Aggressive Benign Tumors Research Articles

Wrist fusion with ulnar translocation for aggressive giant cell tumour of distal end radius: Short-term functional and clinico-radiological outcomes

BackgroundThe distal end radius (DER) is the third most common site of a giant cell tumour (GCT) in bone. GCT is a locally aggressive benign tumour with metastatic potential. The main goals in the management of GCT of DER are the complete removal of the tumours, the prevention of recurrence, and the restoration of the functional wrist. PurposeThis case series reports the clinical and functional outcome of en bloc tumour resection, ulnar translocation and wrist arthrodesis in patients with high-grade GCT of DER. MethodsSixteen patients with Campanacci grade II & III GCT of distal end radius who underwent the procedure between 2009 and 2018 and had a follow-up of 2 years were included. Patients with distant metastasis, chronic severe systemic illness, operated previously, and follow-up dropouts were excluded from the study. The patient's demographic profile and tumour characteristics were collected at the initial visit. Grip strength, modified MAYO wrist score, and VAS score for wrist pain were collected preoperatively and at 1-year and 2-year follow-ups. ResultsThe study included predominantly females (13 vs 3). The mean age was 31.5 ± 8 years with a symptom duration of 6.5 ± 3.1 months (range 3–14). The mean resection length was 8.31 ± 1.5 cm (range 6–12). The mean Ulnoradial and ulnocarpal union time was 22.7 ± 8.0 and 17.5 ± 2.3 weeks, respectively. The mean modified Mayo Wrist score was 20.63 ± 9.4, 48.7 ± 5.6 and 60.6 ± 4.0 at preop, 1 year and 2 years follow-up. The mean MSTS score was 22.68 ± 1.8 (range 19–26). Two patients had ulnoradial nonunion. ConclusionWrist arthrodesis by ulnar translocation and plate fixation is a viable option in the management of GCT of distal end radius. The short-term clinical and functional outcomes are favourable for restoring adequate wrist function.

Mandibular Ameloblastoma: A Reported Case

Ameloblastoma is a benign aggressive odontogenic tumor which requires early diagnosis and appropriate treatment. It commonly affects the mandible and radical surgery is the gold standard treatment. We report the case of a patient with ameloblastoma in extremely advanced phase affecting the retromolar trigone who was treated with concomitant chemoradiation therapy. Mandibular ameloblastoma is not well documented in the literature. It is usually diagnosed at the later stage when optimal surgery cannot be performed. This case study aimed to demonstrate that radiation therapy is a real therapeutic alternative in the treatment of advanced and inoperable forms of ameloblastoma.

Definitive Sclerosing Intensity Modulated Radiotherapy in A Child with Recurrent Aneurysmal Bone Cyst- A Clinical Case from our Practice

Aneurysmal bone cysts (ABCs) are rarely diagnosed aggressive benign bone tumors. They are mainly diagnosed in children and adolescents in the long bones of the limbs, but in 12 %-30% are localized in the spine. Since the value of external beam radiotherapy (EBRT) for ABC has not been well defined, we present our observations and treatment outcome after one year from the definitive intensity modulated radiotherapy (IMRT) up to 30 Gy. for recurrent spine ABC in adolescent age

Intraoperative near-infrared fluorescence guided surgery using indocyanine green (ICG) may aid the surgical removal of benign bone and soft tissue tumours

BackgroundBenign bone and soft tissue tumours encompass a broad, heterogenous range of tumours with varying clinical characteristics. These are often managed surgically with either curettage or marginal excision, but unfortunately have high rates of local recurrence. Indocyanine green (ICG) is a fluorescent dye which can be used to identify solid malignancies intraoperatively but its use is not yet established in benign bone and soft tissue tumours. This study aims to assess whether these tumours fluoresce when administered with ICG pre-operatively and whether this helps surgeons to identify tumour intra-operatively. Patients and methodsPatients with locally aggressive benign bone and soft tissue tumours were administered with 25–75 mg of ICG preoperatively at the induction of anaesthesia. Fluorescence was imaged intraoperatively using the Stryker SPY-PHI camera. ResultsOf the 12 patients included, 11 tumours fluoresced. The surgeons felt the fluorescence guided the procedure in 7 out of the 11 cases which fluoresced. It was felt to be particularly useful in the curettage of bone tumours, in which curettage could be repeated until the absence of fluorescence on imaging. After 12 months, no patients had local recurrence of the tumour. There were no adverse events recorded in this study and surgeons found the technology acceptable. ConclusionsThe use of ICG for fluorescence guided surgery is a promising technology to improve outcomes of surgery for benign bone and soft tissue tumours. Further, longer term, study with a control arm is needed to identify whether it results in a reduction in the local recurrence rate.

