Possible role of TGF β1 in inflammatory pseudotumor associated with multiple neuroendocrine tumors of the small intestine.

INTRODUCTION

Inflammatory myofibroblastic tumors (IMTs), a rare condition of unknown etiology, have often been reported to be associated with specific infections or malignant tumors. The question of whether IMT themselves are an inflammatory or a neoplastic process is still going on.

CASE REPORT: A 57-year-old female patient was transferred to our hospital with ileus caused by a mesenterial tumor. Intraoperatively, the mesenteric mass and the dependent small intestine segment, as well as a suspect hepatic lesion, were resected. The histopathological investigation revealed 8 malignant neuroendocrine tumors (NET) of the small intestine with lymphatic and hepatic metastasis and a mesenteric IMT. The postoperative course was uneventful, and the patient was discharged on the 18th postoperative day. The last follow-up after 30 months showed no recurrence of the IMT but clinical and radiological evidence of a persistent hepatic metastasis of the NETs. While plasma Chromogranin A remained suppressed by Sandostatin, the TGF β1 level was markedly elevated.

DISCUSSION: Based on the current literature and our previous experiences, we can state that IPT are an aberrant secondary immunological process possibly induced by excessive TGF β1 and not a neoplasia. Nevertheless, the tumorous behavior points to a continuity between inflammation and neoplasia. Differential diagnoses and the potential molecular pathogenesis are further discussed.