Surgical Management of Wilms Tumor with Intravascular Extension: A Single-Institution Experience

The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular extension treated at Virgen del Rocio Children's Hospital between 1992 and 2010. Seven patients (median age 3.4 years, range 2–8.1 years) were identified. At diagnosis, 6 of the 7 patients (85.7%) presented with tumor thrombus that reached the right atrium (RA) and 1 patient with infrahepatic inferior vena cava (IVC) thrombus. All patients received neoadjuvant chemotherapy (SIOP 2001 protocol) with vincristine, doxorubicin, and actinomycin D. Regression of the intravascular extension of the tumor was documented in all patients. Postchemotherapy level of extension was suprahepatic IVC in 1 patient, infrahepatic IVC in 2 patients, renal vein (RV) in 1 patient, and RA in 3 patients. Nephrectomy and thrombectomy were performed in all cases, requiring cardiopulmonary bypass for the 4 patients who presented with suprahepatic IVC and RA thrombus. The other 3 patients with infrahepatic IVC and RV involvement underwent cavotomy and thrombus extraction. Computed tomography, ultrasonography, and echocardiography were used for diagnosis and follow-up. All patients remain disease-free with a median follow-up of 6.3 years (range, 2–19 years). Neoadjuvant chemotherapy for WT with intravascular extension may facilitate the resection by decreasing the extent of the tumor thrombus. Cardiopulmonary bypass is indicated for suprahepatic IVC and RA involvement. Accurate diagnostic imaging is necessary.     

Surgical management of Wilms tumor with intravascular extension: a single-institution experience

The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular extension treated at Virgen del Rocio Children's Hospital between 1992 and 2010. Seven patients (median age 3.4 years, range 2-8.1 years) were identified. At diagnosis, 6 of the 7 patients (85.7%) presented with tumor thrombus that reached the right atrium (RA) and 1 patient with infrahepatic inferior vena cava (IVC) thrombus. All patients received neoadjuvant chemotherapy (SIOP 2001 protocol) with vincristine, doxorubicin, and actinomycin D. Regression of the intravascular extension of the tumor was documented in all patients. Postchemotherapy level of extension was suprahepatic IVC in 1 patient, infrahepatic IVC in 2 patients, renal vein (RV) in 1 patient, and RA in 3 patients. Nephrectomy and thrombectomy were performed in all cases, requiring cardiopulmonary bypass for the 4 patients who presented with suprahepatic IVC and RA thrombus. The other 3 patients with infrahepatic IVC and RV involvement underwent cavotomy and thrombus extraction. Computed tomography, ultrasonography, and echocardiography were used for diagnosis and follow-up. All patients remain disease-free with a median follow-up of 6.3 years (range, 2-19 years). Neoadjuvant chemotherapy for WT with intravascular extension may facilitate the resection by decreasing the extent of the tumor thrombus. Cardiopulmonary bypass is indicated for suprahepatic IVC and RA involvement. Accurate diagnostic imaging is necessary.     

Wilms tumor with extension to the right atrium

A seven-year-old boy had cardiac symptoms for five months before the diagnosis of Wilms tumor was made. The tumor extended into the inferior vena cava through the renal vein and protruded into the right atrium. All demonstrable tumor masses could be removed at a one-step surgical procedure without using cardiopulmonary bypass. Intensive postoperative chemotherapy has been given, and 15 months after surgery he was without signs of relapse.     

Wilms tumor with extension to the right atrium.

A seven-year-old boy had cardiac symptoms for five months before the diagnosis of Wilms tumor was made. The tumor extended into the inferior vena cava through the renal vein and protruded into the right atrium. All demonstrable tumor masses could be removed at a one-step surgical procedure without using cardiopulmonary bypass. Intensive postoperative chemotherapy has been given, and 15 months after surgery he was without signs of relapse.     