Perioperative complications of en bloc resection and anterior column reconstruction for thoracic and lumbar spinal tumors

PurposeTo evaluate the perioperative clinical outcomes of en bloc resection and anterior column reconstruction for thoracolumbar spinal tumors.MethodsThis study conducted a retrospective analysis of prospective data collection of 86 consecutive patients, including 40 males and 46 females, with an average age of 39 years (ranged from 10 to 71 years). There were 35 cases of a malignant primary tumor,42 cases of an aggressive benign tumor, and nine cases of metastases. The main lesions were located in 65 cases of thoracic spine, 17 cases of lumbar spine, and 4 cases of thoracolumbar spine. Tumors involved one level in 45 patients, two levels in 12 patients, three levels in 21 patients, four levels in five patients, five levels in two patients, and six levels in one patient.ResultsAccording to the Weinstein-Boriani-Biagini surgical staging system, all patients achieved en bloc resections, including 74 cases of total en bloc spondylectomy and 12 cases of sagittal resections. The mean surgical time was 559 min (210–1208 min), and the mean total blood loss was 1528 ml (260–5500 ml). A total of 122 complications were observed in 62(72.1%) patients, of which 18(20.9%) patients had 25 major complications and one patient (1.2%) died of complications. The combined approach (P = 0.002), total blood loss (P = 0.003), staged surgery (P = 0.004), previous surgical history (P = 0.045), the number of involved vertebrae (P = 0.021) and lumbar location (P = 0.012) were statistically significant risk factors for major complication. When all above risk factors were incorporated in multivariate analysis, only the combined approach (P = 0.052) still remained significant.ConclusionsEn bloc resection and anterior column reconstruction is accompanied by a high incidence of complications, especially when a combined approach is necessary.

Is 3D-printed self-stabilizing endoprosthesis reconstruction without supplemental fixation following total sacrectomy a viable approach for sacral tumours?

The spinopelvic reconstruction poses significant challenges following total sacrectomy in patients with malignant or aggressive benign bone tumours encompassing the entire sacrum. In this study, we aim to assess the functional outcomes and complications of an integrated 3D-printed sacral endoprostheses featuring a self-stabilizing design, eliminating the requirement for supplemental fixation. We retrospectively analyzed patients with sacral tumours who underwent total sacrectomy followed by reconstruction with 3D-printed self-stabilizing endoprosthesis. Clinically, we evaluated functional outcomes using the 1993 version of the musculoskeletal tumour society (MSTS-93) score. Perioperative and postoperative complications were also documented. 10 patients met final inclusion criteria. The median age was 49years (range, 31-64years). The median follow-up time was 26.5months (range, 15-47months). Median postoperative functional MSTS-93 was 22.5 (range, 13-25). The median operation time was 399.5min (305-576min), and the median intraoperative blood loss was and 3200ml (2400-7800ml). Complications include wound dehiscence in one patient, bowel, bladder, and sexual dysfunction in four patients, cerebrospinal fluid leak in one patient, and tumour recurrence in one patient. There were no mechanical complications related to the endoprosthesis at the last follow-up. The utilization of 3D-printed self-stabilizing endoprosthesis proved to be a viable approach, yielding satisfactory short-term outcomes in patients undergoing total sacral reconstruction without supplemental fixation.

Clinical application of LARS tumor tube in joint function reconstruction of tumor type artificial hip replacement.

Proximal femur tumor resection often leads to hip joint instability and functional loss. Various methods have been clinically applied to repair hip joint soft tissue function, but deficiencies remain. This study aims to evaluate the advantages and disadvantages of the ligament advanced reinforcement system (LARS) tumor tube in assisting soft tissue function reconstruction in patients undergoing tumor type artificial hip replacement surgery. This study included 85 patients (41 males, 44 females) with proximal femoral tumors treated at the Xiangya Bone Tumor Treatment Center from January 2012 to January 2022, aged 10 to 79 (38.5±18.2) years. Among them, 13 cases had benign aggressive tumors, 45 had primary malignant bone tumors, and 27 had bone metastases. Clinical data, imaging data, and intraoperative photos were collected. Patients were followed up and postoperative functional evaluations were conducted using the Musculoskeletal Tumor Society (MSTS) scoring system and Harris hip joint scoring system to assess limb function and hip joint function. Preoperative pathological fractures were present in 37 cases (43.5%), with a lesion length of (9.4±2.9) cm. Among non-metastatic tumor patients, 7 experienced postoperative recurrence, including 6 cases of osteosarcoma and 1 case of fibrosarcoma. Pulmonary metastases occurred in 9 osteosarcoma patients. Five patients required reoperation due to postoperative complications, including 3 cases of deep vein thrombosis, 1 case of giant cell granuloma, and 1 case of prosthesis infection. Postoperatively, 5 patients exhibited Trendelenburg gait, and 6 had leg length discrepancies. The postoperative MSTS score was 26.7±1.4, and the Harris score was 89.6±5.3. The LARS tumor tube can effectively assist in reconstructing the soft tissue function of the hip joint and greatly reduce postoperative complications, making it an effective technical improvement in joint function reconstruction in tumor type artificial hip replacement surgery.

The Effectiveness of Denosumab in Middle Eastern Patients With Giant Cell Tumor of the Bone: A Single-Center, Retrospective Study.

Giant cell tumor of the bone (GCTB) is an aggressive benign tumor, which constitutes 5% of all primary bone tumors. Denosumab, a receptor activator of nuclear factor κB ligand monoclonal antibody, inhibits osteoclast-induced bone destruction and has demonstrated promising results in patients with GCTB. However, the long-term efficacy of the drug has not been extensively studied, especially in the Middle East. In this study, we retrospectively analyzed the five-year progression-free survival (PFS) in patients with GCTB at a single Saudi center. PFS was defined as the time from diagnosis until disease progression, relapse, or death. Events were censored after five years from diagnosis. Sixty-two patients with GCTB were included in the study. The median age at diagnosis was 31.16 years, and 38 (61.3%) patients were female. Twenty-nine patients (46.8%) received denosumab during the study period. The median duration of denosumab treatment was 5.06 months, and the median number of cycles was 6. The median PFS was not reached, and the five-year PFS rate was 60.3%. Age, gender, body mass index, performance status at presentation, and tumor location had no impact on five-year PFS. Denosumab treatment prolonged PFS; however, this was not statistically significant compared to non-denosumab patients (P= 0.603). Denosumab does not seem to provide superior long-term outcomes compared to surgery alone. Although our findings are generally consistent with other studies in the literature, larger long-term studies are needed to confirm our findings.

Aggressive Thoracic Spine Hemangioma Treated by Total En Bloc Spondylectomy: A Case Report

Introduction: Vertebral hemangioma (VH) is the most widely faced tumor in the vertebral column. It is generally asymptomatic and slowly growing. Moreover, the appearance of extraosseous extensions in vertebral hemangiomas is locally aggressive, causing neurological deficits, and should be distinguished from other vertebral hemangiomas based on its prognosis and treatment. Here we present a rare case of vertebral hemangioma caused by thoracic cord compression and healed by total en block spondylectomy.Objectives: We present a case of VH with extraskeletal expansion that caused the progressive neurologic deficit and was medicated by resection of the entire tumor.Methods: A 33-year-old female came to the Soetomo General Hospital outpatient clinic with the main complaints of back pain and being unable to move her legs since September 2021. In July 2021, the patient felt tingling in her lower extremities. The patient was still able to walk, but she felt the weakness develop. She still can urinate, but she has a defecation problem. There is no history of a palpable mass in some places. The patient is a housewife and she has no previous history of any disease.Results: The patient felt tingling in her lower limbs. She didn’t lose her to walk, but she felt the weakness develop. On the physical examination, there was a neurological deficit at the lower extremity. According to radiographic and histopathologic examination, the patient was diagnosed with VH at the T5 level. We then performed total en-block spondylectomy and posterior stabilization. As a result, the motoric part improved but sensory deficits still occurred. Neurological improvement happened during the follow-up in three months. An inevitable challenge awaits in terms of the medication of VH with extraskeletal expansion that causes neurologic deficits. Pre-operative diagnosis including radiologic and pathologic findings is essential for the strategy and decision for combative hemangioma. An unjustified diagnosis may lead to an inappropriate surgical strategy or the emergence of various complications.Conclusions: Vertebral hemangiomas with extraosseous extension causing spinal cord compression should be considered an aggressive benign tumor, and total excision that includes a tumor margin is indicated.

