Primary adenocarcinoma of the stomach associated with peripheral neurofibromatosis: Report of a case

Abstract

A 55-year old man with von Recklinghausen's disease was admitted to our hospital for investigation of weight loss, anorexia, and abdominal discomfort. Endoscopy findings revealed impairment in the extendability of the stomach by air, and barium meal studies showed a well-contracted stomach. On exploratory laparotomy, the whole of the stomach was found to be grossly shrunken with thickening of the walls suggesting a "linitis plastica" stomach. Histological findings confirmed stage III poorly differentiated diffuse infiltrative adenocarcinoma of the stomach. This report describes a rare case of "linitis plastica" stomach in a patient with peripheral neurofibromatosis (NF-1) indicating that the NF-1 disorder does not spare an individual from other common neoplastic disorders.

Totally Laparoscopic Gastrectomy for Gastric Cancer Associated with Recklinghausen's Disease

“…Some reports have also demonstrated an association of nonepithelial tumors in gastrointestinal tract [3]. In contrast, there have been a few reports of primary adenocarcinoma of the gastrointestinal tract, such as the stomach, small intestine, or colon [5–7]. The present study reports a rare case, which was associated with gastric cancer without neural tumors other than cutaneous neurofibromas.…”

“…This disease shows a high incidence of malignant tumors, but most of those are neural tumors [2, 3]. There have been a few reports of gastrointestinal cancer with Recklinghausen's disease [4–6], and gastric cancer is rare as well [7]. In the surgical treatment of these cases, the skin incision may be limited by the presence of cutaneous neurofibromas.…”

A rare malignancy in a patient with neurofibromatosis: Gastric adenocarcinoma.

“…In a few cases concomitancy between neurofibromatosis, and gastric adenocarcinoma has been reported [6]. In neurofibromatosis patients with gastrointestinal tumors signs, and symptoms as abdominal pain, bleeding, and manifestations of intussusception are present, and most of the cases are incidentally detected during laparotomies performed for other indications, postmortem examinations, and gastrointestinal barium series [7].…”

Benign giant mediastinal schwannoma presenting as cardiac tamponade in a woman: a case report

“…GISTs are the most common of these forms [1]. Although there have been scattered reports of adenocarcinoma of the GI tract complicating peripheral neurofibromatosis [1,3], few cases have explored the association of primary infiltrating adenocarcinoma of the stomach [5]. It has been indicated that loss of function of the NF-1 gene results in peripheral neurofibromatosis [6], but no definite reasons have been cited for the high incidence of primary malignant tumors in these patients.…”