Cardiac function in pediatric Ewing sarcoma during and after treatment: A longitudinal study.

BACKGROUND: While anthracycline therapy has been shown to improve outcomes in Ewing sarcoma, it may be associated with severe and even fatal cardiac dysfunction. We evaluated the burden and determinants of cardiac dysfunction in pediatric Ewing sarcoma (pES). METHODS: This retrospective study included children aged 0-18 years with pES treated at our center with the EFT 2001 protocol (anthracycline and cyclophosphamide containing regimen), with/without radiation therapy from January 2001 to December 2018. Cardiac dysfunction was defined as left ventricular (LV) ejection fraction with an absolute value <50%. RESULTS: Amongst 650 eligible patients (median age at diagnosis 12 years and median follow-up duration 69 months), 85 (13%) developed cardiac dysfunction, at a median 13 months (range: 1-168 months). The cumulative incidence of cardiac dysfunction was 5.7% at 12 months, 12% at 2 years, 13% at 3 years, 14% at 5 years, and 15 % at 10 years. At a median follow-up duration of 25 (range: 3-212) months, 21 (24.7%) patients had normalization of LV function, whereas nine (10.6%) patients died of cardiac causes. Older age at diagnosis (7-12 years OR 5.1, p = .01, 13-18 years, OR 3.9, p = .03), female sex (OR 2.3, p = .004), undernutrition (OR 2.9, p = .001), and chest wall location (OR 8.7, p = .08) were risk factors for cardiac dysfunction. CONCLUSIONS: Children with Ewing sarcoma have a high incidence of cardiac dysfunction, which continues to develop even years after therapy, underlining the need for life-long surveillance. Undernourished children are at a higher risk for cardiac dysfunction and need stringent monitoring.

Solitary Bone Plasmacytoma of Humerus Presenting as a Nonhealing Fracture in a Child: A Rare Entity.

Solitary bone plasmacytoma is an extremely rare entity and is characterized by localized proliferation of monoclonal plasma cells. Plasmacytomas are extremely rare in the pediatric population. The median age at diagnosis is usually the fifth or sixth decade, with axial skeleton being more commonly involved than appendicular. We hereby, report the case of a 13-year-old boy with solitary bone plasmacytoma of the right humerus. Though extremely rare in the pediatric age group, plasmacytomas may be considered as one of the remote differentials in children presenting with solitary bone tumors.

Reconstructing diaphyseal tumors using radiated (50 Gy) autogenous tumor bone graft.

AIMS: Establish oncologic safety using 50 Gy to sterilize tumor bearing bone before reimplantation in primary diaphyseal high grade extremity tumors, determine extracorporeal radiotherapy (ECRT) graft survival, and analyze factors that affect union at osteotomy sites. MATERIALS AND METHODS: Seventy non metastatic patients underwent reconstruction with intercalary ECRT grafts sterilized with 50 Gy. Diagnosis included osteosarcoma (38) and Ewing's sarcoma (32). At last follow-up-49 patients were alive, 19 had died and 2 were lost to follow up. Survivors had minimum follow up of 3 years (range 39-127 months). RESULTS: Ninty one percent metaphyseal osteotomies united without additional intervention compared to 71% diaphyseal osteotomies. Additional small plate at diaphyseal osteotomy apparently reduced incidence of non-union (17% vs 31%) (P = 0.49). Addition of morsellised allograft did not help union. There were seven (10%) local recurrences, all in soft tissue. Seventeen of 69 patients (25%) needed removal of ECRT graft. Five year survival for ECRT graft (removal for all causes) was 79% and 84% (excluding removal for local recurrence). CONCLUSIONS: Reimplanting sterilized tumor bone using 50 Gy for tumor ablation is an easily applicable, oncologically safe, biological reconstruction option for primary diaphyseal extremity tumors.

Unfavorable presentation but comparable outcome: Presentation and outcome of children with nodular lymphocyte predominant Hodgkin lymphoma from India.

