A tribute Antonio gentil martins, M.D
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to B Rao.
Explore the source record for details and available documents.
PURPOSE: To prospectively study the value of adjuvant chemotherapy in pediatric patients with surgically resected nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS). PATIENTS AND METHODS: From June 1986 to May 1992, after complete surgical resection (+/-radiotherapy) of their NRSTS, 81 eligible patients either received adjuvant chemotherapy comprising vincristine, dactinomycin, cyclophosphamide, and doxorubicin or were observed. Only 30 patients accepted randomization, and 15 were assigned to each regimen. Of the remaining 51 patients, 19 elected adjuvant chemotherapy and 32 elected observation. RESULTS: Patients were predominantly male, and 69% of all patients were white. The median age at diagnosis was 12.3 years (range, 9.2 to 20.7 years). For the 81 eligible patients, the 5-year overall survival estimate was 84.5% +/- 4.4% and event-free survival was 72.2% +/- 5.4%. Among randomized patients, the 5-year estimated overall survival rate was 93.3% +/- 7%, and the event-free survival rate was 86.7% +/- 9.5% for the observation group, compared with 69.2% +/- 13% and 40.7% +/- 14%, respectively, for those who received chemotherapy. The significantly worse outcome of patients who received adjuvant chemotherapy disappeared when survival was stratified by tumor grade. Among all patients, a grade 3 lesion conferred a significant disadvantage with respect to event-free survival (P =.0001). CONCLUSION: The administration of adjuvant chemotherapy according to the schedule and dosages used in our trial did not improve the outcome of children with resected NRSTS. In this study, tumor grade was the most important predictor of clinical outcome in patients with resected NRSTS, and this factor should be incorporated into the stratification of patients in future trials.
The case of a 2-mm Spitz nevus is reported. We comment on the case and describe the skin-surface microscopy features.
BACKGROUND: When to do a neck dissection as part of the surgical treatment for a patient with squamous carcinoma of the oral tongue is controversial, particularly when the primary can be resected without entering the neck. If the patient who is at high risk for having occult nodal disease in the neck can be identified, node dissection with the glossectomy could be justified. To better identify patients for this procedure, we correlated various tumor and patient factors along with preoperative diagnostic studies with the presence or absence of pathologically positive nodes in a group of patients who underwent node dissection. METHODS: Ninety-one previously untreated patients with biopsy-proved squamous carcinoma of the oral tongue were prospectively studied. All patients had a glossectomy and neck dissection as their initial treatment. The pathology findings (ie, lymph nodes with squamous cancer) were correlated with many preoperative and intraoperative factors, and a statistical analysis was made. RESULTS: The use of computed tomography and ultrasound was not better than the clinical examination in determining the presence or absence of nodal metastases. The best predictors were depth of muscle invasion, double DNA aneuploidy, and histologic differentiation of the tumor. CONCLUSIONS: All patients with stage T2-T4 squamous cancers of the oral tongue should have an elective dissection of the neck. Patients with T1N0 cancer who have a double DNA-aneuploid tumor, depth of muscle invasion > 4 mm, or have a poorly differentiated cancer should definitely undergo elective neck dissection. Ultrasound and computed tomography are of little value in predicting which patients have positive nodes.
OBJECTIVE: To determine whether thrombi or vascular occlusion represent a late complication persisting several years after removal of central venous lines (CVLs) in children and adolescents treated for childhood cancer. METHODS: Children whose treatment for malignancy included placement of a CVL that had been removed at least 2 months previously were studied during scheduled follow-up that included contrast-enhanced computed tomography. Spiral volume acquisition was used to obtain 3-mm images from the chest apices through the right hilum, and three-dimensional reconstruction of angiograms was performed. Thrombosis/occlusion was defined as narrowing, obstruction, or filling defect of the deep venous system, with or without the formation of collateral veins. Charts were reviewed to document patient characteristics, previous CVL complications, administration of hyperalimentation, use of urokinase, and family history of venous thrombosis. RESULTS: Twenty-three patients treated for solid tumors and 2 treated for B-cell acute lymphocytic leukemia or lymphoma were studied. Lines had been in place from 0.2 to 36 months (median, 7.4) and were removed at 2.3 to 121.8 months (median, 32.5) before study. Nine patients received hyperalimentation for periods ranging from 2 to 38 weeks (median, 12). Four patients had required urokinase instillations, and one developed superior vena cava syndrome; 4 had a CVL-related infection (two superficial and two Candida line infections). Occlusion was seen on computed tomography angiograms in 3 of the 25 patients (12%; 95% confidence interval: 4.5-31%). One of the patients with occlusion had superior vena cava syndrome; none had a family history of thrombosis, use of a double lumen CVL, or multiple instillations of urokinase. CONCLUSIONS: Persistent asymptomatic vascular occlusion does occur as a late complication of CVL placement for treatment of childhood malignancies, although the frequency appears low among patients treated primarily for solid tumors. Prospective studies of large numbers of patients with a broader spectrum of diagnoses are necessary to define the incidence of and risk factors for this complication and to assess the need for prevention with anticoagulation or other therapy. Pediatricians caring for patients with a history of cancer and CVLs should be aware that these patients may have persistent vascular occlusion that could predispose them to recurrent thrombosis or postphlebitic syndrome.
