Treatment of histiocytic neoplasms with intralesional steroids: A case series report.

INTRODUCTION AND IMPORTANCE: Rosai-Dorfman disease was first described in the 1960s and is characterized by hyperproliferation of histiocytes, which presents as painless lymphadenopathy with fever, night sweats, and weight loss. This disease often affects young adults and children of African descent. Different types of RDD exist, including familial, classical (nodal), extranodal, neoplasia-associated, and immune-associated. While this disease can cause significant pain, there is currently no consensus on treatment. CASE PRESENTATION: This case series outlines three cases of bony involvement of histiocytic neoplasms and our technique for intralesionoal steroid injections. Two cases were successfully treated with intralesional steroid injections and one patient was treated with oral steroids. CLINICAL DISCUSSION: Histiocytic neoplasms can cause significant pain for patients and there is no standardized treatment as of yet. An interesting finding on MRI was a characteristic peripheral edema we termed the "Halo Sign". Steroids are believed to exert their benefit in treating this disease by apoptosis and reducing tumoral swelling. CONCLUSION: This case series demonstrates the successful management of bony involvement of RDD with intralesional steroids similar to eosinophilic granuloma. While intralesional steroid injection has previously been described for other types of RDD, this is the first description of bony RDD treated with this technique to our knowledge.

MYC-Nonamplified Secondary Lymphatic-Type Angiosarcoma With Prominent Lymphocytic Infiltrate Following Radiation Therapy for Myxoid Liposarcoma.

ABSTRACT: Cutaneous angiosarcomas (AS) are uncommon and morphologically heterogeneous. Recently, a distinctive lymphatic-type AS with prominent lymphocytic infiltrate has been observed. Although conventional AS typically bear poor prognosis, lymphatic-type AS with prominent lymphocytic infiltrate and pseudolymphomatous AS show prolonged survival with rare extracutaneous spread. We describe a unique case of AS in a 55-year-old woman who received surgical resection and radiation therapy for her prior myxoid liposarcoma. She developed a suspected recurrence 15 years later. Microscopically, the lesion showed an infiltration of the reticular dermis by irregular interanastamosing vascular spaces lined by atypical endothelial cells with nuclear "hobnailing" and hyperchromasia. A prominent intratumoral and peritumoral lymphocytic infiltrate obscuring the tumor cells was also present. The tumor cells were diffusely positive for endothelial cell markers, including D2-40. Notably, there was no evidence of MYC gene amplification by FISH. Additional NGS-based molecular analysis demonstrated no significant genetic mutations. The patient is alive with a history of two local recurrences, but no evidence of metastasis. We present this case to raise awareness of MYC-nonamplified secondary lymphatic-type AS with prominent lymphocytic infiltrate (pseudolymphomatous AS) and to discuss its differential diagnosis.

Short-term Clinical Outcomes of Unexpected Culture-positive Cutibacterium acnes (Formerly Propionibacterium acnes) in Open Orthopaedic Surgery.

INTRODUCTION: The clinical significance and treatment recommendations for an unexpected positive Cutibacterium acnes (C acnes) culture remain unclear. The purpose of our study was to evaluate the clinical effect of a C acnes positive culture in patients undergoing open orthopaedic surgery. METHODS: Patients with a minimum of one positive C acnes intraoperative culture were retrospectively reviewed over a 7-year period. True C acnes infection was defined as culture isolation from ≥1 specimens in the presence of clinical or laboratory indicators of infection. RESULTS: Forty-eight patients had a positive intraoperative C acnes culture. 4.2% had a C acnes monoinfection, and 12.5% of the patients had a coinfection. The remainder was classified as indeterminate. Significant differences were identified between the indeterminate and true C acnes infection groups, specifically in patients with surgery history at the surgical site (P = 0.04), additional antibiotic therapy before surgery (P < 0 .001), and postoperative clinical signs of infection (P < 0 .001). DISCUSSION: Suspicion for true C acnes infection should be raised in patients with surgery site history, antibiotic therapy before surgery, and clinical infectious signs. The indeterminate unexpected positive culture patients had a low risk of developing a true clinical infection that required antibiotic therapy.

A Rare Case of Diffuse-type Tenosynovial Giant Cell Tumor in a Teenager With Noonan Syndrome.

