Impact of Surgical Timing (Primary, Delayed, or Second Look) on Surgical Morbidity and Outcomes in Malignant Germ Cell Tumor of the Ovary in Children.

BACKGROUND: Malignant ovarian germ cell tumors (MOGCT) are rare in children. Surgery with or without chemotherapy is the primary treatment approach. This study aimed to analyze the impact of primary and delayed surgery on surgical morbidity and outcomes. Second-look surgery after inadequate surgical staging and the various components of surgical staging were also evaluated. METHODS: Children below 15 years with MOGCT treated between 2006 and 2022 were analyzed. A comparison of patients undergoing primary, delayed, and second-look surgery was performed. RESULTS: 118 patients with a median age of 12 (0.11-15) years were eligible. Forty patients underwent primary, 51 delayed, and 27 second-look surgeries. Overall complications, including tumor rupture, blood loss, and adjacent organ removal, were significantly higher in the primary compared to the delayed surgery group (p = 0.0001). Second-look surgery conceded more blood loss (p = 0.0001), extended duration (p = 0.03), and complications (p = 0.004) than delayed surgery. The compliance with surgical guidelines was 100% for most components, with a positive yield rate of 10-80%. At a median follow-up of 5.2 years, the 5-year event-free survival (EFS) and overall survival (OS) for the entire cohort are 86% and 89%, respectively. The OS and EFS did not differ by the timing of surgery, although the second-look surgery demonstrated relatively inferior outcomes consequential to initial suboptimal surgery. CONCLUSIONS: MOGCT shows favorable outcomes. Delayed surgery after chemotherapy in appropriately selected patients minimizes the morbidity of surgery with similar outcomes compared to primary surgery. An optimal initial surgery is essential since second-look surgery produces significant morbidity. Prognosis Study, Level II evidence.

Feasibility of Nonanatomical Liver Resection in Diligently Selected Patients with Hepatoblastoma and Comparison of Outcomes with Anatomic Resection.

INTRODUCTION: Treatment guidelines for hepatoblastoma discourage nonanatomic liver resections. However, the evidence for this is inadequate and comes from a study performed almost two decades ago which additionally contained inherent limitations. This study aimed to assess the feasibility and oncologic outcomes of nonanatomic resections (NAR) performed in diligently selected patients and compare the results with anatomic resections (AR). MATERIALS AND METHODS: A total of 120 patients who underwent liver resections for hepatoblastoma between January 2008 and July 2019 were reviewed. Feasibility of NAR was based on postchemotherapy relations to vessels, site of the lesion, and possibility of achieving negative resection margins. RESULTS: AR was performed in 95 patients and 25 had NAR. The NAR cohort had similar International Childhood Liver Tumors Strategy Group (SIOPEL) risk group distribution. Blood loss and operative times were lower in patients undergoing NAR. No differences were noted between the two groups concerning postoperative morbidity and hospitalization. There were no pathologic positive margins or local recurrences in the NAR patients. Relapse free (RFS) and overall survival (OS) was similar in the two groups (p = 0.54 and 0.96, respectively). Subgroup analysis of only posttreatment extent of tumor (POSTTEXT) I and II patients also showed no difference in RFS or OS for the two groups with a persistent significant difference in operative times and blood loss. CONCLUSION: NAR is feasible with clear margins in carefully selected patients. It is not associated with more complications and outcomes are not inferior to AR. NAR is associated with lesser blood loss and operative time.

Intravascular extension of Wilms tumor: Characteristics of tumor thrombus and their impact on outcomes.

BACKGROUND: Studies describing intravascular involvement in Wilms tumor have focused on illustrating individual institutional experience and the elements of surgical management. Thrombus characteristics like extent, patterns of regression, and correlation with the surgical findings, intraluminal adhesion, and viable tumor in the thrombus, and patency of the inferior vena cava (IVC) have not been systematically described. OBJECTIVES: The aim of this study is to evaluate these thrombus characteristics and explore their impact on the overall outcomes. METHODS: All patients with histologically confirmed Wilms tumors with intravascular thrombus diagnosed in the pediatric oncology unit of Tata Memorial Hospital registered from 2006 to 2019 were included. Data regarding clinical, radiological, and surgical particulars were retrieved from the prospectively maintained institutional database. Specific data for the thrombus included: distal extent before and after neoadjuvant chemotherapy, correlation of extent with the surgical findings, completeness of thrombectomy, the presence of a viable tumor in the thrombus, and the patency of the IVC. Survival analysis was performed utilizing the Kaplan-Meier method on SPSS software version 25. RESULTS: The study included 43 (9.9%) of the 432 patients with Wilms tumor having intravascular extension. Retrohepatic IVC (33.3%) followed by atrioventricular (26%) formed the frequent levels of thrombus with maximum regression occurring after chemotherapy in the latter (Summary figure). The overall concordance rate between computed tomography (CT) scan and surgical findings for the presence of thrombus was 86% and 4 patients had the thrombus limited to a lower level than the preoperative scan. At a median follow-up of 5-years, the 5-year event-free and overall survival was 81% and 82.2% respectively. Atrioventricular thrombus (p = 0.003) and postoperative patency of IVC (p = 0.02) were significantly associated with inferior survival, while the extent of regression, thrombus fracture, and viability was not significant. DISCUSSION: The findings of this study bring forth the characteristics of intravascular tumor thrombus affecting the outcomes which can be validated in future prospective studies. Although the ideal method for radiological assessment of the intravascular thrombus is elusive, CT scan provided adequate information for the presence and level of the intravascular thrombus with reasonable accuracy in this study. Study limitations include small sample size, the limited number of events, and lack of multivariate analysis to rule out confounding factors that could influence the observed findings. CONCLUSION: Atrioventricular thrombus and occlusion of IVC represent adverse prognostic factors. The extent of regression, fracture, and viability of thrombus did not affect survival in this study.