En-bloc spondylectomy in the lumbar spine: indications, results and complications in a series of 47 patients affected by primary malignant bone tumors

IntroductionWide Surgery is the reference treatment for malignant and aggressive benign primary bone tumors in the spine. When located in the lumbar spine, En-Bloc Spondylectomy (EBS) remains a complex challenge. Moreover, surgery is complicated by the presence of the diaphragm in the thoracolumbar junction and the hinderance of the iliac wings at the lumbosacral levels. Therefore, EBS in the lumbar spine frequently requires combined approaches.The purpose of this study is to describe clinical presentation, tumor characteristics and results of a series of 47 consecutive patients affected by malignant primary bone tumors of the lumbar spine who underwent EBS.Materials and methods47 patients were reviewed. Complications were distinguished in early and late whether they occurred before or after 30 days from surgery.Overall survival (OS), disease-free survival (DFS) and local recurrence-free survival (LRFS) were calculated by the Kaplan–Meier product-limit method from surgery until relapse or death.Results27 patients presented to observation after a first intralesional approach in a non-specialized center. Chordoma was the most represented histotype. Vertebrectomies were: 23 one-level, 10 two-level, 12 three-level and 2 four-level. Reconstructions were always carried out with screws and rods. The main postoperative complication was blood loss, while hardware failure was the main long-term complication.The 5-year LRFS was 75.5%, the 5-year DFS was 54.3%, and 5-year OS was 63.6%.ConclusionsThe surgical margin obtained during the index surgery was statistically associated with Local Recurrence, DFS and OS, underlining the importance of treating patients in reference centers.

Intercalary Resection of the Tibia for Primary Bone Tumors: Are Vascularized Fibula Autografts With or Without Allografts a Durable Reconstruction?

Intercalary Resection of the Tibia for Primary Bone Tumors: Are Vascularized Fibula Autografts With or Without Allografts a Durable Reconstruction?

What factors are associated with osteoarthritis after cementation for benign aggressive bone tumor of the knee joint: a systematic review and meta-analysis.

The current systematic review aimed to answer the following questions: (i) Does extended curettage combined with the PMMA technique for the treatment of aggressive bone tumors around the knee led to the development of knee osteoarthritis? (ii) What factors are associated with osteoarthritis after bone cementation around the knee joint? This study was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. All electronic searches were performed on November 20, 2022, by a single researcher who evaluated the full texts of potentially eligible studies to determine inclusion. In these patients, the presence of osteoarthritis secondary to the surgical procedure was investigated. Data extracted included study type, characteristics of participants, sample size, gender, tumor site (femur or tibia), secondary osteoarthritis, tumor volume, distance from the joint cartilage, reoperation, follow-up time, Campanacci grade, and pathological fracture. In total, 11 studies comprising 204 patients were evaluated, and it was found that 61 (30%) patients developed knee osteoarthritis due to extensive curettage and bone cement application for benign aggressive tumor treatment. According to the results obtained based the random effects model with the 11 studies included in the meta-analysis, the mean odds ratio of development knee OA with the 95% CI was calculated as -2.77 (-3.711, -1.83), which was statistically significant (z = -5.79; P < 0.000). The association of distance between the tumor and joint cartilage and development of osteoarthritis was not shown in this meta-analysis. Level IV prognostic study.