Noonan syndrome is a common autosomal dominant disorder associated with an increased risk of malignancy. We report a 16-year-old female with Noonan syndrome (KRAS gene variant, Q22R) and diffuse-type tenosynovial giant cell tumor, a proliferative disorder that has been rarely reported in this population. These tumors may represent a complication of the dysregulated RAS/MAPK signaling pathway that underlies Noonan syndrome. They lack typical clinical features, causing misdiagnosis and delays in management, which could lead to osseous invasion requiring more complicated surgical procedures. Increased awareness of this association will improve the clinical outcomes of patients with Noonan syndrome who develop diffuse-type tenosynovial giant cell tumors.

Phosphaturic Mesenchymal Tumor-induced Osteomalacia Masquerading as an Orthopedic Polytrauma Patient: A Case Report and Review of the Literature.

Introduction: Phosphaturic mesenchymal tumor (PMT) is a rare benign tumor (500 cases to date) that can present in combination with a paraneoplastic syndrome called tumor-induced osteomalacia (TIO). To the best of our knowledge, it is the first case to date that presented as an orthopedic trauma patient. Case Report: This is a case of a 61-year-old male who initially presented as a polytrauma patient, but further investigation revealed a PMT causing TIO. This report describes his initial diagnosis and management from 2015 to 2021. Conclusion: TIO resultant of PMT may lead to severe bone pain, impending fractures, and delayed or misdiagnosis. This case demonstrates the importance of careful diagnosis and a team-based approach to managing PMT and its sequelae.

Pseudomyogenic Hemangioendothelioma: A Case of a Solitary Lesion With a Very Indolent Clinical Course.

Pseudomyogenic hemangioendothelioma (PMH), also known as epithelioid sarcoma-like hemangioendothelioma, is a rare epithelioid vascular neoplasm predominantly affecting young adult males at an average age of approximately 30 years. This tumor is rare; therefore, detailed information regarding this tumor is still lacking. Here, we report a case of a man in his 20s presenting with left foot pain for about one year. Imaging showed a 2-cm ovoid, cortically based lesion with a lytic defect of the cortex at the fifth metatarsal proximal shaft. Histologically, the lesion presented as an infiltrating proliferation of distinctly myoid-appearing spindled cells with eosinophilic cytoplasm and mildly atypical vesicular nuclei. Scant mitoses were identified with no areas of necrosis. Tumor cells exhibited strong, diffuse cytokeratin expression as well as CD31 and ERG. CD34 was positive in a few tumor cells, and integrase interactor 1 (INI1) retained nuclear expression. No reactivity for S100, desmin, smooth muscle actin (SMA), epithelial membrane antigen (EMA), and CD1a was present. Over half of the patients with PMH develop multifocal lesions, often involving several tissue planes; however, distant metastasis is very infrequent. This patient underwent curettage and internal fixation of the left fifth metatarsal and had no evidence of recurrence or distant metastasis after seven years of follow-up. Our case contributes to the growing knowledge of PMH and sheds light on the prognosis of these lesions.

Hypofractionated radiation therapy and wound healing after massive sarcoma resection: Case report and review of the literature.

INTRODUCTION: Large high-grade sarcomas are commonly managed with five weeks of pre-operative radiation with chemotherapy followed by surgical resection. Wound complications occur in about one out of three patients with this regimen. Hypofractionated radiation therapy (HFRT) is a developing pre-operative approach that delivers radiation over a shorter duration of 5-10 treatments. PRESENTATION OF CASE: Two patients underwent HFRT with neoadjuvant chemotherapy followed by tumor resection. The first patient had high-grade de-differentiated liposarcoma, and the second patient a high-grade myxofibrosarcoma. Neither patient developed post-operative wound complications despite the massive tumor size. DISCUSSION: Less is understood regarding rates and risk factors associated with wound complications using this shortened radiation approach. With attention to surgical detail, and advancing radiation delivery technologies, rates of complications can be minimized. CONCLUSION: We discuss our experience with a neoadjuvant hypofractionated chemoradiation protocol in two patients with large volume sarcomas resected from the chest wall and the thigh who did not develop acute wound complications. Further evaluation of this shortened regimen is warranted.

Distal fibular excision: A review of the literature and presentation of our reconstruction technique case series.

