Pubic Aneurysmal Bone Cyst Following Penile Inversion Vaginoplasty: A Case Report.

Background: Aneurysmal bone cysts (ABCs) are aggressive, expansile, and locally destructive vascular lesions. The exact etiology of ABCs is currently unknown and hypothesized to be related to vascular malformations or disruption of osseous vascularity. To date, there have been no reports describing the development of pubic ABCs following penile inversion vaginoplasty (PIV). Methods: This report describes the development of a pubic ABC in a transgender patient who had previously undergone PIV, possibly indicating a very rare complication of this gender-affirming operation. Results: A 37-year-old transgender female was initially referred to the orthopedic oncology clinic for evaluation of a 12-month history of left hip and groin pain. She had undergone gender-affirming PIV about 19 months prior to presentation. Magnetic resonance imaging (MRI) with contrast revealed a low T1 signal intensity and heterogenous T2 hyperintensity 7.5 × 4.9 × 4.3-cm destructive mass in the left superior pubic ramus extending across the pubic symphysis into the right superior pubic ramus. A needle core bone biopsy demonstrated a variably cellular spindle and round lesion with islands of osteoid formation and focal necrosis. The cells were negative for CD34, S100, and desmin. There was no evidence suggesting osteosarcoma, and final review favored the diagnosis of an ABC. Given the highly destructive nature of the mass, it was resected, and the resulting wound was reconstructed with a biologic dermal mesh. Conclusions: Although it is impossible to distinguish coincidence from causation in this case, the patient's recency of PIV and development of a rare ABC in a nearby bone warrants the speculation and discussion provided in this report.

Idiopathic Spontaneous Occurrence of Heterotrophic Occurrence During Pregnancy.

Heterotopic ossification (HO) typically presents in the hip, knee, and elbow joints in the setting of trauma or postsurgical intervention. Less commonly, it may occur secondary to neurologic dysfunction or underlying genetic conditions, but idiopathic HO is rare. Most cases of HO are managed nonoperatively with surgical resection remaining a controversy due to high recurrence rates. We describe a case of idiopathic HO of the shoulder that occurred in the absence of trauma, neurologic dysfunction, or underlying genetic disorder that was treated with surgical excision.

Novel Positioning Feedback System as a Guidance in Bone Tumor Resection.

Need: Bone resection using customized 3D-printed guides can improve accuracy, but the technique is still associated with clinically significant errors.Technical solution: We developed an inexpensive optical feedback system (OFS) that compares intraoperative 2D camera images to the pre-operative plan, and accurately depicts the surgeon's guide placement prior to cutting, reducing the errors in resection.Proof of concept: We simulated wide resections of a bone sarcoma on 24 cadaver femurs using 3 cutting guide types. Guide placement was measured using the OFS and compared to CT-scans showing the actual guide position. We carried out a second, controlled study on 20 sawbones, comparing the accuracy of the final bone cuts with and without the surgeon actively using the OFS to adjust the guide position before cutting.Results: For cadavers, in 2 of 3 planes, the position of the jig recorded by the OFS closely matched its actual position, with an accuracy of .87° ± .65°(r = .94) and 1.2° ± 1.3°(r = .81) in the transverse and sagittal planes, respectively. In the second study, OFS increased accuracy of the final cut about the transverse and sagittal planes, respectively by 53.1% (P = .011)/54.7% (P = .04) and 33% (P = .051)/38% (P = .042) in terms of rotation and translation.Next steps: Developing the OFS as a mobile application to reduce the processing time and improve accessibility in the operating room.Conclusion: The OFS could accurately depict the guide placement on the bone and significantly improve the surgical accuracy of 3D printed jigs.

Glomus Tumor of the Lower Extremity Previously Misdiagnosed as Complex Regional Pain Syndrome in Close Proximity to a Myxofibrosarcoma: A Case Report.

