Atypical bone angiomas in children.
Bone angiomas can present in a variety of clinical, radiological and histological forms. The two cases reported illustrate unusual and fairly rare aspects of this vascular tumor.
Biomedical subjects
Publications and source records attributed to E Sonsino.
Bone angiomas can present in a variety of clinical, radiological and histological forms. The two cases reported illustrate unusual and fairly rare aspects of this vascular tumor.
We report the case of a 6 year-old boy with rhabdomyolysis involving only the inferior limbs. It was complicated by renal failure with anuria and intravenous coagulopathy and associated with encephalitis. Virologic investigations showed a recent infection due to Coxsackie B5 virus. Full recovery was observed after symptomatic treatment with peritoneal dialysis, exchange transfusion and heparin therapy.
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Hamartomatos inverted polyps of the rectum were observed in a 12 years-old boy suffering from recurrent rectal discharges of mucus. Diagnosis was made on examination of rectal specimens obtained by local excisions and finally by recto-sigmoïdectomy, showing ectopic lobulated glandular structures deep to the muscularis mucosae. This histological aspect must be differentiated from adenocarcinoma, but in the paediatric age, prognosis depends on the degree of diffusion of the hamartomatous tissue, which may lead to extensive surgery such as recto-sigmoïdectomy. Whether this lesion is dysgenetic or acquired is discussed, and what relation can be presumed with solitary ulcer of the rectum is not clear.
A case of Steiner type neuroapudomatosis presented with a pseudo-Hirschsprung disease in an infant. Secondarily, the facies became typical, while hyperthyrocalcitoninaemia led to the discovery at the age of 2 years and 8 months of a medullary carcinoma of the thyroid. Despite early surgery in the absence of any clinical signs, it was not possible to avoid cervical lymphatic spread.
The syndrome known as the Multiple Endocrine Neoplasia type III or type IIb presents a particular histological intestinal lesion: hyperplasia of the myenteric plexus. The electron microscopic study of the plexus of Auerbach in our observation suggests a disequilibrium of the different types of individual axonal sheaths compared to the descriptions of a normal plexus. The possible role of this anomaly in the genesis of intestinal manifestations in this syndrome is here discussed.
A child is described who developed acute ischaemic necrosis of the oesophagus complicating an otherwise typical case of anaphylactoid purpura. This episode was preceded by a necrotising ileitis requiring intestinal resection and ileostomy. The histological similarities of the lesions in the oesophagus and the ileum suggest a common cause connected with the anaphylactoid purpura.
Involvement of the ureters (rigid and segmented) and bladder was seen in a 6-year-old child whose clinical lesions were typical of Henoch-Schoenlein purpura. Infrapelvic left uretic stenosis developed secondarily whilst the other lesions disappeared. Histological study and in particular cutaneous and renal immunofluorescence made it possible to eliminate the possible diagnosis of polyarteritis nodosa, and to confirm that of Henoch-Schoenlein purpura. An ischaemic vasculitis was the cause of the ureteric and bladder lesions, and of the progression to stenosis. The existence of this possibility should lead to routine examination to detect such lesions. Sequential radiological studies are necessary whenever such lesions are recognised.
Study of a human abdominal paraganglioma under electron microscopy revealed an unusual cytoplasmic inclusion appearing as a hollow cylinder made up of one or several concentric lamellae surrounded by single or aggregated granules. Cross sections of the lamellar element show a discontinuous juxtaposition of particles. It is moderately dense and is bordered by 140--to 250-A granules of sure ribosomal nature. The inclusion is closely connected to the rough endoplasmic reticulum. The origin and significance of this complex are discussed.
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