Thomas J. Scharschmidt

Diagnosis and Management of Soft-tissue Masses

Soft-tissue masses of the extremities are common entities encountered by nearly all providers of musculoskeletal patient care. Proper management of these lesions requires a specific process of evaluation. A detailed history and physical examination must be performed. Appropriate imaging studies must be obtained based on clinical indications. MRI is the imaging modality of choice for diagnosis of soft-tissue masses, with CT and ultrasonography used as secondary options. These modalities aid the clinician in developing an appropriate differential diagnosis and treatment plan. When the initial evaluation is inconclusive, biopsy must be performed. A diagnosis must be established before definitive treatment with surgical excision or, in rare cases, radiation therapy is performed. Clinicians without substantial experience in treating soft-tissue masses should consider referral to a musculoskeletal oncologist for specialized care when a definitive diagnosis of a benign lesion cannot be made. Several studies have shown that multidisciplinary care in specialized referral centers optimizes outcomes and diminishes comorbid complications.A retrospective review of twenty-one patients with Olliers disease showed that the lesion involved the femur and tibia most frequently, and that those bones accounted for the large majority of clinical problems. Angular deformities were common; 80 per cent of the affected femora had clinically significant varus or valgus angulation in the distal part and 42 per cent of the affected tibiae had proximal or distal deformity. The apex of the angulation, when present, was metaphyseal, with the concavity on the side that was more extensively involved by the enchondromas. Osteotomies were done frequently to correct angulation; all healed well. Deformity in the distal part of the femur frequently required repeat osteotomy to achieve a straight bone at skeletal maturity, The extent of shortening, which always was present in the involved limb, closely paralleled the extent of involvement, The discrepancies in limb lengths prior to surgical treatment averaged 9.8 centimeters (range, 4.3 to 35.7 centimeters). Epiphyseal arrest, when appropriately timed, was effective in correcting or limiting the discrepancies, but partial (medial or lateral) epiphyseal arrest to correct angular deformity was ineffective. Diaphyseal lengthening was done on six occasions, once in the femur and five times in the tibia and fibula, with good results. Fourteen pathological fractures occurred in seven of the twenty-one patients, and all healed uneventfully with conservative treatment.

The cancer-testis antigen NY-ESO-1 is highly expressed in myxoid and round cell subset of liposarcomas

Liposarcomas are a heterogenous group of fat-derived sarcomas, and surgery with or without chemoradiation therapy remains the main stay of treatment. NY-ESO-1 is a cancer-testis antigen expressed in various cancers where it can induce both cellular and humoral immunity. Immunotherapy has shown promise in clinical trials involving NY-ESO-1-expressing tumors. Gene expression studies have shown upregulation of the gene for NY-ESO-1, CTAG1B, in myxoid and round cell liposarcomas. Herein, we evaluated the expression of NY-ESO-1 among liposarcoma subtypes by quantitative real-time PCR, western blot analysis, and immunohistochemistry. Frozen tissue for quantitative real-time PCR and western blot analysis was obtained for the following liposarcoma subtypes (n=15): myxoid and round cell (n=8); well-differentiated (n=4), and dedifferentiated (n=3). Formalin-fixed paraffin-embedded blocks were obtained for the following liposarcoma subtypes (n=44): myxoid and round cell (n=18); well-differentiated (n=10); dedifferentiated (n=10); and pleomorphic (n=6). Full sections were stained with monoclonal antibody NY-ESO-1, and staining was assessed for intensity (1−3+), percentage of tumor positivity, and location. In all, 7/8 (88%) and 16/18 (89%) myxoid and round cell expressed CTAG1B and NY-ESO-1 by quantitative real-time PCR and immunohistochemistry, respectively. Western blot correlated with mRNA expression levels. By immunohistochemistry, 94% (15/16) of positive cases stained homogenously with 2−3+ intensity. Also, 3/6 (50%) pleomorphic liposarcomas demonstrated a range of staining: 1+ intensity in 50% of cells; 2+ intensity in 5% of cells; and 3+ intensity in 90% of cells. One case of dedifferentiated liposarcoma showed strong, diffuse staining (3+ intensity in 75% of cells). Our study shows that both CTAG1B mRNA and protein are overexpressed with high frequency in myxoid and round cell liposarcoma, enabling the potential use of targeted immunotherapy in the treatment of this malignancy.

