-
APMIS : Acta Pathologica,... Apr 2022The use of denosumab to treat giant cell tumors of bone (GCT) and other giant cell-containing bone tumors has become more common. While the clinicopathologic features of...
The use of denosumab to treat giant cell tumors of bone (GCT) and other giant cell-containing bone tumors has become more common. While the clinicopathologic features of denosumab-treated giant cell tumors of bone have been well-illustrated, descriptions of other denosumab-treated bone tumors are very limited. Surgical pathology files of two institutions and consultation files from two authors were searched for denosumab-treated aneurysmal bone cysts and denosumab-treated osteoblastomas. Clinicopathologic features were reviewed and analyzed. We identified four patients with denosumab-treated bone tumors other than GCT from our surgical pathology and consultation files, including two aneurysmal bone cysts and two osteoblastomas. All were treated with denosumab for 0.5-7.0 (median 4.5) months. Radiologically, denosumab-treated tumors showed decreased size with increased ossification and mineralization on CT and heterogeneous intermediate to hypointense signal on MRI. Histologically, denosumab-treated aneurysmal bone cyst contained thin, elongated, curvilinear, and anastomosing strands of bone with empty lacunae, while denosumab-treated osteoblastoma showed circumscribed nodules of woven bone lined by small osteoblasts. Denosumab-treated aneurysmal bone cyst and osteoblastoma showed treatment-related morphologic changes that can mimic other bone neoplasms. Their recognition requires correlation with the clinical history of denosumab use and radiologic findings.
Topics: Bone Cysts, Aneurysmal; Bone Neoplasms; Denosumab; Giant Cell Tumor of Bone; Humans; Neoadjuvant Therapy; Osteoblastoma
PubMed: 35114728
DOI: 10.1111/apm.13211 -
Human Pathology Jul 2022Seventeen cases of epithelioid osteoblastoma were reviewed. The tumors most commonly arose from the vertebrae (7 cases), followed by the mandible (3), sacrum (2), bones...
Seventeen cases of epithelioid osteoblastoma were reviewed. The tumors most commonly arose from the vertebrae (7 cases), followed by the mandible (3), sacrum (2), bones of the foot (2), and femur, rib, and scapula (1 each). Patients' ages ranged from 5 to 33 years. The tumors measured from 2.0 to 6.5 cm in the greatest diameter (mean = 4.1 cm) and most patients presented with low-grade pain at the affected site. Imaging studies showed expansile lytic lesions with cortical thickening and a mild rim of sclerosis. Histologically all tumors were characterized by active production of bone with a fibrovascular stroma containing microtrabecular aggregates of bone matrix. The osteoblastic proliferation was atypical and showed enlarged cells with prominent nucleoli and abundant cytoplasm imparting them with a striking epithelioid appearance. Immunohistochemical studies showed variable results that caused difficulties for interpretation; 4 of 12 cases showed strong nuclear positivity for FOS, 2 of 12 cases showed strong diffuse nuclear positivity for FOSB; the remaining cases showed variable, sometimes overlapping patterns, considered to be indeterminate. Ki-67 proliferation marker showed low nuclear positivity (∼2%) in 10 cases and a slight increase (<10%) in two cases. Clinical follow-up was available in 14 patients; one patient experienced a recurrence at six months that was treated with additional curetting; the remainder of the patients were all alive and well without evidence of recurrence from 1 to 22 years (median follow up = 3 years). Epithelioid osteoblastoma is an unusual variant of osteoblastoma that has the potential for simulating a malignancy and does not appear to be associated with a more aggressive behavior.
