Tumor-Induced Osteomalacia Localized and Excised After Pregnancy.

OBJECTIVE: Tumor-induced osteomalacia (TIO) is a rare osteomalacia characterized by paraneoplastic secretion of fibroblast growth factor 23. Concomitant occurrence of TIO during pregnancy is rarer still. Our objective was to report a young patient with debilitating fractures diagnosed with TIO who became pregnant and subsequently had her tumor localized by gallium-68 (Ga-68) DOTATATE positron emission tomography/magnetic resonance imaging (PET/MRI). CASE REPORT: A 28 year-old woman with a 2-year history of stress fractures was found to have the following: (1) alkaline phosphatase level, 220 (reference range, 30-95) U/L; (2) phosphorus level, 2.1 (2.5-5.0) mg/dL; (3) 1,25-dihydroxyvitamin D3 level, <8 (18-72) pg/mL; (4) 24-hour urine phosphorus level, 0.5 (0.3-1.3) g; and (5) fibroblast growth factor 23 levels, 1241 (reference range, <180) RU/mL. The patient became pregnant, and at term, a cesarean delivery was performed. Ga-68 DOTATATE PET/MRI showed a 9-mm intracortical mass in the right fibular head and right femoral and bilateral calcaneal stress fractures. The fibular lesion was resected; pathology showed a 1.5-cm lesion with positive fibroblast growth factor receptor 1 staining. DISCUSSION: This patient with TIO had an uneventful pregnancy and delivery. TIO is typically caused by benign mesenchymal tumors. Ga-68 DOTATATE PET/computed tomography has been used for localizing tumors causing TIO, yet MRI has superior contrast resolution over computed tomography. Therefore, it is not surprising that Ga-68 PET/MRI successfully localized this patient's tumor to the intracortical space of the fibular head and distinguished it from insufficiency fractures. CONCLUSION: To our knowledge, this is the first report of phosphate treatment in a pregnant patient with TIO and the first report of a tumor-inducing TIO being localized by Ga-68 DOTATATE PET/MRI.

Modern Management of High-risk Soft Tissue Sarcoma With Neoadjuvant Chemoradiation: A Single-center Experience.

OBJECTIVE: Neoadjuvant chemoradiation (NA-CRT), followed by resection of high-risk soft tissue sarcoma (STS), may offer good disease control and toxicity outcomes. We report on a single institution's modern NA-CRT experience. MATERIALS AND METHODS: Delay to surgical resection, resection margin status, extent of necrosis, tumor cell viability, presence of hyalinization, positron emission tomography (PET)/computed tomography data, and treatment toxicities were collected. Using the Kaplan-Meier survival analysis, 5-year overall survival, disease-free survival, distant metastasis-free survival, and local control (LC) were estimated. Clinicopathologic features and PET/computed tomography avidity changes were assessed for their potential predictive impact using the log-rank test. RESULTS: From 2011 to 2018, 37 consecutive cases of localized high-risk STS were identified. Twenty-nine patients underwent ifosfamide-based NA-CRT to a median dose of 50 Gy before en bloc resection. At a median follow-up of 40.3 months, estimated 5-year overall survival was 86.1%, disease-free survival 70.2%, distant metastasis-free survival 75.2%, and LC 86.7%. Following NA-CRT, a median reduction of 54.7% was observed in tumor PET avidity; once resected, median tumor necrosis of 60.0% with no viable tumor cells was detected in 13.8% of the cases. Posttreatment resection margins were negative in all patients, with 27.6% having a margin of ≤1 mm. Delays of over 6 weeks following the end of radiation treatment to surgical resection occurred in 20.7% cases and was suggestive of inferior LC (92.8% vs. 68.6%, P=0.025). CONCLUSIONS: This single-institution series of NA-CRT demonstrates favorable disease control. Delay in surgical resection was associated with inferior LC, a finding that deserves further evaluation in a larger cohort. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

Excision of Prominent Bony Mass due to Skeletal Fluorosis: A Case Report.

