An asymptomatic tumor of the head of the pancreas observed in a 12‐year‐old girl was removed by radical excision. the cell of origin of the tumor is demonstrated ultrastructurally to be other than islet or exocrine, and probably to have been derived from ductular epithelium. the malignant or benign nature of this tumor is discussed and requires longer follow‐up for clarification.
A 1-month-old boy was admitted because of failure to thrive. He was floppy and had bilateral ptosis, diminished reflexes, and poor suck. He had aspiration pneumonia, developed seizures, and died at age 3 1/2 months. Laboratory data showed lactic acidosis, proteinuria, glycosuria and generalized aminoaciduria. He was an only child, and family history was negative. Muscle biopsy showed large clumps of granules positive with oxidative enzyme stains and increased lipid droplets. Ultrastructural studies showed large aggregates of mitochondria, many of which were greatly enlarged and contained disoriented or concentric whorls of cristae and paracrystalline inclusions. Cytochrome c oxidase was absent in fresh frozen sections by histochemical staining. By biochemical assay, cytochrome c oxidase (cytochrome aa3) was 6% of normal in muscle biopsy and undetectable in autopsy muscle; spectra and content of cytochromes showed lack of cytochrome aa3, decreased cytochrome b and normal cytochrome cc1. In kidney, cytochrome-c-oxidase activity was 38% of normal and spectra showed decreased cytochromes aa3 and b. The association of fatal infantile mitochondrial myopathy, lactic acidosis and renal dysfunction was previously reported by Van Biervliet et al and appears to be a distinct nosologic entity, one of the few biochemically defined mitochondrial myopathies.
Deciding whether a visible or palpable lump should be excised is a trivial problem if one believes that every unexplained mass in an infant or a child must be promptly removed. But with the present increased public awareness of cancer, this approach would unnecessarily raise parental anxiety. This review of superficial lumps excised in infants and children shows that approximately 1% are malignant. Although a precise diagnosis on the basis of clinical findings remains imperfect, four fifths of the malignant lesions can be recognized on the basis of five risk factors: onset in the neonatal period, a history of rapid or progressive growth, skin ulceration, fixation to or location deep to the fascia, and a firm mass >3 cm in diameter. In the abscence of any of these risk factors, parents can be reassured with a 99.7% accuracy about the benign nature of their child's lump at the initial consultation. Approximately 6% of these lumps will spontaneously regress and, therefore, do not require excision. However, more than 90% of superficial lumps will persist or slowly enlarge and should be electively excised for cosmetic reasons, to prevent late infection or inflammation, and to diagnose the remaining three malignant lesions per thousand lumps that would not be recognized using the above five risk factors.
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