Cavoatrial tumor extension in children with wilms tumor: a retrospective review of 17 children in a single center

The aim of this study was to evaluate the clinical characteristics and treatment results of 17 children with cavoatrial tumor extension of Wilms tumor. Of the 360 Wilms tumors diagnosed between 1980 and 2000, 17 patients with intracaval thrombus were identified from the medical records at the pediatric oncology department of Hacettepe University. The following data were collected and reviewed: age, sex, presenting symptoms, tumor site, presence of anaplasia, stage, associated congenital anomalies, localization of tumor thrombus, radiologic findings, type and duration of preoperative chemotherapy, response to preoperative chemotherapy, recurrences, and survival. The frequency of cavoatrial extension in this group was 4.7% (15 in the inferior vena cava and 2 in the right atrium). Fourteen patients received preoperative chemotherapy consisting of two-drug regimen (vincristine and actinomycin D) ranging from 1 to 12 weeks (median 4 weeks). Since intravascular invasion is often asymptomatic, a careful radiologic examination to detect tumor thrombus before surgery is essential. There is no need for aggressive surgery in the presence of tumor thrombus. It may be resolved by preoperative chemotherapy. Surgical removal of the thrombus should be considered in the presence of life-threatening tumor thrombosis at diagnosis and in patients who had residual thrombus after chemotherapy.     

Wilms' tumor with vena caval, atrial, and middle hepatic vein tumor thrombus

A 3-year-old male with a right-sided Wilms' tumor presented with tender hepatomegaly and bilateral lower-limb edema. Ultrasound and echocardiography showed a massive tumor thrombus completely occluding the inferior vena cava, right atrial cavity, and extending retrogradely into the middle hepatic vein. Two courses of preoperative chemotherapy (vincristine, actinomycin D, adriamycin) caused minimal shrinkage of the thrombus. The tumor and thrombus were successfully removed with the patient under cardiopulmonary bypass and deep hypothermic circulatory arrest followed by multiagent chemotherapy (vincristine, actinomycin D, adriamycin, cyclophosphamide). The child is alive and well with no evidence of disease 15 months later. Occlusion of the hepatic vein by a tumor thrombus in Wilms' tumor is a very rare event. It was completely removed by the right atrial route under direct vision in this child. Accepted: 26 April 1999     

Successful management of inferior vena cava and right atrial nephroblastoma tumor thrombus with preoperative chemotherapy.

Involvement of inferior vena cava (IVC) by tumor thrombus occurs in up to 10% of patients with nephroblastoma. Right atrial involvement by tumor thrombus is much less frequent. Four patients presenting with advanced nephroblastoma were diagnosed as having IVC involvement with tumor thrombus. Two of these patients had in addition thrombus extending up to the right atrium. All 4 patients were treated with preoperative chemotherapy (vincristine, actinomycin D, 4-epi-Adriamycin). Nephrectomy was subsequently performed without undue difficulty on all 4 patients. The intravascular tumor thrombus had completely cleared in all 4 patients and most of the primary renal tumor was necrotic. The results obtained with preoperative chemotherapy as given to these patients mediates strongly against difficult surgery being undertaken as primary treatment for such patients.     

Successful management of inferior vena cava and right atrial nephroblastoma tumor thrombus with preoperative chemotherapy

Involvement of inferior vena cava (IVC) by tumor thrombus occurs in up to 10% of patients with nephroblastoma. Right atrial involvement by tumor thrombus is much less frequent. Four patients presenting with advanced nephroblastoma were diagnosed as having IVC involvement with tumor thrombus. Two of these patients had in addition thrombus extending up to the right atrium. All 4 patients were treated with preoperative chemotherapy (vincristine, actinomycin D, 4-epi-Adriamycin). Nephrectomy was subsequently performed without undue difficulty on all 4 patients. The intravascular tumor thrombus had completely cleared in all 4 patients and most of the primary renal tumor was necrotic. The results obtained with preoperative chemotherapy as given to these patients mediates strongly against difficult surgery being undertaken as primary treatment for such patients.     

Enormous tumour in the right atrium of a Wilms' tumour patient

Formation of neoplastic thrombus in vena cava inferior (VCI) is well known in Wilms' tumour. We demonstrate a case of large neoplastic mass in the right atrium as prolongation from vena cava inferior in a 2 years and 2 months old girl with Wilms' tumour. The long preoperative chemotherapy (7 months) reduced the tumour and the thrombus. The surgical removal of the tumour and the thrombus was followed by further chemotherapy. During postoperative chemotherapy the control USG examinations showed the presence of a stable mass in a caval wall (a neoplastic mass or a scar?). The extension of the thrombus into the right atrium remains a therapeutic problem which has not been yet resolved.     

