LARGE PLEXIFORM NEUROFIBROMA OF THE SACRO-GLUTEAL REGION – A RARE CASE REPORT

2021 ◽  
pp. 30-31
Author(s):  
Surya Rao Rao Venkata Mahipathy ◽  
Alagar Raja Durairaj ◽  
Narayanamurthy Sundaramurthy ◽  
Anand Prasath Jayachandiran ◽  
Suresh Rajendran
Keyword(s):  
Rare Case ◽  
Primary Closure ◽  
Plexiform Neurofibroma ◽  
Surgical Excision ◽  
Gluteal Region ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Rare Case Report ◽  
Von Recklinghausen

Plexiform neurobroma is a rare benign nerve sheath tumor that develops in the perineurium and is often considered pathognomonic of neurobromatosis type 1 (NF1 or von Recklinghausen disease). They occur most frequently in the craniomaxillofacial region, rarely on back and extremities. These lesions are highly vascular and there is 15-20% potential for malignant transformation. Here, we present a 26-year-old female with neurobromatosis all over her body, but with a large plexiform neurobroma in the sacral region which was causing difculty in sitting and lying supine as well as disgurement of the gluteal region. Surgical excision with primary closure of the swelling was done. Histopathology ndings were consistent with neurobromatosis.

PLEXIFORM NEUROFIBROMA OF THE BRACHIAL PLEXUS – A RARE CASE REPORT

2021 ◽  
pp. 13-15
Author(s):  
Surya Rao Rao Venkata Mahipathy ◽  
Alagar Raja Durairaj ◽  
Narayanamurthy Sundaramurthy ◽  
Anand Prasath Jayachandiran ◽  
Suresh Rajendran
Keyword(s):  
Brachial Plexus ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Plexiform Neurofibroma ◽  
Autosomal Dominant Disorder ◽  
Surgical Debulking ◽  
Rare Case Report ◽  
Benign Tumours ◽  
Common Benign Tumour

Neurobroma is a common benign tumour occurring as part of an autosomal dominant disorder, neurobromatosis type 1, leading to the formation of benign tumours or neurobromas of the peripheral nervous system. Large neurobromas of the brachial plexus are rare and present a difcult challenge for surgeon due to the anatomical complexity of the brachial plexus. Dermal neurobromas usually present with swelling and occasional pain, but neurobromas associated with the brachial plexus present with pain and neurological symptoms. These plexiform neurobromas of the brachial plexus are known to undergo malignant transformation. Here, we present a case of a large plexiform neurobroma affecting the left brachial plexus and extending till the elbow, conrmed with MRI and surgical debulking was done.

Malignant peripheral nerve sheath tumor (MPNST) arising from angiosarcoma: a rare case report

2019 ◽  
Vol 23 (1) ◽  
pp. 109-112
Author(s):  
Maria Lazaridou ◽  
Eleni Bourlidou ◽  
Margarita Vafeiadou ◽  
Yiannis Papadiochos ◽  
Nikolaos Barbetakis ◽  
...  
Keyword(s):  
Case Report ◽  
Peripheral Nerve ◽  
Rare Case ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Rare Case Report

scholarly journals Primary intracardiac malignant peripheral nerve sheath tumor: A rare case report

2015 ◽  
Vol 58 (4) ◽  
pp. 531 ◽  
Author(s):  
Sudheer Arava ◽  
Saphalta Bagmar ◽  
Parul Jain ◽  
Muthu Kumaran ◽  
Sanjeev Kumar ◽  
...  
Keyword(s):  
Case Report ◽  
Peripheral Nerve ◽  
Rare Case ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Rare Case Report

scholarly journals Giant Malignant Peripheral Nerve Sheath Tumor of Scalp in Non-neurofibromatosis Person: A Rare Case Report

2017 ◽  
Vol 07 (01) ◽  
pp. 054-057
Author(s):  
Amit Gupta ◽  
Rakesh Kumar ◽  
Bal Ojha ◽  
Anil Chandra
Keyword(s):  
Peripheral Nerve ◽  
Histopathological Examination ◽  
Bone Destruction ◽  
Surgical Excision ◽  
Intracranial Extension ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Complete Surgical Excision ◽  
Rare Case Report

AbstractMalignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from components of nerve sheath such as perineural fibroblasts or Schwann cells. Primary scalp MPNST is an exceptionally rare entity and only few cases have been reported till date. We encountered an unusual case of giant scalp MPNST in a 70-year-old man without any history suggestive of neurofibromatosis associated with intracranial extension with underlying bone destruction. The tumor was treated with complete surgical excision followed by adjuvant radiotherapy. Histopathological examination showed hyper- and hypocellular areas of spindle cells admixed with myxoid areas and immunohistochemistry revealed positivity to vimentin and S100 on the basis of which the diagnosis of MPNST was made.

Malignant Peripheral Nerve Sheath Tumor Arising from a Solitary Neurofibroma in a Patient without Neurofibromatosis Type 1

2016 ◽  
Vol 78 (5) ◽  
pp. 516-521
Author(s):  
Fumihisa SAWADA ◽  
Eiichi MAKINO ◽  
Takenobu YAMAMOTO ◽  
Ryo TANAKA ◽  
Yutaka FUJIWARA ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Solitary Neurofibroma

scholarly journals Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

2017 ◽  
Vol 75 (6) ◽  
pp. 366-371 ◽  
Author(s):  
Roberto André Torres de Vasconcelos ◽  
Pedro Guimarães Coscarelli ◽  
Regina Papais Alvarenga ◽  
Marcus André Acioly
Keyword(s):  
Overall Survival ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Tumor Size ◽  
Retrospective Chart Review ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population.

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