Complex regional pain syndrome (CRPS) is a potentially devastating condition that can result in severe psychological and social morbidity. It is a diagnosis of exclusion, and other pathologic entities must be ruled out first. Glomus tumors are exquisitely painful benign vascular tumors that are most common in the hand and are rarely found in the lower extremity. Here, we present a case of a patient who developed a focus of severe anterior knee pain and tenderness a few months after a car accident that had been misdiagnosed as CRPS for 15 years. She coincidentally developed a sarcoma of her ipsilateral leg distal to this site. Magnetic resonance imaging of the sarcoma included the area of knee pain where, interestingly, it identified a separate small soft-tissue mass. A glomus tumor was diagnosed histologically in a needle biopsy specimen from this mass, which was resected along with the sarcoma. For the first time in 15 years, despite the additional sarcoma surgery, she reported relief of her pain and complete resolution of her "CRPS."

The Management of an Open Biopsy Tract Site Infection and Ulceration in the Setting of Osteosarcoma.

Biopsy site infection in the setting of osteosarcoma is a potentially devastating complication. We present the case of a 16-year-old adolescent girl with a distal femur osteosarcoma who developed an open biopsy site ulceration and infection after initiation of neoadjuvant chemotherapy. This was treated with careful local excision of the ulcerated biopsy site and systemic antibiotic therapy throughout the duration of her chemotherapy course. She subsequently underwent wide resection of the tumor en bloc with a generous ellipse around the biopsy scar and reconstruction with cemented knee megaprosthesis. No recurrence of either infection or malignancy was observed. This case represents the successful treatment of a biopsy site ulceration and infection in a patient with osteosarcoma and may merit adoption in future instances of this complication.

Negative-Pressure Wound Therapy versus Standard Surgical Dressings after Malignant Tumor Resection: A Systematic Review and Meta-Analysis.

BACKGROUND: Negative-pressure wound therapy offers many advantages over standard surgical dressings in the treatment of open wounds, including accelerated wound healing, cost savings, and reduced complication rates. Although contraindicated by device manufacturers in malignancy-resected wounds because of hypothesized risk of tumor recurrence, negative-pressure wound therapy is still applied postoperatively because of limited clinical support. The authors performed a systematic review with meta-analysis to compare negative-pressure wound therapy outcomes with those of standard surgical dressings on open wounds, with their null hypothesis stating there would be no outcome differences. METHODS: A systematic review of the literature on negative-pressure wound therapy and standard surgical dressings on malignancy-resected wounds was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using PubMed, Embase, CINAHL, and Cochrane Central databases. Meta-analysis compared group outcomes, including malignancy recurrence, wound complication, and surgical site infection rates, with a random effects model. RESULTS: A total of 1634 studies were identified and 27 met eligibility criteria, including four randomized controlled trials, four prospective cohort studies, and 19 retrospective reviews. Eighty-one percent of articles ( n = 22) recommended negative-pressure wound therapy in malignancy-resected wounds. Meta-analysis determined that the treatment yielded significantly lower overall surgical site infection ( p = 0.004) and wound complication ( p = 0.01) rates than standard surgical dressings; however, there were no statistically significant differences found for other outcomes between the two groups. CONCLUSIONS: This review demonstrates favorable outcomes of negative-pressure wound therapy over standard surgical dressings for malignancy-resected wounds without an increased risk of malignancy recurrence. However, because limited randomized controlled trials (detailing only incisional wounds for limited malignancies and anatomic regions) are available, additional high-power randomized controlled trials are recommended.

A novel intraoperative method to project osteotomy lines for accurate resection of primary bone sarcomas.

Background: Accurate reproduction of a preoperative plan is critical in wide resection of bone sarcomas. Recent advances in computer navigation and 3D-custom jigs have increased resection accuracy, although with certain practical drawbacks. Methods: We developed a novel "projector method" that projects the preoperative osteotomy lines onto the bone. A sawbone study was conducted to evaluate accuracy in reproducing preoperative resection plans. An additional cadaver experiment was conducted to evaluate feasibility in a more realistic operating room setting. Results: Based on the results of experiments conducted on sawbones, the proposed light projector method was more accurate at depicting desired osteotomy lines than a traditional manual method, reducing the corner deviation from 2.53 mm to 0.35 mm, angular deviation from 2.10° to 0.31°, and point deviation from 4.66 mm to 0.48 mm (p < 0.001). Results of the cadaver experiment were consistent with those of sawbone experiments. Conclusions: The new projector method can accurately assist surgeons in visualizing the preoperative plan of osteotomy lines accurately in surgery.

