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International Journal of Research in Medical Sciences | July 2016 | Vol 4 | Issue 7 Page 2521
International Journal of Research in Medical Sciences
Vagholkar K et al. Int J Res Med Sci. 2016 Jul;4(7):2521-2523
www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012
Review Article
Inflammatory fibroid polyp (Vanek’s tumour) of the bowel
Ketan Vagholkar*, Suvarna Vagholkar
INTRODUCTION
Inflammatory fibroid poly (IFP) is one of the rare
tumours arising from any part of the gut more commonly
in the stomach followed by the small intestine. It was first
described by Vanek in 1949.
He described the lesion in the stomach as a benign, non-
encapsulated, submucosal granuloma composed mainly
of loose connective tissue, vessels and abundant
eosinophilic inflammatory component.1
It is also called as
eosinophilic granuloma, submucosal fibroma or
inflammatory pseudotumor. It is equally seen in both the
sexes involving all age groups.
Site of origin
IFP’s are idiopathic, localized, benign neoplastic lesions
originating in the submucosa of the gastrointestinal tract.
Gastric antrum is the commonest site followed by small
bowel, colorectal, gall bladder, oesophagus and
duodenum.2,3
Ileal IFP’s deserve special mention as they
usually present as intussusception in adults.3
Pathology
Macroscopically the tumours are either pedunculated or
sessile in nature. Majority of them are in the form of
pedunculated polyps (Figure 1). Their size may vary from
0.2 cm to 20 cms. They project into the lumen of the
bowel. The surface of the tumour is ulcerated and pale.
Figure1: Pedunculated IFP arising from the terminal
ileum was the apex of the intussusception.
ABSTRACT
Inflammatory fibroid polyp (IFP) or Vanek’s tumour is a rare benign tumour of the gastrointestinal tract. Lower
gastrointestinal IFP’s may present as an acute abdomen in the form of an intussusception in an adult. The surgeon
needs to be aware of this entity to avert misdiagnosis. An abdominal CT or an MRI is helpful in diagnosing an acute
abdomen due to IFP in an adult. Surgery is the mainstay of treatment. The etiopathology and management of this rare
tumour is discussed.
Keywords: Inflammatory fibroid polyp, Vanek’s tumour
Department of Surgery, D.Y. Patil University School of Medicine, Navi Mumbai, India
Received: 24 May 2016
Accepted: 28 May 2016
*Correspondence:
Dr. Ketan Vagholkar,
E-mail: kvagholkar@yahoo.com
Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.
DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20161846
Vagholkar K et al. Int J Res Med Sci. 2016 Jul;4(7):2521-2523
International Journal of Research in Medical Sciences | July 2016 | Vol 4 | Issue 7 Page 2522
Microscopically IFP’s exhibit peculiar features. Vascular
and fibroblastic proliferation accompanied by an
inflammatory response which is predominantly
eosinophilic in nature is characteristic. Mononuclear
spindle shaped cells arranged in whorls around the blood
vessels or mucosal glands are typically seen (Figure 2).
There is an abundant inflammatory response comprising
of eosinophils, lymphocytes, macrophages and
mastocytes.4
However the eosinophil component
predominates. The background matrix contains collagen.
This was described by Vanek.1
Based on this component
he classified IFP’s into two types. The classic type
typically seen in the stomach comprised of heavy
inflammatory infiltrate or eosinophilic granuloma but
sparse amount of collagen. The intestinal type was
paucicellular but collagen rich. Fibroblastic spindle cells
and inflammatory infiltrates were scant. Recurrence in
completely resected tumours is extremely rare.2-4
Figure 2: HP reveals spindle shaped cells in a
background of eosinophilic inflammatory infiltrate.
(H&E staining, Magnification 4X).
Genetics and immunohistochemistry
IFP’s are associated with genetic mutations in PDGFRA.
