PubMed Health⌕ Search

Biomedical subjects

A Cangir

Publications and source records attributed to A Cangir.

At least 19 recordsLinked to original sources
PubMed2001

Synovial sarcoma in children and adolescents: thirty three years of experience with multimodal therapy.

BACKGROUND: Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. PROCEDURE: We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. RESULTS: With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [CI] 62-89.2%) and 87.7% (95% CI 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (< 5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. CONCLUSIONS: Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers. Med Pediatr Oncol 2001;37:90-96.

Adolescent↗
PubMed1999

Non-metastatic Ewing's sarcoma: twenty years of experience suggests that surgery is a prime factor for successful multimodality therapy.

Eighty-five patients (37 female, 48 male; median age 14 years) with non-metastatic Ewing's sarcoma received definitive treatment at the University of Texas M.D. Anderson Cancer Center between 1969 and 1988. Multidisciplinary therapy was administered as follows: combination chemotherapy (CC) and local radiotherapy (XRT): 65 patients; CC, XRT and surgery, 19 patients; and XRT and surgery, 1 patient. This permitted a 10-20 year follow-up for 75% of our patients. The overall survival at 5 and 10-20 years was 46.1%, and 37.2%, respectively. At 5 years, 80.5% of live patients had control of local disease. The influence of sex, age, ethnicity, primary site, size, lactic dehydrogenase (LDH) level, presence or absence of systemic symptoms, and XRT dose (<60 Gy and </=60 Gy) was analyzed and was not found to be of prognostic significance in survival. The presence of a soft tissue mass at diagnosis was found to be a significant unfavorable prognostic variable. Nine of 11 patient who underwent resection after CC and/or XRT had residual tumor in the surgical specimen. Patients who received surgery as part of the planned treatment of their primary tumor had significantly better local control and disease-free survival than those who did not undergo resection. Complications in long-term survivors are described.

Adolescent↗
PubMed1996

Fractionated high-dose cyclophosphamide for advanced pediatric solid tumors.

PURPOSE: The objective of this study was to determine the tolerance and toxicities of high-dose cyclophosphamide (CPA) at 7 g/m2 given in four fractions over 8 h in children with advanced solid tumors. PATIENTS AND METHODS: Twenty children aged 1 1/2-19 years (median, 12 years) received 24 courses of high-dose CPA at 7 g/m2 for the treatment of advanced malignant solid tumor. CPA was given in four 1-h infusions of 1.75 g/m2 each, with 1 h of rest between each dose. MESNA was used as a uroprotective agent and was continued for 24 h after the final dose of CPA. With only one exception, all patients were discharged at the end of MESNA infusion and received granulocyte colony-stimulating factor, prophylactic ciprofloxacin, and co-trimoxazole. RESULTS: Severe but transient myelosuppression was observed. The median time to neutrophil and platelet recovery was 17 and 19 days, respectively. Fever developed after 13 of the 24 courses, and hospitalization was required. Extramedullary toxicities were mild. No patient showed cardiomyopathy or hemorrhagic cystitis. Forty-six percent of the courses were managed entirely on an outpatient basis. Objective tumor response was seen in five patients. CONCLUSIONS: CPA at 7 g/m2 is well tolerated by children with advanced malignancies and should be considered in earlier phases of antineoplastic therapy.

Adolescent↗
PubMed1995

Malignant solid tumors in neonates: a 40-year review.

To evaluate the outcome of neonatal malignant solid tumors, we reviewed the records of 222 infants under the age of 1 year with malignant disease who were treated at the University of Texas M.D. Anderson Cancer Center over a 40-year period. Forty-five cases of neonatal (< 30 days old at the time of presentation) malignancies were found. Thirty-two infants had solid tumors and form the basis of this report. Diagnoses included soft tissue sarcoma (13), brain tumor (5), neuroblastoma (6), retinoblastoma (3), malignant melanoma (2), hemangiopericytoma (2), and nephroblastoma (1). The mean age at which initial signs and symptoms were noted was 9 days of life. Fifty-nine percent (19) presented within the first week of life, and 47% (15) presented at birth. The mean age at histological diagnosis was 54 days. The head and neck region was the most common site (18), followed by trunk (9), and extremities (5). Thirty-one patients underwent surgical resection of the primary tumor. Thirteen of those neonates received no additional chemotherapy and/or radiation therapy, whereas 18 received some combination of surgery plus perioperative chemotherapy and/or radiation therapy. Overall survival was 78% (25 of 32) with an average follow-up of 8 years (range, 2 months to 29 years). There were no survivors among those patients with distant metastatic disease at the time of diagnosis. Despite delays, prognosis is excellent in the absence of distant metastatic disease, particularly for extracranial tumors.

