Sheila A. Conway

Unplanned excision of soft-tissue sarcomas: current concepts for management and prognosis.

➤ Biopsy, staging, preoperative imaging and planning, as well as surgical treatment of soft-tissue sarcomas, are best carried out in specialized sarcoma centers, with the support of a multidisciplinary tumor board.➤ Tumor bed excision is recommended after most unplanned excisions, with a goal of obtaining complete tumor removal with an appropriately wide margin of resection.➤ The surgical resection area tends to be more extensive during tumor bed excision than during primary resection because of the need to resect potential areas of contamination, resulting in the need for more reconstructive procedures (flaps and skin grafts) and wider radiation fields.➤ Unplanned excisions are associated with an increased rate of local recurrence related to residual disease and positive margins after tumor bed excision, a deep location, and certain histologic subtypes, such as malignant peripheral nerve sheath tumor, myxofibrosarcoma, and dermatofibrosarcoma protuberans.➤ While adjuvant radiation therapy has not been found to mitigate the risk of local recurrence in unplanned excisions, it is generally utilized in the treatment of unplanned excisions as it is in the treatment of primary soft-tissue sarcomas.➤ Given the surgical and oncologic sequelae of unplanned excisions, prevention through the diffusion of concepts by means of provider education on how to approach soft-tissue masses, which can be potential soft-tissue sarcomas, is the best strategy.

Locally Aggressive Fibrous Dysplasia Mimicking Malignancy: A Report of Four Cases and Review of the Literature

BackgroundFibrous dysplasia is a benign fibroosseous bone tumor that accounts for 5% to 10% of benign bone tumors. It can present as monostotic fibrous dysplasia (70% to 80%), polyostotic fibrous dysplasia (20% to 30%), McCune-Albright syndrome (2% to 3%), or Mazabraud’s syndrome in rare cases. Bone lesions in fibrous dysplasia arise in the medullary canal and usually are confined to the bone. Cortical destruction and extension into soft tissue usually indicates malignant transformation or secondary aneurysmal bone cyst formation. Locally aggressive fibrous dysplasia with cortical destruction and extension into soft tissue in the absence of these two possibilities is extremely rare. It is important for the treating physician to distinguish this entity from more aggressive or malignant tumors to avoid overtreating the patient for a benign condition or inattention to a malignant tumor.Case DescriptionsWe report four unusual cases of fibrous dysplasia with an aggressive radiographic appearance. They occurred in the rib (1), ilium (2), and distal femur (1). Two patients had pain and two had swelling. Radiologically, all were associated with cortical destruction and an associated soft tissue mass, and initially they were interpreted as potentially malignant. Three patients underwent biopsy and one patient did not have a biopsy. Histopathologic analysis by an experienced bone pathologist confirmed fibrous dysplasia in all patients. Two patients were treated surgically; one patient with zoledronic acid and one patient currently is being followed by observation alone.Literature ReviewThere are only a few reports in the literature that describe the locally aggressive variant of fibrous dysplasia that presents with pain and progressive swelling clinically and with cortical destruction and soft tissue extension on imaging which suggest malignancy. We could not find any article that describes the use of bisphosphonates in such lesions or the response to bisphosphonates clinically, on laboratory parameters or imaging. To our knowledge, this is the largest case report published regarding locally aggressive fibrous dysplasia arising outside the craniofacial skeleton.Clinical RelevanceThe locally aggressive variant of fibrous dysplasia may be confused with a malignant tumor or malignant degeneration of fibrous dysplasia. It is important to properly evaluate these lesions to ensure that a proper diagnosis is made, especially with respect to a malignant versus benign mass.

Five Polyostotic Conditions That General Orthopedic Surgeons Should Recognize (or Should Not Miss)

General orthopedic surgeons frequently encounter patients with conditions affecting multiple bones. It is important to recognize common polyostotic diseases. This article describes five polyostotic conditions: Multipe Enchondromatosis (Ollier Disease and Maffucci syndrome), Multiple Hereditary Exostosis (Diaphyseal Aclasis), Fibrous Dysplasia (McCune-Albright syndrome and Mazabraud syndrome), Pagets Disease of bone (Osteitis Deformans), and Skeletal Metastases. This is a survey of the clinical, pathologic and radiographic features that assist in diagnosing these conditions. Also, an overview of the laboratory findings, treatment, follow-up, and prognosis is presented. Recognizing these diseases will aid in prompt and accurate diagnosis and appropriate referral and therapy.