The frequency of mutations ranges from 21% to 69%.5,6
In the gastric type of IFP, spindle cells are positive for
CD 34. However in the intestinal type this is not seen.7
Immunostaining for kIT, DOG -1, S 100 and EMA are
negative. However IFP is strongly positive for vimentin
but absent for HAM-56. This phenomenon is suggestive
of a major component of spindle cells best recognizable
as fibroblasts.2
Thus IFP’s exhibit variable activity for
actin, CD 34, desmin, CD117 and S100.7
Clinical features
The site of the tumour determines the mode of
presentation.8
Tumours situated above the level of the
ligament of Treitz are usually asymptomatic. These may
present with vague symptoms of abdominal discomfort,
upper abdominal pain, vomiting and bleeding. Tumours
situated below the ligament of Treitz usually present as
an acute abdomen. Acute onset colicky abdominal pain,
lower GI bleeding and in certain cases a complicated
intussusception. IFP’s can cause intussusception in adults
just as other benign tumours such as lipomas.8
Diagnosis and treatment
An elaborate history can provide suggestive leads
provided one is aware of such pathology. IFP’s in the
stomach can only be picked up by gastroscopy. This
enables both diagnosis as well as therapy. However
differentiation from GIST remains a big challenge. This
can only be ascertained by detailed immune
histochemical studies. Immunohistochemistry of the
tumour tissue in GIST exhibits positivity for CD 34,
vimentin and CD 117. Whereas gastric IFP exhibits
positivity only for CD 117.9,10
It is the lower
gastrointestinal IFP’s which pose a diagnostic dilemma.
Push and pull enteroscopy or double balloon enteroscopy
can aid in the diagnosis. Capsule endoscopy is yet
another option but restricted to non-emergency situations
only. Colonic lesions can be diagnosed by colonoscopy.
Lower gastrointestinal IFP’s usually present with lower
GI symptoms and many a times with features suggestive
of an acute abdomen. Physical examination in cases
presenting as acute abdomen by virtue of intussusception
may have a palpable lump. An abdominal x ray will
reveal air fluid levels only in cases of complicated
intussusception. Ultrasound may show a lump or mass.
However diagnostic signs may not be picked up by USG
as it is performer dependent.11
An abdominal CT is
invaluable for diagnosis. It will help in identifying
intussusception. In a transverse view a typical target or
doughnut sign is seen. While in a longitudinal view a
pseudo kidney, sandwich or hayfork sign may be seen.12
MRI of the abdomen can also help in diagnosis with
atypical bowel in bowel or coiled spring appearance seen.
The sensitivity, specificity and accuracy of MRI is higher
that CT (Table 1).12,13
Small bowel intussusception in
adults can be caused by a variety of benign causes. Hence
the final diagnosis can only be reached by a diagnostic
laparoscopy or an open laparotomy followed by
histopathological and immune histochemical studies of
the specimen.14
Table 1: Diagnostic efficacy of MRI vs CT.
MRI CT
Sensitivity % 95 71
Specificity % 100 71
Accuracy % 96 71
Intraoperative reduction of the intussusception continues
to be a contentious issue.14,15
In cases of exclusive small
bowel intussusceptions, intraoperative reduction may be
attempted.13
The advantage of reduction is that the length
of the bowel to be resected is reduced.15,16
However in
cases of ileocolic or a colocolic intussusceptions, as the
risk of a malignant lesion looms high it is preferable to do
en bloc resection of the unreduced specimen based on
oncological principles. The same applies to
Vagholkar K et al. Int J Res Med Sci. 2016 Jul;4(7):2521-2523
International Journal of Research in Medical Sciences | July 2016 | Vol 4 | Issue 7 Page 2523
intussusceptions complicated by ischaemia, necrosis or
perforation.16,17
CONCLUSION
Inflammatory fibroid polyp or Vanek’s tumour is a rare
type of small intestinal tumour. It presents as an acute
abdomen in the form of an intussusception in an adult.
This poses a diagnostic dilemma. Endoscopy is
diagnostic for upper GI IFP’s. Whereas abdominal CT or
MRI can be diagnostic for lower GI IFP’s. Diagnostic
laparoscopy or open laparotomy is essential for safe
surgical treatment and confirmation of the diagnosis.
ACKNOWLEDGEMENTS
Authors would like to thank Mr. Parth K. Vagholkar for
his help in typesetting the manuscript.