Brain Neoplasms↗
PubMed1995

Clinical use of molecular information in the management of multiple endocrine neoplasia type 2A.

One hundred and ninety-seven members of 28 kindreds with multiple endocrine neoplasia type 2A (MEN 2A) were screened for RET proto-oncogene exon 10 and 11 mutations. Seventy-one known affected individuals had mutations of codons 609, 618, 620 or 634, whereas 53 unaffected individuals had no abnormalities. Nineteen out of 54 individuals of unknown status, mostly children, had RET mutations. Four of these children had thyroidectomy based on this analysis and were found to have C-cell abnormalities. We identified one false negative mutation analysis because of a codon 691 polymorphism. We conclude that RET mutational analysis is a cost-effective and accurate method for determination of gene carrier status in MEN 2A.

Amino Acid Sequence↗
PubMed1994

Iodine 131 thyroid ablation in female children and adolescents: long-term risk of infertility and birth defects.

BACKGROUND: The use of radioactive iodine, or iodine 131 (131I), for remnant thyroid ablation and the treatment of cervical and distant metastatic disease in patients with thyroid cancer is well accepted. 131I concentrates in the bladder, and irradiation to the ovaries has been theorized to increase the risk of infertility and birth defects in subsequent offspring. METHODS: We conducted a retrospective review of the charts of 154 children and adolescents treated at our institution for thyroid cancer between 1951 and 1991. Review of these charts identified 68 females diagnosed with thyroid cancer, < or = 20 years of age, who received 131I as part of their therapy at our institution. Charts were reviewed and patients recontacted, and initial tumor, date of diagnosis, and 131I administration, including doses, were recorded. Complete pregnancy histories including current health status of the children were also recorded. RESULTS: Twenty-two patients who never attempted pregnancy were excluded from analysis. Eleven patients could not be contacted and were considered lost to follow-up and thus excluded from the study. In the remaining 35 patients, mean age at 131I administration was 18.3 years (range 14.1-20.8), mean follow-up, 16.8 years (range 5.6-39.8), and mean 131I dose, 148.53 mCi (range 77.2-250). Three patients were diagnosed infertile after extensive workup (8.6%). The remaining 32 patients had 69 pregnancies resulting in 60 term and four premature deliveries. There were two elective abortions for nonmedical reasons and three spontaneous abortions. Only two children were conceived within 1 year of 131I therapy. Both were born with birth defects that proved fatal within 8 months. No other children were born with birth defects. One other child born with an estimated gestational age of 27 weeks died due to complications related to his prematurity. No anomalies were noted at autopsy. Of the 61 children alive for follow-up, no major health problems were identified other than asthma in two children. CONCLUSIONS: 131I, used in doses up to 250 mCi, is not associated with any long-term risk of infertility. The risks of infertility or birth defects are not different from those of the general population. Because the two children with birth defects were born to mothers treated either during pregnancy or 6 months before conception, it might be wise to suggest avoiding pregnancy for up to 1 year after 131I treatment.

Abnormalities, Radiation-Induced↗
PubMed1994

Cardiac diastolic function in pediatric patients receiving doxorubicin.

The purpose of the study was to compare systolic and diastolic function in pediatric patients treated with doxorubicin. Left ventricular function was evaluated in 61 children prior to and following chemotherapy. None had clinical evidence of cardiac decompensation prior to treatment. All received relatively low cumulative doses of doxorubicin; the majority received the drug by continuous infusion. Systolic function was estimated using fractional shortening; diastolic function was estimated using A wave velocity, E wave velocity, E to A ratio, and deceleration time. There was a small but significant decline in systolic cardiac function as estimated from changes in fractional shortening that could not be appreciated in any of the measured parameters of diastolic function. A variety of reasons that could be responsible for the absence of significant changes in diastolic function are discussed. For the present, estimations of systolic function are preferred over the studied parameters of diastolic function in the evaluation of cardiac status in pediatric patients receiving doxorubicin containing regimens.

Adolescent↗
PubMed1993

Pulmonary metastases in children and young adults with differentiated thyroid cancer.

