Intestinal vasculitis is an infrequent entity in the absence of systemic vasculitis or chronic inflammatory bowel disease. The primary involvement of gut restricted to mesenteric venous territory is exceedingly rare. We report a pseudotumoural lesion of the cecum caused by localized phlebitis and venulitis associated with thrombosis, with a putative immune etiology, in a young adult presenting with obstructive intestinal crisis

Almeida, M

Cruz, J

Soares, J

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ARTICLE IN PRESS0344-0338/$ - sedoi:10.1016/j.prCorrespondfax: +351 210 0E-mail addrePathology – Research and Practice 205 (2009) 726–729www.elsevier.de/prpTEACHING CASE‘‘Enterocolic phlebitis’’ mimicking a primary tumor of the cecum – A rarepresentation of an unusual entityJoa˜o Cruza,, Mendes de Almeidab, Jorge SoaresaaHospital CUF Descobertas, Department of Pathology, R. Dr. Ma´rio Botas – Parque das Nac¸o˜es, 1998-018 Lisboa, PortugalbHospital CUF Descobertas, Department of Surgery, Lisboa, PortugalReceived 28 May 2008; received in revised form 13 December 2008; accepted 17 December 2008AbstractIntestinal vasculitis is an infrequent entity in the absence of systemic vasculitis or chronic inﬂammatory boweldisease. The primary involvement of gut restricted to mesenteric venous territory is exceedingly rare. We report apseudotumoural lesion of the cecum caused by localized phlebitis and venulitis associated with thrombosis, with aputative immune etiology, in a young adult presenting with obstructive intestinal crisis.r 2009 Elsevier GmbH. All rights reserved.Keywords: Enterocolic phlebitis; Phlebitis; VenulitisIntroductionA 29-year-old female with long-standing abdominaldiscomfort and cramping midabdominal pain wasadmitted to our hospital for sudden onset of semi-occlusive intestinal crisis without vomiting, fever,diarrhea, or blood in the stool. On physical examina-tion, the abdomen was moderately tender at the rightlower quadrant and the periumbilical area. Neitherstigmata of cutaneous nor mucosal lesions suggestive ofsystemic vasculitis were detected on physical examina-tion. The patient is a mild to moderate smoker, and shehad been on oral estrogenic hormone therapy forcontraception for more than 5 years. Other factorspotentially associated with venous thrombosis wereexcluded. She denied taking any other drugs. Abdom-inal X-ray ﬁndings were not conclusive. Pelvic andabdominal ultrasound study revealed an ill-deﬁned masse front matter r 2009 Elsevier GmbH. All rights reserved.p.2008.12.018ing author. Tel.: +351 210 025 392;25 394ss: joao.cruz@hospitalcufdescobertas.pt (J. Cruz).formed by adherences of small intestinal loops to theterminal segment of the ileum associated with luminaldistention, the intestinal wall being mildly increased inthickness. The clinical picture was suggestive of partialmechanical obstruction, and intussusception and volvu-lus were suspected. An elective right hemicolectomy wasperformed.Materials and methodsSelected tissue samples were formalin ﬁxed andparafﬁn embedded. Four-micrometer-thick routinelystained hematoxylin–eosin sections were microscopicallyevaluated by two of the authors. Immunohistochemistrywas performed on 4-mm representative sections afterantigen retrieval using the standard avidin–biotinimmunoperoxidase detection technique and the follow-ing antisera: anti-CD20 (monoclonal, 1:2000; Dako),anti-CD3 (polyclonal, 1:300; Dako), anti-CD4 (mono-clonal, 1:60; Novocastra), and anti-CD8 (monoclonal,1:15; Novocastra). Positive controls were included inARTICLE IN PRESSJ. Cruz et al. / Pathology – Research and Practice 205 (2009) 726–729 727each slide run with satisfactory results. Clinical recordswere reviewed to assess the medical and surgical dataand patient follow-up.Fig. 2. Late-stage phlebitis with organized thrombosis andresidual lymphocytic inﬁltration (hematoxylin–eosin, originalmagniﬁcation  400).Fig. 3. CD20 immunoreactivity (original magniﬁcationResultsThe surgical specimen