ISPUB.com / IJS/31/2/18828
  • Author/Editor Login
  • Registration
  • Facebook
  • Google Plus

ISPUB.com

Internet
Scientific
Publications

  • Home
  • Journals
  • Latest Articles
  • Disclaimers
  • Article Submissions
  • Contact
  • Help
  • The Internet Journal of Surgery
  • Volume 31
  • Number 2

Case Study

Inflammatory Fibroid Polyp Of The Jejunum Presenting As Intussusception: A Case Report And Review Of The Literature.

A Klimis, C Chloptsios, F Chrysovergidιs, A Dimitriadi, T Goulopoulos, A Androulaki

Keywords

diagnosis, immunohistochemistry, inflammatory fibroid polyp, intussusception

Citation

A Klimis, C Chloptsios, F Chrysovergidιs, A Dimitriadi, T Goulopoulos, A Androulaki. Inflammatory Fibroid Polyp Of The Jejunum Presenting As Intussusception: A Case Report And Review Of The Literature.. The Internet Journal of Surgery. 2014 Volume 31 Number 2.

Abstract

Adult  intussuseptions   are  rare entities ,  almost  always  associated  with a  demonstrable  lead  point.  There  has  been  a  notable  association   between  adult  intussusceptions  and   inflammatory  fibroid  polyps(IFPs).  These  are  benign  mesenchymal  tumor  masses,  composed  of  a  spindle  cell  proliferation,  accompanied  by  an  inflammatory  reaction  and  variably  prominent  blood  vessels. The  histogenesis  of  these  tumors  remain   controversial,  but  they  are  believed  to  occur  in  response  to  local  noxious  stimuli.

 

Introduction

Adult  intussusception  is  a  rare  entity  and  causes  only  1%  of  all  bowel  obstruction1.  In  the  small  bowel,  benign  lesions  account for   ̴70%,  with  malignant  lesions  responsible  for  the  remainder2.  Inflammatory  fibroid  polyp (IFP)  is  a  type  of  non-neoplastic  inflammatory  pseudotumor  that  occurs  mainly  in  the  gastric  antrum  or  in  the  ileum,  however,  they  are  considered  to  be  very  rare  in  the  jejunum  and  duodenum3.  IFP  has  been  reported  to  cause  gastrointestinal  bleeding  and  simple  mechanical  obstruction,  but  rarely  cause  intussusception. 

Here,  we  present  a  rare  case  of  Inflammatory  fibroid  polyp (IFP) confined   to  the  jejunum  presenting  as  intestinal  intussusception  in  an  adult  female  patient.

Case presentation

A  63-year-old  female  patient  presented  to  the  hospital  after  experiencing  colicky  abdominal  pain,  nausea  and  vomiting  that  had  begun  36  hours  earlier.  Similar  attacks  of  pain  she  had  experienced  time  after  time  over  the  past  four  months  which  had  resolved  spontaneously.  There was no history of prior abdominal operations.  Between the episodes the patient reported normal bowel habits.  Gastroscopy  which  had  been  undertaken  previously  was  found  to  be  normal.  Significant  abdominal  findings  were confined  to  the  abdomen  that  was  distended  with  mild  peri-umbilical  tenderness  and  hyperactive  bowel  sounds. Digital  rectal  examination  did not  reveal  the  presence  of  feces, mucus  or  blood.  An  upright  plain  abdominal  film  revealed  multiple  loops  of  dilated  small  bowel  in  the  left  upper  quadrant  of  the  abdomen(Fig.1).

Figure 1
Plain abdominal radiograph showing distended loops of small bowel.

Abdominal ultrasonography failed to reveal the intussusception.  Computed  tomography (CT)   was  performed  and  showed  a  typical “ sausage”-shaped  soft  tissue  mass  suggesting  small  bowel  intussusception.                                      

Vitals were  stable:  pulse  78/min,  blood  140/80mm Hg,  respiratory  rate  16/min  and  temperature  normal.

