Reconstruction with a double-constrained implant design after complex shoulder extra-articular resection.

BACKGROUND: Approximately, one-third of patients with tumors of proximal humerus will require an extra-articular resection to achieve oncologic margins. This procedure yields poor functional outcomes with a considerable rate of revisions. Unconstrained implants are prone to instability hindering also function of the elbow and hand, whereas constrained shoulder reconstructions suffer from early aseptic loosening of the glenoid component due to bone overload. The purpose of this study was to develop a constrained implant suitable for extra-articular resection with loss of function in deltoid and rotator cuff, which would provide both stability and passive motion, whilst also decreasing the risk of aseptic loosening of the glenoid component. METHODS: In cooperation with Czech Technical University in Prague, we devised an implant consisting of two constrained joints in series connected by a dumbbell piece. The biomechanical analysis showed a reduction of load transfer to the glenoid component with a torque of 8.6 Nm capable of generating an 865-N pulling force on bone screw to just 0.07 Nm, hence shielding the glenoid component from undesired forces and decreasing the risk of aseptic loosening. Three patients with extra-articular resection with a total loss of function of both rotator cuff and deltoid muscle received this type of reconstruction. The average follow-up was 16 months. RESULTS: The surgical technique is straightforward. The surgery took 175 min on average with average blood loss of 516 ml. There were no surgical- or implant-related complications. All three patients were pain-free and had a stable shoulder joint after the reconstruction. All had fully functional elbow, wrist, and hand joints. The average Musculoskeletal Tumor Society (MSTS) score was 21/30 (70%). All patients were pleased with the results. CONCLUSION: The presented innovative implant design has demonstrated to be a promising alternative for reconstruction in these challenging cases.

A quantitative CT analysis of fibula inlayed in a massive allograft for femoral diaphysis reconstruction.

Introduction: In diaphyseal reconstructions for bone tumor resection, massive bone allografts (MBA) are historically regarded as the gold standard. However, these are not without complications, and they present an elevated risk of infection, nonunion and structural failure that increases over time as the graft remains largely avascular. To counteract this disadvantage, a combination of allograft with a vascularized fibula has been proposed. The aim of our study was to objectively review the results of combined vascularized fibula-allograft constructs compared to plain allograft reconstruction for bone defects in tumor patients and to assess fibular vitality predictive factors from imaging studies. Materials and methods: Our data was retrospectively reviewed for patients with femoral diaphysis reconstructions in the past ten years. Ten patients (six males and four females) with a mean average follow-up time of 43.80 months (range 20-83, SD 18.17) with combined graft (Group A) were included in the study. As a control group 11 patients (six males and five females) with a mean average follow-up of 56.91 months (range 7-118, SD 41.33) with a simple allograft reconstruction were analyzed (Group B). Demographic and surgical data, adjuvant therapy as well as complications were analyzed in both groups. Both groups were assessed with plain radiographs for bony fusion at the osteotomy sites. Patients in "Group A" had consecutive CT scans at 6 months and then annually to check for potential bone stock and bone density changes. We analyzed total bone density as well as incremental changes in three different areas of the reconstruction. This was done at two defined levels for each patient. Only patients with at least two consecutive CT scans were included in the study. Results: There were no statistical differences between the groups in terms of demographics, diagnosis or adjuvant therapy (p = 1.0). The mean average surgical time (599.44 vs 229.09) and mean average blood loss (1855.56 ml vs. 804.55 ml) were significantly higher in the combined graft group A (p < 0.001 and p = 0.01, respectively). The mean average length of resection (19.95 cm vs. 15.50 cm) was higher in the combined graft group (p = 0.04). The risk for non-union and infectious complication was higher in the allograft group, however, the difference was not significant (p = 0.09 and p = 0.66, respectively). The mean average time to union at junction sites was 4.71 months (range 2.5-6.0, SD 1.19) for cases of successful fibula transfer, 19.50 months (range 5.5-29.5, SD 12.49) for the three cases where we presumed the fibula was not viable and 18.85 months (range 9-60, SD 11.99) for the allograft group. The difference in healing time was statistically significant (p = 0.009). There were four cases of non-union in the allograft group.Seven out of ten patients in Group A exhibited incremental changes in all CT scan measured values. This difference was statistically significant already at 18 months from the index surgery (p = 0.008). The patients with a non-viable fibula had a smaller increase in the percentage of total bone density area measured in the CT scan compared to those patients with a successful fibula transfer (4.33, SD 2.52 vs. 52.29, SD 22.74, p = 0.008). The average bone density incremental increase in-between the fibula and allograft was different among patients with an unsuccessful fibula transfer (32.22, SD 10.41) and the ones with a viable fibula (288.00, SD123.74, p = 0.009). Bony bridges were observed in six cases of viable fibula and in none of the tree presumably dead fibulas (p = 0.03). The mean average MSTS score was higher for the subgroup of successful fibular transfer (26.7/30, SD 2.87) when compared to the group of non-viable fibular graft (17.00/30, SD 6.08) and this was also statistically significant (p = 0-007). Conclusion: A viable fibula enhances incorporation of the allograft and decreases the risk for both structural failure as well as infectious complications. Viable fibula also contributes to better functional status of the recipient. Consecutive CT scans proved to be a reliable method for assessing fibular vitality. When no measurable changes are present at 18-month follow-up, we can declare the transfer unsuccessful with a good amount of certainty. These reconstructions behave as simple allograft reconstructions with analogue risk factors. The presence of either axial bridges between the fibula and allograft or newly formed bone on the inner surface of the allograft is indicative of a successful fibular transfer. The success rate of fibular transfer in our study was only 70% and skeletally mature and taller patients seem to be at increased risk for failure. The longer surgical times and donor site morbidity therefore warrant stricter indications for this procedure.

