Plexiform Neurofibroma

Tchernev, Georgi; Chokoeva, Anastasiya Atanasova; Patterson, James W.; Bakardzhiev, Ilko; Wollina, Uwe; Tana, Claudio

doi:10.1097/md.0000000000002663

Cited by 49 publications

(52 citation statements)

References 7 publications

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“…Plexiform neurofibromas occur in up to 30% of cases of NF-1, most frequently in the craniomaxillofacial region [5]. They can be present at birth, but often appear between the ages of 2 and 5 [6].…”

Section: Discussionmentioning

confidence: 99%

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Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Senhaji¹

2018

JDC

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Plexiform neurofibroma is a rare benign tumor of the peripheral tissue cells developed in the perineurium, generally associated with neurofibromatosis type 1 (NF1). These entities consist of multiple, twisted masses that grow along the axis of a large nerve, infiltrating and separating normal nerve fascicles. The diagnosis is essentially pathological and treatment is primarily surgical to avoid malignant degeneration. Giant neurofibromas are very difficult to manage surgically as they are extensively infiltrative and highly vascularized. This report describes a case of an advanced plexiform neurofibroma of the right lower back and pelvic region in a 25-year-old female with neurofibromatosis type 1, discovered in early childhood, with limited normal living due to the severe disfigurement as the result of progression of the disease.

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Section: Discussionmentioning

confidence: 99%

“…Masses can lead to disfigurement, erosion of adjacent bone, and scoliosis [10]. They are generally diagnosed clinically with appreciation of the typical features, and histopathology is useful to exclude malignant transformation [5].…”

Section: Discussionmentioning

confidence: 99%

Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Senhaji¹

2018

JDC

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“…Th e diagnosis is established based of clinical (14). Our patient presented with thousands of cutaneous neurofi bromas which is the third hallmark signs of NF1.…”

Section: Discussionmentioning

confidence: 99%

“…"Subcutaneous" tumors are fi rm and nodular. Pregnancy induces tumor growth (9,14). Th e tumors are composed of Schwann cells, fi broblasts, mast cells, and perineural cells.…”

Section: Discussionmentioning

confidence: 99%

Extensive Peculiar Cutaneous Form of Neurofibromatosis Type I as a New Mutation - a Case Report

Balaban

Popović

2016

Serbian Journal of Dermatology and Venereology

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Neurofi bromatosis-1 (NF1) is one of the most common hereditary multisystemic disorders. The disease manifests a variety of characteristic features that include: hyperpigmentary abnormalities of the skin (café-au-lait macules, freckles in the axillae, and iris Lisch nodules) and growth of benign peripheral nerve sheath tumors (neurofi bromas) in the skin. Associated extracutaneous clinical features include: skeletal abnormalities, neurological, cardiovascular, endocrine and other malformations. NF1 is caused by mutation in the neurofi bromatosis-1 gene, which codes for the protein neurofi bromin. The inheritance of NF1 follows an autosomal dominant trait, although about 50% of patients present with new ("de novo") mutations, and represent the fi rst member of their family. No difference in the severity of the disease can be found in patients with familial mutations versus those with new mutations. We present a 78-year-old female patient with an extreme cutaneous form of neurofi bromatosis who reported no affected family member. Apart from skin problems, she had no major health issues in childhood and adolescence, but in recent decades she had frequent headaches, occasional abdominal pain, and vision and hearing impairment. About 10 to 14 days before admission, she developed a severe cough, shortness of breath, and chest and abdominal pain. On examination, the patient of short stature (hight: 152 cm, weight: 49 kg) presented with thousands of soft nodules dispersed over the whole body, except on extensor sides of thighs and lower legs; the nodules varied in color from skin-colored, livid erythematous, to brown-grey; the nodules on the abdomen were moist, partly bleeding from the base, and accompanied by an unpleasant odor. Her feet were also densely covered by dark purple lumps, with dystrophic changes of the toe nails that were thickened, frayed, and yellowish. The skeletal abnormalities included: short stature, severe osteoporosis and osteosclerosis of the head bone structure; degenerative arthropathc-spondylotic changes of the thoracolumbar spine segment with signs of diffuse skeletal hyperostosis; pronounced degenerative changes of the lumbar spine. CT scans of the head, chest and abdomen showed the following abnormalities: fl attening of the paraventricular gyri and reduction of brain parenchyma with hypodensity of the white matter in terms of cortical atrophy; periventricular bilateral small post-ischemic microvascular brain lesions of varying chronicity; in the parenchyma of the upper left lung lobe the apical presence of small areas of pleural effusion with consequent subatelectic region; distended stomach and a small inner wall herniation; hypotrophic right kidney; atherosclerotic lesions of the abdominal aorta; low grade infrarenal kinking of the abdominal aorta. Pathohistological analysis of biopsy specimen taken from the nodule corresponded with cutaneous neurofi broma. Consultative examinations of various specialists pointed to the existence of the following comorbidities: obstructive respira...

