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.

[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.

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.

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.

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.

[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.

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.

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.

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.

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.

Use of Surgical Adjuvants Does Not Decrease Recurrence of Aneurysmal Bone Cysts in Surgical Intervention With Pediatric Patients.

BACKGROUND: Aneurysmal bone cysts (ABCs) are benign active tumors often requiring intralesional curettage with or without adjuvants. The primary aim of this study was to analyze whether recurrence is influenced by the use of surgical adjuvants in pediatric patients with ABCs. Secondary aims examined recurrence rates based on age, sex, and physeal contact. METHODS: A retrospective review was performed at a tertiary pediatric hospital from 2004 to 2020. Inclusion criteria consisted of patients treated surgically for histologically confirmed ABCs with a minimum of 6 months follow-up. Patients with treatment for a recurrent tumor or incomplete records were excluded. Patient demographics, location of the lesion, treatment technique, and incidence of recurrence were collected. Statistical analyses were performed using STATA. RESULTS: There were 129 patients (74 males and 55 females) with a mean age of 11.5 ± 4.1 years and an average follow-up of 29.0 ± 25.4 months. The most common locations for ABCs were the femur and tibia. Of the patients, 53.5% had tumors abutting the physis, 28.7% had no physeal contact, and 17.8% had insufficient imaging to evaluate physeal contact. Surgical adjuvants (high-speed burr, coagulation, liquid nitrogen, and/or hydrogen peroxide) were used in 91 of the 129 cases (70.5%). There was no significant difference in recurrence when comparing those who received an adjuvant and those who did not (25.3% vs 23.7%, P = 1.000). Physeal contact was also not significantly associated with recurrence ( P = 0.146). Finally, patients younger than 6 years old were significantly more likely to have recurrence compared with those 6 years old or older (66.7% vs 21.7%, P = 0.007). CONCLUSION: Our study found no association between the use of surgical adjuvants and the risk of recurrence after intralesional curettage for ABCs. Although our study did demonstrate that patients 6 years old or younger had an increased rate of recurrence, no significant association was found regarding physeal contact or sex. These data indicate that surgical adjuvant may not affect the recurrence rates of pediatric patients with ABCs. LEVEL OF EVIDENCE: Level III. This retrospective review compares rates of recurrence based on the choice of surgical adjuvant.

Treatment of Spinal Aneurysmal Bone Cyst with Percutaneous Injection of Hydroxyapatite Osteoconductive Cement.

PURPOSE: The aim of this study was to evaluate treatment efficacy of percutaneous injection of hydroxyapatite-osteoconductive-cement in patients with spinal aneurysmal bone cysts. MATERIALS AND METHODS: The study was designed as a retrospective observational clinical study. We included patients who were diagnosed with of spinal aneurysmal bone cyst, at our institution between 2013 and 2020, and treated with percutaneous injection of osteoconductive cement: "Cerament"® (BONESUPPORT AB, Lund, Sweden). Typical clinical and radiological features of the ABCs treatment and follow-up were investigated. RESULTS: Our study included nine patients, two children and seven adults. Three different types of approaches were applied: (single pedicle approach in 3 patients; double pedicle approach in 2 patients; while in the remaining cases, a multiple access approach was used. VAS score decreased from 8.5 ± 0.5 before treatment to 4.1 ± 0.9 at 6-months-follow up. All of the patients reacted well to treatment, with none neurological complications, complete loss of pain and achieved osteosclerosis as radiological marker of treatment success. CONCLUSION: Treatment of symptomatic spinal ABC's with hydroxyapatite cement is effective to achieve complete pain reduction and sclerosis.

Large skull osteoblastoma presented as aneurysmal bone cyst (ABC).

Aneurysmal bone cysts (ABCs) are rare benign vascular bony lesions mostly encountered in young patients. These cysts can occur as primary lesions or, less frequently, secondary to other pathologies such as osteoblastomas. Skull ABCs are rare and can extend intracranially, presenting with hydrocephalus and bleeding. Here we illustrate the case of a 9-year-old male who presented with headache, nausea, and vomiting, without neurological deficit. Radiological investigations showed a soap-bubble lesion with mass effect over the right cerebellum. The patient underwent right sub-occipital craniotomy with marginal wide resection of the cystic lesion. The patient had excellent outcomes. The histopathological report was consistent with osteoblastoma with an aneurysmal bone cyst.

Aneurysmal bone cyst: Is selective arterial embolization effective as curettage and bone grafting?

