A novel osseointegrated percutaneous prosthetic system for the treatment of patients with transfemoral amputation: A prospective study of 51 patients.

Patients with transfemoral amputation (TFA) often experience problems related to the use of socket-suspended prostheses. The clinical development of osseointegrated percutaneous prostheses for patients with a TFA started in 1990, based on the long-term successful results of osseointegrated dental implants. Between 1999 and 2007, 51 patients with 55 TFAs were consecutively enrolled in a prospective, single-centre non-randomised study and followed for two years. The indication for amputation was trauma in 33 patients (65%) and tumour in 12 (24%). A two-stage surgical procedure was used to introduce a percutaneous implant to which an external amputation prosthesis was attached. The assessment of outcome included the use of two self-report questionnaires, the Questionnaire for Persons with a Transfemoral Amputation (Q-TFA) and the Short-Form (SF)-36. The cumulative survival at two years' follow-up was 92%. The Q-TFA showed improved prosthetic use, mobility, global situation and fewer problems (all p < 0.001). The physical function SF-36 scores were also improved (p < 0.001). Superficial infection was the most frequent complication, occurring 41 times in 28 patients (rate of infection 54.9%). Most were treated effectively with oral antibiotics. The implant was removed in four patients because of loosening (three aseptic, one infection). Osseointegrated percutaneous implants constitute a novel form of treatment for patients with TFA. The high cumulative survival rate at two years (92%) combined with enhanced prosthetic use and mobility, fewer problems and improved quality of life, supports the 'revolutionary change' that patients with TFA have reported following treatment with osseointegrated percutaneous prostheses.

Long-term outcome of isolated limb perfusion in advanced soft tissue sarcoma of the extremity.

BACKGROUND: Isolated limb perfusion with tumor necrosis factor alpha and melphalan (TM-ILP) has proven to be a successful option in treating advanced soft tissue sarcomas (STS), where amputation otherwise is needed to achieve safe surgical margins. METHODS: From 2000 to 2009, 54 patients with locally advanced STS, who all were candidates for amputation, were treated with totally 57 TM-ILP procedures and then followed prospectively. The median follow-up time was 30 months. Median tumor size was 10 cm, and 94% of the patients had high-grade tumors. RESULTS: The clinical overall response after TM-ILP was 71% (including 21% CR), and 60% of the patients underwent resection of the tumor remnant after a median of 2 months. The histopathologic response rate in the resected specimens was 76%. Local recurrence/progress occurred in 37% of the patients after a median of 7 months. Thirteen patients finally underwent amputation after a median of 11 months, giving a long-term limb salvage of 76%. CONCLUSIONS: TM-ILP of advanced soft tissue sarcoma of the extremities makes limb-sparing surgery possible in a high proportion of patients.

Fine-needle aspiration characteristics of hibernoma.

BACKGROUND: Hibernoma is a rare, benign lipomatous tumor with features of brown fat. The preoperative diagnosis of hibernoma is difficult at times because its clinical, radiographic, and fine-needle aspiration (FNA) characteristics overlap with those of liposarcoma. METHODS: The preoperative FNA findings of eight surgically excised hibernomas from seven patients (three men and four women, ages 24-60 years) were reviewed. The cytologic features were compared with the histologic features of the corresponding surgical specimens as well as lipomatous tumors and other lesions that may cause confusion in the differential diagnosis. RESULTS: The FNA cytologic features of the hibernomas were found to correspond well with their histologic appearance. The FNA findings included small, round, brown fat-like cells with uniform, small cytoplasmic vacuoles and regular, small, round nuclei; delicate branching capillaries; and variable numbers of mature fat cells. CONCLUSIONS: The FNA cytologic features of hibernoma are characteristic and useful in the preoperative investigation of lipomatous tumors, particularly with regard to excluding a diagnosis of liposarcoma.

Prognostic factors and outcome of pelvic, sacral, and spinal chondrosarcomas: a center-based study of 69 cases.

