NCCN Guidelines Insights: Multiple Myeloma, Version 3.2018.

The NCCN Guidelines for Multiple Myeloma provide recommendations for diagnosis, evaluation, treatment, including supportive-care, and follow-up for patients with myeloma. These NCCN Guidelines Insights highlight the important updates/changes specific to the myeloma therapy options in the 2018 version of the NCCN Guidelines.

Surgical Approach to Bone Metastases.

PURPOSE OF REVIEW: The purpose of this review was to examine the recent changes in the surgical treatment of bone metastases and how the treatment paradigm has shifted with the improvement of adjuvant therapies. How surgery fits into the local and systemic treatment was reviewed for bone metastases in different areas. RECENT FINDINGS: The more common use of targeted chemotherapies and focused high-dose radiation have altered the treatment paradigm of bone metastases. Overall changes in the surgical treatment of bone metastases have been driven by an increased multidisciplinary approach to metastatic cancer and the awareness that one type of surgery does not work for all patients. The individual patient treatment goals dictate the surgical procedures used to achieve these goals. Advancements in adjuvant therapy-like radiation and more targeted chemotherapies have allowed for less invasive surgical approaches and therefore faster recoveries and reduced surgical morbidity for patients.

Multiple Myeloma, Version 3.2017, NCCN Clinical Practice Guidelines in Oncology.

Multiple myeloma (MM) is caused by the neoplastic proliferation of plasma cells. These neoplastic plasma cells proliferate and produce monoclonal immunoglobulin in the bone marrow causing skeletal damage, a hallmark of multiple myeloma. Other MM-related complications include hypercalcemia, renal insufficiency, anemia, and infections. The NCCN Multiple Myeloma Panel members have developed guidelines for the management of patients with various plasma cell dyscrasias, including solitary plasmacytoma, smoldering myeloma, multiple myeloma, systemic light chain amyloidosis, and Waldenström's macroglobulinemia. The recommendations specific to the diagnosis and treatment of patients with newly diagnosed MM are discussed in this article.

NCCN Guidelines Insights: Bone Cancer, Version 2.2017.

The NCCN Guidelines for Bone Cancer provide interdisciplinary recommendations for treating chordoma, chondrosarcoma, giant cell tumor of bone, Ewing sarcoma, and osteosarcoma. These NCCN Guidelines Insights summarize the NCCN Bone Cancer Panel's guideline recommendations for treating Ewing sarcoma. The data underlying these treatment recommendations are also discussed.

NCCN Guidelines Insights: Multiple Myeloma, Version 3.2016.

These NCCN Guidelines Insights highlight the important updates/changes specific to the 2016 version of the NCCN Clinical Practice Guidelines in Oncology for Multiple Myeloma. These changes include updated recommendations to the overall management of multiple myeloma from diagnosis and staging to new treatment options.

Multiple Myeloma, Version 2.2016: Clinical Practice Guidelines in Oncology.

Multiple myeloma (MM) is a malignant neoplasm of plasma cells that accumulate in bone marrow, leading to bone destruction and marrow failure. Recent statistics from the American Cancer Society indicate that the incidence of MM is increasing. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) included in this issue address management of patients with solitary plasmacytoma and newly diagnosed MM.

Sonography of cat scratch disease.

OBJECTIVES: To characterize the sonographic features of cat scratch disease and to identify features that allow differentiation from other causes of medial epitrochlear masses. METHODS: After Institutional Review Board approval was obtained, patients who underwent sonography for a medial epitrochlear mass or lymph node were identified via the radiology information system. Patients were divided into 2 groups: cat scratch disease and non-cat scratch disease, based on pathologic results and clinical information. Sonograms were retrospectively reviewed and characterized with respect to dimension, shape (round, oval, or lobular), symmetry, location (subcutaneous or intramuscular), multiplicity, echogenicity (anechoic, hypoechoic, isoechoic, hyperechoic, or mixed), hyperechoic hilum (present or absent), adjacent anechoic or hypoechoic area, hyperemia (present or absent), pattern of hyperemia if present (central, peripheral, or mixed), increased posterior through-transmission (present or absent), and shadowing (present or absent). Sonographic findings were compared between the patients with and without cat scratch disease. RESULTS: The final patient group consisted of 5 cases of cat scratch disease and 16 cases of other causes of medial epitrochlear masses. The 2 sonographic findings that were significantly different between the cat scratch disease and non-cat scratch disease cases included mass asymmetry (P = .0062) and the presence of a hyperechoic hilum (P = .0075). The other sonographic findings showed no significant differences between the groups. CONCLUSIONS: The sonographic finding of an epitrochlear mass due to cat scratch disease most commonly is that of a hypoechoic lobular or oval mass with central hyperemia and a possible adjacent fluid collection; however, the presence of asymmetry and a hyperechoic hilum differentiate cat scratch disease from other etiologies.