The impact of perioperative chemotherapy timing in conjunction with postoperative external-beam radiation therapy on extremity soft-tissue sarcomas outcome

Purpose:The perioperative management of primary extremity soft-tissue sarcomas (ESTS) is multidisciplinary including radiation therapy and chemotherapy (CT). The interplay between these modalities and the relative importance of each remain unclear. Our study aims to determine the relative impact of CT and radiotherapy on the outcome of ESTS patients treated with limb-sparing surgery. Materials and Methods:A retrospective review of ESTS registry yielded 97 patients who received neoadjuvant chemotherapy (NCT) and/or adjuvant CT with or without external-beam radiation therapy (EBRT) from January 1, 1999 through December 31, 2009. The cohort comprised 56 males and 41 females whose age at surgery ranged from 17 to 83 years (median, 56 y). Tumor characteristics included the following: 73 lower ESTS; 70 grade 3 lesions; 63 American Joint Committee on Cancer stage III tumors; and 27 lesions with positive microscopic margins. The following outcome parameters were evaluated for the patients’ subgroups: overall survival (OS), locoregional control (LRC), and disease-free survival (DFS). Results:EBRT was delivered postoperatively to 81 patients and 49 received CT. Median EBRT dose was 63 Gy (range, 50 to 72 Gy). At median follow-up of 54.6 months, the 5-year OS, LRC, DFS was 68.9%, 87.1%, 66.5%, respectively. On multivariate analysis, positive surgical margins negatively impacted LRC, DFS, and OS (hazard ratio [HR]=10.43, P=0.004), (HR=2.37, P=0.03), (HR=2.26, P=0.038), respectively. EBRT use improved LRC (HR=0.24, P=0.018) and DFS (HR=0.36, P=0.021). The impact of EBRT on DFS was retained (HR=0.28, P=0.006) in the high-grade ESTS subgroup who received CT. The 5-year local failure rate was 6.5%, 28.6%, and 22.2% (P=0.019) for patient receiving NCT, adjuvant chemotherapy, and no CT, respectively. Conclusion:Our data support the use of NCT followed by limb-sparing surgery and adjuvant EBRT in ESTS for local failure reduction with a trend toward improved DFS.

Distal biceps brachii tendon repair complicated by a suture granuloma mimicking a soft-tissue sarcoma: a case report and review of the literature

The incidence of distal biceps brachii rupture is 1.2 per 100,000 persons per year; it most commonly occurs in the dominant elbow of men aged in their 40s. The repair, which includes several described techniques, is generally successful in restoring elbow strength, allowing for an early resumption of daily activities. Foreign-body granulomas have been extensively reported in the literature, occurring in a wide variety of operations and anatomic locations. Different surgical materials such as surgical sponges and silicone have been associated with the formation of this benign inflammatory lesion. Nonabsorbable sutures such as Ticron (Tyco, Waltham, MA, USA), FiberWire (Arthrex, Naples, FL, USA), and Ethibond (Ethicon, Somerville, NJ, USA), as in our case, used during tendon repairs can also elicit such a response. Though rare, these reactions can act as malignant neoplasms. However, no cases of suture granulomas after distal biceps brachii tendon repair presenting as a soft-tissue sarcoma (STS) have been reported.

Evaluating Treatment Strategies for Spinal Lesions in Multiple Myeloma: A Review of the Literature

ABSTRACT Background: Vertebral disease is a major cause of morbidity in 70% of patients diagnosed with multiple myeloma (MM). Associated osteolytic lesions and vertebral fractures are well documented in causing debilitating pain, functional restrictions, spinal deformity, and cord compression. Currently, treatment modalities for refractory MM spinal pain include systemic therapy, radiotherapy, cementoplasty (vertebroplasty/kyphoplasty), and radio frequency ablation. Our objectives were to report on the efficacy of existing treatments for MM patients with refractory spinal pain, to determine if a standardized treatment algorithm has been described, and to set the foundation upon which future prospective studies can be designed. Methods: A systematic search of the PubMed database was performed for studies relevant to the treatment of vertebral disease in MM patients. A multitude of search terms in various combinations were used, including but not limited to: “vertebroplasty,” “kyphoplasty,” “radiation,” “multiple myeloma,” “radiotherapy,” and “radiosurgery.” Results: Our preliminary search resulted in 219 articles, which subsequently resulted in 19 papers following abstract, title, full-text, and bibliography review. These papers were then grouped by treatment modality: radiotherapy, cementoplasty, or combination therapy. Significant pain and functional score improvement across all treatment modalities was found in the majority of the literature. While complications of treatment occurred, few were noted to be clinically significant. Conclusions: Treatment options—radiotherapy and/or cementoplasty—for vertebral lesions and pathologic fractures in MM patients demonstrate significant radiographic and clinical improvement. However, there is no consensus in the literature as to the optimal treatment modality as a result of a limited number of studies reporting head-to-head comparisons. One study did find significantly improved pain and functional scores with preserved vertebral height in favor of kyphoplasty over radiotherapy. When not contraindicated, we advocate for some form of cementoplasty. Further prospective studies are required before implementation of a standardized treatment protocol. Level of Evidence: 5.

