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Inflammatory fibroid polyp: a rare benign tumor of the alimentary tract in children presenting as intussusception-case report and review of literature.

ABSTRACT: Inflammatory fibroid polyp (IFP) represents a rare cause of gastrointestinal polypoid disease in childhood. Τhe lesion has been described by various names beyond the currently accepted term, including "Vanek's tumour," eosinophilic or submucosal granuloma, gastric fibroma with eosinophilic infiltration, inflammatory pseudotumor, and hemangiopericytoma. The etiopathogenesis and origin of the mesenchymal spindle-shaped cells that comprise the polyp remains enigmatic. Recent studies have shown familial occurrence, expression of platelet-derived growth factor receptor (PDGFRA) and oncogenic PDGFRA mutations in the majority of lesions, suggestive of a neoplastic nature. We present a rare case of a 10-year-old boy with an IFP of the terminal ileum, who presented acutely with intussusception and was treated with a right hemicolectomy. Postoperative course was uneventful and the patient has been asymptomatic during follow-up. Histopathology and immunohistochemical analysis excluded inflammatory myofibroblastic tumor (negative for Alk1, desmin, smooth muscle actin [SMA]), gastrointerstinal stromal tumors (GIST) (negative for CD117) and schwannoma (negative for S100). The lesion was positive for CD34 and faintly for vimentin. Despite the classification of IFPs as a mesenchymal benign neoplasm, in the vast majority of cases, surgical excision alone was curative, and no reports exist of a malignant transformation. A cautious approach with periodic surveillance of the affected children seems reasonable though.

SUBMITTER: Siminas S

PROVIDER: S-EPMC4336055 | biostudies-other | 2014 Jun

REPOSITORIES: biostudies-other

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Project description:Introduction Benign multicystic peritoneal mesothelioma (BMPM) represents a very rare clinical entity, with only 130 registered cases in the medical literature, therefore it is usually overlooked from the list of differential diagnoses. The treatment consists of surgery and other authors suggest complementing it with hyperthermic intraperitoneal chemotherapy. Presentation of case A 25-year-old multiparous female experienced periodic abdominal discomfort for two weeks. She developed constipation, urinary urgency, and irregular menstruation. Family history was remarkable for endometrial and breast cancer. Abdominal examination revealed a palpable mass. Abdominal ultrasound and computed tomography scan identified the multicystic appearance of the mass. The diagnosis was unclear, therefore exploratory laparotomy was performed, which revealed multiple grape-like clusters of cysts that were excised immediately. BMPM was diagnosed based on the pathology report. Eventually, the follow-up did not reveal any recurrence. Discussion Mesothelial tumors include three pathological entities, including Benign multicystic peritoneal mesothelioma (BMPM). BMPM is an uncommon neoplasm and has a high recurrence rate after surgery. BMPM consists of clear cysts that take the shape of a grape-like cluster. Clinically, BMPM resembles a tangible abdominal mass and it is challenging to be diagnosed, due to its numerous differential diagnoses. Conclusion The definitive diagnosis of intraperitoneal cystic masses is usually challenging. Therefore, BMPM -although very rare- should always be thought of when dealing with an intraperitoneal cystic mass, especially in women in the reproductive years. In our case cytoreductive surgery solely was sufficient to achieve a disease free follow up, however, further studies regarding treatment and follow-up are required. Highlights • The incidence of Benign multicystic peritoneal mesothelioma (BMPM) is exceptionally rare.• Pathological evaluation is considered the best technique to diagnose.• BMPM is most common in women in reproductive age.• BMPM should be considered as a differential diagnosis for abdominal masses.

Project description:Acute abdominal syndrome can be caused by several possible reasons. The most common causes are perforation of a gastroduodenal ulcer, peritonitis, intestinal obstructions, and perforation of an appendix or fallopian tube. Fever and pain can be caused by an appendicitis or sigmoiditis. Appendiceal lymphoma is a rare disease that is usually found incidentally during appendectomy. Most of the cases are non-Hodgkin's lymphomas. Mantle cell lymphoma is an aggressive B-cell non-Hodgkin's lymphoma with a poorer prognosis than other B-cell lymphomas; thus, a definitive diagnosis is essential. A 60-year-old man presented with right lower quadrant pain. He denied any nausea, vomiting or anorexia and was afebrile. The physical examination revealed right lower quadrant abdomen tenderness. The computed tomography scan revealed periappendiceal fatty stranding with a swollen appendix, approximately 2 cm in diameter and prominent paraaortic, portacaval and mesenteric lymph nodes. A diagnosis of acute appendicitis was made, and laparoscopic appendectomy was performed immediately. The subsequent pathological examination revealed severe congestion with lymphoid hyperplasia. The immunohistochemistry stains revealed positive staining for cluster of differentiation (CD) CD20, B-cell lymphoma-2 (Bcl-2), cyclin D1, SRY-box transcription factor-11 (SOX-11), immunoglobulin D (IgD) and immunoglobulin M (IgM) but negative staining for CD3, CD5, CD10 and CD23. 18F-FDG positron emission tomography showed peripheral lymph node involvement, while the bone marrow biopsy showed negative findings. Therefore, a diagnosis of mantle cell lymphoma, Ann Arbor stage IVA, was made. The patient received postoperative combination chemotherapy and remained in a stable condition over a 1-year follow-up period. We report an uncommon case that initially presented as acute appendicitis, for which a final diagnosis of mantle cell lymphoma was made. In comparison with other B-cell lymphomas, mantle cell lymphoma has a poorer prognosis, and positive immunochemical staining of cyclin D1 and SOX-11 is useful for differentiating mantle cell lymphoma from other appendiceal lymphomas and treating patients appropriately. Physicians and nursing staff should be also aware of the associated complications and management in these patients.

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Inflammatory fibroid polyp: a rare benign tumor of the alimentary tract in children presenting as intussusception-case report and review of literature.

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