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Venous thoracic outlet syndrome (vTOS) usually results from compression of the subclavian vein classically as a result of narrowing of the costoclavicular space. We report 2 rare cases of soft tissue neoplasms resulting in apparent vTOS. The first case is a 46-year-old female with a 2-year history of intermittent unilateral shoulder pain, who was initially diagnosed with intervertebral disk herniation. Cervical fusion was performed; however, her symptoms progressed and she additionally developed paresthesias and venous congestion. Computed tomography (CT) angiogram demonstrated a 13-cm-encapsulated mass within the subscapularis muscle compressing the axillary vein. Radiological findings suggested lipoma. She subsequently underwent complete resection via a transaxillary approach with extension along the lateral border of the latissimus. Final pathology confirmed an intramuscular lipoma. The second case is a 21-year-old female who presented with acute onset of unilateral chest wall pain, palpable nodularity, and venous congestion. CT chest showed pulmonary embolism and an anterior chest wall mass. An initial attempt at resection was aborted due to proximity of the mass to the subclavian vein. The mass enlarged on serial imaging, measuring 3.8 cm in greatest dimension. Additionally, tumor thrombus was seen, and a subsequent ultrasound-guided biopsy was positive for high-grade synovial sarcoma. Positron emission tomography scan showed a pulmonary nodule that was resected thoracoscopically with pathology confirming metastatic synovial sarcoma. Subsequently, she underwent neoadjuvant chemoradiation followed by successful resection of the chest wall mass. An extended infraclavicular approach with a secondary transaxillary incision was utilized to achieve adequate exposure and margins. Final pathology was consistent with preoperative biopsy. Venous reconstruction was not needed. Although rare, an extrinsic mass as a cause of apparent TOS should be in the differential diagnosis. Surgical approach is based on tumor type, location, and proximity to the neurovascular bundle., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Chondroblastomas are benign tumors that typically occur in the epiphysis of long bones. Carpal bone chondroblastomas are very rare and are known to have less aggressive behavior with no evidence of recurrence reported. Methods: We present a case of a recurrent chondroblastoma in the capitate that was treated with repeat curettage, application of phenol, and bone grafting. Results: At 3 years post surgery, the patient is disease free with excellent functional return. Conclusion: Chondroblastomas are rare within the carpus. We present a review of the literature detailing their occurrence and treatment., Competing Interests: Declaration of Conflicting Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Scapulectomy can dramatically alter glenohumeral function and the ability of patients to conduct activities of daily living. In oncologic cases, treatment of the tumor can compromise local musculature, making successful reconstruction difficult to achieve. Depending on the resection level, local musculature may be inadequate to restore shoulder range of motion and/or glenohumeral stability. Surgeons have attempted to address these issues via soft-tissue repairs, allograft replacement, and prosthetic replacement, with variable success. Outcomes are better when a greater portion of the scapula is preserved, thus saving functional rotator cuff muscle bellies. However, preservation of significant rotator cuff musculature is not routinely possible. To our knowledge, no authors in the English-language orthopaedic literature have reported on local tendon transfers as a technique to augment and reconstruct the rotator cuff in a patient with previous scapulectomy.

Purpose: To determine if aerosol granulocyte macrophage-colony-stimulating factor (GM-CSF) decreases the 2-year pulmonary metastasis rate for soft tissue sarcoma., Patients and Methods: Patients with high-grade soft tissue sarcomas were treated with 2 cycles of ifosfamide, mitomycin, doxorubicin, and cisplatin plus GM-CSF subcutaneous followed by 45 Gy irradiation with concurrent 2 cycles of mitomycin, doxorubicin, and cisplatin followed by surgery +/- intraoperative radiation or brachytherapy. Aerosol GM-CSF (250 mcg twice a day) was administered for 1 week every other week 3× during neoadjuvant therapy and beginning 4 weeks postoperatively every other week 5×., Results: A total of 39 patients were enrolled between November 2001 and April 2006. The median age was 51 years (range, 19 to 65 y). The median lesion size was 9 cm (range, 2.3 to 26.7 cm). Seventy-six percent experienced grade 3-4 hematologic toxicity. Twenty-four of the first 35 evaluable patients (69%; 95% CI, 41%-84%) were free of pulmonary metastasis at 2 years. A total of 82% (95% CI, 70%-95%) of patients were still alive after 3 years, with a median follow-up of 5.5 years (range, 3.4 to 7.6 y). A total of 58% (95% CI, 44%-76%) of patients remained progression free after 3 years., Conclusions: The addition of aerosol GM-CSF to combined chemotherapy, irradiation, and surgery for soft tissue sarcomas did not achieve the study endpoint to decrease the 2-year pulmonary metastasis rate.