BACKGROUND: The surgical treatment of chondrosarcoma of the pelvis, sacrum, and spine is complex and technically demanding. As such, adequate surgical margins have been difficult to achieve, resulting in poor local control and survival. The objective of this study was to assess the outcome of patients with chondrosarcomas in these sites who were treated at a tumor center by using modern, aggressive surgical techniques and to identify prognostic factors. METHODS: Sixty-nine consecutive patients with chondrosarcoma of the pelvis (46 cases), sacrum (11 cases), and mobile spine (12 cases) who were treated at Sahlgrenska University Hospital from 1967 to 1999 were included in this study. Demographic information and follow-up data were obtained and statistically analyzed. RESULTS: There were 53 men and 16 women with a mean age of 45 years and a mean tumor size of 12 cm. There were 61 conventional chondrosarcomas, Grades 1-3 (with 13 arising in a preexisting osteochondroma) and 8 Grade 4 chondrosarcomas (7 dedifferentiated and one mesenchymal). The overall local recurrence rate was 27%, and the estimated overall 5- and 10-year survival rates were 72% and 67%, respectively. In contrast, the observed local recurrence rate was 3% (1 patient) in 31 patients whose conventional chondrosarcomas were resected with adequate surgical margins; 90% of these patients survived and most of them (26 of 31 or 84%) were continuously disease free. Significant factors associated with a worse prognosis with respect to local control and/or survival were high histologic tumor grade, increasing patient age, primary surgery outside of a tumor center, incisional biopsy versus a noninvasive diagnostic procedure, and inadequate surgical margins. CONCLUSIONS: Center-based diagnosis and treatment using modern aggressive surgical techniques significantly improve the prognosis of patients with chondrosarcoma of the pelvis, sacrum, and spine.

Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients.

BACKGROUND: The prognosis of patients with chordoma of the sacrum and mobile spine has been reported to be dismal and attributable in the majority of cases to intralesional surgery. The purpose of this study was to evaluate the clinical outcome of these patients using modern surgical principles aimed at complete resection and to identify prognostic factors. METHODS: The clinical and morphologic features, type of surgery, and follow-up of 39 consecutive patients with chordoma were reviewed and analyzed statistically. RESULTS: Thirty sacral and 9 mobile spine chordomas (size range, 3-20 cm; mean, 8 cm) occurring in 22 women and 17 men (median age, 55 years) were analyzed. The preoperative morphologic diagnosis was based on fine-needle aspiration (FNA) biopsy, core needle biopsy, or incisional biopsy. The final surgical margins were wide in 23 patients and marginal or intralesional in 16. The mean follow-up was 8.1 years (range, 0.1-23 years). Seventeen patients (44%) developed local recurrences and 11 patients (28%) developed metastases. The estimated 5-, 10-, 15-, and 20-year survival rates were 84%, 64%, 52%, and 52%, respectively. Local recurrence was associated significantly with an increased risk of metastasis and tumor-related death (P < 0.001). CONCLUSIONS: New surgical techniques have improved local control and survival of patients with sacral or spinal chordoma significantly and have decreased progressive neurologic deterioration. Larger tumor size, performance of an invasive morphologic diagnostic procedure outside of the tumor center, inadequate surgical margins, microscopic tumor necrosis, Ki-67 > 5%, and local recurrence were found to be adverse prognostic factors. FNA is the preferred method for establishing the preoperative morphologic diagnosis of chordoma.

del(6)(q12q15) as the sole cytogenetic anomaly in a case of solitary infantile myofibromatosis.

We describe a case of light microscopically typical solitary, infantile myofibromatosis in a 6-month old boy. The myofibroblastic differentiation of the tumor was supported by immunohistochemical and ultrastructural analyses. Cytogenetic and FISH analyses revealed a pseudodiploid karyotype with an interstitial deletion of the long arm of one chromosome 6, del(6)(q12q15), as the sole anomaly. The results demonstrate the usefulness of cytogenetics and FISH in distinguishing this type of lesion from infantile fibrosarcoma. To the best of our knowledge this is the first cytogenetic analysis of solitary infantile myofibromatosis.

Synovial sarcoma: identification of low and high risk groups.

BACKGROUND: Synovial sarcoma, one of the most common soft tissue sarcomas that occur in adolescents and young adults, is generally viewed and treated as a high grade sarcoma. However, the authors' own experience and some previous studies have indicated that it has a wide spectrum of biologic behavior and that low and high risk subgroups of patients with synovial sarcoma can be identified. METHODS: A total of 121 consecutive patients with synovial sarcoma (including 66 males and 55 females ages 9-74 years), treated primarily or secondarily at 2 large referral centers for musculoskeletal tumors, were included in a statistical analysis conducted to identify independent prognostic factors. RESULTS: There were local recurrences in 38 patients (31%), usually after inadequate primary surgery outside the referral centers; 64 patients (54%) developed metastasis, primarily to the lungs. The estimated 5-, 10-, and 15-year survival rates were 60%, 50%, and 45%, respectively (the mean follow-up for surviving patients was 9.8 years, with a range of 1-30 years). In multivariate analysis, independent risk factors for local recurrence included larger tumor size and primary surgical resection outside the referral center. Independent risk factors for metastasis were older patient age, tumor with poor histologic differentiation, and tumor necrosis. For tumor-related death, the independent risk factors were older patient age, tumor with poor histologic differentiation, and larger tumor size. Local recurrence was associated with a 3.66-fold increased risk of tumor-related death. A low risk group (patient age <25 years, tumor size <5 cm, and no histologic evidence of poorly differentiated tumor) with 88% overall disease free survival was identified, as was a high risk group (patient age > or = 25 years, tumor size > or = 5 cm, and poorly differentiated tumor) with 18% overall disease free survival (P < 0.001). CONCLUSIONS: The identification of low and high risk synovial sarcoma patients indicates that synovial sarcomas are not uniformly high grade tumors. It also indicates that treatment strategies should be modified for these risk groups. Adequate primary surgery is essential to both local control and outcome for synovial sarcoma patients.

