Multidisciplinary Approach for Management of Pediatric Tumors: a Case Series

Abstract

Pediatric solid tumors have wide range of presentations. Multidisciplinary approach is often needed for their optimum management. There are no reports discussing such an approach to pediatric tumors involving pediatric surgeons, oncologists and cardiothoracic surgeons together for the management. We report 5 such cases being managed in our institution from 2010 to 2016. All cases needed chemotherapy followed by resection with a team of surgeons involving pediatric surgeon and cardiothoracic surgeon

Introduction

Pediatric solid tumors account for 2% of all tumor cases. They have wide range of presentations. Multidisciplinary approach is often needed for their optimum management. There are no reports discussing such an approach to pediatric tumors involving pediatric surgeons, oncologists, and cardiothoracic surgeons together for the management. We report 5 such cases being managed in our institution from 2010 to 2016.

There were 2 cases of Wilms’ tumor with inferior vena cava (IVC) extension, an adrenocortical carcinoma, a pheochromocytoma with pancreatic neuroectodermal tumor (PNET), and a neuroblastoma encasing major vessels. All cases needed chemotherapy followed by resection with a team of surgeons involving pediatric surgeon and cardiothoracic surgeon.

Case Series

Case 1 [a]

A 1-year-9-month girl presented with abdominal distension for 15 days and was diagnosed as right sided Wilms’ tumor with invasion of IVC and thrombosis within it with engulfment of right renal vein by mass on CECT abdomen. It was a right upper pole mass of size 11 cm × 8 cm × 8 cm with inferior vena cava invasion by tumor thrombus from infrahepatic to retrohepatic extending to suprahepatic inferior vena cava into left atrium. 2D ECHO was normal. Considering unresectability, 4 cycles of chemotherapy (vincristine and adriamycine) was given after CT guided biopsy which was suggestive of biphasic Wilms^’ tumor. Cardiothoracic surgeons’ opinion was taken for IVC thrombosis and atrial extension and need for cardiopulmonary bypass during excision of mass. With meticulous team work, right radical nephrectomy with excision of inferior vena caval thrombus was accomplished on cardiopulmonary bypass and stable patient was discharged on postoperative day 19. Child had received postoperative chemoradiation and on follow up of 2 years had no recurrence.

Case [1b]

Similar case as above was a 7 years female referred as suspected inferior vena cava leiomyomatosis and was diagnosed as right Wilms’ with IVC and right atrial extension. She was managed with CT guided biopsy followed by preoperative chemotherapy with vincristine, adriamycin, and actinomycin, 8 cycles followed by radical nephrectomy with inferior vena cavotomy, and median sternotomy with removal of intra atrial extension on cardiopulmonary bypass with the assistance of cardiothoracic surgeon. This child had a 5-year follow up which was disease free.

Case 2

A 2-year-old boy presented with high grade fever on and off for 3 months, vomiting for 8 days, distension, and pain in abdomen. On USG abdomen, there was hyperechoic lesion in retroperitoneum 4.1 × 6.2 × 8.1 cm with few calcifications within and encasement of mesenteric vessels. CECT abdomen was suggestive of large retroperitoneal mass 94 × 72 mm with heterogeneous enhancement with few foci of calcification. Superior border of mass starts at hilum of liver extending inferiorly distal to renal hilar level and encases SMA and bilateral renal arteries completely. Multiple nodes were also noted in mesenteric region. Bone marrow biopsy was normal, peripheral smear was normal, and urinary VMA was 5.3 mg/24 h. FDG-PET scan suggestive of metabolically active disease in mid abdomen. 2D ECHO, MIBG scan, and Bone scan were normal. CT guided biopsy was done suggestive of poorly differentiated Neuroblastoma with high Mk-1 unfavorable histology type with n-MYC amplification indicating high-risk disease. The child received 8 cycles of chemotherapy (CECA protocol) preoperatively. FDG-PET scan after 3 cycles showed no evidence of any metabolically active disease in body, and FDG-PET scan after completion of 8 cycle of neoadjuvant chemotherapy has focus in right suprarenal region. Ultrasound of the abdomen showed well-defined hypoechoic mass in right suprarenal region of 1.3 × 1.6 cms and CT abdomen (P + C) suggestive of 3.7 × 2.6 cm hypodense non-enhancing mesenteric mass with multiple specks of calcification within and multiple mesenteric lymph nodes suggestive of metastatic disease. A 5 × 4-cm right suprarenal mass superiorly infiltrating into liver and encapsulating abdominal aorta was excised. IVC was not involved. Porta and retroduodenal lymph nodes were removed. Part of tumor around aorta could not be removed. Histopathology showed neuroblastoma with extensive ganglionic and glial differentiation. One out of 2 retroduodenal lymph nodes were involved. Two portal lymph nodes were also involved. He was given oral cyclophospamide (NB 2004 trial protocol) and radiotherapy. This child was lost to follow up.

