Sustained complete response on crizotinib in primary lung inflammatory myofibroblastic tumor - Case report and literature review
Inflammatory myofibroblastic tumors (IMT) are rare soft tissue tumors of intermediate malignant potential occurring usually in children and adolescents. Treatment options for advanced diseases are limited. A 35-year-old lady presented to us with fever, cough and decreased appetite. On evaluation, she was diagnosed with left lung IMT. She underwent surgery and developed recurrence with pleural nodules after two years. Immunohistochemistry showed positivity for ALK (diffuse). Since recent evidence suggested that crizotinib is effective in advanced IMT with 50% response rates, she was treated with crizotinib 250mg BD with which she had a complete radiological response at three months. She has completed one year of treatment thus far and continues to be in complete remission. Treatment with ALK inhibitors like crizotinib has brought about a paradigm shift in the management of advanced ALK-positive IMT’s with excellent clinical responses which are durable in a majority of cases.
Brunn H. Two interesting benign lung tumors of contradictory histopathology. J ThoracSurg 1939;9:119–31.
Meis JM, Enzinger FM. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor. Am J Surg Pathol 1991;15:1146–56. DOI: https://doi.org/10.1097/00000478-199112000-00005
Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19:859‐72. DOI: https://doi.org/10.1097/00000478-199508000-00001
Cook JR, Dehner LP, Collins MH, et al. Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: a comparative immunohistochemical study. Am J SurgPathol 2001;25:1364–71. DOI: https://doi.org/10.1097/00000478-200111000-00003
Saxena S, Dhal I, Mohanpuria A, et al. Extrapulmonary inflammatory myofibroblastic tumor at different sites with histopathology and immunohistochemical analysis: A case series. Oncol J India 2018;2:80-5. DOI: https://doi.org/10.4103/oji.oji_38_18
Ray A, Suri JC, Bhattacharya D, Gupta A. Bronchoscopic resection of endobronchial inflammatory myofibroblastic tumor: A case report and systematic review of the literature. Lung India 2014;31:172–5. DOI: https://doi.org/10.4103/0970-2113.129866
Maturu VN, Bal A, Singh N. Inflammatory myofibroblastic tumor of the lung in pregnancy mimicking carcinoid tumor. Lung India 2016;33:82–4. DOI: https://doi.org/10.4103/0970-2113.173058
Cerfolio RJ, Allen MS, Nascimento AG, et al. Inflammatory pseudotumors of the lung. Ann ThoracSurg 1999;67:933-6. DOI: https://doi.org/10.1016/S0003-4975(99)00155-1
Agrons GA, Rosado-de-Christenson ML, Kirejczyk WM, et al. Pulmonary inflammatory pseudotumor: radiologic features. Radiology 1998;206:511-8. DOI: https://doi.org/10.1148/radiology.206.2.9457206
Dongel I, Yazkan R, Duman L, et al. Huge inflammatory myofibroblastic tumor of pleura with concomitant nuchal fibroma. Ann Thorac Surg 2013;96:1461–4. DOI: https://doi.org/10.1016/j.athoracsur.2013.01.082
Ueno T, Yamashita M, Sawada S, et al. A rare case of inflammatory myofibroblastic tumor of the diaphragmatic parietal pleura with dissemination. Acta Med Okayama 2015;69:65–8.
Coffin CM, Patel A, Perkins S, et al. ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor. Mod Pathol 2001;14:569–76. DOI: https://doi.org/10.1038/modpathol.3880352
Fuehrer NE, Keeney GL, Ketterling RP, et al. A protein expression and ALK gene rearrangements aid in the diagnosis of inflammatory myofibroblastic tumors of the female genital tract. Arch Pathol Lab Med 2012;136:623-6. DOI: https://doi.org/10.5858/arpa.2011-0341-OA
Butrynski JE, D'Adamo DR, Hornick JL, et al. Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med 2010;363:1727–33. DOI: https://doi.org/10.1056/NEJMoa1007056
Schöffski P, Sufliarsky J, Gelderblom H, et al. Crizotinib in patients with advanced, inoperable inflammatory myofibroblastic tumours with and without anaplastic lymphoma kinase gene alterations (European Organisation for Research and Treatment of Cancer 90101 CREATE): a multicentre, single-drug, prospective, non-randomised phase 2 trial. Lancet Respir Med 2018;6:431-41. DOI: https://doi.org/10.1016/S2213-2600(18)30116-4
Antonescu CR, Suurmeijer AJ, Zhang L, et al. Molecular characterization of inflammatory myofibroblastictumors with frequent ALK and ROS1 gene fusions and rare novel RET rearrangement. Am J Surg Pathol 2015;39:957–67. DOI: https://doi.org/10.1097/PAS.0000000000000404
Li Y, Chen X, Qu Y, Fan JM, Li Y, Peng H et al. Partial response to ceritinib in a patient with abdominal inflammatory myofibroblastictumor carrying a TFG-ROS1 fusion. J Natl Compr Canc Netw 2019;17:1459-62. DOI: https://doi.org/10.6004/jnccn.2019.7360
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