Literature DB >> 22728486

A case of neurofibromatosis type 1 coinciding with bilateral pheochromocytomas, multiple gastrointestinal stromal tumors, and malignant peripheral nerve sheath tumor.

Yuji Hataya1, Yasato Komatsu, Keisuke Osaki, Yorihide Fukuda, Teruyuki Sato, Taisuke Morimoto.   

Abstract

Neurofibromatosis type 1 (NF1) is associated with benign and malignant neoplasms, but the coincidence of abdominal neoplasms is rare. A 65-year-old woman with NF1 had episodes of nausea, tachycardia, hypertension, and loss of consciousness. Bilateral adrenal tumors were detected by abdominal computed tomography, and plasma and urinary catecholamine levels were elevated. Open bilateral adrenalectomy and histological findings revealed bilateral pheochromocytomas (PCCs). Furthermore, malignant peripheral nerve sheath tumor (MPNST) and multiple gastrointestinal stromal tumors (GISTs) were incidentally found in the abdominal cavity. Early diagnosis of abdominal neoplasms in NF1 patients is important because of the risk of malignancy, organic complications and hemorrhagic-obstructive complications.

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Year:  2012        PMID: 22728486     DOI: 10.2169/internalmedicine.51.6385

Source DB:  PubMed          Journal:  Intern Med        ISSN: 0918-2918            Impact factor:   1.271


  2 in total

Review 1.  Pheochromocytoma and gastrointestinal stromal tumours in an adult neurofibromatosis type 1 patient: a rare co-occurrence.

Authors:  Nuttawut Vongsumran; Sarawut Kongkarnka; Pittaporn Watanawittawas; Worapaka Manosroi
Journal:  BMJ Case Rep       Date:  2020-06-28

2.  Unexpected discovery of a diffuse astrocytoma of the conus medullaris in an elderly NF1 patient.

Authors:  Takamistu Uchi; Tsutomu Inaoka; Noriko Kitamura; Rumiko Ishikawa; Tomoya Nakatsuka; Shusuke Kasuya; Wataru Tokuyama; Nobuyuki Hiruta; Hiroshi Takahashi; Hitoshi Terada
Journal:  Radiol Case Rep       Date:  2020-04-18
  2 in total

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