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Biomedical subjects

B Jereb

Publications and source records attributed to B Jereb.

At least 37 records · Page 2Linked to original sources

Age and prognosis in neuroblastoma. Review of 112 patients younger than 2 years.

The results of 112 children with neuroblastoma treated at the Memorial Sloan-Kettering Cancer Center between 1949 and 1980 were analyzed. Of these children, 58 were 0-11 months old and 54 were 12-23 months old and there was a median follow-up of 111 months. All 10 patients with Stage I are alive, 21/27 with Stages II and III (78%) are alive, 5/67 patients (7%) with Stage IV are alive, and 7/8 patients with Stage IVS are alive. Age of the children is an independent prognostic factor. The survival of infants with Stage IV is significantly better than it is for older children of the same stage. Two of 15 infants in Stages II and III died, both of early complications, whereas 4/12 older children with the same stages died. Minimal individualized treatment is recommended for children 0-11 months old who have localized and Stage IVS neuroblastoma. Children less than 1 year old with localized and Stage IVS neuroblastoma had an extremely good prognosis (90% survival) and were usually cured without intensive chemotherapy. Surgical removal of the primary tumor was sufficient for Stage I, and partial tumor removal followed by conservative radiation or chemotherapy was sufficient in most Stage II and III patients. Gentle, individualized treatment was adequate for Stage IVS. Children less than 1 with Stage IV neuroblastoma had a significantly better prognosis than older children of the same stage, but their prognosis was still poor (18% survival).

Age Factors

Primary hepatic or hepatosplenic non-Hodgkin's lymphoma in children.

Non-Hodgkin's lymphoma confined to the liver is unusual in adults and has not been reported in children. Two children with primary hepatic and one with hepatosplenic NHL are described. All three patients are males, and both tumors examined for cell surface markers were of B-cell origin. Two patients, treated with chemotherapy and radiation, developed disseminated disease and died with 7 months of diagnosis. A third patient treated aggressively with radiotherapy and multiagent chemotherapy (LSA2-L2 protocol) remains in complete continuous remission 22 months after initiation of therapy. Hepatic NHL, even if well localized at diagnosis appears to warrant an aggressive therapeutic approach.

Adolescent

Preradiation chemotherapy for newly diagnosed childhood brain tumors. A modified Phase II trial.

A poor-risk population of children with primary malignant central nervous system (CNS) tumors, other than gliomas, can be identified by their young age, by the presence of disease dissemination at diagnosis, and possibly by subtotal resection of the primary tumor. These children require at least neuraxis radiation therapy and possibly chemotherapy for disease control. Unfortunately, once neuraxis radiation is administered, tolerance of subsequent chemotherapy is limited. The authors have explored a multimodal treatment approach in 14 poor-risk patients initially consisting of a modified Phase II chemotherapy trial followed by neuraxis radiation. The diagnoses were medulloblastoma (5), pineoblastoma (3), cerebral primitive neuroectodermal tumor (3), germinoma (2), and choroid plexus carcinoma (1). Eleven patients had disseminated CNS disease, and two had bone marrow involvement at diagnosis. Nine patients received 2 courses of intravenous cyclophosphamide (80 mg/kg) alone over 8 weeks, and five others received three daily doses of intrathecal Ara-C (50 mg/m2) and oral hydroxyurea (40 mg/kg) with each course of cyclophosphamide. There were four complete responses (two dysgerminomas, one pineoblastoma, and one primitive neuroectodermal tumor), one partial response (medulloblastoma), and three mixed responses (two medulloblastomas, one pineoblastoma) to chemotherapy alone, for a response rate of 57%. Twelve patients subsequently tolerated the planned dose of neuraxis radiation. The median survival of all patients was 11 months, and seven of eight deaths were related to recurrent disease. The hematologic toxicity was appreciable, and one death resulted from gram-negative septicemia. Through the use of this type of Phase II trial, valuable information can be obtained on the response rates to specific chemotherapy agents administered prior to radiation. Although cyclophosphamide alone was an active agent in this context, these treatment regimens did not have an important affect on survival.

Age Factors

Brainstem glioma: clinical manifestations of meningeal gliomatosis.

