Giant plexiform neurofibroma with hemorrhage in cranio-maxillofacial region as depicted on CT and MRI

Feng, Yu; Yang, Zhi‐gang; Chen, T; Wang, Q; Deng, Weiming

doi:10.1186/2047-783x-15-2-84

Cited by 11 publications

(8 citation statements)

References 10 publications

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“…Only a few cases have been described of the use of CT in giant plexiform neurofibromas affecting the scalp and cranio-maxillofacial regions. [ 5 6 ] These reports show old (non-active) areas of hemorrhage with heterogeneous density. This imaging presentation also differs from the fully active hemorrhage which is hyperdense in CT as in our case.…”

Section: Discussionmentioning

confidence: 97%

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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Section: Discussionmentioning

confidence: 97%

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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“…Neurofibromatosis (NF) is an autosomal dominant phakomatoses or neurocutaneous syndrome which is clinically diagnosed by the typical presence of café-au-lait macules, intertriginous freckling, Lisch nodules and neural tumors such as neurofibromas and gliomas [4] , [5] , [6] . The disorder occurs in 1/4,000 births and is inherited or may occur by spontaneous genetic mutation [3] .…”

Section: Discussionmentioning

confidence: 99%

“…The disorder occurs in 1/4,000 births and is inherited or may occur by spontaneous genetic mutation [3] . There are two distinctly recognized varieties of neurofibromatosis, NF I and NF II, based on differences in clinical and genetic features [4 , 6 , 7] . Type I disease, also known as von Recklinghausen's disease, which occurs due to loss of function of the NF I gene, presents with neurofibromas, café au lait spots, freckling and optic gliomas.…”

Section: Discussionmentioning

confidence: 99%

Imaging diagnosis of plexiform neurofibroma- unravelling the confounding features: A report of two cases

Grover

Kundra

Grover

et al. 2021

Radiology Case Reports

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Peripheral nerve sheath tumors such as neurofibroma, comprise 5% of all benign soft tissue tumors and usually occur due to an underlying neurofibromatosis. A plexiform neurofibroma, which is a tumor occurring exclusively in neurofibromatosis1, is a rare entity and is an uncommon variant of neurofibroma. We report the clinical and imaging features of plexiform neurofibroma in two young male patients, in whom the imaging diagnosis was confirmed after biopsy. The report not only aims to highlight the characteristic imaging features of plexiform neurofibroma but we also emphasize the ultrasound appearances which are significantly characteristic and can effectively lead to the correct diagnosis at the preliminary stage of investigation. The tumors which originate from nerve sheath, are large, lobulated masses and demonstrate typical imaging features of simultaneous involvement of subcutaneous and cutaneous tissues along with infiltrative invasion of deeper structures. The tumors characteristically display fat and fluid contents and a “target sign’ on evaluation by ultrasound, CT and MRI. Imaging plays an important role in confirming the diagnosis, delineating involved structures, excluding simulating conditions and forewarning a possible malignant transformation.

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“…6 Spontaneous massive bleed as the initial presentation of such tumors was also described involving the head, neck and trunk. [7][8][9][10] It is also worth mentioning that under the ''no complication'' category the authors included cases where complications were not described; the authors assumed that if a significant complication had occurred it would have been mentioned. Malignant transformations to malignant peripheral nerve sheath tumors were rare (5%).…”

Section: Discussionmentioning

confidence: 99%

Massive Soft Tissue Neurofibroma (Elephantiasis Neuromatosa)

et al. 2015

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The authors review the literature on massive soft tissue neurofibroma. The methods included a review of 71 reports (PubMed search 1929-2012) with a total of 91 massive soft tissue neurofibroma patients and illustration of clinical and radiological progression of massive soft tissue neurofibroma on a patient with neurofibromatosis type 1. The mean age at initial examination was 21 years. Tumor onset was mostly in childhood years. The commonest affected body segment was the lower extremity (46%), followed by head/neck (30%). Surgical management was pursued in the majority of cases (79%). Bleeding was a common complication (25%). Recurrence was described in 12%; multiple resections cases were described. Malignant transformation occurred in 5%. Although massive soft tissue neurofibroma may be present early in life, massive tumor overgrowth may take years. Predicting disease progression and/or benefit of surgical intervention early in the disease course is challenging. Recurrence and malignant transformation are possible. Massive soft tissue neurofibroma does not respond to chemotherapy or radiotherapy and is associated with life-threatening surgical complications.

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Giant plexiform neurofibroma with hemorrhage in cranio-maxillofacial region as depicted on CT and MRI

Cited by 11 publications

References 10 publications

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

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

Imaging diagnosis of plexiform neurofibroma- unravelling the confounding features: A report of two cases

Massive Soft Tissue Neurofibroma (Elephantiasis Neuromatosa)

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