[Non-conventional therapies in childhood cancer: guidelines for distinguishing non-harmful from harmful therapies: a report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology]. / Unkonventionelle Therapien bei Krebserkrankungen im Kindesalter: Richtlinien zur Abgrenzung unbedenklicher von schädlichen Behandlungsmethoden: Ein Bericht der SIOP-Arbeitsgruppe für Psychosoziale Fragen in der Pädiatrischen Onkologie.

This is the 11th official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, instituted in 1991. There is a tendency for some physicians to make blanket statements against the use of non-proven, nonconventional therapies, even when these therapies are not harmful. There is an equal and opposite tendency on the part of many parents to do all that they possibly can for their children, including using any non-conventional therapy they feel might do some good. The health care team must open a healthy dialogue with parents that will lead to a clear distinction between those complementary therapies that are harmful and those that are not, indeed, might even be helpful psychologically if not therapeutically.

Central nervous system involvement in children with sarcoma.

OBJECTIVES: To summarize and analyze the experience in CNS involvement (CNSI) in children with sarcomas treated in the above-mentioned institutions. PATIENTS AND METHODS: From 1990 to 2001, all medical charts were retrospectively reviewed: 19 sarcoma patients (12 boys and 7 girls) were diagnosed with CNSI (4 osteogenic sarcomas, 11 Ewing sarcomas, 2 rhabdomyosarcomas, 1 alveolar soft part sarcoma and 1 mesenchymal chondrosarcoma). Mean age of all patients at the time of initial diagnosis was 14.9 years (range: 4-24 years), mean age at the time when CNSI was diagnosed was 16.9 years (range: 5.5-27 years). RESULTS: The frequency of CNSI among our patients was 6.17%. The following symptoms and signs (sometimes combined) presented: headache (10 patients), nausea and vomiting (6 patients), seizures (11 patients) and focal neurological signs (9 patients). The mean duration of time elapsed since diagnosis of CNSI till death or last follow-up was 5.2 months (SD: +/-5.7 months). Four patients received chemotherapy (CT) alone, 8 CT and radiotherapy (RT), 2 RT alone, 3 supportive treatment only, 1 CT and surgery and 1 surgery alone. Sixteen patients died; there was no significant difference in the duration of survival between those who were treated with RT or surgery (mean +/- SD: 6.77 +/- 6.56 months) and those who received only CT or supportive treatment (mean +/- SD: 2.60 +/- 2.94 months) (p = 0.07). Brain disease was the main cause of death in all but 1 patient who died 4 days after autologous bone marrow transplantation from uncontrolled sepsis. In 16 patients, CNSI was part of a metastatic disease. CONCLUSIONS: Among children with sarcoma, CNSI is encountered in 6.17% of cases. More effective therapy has to be developed in order to improve their outcome.

Unusual presentation of mastoid eosinophilic granuloma in a young patient.

Eosinophilic granuloma is a well-recognized form of Langerhans cell histiocytosis, most commonly involving the skull bones, usually with an excellent prognosis. Recurrent and difficult to recognize osteolytic lesions of the skull are encountered only rarely. A patient with recurrent eosinophilic granuloma of the skull is reported. In spite of appropriate multimodality treatment, there were several recurrences, most recently with involvement of the mastoid process. Imaging studies revealed extensive involvement of surrounding structures with expansion of the tumor into the middle cranial fossa and slight pressure on the antero-medial portion of the temporal lobe of the brain. Despite extensive involvement, the patient had no complaints. Because of the rarity of such silent and unpredictable lesions, a systematic approach with regular CT and MRI follow-up is suggested.

Detection of isolated distant metastasis in soft tissue sarcoma by fluorodeoxyglucose positron emission tomography: case report.

Fluorodeoxyglucose (FDG), labeled with F-18, is a glucose analog that accumulates in cells in proportion to the rate of glucose metabolism, and increased carbohydrate metabolism has been recognized as a feature of malignant cells versus normal cells. In addition, it permits the detection of metastases not discovered by bone scan. Although detection of the primary site of disease is usually accomplished well with conventional techniques, the performance of FDG positron emission tomography (PET) may be useful to determine metastases that are not clinically evident. The authors describe a case of early detection of distant metastases by FDG-PET in a young patient diagnosed with rhabdomyosarcoma of the hand.

