Juvenile Psammomatoid Ossifying Fibroma (JPOF) is a type of noncancerous bone tumor that usually affects adolescents in the craniomaxillofacial area. Clinical manifestations are usually symptoms ...caused by the tumor's invasive compression of surrounding tissues. Aneurysmal Bone Cyst (ABC) is also a benign bone tumor, and it typically occurs in long bones and the spine. Only 2% to 3% of cases occur in the head and neck. Due to the rarity of this combination of clinical cases, clinicians face difficulties in comprehensively understanding this complex lesion. Therefore, a comprehensive review of the clinical manifestations and characteristic imaging findings is necessary for surgeons.
On April 6, 2019, a 13-year-old boy presented with left maxillofacial bulge and pain for 1 month. Magnetic resonance imaging of the paranasal sinuses showed an irregular hive-like mass signal in the left maxillary sinus, and cystic changes with fluid levels were seen in the lesion. After the initial diagnosis of JPOF with primary ABC, we decided to perform a facial mid-facial resection of maxillary sinus tumor to remove the tumor tissue. Finally, after 3 recurrences and 4 operations, there was no tumor recurrence for 20 months after the last operation, and the patient was still under continuous follow-up.
This case provided a reference for the diagnosis and treatment of JPOF combined with ABC. In particular, a new understanding of the association between the two diseases and the management of recurrence were proposed, which had the potential to improve clinical understanding of this complicated condition.
Background
To decrease the recurrence rate after intralesional curettage for aneurysmal bone cysts, different adjuvant treatments have been recommended. Liquid nitrogen spray and argon beam ...coagulation have provided the lowest recurrence rates, but unlike the high-speed burr, these adjuvants are not always available in operating rooms.
Questions/purposes
We asked: (1) Is high-speed burring alone sufficient as an adjuvant to curettage with respect to recurrence rates? (2) What is the complication rate from this technique? (3) What are the risk factors for local recurrence?
Methods
A retrospective review of the database of the University Musculoskeletal Tumor Unit and the private files of the senior author (EHW) for a period of 19 years (1993–2011) was performed to identify all patients histologically diagnosed with primary aneurysmal bone cyst. During that period, patients with aneurysmal bone cysts were treated with intralesional curettage, burring, and bone grafting if the lesions showed an adequate cortical wall or a wall with thinned out portions which could be reconstructed with bone grafting. Based on those indications, we treated 54 patients for this condition. Of those, 18 were treated using approaches other than burring because they did not meet the defined indications, and an additional five patients were lost to followup before 2 years, leaving 31 patients for analysis, all of whom were followed up for at least 2 years (mean, 7 years; range, 2–18 years).
Results
Of these 31 patients, one had a recurrence (3.2%). Complications using this approach occurred in three patients (9.7%), and included growth plate deformity (1) and genu varus (2) secondary to collapse of the reconstructed condyle. With only one recurrence, we cannot answer what the risk factors might be for recurrence; however, the one patient with recurrence presented with a large lesion and a pathologic fracture.
Conclusions
Curettage, burring, and bone grafting compare favorably in the literature with other approaches for aneurysmal bone cysts, such as cryotherapy and argon-beam coagulation. We conclude that high-speed burring alone as an adjuvant to intralesional curettage is a reasonable approach to achieving a low recurrence rate for aneurysmal bone cysts.
Level of Evidence
Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.