INTRODUCTION: Aneurysmal bone cyst (ABC) is a lytic benign bone lesion representing about 1% of all primary bone tumors. Method to treat ABC's have developed over time. The standard of care cure for ABC has been curettage with or without bone grafting of the defect but is burdened by recurrence rates of approximately 25%-31%. Based on the assumption that ABCs usually supplied by one or more pathological feeding arteries, selective arterial embolization has been described as an adjuvant preoperative procedure to reduce intra-operative hemorrhage, and as primary treatment for lesions in difficult surgical access. In the current study, we therefore asked whether (1) a single or a repeat selective arterial embolization (SAE) for treating ABCs would produce comparable healing rates compared with curettage and bone grafting; (2) evaluated the relationship of recurrence in relation to the site of the cyst, the age, and gender of the patients; and (3) the two techniques differ in term of long-term complication. MATERIAL AND METHODS: We retrospectively reviewed 265 patients who underwent curettage and bone grafting or SAE performed at our institute from 1994 to 2018. The diagnosis of ABC was always established with percutaneous CT-guided biopsy or open biopsy. Patients were followed clinically with plain radiographs or CT scan at 3, 6, 9, and 12 months then annually in the absence of symptoms. Treatment success was determined evaluating pre- and postprocedural imaging according to Chang classification. RESULTS: Two hundred and nineteen were treated with curettage and bone grafting (curettage group), and 46 with SAE Group. Of the 219 patients treated with Curettage and bone grafting (curettage group), 165 out of 219 (75.3%) experienced bone healing, while local recurrence was observed in 54 cases (24.7%) after 12 months on average (range: 3-120 months) from surgery. After the first SAE, bone ossification was seen in 27 (58.7%), without needing any further treatment. Eleven recurred patients were treated with SAE (four patients need two while seven need three SAE to heal), and eight patients with curettage and bone grafting. Thirty-eight out of 46 (82%) patients experienced bone ossification regardless the number of SAE. The overall rate of local recurrence for all patients was 26.7%. SAE group presented a lower complication rate (6%) where two patients experienced skin necrosis, and one limb-length discrepancies (2% of all cohort). DISCUSSION: The use of SAE is an attractive option to treat ABC as it combines on one hand a lower complication rate than curettage and bone grafting, on the other it can be carried out in case of nonresectable ABCs, significantly reducing the size of viable ABC lesions, fostering bone remodeling and mineralization, and most importantly, significantly improving the patient's quality of life.

Management of advanced metacarpal aneurysmal cysts: Surgical technique.

The hand is a rare site for aneurysmal bone cysts (ABCs). Depending on their degree of invasiveness, treatment of ABCs ranges from medical therapy to surgical curettage and bone filling. In the most advanced cases, bone resection and reconstruction, with or without adjuvant or neoadjuvant treatment, are indicated. We describe a technique involving segmental bone resection and structural iliac graft reconstruction with carpo-metacarpal plate arthrodesis, for the management of advanced ABC involving the base of the metacarpals. The radio-clinical results of two patients operated on using this technique are presented, at 18 and 36 months. At the last follow-up, the results showed a QDASH of 15 and 10, and a PRWE of 9 and 11. Satisfaction was 10/10 in both cases. Grasp strength was 25 kg and 42 kg versus 28 kg and 40 kg on the opposite side. Pinch strength was 6 kg and 11 kg versus 7 kg and 10 kg. Metacarpophalangeal flexion was 80° and 90°. Extension was complete in both cases. Radiographs showed good graft fusion, with no lysis or signs of ABC recurrence at the last follow-up. Segmental bone resection and iliac autograft reconstruction with carpo-metacarpal plate arthrodesis represents a therapeutic option in the management of advanced ABCs of the metacarpal base.

Computed tomographic features and surgical treatment of equine mandibular aneurysmal bone cysts with ß-tricalcium phosphate in a multicenter case series.

OBJECTIVE: To describe clinical and imaging features and surgical treatment of equine mandibular aneurysmal bone cysts (ABCs) with ß-tricalcium phosphate (TCP). ANIMALS: 3 horses (cases 1, 2, and 3) and 1 pony (case 4) with histologically confirmed ABC. CLINICAL PRESENTATION: 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). RESULTS: 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. CLINICAL RELEVANCE: 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.

Langerhans cell histiocytosis with aneurysmal bone cyst-like changes: a case-based literature review.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a neoplastic transformation of myeloid precursors that commonly presents as an osteolytic lesion of the long or flat bones in children. Aneurysmal bone cysts (ABC) are benign neoplasms that frequently affect the metaphysis of long bones and the spine, often revealing a rapidly expansile lesion with fluid-fluid levels. LCH with secondary ABC-like changes is a rare condition that has only been reported five times, with two presentations in the cranium. The aim of this paper is to review the etiology, clinical and radiographic presentations, and treatment of this condition, as well as to present a novel case on the topic. CASE DESCRIPTION: We describe a 5-year-old boy with a rapidly growing head mass and eye pain resulting in a diagnosis of LCH with secondary ABC-like changes. Radiography demonstrated an expansile, lytic lesion of the left parietal bone with fluid-fluid levels. A confirmatory diagnosis was made through histopathology, demonstrating an inflammatory, histiocytic infiltrate staining positive for CD1a, CD68, CD207 (Langerin), and S-100. The lesion was surgically excised, and the patient recovered without any complications. CONCLUSION: We present a novel case of LCH with secondary ABC-like changes managed with surgical excision. While a radiographic workup with multiple imaging modalities is helpful for diagnosis, a thorough immunohistochemical analysis is essential as imaging characteristics are variable and nonspecific. Furthermore, surgical excision should be considered first-line treatment for lesions involving the skull in surgically accessible areas as it is curative, alleviates symptoms, and allows for histopathological diagnosis.