Inflammatory myofibroblastic tumors in childhood: a report from the Italian Cooperative Group studies.
BACKGROUND
Inflammatory myofibroblastic tumors (IMTs) are myofibroblastic lesions with unpredictable biologic behavior that occur at a young age. For this report, the authors investigated clinicopathologic features in a series of pediatric IMTs. The objective of the study was to identify morphologic or immunohistochemical prognostic markers and the possible pathogenic role of human herpes virus 8 (HHV-8).


METHODS
Twenty-six patients were observed over a period of 18 years. Clinical/histologic data were reviewed, and immunohistochemical/molecular studies were performed.


RESULTS
Patients ages 8-216 months (median age, 60 months) presented with tumors of the lung-bronchus (8 patients), abdomen (17 patients), and thoracic wall (1 patient). Twenty-one patients underwent complete excision, and microscopic or macroscopic residual disease was present in 5 of those patients. Chemotherapy was received by 5 patients. After a median follow-up of 6.6 years, 24 patients were in complete remission, and 2 patients had died of disease. Local recurrences were observed in 6 patients (including 4 recurrences that occurred after a complete excision). Cytologic atypia, low inflammatory infiltrate, and a rich myxoid pattern were detected in patients who had recurrent disease or a poor prognosis. Anaplastic lymphoma kinase (ALK) was positive in 7 patients (including 2 patients with recurrent disease). No correlation between clusterin expression and prognosis was demonstrated. HHV-8 was identified in 1 pulmonary IMT.


CONCLUSIONS
IMTs are locally aggressive lesions. In this series, the local recurrence rate was 23%, and the 5-year and 10-year event-free survival rates were 87.4% and 72.8%, respectively. The results indicated that the treatment of choice is a complete, nonmutilating excision; chemotherapy may be given to patients who have microscopic or macroscopic residual disease, although the results are controversial; cytologic atypia and positive ALK status are more frequent in aggressive tumors, whereas metastatic tumors are negative for ALK; and HHV8 is not involved in the pathogenesis of IMT.
Pathology Department, University Hospital of Padova, Padova, Italy. rita.alaggio@gmail.com
Rita
R
Alaggio
Giovanni
G
Cecchetto
Gianni
G
Bisogno
Claudio
C
Gambini
Maria Luisa
ML
Calabrò
Alessandro
A
Inserra
Renata
R
Boldrini
Gian Luca
GL
De Salvo
Emanuele S
ES
G d'Amore
Patrizia
P
Dall'igna
D000293:Adolescent; D000077548:Anaplastic Lymphoma Kinase; D002648:Child; D002675:Child, Preschool; D005260:Female; D019288:Herpesvirus 8, Human / Q000302:isolation & purification; D006801:Humans; D007223:Infant; D007249:Inflammation / Q000473:pathology*; D007558:Italy; D008297:Male; D047708:Myofibroma / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000378:metabolism; D020794:Receptor Protein-Tyrosine Kinases
C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases