Rare case of juvenile ossifying fibroma of the lumbar spine causing vertebra plana

2021 ◽  
Vol 14 (1) ◽  
pp. e239097
Author(s):  
Bhavuk Garg ◽  
Nishank Mehta ◽  
Devasenathipathy Kandasamy ◽  
Asit Ranjan Mridha
Keyword(s):  
Osteolytic Lesion ◽  
Successful Outcome ◽  
Ossifying Fibroma ◽  
Subtotal Resection ◽  
Entire Body ◽  
Residual Tumour ◽  
Fibrous Stroma ◽  
Posterior Stabilisation ◽  
Juvenile Ossifying Fibroma ◽  
Vertebra Plana

Juvenile ossifying fibroma (JOF) is a rare benign fibro-osseous tumour occurring in craniofacial bones of children and young adults. An 8-year-old girl presented with low back pain since 10 months. Plain radiographs revealed a vertebra plana-like lesion of L3 vertebra. CT scan showed an osteolytic lesion with areas of ground-glass appearance interspersed with bone flecks involving the entire body, pedicles and laminae of L3 with well-circumscribed cortical margins. Biopsy was done—histopathological findings showed cellular fibrous stroma with bony trabeculae—which, on corroboration with clinical and radiological findings noted previously, confirmed the diagnosis of JOF. The patient underwent preoperative angioembolisation, curettage and posterior stabilisation. At follow-up of 15 months, the patient was asymptomatic—imaging revealed residual tumour in pedicles—which however did not progress radiologically. The case raises the importance of including this rare condition as a differential diagnosis in children and reports successful outcome with subtotal resection.

scholarly journals Trabecular Juvenile Ossifying Fibroma of the Craniofacial Skeleton: Etiopathogenesis and a Case Report of the Rare Entity

2014 ◽  
Vol 4 (1) ◽  
pp. 51-55
Author(s):  
Shwetha Kumar ◽  
Rahul Kadam
Keyword(s):  
Case Report ◽  
Age Of Onset ◽  
Bone Architecture ◽  
Ossifying Fibroma ◽  
Rare Entity ◽  
Craniofacial Skeleton ◽  
High Tendency ◽  
Fibrous Stroma ◽  
Juvenile Ossifying Fibroma ◽  
Hrpt2 Gene

ABSTRACT The term, fibro-osseous lesions, is used for a group of pathological disturbances encompassing developmental, reactive or dysplastic lesions and neoplasms characterized by replacement of normal bone architecture by tissue composed of collagen fibers and fibroblasts containing various amount of calcified tissue. The groups of the fibro-osseous lesions are best considered as a spectrum of processes arising from cells in the periodontal ligament. Juvenile ossifying fibroma (JOF) is a benign, but potentially aggressive, fibro-osseous tumor of the craniofacial bones. This uncommon neoplasm is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation, potential behavior and the high tendency to recur. Clinically presenting as an actively growing lesion. Histopathologically consists cell rich fibrous stroma containing bands of cellular osteoid without osteoblastic lining together with trabeculae of more typical woven bone. Pathogenesis of JOF may be related to mutations of HRPT2 gene which may arises due to haploinsufficiency of the HRPT2 gene. Here, we reported a case of trabecular JOF (TJOF) which had variations in clinical, radiographic features and histopathological characteristics and it's etiopathogenesis in detail. How to cite this article Kadam R, Patel S, Pathak J, Swain N, Kumar S. Trabecular Juvenile Ossifying Fibroma of the Craniofacial Skeleton: Etiopathogenesis and a Case Report of the Rare Entity. J Contemp Dent 2014;4(1):51-55.

Management of an Extensive Orbitofrontal Juvenile Ossifying Fibroma

2012 ◽  
Vol 73 (S 02) ◽  
Author(s):  
K. Tekeli ◽  
A. Toma ◽  
P. Minhas ◽  
M. Manisali
Keyword(s):  
Ossifying Fibroma ◽  
Juvenile Ossifying Fibroma

scholarly journals HGG-48. ROS1 INHIBITOR ENTRECTINIB USE IN RELAPSE/REFRACTORY INFANTILE GLIOBLASTOMA WITH POSITIVE ROS1 FUSION - A CASE REPORT WITH PROMISING RESPONSE

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii352-iii352
Author(s):  
Dennis Tak-Loi Ku ◽  
Matthew Ming-Kong Shing ◽  
Godfrey Chi-Fung Chan ◽  
Eric Fu ◽  
Ping-Wa Yau ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Facial Nerve ◽  
Nerve Palsy ◽  
Facial Nerve Palsy ◽  
Tumour Progression ◽  
Molecular Study ◽  
Subtotal Resection ◽  
Residual Tumour ◽  
Molecular Assay ◽  
Debulking Surgery

