Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children20210.24287/1726-1708-2018-17-3-93-102CLINICAL OBSERVATIONSКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCase report of the inflammatory myofibroblastic tumor of the liver in infantОписание клинического случая развития воспалительной миофибробластической опухоли печени у пациента раннего возрастаhttps://orcid.org/0000-0002-5489-1879SuleymanovaA. M.СулеймановаА. М.
Russian Federation

Amina M. Suleymanova, MD, рediatric oncologist, Department of сlinical оncology

117997, Moscow, Samory Mashela st., 1

Сулейманова Амина Магомедовна, врач-детский онколог, отделение клинической онкологии 

117997, Москва, ГСП-7, ул. Саморы Машела, 1

aminasuleymanova313@gmail.comFilinA. V.ФилинА. В.
Russian Federation
https://orcid.org/0000-0002-3704-8783KachanovD. Yu.КачановД. Ю.
Russian Federation
https://orcid.org/0000-0002-3767-4477ShamanskayaT. V.ШаманскаяТ. В.
Russian Federation
MetelinA. V.МетелинА. В.
Russian Federation
FeoktistovaE. V.ФеоктистоваЕ. В.
Russian Federation
RoschinV. Yu.РощинВ. Ю.
Russian Federation
ShcherbakovA. P.ЩербаковА. П.
Russian Federation
https://orcid.org/0000-0001-7317-7104TereschenkoG. V.ТерещенкоГ. В.
Russian Federation
ZemcovaL. V.ЗемцоваЛ. В.
Russian Federation
PreobrazhenskayaE. V.ПреображенскаяЕ. В.
Russian Federation
https://orcid.org/0000-0001-9764-2090SuspitsinE. N.СуспицынЕ. Н.
Russian Federation
VarfolomeevaS. R.ВарфоломееваС. Р.
Russian Federation
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева»B.V. Petrovsky Russian Scientific Center of SurgeryФГБНУ «Российский научный центр хирургии им. академика Б.В. Петровского»N.N. Petrov National Medical Research Center of OncologyФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Петрова»St. Petersburg State Pediatric Medical UniversityФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет»24092018173931022409201824092018Copyright ©; 2018, «D. Rogachev NMRCPHOI»Copyright ©; 2018, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2018«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/202

The inflammatory myofibroblastic tumor (IMT) is an extremely rare type of liver tumors in young children. This article presents a clinical case of the development of IMT of the liver in 11-month-old child with the detected chimeric TPM3ex6 / ALKex20 transcript. State-of-the-art data on the clinical presentation of IMT, histological and molecular genetic characteristics of the tumor, differential diagnosis of hepatic masses depending on the level of alpha-fetoprotein, the principles of treatment of IMT, based on a review of the scientific literature, including data on the effectiveness of ALK inhibitors are described.

Воспалительная миофибробластическая опухоль (ВМО) печени – крайне редкий вид опухолевого поражения печени у детей раннего возраста. В настоящей статье представлен клинический случай развития ВМО печени у ребенка 11 месяцев с выявленным химерным транскриптом TPM3ex6/ALKex20. Приведены современные данные о клинической картине заболевания, гистологических и молекулярно-генетических характеристиках опухоли, дифференциальной диагностике объемных образований печени в зависимости от уровня альфа-фетопротеина, принципах лечения ВМО¸ основанные на обзоре научной литературы, включая данные об эффективности ингибиторов ALK.

