Онкопедиатрия. 2017; 4: 68-73
КОМПЛЕКСНАЯ ЛУЧЕВАЯ ДИАГНОСТИКА НЕДИФФЕРЕНЦИРОВАННОЙ ЭМБРИОНАЛЬНОЙ САРКОМЫ ПЕЧЕНИ У ДЕТЕЙ: СОБСТВЕННЫЙ ОПЫТ И ОБЗОР ЛИТЕРАТУРЫ
https://doi.org/10.15690/onco.v4i1.1686Аннотация
Обоснование. Недифференцированная эмбриональная саркома печени — редкая злокачественная опухоль мезенхимального происхождения, встречающаяся преимущественно в детском возрасте. Отсутствие специфических клинических, лучевых, лабораторных признаков приводит к поздней диагностике заболевания. Цель исследования: описание лучевой картины и определение характерных особенностей этой опухоли у детей по результатам анализа собственных наблюдений (n=6) и данных литературы. Методы. С 2008 по 2016 г. в НИИ детской онкологии были обследованы и получали лечение 6 пациентов с диагнозом недифференцированной эмбриональной саркомы печени (5 девочек и 1 мальчик) в возрасте от 4 мес до 11 лет (средний возраст составил 6,5 года). Диагноз подтвержден морфологически и иммуногистохимически. Результаты. Были установлены общие признаки недифференцированной эмбриональной саркомы, встречающиеся при всех лучевых методах исследования: образование имело большие размеры (в среднем 11,8 см), бугристые четкие контуры (6/6), было представлено солитарным узлом (6/6), не инфильтрировало сосуды печени (6/6). Не отмечалось нарушения капсулы печени и выхода опухоли за пределы органа (5/6). Также были выявлены отличия в данных, полученных при сонографии, магнитно-резонансной (МРТ) и компьютерной томографии (КТ). Так, при ультразвуковом исследовании в 5 случаях опухоль имела преимущественно солидное строение с наличием анэхогенных (кистозных) включений. При МРТ и КТ основная часть опухоли имела кистоподобную структуру с высоким содержанием жидкости. Заключение. Результаты данных комплексной лучевой диагностики позволяют заподозрить диагноз недифференцированной саркомы печени у детей благодаря многообразной картине структуры опухоли. Во всех случаях, подозрительных в отношении недифференцированной эмбриональной саркомы печени, морфологическое подтверждение до выработки плана лечения считаем обязательным.
Список литературы
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4. Li Y, Cai Q, Jia N, et al. Pre-operatively misdiagnosed undifferentiated embryonal sarcoma of the liver: analysis of 16 cases. Ann Transl Med. 2015;3(22):353. doi: 10.3978/j.issn.2305-5839.2015.11.20.
5. Gao J, Fei L, Li S, et al. Undifferentiated embryonal sarcoma of the liver in a child: A case report and review of the literature. Oncol Lett. 2013;5(3):739–742. doi: 10.3892/ol.2012.1087.
6. Lyu S, Shi X, Liang Y, et al. Diagnosis and therapy of primary undifferentiated embryonal sarcoma of the liver. Chin Med J (Engl). 2014;127(8):1585–1587.
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11. Arkh Patol. 2007;69(1):40–47. (In Russ).]
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13. Walther A, Geller J, Coots A, et al. Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma. Liver Transpl. 2014;20(2):191– 199. doi: 10.1002/lt.23773.
14. Buetow PC, Buck JL, Pantongrag-Brown L, et al. Undifferentiated (embryonal) sarcoma of the liver: pathologic basis of imaging findings in 28 cases.
15. Radiology. 1997;203(3):779–783. doi: 10.1148/radiology.203.3.9169704.
16. Sodhi KS, Bekhitt E, Rickert C. Paradoxical hepatic tumor: undifferentiated embryonal sarcoma of the liver. Indian J Radiol Imaging. 2010;20(1):69–71. doi: 10.4103/0971-3026.59760.
17. Helmberger TK, Ros PR, Mergo PJ, et al. Pediatric liver neoplasms: a radiologic-pathologic correlation.
18. Eur Radiol. 1999;9(7):1339–1347. doi: 10.1007/ s003300050845.
19. Putra J, Ornvold K. Undifferentiated embryonal sarcoma of the liver: а concise review. Arch Pathol Lab Med. 2015;139(2):269–273. doi: 10.5858/arpa.2013-0463-RS.
20. Jha P, Chawla SC, Tavri S, et al. Pediatric liver tumors — a pictorial review. Eur Radiol. 2009;19(1):209–219. doi: 10.1007/s00330-008-1106-7.
21. Das CJ, Dhingra S, Gupta AK, et al. Imaging of pediatric liver tumors with pathological correlation. Clin Radiol. 2009;64(10):1015–1025. doi: 10.1016/j. crad.2009.04.014.