A 17-year-old girl presented with chondroblastoma of the fibula and pulmonary metastases. Serum creatine kinase (CK) was elevated for a decade preoperatively and fell to near normal after tumor excision. An episode of aseptic meningitis 3 months later led to serologic detection of three antibody markers of paraneoplastic neurologic autoimmunity that in adult patients are characteristic of thymoma and lung carcinoma: muscle-type acetylcholine receptor (AChR) autoantibody (Ab), type 2 Purkinje cell cytoplasmic Ab (PCA-2), and collapsin response-mediator protein-5-IgG (CRMP-5-IgG). Locally recurrent chondroblastoma was excised 1 year later. Serum CK was elevated, CRMP-5-IgG was positive, PCA-2 was lower, and AChR Ab was undetectable. Three years after diagnosis she has stable pulmonary metastases, with elevated CK, higher PCA-2, positive CRMP-5-IgG, and undetectable AChR Ab. The association of metastatic chondroblastoma, elevated CK, and an evolving profile of paraneoplastic autoantibodies reveals a previously unrecognized immunobiologic dimension of chondroblastoma.

Background: Most functional analyses after limb salvage operations about the shoulder have focused on proximal function with the assumption that distal function is largely unaffected. This analysis examines distal function objectively., Methods: Objective laboratory data regarding distal upper extremity strength after reconstructive procedures for tumors near the shoulder joint was collected over a 16-year period. Thirty-two patients were able to participate fully in the data collection at an average most recent follow-up duration of > 3.5 years., Results: Statistically significant reductions on the involved side compared with the uninvolved side in grip, forearm pronation, forearm supination, elbow flexion, and elbow extension strength were documented (p < 0.05). The magnitude of reduction in strength diminishes distally, with the greatest effect in this group of patients being observed in elbow extension, followed by elbow flexion, forearm supination, and forearm pronation. Grip strength consistently showed the least amount of strength reduction compared with the uninvolved side, even within resection and reconstruction groups. Subjective patient rating of dexterity was no less than 3 of 5. Ninety percent of patients rated their dexterity 4 of 5 (52%) or 5 of 5 (38%)., Conclusions: Despite the insistence of "normal" function in the distal upper extremity after limb salvage procedures, complete normality is not maintained. However, the degree of maintenance of distal function appears to be high, especially for grip strength and forearm pronation strength, and patient satisfaction is acceptable.

A multiinstitutional study was carried out to evaluate immunologic responses for human recipients of massive frozen (-80 degrees C) osseous and osteochondral allografts. Allografts were used to reconstruct skeletal defects associated with a variety of traumatic degenerative and neoplastic disorders. Serum samples were obtained before surgery and from 1 month to 4 years after surgery. Sera were tested by microcytotoxicity against T cells from 60 donors for human leukocyte antigen Class I antibodies and against beta 2-microglobulin treated B cells from 40 donors for human leukocyte antigen Class II antibodies. Panels were selected to represent the majority of known human leukocyte antigen specificities. Of the 84 cases evaluated, 62 (74%) received blood transfusions and 28 of 44 (64%) female recipients had been previously pregnant. Sensitization before transplant was shown in 33 of 84 (39%) patients. After grafting, 49 of 84 (58%) recipients showed evidence of sensitization to Class I antigens and 46 of 84 (55%) recipients showed evidence to sensitization to Class II antigens. Overall sensitization was 67%.

During a 10-year period, 17 patients with segmentally destructive bone lesions of the humeral diaphysis in disseminated malignancies resulting in impending fracture (8 patients), pathologic fracture (6 patients), or failure of attempted internal fixation techniques (3 patients) were treated with resection of the involved diaphyseal segment and reconstruction with a cemented modular intercalary humeral spacer. Fourteen patients had metastatic cancer, 2 had multiple myeloma, and 1 had lymphoma. Breast and renal carcinoma were the most common pathologic diagnoses. The involved site was within the middle 1/3 in 8 patients, in the proximal-middle junction in 5, in the middle-distal junction in 2, and within the proximal and distal 1/3 in 1 patient each. Early pain relief was successful in 88% of patients. Early in the postoperative hospital course, patients generally were able to use the ipsilateral hand to assist feeding. Radiographic analysis revealed that the limited selection of stem lengths led to 76% of the distal stems and 47% of the proximal stems being shorter than the ideal length. The complication rate independent of disease progression was 29%. The most common complication was temporary radial nerve injury (3 patients). There were 3 implant failures, most commonly due to disengagement of the male-female junction. Two periprosthetic fractures occurred, 1 proximally (due to tumor progression) and 1 distally. Suggestions are given for modification of the implants to improve the major problems of limited versatility in intramedullary stem length and inadequate mating at the junction.

Massive osteolysis is rare, peculiar, and enigmatic. The clinical, radiographic, and histologic features were reviewed in 11 cases. The patients were generally young (mean age, 20.7 years), and males outnumbered females. More than half the patients had involvement of either the femur or the ribs alone or including the vertebrae. On the basis of radiographic studies, the disease process is capable of originating either in bone or adjacent soft tissues. Histologically, the authors were unable to identify pathognomonic features. Other than excision of all involved tissue, surgical treatment was generally ineffective. Radiation therapy may be successful in eradicating the disease in selected cases.

Biopsy is an integral part of the overall management of patients with soft-tissue sarcoma. The types of biopsy are fine needle, trocar, open incision or en bloc excision. There are advantages and disadvantages of each. Open biopsy requires strict adherence to a number of surgical principles. Proper execution requires determination of appropriate biopsy site, meticulous technique, and close collaboration with an experienced pathologist. Failure to adhere to these principles may result in untoward consequences for patients.

This is a case report of an enterocutaneous fistula complicating total hip arthroplasty, an extremely rare but potentially fatal complication. A 76-year-old woman survived but required extensive prolonged surgical treatment.

Congenital fibromatosis is a rare and benign myofibroblastic tumor that may occur in either a solitary or multicentric form. The soft-tissue form of this entity is well recognized. This report analyzes the clinical and pathologic features of 14 cases of congenital fibromatosis in which the tumor existed as a solitary lesion in bone. Most of the tumors (71%) were in patients who were 2 years old or younger. All but one tumor involved the craniofacial bones. Radiographically, they were purely lucent lesions in which a sclerotic rim was visible in each skull lesion. The myofibroblastic appearance closely resembled the microscopic features seen in the soft-tissue counterpart. For appropriate surgical management, it is imperative to recognize that this tumor occurs as a solitary lesion in bone and that it is distinct from other, more aggressive fibrous lesions.

Reconstruction after resection of malignant bone tumors about the shoulder is difficult. Twelve patients had reconstruction using a new titanium modular spacer. This modular system allows the surgeon to reconstruct variable lengths of the proximal humerus. Although the patient has no active shoulder motion, excellent hand and elbow function is preserved. The spacer serves as a temporary device in the young active patient. A more definitive reconstructive procedure can be performed after the completion of chemotherapy and a sufficient disease-free interval.

A retrospective study of the surgical treatment of 166 metastatic lesions of the humerus and femur in 147 patients was performed. There were 106 women and 41 men whose average age was 62 years. Two-thirds of the patients were treated for complete fractures, while one-third were treated for impending fractures. Breast, lung, and kidney carcinoma accounted for the majority of the primary lesions. One-half of the patients died within nine months of surgery, while one-quarter were alive 19.1 months after surgery. The patients with breast cancer had the best prognosis, while the patients with lung cancer had the worst. The probability of implant failure increased linearly with time to 33% at 60 months. The probability of failure for the femoral lesions was greater, with 44% at 60 months. The average survival in the patients with failed fixation in the femoral lesions was 34.5 months with a mean interval to failure at 17.7 months. The failure rate was high (23%) in proximal femoral lesions treated with a compression screw or nail plate. Common reasons for failure included poor initial fixation, improper implant selection, and progression of disease within the operative field. Bone cement augmentation should be used with the fixation device when possible. Complications due to hip-screw cut-out from the head may also be reduced by applying bone cement around the screw threads.

