Prophylactic therapy with enoxaparin during L-asparaginase treatment in children with acute lymphoblastic leukemia.

Forty-one consecutive children with acute lymphoblastic leukemia (ALL) received prophylaxis therapy with the low molecular weight heparin (LMWH) enoxaparin during L-asparaginase treatment. Enoxaparin was given every 24 h subcutaneously at a median dose of 0.84 mg/kg per day (range, 0.45-1.33 mg/kg per day) starting at the first dose of L-asparaginase until 1 week after the last dose. Molecular analysis for thrombophilic polymorphisms documented prothrombin G20210A mutation in 3/27 (11%), homozygosity for MTHFR C677T mutation in 5/27 (18.5%, and heterozygosity for factor V Leiden mutation in 5/27 (18.5%) children. There were no thrombotic events during 76 courses of L-asparaginase in 41 patients who had received enoxaparin. One patient suffered brain infarct 7 days after enoxaparin was stopped. There were no bleeding episodes. In a historical control group of 50 ALL children who had not received prophylactic enoxaparin during L-asparaginase treatment, two had thromboembolisms (one deep vein thrombosis and one pulmonary embolism). Enoxaparin is safe and seems to be effective in prevention of thromboembolism in ALL patients during L-asparaginase therapy. This study provides pilot data for a future randomized trial of the use of LMWH during ALL therapy for the prevention of asparaginase-associated thrombotic events.

Long-term follow-up of children with retinoblastoma.

Twenty-one pediatric retinoblastoma (RB) patients treated between 1976 and 1994 were evaluated for late treatment-related complications. Median age at diagnosis was 24 months; median age at follow-up was 12 years; median follow-up time was 12 years. Of the 21 patients, 14 had unilateral RB and 7 had bilateral RB. Thirteen patients had received external radiotherapy and 8 children were treated by chemotherapy. Twenty-one patients had undergone enucleation. Radiation-induced cataracts were found in 3 patients, radiation retinopathy in 1, enucleation and postradiotherapy contracted socket in 1, very low visual acuity postradiotherapy in 3, severe hypotelorism in 2, growth hormone deficiency in 2, neurocognitive disorders in 6, and orbital deformation due to radiation bone atrophy was moderate-severe in 12 patients. Azoospermia was found in 1 patient treated by cyclophosphamide and vincristine. The most frequent sequela in this group of RB-cured children were postradiotherapy orbital deformation due to bone atrophy and neurocognitive disabilities. Late radiation effects must be avoided by using modern, innovative, and more sophisticated radiotherapeutic techniques. Late treatment-related complications justify the long-term follow-up of childhood RB survivors.

Internal hemipelvectomy for bone sarcomas in children and young adults: surgical considerations.

AIMS: Pelvic bone sarcomas in children and young adults are rare, and associated with a poor prognosis and a high rate of local recurrence. Primary goals of treatment include prevention of local recurrence and distant metastases. A secondary goal is maintenance of quality of life by avoiding major amputative surgery. This is why internal hemipelvectomy (a limb-sparing surgery) is advocated whenever possible. The focus of our presentation is surgical issues in the context of resection and reconstruction of the pelvis in the first two decades of life. MATERIALS AND METHODS: Between January 1988 and June 1998, 27 patients were treated and operated on (follow-up time 1.5-12 years). There were 17 males and 10 females. Their age ranged between 2 and 22 years. There were 24 patients with Ewing's sarcoma (ES) and three with other bone sarcomas. In 19 patients the tumour involved the entire or part of the iliac bone (in some cases with extension to the sacrum). In five patients the tumour involved the pubis and/or ischium. In three patients the tumour involved the sacrum with some extension to the posterior iliac bone. All patients received neoadjuvant and adjuvant chemotherapy and radiotherapy with different protocols (related to the origin of referral). RESULTS: Twenty-seven patients underwent internal hemipelvectomy. According to Enneking's classification there were: type I-10; type II-one; type III-six; type IV-five (including one localized sacrectomy); type I+IV-five patients. In 15 patients some kind of reconstruction was needed and in 12 no reconstruction was done. Four wound infections occurred that were managed successfully by surgical debridement, antibiotics and local wound care. In one case removal of the 'implant' was needed. No primary or secondary amputations were performed in the series. The rate of local recurrence was 22%. Functional status at the last follow-up visit or before death, according to the AMSTS functional rating system: excellent-six; good-17; fair-three and poor-one. All patients except the one poor result maintained their walking ability during the follow-up time. CONCLUSIONS: Internal hemipelvectomy is achievable in most cases and justified for better quality of life in children, adolescents and young adults with sarcomas. Further efforts are needed to improve the reconstructive options in the pelvis.

Nutritional status of children with solid tumors.

