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At least 613 records · Page 34Linked to original sources
PubMed1977

Malignant non-Hodgkin's lymphoma (NHL) in childhood. Retrospective analysis of 34 cases.

Of 47 children with an initial diagnosis of lymphosarcoma, reticulosarcoma or Non-Hodgkin's lymphoma (NHL), 13 had to be excluded at the histologic reevaluation: in 10 an undifferentiated sarcoma, in 2 Hodgkin lymphoma was found; in one patient no definite classification of the tumor was possible. Of the remaining 34 patients there were 26 boys and 8 girls. One patient had a nodular, 33 a diffuse NHL. Of the latter 16 had a Burkitt-type (LB-), 3 a lymphoblastic, convoluted (LC-), 8 a lymphoblastic, "other" (LO-) and 6 a histiocytoid (H-) NHL. Primary localization: abdomen: 13/34; "peripheral" lymph nodes: 9/34; mediastinum: 5/34; nasopharynx: 4/34; subcutis: 2/34; skeleton: 1/34. Twelve of 17 NHL with primary localization in the abdomen or nasopharynx were LB-NHL, 8/14 NHL with primary localization in "peripheral" nodes or mediastinum were LC- or LO-NHL. Only 2/17 NHL with abdominal or nasopharyngeal primary, but 9/14 NHL with "peripheral" nodal or mediastinal primary developed leukemic extension and/or CNS involvement. 6 of 34 patients are living without evidence of disease for 1 1/2+ to 13+ years; 5/34 died but lived for 85, 57, 37, 22 and 22 months; 9/34 lived 6--12 months; 14/34 died within less than 6 months. Patients with abdominal primary either died within 5 months or survived (for 165+, 63+ and 25+ months). Aggressive local therapy (surgery and radiotherapy with approximately 4000 R) may be adequate for strictly localized (stage I) disease, particularly if the primary localization is abdominal. In all other diffuse NHL of childhood an early, aggressive chemotherapy, later combined with radiotherapy to bulk disease and prophylactic CNS-treatment is essential for inducing long-term remissions and, possibly, cures. For prognosis the primary localization appeared to be more important than histology and stage. The most decisive factor, however, is therapy.

Adolescent↗
PubMedDate not supplied

[Sarcoma of the breast].

Reports of 4 personal cases of breast sarcomas (3 cases of cystosarcoma phyllodes and 1 angiosarcoma), the authors review the literature concerning these rare tumors which account for less than 1% of malignant tumors of the breast. Whilst the usual clinical picture is that of a large tumor deforming the breast with pseudo-inflammatory signs, sarcomas 1 to 3 cm in diameter were discovered and treated with good chances of recovery. The natural history of breast sarcomas is marked above all by the risk of hematogenic metastatic spread (30% of cases) whilst very few (5%) local recurrences are seen following surgical treatment by mastectomy. Emphasis is placed upon problems of histological diagnosis of the variety of sarcoma and the demonstration of histoprognostic factors which can be used to predict the outcome (mitotic activity, grading) showing evidence of good correlation in the series analyzed. The various characteristics of cystosarcoma phyllodes are described emphasizing the conditions surrounding its development, relations with benign phyllodes tumors. The particular severity of this sarcomatous form in the very young woman is mentioned. Prevention lies in the sufficiently extensive excision of the phyllodes tumor. Mesenchymatous sarcomas of the breast are also accompanied by a fairly favorable prognosis (5 year actuarial survival of 50 to 80% according to series). By contrast, two special and rare histological varieties, angiosarcoma and lymphosarcoma, are characterized by a much more gloomy prognosis and require different treatment including radiotherapy and chemotherapy.

Adult↗