Funding: No funding sources
Conflict of interest: None declared
Ethical approval: Not required
REFERENCES
1. Vanek J. Gastric submucosal granuloma with
eosinophilic infiltration. Am J Pathol. 1949;25:397-
411.
2. Liu TC, Lin MT, Montgomery EA, Singhi AD.
Inflammatory fibroid polyps of the gastrointestinal
tract: spectrum of clinical, morphologic, and
immunohistochemistry features. Am J Surg Pathol.
2013;37:586-92.
3. Akbulut S. Intussusception due to inflammatory
fibroid polyp: a case report and comprehensive
literature review. World J Gastroenterol.
2012;18:5745-52.
4. Daum O, Hes O, Vanecek T, Benes Z, Sima R,
Zamecnik M, Mukensnabl P, Hadravska S, Curik R,
Michal M. Vanek’s tumor (inflammatory fibroid
polyp) report of 18 cases and comparison with three
cases of original vanek’s series. Ann Diagn Pathol.
2003;7:337-47.
5. Schildhaus HU, Cavlar T, Binot E, Büttner R,
Wardelmann E, Merkelbach-Bruse S. Inflammatory
fibroid polyps harbour mutations in the platelet-
derived growth factor receptor alpha (PDGFRA)
gene. J Pathol. 2008;216:176-82.
6. Huss S, Wardelmann E, Goltz D, Binot E, Hartmann
W, Merkelbach BS, Buttner R, Schildhaus HU.
Activating PDGFRA mutations in inflammatory
fibroid polyps occur in exons 12, 14 and 18 and are
associated with tumour localization. Histopathology.
2012;61:59-68.
7. Kolodziejczyk P, Yao T, Tsuneyoshi M.
Inflammatory fibroid polyp of the stomach a special
reference to an immunohistochemical profile of 42
cases. Am J Surg Pathol. 1993;17:1159-68.
8. Vagholkar K, Chavan R, Mahadik A, Maurya I.
Lipoma of the small intestine: a cause for
intussusception in adults. Hindawi.
2015;2015:856030.
9. Paikos D, Moschos J, Tzilves D, Koulaouzidis A,
Kouklakis G, Patakiouta F, Kontodimou K,
Tarpagos A, Katsos I. Inflammatory fibroid polyp or
Vanek’s tumour. Dig Surg. 2007;24:231-3.
10. Nonose R, Valenciano JS, da Silva CM, de Souza
CA, Martinez CA. Ileal intussusception caused by
vanek’s tumor: a case report. Case Rep
Gastroenterol. 2011;5:110-6.
11. Harned RK, Buck JL, Shekitka KM. Inflammatory
fibroid polyps of the gastrointestinal tract:
radiologic evaluation. Radiology. 1992;182:863-6.
12. Sundaram B, Miller CN, Cohan RH, Schipper MJ,
Francis IR. Can CT features be used to diagnose
surgical adult bowel intussusceptions? AJR Am J
Roentgenol. 2009;193:471-8.
13. Beall DP, Fortman BJ, Lawler BC, Regan F.
Imaging bowel obstruction: a comparison between
fast magnetic resonance imaging and helical
computed tomography. Clin Radiol. 2002;57:719-
24.
14. Ghaderi H, Jafarian A, Aminian A, Mirjafari
Daryasari SA. Clinical presentations, diagnosis and
treatment of adult intussusception, a 20 years
survey. Int J Surg. 2010;8:318-20.
15. Yakan S, Caliskan C, Makay O, Denecli AG,
Korkut MA. Intussusception in adults: clinical
characteristics, diagnosis and operative strategies.
World J Gastroenterol. 2009;15:1985-9.
16. Wang N, Cui XY, Liu Y, Long J, Xu YH, Guo RX,
Guo KJ. Adult intussusception: a retrospective
review of 41 cases. World J Gastroenterol.
2009;15:3303-8.
17. Marinis A, Yiallourou A, Samanides L, Dafnios N,
Anastasopoulos G, Vassiliou I, Theodosopoulos T.
Intussusception of the bowel in adults: a review.
World J Gastroenterol. 2009;15:407-11.
Cite this article as: Vagholkar K, Vagholkar S.
Inflammatory fibroid polyp (Vanek’s tumour) of the
bowel. Int J Res Med Sci 2016;4:2521-3.

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Rare bowel tumor: Inflammatory fibroid polyp

  • 1. International Journal of Research in Medical Sciences | July 2016 | Vol 4 | Issue 7 Page 2521 International Journal of Research in Medical Sciences Vagholkar K et al. Int J Res Med Sci. 2016 Jul;4(7):2521-2523 www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012 Review Article Inflammatory fibroid polyp (Vanek’s tumour) of the bowel Ketan Vagholkar*, Suvarna Vagholkar INTRODUCTION Inflammatory fibroid poly (IFP) is one of the rare tumours arising from any part of the gut more commonly in the stomach followed by the small intestine. It was first described by Vanek in 1949. He described the lesion in the stomach as a benign, non- encapsulated, submucosal granuloma composed mainly of loose connective tissue, vessels and abundant eosinophilic inflammatory component.1 It is also called as eosinophilic granuloma, submucosal fibroma or inflammatory pseudotumor. It is equally seen in both the sexes involving all age groups. Site of origin IFP’s are idiopathic, localized, benign neoplastic lesions originating in the submucosa of the gastrointestinal tract. Gastric antrum is the commonest site followed by small bowel, colorectal, gall bladder, oesophagus and duodenum.2,3 Ileal IFP’s deserve special mention as they usually present as intussusception in adults.3 Pathology Macroscopically the tumours are either pedunculated or sessile in nature. Majority of them are in the form of pedunculated polyps (Figure 1). Their size may vary from 0.2 cm to 20 cms. They project into the lumen of the bowel. The surface of the tumour is ulcerated and pale. Figure1: Pedunculated IFP arising from the terminal ileum was the apex of the intussusception. ABSTRACT Inflammatory fibroid polyp (IFP) or Vanek’s tumour is a rare benign tumour of the gastrointestinal tract. Lower gastrointestinal IFP’s may present as an acute abdomen in the form of an intussusception in an adult. The surgeon needs to be aware of this entity to avert misdiagnosis. An abdominal CT or an MRI is helpful in diagnosing an acute abdomen due to IFP in an adult. Surgery is the mainstay of treatment. The etiopathology and management of this rare tumour is discussed. Keywords: Inflammatory fibroid polyp, Vanek’s tumour Department of Surgery, D.Y. Patil University School of Medicine, Navi Mumbai, India Received: 24 May 2016 Accepted: 28 May 2016 *Correspondence: Dr. Ketan Vagholkar, E-mail: kvagholkar@yahoo.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20161846
  • 2. Vagholkar K et al. Int J Res Med Sci. 2016 Jul;4(7):2521-2523 International Journal of Research in Medical Sciences | July 2016 | Vol 4 | Issue 7 Page 2522 Microscopically IFP’s exhibit peculiar features. Vascular and fibroblastic proliferation accompanied by an inflammatory response which is predominantly eosinophilic in nature is characteristic. Mononuclear spindle shaped cells arranged in whorls around the blood vessels or mucosal glands are typically seen (Figure 2). There is an abundant inflammatory response comprising of eosinophils, lymphocytes, macrophages and mastocytes.4 However the eosinophil component predominates. The background matrix contains collagen. This was described by Vanek.1 Based on this component he classified IFP’s into two types. The classic type typically seen in the stomach comprised of heavy inflammatory infiltrate or eosinophilic granuloma but sparse amount of collagen. The intestinal type was paucicellular but collagen rich. Fibroblastic spindle cells and inflammatory infiltrates were scant. Recurrence in completely resected tumours is extremely rare.2-4 Figure 2: HP reveals spindle shaped cells in a background of eosinophilic inflammatory infiltrate. (H&E staining, Magnification 