Wenger, Doris E.

AbstractThe role of quantitative magnetic resonance imaging (MRI) and positron emission tomography/computed tomography (PET/CT) techniques continues to grow and evolve in the evaluation of musculoskeletal tumors. In this review we discuss the MRI quantitative techniques of volumetric measurement, chemical shift imaging, diffusion-weighted imaging, elastography, spectroscopy, and dynamic contrast enhancement. We also review quantitative PET techniques in the evaluation of musculoskeletal tumors, as well as virtual surgical planning and three-dimensional printing.

AbstractBackgroundExtraskeletal myxoid chondrosarcoma (ESMC) is a rare type of soft‐tissue sarcoma with limited series reporting outcome of treatment. Currently there is limited data on the incidence and impact on patient outcome in those with metastatic disease to lymph nodes in ESMC.MethodsThirty (21 males, 9 females) patients, mean age 50 ± 16 years, with ESMC were reviewed. The tumors were most commonly located in the lower extremity (n = 23, 77%) and the mean tumor size and volume were 9 ± 5 cm and 490 ± 833 cm3. Mean follow up was 7 ± 4 years.ResultsSix (20%) patients either presented (n = 3, 10%) or developed (n = 3, 10%) lymph node metastatic disease. When comparing patients without, with lymph node metastasis and metastasis elsewhere, patients with lymph nodes metastasis had worse survival than those without metastasis, however better 10‐year disease specific survival than those with metastasis elsewhere (100% vs 62% vs 0%; P < .001).ConclusionThere is a high incidence of lymph node metastatic disease in patients with ESMC. Although survival in these patients is worse compared to those without metastasis, their survival is better than those with metastasis elsewhere. Due to the high incidence of lymph node metastatic disease, preoperative staging of the lymph node should be considered.

AbstractBackgroundConventional primary pelvic chondrosarcoma often presents as a low‐ or intermediate‐grade tumor in older patients. Although this is the most common variant of pelvic chondrosarcoma, studies examining treatment outcomes are lacking. The purpose of this study was to evaluate patients with these tumors to determine their outcomes of treatment.MethodsSeventy‐three patients (grade I [n = 19, 26%] and grade II [n = 54, 74%]) were reviewed including 55 (75%) males and 18 (25%) females, with a mean age of 51 (range, 17‐81) years and follow‐up of 9 ± 5 years.ResultsThe 10‐year disease‐specific survival was 71%. Grade II disease (hazard ratio [HR], 6.74; P = .04) and age ≥50 years (HR, 3.97; P = .02) was associated with death due to disease. The 10‐year local recurrence‐ and metastatic‐free survival were 79% and 72%. Of the patients with a local recurrence (n = 11), 7 (64%) recurred at a higher histological grade. Patient age ≥50 years was associated with local recurrence (HR, 10.03; P = .02) and metastatic disease (HR, 4.20; P = .02).ConclusionAdvancing patient age was an independent risk factor for worse survival and disease recurrence. Tumors often recurred locally at a higher grade and as such wide local excision remains the treatment of choice for these tumors.

AimsChondroblastomas (CB) are rare bone tumours that typically arise in the epiphysis/apophysis of long bones in skeletally immature patients. We explore the clinicopathological features of CB presenting in adults.Methods and resultsCB in patients ≥20 years of age were retrieved from our institutional archives. Thirty‐nine CB were identified (29 male/10 female; aged 20–54 years). Twenty (51%) cases occurred in long tubular bones, 10 (26%) in small bones of the feet, five (13%) in flat bones and four (10%) in the patella. All cases showed classic cytological features of CB, and chondroid matrix was universally present. Calcification was identified in 10 cases (26%), including various combinations of serpiginous (n = 7), punctate (n = 6), classic chicken‐wire (n = 4) and psammomatous (n = 2) patterns. Haemosiderin (n = 19), woven bone (n = 13), secondary aneurysmal bone cyst formation (n = 8), foamy macrophages (n = 4), hyalinised vascular spaces (n = 2) and cholesterol clefts (n = 2) were noted. Follow‐up information (n = 32, 1–452 months) revealed local recurrence in three patients, all >40 years of age with flat bone origin, one of which developed pulmonary metastases 132 months after initial diagnosis.ConclusionsCB in patients >20 years of age more frequently involves the short bones of the hands/feet and flat bones compared to those arising in their younger counterparts. A subset may harbour extensive serpiginous or psammomatous calcification rather than the classic chicken‐wire pattern. Although the overall local recurrence rate in adulthood is approximately 10%, all three patients with recurrent disease had tumours involving flat bones, suggesting that tumours arising in these sites may behave more aggressively.

Abstract Soft tissue sarcomas are a rare and heterogeneous group of malignancies that present significant diagnostic and therapeutic challenges. Patient stratification based on tumor aggressiveness and early therapeutic response based on quantitative imaging may improve prediction of treatment response and the evaluation of new treatment strategies in clinical trials. The purpose of this pilot study was to determine the technical feasibility of magnetic resonance elastography (MRE) and dynamic contrast-enhanced (DCE) MRI for the evaluation of sarcoma stiffness and perfusion in 9 patients with histologically confirmed sarcoma. Additionally, we assessed the feasibility of utilizing MRE and DCE-MRI for the early evaluation of response to radiation therapy in 4 patients to determine the utility of further evaluation in a larger cohort study. Tumor size, stiffness, and perfusion parameters all decreased from baseline at the time of the pre-surgery or follow-up MRI, and results were compared to pathology or conventional imaging. MRE and DCE-MRI may be useful for the quantitative evaluation of tumor stiffness and perfusion, and therapy response assessment in soft tissue sarcomas.

