Three Saudi siblings presented with clinical features of chronic myelocytic leukaemia in early infancy. Their parents are first cousins. The mother was 22 years old during her first pregnancy. She had no history of abortion. The possibility of this familial disorder being congenital is discussed.
A 4 1/2-year-old girl had colonic malacoplakia of two years' duration, the presenting symptom being rectal bleeding. Abdominal tuberculosis and Escherichia coli lumbar abscess were diagnosed at the age of 3 1/2 years. Despite antituberculous treatment, there was no improvement and she died from protein-losing enteropathy. The patient is discussed and the literature reviewed, with special emphasis on the incidence of malacoplakia in children, the aggressive nature of colonic malacoplakia, and the lack of response to treatment. A brief review of the pathogenesis of malacoplakia is considered.
Neuroblastoma is an embryonic tumor and constitutes about 7% of all childhood cancer. 1 In Saudi Arabia, it has been observed to be the seventh most common pediatric malignancy. 2 The high spontaneous regression potential compounds the difficulty of assessing the effects of therapy.3 Nevertheless, patients who are free of disease 24 months after diagnosis are generally considered cured. 4 Here we report a neuroblastoma with late recurrence. A 20-month-old Saudi girl had a history of abdominal swelling over a period of six weeks. Physical examination showed a left hypochondrial mass that was firm, nontender, nodular, measuring 15 cm by 10 cm in diameter. The VMA and HVA excretion in 24-hour urine collection were 9.5 mg and 36 mg, respectively. An intravenous pyelogram and CT scan of abdomen revealed a huge suprarenal mass. Complete excision of the tumor was performed, and histopathologic studies confirmed the diagnosis of neuroblastoma stage II. The patient was treated with a course of vincristine and cyclophosphamide for six months. Three-and-a-half years after diagnosis, she was admitted with chief complaint of limping and inability to walk. X-ray examination of the vertebral region showed increased density over the fourth lumbar vertebra (Figure 1). Radioisotope bone scanning revealed increased uptake at various levels of the lumbar and thoracic vertebrae ( Figure 2). Treatment with combination chemotherapy and radiotherapy was elected, and currently she is in clinical remission.Late relapse of neuroblastoma is uncommon, with few cases reported in the literature. 5 The period of risk is two years from diagnosis, and absence of disease during this period is considered equivalent to cure. 4 In this case, the patient showed evidence of recurrence after 40 months, which is not as long as in cases reported previously, but is considered to be a late relapse according to the criteria discussed. Follow-up is highly recommended at least until puberty, with clinical and radiological assessments and urine catecholamine determinations. 6 A urine spot test is available for diagnosis and follow-up of patients with neuroblastoma. This correlates very well with 24-hour urine collection for quantitation of urinary VMA, particularly when expressed as µg/mg of urinary creatinine. 6,7 Once the tumor recurs, prognosis is extremely poor, particularly if it is disseminated.
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