Metaphyseal chondroblastoma in a pediatric patient.

Chondroblastoma is a rare, benign neoplasm usually located in the epiphyses and apophyses of the long bones in the immature skeleton. Radiologically, these tumors have a classic appearance of a lytic lesion with chondroid matrix surrounded by a thin sclerotic rim. Here, we describe the case of a 5-year-old male who presented with a chondroblastoma unusually located exclusively in the metaphyseal region, which led to an elusive diagnosis. The presence of tumors outlying the traditional location or epidemiological spectrum, along with the potential for histopathological misdiagnosis, can pose a diagnostic and therapeutic challenge for the treating team.

Aggressive atraumatic myositis ossificans in a toddler.

Myositis ossificans (MO) is a benign disorder where bone forms within muscles or other soft tissues. This condition usually follows trauma and is rare in pediatric patients. Here we present the case of a 2-year-old male who developed MO of his right elbow without obvious trauma to the area. Imaging of MO in the initial phase is highly unspecific and obtaining tissue samples through a biopsy can render misleading reports. In most cases MO is a self-limited process with complete resolution, however, some cases may present a diagnostic and therapeutic challenge.

Multifocal eosinophilic granuloma with femoral epiphyseal lesion mimicking an aneurysmal bone cyst.

Eosinophilic granuloma (EG) is a rare benign tumor-like disorder characterized by abnormal proliferation Langerhans cells. EG frequently presents as a solitary lesion in the axial skeleton and diaphysis long bones. Here we present the case of a 14-year-old male with multifocal EG with a lesion located in the femoral epiphysis mimicking an aneurysmal bone cyst that presented a diagnostic challenge. While the initial presentation of EG patients may appear uncommon, its overlapping features with other benign and malignant etiologies highlight the importance of increased awareness of this condition, as well as the need for an experienced multidisciplinary team in its diagnosis and treatment.

Sclerosis of the clavicle--A challenging diagnosis.

Condensing osteitis of the clavicle is a rare benign disease described as an increase in bone density at the medial end of the clavicle. Its clinical and radiographic presentation can frequently be equivocal and tissue sampling is necessary for diagnostic confirmation. Here we present the case of a 29-year-old female with condensing osteitis of the right medical clavicle, who remained undiagnosed for many years despite obtaining imaging studies and undergoing an initial biopsy. This disease presents oftentimes a challenging diagnosis due to its imaging features overlapping with many benign and malignant bone lesions. A qualified multidisciplinary team with expertise in rare bone conditions becomes oftentimes essential to arrive at an accurate diagnosis.