Metachronous Wilms tumor associated with pulmonary embolism: how can we detect these cases early? A case report and literature review

A 4-year-old girl developed right metachronous Wilms tumor 2 years after completing treatment for a left-sided stage I Wilms tumor. The original treatment included 7 weeks of chemotherapy, delayed nephrectomy, and another 3 weeks of chemotherapy. The metachronous tumor on the right side extended into the inferior vena cava and right atrium. She developed pulmonary embolism as a result. She received chemotherapy and developed liquifaction of the tumor and toxic shock. She also had surgery. The patient is alive 3 years after the original diagnosis and 10 months after the relapse. The authors report this unusual case and discuss whether these cases can be identified early.     

Atypical leiomyoma arising in an hepatic vein with extension into the inferior vena cava and right atrium

We report an atypical leiomyoma arising in an hepatic vein and extending into the inferior vena cava and right atrium in a fourteen year old boy. US, CT and MRI facilitated diagnosis and removal of this tumor.     

Inferior vena caval and right atrial thrombosis: Complicating pyogenic liver abscess

Vascular complication of liver abscess are rare but life-threatening. We herein report a 2 year 9 month boy with pyogenic hepatic abscess complicated by inferior vena cava thrombus extending to right atrium. Early clinical suspicion aided by ultrasonography and echocardiography confirmed the diagnosis. The child was treated successfully with timely medical and surgical intervention.     

Rhabdoid tumour of the kidney with caval and cardiac extension

Rhabdoid tumour of the kidney is a rare paediatric neoplasm usually associated with a grave prognosis. We report a case of left renal rhabdoid tumour with involvement of the inferior vena cava (IVC) and right atrium in a 13-year-old girl. Left nephrectomy together with removal of the IVC and atrial extension was undertaken on cardiopulmonary bypass and hypothermia. She remained disease-free and in good health for 1 year, however, recent review has shown recurrence in the abdominal lymph nodes.     

Right heart thrombus in infants and children

Summary The echocardiographic features and management of 10 children with right heart thrombus (RHT) are presented. The patients' ages ranged from 2 weeks to 12 years. Three patients were less than 3 weeks old. The echocardiographic diagnosis was confirmed in five patients by surgery or autopsy. The thrombus was detected in the right atrium (five patients), in the right ventricle (three), on the tricuspid valve or within the inferior vena cava, the superior vena cava, or its extension following Senning's operation for transposition of great arteries (TGA). Seven patients had structural heart disease: TGA in four, tricuspid atresia in three. In two of three patients with anatomically normal heart, the thrombus occurred as a complication of a ventriculoatrial and a lymphovenous shunt system. The third patient had no predisposing factor. Five patients were treated with anticoagulants or platelet anti-aggregating agents and four by surgery. Seven patients survived and were followed by echocardiography.     

Anomalous Insertion of the Superior or the Inferior Vena Cava into the Right Atrium

Three cases with anomalous insertion of the superior or inferior vena cava into the right atrium are presented. One case was a 25-year-old healthy man with anomalous low insertion of the right superior vena cava into the right atrium. The remaining two cases were infants with complex cardiac anomalies showing anomalous high insertion of the inferior vena cava into the right atrium. The congenital anomalies of the connection between the superior and the inferior vena cava and the right atrium are rare. Angiographic and computed tomographic findings of these anomalies were reported.     

BCOR‐CCNB3 fusion‐positive clear cell sarcoma of the kidney

Clear cell sarcoma of the kidney (CCSK) is the second most common malignant pediatric renal tumor. Two of the recurrent somatic alterations reported in CCSK are BCL‐6 corepressor (BCOR) internal tandem duplication (ITD) and YWHAE‐NUTM2B/E gene fusion. A minority of patients with CCSKs have other rare somatic alterations. We report two patients with CCSK showing BCOR‐CCNB3 (where CCNB3 is cyclin B3) fusion, who had similar clinical presentation of a large renal mass with tumor thrombus extending through the inferior vena cava into the right atrium and a favorable response to chemotherapy. We recommend BCOR‐CCNB3 fusion testing for all patients with CCSK who lack BCOR‐ITD or YWHAE‐NUTM2B/E gene fusions.     