Aneurysmal bone cyst Rapp, Timothy B; Ward, James P; Alaia, Michael J
Journal of the American Academy of Orthopaedic Surgeons
20, Številka:
4
Journal Article
Recenzirano
Aneurysmal bone cysts are rare skeletal tumors that most commonly occur in the first two decades of life. They primarily develop about the knee but may arise in any portion of the axial or ...appendicular skeleton. Pathogenesis of these tumors remains controversial and may be vascular, traumatic, or genetic. Radiographic features include a dilated, radiolucent lesion typically located within the metaphyseal portion of the bone, with fluid-fluid levels visible on MRI. Histologic features include blood-filled lakes interposed between fibrous stromata. Differential diagnosis includes conditions such as telangiectatic osteosarcoma and giant cell tumor. The mainstay of treatment is curettage and bone graft, with or without adjuvant treatment. Other management options include cryotherapy, sclerotherapy, radionuclide ablation, and en bloc resection. The recurrence rate is low after appropriate treatment; however, more than one procedure may be required to completely eradicate the lesion.
To describe clinical and imaging features and surgical treatment of equine mandibular aneurysmal bone cysts (ABCs) with β-tricalcium phosphate (TCP).
3 horses (cases 1, 2, and 3) and 1 pony (case 4) ...with histologically confirmed ABC.
All cases had mandibular swelling with intact adjacent skin. Cases 1 to 3 had a body condition score of 3/5 and case 4 had 2/5 and showed quidding during mastication and, at oral examination, large interdental spaces and loose elements adjacent to the swelling. Radiography or CT was performed in all cases. In cases 1, 3, and 4, an expansile septate cystic space-occupying lesion with mass effect on the adjacent cortices and teeth was seen without compact bone destruction. Case 2 showed a heterogeneous osteolytic mass with multifocal cortical lysis and interruption. Case 4 had severe dental abnormalities of deciduous and precursors of permanent teeth. ABCs were surgically treated and filled with only TCP (case 3) or in combination with autologous bone marrow (cases 1, 2, and 4).
Cases 1 through 3 showed an uneventful reduction in ABC size with increased opacity/attenuation. In case 4, a surgical site infection occurred. After removal of TCP remnants, the ABC healed satisfactorily, but remaining dental abnormalities necessitated dietary adjustments to maintain an acceptable body condition score.
Treatment of ABCs with TCP had a favorable outcome and good long-term prognosis. In young specimens, the expansile effect on the development and eruption of neighboring teeth can influence and determine final functionality of the diseased dental quadrant.
Aneurysmal bone cyst is a benign bone neoplasm that most commonly arises from the metaphyses of long bones in the first and second decades of life. Here, we describe a case of an aneurysmal bone cyst ...that occurred in the distal tibial diaphysis of a 72‐year‐old female that was concerning for malignancy on imaging, demonstrating cortical breakthrough and soft tissue extension. Histologically, the tumor showed the characteristic morphologic features of aneurysmal bone cyst. Fluorescence in situ hybridization was positive for USP6 rearrangement, and RNA sequencing revealed a USP6 gene fusion with VDR, a novel partner that encodes the vitamin D receptor and that has not been implicated previously in human neoplasia. This case highlights the diagnostic challenges presented by aneurysmal bone cyst in elderly adults, and it expands the genetic spectrum of USP6 rearrangements.
Aneurysmal bone cyst: a review of 150 patients Mankin, Henry J; Hornicek, Francis J; Ortiz-Cruz, Eduardo ...
Journal of clinical oncology,
09/2005, Letnik:
23, Številka:
27
Journal Article
Recenzirano
We have reviewed a series of 150 aneurysmal bone cysts treated over the last 20 years.
The lesions were principally located in the tibia, femur, pelvis, humerus, and spine and, in most cases, ...presented the imaging appearance originally described by Jaffe and Lichtenstein as a blowout with thin cortices.
Only one of the patients was believed to have an osteoblastoma of the spine with secondary development of an aneurysmal bone cyst, and none of the patients developed additional lesions. The patients were treated primarily with curettage and implantation of allograft chips or polymethylmethacrylate, but some patients were treated with insertion of autografts or allografts. The local recurrence rate was 20%, which is consistent with that reported by other centers.