Extraskeletal myxoid chondrosarcoma: a reappraisal of its morphologic spectrum and prognostic factors based on 117 cases.

Extraskeletal myxoid chondrosarcoma (EMC), a phenotypically and genotypically distinctive entity, has generally been viewed as a low-grade sarcoma. No studies regarding clinical and morphologic prognostic factors have been performed on a large series of cases with long-term follow-up because of the rarity and protracted clinical course of EMC. The clinical, morphologic, and immunohistochemical features of 117 previously unreported cases were studied and statistically analyzed. The male-to-female ratio was 2:1. The median patient age was 52 years (range, 6-89 years), and the median tumor size was 7 cm (range, 1.1-25 cm). All tumors occurred within the deep subcutis or deeper soft tissues, with 80% occurring in the proximal extremities or limb girdles and 20% in the trunk. Most initial tumor excisions were intralesional or marginal. Follow-up information was available in 99 cases (median, 9 years: range, 2 months-22 years). Forty-eight patients were disease-free, and 41 patients had evidence of disease (18 of these had died of disease). Ten additional patients survived, but their disease status was unknown. There were local recurrences in 40 (48%) of 83 patients, 23 (58%) of whom had multiple local recurrences. Metastases occurred in 35 (46%) of 76 patients. The estimated 5-, 10-, and 15-year survival rates were 90%, 70%, and 60%, respectively. All cases had histologic features characteristic of classical EMC, at least focally. Cellular foci devoid of myxoid matrix and reminiscent of chondroblastoma, Ewing's sarcoma, monophasic and poorly differentiated synovial sarcoma, fibrosarcoma, and rhabdoid tumor were identified in 29% cases. Older patient age, larger tumor size, and tumor location in the proximal extremity or limb girdle were adverse prognostic factors identified by multivariate analysis. Metastasis also adversely affected survival, although local recurrence did not. This study shows that EMC has a unique clinical course, including a high rate of local recurrence, prolonged survival after metastasis in some cases, and eventually a high rate of death due to tumor. These features distinguish EMC from low-grade sarcomas. This study shows that histologic grading is of no prognostic value in EMC because prognosis is dictated primarily by certain clinical features. Histologic recognition of classical EMC and cellular and solid, nonmyxoid variants is important, however, in view of EMC's distinctive biologic behavior.

Light and electron microscopic examination of fine-needle aspirates in the preoperative diagnosis of cartilaginous tumors.

Twenty-eight patients with chondrogenic tumours--2 chondroblastomas, 4 chondromas, 18 chondrosarcomas, 1 clear-cell chondrosarcoma, and 3 mesenchymal chondrosarcomas--underwent fine-needle aspiration biopsy (FNAB) in the preoperative investigation. The cytologic features in smears were compared with the histopathologic findings in the surgical specimens; in 14 cases they were also compared with the light and electron microscopic findings in resin-embedded fine-needle aspirates. The smears of the vast majority of the classical chondrosarcomas presented features that made possible the FNAB diagnosis of a chondrogenic tumor to be made. In the case of the low-grade chondrosarcomas in particular, which were poorly or moderately cellular in smears and showed chondroblastic cells often in lacunary structures of hyaline matrix, consideration of the clinical presentation, size, location, and roentgenographic appearance was essential for the diagnosis of chondrosarcoma. On the other hand, the high-grade chondrosarcomas presented cytologic features that clearly indicated their malignancy and they usually had a myxoid matrix. The possible differential diagnoses that may arise from the FNAB diagnosis of cartilaginous tumors are discussed. The resin-embedding technique for the light and electron microscopic examination of FNABs, along with the histochemical analysis for the demonstration of sulphated glucosaminoglycans and the immunocytochemistry applied to smears, was found to be of value in the definite diagnosis, especially in the distinction of chondrogenic tumors from chordoma and metastatic mucous-producing carcinoma.

Tumoral calcinosis. An ultrastructural analysis and consideration of pathogenesis.

