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Biomedical subjects

B N Rao

Publications and source records attributed to B N Rao.

At least 37 records · Page 2Linked to original sources
PubMed2000

Childhood carcinoid tumors: the St Jude Children's Research Hospital experience.

BACKGROUND/PURPOSE: To better characterize childhood carcinoid tumors, the authors reviewed the clinical presentation, treatment, and outcomes of pediatric patients with these rare tumors. METHODS: A retrospective review was conducted of medical records and pathologic materials of all children with carcinoid tumors treated at St Jude Children's Research Hospital between December 1977 and March 1999. RESULTS: Eight patients (median age, 12.7 years) were identified; 2 were boys, and 7 were white. Primary tumor sites were the appendix (n = 5), small intestine (n = 1), bronchus (n = 1), and 1 unknown site. In 7 cases, carcinoid tumor was not suspected at the time the tumor was identified. Seven patients had localized disease; 5 remain disease-free after complete resection, and 2, whose carcinoid tumors were identified incidentally, died of metastatic mucinous adenocarcinoma of the colon. One patient who presented with symptoms of carcinoid syndrome had metastatic disease that responded poorly to cytotoxic chemotherapy and remains alive with active disease. CONCLUSIONS: Although most pediatric carcinoid tumors arise in the appendix, these tumors also occur in other primary sites. Clinical awareness and early diagnosis are important factors in preventing morbidity and mortality. Outcomes are excellent for patients with localized disease that is completely resected, but those with metastatic disease fare poorly. New therapeutic strategies are needed for these patients.

Adolescent↗
PubMed2000

Clinical profile and multimodality approach in the management of juvenile nasopharyngeal angiofibroma.

Our experience with 19 cases of juvenile nasopharyngeal angiofibroma (JNA) over a period of 10 years is discussed. All cases are managed with combined modalities which include pre-operative hormonal therapy, radiotherapy and/or surgical resection via transpalatal approach. All patients became asymptomatic with these treatment modalities. The use of combined modalities of treatment is advocated so as to achieve decreased intraoperative blood loss and cure, a reality.

Adolescent↗
PubMed1999

Metastases detected at the time of diagnosis of primary pediatric extremity osteosarcoma at diagnosis: imaging features.

BACKGROUND: The authors performed a retrospective study to estimate the incidence rate of metastatic disease at the time of diagnosis of extremity osteosarcoma (OS), to characterize its pattern of presentation, and to identify factors predictive of survival within a cohort of patients with pulmonary metastatic disease at diagnosis. METHODS: From the institutional solid tumor database, the authors identified all patients diagnosed with extremity OS since CT became available at the study institution (1977). The authors recorded patient demographics, the site of primary disease, the histologic subtype of OS, and the presence of metastases at diagnosis. In those patients with pulmonary metastases at diagnosis, the presence of calcifications, the primary tumor volume, the number of pulmonary lobes with disease, and the number of pulmonary nodules were recorded. RESULTS: Of an evaluable population of 215 patients, 32 (15%) had bone or pulmonary metastases at diagnosis, of whom original imaging from 28 patients was available for review. Osteoblastic histology correlated with lung metastases at diagnosis (P = 0.049). One of the 32 patients had a solitary bone metastasis without lung metastases. Four of 28 patients (14%) with original imaging available had calcifications within the pulmonary nodules. Both the number of nodules and the number of lobes involved were found to be significant predictors of survival (P = 0.0009 and P = 0. 04, respectively); multiple nodules were bilateral in 61% of patients. CONCLUSIONS: The rate of incidence of computed tomography detected pulmonary metastases was found to be 14% (31 of 215 patients) at diagnosis and 0.5% (1 of 215 patients) for bone metastases in patients with primary extremity OS. Pulmonary metastases usually are multiple and bilateral and infrequently calcify. The number of nodules and lobes involved are predictors of patient survival.

Adolescent↗
PubMed1999

Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: the St. Jude Children's Research Hospital experience.

