Denosumab and sclerotherapy for recurrent spinal aneurysmal bone cyst in a child.

Aneurysmal bone cyst (ABC) is a non-malignant, locally destructive, blood-filled lesion in the bone that tends to grow aggressively. A young girl presented with a rapid recurrence after aggressive surgery of a large symptomatic sacral-spinal ABC. After a multidisciplinary tumour board, she was successfully treated with sclerotherapy and monthly intravenous denosumab. The patient has maintained asymptomatic for over 36 months now and has returned to full activity and strength. She never required surgery and has had radiologic resolution of the lesions. Treatment of recurrent ABC requires a multidisciplinary team approach. We believe this to be the first report to use this combined therapy to provide an alternative to morbid surgery for children with ABCs.

Surgical management of a multilevel thoracolumbar aneurysmal bone cyst.

Aneurysmal bone cysts (ABCs) are primary bone tumours that rarely occur in the spine and generally affect one vertebral level in adolescents. Here, we present an unusual case of a multilevel thoracolumbar ABC, which presented a unique surgical challenge due to its infiltrative and destructive nature. A teenage male presented with back pain, paresthesias and a mildly spastic gait. MRI of the thoracolumbar spine revealed an expansive, multicystic mass extending from the left T12-L1 vertebral bodies into adjacent musculature. The patient underwent a two-stage surgical approach with decompression of the spinal cord and instrumentation to stabilise the vertebral column. The first stage involved posterior decompression, laminectomy and facetectomies, followed by pedicle-based instrumentation from T10 to L3. This was followed by a vertebrectomy and anterior stabilisation with an expansile cage from T11 to L2. A gross total resection was achieved with the patient maintaining full neurological function.

Hexapod Frame Correction of Proximal Tibial Recurvatum Caused Indirectly by a Femoral Aneurysmal Bone Cyst in an Adolescent: A Case Report and Systematic Review.

CASE: A 16-year-old boy presented with a recurrent distal femur aneurysmal bone cyst accompanied by a combined sagittal knee deformity (20° of femoral antecurvatum and 26.8° of tibial recurvatum) and limb shortening. After preoperative planning, the treatment involved new intralesional curettage, phenolization, and bone allograft filling. Additional procedures included distal extension femoral osteotomy with plate fixation, and proximal tibial osteotomy with, gradually corrected through a hexapod frame. At 2-year follow-up, lower limbs exhibited normoalignment and equal length. CONCLUSION: Complex knee deformities may occur with tumoral lesions around the knee but can be effectively addressed through double osteotomy and application of a hexapod frame.

[Aneurysmal Bone Cyst of the Rib:Report of a Case].

We report a case of an aneurysmal bone cyst (ABC) originating in a rib. A 34-year-old woman was admitted to our medical department for evaluation of left rib pain and an abnormal shadow in the left 7th rib observed on chest radiography. Computed tomography (CT) revealed an osteolytic lesion involving the left 7th rib. Positron emission tomography/CT showed slight fluorodeoxyglucose uptake in the lesion. We performed 7th rib resection with a 4 cm margin from the tumor, including the intercostal muscles in the 6th and 7th interspaces. Histopathological examination of the resected specimen showed multiple blood-filled spaces and fibrous trabeculae, which confirmed the diagnosis of an ABC. The patient's postoperative course was uneventful. Although rare, clinicians should consider ABCs in the differential diagnosis of rib tumors.

[Clinical, imaging and pathological and molecular characteristics of simple bone cyst].