BACKGROUND: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an uncommon subtype of Hodgkin lymphoma (HL) with few published studies in children, entirely from North America and Europe. We analyzed clinical features and treatment outcome of pediatric NLPHL. PROCEDURE: Children less than 18 years of age diagnosed after histopathology review to have NLPHL between June 1998 and August 2016 were retrospectively analyzed. Descriptive details of clinical presentation and treatment were collected, and outcomes analyzed using Kaplan-Meier survival analysis. RESULTS: Of the 42 patients with a confirmed diagnosis of NLPHL during this period, there was complete information on 35. Median age was 11 years (range 6-16 years), male:female ratio was 4.8:1, there were 15, 11, 6, and 3 patients with Stage I, Stage II, Stage III, and Stage IV disease, respectively. Six patients had B symptoms, 10 had bulky disease, and 3 had bone marrow as well as extranodal involvement. Histology was typical NLPHL in 23 and variant in 12. Twenty-nine received chemotherapy, 10 with additional radiation, 3 patients with early stage disease received only radiotherapy and three others underwent complete node resection alone. Median follow-up was 55 months (range 7-165 months), 5 year event-free survival (EFS) was 83.3%, and overall survival 97.1%. Variant NLPHL histology was associated with higher incidence of unfavorable presentation and lower EFS. CONCLUSIONS: NLPHL in India has an excellent outcome, despite a higher incidence of unfavorable presentations such as advanced stage disease, B symptoms, and bulky disease. Variant histology is an adverse prognostic factor.

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.

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.

A Retrospective Cohort Study to Evaluate the Outcomes of HIV-Associated High-Grade B-Cell Non-Hodgkin Lymphoma (NHL) Treated with Dose Adjusted EPOCH (+/-R) Regimen.

High-grade B-cell NHL's are more common in seropositive patients. They are biologically different from their seronegative counterparts. We report our analysis on our cohort of patients who were treated with DA-EPOCH(+/-R). We retrospectively analyzed treatment-naïve HIV-associated High-grade B-cell NHL patients (aged ≥ 18) treated with DA-EPOCH(+/-R) regimen from 2011 to 2015. Descriptive statistics were summarized with median and range; survival outcomes were analyzed with Kaplan-Meier method. The cohort comprised of 40 patients [DLBCL(19), Burkitt's Lymphoma(16), High-grade B-Cell Lymphoma-Unclassifiable(09), and Plasmablastic Lymphoma(01)] and the median CD4 + T cell count was 202/mm3. CNS prophylaxis was administered with intrathecal methotrexate to 90% of patients. With a median follow-up of 72 months, an estimated 5-year OS was 82.5%, and 5-PFS was 77.5%. There were 9 deaths, and 9 patients had progression. At least 4 cycles of chemotherapy were administered to 35 (93%) patients, with 28 (70%) receiving 6 cycles. Grade 3-4 toxicities were seen in 33 (83%) patients- febrile neutropenia (65%) being the most common followed by mucositis (25%) and peripheral neuropathy (13%). There was no difference in survival based on IPI, CD 4 + T cell count, CDI, or duration of HIV. DA-EPOCH(+/-R) is a highly effective regimen in seropositive high-grade B-cell lymphoma, even in the presence of adverse features. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-023-01652-3.

Evaluation of Metronomic Therapy as a Low-Cost, Sustainable, Standard-of-Care Option in Desmoid Fibromatosis: Real-World Data From a Tertiary Care Center in India.

PURPOSE: Desmoid fibromatosis (DF) is a locally aggressive tumor with low mortality but significant morbidity. There is a lack of standard of care, and existing therapies are associated with significant barriers including access, cost, and toxicities. This study aimed to explore the efficacy and safety of the metronomic therapy (MT) in DF in a large, homogenous cohort from India. PATIENTS AND METHODS: This study involved histologically confirmed DF cases treated with MT comprising vinblastine (6 mg) and methotrexate (15 mg) both once a week, and tamoxifen (40 mg/m2) in two divided doses once daily between 2002 and 2018. RESULTS: There were 315 patients with a median age of 27 years; the commonest site was extremity (142 of 315; 45.0%). There were 159 (50.1%) male patients. Of the 123 (39.0%) prior treated patients, 119 had surgery. Of 315 patients, 263 (83.5%) received treatment at our institute (MT-151, 77-local treatment, 9-tyrosine kinase inhibitor, and 26 were observed). Among the MT cohort (n = 163, 61.2%), at a median follow-up of 36 (0.5-186) months, the 3-year progression-free and overall survival were 81.1% (95% CI, 74.3 to 88.4) and 99.2% (95% CI, 97.6 to 100), respectively. There were 35% partial responses. Ninety-two patients (56.4%) completed 1-year therapy, which was an independent prognosticator (P < .0001; hazard ratio, 0.177 [95% CI, 0.083 to 0.377]). MT was well tolerated. Predominant grade ≥3 toxicities were febrile neutropenia, 12 (7.4%) without any chemotoxicity-related death. The annual cost of MT was $130 US dollars. CONCLUSION: The novel, low-cost MT qualifies as one of the effective, less toxic, sustainable, standard-of-care options for the treatment of DF with global reach and merits wide recognition.