CASE: A 72-year-old man presented for evaluation of bony prominences over extremities. Radiographic imaging demonstrated masses of varying sizes extending from the cortical surfaces without medullary continuity. The patient had a history of Freon inhalation abuse and was diagnosed with skeletal fluorosis due to elevated serum fluoride levels. He underwent an uncomplicated excision of a left fibular mass that was threatening skin breakdown. CONCLUSIONS: This is the first reported surgical case of skeletal fluorosis demonstrating continued enlargement of bony prominences throughout the body. Skeletal fluorosis not only causes diffuse mineralization but may also lead to protruding lesions throughout the body.

Diagnosis and management of primary malignant tumors in the upper extremity.

Bone and soft tissue sarcomas of the upper extremity are relatively uncommon. In many cases, they are discovered incidentally during evaluation of traumatic injuries or common ailments such as rotator cuff tendonitis or tennis elbow. Thus, it is important for all orthopedic surgeons to understand the differential diagnosis, workup, and treatment for upper extremity lesions. An appreciation of the clinical and radiographic features of primary malignant lesions aids in identifying patients that need referral to an orthopedic oncologist and a multidisciplinary team.

Flap Reconstruction of Sarcoma Defects in the Setting of Neoadjuvant and Adjuvant Radiation.

BACKGROUND: Limb-sparing treatment of extremity soft tissue sarcomas requires wide resections and radiation therapy. The resulting complex composite defects necessitate reconstructions using either muscle or fasciocutaneous flaps, often in irradiated wound beds. METHODS: A retrospective chart review was performed of all limb-sparing soft tissue sarcoma resections requiring immediate flap reconstruction from 2012 through 2016. RESULTS: Forty-four patients with 51 flaps were identified: 25 fasciocutaneous and 26 muscle-based flaps. Mean defect size, radiation treatment, and follow-up length were similar between groups. More often, muscle-based flaps were performed in younger patients and in the lower extremity. Seventeen flaps were exposed to neoadjuvant radiation, 12 to adjuvant radiation, 5 to both, and 17 to no radiation therapy. Regardless of radiation treatment, complication rates were comparable, with 28% in fasciocutaneous and 31% in muscle-based groups (p < 0.775). Muscle-based flaps performed within 6 weeks of undergoing radiotherapy were less likely to result in complications than those performed after greater than 6 weeks (p < 0.048). At time of follow-up, Musculoskeletal Tumor Society scores for fasciocutaneous and muscle-based reconstructions, with or without radiation, showed no significant differences between groups (mean [SD]: 91% [8%] vs. 89% [13%]). CONCLUSION: The similar complication rates and functional outcomes in this study support the safety and efficacy of both fasciocutaneous flaps and muscle-based flaps in reconstructing limb-sparing sarcoma resection defects, with or without radiotherapy.

Posterior "Open Book" approach for type 1 internal hemipelvectomy.

INTRODUCTION: Internal limb-sparing hemipelvectomy is currently the preferred surgical option for resection of pelvic tumours. Obtaining an acceptable functional outcome through the standard ilioinguinal or iliofemoral incisions, only compounds the already challenging dissection, resection and reconstruction of these extensive en-bloc extirpative oncologic operations. SURGICAL TECHNIQUE: We describe a novel surgical approach to the lateral pelvis that minimises injury to the gluteal muscles, spares the gluteal vessels and provides a broad yet shallow operative field conducive to microsurgery with sizeable vasculature in close proximity to facilitate any microsurgical reconstruction. Access to the ilium and sacrum for Enneking Type 1 and Type 4 resections respectively is obtained by reflecting a skin and soft tissue flap anteriorly while the gluteal muscles are reflected posteriorly and inferiorly. This technique minimises the amount of tissue reflected in any particular direction, therefore, providing a broad and shallow operative field which benefits the orthopaedic oncologic surgeon and the plastic reconstructive surgeon. CONCLUSION: The "posterior open-book" approach offers a promising alternative to the standard ilioinguinal or iliofemoral incisions for internal limb-sparing Type 1 hemipelvectomies while also optimising the exposure for subsequent reconstructive procedures.