Oncopediatrics. 2017; 4: 68-73
THE COMPLEX IMAGING DIAGNOSIS OF UNDIFFERENTIATED EMBRYONAL SARCOMA OF THE LIVER IN CHILDREN: THE OWN EXPERIENCE AND LITERATURE REVIEW
https://doi.org/10.15690/onco.v4i1.1686Abstract
Background. Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mes-enchymal tumor that occurs predominantly in children. Diagnosis delay often happens due to the deficiency of clinical symptoms, radiological findings, and serological markers. Objektive was to describe the imaging features of the undifferentiated embryonal sarcoma of the liver in children. Materials. Six children with UESL (5 girls and 1 boy) were observed and treated at the Research Center of Pediatric Oncology between 2008 and 2016. The median age was 6.5 years (from 4 months to 11 years). The diagnosis was confirmed by histological and immunohistochemical analysis. Abdominal ultrasonography (US), abdominal computed tomography (CT), and magnetic resonance imaging (MRI) were performed. Results. The enormous solitary well circumscribed mass with irregular contours without invasion of liver vessels were revealed by US, CT, and MRI. In 5 cases, the tumors didn’t disturbed the fibrocapsule. Five tumors showed solid appearance with some little anechoic areas in abdominal US, while at CT and MRI UESL had predominantly cyst-like structure due to the high water content of the prominent myxoid stroma. After intravenous contrast administration predominantly peripheral enchancement and solid parts of the mass was determined at CT and MRI in all cases. Conclusion. Imaging data reveal discrepancy between US and CT/MRI appearance of UESL that can help to suspect this tumor. It is necessary to perform the morphological investigation when UESL is suspected.
References
1. Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver. Cancer. 1978;42(1):336–348. doi: 10.1002/1097-0142(197807)42:13.0.co;2-v.
2. Zhang H, Lei L, Zuppan CW, Raza AS. Undifferentiated embryonal sarcoma of the liver with an unusual presentation: case report and review of the literature. J Gastrointest Oncol. 2016;7(Suppl 1):S100–S106. doi: 10.3978/j.issn.2078-6891.2015.020.
3. Lack EE, Schloo BL, Azumi N, et al. Undifferentiated (embryonal) sarcoma of the liver: clinical and pathologic study of 16 cases with emphasis on immunohistochemi-cal features. Am J Surg Pathol. 1991;15(1):1–16. doi: 10.1097/00000478-199101000-00001.
4. Li Y, Cai Q, Jia N, et al. Pre-operatively misdiagnosed undifferentiated embryonal sarcoma of the liver: analysis of 16 cases. Ann Transl Med. 2015;3(22):353. doi: 10.3978/j.issn.2305-5839.2015.11.20.
5. Gao J, Fei L, Li S, et al. Undifferentiated embryonal sarcoma of the liver in a child: A case report and review of the literature. Oncol Lett. 2013;5(3):739–742. doi: 10.3892/ol.2012.1087.
6. Lyu S, Shi X, Liang Y, et al. Diagnosis and therapy of primary undifferentiated embryonal sarcoma of the liver. Chin Med J (Engl). 2014;127(8):1585–1587.
7. Chung EM, Lattin GE Jr, Cube R, et al. From the archives of the AFIP: Pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors. Radiographics. 2011;31(2):483–507. doi: 10.1148/rg.312105201.
8. Harman M, Nart D, Acar T, Elmas N. Primary mesenchymal liver tumors: radiological spectrum, differential diagnosis, and pathologic correlation. Abdom Imaging. 2015;40(5):1316–1330. doi: 10.1007/s00261-014-0268-9.
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11. Arkh Patol. 2007;69(1):40–47. (In Russ).]
12. Bisogno G, Pilz T, Perilongo G, et al. Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer. 2002;94(1):252–257. doi: 10.1002/cncr.10191.
13. Walther A, Geller J, Coots A, et al. Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma. Liver Transpl. 2014;20(2):191– 199. doi: 10.1002/lt.23773.
14. Buetow PC, Buck JL, Pantongrag-Brown L, et al. Undifferentiated (embryonal) sarcoma of the liver: pathologic basis of imaging findings in 28 cases.
15. Radiology. 1997;203(3):779–783. doi: 10.1148/radiology.203.3.9169704.
16. Sodhi KS, Bekhitt E, Rickert C. Paradoxical hepatic tumor: undifferentiated embryonal sarcoma of the liver. Indian J Radiol Imaging. 2010;20(1):69–71. doi: 10.4103/0971-3026.59760.
17. Helmberger TK, Ros PR, Mergo PJ, et al. Pediatric liver neoplasms: a radiologic-pathologic correlation.
18. Eur Radiol. 1999;9(7):1339–1347. doi: 10.1007/ s003300050845.
19. Putra J, Ornvold K. Undifferentiated embryonal sarcoma of the liver: a concise review. Arch Pathol Lab Med. 2015;139(2):269–273. doi: 10.5858/arpa.2013-0463-RS.
20. Jha P, Chawla SC, Tavri S, et al. Pediatric liver tumors — a pictorial review. Eur Radiol. 2009;19(1):209–219. doi: 10.1007/s00330-008-1106-7.
21. Das CJ, Dhingra S, Gupta AK, et al. Imaging of pediatric liver tumors with pathological correlation. Clin Radiol. 2009;64(10):1015–1025. doi: 10.1016/j. crad.2009.04.014.