BACKGROUND: Recently, the Pigmented Lesion Group at the University of Pennsylvania described a 4-variable model for predicting 10-year survival for patients with primary cutaneous melanoma. The variables are tumor thickness, anatomic site of the lesion, age, and gender. The objective of the current study was to test the validity of this model, employing the large data base of the New York University Melanoma Cooperative Group. METHODS: The predicted probabilities of 10-year survival for 780 patients with primary cutaneous melanoma were determined by multivariate logistic regression, using the 4 variables. RESULTS: The overall 10-year survival rate of the current study group was 78.4%. Of the four variables, tumor thickness, anatomic site of the lesion, and age were found to be independent predictors of survival. Although survival was better for women, gender was not a statistically significant factor in predicting 10-year survival when entered into the multivariate logistic regression model. In the current study, the probability of 10-year survival of patients with melanomas < 0.76 mm ranged from 93-99%, depending on the age and primary site. Age and site had more impact on the prognosis of intermediate and thick melanomas than on thin melanomas. Thus, for melanomas 0.76-1.69 mm, 1.70-3.60 mm, and thicker than 3.60 mm, the probabilities of survival ranged from 70-94%, 39-82%, and 23-68%, respectively. CONCLUSIONS: The wider ranges in survival rates for thicker melanomas, depending on the other variables, emphasize the importance of including variables in addition to tumor thickness in a prognostic model. Using a large data base from a medical center, the current study supports the prognostic multivariate model of the Pigmented Lesions Group of the University of Pennsylvania; however, the authors of the current study did not find gender to be statistically significant in this multivariate model.
BACKGROUND & AIMS: We showed previously that the peroxisome proliferators di(2-ethylhexyl)phthalate (DEHP), clofibrate, and 4-chloro-6-(2,3 xylidino)-2-pyrimidinylthio (N-beta-hydroxyl)acetamide (BR931) alter hepatic sex steroid metabolism and receptor expression during induction of hepatic hyperplasia and hepatocellular carcinoma (HCC) in rats. The aim of this study was to identify metabolic changes associated with cell growth during hyperplasia and HCC. METHODS: Hepatic hyperplasia was induced in male rats by a diet containing DEHP and clofibrate for 3-60 days. HCC was induced by feeding a diet containing BR931, a more potent hepatocarcinogen, for 10 months. RESULTS: Cholesterol biosynthesis was depressed in hyperplastic livers but increased in HCC. Glucose-6-phosphate dehydrogenase (G6PD) activity was inhibited in hyperplastic liver as well as in HCC, whereas malic enzyme activity increased severalfold. Protein and messenger RNA (mRNA) levels for both G6PD and malic enzyme increased in hyperplastic livers and HCC. mRNA levels for 3-hydroxy-3-methylglutaryl-coenzyme A reductase decreased in hyperplasia and increased in HCC, whereas low-density lipoprotein receptor mRNA increased in hyperplasia and decreased in HCC. CONCLUSIONS: Neoplastic cells acquire a growth advantage by their capacity to synthesize cholesterol and obtain reduced nicotinamide adenine dinucleotide phosphate by the malic enzyme pathway when G6PD activity is inhibited by peroxisome proliferators.
Dysembryoplastic neuroepithelial tumour (DNT) of the central nervous system is a recently recognized entity. We describe the clinical and characteristic histomorphological features in a patient with DNT. As this lesion carries a favourable prognosis, it becomes essential to make an accurate histopathological diagnosis.
