Reactive nodular fibrous pseudotumors of the gastrointestinal tract: report of 8 cases.

Department of Pathology, Medical Faculty Hospital, Plzen, Czech Republic.
International Journal of Surgical Pathology (Impact Factor: 0.96). 11/2004; 12(4):365-74.
Source: PubMed

ABSTRACT Eight cases of reactive nodular fibrous pseudotumor of the gastrointestinal tract are presented. The patients included 6 males and 2 females between the ages of 1 and 68 years (mean age 41.5 years). Three tumors involved the small intestine, and 5 of the investigated lesions were located in the large bowel. Of these, 2 originated in the sigmoid colon, 1 in the cecum, 1 in the appendix, and 1 in the large bowel not otherwise specified. The tumors' size varied from 3 to 10 cm in the greatest diameter (mean 6.2 cm). Histologically they were composed of stellate or spindle shaped cells resembling fibroblasts arranged haphazardly or in intersecting fascicles, embedded in a collagen-rich stroma, with sparse intralesional mononuclear cells frequently arranged in lymphoid aggregates. Immunohistochemically, the lesions were positive for vimentin (7/7), smooth muscle actin (8/8), muscle-specific actin (5/7), cytokeratins AE1/AE3 (6/7), and CAM 5.2 (1/7), and antigen CD68 (1/7). No case (0/8) reacted positively with antibody to CD117 (c-kit). Genetically no substitutions, deletions, or insertions occurred in exon 11 in all analyzed samples. Likewise, no deletions or insertions in part of exon 9 were observed. Ultrastructurally the tumor cells revealed features typical of myofibroblasts. According to the morphologic, immunohistochemical, and ultrastructural features mentioned above, especially to the positivity of low-molecular-weight cytokeratins, we propose this lesion to be related to a proliferation of multipotential subserosal cells rather than ordinary myofibroblasts or fibroblasts.

  • [Show abstract] [Hide abstract]
    ABSTRACT: BACKGROUND: Heterotopic mesenteric ossification (HMO) is rare condition with unclear multifactorial etiology. When presented with enterocutaneous fistulae, HMO may be devastating for the patient. METHODS: We herein present the case of a 59-year-old woman with persistent enterocutaneous fistula secondary to heterotopic mesenteric ossification, which developed after a complicated gynecologic laparoscopic oophorectomy, abdominal sepsis, multiple small-bowel resections, and skin grafting for an open abdomen. RESULTS: Nonoperative management with nil per os status and total parenteral nutrition of her fistulae was unsuccessful. The patient subsequently underwent en masse resection of her mesenteric ossification with associated small and large intestine. CONCLUSIONS: This case represents a rare occurrence of chronic enterocutaneous and/or enteroatmospheric fistulae secondary to heterotopic mesenteric ossification. Diagnosis, treatment, and histologic appearance of heterotopic mesenteric ossification are discussed in a review of the literature.
    European Surgery 08/2011; 44(4). · 0.26 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Reactive nodular fibrous pseudotumor (RNFP), which presents abdominal clinical manifestations and malignant radiographic results, usually requires radical resection as the treatment. However, RNFP has been recently described as an extremely rare benign post-inflammatory lesion of a reactive nature, which typically arises from the sub-serosal layer of the digestive tract or within the surrounding mesentery in association with local injury or inflammation. In addition, a postoperative diagnosis is necessary to differentiate it from the other reactive processes of the abdomen. Furthermore, RNFP shows a good prognosis without signs of recurrence or metastasis. A 16-year-old girl presented with a 3-mo history of epigastric discomfort, and auxiliary examinations suggested a malignant tumor originating from the stomach; postoperative pathology confirmed RNFP, and after a 2-year follow-up period, the patient did not display any signs of recurrence. This case highlights the importance of preoperative pathology for surgeons who may encounter similar cases.
    World journal of clinical cases. 04/2014; 2(4):111-9.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: We will describe a case of a patient diagnosed with a rare identity of a benign lesion, "reactive nodular fibrous pseudotumor" (RNFP). It is a tumor which preoperatively can present as a malignant tumor and is only reported in 19 cases. According to the very limited amount of information on this tumor in the literature it is mostly seen after trauma or intraperitoneal inflammation. Our case is the second one of RNFP associated with endometriosis, which is a frequently seen intraperitoneal inflammation process in women. Knowledge that these large pseudotumoral lesions can occur is important to direct the management of these patients.
    Case reports in obstetrics and gynecology. 01/2014; 2014:421234.