Brainstem gliomas of children are variably malignant tumors that rarely have been reported to produce subarachnoid dissemination. Nevertheless, during a two-year period, 5 of 15 such patients treated by us developed symptoms of leptomeningeal metastases. The diagnosis of an anaplastic astrocytoma with meningeal gliomatosis was confirmed postmortem in all 5. In 3 children, meningeal symptoms preceded other signs of posterior fossa recurrence. Symptoms of meningeal gliomatosis included local or radiating back pain (5 patients), segmental weakness (3), paresthesia (2), and incontinence (2). Myelography, performed in 4 patients, was the most useful diagnostic technique, disclosing multiple intradural filling defects or a high degree of block in 3 patients. Although the cerebrospinal fluid was abnormal in all 4 examined patients, in only 1 were malignant cells detected. Prolonged survival, which appears to predispose to dissemination of adult malignant gliomas, was not an apparent factor in our patients.

Adult

Prognostic factors in neuroblastomas.

The clinical and histological factors affecting prognosis for 70 patients with neuroblastomas were tested with regression analysis. Generalized tumor disease was found to be of primary importance. Significant predictors for prognosis in patients without distant metastases were operation, with or without other therapy, age less than one year and histology. When all recorded factors were analyzed the explanation value only reached 0.42. This indicates that the variation in prognosis depends upon other factors as well.

Adolescent

Primary non-Hodgkin's lymphoma ('reticulum cell sarcoma') of bone in adults.

Thirty-one adult patients with a primary non-Hodgkin's lymphoma ('reticulum cell sarcoma') in the bone were analyzed. Twenty-one patients had stage I disease, one stage II disease and 9 stage IV disease. The relapse-free 5-year survival rate for these patients was 42 per cent. The authors regard radiation therapy as the treatment of choice in localized tumors. Chemotherapy did not seem to affect the long-term survival in this small series. However, clear responses to multidrug chemotherapy were observed in some patients with recurrent or disseminated disease.

Adolescent

Lymph node invasion and prognosis in nephroblastoma.

Five hundred and twelve children with Wilms' tumor Stages I, II, and III registered to the International Society of Pediatric Oncology (SIOP) nephroblastoma trial were analyzed as to the prognostic value of metastatic lymph node involvement. The lymph nodes of 300 patients were submitted for microscopic examination; malignant invasion was reported in 15%. The disease-free and the actuarial survival rates for patients with metastatic lymph node invasion were significantly worse than for those without metastatic lymph node involvement. Radical lymph node dissection neither improved the cure rate nor decreased the rate of abdominal recurrence in patients with lymph node metastases.

Adolescent

Radiation in multidisciplinary treatment of children with malignant ovarian tumors.

Thirteen girls with malignant ovarian tumors treated at Memorial Hospital from August 1971 through October 1975 with extensive surgery, multi-drug chemotherapy (T-2 protocol) and postoperative radiation were reviewed. The plan of radiation was to deliver 3,000 rads to the whole abdomen and boost the para-aortic and pelvic area with 1,500 rads in five to six weeks. In only two patients was the radiation conducted as planned. In others the treatment was either interrupted or discontinued due to complications. Four of the thirteen patients are dead. The cause of death in two of these patients was distant metastases; the two other patients died without evidence of tumor due to treatment complications. Three of the nine patients who are alive without evidence of disease have late complications. Following radical surgery, lower doses of radiation given concomitantly with T-2 protocol chemotherapy seem to be sufficient for controlling the disease.

Adolescent

Thin needle biopsy of solid tumors in children.

Thin needle biopsy of a suspected tumor will usually yield material amenable to cytologic examination and sufficient for diagnosis. Sixty children with suspected solid malignant tumors were subjected to this procedure. The masses biopsied were in bone, abdomen, mediastinum, tongue, pharynx, soft tissue, and peripheral lymph nodes. The diagnostic yield was 66% on first biopsy and rose to 93% on repeated biopsies. The diagnostic accuracy was 97% in malignant tumors with histologic confirmation. There were no proved false-negative diagnoses, and two (3%) false-positive (for malignancy) diagnoses. All patients were followed up. There were no complications of note.