BACKGROUND: Because the natural history of pediatric patients with metastatic nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) had not been well described, we retrospectively reviewed our single-institution experience with these tumors. PROCEDURE: We identified 26 patients with metastatic NRSTS who were treated at St. Jude Children's Research Hospital from December 1971 through July 1995. We evaluated the characteristics of each patient, including age, sex, primary site, stage, type of therapy received, and outcome. Sites of metastatic disease at diagnosis and relapse were noted. RESULTS: The median age of the 26 study patients at diagnosis was 14. 8 years; 54% of patients were male and 69% were white. The most common histologies were synovial sarcoma, alveolar soft-part sarcoma, and malignant fibrous histiocytoma. Most primary tumors (73% of cases) occurred in the trunk or extremities. Most patients presented with large (>5 cm), high-grade lesions. All 26 patients received chemotherapy, and 8 responded to an ifosfamide- or cyclophosphamide-doxorubicin-based regimen. Radiotherapy was administered to 15 patients, and 13 had a partial or complete resection of the primary tumor. Six patients underwent thoracotomy. The estimated 2-year survival for the cohort was 34.6% +/- 8.9%; the 2-year progression-free survival was 15.4% +/- 6.3%. The lung was the most common site of failure. CONCLUSIONS: Children with metastatic NRSTS have a poor outcome, which is similar to that in adults. More effective systemic chemotherapy is needed to facilitate complete surgical resection of primary and metastatic sites. Aggressive pulmonary metastatectomy can increase disease control.

Adolescent↗
PubMed1999

Soft tissue sarcomas in children.

In childhood, soft tissue sarcomas comprise a complex group of malignancies of varied histologic subtypes, the prognoses of which depend on the histology, age, site, extent of involvement and a variety of other factors. This paper discusses the varieties of tumors classified as soft tissue sarcomas in childhood and the multimodal approach taken to cure these tumors, with particular attention to the details of difficult surgical problems.

Child↗
PubMed1999

Applications of 3D contrast-enhanced MR angiograpy in pediatric oncology.

Contrast-enhanced 3D MR angiography can be used for imaging of children. This technique can improve the delineation of the vasculature that is more anatomically familiar to surgeons. Here we illustrate the usefulness of contrast-enhanced 3D MR angiography in the diagnosis and follow-up of pediatric oncology patients.

Adolescent↗
PubMed1999

The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma.

BACKGROUND/PURPOSE: Because the management of pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) is determined by extrapolation from adult studies, the effect of margin of tumor resection and postoperative radiation therapy (RT) on local tumor recurrence in children has not been assessed. METHODS: Records of NRSTS patients from a single institution were reviewed with regard to demographic data, TNM staging, grade, histological type and site of primary tumor, RT, and local tumor recurrence. The margin of resection was determined by pathological review and did not necessarily reflect operative margins. RESULTS: Eighty-eight clinical group I patients were treated over a 30-year period. The most common histological tumor subtypes were synovial cell sarcoma (n = 26), malignant fibrous histiocytoma (n = 17), and fibrosarcoma (n = 7). The mean age was 9.4 years (range, 0 to 29 years). Thirty-four patients had high-grade tumors. Two of ten patients with low-grade tumors and margins less than 1 cm, including one of five who had received RT, had a local recurrence. Patients with low-grade tumors and margins greater than 1 cm (n = 44) had a lower recurrence rate (2 of 44, 4.5%). None of these patients had received RT. Fourteen patients with high-grade tumors had margins less than 1 cm. Seven of these had RT and had no recurrence. Three of the seven patients who received no RT had a recurrence (42.9%). None of the 20 patients with high-grade tumors and margins greater than 1 cm received RT; four of these patients had recurrences (20%). Seven of the 12 irradiated patients (58.3%) had serious radiation-associated complications (wound dehiscence, fracture, growth retardation, and joint dysfunction). CONCLUSIONS: Grade alone does not determine the rate of local recurrence. In both low- and high-grade tumors, a pathological margin of resection greater than 1 cm reduced local recurrence. Radiotherapy provided no advantage in low grade tumors but did decrease local recurrence rates in high-grade tumors with less than 1 cm pathological margins.

Adolescent↗
PubMed1999

Multifocal osteosarcoma following retinoblastoma.

