223 results match your criteria Osteofibrous Dysplasia


Approach to Primary Vertebral Tumors in the Light of the 2020 Updated World Health Organization Classification of Bone Tumors.

Turk Neurosurg 2021 Dec 22. Epub 2021 Dec 22.

Umraniye Education and Research Hospital, Department of Pathology, Istanbul, Turkey.

Aim: The spinal canal frequently harbors primary and secondary bone tumors. These tumors have specific localization, age range, and clinical and radiological manifestations, and their treatment requires a multidisciplinary approach. In most cases, a definitive diagnosis is made based on clinical, radiological, and histopathological findings. Read More

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December 2021

Osteofibrous dysplasia-like adamantinoma of isolated fibula in a child mimicking chronic osteomyelitis with pathological fracture.

J Surg Case Rep 2022 Jun 7;2022(6):rjac196. Epub 2022 Jun 7.

Cliniques Universitaires Saint-Luc, Service de Chirurgie Orthopédique et Traumatologique, Bruxelles, Belgium.

The occurrence of a pathological fracture in children requires a rigorous diagnostic approach in order to establish the etiology and to develop a precise therapeutic strategy. Several causes are associated with these fractures, the most frequent being benign tumors in children in developed countries and chronic osteomyelitis in developing countries. More rarely, malignant tumors must however always be considered. Read More

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Osteofibrous dysplasia of the tibia : the importance of deformity in surveillance.

Bone Joint J 2022 Feb;104-B(2):302-308

Great Ormond Street Hospital for Children, London, UK.

Aims: Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. Read More

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February 2022

Osteofibrous dysplasia: A rare case in 3-day-old female.

Radiol Case Rep 2022 Mar 31;17(3):825-831. Epub 2021 Dec 31.

Department of Radiology, AORN Santobono Pausilipon, Pediatric Hospital, Naples, Italy.

Osteofibrous dysplasia (OFD) is a nonneoplastic tumor-like lesion, made up of fibrous matrix with immature bone tissue surrounded by osteoblasts, occurring usually in the cortex of tibial diaphysis. OFD is usually seen in the first decade of life and, according to literature, it is rarely seen in the newborn period. Diagnosis of congenital OFD in the newborn is challenging because it is uncommon in this age group and can be confused with other bone benign or malignant lesions. Read More

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Hemi-tibia allograft and free microvascularized fibula transplant reconstitute the tibia shaft with side to side healing: 7 year follow up of a 14-year-old boy with adamantinoma.

Case Reports Plast Surg Hand Surg 2021 11;8(1):175-180. Epub 2021 Nov 11.

Morristown Medical Center, Morristown, NJ, USA.

Adamantinoma is a malignant tumor that usually presents in adult men between 20 and 50 years. Due to its metastatic potential, differentiating Adamantinoma from Osteofibrous dysplasia is essential as treatment varies greatly. We present a case of limb salvage using a free microvascularized fibula transplant and hemi-tibia allograft. Read More

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November 2021

Osteofibrous Dysplasia and Adamantinoma.

Surg Pathol Clin 2021 Dec 7;14(4):723-735. Epub 2021 Oct 7.

Department of Pathology, L25, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA. Electronic address:

For decades, the diagnosis, treatment, and even pathogenesis of the osteofibrous dysplasia/osteofibrous dysplasia-like adamantinoma/classic adamantinoma spectrum of neoplasms have been controversial. Herein, we discuss and illustrate the radiographic and histologic spectrum, differential diagnoses, unifying chromosomal and molecular abnormalities, and current controversies and treatment recommendations for each entity. Read More

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December 2021

A simple management of massive bone defect after en-bloc resection of osteofibrous dysplasia of tibial shaft: A case report.

Int J Surg Case Rep 2021 Aug 20;85:106213. Epub 2021 Jul 20.