Objective: To investigate the radiologic, pathologic, and molecular features of simple bone cysts (SBC), and their differential diagnoses. Methods: Fourteen cases of SBC were collected at the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from 2017 to 2022, and fluorescence in situ hybridization (FISH) was performed for retrospective analysis. Results: There were 14 patients, including 7 females and 7 males, with age range of 7 to 45 (median 29) years. The most common complaint was pain, including 4 cases with pathological fracture and 5 with history of previous trauma. The tumor size ranged from 3.4 to 13.5 (median 5.6) cm. The lesion involved the femur (n=4), humerus (n=5) and iliac bone (n=5). Radiologic diagnoses included SBC, aneurysmal bone cyst, and giant cell tumor of the bone or its combination with aneurysmal bone cyst-like region and fibrous dysplasia. Histologically, the cyst walls of the lesions were composed of fibrous tissue, fibrin-like collagen deposits, bone-like matrix and occasional woven bone. The lesional cells were spindled to ovoid, with scattered osteoclast-like giant cells, foamy histiocytes, hemosiderin deposits and cholesterol clefts. In 6 cases there were nodular fasciitis-like areas. Immunohistochemically, the spindled to ovoid cells were positive for SMA, EMA and SATB2 in varying degrees. FISH detection was performed in all 14 cases and EWSR1/FUS rearrangement were found in 9 cases. One case of FUS::NFATC2 fusion was detected by next-generation sequencing. Nine cases of SBC with the rearrangement were more cellular, and there were more mitotic figures in the recurrent FUS::NFATC2 fusion tumor. Clinical follow-up was obtained in all 14 cases with the time ranging from 5 to 105 (mean 46) months. Amongst them, the tumor with FUS::NFATC2 rearrangement had local recurrence twice after the first local excision, but had no more recurrence or metastasis 34 months after the subsequent segmental resection. The other 13 cases had no recurrence. Conclusions: EWSR1 or FUS rearrangement is most commonly identified in SBC, suggesting that SBC might be a neoplastic disease. In cases where the radiologic appearance and histomorphology are difficult to differentiate from aneurysmal bone cyst, FISH detection can aid in the definitive diagnosis.

Fibrous dysplasia with aneurysmal bone cyst-like change occurring in pediatric orbit: case report and literature review.

PURPOSE: We report a case of fibrous dysplasia (FD) with aneurysmal bone cyst (ABC)-like change in a child with orbital involvement, review the related cases, and discuss clinical features, therapy, and prognosis of this disease. CASE PRESENTATION: A 10-year-old girl had right proptosis (degree of exophthalmos: OD 16 mm, OS 13 mm) and limited vision (visual acuity: OD 1.0, OS 0.8) without trauma. Preoperative CT showed a 5.0*4.3 cm right-sided crania-orbital communicating tumor. MRI indicated a well-defined multicystic mass with scattered fluid levels and soap bubble-like alterations. The child underwent total tumor resection and orbital parietal titanium mesh reconstruction. At 20 months of follow-up, the child has recovered from ocular problems, and the tumor has not recurred. CONCLUSION: FD combined with ABC rarely occurs in orbit and generally begins with ocular symptoms. The etiology is uncertain. Early diagnosis and surgery are essential. Complete resection is suggested whenever possible because residual lesions may recur.

Analysis of Diagnosis and Treatment Strategies for Primary Juvenile Psammomatoid Ossifying Fibroma Complicated With Primary Aneurysmal Bone Cyst.

BACKGROUND: 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. CASE PRESENTATIONS: 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. CONCLUSIONS: 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.

A rare case of aneurysmal bone cyst of the anterior clinoid process in an 18-year-old female mimicking optic neuritis.

BACKGROUND: Aneurysmal bone cyst (ABC) is an uncommon, benign, vascular multicystic bony lesion that most frequently develops in the first two decades of life. The metaphysis of long bones, pelvic, and vertebral column are the most common locations. The precise underlying pathophysiology of ABCs formation remains unclear; however, it is believed that reactive processes subsequent to trauma or vascular disturbance may play an important role. Involvement of the skull base rarely occurs with a prevalence of up to 5% of intracranial ABCs. CASE PRESENTATIONS: An 18-year-old adolescent female with a history of progressive blurred vision since three months ago presented to our office. The brain and orbital MRI demonstrated no abnormal findings. After three months of glucocorticoid treatment with the diagnosis of multiple sclerosis, the visual impairment of the left eye deteriorated abruptly. The patient underwent an MRI and the imaging study demonstrated a well-defined 30 × 22 × 20-mm lesion at the anterior clinoid process with an extension to the optic canal and ethmoid sinus. The patient underwent pterional craniotomy, and the tumor was resected. The histopathological examination was suggestive of ABC. CONCLUSION: ABC and other conditions should be considered in young-age people with an early unilateral decline in vision and imaging studies should be obtained in early stages and during follow-ups.

