Introduction: Plexiform Fibromyxoma (PF), also known as Plexiform Angiomyxoid Myofibroblastic Tumour (PAMT) is a rare mesenchymal myxoid tumour originating in different areas of the digestive system. Symptoms and signs are usually elusive: the most common are abdominal discomfort, dyspepsia, nausea and gastrointestinal bleeding with anemia and hematemesis. Imaging tests are useful to identify plexiform fibromyxoma, but only pathology provides the definitive diagnosis and differentiate it from other mesenchymal tumours. PF seems to display a benign behaviour and the only complete surgical removal of the mass represents the gold standard of treatment; no recurrence or metastasis has been described so far. A little more than 100 cases have been described in adulthood with only 14 cases reported up to now in pediatrics. Case report and review: We report the case of a gastric plexiform fibromyxoma occurred in an 8-year old girl went to our observation with a story of dyspepsia, vomiting, weight loss and anemia. A 4 X 2.5 cm mass was found in the gastric antrum. The tumour was removed by laparoscopic antrectomy and the pathological exam was consistent with PF. A detailed review of PF pediatric literature is also provided. Keywords: plexiform fibromyxoma; gastric tumour; pediatrics; laparoscopic gastric resection.

Gastric plexiform fibromyxoma in children: A new case report and review of pediatric literature

Cristina Moglia;Filippo Parolini;Vincenzo Villanacci;Giuseppe di Gaetano;Della Casa Domenico;Giovanni Boroni;Daniele Alberti;
2021-01-01

Abstract

Introduction: Plexiform Fibromyxoma (PF), also known as Plexiform Angiomyxoid Myofibroblastic Tumour (PAMT) is a rare mesenchymal myxoid tumour originating in different areas of the digestive system. Symptoms and signs are usually elusive: the most common are abdominal discomfort, dyspepsia, nausea and gastrointestinal bleeding with anemia and hematemesis. Imaging tests are useful to identify plexiform fibromyxoma, but only pathology provides the definitive diagnosis and differentiate it from other mesenchymal tumours. PF seems to display a benign behaviour and the only complete surgical removal of the mass represents the gold standard of treatment; no recurrence or metastasis has been described so far. A little more than 100 cases have been described in adulthood with only 14 cases reported up to now in pediatrics. Case report and review: We report the case of a gastric plexiform fibromyxoma occurred in an 8-year old girl went to our observation with a story of dyspepsia, vomiting, weight loss and anemia. A 4 X 2.5 cm mass was found in the gastric antrum. The tumour was removed by laparoscopic antrectomy and the pathological exam was consistent with PF. A detailed review of PF pediatric literature is also provided. Keywords: plexiform fibromyxoma; gastric tumour; pediatrics; laparoscopic gastric resection.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11379/622078
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