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  • 1
    ISSN: 1432-2307
    Keywords: Giant pigmented naevi ; Naevocytoma ; Congenital tumours
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We describe two instances of a previously unrecognized variant of congenital giant pigmented naevus (GPN), presenting as a bulky naevocytic tumour in the perineal region. In both cases the lesion was present at birth and attained massive dimensions. In addition to the characteristic histological patterns found in GPN, which included extensive areas with a neural appearance, these tumours presented an uncommon tendency to form pseudo-follicular structures lined by naevus cells. No features suggestive of malignant transformation were found. Because GPN may associate with an underlying malignancy, accurate diagnosis of this lesion is important in clinical practice.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 14 (1998), S. 537-546 
    ISSN: 1433-0350
    Keywords: Key words Fourth ventricle ; Brain stem ; Cerebellum ; Astrocytoma ; Brain neoplasms ; Craniotomy ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The authors conducted a study of 21 children with benign astrocytomas in the IV ventricle treated with radical tumor resection from 1982 through 1991. The purposes of this study were to identify the tumor origin and neural involvement, and to determine the natural history following surgical resection. Pathological studies showed that 18 were pilocytic astrocytomas (pure pilocytic in 12, mixed in 6), 2 fibrillary, and 1 gemistocytic. In the IV ventricle, 12 patients had a transependymal involvement of the floor (brain stem), 6 had an involvement of the wall (cerebellar peduncle), and 3 had involvement of both floor and wall. A gross total resection was performed in 9 patients, and the remaining 12 patients underwent a subtotal resection. All patients were followed without radiation therapy (RT) or chemotherapy. During a follow-up period of 6.5–15 years, all patients were alive. Eight patients suffered recurrence between five months and 66 months after diagnosis. Of these, five received RT for recurrence and had a complete response in all cases. The remaining 13 patients showed no evidence of disease and one had a stable residual tumor. The recurrence-free 5-year and 10-year survival rates were 62.5% and 57% respectively. Patients without brainstem involvement, with total resection, or with pure pilocytic astrocytoma had a better outcome than those with brain stem involvement, with subtotal resection, or with nonpilocytic or mixed histology. In summary, a great majority of benign IV ventricle astrocytomas involve the floor of the IV ventricle. It is often difficult to determine the origin of these tumors in most cases. Benign IV ventricle astrocytomas may not recur even after incomplete resection, and close observation without RT is recommended, although RT appears to be effective for these tumors when they recur.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric and developmental pathology 3 (2000), S. 61-69 
    ISSN: 1615-5742
    Keywords: Key words: hypophosphatemia, oncogenic osteomalacia, phosphaturic mesenchymal tumor, rickets
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: ABSTRACT We describe two prepubertal girls with oncogenic rickets. The first patient, 9 years of age, presented with recent-onset lower-extremity pain. The second girl, presented at 4 years of age following a 9-month period of muscle weakness, bone pain, and poor linear growth. Laboratory analyses in both patients revealed hypophosphatemia and hyperphosphaturia; elevated circulating alkaline phosphatase activity was present in one of them. Radiographic evidence of a generalized rachitic process was evident in both cases. Computerized tomography of the paranasal sinuses and facial bones in patient 1 revealed a small lesion eroding through the inner table of the left mandibular ramus. Microscopic examination of this mass revealed a spindle cell neoplasm with chondroid material, dystrophic calcification, and both osteoclast-like and fibroblast-like cells. Prominent vascularity and marked atypia were present. These features are consistent with a phosphaturic mesenchymal tumor of the mixed connective tissue variant. In the second patient, computerized tomography revealed a lytic lesion located in the right proximal tibia, with histologic features consistent with a phosphaturic mesenchymal tumor of the nonossifying fibroma-like variant. Resection of each tumor resulted in rapid correction of the phosphaturia and healing of the rachitic abnormalities. A careful search for small or occult tumors should be carried out in cases of acquired phosphaturic rickets.
    Type of Medium: Electronic Resource
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