Standardizing lymph nodal sampling for Wilms tumor: A feasibility study with outcomes.

BACKGROUND: Despite being mandated by cooperative groups, omission of nodal sampling is the most frequent protocol deviation in surgery for Wilms tumor. The stations as well as the number of nodes that should be sampled are not clearly defined resulting in a marked variation in practices among surgeons. We propose a systematic method for nodal sampling intending to reduce interoperator variation. In this study, we have assessed the feasibility and yield of systematic lymph node sampling and also evaluated the factors influencing nodal metastasis. METHODS: Prospective evaluation of 113 Wilms tumor patients operated at a single tertiary cancer center between 2015 and 2019. All these patients underwent a systematic 5-station nodal sampling. RESULTS: Median lymph node yield was 8 and 13.2% (15/113) patients harbored a histologically positive nodal disease. Of the patients with positive nodal disease, interaortocaval nodes had metastasis in 46.7% (n = 7). They represented isolated sites of nodal disease (skip metastases) in 28.6% (n = 4) of patients. Right-sided tumors had more frequent involvement of interaortocaval nodes and skip disease. Tumors with high-risk histology had 12.5 times more odds of harboring nodal disease as compared to low and intermediate-risk histology Wilms tumor. CONCLUSIONS: The proposed method of systematic station wise sampling provides a template to guide surgeons in performing lymph node harvesting. Interaortocaval nodes sampling should be performed routinely as the incidence of disease at this station is sufficiently high and metastasis may skip hilar nodes. STUDY OF DIAGNOSTIC TEST: Level III evidence.

Customized approach for upfront or delayed resection using radiological criteria in unilateral, nonmetastatic pediatric renal tumors: A prospective study.

BACKGROUND: The availability of robust, equivalent data regarding outcomes for upfront or delayed surgery for renal tumors in children leads to a dilemma in selecting the initial treatment. Imaging criteria associated with the probability of rupture or incomplete resection may provide a more objective assessment for customization for the timing of surgery. PROCEDURE: Eighty-three children with unilateral, nonmetastatic renal tumors were enrolled between January 2012 and April 2018. Upfront nephrectomy was performed in the absence or delayed surgery (after a biopsy and chemotherapy) in the presence of one or more imaging-based high-risk features, including perinephric spread or adjacent organ infiltration, tumors crossing the midline, intravascular thrombus, and extensive adenopathy. Post hoc analysis for interobserver concordance for high-risk imaging features was also performed. RESULTS: The upfront surgery group (19) had predominantly stage I or II diseases (89%) and the histological types were Wilms (13), non-Wilms (5) renal tumor, and an inflammatory lesion. The delayed surgery group had 60% with stage I or II diseases and the histological types were Wilms (60) and non-Wilms (4) tumor. In addition, high-risk pathology was identified in nine patients. Overall, 27 patients with Wilms tumors required radiotherapy and anthracycline because of stage III disease, including one in the immediate surgery group. The event-free and overall survival (OS) at a median follow-up of 39 months for Wilms tumor are 88% (95% confidence interval [CI]: 78.5-94.9%) and 89% (95% CI: 81.4-96.6%), 85.1% (95% CI: 73.8-93.4%) and 86.5% (95% CI: 77.4-95.8%) for the delayed, and 100% event-free survival as well as OS (P = .1) in the upfront surgery group. CONCLUSION: A customized approach pivoted on image-based high-risk features facilitates identification of patients with early-stage renal tumor when the timing of surgery is tailored. Moreover, non-Wilms tumor and high-risk pathology are also identified.

A cross-sectional study of the distribution of pediatric solid tumors at an Indian tertiary cancer center.