childreninflammatory myofibroblastic tumorALK geneliver tumorsдетивоспалительная миофибробластическая опухольген ALKопухоли печени1. Coffin C.M., Fletcher J.A. Inflammatory myofibroblastic tumour. WHO Classi-fication of Tumours of Soft Tissue and Bone. WHO/IARC Classification of Tumours, 4th Edition, Volume 5. Edited by Fletcher C.D.M., Bridge J.A., Hogendoorn P.C.W., Mertens F. International Agency for Research on Cancer. Lyon, 2013; р. 83–6.Coffin C.M., Fletcher J.A. Inflammatory myofibroblastic tumour. WHO Classi-fication of Tumours of Soft Tissue and Bone. WHO/IARC Classification of Tumours, 4th Edition, Volume 5. Edited by Fletcher C.D.M., Bridge J.A., Hogendoorn P.C.W., Mertens F. International Agency for Research on Cancer. Lyon, 2013; р. 83–6.2. Coffin C.M., Watterson J., Priest J.R., Dehner L.P. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995; 19 (8): 859–72.Coffin C.M., Watterson J., Priest J.R., Dehner L.P. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995; 19 (8): 859–72.3. Качанов Д.Ю., Шаманская Т.В., Сулейманова А.М., Варфоломеева С.Р., Меркулов Н.Н., Феоктистова Е.В. и др. Воспалительная миофиб-робластическая опухоль брыжейки тонкой кишки, осложнившаяся острой кишечной непроходимостью: описание клинического случая и обзор литературы 2017; 16 (1): 54–61.Качанов Д.Ю., Шаманская Т.В., Сулейманова А.М., Варфоломеева С.Р., Меркулов Н.Н., Феоктистова Е.В. и др. Воспалительная миофиб-робластическая опухоль брыжейки тонкой кишки, осложнившаяся острой кишечной непроходимостью: описание клинического случая и обзор литературы 2017; 16 (1): 54–61.4. Качанов Д.Ю., Шаманская Т.В., Добреньков К.В., Варфоломеева С.Р. Воспалительная миофибробластическая опухоль у детей. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2008; 7 (3): 16–20.Качанов Д.Ю., Шаманская Т.В., Добреньков К.В., Варфоломеева С.Р. Воспалительная миофибробластическая опухоль у детей. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2008; 7 (3): 16–20.5. Blohm M.E., Vesterling-Hörner D., Calaminus G., Göbel U. Alpha-1-fetoprotein (AFP) reference values in infants up to 2 years of age. Pediatr Hematol Onco 1998; 15 (2): 135–42.Blohm M.E., Vesterling-Hörner D., Calaminus G., Göbel U. Alpha-1-fetoprotein (AFP) reference values in infants up to 2 years of age. Pediatr Hematol Onco 1998; 15 (2): 135–42.6. Dalton B.G., Thomas P.G., Sharp N.E., Manalang M.A., Fisher J.E., Moir C.R., et.al. Inflammatory myofibroblastic tumors in children. J Pediatr Surg 2016; 51 (4): 541–4.Dalton B.G., Thomas P.G., Sharp N.E., Manalang M.A., Fisher J.E., Moir C.R., et.al. Inflammatory myofibroblastic tumors in children. J Pediatr Surg 2016; 51 (4): 541–4.7. Alaggio R., Cecchetto G., Bisogno G., Gambini C., Calabrò M.L., Inserra A., et al. Inflammatory myofibroblastic tumors in childhood: a report from the Italian Cooperative Group studies. Cancer 2010; 116 (1): 216–26.Alaggio R., Cecchetto G., Bisogno G., Gambini C., Calabrò M.L., Inserra A., et al. Inflammatory myofibroblastic tumors in childhood: a report from the Italian Cooperative Group studies. Cancer 2010; 116 (1): 216–26.8. Pack G.T., Baker H.W. Total Right Hepatic Lobectomy: Report of a Case. Ann Surg. 1953; 138 (2): 253–8.Pack G.T., Baker H.W. Total Right Hepatic Lobectomy: Report of a Case. Ann Surg. 1953; 138 (2): 253–8.9. Goldsmith P.J., Loganathan A., Jacob M., Ahmad N., Toogood G.J., Lodge J.P., et al. Inflammatory pseudotumours of the liver: a spectrum of presentation and management options. Eur J Surg Oncol 2009; 35 (12): 1295–8.Goldsmith P.J., Loganathan A., Jacob M., Ahmad N., Toogood G.J., Lodge J.P., et al. Inflammatory pseudotumours of the liver: a spectrum of presentation and management options. Eur J Surg Oncol 2009; 35 (12): 1295–8.10. Torzilli G., Inoue K., Midorikawa Y., Hui A.M., Takayama T., Makuuchi M. Inflammatory pseudotumours of the liver: prevalence and clinical impact in surgical patients. Hepatogastroente-rology 2001; 48 (40): 1118–23.Torzilli G., Inoue K., Midorikawa Y., Hui A.M., Takayama T., Makuuchi M. Inflammatory pseudotumours of the liver: prevalence and clinical impact in surgical patients. Hepatogastroente-rology 2001; 48 (40): 1118–23.11. Teranishi N., Yoshida H., Mamada