Mandible Reconstruction with 3D Printed Models

Background: When surgeons operate under general anesthesia, length of surgery, not only its outcome, matters. In maxillofacial surgery we can reduce the time in reconstructive surgery by using presurgical bended plates in presurgically designed 3D models. Method: The study measured the mean time to bend the plates preoperatively on a printed three-dimensional model and the capability of the plates to adapt well on the mandible intraoperatively in cases of mandibular reconstruction after partial resection of the mandible due to pathology, such as benign aggressive tumors. Results and conclusions: The mean time to bend the plates preoperatively was 17 minutes, with rapid adaptation during surgery. This is important in minimizing the time that the wound is exposed to the environment and minimizes the trauma to the surrounding soft tissues. Multiple checks of the plate if bended at time of surgery can increase trauma to the surrounding soft tissues. The study supports the recommendation of the use of the preoperatively corrected and printed 3D models to prebend the plates pre-operatively. This step should be included as a routine workflow in elective cases, since it is not be possible in cases of emergency or urgency.

Aggressive Giant Cell Tumor of Proximal Tibia Treated with Resection and Arthrodesis using Limb Reconstruction System – A Case Report

Introduction: Giant cell tumors (GCTs) are one of the most aggressive benign tumors of the bone. For tumors around the knee joint, excision with preservation of joint movement is preferred. Resection arthrodesis or an amputation might be necessary sometimes. Case Report: In this case study, we report a 23-year-old female who had an aggressive osteolytic lesion of the proximal tibia with articular breach. The biopsy revealed an aggressive GCT. Conclusion: Then the patient was managed with resection arthrodesis with fibular strut graft, limb reconstruction system, and augmentation plating. The limb reconstruction system was removed after the union was achieved. Keywords: Giant cell tumors, resection, arthrodesis, limb reconstruction system.

Overview of Pharmacological Therapies for Diffuse Tenosynovial Giant Cell Tumor

Tenosynovial giant cell tumor (TGCT) is a rare and locally aggressive benign tumor arising from the synovium of joints, bursae, and tendon sheaths. It is classified into localized (L-TGCT) and diffuse (D-TGCT) forms based on the extent of involvement. Surgical resection is the primary treatment, though achieving a definitive cure remains challenging due to the high recurrence rates, especially in D-TGCT. Systemic therapies targeting the CSF1-CSF1R axis have emerged as promising treatment options. CSF1R tyrosine kinase inhibitors (TKIs) such as imatinib, nilotinib, pexidartinib, and vimseltinib, alongside anti-CSF1R antibodies like emactuzumab, cabiralizumab, and lacnotuzumab, have shown encouraging results in managing TGCT, particularly when surgery is not feasible or poses significant morbidity. Other potential therapies, including local treatments and anti-inflammatory drugs, are being explored for TGCT management. This review provides an overview of systemic treatment options for D-TGCT, highlighting emerging therapeutic modalities and their potential implications. Effective management is crucial due to TGCT’s significant morbidity despite its non-life-threatening nature, necessitating novel approaches to improve patient prognosis and quality of life.

Digitalized diagnosis and treatment of pigmented villonodular synovitis of temporomandibular joint: a case report.

Pigmented villonodular synovitis (PVNS) is a rare locally aggressive benign tumor in the temporomandibular joint (TMJ). This paper presents a patient with TMJ-PVNS involving masseteric space, temporal bone, zygomatic process, and mandibular ramus. Digital technique was used to determine the boundary of the lesion and reconstruct the normal glenoid fossa. The temporalis myofascial flap was transplanted between titanium mesh and condyle to reconstruct the disk after the complete resection of the tumor. The patient's facial profile is symmetrical, with a mouth ope-ning of 43 mm. No local recurrence and complications, such as cerebrospinal fluid fistula and encephaloceles, were detected during 30-month follow-up period.

Is higher body mass index associated with secondary osteoarthritis in patients treated with bone cement for benign aggressive bone tumors? Long-term evaluation of 43 patients