At the Mayo Clinic between 1950 and 1975, initial fusion of the interphalangeal joint of the great toe was attempted 166 times in 139 patients utilizing a Kirschner wire for stabilization. Follow-up examination of these patients disclosed an overall pseudarthrosis rate of 44%. Because of these disappointing results, a technique involving use of a longitudinal mini-cancellous bone screw was devised which provides increased stability and compression at the arthrodesis site. With this method, used 20 times in 18 patients, the pseudoarthrosis rate was decreased to 10%.

At our institution from 1980 to 1985, 30 patients underwent resection of malignant or locally aggressive bone tumors, with limb salvage and reconstruction by free vascularized bone grafts. Of the 26 patients followed up for at least four months (average, 21 months), four had complications. In these four, there were three nonunions, two infections, and one stress fracture. The average duration of immobilization was 7.6 months in the lower extremity, five months in the pelvis, and 3.8 months in the upper extremity. The average time to union was 6.3 months in the lower extremity, five months in the pelvis, and five months in the upper extremity. Although the technique of oncologic reconstruction must be individualized, our experience indicates that vascularized bone grafts offer significant advantages over conventional methods in selected patients.

Thirteen cases of giant sacral schwannomas with erosion of the anterior aspect of the sacrum and associated intrapelvic extension are reviewed. These tumors showed no sex predilection; the patients' mean age was 38.6 years at the time of diagnosis, and their symptoms predated the diagnosis by an average of 5.2 years. The most common symptoms were low-back pain and lower-extremity dysesthesiae. Plain roentgenograms, myelography, and computerized tomography constituted essential and complementary studies in the preoperative assessment. Choice of surgical approach (anterior transabdominal vs. posterior transsacral) was dependent upon the amount of sacral destruction, intrapelvic extension, and sacroiliac joint involvement. Microscopic examination revealed classic features of benign schwannoma in all but three cases, which were classified as cellular schwannomas. Patients who presented with pain and dysesthesiae reported immediate and complete relief of symptoms following surgery. In addition, all 13 patients were ambulatory and able to resume their routine daily activities postoperatively. At the last reported follow-up examination, which ranged from 5 months to 33 years and 3 months (mean 9 years) after surgery, two patients had died of unrelated causes, two reported return of preoperative symptoms, and the remainder were asymptomatic. This experience suggests that these histologically benign but neurologically devastating tumors should be aggressively resected with the intent of complete extirpation, and that this goal may be accomplished with minimal risk and an excellent prognosis.

Sixty-nine patients were identified who had survived for at least 10 years after therapy for ordinary-type intramedullary osteosarcoma and for whom adequate follow-up data were available to determine whether second malignancies had developed. Eight patients (11.6%) had developed second malignant tumors, 5 of whom had died of their second malignancies. Although these figures are higher than those expected for cancer incidence and death rates, the differences are not statistically significant.

The surgical treatment of extensive metastatic diaphyseal lesions of the humerus with pathologic fractures is difficult. Patients with failed internal fixation often have large segments of bone loss. Rigid fixation often is not possible in these cases. Four patients were treated with a titanium segmental defect (SD) prosthesis with good clinical results. A comparative in vitro analysis of fixation with the SD prosthesis, a Rush rod, and a dynamic compression plate (DCP) augmented with methylmethacrylate was performed. The SD prosthesis performed better than the Rush rod and DCP in both the nondestructive four-point bending test and the destructive torsional test. The reconstructed humeri using the SD prosthesis had a torsional strength approaching that of an intact specimen. Hence, the SD prosthesis is a useful adjunct device in treating patients with extensive destructive lesions of the humerus secondary to metastatic disease and hematologic malignancies and in patients with malignant diseases who have had failed attempts at internal fixation.