PURPOSE: The objective of this study was to assess the complications and efficacy of gastrostomy (GT) feedings in pediatric cancer patients. PATIENTS AND METHODS: We reviewed the medical records of 33 pediatric cancer patients who received enteral nutrition via a GT. RESULTS: Median age was 9.4 years (range, 1-19.8 years), and 28 of the 33 patients had solid tumors. Seventeen patients had a significant weight loss (median, 8.5%) and therapy-related weight loss was anticipated in 16 patients. The GT device was placed an average of 5.5 months after diagnosis. Twenty-five patients were fed via a tube and eight via a button device. The tube was placed surgically in 21 cases (including all eight button types) and endoscopically in 12. Nutritional support lasted a median of 9.5 months. One or more complications occurred in 30 patients and were categorized as (a) insertion site reactions (inflammation, 23; infection/colonization, 14; exuberant granulation tissue, 6); (b) mechanical problems (leaking, 3; obstruction, 2; breakage, 1; accidental dislodgement, 2); (c) insertion site bleeding, 8; and (d) feeding intolerance, 12. Only one insertion site cellulitis progressed to a systemic infection. All eight patients with a button GT experienced insertion site complications, with local infection occurring significantly more often in patients with the button than in those with the tube GT. There were no significant associations between insertion technique and type of complication. Twenty-seven patients (82%) achieved or maintained ideal body weight with this intervention. CONCLUSIONS: GT feeding was associated with minor complications, but permitted effective nutritional support for pediatric cancer patients.
Gangliogliomas are one of the uncommon tumours of central nervous system. Since these tumours have been associated with an indolent clinical course and benign biological behaviour, it is essential to make an accurate histopathological diagnosis of ganglioglioma. In this study the salient clinical and histopathological features in six patients with intracranial ganglioglioma are described.
BACKGROUND: The incidence of malignant melanoma (MM) in the United States (US) must be known to accurately evaluate the costs that MM imposes on the health care system and society in general. Furthermore, knowledge of the incidence is needed to determine the benefit of MM prevention programs. OBJECTIVE: To obtain an estimate of the incidence of MM in the US. METHODS: The data for this study were collected by means of a questionnaire that was sent to all members of the American Academy of Dermatology practicing in the US (N = 7412). RESULTS: Based on the mean number of MMs seen annually per dermatologist in each state and the number of dermatologists per state, the number of new in situ and invasive MMs in the US in 1992 was calculated to be 80,000. This translates to an incidence of 32 MMs per 100,000 persons. CONCLUSIONS: Our estimate of 80,000 new MMs diagnosed in 1992 in the US suggests that MM places much greater burdens on the US health care system and society than that based on current published estimates.
BACKGROUND: The combined modalities of surgery, chemotherapy, and radiation therapy have greatly improved the survival rate in childhood paratesticular rhabdomyosarcoma, but the incidence of complications and late side effects is a cause for concern. METHODS: We reviewed the records of 18 patients treated for paratesticular rhabdomyosarcoma at St. Jude Children's Research Hospital between 1962 and 1989. Patients with Group I disease were treated with orchiectomy, retroperitoneal lymph node dissection, and multi-agent chemotherapy; more advanced cases also received radiation therapy with concurrent chemotherapy. RESULTS: Sequelae included esophageal and common bile duct stricture, inguinal nerve entrapment syndrome, and small bowel obstruction. Short stature was found in all children whose spines were irradiated via para-aortic fields (34-37 Gy) prior to puberty. Two of 18 patients died from treatment complications and one from progressive disease. CONCLUSIONS: Multimodality treatment offers an excellent prognosis in paratesticular rhabdomyosarcoma, but is associated with significant morbidity and mortality rates. A discussion of therapy components and their application to disease stages suggests possible approaches to optimizing treatment for this therapy-sensitive malignancy.
Thirty-two cases of neurofibromatosis Type I (NF1) were identified among 6,678 pediatric cancer patients treated at St. Jude Children's Research Hospital over a 29-year period. A total of 35 malignant neoplasms have been diagnosed in these patients. Two of three patients with second malignant neoplasms had colon cancer at the primary or second tumor. Of particular interest are two cases in which both NF1 and malignant peripheral nerve sheath tumors were present in multiple successive generations: a patient with colon cancer and non-Hodgkin lymphoma who has a constitutional abnormality of the p53 gene, and a patient with acute lymphoblastic leukemia with the Philadelphia chromosome and other cytogenetic abnormalities, including the t(8;14). Outcome of patients in the largest subgroup, that of malignant peripheral nerve sheath tumors, was favorable only for those patients having resectable extremity lesions. In contrast, all patients with central nervous system tumors are surviving. These cases reflect the molecular and cytogenetic abnormalities that can be present in NF1 and the variety of tumors that may result in these patients.