Evaluation and Management of Pediatric Bone Lesions.

Bone abnormalities on pediatric radiographs are not uncommon findings for both the general orthopedist as well as the specialist. Although the majority of lesions encountered are benign, the treating physician should also be aware of more concerning diagnoses. General orthopedists and pediatric orthopedists should exhibit a basic level of comfort with working up and diagnosing these benign lesions. When evaluating the pediatric patient with a bone lesion it is crucial to keep in mind important aspects of the clinical history, physical exam, and radiographic findings. Here we provide a review of important findings for the orthopedic surgeon in each of these areas. Many times diagnoses can be made with these alone. With better understanding of clinical and radiographic features of these lesions the orthopedist should be comfortable knowing which lesions he or she can reasonably treat and which should be referred to an oncologic specialist.

Orthopedic Oncology Caseload Among Orthopedic Surgery Residents.

Despite educational focus regarding orthopedic oncology during residency, assessment of resident orthopedic oncology caseload has not been performed. The purpose of this study was to evaluate orthopedic oncology caseload trends and variation among residents. The Accreditation Council for Graduate Medical Education case log reports for orthopedic surgery residents were reviewed for graduating years 2007 to 2013. Trends in orthopedic oncology cases and variation in the median number of cases performed by residents in the 90th, 50th, and 10th percentiles of caseload were evaluated. The proportion of orthopedic oncology caseload among all cases performed by residents increased significantly (P = 0.005) from 2007 to 2013. Likewise, the mean number of adult (P = 0.002), pediatric (P = 0.003), and total orthopedic oncology cases increased significantly (P = 0.002). On average, residents in the 90th, 50th, and 10th percentiles performed 83, 28, and 3 cases, respectively. The current study demonstrates a significant increase in adult, pediatric, and total orthopedic oncology caseload. There is also evidence of substantial caseload variation among residents. Caseload variation may influence the education and technical proficiency of orthopedic residents.

Limb-sparing sarcoma reconstruction with functional composite thigh flaps.

INTRODUCTION: Innervated muscle transfer can improve functional outcomes after extensive limb-sparing sarcoma resections. We report our experience using composite thigh flaps for functional reconstruction of large oncologic extremity defects. PATIENTS AND METHODS: Between 2011 and 2014, four limb-sparing oncologic resections (3 lower extremities, 1 upper extremity) underwent immediate functional reconstruction with composite thigh free flaps in three males and one female. The age of the patients ranged from 36 to 73 years. There were 3 soft-tissue sarcomas and one giant cell tumor, all required resection of entire muscle compartments. Flap components included fasciocutaneous tissue with sensory nerve, plicated iliotibial band (ITB), and variable amounts of motorized vastus lateralis (VL). RESULTS: All flaps survived without complications. All patients showed VL motor innervation by six months. Follow-up ranged from 20 to 36 months. Motor strength ranged from 2 to 5 out of 5, active range of motion was 25-92% of normal, and Musculoskeletal Tumor Society (MSTS) Scores were between 22 and 29 out of 30. CONCLUSIONS: Limb-sparing techniques for upper and lower extremity sarcomas continue to evolve. Our experience has validated the composite thigh free flap as an excellent option for one-stage functional reconstruction of large limb defects.

Bone Grafts, Substitutes, and Augments in Benign Orthopaedic Conditions Current Concepts.