Distal fibula resection is a procedure that has been described as early as 1938 for the treatment of neoplastic lesions. There have been several techniques described for the reconstruction of the remaining tibiotalar joint to prevent deformity and maintain as much function as possible. While these reconstruction techniques provide an option for limb salvage with the removal of disease, patients are faced with chronic pain, loss of function, valgus instability, need for long term orthosis, early arthritis, or significant morbidity related to proximal dissection and disruption of native knee anatomy. We present a case series that is compliant with PROCESS1 criteria to demonstrate the effective treatment with distal fibular excision and a reconstruction technique inspired by the original Tommy John procedure of the elbow. This procedure has allowed these two patients long-term stability of the ankle, maintenance of full function, and high levels of function. The first case is a 23-year-old female with high-grade osteosarcoma and the second patient is a 19-year-old female with Ewing sarcoma. Details of the procedure as well as clinical and radiographic follow up of these two patients will be described.

Squamous Cell Carcinoma Arising from a Morel-Lavallée Lesion: A Case Report.

CASE: A 72-year-old man presented 20 years after a Morel-Lavallée (ML) lesion with pain and drainage. Biopsies of the lesion and lymph nodes were positive for squamous cell carcinoma (SCC). There was no cutaneous involvement or distant metastasis. After chemotherapy and radiation, he underwent resection of the lesion and lymph nodes with flap closure. Two months postoperatively, he unfortunately developed malignant pleural effusions, hypercalcemia, and kidney injury and was eventually transferred to hospice care and died. CONCLUSION: This is the first report of SCC arising from a ML lesion. Chronic ML lesions should be treated aggressively and monitored for transformation into malignancy, even without cutaneous involvement.

Correction to: Early postoperative compilations of bone filling in curettage defects.

In the original publication of this article [1], there was a mistake in Figure 2. Figure 2a and Fig 2c should be swapped. The revised Figure 2 is shown below.

Early postoperative compilations of bone filling in curettage defects.

BACKGROUND: Curettage is widely used in orthopedic oncology; the defect created frequently requires filling for mechanical and functional stability for the bones and adjacent joint. Allograft, bone graft substitute, and polymethyl methacrylate (PMMA) are the most common substances used each with their benefits and drawbacks. The aim of the study is to show that good functional result can be achieved with curettage and bone filler, regardless of type. METHODS: A series of 267 cases were reviewed between 1994 and 2015 who received curettage treatment and placement of a bone filler. Endpoints included fracture, infection, cellulitis, pulmonary embolism, and paresthesia. Complication rates at our single institution were compared against literature values for three study cohorts: allograft, bone graft substitute, and PMMA bone fillers. Friedman test, Wilcoxon test, and Z-score for two populations were used to compare our subset against literature values and between different bone filling types. RESULTS: Our cases included 18 autografts, 74 allografts, 121 bone graft substitute, and 54 PMMA of which the bulk of complications occurred. Our overall complication rate was 3.37%. Allograft has a complication rate of 1.35%, bone graft substitute of 4.13%, and PMMA of 5.56%. Other techniques did not yield any complications. Combination filling techniques PMMA + allograft and PMMA + bone graft substitute had sample sizes too small for statistical comparison. Statistical comparison yielded no significant difference between complications in any of the filling groups (P = 0.411). CONCLUSIONS: Some has even argued that bone defects following curettage do not require bone filling for good outcome. However, many structural or biologic benefits that aid in earlier return to functionality can be conferred by filling large bone defects. There was no significant difference in postoperative complication rates between allograft, bone graft substitute, and PMMA when compared at our institution and with literature values. Nevertheless, one complication with a large defect filled with allograft, requiring a subsequent reconstruction using vascularized fibular graft. Taking everything into account, we see bone graft substitute as a suitable alternative to other bone filling modalities.

Inflammatory pseudotumor after ceramic-on-ceramic total hip arthroplasty.

We present a unique case of a symptomatic adverse local tissue reaction in a patient with a ceramic-on-ceramic total hip bearing surface. To our knowledge, this pathological finding has not yet been described in a ceramic-on-ceramic articulation without a cobalt-chromium alloy trunnion or modular neck component as a source of metal wear. We conclude that despite its mechanical mostly benign wear characteristics, ceramic wear debris is not entirely inert and may lead to the development of adverse local tissue reaction.

Postoperative complications with cryotherapy in bone tumors.

