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Ruengwanichayakun P, Marrari A, Mazzatenta D, Baldi GG, Bertolini P, Piwchan S, Bercich L, Facco C, Turri-Zanoni M, Silini EM, Spinnato P, Gentilini F, Gianfranco V, Riccardo D, Tonon C, Gambarotti M, Lodi R, Foschini MP, Asioli S, Righi A. Skull Base Osteosarcoma: An Analysis of 9 Cases and Literature Review. Head Neck Pathol 2025; 19:64. [PMID: 40374813 DOI: 10.1007/s12105-025-01801-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2025] [Accepted: 04/28/2025] [Indexed: 05/18/2025]
Abstract
PURPOSE Skull base osteosarcoma is an exceedingly rare malignancy. Unlike maxillofacial osteosarcomas, there are only few cases of skull base tumors reported in literature, and their clinical behavior and treatment outcome are not well defined. This study aims to characterize the clinical features and outcomes of primary skull base osteosarcoma based on our experiences of 9 cases and to review available literature data. METHODS A retrospective analysis was done on 9 cases of skull base osteosarcoma diagnosed at IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy, from 1995 to 2023. Clinicopathologic features were reported, and survival outcomes were analyzed. Literature review was performed by searching the PubMed database for published cases of skull base osteosarcoma. Kaplan-Meier survival analysis was used to calculate 1-year, 3-year, and 5-year survival rates. RESULTS Patients were 5 males and 4 females, with an age range of 5-72 years (mean: 39 years). Tumor location was frontal (3), sphenoid/ethmoid (3), occipital (2), and temporal (1) bone. Seven patients had subtotal resections; Two had total resection, but only one achieved negative margin. Follow-up duration ranged from 4 to 256 months (median: 28 months). Local recurrence occurred in 4 patients. At last follow-up, 5 patients were alive, with 3 had no evidence of disease (NED) and 2 alive with disease (AWD), while 4 patients died of disease (DOD). One-year, 3-year, and 5-year survival were 88.9%, 76.2%, and 57.1%, respectively. Literature review showed 1-year, 3-year, and 5-year survival of 50.3%, 22.6%, and 6.7%, respectively. CONCLUSION Skull base osteosarcoma is a rare malignancy with limited data on its clinical behavior. Surgical resection with negative margins is critical for better outcomes but is challenging due to the complex anatomy. Multidisciplinary treatment approaches are essential to improve our understanding of the tumor and survival outcomes.
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Affiliation(s)
| | - Andrea Marrari
- Innovative Therapy Unit, Osteoncology, Soft Tissue and Bone Sarcomas, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
| | - Diego Mazzatenta
- Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
- IRCCS Istituto Delle Scienze Neurologiche Di Bologna, Programma Neurochirurgia Ipofisi - Pituitary Unit, Bologna, Italy
| | - Giacomo Giulio Baldi
- Department of Oncology, Hospital of Prato, Azienda USL Toscana Centro, Prato, Italy
| | - Patrizia Bertolini
- Dipartimento Materno-Infantile, UOC Oncoematologia Pediatrica, Azienda Ospedaliero Universitaria Di Parma, Parma, Italy
| | - Setthachai Piwchan
- Department of Medicine, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand
| | - Luisa Bercich
- Department of Pathology, ASST Spedali Civili of Brescia, Brescia, Italy
| | - Carla Facco
- Department of Pathology, ASST Settelaghi, Ospedale Di Circolo, Varese, Italy
| | - Mario Turri-Zanoni
- Department of Otorhinolaryngology, Ospedale Sant'Anna, ASST Lariana, University of Insubria, Como, Italy
| | - Enrico Maria Silini
- Surgical Pathology Unit, Azienda Ospedaliero-Universitaria Di Parma, Parma, Italy
| | - Paolo Spinnato
- Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
| | | | - Vornetti Gianfranco
- Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
- IRCCS Istituto Delle Scienze Neurologiche Di Bologna, Bologna, Italy
| | - Draghi Riccardo
- Maria Cecilia Hospital GVM Care& Research, Cotignola, Ravenna, Italy
| | - Caterina Tonon
- Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
- Functional and Molecular Neuroimaging Unit, IRCCS Istituto Delle Scienze Neurologiche Di Bologna, Bologna, Italy
| | - Marco Gambarotti
- Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
| | - Raffaele Lodi
- Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
- IRCCS Istituto Delle Scienze Neurologiche Di Bologna, Bologna, Italy
| | - Maria Pia Foschini
- Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
- Section of Anatomic Pathology, Bellaria Hospital, Bologna, Italy
| | - Sofia Asioli
- Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
- IRCCS Istituto Delle Scienze Neurologiche Di Bologna, Bologna, Italy
| | - Alberto Righi
- Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.
