Understanding multicentric haemangioendothelioma: diagnostic dilemmas and treatment strategies.

Epithelioid haemangioendothelioma (EH) is a rare malignant vascular tumour occurring mainly in the liver and lungs, with bones being a rare site and primarily seen in the adult population. This case presents a male patient in his 40s who presented to the outpatient department with a chief issue of a painless swelling over the inguinal region for 4 months, gradually increasing in size, along with a history of a gradually enlarging, painless mass on his left knee over the past 5 years. Despite occasional discomfort during physical activities, the mass exhibited no associated trauma, fever, weight loss or systemic symptoms. Physical examination revealed a firm mass on the left knee and a matted lymph nodal swelling in the left inguinal region. Subsequent imaging studies identified multiple soft tissue lesions, osseous involvement and pulmonary metastases, suggestive of multicentric haemangioendothelioma. The patient underwent surgical excision of the inguinal mass and fixation of a pathological fracture in the left femur. He is currently undergoing chemotherapy and is scheduled for regular follow-up appointments. This case underscores the importance of thorough diagnostic evaluation and multidisciplinary management in complex oncological conditions like multicentric haemangioendothelioma.

Patient-Reported Outcomes After Intramedullary Nailing of Oncologic Impending or Pathologic Fractures With Carbon Fiber or Titanium Implant.

INTRODUCTION: Despite the benefits of intramedullary nailing (IMN) of impending or pathologic fractures in oncologic patients, literature on patient-reported outcomes (PROs) is scarce in patients treated with carbon fiber (CF) nails. Our study compared postoperative PROs after IMN with CF or titanium implants. METHODS: We conducted a retrospective propensity score-matched cohort study of patients treated at our institution with CF or titanium nails for impending or pathologic fractures from metastatic bone disease. Patient-Reported Outcomes Measurement Information System (PROMIS) Global Health Short Form (SF) Physical, Mental, and Physical Function 10a scores were collected. Pain was assessed using visual analog scale (VAS). Absolute and differential scores were compared between groups. RESULTS: We included 207 patients, 51 treated with CF and 156 with titanium nails. One month postoperatively, patients had a one-point decrease in the pain VAS score while PROMIS scores did not improve. At 3 months, PROMIS SF Physical and SF 10a scores improved from preoperative values. Six months postoperatively, median PROMIS SF Physical, SF Mental, and SF 10a scores were higher than preoperative scores. Absolute and differential PROMIS and pain VAS scores were similar between groups at the 6-month and 1-year marks. CONCLUSION: Patient-reported outcomes were similar after intramedullary nailing with either CF or titanium implants.

Tumour-induced osteomalacia: the long road to diagnosis and recovery.

Tumour-induced osteomalacia is caused by tumorous production of fibroblast growth factor 23 (FGF23) leading to urinary phosphate wasting, hypophosphataemia and decreased vitamin D activation. The resulting osteomalacia presents with muscle weakness and bone pain but progresses to multiple pathological fractures. Patients often remain undiagnosed for years with severe physical, psychological and economic ramifications. A young woman presented with multiple spontaneous fractures including bilateral femoral fractures. Laboratory tests revealed severe hypophosphataemia, elevated bone turnover markers and low to normal calcium and 25-hydroxy-vitamin D levels. Treatment with phosphate, alfalcalcidol, calcium and magnesium was initiated. 68Gallium-DOTATOC positron emission tomography imaging revealed a mass in the right foot and venous sampling of FGF23 from all extremities confirmed this tumour as the culprit. Biopsy and histology were consistent with a phosphaturic mesenchymal tumour, which was surgically resected. Phosphate levels quickly normalised postoperatively but a long convalescence with hungry bone syndrome, fracture healing and physical therapy followed.

Implantation of C2 prosthesis with dorsal fusion C0-C4 due to pathologic C2 fracture. Case report and literature review.

