Clear cell sarcoma of soft tissues. Mayo Clinic experience with 35 cases.

Thirty-five cases of clear cell sarcoma of soft tissues were studied to determine the clinical or morphologic features that are important in predicting prognosis. Tumors occurred most commonly in the extremities, and the majority of the patients were young women. Surgery was the elected treatment in every case. Five patients experienced local recurrences, and metastases developed in 22. Fifty-four percent of the patients died of tumor, 11% are alive with disease, and the remaining 34% are alive and well; the average survival for each group was 67 months, 113 months, and 103.5 months, respectively. This sarcoma is characterized by small clusters of polygonal to spindle cells featuring clear to slightly basophilic cytoplasm and vesicular nuclei with prominent nucleoli. The clusters are separated by delicate fibrous septa. In a deletion, clear cell sarcoma has low mitotic activity, little or no necrosis, and mild nuclear pleomorphism. Tumor size and the presence of necrosis are statistically significant predictors of prognosis. All 12 patients with tumors measuring > 5 cm died of disease or are alive with disease. Eleven of the 20 patients with tumors measuring < 5 cm are alive with no evidence of disease. Tumor necrosis was present in 10 cases; eight of these patients died of disease and one is alive with disseminated metastases.     

Oligodendrogliomas: the Mayo Clinic experience.

Eighty-one patients with pure supratentorial oligodendrogliomas underwent surgery alone (19 patients) or surgery plus postoperative radiation therapy (63 patients) between the years 1960 and 1982. The median survival time and the 5-, 10-, and 15-year survival rates for these 82 patients were 7.1 years, 54%, 34%, and 24%, respectively; these values were significantly different from those for an age- and sex-matched normal reference population. Univariate and multivariate survival analyses were performed on 13 possible prognostic factors including: patient age and sex; presence of seizures; site, size, side, computerized tomography (CT) enhancement, grade, and calcification of the tumor; and treatment (extent of surgical resection, lobectomy, radiation dose, and radiation field). Of these factors, tumor grade as classified by the Kernohan and St. Anne-Mayo methods was most strongly associated with survival. Patients with Grade 1 or 2 tumors by either grading method had a median survival time and 5- and 10-year survival rates of approximately 9.8 years. 75%, and 46%, respectively, compared to 3.9 years, 41%, and 20% for those with Grade 3 or 4 tumors. The extent of surgical resection was also associated with survival. The 19 patients who underwent gross total resection of their tumor had a median survival time and 5- and 10-year survival rates of 12.6 years, 74%, and 59%, compared to 4.9 years, 46%, and 23%, respectively, for the 63 who had subtotal resection. When comparing the 19 patients who underwent surgery alone with the 63 who had surgery plus postoperative radiation therapy, there did not appear to be a survival benefit to be gained from the addition of postoperative radiation therapy. However, the patients who had surgery alone tended to have gross total resections and lower tumor grades. Analysis of the subset of 63 patients who underwent subtotal resection alone or with radiation therapy showed that the median survival time and 5- and 10-year survival rates were: 2 years, 25%, and 25% for the eight patients with subtotal resection alone; 4.5 years, 39%, and 20% for the 26 patients with surgery and low-dose (less than 5000 cGy) radiation therapy; and 7.9 years, 62%, and 31% for the 29 patients receiving surgery and high-dose radiation therapy (greater than or equal to 5000 cGy), respectively.     

Early experience and results with the periacetabular osteotomy. The Mayo Clinic experience.