Internal and external hemipelvectomy or flail hip in patients with sarcomas quality-of-life and functional outcomes

ObjectiveWe evaluated the quality-of-life of patients who have had an internal hemipelvectomy with and without (flail hip) prosthetic reconstruction and external hemipelvectomy. DesignWe reviewed the cases of 15 patients who had undergone either internal or external hemipelvectomy for tumor. Fifteen patients who were previously treated operatively with either a type II periacetabular internal (n = 5) or external (n = 10) hemipelvectomy were evaluated using the Toronto Extremity Salvage Score (TESS), Musculoskeletal Tumor Society (MSTS), and the 36-item Short-Form Health Survey. There were 11 (73%) men and 4 (27%) women in the study, with a mean age at operation of 46.9 ± 18.0 yrs (range, 18–69 yrs). ResultsFollow-up was 30.6 ± 19.6 mos (range, 6–70 mos). Overall mean MSTS score was 45.2 (range, 6.7 to 83.3), and TESS score was 60.4 ± 16.1 (range, 31.8–88.0). The 36-item Short-Form Health Survey physical component score results were lower than the general population. TESS and MSTS were all positively correlated to physical component score. There were no significant influences of postsurgery time on MSTS, TESS, or physical component score. Age had a negative correlation with physical function. ConclusionsQuality-of-life and functional outcome were significantly reduced for patients with internal and external hemipelvectomies on the TESS, MSTS, and the 36-item Short-Form Health Survey physical component scores.

Targeted Chemotherapy in Bone and Soft-Tissue Sarcoma.

Historically surgical intervention has been the mainstay of therapy for bone and soft-tissue sarcomas, augmented with adjuvant radiation for local control. Although cytotoxic chemotherapy revolutionized the treatment of many sarcomas, classic treatment regimens are fraught with side effects while outcomes have plateaued. However, since the approval of imatinib in 2002, research into targeted chemotherapy has increased exponentially. With targeted therapies comes the potential for decreased side effects and more potent, personalized treatment options. This article reviews the evolution of medical knowledge regarding sarcoma, the basic science of sarcomatogenesis, and the major targets and pathways now being studied.

Prognostic factors and staging for soft tissue sarcomas: an update.

Soft tissue sarcoma (STS) staging is a constantly evolving process. Grading is still of utmost importance and has been adapted into a three-tier system. The STS most difficult to categorize are those with uncertain malignant potential, such as solitary fibrous tumors, gastrointestinal stromal tumors, and glomus tumors, some of which have developed completely separate staging systems and may not even be considered sarcomas. Beyond the current TNM staging system, a multitude of prognostic factors for STS will continue to be discovered and ultimately incorporated into future revisions of the staging system.

Periostitis Secondary to Prolonged Voriconazole Therapy in a Lung Transplant Recipient

