Intensive chemotherapy in children with stage IV neuroblastoma.

A retrospective analysis of effectiveness of sequential chemotherapy with cyclophosphamide, doxorubicin, cisplatin and etoposide in children with stage IV neuroblastoma was undertaken. Study group included 17 children of mores than one year old with median age of 3 years (range 18 months to 7 years). Fourteen were males and three females. Sites of primary tumor were abdomen in 12 patients, pelvis in 3, paravertebral in 1 and unknown in 1. Metastatic sites included bone marrow (88%), bone (82%), orbit (29.4%) and lymph node (11.7%). One patient had brain parenchymal disease and another had cerebrospinal fluid positivity for malignant cells. Fifteen of the 17 patients had major response with chemotherapy (complete response in two and partial response in 13). Ten of the 15 patients completed four courses of chemotherapy and five patients progressed while on chemotherapy and died. Only two of the ten patients, who had four courses chemotherapy are alive after 2 years. Hence the 2-year survival in this series is 11.7%. There was no toxic death in this study.

Langerhans cell histiocytosis in children less than 2 years of age.

OBJECTIVE: To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life. DESIGN: Retrospective analysis. METHODS: Medical records of Children upto 2 years of age with a diagnosis of langerhans cell histiocytosis (LCH) were analyzed. Their clinical pattern, treatment modalities and outcome were studied. The patients Were categorized into 2 groups according to their clinical presentation: (i) Subject without organ dysfunction; and (ii) cases with organ dysfunction. Treatment considered of surgical intervention, radiotherapy, chemotherapy or combination of all these modalities depending upto the extent of disease. RESULTS:There were 20 children upto 2 years of age with histiocytosis during the 12 year period (January 1983 - December 1994). The median age at diagnosis was 18 months (range 52 days - 24 months). Of the twenty patients,13 patients didn't have organ dysfunction and 7 had organ dysfunction. Out of the 13 children without organ dysfunction eleven patients received treatment and all of them are alive free of disease with a median follow up of 62 months. But all children with organ dysfunction succumbed to disease within a few weeks. CONCLUSION: Children under 2 years of age with localised and or multifocal LCH without organ dysfunction have a good prognosis and they should not be exposed to aggressive form of treatment. All children with organ dysfunction require multi-agent chemotherapy.

Pattern and outcome of neuroblastoma. A 10 year study.

OBJECTIVE: To determine the clinical pattern and outcome of children with neuroblastoma. DESIGN: Retrospective analysis. SETTING: Hospital based information from the case sheets. SUBJECTS: 91 children with age upto 14 years treated in the Regional Cancer Center, Trivandrum. METHODS: Clinical presentation, metastatic pattern and treatment outcome were analyzed. RESULTS: Median age of the study group was 2.5 years with a male:female ratio of 1.6:1. Fifteen per cent children had early stage disease and 85% advanced disease. Five children with stage III and 22 with stage IV disease did not receive may active treatment. The remaining 64 children were evaluated for this analysis with a survival rate of 25%. Two of the four (50%) stage I, four of the six (83.3%) stage II, five of the 18 (27.7%) stage III, three of the 32 (9%) stage IV and two of the four (50%) stage IVs patients are long term survivors. CONCLUSIONS: Majority of the children (> 80%) presented with advanced disease and outcome remained poor. Only 27% of stage III and 9% of stage IV patients are long term survivors in our series. In future, approaches to detect disease at an early stage and aggressive therapeutic strategies in selected patients may improve survival.

Early diagnosis of bone biopsies--the role of sediment cytology.

This study analyses the role of the cytological examination of the sediment of the formalin in which bone biopsy specimens are received, in the early diagnosis of bone lesions. Of the one hundred lesions studied using this technique, twenty six of thirty three malignant lesions (seventy nine percent) could be diagnosed as malignant when relevant clinical and radiological data were considered. Sixty six benign lesions yielded sparsely cellular smears with benign cell. There was one false positive for malignancy. This simple rapid diagnostic technique can be used to advantage in the early diagnosis, and treatment planning of bone lesions.

Chemotherapy in Hodgkin's disease.

OBJECTIVE: To assess the efficacy of primary chemotherapy in patients with clinically staged Hodgkin's disease. DESIGN: Non randomized study. SETTING: Pediatric Oncology Division of Regional Cancer Center. SUBJECTS: Twenty nine children with Hodgkin's disease. INTERVENTIONS: Chemotherapy was given to 21 patients whose parents agreed for the same. Sixteen children received COPP regimen and 5 received MOPP regimen. RESULTS: Complete remission was achieved in 19 patients. The relapse free survival and overall survival in these patients were 76% and 86%, respectively at 5 years. There was no death related to chemotherapy toxicity. CONCLUSION: Combination chemotherapy is an effective modality of treatment for children with Hodgkin's disease.

Neuroblastoma with testicular metastasis. Review of literature and report of a case.

Eventhough neuroblastoma presents with wide spread tumour dissemination, metastasis to testes had rarely been reported. We report a case of Stage IV neuroblastoma who developed metastasis to both testes. He had massive infra-diaphragmatic disease with involvement of left supraclavicular lymph node at presentation. Treatment received by him was not adequate and five months later he developed metastasis to both testes. Hematogenous spread might have been the possibility in this case.

Clear cell sarcoma of kidney (CCSK)--a clinicopathological study of 4 cases.

The clinical and histopathological features of four cases of clear cell sarcoma of kidney (CCSK) or Bone metastasising renal tumour of childhood (BMRTC) are presented. These cases were identified among 107 primary renal tumours in childhood over a period of 15 years (1973-1987) in the Trivandrum Medical College. Of the 107 cases 96 (89.7 percent) were nephroblastomas and 7 (6.6 percent) were Mesoblastic Nephromas. The incidence of Clear cell sarcoma was 3.7 percent. Abdominal mass and haematuria were the most common clinical features. All the four cases occurred in male children with no predilection for the right or the left kidney. At the time of presentation bone metastasis was not present in any of the four cases. Metastasis to scapula and skull was detected ten months after nephrectomy in one case. Of the four patients three were in stage I disease at the time of diagnosis. All the four cases showed the typical gross morphology and the classic microscopic pattern of Clear Cell sarcoma kidney. The treatment was similar in all the four cases with Surgery followed by radiotherapy and chemotherapy (Vincristine, Adriamycin Actinomycin D and cyclophosphamide). Only one of the four patients is alive and well 12 months after surgery. The literature is reviewed along with a discussion of the gross pathology, histology and histogenesis of clear cell sarcoma of kidney.