| 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. ( info) |
2/75. Plexiform neurofibroma of the small bowel infiltrated with metastatic adenocarcinoma. Neurofibromatosis Type 1 (NF1) is not classically associated with gastrointestinal manifestations although these patients are at increased risk of several GI complications. We describe the ultrasound, CT and barium findings in a patient with NF1 who had a huge benign plexiform neurofibroma of the ileum that was infiltrated with metastatic adenocarcinoma. ( info) |
3/75. Massive plexiform neurofibroma of the sympathetic trunk. A case report of a massive plexiform neurofibroma of the sympathetic trunk is reported. Symptoms included dysphagia, shortness of breath and paraesthesia of the right hand. The nerve of origin and extent of the tumour were unusual. The tumour mass partially filled the right hemithorax, surrounded the right brachial plexus, extended to the base of skull and invaded the cervical vertebrae threatening to compromise the spinal cord. Surgical resection required combined cardiothoracic, neurosurgical and plastic and reconstructive surgical teams. Early outcome following surgery was excellent. This case illustrates the need for a combined surgical approach for such a lesion and we believe it is the first reported case of plexiform neurofibroma of the sympathetic trunk. ( info) |
4/75. 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. ( info) |
5/75. 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. ( info) |
6/75. 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. ( info) |
| 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. ( info) |
8/75. Giant plexiform neurofibroma of the back. Complete excision of a giant neurofibroma can be technically difficult. Thorough preoperative planning with magnetic resonance imaging, computed tomography, and arteriography are indicated to define the extent of the mass and to facilitate operative planning. By following the treatment guidelines discussed in this case report, the authors feel that these tumors can be excised safely with minimal morbidity. ( info) |
9/75. 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. ( info) |
10/75. 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. ( info) |