Intraosseous hemangioma with aneurysmal bone cyst-like changes of the hyoid bone: Case report and literature review.

RATIONALE: Intraosseous hemangioma is a rare benign vascular tumor of the bone that can affect any body part; however, the most common site is the vertebra, followed by calvarial bones. PATIENT CONCERNS: We present a case of intraosseous hemangioma in a 23-year-old male who presented a feeling of fullness in the throat for 3 months. The hyoid bone level had a hard mass of about 5 cm. Fine needle aspiration showed 5 mL dark bloody aspirates. Magnetic resonance image showed a 5.3 cm mixed signal intensity lesion in the hyoid body. DIAGNOSIS: Histopathologic examination showed intraosseous hemangioma with aneurysmal bone cyst (ABC)-like changes in the hyoid bone. INTERVENTIONS: The mass was completely removed without significant problems. OUTCOMES: Complete mass excision and symptomatic improvements were achieved, and no subsequent relapses were observed. LESSONS: The authors experienced a case of intraosseous hemangioma with ABC-like changes. There has been no case report of intraosseous hemangioma in the hyoid bone. This case showed a spectral pattern of the ABC-like changes developing from the underlying bone tumor as a secondary change. ABC-like changes in bone tumors can mislead the diagnosis. Careful examination of the tumor is essential for the correct diagnosis of ABC or ABC-like changes.

[Pediatric neurosurgical treatment of aneurysmal bone cyst at the level of thoracic spine]. / Aneurysmás csontcysta gyermek-idegsebészeti megoldása a háti gerinc magasságában.

Aneurysmal bone cysts are benign but locally aggressive bone tumours, most often affecting children and young adults. In this case report, we present the clinical  picture of a 15-year-old boy with progressive, chronic back pain. An MRI of thoracic spine  confirmed a T2 cystic spinal tumour. After considering potential options surgical removal was our choice and gross total removal was achieved with T1-3 short-segment fixation. Aneurysmal bone cysts are often rapidly expanding lesions with vascular  transformation. In order to avoid irreversible damage, in addition to early diagnosis, it is necessary to carefully consider the therapeutic options, perform surgical removal and stabilization as necessary. In case of the presented patient, extensive surgical removal and short-segmentation were performed. At 18 months of follow-up, he had no complaints and was asymptomatic. Follow-up imaging studies showed no residual or recurrent tumour to date.

Using Denosumab as a Nonsurgical Management of Aneurysmal Bone Cysts in the Pelvis.

Background: Aneurysmal bone cysts (ABCs) are infrequent, benign, and locally destructive lesions that most commonly occur during the first two decades of life. They usually affect the metaphysis of the long bones, but the pelvis is involved in 8%-12% of the cases. The management of pelvic ABCs is a challenging issue due to difficulties in choosing the appropriate approach, adjacent neurovascular bundles, the risk of intraoperative bleeding with difficulty achieving good hemostasis, and the risk of injury to the hip or sacroiliac joints. Limited data exist concerning the use of denosumab as a non-surgical treatment for pelvic ABCs. Our hypothesis was that denosumab might be an effective and safe solo treatment of cases with ABCs in the pelvis. Methods: We retrospectively assessed 20 patients with ABCs in the pelvis, who were treated by denosumab as a solo agent without surgery. Patients were assessed regarding disease control, the incidence of recurrence and non-oncological complications, and functional outcome. Results: The mean follow-up period was 38.5 months. Disease control was achieved in 16 patients (80%), with no local recurrence. Tolerable drug-related complications occurred in 15% of cases. The mean Musculoskeletal Tumor Society score was 92.3%. Conclusions: Denosumab may provide a reliable option in the nonsurgical treatment of ABCs of pelvic origin with expected lower morbidity than the surgical solution and tolerable complications. Further studies on the safety profile and long-term effects of denosumab especially in skeletally immature patients are required.