Aneurysmal bone cysts are enigmatic lesions of unknown cause and presentation and are difficult to distinguish from other lesions. Overall, the treatment is satisfactory, but it is possible that newer approaches, such as improved magnetic resonance imaging studies, may help diagnose the lesions and allow the physicians to plan for more effective treatment protocols.
The treatment of aneurysmal bone cysts van Geloven, Thomas P G; van de Sande, Michiel A J; van der Heijden, Lizz
Current opinion in pediatrics,
02/2023, Letnik:
35, Številka:
1
Journal Article
Recenzirano
Odprti dostop
Aneurysmal bone cysts are rare, locally aggressive bone tumors. Optimal treatment of ABCs is still matter of debate as therapies including sclerotherapy, selective arterial embolization and systemic ...treatment with denosumab are increasingly utilized, in addition to or instead of traditional curettage. The purpose of this review is to discuss current concepts and difficulties in diagnosing and treating primary ABCs, based on latest available literature.
In diagnostics, multiple new fusion partners of USP-6 have been described on next-generation sequencing specifically for primary ABCs. In a recent systematic review, failure rates of percutaneous injections and surgery were comparable. In a literature review, the use of denosumab seemed effective but resulted in multiple cases of severe hypercalcemia in children.
Accurately diagnosing primary ABC is crucial for treatment decisions. Curettage remains a valid treatment option, especially with adjuvant burring, autogenous bone grafting and phenolization. Percutaneous sclerotherapy represents a solid alternative to surgery, with polidocanol showing good results in larger studies. Systematic therapy with denosumab exhibits favorable results but should be reserved in the pediatric population for unresectable lesions, as it may result in severe hypercalcemia in children. When selecting a treatment option, localization, stability and safety should be considered.
A 13-year-old female patient presented with painless vision loss and proptosis for 18 months. Imaging findings were highly suggestive of a supraorbital aneurysmal bone cyst (ABC) for which she ...underwent complete surgical excision. Postoperatively, she developed left hemiparesis. Computed tomography angiography (CTA) revealed right complete internal carotid arterial (ICA) thrombosis. This was managed conservatively, and she improved in hemiparesis over the next 3 weeks. Histopathology report revealed osteosarcoma with secondary ABC, for which she was referred for radiotherapy. At 1.5 months follow-up, the patient’s left lower limb power improved to 4 + /5. She was walking without support, and her left upper limb power was 4/5.
The receptor-activator of nuclear kappaB ligand (RANKL) signaling pathway plays an important role in the regulation of bone growth and mediates the formation and activation of osteoclasts. ...Osteoclasts are involved in significant bone resorption and destruction. Denosumab is a fully human monoclonal antibody against RANKL that specifically inhibits osteoclast differentiation and bone resorption. It has been approved for use for multiple myeloma and bone metastases, as well as for giant cell tumor of bone. However, there is no previous report quantitatively, comparing RANKL expression in histologically varied bone tumors. Therefore, we analyzed the mRNA level of various bone tumors and investigated the possibility of these tumors as a new therapeutic target for denosumab. We examined RANKL mRNA expression in 135 clinical specimens of primary and metastatic bone tumors using real-time PCR. The relative quantification of mRNA expression levels was performed via normalization with RPMI8226, a human multiple myeloma cell line that is recognized to express RANKL. Of 135 cases, 64 were also evaluated for RANKL expression by using immunohistochemistry. Among all of the tumors investigated, RANKL expression and the RANKL/osteoprotegerin ratio were highest in giant cell tumor of bone. High RANKL mRNA expression was observed in cases of aneurysmal bone cyst, fibrous dysplasia, osteosarcoma, chondrosarcoma, and enchondroma, as compared to cases of multiple myeloma and bone lesions from metastatic carcinoma. RANKL-positive stromal cells were detected in six cases: five cases of GCTB and one case of fibrous dysplasia. The current study findings indicate that some primary bone tumors present new therapeutic targets for denosumab, particularly those tumors expressing RANKL and those involving bone resorption by osteoclasts.
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