1/8. A case of familial angiolipomatosis with Lisch nodules.Familial angiolipomatosis is a rare syndrome that may be confused clinically with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, although somewhat disfiguring, is a benign condition with no known association with malignant neoplasms. This is in contradistinction to neurofibromatosis, an autosomal dominant syndrome associated with a myriad of benign and malignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a prison inmate with a history of seizures and "neurofibromatosis" without clinical documentation. Lisch nodules were noted on the irides. Postmortem examination showed multiple subcutaneous yellow tumors on the chest and arms. Fine-needle aspiration of 1 mass yielded adipose tissue with prominent vessels; histologic sections of another mass showed angiolipoma. The remainder of the autopsy showed significant coronary artery disease and a remote cerebral infarction of the temporal lobe but no signs of neurofibromatosis. We feel that the presence of multiple angiolipomas in combination with Lisch nodules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/8. A case of multiple schwannomas of the trigeminal nerves, acoustic nerves, lower cranial nerves, brachial plexuses and spinal canal: schwannomatosis or neurofibromatosis?In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
3/8. Malignant fibrous histocytoma of bone in neurofibromatosis--a case report.Multiorgan neoplasms are encountered frequently in patients with neurofibromatosis, which is a genetic disorder. Though skeletal abnormalities are common in these patients, the occurrence of primary bone sarcomas is very rare. We hereby report a case of post-radiation malignant fibrous-histiocytoma of bone developing in an elderly patient with neurofibromatosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
4/8. Neurofibromatosis: a diagnostic mimicker on CT in a known case of malignancy.A known case of early carcinoma cervix was found to have mediastinal widening on chest radiograph and hypoechoic oval retroperitoneal lesions on USG abdomen. CECT chest and abdomen showed these to be non enhancing lymph node like round to oval discrete mass lesions in mediastinum, abdomen and pelvis. With no other suggestion of carcinoma spread, local or distant and uncommon incidence of extensive lymphadenopathy in a early carcinoma cervix, biopsy from one of the representative lesion was performed which revealed it to be benign neurofibroma. Differentiation of these strategically located benign nerve sheath tumors from lymphadenopathy can sometimes be challenging on CT scan and in a known case of malignancy or with history of surgery for malignant neoplasm it may cause concern for disease spread or local tumor recurrence. Associated imaging and clinical features can sometimes be helpful in reaching the correct diagnosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
5/8. Plexiform neurofibroma of the uterine cervix: a case report and review of the literature.The female genital system is rarely affected in von Recklinghausen neurofibromatosis. The vulva is the most frequent genital location, but vaginal, cervical, uterine, and ovarian neurofibromas have rarely been reported. We describe a case of plexiform neurofibroma affecting the uterine cervix in a patient with chronic pelvic pain and menorrhagia who had multiple cutaneous neurofibromas and 1 large paraspinal neurofibroma. A small plexiform neurofibroma, which was not grossly visible, was confined to the uterine cervix and coexisted with a uterine leiomyoma and adenomyosis. There were no neurofibromas in the myometrium, fallopian tubes, or ovaries. Plexiform neurofibroma is a neoplasm that should be considered in the differential diagnosis of spindle cell neoplasms of the uterine cervix, especially in specimens from patients with neurofibromatosis.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/8. Acoustic neuroma in an adolescent without neurofibromatosis: case study.When acoustic neuromas are found in younger age groups, they are typically bilateral and associated with neurofibromatosis (NF). A unilateral acoustic neuroma in a child or adolescent without NF is rare. We report audiometric, auditory brainstem response (ABR) and magnetic resonance imaging (MRI) data for a 15-year-old male with an acoustic neuroma but without associated NF. The patient was seen for audiologic assessment on three separate occasions over a period of 5 years. The first two assessments produced unremarkable audiometric and immittance data. The third assessment, subsequent to a failed school hearing screening, demonstrated a flat unilateral sensorineural hearing loss, unilateral absent acoustic reflexes, and abnormal bilateral ABR recordings. MRI with contrast demonstrated a unilateral mass extending out of the internal auditory meatus. Microscopic examination of the removed tumor confirmed a vestibular schwannoma. An enhanced MRI 3 years post neuroma removal demonstrated no evidence of tumor regrowth and no evidence of other neoplasms. ABR recordings for the uninvolved ear continued to demonstrate neural synchrony as evidenced by normal absolute wave latencies; however, the III-V and I-V interwave latencies remained extended beyond the 99th percentile. Postoperatively, the young man was fitted with a high gain in-the-ear hearing aid in the involved ear, which he has continued to wear on a daily basis for the past 3 years.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
7/8. Multiple cutaneous plexiform schwannomas. Report of a case and review of the literature with particular reference to the association with types 1 and 2 neurofibromatosis and schwannomatosis.Plexiform schwannomas are relatively rare, benign peripheral nerve sheath tumors, which usually arise in either the dermis or subcutaneous tissue, although rare cases originate in skeletal muscle or other deep somatic soft tissue sites. These tumors may occur singly or as multiple lesions and may be localized to one anatomic site or diffusely distributed. Rare cases have been associated with "schwannomatosis" as well as type 1 neurofibromatosis (von Recklinghausen's disease). We report an unusual case of multiple cutaneous plexiform schwannomas associated with bilateral acoustic neuromas as well as other intracranial and intraspinal neoplasms. In addition, we examine the relationship between the various forms of cutaneous schwannoma, particularly the plexiform variant, and both types 1 and 2 neurofibromatosis; we also examine several purported cases of schwannomatosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
8/8. Sarcomatosis of leptomeninges, brain and spinal cord coexisting with von Recklinghausen's neurofibromatosis. A case report and review of the literature.A case of 56-year-old male with sarcomatosis of leptomeninges as well as of the brain and spinal cord coexisting with Recklinghausen's neurofibromatosis is presented. Neurological and neurophysiological symptoms of the disease resembled those of amyotrophic lateral sclerosis (ALS). Patient died 7 months after onset of the initial symptoms. The post-mortem examination revealed neoplastic infiltration of the leptomeninges of brain and spinal cord. Histologically sarcomatosis of the leptomeninges was diagnosed and immunohistochemical analysis of the neoplastic infiltrates can indicate fibrohistiocytic origin of the neoplasm, suggesting also a probable contribution of perineurial cells in the pathogenesis of the tumor. On the grounds of the performed immunohistochemical study together with a review of the literature, the differential diagnosis of malignant mesenchymal tumors of the CNS is discussed with a special regard to their histogenesis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |