Yang Qinglin,Guo Weihong,Zhang Weiping,et al.Intestinal infantile fibrosarcoma: a case report and literature review[J].Journal of Clinical Pediatric Surgery,,19():1107-1112.[doi:10.3969/j.issn.1671-6353.2020.12.009]
Intestinal infantile fibrosarcoma: a case report and literature review
- Keywords:
- Colonic Neoplasms; Fibrosarcoma/SU; Fibrosarcoma/ET; Treatment Outcome
- CLC:
- R735.3+5;R730.262
- Abstract:
- Objective To explore the clinical manifestations,pathological features,treatments and outcomes of intestinal infantile fibrosarcoma (IFS).Methods In April 2019,clinical data were retrospectively analyzed for one case of intestinal infantile fibrosarcoma.And the relevant literature reports were retrieved through the databases of PubMed and Wanfang.Results One 43-day-old boy was hospitalized for 3-week hematochezia.Preoperative contrast enhanced computed tomography (CT) hinted at a heterogeneously intensified mass in right lower abdomen with partial extension into enteric cavity.Laparoscopic exploration was performed and lesion completely resected.Pathological diagnosis confirmed IFS.During a follow-up period of 6 months,there was no sign of relapse or transformation on abdomen ultrasound.A total of 19 IFS cases were reported.Most cases (16/20,80.0%) occurred within the first week after birth.There were intestinal enterobrosis (9/20,45.0%) and intestinal obstruction (5/20,25.0%).Surgery was effective while adjuvant chemotherapy was required for relapse.A definite diagnosis was dependent upon histology,immunohistochemistry and molecular diagnostics.Ectopia of ETV6-NTRK3 was detected in 46.1%(6/13) of intestinal IFS.The overall prognosis was decent.Conclusion Intestional IFS is a rare soft-tissue sarcoma usually occurring during early neonatal period.Proper surgical approach should be employed for complete lesion resection.And the overall prognosis is excellent.
References:
1 Al-Salem AH.Congenital-infantile fibrosarcoma masquerading as sacrococcygeal teratoma[J].J Pediatr Surg,2011,46(11):2177-2180.DOI:10.1016/j.jpedsurg.2011.08.011.
2 van Niekerk ML,Nel WA,Slavik T.Infantile fibrosarcoma of the ileum presenting with congenital bowel obstruction[J].J Pediatr Surg,2010,45(2):461-462.DOI:10.1016/j.jpedsurg.2009.10.092.
3 Islam S,Soldes OS,Ruiz R,et al.Primary colonic congenital infantile fibrosarcoma presenting as meconium peritonitis[J].Pediatr Surg Int,2008,24(5):621-623.DOI:10.1007/s00383-008-2113-0.
4 Buccoliero AM,Castiglione F,Rossi Degl’Innocenti D,et al.Congenital/Infantile fibrosarcoma of the colon:morphologic,immunohistochemical,molecular,and ultrastructural features of a relatively rare tumor in an extraordinary localization[J].J Pediatr Hematol Oncol,2008,30(10):723-727.DOI:10.1097/MPH.0b013e31817541df.
5 Parmar V,Peters RT,Cheesman E,et al.Congenital infantile fibrosarcoma of the colon:a case series and literature review[J].Pediatr Surg Int,2014,30(10):1079-1085.DOI:10.1007/s00383-014-3589-4.
6 Kim HY,Cho YH,Byun SY,et al.A case of congenital infantile fibrosarcoma of sigmoid colon manifesting as pneumoperitoneum in a newborn[J].J Korean Med Sci,2013,28(1):160-163.DOI:10.3346/jkms.2013.28.1.160.
7 Zeytun H,Okur MH,Basuguy E,et al.Congenital-infantile fibrosarcoma of the ileocecal region:the first case presentation[J].Pediatr Surg Int,2016,32(1):97-99.DOI:10.1007/s00383-015-3802-0.
8 Obayashi J,Koizumi H,Hoshikawa M,et al.A case of congenital infantile fibrosarcoma of the bowel presenting as a neonatal intussusception[J].Pathol Int,2017,67(12):644-648.DOI:10.1111/pin.12603.
9 Scirè G,Mantovani A,Zampieri N,et al.Transumbilical laparoscopic treatment of Congenital Infantile Fibrosarcoma of the Ileum[J].Pediatr Med Chir,2014,36(4):93.DOI:10.4081/pmc.2014.93.
10 Rizkalla H,Wildgrove H,Quinn F,et al.Congenital fibrosarcoma of the ileum:case report with molecular confirmation and literature review[J].Fetal Pediatr Pathol,2011,30(3):156-160.DOI:10.3109/15513815.2010.547554.
