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Intestinal neurofibromatosis in von Recklinghausen's disease: presenting as chronic anemia due to recurrent intestinal hemorrhage.

Hahn JS, Chung JB, Han SH, Lee SW, Noh SH, Lee JT, Chun SI, Kim GH - Korean J. Intern. Med. (1992)

Bottom Line: Neurofibromatosis (von Recklinghausen's disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities.The involvement of gastrointestinal tract in neurofibromatosis is not common.The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

ABSTRACT
Neurofibromatosis (von Recklinghausen's disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities. The involvement of gastrointestinal tract in neurofibromatosis is not common. The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain. We experienced a case of intestinal neurofibroma in von Recklinghausen's disease. The patient was a 39 year-old female who had suffered from chronic iron deficiency anemia and recurrent gastrointestinal hemorrhage due to two neurofibromas of jejunum for 3 years, which was diagnosed by superior mesenteric and ileal arteriogram and 99mTc pertechnetate-labelled RBC scan, and treated by segmental resection of jejunum with end to end anastomosis.

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The cut surface of the jejunal mass showing a gray-white myxoid appearance.
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f6-kjim-7-2-137-10: The cut surface of the jejunal mass showing a gray-white myxoid appearance.

Mentions: Segmental resection of jejunum with end to end anastomosis and cholecystectomy were performed. Also the skin biopsy on the left breast was performed during the operation. The pathologic diagnosis was neurofibromas of the jejunum (Fig. 5–, 8) and the skin (Fig. 9). The patient has been in good health without intestinal bleeding until now.


Intestinal neurofibromatosis in von Recklinghausen's disease: presenting as chronic anemia due to recurrent intestinal hemorrhage.

Hahn JS, Chung JB, Han SH, Lee SW, Noh SH, Lee JT, Chun SI, Kim GH - Korean J. Intern. Med. (1992)

The cut surface of the jejunal mass showing a gray-white myxoid appearance.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4532108&req=5

f6-kjim-7-2-137-10: The cut surface of the jejunal mass showing a gray-white myxoid appearance.
Mentions: Segmental resection of jejunum with end to end anastomosis and cholecystectomy were performed. Also the skin biopsy on the left breast was performed during the operation. The pathologic diagnosis was neurofibromas of the jejunum (Fig. 5–, 8) and the skin (Fig. 9). The patient has been in good health without intestinal bleeding until now.

Bottom Line: Neurofibromatosis (von Recklinghausen's disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities.The involvement of gastrointestinal tract in neurofibromatosis is not common.The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

ABSTRACT
Neurofibromatosis (von Recklinghausen's disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities. The involvement of gastrointestinal tract in neurofibromatosis is not common. The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain. We experienced a case of intestinal neurofibroma in von Recklinghausen's disease. The patient was a 39 year-old female who had suffered from chronic iron deficiency anemia and recurrent gastrointestinal hemorrhage due to two neurofibromas of jejunum for 3 years, which was diagnosed by superior mesenteric and ileal arteriogram and 99mTc pertechnetate-labelled RBC scan, and treated by segmental resection of jejunum with end to end anastomosis.

Show MeSH
Related in: MedlinePlus