CONTEXT: Pediatric solid tumors include a heterogeneous group of tumors, and the burden of these tumors, especially from resource-challenged countries, is not well described. AIMS: The aim of this study was to describe the distribution of solid tumors in children and the treatment outcome of Wilms tumor and hepatoblastoma. PATIENTS AND METHODS: All patients under 15 years of age with histologically confirmed tumors presenting at a tertiary cancer center from January 2012 to December 2016 were identified from the hospital database. Patients with lymphomas, bone, and central nervous tumors were excluded. The demographic profile including age, sex distribution, and the treatment received were recorded for all patients. RESULTS: The mean age of the eligible 1944 patients was 5.7 years with majority (57.3%) in the 0-4 years age group. The male-to-female ratio was 1.4:1 with a male predominance in all tumors except germ cell tumors. Soft tissue tumors were the most common tumors followed by neuroblastoma and renal tumors, whereas liver tumors formed only 6.7% of all tumors. Seventy percent of the patients received treatment completely or partially at our institute, whereas 18.3% had no cancer-directed treatment. The 3-year overall survival of patients with Wilms tumor and hepatoblastoma was 85.4 and 78.5%, respectively. CONCLUSIONS: Extracranial and extraosseous pediatric solid tumors include a wide range of tumors with a predilection for male sex and children below 4 years of age. Soft tissue tumors, neuroblastoma, and renal tumors are the most common; the outcomes of Wilms tumor and hepatoblastoma are favorable.

The effect of chemotherapy on the mammographic appearance of breast cancer and correlation with histopathology.

OBJECTIVE: To document the mammographic changes after neoadjuvant chemotherapy with histopathological correlation, to calculate the accuracy of mammography (MG) in predicting residual tumour size and to measure the interobserver agreement in reading mammograms. METHODS: In 446 consecutive cases, the pre- and post-chemotherapy mammograms were retrospectively evaluated by two blinded observers, and consensus findings were compared with reference standard of surgical specimen. The accuracy of MG in predicting residual tumour size was calculated. Kappa statistics were calculated for measuring the interobserver agreement for reading mammograms. The sensitivity, specificity, positive-predictive value and negative-predictive value for the prediction of residual disease were calculated. RESULTS: The most common primary abnormalities were mass lesions without and with microcalcifications. After chemotherapy, there was decrease in size of most (95.1%) of the measurable masses, with decrease in the mean tumour size from 4.1 to 2.5 cm. The density of the tumour decreased in 66.6% (241/362) cases with residual disease. There was almost perfect interobserver agreement for describing the primary abnormality in the pre- as well as post-chemotherapy mammograms (k = 0.87 and 0.81, respectively) with substantial agreement for measurement of the mass lesions before and after chemotherapy (k = 0.69 and 0.68, respectively). MG showed accuracy of 60.0%, sensitivity of 94.4%, specificity of 50.0%, positive-predictive value of 91.3% and negative-predictive value of 61.8%. CONCLUSION: MG remains a highly sensitive and reproducible investigation for the assessment of residual disease after chemotherapy. ADVANCES IN KNOWLEDGE: There is substantial interobserver agreement in characterizing and measuring breast tumours on mammograms.

Imaging in neuroblastoma: An update.

Neuroblastoma is the third common tumor in children. Imaging plays an important role in the diagnosis, staging, treatment planning, response evaluation and in follow-up of a case of Neuroblastoma. The International Neuroblastoma Risk Group task force has recently introduced an imaging-based staging system and laid down guidelines for uniform reporting of imaging studies. This review is an update on imaging in neuroblastoma, with emphasis on these guidelines.

Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt.

Although rare, pancreatoblastoma is the most common pancreatic tumor in children. Cushing syndrome secondary to ectopic secretion of adrenocorticotropic hormone (ACTH) from a pancreatoblastoma is very rare with only two previously reported cases. We present the management and the lesson learnt in a 3-year-old child with recurrent pancreatoblastoma with Cushing syndrome.

Reactive intramammary lymph node mimicking recurrence on MRI study in a patient with prior breast conservation therapy.

Breast conservative therapy (BCT) is a well accepted form of treatment for patients with early stage breast cancer. The incidence of ipsilateral breast tumor recurrence is higher in patients undergoing BCT than in those patients undergoing Modified Radical Mastectomy (MRM) without any adverse effect on survival. Patients treated with BCT are put on active surveillance using clinical breast examination and mammography. The radiologist reading the follow-up mammograms is on high alert and any neo-density is viewed with suspicion. MRI may be used as a problem solving tool. At such a time, an innocuous intra-mammary node can mimic malignancy on MRI. We want to showcase one such typical example with histological proof and highlight that type III curve may be seen in an intramammary node. Our case also reinforces the utility of second look ultrasound which is a faster, cheaper and easier method for localization and biopsy of abnormalities seen on MRI.

Pictorial essay: Acute neurological complications in children with acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) is the commonest childhood malignancy with high cure rates due to recent advances in central nervous system (CNS) prophylaxis. The disease per se, as well as the prophylactic therapy, predisposes the child to complications such as cerebrovascular events, infections, drug toxicities, etc. The purpose of this study is to highlight the pathophysiology and the imaging features (with appropriate examples) of these complications and to propose a diagnostic algorithm based on MRI. Interpreting these scans in the light of clinical inputs very often helps the radiologist reach an appropriate diagnosis and help treatment and management.