Case 3

A 12-year-old boy presented with headache and pain in abdomen since 3 months. He had hypertension and was started on antihypertensive. Ultrasonography and CT scan showed mass in bilateral adrenals with calcifications more likely suggestive of pheochromocytoma and mass involving head, uncinate process, and body of pancreas with calcifications. Normetanephrine levels were elevated. MIBG scan showed uptake by both adrenals and pancreas. Bilateral adrenalectomy with FNAC of pancreatic mass was done in first sitting. FNAC showed neuroendocrine tumor (NET). Histopathology of bilateral adrenal mass showed pheochromocytoma. Pylorus preserving Whipples procedure was done after 5 months for pancreatic-NET as a combined team with cardiothoracic surgeons. Histopathology showed neuroendocrine carcinoma with perineural and lymphovascular invasion. Patient had uneventful recovery and is asymptomatic in 18 months of follow up period.

Case 4

A 12-year female child presented with pain in abdomen and lump in upper abdomen since 1 month. Patient was hypertensive and started on antihypertensives and evaluated in endocrinology as secretory right adrenocortical mass. Basal cortisol (12.7) and DHEAS (970) were raised. USG abdomen suggestive of 16 × 14 cm well defined hypoechoic lesion in segment VI, VII, and VIII of liver and was very vascular. CECT abdomen suggestive of 15 cm heterogeneously enhancing mass VI, VII, and VIII segment of liver and IVC compressed anteromedially. Right adrenal could not be visualized separate from mass, highly vascular, specks of calcification present, fat planes between liver and mass, and between mass and right kidney and renal vessels obliterated. MIBG scan showed no focus of abdominal tracer uptake. FDG-PET scan showed metabolically active mass with calcification in right suprarenal region not separate from adrenal neuroblastoma. VMA and metanephrines were normal. Two cycles of chemotherapy were given followed by excision of right adrenal mass with right nephrectomy as a team with cardiothoracic surgeons in view of IVC being adherent to mass. Histopathology showed adrenocortical carcinoma. Subsequently 2 more chemotherapies were given. Follow-up DHEAS and cortisol were normal. On 2 years of follow up, the child was on mitotane for recurrence.

Discussion

Cancer is the second most common cause of death among children aged 1 to 14 years surpassed only by accidents. Leukemia (77% of which are lymphoid leukemia) accounts for 30% of all childhood cancers (including benign brain tumors). Cancers of the brain and other nervous system are the second most common cancer type (26%), followed by neuroblastoma (6%), soft tissue sarcomas (6%, one-half of which are rhabdomyosarcoma), renal (Wilms’) tumors (5%), non-Hodgkin lymphomas (including Burkitt lymphoma) (5%), and Hodgkin lymphomas (3%) [1].

Progress in treatment of childhood cancer has led to an overall cure rate of nearly 80%.[2]Many of the morbidities associated with childhood cancer survivorship have been shown to impact on quality of life, and can include organ damage, cognitive impairments, and psychosocial dysfunction. A multidisciplinary approach to the complex healthcare needs of childhood cancer survivors has been proposed to be an efficient way to deliver care that is beneficial to patients, providers, and institutions [3]. In some circumstances, we may need team work for the management of tumors cases in children. Wilms’ with IVC extension has an incidence of 4 to 11% and needs cardiopulmonary bypass thus assistance by cardiothoracic surgeons for the same accounts for better outcome of the patient.

Most of the time, we read about collaboration of pediatric oncologists, surgeons radiotherapist, pathologists, radiologists, and nursing staff for management of pediatric tumors. This is to emphasize that cardiothoracic surgeons with pediatric surgeons make a good team for management of cases with major vascular invasion, encasement, or involvement. It is always safe to keep them together for such cases than to run about in adverse circumstances of major vascular injury intraoperatively and endangering life of child. Meticulous dissection and precision of vascular repair by cardiothoracic surgeon may improve resectability rates and survival chances of such pediatric tumors with vascular involvement. Need for vascular reconstruction with grafts or for cardiopulmonary bypass requires them to be in and around during the excision of such tumors.

Albeit a few adverse effects, combined approach may lead to a time-consuming procedure for management of such patients. It may create confusion for the child and difference of opinion for management may occur.

Overall a team work makes the management of cases of pediatric tumors more efficient and promising for better results in long run. The trained personal in their respected fields can offer the best for the sick child in the multidisciplinary approach and thereby reducing chances of complications.

Conclusion

Multidisciplinary approach for management of pediatric tumors can lead to planned and prompt management of tumors with better outcomes in terms of morbidity and survival.