vascularity of neurofibroma

Neurofibromas predominately consist of Schwann cells and fibroblasts, which mainly originate from neurofibromatosis type 1. However, solitary cervical vagal nerve neurofibroma (VNN) has rarely been reported in previously published literature. Additionally, the onset of VNN is characteristically chronic, asymptomatic, and insidious, and is often discovered unexpectedly through physical examination or imaging. The exact etiology and pathogenesis of VNN are yet to be clarified and need further exploration. Consequently, the definitive diagnosis of VNN mainly depends on pathological and immunohistochemical examinations...

BACKGROUND: Xeroderma pigmentosum (XP) is a rare genodermatosis with a propensity to develop malignant skin tumors lifelong. METHODS: In this retrospective study, 24 XP patients were evaluated with regard to frequency and clinicopathological features of benign and malignant skin tumors. RESULTS: Seventeen patients had at least one malignant skin tumor diagnosed; basal cell carcinoma in 13 patients (n = 72), basosquamous carcinoma in three patients (n = 4), squamous cell carcinoma in six patients (n = 13), keratoacanthoma in three patients (n = 15) and melanoma in six patients (n = 18)...

BACKGROUND AND OBJECTIVE: Clinicopathological features of cutaneous neurofibromas presenting as large irregularly shaped congenital café-au-lait macules (CALM) in Neurofibromatosis type 1 (NF1) patients have not been well characterized. We aimed to analyze the histopathological findings of large "atypical" CALM in children with NF1. PATIENTS AND METHODS: In this retrospective observational study we analyzed histopathological and immunostaining features of 21 biopsy specimens from 18 large hyperpigmented macules with irregular borders with or without hypertrichosis present during the first months of life in NF1 diagnosed children...

BACKGROUND Type 1 neurofibromatosis (NF1) is known to be associated with not only neurogenic tumors but also gastrointestinal (GI) neoplasms. However, there are few reports on vascular lesions and the incidence is unknown. CASE REPORT We report here the case of a 45-year-old woman with a history of NF1 referred to our hospital for the purpose of detailed examination for positive fecal occult blood test. On the basis of the investigation reports, she was diagnosed with a neuroendocrine tumor (NET)-G1. We planned a subtotal stomach-preserving pancreaticoduodenectomy...

BACKGROUND: Primary intradural extramedullary (IDEM) lesions are rare, with an incidence of about 1/100,000 person-years. The aim of this study was to investigate their demographic, clinical, imaging, management, histopathological and outcome parameters. Another objective was to evaluate the various predicting factors leading to long-term favorable outcomes, thereby answering the controversial question: when to operate? METHODS: This study observed 212 patients of primary IDEM lesions and followed-up for a mean of 53...

OBJECTIVE. Loss of the neurofibromatosis type 1 (NF1) tumor suppressor protein causes uninhibited activation of the RAS oncogene, which leads to tumorigenesis in patients with NF1. This case-based review discusses imaging manifestations of NF1 in the abdomen and pelvis, highlighting key genetic associations and management to elucidate features different from the general population. CONCLUSION. The spectrum of pathologic findings includes gastrointestinal tumors such as gastrointestinal stromal tumors, genitourinary lesions including urogenital neurofibromas, vascular entities such as renal artery stenosis, and less common associations like lymphoma...

Neurofibroma is an uncommon benign tumor arising from nerve sheath fibroblasts. The diagnosis of solitary lesions becomes difficult in patients who do not have any family history of neurofibroma. An intra-oral solitary neurofibroma comprises 6.5% of reported cases of neurofibroma. Few cases of intraosseous solitary neurofibroma have been published as per literature. Treatment-surgical resection of tumor has an excellent prognosis with extreme rare malignant transformation which is more commonly seen in neurofibromatosis...

BACKGROUND: Neurofibromatosis type 1 (NF1) is a hereditary disease that causes neurofibromas generally, but it has been reported to sometimes be associated with various forms of blood vessel stenosis, occlusion and vascular abnormalities of unknown mechanism. However, a symptomatic case with simultaneous ophthalmic artery stenosis and internal carotid artery stenosis is an extremely rare pathogenesis in a child with NF1. In this report, we performed the diagnosis and observation using various imaging modalities for this rare pediatric case...

Carney complex (CNC) is a rare, autosomal dominant multiple neoplasia syndrome. Although cutaneous myxomas commonly occur in CNC patients, intraoral myxomas are extremely rare. We present a case of a palatal myxoma in a 21-year-old female patient with CNC, along with a review of the pertinent literature. She presented with a sessile nodule on the hard palate that microscopically showed a multilobulated and highly vascularized myxomatous tissue composed of loosely-arranged spindle, polygonal, and stellate cells, suggestive of myxoid neurofibroma...

Osseous manifestations of neurofibromatosis 1 (NF-1) occur in a minority of the affected subjects but may be because of significant clinical impairment. Typically, they involve the long bones, commonly the tibia and the fibula, the vertebrae, and the sphenoid wing. The pathogenesis of NF-1 focal osseous lesions and its possible relationships with other osseous NF-1 anomalies leading to short stature are still unknown, though it is likely that they depend on a common mechanism acting in a specific subgroup of NF-1 patients...