Displaced humeral pathological fracture secondary to intraosseous hemangioma.

Intraosseous hemangiomas are uncommon slow-growing benign bone tumors. Most of these lesions are located in the spine or skull and long bone location is rare. Here we present the case of a 63-year-old female with a pathological fracture of the left proximal humerus through an intraosseous hemangioma. Imaging features can be highly unspecific when these tumors are found in the long bones. In this case a pathological fracture obscured the diagnosis even further, prompting the need for tissue sampling to exclude an underlying malignancy.

Missed pathological femoral neck fracture undergoes spontaneous healing.

Pathologic fractures are common complications of metastatic bone disease in patients with breast cancer. Fractures involving the proximal femur generally cause significant pain that is exacerbated by ambulation. Due to excessive stress on the weight-bearing hip joint, these fractures present a significant burden on the quality of life among patients. Here we describe a case of a 38-year-old female patient who was found to have a pathologic fracture of the proximal femur missed on imaging studies that underwent spontaneous union. Pathologic fractures rarely heal on their own, since a tumor at the fracture site interferes with bone healing and most fractures have to be managed with surgical intervention. Fractures can be missed on imaging studies in the setting of extensive metastatic disease. Physicians should be cognizant of this fact and maintain a high level of suspicion to recognize fractures with unusual presentations where patients may not present with the typical findings of acute onset of pain and inability to ambulate or bear weight.

Rapidly acquired valgus deformity of the knee after osteochondroma resection in multiple hereditary exostoses pediatric patients: A report of two cases.

Patients with multiple hereditary exostoses (MHE) often develop leg length discrepancies and limb alignment deformity around the knee as part of the natural course of the disease. Limb alignment deformity occurring post-resection of an osteochondroma has been described in one case report and only pertaining to the proximal medial tibia location. Here we describe the case of 2 patients with MHE, a 7-year-old female who underwent resection of distal femur and proximal tibia osteochondromas and a 9-year-old female who had a distal femur osteochondroma resected. Both patients developed rapidly progressive valgus knee deformity requiring surgical intervention. Excision of osteochondromas near the physis of a skeletally immature patient can cause overgrowth from the involved side of the growth plate resulting in a rapidly progressing unilateral coronal plane deformity. Surgeons should be aware of this potential complication and closely follow growing patients with serial alignment radiographs and counsel the family regarding the potential of acquired limb deformity and subsequent surgeries.

Intramedullary rod failure in metastatic breast cancer: Do triple negative cancer patients have more revision surgery?

BACKGROUND: Breast cancer is the most common cancer and second cause of death in women worldwide. Patients with breast cancer are classified into subgroups based on the presence or absence of hormone receptors and the human epidermal growth factor 2-neu (HER-2) marker, the different molecular profiles come with an associated prognosis and variety of possible treatment options. Patients with triple negative cancer have a worse prognosis, a more aggressive behavior, higher likelihood of spreading, a higher risk of recurrence and a poorer outcome overall. Intramedullary rod fixation has proven to provide a good outcome and function in patients with metastatic breast cancer, but no study has addressed the receptor-status potential outcome differences that may affect disease progression at an orthopaedic surgery site. QUESTIONS/PURPOSES: (1) Do patients with triple negative breast cancer have a higher revision rate of intramedullary rod fixation of bone metastases? (2) Do patients with metastatic triple negative breast cancer have a higher revision rate of intramedullary rod fixation due to local disease progression? METHODS: This was a single-center, observational, retrospective cohort study. Fifty-seven patients with a diagnosis of breast cancer metastatic to long bones who underwent surgical fixation with an intramedullary rod for a pathological fracture or an impending fracture due to a bone metastasis with a Mirels' score equal or above 8 between January 2004 and December 2016 at our institution were included. All implants used were from the same manufacturer (Stryker Corp., Mahwah, NJ, USA). Patients were divided into two groups based on the receptor status of the tumor and were classified either as triple negative, when the tumor lacked progesterone, estrogen and HER-2 receptors, or as receptor-positive when the presence of one or a combination of either three was proven. In the triple-negative tumor group the mean follow up time was 26 months (SD 29) and median follow up time was 16 months. In the receptor-positive tumor group mean follow up was 27 months (SD 24) with a median follow up of 19 months. To assess possible associations between different factors and the outcomes of interest, we used either the chi-square test or Fisher's exact test for categorical variables and the ANOVA test for continuous variables. For the survival assessment, a Kaplan-Meier analysis was performed and for the cumulative incidence a competing risk analysis was utilized. RESULTS: The intramedullary rod revision rate for patients in the triple-negative tumor group was 17%, while for the receptor-positive group it was 12%, this was not statistically different for our sample size. The mean time for revision of the intramedullary rod in the whole sample was 19 months (SD 11, range 6-40). The causes of revision were disease progression (43%), nonunion (29%) and surgeon error (29%). The cumulative incidence of revision surgery was 6% (CI 95%, 2-14%) at 12 months and 20% (CI 95%, 8-36%) at 60 months. CONCLUSIONS: Intramedullary rodding can be considered for the treatment of long bones metastases in breast cancer patients for an impending or actual pathological fracture. There is no difference in the intramedullary rod revision rate among patients with different receptor-status when comparing triple-negative tumor patients and receptor-positive ones. LEVEL OF EVIDENCE: Level III, therapeutic study.