4X). Genetics and immunohistochemistry IFP’s are associated with genetic mutations in PDGFRA. The frequency of mutations ranges from 21% to 69%.5,6 In the gastric type of IFP, spindle cells are positive for CD 34. However in the intestinal type this is not seen.7 Immunostaining for kIT, DOG -1, S 100 and EMA are negative. However IFP is strongly positive for vimentin but absent for HAM-56. This phenomenon is suggestive of a major component of spindle cells best recognizable as fibroblasts.2 Thus IFP’s exhibit variable activity for actin, CD 34, desmin, CD117 and S100.7 Clinical features The site of the tumour determines the mode of presentation.8 Tumours situated above the level of the ligament of Treitz are usually asymptomatic. These may present with vague symptoms of abdominal discomfort, upper abdominal pain, vomiting and bleeding. Tumours situated below the ligament of Treitz usually present as an acute abdomen. Acute onset colicky abdominal pain, lower GI bleeding and in certain cases a complicated intussusception. IFP’s can cause intussusception in adults just as other benign tumours such as lipomas.8 Diagnosis and treatment An elaborate history can provide suggestive leads provided one is aware of such pathology. IFP’s in the stomach can only be picked up by gastroscopy. This enables both diagnosis as well as therapy. However differentiation from GIST remains a big challenge. This can only be ascertained by detailed immune histochemical studies. Immunohistochemistry of the tumour tissue in GIST exhibits positivity for CD 34, vimentin and CD 117. Whereas gastric IFP exhibits positivity only for CD 117.9,10 It is the lower gastrointestinal IFP’s which pose a diagnostic dilemma. Push and pull enteroscopy or double balloon enteroscopy can aid in the diagnosis. Capsule endoscopy is yet another option but restricted to non-emergency situations only. Colonic lesions can be diagnosed by colonoscopy. Lower gastrointestinal IFP’s usually present with lower GI symptoms and many a times with features suggestive of an acute abdomen. Physical examination in cases presenting as acute abdomen by virtue of intussusception may have a palpable lump. An abdominal x ray will reveal air fluid levels only in cases of complicated intussusception. Ultrasound may show a lump or mass. However diagnostic signs may not be picked up by USG as it is performer dependent.11 An abdominal CT is invaluable for diagnosis. It will help in identifying intussusception. In a transverse view a typical target or doughnut sign is seen. While in a longitudinal view a pseudo kidney, sandwich or hayfork sign may be seen.12 MRI of the abdomen can also help in diagnosis with atypical bowel in bowel or coiled spring appearance seen. The sensitivity, specificity and accuracy of MRI is higher that CT (Table 1).12,13 Small bowel intussusception in adults can be caused by a variety of benign causes. Hence the final diagnosis can only be reached by a diagnostic laparoscopy or an open laparotomy followed by histopathological and immune histochemical studies of the specimen.14 Table 1: Diagnostic efficacy of MRI vs CT. MRI CT Sensitivity % 95 71 Specificity % 100 71 Accuracy % 96 71 Intraoperative reduction of the intussusception continues to be a contentious issue.14,15 In cases of exclusive small bowel intussusceptions, intraoperative reduction may be attempted.13 The advantage of reduction is that the length of the bowel to be resected is reduced.15,16 However in cases of ileocolic or a colocolic intussusceptions, as the risk of a malignant lesion looms high it is preferable to do en bloc resection of the unreduced specimen based on oncological principles. The same applies to