Neurofibromatosis (NF) is a common autosomal dominant disorder that can be subdivided into type 1, type 2, and schwannomatosis. Patients with NF1 typically develop café-au-lait spots, scoliosis, and benign neurofibromas. In addition, NF1 predisposes to vascular complications including stenosis, arterial ectasia, and aneurysms. Here, we report the case of an otherwise healthy 32-year-old man who developed a fatal tension hemothorax due to vertebral artery aneurysm rupture. Based on the available literature, we discuss the presentation, workup, and available therapeutic approaches to this complication of neurofibromatosis...

INTRODUCTION: Transradial access is increasingly used among neurointerventionalists as an alternative to the transfemoral route. Currently available data, building on the interventional cardiology experience, primarily focus on right radial access. However, there are clinical scenarios when left-sided access may be indicated. The purpose of this study was to evaluate the technical feasibility of left transradial access to cerebral angiography across three institutions. METHODS: A retrospective chart review was performed for patients who underwent cerebral angiography accessed via the left radial artery at three institutions between January 2018 and July 2019...

Resection of giant neurofibroma in neurofibromatosis type 1 has a high risk of perioperative bleeding because the tumors are enriched in blood vessels, which are weakened due to the loss and thinning of vascular smooth muscle. Therefore, we combined skin ligation around the tumor and use of an argon beam coagulator (ABC) for hemostasis during resecting the giant neurofibroma. The ABC is a non-contact-type hemostasis device employing argon gas as a medium. We examined the usefulness of our method by retrospectively comparing the outcomes of the ABC-use group with those of the non-use group (7 patients, 9 tumors)...

Hashimoto's thyroiditis is a common form of chronic autoimmune thyroid disease (AITD) and it often coexists with other autoimmune diseases, but Hashimoto's thyroiditis associated with an autosomal dominant neurofibromatosis type 1 is exceedingly rare. A 30-year-old woman presented with complaints of headache for 1 year on and off. Physical examination revealed nodular swelling in the neck, cafe-au-lait spots, and neurofibromas covering the entire surface of her body. Her thyroid hormones were within normal limits...

Vascular tumors of spine range from benign hemangiomas to malignant angisarcomas. Hemangioendotheliomas of spine are tumors of intermediate grade malignancy with rare occurrence in the intradural location. The imaging and histopathology may mimic other common lesions occurring at this location. A 70 year old lady presented with lower limb weakness and sensory impairment along L5-S1 dermatome. With the radiological diagnosis of neurofibroma at the intradural location of L4-L5 level, total excision of the space occupying lesion was done...

Purpose: The aim of this study was to review the common conditions associated with mandibular canal widening. Materials and Methods: General search engines and specialized databases including Google Scholar, PubMed, PubMed Central, Science Direct, and Scopus were used to find relevant studies by using the following keywords: "mandibular canal," "alveolar canal," "inferior alveolar nerve canal," "inferior dental canal," "inferior mandibular canal," "widening," "enlargement," "distension," "expansion," and "dilation...

Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen's disease is an autosomal dominant genetic disorder. It is the most common of phacomatoses. Pulmonary complications have been rarely described in the literature. It is generally a benign disease, but has the potential for rare and fatal complications, such as spontaneous hemothorax. We reported two cases of patients with a history of von Recklinghausen's disease. Both of them suffered sudden chest pain. Chest-X ray revealed a hemi-thoracic opacity...

BACKGROUND Von Recklinghausen's disease, also known as Type 1 neurofibromatosis (NF1), is a genetic disorder characterized by skin tumors, neurofibromas of multiple organs and vascular abnormalities. Spontaneous thoracic hemorrhage is a rare but potentially fatal consequence of this disorder. After a review of the literature over the last 10 years and on the basis of a case study, the aim of this study was to report the challenges of management of this pathology. CASE REPORT We report a rare case of a 45-years-old male with a medical history of neurofibromatosis who complained of a 3-day history of progressive dyspnea...

Accumulating evidence has demonstrated the significant progression of cutaneous neurofibroma (cNF) without necrosis during puberty. However, the molecular events involved in this process remain unclear. The alteration of the steroid hormone levels during puberty has led to the investigation of the expression levels of the androgen receptor (AR). A positive correlation between AR expression and microvessel density has been reported in human cNF tissues in combination with enhanced endothelial cell tube formation in vitro...

PURPOSE: To review the pertinent anatomy and the imaging features of common and uncommon benign and malignant neoplasms and masses of the ischiorectal fossa. RESULTS: The ischiorectal or ischioanal fossa is the largest space in the anorectal region. The benign neoplasms that develop in the ischiorectal originate from the different components that forms the fossa including vascular tumors such as aggressive angiomyxoma or hemangioma; neural tumors as plexiform neurofibroma or schwannoma; fat tumors as lipoma; skin/skin appendages tumors as hidradenoma papilliferum; smooth or skeletal muscle tumors as solitary fibrous tumor...