Musculoskeletal tumors are relatively rare diagnoses made by orthopaedic surgeons. While approximately 2,500 primary bone sarcomas are diagnosed annually in the USA, the number of benign orthopaedic tumors encountered annually is far more difficult to quantify. Some studies have documented between 3% and 10% of the general population having benign bony lesions. Many of these conditions can be simply observed, while others will require surgical intervention. Surgical treatments for benign conditions range from a one-step curettage to extensive resection and reconstruction. With treatment of larger lesions, significant bony defects may need to be addressed surgically. Treatment options have evolved over time with the use of various bone graft and bone void fillers, including methyl methacrylate cement, autograft, allograft bone chips, struts and osteoarticular segments, synthetic bone graft substitutes, and metal augments. This review provides an overview of the present status of bone graft, substitutes, and augment options for the orthopaedic surgeon treating benign musculoskeletal conditions.

Synovial Hemangioma Presenting as a Painful Locked Knee A Case Report.

A 39-year-old man presented to orthopaedic care with a painful, fully locked knee. Workup revealed free intraarticular nodules, which were subsequently arthroscopically removed and identified to be synovial hemangioma. To the investigators' knowledge, this is the second reported case of synovial hemangioma presenting as a painful, definitively locked knee. Synovial hemangioma should be considered in the differential diagnosis of knee pain, particularly after more common diagnoses have been ruled out. Efficient and appropriate diagnosis and treatment may result in favorable patient outcomes and avoid long-term disability and dysfunction.

Management of Lower Extremity and Pelvic Tumors Using Computer Assisted Modeling (CAM) A Case Series.

Computer assisted modeling (CAM) has become an important tool in surgical oncology and reconstructive surgery. The preservation of the limb is an important consideration when approaching the treatment of lower extremity and pelvic tumors. The use of cutting guides allows for optimal conservation of disease-free bone and maintenance of function. We present a small case series that illustrates the use of CAM in patients with lower extremity and pelvic bone tumors.

Is Prophylactic Intervention More Cost-effective Than the Treatment of Pathologic Fractures in Metastatic Bone Disease?

BACKGROUND: Metastatic bone disease is a substantial burden to patients and the healthcare system as a whole. Metastatic disease can be painful, is associated with decreased survival, and is emotionally traumatic to patients when they discover their disease has progressed. In the United States, more than 250,000 patients have metastatic bone disease, with an estimated annual cost of USD 12 billion. Prior studies suggest that patients who receive prophylactic fixation for impending pathologic fractures, compared with those treated for realized pathologic fractures, have decreased pain levels, faster postoperative rehabilitation, and less in-hospital morbidity. However, to our knowledge, the relative economic utility of these treatment options has not been examined. QUESTIONS/PURPOSES: We asked: (1) Is there a cost difference between a cohort of patients treated surgically for pathologic fractures compared with a cohort of patients treated prophylactically for impending pathologic lesions? (2) Do these cohorts differ in other ways regarding their utilization of healthcare resources? METHODS: We performed a retrospective study of 40 patients treated our institution. Between 2011 and 2014, we treated 46 patients surgically for metastatic lesions of long bones. Of those, 19 (48%) presented with pathologic fractures; the other 21 patients (53%) underwent surgery for impending fractures. Risk of impending fracture was determined by one surgeon based on appearance of the lesion, subjective symptoms of the patient, cortical involvement, and location of the lesion. At 1 year postoperative, four patients in each group had died. Six patients (13%) were treated for metastatic disease but were excluded from the retrospective data because of a change in medical record system and inability to obtain financial records. Variables of interest included total and direct costs per episode of care, days of hospitalization, discharge disposition, 1-year postoperative mortality, and descriptive demographic data. All costs were expressed as a cost ratio between the two cohorts, and total differences between the groups, as required per medical center regulations. All data were collected by one author and the medical center's financial office. RESULTS: Mean total cost was higher in patients with pathologic fractures (cost unit [CU], 642 ± 519) than those treated prophylactically without fractures (CU, 370 ± 171; mean difference, 272; 95% CI, 19-525; p = 0.036). In USD, this translates to a mean of nearly USD 21,000 less for prophylactic surgery. Mean direct cost was 41% higher (nearly USD 12,000) in patients with a pathologic fracture (CU, 382 ± 300 versus 227 ± 93; mean difference, 155; 95% CI, 9-300; p = 0.038). Mean length of stay was longer in patients with pathologic fractures compared with the group treated prophylactically (8 ± 6 versus 4 ± 3 days; mean difference, 4; 95% CI, 1-7; p = 0.01). CONCLUSIONS: These findings show economic and clinical value of prophylactic stabilization of metastatic lesions when performed for patients with painful lesions compromising the structural integrity of long bones. Patients sustaining a pathologic fracture may represent a more severe, sicker demographic than patients treated for impending pathologic lesions. LEVEL OF EVIDENCE: Level IV, economic and decision analysis.