The technique of cryosurgery has been used to control local recurrence in a variety of benign and malignant bone tumors. Early studies revealed significant complication rates (25%) that included fracture, infection, and soft tissue injury. Our method of cryosurgery has yielded excellent tumor control with improved complication rates. The objective of this study is to determine the characteristics of postoperative complications after pouring liquid nitrogen into curettaged bone defects, and to review our current indications and surgical technique in bone tumor management. We reviewed charts in over 200 patients who received cryoablation for bone tumors from 1994 to 2015. Imaging studies were evaluated in all patients diagnosed with a complication. All patients receiving cryotherapy had soft tissue management intraoperatively that included warm saline directed to the structures. Liquid nitrogen was poured into the bone defect and in some cases, additional spraying with a cryogun into the defect was performed. The majority of cryotherapy was used in cases of active or aggressive benign tumors. Our low complication rate of 2.34% included 1 post-operative fracture, 3 infection, and 1 paraesthesia. Bone graft or cementation was used in the majority of patients, all of which fully incorporated. Cryoablation is an excellent from of adjuvant therapy for active and aggressive benign tumors and may be used in malignant tumors as well. Soft tissue protection is critical to avoid skin necrosis and wound breakdown. We recommend the use of cryotherapy in active and aggressive bone tumors as an adjuvant treatment prior to bone grafting or cementation.

Image-guided resection of aggressive sacral tumors.

OBJECTIVE The aim of this study was to identify and discuss operative nuances utilizing image guidance in the surgical management of aggressive sacral tumors. METHODS The authors report on their single-institution, multi-surgeon, retrospective case series involving patients with pathology-proven aggressive sacral tumors treated between 2009 and 2016. They also reviewed the literature to identify articles related to aggressive sacral tumors, their diagnosis, and their surgical treatment and discuss the results together with their own experience. Information, including background, imaging, treatment, and surgical pearls, is organized by tumor type. RESULTS Review of the institutional records identified 6 patients with sacral tumors who underwent surgery between 2009 and 2016. All 6 patients were treated with image-guided surgery using cone-beam CT technology (O-arm). The surgical technique used is described in detail, and 2 illustrative cases are presented. From the literature, the authors compiled information about chordomas, chondrosarcomas, giant cell tumors, and osteosarcomas and organized it by tumor type, providing a detailed discussion of background, imaging, and treatment as well as surgical pearls for each tumor type. CONCLUSIONS Aggressive sacral tumors can be an extremely difficult challenge for both the patient and the treating physician. The selected surgical intervention varies depending on the type of tumor, size, and location. Surgery can have profound risks including neural compression, lumbopelvic instability, and suboptimal oncological resection. Focusing on the operative nuances for each type can help prevent many of these complications. Anecdotal evidence is provided that utilization of image-guided surgery to aid in tumor resection at our institution has helped reduce blood loss and the local recurrence rate while preserving function in both malignant and aggressive benign tumors affecting the sacrum.

Resection of spinal column tumors utilizing image-guided navigation: a multicenter analysis.

OBJECTIVE The use of intraoperative stereotactic navigation has become more available in spine surgery. The authors undertook this study to assess the utility of intraoperative CT navigation in the localization of spinal lesions and as an intraoperative tool to guide resection in patients with spinal lesions. METHODS This was a retrospective multicenter study including 50 patients from 2 different institutions who underwent biopsy and/or resection of spinal column tumors using image-guided navigation. Of the 50 cases reviewed, 4 illustrative cases are presented. In addition, the authors provide a description of surgical technique with image guidance. RESULTS The patient group included 27 male patients and 23 female patients. Their average age was 61 ± 17 years (range 14-87 years). The average operative time (incision to closure) was 311 ± 188 minutes (range 62-865 minutes). The average intraoperative blood loss was 882 ± 1194 ml (range 5-7000 ml). The average length of hospitalization was 10 ± 8.9 days (range 1-36 days). The postoperative complications included 2 deaths (4.0%) and 4 radiculopathies (8%) secondary to tumor burden. CONCLUSIONS O-arm 3D imaging with stereotactic navigation may be used to localize lesions intraoperatively with real-time dynamic feedback of tumor resection. Stereotactic guidance may augment resection or biopsy of primary and metastatic spinal tumors. It offers reduced radiation exposure to operating room personnel and the ability to use minimally invasive approaches that limit tissue injury. In addition, acquisition of intraoperative CT scans with real-time tracking allows for precise targeting of spinal lesions with minimal dissection.

Proximal femoral reconstruction with a constrained acetabulum in oncologic patients.