Autogenous fibula head transplantation for aneurysmal bone cyst of distal radius: A case report followed up for 7 years.

RATIONALE: Aneurysmal bone cyst (ABC) is a rare primary or secondary tumor that usually occurs in young women aged between 10 and 20 years, mostly in the long tubular bone and spine. However, there are no definite standards for its clinical treatment. To our knowledge, this is the first report of a young female patient with distal radius ABC who was successfully treated with tumor resection and autogenous fibular head transplantation. PATIENT CONCERNS: A 28-year-old married Chinese young woman presented to our hospital with swelling and pain in her right wrist for 2 years and aggravation of wrist movement restriction for 1 week. DIAGNOSES: Pathological biopsy confirmed ABC. INTERVENTIONS: We performed a pathological examination of the tumor on the right wrist and preliminarily confirmed the diagnosis of ABC. The right wrist joint was reconstructed by total surgical resection of the ABC tumor in the right wrist joint and autogenous fibular head transplantation. OUTCOMES: During follow-up within 7 years, good right wrist function was confirmed. The tumor did not recur, the swelling of the right wrist disappeared, the joint pain and limitation of movement significantly improved, and the function of the right wrist was not impaired in daily activities. Radiography showed that the fracture had healed. LESSONS: Our results suggest that autofibular head transplantation is an effective treatment for reconstruction of wrist function in adult patients with ABC of the distal radius.

Maxillary Aneurysmal Bone Cyst in a Young Dog-A Case Report.

This case report describes an aneurysmal bone cyst in the maxilla of a young dog. It describes the clinical presentation, diagnostics, management, and successful outcome of this highly unusual case. Bone cysts are described as benign, cavitated lesions within bone that are lined by reactive tissues. There is no epithelial lining in the lesions. Bone cysts usually contain hemorrhage or serosanguinous fluid. They usually appear in the long bones, and present as a swelling with or without pain.

Aneurysmal bone cysts: A UK wide tumor center experience.

BACKGROUND AND OBJECTIVES: This multicenter retrospective series of consecutive extra-spinal aneurysmal bone cysts aims to identify risk factors for treatment failure. METHODS: Aneurysmal bone cysts treated within seven collaborating centers with over 12-months follow-up were eligible for inclusion. Survival analyses were performed to identify variables associated with recurrence using log-rank tests and Cox proportional hazard regression. RESULTS: One hundred and fifteen (M:F 60:55) patients were included. Median age at presentation was 13 years and median follow-up was 27 months. Seventy-five patients underwent surgical curettage and 27% of these required further intervention for recurrence. Of the 30 patients who underwent biopsy with limited percutaneous curettage as initial procedure, 47% required no further treatment. Patients under 13 years (log-rank p = 0.006, HR 2.3, p = 0.011) and those treated who had limited curettage (log-rank p = 0.001, HR 2.7, p = 0.002) had a higher risk of recurrence/persistence. CONCLUSIONS: There is a high risk of recurrence following surgical treatment for aneurysmal bone cysts and this risk is higher in young patients. However, the cyst heals in a substantial number of patients who have a limited curettage at the time of biopsy.

Keratin-Positive Giant Cell-Rich Tumor of Bone Harboring an HMGA2::NCOR2 Fusion: Two Cases, Including a Patient With Metastatic Disease, and Review of the Literature.

Giant cell-rich lesions of bone represent a heterogeneous group of entities which classically include giant cell tumor of bone, aneurysmal bone cyst, nonossifying fibroma, and Brown tumor of hyperparathyroidism. A recently described subset of giant cell-rich tumors involving bone and soft tissue has been characterized by recurrent HMGA2::NCOR2 fusions and keratin expression. The overlapping clinical, radiographic, and morphological features of these giant cell-rich lesions provide a unique diagnostic challenge, particularly on biopsy. We present 2 additional cases of keratin-positive giant cell-rich tumor of bone with HMGA2::NCOR2 fusions, including 1 patient who developed metastatic disease.

Adjunct role of liquid absolute alcohol intralesional sclerotherapy during the surgical management of spinal primary aneurysmal bone cyst in a child: a technical case report.

BACKGROUND: Aneurysmal bone cyst is composed of variable -sized cystic blood-filled spaces separated by connective tissue septae. First-line surgical resection of spinal aneurysmal bone cyst in a child with limited total blood volume can lead to massive intraoperative bleeding, thus limiting extent of resection. Our Centre's has good experience of using absolute alcohol as an effective immediate devascularizing agent during vertebral hemangioma surgery in children. MATERIAL AND METHODS: We report the first case of pediatric lumbar primary aneurysmal bone cyst in which completely blood-less piecemeal total resection of the lesion was performed after intraoperative absolute alcohol intralesional sclerotherapy. RESULTS: Completely blood-less piecemeal total resection of the lumbar aneurysmal bone cyst was performed after intraoperative absolute alcohol intralesional sclerotherapy. CONCLUSION: Intraoperative absolute alcohol intralesional sclerotherapy is a very effective devascularizing adjunct for complete piecemeal resection of spinal aneurysmal bone cyst in children with limited blood volume.

Unusual presentation of aneurysmal bone cyst (ABC) in children: pediatric intracranial osteosarcoma with secondary ABC.

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.

How successful is synthetic graft treatment for children with pathological hip fractures?

PURPOSE: To determine whether synthetic grafts are a satisfactory treatment option for pathological proximal femoral fractures in children. METHODS: Paediatric patients treated for pathological fractures of the proximal femur between 2013 and 2020 were evaluated retrospectively. 17 patients with a mean age of 10.7 years (range 6-16 years) were assessed. The definitive histopathological diagnoses were SBC (simple bone cyst) (12) and ABC (aneurysmal bone cyst) (5). The median duration of follow-up was 37 months (range 12-70 months). RESULTS: All patients returned to their normal daily routine within 3-8 months following surgery. The mean post-op recovery time was 3.2 months (range 3-6 months). Graft was incorporated at approximately 12 months. No significant radiographic healing was observed in 2 patients. In the remaining 15 patients, the mean duration of healing was 14 months (range 8-24 months). CONCLUSION: Synthetic grafts are a satisfactory treatment option for pathological proximal femoral fractures in children.

Does cryotherapy decrease the local recurrence rate in the treatment of an aneurysmal bone cyst? A comparative assessment.

Curettage with or without the use of adjuvants is the standard of care in the treatment of an aneurysmal bone cyst (ABC). Historically, our approach combined curettage, high-speed burr drilling, and cryoablation. However, treatments varied based on age, tumor location, and surgeon preference. We asked: (1) Does cryoablation in addition to curettage and burr drilling decrease the local recurrence rates? (2) Are there any risk factors for the local recurrence rate? (3) Does cryoablation improve postsurgical functional outcomes in these patients? Patients treated for an ABC, between January 2006 and December 2019 were included in this retrospective analysis. Patient and surgical characteristics, such as age, gender, tumor location, type of treatment, time of follow-up, recurrence rate, and functional outcome measured by the Musculoskeletal Tumor Society Score 1993 (MSTS93) score were compared between those treated with and without cryoablation. Both groups, without cryoablation (n = 88) and with cryoablation (n = 42), showed no significant difference in local recurrence rates (9.1% vs. 7.1%, p = 0.553) and functional outcomes as measured by the MSTS93 score (28.9 vs. 27.8, p = 0.262). Risk factors analyzed did not significantly affect local recurrence risk, except for secondary ABC diagnosis (p = 0.017). The cryoablation group had a more extended follow-up (45.6 vs. 73.2 months, p < 0.001), reflecting a shift in practice over time. We found no significant difference in local recurrence rate or functional outcome in patients treated with or without cryoablation. Formal curettage with additional high-speed burr drilling provides effective tumor control and favorable functional outcomes, negating the need for adjuvant cryoablation.