Thirty-five cases of neurogenic sciatic nerve tumors are described. Pain is the major clinical manifestation but may give rise to diagnostic confusion because of its often prominent site far distant from the neoplasm. Nonpalpable tumors are best identified by computed tomographic scan of the pelvis and thighs. Early surgical therapy is advisable. Neurilemomas are treated by simple enucleation with preservation of the sciatic nerve. They rarely recur. For neurofibromas, total excision is often not possible. As long as they are small and neurologically nondestructive, subtotal excision with preservation of the nerve trunk is recommended. When they are large or have caused marked neurologic dysfunction, radical tumor and nerve resection is required. In the usually highly malignant neurofibrosarcomas, disarticulation of the hip joint or hindquarter amputation affords the best chance for cure and should not be delayed once the diagnosis has been established.

Distinguishing superficial plantar fibromatosis from deep fibromatosis of the plantar aspect of the foot is difficult both clinically and histologically. An illustrative case is presented. Clinical factors suggestive of aggressive fibromatosis include presentation at an age younger than 20 years, tumor dimension exceeding 3 cm (without prior surgical excision), and multicentric occurrence in the affected limb. On histological review, the absence of nodule formation favors the diagnosis of deep aggressive fibromatosis.

A case of aneurysmal bone cyst is presented, and the radiographic, computed tomographic, and magnetic resonance imaging findings are discussed. The potential advantages of magnetic resonance imaging compared with those of computed tomography are explained. The case is presented to illustrate the potential value of magnetic resonance imaging in the evaluation of bone and soft tissue tumors.

Limb-saving resection has become a valid alternative to amputation in carefully selected patients with bone tumors. As interest grows in this technique, efforts continue to improve our methods of bone and joint reconstruction to restore a functional extremity. Previously there was little interest in this form of treatment despite a limb-saving potential, because of inadequate methods of skeletal reconstruction. The purpose of this paper is to review these previous methods and to outline our present approach to the problem.

Forty-six patients with Kienböck's disease were evaluated over a 2- to 27-year period; 72% had a history of wrist injury prior to diagnosis; 67% had evidence of fracture or fragmentation of the lunate. Documented fractures of the lunate were identified followed by both the presence and absence of subsequent Kienböck's disease and this may be explained by the variable blood supply of the lunate. Ten patients were not treated; 36 patients were treated surgically. Patients were relieved of pain and had functional wrists whether they were treated or not and regardless of the type of surgical treatment.

This study was done to describe the magnetic resonance imaging (MRI) characteristics of osteosarcomas and to determine the clinical usefulness of MRI in the evaluation of patients with these tumors. Ten patients with osteosarcomas were examined with a Picker MRI scanner (0.15 Tesla resistive magnet). All had computed tomograms available for comparison. For demonstrating tumor extent in marrow, MRI was superior to computed tomography (CT) in 60% and about equal to CT in 40%. For defining soft tissue mass, MRI was superior to CT in 40% and about equal to CT in 60%. Direct sagittal and coronal images on MRI and superior contrast differences between tumor and normal tissues with MRI were the reasons for the advantage of MRI in these instances. CT was superior in all cases for demonstrating calcifications. Overall, MRI is better than CT for defining local extent of osteosarcomas. It is of greatest value in the peripheral skeleton, in the medullary canal, and in soft tissues.

Adamantinoma is a rare, primary, malignant bone tumor; only one such case involving the radius has been reported. A second case is documented. This patient was treated by en bloc excision and reconstruction with microvascular fibula transfer. Although adamantinoma is not considered to be a high-grade lesion, aggressive treatment is warranted for lesions of the radius.

Eighteen patients had prosthetic proximal humeral replacement with either a metal or ceramic prosthesis. Three replacements were performed for fracture nonunions, five for benign neoplasms, six for low-grade malignancies, and four for high-grade malignancies. Retention of elbow and hand function was good. In five of the 11 ceramic prostheses, failure occurred at the humeral-prosthetic junction even though it was designed for biologic fixation. Ten of 18 prostheses subluxated or dislocated. Twelve of 18 patients have had revision operations. While the revision rate in this initial series was high, valuable experience was gained for further investigations of shoulder arthroplasty.