Vascular control for a forequarter amputation of a massive fungating humeral osteosarcoma.

Forequarter amputation is a radical operation performed for treatment of malignant neoplasms of the shoulder girdle not amenable to limb salvage. Traditional approaches involve bone and soft tissue resection, followed by ligation of the axillary vessels. We describe a technique to minimize blood loss whereby control of the subclavian vessels is performed before amputation of a large tumor associated with extensive venous congestion. A 34-year-old man presented with proximal humeral osteosarcoma. Surgery involved claviculectomy to facilitate vascular control of the subclavian vessels, followed by guillotine amputation at the proximal upper arm level and completion of the amputation as conventionally described.

Identification of Risk Factors for Acute Surgical Site Infections in Musculoskeletal Tumor Patients Using CDC/NHSN Criteria.

BACKGROUND: Acute surgical site infections (SSI) are well-recognized postoperative complications, representing a significant source of patient morbidity and cost to the healthcare system. This study is among the first to use standardized criteria for the diagnosis of acute SSI in orthopaedic oncology. METHODS: The medical records of 165 patients were retrospectively reviewed for the occurrence of superficial or deep SSI as defined by the Center for Disease Control's National Healthcare Safety Network (CDC/NHSN) criteria. Patient, disease, and procedure-specific variables were evaluated as potential risk factors for infection. RESULTS: The overall rate of acute SSI was 10.3%. Univariate analysis demonstrated the significance of malignant pathology (p < 0.001), ASA classification (p = 0.009), operative duration (p < 0.001), intraoperative RBC transfusions (p = 0.03), the performance of an amputation (p = 0.016), and race (p = 0.008) on the incidence of SSI. Prolonged operative duration (p = 0.014) and race (p = 0.005) were found to be independent risk factors with odds ratios of 1.89 (95%, CI: 1.14 to 3.14) and 0.047 (95%, CI: 0.006 to 0.387), respectively. CONCLUSIONS: By using the CDC/NHSN guidelines for the diagnosis of acute SSI, we identified prolonged operative time and non-Caucasian race as independent risk factors for infection in musculoskeletal tumor patients.

Maffucci Syndrome. An Interesting Case and a Review of the Literature.

Maffucci syndrome, a rare sporadic form of enchondromatosis, is characterized by hemangiomas and multiple enchondromas, benign cartilaginous tumors that arise near growth plates. Previous studies demonstrate that individuals diagnosed with Maffucci syndrome have approximately 100% lifetime risk of malignant transformation. Identification of Maffucci syndrome by surgical excision and pathological diagnosis can be life-saving due to its high malignant potential relative to other subtypes of enchondromatosis such as Ollier's disease. We report a case of a 58-year-old man with enchondromatosis who experienced malignant transformation of the enchondroma in his distal femur into a chondrosarcoma. He underwent a right distal femoral replacement without complications. Two years later, new masses were identified in his left hand and excised following progressive growth, pain, and functional limitation. Pathology confirmed these to be hemangiomas, and he was diagnosed with Maffucci syndrome. At last follow-up, patient reported additional nodular tumor growths occurring unilaterally on the left side. For patients with Maffucci syndrome, continual follow-up and careful surveillance of these masses is crucial as these lesions can cause fractures, deformities, pain, and undergo malignant transformation. Our report reviews the literature and outlines the treatment and management plans for patients with this rare and potentially dangerous disorder.