Topics: Adolescent; Adult; Bone Neoplasms; Child; Child, Preschool; Humans; Osteoblastoma; Young Adult
PubMed: 35337839
DOI: 10.1016/j.humpath.2022.03.006 -
Journal of Oral Pathology & Medicine :... Apr 2023The advances in molecular technologies have allowed a better understanding of the molecular basis of odontogenic cysts and tumours. PTCH1 mutations have been reported in... (Review)
Review
The advances in molecular technologies have allowed a better understanding of the molecular basis of odontogenic cysts and tumours. PTCH1 mutations have been reported in a high proportion of odontogenic keratocyst. BRAF p.V600E are recurrent in ameloblastoma and KRAS p.G12V/R in adenomatoid odontogenic tumour, dysregulating the MAPK/ERK pathway. Notably, BRAF p.V600E is also detected in ameloblastic carcinoma, but at a lower frequency than in its benign counterpart ameloblastoma. Recently, adenoid ameloblastoma has been shown to be BRAF wild-type and to harbour CTNNB1 (β-catenin gene) mutations, further suggesting that it is not an ameloblastoma subtype. CTNNB1 mutations also occur in other ghost-cell-containing tumours, including calcifying odontogenic cysts, dentinogenic ghost cell tumours and odontogenic carcinoma with dentinoid, but the link between CTNNB1 mutations and ghost cell formation in these lesions remains unclear. Regarding mixed tumours, BRAF p.V600E has been reported in a subset of ameloblastic fibromas, ameloblastic-fibrodentinomas and fibro-odontomas, in addition to ameloblastic fibrosarcoma. Such mutation-positivity in a subset of samples can be helpful in differentiating some of these lesions from odontoma, which is BRAF-wild-type. Recently, FOS rearrangements have been reported in cementoblastoma, supporting its relationship with osteoblastoma. Collectively, the identification of recurrent mutations in these aforementioned lesions has helped to clarify their molecular basis and to better understand the interrelationships between some tumours, but none of these genetic abnormalities is diagnostic. Since the functional effect of pathogenic mutations is context and tissue-dependent, a clear role for the reported mutations in odontogenic cysts and tumours in their pathogenesis remains to be elucidated.
Topics: Humans; Ameloblastoma; Proto-Oncogene Proteins B-raf; Odontogenic Tumors; Odontogenic Cysts; Odontoma; Mouth Neoplasms; Carcinoma
PubMed: 36629457
DOI: 10.1111/jop.13401 -
FP Essentials Jun 2020Primary benign bone tumors are uncommon and most often affect children and young adults. They typically are detected incidentally on imaging, though some patients...
Primary benign bone tumors are uncommon and most often affect children and young adults. They typically are detected incidentally on imaging, though some patients present with pain, swelling, or other symptoms. The four main categories of benign bone tumor are: bone-forming (eg, osteoid osteoma, osteoblastoma, fibrous dysplasia, enostosis), cartilage-forming (eg, osteochondroma, enchondroma), connective tissue, and vascular; the latter two are rare. A fifth category is idiopathic (eg, giant cell tumor, aneurysmal bone cyst, simple bone cyst). Osteochondromas are the most common, accounting for 30% to 35% of benign bone tumors. Giant cell tumors account for 20%, osteoblastomas for 14%, and osteoid osteomas for 12%. All others are less common. Diagnosis mainly is via imaging; biopsy rarely is required. Management varies with tumor type, location, symptoms, and risk of recurrence. Some (eg, enchondroma, osteochondroma, fibrous dysplasia, enostosis) typically are asymptomatic, and generally require no intervention. Others (eg, osteoid osteoma, aneurysmal bone cyst, simple bone cyst) can cause symptoms and require percutaneous ablation or surgery. Still others (eg, giant cell tumor, osteoblastoma) can be aggressive and require surgery and other therapies. Malignant transformation is rare for all benign bone tumors, but patients with these tumors should be monitored with serial imaging.
Topics: Biopsy; Bone Neoplasms; Child; Diagnosis, Differential; Humans; Osteoblastoma; Osteoma, Osteoid; Young Adult
PubMed: 32573182
DOI: No ID Found -
The American Journal of Surgical... May 2021Cementoblastomas are rare odontogenic tumors developing in close proximity to the roots of teeth. Due to their striking morphologic resemblance to osteoblastomas of the...
Cementoblastomas are rare odontogenic tumors developing in close proximity to the roots of teeth. Due to their striking morphologic resemblance to osteoblastomas of the peripheral skeleton, we set out to determine whether cementoblastomas harbor the same FOS rearrangements with overexpression of c-FOS as has recently been described for osteoblastomas. In total, 16 cementoblastomas were analyzed for FOS expression by immunohistochemistry and for FOS rearrangements by fluorescence in situ hybridization (FISH). We observed strong and diffuse staining of c-FOS in 71% of cementoblastomas and identified a FOS rearrangement in all cases (n=3) applicable for FISH. In the remaining cases, FISH failed due to decalcification. Cementoblastomas harbor similar FOS rearrangements and show overexpression of c-FOS like osteoblastomas, suggesting that both entities might represent parts of the spectrum of the same disease.