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.

Outcomes and prognostic factors of patients with Extranodal NK T cell lymphoma (ENKTL).

The staging, prognostication, and treatment of ENKTL has evolved over the years with better understanding of the disease biology. There is significant heterogeneity in the treatment followed across the world. Literature from India have been few with small number of patients. We studied the outcomes and prognostic factors of patients with ENKTL treated between May 2010 and December 2021 at our center. A total of 78 patients diagnosed with ENKTL were treated at our center. L-asparaginase based chemotherapy was administered in 84% of the patients. Close to 2/3rd patients received SMILE chemotherapy. After a median follow-up of 30 months (18.5-41.4 months), the median relapse free survival and overall survival for the overall population was 45 months (12-118 months) and 45 months (14-118 months) respectively. By multivariate analysis, PINK score of 2-4, non-receipt of RT and non-achievement of CR were associated with poor survival.

Impact of the First Wave of COVID-19 Pandemic on Radiotherapy Practice at Tata Memorial Centre, Mumbai: A Longitudinal Cohort Study.

PURPOSE: Delivery of cancer care during the pandemic required adopting various changes in the standard management. We analyzed the impact of the first wave of the COVID-19 pandemic on radiation oncology treatment practices at Tata Memorial Hospital in India. MATERIALS AND METHODS: From March 1 to October 31, 2020, all consecutive patients who attended the radiation oncology department for radiotherapy treatment were included in this study. Electronic medical records, patient files, and telephonic consult were used to collect patient's data including changes in the standard treatment practice, COVID-19 testing and its results, and subsequent impact on radiotherapy treatment. Comparison was done with the same period data of 2019 for the number of the caseload, radiotherapy regimen, referral rates, and noncompliance rates. RESULTS: Our study included 4,256 patients with a median age of 52 years (interquartile range 41-61 years). There was a significant drop in the new-patient registrations (approximately 63%), radiotherapy consultations (44.9%), and referrals to other centers (27.8%). The reduction in the caseload was highest for genitourinary cases (-58.5%) and the lowest for breast cases (-11.5%) when compared with the 2019 cohort. Among those treated with radical intent, the noncompliance rate was 15%. Hypofractionation was the commonly adopted regimen across all sites. Compared with 2019, the maximum reduction in the average fractions per patient was seen in the breast cancer cases (-8.2 fraction), followed by genitourinary cases (-4.9 fraction). Of the 27.8% of patients tested for COVID-19, 13.4% turned positive and 3.4% died due to the disease. CONCLUSION: The COVID-19 pandemic adversely affected the number of radiotherapy consultations and treatments at our institute. However, our department offered uninterrupted services despite grave challenges. Hypofractionated regimen was used across disease sites to minimize patient visits and allow planned treatment completion. Radiotherapy was delivered safely, and patients experienced low rates of COVID positivity during radiotherapy and even lower mortality.

Assessment of outcomes of elective cancer surgeries in children during coronavirus disease 2019 pandemic: Retrospective cohort study from a tertiary cancer center in India.