Metallic endoprostheses are used for oncological reconstruction around the proximal femur and hip joint. Common modes of failure with hemiarthroplasty or standard hip arthroplasty after proximal femoral replacement include dislocation, late hip pain, and infection. The authors reviewed hospital records to identify patients undergoing constrained tripolar hip arthroplasty for oncological reasons between 2002 and 2012. Inclusion criterion was at least 12-cm proximal femoral resection, including patients with total femur reconstruction. A total of 33 patients were reviewed. Information regarding demographics, length of follow-up, treatment characteristics, and patient outcomes was extracted. Average follow-up for all patients was 912.33 days (30.4 months). Average follow-up was 1396.1 days for living patients and 428.6 days for deceased patients. Average estimated blood loss was 462.12 cc: an average of 1080 cc for patients undergoing total femoral resection and replacement and 315.8 cc for patients undergoing proximal femoral resection and replacement. Average operative time was 137.7 minutes: an average of 205 minutes for patients undergoing total femoral resection and replacement and 119.1 minutes for patients undergoing proximal femoral resection and replacement. Average Musculoskeletal Tumor Society score was 21.7. There were no dislocations in the cohort. A constrained tripolar device can be safely used for oncological proximal femoral reconstructions while minimizing the risk of dislocation. Positioning of the acetabular implant in neutral anatomic version in conjunction with a neutral-placed femoral component provides the greatest range of motion, reduction of liner impingement, and improved hip stability.

Giant cell tumor of bone in childhood: clinical aspects and novel therapeutic targets.

Giant cell tumor of bone (GCTB) is a rare primary bone tumor that primarily affects young adults, but can be seen in children. The primary modality of treatment is surgical resection; however, this is not always possible given the location and extent of the neoplasm. Recent developments in the understanding of the underlying molecular pathogenesis of disease have pointed to interactions between the stromal component producing receptor activator of nuclear factor-kappaB (RANK) and RANK-ligand (RANKL) causing the formation of osteoclast-like giant cells that drive bone destruction. The development of a monoclonal humanized antibody to RANKL, denosumab, has been shown to reduce skeletal-related events from osteoporosis and from bony metastases from solid tumors. Recent phase II clinical trials with denosumab in skeletally mature adolescents over age 12 years and adults with GCTB, have shown both safety and efficacy, leading to its accelerated US FDA approval on 13 June 2013. In children who are skeletally immature, safety and efficacy has not been established, and there has been only published anecdotal use.

Limb-sparing surgery for distal femoral and proximal tibial bone lesions: imaging findings with intraoperative correlation.

OBJECTIVE: Our purpose is to present normal and abnormal imaging findings associated with endoprosthetic reconstruction after limb-salvage surgery. CONCLUSION: Endoprosthetic reconstruction varies with the location and size of the tumor, implant designs, and complications. Radiologists need to be aware of associated imaging findings seen in postoperative infection, tumor recurrence, and hardware failure. With a thorough understanding of the normal postoperative radiographic findings after complex reconstructions, subsequent abnormalities are readily identified and timely diagnosis can be obtained.

Thermal ablation of osteoid osteoma: overview and step-by-step guide.

Osteoid osteoma is a small, benign but painful lesion with specific clinical and imaging characteristics. Computed tomography is the imaging modality of choice for visualization of the nidus and for treatment planning. Complete surgical excision of the nidus is curative, providing symptomatic relief, and is the traditionally preferred treatment. However, surgery has disadvantages, including the difficulty of locating the lesion intraoperatively, the need for prolonged hospitalization, and the possibility of postoperative complications ranging from an unsatisfactory cosmetic result to a fracture. Percutaneous radiofrequency (RF) ablation, which involves the use of thermal coagulation to induce necrosis in the lesion, is a minimally invasive alternative to surgical treatment of osteoid osteoma. With reported success rates approaching 90%, RF ablation should be considered among the primary options available for treating this condition.

Pigmented villonodular synovitis of the foot and ankle.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare disease of uncertain etiology usually affecting the synovium of weightbearing joints. METHODS: We retrospectively evaluated 11 patients who were diagnosed and treated for PVNS of the ankle and foot over a 13-year period with a minimum of 2-year followup. Four patients with ankle joint PVNS and one patient with PVNS of the fifth metatarsophalangeal joint were seen initially at our institution and were treated with surgery alone. Six patients with ankle joint PVNS were referred to our institution for recurrent PVNS lesions; two of these patients were treated with excision alone, and the other four patients had surgical excision followed by radiation therapy with dosages ranging from 3600-4000 cGy. RESULTS: No recurrence was noted at a mean followup of 9 years for primary lesions and 3.5 years for recurrent lesions. CONCLUSION: Based on these results, surgical excision of primary lesions and excision with postoperative radiation for recurrent lesions are recommended.