Topics: Adolescent; Adult; Biomarkers, Tumor; Bone Neoplasms; Child; Dental Cementum; Female; Gene Rearrangement; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Male; Middle Aged; Netherlands; Odontogenic Tumors; Osteoblastoma; Proto-Oncogene Proteins c-fos; Switzerland; Tooth Root; Young Adult
PubMed: 33653978
DOI: 10.1097/PAS.0000000000001695 -
Journal of Clinical Medicine Dec 2021Osteoblastoma (OB) is a rare, benign bone tumor, accounting for 1% of all primary bone tumors, which occurs usually in childhood and adolescence. OB is histologically... (Review)
Review
Osteoblastoma (OB) is a rare, benign bone tumor, accounting for 1% of all primary bone tumors, which occurs usually in childhood and adolescence. OB is histologically and clinically similar to osteoid osteoma (OO), but it differs in size. It is biologically more aggressive and can infiltrate extraskeletal tissues. Therapy is required because of severe bone pain worsening at night. Moreover, non-steroid anti-inflammatory drugs (NSAIDs) are not a reasonable long-term treatment option in young patients. Surgical excision, considered the gold standard in the past, is no longer attractive today due to its invasiveness and the difficulty in performing a complete resection. The treatment of choice is currently represented by percutaneous thermoablation techniques. Among these, Radiofrequency ablation (RFA) is considered the gold standard treatment, even when the lesions are located in the spine. RFA is a widely available technique that has shown high efficacy and low complication rates in many studies. Other percutaneous thermoablation techniques have been used for the treatment of OB, including Cryoablation (CA) and laser-ablation (LA) with high success rates and low complications. Nevertheless, their role is limited, and further studies are necessary.
PubMed: 34945013
DOI: 10.3390/jcm10245717 -
Journal of Clinical Medicine Jun 2021Pediatric benign osteocytic tumors include osteoma, enostosis, osteoid osteoma, and osteoblastoma. In pediatric populations, benign bone tumors are more common than... (Review)
Review
Pediatric benign osteocytic tumors include osteoma, enostosis, osteoid osteoma, and osteoblastoma. In pediatric populations, benign bone tumors are more common than malignancies. Benign osteocytic tumors may have a unique clinical presentation that helps narrow the differential diagnosis. A systemic imaging approach should be utilized to reach the diagnosis and guide clinicians in management. Radiographs are the most prevalent and cost-effective imaging modality. Cross-sectional imaging can be utilized for tissue characterization and for evaluation of lesions involving complex anatomical areas such as the pelvis and spine. Computed Tomography (CT) is the modality of choice for diagnosis of osteoid osteoma. CT scan can also be utilized to guide radiofrequency ablation, which has been found to be highly effective in treating osteoid osteoma and osteoblastoma. Enostosis is a no-touch lesion. Osteoma is commonly located in the paranasal sinuses. Osteoma needs an excision if it causes complications due to a mass effect.
PubMed: 34206870
DOI: 10.3390/jcm10132823 -
Pediatric Clinics of North America Jun 2023Back pain is common, in up to 30% of children, increasing with age. Eighty percent is benign, mechanical type, improving within 2 weeks of conservative care. Required... (Review)
Review
Back pain is common, in up to 30% of children, increasing with age. Eighty percent is benign, mechanical type, improving within 2 weeks of conservative care. Required for those not improving is in-depth evaluation, including MRI, laboratory, and peer consultations. Spondylolysis and spondylolisthesis comprise almost 10% of pediatric back pain, often caused by lumbar hyperextension activities and treated conservatively in most cases. Osteoid osteomas and osteoblastomas constitute the most common benign spinal tumors in childhood. Aggressive and malignant tumors of the spine are rare but when present require tertiary care referral and a comprehensive oncology team for optimal life-sustaining outcomes.
Topics: Humans; Child; Back Pain; Spondylolysis; Spondylolisthesis; Magnetic Resonance Imaging; Lumbosacral Region
PubMed: 37121642
DOI: 10.1016/j.pcl.2023.01.013 -
Journal of Pediatric Orthopedics Apr 2021Primary benign osseous tumors and tumor-like lesions at the sacrum are rare in the pediatric population and exact surgical strategy is still unclear. In this study, we...
BACKGROUND
Primary benign osseous tumors and tumor-like lesions at the sacrum are rare in the pediatric population and exact surgical strategy is still unclear. In this study, we evaluate the outcome for pediatric patients with benign tumors and tumor-like lesions at the sacrum who were receiving surgical treatment according to our proposed surgical strategy and classification.
METHODS
We analyzed 49 pediatric patients with sacral benign tumors or tumor-like lesions aged 18 years and below from 2005 to 2018. There were 23 men and 26 women with a mean age of 14.0±3.8 years. Nineteen patients had giant cell tumors (GCTs), 9 aneurysmal bone cysts, 5 osteoblastomas, 5 neurogenic tumors, 3 hemangiomas, 3 teratomas, 2 Langerhans cell histiocytosis, 1 chondroblastoma, 1 fibrous dysplasia, and 1 GCT of tendon sheath. We proposed our surgical plan and surgical classification for pediatric patients with sacral benign tumors or tumor-like lesions.