BACKGROUND: The purposes of the study were to evaluate prospectively the nutritional status of children with solid tumors who were receiving chemotherapy, to find the most sensitive parameter of protein energy malnutrition, and to determine whether the stage of disease and aggressiveness of chemotherapy have any influence on nutritional status. METHODS: Fifty patients were followed prospectively from the time of diagnosis throughout chemotherapy. Serum albumin, prealbumin, and weight were measured at the time of diagnosis and before each course of chemotherapy. RESULTS: At diagnosis, only 2.7% of patients had albumin levels < 3.5 g/dL whereas 36% had prealbumin levels below the normal limit. All patients showed a weight increment of 81 g/day (P = 0.0001), an albumin increment of 0.001 U/day (P = 0.0001), and a prealbumin increment of 0.044 U/day (P = 0.0407). The change in prealbumin values was much more prominent (10-fold higher) in children age < 2 years. Changes in albumin values were not statistically significant by stage of disease but the increment of prealbumin did show statistical significance, which was most prominent in patients with Stage IV disease CCG (children's cancer group classification ) (P = 0.0003). The intensity of chemotherapy had no influence on changes in weight or albumin levels. However, it did influence changes in prealbumin levels, which were most pronounced in the group receiving high dose chemotherapy. CONCLUSIONS: Based on the results of the current study, the authors believe prealbumin is the most powerful test overall with which to evaluate the nutritional status of children with solid tumors both at the time of diagnosis and throughout chemotherapy.

Multifocal desmoid tumor in childhood: report of two cases and review of the literature.

We present two cases of multifocal desmoid tumors arising in two teenagers. Different, separate lesions developed in the same limb over a period of several years, one of which recurred on several occasions. The literature regarding the therapeutic aspects of multifocal fibromatosis is reviewed and discussed.

T cell lymphoma of the ear presenting as mastoiditis.

Mastoiditis is a complication of otitis media characterized by suppuration and destruction of air cell septa in the mastoid and petrous pyramid. Diagnosis is made by clinical findings and computerized tomography (CT) of the temporal bone. We present a patient initially diagnosed by CT as having chronic mastoiditis who was subsequently shown to have an unusual large-cell malignant lymphoma of T cell type.

Solitary extranodal anaplastic large cell lymphoma, Ki-1+, of the eyelid.

PURPOSE: To report a rare case of solitary anaplastic large cell lymphoma, Ki-1+, of the eyelid. METHOD: Case report. A firm ulcerated mass of the lower eyelid in a 10-year-old boy was the initial and only sign of anaplastic large cell lymphoma. RESULTS: A local excision of the mass was performed. Histologic examination disclosed large lymphoid anaplastic cells that reacted positively for T-cell markers and CD30 antigen. CONCLUSION: A solitary eyelid mass can be an initial sign of anaplastic large cell lymphoma in children.

Neurologic complications in pediatric solid tumors.

The cases of 61 children, consecutively diagnosed during 1986-1990 as having malignant solid tumors (but excluding those with brain tumors and lymphoproliferative diseases), were reviewed. Neurologic complications occurred in 19 (31%), most often in association with neuroblastomas and sarcomas. Complications observed in order of frequency were: brain metastases in 6 children, spinal cord compression in 5, peripheral or cranial neuropathies in 4, and seizures in the remaining 4. Early recognition of neurologic compromise and rapid initiation of treatment are mandatory in order to prevent permanent disability.

Ataxia telangiectasia and lymphoma: an indication for individualized chemotherapy dosing--report of treatment in a highly inbred Arab family.

Ten of 18 children in a highly inbred Arab kindred suffered from either ataxia telangiectasia (AT) or a variant syndrome consisting of ataxia, microcephaly, and congenital cataract (AMC). Four of the nine afflicted children were treated in our unit when they developed lymphomas (both Hodgkin's and non-Hodgkin's including Burkitt's). They were given chemotherapy (either standard COMP or low-dose ABV/CVPP). The children with non-Hodgkin's lymphomas died of sepsis after receiving full-dose COMP. Low-dose ABV/CVPP brought about a 20-month remission in one child with nodular sclerosing Hodgkin's lymphoma and both AT and AMC, but she developed a preleukemic syndrome and her parents refused further treatment; she too died. A fourth child, also with nodular sclerosing Hodgkin's lymphoma, is currently in complete remission after ABV/CVPP. Treatment of lymphomas in patients with AT is extraordinarily difficult and has potential side effects so grave as to necessitate careful monitoring and individualized protocols.

Fibromatosis (desmoid tumor) following radiation therapy for Hodgkin's disease.

Fibromatosis (desmoid tumor) can sometimes develop at sites of previous surgery or previous trauma, but it is very rarely detected at sites of prior irradiation. We report two cases of fibromatosis following radiotherapy for Hodgkin's disease. The fibromatoses, developing in the radiation fields, were detected at the posterior area of the neck 11 years after irradiation in one case and in the peritoneal cavity 3 years after laparotomy and irradiation in the second case. The tumors did not differ clinically or histologically from those seen in patients who did not undergo irradiation. Although rare, fibromatosis should enter the differential diagnosis of masses developing in irradiated fields in patients with treated Hodgkin's disease.

Ollier's disease associated with ovarian Sertoli-Leydig cell tumor and breast adenoma.

An ovarian Sertoli-Leydig cell tumor in a 15-year-old girl is reported. The patient had enchondromatosis (Ollier's disease), and a review of the literature revealed a few previous reports linking enchondromatosis with ovarian sex-cord stromal tumors. This association between these two unusual lesions indirectly supports a generalized mesodermal dysplasia in patients with enchondromatosis.