Laboratory  investigations  showed :  Urea 22mg/dl(normal: 15-42 mg/dl,  creatinine 0.7mg/dl(normal:0.6-1.3mg/dl),  SGOT(ALT) 23u/l(normal:5-37u/l),  SGPT(AST)52(normal  12-70u/l), Sodium  137mmol/l,  potassium  4.4 mmol/l,  haemoglobin  10.8g/dl,  total  leucocyte  count  10.8x103/mm3(normal: 4-11.000/l).

The  clinical  symptoms  and  CT  images  were  consistent  with  small  bowel  obstruction.  Thus urgent   laparotomy with midline incision was performed.  On  exploration,  an  intussusception  at  the  jejunum   was  found  40 cm  distal  to  the  duodeno- jejunal  flexure(Fig.2),  with  an  intraluminal  obstructive  mass  as  a  lead  point (Fig.3).  The small bowel was dilated proximally.  The intussuscepted   segment was resected and end-to-end anastomosis was carried out.  The resected specimen was sent  for  histopathology.

Figure 2
Jejuno-jejunal intussusception.

Figure 3
IFP after section of the S-bowel.

Gross  pathologic  examination  of  the  resected  segment  of  the  jejunum  revealed  a  25x20x15mm  sessile  polypoid  mass,  located  perpendicular  to  the  plane  of  mucosa. Sectioning  of  the  lesion  revealed  a  homogeneous,  hemorrhagic, tan  with  glistening  surface.  Sixteen lymph nodes were present in the attached mesentery.

Light  microscopic  examination  showed  portion  of  small  bowel  containing  a  submucosal  mass(Fig. 4)  with  extension  into  the  muscularis  propria.  The overlying mucosa was hemorrhagic with patchy area of ulceration.  The  mass  was  composed  of  a  proliferation    of  spindle-shaped  and  stellate  fibroblasts  embedded  within  loose, oedematous  stroma,  which  contained  numerous  blood  vessels  and  an  abundant  inflammatory  infiltrate  comprising  eosinophils,  plasma  cells  and  lymphocytes.  All resected mesenteric lymph nodes showed reactive changes only.

Figure 4
Histological appearance of IFP(H-E stain, original maginification x100).

The  primary  diagnoses  were  doubted  between  gastro-intestinal  stromal  tumor(GIST)  and  inflammatory  fibroid  polyp.  Immunohistochemical study was done on the specimen.  The  spindle  cells  were  negative  for  CD117(C-kit), smooth  muscle  actin, desmin  and  S-100.  Vimentin, fascin and cyclin D1 were strongly and diffusely  positive.  CD34  highlighted  the  vascular  supply, although  some  of  the  spindle  cells  located  between  vessels  were  positive (Fig. 5).

Figure 5
Positive Immunostaining of spindle cells and vascular supply for CD 34(Immuno- histochemical stain, original maginificationx100).

The differential diagnosis  also  included  neural  and  smooth  muscle  tumors.  Immunohistochemistry  performed  on  this  lesion  was  negative  for  S100  and  desmin,  excluding  a  neurogenic  and  smooth  muscle  tumors.  A  final  diagnosis  of  an  inflammatory  fibroid  polyp  of  the  jejunum   was  made.

The  patient  had  an  uneventful  post-operative  recovery,  and  was  discharged  7  days  after  surgery . Six  months  after  the   surgery  she  remained  well  and  symptom  free.

Discussion

Intussusception  is  a  rare  condition  in  adults,  but  can  be  the  first  manifestation  of  a  tumor.  It  represents  only  1% of  all cases  of    intestinal  obstructions  in  adults.1,2,3  The   jejunum   is  the  least  likely  location  for  intussusceptions  to  occur  because  the  leading  pathology  is  rare   at  this  location  of  the  gastrointestinal  tract4,5,6,7.  A  jejuno-jejunal  intussusception  resulting  from  an  IFP  was  first  described  by  Winkler et al.  in 1986.4  Since  then,  to  the  best  of  our  knowledge,  about  17  cases  of  jejunal  IFPs,  producing  intussusception  ,  have  been  described 2,4,5,6,7,8,9,10,11,12,13.  IFPs   can  affect    any  portion  of  the GI   tract,  but  they  are  most  common  in  the  gastric  antrum  (70%)  and  the  terminal  ileum(20%),  but  rarely  in  the  jejunum.  Akbulut  et  al.13 reported  that  among  85  patients  with  IFPs  of  the  small  bowel  ileal  intussusception  was  found  in  63  patients  and  17 had  jejunal,  three  had  colonic  and  two  had  ileojejunal  intussusception.  IFPs  affect  both  sexes  and  all  ages,  with  peak  incidence  in  the  fifth  and  sixth  decades.  This  is  likely  because  the  lesions  produce  local  symptoms  that  are  size  dependent.

