Kikuchi-Fujimoto disease: a rare but important cause of lymphadenopathy.

UNLABELLED: Cervical lymphadenopathy is a common problem in children. Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis) is a rare condition that causes persistent lymphadenopathy and has a characteristic histological appearance. Kikuchi-Fujimoto disease is well recognized in Japan, where it was first described, but descriptions in the paediatric literature are sparse. Paediatricians may therefore be unaware of this rare but important condition. The case is described of a 14-y-old girl suffering from fatigue, weight loss, night sweats and lymphadenopathy. The predominance of systemic symptoms prompted an extensive investigation. The diagnosis of Kikuchi-Fujimoto disease was made after direct histological examination of a lymph node biopsy. The case illustrates the clinical features of this little-known condition and highlights the potential confusion with other diagnoses. The pathological features are discussed and the literature reviewed. CONCLUSION: Kikuchi-Fujimoto disease should be considered in cases of persistent lymphadenopathy. An early biopsy can be instrumental in preventing unnecessary investigations and potentially harmful treatments.

Small round cell tumor with biphenotypic differentiation and variant of t(21;22)(q22;q12).

A 14-year-old boy presented with a soft tissue swelling on the outer aspect of his left upper arm. Examination of the tumor by light microscopy showed a small round cell tumor with a rare focus of myogenic differentiation. Myogenic differentiation was confirmed on ultrastructural examination by immunohistochemistry and reverse transcriptase polymerase chain reaction (RT-PCR). Conventional G-banding and fluorescent in situ hybridization (FISH) demonstrated a complex variant of t(21;22)(q22;q12). By RT-PCR, the EWS-ERG fusion transcript was defined as type 9e. This tumor was unusual in that it showed characteristics of myogenic and neural differentiation, and contained a rearrangement of the EWS gene consistent with a diagnosis of Ewing's sarcoma. This supports the hypothesis that a class of biphenotypic childhood sarcomas, with features of myogenic and neural differentiation, exists that may be related to the Ewing's sarcoma family of tumors.

Leptomeningeal melanoma in childhood.

BACKGROUND: Malignant melanoma (MM) is one of the least common types of childhood cancer, accounting for less than 1% of all pediatric malignancies. Neurocutaneous melanosis (NCM) is a rare phakomatosis consisting of congenital abnormal pigmentation of the skin and meninges. The meningeal lesions are particularly prone to malignant change. METHODS: The authors describe 5 patients with NCM and 1 with primary leptomeningeal melanoma (LMM) seen at 2 treatment centers in the north of England over a 13-year period (1984-1997). RESULTS: The clinical features, progress, radiological findings, and treatment of these patients are discussed. All six died within eight months of their diagnosis, illustrating the difficulties faced in treating patients with these conditions. The authors reviewed the published literature on NCM, concentrating on the various therapeutic strategies that have been tried. Very little consistency in approach was found. Malignant skin lesions in NCM may be less responsive than primary malignant melanoma, but the small number of patients with primary LMM or brain metastases of MM make comparisons with NCM difficult. The authors' own series illustrates well the piecemeal nature of therapy for patients with these rare conditions. CONCLUSIONS: The rate of incidence of MM melanoma in the U.K. is increasing, and it will represent an increasing proportion of the pediatric oncologist's workload. A consistent approach to the therapy of patients with metastatic MM and NCM is needed if we are to have any hope of offering more than palliative therapy to these children in the future.

Central nervous system metastasis in Wilms' tumor: a review of three consecutive United Kingdom trials.

BACKGROUND: Central nervous system (CNS) metastasis is an uncommon event in pediatric oncology, and typically occurs at the end stage of the disease. Previous reports suggest that brain metastases in patients with Wilms' tumor might behave differently. METHODS: This study reviews the data from three consecutive United Kingdom Children's Cancer Study Group trials (UKW 1, 2, and 3) focusing on this entity. RESULTS: Seven children of 1249 (0.6%) entered into Wilms' tumor studies developed CNS metastases between 2-27 months after initial diagnosis. At last follow-up 3 patients still were alive and 4 had died; the mean follow-up from recurrence in the surviving patients was 63 months. Radiotherapy and chemotherapy were administered to all surviving patients. Those patients who died had tumors with particularly aggressive features or extensive disease. CONCLUSIONS: CNS metastasis of Wilms' tumor is not in itself a terminal event. With regard to other sites of recurrence, salvage therapy can be expected to be effective in patients without other adverse prognostic features.