BackgroundAdamantinomas are rare bone tumors, commonly affecting the tibia. Due to the rare nature of disease, previous studies are small or from multiple centers. The purpose of this study is to investigate outcomes of patients with adamantinoma treated in a single institution.MethodsForty‐six histological confirmed adamantinomas of the extremities were reviewed at our institution between 1939 and 2012. Follow‐up data included clinical and radiographical information focusing on complications, local recurrence, metastasis, and overall survival after the treatment. The mean follow‐up was 16 years (range 2‐42 years).ResultsThe most common location was the tibia (n = 31). Patients commonly presented with pain and swelling. The mean age was 24 years (7‐79 years). Thirty‐seven patients were treated with limb salvage. The 39% of patients required a reoperation. The 10‐year disease specific‐ and recurrence free survival was 92% and 72%, with three patients having a recurrence over 15 years postoperative. Older (> 20 years) patients and males were at increased risk of local recurrence (P < 0.05).ConclusionTreatment of adamantinoma of the long bone consists of limb‐salvage surgery. Male patients should be cautioned on their increased risk of disease recurrence, and advocate for continued surveillance of patients even greater than 15‐years postoperatively due to late tumor recurrence.

Background An anteroinferior osseous defect of the glenoid rim is sometimes encountered in patients with recurrent anterior dislocations of the shoulder. A defect of more than 21% of the glenoid length is reported to cause instability after Bankart repair. Hypothesis We can estimate the critical size of glenoid defects by using radiography or computed tomography. Study Design A controlled laboratory study. Methods Osseous defects of 0%, 9%, 21%, 34%, and 46% of the glenoid length were created stepwise in 12 cadaveric scapulae, and plain radiographs simulating the axillary and West Point views and computed tomographic images were obtained. The maximum width of the remnant glenoid was measured under each condition and expressed as a percentage of the width of the intact glenoid. Results A 21% defect appeared to be 18.6% of the intact glenoid on the West Point view. With computed tomography, a 21% defect resulted in loss of 50% of the width on a single slice across the lower one-fourth of the glenoid. Conclusions We can estimate the size of a glenoid defect by using the West Point radiographic view or computed tomogram. Clinical Relevance These images gave decisive information as to whether an osseous glenoid defect required bone grafting to achieve stability after Bankart repair.

By the current WHO classification, benign notochordal cell tumor (BNCT) and chordoma comprise the entire spectrum of notochordal-derived tumors. They have defined radiologic and histologic criteria, and differ considerably in management and clinical outcome. Chordomas are malignant tumors; they show progressive, destructive growth and have the capacity for metastasis. In contrast, BNCT are benign and show limited intraosseous growth. Patients with BNCT can be managed with serial imaging or conservative excision, whereas patients with spinal/sacral chordomas typically undergo radical en bloc resection often with adjuvant therapy and significant morbidity. As such, the distinction between BNCT and chordoma is critically important. We have seen 4 unusual notochordal tumors with radiologic and/or histologic features that defy classification as either BNCT or chordoma. Cases occurred in 4 adults (53 to 83 y), and involved the lumbar spine (N=2) and sacrum (N=2). Three cases had subtle radiologic features of cortical permeation with minimal soft tissue extension. All 4 cases had the characteristic histologic features of BNCT; however, 2 cases also had focal myxoid change. Three patients were followed with serial imaging (follow-up range, 26 to 120 mo); 2 showed no disease progression and 1 had a 10-year cumulative interval growth of 3.7 mm. One patient underwent sacrectomy. The tumor was examined in toto and had the characteristic histologic features of BNCT, with the exception of minimal soft tissue extension. On the basis of these observations, we propose a provisional designation of atypical notochordal cell tumors (ANCT) be used for the subset of notochordal-derived tumors that fail to fulfill current diagnostic criteria for either BNCT or chordoma. We would argue that designating these atypical notochordal tumors as chordoma precipitates potentially overly aggressive surgical management. Patients with ANCT may be better managed by close observation and serial imaging. Additional studies with more cases and longer clinical follow-up should clarify the relationship of ANCT to BNCT and chordoma.

Although the majority of giant cell tumors (GCTs) of the bone occur in adult patients, occasionally they arise in the pediatric population. In this setting they may be mistaken for tumors more commonly seen in this age group, including osteosarcoma, aneurysmal bone cyst, and chondroblastoma. All cases of primary GCT of the bone arising in patients 18 years and below were retrieved from our institutional archives and examined with emphasis on the evaluation of various morphologic patterns. Clinical/radiologic records were reviewed when available. Analysis for H3F3A/H3F3B mutations was performed in a subset of cases. Sixty-three (of 710) patients treated at our institution for GCT were 18 years of age and below. The following morphologic patterns were identified: fibrosis (31 cases, 49%), reactive-appearing bone (26, 41%), cystic change (7, 11%), foamy histiocytes (6, 10%), secondary aneurysmal bone cyst (3, 5%), and cartilage (2, 3%). Infarct-like necrosis was present in 17 tumors (27%), and the mitotic rate ranged from 0 to 35 mitoses/10 high-power fields (median 5 mitoses/10 high-power field). Follow-up information (n=55; 6 mo to 69.6 y; median, 11.6 y) showed 21 patients with local recurrence (38%) and 2 patients with lung metastasis (4%). Polymerase chain reaction with sequencing showed that 5 of 5 tested cases harbored H3F3A mutations. In summary, GCT arising in the pediatric population is rare, representing 9% of GCTs seen at our institution. The morphologic spectrum of these tumors is broad and similar to that seen in patients above 18 years of age. It is important to recognize that matrix formation may be observed in GCT, including reactive-appearing bone and cartilage, as well as areas of fibrosis mimicking osteoid production, to avoid misclassification as osteosarcoma or other giant cell–rich lesions common in children.