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.

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.

Cherubism in a 12-year old Child; A Rare Case Report

2020 ◽  
Vol 12 (2) ◽  
pp. 39-42
Author(s):  
Dr. Mallayya C. Hiremath ◽  
Dr. SK. Srinath ◽  
Dr. Bineesh Balan ◽  
Dr. Sushma H S
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Growth Period ◽  
Individual Case ◽  
Radiographic Examination ◽  
Facial Deformity ◽  
Autosomal Dominant Disorder ◽  
Radiological Features ◽  
Rare Case Report

Cherubism is a non-neoplastic, fibro-osseous, self-limiting, autosomal dominant disorder of the jaws. It is also called as familial fibrous dysplasia and familial multi-locular cystic lesion of the jaws. Its typical dento-facial deformities are caused by mutations in the SH3BP2 gene. The lesion regresses during puberty and the disease stabilizes after the growth period. But it leaves some facial deformity along with disturbances in occlusion. Cherubism may occur as an individual case or in multiple members of the same family, oftenly in multiple generations. Radiographic examination of the lesion shows bilateral multi-locular radiolucent areas.The aim of this case report is to present a case of 12-years old cherubic child, with its clinical and radiological features and discussion about the clinical outcome.This patient was diagnosed with Cherubism and being followed up periodically.

scholarly journals A Rare Case of Type 1 Neurofibromatosis-Associated Unilateral Gigantomastia: A Case Report and Literature Review

2020 ◽  
Vol 6 ◽  
pp. 2513826X2096470
Author(s):  
Kim Phan ◽  
Muralie Vignarajah ◽  
Nasimul Huq
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Neurofibromatosis Type ◽  
Flap Reconstruction ◽  
Autosomal Dominant Disorder ◽  
Nerve Sheath ◽  
Type 1 Neurofibromatosis ◽  
The Right

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by the formation of benign nerve sheath tumours known as neurofibromas. A 28-year-old female presented with a large neurofibroma extending from the elbow of the right arm into the axilla and across the right breast. A mastectomy was performed to excise 4 kg of tissue with an immediate flap reconstruction. Post-operatively, the patient’s hemoglobin levels dropped significantly due to excessive blood loss, but the patient was discharged by post-operative day 5 with no additional complications. This study raises awareness for NF1, describes possible complications that may arise following excision of highly vascularized neurofibromas, and outlines possible techniques for breast reconstruction.

scholarly journals Gingival Lipoma: A Rare Case Report

2017 ◽  
Vol 1 (1) ◽  
pp. 37-39
Author(s):  
Sweta Shrestha ◽  
Shaili Pradhan ◽  
Ranjita Shrestha Gorkhali
Keyword(s):  
Rare Case ◽  
Surgical Excision ◽  
Soft Tissue Tumors ◽  
Rare Case Report ◽  
Postsurgical Complication ◽  
Benign Tumours ◽  
One Year ◽  
Slow Growing ◽  
Oral Soft Tissue

Lipomas are benign tumours of mesenchymal origin (mature adipocytes) that are comparatively uncommon in the oral cavity corresponding to less than 4.4% of all benign oral soft tissue tumors. Clinically, they present as slow growing, soft, asymptomatic masses. Histopathologically, they appear as thinly encapsulated lesion composed of mature adipocytes with inconspicuous vascularity. The pathogenetic mechanisms of oral lipomas are still unclear. They are usually treated by surgical excision and bear excellent prognosis. Here we report a case of intraoral lipoma in 54 year old male patient in the left lower lingual alveolar mucosal region that was treated by surgical excision using electrocautery without any postsurgical complication. One-year follow-up showed no evidence of recurrence.

scholarly journals Lactulose to the Rescue: A Case of Toxic Hepatic Encephalopathy Caused by Portosystemic Shunting and Epistaxis in a Patient with Hereditary Hemorrhagic Telangiectasia

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Ruchit N. Shah ◽  
Michael Makar ◽  
Nasir Akhtar ◽  
Erin Forster
Keyword(s):  
Hepatic Encephalopathy ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Rare Complication ◽  
Multiple Organ ◽  
Autosomal Dominant Disorder ◽  
Life Saving ◽  
Organ Systems ◽  
Portosystemic Shunting

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder characterized by telangiectasias and arteriovenous malformations. Multiple organ systems are involved including the skin, lungs, gastrointestinal tract, and brain. Hepatic encephalopathy is an extremely rare complication of HHT and early diagnosis and treatment can be life-saving. We present a rare case of hepatic encephalopathy caused by HHT-induced portosystemic shunting treated with lactulose.

scholarly journals Desquamative interstitial pneumonia in a non-smoker with neurofibromatosis type 1 (Von Recklinghausen syndrome)

2020 ◽  
Vol 13 (1) ◽  
pp. e227379
Author(s):  
Gustavo Ferrer ◽  
Alwiya Omar Saleh ◽  
Henry D Tazelaar ◽  
Andrea V Arrossi
Keyword(s):  
Interstitial Pneumonia ◽  
Neurofibromatosis Type 1 ◽  
Autosomal Dominant ◽  
Interstitial Fibrosis ◽  
Neurofibromatosis Type ◽  
Pulmonary Involvement ◽  
Autosomal Dominant Disorder ◽  
Systemic Manifestations ◽  
Von Recklinghausen

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with multiple systemic manifestations. Pulmonary involvement has been reported in the form of interstitial fibrosis, emphysema, pulmonary hypertension and thoracic neoplasm. We report a case of desquamative interstitial pneumonia in a non-smoker with NF1.

Plexiform Neurofibromatosis with Abnormal Pachydermatocele: A Rare Case Report

2013 ◽  
Vol 24 (4) ◽  
pp. 110-113
Author(s):  
AK Agarwal ◽  
Dileep Kumar ◽  
Anil Kumar Gupta ◽  
Ganesh Yadav
Keyword(s):  
Rare Case ◽  
Functional Disability ◽  
Indian Subcontinent ◽  
Plexiform Neurofibroma ◽  
Subcutaneous Fat ◽  
Left Thigh ◽  
Optimal Management ◽  
Rare Type ◽  
Rare Case Report ◽  
Plexiform Neurofibromatosis

Abstract Plexiform neurofibromatosis, also known as pachydermatocele, is a rare type of neurofibromatosis, caused by excessive growth of the neural tissue in the subcutaneous fat. This condition is rare in Indian subcontinent but when it occurs, it causes cosmetic as well as functional disability. The optimal management of plexiform neurofibroma is not well defined and surgery is often delayed. We report a rare case of neurofibromatosis with pachydermatocele involving the nerves of the left thigh and leg with disfigurement and associated disability.

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