BackgroundThis study was designed to investigate the secondary osteoarthritis rate in patients with benign aggressive bone tumors treated with curettage and cementing at long-term follow up. MethodsPatients diagnosed with benign aggressive bone tumor (giant cell or aneurysmal bone cyst), treated with extended curettage and cementation with polymethylmethacrylate, who had a minimum of 60 months of follow up after surgery were included in this study. After definitive diagnoses were confirmed by a histopathologist, we decided to perform standard surgical management. Osteoarthritis was staged radiologically using the Kellgren–Lawrence scoring system, and the contralateral knees of the patients were used as the control group. Based on the Kellgren–Lawrence classification system, stages 3 and 4 were accepted as the existence of osteoarthritis. Body mass index, the distance to the subchondral joint line, tumor mass volume, the location of the tumor (i.e., femur, tibia, medial condyle, or lateral condyle), age, and sex were also investigated, all of which are factors that can affect the occurrence of osteoarthritis. ResultsForty-three patients, 24 male (56%) and 19 female (44%), were included in the study. The mean age of the patients was 29.5 ± 10 years, and mean follow up duration was 128.7 months. Tumor localization was the distal femur in 20 patients (46.5%) and the proximal tibia in 23 patients (53.5%). The mean tumor mass volume was 77.84 cm3 and the distance to the knee joint subchondral line was 3.2 ± 2 mm. According to this scoring system, 14 patients were at stage 0, 10 patients were at stage 1, 10 patients were at stage 2, four patients were at stage 3, and five patients were at stage 4. When we compared osteoarthritis development, the affected knee had a significantly higher rate of osteoarthritis development than the contralateral knee. A univariate analysis demonstrated that age (P = 0.002) and body mass index (P = 0.045) were associated with secondary osteoarthritis. Moreover, multivariate analysis demonstrated that none of the variables were independently associated with secondary osteoarthritis. ConclusionPatients with contralateral osteoarthritis had bilateral knee osteoarthritis, indicating that primary osteoarthritis progressed in both knees. Seven of the 43 patients (16.2%) showed secondary osteoarthritis. Although age and body mass index were associated with secondary osteoarthritis in univariate analysis, none of the variables were independently associated with secondary osteoarthritis in the multivariate analysis.

Giant Cell Tumor in the Proximal Femur of an Eight-Year-Old Boy: A Case Report

Background: Giant cell tumor (GCT) is a benign aggressive bone tumor that occurs mostly in young adult patients after puberty. The most common location is around the knee joint. Occurrence in pre-puberty pediatric patients is very rare and accounts for 1.8% to 10% of all known GCTs.&#x0D; Case Report: Here, we report an eight-year-old boy who complained of pain and loss of range of motion (ROM) in the right hip. Radiological and pathological studies revealed GCT of the femoral head with joint expansion. We treated the patient by wide resection and osteochondral allograft reconstruction.&#x0D; Conclusion: We believe this is the first reported case of GCT in the proximal femoral bone of pediatric patients, which is proven by pathology. Studying the current case may help tumor surgeons to become aware of this possibility.

Partial sternectomy with reconstruction of a giant cell tumor of the sternum, a case report, Saudi, Arabia

BackgroundGiant cell tumor (GCT) is a relatively common and locally aggressive benign bone tumor that rarely affects the sternum.Case presentationWe report a case of giant cell tumor of the sternum in a 28-year-old Saudi with painful swelling at the lower part of the sternum. Subtotal sternectomy and reconstruction with a neosternum using two layers of proline mesh, a methyl methacrylate prosthesis, and bilateral pectoralis muscle advancement flaps were performed.ConclusionsGiant cell tumor of the sternum is a rare diagnosis. Surgical resection with negative margins is the ideal management. To avoid defects or instability of the chest wall, reconstruction of the chest wall with neosternum should be considered.

Giant Cell Tumour of Bone: A Comprehensive Review of Pathogenesis, Diagnosis, and Treatment.

The benign aggressive tumour known as a giant cell tumour of bone (GCTB) frequently affects the knee bones.Patients suffering from GCTB present with pain, swelling, joint effusion, loss of ability to bear weight on the involved extremity and a restriction in the range of motion of the afflicted joint may also exist, depending on the tumour's size. GCTB makes up 20% of benign skeletal tumours and 5% of all primary bone tumours. Although it has an equal distribution of the sexes, the majority reveal a higher frequency among women. Eighty per centof GCTB instances were recorded in patients between the ages of 20 and 50 during the third decade. The femur, tibiaand radius are where GCTB is most frequently discovered. Lesions can be rated using the Campanacci grading method based on the plain radiograph's results. Plain radiography, CT and MRI are used to diagnose the tumour. Surgery is the only curative treatment which is determined by the Campanacci grade and the tumour's location. Recurrence of GCTB is observed in about 25% of patients, with curettage being associated with rates as high as 50%. We evaluated the GCTB-related articles and summarised the developments in diagnosis, treatment and reducing risk of recurrence.