1/53. Plexiform neurofibroma of the cauda equina. Case report.Plexiform neurofibroma of the cauda equina has been reported only twice previously. The authors report the first pediatric patient in whom such a tumor has been found. A 4-year-old boy presented with low-back pain that radiated bilaterally into the L-4 and L-5 dermatomes. A dermal sinus noted at the midthoracic level was surrounded by a hemangiomatous lesion. magnetic resonance imaging confirmed the presence of the dermal sinus and revealed a well-defined lumbosacral mass that showed heterogeneous intensity with irregular enhancement. Intraoperatively, a solid mass, which engulfed the entire cauda equina, could not be dissected from the roots. The dermal sinus tract, however, was excised from the thoracic spine. The patient underwent radiotherapy to control the tumor and relieve his pain. Plexiform neurofibromas of the cauda equina are characterized by an insidious and progressive clinical course. The tumor mass may engulf all the roots of the cauda equina. No plexiform neurofibroma of the cauda equina has been reported to be associated with neurofibromatosis Type 1. The authors assume that the thoracic-level dermal sinus observed in this child was an incidental finding.- - - - - - - - - - ranking = 1keywords = neurofibromatosis (Clic here for more details about this article) |
2/53. Plexiform schwannoma of the cheek.We present a case of subcutaneous plexiform schwannoma, a rare benign peripheral nerve sheath tumour characterized by a multinodular and plexiform growth pattern. A review of the literature was performed to identify the relationship between plexiform schwannoma and neurofibromatosis types 1 and 2, and schwannomatosis. It is also important to distinguish plexiform schwannoma from plexiform neurofibroma, a particular type 1 neurofibromatosis lesion, because of the propensity of the latter for malignant degeneration.- - - - - - - - - - ranking = 2.00004007942keywords = neurofibromatosis, peripheral (Clic here for more details about this article) |
3/53. Surgical debulking of eyelid and anterior orbital plexiform neurofibromas by means of the carbon dioxide laser.PURPOSE: To present the carbon dioxide laser as an effective tool for surgical debulking of eyelid and orbital neurofibromas. METHOD: Two patients with neurofibromatosis underwent surgical debulking of their eyelid and orbital plexiform neurofibromas by means of the carbon dioxide laser. RESULTS: Acceptable cosmetic results were obtained with the removal of eyelid and orbital neurofibromas with improved hemostasis and minimal destruction of surrounding tissue when compared with conventional methods of removal. CONCLUSIONS: The carbon dioxide laser may allow significant improvement in the removal of plexiform neurofibromas.- - - - - - - - - - ranking = 1keywords = neurofibromatosis (Clic here for more details about this article) |
4/53. CO2-laser in the removal of a plexiform neurofibroma from the eyelid.We present a child with neurofibromatosis type I (NF-I) who underwent excision of a large upper lid neurofibroma using a CO2-laser. Plexiform neurofibromas are notorious for their bleeding tendency and the inability to achieve complete surgical excision. The CO2-laser is an adjunct to achieving better hemostasis and delineation of the tissue in the absence of natural tissue planes caused by the tumor's diffuse mode of growth. Using the CO2-laser achieves better cosmesis, while reducing operation time and limiting complications.- - - - - - - - - - ranking = 1keywords = neurofibromatosis (Clic here for more details about this article) |
5/53. Plexiform neurofibroma of the submandibular gland.A rare plexiform neurofibroma of the submandibular gland in a patient with neurofibromatosis is presented. The clinical manifestations of the disease are reviewed. The need for early diagnosis of neurofibroma is emphasized because of malignant transformation.- - - - - - - - - - ranking = 1keywords = neurofibromatosis (Clic here for more details about this article) |
6/53. Isolated bilateral orbital neurofibromatosis in a twelve-year-old.A patient with bilateral orbital neurofibromatosis is described. This patient had no other dysmorphic features and no systemic stigmata indicative of Von Recklinghausen's disease or endocrine syndromes. To our knowledge, this is the first report in the literature of isolated bilateral orbital neurofibromatosis in an otherwise normal patient.- - - - - - - - - - ranking = 6keywords = neurofibromatosis (Clic here for more details about this article) |
7/53. Diffuse, retroperitoneal mesenteric and intrahepatic periportal plexiform neurofibroma in a 5-year-old boy.We present a case of plexiform neurofibroma involving the retroperitoneum, mesentery, and liver in a 5-year-old boy who underwent evaluation for extent of a palpable left neck mass. The mass had intrathoracic extension with great vessel encasement and extension into the abdomen. Abdominal CT revealed a diffuse low-attenuation non-enhancing mass encasing the retroperitoneal vessels with serpiginous extension into the liver along the portal vein. This spread pattern of plexiform neurofibroma is an unusual manifestation of neurofibromatosis in a young child.- - - - - - - - - - ranking = 1keywords = neurofibromatosis (Clic here for more details about this article) |
8/53. Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.- - - - - - - - - - ranking = 5.00004007942keywords = neurofibromatosis, peripheral (Clic here for more details about this article) |
9/53. Plexiform neurofibroma of the submandibular salivary gland in a child.Plexiform neurofibromas in major salivary glands are rarely described. In the literature, most reported tumours have been present in the parotid gland region. A three-year-old boy with a family history of neurofibromatosis presented with a rapidly growing left submandibular mass. The clinical diagnosis was that of a neurofibroma rather than a primary salivary gland tumour. Resection of the lesion revealed a plexiform neurofibroma involving the submandibular gland. Although these tumours have a neurogenic rather than a salivary gland origin, they must be considered in the differential diagnosis of a salivary gland lesion in a patient with a history of neurofibromatosis.- - - - - - - - - - ranking = 2keywords = neurofibromatosis (Clic here for more details about this article) |
10/53. Segmental colonic involvement of plexiform neurofibroma in neurofibromatosis type 1.In a 36-year-old man with neurofibromatosis type 1, rare colonic involvement of plexiform neurofibroma is presented. The diagnosis was confirmed by operation. Radiologic findings consisted of marked concentric thickening of the colonic wall with variable attenuation, namely, a "multilayer appearance," as well as clusters of multiple soft tissue nodules in the mesentery.- - - - - - - - - - ranking = 5keywords = neurofibromatosis (Clic here for more details about this article) |
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