ABSTRACT: To describe the outcomes of elective cancer surgeries and adverse consequences on the patients and medical staff due to the surgical interventions in children during the Coronavirus Disease 2019 (COVID-19) pandemic.The study included children younger than 15 years who underwent elective cancer surgeries from March 4, 2020 and December 3, 2020.A total of 121 patients (62% male; median age, 3 years) underwent surgery. The surgical procedures included nephrectomies (n = 18), neuroblastoma (n = 26) and soft tissue tumor resections (n = 24) and complex surgical procedures like extended liver resections (n = 2), intra-atrial thrombectomy under cardiopulmonary bypass (n = 2), pancreatoduodenectomy (n = 1), and free microvascular flaps (n = 7). Clavien-Dindo Grade III complications were 5% (n = 6), and there were no postoperative deaths. Preoperative COVID-19 testing was performed in 82% of children, and only 2% showed severe acute respiratory syndrome coronavirus 2 positivity. Postoperatively, 26 children were tested because of specific symptoms and, 6 tested positive for severe acute respiratory syndrome coronavirus 2. Except for a median delay of 23 days in treatment, none of the patients with COVID-19 required critical hospital management. None of the surgical residents or faculty acquired COVID-19, while 4 each medical and support staff were tested positive in the study period.COVID-19 was not a deterrent for continued cancer care, and surgeries could be safely performed adopting universal preventive measures without any added morbidity from COVID-19. Caregivers and centers dealing with childhood cancers can be encouraged to sustain or seek early healthcare.

Prognosis of metastasis based on age and serum analytes after follow-up of non-metastatic lung cancer patients.

At the diagnostic stage, metastasis detection is around 75% in the lung cancer patients. Major clinical challenge faced by medical oncologists is the unpredictable metastasis development in non-metastatic patients. The literature regarding the biomarkers/factors prognosticating metastasis in non-metastatic patients during follow-up is very limited. In this pilot study, the levels of serum biomarkers (IL-8, VEGF, MMP-2, MMP-9) were measured at diagnosis stage of non-metastatic lung cancer patients and these observations were evaluated for metastasis development after follow-up of median 29.2 months. After follow-up, ∼40% of these patients developed metastasis. The average age of non-metastatic patients which later developed metastasis, was found to be lower than the patients continued to be non-metastatic. These patients also showed higher levels of IL-8 and MMP-9 than the patients which did not develop metastasis. Analysis of Receiver Operating Characteristic Curves, Youden's Index and positive likelihood ratio values showed better diagnostic ability for IL-8 and MMP-9, which improved when both markers used together. Moreover, patients with age ≤60 years showed higher prognostic ability of metastasis development, which was significantly enhanced when patient age was analysed with IL-8. These results suggest potential of serum analytes (IL-8, MMP-9) and/or patient age in prognosticating the metastasis development in non-metastatic patients.

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.

Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution.

INTRODUCTION: Non-Wilms renal tumors represent a compelling subset of childhood renal tumors. However, their relative rarity renders accurate diagnosis, and therapy challenging which in some instance is inferred from their adult counterparts. OBJECTIVE: To describe the incidence and analyze the diagnostic challenges, therapies and, outcomes of non-Wilms renal tumors at the largest tertiary cancer centre in India. METHODS: All patients with histologically confirmed non-Wilms renal tumours diagnosed in the paediatric oncology unit of Tata Memorial Hospital between 2006 and 2019 were included. Data regarding clinical and radiological features and treatment outcomes were retrieved from the prospectively maintained institutional database. At the outset, histological types were categorised into a high and low-risk group depending on anticipated survival. Survival analysis was performed utilising the Kaplan-Meier method on SPSS software version 24.0. RESULTS: Of the 569 patients with renal tumors, 109 (19%) patients with primary (n = 97) or recurrent (n = 12) non-Wilms renal tumors were included. Histological high-risk group included clear cell sarcoma (CCSK) (39.4%), renal cell carcinoma (RCC) (19.3%), malignant rhabdoid tumor (MRTK) (12.8%), Ewing's sarcoma (rES) (15.6%), synovial sarcoma (2%), and undifferentiated sarcoma (2%). The low-risk group comprised of congenital mesoblastic nephroma (CMN) (4.6%), cystic partially differentiated nephroblastoma (2%), and other rare tumors (3%). Diagnostic error occurred in 2 patients in the high-risk group. All low-risk tumours were treated with surgery alone and most (97%) high-risk tumors were operated either upfront (61.5%) or after preoperative chemotherapy (38.4%). Adjuvant therapy based on histology was offered to 70%. The recurrent tumors received various salvage treatments including chemotherapy; radiotherapy; surgery and immunotherapy, however, only 2 patients could be salvaged. The 3-year overall survival for the entire cohort with primary tumors was 59%, and the survival rates were 76.7%, 77.9%, 0.0%, and 52% for CCSK, RCC, MRTK, and rES (summary figure). Low-risk tumors had 100% survival while the recurrent tumors had a median survival of 10.5 months. CONCLUSIONS: Non-Wilms renal tumors constitute a heterogeneous group of tumors, accounting for less than 20% of all renal tumors. Low-risk tumors are associated with excellent outcomes following surgery alone while the high-risk tumours have a variable outcome. MRTK and recurrent non-Wilms tumour have the worst survival. Favourable outcomes for CCSK and RCC and worst outcomes for MRTK were observed in this study. Renal ES have higher incidence of treatment failure and unsatisfactory outcomes. Recurrent non-Wilms tumours have an extremely poor outcome and more alternative or innovative approaches are needed for their treatment.