INTRODUCTION: Pathological destruction of the axis vertebra leads to a highly unstable condition in an upper cervical spine. As surgical resection and anatomical reconstruction of the second cervical vertebrae represents a life threatening procedure, less radical approaches are preferred and only few cases of C2 prosthesis are described in literature. CASE DESCRIPTION: The focus of this case report is a 21-year-old man with a pathological fracture of C2 managed primarily surgically with the C1-C3 dorsal fusion. Due to the progression of giant cell tumor and destruction of the axis vertebra, C2 prosthesis through anterior approach and dorsal occipito-cervical fusion C0-C4 were performed. Postoperative infection was managed surgically with a 2-staged dorsal debridement, ostheosynthesis material change and autologous bone graft. After a 4 week-intravenous therapy with the ceftriaxone in combination with the amoxicillin/clavulanate, followed by 12 week per oral therapy with amoxicillin/clavulanate in combination with ciprofloxacin, the complete recovery of the infection was achieved. Radiotherapy was initiated 2 months after the last revision surgery and the patient showed a good clinical outcome with stable construct at a 1 year follow-up. A review of literature of all reported C2 prosthesis cases was performed CONCLUSION: C2 prosthesis allows a more radical resection in pathological processes involving the axis vertebra. Combined with the posterior fusion, immediate stability is achieved. Anterior surgical approach is through a highly unsterile oral environment which presents a high-risk of postoperative infection.

Factors which can influence elastic stable intramedullary nailing removal in healed bone cysts in children.

Elastic stable intramedullary nailing (ESIN) internal fixation is used clinically to treat pathological fractures of bone cysts in children. However, one of the most important complications was removal difficulty. In this study, we aim to analyse the factors which can influence ESIN removal in healed bone cysts in children. From April 2014 to November 2020, the clinical data of 49 children who underwent elastic stable intramedullary nail removal for pathological fractures of the bone cysts in our hospital were retrospectively analysed. The following data, including age, sex, pathological fracture site, with bone graft, number of ESINs, ESIN indwelling time, and extraosseous length of ESIN were collected, and univariate analysis and logistic regression analysis was performed. The frequency of difficulty in ESIN extraction was 44.90% (22/49). The univariate logistic regression analysis showed that age,ESIN indwelling time,with bone garft and extraosseous length of ESIN may be correlated with the difficulty in removing ESIN (P < 0.05), while sex, pathological fracture site, number of ESIN may not be correlated with the difficulty in removing ESIN (P > 0.05).The multivariate logistic regression analysis showed that the ESIN indwelling time was the independent influencing factor for difficulty in removing ESIN (P < 0.05). The factors influencing the ESIN removal in healed bone cysts in children include over 11.79 years old, the long indwelling time of the ESIN(over 10.5 months),with bone graft and short extraosseous length of ESIN(≤ 0.405 cm). These factors influencing ESIN removal in healed bone cysts in children should be considered.

Reconstruction of the extensor mechanism using polypropylene mesh in a displaced pathological fracture of the patella affected by giant cell tumour.

A man in his 30s came to our clinic with a year-long history of progressive pain and swelling in his knee. Diagnostic imaging revealed a displaced patellar fracture with an osteolytic, septated lesion and thinned expanded cortex in both fracture fragments. A core needle biopsy confirmed the diagnosis of giant cell tumour. Treatment involved wide excision of the tumour and the use of polypropylene mesh and a peroneal longus tendon autograft to reconstruct the extensor mechanism of the knee joint. One year postoperatively, the patient experienced no pain, demonstrated full range of motion and showed no signs of functional impairment or local tumour recurrence. This case highlights that reconstruction of the extensor mechanism of the knee after tumour excision with synthetic mesh is an affordable, user-friendly and widely accessible method. It can address large defects effectively while minimising the risks of disease transmission and graft lengthening, resulting in satisfactory outcomes.

Neoplastic pathologic hip fractures are associated with a higher risk of post-operative bleeding and thromboembolic events.