Three survivors of retinoblastoma, one with hereditary bilateral and two with nonhereditary (spontaneous) unilateral disease, developed multifocal osteosarcoma. For one patient, unilateral retinoblastoma was followed by primitive neuroepithelioma at age 13 years. Multifocal chondroblastic osteosarcoma represented the patient's third malignant neoplasm. The course of multifocal osteosarcoma in these three patients compares to that of multifocal osteosarcoma which presents de novo in other patients without prior retinoblastoma.

Adult↗
PubMed1999

Chemotherapy dose-intensification for pediatric patients with Ewing's family of tumors and desmoplastic small round-cell tumors: a feasibility study at St. Jude Children's Research Hospital.

PURPOSE: To evaluate the feasibility of dose-intensification for patients with Ewing's family of tumors (EFT) and desmoplastic small round-cell tumors. PATIENTS AND METHODS: From February 1992 to June 1996, we treated 53 consecutive patients on our Ewing's protocol. Induction comprised three cycles of ifosfamide/etoposide on days 1 to 3 and cyclophosphamide (CTX)/doxorubicin on day 5, followed by granulocyte colony-stimulating factor. Local control using surgery and/or radiotherapy started at week 9 along with vincristine/dactinomycin. Maintenance included four alternating cycles of ifosfamide/etoposide and doxorubicin/CTX, with randomization to one of two CTX dose levels to determine the feasibility of dose-intensification during maintenance. RESULTS: Patients had a median age of 13.4 years (range, 4.5 to 24.9 years); 34 patients were male and 43 patients were white. Nineteen patients presented with metastatic disease, 29 had tumors greater than 8 cm in diameter, and 26 had primary bone tumors. These patients received 155 induction cycles, 91% of which resulted in grade 4 neutropenia, 68% in febrile neutropenia, and 68% in grade 3 to 4 thrombocytopenia. During maintenance, grade 4 neutropenia and grade 3 to 4 thrombocytopenia occurred in 81% and 85% of cycles, respectively. Thirty-five patients (66%) completed all therapy, only 13 without significant delays; three developed secondary myeloid malignancies. The toxicity and time to therapy completion were similar in both CTX arms. Estimated 3-year survival and event-free survival were 72%+/-8% and 60%+/-9%, respectively. CONCLUSION: Although intensifying therapy seems feasible for 25% of patients on this study, toxicity was considerable. Therefore, the noninvestigational use of dose-intensification in patients with EFT should await assessment of its impact on disease-free survival.

Adolescent↗
PubMed1999

Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children's Research Hospital.

PURPOSE: The rarity and heterogeneity of pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) has precluded meaningful analysis of prognostic factors associated with surgically resected disease. To define a population of patients at high risk of treatment failure who might benefit from adjuvant therapies, we evaluated the relationship between various clinicopathologic factors and clinical outcome of children and adolescents with resected NRSTS over a 27-year period at our institution. PATIENTS AND METHODS: We analyzed the records of 121 consecutive patients with NRSTS who underwent surgical resection between August 1969 and December 1996. Demographic data, tumor characteristics, treatment, and outcomes were recorded. Univariate and multivariate analyses of prognostic factors for survival, event-free survival (EFS), and local and distant recurrence were performed. RESULTS: At a median follow-up of 9.2 years, 5-year survival and EFS rates for the entire cohort were 89% +/- 3% and 77% +/- 4%, respectively. In univariate models, positive surgical margins (P =.004), tumor size > or = 5 cm (P <.001), invasivene (P =.002), high grade (P =.028), and intra-abdominal primary tumor site (P =.055) adversely affected EFS. All of these factors except invasiveness remained prognostic of EFS and survival in multivariate models. Positive surgical margins (P =.003), intra-abdominal primary tumor site (P =.028), and the omission of radiation therapy (P =.043) predicted local recurrence, whereas tumor size > or = 5 cm (P <.001), invasiveness (P <.001), and high grade (P =.004) predicted distant recurrence. CONCLUSION: In this largest single-institution analysis of pediatric patients with surgically resected NRSTS, we identified clinicopathologic features predictive of poor outcome. These variables should be prospectively evaluated as risk-adapted therapies are developed.

Adolescent↗
PubMed1999

Extracortical bridging callus after limb salvage surgery about the knee.