Nodular fasciitis: characteristic imaging features on sonography and magnetic resonance imaging.

OBJECTIVES: The purpose of this study was to evaluate the imaging features of nodular fasciitis on sonography and magnetic resonance imaging (MRI). METHODS: A retrospective search of the radiologic and pathologic databases over the past 10 years for the diagnosis of nodular fasciitis was performed. Sonographic and MRI features were described. Pathologic specimens were reviewed. RESULTS: Six pathologically confirmed cases of nodular fasciitis were found. The mean patient age was 19.5 years (range, 8-33 years); 3 patients were male and 3 were female. Four patients had sonography only; 1 patient had both sonography and MRI; and 1 patient had MRI only. Three masses were located in the subcutaneous tissue adjacent to fascia; 2 were at the subcutaneous/muscular border; and 1 was intramuscular; however, all were in contact with fascia and showed a fascial tail on sonography and MRI. On sonography and MRI, masses were oval with poorly defined lobulated borders, averaging 2.6 cm (range, 1.8-3.5 cm). On sonography, all masses were hypoechoic. On MRI, the masses were isointense to muscle on T1-weighted sequences, hyperintense to muscle on fluid-sensitive sequences, and enhanced avidly but heterogeneously. The masses were surrounded by fat. CONCLUSIONS: When a rapidly growing oval mass in contiguity with a fascial plane is recognized, the diagnosis of nodular fasciitis should be entertained. More importantly, the possibility of an inaccurate diagnosis by core biopsy exists, which may warrant gross resection.

Updates in the treatment of bone cancer.

Although extremely rare, primary bone cancers are often curable with proper treatment. Effective management of primary bone tumors hinges on the involvement of a multidisciplinary team of physicians with expertise in this area, both in the realms of diagnosis and treatment. In her presentation at the NCCN 18th Annual Conference, Dr. J. Sybil Biermann reviewed the changes to the 2013 NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for bone cancer, featuring the introduction of new sections on giant cell tumor of bone (GCTB) and chordoma. The benefits of denosumab for the benign GCTB and the unique challenges associated with the malignant chordoma are also explored.

NCCN Task Force Report: Bone Health In Cancer Care.

Bone health and maintenance of bone integrity are important components of comprehensive cancer care. Many patients with cancer are at risk for therapy-induced bone loss, with resultant osteoporotic fractures, or skeletal metastases, which may result in pathologic fractures, hypercalcemia, bone pain, and decline in motility and performance status. Effective screening and timely interventions are essential for reducing bone-related morbidity. Management of long-term bone health requires a broad knowledge base. A multidisciplinary health care team may be needed for optimal assessment and treatment of bone-related issues in patients with cancer. Since publication of the previous NCCN Task Force Report: Bone Health in Cancer Care in 2009, new data have emerged on bone health and treatment, prompting NCCN to convene this multidisciplinary task force to discuss the progress made in optimizing bone health in patients with cancer. In December 2012, the panel members provided didactic presentations on various topics, integrating expert judgment with a review of the key literature. This report summarizes issues surrounding bone health in cancer care presented and discussed during this NCCN Bone Health in Cancer Care Task Force meeting.

Multiple myeloma, version 1.2013.

These NCCN Guidelines Insights highlight the important updates/changes specific to the management of relapsed or progressive disease in the 2013 version of the NCCN Clinical Practice Guidelines in Oncology for Multiple Myeloma. These changes include the addition of new regimens as options for salvage therapy and strategies to mitigate the adverse effects and risks associated with newer regimens for the treatment of multiple myeloma.

Bone cancer.

Primary bone cancers are extremely rare neoplasms, accounting for fewer than 0.2% of all cancers. The evaluation and treatment of patients with bone cancers requires a multidisciplinary team of physicians, including musculoskeletal, medical, and radiation oncologists, and surgeons and radiologists with demonstrated expertise in the management of these tumors. Long-term surveillance and follow-up are necessary for the management of treatment late effects related to surgery, radiation therapy, and chemotherapy. These guidelines discuss the management of chordoma, giant cell tumor of the bone, and osteosarcoma.

Waldenström's macroglobulinemia/lymphoplasmacytic lymphoma, version 2.2013.

