Instructions to authors
Società Italiana di Cancerologia
Associazione Italiana di Radioterapia Oncologica
Associazione Italiana di Oncologia Medica
Società Italiana di Chirurgia Oncologica
Gli osservatori di Tumori
A cura di Giulia Volpe
Send an article
2013 Vol. 99 N. 4 July-August
2013 Vol. 99 N. 3 May-June
2013 Vol. 99 N. 2 March-April
2013 Vol. 99 N. 1 January-February
2012 Vol. 98 N. 6 November-December
2012 Vol. 98 N. 5 September-October
2012 Vol. 98 N. 4 July-August
2012 Vol. 98 N. 3 May-June
2012 Vol. 98 N. 2 March-April
2012 Vol. 98 N. 1 January-February
2011 Vol. 97 N. 6 November-December
2011 Vol. 97 N. 5 September-October
2011 Vol. 97 N. 4 July-August
2011 Vol. 97 N. 3 May-June
2011 Vol. 97 N. 2 March-April
2011 Vol. 97 N. 1 January-February
2010 Vol. 96 N. 6 November-December
2010 Vol. 96 N. 5 September-October
2010 Vol. 96 N. 4 July-August
2010 Vol. 96 N. 3 May-June
2010 Vol. 96 N. 2 March-April
2010 Vol. 96 N. 1 January-February
2009 Vol. 95 N. 6 November-December
2009 Vol. 95 N. 5 September-October
2009 Vol. 95 N. 4 July-August
2009 Vol. 95 N. 3 May-June
2009 Vol. 95 N. 2 March-April
2009 Vol. 95 N. 1 January-February
2008 Vol. 94 N. 6 November-December
2008 Vol. 94 N. 5 September-October
2008 Vol. 94 N. 4 July-August
2008 Vol. 94 N. 3 May-June
2008 Vol. 94 N. 2 March-April
2008 Vol. 94 N. 1 January-February
2007 Vol. 93 N. 6 November-December
2007 Vol. 93 N. 5 September-October
2007 Vol. 93 N. 4 July-August
2007 Vol. 93 N. 3 May-June
2007 Vol. 93 N. 2 March-April
2007 Vol. 93 N. 1 January-February
2006 Vol. 92 N. 6 November-December
2006 Vol. 92 N. 5 September-October
2006 Vol. 92 N. 4 July-August
2006 Vol. 92 N. 3 May-June
2006 Vol. 92 N. 2 March-April
2006 Vol. 92 N. 1 January-February
2005 Vol. 91 N. 6 November-December
2005 Vol. 91 N. 5 September-October
2005 Vol. 91 N. 4 July-August
2005 Vol. 91 N. 3 May-June
2005 Vol. 91 N. 2 March-April
2005 Vol. 91 N. 1 January-February
2004 Vol. 90 N. 6 November-December
2004 Vol. 90 N. 5 September-October
2004 Vol. 90 N. 4 July-August
2004 Vol. 90 N. 3 May-June
2004 Vol. 90 N. 2 March-April
2004 Vol. 90 N. 1 January-February
2003 Vol. 89 N. 6 November-December
2003 Vol. 89 N. 5 September-October
2003 Vol. 89 N. 4 July-August
2003 Vol. 89 N. 3 May-June
2003 Vol. 89 N. 2 March-April
2003 Vol. 89 N. 1 January-February
2002 Vol. 88 N. 6 November-December
2002 Vol. 88 N. 5 September-October
2002 Vol. 88 N. 4 July-August
2002 Vol. 88 N. 3 May-June
2002 Vol. 88 N. 2 March-April
2002 Vol. 88 N. 1 January-February
Ileal Vanek’s tumor mimicking cecal carcinoma
, Pedro A García-Muñoz
, María J López
Jose A Ruiz-Maciá
, Joaquín Sola
, and Jose A Pons
Department of Surgery,
Department of Pathology, and
Department of Digestive Diseases,
Mesa del Castillo Hospital, Murcia, Spain
inflammatory fibroid polyp, Vanek’s tumor, ileal tumor, carcinoembryonic antigen.
We are grateful to Dr Mike Tobin for his help in reviewing and translating the manuscript.
Antonio Piñero, MD PhD, Mesa del Castillo Hospital, Ronda Sur 20, 30010 Murcia, Spain.
Received February 17, 2011;
accepted May 19, 2011.
Inflammatory fibroid polyps or Vanek’s tumors are rare benign pseudotumoral lesions with morphological characteristics similar to those of submucosal mesenchymal tumors. They have been described in the gastrointestinal tract, most frequently in the gastric antrum. We present a case of ileal Vanek’s tumor associated with a raised carcinoembryonic antigen level and with radiological and endoscopic features mimicking cecal carcinoma.
Subepithelial lesions are a heterogeneous group of digestive tract tumors which can be difficult to differentiate from malignant epithelial tumors but whose management and prognosis are very different. Submucous tumors account for less than 1% of all gastrointestinal neoplasms
. Eighty percent are gastrointestinal stromal tumors (GISTs), which arise from Cajal’s interstitial cells and are related to peristaltic activity. The remainder develop from other subepithelial tissues resulting in leiomyomas, schwannomas, lipomas, glomic tumors and their malignant variants
. There is also an even rarer group of submucous tumors that do not arise from the mesenchyma and that produce lesions such as carcinoid tumors, ectopic tissue, or inflammatory fibroid polyps (IFP). We present a patient with an unusual clinical presentation that illustrates the difficulty in differentiating between cecal carcinoma and this benign entity.
