212 results match your criteria Osteofibrous Dysplasia


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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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 2020 Dec 6. 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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December 2020

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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October 2020

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 Oct;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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Osteolytic lesion of the tibial shaft in a young boy.

BMJ Case Rep 2020 Feb 11;13(2). Epub 2020 Feb 11.

Pathology, Indraprastha Apollo Hospital, New Delhi, India.

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February 2020

AMPH-1 As A Critical Tumor Suppressor That Inhibits Osteosarcoma Progression.

Cancer Manag Res 2019 25;11:9913-9919. Epub 2019 Nov 25.

Department of Laboratory Medicine, The Fourth People's Hospital of Nantong, Jiangsu 226000, People's Republic of China.

Introduction: Amphiphysin 1 (AMPH-1) is involved in endocytosis, and its expression is upregulated in osteosarcoma compared with osteofibrous dysplasia.

Methods: We investigated the role of AMPH-1 in osteosarcoma cells via both gain-of-function and loss-of-function experiments.

Results: Knockdown of in osteosarcoma cells promoted cell cycle progression and cell proliferation and attenuated apoptosis. Read More

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November 2019

Growth hormone receptor promotes osteosarcoma cell growth and metastases.

FEBS Open Bio 2020 01 18;10(1):127-134. Epub 2019 Dec 18.

Department of Musculoskeletal Surgery, Fudan University Shanghai Cancer Center, China.

Osteosarcoma (OS) is the primary bone malignancy in children and adolescents, with a high incidence of lung metastasis and poor prognosis. Here, we report that growth hormone receptor (GHR) is overexpressed in OS samples compared with osteofibrous dysplasia. We subsequently demonstrated that GHR knockdown inhibited colony formation, promoted cell apoptosis and decreased the number of cells at G2/M phase in 143B and U2OS cells. Read More

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January 2020

Adamantinoma filling the medullary space of the tibia: A case report.

Authors:
Jialu Chen Ji Zhang

Radiol Case Rep 2019 Nov 5;14(11):1330-1333. Epub 2019 Sep 5.

Department of Medical imaging, Hefei Second People's Hospital of Wannan Medical College, Guangde Road, Hefei, 230011, China.

Adamantinoma of the bone is a rare low-grade bony tumor that accounts for less than 1% of all primary bone tumors. On imaging, adamantinoma may be similar to other tumors such as osteofibrous dysplasia, for which the treatment protocol is completely different. Therefore, correct diagnosis and staging of adamantinoma ensures that the patient will undergo appropriate surgery. Read More

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November 2019

Osteofibrous Dysplasia of the Tibia in Children: Outcome Without Resection.

J Pediatr Orthop 2019 Sep;39(8):e614-e621

The Hospital for Sick Children, Toronto, ON, Canada.

Background: The proposed association between osteofibrous dysplasia and adamantinoma has led some to advocate resection of the entire lesion, which can require major subsequent reconstruction. However, this link remains unproven and there is some support in more recent literature for a less aggressive approach. This study aims to describe our experience managing pediatric tibial osteofibrous dysplasia with such an approach and to report functional outcomes in children treated thus. Read More

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September 2019

Comprehensive Molecular Characterization of Adamantinoma and OFD-like Adamantinoma Bone Tumors.

Am J Surg Pathol 2019 07;43(7):965-974

Institute of Cancer and Genomic Sciences, College of Medical and Dental Sciences, University of Birmingham.

Adamantinoma and osteofibrous dysplasia (OFD)-like adamantinoma are rare primary bone tumors that are predominantly confined to the tibia. These 2 entities show similarities in location, histology, and radiologic appearance; however, adamantinoma is malignant and therefore differentiating between these bone tumors is essential for optimal patient care. To elucidate their genomic and transcriptomic alteration profiles and expand their etiological mechanisms, whole exome sequencing (WES) and RNA sequencing (RNA-Seq) were conducted on adamantinoma and OFD-like adamantinoma tumors. Read More

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Osteofibrous dysplasia arising in the humerus: A case report.

Rare Tumors 2018 5;10:2036361318808852. Epub 2018 Nov 5.

Department of Pathology, Tohoku University Hospital, Sendai, Japan.

Osteofibrous dysplasia is a benign fibro-osseous lesion of bone which is most commonly occurred in cortical bone of anterior mid-shaft of the tibia of infancy and childhood. This study reported a case of osteofibrous dysplasia arising in the humerus of adult, resulting in good prognosis after a surgical treatment. A 34-year-old male had felt left upper arm pain and was suspected as having a bone tumor at the humeral shaft by X-ray pictures. Read More

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November 2018

Giant monostotic osteofibrous dysplasia of the ilium: A case report and review of literature.

World J Clin Cases 2018 Nov;6(14):830-835

Department of Orthopedics, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215002, Jiangsu Province, China.

Background: Osteofibrous dysplasia (OFD) is a developmental skeletal disorder, and cases with a giant affected area in the pelvis are rare.

Case Summary: In this case report, a 48-year-old man presented with a large tumor in the right iliac region that turned out to be OFD. The patient had rebound tenderness in his right hip. Read More

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November 2018

Extraperiosteal segmental excision for osteofibrous dysplasia of tibia with reconstruction by liquid nitrogen-treated recycled autograft.

J Orthop Sci 2019 Mar 30;24(2):342-346. Epub 2018 Oct 30.

Department of Orthopedics, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, People's Republic of China. Electronic address:

Background: Osteofibrous dysplasia usually progresses until ten years of age and occasionally regresses spontaneously after puberty. Patients with osteofibrous dysplasia usually require close observation. Surgery is an option considered only for extensive, deforming lesions and those with pathological fractures and rapid progression prior to puberty. Read More

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Adamantinoma of bone: Long-term follow-up of 46 consecutive patients.

J Surg Oncol 2018 Dec 17;118(7):1150-1154. Epub 2018 Oct 17.

Department of Orthopedic Surgery, Mayo Clinic, Rochester, Minnesota.

Background: Adamantinomas are rare bone tumors, commonly affecting the tibia. Due to the rare nature of disease, previous studies are small or from multiple centers. The purpose of this study is to investigate outcomes of patients with adamantinoma treated in a single institution. Read More

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December 2018