Accuracy of contrast-enhanced CT in liver neoplasms in children under 2 years age.

BACKGROUND: Multiple differentials exist for pediatric liver tumors under 2 years. Accurate imaging diagnosis may obviate the need for tissue sampling in most cases. OBJECTIVE: To evaluate the imaging features and diagnostic accuracy of computed tomography (CT) in liver tumors in children under 2 years. METHODS: Eighty-eight children under 2 years with treatment naive liver neoplasms and baseline contrast-enhanced CT were included in this institutional review board approved retrospective study. Two blinded onco-radiologists assessed these tumors in consensus. Findings assessed included enhancement pattern, lobulated appearance, cystic change, calcifications, central scar-like appearance, and metastases. The radiologists classified the lesion as hepatoblastoma, infantile hemangioma, mesenchymal hamartoma, rhabdoid tumor, or indeterminate, first based purely on imaging and then after alpha-fetoprotein (AFP) correlation. Multivariate analysis and methods of comparing means and frequencies were used for statistical analysis wherever applicable. Diagnostic accuracy, sensitivity, and positive predictive values were analyzed. RESULTS: The mean age of the sample was 11.4 months (95% CI, 10.9-11.8) with 50/88 (57%) boys. The study included 72 hepatoblastomas, 6 hemangiomas, 4 mesenchymal hamartomas, and 6 rhabdoid tumors. Presence of calcifications, multilobular pattern of arterial enhancement, lobulated morphology, and central scar-like appearance was significantly associated with hepatoblastomas (P-value < 0.05). Fourteen out of eighty-eight lesions were called indeterminate based on imaging alone; six lesions remained indeterminate after AFP correlation. Pure radiology-based diagnostic accuracy was 81.8% (95% CI, 72.2-89.2%), which increased to 92.1% (95% CI, 84.3-96.7%) (P-value > 0.05) after AFP correlation, with one hepatoblastoma misdiagnosed as a rhabdoid tumor. If indeterminate lesions were excluded for biopsy, the accuracy would be 98.8% (95% CI, 93.4-99.9%). CONCLUSION: CT had high accuracy for diagnosing liver neoplasms in the under 2-year age population after AFP correlation. Certain imaging features were significantly associated with the diagnosis of hepatoblastoma. A policy of biopsying only indeterminate lesions after CT and AFP correlation would avoid sampling in the majority of patients.

Outcomes of Elective Major Cancer Surgery During COVID 19 at Tata Memorial Centre: Implications for Cancer Care Policy.

BACKGROUND: Overburdened systems and concerns of adverse outcomes have resulted in deferred cancer surgeries with devastating consequences. In this COVID pandemic, the decision to continue elective cancer surgeries, and their subsequent outcomes, are sparsely reported from hotspots. METHODS: A prospective database of the Department of Surgical Oncology was analysed from March 23rd to April 30th, 2020. FINDINGS: Four hundred ninety-four elective surgeries were performed (377 untested and 117 tested for Covid 19 before surgery). Median age was 48 years with 13% (n = 64) above the age of 60 years. Sixty-eight percent patients were American Society of Anaesthesiology (ASA) grade I. As per surgical complexity grading, 71 (14·4%) cases were lower grade (I-III) and 423 (85.6%) were higher grade complex surgeries (IV - VI).Clavien-Dindo ≥ grade III complications were 5.6% (n = 28) and there were no postoperative deaths. Patients >60 years documented 9.3% major complications compared to 5.2% in <60 years (P = 0.169). The median hospital stay was 1 to 9 days across specialties.Postoperatively, 26 patients were tested for COVID 19 and 6 tested positive. They all had higher grade surgeries but none required escalated or intensive care treatment related to COVID infection. INTERPRETATION: A combination of scientific and administrative rationale contributed to favorable outcomes after major elective cancer surgeries. These results support the continuation of elective major cancer surgery in regions with Covid 19 trends similar to India.

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.

Outcomes and complications of surgery in patients with intermediate-risk neuroblastoma: experience from an Indian tertiary Cancer Centre.

