Inflammatory Myofibroblastic Tumor: A Rare Presentation and an Effective Treatment with Crizotinib

Abstract

<jats:p>Inflammatory myofibroblastic tumor (IMT) is a rare entity of neoplastic origin. It usually occurs in children and adolescents and most commonly involves pulmonary and gastrointestinal sites. Here, the authors present two cases; one is the nine months old boy with a subcutaneous IMT in the left temporal region that was treated successfully with surgical resection. To the best of our knowledge, this is the first reported case of a subcutaneous IMT in this particular region. The second is an eight years old girl with an IMT of the right hemi-pelvis. The mass had complete surgical excision with clear margins and no residual disease. She was kept on regular follow-up with ultrasound abdomen. However, her disease relapsed with the appearance of lesions in right iliac fossa, right ovary, and liver. Biopsy of the relapsed abdominal mass confirmed ALK-positive IMT. She was treated with ALK inhibitor Crizotinib. She was monitored with regular blood complete picture, hepatic and renal function test, and ultrasound abdomen. Her lesions started regressing within one month, and she achieved complete remission after 6 months of treatment.</jats:p>

Rare presentation of inflammatory myofibroblastic tumor on a digit

“…ALK inhibitors, such as crizotinib, may be used for IMT. Crizotinib may be used for visceral IMT 2,15,16 . In a phase II clinical trial of patients with advanced, inoperable IMT treated with crizotinib, a 50% objective response rate was observed, supporting its use in this patient population 17 …”

“…Genomic analysis has shown that 85% of IMT have kinase fusions, involving either ALK , ROS1 , or PDGFRB genes, with a preponderance having the ALK rearrangement 2 . Multiple ALK fusion partners exist, including CLTC , RANBP2 , FN1 , TPM3 , TPM4 , SEC31A , CARS , and TF3 , among others 2,13,15 …”

Liver transplantation for hilar inflammatory myofibroblastic tumor: Case report and review of the literature

“…Because active ALK gene rearrangements have been identified in 50% of cases, targeted ALK inhibition is a promising treatment option 18 . Khail et al 17 reported that it is safe and effective to use ALK inhibitors like crizotinib for patients with unresectable and recurrent ALK‐1 positive tumors in the pediatric population. Dong et al 18 proposed a treatment based on risk stratification for IMT in a retrospective study of 6 described children.…”

“…Surgical treatment is well established in resectable lesions, and complete surgical resection with clear margins is associated with a favorable prognosis. 17,18 Hilar tumors can involve the portal vein, hepatic artery and bile duct, and spread to the branches of the portal triad, causing obstructive symptoms, occlusive phlebitis, and portal hypertension. 19 Thus, liver transplantation is an excellent alternative for locally advanced hilar tumors, since it ensures complete tumor resection with free margins (Table 1).…”

Inflammatory myofibroblastic tumor of the lung - an explosive metastatic case

“…A ressecção cirúrgica completa com margens claras está associada a um prognóstico favorável. (4) A radiação também pode ser aplicada em casos recorrentes ou situações de invasão local de órgãos. (5) Apesar das escassas evidências referentes ao tratamento desses tumores em adultos, o Crizotinibe é amplamente utilizado e eficaz em pacientes com câncer de pulmão…”