RESULTS
The mean follow-up duration was 6.2 years (range, 1.0 to 18.9 y). GCTs (39%, 19/49) and primary aneurysmal bone cysts (18%, 9/49) are the top 2 common histologic types. Preoperative selective arterial embolization (SAE) was performed in 12 cases and 24 patients received intraoperative aortic balloon occlusion (ABO) as the preoperative surgical plan. Furthermore, according to tumor location at the sacrum, we classified surgical excision of sacral benign tumors and tumor-like lesions into 3 types. Fourteen cases were classified as type I, 27 as type II, 3 as type III, and 5 patients with neurogenic tumors cannot be classified into this surgical classification. Ten patients had wound complications. Two had femoral artery thrombosis because of ABO application. One had mechanical failure. Rate of local recurrence was 16%. Seven patients with GCTs and 1 with neurogenic tumor had local recurrence. No patient died of disease at the last follow-up. For the assessment of neurological function, the rate of neurological dysfunction was 12% (6/49). Four cases had urinary incontinence, 3 fecal incontinence, and 3 had bowel obstruction. Next, univariate analysis for influence of preoperative SAE and intraoperative ABO on complications demonstrated that both of them exerted no significant influence on the occurrence of oncological and nononcological complications.
CONCLUSIONS
The proposed surgical strategy can provide an excellent therapeutic effect for pediatric benign tumors and tumor-like lesions at the sacrum. Preoperative SAE and intraoperative ABO can safeguard pediatric patients with high vascularity of benign tumor at the sacrum during the operation.
LEVEL OF EVIDENCE
Level IV.
Topics: Adolescent; Aorta; Balloon Occlusion; Bone Cysts, Aneurysmal; Child; Child, Preschool; Chondroblastoma; Fecal Incontinence; Female; Fibrous Dysplasia of Bone; Follow-Up Studies; Giant Cell Tumor of Bone; Hemangioma; Histiocytosis, Langerhans-Cell; Humans; Intestinal Obstruction; Male; Neoplasm Recurrence, Local; Nervous System Diseases; Osteoblastoma; Postoperative Complications; Sacrum; Spinal Neoplasms; Teratoma; Treatment Outcome; Urinary Incontinence
PubMed: 33655901
DOI: 10.1097/BPO.0000000000001738 -
Journal of Pediatric Neurosciences 2021Skull base tumors are varied in children and are particularly challenging to pediatric neurosurgeons, with few papers in the literature describing the evolution,...
CONTEXT
Skull base tumors are varied in children and are particularly challenging to pediatric neurosurgeons, with few papers in the literature describing the evolution, complications, and outcome. The authors evaluated long-term outcomes in children submitted to skull base tumor surgery and performed a literature review.
AIMS
The aim of this study was to analyze surgical results, complications, and outcomes, on comparison with previous publications.
MATERIALS AND METHODS
A retrospective analysis of children undergoing surgery at a single institution between 2000 and 2018 for lesions of the cranial base was carried out. In addition, a literature review was carried out describing a total of 115 children operated on for skull base tumors.
STATISTICAL ANALYSIS
Chi-squared and Fisher's exact tests were performed to compare the distribution of categorical variables and a nonparametric Mann-Whitney test was used to perform intergroup comparisons of continuous variables.
RESULTS
Seventeen children ranging in age from 8 months to 17 years (mean, 10.9 years) underwent skull base approaches. Tumor types included schwannoma, meningioma, chondroid chordoma, mature teratoma, epidermoid cyst, hemangiopericytoma, rhabdomyosarcoma, myofibroblastic inflammatory tumor, fibromyxoid sarcoma, Crooke's cell adenoma, ossifying fibroma, osteoblastoma, nasopharyngeal angiofibroma and Ewing's sarcoma. Gross total resection was achieved in 6 patients (35.3%), 12 patients (70.6%) had benign histology, and 5 patients (29.4%) had a malignant tumor. Transient postoperative cerebrospinal fluid leak affected only one patient. Thirteen children (76.4%) had a residual neurological deficit at last follow-up evaluation. Three (17.6%) surviving patients received adjuvant therapy. The rate of recurrence or lesion progression was 17.6%.
CONCLUSIONS
Skull base tumors in children present a therapeutic challenge because of their unique pathological composition and can lead to considerable morbidity and mortality in pediatric age.
PubMed: 34316306
DOI: 10.4103/jpn.JPN_56_20