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Bin Alamer O, Haider AS, Haider M, Sagoo NS, Robertson FC, Arrey EN, Aoun SG, Yu K, Cohen-Gadol AA, El Ahmadieh TY. Primary and radiation induced skull base osteosarcoma: a systematic review of clinical features and treatment outcomes. J Neurooncol 2021; 153:183-202. [PMID: 33999382 PMCID: PMC9312842 DOI: 10.1007/s11060-021-03757-z] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2021] [Accepted: 04/09/2021] [Indexed: 01/28/2023]
Abstract
PURPOSE We aim to systematically review and summarize the demographics, clinical features, management strategies, and clinical outcomes of primary and radiation-induced skull-base osteosarcoma (SBO). METHODS PubMed, Scopus, and Cochrane databases were used to identify relevant articles. Papers including SBO cases and sufficient clinical outcome data were included. A comprehensive clinical characteristic review and survival analysis were also conducted. RESULTS Forty-one studies describing 67 patients were included. The median age was 31 years (male = 59.7%). The middle skull-base was most commonly involved (52.7%), followed by anterior (34.5%) and posterior (12.7%) skull-base. Headache (27%), exophthalmos (18%), and diplopia (10%) were common presenting symptoms. Sixty-eight percent of patients had primary SBO, while 25% had radiation-induced SBO. Surgery was the main treatment modality in 89% of cases. Chemotherapy was administered in 65.7% and radiotherapy in 50%. Median progression-free survival (PFS) was 12 months, and the overall 5-year survival was 22%. The five-year survival rates of radiation-induced SBO and primary SBO were 39% and 16%, respectively (P < 0.05). CONCLUSION SBO is a malignant disease with poor survival outcomes. Surgical resection is the primary management modality, in conjunction with chemotherapy and radiotherapy. Radiation-induced SBO has a superior survival outcome as compared to its primary counterpart. Complete surgical resection showed a statistically insignificant survival benefit as compared to partial resection.
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Affiliation(s)
- Othman Bin Alamer
- College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
| | - Ali S Haider
- Texas A&M University College of Medicine, Houston, TX, USA
| | - Maryam Haider
- McGovern Medical School at University of Texas Health, Houston, TX, USA
| | - Navraj S Sagoo
- University of Texas Medical Branch School of Medicine, Galveston, TX, USA
| | - Faith C Robertson
- Department of Neurosurgery, Massachusetts General Hospital, Boston, MA, USA
| | - Eliel N Arrey
- Department of Surgery, Morehouse School of Medicine, Atlanta, GA, USA
| | - Salah G Aoun
- Department of Neurological Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, USA
| | - Kenny Yu
- Department of Neurosurgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Aaron A Cohen-Gadol
- Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN, USA
| | - Tarek Y El Ahmadieh
- Department of Neurological Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, USA.
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Mathkour M, Garces J, Beard B, Bartholomew A, Sulaiman OAR, Ware ML. Primary High-Grade Osteosarcoma of the Clivus: A Case Report and Literature Review. World Neurosurg 2016; 89:730.e9-730.e13. [PMID: 26836700 DOI: 10.1016/j.wneu.2016.01.054] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2015] [Revised: 01/17/2016] [Accepted: 01/19/2016] [Indexed: 12/26/2022]
Abstract
BACKGROUND Osteosarcoma is the second most common primary tumor of the skeletal system and the most common primary bone tumor. Usually occurring at the metaphysis of long bones, osteosarcomas are highly aggressive lesions that comprise osteoid-producing spindle cells. Craniofacial osteosarcomas comprise <8% and are believed to be less aggressive and lower grade. Primary osteosarcomas of the skull and skull base comprise <2% of all skull tumors. Osteosarcomas originating from the clivus are rare. We present a case of a primar, high-grade clival osteosarcoma. CASE DESCRIPTION A 29-year-old man presented to our institution with a progressively worsening right frontal headache for 3 weeks. There were no sensory or cranial nerve deficits. Computed tomography revealed a destructive mass involving the clivus with extension into the left sphenoid sinus. Magnetic resonance imaging revealed a homogenously enhancing lesion measuring 2.7 × 2.5 × 3.2 cm. The patient underwent endonasal transphenoidal surgery for gross total resection. The histopathologic analysis revealed proliferation of malignant-appearing spindled and epithelioid cells with associated osteoclast-like giant cells and a small area of osteoid production. The analysis was consistent with high-grade osteosarcoma. The patient did well and was discharged on postoperative day 2. He was referred for adjuvant radiation therapy and chemotherapy. Two-year follow-up showed postoperative changes and clival expansion caused by packing material. CONCLUSIONS Osteosarcoma is a highly malignant neoplasm. These lesions are usually found in the extremities; however, they may rarely present in the craniofacial region. Clival osteosarcomas are relatively infrequent. We present a case of a primary clival osteosarcoma with high-grade pathology.