PURPOSE: The treatment of intermediate risk (IR) neuroblastoma has evolved with the focus now on reducing the drugs, dosage, and duration of chemotherapy. The aim of this study is to present the outcomes of treatment and the complications of surgery in patients with IR neuroblastoma treated at a tertiary cancer center in India. METHODS: All eligible patients with IR neuroblastoma treated between April 2005 and August 2016 were identified. The presence and number of image-defined risk factors (IDRF) before and after neoadjuvant chemotherapy were retrospectively analyzed as were the extent of surgery, complications, and outcomes. RESULTS: Of 282 neuroblastoma patients treated during the study period, 54 had IR neuroblastoma. Complete excision was achieved in 25 patients. There were 26 surgical complications in 22 patients with a similar incidence in patients with complete (n = 13) or incomplete (n = 13) resection (p = 0.78). After a median follow-up of 47 months, the 4-year overall and event-free survival was 91.5% and 75%, respectively. There was no difference in survival between patients who underwent complete resection versus those with incomplete resection (p = 0.9). CONCLUSION: Outcomes of IR neuroblastoma are favorable. The extent of resection does not affect the survival and complications can occur even when the resection is incomplete.

Transversus abdominis plane catheters for postoperative pain relief in pediatric patients.

Regional techniques provides excellent post operative pain relief in pediatric patients. Transversus abdominis plane (TAP) block is a newer regional technique available. Though there is emerging evidence proving the efficacy of TAP blocks, there is limited literature on use of TAP catheters in pediatric patients. TAP catheters were placed in two children following laparotomy with transverse incisions and in both epidural was avoided, with good post operative pain relief. Ultrasound guidance was used in one child, while in the other the catheter was placed under direct vision after dissection of the plane between transversus abdominis and internal oblique. Intermittent boluses of high volumes of local anesthetic (0.6-0.7 ml/kg) were used through the TAP catheter, ensuring that the maximum permissible level of bupivacaine was not exceeded. In adults, continuous abdominal catheters have found a place for post-operative pain management, when epidural analgesia is contraindicated. At present, the use of TAP catheters by pediatric anesthesiologists is limited, though there exists diverse clinical scenarios when these catheters may be of benefit. Contraindication of neuraxial blockade and septic patients are the two scenarios we have reported. In conclusion, TAP catheters are effective analgesia technique for laparotomies with transverse incision in pediatric patients.

Rhabdomyosarcoma of the breast: Report of two cases with the review of literature.

Rhabdomyosarcoma (RMS) is a common soft-tissue neoplasm in the pediatric age group. Common locations are head and neck, genitourinary areas, trunk, and extremities. Two pathologic variants of this malignancy are embryonal and alveolar. The involvement of breast is rare. Herein, we report two cases of alveolar RMS of the breast of which one is an isolated breast metastasis from an orbital primary, whereas the other is a primary RMS of the breast. Both the patients were treated with surgery followed by adjuvant chemotherapy and radiotherapy and are currently disease free at six and three year respectively, following completion of treatment.

Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma.

Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar resemblance. We report a case of a 4-year-old boy with a right hypochondriac region mass, which was diagnosed as hepatoblastoma on the basis of imaging, raised tumor marker, and biopsy; however, pancreatic origin of the mass was ascertained on exploration and pancreatoblastoma was confirmed on histopathology.

Aggressive cervical neuroblastoma with a rare paraneoplastic syndrome: A therapeutic dilemma.

Neuroblastoma is infrequently associated with paraneoplastic syndromes. Amongst the few, opsomyoclonus (Kinsbourne syndrome) is the most common neurological paraneoplastic syndrome and diarrhea secondary to increased secretion of vasoactive intestinal peptide (Kerner-Morrison syndrome), hormonal paraneoplastic syndrome. Hypothalamic dysfunction (HD) is a rare disorder and its manifestation as a paraneoplastic syndrome of neuroblastoma is uncommonly reported. We present an interesting case of an unrelenting cervical neuroblastoma associated with HD, which posed a therapeutic challenge.

PRETEXT II-III multifocal hepatoblastoma: significance of resection of satellite lesions irrespective of their disappearance after chemotherapy.