INTRODUCTION: Surgical treatment of hip fractures leads to significant post-operative complications. Although pathologic fractures (PF) are associated with worse outcomes, most studies do not differentiate between etiology (neoplastic and non-neoplastic PF). We seek to compare 30-day complication rates between 1) native hip fractures and neoplastic PF, and 2) neoplastic and non-neoplastic PF. MATERIALS AND METHODS: A total of 127,819 patients with hip fractures and 5104 with PF diagnosed from 2005 to 2021 were retrieved from the NSQIP database. We included 1843 patients with neoplastic PF and 3261 with non-neoplastic PF. Demographics, pre-operative labs and co-morbidities, and post-operative outcomes were analyzed. Propensity-score matching was conducted to control for confounders. RESULTS: Patients with a neoplastic PF had a significantly higher rate of deep venous thrombosis (DVT) (4 % vs 1.2 %, p = 0.001) and pulmonary embolism (PE) (2.4 % vs 0.7 %, p < 0.001), than native hip fractures. Rates of post-operative bleeding were significantly higher in the neoplastic PF group (29.3 % vs 23.9 %, p < 0.001) than non-neoplastic PF. No differences in soft tissue complications were found. When comparing neoplastic and non-neoplastic PF, the former had a higher rate of PE (2.5 % vs 1.0 %, p = 0.015) and post-operative bleeding (27.6 % vs 22.0 %, p = 0.009). Unplanned readmission rates and 30-day mortality rate were also higher in the neoplastic PF group. CONCLUSION: Neoplastic PF of the hip are associated with higher risk of thromboembolic event rates and post-operative bleeding than both native hip fractures and non-neoplastic PF. No differences in rates of soft tissue complications were found between groups.

Intravenous tranexamic acid reduces complications following surgical treatment of pathologic fractures of the lower extremity.

BACKGROUND AND OBJECTIVES: This study aimed to evaluate the postoperative complications associated with administering intravenous (IV) tranexamic acid (TXA) in patients undergoing surgical fixation for neoplastic pathologic fractures of the lower extremities. METHODS: Patients ≥18 years old who underwent surgical intervention for neoplastic pathologic lower extremity fractures from 2015 to 2021 were identified using the Premier Healthcare Database. This cohort was divided by TXA receipt on the index surgery day. Patient demographics, hospital factors, patient comorbidities, and 90-day complications were assessed and compared between the cohorts. RESULTS: From 2015 to 2021, 4497 patients met inclusion criteria (769 TXA[+] and 3728 TXA[-]). Following propensity score matching, patients who received TXA had a significantly shorter length of stay than those who did not (7.6 ± 7.3 days vs. 9.0 ± 15.2, p = 0.036). Between the two cohorts, there were no significant differences in comorbidities. Regarding differences in postoperative complications, TXA(+) patients had significantly decreased odds of deep vein thrombosis (DVT) (1.87% vs. 5.46%; odds ratio [OR]:0.33; 95% confidence interval: 0.17-0.62; p = 0.001). CONCLUSION: Administration of IV TXA may be associated with a decreased risk of postoperative DVT without an increased risk of other complications. Orthopedic surgeons should consider the utilization of IV TXA in patients treated surgically for neoplastic pathologic fractures of the lower extremity.

Non-metastatic hip fractures surgery in patients with active cancer: benefit and risk.

PURPOSE: Although rare, non-metastatic proximal femoral fracture (PFF) can develop in patients with active cancer. However, little data are available regarding the risks and benefits of surgical treatment in such patients. The purpose of his study was to investigate the risks and benefits of surgical treatment of PFF in patients with and without cancer. METHODS: We retrospectively examined the medical records of all patients treated for PFF, excluding those with pathological fracture, at our hospital from July 2013 to December 2020. The patients were divided into two groups; The active cancer group and the standard group. We investigated in both groups about surgical and medical complications during the perioperative period, walking ability two weeks postoperatively, and one-year postoperative mortality rate. RESULT: After the inclusion and exclusion criteria, 39 patients in the active cancer group and 331 patients in the standard group were finally investigated. There were no statistically significant differences between the two groups. The complication rate did not appear statistical significance between two groups (16.7% in active cancer group vs 10.7% in standard group: p = 0.272). Walking ability was also similar in two groups. Mortality rate at one year was significantly higher in the active cancer group. (41.2% in active cancer group vs 6.0% in standard group: p < 0.05). CONCLUSION: Although the active cancer group had a higher mortality rate at one year, which was influenced by the prognosis of the cancer, the benefits of surgical intervention, such as regaining walking ability, were the same in patients with and without active cancer.

Photodynamic bone stabilization for traumatic and pathologic fractures: a systematic review of utilization, complications, and patient-reported outcomes.