11 Bruno C,Caliari G,Zampieri N,et al.Congenital fibrosarcoma of the bowel:sonographic description of a rare case of neonatal intestinal obstruction[J].J Clin Ultrasound,2014,42(6):363-366.DOI:10.1002/jcu.22117.
12 See WSQ,Cheuk DKL,To KF,et al.Congenital intestinal fibrosarcoma with rapid recurrence requiring adjuvant chemotherapy[J].Pediatr Int,2017,59(6):733-736.DOI:10.1111/ped.13252.
13 Berrebi D,Fournet JC,Boman F,et al.Intestinal congenital/infantile fibrosarcoma:a new clinico-pathological entity?[J].Pediatr Surg Int,2015,31(4):375-379.DOI:10.1007/s00383-015-3670-7.
14 Shima Y,Ikegami E,Takechi N,et al.Congenital fibrosarcoma of the jejunum in a premature infant with meconium peritonitis[J].Eur J Pediatr Surg,2003,13(2):134-136.DOI:10.1055/s-2003-39560.
15 Kaiser M,Liegl-Atzwanger B,Nagy E,et al.Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn[J].Case Rep Pediatr,2017,2017:2969473.DOI:10.1155/2017/2969473.
16 Shearburn EW,Teja K,Botero LM,et al.Pancreaticoduodenectomy in the treatment of congenital fibrosarcoma of the duodenum[J].J Pediatr Surg,1975,10(5):801-806.DOI:10.1016/0022-3468(75)90388-7.
17 Stout AP.Fibrosarcoma in infants and children[J].Cancer,1962,15(5):1028-1040.DOI:10.1002/1097-0142(196209/10)15:53.0.CO;2-X.
18 Steelman C,Katzenstein H,Parham D,et al.Unusual presentation of congenital infantile fibrosarcoma in seven infants with molecular-genetic analysis[J].Fetal Pediatr Pathol,2011,30(5):329-337.DOI:10.3109/15513815.2011.587497.
19 Orbach D,Rey A,Cecchetto G,et al.Infantile fibrosarcoma:management based on the European experience[J].J Clin Oncol,2010,28(2):318-323.DOI:10.1200/jco.2009.21.9972.
20 Ortega-Garcia JA,Soldin OP,Lopez-Hernandez FA,et al.Congenital fibrosarcoma and history of prenatal exposure to petroleum derivatives[J].Pediatrics,2012,130(4):e1019-e1025.DOI:10.1542/peds.2011-1307.
21 Romano C,Oliva S,Martellossi S,et al.Pediatric gastrointestinal bleeding:Perspectives from the Italian Society of Pediatric Gastroenterology[J].World J Gastroenterol,2017,23(8):1328-1337.DOI:10.3748/wjg.v23.i8.1328.
22 Braun P,Fernández-Montes JG,Calatayud AV.Congenital infantile fibrosarcoma:Report of four cases and review of the literature[J].Eur J Radiol Extra,2007,61(1):33-39.
23 Lee MJ,Cairns RA,Munk PL,et al.Congenital-infantile fibrosarcoma:magnetic resonance imaging findings[J].Can Assoc Radiol J,1996,47(2):121-125.
24 Knezevich SR,McFadden DE,Tao W,et al.A novel ETV6-NTRK3 gene fusion in congenital fibrosarcoma[J].Nat Genet,1998,18(2):184-187.DOI:10.1038/ng0298-184.
25 Alassiri AH,Ali RH,Shen Y,et al.ETV6-NTRK3 Is Expressed in a Subset of ALK-Negative Inflammatory Myofibroblastic Tumors[J].Am J Surg Pathol,2016,40(8):1051-1061.DOI:10.1097/pas.0000000000000677.
26 Hayes-Jordan A.Recent advances in non-rhabdomyosarcoma soft-tissue sarcomas[J].Semin Pediatr Surg,2012,21(1):61-67.DOI:10.1053/j.sempedsurg.2011.10.006.
27 Thebaud E,Mezel A,Leroy X,et al.Fibrosarcoma in children and adolescents:different entities for the same name[J].Bull Cancer,2012,99(6):715-722.DOI:10.1684/bdc.2012.1597.
28 Bourgeois JM,Knezevich SR,Mathers JA,et al.Molecular detection of the ETV6-NTRK3 gene fusion differentiates congenital fibrosarcoma from other childhood spindle cell tumors[J].Am J Surg Pathol,2000,24(7):937-946.DOI:10.1097/00000478-200007000-00005.
Memo
收稿日期:2019-10-08。
基金项目:北京市医院管理局"扬帆计划"（编号：ZYLX201709）：北京市医院管理局"登峰"人才培养计划（编号：DFL20151102）
通讯作者:郭卫红,Email:guo_weihong@163.com;张潍平,Email:zhangwpp@163.com