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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

Involved field (IF) irradiation with or without chemotherapy in the management of children with Hodgkin's disease.

The present policy at Memorial Sloan Kettering Cancer Center (MSKCC) of treating children with Hodgkin's disease [HD] is as follows: involved field (IF) irradiation only (3,600 rad) for Stages IA and IIA; IF irradiation (2,400 or 2,000 rad) combined with multidrug chemotherapy (MDP) protocol for all other stages. A somewhat higher recurrence rate is accepted for Stages IA and IIA in view of the good salvage rate for these recurrences and in view of side effects of more aggressive types of radiation treatment. One hundred forty-two patients with HD, 2-19 years of age, were treated at MSKCC between 1970 and 1981; 98 of these were treated according to the present policy (SP group), and 44 (NP group) were treated differently. All SP patients underwent staging laparotomy. The follow-up time was 12 to 146 months with a median of 65 months; two patients were lost to follow-up. For the SP group, all stages, 10-year disease-free survival is 77%, and 10-year survival is 93%. By comparison, in the NP group 10-year disease-free survival is 64%, and 10-year survival is 80%. The disease-free survival of SP patients in Stages IA and IIA treated with IF radiation alone is 72%, and survival is 95%. The disease-free survival of SP patients in advanced stages treated with combined radiation and chemotherapy is 87%; the salvage rate of recurrent disease in these stages is poor. The survival was apparently better (P = 0.07) in the SP group as compared to the NP group. All 6 patients of the SP group who died had a nodular sclerosing type of HD. None of the patients in the SP group have developed secondary malignancies, and no severe bone growth retardations or late effects to other organs were observed. In our opinion, IF irradiation alone might at present be suitable treatment for children in Stages IA and IIA of Hodgkin's disease, and addition of IF radiation with low doses of MPD improves the survival of patients in advanced stages.

Adolescent

Radiation therapy of conjunctival and orbital lymphoid tumors.

Lymphoid tumors of the conjunctiva and orbit are rare and remain localized in the majority of cases. Sometimes it is not possible either clinically or histologically to differentiate between a non-Hodgkin's lymphoma (NHL) and benign lymphoid hyperplasia. A series of 24 patients is reported. Nineteen were classified as having malignant NHL and 5 benign hyperplasia; 1 of these 5 later developed metastases, however. All patients had systemic work-up: 18 had Stage I, 1 had Stage II, and 5 had Stage IV disease. All patients received local radiation therapy with doses of 2400 to 2750 rad in 2-3 weeks for lesions of the eyelid and conjunctiva, and between 3000 and 3750 rad in 3-4 weeks for retrobulbar lesions. The lens was shielded in all patients except in 2 who had NHL of the vitreous body. A method of shielding the lens with a lead block mounted on a "low vac lens" is described, and the dose distribution within the eye and orbit is presented. The dose to the ocular lens is reduced to about 10% of the tumor dose with this technique. Patients who were treated with doses higher than 3000 rad experienced conjunctivitis and skin erythema that resolved completely. No other effects of radiation on normal structures of the ocular adnexa were observed in the 20 patients who are alive and without signs of tumor 10-46 months with a median follow-up time of 22 months.

Adolescent

Treatment of genitourinary rhabdomyosarcoma in children.

We report on 27 children with rhabdomyosarcoma of the bladder, prostate, uterus, cervix and vagina treated with combined therapy of surgery, radiotherapy and multiple drug chemotherapy. Of the 27 patients 18 are alive for 13 to 147 months (median 86 months) and 9 have retained a functional bladder. Extirpative surgery followed by adjuvant chemotherapy and occasionally radiation therapy was most effective for local tumor control and 7 of 10 patients survived, including 5 with an intact functional bladder. Primary use of chemotherapy in 13 patients with extensive disease resulted in complete tumor control in only 3 but 7 survived, including 4 with an intact bladder. Radiation therapy used concomitantly with chemotherapy failed to control the local tumor but with subsequent extirpative surgery all 4 patients survived.

Adolescent

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

Retreatment of retinoblastoma with external beam irradiation.

A retrospective review of cases on file at the Ophthalmic Oncology Center of New York Hospital-Cornell Medical Center, New York, was undertaken to examine the effectiveness of a second course of radiotherapy on retinoblastoma. One hundred four patients were found to have been treated with at least two courses of external beam irradiation to one eye. All but one of the cases were bilateral, the other eye with more advanced disease having been previously enucleated. At the time the decision was made to repeat radiotherapy, 15 eyes could be classified group I, II, or III. Twelve (80%) of these eyes survived. Of the 89 eyes that showed more advanced growth (group IV or V), only two (2.2%) survived. In all, 14 of the 104 eyes wer saved--nine with useful (macula) vision, five without. Second nonocular tumors developed in 18 patients (22.8% of those who survived retinoblastoma itself). There appears to be no increased risk of second tumors from the second course of irradiation.

Child, Preschool

Supratentorial recurrences in medulloblastoma.

Four children with medulloblastoma had massive supratentorial recurrences in the region of the cribriform plate after adequate craniospinal irradiation. The pathogenesis of these recurrences is probably related to underdosage to this region by shielding of the eyes. This hypothesis was corroborated by autopsy findings in two other patients in whom subfrontal implants were histologically different from recurrences elsewhere. Two possible solutions to avoid this problem in the future are suggested.

Brain Neoplasms

Nasopharyngeal carcinoma--a retrospective review of patients less than thirty years of age: a report of Children's Cancer Study Group.

Data from 119 patients with nasopharyngeal carcinoma who were under 30 years of age were retrospectively analyzed by the investigators of the Children's Cancer Study Group. The overall five-year survival and relapse-free survival rates from diagnosis were 51 and 36%, respectively. When the tumor was confined to the nasopharynx (1 + T2, 41 patients), the five-year survival was 75%. No significant correlation was found between survival or local recurrence and the radiation dose to the primary site. The five-year survival from relapse was 37% for 21 reirradiated patients with local and/or regional relapse compared with 11% for 45 patients with other forms of relapse. It is concluded that the optimal radiation dose for tumor eradication in young patients is now known.

Adolescent

The role of local radiation in the treatment of non-Hodgkin lymphoma in children.

Eighty-one patients between 1 and 15 years of age with non-Hodgkin lymphoma were seen at Memorial Sloan-Kettering Cancer Center (MSKCC) in an 8-year period ending December 1973. There was no statistically significant difference among the survival distributions for site or histologic type. The patients also were divided into three groups according to the chemotherapeutic regimen employed in their care, and further subdivided as to whether they received "curative" radiotherapeutic attempts. Children treated with multiple agent chemotherapeutic regimen (LSA-2) did significantly better than children of the LSA-1 or nonprotocol (NP) group. Children with Stage I and Stage II disease did significantly better than children with Stage III and Stage IV disease. Radiation therapy as employed in this study prevented recurrence of local disease. All patients in whom the disease recurred died within a year from the appearance of recurrence. However, no statistically significant association between radiation and survival could be shown in this series.

Abdominal Neoplasms