Department of Orthopaedic and Traumatology, Faculty of Medicine Universitas Indonesia, Cipto Mangunkusumo General Hospital, Jakarta, Indonesia. Electronic address:

Introduction: Osteofibrous dysplasia is a relatively rare disease, exclusively found in children, affecting the tibial diaphysis. Various management approaches are already available, but an internationally approved management guideline is not yet established. There is a major concern in the current management of wide excision technique as it frequently results in massive bone defect. Read More

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[Osteofibrous dysplasia-like adamantinoma of bone: a clinicopathological study of five cases].

Zhonghua Bing Li Xue Za Zhi 2021 Aug;50(8):943-945

Department of Pathology, Xijing Hospital, the Fourth Military Medical University, Xi'an 710032, China.

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Osteofibrous dysplasia and adamantinoma: A summary of diagnostic challenges and surgical techniques.

Surg Oncol 2021 Sep 16;38:101626. Epub 2021 Jun 16.

Department of Orthopaedic Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Osteofibrous dysplasia is an indolent benign fibro-osseous tumor, while adamantinoma is a locally aggressive biphasic malignancy with epithelial and fibro-osseous components. Predominantly arising in the tibial diaphysis of children and young adults, both tumors are resistant to chemotherapy and radiation. Wide surgical resection is regarded as the mainstay of therapy for adamantinoma, and limb-salvage reconstructive procedures can achieve good functional outcomes, albeit with non-negligible rates of complications. Read More

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September 2021

An unusual case of adamantinoma of long bone.

Autops Case Rep 2021 27;11:e2021276. Epub 2021 May 27.

School of Medical Sciences and Research, Sharda Hospital, Department of Pathology, Greater Noida, Uttar Pradesh, India.

Adamantinoma of the long bones is an exceedingly rare and slow-growing tumor that affects the diaphysis of long bones, particularly the tibia. Based on the pattern of the epithelial cell component and the presence or absence of the osteofibrous dysplasia-like element, several histological variants have been described, such as (i) tubular (the most frequent), (ii) basaloid, (iii) squamous, (iv) spindle variant, (v) osteofibrous dysplasia -like variant, and (vi) Ewing's sarcoma - like adamantinoma (the least frequent). The diagnosis may be challenging since this tumor may be mistakenly interpreted as carcinoma, myoepithelial tumor, osteofibrous dysplasia, and vascular tumor. Read More

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Increased uptake of Ga-DOTA-FAPI-04 in bones and joints: metastases and beyond.

Eur J Nucl Med Mol Imaging 2022 01 9;49(2):709-720. Epub 2021 Jul 9.

Department of Nuclear Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1277 Jiefang Ave, Wuhan, 430022, China.

Purpose: To describe the uptake of Gallium-labelled fibroblast activation protein inhibitor (Ga-FAPI) in the bones and joints for better understanding of the role of Ga-FAPI PET in benign and malignant bone lesions and joint diseases.

Methods: All 129 Ga-FAPI PET/MR or PET/CT scans from June 1, 2020, to February 20, 2021, performed at our PET center were retrospectively reviewed. Foci of elevated Ga-FAPI uptake in the bones and joints were identified. Read More

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January 2022

Quantitative bone SPECT/CT applications for primary bone neoplasms.

Hell J Nucl Med 2021 Jan-Apr;24(1):36-44. Epub 2021 Apr 20.

Department of Radiology, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501 Japan.

Objective: To evaluate the clinical utility of quantitative values obtained with bone single photon emission computed tomography/computed tomography (SPECT/CT) for primary bone neoplasms.

Subjects And Methods: Bone SPECT/CT scans of 23 patients with 19 benign bone neoplasms (5 osteoid osteomas, 4 bone giant cell tumor, 4 osteofibrous dysplasia, 3 intraosseous ganglion, 2 aneurysmal bone cyst, 1 intraosseous hemangioma) and 5 malignant bone neoplasms (2 osteosarcoma, 1 periosteal osteosarcoma, 1 malignancy in bone giant cell tumor, 1 Ewing sarcoma) were retrospectively analyzed with maximum standardized uptake value (SUVmax), peak SUV (SUVpeak), mean SUV (SUVmean), metabolic bone volume (MBV), and total bone uptake (TBU) of primary lesions.