Abstract INTRODUCTION Infantile glioblastoma is rare with poor prognosis. Recent molecular study for infantile hemispheric high grade glioma found its association with ALK/ROS1/NTRK/MET pathway. This suggested the potential use of targeted therapy for refractory / relapse patients. CASE: A newborn presented with apnea, CT brain showed intracranial haemorrhage. MRI then showed a left parietal tumour with bleeding and mass effect. Craniotomy achieved subtotal resection. Chemotherapy VCR/CPM alternating with CDDP/VP-16 was given for one year. Patient was stable with static residual tumour during chemotherapy. However patient developed status epilepticus two weeks after off treatment. MRI showed significant tumour progression which required 2nd & 3rd debulking surgery. Molecular assay by nanostring panel showed BRAF-KIAA1549 fusion. MEK inhibitor Trametinib was tried for 3 months and stopped as disease progression. Further molecular assay by RNASeq showed presence of ROS1 fusion (ZCCHC8-ROS1) while absent of BRAF fusion. Patient underwent 4th debulking surgery as impending herniation while waiting for the targeted therapy. It was complicated with right hemiplegia and facial nerve palsy postoperatively. Finally, ROS1 inhibitor Entrectinib was started 2 weeks later. It was well tolerated without significant adverse reaction. Patient made dramatic neurological recovery including improved facial nerve palsy, able to walk unaided and self feed. MRI brain 1 and 3 months after Entrectinib showed interval reduction in residual tumour. Patient is currently progression-free for 6 months. CONCLUSION Early molecular study for infantile glioblastoma is useful to guide novel therapy. Molecular result may varies between different panels or change over time, to be interpreted with caution.

Juvenile ossifying fibroma of the jaw: a retrospective study of 15 cases

2016 ◽  
Vol 45 (3) ◽  
pp. 368-376 ◽  
Author(s):  
J. Han ◽  
L. Hu ◽  
C. Zhang ◽  
X. Yang ◽  
Z. Tian ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Ossifying Fibroma ◽  
Juvenile Ossifying Fibroma

scholarly journals Juvenile Aggressive Trabecular Ossifying Fibroma of Mandible: A Rare Case Report

2016 ◽  
Vol 6 (1) ◽  
pp. 45-51
Author(s):  
Deepa Das Achath ◽  
Abhishek Sanjay Ghule ◽  
Preeti Kanchan-Talreja ◽  
Sunanda Bhatnagar
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Histological Analysis ◽  
High Recurrence Rate ◽  
Ossifying Fibroma ◽  
Appropriate Treatment ◽  
Juvenile Ossifying Fibroma ◽  
Rare Case Report

ABSTRACT Fibroosseous lesions of the jaws, including juvenile ossifying fibroma (JOF), pose diagnostic and therapeutic difficulties due to their clinical, radiological, and histological variability. There are two histological varieties of it, one as psammomatoid type and second as trebacular type; here, we present a trebacular type, which is a rare variety. After the clinical examination, radiological and histological analysis, it was diagnosed as juvenile trebacular ossifying fibroma. Although JOF is an uncommon clinical entity, its aggressive local behavior and high recurrence rate means that it is important to make an early diagnosis, apply the appropriate treatment, and, especially, follow-up the patient over the long term. How to cite this article Ghule AS, Achath DD, Kanchan- Talreja P, Bhatnagar S. Juvenile Aggressive Trabecular Ossifying Fibroma of Mandible: A Rare Case Report. J Contemp Dent 2016;6(1):45-51.

scholarly journals Juvenile Ossifying Fibroma of the Maxilla: A Case Report

1970 ◽  
Vol 9 (1) ◽  
pp. 49-52
Author(s):  
NTH Syarifah ◽  
AR Roselinda ◽  
M Irfan
Keyword(s):  
Case Report ◽  
Clinical Presentation ◽  
Age Of Onset ◽  
Medical Science ◽  
Ossifying Fibroma ◽  
Local Growth ◽  
Juvenile Ossifying Fibroma

Juvenile ossifying fibroma (JOF) is an uncommon, benign, bone-forming neoplasm with aggressive local growth that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation and potential behaviour. We reported a 10 year old girl presenting with a growth in the left upper buccal gingiva which later was diagnosed as juvenile ossifying fibroma. The nature of the disease and outline of management were discussed. Keywords: Ossifying fibroma; Juvenile; Maxilla. DOI: 10.3329/bjms.v9i1.5231 Bangladesh Journal of Medical Science Vol.09 No.1 Jan 2010 49-52

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