TY - JOUR
T1 - Langerhans cell histiocytosis mimicking malignancy
T2 - A radiological appraisal
AU - Potepan, Paolo
AU - Tesoro-Tess, John David
AU - Laffranchi, Alberto
AU - Danesini, Gian Maria
AU - Gianni, Cristina
AU - Uslenghi, Elisabetta
AU - Spagnoli, Ivo
PY - 1996/11
Y1 - 1996/11
N2 - Aims: To analyze the radiologic characteristics, clinical course and long-term follow-up of 7 radiologically uncommon pediatric cases of Langerhans cell histiocytosis and to identify prognostic factors related to imaging patterns. Methods: The clinical records and complete imaging data of 75 patients with LCH diagnosed and treated at the National Cancer Institute of Milan between January 1975 and December 1993 were analyzed, and 43 cases presenting as unifocal bone lesions were identified. The plain film, computed tomography and magnetic resonance characteristics enabled the identification of 7 radiologically aggressive and rapidly progressive cases, which were analyzed at presentation and during follow-up. Results: Although at disease presentation bone lesions appeared lytic destructive, rapidly progressive and often involved adjacent soft tissues, after adequate therapy the disease course was invariably benign and led to almost complete restoration of normal structure and function. Long-term follow-up confirmed the favorable outcome and lack of disease recurrence in all cases. Conclusions: There is no correlation between radiologically aggressive characteristics and final outcome in Langerhans cell histiocytosis. Radiologists and pediatric oncologists should be acquainted with less common radiologic forms which, at presentation, can mimic more ominous diseases. If recognized and adequately treated, monostotic forms almost invariably have a benign prognosis.
AB - Aims: To analyze the radiologic characteristics, clinical course and long-term follow-up of 7 radiologically uncommon pediatric cases of Langerhans cell histiocytosis and to identify prognostic factors related to imaging patterns. Methods: The clinical records and complete imaging data of 75 patients with LCH diagnosed and treated at the National Cancer Institute of Milan between January 1975 and December 1993 were analyzed, and 43 cases presenting as unifocal bone lesions were identified. The plain film, computed tomography and magnetic resonance characteristics enabled the identification of 7 radiologically aggressive and rapidly progressive cases, which were analyzed at presentation and during follow-up. Results: Although at disease presentation bone lesions appeared lytic destructive, rapidly progressive and often involved adjacent soft tissues, after adequate therapy the disease course was invariably benign and led to almost complete restoration of normal structure and function. Long-term follow-up confirmed the favorable outcome and lack of disease recurrence in all cases. Conclusions: There is no correlation between radiologically aggressive characteristics and final outcome in Langerhans cell histiocytosis. Radiologists and pediatric oncologists should be acquainted with less common radiologic forms which, at presentation, can mimic more ominous diseases. If recognized and adequately treated, monostotic forms almost invariably have a benign prognosis.
KW - bone neoplasms
KW - Langerhans cell histiocytosis
KW - magnetic resonance imaging
UR - http://www.scopus.com/inward/record.url?scp=0030425001&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0030425001&partnerID=8YFLogxK
M3 - Article
C2 - 9061074
AN - SCOPUS:0030425001
VL - 82
SP - 603
EP - 609
JO - Tumori
JF - Tumori
SN - 0300-8916
IS - 6
ER -