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Henry J Mankin

Publications and source records attributed to Henry J Mankin.

At least 19 recordsLinked to original sources

Clear cell sarcoma of soft tissues.

Clear cell sarcoma (CCS) is a rare but highly malignant tumor of soft tissues often appearing as a small tender mass in the deep tissues of the distal extremities. We have studied 17 patients with such lesions treated since 1986 and have a high incidence of local recurrence and metastasis with a survival rate of only 47% despite surgery and for many of the patients, adjuvant therapy. The purpose of this article is to warn the readers of the dangers related to treating this seemingly benign lesion and urge them to perform wide surgery and utilize adjuvant therapy.

Adolescent↗

Improved survival for sarcomas of the wrist and hand.

PURPOSE: To evaluate survival characteristics of malignant hand tumors compared with those occurring in other musculoskeletal locations over a 30-year period. METHODS: Patients were identified through a computerized database maintained at the authors' institution over the past 30 years. Each patient's records were reviewed retrospectively. Diagnoses were categorized and survival data for patients with hand malignancies were compared with those of patients having similar tumors in other musculoskeletal sites by using multivariate statistical analysis. RESULTS: The most common malignancies were epitheloid sarcoma, synovial sarcoma, and malignant fibrous histiocytoma. Improved survival during the follow-up period was found for hand malignancies versus those occurring in other musculoskeletal sites as a whole, and this difference was statistically significant. No significant survival benefit was identified for individual tumors. CONCLUSIONS: Patients presenting with primary malignancies of the hand may survive longer than those with similar tumors in other musculoskeletal locations TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic, Level II.

Adolescent↗

Advantage of limb salvage over amputation for proximal lower extremity tumors.

UNLABELLED: Although function after lower extremity amputation and limb salvage has been compared, no study has assessed individual functional variables by surgical level. Our aim was to determine whether risks of long-term psychologic and physical limitations were associated with amputation or limb salvage at four levels: below-knee, above-knee, hip, and pelvis. We included 408 patients with sarcomas and postoperative followup of 2 years or greater who had completed a quality-of-life self-report questionnaire. The mean length of followup was 8.91 +/- 5.15 years (range, 2-27 years). Relative risk analysis was done on 12 dichotomous general health, psychologic, and physical function variables. At the below-knee level, outcomes were similar after both procedures. At the above-knee level, amputation was associated with increased risk of limp (RR = 1.6), walking aid use (RR = 2.1), anxiety (RR = 2.4), and inability to drive (RR = 3), and decreased risk of muscle weakness (RR = 0.57). At the hip and pelvic levels, outcomes were descriptively compared because of the small number of amputations. At these higher levels, limitations were more common after amputation. The difference in results between the below-knee and above-knee levels supports the importance of distinguishing surgical levels. Limb salvage offers a functional advantage at proximal tumor locations. LEVEL OF EVIDENCE: Therapeutic study, Level III (retrospective, comparative study). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗

Aneurysmal bone cyst: a review of 150 patients.

PURPOSE: We have reviewed a series of 150 aneurysmal bone cysts treated over the last 20 years. PATIENTS AND METHODS: The lesions were principally located in the tibia, femur, pelvis, humerus, and spine and, in most cases, presented the imaging appearance originally described by Jaffe and Lichtenstein as a blowout with thin cortices. RESULTS: Only one of the patients was believed to have an osteoblastoma of the spine with secondary development of an aneurysmal bone cyst, and none of the patients developed additional lesions. The patients were treated primarily with curettage and implantation of allograft chips or polymethylmethacrylate, but some patients were treated with insertion of autografts or allografts. The local recurrence rate was 20%, which is consistent with that reported by other centers. CONCLUSION: Aneurysmal bone cysts are enigmatic lesions of unknown cause and presentation and are difficult to distinguish from other lesions. Overall, the treatment is satisfactory, but it is possible that newer approaches, such as improved magnetic resonance imaging studies, may help diagnose the lesions and allow the physicians to plan for more effective treatment protocols.

Adolescent↗

Guidance on the use of miglustat for treating patients with type 1 Gaucher disease.

Type 1 Gaucher disease (GD) is a progressive lysosomal storage disorder due to an autosomal recessive deficiency of glucocerebrosidase. Clinical manifestations include anemia, thrombocytopenia, hepatosplenomegaly, and bone and pulmonary disease. Intravenous enzyme replacement (ERT) with imiglucerase is the accepted standard for treatment of symptomatic patients. More than 3,500 patients worldwide have received ERT with well-documented beneficial effects on the hematological, visceral, skeletal, and pulmonary manifestations, and with resultant improvement in health-related quality of life. Miglustat, an imino sugar that reversibly inhibits glucosylceramide synthase and reduces intracellular substrate burden, is an oral treatment for patients with type 1 GD that was recently approved in the United States for symptomatic patients with mild to moderate clinical manifestations for whom ERT is not an option. Because responses to miglustat are slower and less robust than those observed with ERT, and because miglustat is associated with significant side effects, clinicians who care for patients with GD should become familiar with the limited indications for miglustat use and the circumstances when it may be prescribed appropriately. This review article and position statement represents the current opinion of American physicians with extensive expertise in GD regarding patient management in the context of the availability of standard imiglucerase treatment and the recent introduction of miglustat.

