Superficial Femoral Vein Invasion by a Benign Neurofibroma in a Non-neurofibromatosis Patient: Case Report

Neurosurgery ◽  
2006 ◽  
Vol 58 (5) ◽  
pp. E997-E997 ◽  
Author(s):  
Claude-Edouard Châtillon ◽  
Marie-Christine Guiot ◽  
Marc-Michel Corriveau ◽  
Line Jacques
Keyword(s):  
Femoral Vein ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Vascular Involvement ◽  
Surgical Removal ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
En Bloc ◽  
Superficial Femoral Vein ◽  
The Right

Abstract OBJECTIVE AND IMPORTANCE: Neurofibromas are benign neural sheath tumors arising from intraneural supporting cells. Such tumors are characteristic of neurofibromatosis Type I (von Recklinghausen disease) but also occur sporadically. Vascular involvement by neurofibromata is rare, but has been described in the past in the context of neurofibromatosis. There is, to our knowledge, no description of vascular involvement by a neurofibroma in a non-neurofibromatosis patient. CLINICAL PRESENTATION: A 40-year-old woman presented with a 4 year history of a right thigh mass associated with diffuse lower extremity pain. She had no other clinical manifestations of neurofibromatosis and no known family member with neurofibromatosis Type I. Magnetic resonance imaging scans revealed a well-defined solid mass in the anteromedial aspect of the right thigh closely associated with the superficial femoral vein. INTERVENTION: The vessel segment and encapsulated mass were resected “en bloc” after proximal and distal ligation of the vein. The pathological appearance of the mass was consistent with a benign neurofibroma that had infiltrated all layers of the vessel. CONCLUSION: Vessel invasion by a benign sporadic neurofibroma is a rare occurrence with potentially severe implications for the patient. It suggests that surgical removal of asymptomatic benign-appearing lesions of that type should be considered if they are adjacent to important anatomical structures.

scholarly journals Sepsis from Neurofibromatosis

2006 ◽  
Vol 33 (3) ◽  
pp. 328-328
Author(s):  
Navdeep Tangri ◽  
Shireen Sirhan ◽  
Gordon Crelinsten
Keyword(s):  
Autosomal Dominant ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Large Mass ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
Autosomal Dominant Disorder ◽  
Chest Wound ◽  
History Of ◽  
The Right

Neurofibromatosis Type I or von Recklinghausen’s neurofibromatosis is an autosomal dominant disorder with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. We present a case of sepsis secondary to an infected hematoma, enclosed within a massive neurofibroma.A 42-year-old man presented to the emergency department with a one week history of fever and chills. He reported an increase in pain and size of a growth near his chest. The patient was noted to be febrile on arrival. On physical examination, a very large neurofibroma was seen extending from the right upper chest. Wound and blood cultures were obtained. Computed Tomography (CT) of the thorax revealed a hematoma contained within the large mass.

scholarly journals Optic pathway gliomas associated with neurofibromatosis type I in children

2019 ◽  
Vol 18 (4) ◽  
pp. 29-38
Author(s):  
E. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
O. A. Medvedeva ◽  
Y. Y. Trunin ◽  
E. M. Tarasova ◽  
...  
Keyword(s):  
Survival Rate ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Type I ◽  
Optic Pathway ◽  
Neurofibromatosis Type I ◽  
National Medical ◽  
Optic Pathway Gliomas ◽  
Visual Disturbances

Neurofibromatosis type I (NFI) is one of the most common brain tumor predisposition syndromes. Children with NFI are prone to develop a low grade gliomas, which can be localized in various areas of the brain, however, most of them occur in the structures of the optic pathway: optic nerves, chiasm, tracts and optic radiations – that is, are optic pathway gliomas (OPG). This retrospective study included children with newly diagnosied low grade glioma of the optic pathway at the age from 0 to 18 years with NFI, who underwent medical examination and / or treatment at the Burdenko Neurosurgery Institute from January 1, 2003 till December 31,2015. Atotal from 264 patients 42 (16%) had clinical manifestations of NFI. The ratio of boys and girls was 1:1. The median age was 4.25 years (range 4.5 months – 17 years). Visual disturbances were the most frequent clinical manifestation of the tumor. Surgical resection was performed in 18 patients. The remaining 24 patients OPG were diagnosed based on clinical and radiological findings: 9 patients were in observation group, 11 patients chemotherapy was carried out, three were given radiation therapy, and spontaneous regression of the tumor was recorded in 1 patient. Progression of the disease was observed in 14 patients in our cohort. The overall survival rate in patients with NFI was 98 ± 2% at 5 years. Event free survival rate was 68 ± 7% at 5 years.The study was approved by the Independent Ethics Committee of N.N. Burdenko National Medical Research Center of neurosurgery Ministry of healthcare ofRussian Federation.

scholarly journals Large Invasive Adrenal Cortical Carcinoma in a Patient With Neurofibromatosis Type I

