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.

Malignancies in Down syndrome.

Down Syndrome (DS) is associated with an increased incidence of malignancies, especially leukaemias. We came across 8 DS children presenting with malignancies and having trisomy 21 as the sole cytogenetic abnormality. Of these 8 DS cases, 4 presented with acute lymphocytic leukaemia, 2 with acute myeloid leukaemia and one case each with Hodgkin's disease and Wilms' tumour. There are contradictory reports regarding the distribution of myeloid versus lymphoid malignancies in DS children and their response to therapy. The exact mechanism by which patients with DS are predisposed to develop malignancies is unclear. However, presence of the extra chromosome no. 21 is presumed to disrupt the genetic balance which increases generalized susceptibility to genetic and environmental trauma. Furthermore, an increased methotrexate toxicity observed in these patients should also be taken into consideration in designing treatment for DS children with malignancies.

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.