consisted of an 18-cm-longright hemicolectomy, with a 4.5 3 3 cm partiallyulcerated soft and edematous mass, with irregularborders and serosal umbilication, partially involvingthe ileo-cecal valve. The ileal wall was slightly thickened,and the remaining colon and the appendix wereunremarkable macroscopically. Eleven lymph nodeswere dissected from peri-intestinal mesenteric fat.Histopathological examination showed mucosal ero-sion associated with prominent submucosal edema andischemia, as well as inﬂammatory lesions affectingsmall- and medium-sized venous vessels, transmural indistribution but with submucosal predominance. Thevasculitic lesions were present at the tumoural mass andin the remaining, grossly uninvolved colonic wall. Theinﬂammatory cell inﬁltrate of the venous walls waspolymorphic in composition (mature small lymphocytes,neutrophils, few eosinophils, and scattered histiocytesand giant cells), but was predominantly lymphocytic andneutrophilic.These morphological changes were associated withsigns of venous thrombosis showing different stages oforganization and conspicuous foci of myointimalhyperplasia, resulting in near-complete occlusion ofvascular lumina. Vasculitic lesions involving arteriolesand arteries were never found, as well as granulomas(Figs. 1 and 2). The appendix presented subserosal fociwith similar vascular lesions, although of much lessseverity. The regional lymph nodes had non-speciﬁcreactive changes.Fig. 1. Myointimal hyperplasia and lumen narrowing in amedium-sized vein with signs of recent thrombosis (hematoxylin–eosin, original magniﬁcation  400). 400).Fig. 4. CD3 immunoreactivity (original magniﬁcation  400).ARTICLE IN PRESSFig. 5. CD4 immunoreactivity (original magniﬁcation  400).Fig. 6. CD8 immunoreactivity (original magniﬁcation  400).J. Cruz et al. / Pathology – Research and Practice 205 (2009) 726–729728Immunohistochemistry for CD20, CD3, CD4, andCD8 was performed to characterize the lymphocyticinﬁltrate in the venulitic lesions. Few or no B-celllymphocytes were identiﬁed. We observed a brisk CD3-positive lymphocyte inﬁltrate, composed both of CD4-and CD8-positive lymphocytes, the former outnumber-ing the latter (Figs. 3–6).DiscussionVasculitis of the gastrointestinal tract has occasionallybeen described in systemic lupus erythematosus patients,affecting both arterioles and venules with a predomi-nantly arteriolar involvement [6]. Examples of intestinalvasculitis have also been reported in Churg–Strausssyndrome, Behc¸et’s disease, polyarteritis nodosa, andHenoch–Scho¨nlein purpura, but venous inﬂammation isnot a frequent feature of these entities [5]. Ischemiclesions due to vasculitis limited to gastrointestinal siteshave also been reported in association with Crohn’sdisease, and the inﬂammatory process also affects thearterial vasculature [1,3].Primary intestinal phlebitis and venulitis are exceed-ingly rare. Very few reports received descriptivedesignations, namely mesenteric inﬂammatory veno-occlusive disease, enterocolic (lymphocytic) phlebitis,lymphocytic phlebitis, necrotizing and giant cell granu-lomatous phlebitis, idiopathic myointimal hyperplasiaof mesenteric veins, intramural mesenteric venulitis, andidiopathic colonic phlebitis [2,4].The larger series of this entity were reported byFlaherty et al. and Saraga et al., who described themorphologic spectrum of lesions found on surgicalspecimens from 13 patients, seven females and six males,aged 27–78 years. The large bowel and the small bowelwere the most involved sites. Ischemic lesions associatedwith vasculitis and thrombosis of veins and venules withsparing of arterial tributaries were common to all cases.The vascular inﬂammatory inﬁltrate varied in composi-tion from predominantly lymphocytic to neutrophilicwith ﬁbrin deposition (necrotizing venulitis). Granulo-matous vasculitis was identiﬁed in some cases, butmyointimal hyperplasia with reduction of vascularlumina was frequently present. Flaherty et al. suggestedthat a temporal