Minimizing Blood Loss in Orthopaedic Surgery The Role of Antifibrinolytics.

Many common orthopaedic procedures are associated with a large volume of blood loss. Antifibrinolytics have a well-elucidated mechanism of action and a long history of clinical use. By inhibiting the enzymatic breakdown of fibrin, there has been a demonstrated ability to decrease total blood loss and reduce postoperative transfusion requirements. Despite substantial use in cardiac surgery, antifibrinolytic use in orthopaedic surgery has been limited due to concerns over the potential for venous thromboembolism. A growing body of evidence demonstrates the medications' efficacy and safety in arthroplasty and spine surgery. Due to the risks associated with allogenic blood transfusions and the limitations of alternative blood conservation strategies, antifibrinolytics are a reasonable strategy in blood conservation programs for patients undergoing major orthopaedic procedures.

Large solitary glomus tumor of the wrist involving the radial artery.

Glomus tumors are neuromyoarterial glomus body neoplasms that most commonly occur in the subungual areas of the hand. These tumors typically present as painful solitary lesions with localized tenderness. Extradigital glomus tumors are rare and difficult to diagnose because of their varying symptoms and presentation. Misdiagnosis can result in significant chronic pain, disuse syndromes, and disability over prolonged periods. Timely diagnosis and surgical resection are generally curative with complete resolution of symptoms. Surgeons should be aware of this rare condition and consider it in the differential diagnosis when treating a painful soft-tissue mass of the wrist. We report a case of a large solitary glomus tumor that occurred on the volar-radial aspect of the wrist and involved the radial artery. The patient was successfully treated with complete surgical excision of the tumor. There were no symptoms or recurrence at 3-year follow-up. In the literature, this is the first case of large atypical wrist glomus tumor involving the radial artery to be reported with corresponding magnetic resonance imaging, intraoperative, histology, and immunohistochemistry findings.

Feasibility and efficacy of local radiotherapy with concurrent novel agents in patients with multiple myeloma.

INTRODUCTION: This study evaluated the safety and efficacy of radiotherapy (RT) with concurrent novel agents (NAs), cytotoxic therapy (CTx), or both in the management of osteolytic bone lesions in multiple myeloma (MM). PATIENTS AND METHODS: A total of 39 patients with MM received RT to 64 different bone sites during the 2007-2012 period, with a dose of 8 to 37.5 Gy (mean, 26.8 Gy) delivered in 1 to 15 fractions (median, 10 fractions). Of these patients, 21 also received concurrent NAs or CTx. Pain response, M protein and κ light chain response, and adverse events were evaluated. RESULTS: RT was completed in 35 of 39 patients (89.7%) in this study. Pain relief was observed in 30 of 31 patients (96.7%). Hematologic toxicity (grade 3 or 4 by the Radiation Therapy Oncology Group system) was seen in 43.2% of treated patients, and NA therapy was stopped in 2 patients owing to grade 4 toxicity. RT adverse effects resolved at 4 to 6 weeks posttreatment. Changes in pre- and posttreatment levels of M protein trended toward significance in patients treated with RT + systemic therapy (ST) versus. RT alone (ΔM ProteinRT+ST = 5.6 g/L; ΔM ProteinRT = 0 g/L; P = .089). CONCLUSION: Treating MM with RT concurrently with CTx including NAs was safe and well tolerated in the majority of patients (14 of 16 [87.5%] for those taking NAs and 19 of 21 [90.5%] for all patients). Excellent clinical pain response (> 95%) was also seen in patients regardless if they were treated with RT + ST or RT alone.