A Novel 3D Light Assisted Drawing (3D-LAD) Method to Aid Intraoperative Reproduction of Osteotomy Lines Surrounding a Bone Tumor During Wide Resection: An Experimental Study.

Introduction: Computer navigation and customized 3D-printed jigs improve accuracy during bone tumor resection, but such technologies can be bulky, costly, and require intraoperative radiation, or long lead time to be ready in OR. Methods: We developed a method utilizing a compact, inexpensive, non-X-ray based 3D surface light scanner to provide a visual aid that helps surgeons accurately draw osteotomy lines on the surface of exposed bone to reproduce a well-defined preoperative bone resection plan. We tested the accuracy of the method on 18 sawbones using a distal femur hemimetaphyseal resection model and compared it with a traditional, freehand method. Results: The method significantly reduces the positional error from 2.53 (±1.13) mm to 1.04 (±0.43) mm (p<0.001), and angular error of the front angle from 2.10° (±0.83°) to 0.80° (±0.66°) (p=0.001). The method also reduces the mean maximum deviation of the bone resection, with respect to the preoperative path, from 3.75mm to 2.69mm (p=0.003). However, no increased accuracy was observed at the back side of the bone surface where this method would not be expected to provide information. Discussion: In summary, we developed a novel 3D-LAD navigation technology. From the experimental study, we demonstrated that the method can improve the ability of surgeons to accurately draw the preoperative osteotomy lines and perform resection of a primary bone sarcoma, with comparison to traditional methods, using 18 sawbones.

A novel method of light projection and modular jigs to improve accuracy in bone sarcoma resection.

We developed a novel method using a combined light-registration/light-projection system along with an off-the-shelf, instant-assembly modular jig construct that could help surgeons improve bone resection accuracy during sarcoma surgery without many of the associated drawbacks of 3D printed custom jigs or computer navigation. In the novel method, the surgeon uses a light projection system to precisely align the assembled modular jig construct on the bone. In a distal femur resection model, 36 sawbones were evenly divided into 3 groups: manual-resection (MR), conventional 3D-printed custom jig resection (3DCJ), and the novel projector/modular jig (PMJ) resection. In addition to sawbones, a single cadaver experiment was also conducted to confirm feasibility of the PMJ method in a realistic operative setting. The PMJ method improved resection accuracy when compared to MR and 3DCJ, respectively: 0.98 mm versus 7.48 mm (p < 0.001) and 3.72 mm (p < 0.001) in mean corner position error; 1.66 mm versus 9.70 mm (p < 0.001) and 4.32 mm (p = 0.060) in mean maximum deviation error; 0.79°-4.78° (p < 0.001) and 1.26° (p > 0.999) in mean depth angle error. The PMJ method reduced the mean front angle error from 1.72° to 1.07° (p = 0.507) when compared to MR but was slightly worse compared to 0.61° (p = 0.013) in 3DCJ. The PMJ method never showed an error greater than 3 mm, while the maximum error of other two control groups were almost 14 mm. Similar accuracy was found with the PMJ method on the cadaver. A novel method using a light projector with modular jigs can achieve high levels of bone resection accuracy, but without many of the associated drawbacks of 3D printed jigs or computer navigation technology.

A novel bone registration method using impression molding and structured-light 3D scanning technology.

