Juvenile ossifying fibroma of the orbit: a rare location

<p class="abstract">Osseous tumors in the craniofacial skeleton of young patients are not very frequent, and tumors involving the walls of the orbital cavity are even more infrequent. Despite being usually slow-growing, even small neoformations can have a local aggressive behavior, displacing and compressing vital structures, and so it is important to perform an early diagnosis in order to avoid the tumor to pose problems on the optic nerve and threaten vision. The case that we are presenting shows an ossifying fibroma in a 19-year-old male from Ethiopia, a benign lesion whose progressive growth caused proptosis and downward displacement of the left eye. Following an adequate radiological diagnosis, we were able to delimit the fibroma existence and location. The surgery was conducted in a conservative form, allowing the patient not to suffer from post-surgery malformations. Histopathological diagnosis was compatible with juvenile ossifying fibroma, psammomatoid variant, arising from an uncommon location – the roof of the orbit. </p>

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Juvenile Ossifying Fibroma in a Juvenile Diabetic: A Case Report with Surgical Management

World Journal of Dentistry ◽

10.5005/jp-journals-10015-1012 ◽

2010 ◽

Vol 1 (1) ◽

pp. 59-64

Author(s):

Jagadish Hosmani ◽

Sujata M Byahatti ◽

Ranganath Nayak ◽

Ramakant Nayak ◽

Bhushan Jayade

Keyword(s):

High Recurrence Rate ◽

Ossifying Fibroma ◽

Histopathological Diagnosis ◽

Massive Growth ◽

Juvenile Ossifying Fibroma ◽

Histological Variants ◽

Long Term Follow Up ◽

Juvenile Diabetic

ABSTRACT Juvenile ossifying fibroma (JOF) is a highly aggressive uncommon fibro-osseous lesion occurring in the facial bones. Two histological variants of JOF, i.e. trabecular and psammomatoid have been reported. Here we present a case of a 20-year-old juvenile diabetic patient with massive growth on her left side of the jaw which was diagnosed as juvenile ossifying fibroma. This huge aggressive mass in the left side of the mandible had lead to complete lingual tilting of molars and premolars. After clinical and radiological examination, a biopsy led to the histopathological diagnosis of mixed variant of juvenile ossifying fibroma. The required surgical treatment (hemimandibulectomy) was undertaken under GA. The aggressive local behavior and high recurrence rate of JOF emphasizes the need for an early diagnosis, and appropriate radical treatment with long-term follow-up. One such case is reviewed and discussed in detail in the following article.

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Rare case of a recurrent juvenile ossifying fibroma of the lumbosacral spine

Journal of Neurosurgery Spine ◽

10.3171/2017.10.spine17947 ◽

2018 ◽

Vol 28 (6) ◽

pp. 647-653 ◽

Cited By ~ 2

Author(s):

Cecilia L. Dalle Ore ◽

Darryl Lau ◽

Jessica L. Davis ◽

Michael M. Safaee ◽

Christopher P. Ames

Keyword(s):

Surgical Management ◽

Case Reports ◽

Benign Lesion ◽

Spinal Column ◽

Spinal Tumors ◽

Ossifying Fibroma ◽

Lumbosacral Spine ◽

Spinal Reconstruction ◽

Juvenile Ossifying Fibroma ◽

High Propensity

Juvenile ossifying fibroma (JOF) is a rare benign bone tumor that occurs most frequently in the craniofacial bones of children and young adults. There are few case reports that describe its involvement outside the craniofacial skeleton, especially within the spinal column. While JOF is classified as a benign lesion, it may be locally aggressive and demonstrate a high propensity for recurrence, even after resection. Definitive surgical management may be challenging in naive cases, but it is particularly challenging in recurrent cases and when extensive spinal reconstruction is warranted. In this report, the authors describe the diagnosis and surgical management of a 29-year-old man who presented with a large recurrent sacral trabecular-subtype JOF. A review of literature regarding JOFs, management of recurrent primary spinal tumors, and sacral reconstruction are discussed.

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Juvenile psammomatoid ossifying fibroma with fluid–fluid levels: an unusual presentation

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-021-00570-6 ◽

2021 ◽

Vol 52 (1) ◽

Author(s):

Linda Kalliath ◽

D. Karthikeyan ◽

Nataraj Pillai ◽

Deepthi Padmanabhan ◽

Partheeban Balasundaram ◽

...

Keyword(s):

Recurrence Rate ◽

Nasal Obstruction ◽

Histopathological Examination ◽

Young Patients ◽

High Recurrence Rate ◽

Ossifying Fibroma ◽

Imaging Findings ◽

Juvenile Ossifying Fibroma ◽

Adjacent Structures ◽

Facial Swelling

Abstract Background Juvenile ossifying fibroma is an uncommon benign fibro-osseous tumor commonly involving the craniofacial skeleton of young patients with locally aggressive behavior and a high recurrence rate. Depending on the site of involvement it can present clinically as nasal obstruction, facial swelling, or proptosis. Case presentation Here we present a case of juvenile ossifying fibroma with an aneurysmal bone cyst component in the left nasal cavity in a 15-year-old boy who presented with gradually progressing left-sided nasal obstruction. Imaging findings were consistent with juvenile ossifying fibroma. Endoscopic resection of the tumor was done, and histopathological examination revealed it to be a psammomatoid variant of juvenile ossifying fibroma. Here we discuss its imaging findings, differential diagnosis, treatment options, and histopathological features. Conclusion Despite being a slow-growing benign tumor, early diagnosis and treatment are necessary due to its locally aggressive nature and invasion of adjacent structures. Complete surgical resection is the mainstay of treatment because of the high recurrence rate.