An asymptomatic 49-year-old man undergoing routine analysis was found to have a high level of carcinoembryonic antigen (CEA) (14 ng/mL, normal <5 ng/mL). Colonoscopy showed a 4-cm exophytic polypoid cecal mass, with histology revealing nothing more than inflammatory changes within normal colonic epithelium. Barium enema confirmed a cecal tumor (Figure 1) and abdominal ultrasonography reported a mass compatible with a cecal or appendicular tumor.
At laparotomy, a small bowel intussusception was seen with a polypoid ileal tumor being the precipitating lesion with no other abnormality. An ileocecal resection was performed and the macroscopic appearance was that of a 4-cm terminal ileal mass, 15 cm from the ileocecal valve. The patient made an unremarkable postoperative recovery and was discharged well.
Histological examination showed a well-demarcated non-ulcerated tumor with submucosal proliferation of mononuclear and monomorphic spindle and stellate cells in a fibromixoid stroma, with vascular proliferation and inflammatory infiltration dominated by mast cells and eosinophils. Immunohistochemistry gave negative results for S-100, ALK and CEA, and only mast cells were positive for CD117 (c-kit). The tumor showed a diffuse positive pattern for vimentin and focal positive expression of cyclin D1, and the perivascular stromal cells stained positive for CD34 (Figure 2). At follow-up 6 months following discharge, the patient remained well with normal CEA.
IFP or Vanek’s tumor is a benign pseudotumoral lesion that has morphological features similar to those of submucosal mesenchymal tumors. It consists of cells reminiscent of fibroblasts, inflammatory eosinophilic infiltrate, collagenous fibrils, and abundant vasculature. These features were described by Vanek in 6 gastric tumors
. IFPs seem to occur most frequently between the fifth and seventh decades and can be found throughout the gastrointestinal tract but most commonly in the gastric antrum (70%) and ileum (20%), and rarely in the duodenum or jejunum
. They can be distinguished from GISTs and inflammatory myofibroblastic tumors because these 2 tumors show immunohistochemical positivity for CD117 and smooth muscle actin, respectively.
The reactive rather than neoplastic nature of the lesion is suggested by its low mitotic activity, low recurrence rate, absence of necrosis, and absence of metastases. Although Vanek initially suggested an allergic etiology because of the presence of eosinophils, its etiopathogenesis is not clear and a form of reparative tissue response to injury has been suggested
. It is not, however, a clearly defined pseudotumor and it is possible that 2 histogenetically different lesions exist: CD34-positive typical Vanek’s tumors and less typical CD34-negative lesions that may be related to inflammatory myofibroblastic tumors
have studied overexpression of cyclin D1 in these tumors and they concluded that the stromal cells derive from dendritic cells with myofibroblastic differentiation and a possible defect in cell cycle regulation. Small bowel obstruction secondary to intussusception is the most frequent clinical presentation described in the literature
and its presentation as an isolated finding of raised CEA in an asymptomatic individual has not been previously reported. The CEA level increase should be related to its expression by the epithelial component of the tumor, although, as in other diseases
, the source of CEA production at the cellular level has not been elucidated.
1. Nowain A, Bhakta H, Pais S, Panel G, Verma S: Gastrointestinal stromal tumors: clinical profile, pathogenesis, treatment strategies and prognosis. J Gastroenterol Hepatol, 20: 818-824, 2005.
2. Stupnik S, Rafaelli C, Osorio-González G, Luján-Pestalardo M, Quesada M, Viudez P: Tumores subepiteliales del tracto gastrointestinal. Acta Gastroenterol Latinoam, 39: 118-124, 2009.
3. Vanek J: Gastric submucosal granuloma with eosinophilic infiltration. Am J Pathol, 25: 397-412, 1949.
4. Wysocki AP, Taylor G, Windsor JA: Inflammatory fibroid polyps of the duodenum: a review of the literature. Dig Surg, 24: 162-168, 2007.
5. Gara N, Falzarano JS, Limm WML, Namiki TS, Tom LKS: Ileal inflammatory fibroid polyp causing chronic ileocolic intussusception and mimicking cecal carcinoma. World J Gastrointest Oncol, 15: 89-92, 2009.
6. De Petris G, Leung ST: Pseudoneoplasms of the gastrointestinal tract. Arch Pathol Lab Med, 134: 378-392, 2010.
7. Daum O, Hes O, Vanecek T: Vanek’s tumor (inflammatory fibroid polyp). Report of 18 cases and comparison with three cases of original Vanek’s series. Am Diagn Pathol, 7: 337-347, 2003.
8. Pantanowitz L, Antonioli DA, Pinkus GC, Shahsafaei A, Odze RD: Inflammatory fibroid polyps of the gastrointestinal tract: evidence for a dendritic cell origin. Am J Surg Pathol, 28: 107-114, 2004.
9. Malik KA, Pande GK, Aftab Z, Nirmala V: Inflammatory fibroid polyp of the ileum causing intussusception. Saudi Med J, 26: 995-998, 2005.
10. Karamercan A, Kurukahvecioglu O, Yilmaz TU, Aygencel G, Aytac B, Sare M: Adult ileal intussusception: an unusual emergency condition. Adv Ther, 23: 163-168, 2006.
11. Costamagna D, Erra S, Zullo A, Sevente G, Durando R: Small bowel intussusception secondary to inflammatory fibroid polyp of the ileum: report of a case. Chir Ital, 60: 323-327, 2008.
12. Gakiopoulou H, Litsiou E, Valaris K, Balafoutas D, Patsouris E, Tseleni-Balafouta S: Possible association of CEA expression with oxyphilic change but not with C-cell hyperplasia in Hashimoto’s thyroiditis. Endocr J, 57: 693-699, 2010.
Riproduzione e diritti riservati
ISSN online: 2038-2529