Neurofibromatosis associated with malignant neurofibromas

Knight, W. A.

doi:10.1001/archderm.107.5.747

Cited by 17 publications

(4 citation statements)

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“…Although rare, MM has been reported from several sites including leptomeningeal and cutaneous, with ocular MM being the most common in NF‐1 patients 1 . Cutaneous MM has been reported to occur in 0.1% to 5.4% of NF‐1 patients with approximately 26 cases reported in the literature mostly as individual observation or brief series 1,5–7,10–23 . The association between MM and NF‐1 was described for the first time in 1934 by Bjorneboe 4 .…”

Section: Discussionmentioning

confidence: 99%

Fatal malignant melanoma in a child with neurofibromatosis type 1

Amer

Al-Khenaizan

2007

Int J Dermatology

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Neurofibromatosis type 1 is an autosomal dominant disease and is considered one of the most commonly inherited diseases in humans. Malignant melanoma has been reported in up to 5% of patients with neurofibromatosis type 1. We report a young Saudi boy with neurofibromatosis type 1 who developed fatal metastatic malignant melanoma arising from giant melanocytic nevi within speckled lentiginous nevus (SLN).

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Section: Discussionmentioning

confidence: 99%

Fatal malignant melanoma in a child with neurofibromatosis type 1

Amer

Al-Khenaizan

2007

Int J Dermatology

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“…From 1973 through 2000, only 12 similar cases of malignant tumor of the pancreas associated with VRD were reported; our patient is the thirteenth such case (Table 1). 12,[16][17][18][19][20][21][22][23][24][25][26] Histologically, the majority of the tumors were tubular or papillary adenocarcinomas (69%; 9/13). Of the remainder, 2 (15%) were malignant endocrine tumors, 1 (8%) was serous cyst adenocarcinoma, and 1 was a neurofibrosarcoma.…”

Section: Discussionmentioning

confidence: 99%

Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease

Fujisawa

Osuga

Maeda

et al. 2002

Journal of Gastroenterology

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We describe a case of malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease. A 66-year-old woman with familial von Recklinghausen's disease was admitted to our hospital with weight loss and lower abdominal pain. Endoscopy revealed a submucosal tumor in the second part of the duodenum. Contrast-enhanced computed tomography showed a heterogeneous low-density tumor, measuring 20 x 15 mm, in the head of the pancreas, and metastatic lymphadenopathy. Angiography showed faint tumor staining without encasement or apparent increase in vascularity. Pancreatoduodenectomy was performed. Macroscopically, there was a solid tumor, measuring 20 x 18 mm, in the head of the pancreas. Histologically, a malignant endocrine tumor was shown, with direct invasion to the duodenum, and lymph node metastases. This is the thirteenth case of pancreatic malignant tumor and the fourth case of pancreatic endocrine tumor associated with von Recklinghausen's disease reported in the world literature.

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“…Both sexes and all races appear to be affected equally 1 ) . The clinical features of this disorder invariably include cafe-au-lait spots, multiple neurofibromas, lisch nodules or pigmented iris hamartomas 1 , 3 , 4 ) . The Schwann cell most likely is the cell of origin, but some may originate from perineurium or endoneurium 5 ) .…”

Section: Discussionmentioning

confidence: 99%

Intrathoracic Malignant Peripheral Nerve Sheath Tumor in von Recklinghausen`s Disease

Park¹,

Choi²,

Lee³

et al. 2001

Korean J Intern Med

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Malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. MPNST accounts for about 5–10% of all soft tissue tumors and is often associated with neurofibromatosis type I (NF-1, von Recklinghausen’s disease). It is one of the malignant tumors associated with von Recklinghausen’s disease. Its common site is the lower and upper extremities, trunk, head and neck. But intrathoracic manifestations are very rare. We report a case of a 40 year-old man with multiple neurofibromatosis who was presented with an intrathoracic malignant peripheral nerve sheath tumor.

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Neurofibromatosis associated with malignant neurofibromas

Cited by 17 publications

References 0 publications

Fatal malignant melanoma in a child with neurofibromatosis type 1

Fatal malignant melanoma in a child with neurofibromatosis type 1

Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease

Intrathoracic Malignant Peripheral Nerve Sheath Tumor in von Recklinghausen`s Disease

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