PURPOSE: To determine the outcomes of resection of satellite lesions in PRE-treatment Tumor EXTension (PRETEXT) II and III multifocal hepatoblastoma irrespective of their disappearance after chemotherapy. To compare the overall outcomes of multifocal and unifocal hepatoblastoma. METHODS: Fourteen patients with PRETEXT II (n = 7) and III (n = 7) multifocal hepatoblastoma treated between April 2006 and July 2014 were analyzed and their outcomes were compared with PRETEXT II and III unifocal hepatoblastoma treated in the similar period. RESULTS: Satellite lesion or the affected segments with disappeared satellite lesions were resected in 11 patients. Amongst them, all relapses were distant except one in the liver. In contrast, two of three patients developed liver relapse when the affected segments were not resected. None of the patients receiving intensive chemotherapy based on SIOPEL-3 guidelines developed a relapse. The 3-year event-free and overall survival were 38.6 and 42.9% in multifocal hepatoblastoma and 86.4 and 92.4% in unifocal hepatoblastoma (p = 0.001). Multifocality (p = 0.002) and AFP >10,000 after induction chemotherapy significantly affected event-free survival (p = 0.01). CONCLUSION: Multifocal hepatoblastoma is associated with poor outcomes as compared to unifocal hepatoblastoma. These preliminary observations of relapse and the role of chemotherapy intensification deserve further study in a multicenter controlled trial setting.

Benign liver tumors in children: outcomes after resection.

PURPOSE: Benign liver tumors in children are rare and data regarding the surgical outcomes are deficient. We reviewed our experience in the management of these tumors particularly the extent, safety, and effectiveness of surgical resection. METHODS: Between March 2005 and March 2014, 10/90 liver resections were performed for benign liver tumors. Three other patients received conservative treatment. Demographic characteristics, operative management, morbidity, and mortality were analyzed. RESULTS: The median age of the patients was 12 months. The distribution of pathology was infantile hepatic hemangioendothelioma/hemangioma (n = 7), mesenchymal hamartoma (n = 4), adenoma (n = 1) and focal nodular hyperplasia (n = 1). Median tumor size was 12.25 cm (range 3.5-21 cm) with a median tumor volume of 576.64 cm(3) (range 13.9-1822.64 cm(3)). Non-anatomic resection was performed in 6/10 patients and the median blood loss was 100 ml (range 10-850 ml). Median length of hospital stay was 7 days (range 5-9 days). There were no mortality, none of the patients had a local recurrence, and all are alive at a median follow-up of 33 months. CONCLUSIONS: Two-third patients with benign liver tumors were managed with surgical excision, which comprised 11% of our resectional practice. Non-anatomical resection whenever feasible can be performed safely and does not compromise the oncological outcomes.

Non-rhabdomyosarcoma soft-tissue sarcomas in children: Contemporary appraisal and experience from a single centre.

Nonrhabdmyosarcoma soft tissue sarcomas (NRSTS) include a cluster of different types of soft tissue sarcomas clubbed together due to the rarity of individual subtypes. The diagnostic accuracy is lately reinforced due to the availability of immunohistochemical and molecular markers. Surgery is the central modality of treatment since many of them are insensitive to chemotherapy. With the availability of rational risk stratification system, efforts are in progress to evaluate the role of neoadjuvant chemotherapy and radiotherapy to improve outcomes especially for the locally advanced disease. The survival remains dismal for metastatic disease. This review highlights the current status of NRSTS and also describes the experience from a single centre in treatment of NRSTS.

Primary non-metastatic Ewing sarcoma of the jaw in children: results of surgical resection and primary reconstruction.

BACKGROUND AND OBJECTIVE: The rarity of Ewing sarcoma (ES) of the jaw coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data on surgical management of these tumors. The purpose of this study is to describe the results of surgical excision and reconstruction of primary non-metastatic ES of the mandible and maxilla in children. METHODS: Consecutive patients (mandible = 6, maxilla = 5) treated with surgery from August 2005 to January 2013 were selected. All patients received induction chemotherapy and were selected for surgical resection based on the presence of specific criteria for operability. RESULTS: The median age was 11.5 years (range 5-16 years). Free fibular osteocutaneous flap was commonly used for reconstruction. There were no complications related to microvascular anastomosis or flap loss. Five patients had 100% tumor necrosis and did not receive radiotherapy. Teeth alignment, chewing, swallowing, and speech were normal in all and donor site morbidity occurred in one. The 5-year overall, event-free survival, and local control are 87.5%, 72.9%, and 90%, respectively. CONCLUSION: In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcome. Surgery also has the added advantage of identifying patients who may not need radiotherapy.

Outcome and morbidity of surgical resection of primary cervical and cervicothoracic neuroblastoma in children: a comparative analysis.