The  exact   aetiology  and  pathogenesis  of   IFPs  has  not  yet  been  clarified.  Most  regard  IFPs  as  a  reactive  or  inflammatory  process  in  response  to  local  noxious  stimuli,  with  incomplete  differentiation  of  myofibroblasts  and  primitive  submucosal  stromal  cells14,15.  An  allergic  aetiology  has  been  proposed  due  to  the association  with  eosinophils10.  Immunohistochemical   study  with  positive  expression  of  spindle  shaped  cells with  CD34  favor  the  hypothesis  that  IFPs  represent  a  reactive  proliferation  of  primitive  perivascular/vascular  cells14.

Macroscopically,  IFPs  tend  to  appear  as  solitary  polyps,  that  are  well-circumscribed.  Larger  lesions  may  be  pedunculated,   and  the  mucosal  surface  is  often  ulcerated  on  its  apex.  Size  up  to  18  cm  in  the  largest  dimension  has  been  reported9. Histologically,  the  epicenter  of  the  tumor  is  usually  in  the  submucosa,  but  it  can  infiltrate  into  the  overlying   mucosa  and  the  muscularis  propria  or  serosa 6,10,11.  The  microscopic  picture  entails  a  loosely  arranged  fibroconnective  tissue  with  a  prominent  thin-walled  vascular  component  and  an  inflammatory  response  dominated  by  eosinophils.  Cellular  atypia  and  significant   numbers  of  mitoses   are  not  seen.

 The  GIST  is  an  important  consideration  in  the  histological  differential   diagnosis  of  IFPs.  In  morphological  ambiguous  cases,  immunohistochemistry  is  helpful  to  make  a  distinction  between  the  two  entities.  Both  tumors  are  positive  for  CD34  and  Vimentin,  but  GISTs  are  positive  for  CD117(c-kit),while  IFPs  are  not5,10,15.

Negative  staining  to  S-100 protein  distinguishes  an  IFP  from   a  neurogenic  tumors  which  are  positive   for  this  marker.  Some cases  of  IFPs  fail  to  show  positive  staining  for  CD34, and  this  may  be  related  to  the  evolution  of  the  tumor14,16.

Many  IFPs  are  asymptomatic , and  are  typically  identified   during  endoscopy  or  laparotomy.  When  symptomatic,  the  clinical  picture  depends  on  the  size  of  the   tumor   and  on  the  location.  Most  of  the  jejunal  IFPs  have  been  found  to  cause  small  bowel  obstruction  as  a  result  of  intussusception3,4,,5,6,7,9.  If  the  overlying  mucosa  ulcerates  then  GI  bleeding,  anaemia  and  alteration  of  bowel  habits  were  occasionally.  Small  bowel  lesions  are  not  usually  diagnosed  pre-operatively  because  they  present  with  vague  symptoms  of  bowel  obstruction  due  to  intussusception.  Laboratory   investigations  and  plain  radiographs  are  not  helpful  in  making  the  diagnosis  as  they   will  demonstrate  nonspecific  findings  that  are  more  in   keeping  with  bowel  obstruction.   Abdominal  CT scanning,  is  currently  the  diagnostic  instrument  of  choise17,allowing  in  some  series   a  correct   preoperative  diagnosis   in up to 80% of the  cases1. The CT  appearance  is  complex.  According  to  the  cut  axis, the  intussusception  appears  as  a  “sausage”  or  a “ target”  mass.     There  are  no  unique  identifying  features  on  imaging  that  can  distinguish  the  nature  of  the  lead  point.  The  absence  of  pathognomonic  clinical  or  radiological  features  makes  operative  resection  and  microscopic  examination  of  the  resected  lesion  mandatory.

