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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

Radiation and chemotherapy of parameningeal rhabdomyosarcoma involving the orbit.

Eighteen patients with parameningeal rhabdomyosarcoma (RMS) involving the orbit were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and October 1983. Fifteen patients were children with a mean age of 6 years and three patients were adults with a mean age of 21 years. In four patients, the primary tumor originated in the orbit, while the remaining 14 had other parameningeal primary sites. The tumors were in a very progressive local stage, with destruction of facial bones in most patients. Six patients were treated with the T2 chemotherapy protocol and 12 received the T6 protocol. The radiation treatment plan for all patients was designed to deliver between 4500 and 7200 rad delivered to the primary tumor over 4 to 16 weeks. Eleven of the 18 patients (61%) are alive and well with a median follow-up time of six years. Two patients died of therapeutic complications and five died of tumor spread with central nervous system involvement. Ocular complications included acute and chronic conjunctival, corneal, lens, and retinal changes, which were less severe than those reported in previous series.

Cataract↗

Abdominal recurrences in Wilms' tumours: a report from the SIOP Wilms' tumour trials and studies.

The Wilms' tumour trials and studies conducted from 1971 to 1980 registered 1042 patients. Of these, 82 patients developed an abdominal recurrence. Particulars of these were studied. Half of the recurrences occurred in stage III patients. Often several untoward prognostic factors could be identified, such as large tumour size, difficult operation, incomplete excision, peritoneal adhesions or metastases, tumour extending to renal vein or vena cava. A tumour rupture increases the chance for an abdominal recurrence, especially if appropriate radiotherapy is not given. In many of these cases, postoperative radiotherapy seems to have been insufficiently tailored to the operative findings. For stage III cases, a careful discussion between surgeon, radiotherapist, and pathologist should lead to the optimal radiotherapy field size and dose for each individual patient, so that the risk of abdominal recurrence can be reduced.

Abdominal Neoplasms↗

Redefined role of radiation in combined treatment of Ewing's sarcoma.

Eighty-six patients with Ewing's sarcoma were analyzed as to the role of radiation therapy. Fifty-eight patients (P group) had removal of the involved part of the bone after preoperative chemotherapy, and 28 (NP group) had local treatment either at the same time or before chemotherapy. Thirty-six of 58 P-group patients (61%) and 13 of the 28 NP-group patients (48%) are alive. Five of 48 patients in P-group, who had postoperative radiation, had local recurrence, as did 6 of 11 without postoperative radiation, a statistically significant difference (p = 0.001).

Adolescent↗

The incidence of herpes zoster in patients with Hodgkin's disease. An analysis of prognostic factors.

The incidence of zoster in 717 patients with Hodgkin's disease was determined by a retrospective chart review. All patients had been treated and followed in one of six cancer centers. Prognostic factors that might predict the subsequent incidence of zoster were examined by univariate and multivariate analytic techniques. Intensity of treatment was a key factor in the incidence of zoster. Thirty-six months after initiation of treatment, patients receiving chemotherapy-radiation-chemotherapy had twice the attack rate (27.3%) of those receiving radiation alone (11.5%). The pediatric age group had a significantly higher attack rate (26.6%) than did adults (18.7%). Stage, histology, and laparotomy did not influence the incidence of zoster.

Adolescent↗

Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation.

Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.

Adolescent↗

Therapy of CNS leukemia with intraventricular chemotherapy and low-dose neuraxis radiotherapy.

Successful treatment of CNS leukemic relapse has been frustrated by frequent local recurrence and eventual marrow relapse. We describe the treatment of meningeal leukemia in 39 children with intrathecal remission induction followed by the placement of an Ommaya reservoir to facilitate the administration and distribution of chemotherapeutic agents into the CSF. Six hundred or 900 rad of craniospinal radiation and maintenance intraventricular and intrathecal chemotherapy was then administered. Systemic reinduction therapy was added in the later cases. Sixteen children (41%) experienced no further events, with 17+ months to 13+ years (median, 25 months) follow-up . Eleven patients (28%) had CNS recurrence, nine (23%) bone marrow (BM) relapse, and two (5%) testicular relapse as the next adverse event. The course of patients with first isolated CNS relapse differed from that of the others. Eleven (69%) of 16 patients treated for first isolated CNS relapse are alive and 9 are event free, while only 35% of patients whose CNS relapse occurred simultaneously or after recurrent disease at other sites are alive (P = .04). Seven of 23 in the later group are event free. The difference is due to the increased incidence of BM relapse in the later group (30% v 6%; P = .04). For patients with first isolated CNS relapse, the life-table median CNS remission duration is 42 months. The projected CNS relapse-free survival and event-free survival 8 to 10 years after CNS relapse are 40% and 32%, respectively. Headache, nausea, and emesis of short duration were frequent during therapy. In three patients, the reservoir had to be removed for infection. No patient suffered neurologic deficit related to the reservoir. The therapy described can reduce the CNS relapse rate with manageable toxicity. Systemic relapse is still a major problem after multiple CNS relapse and in those in whom the CNS relapse follows or is simultaneous with relapse at other sites.

Adolescent↗

Radiation for medulloblastoma adjusted to prevent recurrence to the cribriform plate region.

In a recent survey of the recurrence pattern in 40 medulloblastoma patients treated at Memorial Hospital between 1970 and 1979, 15% of all recurrences were in the region of the cribriform plate, which is an area that is undertreated with commonly employed radiation therapy techniques. The authors, therefore, modified their technique to increase the dose in this area. They report on the initial results in 15 subsequent patients treated with this modification. Ten of the 15 patients had localized tumors (Groups I and II) and 8 of these patients are alive disease-free compared with 7 of 17 Group I and II patients treated between 1970 and 1979. Three of five patients with disseminated tumors (Groups III and IV) are alive disease-free compared with none in the previous series. Although the period of follow-up is relatively short (median, 32 months), the overall survival of patients with medulloblastoma seems to be improved with additional radiation to the region of the cribriform plate. No side effects attributable to this modification were observed. In the opinion of the authors, this modification should be used routinely in patients with medulloblastoma.

Adolescent↗

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↗