Does an Algorithmic Approach to Using Brachytherapy and External Beam Radiation Result in Good Function, Local Control Rates, and Low Morbidity in Patients With Extremity Soft Tissue Sarcoma?

Background High-dose-rate brachytherapy (HDR-BT) and external-beam radiation therapy (EBRT) are two modalities used in the treatment of soft tissue sarcoma. Previous work at our institution showed early complications and outcomes for patients treated with HDR-BT, EBRT, or a combination of both radiation therapy modalities. As the general indications for each of these approaches to radiation therapy differ, it is important to evaluate the use of each in an algorithmic way, reflecting how they are used in contemporary practice at sites that use these treatments. Question/purposes (1) To determine the proportions of intermediate- and long-term complications associated with the use of brachytherapy in the treatment of primary high-grade extremity soft tissue sarcomas; (2), to characterize the long-term morbidity of the three radiation treatment groups using the Radiation Therapy Oncology Group/ European Organization for Research and Treatment of Cancer (RTOG/EORTC) Late Radiation Morbidity Scoring Scheme; (3) to determine whether treatment with HDR-BT, EBRT, and HDR-BT+EBRT therapy, in combination with limb-salvage surgery, results in acceptable local control in this high-risk group of sarcomas. Methods We retrospectively studied data from 171 patients with a diagnosis of high-grade extremity soft tissue sarcoma treated with limb-sparing surgery and radiation therapy between 1990 and 2012 at our institution, with a mean followup of 72 months. Of the 171 patients, 33 (20%) were treated with HDR-BT, 128 (75%) with EBRT, and 10 (6%) with HDR-BT+EBRT. We excluded 265 patients with soft tissue sarcomas owing to axial tumor location, previous radiation to the affected extremity, incomplete patient records, patients receiving primary amputation, recurrent tumors, pediatric patients, low- and intermediate-grade tumors, and rhabdoid histology. Fifteen patients (9%) were lost to followup for any reason including died of disease or other causes during the first 12 months postoperatively. This included four patients who received HDR-BT (12%), 11 who received EBRT (9%), and none who received HDR-BT+EBRT (0%) with less than 12 months followup. Determination of radiation therapy technique for each patient was individualized in a multidisciplinary forum of sarcoma specialists. Anticipated close or positive surgical margins and a low likelihood of complex soft tissue procedures were factors that encouraged use of brachytherapy, whereas the anticipated need for secondary procedures and/or soft tissue coverage encouraged use of EBRT alone. Combination therapy was used when the treatment volume exceeded the treatment field of the brachytherapy catheters or when the catheters were used to boost a close or positive surgical margin. Local recurrence, complications, and morbidity outcomes scores (RTOG) were calculated based on chart review. Between-group comparisons pertaining to the proportion of patients experiencing complications, morbidity outcomes scores, and local recurrence rates were not performed because of dissimilarities among the patients in each group at baseline. Results The HDR-BT treatment group showed a high incidence of intermediate-term complications, with the three most common being: deep infection (33%, 11 of 33); dehiscence and delayed wound healing (24%, eight of 33); and seroma and hematoma (21%, seven of 33). The EBRT group showed a high incidence of intermediate- and long-term complications with the three most common being: chronic radiation dermatitis (35%, 45 of 128); fibrosis (27%, 35 of 128); and chronic pain and neuritis (13%, 16 of 128). The RTOG scores for each treatment group were: HDR-BT 0.8 ± SD 1.2; EBRT 1.9 ± 2.0; and HDR-BT+EBRT 1.7 ± 1.7. Overall, 142 of 169 (84%) patients were free from local recurrence: 27 (82%) in the HDR-BT group, 108 (86%) in the EBRT group, and seven (70%) in the combination therapy group. Conclusions In this single-institution study, an algorithmic approach to using HDR-BT and EBRT in the treatment of patients with high-grade soft tissue sarcomas can yield acceptable complication rates, good morbidity outcome scores, and a high degree of local control. Based on these results, we believe HDR-BT is best for patients with an anticipated close margin, a positive surgical margin, and for patients who are unlikely to receive a complex soft tissue procedure. Conversely, if a secondary procedure and/or soft tissue coverage are likely to be used, EBRT alone may be reasonable. Finally, combination therapy might be considered when the treatment volume exceeded the treatment field capacity for HDR-BT or when the catheters were used to boost a close or positive surgical margin. Level of Evidence Level IV, therapeutic study.