The purpose of the present study was to review the early results of periacetabular osteotomy in the initial group of patients undergoing this procedure at the authors' institution. The first 21 hips in 19 patients with greater than 2 years followup, which represents the learning curve with this operation, were reviewed retrospectively. There were 14 females and five males with an average age of 21 years (range, 17-43 years). Intertrochanteric osteotomy was performed simultaneously on four patients with coxa valga and inadequate correction with periacetabular osteotomy alone. At an average of 38 months of followup (range, 24-52 months), the Mayo hip scores improved from an average of 46 points (range, 34-58 points) to an average of 68 points (range, 42-80 points). Hip range of motion declined slightly in all three arcs of motion. The lateral center edge angle of Wiberg improved from an average of 2 degrees to an average of 24 degrees. The loading zone angle (T?nnis) improved from an average of 24 degrees to an average of 11 degrees. The anterior center edge angle of Lequesne improved from an average of -6 degrees to an average of 38 degrees. Complications included two peroneal palsies, both of which resolved completely; three ischial fractures that healed uneventfully; three asymptomatic pubic nonunions; and asymptomatic heterotopic ossification in five patients. One patient underwent subsequent total hip arthroplasty for progressive arthritis and pain. Another patient required intertrochanteric osteotomy at a later date. The early results in this initial group of patients treated with periacetabular osteotomy show reliable radiographic correction of deformity and improved function with an acceptable complication rate. Patients should be counseled carefully about possible loss of motion postoperatively. Additional study is necessary to assess the long term results of this procedure.     

The management of soft tissue sarcomas

BackgroundSoft tissue sarcomas are a rare and heterogeneous group of malignancies that are derived from the mesenchymal cell lines. In the last few decades, the management of these lesions has been improved by the introduction of dedicated Multi Disciplinary Teams (MDTs) where most bone and soft tissue tumours are now treated.¹Following the recent changes to management outlined by the NICE/IOGs, we believe it is pertinent to review the current thinking on soft tissue tumour management.² We also discuss the principles of diagnosis and treatment and the role of adjuvant therapy.MethodsThis is a retrospective review. In the preparation of this paper, we have referred to recent NICE guidelines in this field and have performed a Medline search of the existing literature.ResultsThe key to success is early and appropriate patient referral. Whilst the responsibility for performing surgery has shifted away from the generalist and towards the super-specialist, improvements in survivability can be achieved by promoting basic knowledge within the medical profession as a whole.ConclusionsBoth excision and biopsy of a soft tissue sarcoma by a non-specialist surgeon have been shown to increase the risk of tumour recurrence and all invasive procedures should now be performed within the MDT setting.     

The management of soft tissue sarcomas

The management of soft tissue sarcomas presents one of the more exciting areas of the application of multimodal therapy for malignancy. Rapid developments have taken place in the understanding of the histogenesis, histopathology, prognostic indicators and treatment approaches. With the combination of local irradiation and adjuvant chemotherapy, limb sparing operations can now be widely advocated with amputation being reserved for proximal difficult lesions. Retroperitoneal sarcomas, however, remain a major challenge with little progress in results of therapy, other than improved operative morbidity in the last 10-15 years. Current prospective, randomized studies of the management policies designed to maximize survival while minimizing morbidity should provide further insights in the near future. Our current and past experience with more than 150 new cases each year allows a brief summary of the understanding and management of these difficult problems.     

The radiology of bone and soft tissue sarcomas.

Solitary lesions of bone often have characteristic radiographic patterns that suggest the diagnosis of a specific bone tumor. Differentiation of tumor from infection, however, frequently may be impossible, and the majority of bone lesions require biopsy for histologic confirmation of the type of tumor or for identification of the infectious organism. Soft tissue masses, unlike bone tumors, most commonly look alike. They too require biopsy, but in this case to distinguish a benign mass from a soft tissue sarcoma. Special radiographic techniques such as isotopic and CAT scans or angiography add information about the morphology and response of adjacent bone, but fail to differentiate one category of disease from another or to establish a histologic diagnosis of the type of tumor.     

Pediatric soft tissue sarcomas

Soft tissue sarcomas in children are rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or a non-RMS soft tissue sarcoma (NRSTS). RMS is more common in children 14 years old and younger and NRSTS in adolescents and young adults. Infants get NRSTS, but their tumors constitute a distinctive set of histologies. Surgery is a major therapeutic modality and radiation plays a role. RMS is treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the NRSTS that are high grade or unresectable. This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas.     

Clinical features of soft tissue sarcomas.