Adrenocortical carcinoma with extension into inferior vena cava and right atrium: Report of 3 cases in children

We present three cases of adrenocortical carcinoma, a rare tumor in children, with extension into the inferior vena cava and the right atrium. The diagnosis is facilitated by use of ultrasonography, computed tomography and magnetic resonance imaging.     

Ewing's sarcoma metastazing from the right pelvis via the inferior vena cava into the heart: Diagnosis and successful surgical treatment

Summary A case of Ewing's sarcoma metastazing from the pelvis into the heart is presented. Fainting as a result of circulatory obstruction within the right heart was the leading symptom. Open-heart surgery was performed to excise the tumor from the right atrium and the inferior vena cava. Postoperative angiography showed the tumor coming up out of the right iliac vein and growing along the wall of the inferior vena cava.*Current address: 5/8-10 Lugar Street, Waverley, NSW 2024, Australia.     

Virilizing Adrenocortical Carcinoma Invading the Right Atrium with Histological High-Grade Malignancy and p53 Mutation in a 3-Year-Old Child: Indication of Post Operative Adjuvant Chemotherapy

We present a 3-yr-old girl with a virilizing adrenocortical carcinoma invading into the right atrium with histological high-grade malignancy and p53 mutation. Development of facial acne and pubic hair were noted at 3 yr and 2 mo. The levels of androgens were high. Diurnal variation in ACTH and cortisol were absent. Abdominal computed tomography revealed a large right suprarenal mass, with extension into the inferior vena cava and right atrium. Based on the diagnosis of a right virilizing adrenocortical tumor with Cushing syndrome, surgery was performed by a combined thoracoabdominal approach with the patient on cardiopulmonary bypass. The tumor was 7 × 5.5 × 3.5 cm in size, and weighed 95 g. The histological diagnosis was adrenocartical carcinoma with high-grade malignancy according to the category of Weiss. A heterozygous mutation of the p53 tumor-suppressor gene (codon 248 CGC→TGG) was found. We did not perform adjuvant chemotherapy because of radical resection on macroscopic observation and no metastasis in radiological findings. Five months after the surgery, her chest X ray and computed tomography revealed multiple lung metastases and a single liver metastasis. In this type of patient with histological high-grade malignancy and p53 mutations, postoperative adjuvant chemotherapy is indicated even if macroscopic total surgical removal had been performed.     

Surgical management and outcomes of 12 cases of Wilms tumour with intracardiac extension from a single centre

Purpose

To review demographics, effect of preoperative chemotherapy on tumour thrombus, imaging, operative strategy, and outcomes of 12 patients presenting with intracardiac extension of Wilms tumour thrombus.

Methods

A retrospective audit was undertaken on patients with intracardiac extension of Wilms tumour. Patients were identified from the oncology database and information obtained on clinical presentation, stage, preoperative treatment, surgical procedures and complications, histology, and survival status. Ethics approval was obtained from the University of Cape Town Human Research Ethics Committee.

Results

From 1984 to 2016, 337 children with Wilms tumour were treated. Twelve (3.6%) had intracardiac extension of tumour thrombus, nine into the right atrium, and three into the right ventricle. Ultrasound, computerized tomography, magnetic resonance imaging, and echocardiograms were used to assess thrombus level. Patients were staged as stage III(8) and IV(4). All patients received preoperative chemotherapy. Thrombus retracted from the heart in two cases. One patient died preoperatively. Eleven underwent laparotomy, median sternotomy, and cardiopulmonary bypass (CPB). Four underwent cavectomy. Five required cavoatrial patches. Thrombus extending into the hepatic veins was extracted in five patients. There was one intraoperative complication and one perioperative death. Thrombus histology showed viable tumour in 9 of 11 patients. Three patients died of progressive disease. Seven patients are currently disease free.

Conclusion

A combination of imaging is required to determine thrombus extent, and this facilitates surgical planning. Preoperative chemotherapy may cause thrombus regression, thus avoiding CPB. CPB offers appropriate conditions for safe tumour thrombus excision. Full management in centres with appropriately experienced staff and facilities for CPB is recommended.