Y., Taniai N., Mizuguchi Y., Shimizu T., et al. Inflammatory pseudotumor in the Spiegel lobe of the liver of an elderly woman. J Nippon Med Sch 2005; 72: 121–6.Teranishi N., Yoshida H., Mamada Y., Taniai N., Mizuguchi Y., Shimizu T., et al. Inflammatory pseudotumor in the Spiegel lobe of the liver of an elderly woman. J Nippon Med Sch 2005; 72: 121–6.12. Nagarajan S., Jayabose S., McBride W., Prasadh I., Tanjavur V., Marvin M.R., et al. Inflammatory myofibroblastic tumor of the liver in children. J Pediatr Gastroenterol Nutr 2013; 57 (3): 277–80.Nagarajan S., Jayabose S., McBride W., Prasadh I., Tanjavur V., Marvin M.R., et al. Inflammatory myofibroblastic tumor of the liver in children. J Pediatr Gastroenterol Nutr 2013; 57 (3): 277–80.13. Cheuk W., Chan J.K., Shek T.W., Chang J.H., Tsou M.H., Yuen N.W., et al. Inflammatory pseudotumor-like follicular dendritic cell tumor: a distinctive lowgrade malignant intra-abdominal neoplasm with consistent Epstein-Barr virus association. Am J Surg Pathol 2001; 25: 721–31.Cheuk W., Chan J.K., Shek T.W., Chang J.H., Tsou M.H., Yuen N.W., et al. Inflammatory pseudotumor-like follicular dendritic cell tumor: a distinctive lowgrade malignant intra-abdominal neoplasm with consistent Epstein-Barr virus association. Am J Surg Pathol 2001; 25: 721–31.14. Zen Y., Fujii T., Sato Y., Masuda S., Nakanuma Y. Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease. Mod Pathol 2007; 20 (8): 884–94.Zen Y., Fujii T., Sato Y., Masuda S., Nakanuma Y. Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease. Mod Pathol 2007; 20 (8): 884–94.15. Ogawa T., Yokoi H., Kawarada Y.A. Case of inflammatory pseudotumor of the liver causing elevated serum CA19-9 levels. Am J Gastroenterol 1998; 93: 2551–5.Ogawa T., Yokoi H., Kawarada Y.A. Case of inflammatory pseudotumor of the liver causing elevated serum CA19-9 levels. Am J Gastroenterol 1998; 93: 2551–5.16. Brugiěres L. Clinical presentation and diagnosis. In book Pediatric liver tumors, Zimmerman A., Perilongo G (eds). Springer, 2011; р. 59–64.Brugiěres L. Clinical presentation and diagnosis. In book Pediatric liver tumors, Zimmerman A., Perilongo G (eds). Springer, 2011; р. 59–64.17. Brunello F., Caremani M., Marcarino C., Benci A., Menchettiry D., Emanuelli G. Inflammatory pseudotumour of the liver: Diagnosis by fine needle biopsy in two cases and a review of the literature. Ital J Gastroenterol 1994; 26: 151–3.Brunello F., Caremani M., Marcarino C., Benci A., Menchettiry D., Emanuelli G. Inflammatory pseudotumour of the liver: Diagnosis by fine needle biopsy in two cases and a review of the literature. Ital J Gastroenterol 1994; 26: 151–3.18. Goldblum J.R., Folpe A.L., Weiss S.W. Borderline and malignant fibroblastic/ myofibroblastic tumors. In book Enzinger and Weiss’s Soft Tissue Tumors. Goldblum J.R., Folpe A.L., Weiss S.W. (eds). Elsevier Saunders. Philadelphia, 2014; р. 304–10.Goldblum J.R., Folpe A.L., Weiss S.W. Borderline and malignant fibroblastic/ myofibroblastic tumors. In book Enzinger and Weiss’s Soft Tissue Tumors. Goldblum J.R., Folpe A.L., Weiss S.W. (eds). Elsevier Saunders. Philadelphia, 2014; р. 304–10.19. Someren A. Inflammatory pseudotumour of liver with occlusive phlebitis: report of a case in child and review of the literature. Am J Clin Pathol 1978; 69 (2): 176–816.Someren A. Inflammatory pseudotumour of liver with occlusive phlebitis: report of a case in child and review of the literature. Am J Clin Pathol 1978; 69 (2): 176–816.20. Lee S.L., DuBois J.J. Hepatic inflammatory pseudotumor: case report, review of the literature, and a proposal for morphologic classification. Pediatr Surg Int 2001 Sep; 17 (7): 555–9.Lee S.L., DuBois J.J. Hepatic inflammatory pseudotumor: case report, review of the literature, and a proposal for morphologic classification. Pediatr Surg Int 2001 Sep; 17 (7): 555–9.21. Coffin C.M., Patel A., Perkins S., Elenitoba-Johnson K.S., Perlman E, Griffin C.A. ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor. Modern Pathology 2001; 14 (6): 569–76.Coffin C.M., Patel A., Perkins S., Elenitoba-Johnson K.S., Perlman E, Griffin C.A. ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor. Modern Pathology 2001; 14 (6): 569–76.22. Lovly C.M., Gupta A., Lipson D., Otto G., Brennan T., Chung C.T., et al. Inflammatory myofibroblastic tumors harbor multiple potentially actionable kinase fusions. Cancer Discov 2014; 4 (8): 889–95.Lovly C.M., Gupta A., Lipson D., Otto G., Brennan T., Chung C.T., et al. Inflammatory myofibroblastic tumors harbor multiple potentially actionable kinase fusions. Cancer Discov 2014; 4 (8): 889–95.23. Antonescu C.R., Suurmeijer A.J., Zhang L., Sung Y.S., Jungbluth A.A, Travis W.D., et al. Molecular characterization of inflammatory myofibroblastic tumors with frequent ALK and ROS1 gene fusions and rare novel RET rearrangement. Am J Surg Pathol 2015; 39 (7): 957–67.Antonescu C.R., Suurmeijer A.J., Zhang L., Sung Y.S., Jungbluth A.A, Travis W.D., et al. Molecular characterization of inflammatory myofibroblastic tumors with frequent ALK and ROS1 gene fusions and rare novel RET rearrangement. Am J Surg Pathol 2015; 39 (7): 957–67.24. Alassiri A., Ali R.H., Shen Y., Lum A., Strahlendorf C., Deyell R., et. al. ETV6-NTRK3 Is Expressed in a Subset of ALK-Negative Inflammatory Myofibroblastic Tumors. Am J Surg Pathol 2016; 40 (8): 1051–61.Alassiri A., Ali R.H., Shen Y., Lum A., Strahlendorf C., Deyell R., et. al. ETV6-NTRK3 Is Expressed in a Subset of ALK-Negative Inflammatory Myofibroblastic Tumors. Am J Surg Pathol 2016; 40 (8): 1051–61.25. Lawrence B., Perez-Atayde A., Hibbard M.K., Rubin B.P., Dal Cin P., Pinkus J.L., et al. TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors. Am J Pathol 2000; 157 (2): 377–84.Lawrence B., Perez-Atayde A., Hibbard M.K., Rubin B.P., Dal Cin P., Pinkus J.L., et al. TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors. Am J Pathol 2000; 157 (2): 377–84.26. Zavaglia C., Barberis M., Gelosa F., Cimino G., Minola E., Mondazzi L., et al. Inflammatory pseudotumour of the liver with malignant transformation. Report of two cases. Ital J Gastroenterol 1996; 28: 152–9.Zavaglia C., Barberis M., Gelosa F., Cimino G., Minola E., Mondazzi L., et al. Inflammatory pseudotumour of the liver with malignant transformation. Report of two cases. Ital J Gastroenterol 1996; 28: 152–9.27. Pecorella I., Ciardi A., Memeo L., Trombetta G., Quarto А., Simone P., et al. Inflammatory pseudotumour of the liver-evidence for malignant transformation. Pathol Res Pract 1999; 195: 115–20.Pecorella I., Ciardi A., Memeo L., Trombetta G., Quarto А., Simone P., et al. Inflammatory pseudotumour of the liver-evidence for malignant transformation. Pathol Res Pract 1999; 195: 115–20.28. Yamaguchi J., Sakamoto Y., Sano T., Shimada K., Kosuge T. Spontaneous regression of inflammatory pseudotumor of the liver: report of three cases. Surg Today 37: 525–9.Yamaguchi J., Sakamoto Y., Sano T., Shimada K., Kosuge T. Spontaneous regression of inflammatory pseudotumor of the liver: report of three cases. Surg Today 37: 525–9.29. Tang L., Lai E.C., Cong W.M., Li A.J., Fu S.Y., Pan Z.Y., et al. Inflammatory myofibroblastic tumor of the liver: a cohort study. World J Surg 2010; 34 (2): 309–13.Tang L., Lai E.C., Cong W.M., Li A.J., Fu S.Y., Pan Z.Y., et al. Inflammatory myofibroblastic tumor of the liver: a cohort study. World J Surg 2010; 34 (2): 309–13.30. Mosse Y.P., Lim M.S., Voss S.D., Wilner K., Ruffner K., Laliberte J., et al. Safety and activity of crizotinib for paediatric patients with refractory solid tumours or anaplastic large-cell lymphoma: a Children's Oncology Group phase 1 consortium study. Lancet Oncology 2013; 14 (6): 472–80.Mosse Y.P., Lim M.S., Voss S.D., Wilner K., Ruffner K., Laliberte J., et al. Safety and activity of crizotinib for paediatric patients with refractory solid tumours or anaplastic large-cell lymphoma: a Children's Oncology Group phase 1 consortium study. Lancet Oncology 2013; 14 (6): 472–80.31. Mosse Y.P., Voss S.D., Lim M.S., Rolland D., Minard C.G., Fox E., et al. Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. J Clin Oncol 2017; 35 (28): 3215–21.Mosse Y.P., Voss S.D., Lim M.S., Rolland D., Minard C.G., Fox E., et al. Targeting ALK With Crizotinib in Pediatric Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumor: A Children's Oncology Group Study. J Clin Oncol 2017; 35 (28): 3215–21.