INTRODUCTION: The photodynamic bone stabilization system (PBSS) was was developed in 2010, and in 2018 gained FDA approval in the United States. Given its relative novelty, our analysis sought to analyze the available literature exploring the indications, outcomes, and complications of the PBSS. METHODS: We performed a systematic review (PROSPERO registration of study protocol: CRD42022363065, October 8th, 2022). PubMed, EBSCOHost, and Google Scholar electronic databases were queried to identify articles evaluating PBSS in the treatment of pathologic or traumatic fractures between January 1 2010 and 15 October 2022. The quality of the included studies was assessed using the Methodological Index for Nonrandomized Studies tool. RESULTS: Our initial search yielded 326 publications, which were then screened for appropriate studies that aligned with the purpose of our review. A total of thirteen studies, comprising seven case series, four case reports, and two cohort studies. The total sample size of the included studies consisted of 345 patients, with 242 females (70%) and 103 males (30%). The implants were most commonly utilized in the humerus (41%), radius (12%), and metacarpal (12%). The most common complications were related to broken implants (5%) and dislocation (1%). Most studies reported complete fracture healing and return of full strength and range of motion. CONCLUSION: Despite being a relatively novel technology, PBSS appears to be a viable option for fracture stabilization. Most studies included in our analysis reported complete fracture healing and return of function with minimal complications.

Impact of metastatic epidural spinal cord compression (MESCC) and pathological vertebral compression fracture (pVCF) in neurological and survival prognosis.

BACKGROUND: Metastatic epidural spinal cord compression (MESCC) and pathological vertebral compression fractures (pVCF) are the most serious debilitating morbidities of spine metastases (SpMs) causing devastating neurological damages. The respective impact of these two metastasis-spreading entities on survival and on neurological damage is debated. METHODS: A French prospective cohort study collected 279 consecutive patients presenting with SpMs between January 2017 and 2021. We compared 174 patients with MESCC and 105 patients with pVCF. RESULTS: The median Overall Survival (OS) for the MESCC group was 13.4 months (SD 1.5) vs 19.2 months (SD 2.3) for pVCF patients (p = 0.085). Sixty-five patients (23.3 %) were operated on: 49/65 (75.4 %) in the MESCC group and 16/65 (15.2 %) in the pVCF group, p < 0.0001. At 6 months FU, in the MESCC group, 21/44 (45.4 %) of non-ambulatory patients at onset improved to ambulatory status (Frankel D-E) vs 10/13 (76.9 %) in the pVCF group (p = 0.007). In multivariable analysis with the Cox proportional hazard model, good ECOG-PS and SINS Score 7-12 [HR: 6.755, 95 % CI 2.40-19.00; p = 0.001] were good prognostic factors for preserved ambulatory neurological status. However, SpMs diagnosed synchronously with the primary tumor [HR: 0.397, 95 % CI 0.185-0.853; p = 0.018] and MESCC [HR: 0.058, 95 % CI 0.107-0.456; p = 0.007] were independent risk factors for impaired neurological function. CONCLUSION: Contrary to pVCF, MESCC causes neurological damage. Nevertheless, neurological recovery remains possible. MESCC and pVCF have no impact on survival. The management of MESCC remains to be clarified and optimized to reduce neurological damage.

Durability of intercalary endoprosthesis for humeral reconstruction.

INTRODUCTION: The humerus is a common site of metastases and primary tumors. For some patients with a segmental defect and/or diaphyseal cortical destruction a cemented intercalary device may provide a more reliable construct, however data on their use is limited. METHODS: We reviewed 43 (28 male and 15 female) patients treated with an intercalary humeral spacer at a single tertiary referral center between 1989 and 2022. Humeral lesions were most commonly secondary to metastatic disease (n = 29, 68%), with 25 (58%) patients presenting with a pathologic fracture. Mean age and body mass index were 66 years and 27.9 kg/m2 . First generation taper joint device were used in 22 patients and second-generation lap device in 21 patients. RESULTS: Following reconstruction the 2-year overall survival was 30%. Mechanical complications occurred in 11 patients, most commonly aseptic loosening (n = 6, 14%). With death as a competing risk, the cumulative incidence of mechanical failure was 28% at 2-years postoperative. Following the procedure, mean Musculoskeletal Tumor Society scores was 70% and mean shoulder elevation was 87°. CONCLUSION: Reconstruction of the humeral diaphysis with an intercalary endoprosthesis provides restoration of function of the upper extremity, however, is associated with one in four patients having mechanical failure.

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.