Accurate bone registration is critical for computer navigation and robotic surgery. Existing registration systems are expensive, cumbersome, limited in accuracy and/or require intraoperative radiation. We recently reported a novel method of registration utilizing an inexpensive, compact, and X-ray-free structured-light 3D scanner. However, this technique is not always practical in a real surgical setting where soft tissue and blood can obstruct the continuous line-of-sight required for structured-light technology. We sought to remedy these limitations using a novel technique using rapid-setting impression molding to capture bone surface features and scan the undersurface of the mold with a structured-light scanner. The photonegative of this mold is compared to the preoperative computed tomography (CT)-scan to register the bone. A registration accuracy study was conducted on 36 CT-scanned femur sawbones, simulating typical exposure in hip/knee arthroplasty and bone tumor surgery. A cadaver experiment was also conducted to evaluate the feasibility of using the impression molding in a more realistic operating room setting. The registration accuracy of the proposed technique was 0.50 ± 0.19 mm. This was close to the reported accuracy of 0.43 ± 0.18 mm using a structured-light scanner without impression molding (p = 0.085). In comparison, historical values for "paired-point" and intraoperative CT image-based registration methods currently used in modern robotic/computer-navigation systems were 0.68 ± 0.14 mm (p = 0.004) and 0.86 ± 0.38 mm, respectively. The registration accuracy of the cadaver experiment was consistent with that of sawbone experiments. Although future studies are needed to extend to human subjects, this study shows that the impression molding method can produce comparable or better registration accuracy than the existing techniques.

3D-Printed Guides in Bone Tumor Resection: Studying Their Error and Determining a Safety Margin for Surgery.

3D-printed guides, which have recently been introduced in orthopedic oncology, improve resection accuracy compared with traditional bone resection methods, but there are inaccuracies associated with them. These inaccuracies could lead to disastrous outcomes such as positive tumor resection margins. In this Sawbone study, we sought to quantitatively investigate the margin of error for various jig types and to determine a "safety margin" that could serve as a guide for surgeons and jig engineers in creating 3D-printed jigs that would reduce the risk of potential disastrous results such as positive margins. Various 3D-printed jigs were used to simulate wide resection of a distal femoral bone sarcoma on Sawbone specimens by 10 individuals with no specific prior expertise in cutting guides. We developed a mathematical model using kinematic theory. We defined a safety margin as the amount of change in the osteotomy lines that must be incorporated into the jig design to ensure that the surgeon is at least 98% likely not to have a positive tumor margin. Experiments were conducted to determine the mean deviation experienced in placing cutting guides on the bones. The mean deviation for the four types of cutting guides ranged from 2.86 mm to 6.54 mm. We determined that a jig design should have a safety margin of 4.8 mm for standard guides and 8.65 mm for gusset guides to minimize the possibility of cutting into the tumor as a result of human error in guide placement. Further studies involving cadavers and patients are warranted. [Orthopedics. 2022;45(3):169-173.].

Systemic Mastocytosis Presenting as Pathologic Intertrochanteric Femur Fracture.

Systemic mastocytosis (SM) is pathologically characterized by the proliferation of mast cells with infiltrates in various organs, almost always including bone marrow, leading to defects in bone remodeling. Osteoporosis and subsequent fragility fractures are the most common and clinically relevant presentation, although pathologic fracture through the focal lytic lesions can also be observed. Here, we report the case of a 54-year-old woman, with a recent history of unexplained severe allergic reactions, presenting with intertrochanteric fracture of the left femur which on careful history, physical and radiological evaluation was determined to be pathological. The patient was found to have lytic lesions on the CT scan at the fracture site and the pelvis, bilateral femurs, ribs, and sternum, raising suspicion for malignancy. The malignancy workup failed to reveal a primary neoplasm, and the patient was indicated for intramedullary fixation of the left femur along with intraoperative biopsy. Pathologic evaluation of the femoral biopsy was positive for aggregates of mast cells with CD117 (c-KIT, D816V). This finding prompted a bone marrow biopsy, which ultimately led to the diagnosis of aggressive SM. Femoral intramedullary fixation was done with a trochanteric femoral nail, and the patient was postoperatively started on calcium, vitamin D, and physical therapy. Systemic disease was managed by the hematology-oncology team, and the patient was given an epinephrine autoinjector (EpiPen) and managed with midostaurin (Rydapt, Novartis Pharmaceuticals). Treating surgeons should be aware that a pathological long bone fracture can be the initial presentation of SM. Furthermore, surgeons should consider following patients with SM for longer than usual considering a higher risk of complications, such as implant loosening, nonunion, and refracture due to poor and progressively worsening quality of the bone. Our patient was followed with routine visits for 30 months and showed no clinical or radiographical signs of any complications.