  • 3. Vagholkar K et al. Int J Res Med Sci. 2016 Jul;4(7):2521-2523 International Journal of Research in Medical Sciences | July 2016 | Vol 4 | Issue 7 Page 2523 intussusceptions complicated by ischaemia, necrosis or perforation.16,17 CONCLUSION Inflammatory fibroid polyp or Vanek’s tumour is a rare type of small intestinal tumour. It presents as an acute abdomen in the form of an intussusception in an adult. This poses a diagnostic dilemma. Endoscopy is diagnostic for upper GI IFP’s. Whereas abdominal CT or MRI can be diagnostic for lower GI IFP’s. Diagnostic laparoscopy or open laparotomy is essential for safe surgical treatment and confirmation of the diagnosis. ACKNOWLEDGEMENTS Authors would like to thank Mr. Parth K. Vagholkar for his help in typesetting the manuscript. Funding: No funding sources Conflict of interest: None declared Ethical approval: Not required REFERENCES 1. Vanek J. Gastric submucosal granuloma with eosinophilic infiltration. Am J Pathol. 1949;25:397- 411. 2. Liu TC, Lin MT, Montgomery EA, Singhi AD. Inflammatory fibroid polyps of the gastrointestinal tract: spectrum of clinical, morphologic, and immunohistochemistry features. Am J Surg Pathol. 2013;37:586-92. 3. Akbulut S. Intussusception due to inflammatory fibroid polyp: a case report and comprehensive literature review. World J Gastroenterol. 2012;18:5745-52. 4. Daum O, Hes O, Vanecek T, Benes Z, Sima R, Zamecnik M, Mukensnabl P, Hadravska S, Curik R, Michal M. Vanek’s tumor (inflammatory fibroid polyp) report of 18 cases and comparison with three cases of original vanek’s series. Ann Diagn Pathol. 2003;7:337-47. 5. Schildhaus HU, Cavlar T, Binot E, Büttner R, Wardelmann E, Merkelbach-Bruse S. Inflammatory fibroid polyps harbour mutations in the platelet- derived growth factor receptor alpha (PDGFRA) gene. J Pathol. 2008;216:176-82. 6. Huss S, Wardelmann E, Goltz D, Binot E, Hartmann W, Merkelbach BS, Buttner R, Schildhaus HU. Activating PDGFRA mutations in inflammatory fibroid polyps occur in exons 12, 14 and 18 and are associated with tumour localization. Histopathology. 2012;61:59-68. 7. Kolodziejczyk P, Yao T, Tsuneyoshi M. Inflammatory fibroid polyp of the stomach a special reference to an immunohistochemical profile of 42 cases. Am J Surg Pathol. 1993;17:1159-68. 8. Vagholkar K, Chavan R, Mahadik A, Maurya I. Lipoma of the small intestine: a cause for intussusception in adults. Hindawi. 2015;2015:856030. 9. Paikos D, Moschos J, Tzilves D, Koulaouzidis A, Kouklakis G, Patakiouta F, Kontodimou K, Tarpagos A, Katsos I. Inflammatory fibroid polyp or Vanek’s tumour. Dig Surg. 2007;24:231-3. 10. Nonose R, Valenciano JS, da Silva CM, de Souza CA, Martinez CA. Ileal intussusception caused by vanek’s tumor: a case report. Case Rep Gastroenterol. 2011;5:110-6. 11. Harned RK, Buck JL, Shekitka KM. Inflammatory fibroid polyps of the gastrointestinal tract: radiologic evaluation. Radiology. 1992;182:863-6. 12. Sundaram B, Miller CN, Cohan RH, Schipper MJ, Francis IR. Can CT features be used to diagnose surgical adult bowel intussusceptions? AJR Am J Roentgenol. 2009;193:471-8. 13. Beall DP, Fortman BJ, Lawler BC, Regan F. Imaging bowel obstruction: a comparison between fast magnetic resonance imaging and helical computed tomography. Clin Radiol. 2002;57:719- 24. 14. Ghaderi H, Jafarian A, Aminian A, Mirjafari Daryasari SA. Clinical presentations, diagnosis and treatment of adult intussusception, a 20 years survey. Int J Surg. 2010;8:318-20. 15. Yakan S, Caliskan C, Makay O, Denecli AG, Korkut MA. Intussusception in adults: clinical characteristics, diagnosis and operative strategies. World J Gastroenterol. 2009;15:1985-9. 16. Wang N, Cui XY, Liu Y, Long J, Xu YH, Guo RX, Guo KJ. Adult intussusception: a retrospective review of 41 cases. World J Gastroenterol. 2009;15:3303-8. 17. Marinis A, Yiallourou A, Samanides L, Dafnios N, Anastasopoulos G, Vassiliou I, Theodosopoulos T. Intussusception of the bowel in adults: a review. World J Gastroenterol. 2009;15:407-11. Cite this article as: Vagholkar K, Vagholkar S. Inflammatory fibroid polyp (Vanek’s tumour) of the bowel. Int J Res Med Sci 2016;4:2521-3.