Late recurrence of combined hepatocellular carcinoma and hepatoblastoma in a child: case report and review of the literature.

We report on a case of late relapse of hepatocellular carcinoma in a child suffering from combined hepatoblastoma and hepatocellular carcinoma, stage IV. This is a rare event, as it has been accepted that a 5-year period free of any signs of disease in children suffering from malignant hepatic tumors is sufficient to classify such patients as survivors. In our patient, recurrence of the hepatocellular carcinoma component was diagnosed more than five years after the initial diagnosis. This case illustrates the need for more prolonged follow-ups for such children.

Large cell lymphoma of bone presented by limp.

We present a rare case of anaplastic large cell lymphoma of the bone in the leg of a child. The patient initially presented with suspected osteomyelitis of the fibula and was treated by antibiotics without apparent success. Thereafter, an open biopsy of the lesion was performed and the correct diagnosis was established. This rare case demonstrates the difficulties that a treating physician meets in establishing the correct diagnosis in a child presenting with limping. A review of the pertinent literature is introduced.

Guidelines for the recognition, prevention, and remediation of burnout in health care professionals participating in the care of children with cancer: report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology.

This is the eighth official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, instituted in 1991. It deals with a topic discussed and approved by the SIOP Committee; namely, "Recognition, prevention, and remediation of burnout in health care professionals participating in the care of children with cancer." It is addressed to the Pediatric Oncology community and outlines: 1) the general definition of burnout as mental and physical exhaustion, indifference, sense of failure as a professional, and sense of failure as a person; 2) the causes of burnout from the nature of the work itself, the work environment, and the characteristics of the individual; 3) the prevention of burnout, changing the detrimental aspects of one's work environment and modifying one's own behavior; and accepting methods to remediate burnout when it occurs.

Comparing thyroid ultrasonography to thyroid function in long-term survivors of childhood lymphoma.

BACKGROUND: We studied the value of thyroid ultrasonography as a follow-up tool in survivors of childhood lymphomas and correlated morphologic abnormalities with thyroid function tests. PROCEDURE: A prospective follow-up study of 45 long-term survivors of histology-proved childhood lymphomas was performed. Mean age at diagnosis was 9.1 years (range 2.1-16.4 years) and mean follow-up duration 10.9 years (range 3.9-22.2 years). RESULTS: Among the 26 survivors of Hodgkin disease (HD) who received mantle field irradiation, 14 (54%) had abnormal ultrasonograms. Elevated thyroid-stimulating hormone (TSH) concentrations were found in 14 (54%), and 6 of them (42%) had normal thyroid functions. Six of twelve patients with normal ultrasonograms had abnormal thyroid function, and 5 of 11 patients with normal function had abnormal sonograms. Among the 19 non-HD survivors who did not receive radiotherapy, 18 (95%) had both normal sonograms and normal function. Thus thyroid gland abnormalities were detected in 54% of HD survivors after mantle field irradiation. No correlation between the abnormalities detected on ultrasonography and serum levels of TSH and thyroid hormones were found. CONCLUSIONS: Both ultrasound and thyroid function tests independently provide clinically useful information; the former examines gland morphologogy and the latter evaluates hormonal changes associated with thyroid disease. The high frequency of thyroid abnormalities detected by ultrasonography suggests that periodic thyroid ultrasonography is advisable in the follow-up of patients treated with mantle irradiation to screen for morphologic changes that may presage malignant transformation.

Ewing's sarcoma of the facial zygomatic area bones in a young child: a case presentation.

We present the case of a young patient with Ewing's sarcoma of the facial zygomatic area bones. This type of tumor in a very young child is a rare event and poses significant diagnostic and therapeutic challenges for the attending physician. In this case, the diagnosis was made by a computed tomography scan with subsequent histological confirmation. The differential diagnoses and therapeutic options are discussed.