Tenosynovial giant cell tumor of the distal tibiofibular joint.

Tenosynovial giant cell tumors are extremely rare tumors with highly nonspecific symptoms. This benign but aggressive disease has a slow course of progression; however, it can ultimately lead to irreversible damage to a joint. Here we describe a case of a 45-year-old female with a diagnosis of tenosynovial giant cell tumors of the distal tibiofibular joint, the second case described in the literature for such location. Appropriate imaging studies and ultimately histologic studies are necessary for the correct diagnosis. Some locations are particularly unusual for these tumors making a high level of suspicion as well as treatment by an oncology orthopedic surgery specialist at a high-volume center paramount.

Molded, Gamma-radiated, Argon-processed Polyethylene Components of Rotating Hinge Knee Megaprostheses Have a Lower Failure Hazard and Revision Rates Than Air-sterilized, Machined, Ram-extruded Bar Stock Components.

BACKGROUND: Megaprostheses are commonly used for reconstruction after distal femoral resection in orthopaedic oncology. The polyethylene bearings in these reconstructions experience wear and wear-related complications that may result in revision surgery. Improved manufacturing and processing of polyethylene has increased the durability of components commonly used for routine arthroplasty. Alterations in the manufacture of polyethylene is expected to reduce the revision risk of oncologic megaprostheses, resulting in fewer revision procedures, but this has not been proven. QUESTIONS/PURPOSES: Is there a difference in the hazard of polyethylene wear or breakage leading to prosthetic revision between differences in polyethylene manufacture and processing based on a competing risk analysis? METHODS: This was a single-center, observational, retrospective comparative study of 224 patients who had distal femur megaprostheses with identical rotating hinge articulations and knee kinematics after oncologic surgery from 1993 to 2015. No differences in surgical indications, joint articular components and kinematics, age, sex, diagnosis, BMI, use of chemotherapy, or tumor stage were seen with the patient numbers available. Prosthetic survivorship free from prosthetic revision surgery because of polyethylene wear-related revisions, defined as breakage, increased excursion on varus-valgus stress, or new locking or giving way was compared between two groups of patients: group 1 polyethylene (P1) (66 patients) who had air-sterilized machined ram-extruded bar stock or group 2 polyethylene (P2) (158 patients) molded gamma-radiated argon-processed polyethylene components. The mean follow-up duration for the P1 group (89 ± 55 months) was not different from that of patients with P2 polyethylene (79 ± 63 months; p = 0.24) including 27% (18 of 66) of patients in the P1 group and 25% (40 of 158) of patients in the P2 group followed for more than 10 years. More patients in the P2 group were lost to follow-up (9.2%, 16 of 174) than in the P1 group (5.7%, 4 of 70) but this was not statistically different (chi square; p = 0.37). The hazard of revision because of polyethylene wear or breakage was calculated with a competing risk analysis using the Fine-Gray subdistribution hazard model. RESULTS: The P1 implants had a higher hazard ratio for revision caused by polyethylene damage at 120 months than did the P2 polyethylene implants (P1 HR 0.24 [95% CI 0.13 to 0.36] versus HR 0.07 [95% CI 0.03 to 0.12]), which represents an estimated absolute risk reduction of 17% (95% CI 6.15 to 27.9). CONCLUSION: Polyethylene damage can result in megaprosthetic revisions in patients undergoing oncologic procedures. The hazard of polyethylene failure resulting in revision surgery was lower in patients who received recent polyethylene than in patients with polyethylene produced by previous methods, enhancing the durability of distal femoral megaprosthetic reconstructions. Despite improvements in polyethylene manufacture and clinical results, revision solely because of polyethylene damage still occurs in 7% of patients by the 10-year timepoint; thus, more improvement is needed. Patients who receive these implants should be monitored for signs and symptoms of polyethylene damage. LEVEL OF EVIDENCE: Level III, therapeutic study.

