Long-term results of Taiwan Pediatric Oncology Group studies 1997 and 2002 for childhood acute lymphoblastic leukemia.

The long-term outcome of 1390 children with acute lymphoblastic leukemia (ALL), treated in two successive clinical trials (Taiwan Pediatric Oncology Group (TPOG)-ALL-97 and TPOG-ALL-2002) between 1997 and 2007, is reported. The event-free survival improved significantly (P=0.0004) over this period, 69.3+/-1.9% in 1997-2001 to 77.4+/-1.7% in 2002-2007. A randomized trial in TPOG-97 testing L-asparaginase versus epidoxorubicin in combination with vincristine and prednisolone for remission induction in standard-risk (SR; low-risk) patients yielded similar outcomes. Another randomized trial, in TPOG-2002, showed that for SR patients, two reinduction courses did not improve long-term outcome over one course. Decreasing use of prophylactic cranial irradiation in the period 1997-2008 was not associated with increased rates of CNS relapse, prompting complete omission of prophylactic cranial irradiation from TPOG protocols, beginning in 2009. Decreased use of etoposide and cranial irradiation likely contributed to the low incidence of second cancers. High-risk B-lineage ALL, T-cell, CD10 negativity, t(9;22), infant, and higher leukocyte count were consistently adverse factors, whereas hyperdiploidy >50 was a consistently favorable factor. Higher leukocyte count and t(9;22) retained prognostic significance in both TPOG-97 and TPOG-2002 by multivariate analysis. Although long-term outcome in TPOG clinical trials is comparable with results being reported worldwide, the persistent strength of certain prognostic variables and the lower frequencies of favorable outcome predictors, such as ETV6-RUNX1 and hyperdiploidy >50, in Taiwanese children warrant renewed effort to cure a higher proportion of patients while preserving their quality of life.

Nail changes associated with chemotherapy in children.

BACKGROUND: Large series of chemotherapy-induced nail changes in children have rarely been reported in the literature. OBJECTIVE: To study the pattern and onset of nail changes in cancer children receiving various chemotherapy regimens. METHODS: A cross-sectional study was performed on 30 paediatric patients (aged 1-17, mean 8.3 years), including 11 with acute lymphoblastic leukaemia, five with acute myeloid leukaemia, and others. RESULTS: Nail changes developed in 10 children during chemotherapy, five of whom had Muehrcke's lines, three Beau's lines, one Mees' lines and another had trachyonychia. There appeared to be no correlation between the pattern of nail alteration and the underlying cancer types or stages, or the regimens of chemotherapy. CONCLUSION: One third of the children with cancers developed nail changes associated with chemotherapy. Among them, Muehrcke's lines were the most common manifestation, which were unrelated to hypoalbuminaemia in our series.

Primary spinal epidural non-Hodgkin's lymphoma in a child.

Non-Hodgkin's lymphoma usually involves the central nervous system by metastatic disease. Primary spinal epidural non-Hodgkin's lymphoma (PSENL) is a relatively rare cause of spinal cord compression. It mainly occurs in adults past the 4th or 5th decades. This entity is even rarer in children. The proper treatment modalities are controversial in adults with PSENL. Radiotherapy is the main strategy after surgery; the role of chemotherapy is uncertain. Therapeutic experience in childhood PSENL is extremely limited. We report a 10-year-old boy presenting with backache and bilateral lower leg weakness after minor trauma. Small non-cleaved cell non-Hodgkin's lymphoma of the epidural space was proven after subtotal tumor removal. Other investigations including computed tomography of the chest and abdomen, bone scan, gallium scan, bone marrow aspiration, and cerebrospinal fluid study were all negative for occult disease. The patient received combined therapy with irradiation and chemotherapy after surgery. Esophageal stricture resulting from radiotherapy developed during treatment and colon interposition was performed. He has remained disease free 42 months after the diagnosis with normal functional status.

Congenital rhabdomyosarcoma--a case report.

A term female newborn was noted to have a tumor mass in the oral cavity soon after birth. Oral computer tomography revealed a well-enhanced soft tissue mass about 4 x 4 x 3 cm in size in the left buccal area. Group III embryonal type congenital rhabdomyosarcoma was diagnosed after biopsy (gross removal was not feasible). Respiratory distress exacerbated due to rapid tumor growth compressing airway with the result that endotracheal tube had to be intubated. Chemotherapy was done and complicated by two episodes of neutropenic fever and sepsis. Radiotherapy was suggested but refused by the family. Tumor size was slightly reduced and endotracheal tube could be removed four months later. She was taken home under regular chemotherapy. Radiotherapy, was, however, clearly indicated.

Diabetes insipidus in Langerhans' cell histiocytosis: report of a case.

The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in Taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. Skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. Brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.

Thrombocytosis in children at one medical center of southern Taiwan.

Thrombocytosis in children is common, but usually without symptoms. The causes of thrombocytosis in children are considered to be mostly due to infection, trauma, surgery, blood disease, prematurity, renal disease and chronic inflammation. To evaluate the incidence and etiology of thrombocytosis of the hospitalized patients, patients who were admitted to the Pediatric Department of Kaohsiung Medical College Hospital (KMCH) from October 1996 to November 1997 were studied. There were 2910 cases studied and 220 cases (127 male and 93 female) had thrombocytosis (> or = 500 x 10(9)/L) with a rate of 7.6%. The causes of thrombocytosis are infections (49.5%), Kawasaki disease (6.4%), postsplenectomy (7.8%), blood diseases (3.7%), malignancies (3.2%), renal disorders (3.2%), prematurity (3.2%), tissue damage (4.5%), chronic inflammation (1.8%), recovery from marrow suppression (1.3%), immunologic disturbances (2.2%), essential thrombocythemia (0.5%), and miscellaneous factors (3.7%). Thrombocytosis associated with multiple, simultaneous causative factors was found in 9.0% of these cases. Thrombocytosis secondary to infectious diseases or Kawasaki disease was significantly more common in children under 2 years old. The most commonly associated infectious disease was respiratory tract infection (61.1%). There were 29 children (13.2%) presenting a platelet count of more than 800,000/mm3. However, no thrombotic complications were seen in any of the children. By far, the major cause of thrombocytosis in our cases was reactive in character. Most of the thrombocytosis cases were due to infections, inflammatory diseases, or Kawasaki disease.