The prevalence of gastrointestinal involvement in von Recklinghausen’s disease is frequent (11 % – 25 %) [1] with different types of neurofibroma and juvenile-like polyps [2]. However, the association of von Recklinghausen’s disease with inflammatory fibroid polyps has been only rarely described [3]. We report here the case of a 39-year-old woman with past history of type 1 neurofibromatosis who was referred for hematochezia.
Colonoscopy was performed and diagnosed a large pedunculated polyp in the sigmoid with a type Ip shape (Paris classification). Using narrow-band imaging and dual focus magnification (Olympus, Tokyo, Japan), the features of the polyp included an amorphous pit pattern over a large area (Kudo VN). The vascular pattern was patchy avascular areas mixed with large irregular vessels (Sano IIIB) ([Fig. 1], [Fig. 2], [Video 1]). The vascular pattern was present over the whole lesion, without any demarcation line. Using the NICE classification [4], the lesion was classified as type III and was suggestive of a deep submucosal invasive cancer.
Fig. 1 Endoscopic aspect of the inflammatory fibroid polyp. a Macroscopic aspect with white light. b Pedunculated shape. c Narrow-band imaging (NBI) aspect in far view, showing patchy avascular areas. d NBI in close-up view, showing irregular vessels.
Fig. 2 Endoscopic aspect of the inflammatory fibroid polyp. a, b Avascular and amorphous whitish area. c Pedunculated aspect after injection. d Narrow-band imaging aspect of the resected specimen.
Video 1 Endoscopic aspect and resection of an inflammatory fibroid polyp.
The lesion was resected en bloc by endoscopic mucosal resection with a large safety margin on the stalk. Pathological examination, after expert discussion (because of the atypical features), concluded a diagnosis of inflammatory fibroid polyp resected totally with safe margins, and not to a neurofibroma or a stromal tumor.
This case illustrates the lack of specificity of the invasive mucosal and vascular pattern of colorectal lesions, as has been demonstrated previously for inflammatory reactions after diverticulitis [5]. The lack of demarcation line, the pedunculated shape, and the past history of von Recklinghausen’s disease may suggest the possibility of choosing endoscopic resection or biopsy sample instead of sending the patient for surgical management. Endoscopic resection with safe margins facilitates a precise pathological assessment to avoid the risk of incomplete resection and, as in the current case, unnecessary colectomy.
Endoscopy_UCTN_Code_CCL_1AD_2AC
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