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A139-A139
Author(s):  
Brenda G Sandoval ◽  
Maria Del Mar Felix Morales ◽  
Joy Wortham ◽  
Maureen Koops ◽  
Ramona Granda-Rodriguez ◽  
...  
Keyword(s):  
Right Atrium ◽  
Renal Vein ◽  
Adrenal Mass ◽  
Left Renal Vein ◽  
Neurofibromatosis Type ◽  
Adrenal Cortical Carcinoma ◽  
Right Atrial ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
The Right

Abstract Background: Adrenal cortical carcinoma is a rare neoplasm. Adrenal cortical carcinoma has previously been reported in less than 10 cases of patients with Neurofibromatosis Type I. Clinical Case: A 44-year-old Hispanic man with Neurofibromatosis Type I (NF1) presented with progressive hypoxia, tachypnea and abdominal pain. Intubation was required with 100% FiO2. CT angiogram of the chest and abdomen showed a 9.5 cm left adrenal mass with tumor thrombus in the left adrenal vein, distal left renal vein, IVC extending into the right atrium, suggestive of an adrenocortical carcinoma. Café-au-spots, neurofibromas and Lisch nodules were seen on physical exam but no signs suggestive of a functional adrenal cortical carcinoma. Transthoracic echocardiogram demonstrated a large well circumscribed echo-dense mass filling the right atrial cavity. Hemodynamically he was unstable with labile blood pressures due to right atrium thrombus burden. He was supported pending pheochromocytoma investigation. Biochemical work up revealed an elevated 24-hour free cortisol concentration of 95.9 ug/d (<=60ug/d) with a normal 24-hour urine metanephrine and normetanephrine [140ug/d (55-320ug/d) and 448 ug/d (114-865ug/d]. Plasma free metanephrine level was normal 0.43 (0.00-0.49nmol/L) and the normetanephrine levels was increased at 2.7 (0.00-0.89nmol/L) consistent with stress response. Serum aldosterone level and renin activity with potassium and DHEA-S levels were normal. Once pheochromocytoma was ruled out, he successfully underwent a left nephrectomy, left adrenalectomy, IVC thrombectomy, right atrium thrombectomy under a multidisciplinary team of surgeons. Surgery was well tolerated and he was discharged home 11 days later. Pathology confirmed adrenal cortical carcinoma, stage 4, with involvement of endocardium and left renal vein with mitotic count greater than 20/50 high-power fields, consistent with a high-grade tumor. Summary: Patients with NF1 have a higher predilection to develop tumors including pheochromocytoma, paraganglioma, gastrointestinal stromal tumor, and pancreatic neuroendocrine tumor. NF1 association with adrenal tumors is well known, most commonly pheochromocytomas which occur in 0.1–5.7% of patients with NF1. However, based on a literature search there have been less than 10 case reports which postulate an association between NF1 and ACC. Genetic analysis of these reported cases suggest a loss of heterozygosity at the NF1 locus as a possible explanation of development of ACC in patients with NF1. Conclusion: Adrenal cortical carcinoma is rare but should be considered in a patient with NF1 and adrenal mass when plasma/urine metanephrines are not suggestive of pheochromocytoma.

scholarly journals Neurofibroma of the Palate

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Tirumalasetty Sreenivasa Bharath ◽  
Yelamolu Rama Krishna ◽  
Govind Rajkumar Nalabolu ◽  
Swetha Pasupuleti ◽  
Suneela Surapaneni ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Immunohistochemical Analysis ◽  
Neurofibromatosis Type ◽  
Clinical Findings ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Neurofibromatosis Type I ◽  
Solitary Lesion ◽  
Nerve Sheath ◽  
Different Types

Neurofibroma is a benign peripheral nerve sheath tumor comprising variable mixture of Schwann cells, perineurial-like cells, and fibroblasts. Neurofibroma may occur as solitary lesion or as part of a generalised syndrome of neurofibromatosis or very rarely as multiple neurofibromas without any associated syndrome. There are two distinct variants of neurofibromatosis type I and type II. We present a case of neurofibroma of the hard palate associated with neurofibromatosis type I. The diagnosis of the lesion was made based on the clinical findings, family history, histopathology, and immunohistochemistry. Literature was reviewed and different types of neurofibroma, their incidence and frequency in the oral cavity, its association with neurofibromatosis, clinical manifestations, histopathologic characteristics, immunohistochemical analysis, behaviour, treatment, and recurrence are discussed.

scholarly journals Optic pathway gliomas associated with neurofibromatosis type I in children

2019 ◽  
Vol 18 (4) ◽  
pp. 28-38
Author(s):  
E. F. Valiakhmetova ◽  
N. A. Mazerkina ◽  
O. A. Medvedeva ◽  
Y. Y. Trunin ◽  
E. M. Tarasova ◽  
...  
Keyword(s):  
Survival Rate ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Type I ◽  
Optic Pathway ◽  
Neurofibromatosis Type I ◽  
National Medical ◽  
Optic Pathway Gliomas ◽  
Visual Disturbances

