Plexiform neurofibroma affecting the upper parietal scalp, with cerebellar hamartoma<b>:</b> role of histopathology, colour Doppler imaging and magnetic resonance imaging

Sehgal, Virendra; Oberai, Rakesh; Venkatash, P.; Sharma, Sonal; Verma, Prashant; Chatterji, Kabita

doi:10.1111/ced.12027

Cited by 4 publications

(5 citation statements)

References 10 publications

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“…This study's results showed the presence of a power Dop-pler signal in 30 % of the lesions studied. The studies that do mention the Doppler signal of the nodular forms catalog them as hypovascular lesions (Giovagnorio's type 1) [9,13,14,16,20], while this parameter might be slightly elevated in plexiform and diffuse types [6,8,21]. Although in most lesions the presence of vascularization was not observed, it was showed intralesionally in 9.92 % (type III) and both intralesionally and peripherally in 4.13 % (type IV), especially in the forms clinically labeled as superficial plexiform neurofibromas.…”

Section: Discussionmentioning

confidence: 99%

“…Most authors do not refer to the studies of neurofibromas in Doppler mode. Those who refer to the vascularization of these lesions suggest that it is usually absent in the nodular forms [19,20] or is slightly increased in the diffuse and plexiform variants [8,21]. The first goal of this study was to define the ultrasound patterns of the different types of cutaneous neurofibromas in NF1 present in a pediatric popula-tion and the second was to establish a clinical and sonographic classification of cutaneous neurofibromas present in childhood and determine the interobserver diagnostic agreement coefficient (κ) in this classification of neurofibromas.…”

Section: Introductionmentioning

confidence: 99%

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Clinical and Sonographic Classification of Neurofibromas in Children with Neurofibromatosis Type 1 – A Cluster Analysis

et al. 2021

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Purpose High-frequency ultrasound allows the accurate identification of neurofibromas in neurofibromatosis type 1 (NF1). This study aimed to analyze the ultrasound features of neurofibromas in children with NF1, to establish a classification based on the clinical and sonographic patterns of the different types of neurofibromas, and to evaluate the interobserver correlation coefficient (κ) of this classification. Materials and Methods In this prospective, single referral center observational study, clinical and ultrasound findings of neurofibromas in children diagnosed with NF 1 were analyzed. To identify the ultrasound patterns, a cluster analysis allowing the inclusion of both clinical and ultrasound data was designed. The κ coefficient was calculated using 9 external evaluators. Results 265 ultrasound scans were performed on a total of 242 neurofibromas from 108 children diagnosed with NF1. Cluster analysis allowed the identification of 9 patterns (Snedecorʼs F, P < 0.001) classified as “classic” cutaneous neurofibroma, blue-red neurofibroma, pseudoatrophic neurofibroma, nodular subcutaneous neurofibroma, diffuse subcutaneous neurofibroma, congenital cutaneous neurofibroma, congenital plexiform neurofibroma, congenital diffuse and plexiform neurofibroma, and subfascial neurofibroma. The κ coefficient of the interobserver ratings was 0.82. Conclusion Patterns identified in the cluster analysis allow neurofibromas to be classified with a very high interobserver correlation.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical and Sonographic Classification of Neurofibromas in Children with Neurofibromatosis Type 1 – A Cluster Analysis

et al. 2021

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“…In 1987, the NIH published diagnostic criteria for the disease, as shown in Table I. The disease can be confirmed with two or more of these features (Sehgal et al, 2013).…”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment of a Massive Neurofibroma of the Head and Neck in a Patient with Neurofibromatosis Type 1: A Case Report

et al. 2018

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Neurofibromatosis type 1 (NF1) is a rare autosomal dominant neurogenetic disease with variable clinical manifestations, which are primarily manifested as neurofibromas, café-au-lait macules (CALMs) and skeletal deformities. Although generally benign, expansile neurofibromas that are characteristic of NF1 readily lead to disturbing deformities. It is often difficult to surgically extirpate a tumor that involves these important tissues or organs. We report a rare case of a patient with neurofibromatosis Type 1. The patient presented with a congenital giant scalp neurofibroma and CALMs in the occipito-cervical region, in addition to ear and occipital deformities. We performed a challenging surgical intervention (a near-total resection) to reduce the tumor burden and rehabilitate the appearance and function of the patient while preserving the intracranial tissue structure. Here, we review this case and analyze the clinical manifestations, diagnosis and management of NF1.

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“…The ultrasound imaging appearance of uncomplicated plexiform NF in the scalp has been previously described as a hypoechoic solid mass. [ 4 ] This appearance differs greatly in its imaging presentation from the complicated ultrasound features of the same tumor under hemorrhage, which shows a mixed echogenicity (heterogeneous) hypoechoic and anechoic solid-cystic structure. Even though the diagnosis can be performed with 2D ultrasound images, 3D images that require only a 5 to 8 seconds sweep show a more understandable representation of the pathology to the clinician.…”

Section: Discussionmentioning

confidence: 99%

Multidimensional ultrasound and computed tomography imaging support in bleeding plexiform neurofibromatosis of the scalp: A case report and literature review

et al. 2015

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Active bleeding in plexiform neurofibromatosis can be a life-threatening complication in neurofibromatosis type 1 (NF1). The prompt imaging support of 2D-3D ultrasound (US) and computed tomography (CT) during the active hemorrhage phase of cutaneous neurofibromas has not been previously reported. We report a case with NF1 who experienced a sudden swelling in the parieto-temporal region that corresponded to a massive and active hemorrhage within a plexiform neurofibroma. The US and CT imaging characteristics of this bleeding tumor are shown. Active hemorrhage in a plexiform neurofibroma of the scalp appeared in US as a heterogeneous hypodermal mass. CT demonstrated a fully hyperdense soft tissue mass. These characteristics differ from the non-complicated or old hemorrhagic imaging appearances of scalp plexiform neurofibromas and encourage prompt surgical treatment. This case report demonstrates the usefulness of imaging support in the early diagnosis of this hemorrhagic complication of NF1 in the scalp and also stimulates multispecialty management.

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Plexiform neurofibroma affecting the upper parietal scalp, with cerebellar hamartoma: role of histopathology, colour Doppler imaging and magnetic resonance imaging

Cited by 4 publications

References 10 publications

Clinical and Sonographic Classification of Neurofibromas in Children with Neurofibromatosis Type 1 – A Cluster Analysis

Clinical and Sonographic Classification of Neurofibromas in Children with Neurofibromatosis Type 1 – A Cluster Analysis

Surgical Treatment of a Massive Neurofibroma of the Head and Neck in a Patient with Neurofibromatosis Type 1: A Case Report

Multidimensional ultrasound and computed tomography imaging support in bleeding plexiform neurofibromatosis of the scalp: A case report and literature review

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