Results: Mean SUVmax of 19 benign and 5 malignant primary bone neoplasms were 6. Read More

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October 2021

Does the management of osteofibrous dysplasia of the tibia and fibula in children should be tailored to the extent and location of the lesion? A case control study investigating different surgical options.

Orthop Traumatol Surg Res 2021 Mar 10:102888. Epub 2021 Mar 10.

Department of Pediatric Orthopedics, Fuzhou Second Hospital Affiliated to Xiamen University, 47th Shangteng Road of Cangshan District, Fuzhou 350007, Fujian Province, China. Electronic address:

Background: Osteofibrous dysplasia (OFD) is a rare non neoplastic, self-limited intracortical fibro-osseous lesion that most commonly affects the diaphysis of the tibia and fibula of children, the best treatment is still debated. Therefore we performed a retrospective study in children mostly under 10 years old with OFD aiming to determine whether early surgery is necessary and which is the best treatment.

Hypothesis: Symptomatic OFD lesions should be treated proactively, and appropriate treatment can achieve favorable outcome. Read More

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Osteofibrous dysplasia-like adamantinoma versus osteofibrous dysplasia in children: A case report of challenging diagnosis.

Int J Surg Case Rep 2021 Mar 28;80:105599. Epub 2021 Jan 28.

Musculoskletal Pathology Division, Departement of Anatomic Pathology, Faculty of Medicine Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia. Electronic address:

Introduction: Osteofibrous dysplasia (OFD) and Osteofibrous dysplasia-like Adamantinoma have a similar appearance both in clinical and radiography, but different in its histopathology. Despite this similarity, the treatment and prognosis are different, therefore the diagnosis should be established precisely.

Case Illustration: A three-year-old boy was admitted to hospital after falling on his lower leg. Read More

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Bilateral Symmetric Sporadic Osteofibrous Dysplasia: an Unusual Case.

Indian J Surg Oncol 2020 Sep 8;11(Suppl 2):307-310. Epub 2020 Oct 8.

Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029 India.

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September 2020

Fibrous osteodystrophy in a dromedary camel.

J Vet Diagn Invest 2021 Jan 14;33(1):144-148. Epub 2020 Oct 14.

Virginia-Maryland College of Veterinary Medicine, Virginia Tech, Blacksburg, VA.

A 6-y-old female dromedary camel ( L.) was presented for assessment of firm, bilateral swellings rostral and ventral to the eyes. Serum biochemistry revealed hyperglycemia (28. Read More

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January 2021

Recurrent adamantinoma of the mandible.

Int J Oral Maxillofac Surg 2021 Aug 6;50(8):1023-1026. Epub 2020 Dec 6.

Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology, Beijing, China. Electronic address:

Adamantinoma is an extremely rare tumour originating from bone that can be divided into classical and osteofibrous dysplasia (OFD)-like adamantinoma. This low-grade malignancy almost exclusively occurs in long bones, and only few cases of mandibular adamantinoma have been reported. Here, we report the case of a 30-year-old female with a 2-year history of right mandible tenderness. Read More

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High SENP3 Expression Promotes Cell Migration, Invasion, and Proliferation by Modulating DNA Methylation of E-Cadherin in Osteosarcoma.

Technol Cancer Res Treat 2020 Jan-Dec;19:1533033820956988

Department of Plastic Surgery, The 3rd Xiangya Hospital, 504354Central South University, Changsha, PR China.

SENP3, a sentrin/SUMO2/3-specific protease, is recognized as a transcriptional factor that accumulates under cellular oxidative stress and plays a significant role in the removal of SUMO2/3 modification. In our study, we examined a TCGA dataset and found that the transcripts per million (TPM) value of SENP3 is high in sarcoma, including osteosarcoma (OS). We found that SENP3 was highly expressed in OS cancer tissues when compared with osteofibrous dysplasia tissues. Read More

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November 2021

What's New in Pediatric Orthopaedic Tumor Surgery.

J Pediatr Orthop 2021 Feb;41(2):e174-e180

Department of Orthopaedic Surgery, Children's Hospital of Philadelphia, Philadephia, PA.