The presenting features of 526 patients referred to an open access 'lumps and bumps' clinic were reviewed to try and identify whether the four cardinal features of soft tissue malignancy were in fact predictive of this. The features investigated were: size bigger than 5 cm, pain, increase in size, depth beneath the deep fascia. All of these factors were found to be associated more frequently with malignancy than a benign state. Using the summed weights of evidence method we have constructed a graph which will allow prediction of whether a lump is likely to be malignant or not.     

Obturator hernia: the Mayo Clinic experience

Background

Obturator herniae (OH) are rare, with nonspecific signs and symptoms, and diagnosis is usually delayed until laparotomy. The added benefit of preoperative diagnosis with computed tomography (CT) remains unclear.

Methods

We reviewed the clinical characteristics and outcomes of OH repairs performed at our institution over a 58-year period. Outcomes were compared between patients who did or did not have a preoperative CT.

Results

Between 1950 and 2008, 30 patients (median age 82?years, 29 women) underwent OH repair. The most common presenting signs and symptoms were bowel obstruction (63%), abdominal/groin pain (57%), and a palpable lump (10%). The pathognomonic Howship-Romberg sign was present in 11 patients (37%). The diagnosis was made preoperatively in nine patients: clinically in one (3%) and with CT in eight (27%). Nineteen patients (63%) presented emergently. Primary and prosthetic repair were performed in 23 (77%) and seven (23%) patients, respectively. Small-bowel resection was performed in 14 patients (47%). Perioperative morbidity (30%) and mortality (10%) rates were high. Patients with a preoperative CT were less likely to develop a postoperative complication of any type [odds ratio (OR) 0.8, P?=?0.04]; however, time to operation, length of stay, need for bowel resection, and mortality rate did not differ (P?=?NS). No recurrences were detected at a median follow-up of 2?years (range 0?C55).

Conclusion

Although CT imaging provides an excellent means of preoperative diagnosis, suggestive signs and symptoms in a ??skinny old lady?? should prompt immediate operative intervention without delay.     

Radiosensitivity of soft tissue sarcomas

The correlation between the effectiveness of radiation therapy and the histology of soft tissue sarcomas was investigated. Of 31 cases with a soft tissue sarcoma of an extremity treated by conservative surgery and postoperative radiation of 3,000-6,000 cGy, local recurrence occurred in 12; 5 out of 7 synovial sarcomas, 4 of 9 MFH, one of 8 liposarcomas, none of 4 rhabdomyosarcomas and 2 of 3 others. As for the histological subtyping, the 31 soft tissue sarcomas were divided into spindle cell, pleomorphic cell, myxoid and round cell type, and recurrence rates were 75%, 33.3%, 16.7% and 0%, respectively. From the remarkable difference in recurrence rate, it was suggested that round cell and myxoid type of soft tissue sarcomas showed a high radiosensitivity compared to the spindle cell type with low sensitivity. Clarifying the degree of radiosensitivity is helpful in deciding on the management of limb salvage in soft tissue sarcomas of an extremity.     

Experiences from tissue microarray in soft tissue sarcomas

The tissue microarray (TMA) technology was introduced in 1998 as a tissue preserving, high-throughput technique that allows studies of multiple markers in large sample sets. TMA slides can be analyzed using techniques such as immunohistochemistry and in situ hybridization and represents a powerful tool for the investigation of potential diagnostic and prognostic markers identified in DNA microarray studies. We review the TMA method, its reproducibility, advantages, limitations and future perspectives with specific focus on soft tissue sarcomas.     

Inflatable penile prosthesis: Mayo Clinic experience with 175 patients.

Sex prosthetics have become an established alternative of therapy for both the organically and the psychogenically impotent male patient. Functional success with the implantation of the inflatable penile prosthesis can be anticipated in 90 to 95 per cent of the patients. Both mechanical and pathologic complications occur, but with relatively low frequency. There have been no reported operative or postoperative deaths associated with the implantation of more than 6,000 devices. The availability of penile prostheses to impotent patients should be limited only by the patient's ability to meet the rigid criteria for selection of patients. When these criteria are fulfilled and the patient's expectations are in harmony with the known results that can be provided by implantation, uniformly successful results can be expected.     