Intrapelvic melanocytic schwannoma resection with computer-assisted navigation.

Melanocytic schwannoma is a rare nerve tumor characterized by melanin-producing neoplastic Schwann cells. Wide surgical resection is the management of choice for this tumor; however, anatomical location and proximity to nerve roots can make locating this tumor and the surgical resection challenging. Here we describe the case of 49-year-old male with a melanocytic schwannoma in the presacral area adjacent to the second sacral nerve root that was managed by wide resection aided by computer-assisted navigation due to the difficulty in identifying its location intraoperatively. The utilization of computer-assisted navigation improves accuracy and precision through the creation of a virtual continuous tridimensional map, particularly useful when oftentimes tumor margins may seem equivocal and further resection would compromise the patient's functionality. The value of computer-assisted navigation for soft tissue tumor resections in orthopedic oncology is still in its infancy, though, in certain scenarios it may advance the technique for some soft tissue resections.

Intraosseous lipoma of the clavicle with extraosseous extension.

Intraosseous lipomas are rare primary benign bone tumors which present with highly nonspecific radiographic features that may lead to equivocal diagnoses. Advanced imaging studies such as MRI with and without contrast and, in some selected cases, tissue sample analyses are required in the diagnostic pathway. Here we describe the second case in the literature of an intraosseous lipoma of the clavicle and the first with extraosseous extension. Subsequent to histologic confirmation the lesion was monitored with clinical and radiologic evaluation.

Metastatic vertebral lesion mimicking an atypical hemangioma with negative 18F-FDG positron emission tomography-computed tomography.

Atypical hemangiomas of the spine can mimic metastatic lesions on magnetic resonance imaging, therefore making this distinction is a diagnostic challenge. In most cases, this conundrum can usually be solved with positron emission tomography/computed tomography images, because hemangiomas do not usually present with increased uptake while metastatic lesions do. Here we present a case of a patient with a unique diagnosis, myxoid liposarcoma, in which the vertebral metastatic lesion did not present with increased uptake in positron emission tomography/computed tomography scans. While keeping the imaging particularity of this rare sarcoma in mind, proceeding with a biopsy when the suspicion of metastasis remains high will help elucidate the diagnosis and allow for proper management.

A case of a myxoma within the obturator externus muscle.

Intramuscular myxomas (IM) are rare benign tumors of mesenchymal origin. These tumors have a high myxoid content and a nonspecific radiologic appearance. Here we describe the case of a patient with an IM in an infrequent location; within the obturator externus muscle. A singular approach was utilized for the resection of this tumor in such rare location. Despite IM being benign tumors, patients may require a surgical excision due to the difficulty of differentiating these tumors, with unreliable imaging features, from other myxoid soft tissue masses.

Recurrent parachordoma of the lower back: A case report.

Parachordoma is a rare entity with less than 50 cases described in the literature. This soft-tissue tumor resembles chordomas as well as extraskeletal myxoid chondrosarcomas and has only recently been fully characterized. Here we describe the case of a patient with a lower back parachordoma and its subsequent postresection recurrence 9 years after the initial procedure, emphasizing the importance of long-term follow-up in individuals with this diagnosis.