Inflammatory myofibroblastic tumors in childhood

A report from the italian cooperative group studies

Rita Alaggio, Giovanni Cecchetto, Gianni Bisogno, Claudio Gambini, Maria Luisa Calabrò, Alessandro Inserra, Renata Boldrini, Gian Luca De Salvo, Emanuele S G D'Amore, Patrizia Dall'Igna

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish
Pages (from-to)216-226
Number of pages11
JournalCancer
Volume116
Issue number1
DOIs
Publication statusPublished - Jan 1 2010

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Neoplasms
Recurrence
Clusterin
Viruses
Drug Therapy
Lung
Thoracic Wall
Bronchi
Abdomen
Disease-Free Survival
Survival Rate
Pediatrics
anaplastic lymphoma kinase

Keywords

  • Anaplastic lymphoma kinase
  • Children
  • Clusterin
  • Inflammatory myofibroblastic tumors
  • Pseudotumor

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Inflammatory myofibroblastic tumors in childhood : A report from the italian cooperative group studies. / Alaggio, Rita; Cecchetto, Giovanni; Bisogno, Gianni; Gambini, Claudio; Calabrò, Maria Luisa; Inserra, Alessandro; Boldrini, Renata; De Salvo, Gian Luca; D'Amore, Emanuele S G; Dall'Igna, Patrizia.

In: Cancer, Vol. 116, No. 1, 01.01.2010, p. 216-226.

Research output: Contribution to journalArticle

Alaggio, Rita ; Cecchetto, Giovanni ; Bisogno, Gianni ; Gambini, Claudio ; Calabrò, Maria Luisa ; Inserra, Alessandro ; Boldrini, Renata ; De Salvo, Gian Luca ; D'Amore, Emanuele S G ; Dall'Igna, Patrizia. / Inflammatory myofibroblastic tumors in childhood : A report from the italian cooperative group studies. In: Cancer. 2010 ; Vol. 116, No. 1. pp. 216-226.
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abstract = "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.",
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AU - Inserra, Alessandro

AU - Boldrini, Renata

AU - De Salvo, Gian Luca

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N2 - 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.

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