Neurofibromatosis type I (NFI) is one of the most common brain tumor predisposition syndromes. Children with NFI are prone to develop a low grade gliomas, which can be localized in various areas of the brain, however, most of them occur in the structures of the optic pathway: optic nerves, chiasm, tracts and optic radiations – that is, are optic pathway gliomas (OPG). This retrospective study included children with newly diagnosied low grade glioma of the optic pathway at the age from 0 to 18 years with NFI, who underwent medical examination and / or treatment at the Burdenko Neurosurgery Institute from January 1, 2003 till December 31,2015. Atotal from 264 patients 42 (16%) had clinical manifestations of NFI. The ratio of boys and girls was 1:1. The median age was 4.25 years (range 4.5 months – 17 years). Visual disturbances were the most frequent clinical manifestation of the tumor. Surgical resection was performed in 18 patients. The remaining 24 patients OPG were diagnosed based on clinical and radiological findings: 9 patients were in observation group, 11 patients chemotherapy was carried out, three were given radiation therapy, and spontaneous regression of the tumor was recorded in 1 patient. Progression of the disease was observed in 14 patients in our cohort. The overall survival rate in patients with NFI was 98 ± 2% at 5 years. Event free survival rate was 68 ± 7% at 5 years.The study was approved by the Independent Ethics Committee of N.N. Burdenko National Medical Research Center of neurosurgery Ministry of healthcare ofRussian Federation.

scholarly journals CASE OF NEUROFIBROMATOSIS TYPE I WITH ASTROCYTOMA OF THE BRAIN IN THE PRACTICE OF PEDIATRICIAN

2020 ◽  
Vol 75 (3) ◽  
pp. 138-144
Author(s):  
T.N. Buchkova ◽  
◽  
N.I. Zriachkin ◽  
G.I. Chebotareva ◽  
O.V. Tihnenko ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Speech Disorders ◽  
Endocrine Disorders ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
Cutaneous Manifestations ◽  
The Brain

The relevance of the topic is due to insufficient awareness of doctors about neurofibromatosis, which leads to a delay in diagnosis and the development of complications. The diagnostic criteria characteristic of this disease, features of the clinical course, possible complications, methods of treatment, features of dispensary observation are described. A clinical case of neurofibromatosis type I with astrocytoma of the brain is described. The diagnosis of neurofibromatosis type I (NFI) was established in a patient aged 4 years 3 months. The peculiarity of the manifestations of this clinical case is the absence of family history; the combination of cutaneous manifestations, multiple lesions of the nervous system, complicated by chronic subdural hematoma, endocrine disorders and extra-cutaneous manifestations (eye symptoms, hearing and speech disorders); the absence of significant positive dynamics despite repeated surgical treatment and long-term polyochemotherapy. Despite the stabilization of clinical manifestations of the disease, this patient has an unfavorable prognosis with possible progression of the pathological process. For early clinical detection of patients with NFI, using for this purpose diagnostic criteria recommended by the International Committee of experts on neurofibromatosis, continuity of management and adequate measures of primary and secondary prevention of complications of NFI is important knowledge of this pathology by primary health care physicians, including pediatricians, district and family doctors, neurologists, dermatologists, ophthalmologists and surgeons.

Can 18FDG-PET detect malignant transformation of neurofibromas in patients with neurofibromatosis type I?

2006 ◽  
Vol 37 (06) ◽  
Author(s):  
J Neinert ◽  
E Fastnacht ◽  
R Larisch ◽  
E Legius ◽  
R Engelskirchen ◽  
...  
Keyword(s):  
Malignant Transformation ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
18Fdg Pet

Right Ventricle Mass Removal from Tricuspid Valve Apparatus: An Unusual Thromboembolic Complication of Severe Ketoacidosis

2016 ◽  
Vol 19 (2) ◽  
pp. 077
Author(s):  
Ireneusz Haponiuk ◽  
Maciej Chojnicki ◽  
Konrad Paczkowski ◽  
Wojciech Kosiak ◽  
Radosław Jaworski ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Mild Hypothermia ◽  
Heart Function ◽  
Thromboembolic Complication ◽  
Surgical Removal ◽  
Unusual Complication ◽  
Type I ◽  
Definitive Therapy ◽  
The Right

The presence of a pathologic mass in the right ventricle (RV) may lead to hemodynamic consequences and to a life-threatening incident of pulmonary embolism. The diagnosis of an unstable thrombus in the right heart chamber usually necessitates intensive treatment to dissolve or remove the pathology. We present a report of an unusual complication of severe ketoacidosis: thrombus in the right ventricle, removed from the tricuspid valve (TV) apparatus. A four-year-old boy was diagnosed with diabetes mellitus (DM) type I de novo. During hospitalization, a 13.9 × 8.4 mm tumor in the RV was found in a routine cardiac ultrasound. The patient was referred for surgical removal of the floating lesion from the RV. The procedure was performed via midline sternotomy with extracorporeal circulation (ECC) and mild hypothermia. Control echocardiography showed complete tumor excision with normal atrioventricular valves and heart function. Surgical removal of the thrombus from the tricuspid valve apparatus was effective, safe, and a definitive therapy for thromboembolic complication of pediatric severe ketoacidosis.<br /><br />

Sign in / Sign up

Export Citation Format

Share Document