Immune and hematopoietic reconstitution after transplantation of cord blood progenitor cells: case report and review of the literature.

Transplantation using umbilical cord progenitor cells as the source of the stem cells is increasingly recognized as another form of allogeneic transplantation with curative intent. However, the different patterns of hematopoietic and immunological reconstruction have been described in very few patients. A 20-month-old boy presented with acute leukemia. He received standard AML induction and consolidation therapy, after which he underwent allogeneic transplantation using HLA-matched sibling stem cells obtained from the umbilical cord. The preparative regimen consisted of busulfan and cyclophosphamide. White cell recovery, despite concomitant use of G-CSF, was slow, reminiscent of the engraftment pattern without the use of growth factor. Erythroid recovery was best recorded using fetal cell HbF level. Platelet transfusion independence occurred on day +31. Immunologic reconstitution revealed an early NK cell recovery by 6 weeks and progressive T cell recovery until 3 months, with continued increase in counts thereafter. However, the CD4/CD8 ratio remained low even at 14 months post-transplantation. Recovery of B cells was slower until day +120. Proliferative response was within normal range on day +120. This report describes the unique engraftment pattern following umbilical cord blood transplant and emphasizes the pattern of immunological and hematological reconstitution.

Male gonadal function in survivors of childhood Hodgkin and non-Hodgkin lymphoma.

The aim of this study was to investigate the impact of therapy on long-term gonadal function of young people cured of childhood lymphomas and to assess whether a prepubertal state during the treatment protects the gonads from chemotherapy and/or radiotherapy late effects. Clinical evaluation, semen analysis, and endocrine status were studied in 20 survivors of childhood lymphomas. Five patients received Inverted Y radiotherapy, 2320 cGy (1550-4000); all 20 received chemotherapy as follows: MOPP/ABVD protocol, 9 patients; COMP protocol, 5 patients; MOPP protocol, 3 patients; other protocols, 3 patients. Semen analysis results were as follows: normal values, 4/20 patients; oligospermia, 8/20 patients; azoospermia, 8/20 patients; FSH above normal level, 10/20 patients; 4/5 who received Inverted Y irradiation were azoospermic and 1 was severely oligospermic. Treatment damage to the testis involves tubular germinal elements. Radiotherapy and chemotherapy combinations that included nitrogen mustard or cyclophosphamide were associated with high rates of oligospermia and azoospermia. MOPP/ABVD combination did not have a significant better outcome of sperm counts compared to MOPP alone. Age at chemotherapy did not correlate with the sperm count; hence a prepubertal state did not protect the gonad from the late effects of treatment.

Changes in the policies of the department of hematology, 1982-1998, designed to promote the mental health of children with leukemia and enhance their quality of life.

A perceived personal control (PPC) preventive intervention model that had earlier received empirical and theoretical verification was applied to a population of pediatric leukemia patients to promote their mental health and enhance their quality of life. The PPC model entails intervention on two complementary levels. On the personal interaction level, preventive intervention is administered by a network of natural and organized support systems, while the social action level leads to the introduction of changes in policies, structures, allocation of resources, and services. Preventive intervention in both domains is discussed with respect to changes in policies introduced between 1982 and 1998 in the hematology department of Rambam Medical Center in Haifa, Israel, as well as in other relevant departments catering to children with leukemia, to foster the positive mental health of these children.

Pediatric thyroid carcinoma: 22 years of experience at the Northern Israel Oncology Center (1973-1995).

Over the past 22 years, 16 children with thyroid carcinoma were referred to the Northern Israel Oncology Center. All patients had undergone surgical procedures, either total or subtotal thyroidectomy, and 7 patients had undergone cervical lymph node dissections. Postoperatively, 5 patients underwent thyroid ablation with radioactive 131I as first treatment. Two patients received postoperative external radiation therapy to a field encompassing the cervical region, superior mediastinum, and both supraclavicular grooves. After a median follow-up of 60 months (range, 5-169 months), all patients are alive with no evidence of recurrent disease. Two patients who had recurrences, one in the submaxillary lymph nodes and one in the lungs, were salvaged successfully with retreatment with 131I therapy. No severe acute or long-term side effects were exhibited. The long-term results of treatment of pediatric thyroid carcinoma are excellent, but there remains disagreement over the extent of surgical and postsurgical treatment required.