Background: Pediatric Orthopaedic Oncology is a developing subspecialty within the field of Pediatric Orthopaedics. Traditionally, the field of Orthopaedic Oncology has been focused on the skeletally mature individual, and the research tends to be all encompassing rather than truly evaluating isolated populations. The purpose of this review is to summarize the most clinically relevant literature in the field of Pediatric Orthopaedic Oncology over the last 6 years. Read More

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February 2021

Surgical Outcome and Oncological Survival of Osteofibrous Dysplasia-Like and Classic Adamantinomas: An International Multicenter Study of 318 Cases.

J Bone Joint Surg Am 2020 10;102(19):1703-1713

Departments of Orthopedic Surgery (E.M.S., P.D.S.D., and M.A.J.v.d.S.), Histopathology (J.V.M.G.B.), and Pathology (P.C.W.H.), Leiden University Medical Center, Leiden, the Netherlands.

Background: Osteofibrous dysplasia-like adamantinoma (OFD-AD) and classic adamantinoma (AD) are rare, neoplastic diseases with only limited data supporting current treatment protocols. We believe that our retrospective multicenter cohort study is the largest analysis of patients with adamantinoma to date. The primary purpose of this study was to describe the disease characteristics and evaluate the oncological outcomes. Read More

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October 2020

[Tumor-like bony lesions of the skeleton].

Orthopade 2020 Sep;49(9):825-838

Klinik für Orthopädie und Unfallchirurgie, Comprehensive Cancer Center Freiburg CCCF, Medizinische Fakultät, Universitätsklinikum Freiburg, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland.

Tumor-like bony lesions are, by definition bony lesions, which can be clinically, radiologically and histologically mistaken for real bone tumors. This article presents the aneurysmal bone cyst (ABC), solitary bone cyst (SBC), fibrous dysplasia, osteofibrous dysplasia Campanacci and non-ossifying fibroma (NOF). Many tumor-like bony lesions are often incidental findings. Read More

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September 2020

Osteofibrous dysplasia-like adamantinoma treated via intercalary segmental resection with partial cortex preservation using pedicled vascularized fibula graft: a case report.

World J Surg Oncol 2020 Aug 13;18(1):203. Epub 2020 Aug 13.

Department of Orthopedic Surgery, Sapporo Medical University School of Medicine, West 16, South 1, Chuo- ku, Sapporo, Hokkaido, 060-8543, Japan.

Background: Morphologically, osteofibrous dysplasia-like adamantinoma is thought to be intermediate between osteofibrous dysplasia and adamantinoma. Its treatment is not well established owing to its rarity.

Case Presentation: We report about of a 10-year-old girl with osteofibrous dysplasia-like adamantinoma initially diagnosed as osteofibrous dysplasia and treated via intercalary segmental resection with partial cortex preservation using a pedicled vascularized fibula graft for reconstruction. Read More

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Outcome of osteofibrous dysplasia-like versus classic adamantinoma of long bones: a single-institution experience.

J Orthop Surg Res 2020 Jul 16;15(1):268. Epub 2020 Jul 16.

Department of Orthopaedic Oncology Surgery, Beijing Jishuitan Hospital, Peking University, Number 31, Xinjiekoudongjie Street, Xicheng District, Beijing, 100035, China.

Background: The clinical and molecular characteristics of osteofibrous dysplasia (OFD)-like adamantinoma (AD) differ from those of classic AD. Most reports about OFD-like AD are case reports or small case series. More cases from different centers are still warranted. Read More

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Keratinocytic epidermal nevi associated with localized fibro-osseous lesions without hypophosphatemia.

Pediatr Dermatol 2020 Sep 14;37(5):890-895. Epub 2020 Jul 14.

Department of Dermatology, University Hospitals of Leuven, Leuven, Belgium.

