Neurofibrosarcoma is the most common malignant change in patients with neurofibromatosis. But the incidence of neurofibrosarcoma arising from cutaneous neurofibroma is very low. We report two cases of neurofibrosarcoma arising from cutaneous neurofibroma in patients with neurofibromatosis showing typical clinical manifestations.
Humans ; Incidence ; Neurofibroma* ; Neurofibromatoses* ; Neurofibrosarcoma*

Plexiform schwannoma is a rare benign tumor arising from the peripheral nerve sheath and characterized by a multinodular and plexiform growth pattern. This tumor usually arises sporadically. In rare cases, plexiform schwannomas have been associated with neurofibromatosis type 2. Plexiform schwannoma should be differentiated from plexiform neurofibroma, because the latter is pathognomonic tumor of neurofibromatosis type 1 and has a potential of malignant transformation. We report a case of multiple plexiform schwannomas associated with bilateral acoustic neuromas and meningioma.
Meningioma ; Neurilemmoma* ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1 ; Neurofibromatosis 2* ; Neuroma, Acoustic ; Peripheral Nerves

No abstract available.
Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.
Abdominal Wall ; Humans ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibrosarcoma ; Peripheral Nerves ; Prognosis ; Sarcoma

No abstract available.
Humans ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Neurofibroma* ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1

Neurilemoma is a pure proliferation of Schwann cell and its ocular symptoms depend on the location of the tumor and its size. Neurofibromatosis is a multisystemic disease and has two distinct form : Neurofibromatosis type 1 described as peripheral neurofibromatosis and type 2 as central or bilateral acoustic neurofibromatosis. We experienced a case of orbital neurilemoma associated with neurofibromatosis in a 12-year-old male patient who presented with left orbital mass. We report this case with literature review.
Child ; Humans ; Male ; Neurilemmoma* ; Neurofibroma* ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibromatosis 2 ; Orbit*

Plexiform neurofibroma is a benign proliferation that arises from the peripheral nerves and represents one of the diagnostic features of neurofibromatosis type I (NF-1). They are commonly found in the gastrointestinal tract, and intrathoracic neurofibroma is relatively uncommon. Ultimately, this tumor grows along the length of any single nerve and may involve multiple fascicles or branches of major nerves. Here, we report a case of multiple-site plexiform neurofibromas in a 40-year-old man previously diagnosed with NF-1. Although he had no perceptible symptoms, contrast-enhanced CT revealed massive diffuse neurofibromas that involved the mediastinum, periportal region, retroperitoneal space, and the mesentery. Histopathological studies of the neck, skin, and intra-abdominal soft tissue showed loose spindle cells and collagen bundles. The microscopic appearance was typical of a plexiform-type neurofibroma. It was decided that we would observe the patient without surgical management, because the neurofibromas were asymptomatic.
Abdomen ; Adult ; Collagen ; Gastrointestinal Tract ; Humans ; Mediastinum ; Mesentery ; Neck ; Neurofibroma ; Neurofibroma, Plexiform ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nerves ; Retroperitoneal Space ; Skin

Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive upper gastrointestinal bleeding. We conclude that gastrointestinal bleeding and anemia in the setting of NF1 mandates complete endoscopic examination of the digestive tract to rule out neurofibromas. Surgical resection is the standard treatment.
Adolescent ; Anemia ; Gastrointestinal Tract ; Hemorrhage* ; Humans ; Male ; Neurofibroma* ; Neurofibroma, Plexiform ; Neurofibromatoses ; Neurofibromatosis 1* ; Peripheral Nervous System ; Skin ; Stomach

Congenital neurofibromatosis type 1, or Von Recklinghausen's disease is an autosomal dominant disorder characterized by neurofibroma, pigmented skin lesions (Cafe-au-lait macules), iris hamartomas and meningeal tumors, but rarely, by autonomic ganglia tumors, such as pheochromocytomas. We have experienced an intrathoracic and chest wall plexiform neurofibroma intimately contacted with collagenoma-like, dome-shaped skin lesions of type 1 neurofibromatosis, which are relatively rare and interesting, but can be regarded as typical findings in neurofibromatosis. Although intrathoracic neurogenic tumors are not uncommon, cases like ours are interesting, as the feature of collagenoma-like skin neurofibroma was very closely apposited with chest wall neurofibroma. Our case had no aypical features of malignancy and the patient was clinically followed up without recurrence.
Ganglia, Autonomic ; Hamartoma ; Humans ; Iris ; Meningeal Neoplasms ; Neurofibroma* ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1* ; Pheochromocytoma ; Recurrence ; Skin ; Thoracic Wall* ; Thorax*

No abstract available.
Neurofibroma* ; Neurofibromatoses*