关键词:
uterine neoplasms
摘要:
Correspondence to Dr Zheng Yuan Ng, Department of Gynaecological Oncology, KK Women's and Children's Hospital, Singapore 229899; ngzhengyuan@*** Inflammatory myofibroblastic tumors of the uterus are uncommon mesenchymal neoplasms with intermediate malignant potential.1 Previously classified under 'inflammatory pseudo-tumors', inflammatory myofibroblastic tumors most commonly involve the lungs and intra-abdominal soft tissue.2 Accurate identification is important given the risk of local recurrence (25%) and distant metastasis (2%).1 Approximately half of inflammatory myofibroblastic tumors harbor rearrangements in the anaplastic lymphoma kinase (ALK) gene on chromosome 2p23.1 ALK is a transmembrane tyrosine kinase which undergoes translocation with various fusion partners, resulting in the 3′ kinase portion of ALK being joined to the 5′ portion of a constitutively expressed gene, forming a fusion oncogene.3 A common breakpoint is intron19, resulting in dissociation of 3′ exons 20–29 from 5′ exons 1–19.4 Previously described fusion partners with ALK include IGFBP5, THBS1, FN1, and TIMP3.2 Unlike soft tissue or pulmonary inflammatory myofibroblastic tumors, ALK is the only receptor tyrosine kinase implicated in uterine inflammatory myofibroblastic tumors to date.
There should be a higher index of suspicion for inflammatory myofibroblastic tumors when managing women with fibroids in the younger age group, during pregnancy, or with submucosal/polypoidal location.1 Adverse prognostic factors include delayed diagnosis, incomplete resection, tumor size >7 cm, predominant myxoid pattern, moderate–severe atypia, infiltrative borders, and high mitotic count.2 4 Where possible, morcellation should be avoided or contained in-bag to reduce the likelihood of abdominopelvic recurrence.
Accurate diagnosis of inflammatory myofibroblastic tumors may allow targeted therapy with tyrosine kinase inhibitors in cases of recurrence, particularly in young women who want to preserve t