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Affiliation(s)
- Mansour Mathkour
- Department of Neurosurgery, Ochsner Health System, New Orleans, Louisiana, USA; Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA.
| | - Juanita Garces
- Department of Neurosurgery, Ochsner Health System, New Orleans, Louisiana, USA; Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA
| | - Bryce Beard
- Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA
| | - Angela Bartholomew
- Department of Neurosurgery, Ochsner Health System, New Orleans, Louisiana, USA
| | | | - Marcus L Ware
- Department of Neurosurgery, Ochsner Health System, New Orleans, Louisiana, USA
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Vasiliadis HS, Arnaoutoglou C, Plakoutsis S, Doukas M, Batistatou A, Xenakis TA. Low-grade central osteosarcoma of distal femur, resembling fibrous dysplasia. World J Orthop 2013; 4:327-332. [PMID: 24147271 PMCID: PMC3801255 DOI: 10.5312/wjo.v4.i4.327] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/20/2013] [Revised: 06/07/2013] [Accepted: 06/20/2013] [Indexed: 02/06/2023] Open
Abstract
We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intra-operatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision.
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Kim E. Fibrous dysplasia of the clivus. J Korean Neurosurg Soc 2010; 48:441-4. [PMID: 21286483 DOI: 10.3340/jkns.2010.48.5.441] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2010] [Revised: 10/05/2010] [Accepted: 11/25/2010] [Indexed: 11/27/2022] Open
Abstract
Fibrous dysplasia (FD) of craniofacial structures is well documented, however, its involvement of the clivus is seldom described. We report a case of clival FD in a young man who presented with headache localized to the occipital area. The radiological studies revealed a monostotic disease confined to the clivus, with typical findings of hypointensity on magnetic resonance images and ground-glass density on computed tomography. The diagnosis of FD was confirmed on pathological examination of specimens taken through transsphenoidal surgery. The patient showed reduction of symptoms and no change of residual lesion on follow-up imaging taken 2.5 years later after surgery. This study includes clinical aspect, radiographic appearance, differential diagnosis and treatment strategy of this rare skull base lesion.
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Affiliation(s)
- Ealmaan Kim
- Division of Skull Base Surgery, Department of Neurosurgery, Keimyung University School of Medicine, Dongsan Medical Center, Daegu, Korea
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Andresen KJ, Sundaram M, Unni KK, Sim FH. Imaging features of low-grade central osteosarcoma of the long bones and pelvis. Skeletal Radiol 2004; 33:373-9. [PMID: 15175837 DOI: 10.1007/s00256-004-0796-4] [Citation(s) in RCA: 43] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/17/2004] [Revised: 04/01/2004] [Accepted: 04/02/2004] [Indexed: 02/02/2023]
Abstract
PURPOSE To determine the age and gender distribution and imaging features of low-grade central osteosarcoma (LGCOS) of the long bones and pelvis and to discuss our findings in the context of lesions for which LGCOS has been mistaken. MATERIALS AND METHODS We reviewed 99 cases of LGCOS collected between 1919 and 2002 from our institution and pathology consultation files. Adequate imaging was available in 70 cases (36 radiographs only, 17 radiographs/CT, 12 radiographs/MRI, 2 radiographs/CT/MRI, 2 CT only, 1 MRI only, 5 bone scans). RESULTS Patient average age was 30.1+/-14.2 years, with a slight female predominance. The femur and tibia were the most common long bones involved (29 and 20 each) with the majority of these tumors arising around the knee, followed by the fibula, radius, humerus and ulna (four, three, two and one case each). Flat bones were involved in six cases (three pelvis, one rib, two scapulae). Short tubular bones were involved in five cases (two metatarsal, two phalanges, one clavicle). The lesion extended to the end of the affected long bone in 22 of 59 cases. Lesions were large at presentation (mean 7.9+/-4.6 cm, range 2-24). Four radiographic patterns were identified: lytic with varying amounts of thick and coarse trabeculation ( n=22), predominantly lytic with few thin, incomplete trabecula ( n=21), densely sclerotic ( n=17) and mixed lytic and sclerotic ( n=10). Lesions were benign-appearing overall with focally aggressive features. CT or MRI demonstrated cortical breech or extension into the soft tissues in all cases. CONCLUSIONS LGCOS has a variable appearance on radiographs. A frequent pattern is a slow-growing large intracompartmental fibro-osseous lesion with varying amounts of septal ossification associated with focal areas of aggression. A homogeneously sclerotic pattern was also noted. Imaging with CT or MRI was helpful in every instance in our series in identifying areas of soft tissue extension or cortical disruption suggestive of a low-grade malignancy.
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Affiliation(s)
- Kelli J Andresen
- Department of Radiology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA
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