transition from primarily necrotizing tolymphocytic inﬂammation may exist, and Saraga et al.hypothesized that all the cases probably belong to thesame clinicopathologic entity. They suggested thatthrombosis is more likely a consequence of the phlebiticand venulitic lesions and the cause of the ischemicintestinal injury. Secondary myointimal hyperplasiaeventually further reduces mesenteric vascular ﬂowand consequently contributes to increased ischemicintestinal damage [2,4]. Similarly, we identiﬁed thrombionly in inﬂamed veins and venules. These ﬁbrin thrombiwere found at different stages, from recent to organized,indicating a chronic pathway. Moreover, since phlebitiswithout thrombosis is the dominant lesion and theunaffected veins and venules did not show thrombosis,local intravascular coagulation should represent an end-stage secondary event of the chronic phlebitic andvenulitic process.The etiology of this clinicopathological entity remainsto be elucidated, and no association with a deﬁnitepredisposing cause was identiﬁed. A lymphocyte-mediated vascular damage linked to a hypersensitivityreaction is the most likely pathogenesis, as reported bySaraga et al. [2].We studied the immunohistochemical proﬁle of thelymphoid inﬂammatory inﬁltrate and, at variance withSaraga et al., who reported a mixed population of B andT-cells, we identiﬁed only T-lineage elements. CD20-positive lymphocytes were never identiﬁed in thevascular lesions. The T-cell inﬁltrate was composedARTICLE IN PRESSJ. Cruz et al. / Pathology – Research and Practice 205 (2009) 726–729 729both of CD4-positive T helper-inducer cells and CD8-positive cytotoxic lymphocytes, with a CD4 subsetpredominance. Thus, our ﬁndings support Saraga’sview that the vascular injury is caused by a hypersensi-tivity reaction, and further suggest that a delayed-typemechanism mediated by CD4 and CD8 cells might bethe predominant pathogenic pathway of vein andvenular injury.Most of the patients reported by Flaherty et al. andSaraga et al. fully recovered from the surgery withoutany local recurrence during the follow-up. The presentcase illustrates an example of isolated intestinal phlebitisand venulitis with a unique presentation. The colonos-copies performed after surgery were considered normal,and the biopsies taken had mild non-speciﬁc inﬂamma-tory changes. The patient had a follow-up of 24 months,and there are neither signs of local recurrence norevidence of extraintestinal vasculitis and inﬂammatorybowel disease.To the best of our knowledge, this is the ﬁrst reporton a pseudotumoural clinical presentation of a primary‘‘enterocolic phlebitis’’ that needs to be added to thespectrum of mass lesions of the colon occurring inyoung patients without manifestations of systemicvascular disease or chronic inﬂammatory bowel disease.References[1] A.J. Wakeﬁeld, E.A. Sankey, A.P. Dhilon, Granulomatousvasculitis in Crohn’s disease, Gastroenterology 100 (1991)1279–1287.[2] E. Saraga, H. Bouzourenne, Enterocolic (lymphocytic)phlebitis: a rare cause of intestinal ischemic necrosis. Aseries of six patients and review of the literature, Am. J.Surg. Pathol. 6 (2000) 824–829.[3] J.T. Lie, Vasculitis and the gut, J. Rheumatol. 18 (1991)647–649.[4] M.J. Flaherty, J.T. Lie, R.C. Haggit, Mesenteric inﬂam-matory veno-occlusive disease. A seldom recognized causeof intestinal ischemia, Am. J. Surg. Pathol. 18 (1994)779–784.[5] R.G. Lee, The colitis of Behcet’s syndrome, Am. J. Surg.Pathol. 10 (1986) 888–893.[6] T.R. Helliwell, D. Flook, J. Whithworth, D.W. Day,Arteritis and venulitis in systemic lupus erithematosusresulting in massive lower intestinal hemorrhage, Histo-pathology 9 (1985) 1103–1113.

Enterocolic phlebitis" mimicking a primary tumor of the cecum--a rare presentation of an unusual entity.

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Enterocolic phlebitis" mimicking a primary tumor of the cecum--a rare presentation of an unusual entity.

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