Adolescent

Ovarian cancer in children and adolescents: a review of 15 cases.

Fifteen patients, admitted between 1961 and 1975, from 8 to 19 years of age, with malignant ovarian tumors were reviewed. The histology was: dysgerminoma in 5, common "epithelial tumor" in 5, immature teratoma in 4, granulosa cell tumor in one. Of the 15 patients, 5 are alive and well, 4 of these had dysgerminoma, 1 had a papillary cystadenocarcinoma. Of 3 patients with dysgerminoma in Stage III, 2 are among the survivors. Dysgerminoma, in contrast with other types of germ cell tumors, may be curable even in advanced stage.

Adenocarcinoma

Acute and late effects on normal tissues following combined chemo- and radiotherapy for childhood rhabdomyosarcoma and Ewing's sarcoma.

Twenty-three patients with rhabdomyosarcoma and 15 patients with Ewing's sarcoma, treated with radiation therapy to the local site and systemic multiagent chemotherapy are described. Acute reactions from combination chemotherapy and radiation therapy were noted in both groups of patients. These reactions often appeared after low doses of irradiation, required unplanned interruptions of treatments, and in some patients, led to discontinuation of radiation therapy. The chronic effects on normal tissues in both groups of patients have been severe in several cases.

Antineoplastic Agents

Preoperative versus postoperative radiotherapy, single versus multiple courses of actinomycin D, in the treatment of Wilms' tumor. Preliminary results of a controlled clinical trial conducted by the International Society of Paediatric Oncology (S.I.O.P.).

The preliminary results of a controlled clinical trial organized by the S.I.O.P. of radiotherapy and chemotherapy in patients with nephroblastoma are presented. Forty-two centers have participated. Between September 1971 and October 1974, 398 patients were registered; 195 were eligible for the trial and were randomized. The remaining 203 patients were excluded from the trial, but were followed in the same way as the patients in the trial. The results were evaluated in terms of recurrence-free survival rate and survival rate. Results in patients who received preoperative and postoperative radiotherapy (group A, 73 patients) were compared with the results in patients who recieved only postoperative radiotherapy (group B, 64 patients). The tumor ruptured at surgery in three patients of group A, and in 20 patients of group B, a difference that is statistically significant. No significant difference in survival and recurrence-free survival between groups A and B is observed at present. Results in patients treated with a single course of actinomycin D (group I, 80 patients) were compared with the results in patients treated with multiple courses (group II, 80 patients). At present, no significant difference is found between the two groups.

Adolescent

Considerations on treatment of nephroblastoma. A study of 36 cases.

Thirty-six patients with nephroblastoma in Stages I, II and III treated at Radiumhemmet between 1966 and 1973, were analysed. The tumors were classified into three different histological types. All 10 patients in Stage III have developed metastases. All patients in Stages I and II (5 out of 26) who developed metastases had poorly differentiated (histological Type III) tumours. The indications for postoperative irradiation and chemotherapy for the different stages and histological types of the tumour are discussed.

Child

Treatment of metastases in nephroblastoma.

A series of 36 children with nephroblastoma treated between 1966 and 1973 was analysed. None of the patients with stages I or II and microscopic type I or II developed metastases; 5 of 7 patients with stages I or II and microscopic type III developed metastases. All patients in stage III developed metastases. Fifteen patients developed metastases after the termination of the primary treatment. Of 9 patients with pulmonary metastases only, 6 are free of disease, all patients with metastases in other sites are dead.

Child

Anaplastic giant-cell carcinoma of the thyroid. A study of treatment and prognosis.

In 79 cases of histologically verified anaplastic giant-cell carcinoma, symptoms, treatment, and prognosis were documented. Seventy-eight patients are dead, the mean survival time being 2.5 months. One patient is cured. It was concluded that surgery and/or radiotherapy alone are not sufficient. The results from an additional 8 patients also treated with methotrexate indicate a positive therapeutic effect, the mean survival time being 9.4 months. Disappearance of recurrent tumor or pulmonary metastases was noted in 2 patients.

Adult