INTRODUCTION: Primary cervical (CN) and cervicothoracic neuroblastoma (CTN) is generally associated with good outcome; however, surgical resection can be challenging and not without morbidity. The aim of this study is to assess the overall outcome and compare the clinico-radiological features, treatment, and complications of CN and CTN. MATERIALS AND METHODS: Sixteen consecutive patients, (CN = 9, CTN = 7) treated between November 2006 and December 2012 were selected from the prospective database for this analysis. RESULTS: The 2-year overall and event-free survival of entire cohort is 100 and 72 %, respectively. Respiratory symptoms due to compression of airway and intraspinal extension were common in CTN. Gross total resection was feasible in all patients with CN; in contrast, incomplete excision along with significantly longer duration of surgery and more blood loss occurred in CTN. Postoperative morbidity was seen in three patients with CN and only one patient with CTN. The extent of surgery did not affect the overall and event-free survival of CTN (p = NS). CONCLUSION: CN and CTN have characteristic clinico-radiological presentation and surgical specification. However, both have a favorable outcome, even though with a distinct but acceptable morbidity. The favorable outcome in CTN is unrelated to the extent of surgical excision.

Desmoid Fibromatosis of the Oesophagus Creating an Oesophageal Diverticulum in a 2-year-old Girl.

ABSTRACT: Extra-abdominal desmoid fibromatosis arising from the oesophagus and the contemporaneous traction diverticula due to an oesophageal tumour is extremely rare. We present this complex situation in a 2-year-old girl which posed a surgical challenge requiring simultaneous management of multiple pathologies. Surgery addressed both the entities and the presence of the diverticulum facilitated achieving negative surgical margins.

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.

Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin.

PURPOSE: The aim of the study was to determine the impact of negative but close resection margins on local recurrence in children with extraskeletal Ewing sarcoma (EES). METHOD: We reviewed records of 32 patients with EES treated between March 2005 and March 2013. All patients except one underwent surgical excision either upfront or after induction chemotherapy. Patients with viable tumor and negative surgical margins, which were categorized as less than or greater than 1 cm, were selected. Local control and survival analysis were performed for patients in both the groups. RESULTS: The 5-year event-free and overall survival rates of entire cohort is 68 and 77 %, respectively. Surgical margins were negative in 23/26 (90.3 %) patients. There were no local recurrences in any of the patients with margins of less than 1 cm. Only one patient with a margin greater than 1 cm had a local recurrence along with distant metastases. A tumor-free margin of more than 1 cm did not affect overall or event-free survival (p = NS). CONCLUSION: Optimal local control is feasible in children with EES regardless of the quantitative extent of negative margins. Achieving a three-dimensional tumor-free margin should be the goal of surgical resection.

Factors Influencing Cervical Lymph Node Metastasis in Pediatric Differentiated Thyroid Cancers.

Lymph node metastasis is a considerable variable influencing postoperative American Thyroid Association (ATA) risk stratification in pediatric differentiated thyroid cancer (DTC). The primary aim of this study was to ascertain the factors predicting nodal metastasis and describe the outcomes in relation to the ATA risk. Patients 18 years or younger operated between December 2005 and December 2019 were analyzed. Demographic, clinicopathological, treatment, and outcome data were recorded. Factors associated with nodal metastasis were assessed by univariate and multivariate regression analysis. Patients were stratified into low-, intermediate-, and high-risk as per the pediatric ATA guidelines. A total of 86 patients (43% male; median [IQR] age, 12 (10-14) years) underwent surgery during the study period. Lymph node metastases were present in 70 (82.4%) patients involving the lateral (8%) and central compartment (4.7%) alone and both (88.6%) compartments. Extrathyroid extension (ETE) was present in 65%; 35%, minimal; and 30%, extensive. On univariate analysis, nodal metastasis was more frequent in male patients, multifocal tumor, lymphovascular invasion, and ETE. On multivariate analysis, only ETE was predictive of nodal disease with an odds ratio of 8. Minimal and extensive ETEs were both significantly associated with lymph node metastases when compared to the absence of ETE. The 5-year disease-free survival was 100%, 95.7%, and 66% in the low-, intermediate-, and high-risk groups respectively (p < 0.0001). Pediatric DTCs have an exceptionally high incidence of lymph node metastasis. ETE is the single most important predictor of nodal disease. The ATA pediatric risk stratification is useful in predicting clinical outcomes.