Operative  resection  of  the  involved  intestine  with  free  margins  is  sufficient  treatment.  In  conclusion,  IFP  is  a  rare  benign  cause  of  enteric  intussusception  in  adults.  This  diagnosis  should  be  considered  by  Surgeons  when  intussusceptions  are  encountered  in  adults.

References

1. Azar T, Berger DL. Adult intussusception. Ann. Surg 1997;226:134-138.
2. Yakan S, Caliscan C, Makay O. et al. World J Gastroenterol 2009;15:1985-89
3. Toso C, Erne M, Lenzlinger Ph.M et al. Intussusception as a cause of bowel obstruction in adults. Swiss Med.WKLY 2005;135:87-90.
4. Winkler H, Zelikovski A, Gutman H. et al. Inflammatory fibroid polyp of the jejunum causing intussusception. Am J Gastroenterol 1986;81:598-601.
5. Jacobs TM & Lambrianides AL. Inflammatory fibroid polyp presenting as intussusception. J Surg Case rep 2013 (2).
6. Rehman Sh, Gamie Z & Wilson TR. Inflammatory fibroid polyp (Vanek’s tumor), an unusual large polyp of the jejunum: a case report. Cases J 2009;2:7152
7. Cawich S, Gibson T, Mitchel D. et al. Adult intussusception from an inflammatory fibroid polyp: A case report and review of the literature. Inter Jour Path 2007;7:1.
8. Kim JS, Kwon SY, Byun KS et al. Kor Jour Inter Med 1994;9:51-54.
9. Neishaboori H, Maleki I, Emadian O. Jejunal intussusception caused by a huge Vanek’s tumor : a case report. Gastroenterol & Hepatol 2013;6(4):210-213.
10. Patel A, James A, Thomas D.M. Inflammatory fibroid polyp causing jejuno-jejunal intussusception. Gastroenterol Today 2008;18:87-91.
11. Vijayaraghavan R, Sujatha Y, Santosh KV et al. Inflammatory fibroid polyp of jejunum causing jejuno-jejunal intussusception. Indian J Gastroenterol 2004;23:190-2.
12. El Hajji II, Sharara AL. Jejuno-jejunal intussusception caused by an inflammatory fibroid polyp: Case report and review of the literature. J Med Liban 2007;55:108-111.
13. Αkbulut S. Intussusception due to inflammatory fibroid polyp: A case report and comprehensive literature review. World J Gastroenterol 2012;58:5745-5752.
14. Wille P & Borchard F. Fibroid polyps of intestinal tract are inflammatory reactive proliferations of CD34-positive perivascular cells. Histopathol 1998;32:498-502.
15. Pantanowits L, Antonioli D, Pinkus GS et al. Inflammatory fibroid polyps of the gastrointestinal tract. Evidence for a dentritic cell origin. Am J Surg Pathol 2004;28:107-114.
16. Ozolek JA, Sasatorni E, Swalski PA, et al. Inflammatory fibroid polyp of the GI tract: clinical, pathologic, and characteristics. Appl. Immunohistochem Mol Morphol 2004;12:59-66.
17. Gayer G, Zissin R, Apter S et al. Adult intussusception- a CT diagnosis. Brit J Rad 2002;75:185-190.

Author Information

A Klimis
Department of Pathology, General Prefectural Hospital
Elpis, Athens

Ch Chloptsios
Department of Surgery, General Prefectural Hospital
Elpis, Athens

F Chrysovergidιs
Department of Surgery, General Prefectural Hospital
Elpis, Athens

Ai Dimitriadi
Department of Surgery, General Prefectural Hospital
Elpis, Athens

Th Goulopoulos
Department of Surgery, General Prefectural Hospital
Elpis, Athens

A Androulaki
Department of Pathology, General Prefectural Hospital
Elpis, Athens

Download PDF

Your free access to ISPUB is funded by the following advertisements:

 

BACK TO TOP
  • Facebook
  • Google Plus

© 2013 Internet Scientific Publications, LLC. All rights reserved.    UBM Medica Network Privacy Policy