BACKGROUND: The prognostic significance and optimal care of children with differentiated thyroid cancer and pulmonary metastases are not well established. METHODS: Of 209 patients younger than 25 years of age who were treated at University of Texas M. D. Anderson Cancer Center between 1960 and 1990 and for whom there was sufficient information, 19 (9%) had pulmonary metastases at presentation. RESULTS: All of these patients had regional lymphadenopathy at the time of diagnosis. All but two had intense, diffuse radioiodine uptake in the lungs; there were two false-negative scans immediately after surgical procedure caused by competing thyroid residual. The chest radiograph (CXR) was normal in 8 of 17 (42%) patients with abnormal radioiodine scans. After therapy with radioiodine (100-499 mCi), CXR appeared normal in 7 of 9 patients with initial abnormal radiographs (within 6-75 months). Radioiodine uptake by the lungs normalized in 3 of 8 patients with initially normal radiographs, and in 3 of 9 patients with initially abnormal radiographs. There have been no deaths in these 19 patients. CONCLUSION: Pulmonary metastases are not uncommon in children and young adults with differentiated thyroid cancer, especially those who have regional lymphadenopathy. The lung metastases almost always concentrate radioiodine diffusely and may be associated with a normal CXR in almost half of the patients. Pulmonary metastases may be overlooked unless near total thyroidectomy is followed by total body radioiodine scan (TBS) in all children and young adults who have regional lymphadenopathy of the neck.

Adenocarcinoma↗
PubMed1993

Diagnosis of eosinophilic granuloma of bone by fine-needle aspiration with concurrent institution of therapy: a cytologic, histologic, clinical, and radiologic study of 27 cases.

Twenty-seven patients with eosinophilic granuloma (EG) of bone seen at our institution between 1979 and 1991 underwent fine-needle aspiration (FNA) with or without concurrent Tru-Cut biopsy. The 16 males and 11 females ranged in age from 2 1/2 to 61 years (median, 10 yr). Twenty-four patients had monostotic lesions. The clinicoradiologic differential diagnosis included osteomyelitis and Ewing's sarcoma (young patients) and primary and metastatic malignancies (older patients). Twenty-four of 28 FNAs (one patient had two FNAs) were diagnostic of EG, and 10 cases were diagnosed by FNA alone. Smears in these cases showed histiocytes, often with grooved or infolded nuclei, and abundant eosinophils. Multinucleated giant cells, foamy histiocytes, neutrophils, lymphocytes, and plasma cells were present in variable numbers. Four FNAs were misdiagnosed: two as osteomyelitis where smears contained abundant neutrophils, sparse eosinophils, and histiocytes misinterpreted as foamy histiocytes, and two as metastatic carcinoma (in adults) where histiocytes in a scant specimen (one case) and skin appendiceal structures without lesional tissue (one case) were misinterpreted. These cases were correctly diagnosed on repeat FNA (one case), Tru-Cut (two cases), or excisional biopsy (one case); however, three cases diagnosed by FNA had nondiagnostic concurrent Tru-Cut biopsies. Treatment consisted of intralesional injection of 125 mg of methylprednisolone (22 cases). Progressive or complete healing of all lesions occurred. FNA is a rapid and useful technique for the immediate diagnosis of EG that allows concurrent institution of therapy.

Adolescent↗
PubMed1993

Pediatric hepatic tumors: clinical trials conducted in the United States.

Hepatoblastomas (HBs) and hepatocellular carcinomas (HCCs) constitute the majority of hepatic tumors in children. Although most children who have HB or HCC present with unresectable disease, chemotherapy may reduce the size of the primary tumor and metastases, thus allowing subsequent successful removal. Clinical trials conducted over the past decade in the United States have demonstrated that chemotherapy regimens consisting of continuous-infusion doxorubicin and cisplatin or high-dose cisplatin alone effectively reduce tumor size. The prognosis of children with HB is better than that of those with HCC. Over half the patients who initially have unresectable HB can be rendered disease free with chemotherapy and subsequent surgery.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

A rational approach to the use of fine-needle aspiration biopsy in the evaluation of primary and recurrent neoplasms in children.

Fine-needle aspiration (FNA) has been recommended as a technique for accurate evaluation and diagnosis of childhood malignancies. Very little data exist regarding the limitations and utility of FNA in children. We reviewed our experience over the past 5 years to assess the clinical use of FNA and its limitations. One hundred seventy-three FNAs were performed in 156 patients who ranged in age from 7 months to 18 years. Sixty-three patients were female and 110 were male. Thirty-seven aspirations were obtained to evaluate new masses in children with previously diagnosed malignancies. FNA allowed diagnosis of the malignancy in 90% of patients with solid tumors, whereas only 9 of 19 patients with lymphomas were diagnosed accurately with FNA. The specificity was 99.7%. Inadequate material was obtained in 13 aspirates. Insufficient material was obtained in 14% of patients who had FNA as their initial diagnostic tool. Four false-negatives were obtained while evaluating children for recurrent disease. There were 16 true-negative biopsies. These data suggest that FNA is an excellent tool for diagnosing both recurrent malignancies as well as previously undiagnosed solid tumors in the pediatric population but is inadequate to diagnose children with suspected undiagnosed lymphomas. Negative studies should be considered nondiagnostic and followed by open biopsies when clinical suspicion of malignancy is high.