Keratinocytic epidermal nevi (KEN) are characterized clinically by permanent hyperkeratosis in the distribution of Blaschko's lines and histologically by hyperplasia of epidermal keratinocytes. KEN with underlying RAS mutations have been associated with hypophosphatemic rickets and dysplastic bone lesions described as congenital cutaneous skeletal hypophosphatemia syndrome. Here, we describe two patients with keratinocytic epidermal nevi, in one associated with a papular nevus spilus, who presented with distinct localized congenital fibro-osseous lesions in the lower leg, diagnosed on both radiology and histology as osteofibrous dysplasia, in the absence of hypophosphatemia or rickets, or significantly raised FGF23 levels but with distinct mosaic HRAS mutations. Read More

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September 2020

[Tumor-like bony lesions of the skeleton].

Radiologe 2020 Jul;60(7):655-668

Klinik für Orthopädie und Unfallchirurgie, Comprehensive Cancer Center Freiburg CCCF, Medizinische Fakultät, Universitätsklinikum Freiburg, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland.

Tumor-like bony lesions are, by definition bony lesions, which can be clinically, radiologically and histologically mistaken for real bone tumors. This article presents the aneurysmal bone cyst (ABC), solitary bone cyst (SBC), fibrous dysplasia, osteofibrous dysplasia Campanacci and non-ossifying fibroma (NOF). Many tumor-like bony lesions are often incidental findings. Read More

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Fibrous dysplasia limited to an ossicle.

Auris Nasus Larynx 2021 Aug 27;48(4):783-787. Epub 2020 May 27.

Beckman Laser Institute Korea, College of Medicine, Dankook University, Cheonan, South Korea; Department of Otolaryngology-Head & Neck Surgery, College of Medicine, Dankook University Cheonan, South Korea. Electronic address:

Fibrous dysplasia is an unusual pathologic condition caused by abnormal bone metabolism. Temporal bone involvement is often seen, but it is uncommon to find fibrous dysplasia limited to the middle ear, especially originating in and confined to a single ossicle. Here we report a case of osteofibrous dysplasia limited exclusively to an ossicle (malleus) causing gradual conductive hearing loss, which recovered after eventual complete removal of the dysplastic area. Read More

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Adamantinomatous tumors: Long-term follow-up study of 20 patients treated at a single institution.

J Surg Oncol 2020 Aug 25;122(2):273-282. Epub 2020 Apr 25.

Department of Surgery, Orthopaedic Service, Memorial Sloan-Kettering Cancer Center, New York, New York.

Background And Objectives: Adamantinomas are primary, low-grade malignant tumors of the bone that have metastatic potential to the lungs, lymph nodes, and other regions. The rarity of this disease and its nonspecific symptoms complicate diagnosis.

Materials And Methods: Records for 20 patients who underwent treatment for adamantinoma from 1975 to 2018 were reviewed for demographic, clinical, and pathological data, treatment details, postoperative complications, and outcomes. Read More

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Osteofibrous Dysplasia in a Cockatiel ().

J Avian Med Surg 2020 Mar;34(1):83-88

Department of Avian Diseases, Faculty of Veterinary Medicine, University of Tehran, Tehran, Iran.

Osteofibrous dysplasia is a rare and benign nonneoplastic condition of unknown etiology in humans and mammals. An adult female cockatiel () was presented with the following problems: a soft tissue mass on the left frontal area of the head that extended over the left eye, anorexia, lethargy, and emaciation. Computed tomographic imaging and ultrasonography revealed a soft tissue opacity in the left cranial area of the head with multifocal heterogeneous opacities and foci of mineralization. Read More

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Surgical Outcomes, Complications, and Long-Term Functionality for Free Vascularized Fibula Grafts in the Pediatric Population: A 17-Year Experience and Systematic Review of the Literature.

J Reconstr Microsurg 2020 Jun 23;36(5):386-396. Epub 2020 Feb 23.

Division of Orthopedic Surgery, Children's Hospital of Los Angeles, Los Angeles, California.

Background:  In the pediatric population, bony defects of the extremities pose a significant challenge for which free vascularized fibular grafts (FVFGs) represent a valuable reconstructive option. The purpose of this study was to explore surgical outcomes, complications, and long-term functionality of FVFG for this patient group.

Methods:  Using MEDLINE and PubMed databases, studies were identified of pediatric extremity reconstruction using FVFG which reported functional outcomes and/or complications. Read More

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