The clinical presentation and radiographic findings including computed tomography is described in a case of tumoral calcinosis along with a detailed light- and electronmicroscopic analysis. Five main cell types surrounding the calcified masses were identified: fat cells, fibroblasts, macrophages, osteoclast-like giant cells and osteoblast-like cells. The light microscopic and ultrastructural findings suggest an initial tissue damage with fat necrosis and the secondary calcification process seems to be of the mitochondrial type and possibly also of the matrix vesicel type.

Intramuscular haemangioma of the extremities: is computerized tomography useful?

Fourteen patients with intramuscular haemangiomas of the extremities were included in the study. All had computerized tomography (CT) and thirteen had angiography. Twelve of the thirteen were operated upon. CT scan proved to be an extremely valuable tool in the pre-operative planning as the extent of the lesion could be assessed accurately by this method; in most cases this was not possible with angiography.

Gentamicin-PMMA beads in the treatment of chronic osteomyelitis.

Fifty cases of chronic osteomyelitis, associated with septic arthritis in five cases, were treated with radical surgery, systemic antibiotics, and insertion of gentamicin-PMMA beads. Seven cases were lost to follow-up. The remaining 43 cases were followed up for an average of 14 months. All but four cases healed.

Surgical removal of bone and muscle metastases of renal cancer.

Fifteen men and six women with renal cancer underwent surgical removal of metastatic lesions in bone (19 patients) or muscle (two patients). The operation was carried out 2 years before nephrectomy/renal resection in two patients, on the same occasion in four, and 1-196 months after in 15. Surgical interventions of various kinds were undertaken, resulting in the loss of a lower limb in seven patients and an upper limb in one. The observed 5-year survival was 4 out of 10. Six patients were alive at follow-up, five of them without evidence of disease. Eight of the remaining 15 patients died of an unrelated disease (five without evidence of tumor); the other seven patients died of metastatic tumor disease. Local recurrence was diagnosed, and removed, in two patients. The results compare favourably with reports on surgically removed pulmonary metastases of renal cancer and seem to justify an aggressive attitude towards solitary bone and muscle metastases of renal cancer.

Favourable results of acrylic cementation for giant cell tumors.

A consecutive series from two university hospitals of 50 patients with giant cell tumors was reviewed and histologically reconfirmed. The patients treated with curettage and acrylic cement were re-examined concerning function. Two of 14 cemented tumors had recurred but could be treated by additional cementation, so that no final failure of this method had occurred, compared to one of 19 radically resected and 12 of 22 with bone-graft after curettage. Joint function was normal in 11 and radiographic arthrosis of low grade was found in only two of 14 patients. It is concluded that this method gives a minimum of recurrence and a maximum of function. It is suggested that the old name of "giant cell sarcoma" should be reintroduced, bringing the tumor into the group of low-grade sarcomas where it belongs.

Amputation for tumor of the upper arm.

In a 10-year period 35 patients underwent a proximal amputation of the upper limb because of a malignant tumor. In 27 patients a forequarter amputation was made, in one a humeroscapular disarticulation and in seven an amputation through the humerus. The observed 5-year survival was 23 per cent. Twelve out of 23 patients followed for at least 3 years also survived 3 years. Fifteen living patients were questioned concerning prosthetic use and social and psychologic factors. Only three patients used a functional (mechanical) prosthesis and only five used a cosmetic prosthesis. The other seven patients rejected the use of a prosthesis. Half of the patients had the same occupation postoperatively as preoperatively. Activities of daily living did not constitute any major problem. One of three housekeepers needed daily help. One patient seemed to have suffered obvious psychologic damage.

Chordoma. A clinicopathologic and prognostic study of a Swedish national series.

Chordoma is a rare tumor with slow growth developing from remnants of the notochord and thus appears in close relation to the axial skeleton. A Swedish national series collected over a period of 13 years, comprising 51 patients with a follow-up time of 8-20 years, was studied. Histologically all tumors had a typical appearance except two, which had characteristics of "chondroid chordoma". Fifty-seven per cent of the tumors were located in the sacrococcygeal region, 27 per cent in the spheno-occipital region and 16 per cent in the vertebrae. The peak age incidence was in the 6th and 7th decades. Male: female ratio was 1:1. The main symptoms were pain and neurologic disturbances. Skeletal destruction was noted radiographically in most instances. Intratumoral calcifications were rarely seen. The treatment was surgery, radiotherapy or a combination of these. There was only one long-term survivor without evidence of disease, a patient operated on for cervical chordoma 14 years earlier. Six other patients lived 8-18 years after diagnosis with chordoma. All other patients were dead; 39 died of chordoma, five of unrelated causes. Distant metastases were noted in 29 per cent. Chordoma constituted 17.5 per cent of all primary malignant bone tumors of the axial skeleton. The yearly incidence was 0.51 per million inhabitants in Sweden.