Viral haemorrhagic fevers: current status, future threats.

In developing countries, the major outbreaks of viral haemorrhagic fevers such as Marburg, Ebola and Lassa fever viruses have been nosocomially spread. The high mortality and absence of specific treatment have had a devastating effect. Epidemics of this highly contagious disease remain a constant threat to Australia and, as a result, carefully planned laboratory and public health strategies and clinical infection control measures have been instituted for the management of suspected cases.

Multifocal Langerhans' cell histiocytosis in a 4-year-old child: a case report.

A 4-year-old girl presented with pain associated with a mobile mandibular left permanent first molar. The mandibular left primary second molar was also found to be mobile and the gingiva buccal to these teeth was inflamed. A well-defined radioluceny of the left posterior mandible was detected radiographically. Langerhans' cell histiocytosis had been diagnosed 10 months previously when she had presented with an isolated tibial lesion. Curettage of the mandibular lesion and extraction of four adjacent teeth was performed. Post-operatively, radiographic skeletal screening revealed osteolytic lesions in the right parietal bone and right eighth rib. A 6-month course of chemotherapy was instigated which resulted in bony healing of all the osteolytic lesions.

Malignant germ cell tumours in two siblings.

Familial germ cell tumours are well recognised in kinship with gonadal dysgenesis, but their occurrence in siblings with normal chromosomes is rare. We report two sisters who presented within a 4 month period with malignant ovarian germ cell tumours; one a dysgerminoma and the other a mixed tumour with marked choriocarcinomatous elements. Both children had a normal female constitutional karyotype and normal phenotype.

Improved survival for childhood acute lymphoblastic leukemia: possible effect of protocol compliance.

Survival rates were analyzed for an effectively population-based series of 77 children with acute lymphoblastic leukemia treated at one hospital during 1970-1981. Treatment was according to Medical Research Council protocols UKALL I to UKALL VII but with a great emphasis on compliance. The relapse-free survival rate was 54.5% at 5 years. The overall 5-year survival rate of 64% was substantially higher than the 47% recorded during a similar period over the rest of Britain. The difference between survival rates in this series and nationally was especially marked for children aged 2-9 years (76% versus 50%) and with white blood count under 10 x 10(9)/L (87% versus 57%). For both of these groups, the survival rates achieved were similar to those now being recorded for the UKALL VIII trial in which treatment is more sustained than in its predecessors and there is greater emphasis on doctor compliance with protocol. These results suggest that although the advantage of UKALL VIII over previous trials for poor prognosis patients may be attributed to the more sustained treatment, the improvement for good prognosis patients may be due to more rigorous compliance with protocol.

Serological study of Pneumocystis carinii infection in the absence of immunosuppression.

Serum samples from 145 children with no known immunosuppressive illness were examined by indirect immunofluorescence for antibody to Pneumocystis carinii. Positive antibody titres (greater than or equal to 1:8 dilution) were found in 69 children (48%). Antibody could not be detected in the remaining children. Previous studies have shown that at least 75% of children have antibodies to P. carinii by the age of 4 years. This study shows a lower percentage of children with detectable antibody. This may be related to geographical variation of antigen or possibly to the widespread use of co-trimoxazole.

Acute encephalopathy during induction therapy for acute lymphoblastic leukemia.

Twenty-one children who suffered an acute encephalopathic illness during induction chemotherapy for ALL are described. Convulsions and coma were a major feature of this illness. In the absence of other predisposing factors we feel that the encephalopathy was secondary to drug toxicity. Two children died, and 4 of the surviving children have also died. Modifications of antileukemic therapy because of encephalopathic illness may have had a part to play in the outcome for some of these children. The surviving children appear to be neurologically and developmentally normal.