Monophasic Synovial Sarcoma in the Elbow Misclassified but Successfully Treated as Ewing's Sarcoma with Chemotherapy.

To the best of the authors' knowledge, this is the first published case of monophasic synovial sarcoma (SS) initially diagnosed as Ewing's sarcoma (ES), yet successfully treated with chemotherapy in a 24-year-old patient. The initial diagnosis showed a monotonous round cell tumor and positivity for CD99, characteristic of ES; however, the cytology was negative for the classic EWSR1 rearrangement of ES. The patient was treated with the standard chemotherapy protocol of ES - COG AEWS1031 Regimen A with vincristine, doxorubicin, cyclophosphamide, and mesna - as well as with wide resection. Post-resection tissue submission showed additional morphologic features which led to a re-evaluation of the classification of the tumor as well as additional molecular studies; these revealed positivity for translocations of SS18 (18q11.1) in 100% of the nuclei, which is most characteristic of SS, thus, reclassifying the neoplasm as a SS tumor. This case underscores the importance of considering several pathologic entities in the differential diagnosis of small, round blue cell tumors, including ES, SS, and lymphoma. It also demonstrates the importance of using chromosomal identification for a more definitive diagnosis, rather than relying on histological features and markers which are found in more than one tumor classification. There is conflicting evidence of the impact of chemotherapy on survival in SS, as it is primarily treated with radiation therapy. Since SS is rare, prospective studies on the effect of chemotherapy on survival are limited in number. However, our case study demonstrates that chemotherapy is another modality that can be used in the treatment of SS neoplasms.

Inflammatory Myofibroblastic Tumor 12 Years After Treatment for Synovial Sarcoma: A Case Report.

Inflammatory myofibroblastic tumors (IMTs) are mesenchymal neoplasms most seen in the abdominopelvic region, lung, and retroperitoneum; and less commonly seen in virtually any other site. We report a case of two lower limb masses consistent with diagnosis of IMTs. This is a 39-year-old woman with a history of right lower extremity popliteal fossa synovial sarcoma diagnosed 12 years prior and treated with chemotherapy, surgery, and radiation. She presented with two new - one anterior and one posterior - right thigh masses. Biopsies of the lesions demonstrated low-grade inflammatory spindle cell lesions at both sites. Wide resection was performed for both masses and further characterization of the surgical specimens was most consistent with IMT. At follow-up, the patient is well with no signs of recurrence 19 and 7 months postoperative to the resection of the anterior and posterior thigh masses, respectively. This case represents the first reported IMTs occurring as late as 12 years after primary cancer treatment, and the first occurring after synovial sarcoma.

Treatment Approach for Knee Osteoarthritis with Ipsilateral Femoral Cartilage Tumor: A Case Series.

Osteoarthritis (OA) is a common pathology affecting the knee joint. Twenty percent of the cartilaginous tumors of bone are in the distal femur. This presents a challenge for treating patients with knee OA who also have ipsilateral distal femur cartilage tumors. We propose a classification system for knee OA with ipsilateral cartilaginous tumor and a treatment approach to address this issue. Intramedullary guides are avoided when performing total knee arthroplasty (TKA) in these patients so as not to further contaminate the femur with tumor cells. A non-image-based stereotactic surgical system is favored at our institution to achieve this goal. Seven patients underwent classification and treatment with TKA utilizing this approach. Average final follow-up was 15.3 months. Component alignment averaged 89.2° and all patients had 0° of extension with an average flexion to 107.5°. There were no postoperative complications and no radiographic evidence of component complication, tumor recurrence, or tumor expansion. Few studies have reported on the treatment of knee OA with concurrent cartilaginous tumor of the distal femur. All patients treated with the proposed approach had their OA successfully treated without complication related to their cartilaginous tumor or TKA components. This case series presents a novel classification and treatment algorithm to potentially guide arthroplasty surgeons in approaching these often-concurrent occurring pathologies.