This article reports a case of perostitis deformans in a lung transplantation patient taking the fluoride-containing medication voriconazole, a relatively new and potent anti-fungal. The patient had a normal range of motion in all joints and a normal gait. On radiographs at presentation, multifocal areas of periostitis were visualized involving the left-hand first, second, and third proximal phalanx shafts. Similar periostitis was present on the left-hand third, fourth, and fifth middle phalanx shafts. Plain radiographs of the right hand also demonstrated multifocal periostitis of the third and fourth proximal and middle phalanges. Aggressive periostitis at the level of the right fourth proximal and middle phalanges was also present. Given her long-term treatment with voriconazole and a presentation consistent with periostitis deformans, voriconazole was presumed to be the offending agent and was replaced with itraconazole. The patients symptoms resolved shortly after withdrawal of voriconazole.Voriconazole-associated periostitis has only recently been reported in the literature. Food and Drug Administration-approved in 2002, voriconazole is efficacious in treating serious, invasive fungal infections that are generally seen in immunocompromised patients. Due to the novel nature of voriconazole and the uncommon indications for its long-term use, the periostitis deformans described in this article may be unfamiliar to the orthopedic surgeon. Consequently, a patient presenting with bone pain and periosteal involvement on plain radiographs may provoke a broad, expensive, and ultimately unnecessary diagnostic evaluation. The clinical case and imaging findings presented here can help to promote understanding of this benign condition and its simple cure: voriconazole discontinuation.

Exceptional functional recovery and return to high-impact sports after Van Nes rotationplasty.

Rotationplasty involves wide resection of lower-extremity malignancy and approximately 180° rotation of the tibia to allow the ankle to function similarly to the former knee joint. It is most commonly used for sarcomas around the knee, such as the distal femur and proximal tibia, in adolescent and young adult patients and is an option for patients with proximal femoral focal deficiency (congenital short femur). Rotationplasty is an alternative to ablative procedures when functional outcome is a consideration or when resection of involved areas and endoprosthetic reconstruction is not possible. This article describes functional status and return to competitive sport after rotationplasty for a lower-extremity bone sarcoma with 3-year follow-up. Despite a postoperative course complicated by a distal tibial physeal injury and femoral neck stress fracture, the patient recovered fully by 1 year postoperatively. Pain free at rest and with activity and with no loss of function, the patient is a successful athlete, playing basketball and baseball and skiing competitively. The patient used a custom-made prosthesis that likely played a role in his high level of function. The patients high function is evidenced by a maximal or near-maximal possible score on all subsections of the Short Form 36 health survey. Although this level of function is exemplary, it may be more expected in younger, more active, highly motivated, and emotionally and socially mature individuals.

Pelvic resection: current concepts.

Pelvic resection is a technique that involves surgical resection of portions of the pelvic girdle. Historically, this procedure was known as internal hemipelvectomy. Hemipelvectomy is a resection that includes the ipsilateral limb. The main indication for these procedures is primary malignant tumors of the pelvis, but in rare cases they are indicated for metastatic lesions, infection, or trauma. Reconstruction is dictated by the extent of the resection and the remaining structures. Surgical technique is dictated by histology of the tumor and location of the lesion. A multidisciplinary team is required. The patient and family should undergo counseling preoperatively to discuss morbidity and mortality, the extensive rehabilitation process, and life expectancy.

Multiple myeloma: diagnosis and orthopaedic implications.

Abstract Multiple myeloma is a hematologic malignancy that commonly affects the skeletal system. The disease is primarily managed medically with chemotherapeutic agents. Pathologic fractures are common in patients with diagnosed and undiagnosed disease. The number of patients diagnosed with multiple myeloma is increasing, as is the incidence of associated pathologic fractures. Novel chemotherapeutic agents and radiation therapy protocols have been used to extend the average life span of patients with this disease. Various methods that allow for restoration of function and pain reduction can be used to stabilize and manage fractures associated with multiple myeloma. The orthopaedic surgeon and oncology team must work together to develop an individualized treatment plan to improve patient quality of life and provide pain relief.

Surgical management of soft tissue sarcomas: Extremity sarcomas

Wide surgical resection is the recommended treatment for extremity soft tissue sarcomas. Chemotherapy and/or radiotherapy may improve local control, but with marginal effect on overall survival. Advanced reconstructive techniques and multidisciplinary care, including plastic surgery, may allow a higher rate of limb salvage. This report focuses on surgical and reconstructive aspects in the multimodality care of extremity sarcomas. J. Surg. Oncol. 2015 111:540–545.