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Juvenile Psammomatoid Cemeto-ossifying Fibroma of Mandible: a Diagnostic dilemma

BMJ Case Reports ◽

10.1136/bcr-2020-240952 ◽

2021 ◽

Vol 14 (3) ◽

pp. e240952

Author(s):

Santhosh Rao ◽

Vandita Singh ◽

Abdul Hafeez A ◽

Subham S Agarwal

Keyword(s):

Recurrence Rate ◽

Benign Lesion ◽

Malignant Lesion ◽

Clinical Importance ◽

Metastatic Potential ◽

High Recurrence Rate ◽

Ossifying Fibroma ◽

Diagnostic Dilemma ◽

Juvenile Ossifying Fibroma ◽

Rare Phenomena

Psammomatoid Juvenile ossifying fibroma (PsJOF) is a rare benign fibro-osseous lesion characterised to grow to unusually large size very rapidly. Its usual presentation is in younger age group mostly children and predominately involving the Sino-Naso-Orbital region. Its aggressive nature gimmicks a malignant lesion but it is rather a benign lesion with a higher recurrence rate than the conventional ossifying fibroma but lacking metastatic potential. The high recurrence rate makes it essential that the lesion is not reconstructed immediately and thorough monitoring in the follow-up period. Lesion of such clinical importance needs to be diagnosed preoperatively to provide a better and radical surgical treatment option, but the variability in its presentation as seen in this case makes it even harder to diagnose. We aim to draw attention to the rare phenomena that PsJOF presents to help readers broaden their purview in diagnosis and thereby manage them accordingly.

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Management of an Extensive Orbitofrontal Juvenile Ossifying Fibroma

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0032-1314351 ◽

2012 ◽

Vol 73 (S 02) ◽

Author(s):

K. Tekeli ◽

A. Toma ◽

P. Minhas ◽

M. Manisali

Keyword(s):

Ossifying Fibroma ◽

Juvenile Ossifying Fibroma

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Soft tissue masses: distribution of entities and rate of malignancy in small lesions

BMC Cancer ◽

10.1186/s12885-020-07769-2 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Felix G. Gassert ◽

Florian T. Gassert ◽

Katja Specht ◽

Carolin Knebel ◽

Ulrich Lenze ◽

...

Keyword(s):

Soft Tissue ◽

Benign Lesion ◽

Maximum Diameter ◽

Histopathological Diagnosis ◽

Tumor Diameter ◽

Mr Images ◽

Tissue Mass ◽

Malignancy Rate ◽

Significant Difference ◽

Soft Tissue Masses

Abstract Background Small soft tissue masses are often falsely assumed to be benign and resected with failure to achieve tumor-free margins. Therefore, this study retrospectively investigated the distribution of histopathologic diagnosis to be encountered in small soft tissue tumors (≤ 5 cm) in a large series of a tertiary referral center. Methods Patients with a soft tissue mass (STM) with a maximum diameter of 5 cm presenting at our institution over a period of 10 years, who had undergone preoperative Magnetic resonance imaging and consequent biopsy or/and surgical resection, were included in this study. A final histopathological diagnosis was available in all cases. The maximum tumor diameter was determined on MR images by one radiologist. Moreover, tumor localization (head/neck, trunk, upper extremity, lower extremity, hand, foot) and depth (superficial / deep to fascia) were assessed. Results In total, histopathologic results and MR images of 1753 patients were reviewed. Eight hundred seventy patients (49.63%) showed a STM ≤ 5 cm and were therefore included in this study (46.79 +/− 18.08 years, 464 women). Mean maximum diameter of the assessed STMs was 2.88 cm. Of 870 analyzed lesions ≤ 5 cm, 170 (19.54%) were classified as superficial and 700 (80.46%) as deep. The malignancy rate of all lesions ≤ 5 cm was at 22.41% (superficial: 23.53% / deep: 22.14%). The malignancy rate dropped to 16.49% (20.79% / 15.32%) when assessing lesions ≤ 3 cm (p = 0.007) and to 15.0% (18.18% / 13.79%) when assessing lesions ≤ 2 cm (p = 0.006). Overall, lipoma was the most common benign lesion of superficial STMs (29.41%) and tenosynovial giant cell tumor was the most common benign lesion of deep STMs (23.29%). Undifferentiated pleomorphic sarcoma was the most common malignant diagnosis among both, superficial (5.29%) and deep (3.57%) STMs. Conclusions The rate of malignancy decreased significantly with tumor size in both, superficial and deep STMs. The distribution of entities was different between superficial and deep STMs, yet there was no significant difference found in the malignancy rate.

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