical techniques, a new tumor thrombus PUTH grading system was proposed. Results: There were 2 cases of PUTH-1a, 2 cases of PUTH-1b, 6 cases of PUTH-2, 6 cases of PUTH-3,3 cases of PUTH-4. All the operations were successfully performed in 19 patients. Among them, 5 cases (26.3%) were operated by completely laparoscopic approach and 13 cases (68.4%) were operated by open approach. One case (5.3%) was converted from laparoscopic approach to open approach. 5 cases experienced postoperative complications, the incidence was 26.3%. All the cases were followed-up for a median of 14 months. Cancer associated death were occurred in 3 cases. Distant metastases were occurred in 7 cases. Conclusions: In this study, we proposed a new tumor thrombus grading system based on the anatomical characteristics of retroperitoneal tumors with venous tumor thrombus. Retroperitoneal tumor resection and removal of venous tumor thrombus are safe and effective in the treatment of such diseases.