Report on a novel bone registration method: A rapid, accurate, and radiation-free technique for computer- and robotic-assisted orthopedic surgeries.

INTRODUCTION: Computer- and robotic-assisted technologies have recently been introduced into orthopedic surgery to improve accuracy. Each requires intraoperative "bone registration," but existing methods are time consuming, often inaccurate, and/or require bulky and costly equipment that produces substantial radiation. METHODS: We developed a novel method of bone registration using a compact 3D structured light surface scanner that can scan thousands of points simultaneously without any ionizing radiation.Visible light is projected in a specific pattern onto a 3 × 3 cm2 area of exposed bone, which deforms the pattern in a way determined by the local bone geometry. A quantitative analysis reconstructs this local geometry and compares it to the preoperative imaging, thereby effecting rapid bone registration.A registration accuracy study using our novel method was conducted on 24 CT-scanned femur Sawbones®. We simulated exposures typically seen during knee/hip arthroplasty and common bone tumor resections. The registration accuracy of our technique was quantified by measuring the discrepancy of known points (i.e., pre-drilled holes) on the bone. RESULTS: Our technique demonstrated a registration accuracy of 0.44 ± 0.22 mm. This compared favorably with literature-reported values of 0.68 ± 0.14 mm (p-value = 0.001) for the paired-point technique13 and 0.86 ± 0.38 mm for the intraoperative CT based techniques 14 (not enough reported data to calculate p-value). CONCLUSION: We have developed a novel method of bone registration for computer and robotic-assisted surgery using 3D surface scanning technology that is rapid, compact, and radiation-free. We have demonstrated increased accuracy compared to existing methods (using historical controls).

Classification of Soft Tissue Sarcoma Specimens with Raman Spectroscopy as Smart Sensing Technology.

Intraoperative confirmation of negative resection margins is an essential component of soft tissue sarcoma surgery. Frozen section examination of samples from the resection bed after excision of sarcomas is the gold standard for intraoperative assessment of margin status. However, it takes time to complete histologic examination of these samples, and the technique does not provide real-time diagnosis in the operating room (OR), which delays completion of the operation. This paper presents a study and development of sensing technology using Raman spectroscopy that could be used for detection and classification of the tumor after resection with negative sarcoma margins in real time. We acquired Raman spectra from samples of sarcoma and surrounding benign muscle, fat, and dermis during surgery and developed (i) a quantitative method (QM) and (ii) a machine learning method (MLM) to assess the spectral patterns and determine if they could accurately identify these tissue types when compared to findings in adjacent H&E-stained frozen sections. High classification accuracy (>85%) was achieved with both methods, indicating that these four types of tissue can be identified using the analytical methodology. A hand-held Raman probe could be employed to further develop the methodology to obtain spectra in the OR to provide real-time in vivo capability for the assessment of sarcoma resection margin status.

Hip disarticulation with pedicled total leg fillet flap for recurrent fungating sarcoma.

Soft tissue sarcomas may be treated with limb-sparing procedures in the majority of cases; however, certain cases involving significant tumor spread and fungation may call for amputation. In the thigh, hip disarticulation typically involves a pedicled gluteus maximus flap or a pedicled anterior quadriceps flap. In this case report, we describe a rare situation in which the anterior flap, posterior flap, and adductor flap musculature were contaminated with tumor; therefore, a hip disarticulation was performed applying a pedicled total leg fillet flap for closure. Eighteen months after treatment, the patient continues to have no local recurrence of disease, a stable flap site, and ambulates with a walker. We present this amputation and closure method as a potentially effective modality in treating extensive oncologic disease of the proximal lower extremity.