BACKGROUND: Acroangiodermatitis is a benign vesicular process encountered on the lower extremities that histologically resembles the superficial form of stasis dermatitis, but is clinically characterized by circumscribed violaceous, brown or dusky macules, papules, or plaques. Furthermore, unlike stasis dermatitis, acroangiodermatitis is usually associated with minimal epidermal changes and eosinophils in the inflammatory infiltrate in the dermis. Kaposi's sarcoma, pigmented purpura, vasculitis, and lichen planus are other conditions that should be considered when making a diagnosis of acroangiodermatitis. METHODS: Ten patients with acroangiodermatitis were referred to us by area physicians. Hematoxylin and eosin, periodic acid-Schiff, Pearl's (an iron stain), and an immunoperoxidase stain for Factor VIII were performed on routinely embedded paraffin sections. RESULTS: All cases showed new vessel proliferation, perivascular inflammation of superficial and mid-dermis, consisting of lymphocytes, histiocytes, eosinophils, occasional plasma cells, extravasation of red blood cells, and hemosiderin pigment deposition. Dermal fibrosis was observed in all cases. None of the patients showed changes of vasculitis or Kaposi's sarcoma. Only one patient displayed significant epidermal changes of spongiosis and mild acanthosis. CONCLUSIONS: Acroangiodermatitis is an uncommon entity with peculiar clinical and histologic features and should be confirmed by histologic tests.
Explore the source record for details and available documents.
BACKGROUND: Brain metastasis has been considered a rare event in osteosarcoma, although with prolonged survival an increasing incidence has been suggested. There have been no prior reports of long-term survivors among patients with this complication. METHODS: The authors describe a child treated for osteosarcoma who is alive and free of disease 8 years after the detection of brain metastases. Of 254 patients with primary osteosarcoma referred to St. Jude Children's Hospital between 1962 and 1989, 13 developed brain metastases, all after relapse or recurrence in another site. Concomitant active lung metastases were present in all of the patients except the one long-term survivor, whose pulmonary disease had responded to treatment with cisplatin and doxorubicin. Log-rank analyses were used to compare survival duration and the frequency of brain metastases among patients treated before and after 1982, when effective multiagent therapy was initiated. RESULTS: Log-rank analyses comparing patients treated before and after 1982 showed that the introduction of effective modern therapy improved survival among patients at risk for brain metastases (i.e., those with recurrent and progressive disease, P = 0.007) but was not associated with a statistically significant increase in the frequency of brain metastases (15.5% versus 4.5%, P = 0.125). CONCLUSIONS: Although the outlook for patients with this complication remains bleak, the resolution of brain metastases after eight courses of ifosfamide in the patient described in this article suggests that enrollment of selected patients in Phase II trials is merited.
BACKGROUND: Malignant rhabdoid tumor (MRT) is an aggressive, invariably lethal tumor that is resistant to multimodal therapy. METHODS: The authors reviewed the clinicopathologic features, treatment, and outcome of 13 children (7 boys and 6 girls) with diagnoses of primary extracranial MRT at St. Jude Children's Research Hospital between 1981 and 1990. RESULTS: The median age at diagnosis was 8 months (range, 10 weeks-18 years). Primary sites included the kidney (seven patients), liver (three patients), soft tissue of scapula, posterior mediastinum, and retroperitoneum. Seven patients had metastatic disease (lungs, six patients; cutaneous hemangioma, one patient). Ten patients had surgical resection of primary tumor (complete, nine patients; incomplete, one patient). Eleven patients had chemotherapy with multiple agents. Three of four chemotherapy responses observed were with regimens containing ifosfamide. Partial responses (PR, > 50% reduction in tumor size) were obtained in one patient who received single-agent ifosfamide during disease relapse (PR lasting 2 months), one patient who received a combination of ifosfamide, carboplatin, and etoposide at diagnosis (PR lasting 5 months), and one patient who was treated with bleomycin, cyclophosphamide, doxorubicin, and vincristine at diagnosis (PR lasting 5 months) and subsequently with ifosfamide in combination with carboplatin and etoposide during disease relapse (PR lasting 4 months). All patients died at a median period of 5 months (range, 0.5-30 months) after diagnosis. CONCLUSIONS: Based on this review, the authors recommend using ifosfamide alone or in combination with carboplatin and etoposide in front-line therapy for malignant rhabdoid tumor.