The increasing survival of children treated for osteosarcoma has led to an increase in limb sparing surgery. Little published information is available about the postoperative imaging appearance of this technique. Thus, information gleaned from medical records and imaging reviews was correlated with clinical outcome of 19 consecutive children (median age at diagnosis, 12.3 years) treated for distal femoral osteosarcoma with a cemented rotating hinged knee endoprosthesis with porous coated collar. An extracortical bridging bony callus was identified in 16 patients who were followed up after surgery for a median of 2.8 years (range, 1.4-6 years). Extracortical bridging bony callus formation was circumferential in eight and preferentially posterior in nine, and ranged from 0.5 to 0.7 cm in thickness; 14 patients had lucent lines subjacent to the bridging bone. Thirteen patients had good functional use of the surgically treated leg. Five patients had progressive metastatic disease develop. One patient had local disease recurrence 8 months after surgery. Extracortical bridging bone was not identified in three patients, two of whom had infection develop 7 months and 17 months after surgery; both infections required amputation. Extracortical bridging bone preferentially develops posteriorly, along the compression side of the femur, often is associated with subjacent lucency, and seems to reflect increased prosthetic stability.

Adolescent↗
PubMed1998

Treatment of unresectable or metastatic pediatric soft tissue sarcomas with surgery, irradiation, and chemotherapy: a Pediatric Oncology Group study.

BACKGROUND: The objectives of this study were to compare vincristine/actinomycin D/cyclophosphamide/adriamycin (VACA) with VACA/plus imidazole carboxamide (DTIC) (VACAD) therapy in regards to complete/partial response and event free survival rates in children and adolescents with metastatic non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) or previously chemotherapy-naive recurrent NRSTS or locally persistent gross residual tumor after surgery and radiation therapy. PROCEDURES: Between 1986 and March 1994, 75 patients entered this randomized study comparing VACA and VACAD, given at 3 week intervals. Sixty-one patients were considered eligible and received chemotherapy and radiation therapy to the primary tumor and areas of metastases. Thirty-six patients had regional unresected (Group III) disease, and 25 had metastatic disease (Group IV) at time of accession. Thirty-six patients received VACA (11 were not randomized), and 25 received VACAD. RESULTS: With a median follow-up of greater than 4 years, overall and event-free survival for all eligible patients are 30.6% and 18.4% respectively (S.E: 9.5% and 6.8%). There was insufficient evidence that DTIC offered any advantage to event free survival, but there was evidence for better outcome for patients in Group III disease in comparison to patients with Group IV disease, and for patients with a Grade 1 and 2 histology in comparison to Grade 3 lesions. CONCLUSIONS: Combination chemotherapy with VACA and VACAD were insufficient to prevent recurrent or progressive disease in children and adolescents with high stage NRSTS. The use of vincristine/ifosfamide/doxorubicin with cytokine support is under study.

Adolescent↗
PubMed1998

A pilot study of vincristine, ifosfamide, and doxorubicin in the treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas.

BACKGROUND: Standard therapy for pediatric nonrhabdomyosarcoma soft tissue sarcomas (PNRSTS) consists of surgical resection with or without radiotherapy. The role of chemotherapy in the treatment of these tumors has not yet been defined. We investigated the efficacy and toxicity of an ifosfamide-based regimen in controlling disease in children with high-risk PNRSTS. PATIENTS AND METHODS: Between January 1992 and June 1994 at St. Jude Children's Research Hospital, we treated 11 children and young adults with PNRSTS who were at high risk for treatment failure by using a combined modality regimen that comprised aggressive surgery, radiotherapy, and chemotherapy including vincristine, ifosfamide, and doxorubicin (VID). Nine of these patients had grade 3 disease and one had grade 2 tumor; due to insufficient tissue, the disease grade of the remaining patient could not be established. Metastases were present at diagnosis in 2 children. RESULTS: Therapy was generally well tolerated, with minimal morbidity and no mortality. The most common toxicity was grade 4 neutropenia, which occurred in 51% of evaluable courses. Among 4 patients evaluable for response to chemotherapy alone, 1 child attained a partial response and 3 had stable disease. One child had a response to chemotherapy and concurrent irradiation. At a median follow-up of 30 months, 10 of 11 patients are alive; 8 of 11 patients are alive without evidence of disease. CONCLUSION: Aggressive multimodality therapy for PNRSTS is well tolerated, despite frequent and profound neutropenia. Although adjuvant chemotherapy for this group of cancers remains unproved, the rate of tumor control achieved in this pilot study encourages further investigation in a multi-institutional setting.