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“…11 Plexiform neurofibroma is an uncommon variant of neurofibromatosis type 1 occurring along multiple nerves and their branches as an interdigitating network of fingerlike fronds. 12,13 Retroperitoneal plexiform neurofibromas appear as symmetric,bilateral parapsoas or presacral masses along the distribution of lumbosacral plexus. High lipid content, high water content within the mucinous matrix, cystic degeneration and entrapment of perineural adipose tissue are contributing to the hypodensity of these tumors 13 [Fig.5].…”

Section: Solitary Fibrous Tumormentioning

confidence: 99%

Cross - Sectional Imaging Features of Common and Uncommon Primary Retroperitoneal Tumors: An Experience at Our Institute - A Pictorial Essay

Riba¹,

Gunasekaran²,

Hauzel³

et al. 2019

IJAR

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Int. J. Adv. Res. 7(12), 213-227 214 Familiarity with the CT and MR imaging features of various retroperitoneal masses will facilitate accurate diagnosis as well as staging of these lesions.This pictorial essay reviews the imaging features of few of the histopathologically confirmed common and uncommon primary retroperitoneal tumors presented to our institute from 2016 to 2019. Anatomy of retroperitoneumThe retroperitoneum is a compartmentalized space extending between transversalis fascia posteriorly and posterior parietal peritoneal layer anteriorly [ Fig. 1]. It is divided into following three potential spaces: a) Anterior pararenal space, b) posterior pararenal space c) perirenal space and d) great vessel space. Anterior pararenal space is bounded anteriorly by posterior parietal peritoneum, posteriorly by anterior renal fascia / Gerota's fascia and laterally by lateroconal fascia which is a continuation of posterior renal fascia / Zuckerkandl fascia Anterior pararenal space is subdivided into (i) pancreaticoduodenal space comprising pancreas and second, third and fourth portions of the duodenum, and (ii) pericolonic space comprising ascending and descending colon, lymph nodes and fat. Anterior pararenal space is continuous across the midline. Posterior pararenal space extends between Zuckerkandl fascia anteriorly and transversalis fascia posteriorly and contains only small amounts of fat. The perirenal space is situated between anterior and posterior renal fasciae and contains adrenal gland, kidney and renal pelvis, proximal ureter, renal hilar vessels, lymph nodes, and fat. The perirenal space contains bridging septa and a network of lymphatic vessels that allow the spread of disease to or from adjacent spaces. There are three more potential expansile planes composed of apposed layers of embryonic mesentery formed from laminations of renal and lateroconal fasciae: (i) retromesenteric, (ii) retrorenal and (iii) lateroconal planes, formed by splitting of anterior renal fascia, posterior renal fascia and lateroconal fascia respectively. The great vessel space is a fat containing space located anterior to psoas muscles and vertebral bodies that surrounds the aorta and inferior vena cava. A fifth space, the infrarenal retroperitoneal space is formed by the fusion of anterior and posterior pararenal spaces, below the level of the kidneys and is communicating with the prevesical and extraperitoneal compartments of the pelvis. 4,5,6,7

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Plexiform Neurofibroma

Cited by 49 publications

References 7 publications

Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Extensive Peculiar Cutaneous Form of Neurofibromatosis Type I as a New Mutation - a Case Report

Cross - Sectional Imaging Features of Common and Uncommon Primary Retroperitoneal Tumors: An Experience at Our Institute - A Pictorial Essay

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