We present a case of laryngeal neurofibroma associated with neurofibromatosis type 2. Although laryngeal neurofibromas have previously been reported in cases of neurofibromatosis type 1, their presence has never been described in a patient with neurofibromatosis type 2.

the association of osteomalacia with neurofibromatosis is a very rare entity. here a 34 year old man, a known case of neurofibromatosis, is reported who presented with bone pain, hypophosphatemia, rena] phosphorus wasting, multiple looser's pseudofractures, and low bone density. treatment with high dose calcitriol and phosphate resulted in temporary treatment. permanent cure was achieved a...

Neurofibromatosis type 1 (NF-1), first described by Von Recklinghausen (1882), is an autosomal dominant disease caused a spectrum of mutations in NF-1 gene. We reported case giant facial neurofibromas 26 years old male patient without family history neurofibromatosis 1. Features typical neurofibroma, including enlarged nerve fascicle composed elongated nuclei and scant cytoplasmic cells, were i...

Neurofibromatosis is an autosomal dominant disease that is characterized by cutaneous hyperpigmentation and multiple neurofibromas and often accompanied by localized peripheral and central neural tumors. Gastrointestinal involvement is seen in 25% of the cases. Gastrointestinal stromal tumors, pancreatic and duodenal neuroendocrine tumors are the most common tumors in patients with neurofibroma...

Two siblings from a family in which neurofibromatosis was inherited as an autosomal dominant had hypertrophic cardiomyopathy and neurofibromatosis. Idiopathic hypertrophic cardiomyopathy may have occurred by chance in two first degree relatives with neurofibromatosis. An alternative explantation is that these diseases are both manifestations of a common hereditary defect of neural crest tissue....

Neurofibromatosis type 2 is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumors in the region of the cranial nerve VIII usually as schwannoma. We report here a 20-year-old woman with primary intracranial malignant nerve sheath tumor located in the left cerebellopontine angle. Histologically, the tumor showed malignant spindle c...

Neurofibromatosis is a genetic disorder manifested by characteristic cutaneous lesions called neurofibromas. There are two distinct neurocutaneous syndromes named neurofibromatosis type 1 (also called von Recklinghausen disease or NF1) and neurofibromatosis type 2 (NF2). NF1 is by far the most common presentation and is caused by an autosomal dominant mutation in the NF1 gene mapped to chromoso...

Neurofibromatosis (NF) is a genetic and multisystemic disease with autosomal dominant transition. It can affect anesthesia applications by affecting more than one system. In the presence of neurofibromas in airway, it cause airway obstruction difficulties respiratory delivery during general anesthesia. The tumors central nervous system makes spinal risky. Anesthesiologists should act awareness ...

a 61-year-old female presented with complaints of fever, general weakness and hepatosplenomegaly. she had a history of nonfamilial peripheral neurofibromatosis diagnosed as von recklinghausen's disease since 30 years previous. physical examination was remarkable for skin colored cutaneous circumscribed nodules which appeared soft to the touch in both arms, the upper part of her abdomen, back, a...

General introduction to the neurofibromatoses The diseases traditionally known as neurofibromatosis have now been formally separated into two types: neurofibromatosis type 1 or NFl (the type described by von Recklinghausen) and neurofibromatosis type 2 or NF2 (a much rarer form).' It is now recognised that although they have overlapping features, including an inherited propensity to neurofibrom...