Adolescent↗
PubMed1998

A prospective study of bacterial meningitis in children from north-eastern Libya.

During a 14-month period, 77 children (excluding neonates) with bacterial meningitis were studied. The causative organisms were identified in 60 children: Haemophilus influenzae in 26 (34%), Streptococcus pneumoniae in 20 (26%), Gram-negative enteric bacilli in nine (12%) and beta-haemolytic streptococci group A in one. No organism was identified in 17 (22%) cases. The male to female ratio was 1.2:1. The age group most affected was between 2 and 12 months. The case mortality rate was 13% and nine children (12%) were left with permanent neurological sequelae.

Child↗
PubMed1998

Patient satisfaction after limb-sparing surgery and amputation for pediatric malignant bone tumors.

We surveyed 65 patients age 13 years or older who had been treated for malignant bone tumors and were in remission at least 1 year after limb-sparing surgery (LS) or amputation (AMP) to assess general satisfaction with the surgical outcome and its impact on various areas of functioning. Of 130 eligible patients, 65 responded (61 treated for osteosarcoma, 3 for Ewing's sarcoma, and 1 for mesenchymoma). The median current age of the cohort was 25.8 years (range, 14.2 to 47.5 years). The median time from surgery was 14.2 years (range, 4.0 to 30.4 years) for the AMP group, and 5.5 years (range, 2.0 to 13.8 years) for the LS patients. Questionnaire responses of patients treated with AMP compared to those who had LS surgery showed no significant differences in the impact of the surgical procedure on educational and occupational status, functional limitations, pain intensity and degree of pain interference, emotional distress, interpersonal/social interactions and self-image, rehabilitation experience, and overall satisfaction with the surgical procedure. Functional limitation was significantly related to pain interference, as well as emotional distress, self-image, and interpersonal difficulties for the entire cohort. Our findings highlight procedure-related advantages and difficulties that may potentially enhance decision making regarding the selection of the surgical procedure for individual patients with malignant bone tumors of an extremity.

Activities of Daily Living↗
PubMed1998

Levels of serum immunoglobulin G, CSF IgG and IgG index in acute bacterial meningitis.

This study characterised the levels of serum immunoglobulin G (IgG), cerebrospinal fluid IgG (CSF IgG) and IgG index as an aid to the diagnosis and prognosis of acute bacterial meningitis (ABM). A total of 28 patients with proven ABM at admission (age range: one month to 10 years; 17 males, 11 females) (group A) and 17 age- and sex-matched control children (group B) were studied. Levels were also compared between patients with neurological morbidity (n = 4; group C) and without neurological morbidity (n = 24; group D) who were subsets of group A. In addition, patients were divided randomly into two groups based on the treatment received (i.e. ceftriaxone together with dexamethasone [n = 11; group A1] and ceftriaxone only [n = 9; group A2] to assess the effect of dexamethasone. The results (mean +/- SEM) demonstrated intrathecal synthesis of IgG in ABM (group A vs group B: CSF IgG (mg/L): 92.64 +/- 23.54 vs 2.12 +/- 1.08, P < 0.002; IgG index: 0.959 +/- 0.481 vs 0.029 +/- 0.006, P < 0.001) which showed good diagnostic significance. In the patients with permanent neurological morbidity (group C) vs healthy survivors (group D), the CSF IgG and IgG index showed good prognostic significance (group C vs group D: CSF IgG (mg/L): 10.75 +/- 9.75 vs 106.24 +/- 29.37, P < 0.01; IgG index: 0.046 +/- 0.039 vs 1.132 +/- 0.568, P < 0.05). Dexamethasone lowered CSF-IgG and IgG-index levels, but the effect was not statistically significant (group A1 vs group A2: P > 0.1).

Acute Disease↗