Primary diffuse large B cell lymphoma of Uterine Cervix: Treatment outcomes of a rare entity with literature review.

BACKGROUND: Primary lymphomas involving the female genital tract are rare, and those arising from cervix are extremely uncommon. They are often misdiagnosed because of their rarity. METHODS AND CASES: The treatment and clinical outcomes of the four cases treated at our institution were compared with the previously published studies. Written informed consent was taken. We highlight four cases of primary diffuse large B-cell lymphoma of cervix treated at our institution with immunochemotherapy and radiotherapy. The mean age was 50 years (range, 39-62 years). Three patients had stage I disease while one had stage II disease. All the patients were in complete remission following treatment with immunochemotherapy and radiation therapy. The average disease free survival was 20 months (range, 8-43 months). None of the patients had any local or systemic relapse. CONCLUSION: These cases highlight the physicians to be aware of this entity as their management, natural history and prognosis is completely different from squamous carcinomas of the cervix. Surgery should not be attempted in these patients. Immunochemotherapy and radiotherapy results in favorable clinical outcomes.

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.

Does Radiotherapy after Surgery Affect Outcomes in Ewing's Sarcoma of the Pelvis?

BACKGROUND: The impact of postoperative radiotherapy (PORT) on outcomes has been a matter of debate after adequate resection in Ewing's sarcoma of the pelvis. We evaluated our cases after surgical excision in pelvic Ewing's sarcoma and assessed local control and overall survival (OS) with respect to PORT and chemotherapy-induced percentage necrosis. MATERIALS AND METHODS: Forty four surgically operated patients (June 2002-November 2014) of localized Ewing's sarcoma were retrospectively reviewed. There were 31 males and 13 females. Age ranged from 2 to 53 years. All patients received institutional chemotherapy protocol. No patient received preoperative radiotherapy. Specimen was analyzed for margins and chemotherapy-induced percentage necrosis. PORT was offered to patients on case-by-case basis. Presence of a large preoperative soft-tissue component, margin evaluation, and percentage necrosis were factors considered. At time of the last followup, 29 patients were alive, 11 died, and 4 were lost to followup. Survivors had a minimum followup of 2 years (range: 31-118 months, mean = 69 months). RESULTS: One of twenty (5%) patients with PORT had a local recurrence as against 2 of 24 (8%) without PORT. OS of all patients was 76% at 5 years. Twelve patients with <90% necrosis had OS of 56% and 32 with >90% necrosis had OS of 83% (P = 0.040). OS of patients with PORT was 74%, without PORT was 78% (P = 0.629). CONCLUSIONS: The decision to offer PORT after surgical excision in pelvic Ewing's sarcoma is multifactorial; the absence of PORT in selected cases is not detrimental to local control. Poor responders to chemotherapy had poorer survival while PORT did not impact on outcomes.

Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document.

Rhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children. RMS can arise in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph nodal spread, therapeutic response and long term outcome, hence requiring physicians to be familiar with site-specific staging and treatment details. Most common primary sites include the head and neck region, genitourinary tract, and extremities. Prognosis for children and adolescents with RMS has recently improved substantially, especially for patients with local or locally extensive disease because of the development of multi-modal therapy incorporating surgery, dose-intensive combination chemotherapy, and radiation therapy. Despite aggressive approaches the outcome for patients who present with metastatic disease remains unsatisfactory. Clinical trials are ongoing to reduce toxicity and improve outcomes of such patients; newer agents in combination are being investigated.