These NCCN Guidelines Insights highlight the important updates/changes specific to the management of Waldenström's Macroglobulinemia/Lymphoplasmacytic Lymphoma. These include the addition of regimens containing novel agents as primary and salvage therapy options, inclusion of the updated summary of response categories and criteria from the sixth international workshop on Waldenström's Macroglobulinemia, and a section on management of peripheral neuropathy in the accompanying discussion.

Accuracy of CT-guided percutaneous core needle biopsy for assessment of pediatric musculoskeletal lesions.

BACKGROUND: CT-guided percutaneous core needle biopsy has been shown in adults to be an effective diagnostic tool for a large number of musculoskeletal malignancies. OBJECTIVE: To characterize our experience with CT-guided percutaneous core needle biopsy of pediatric bone lesions and determine its utility in diagnosing pediatric osseous lesions, in a population where such lesions are commonly benign. MATERIALS AND METHODS: From 2000 to 2009, 61 children underwent 63 CT-guided percutaneous biopsies. Radiological, pathological and clinical records were reviewed. RESULTS: Fourteen biopsies (22%) were performed on malignant lesions, while 49 biopsies (78%) were performed on benign lesions. Forty-nine of the 63 biopsies (78%) were adequate; these children underwent no further tissue sampling. Fourteen of the 63 biopsies (22%) were inadequate or non-conclusive. Of these patients, 12 underwent open biopsy. Retrospective analysis of percutaneous biopsies in these patients demonstrates that 9/12 provided clinically relevant information, and 4/12 patients received final diagnoses that confirmed initial core biopsy findings. No malignancies were diagnosed as benign on percutaneous biopsy. Overall, percutaneous core needle biopsy provided accurate diagnostic information in 84% (53/63) of biopsies. CONCLUSION: Our results demonstrate that CT-guided percutaneous biopsy is safe and beneficial in children. This study supports the use of CT-guided percutaneous core needle biopsy for primary diagnosis of pediatric bone lesions.

Analysis of the Effect of the Size and Grade of Soft Tissue Sarcoma on Rates of Unplanned Resection, Metastatic Disease, Mortality, and Morbid Re-Resection Over 20 Years.

Soft tissue sarcomas are rare malignancies that are often presumed to be benign and are resected without the typical preoperative workup, such as imaging or biopsy. These unplanned resections occur in approximately 30% of all cases and frequently require further morbid treatments, resulting in worse oncologic outcomes. A retrospective review was performed of all patients who presented to a tertiary sarcoma center with a diagnosis of sarcoma between 1996 and 2017. In-depth chart reviews were performed for the 2600 patients who were identified, with 836 having a primary diagnosis of soft tissue sarcoma in an upper or lower extremity. Data collected included histologic features, grade, size, resection status, demographic features, referral information, metastatic disease, morbid procedures, and mortality rate. Patients were divided into 2 groups based on whether the tumor size was greater or less than 5 cm. This classification was in keeping with the guideline of referring patients to a tertiary sarcoma center for workup for tumors "larger than a golf ball." The difference in the rate of unplanned resection for tumors measuring less than 5 cm (41.6%) and those measuring 5 cm or greater (18.8%) was statistically significant (P<.001), with smaller tumors more likely to undergo unplanned resection, in keeping with the success of the "golf ball rule." The rate of metastatic disease for unplanned resection for tumors measuring 5 cm or greater (50.7%) was significantly greater than that for tumors measuring less than 5 cm (19.7%) (P<.001). The authors found a great deal of morbidity associated with unplanned resection, regardless of tumor size. Before resection is planned, delineation is required beyond tumor size. [Orthopedics. 2021;44(3):166-171.].

Metastatic bone disease: diagnosis, evaluation, and treatment.

The management of bone metastases has changed considerably over the past decade, with the improved longevity of cancer patients, the advent of bisphosphonates, and the availability of other new treatment modalities. Of primary importance is the systematic establishment of the diagnosis of metastasis before treatment, which avoids improper management of primary bone malignancies and optimizes oncologic treatment. The decision to use surgical stabilization rests not only on structural concerns but also on the patient's anticipated longevity, activity goals, and preferences. Minimally invasive options are available to treat bone lesions in areas not amenable to surgical stabilization or in patients who are poor candidates for surgery. Selected patients with spinal metastases, especially those with cord compression, may benefit from decompression and/or stabilization.

Childbearing Decisions in Residency: A Multicenter Survey of Female Residents.