Primary Osteosarcoma of the Bone with Rhabdoid Features: A Rare, Previously Undescribed Primary Malignant Tumor of Bone

Primary osteosarcoma of the bone with rhabdoid features is a rare malignant tumor of bone, not previously described in the literature. Here we report a 69-year-old female who originally presented with a right femur pathologic fracture. Radiographs of the injury showed an aggressive-appearing lesion of the distal femur. Initial biopsy was done, which was not diagnostic; additional advanced imaging studies were performed, which failed to show any other site within the body with detectable disease process. Accordingly, the patient underwent radical resection of the distal femur and reconstruction with endoprosthesis. Histopathology obtained from the operative specimen showed osteosarcoma with rhabdoid features. Two months after surgery, the patient is symptom-free and doing well; she is currently pending adjuvant chemotherapy. Although rhabdoid features have been described in extraskeletal osteosarcoma, this appears to be the first mention of osteosarcoma of bone with rhabdoid features in the literature.

Current concepts review unplanned excision of soft-tissue sarcomas: Current concepts for management and prognosis

➤ Biopsy, staging, preoperative imaging and planning, as well as surgical treatment of soft-tissue sarcomas, are best carried out in specialized sarcoma centers, with the support of a multidisciplinary tumor board.➤ Tumor bed excision is recommended after most unplanned excisions, with a goal of obtaining complete tumor removal with an appropriately wide margin of resection.➤ The surgical resection area tends to be more extensive during tumor bed excision than during primary resection because of the need to resect potential areas of contamination, resulting in the need for more reconstructive procedures (flaps and skin grafts) and wider radiation fields.➤ Unplanned excisions are associated with an increased rate of local recurrence related to residual disease and positive margins after tumor bed excision, a deep location, and certain histologic subtypes, such as malignant peripheral nerve sheath tumor, myxofibrosarcoma, and dermatofibrosarcoma protuberans.➤ While adjuvant radiation therapy has not been found to mitigate the risk of local recurrence in unplanned excisions, it is generally utilized in the treatment of unplanned excisions as it is in the treatment of primary soft-tissue sarcomas.➤ Given the surgical and oncologic sequelae of unplanned excisions, prevention through the diffusion of concepts by means of provider education on how to approach soft-tissue masses, which can be potential soft-tissue sarcomas, is the best strategy.

Unplanned Excision of Soft-Tissue Sarcomas

➤ Biopsy, staging, preoperative imaging and planning, as well as surgical treatment of soft-tissue sarcomas, are best carried out in specialized sarcoma centers, with the support of a multidisciplinary tumor board.➤ Tumor bed excision is recommended after most unplanned excisions, with a goal of obtaining complete tumor removal with an appropriately wide margin of resection.➤ The surgical resection area tends to be more extensive during tumor bed excision than during primary resection because of the need to resect potential areas of contamination, resulting in the need for more reconstructive procedures (flaps and skin grafts) and wider radiation fields.➤ Unplanned excisions are associated with an increased rate of local recurrence related to residual disease and positive margins after tumor bed excision, a deep location, and certain histologic subtypes, such as malignant peripheral nerve sheath tumor, myxofibrosarcoma, and dermatofibrosarcoma protuberans.➤ While adjuvant radiation therapy has not been found to mitigate the risk of local recurrence in unplanned excisions, it is generally utilized in the treatment of unplanned excisions as it is in the treatment of primary soft-tissue sarcomas.➤ Given the surgical and oncologic sequelae of unplanned excisions, prevention through the diffusion of concepts by means of provider education on how to approach soft-tissue masses, which can be potential soft-tissue sarcomas, is the best strategy.