Adolescent↗
PubMed1992

A unique case of retinoblastoma masked by retinal detachment diagnosis and management.

A 4-year-old Latin American boy presented with a history of loss of vision of his left eye for 7 months. He was followed by different ophthalmologists and diagnosed with a retinal detachment. Computerized tomograms and magnetic resonance imaging demonstrated a large calcified left eye and markedly enlarged optic nerve. Based on these radiologic findings diagnosis of retinoblastoma was strongly suspected. Initially enucleation was not done because it was thought that cutting through the involved optic nerve would cause dissemination. The patient was treated with chemotherapy which resulted in marked response. Following normalization of optic nerve and after irradiation the eye was enucleated. The diagnosis of retinoblastoma was confirmed. No tumor was found in the optic nerve. The importance of imaging studies in diagnosis of retinoblastoma and the role of chemotherapy in the treatment of primary retinoblastoma are emphasized.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

Marked response to preoperative high-dose cis-platinum in children with unresectable hepatoblastoma.

Chemotherapy is the only effective method of treating unresectable hepatoblastoma. Most protocols require the administration of multiple highly toxic agents. We evaluated the ability of single-agent high-dose cis-platinum (HD-CDDP) to shrink unresectable primary hepatoblastoma to allow resection. Seven children aged 11 to 72 months had unresectable hepatoblastoma based on size and location. Initial alpha-fetoprotein (AFP) levels were between 4,900 and 1,840,000 ng/mL (mean, 555,900 ng/mL). Chest computed tomography (CT) scans obtained before beginning HD-CDDP therapy showed multiple or bilateral lung masses in 3 patients. Chemotherapy for each of the 7 children consisted of only HD-CDDP, 150 mg/m2, at 3-week intervals. HD-CDDP was stopped and prompt resection performed when AFP levels ceased to decline significantly (n = 4; mean nadir, 18,600); the corrected creatinine clearance decreased below 60 mL/min (n = 2); or, in one case, significant hemorrhage occurred within the tumor. Therefore, the number of HD-CDDP doses given preoperatively varied between 1 and 7 (mean, 3). No children required dialysis. Tumor cells in the bone marrow of one child disappeared completely after one dose of HD-CDDP. Follow-up CT scans showed complete resolution of the pulmonary metastases in 2 children, a partial response in the third, and a marked reduction of primary tumors to resectable sizes. Six children underwent tumor excision with adequate margins; parents of the seventh child refused permission for surgery. There were 2 operative deaths, 3 deaths due to local or distant disease, and 2 patients survived (postoperative follow-up, 22 and 14 months).(ABSTRACT TRUNCATED AT 250 WORDS)

Carcinoma, Hepatocellular↗
PubMed1991

Multimodal therapy for the management of localized Ewing's sarcoma of pelvic and sacral bones: a report from the second intergroup study.

A total of 59 eligible patients with localized Ewing's sarcoma of the pelvic and sacral bones were entered into a multimodal Intergroup Ewing's Sarcoma Study (IESS-II) (1978 to 1982) and compared with a historical control series of 68 patients entered into an earlier multimodal Intergroup Ewing's Sarcoma Study (IESS-I) (1973 to 1978). High-dose intermittent multiagent chemotherapy (vincristine, cyclophosphamide, Adriamycin [doxorubicin; Adria Laboratories, Columbus, OH], and dactinomycin) was given to all patients for 6 weeks before and for 70 weeks following local therapy. All patients who had a tumor biopsy or incomplete resection performed received a dose of 55 Gy to the tumor bed. With a median follow-up time of 5.5 years, two of 59 patients (3%) had a local recurrence, five patients (8%) had a local recurrence and metastases, and 17 patients (29%) developed metastases only. There was significant statistical evidence of an advantage in relapse-free survival (RFS) and survival (S) for patients on IESS-II versus IESS-I, P = .006 and P = .002, respectively. At 5 years, the comparison between IESS-II versus IESS-I was 55% versus 23% for RFS and 63% versus 35% for S.

Adolescent↗
PubMed1990

Ewing's sarcoma metastatic at diagnosis. Results and comparisons of two intergroup Ewing's sarcoma studies.