The model AS 800 artificial urinary sphincter: Mayo Clinic experience

The model AS 800 artificial urinary sphincter was implanted in 100 male and 9 female patients between 7 and 89 years old. Postoperative followup was 1 to 32 months. The indication for implantation was total urinary incontinence in 86 patients (78.9 per cent), stress incontinence in 22 (21.2 per cent) and urgency incontinence in 1 (0.9 per cent). Of the patients 97 (89 per cent) underwent implantation for the first time, 7 (6.4 per cent) had a previous artificial urinary sphincter model replaced by the AS 800 device and 5 (4.6 per cent) underwent reimplantation of a previous model. The cuff was placed around the bladder neck in all 9 female patients, whereas in the male patients the cuff was implanted around the bladder neck in 20 and around the bulbous urethra in 80. Thirty-one patients (28.4 per cent), 29 of whom were continent at night, were practicing nocturnal deactivation of the device. Complete post-activation continence was achieved in 91 patients (83.5 per cent), some leakage occurred in 10 (9.2 per cent) and 8 (7.3 per cent) remained incontinent. A total of 23 patients required 1 or more revisions, the most common indications for the first revision being loss of cuff compression (9), tubing kink (3), cuff erosion (3) and infection (2). At the time of this report 89 patients (81.7 per cent) were continent, 9 (8.3 per cent) still had some leakage, 3 (2.8 per cent) were incontinent, 5 (4.6 per cent) were awaiting reimplantation and 3 (2.8 per cent) had died of unrelated causes.     

Body wall and visceral nonrhabdomyosarcoma soft tissue sarcomas in children and adolescents

Background

Predictors of outcome have not been established for pediatric visceral and body wall nonrhabdomyosarcoma soft tissue sarcomas (NRSTS).

Methods

The study used a retrospective review of clinical features and outcome of 61 patients with visceral and body wall NRSTS evaluated at our institution between March 1962 and December 1999.

Results

Median age at diagnosis was 9.9 years (range, birth to 17.4 years). Tumors were greater than 5 cm in 43 (70%), high grade in 33 (54%), invasive in 25 (41%), and metastatic at presentation in 14 (23%) patients. Visceral tumors (n = 27) were more likely than body wall tumors (n = 34) to be greater than 5 cm (93% vs 53%; P < .001) and invasive (70% vs 18%; P < .001) and were less likely to be resected at diagnosis (44% vs 85%; P = .001). Estimated 10-year event-free survival (EFS) and overall survival (OS) for the entire cohort were 45.5% ± 6.9% and 56.8% ± 6.7%, respectively. The 10-year EFS and OS were better for patients with body wall sites than for those with visceral sites (61.8% ± 8.5% and 67.5% ± 8.2% vs 24.2% ± 9.4% and 43.0% ± 10.3%; P = .004 and P = .004). The 10-year estimated cumulative incidence (CI) of local recurrence was higher for patients with visceral sites than for those with body wall sites (64.3% ± 9.8% vs 26.5% ± 7.7%; P = .004), whereas CI of distant recurrence was similar for the 2 sites (15.2% ± 7.2% vs 23.5% ± 7.4%; P = .39).

Conclusions

Pediatric patients with visceral NRSTS are more likely to have invasive, large, and unresectable tumors compared to those with body wall tumors. More than two thirds of visceral NRSTS recur locally, and fewer than half of patients with visceral tumors survive.     

Radiobiological considerations in radio-induced soft tissue sarcomas

Soft tissue sarcomas are relative rare tumors with an incidence of almost 1% of all malignancies. Although their etiology is not well understood, studies regarding the effect of ionizing radiation, showed their capacity to induce sarcomas in soft tissues, after several years from the treatment of other primary tumor. Their early diagnosis could be achieved by genetic analysis of radio-induced chromosomal translocations specific to each histological type. Dose-response curves reveals the dynamics of the process, taking into consideration the beam energy and linear energy transfer.