PURPOSE: To characterize how female residents make decisions about childbearing, factors associated with the decision to delay childbearing, and satisfaction with these decisions. METHOD: In 2017, the authors sent a voluntary, anonymous survey to 1,537 female residents enrolled across 78 graduate medical education programs, consisting of 25 unique specialties, at 6 U.S. academic medical centers. Survey items included personal, partner, and institutional characteristics, whether the respondent was delaying childbearing during residency, and the respondent's satisfaction with this decision. RESULTS: The survey response rate was 52% (n = 804). Among the 447 (56%) respondents who were married or partnered, 274 (61%) were delaying childbearing. Residents delaying childbearing were significantly more likely to be younger (P < .001), not currently a parent (P < .001), in a specialty with an uncontrollable lifestyle (P = .001), or in a large program (P = .004). Among self-reported reasons for delaying childbearing, which were not mutually exclusive, the majority cited a busy work schedule (n = 255; 93%) and desire not to extend residency training (n = 145; 53%). Many cited lack of access to childcare (n = 126; 46%), financial concerns (n = 116; 42%), fear of burdening colleagues (n = 96; 35%), and concern for pregnancy complications (n = 74; 27%). Only 38% (n = 103) of respondents delaying childbearing were satisfied with this decision, with satisfaction decreasing with increasing age. CONCLUSIONS: Decisions to delay childbearing are more common in certain specialties, and many residents who delay childbearing are not satisfied with that decision. These findings suggest that greater attention is needed overall, and particularly in certain specialties, to promote policies and cultures that both anticipate and normalize parenthood in residency, thus minimizing the conflict between biological and professional choices for female residents.

Biopsy of soft tissue masses: evidence-based medicine for the musculoskeletal tumor society.

UNLABELLED: The literature contains a number of controversies regarding key questions: (1) When is a biopsy indicated? (2) How should the biopsy be placed? (3) How should the biopsy be performed and which has the greatest diagnostic accuracy? (4) Who should perform the biopsy? (5) What clinical parameters present the greatest diagnostic difficulty? Using PubMed and Google Scholar we performed English-language literature searches of clinical studies reporting biopsy of soft tissue masses. Thirty-two studies met the inclusion criteria but were only able to address three of the five questions the authors had hoped to evaluate. Available evidence suggests open biopsy has the highest diagnostic accuracy over core needle biopsy, which was higher than fine needle aspiration. There was no evidence to address who is best suited to perform the biopsy (general surgeon, orthopaedic surgeon, radiologist, pathologist) in terms of accuracy of diagnosis. Frozen section at the time of biopsy may improve diagnostic accuracy. Diagnostic difficulty was associated with myxoid and round cell neoplasms, infections, and tumors located in the paraspinal region. The limited number of references addressing these issues demonstrated the need for more Level I research in the area of biopsy of soft tissue masses. LEVEL OF EVIDENCE: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Differential Outcomes and Biologic Markers of Radiation-Associated vs. Sporadic Osteosarcoma: A Single-Institution Experience.

Background: Radiation-associated osteosarcoma (RAO) is a rare, life-threatening complication from radiation. Many physicians presume RAO has a worse prognosis than sporadic osteosarcoma (SO), although limited objective data exist. We conducted a retrospective study comparing these entities. Methods: We identified adults treated at our institution with osteosarcoma (1990-2016) and categorized tumors as SO or RAO based on location within a prior radiation field. We extracted data on demographics, treatment and primary malignancy and examined available tumor samples for MTA-1 and ezrin using immunohistochemistry (IHC). Results: Of 159 identified patients, 28 had RAO, diagnosed at a median interval from radiation of 11.5 years (1.5-28 years). Median follow-up was 2.8 years (0.1-19.6 years). Median progression free survival (PFS) and overall survival (OS) were not significantly different in the small population of patients with metastases, SO (n = 20) vs. RAO (n = 6): PFS 10.3 months vs. 4.8 months (p = 0.45) and OS 15.6 months vs. 6.1 months (p = 0.96), respectively. For the larger group with localized disease, median relapse-free survival (RFS) and OS were significantly different, NR vs. 12.2 months (p < 0.001) and NR vs. 27.6 months (p = 0.001) in SO (n = 111) vs. RAO (n = 22), respectively. On IHC, there were significant differences in distribution of high, intermediate or low MTA-1 (p = 0.015) and ezrin (p = 0.002) between RAO and SO tumors. Conclusions: Patients with metastases at diagnosis fared poorly irrespective of prior radiation. RAO patients with localized disease had worse outcomes without detectable differences in therapy rendered or treatment effect in resected specimens. Higher expression of MTA-1 in RAO patients may suggest an underlying difference in tumor biology to explain differences in outcomes.