Two Pediatric Intergroup Ewing's Sarcoma studies of patients with metastatic disease (IESS-MD) have used multimodal therapy consisting of intensive combination chemotherapy and radiation therapy (XRT) to areas of gross disease detected at the time of diagnosis. In IESS-MD-I, conducted from 1975 to 1977, 53 eligible patients were entered and received the chemotherapeutic agents vincristine, Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH), cyclophosphamide, and dactinomycin with concomitant XRT (VACA + XRT). In IESS-MD-II, conducted from 1980 to 1983, 69 eligible patients were entered and received 5-fluorouracil (5FU) in addition to the chemotherapeutic agents of IESS-MD-I; initial intensive chemotherapy was given and XRT was delayed until week 10 (VACA + 5FU, delayed XRT). The best response rate (complete and partial remissions combined) was 73% in IESS-MD-I and 70% in IESS-MD-II, so there was no statistical evidence of a difference in response rates (P = 0.62). The length of best response also was similar between studies (P = 0.79), with approximately 30% of the patients on both studies remaining in remission at 3 years. The percentage of patients surviving 5 years or more was 30 on the first study and 28 on the second study (P = 0.49). The major sites of relapse after a response were lung and bone, each occurring with nearly equal frequency. The age of the patient was related to both best response rate and survival: patients 10 years of age or younger had substantially higher response and survival rates than patients 11 years of age or older. The favorable prognosis for younger patients might be explained by a more favorable distribution of primary sites at diagnosis; 39% of patients 10 years of age or younger had rib primary sites, compared with only 16% for patients older than 10 years of age (P = 0.05). The frequency of life-threatening toxicity was substantially higher in IESS-MD-I (30%) than in IESS-MD-II (9%), but the frequency of fatal toxicity was similar (6% to 7%). Fatal complications included Adriamycin-induced cardiomyopathy, Pneumocystis carinii pneumonia, unspecified pneumonitis, and sepsis. The most common toxicity and complications were leukopenia and infections.

Adolescent↗
PubMed1990

Nasopharyngeal carcinoma in the young: a combined M.D. Anderson and Stanford experience.

From 1956 to 1988, 57 children and young adults (age 4-21 years) with a diagnosis of nasopharyngeal carcinoma were treated at The University of Texas M.D. Anderson Cancer Center (42 patients) and Stanford University Medical Center (15 patients). The male to female ratio was 2:1. Forty-three patients had lymphoepithelioma, seven had undifferentiated neoplasms, and seven had squamous cell carcinoma. Two patients had Stage III disease and the remainder had Stage IV disease at the time of presentation. All patients were treated with primary radiotherapy, and 14 patients also had chemotherapy with combinations of the following drugs: dactinomycin, doxorubicin, bleomycin, cisplatin, cyclophosphamide, fluorouracil, methotrexate, and vincristine. Twenty-six patients are alive 6 to 178 months from the first day of treatment (median 93 months). The 5- and 10-year actuarial survival rates are 51% and 36%, respectively, and the corresponding disease specific survival rates were 51% and 51%. There were no recurrences after 42 months. The patterns of failure were as follows: distant metastasis only, 21 patients; locoregional metastasis only, 1; both, 5. Distant metastases most commonly occurred in bones, lungs, liver, and mediastinal lymph nodes. Chronic treatment-related morbidity was encountered in a significant number of long term survivors. Trends in the data not reaching statistical significance suggest a more favorable prognosis for a) females, b) patients less than or equal to 15 years of age, c) lymphoepithelioma or undifferentiated histologies, d) stages T3-4 NO-1 vs T1-2 N2-3 vs T3-4 N2-3, e) primary tumor dose greater than or equal to 65 Gy and f) patients who received chemotherapy.

Adolescent↗
PubMed1990

Lymph node metastasis from papillary-follicular thyroid carcinoma in young patients.

A total of 117 patients under 20 years of age with papillary and/or follicular thyroid cancer presented to the M. D. Anderson Cancer Center between 1949 and 1987. The most common presenting symptom was a cervical mass. Twenty percent of the patients had a history of prior irradiation. Sixty percent initially had palpable lymph nodes, while 26% who had clinically negative examinations had pathologically positive lymph nodes. Recurrence was highest in regional lymph nodes at 24%, with only a 4% recurrence rate at the primary site and a 3% recurrence rate at distant sites. There were no deaths due to the thyroid cancer. To maintain a low rate of recurrence, near-total thyroidectomy with neck dissection followed by iodine 131 treatment should be considered in these young patients.

Adenocarcinoma↗