Successful haploidentical bone marrow transplantation in Fanconi anemia.

A 10-year-old girl with Fanconi anemia and severe aplastic anemia underwent a haploidentical BMT from her mother due to lack of a matched family donor. T cell depletion was done by positive selection of CD34 cells with immunomagnetic beads. Due to graft rejection a second haploidentical BMT from the father was successfully undertaken. No immunosuppression was given after the transplant. Immunological reconstitution took approximately 6 months, with no GVHD or severe infections. Such a transplant, containing a large purified CD34 cell fraction with a minimal number of added T cells, should be considered as the treatment of choice for patients with Fanconi anemia if no HLA matched donor is available.

The role of chemotherapy in childhood soft tissue sarcomas other than rhabdomyosarcomas: experience of the Northern Israel Oncology Center.

Forty-three children with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS) were treated at the Northern Israel Oncology Center in Haifa, Israel, from 1971 to 1996. The male:female ratio was 1.5:1 and the median age of patient was 10 years (range, 3 months-18 years). The most common histopathologic diagnoses were fibrosarcoma (32.5%) and synovial sarcoma (16%). The sites of primary tumor were lower limb (35%), trunk (18%), upper limb (16%), head and neck (16%), and retroperitoneum (11%). By Intergroup Rhabdomyosarcoma Study classifications, 13 patients presented as group I, 15 patients as group II, 10 patients as group III, and 5 patients as group IV. Median follow-up time was 63 months (range, 6 months-18 years). The estimated survival after a 5-year period is 72% (SE +/- 17) for patients in group I, 75 +/- 15% in group II, 90 +/- 9% for patients in group III, and 40 +/- 21% for patients in group IV. Eleven patients relapsed; 4/6 who developed local relapse were cured and are alive with disease, while 4/5 who developed distant metastases are dead. For the 28 patients who underwent complete resection at diagnosis, the estimated survival after a 5-year period is 87 +/- 5% vs. 60 +/- 17% for the 15 patients who underwent partial excision or biopsy. Local radiotherapy was delivered after surgery to group III patients. Preoperative and postoperative chemotherapy was delivered to the patients of groups III and IV, and postoperative chemotherapy only to group II patients. Chemotherapy produced demonstrable gain in survival for group II and III patients but not for patients with metastases. The authors conclude that an aggressive surgical approach is needed in patients with NRSTS. Chemotherapy may help as a preoperative treatment in bulky disease or as a postoperative treatment for microscopic residual disease.

Prognostic significance of DNA ploidy in childhood astrocytomas.

The ability to divide subsets of children with astrocytoma into prognostic groups is limited because only a few clinical and pathologic variables are available. This study evaluated DNA ploidy as a potential prognostic factor in 30 children with diagnosed gliomas and examined the correlation of flow cytometric analysis to other parameters such as sex, age at diagnosis, histologic grading, localization of tumor, and completeness of surgical resection. Seventeen children with low-grade glioma and 13 with high-grade glioma were retrospectively reviewed; mean age of the patients was 8.2 years, and mean follow-up of the population was 7.6 years. The tumor was localized to the cerebrum in 19 patients, the cerebellum in 7 patients, the brain stem in 3 patients, and the spine in 1 patient. Fourteen patients underwent complete excision and 16 patients underwent partial excision. DNA diploidy was demonstrated in 21 patients and aneuploidy in 9 patients. Twenty children had no evidence of disease and 10 died of disease. Of the patients with diploid tumors, 81% survived, compared to only 33% survival among patients with aneuploid tumors (p < .011). By Cox regression analysis with age, gender, type of excision, grade, location of tumor, and ploidy as independent variables, ploidy was a statistically significant predictor of survival (p = .043). This investigation provides further evidence that flow cytometry may have prognostic value in children with gliomas. Thus, a larger number of tumors can be studied to extend and validate these observations.