1-Deoxynojirimycin↗

Total femur replacement procedures in tumor treatment.

UNLABELLED: Replacement of bone segments after resection of a tumor-containing bone has a long history, and currently metallic implants and allografts have reasonable rates of success in terms of patient survival and restoration of useful function. Total femoral resections for extensive tumors are uncommon but clearly represent a major problem, which in the past has required hip disarticulations. In recent years, resection and replacement using custom metallic implants and more recently modular devices have allowed the patients to be restored to reasonable function. Allografts have not been used commonly for this purpose. During the past 23 years, we have done 15 total femoral replacements for osteosarcoma, chondrosarcoma, metastatic carcinoma, and an osteonecrotic fractured femur in a patient with rheumatoid arthritis and for a patient with severe deformity based on Paget's disease. Ten of these patients had allografts implanted with total hip replacement and total knee replacement implants and five patients had metallic implants. Eight of the patients died from disease, but the remaining seven have done reasonably well in terms of function and absence of complications such as fracture or infection. None of the patients required an amputation. The procedure is complex and metallic implant and allograft implantation require wide surgery and special systems of reconstruction. LEVEL OF EVIDENCE: Therapeutic study, Level IV-1 (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗

Infection in massive bone allografts.

We studied a series of 945 patients who received cadaveric allografts implanted for nonpelvic bone or soft tissue tumor lesions since 1971. We specifically were interested in the role of infection in the graft process, and, more specifically its history, frequency, and affect on outcome. Primary infections (not related to reoperations for nonunions or fractures of the graft) occurred in 75 patients (7.9%), and an additional 46 patients had infections related to reoperations, increasing the total number of patients with infections to 121 (12.8%). The highest frequency of infection occurred in patients with soft tissue tumors, radiated sites, Musculoskeletal Tumor Society Stage IIB tumors, or surgeries consisting of an allograft arthrodesis. Most of the infected grafts failed; however, none of the patients died. One patient had hepatitis C develop, and one patient became HIV positive after receiving the virus from a blood transfusion. A comparison with other series of surgically treated patients, including those receiving metallic devices, suggests that the infection rate may be related to the surgery or the graft's immunologic resistance, rather than the graft. The problem of infection is a major issue for tumor surgeons. Suggestions regarding how to decrease the frequency of this complication are presented.

Adult↗

Treatment of giant cell tumors with allograft transplants: a 30-year study.

We retrospectively reviewed 144 patients with giant cell tumors who had resection and implantation of cadaveric allografts from 1971-2001. Most procedures were done in the distal femur, proximal tibia, proximal femur, and proximal humerus. Seventy-eight percent of patients have retained their grafts and remain functional, but with limitations. Tumor complications included local recurrences (eight patients, none required an amputation) and metastases (three patients). None of the patients died. Allograft fracture occurred in 30 (21%) of the 144 patients, nonunion occurred in 12 (8%) patients, and infection occurred in 12 (8%) patients. Only patients with infection had a marked decline in outcome. Four patients required amputations, and only two of the eight patients with infection (17%) retained a functional graft. Eighty of the 144 patients (56%) had no complications, with a 94% success rate for the procedure. There have been fewer grafts done with the advent of burring, phenolization, and insertion of polymethylmethacrylate. However, based on our data and the good outcome for more extensive and destructive tumors, allograft implants can be used for treatment of patients with aggressive tumors, tumors that have caused fractures, or tumors that have recurred after conservative treatment.

Adolescent↗

Chondromyxoid fibroma: a rarely encountered and puzzling tumor.

Chondromyxoid fibroma is an uncommon bone neoplasm, accounting in our series for less than 1% of all connective tissue tumors. The tumor is more common in males, and located mostly in the metaphyseal areas of the lower extremity. The tumor is benign and there have been no reports of metastases. The method of treatment that has been used since the initial identification of the tumor has been curettage, which has a 20-25% recurrence rate. In our 30 patients, the average length of followup was 11 years (range, 1-29 years. Most of the tumors were in the pelvis, proximal tibia, distal femur, and foot. Tumors that were treated with curettage alone did less well than those that were packed with allograft bone or polymethylmethacrylate. Tumors treated by excision did not recur. The most difficult problem with chondromyxoid fibroma is pathologic identity because it often is confused with more aggressive tumors that may metastasize.

Adolescent↗

Alveolar soft part sarcoma: a rare and enigmatic entity.

UNLABELLED: Alveolar soft part sarcoma is a rare malignant tumor with unusual clinical behavior. Treatment of alveolar soft part sarcoma has been difficult to evaluate because of the small numbers of cases seen, but it seemed that although treatment of the primary tumor in alveolar soft part sarcoma often is successful, treatment of metastatic tumors is unsuccessful. A review of outcome after treatment of primary and metastatic disease in the 15 patients in our database with alveolar soft part sarcoma was done in order to evaluate this issue. Nine of 15 patients presented with metastatic disease and one further patient developed metastases. Treatment of primary tumors involved surgical excision in all but one patient and radiation in all patients. Adjuvant chemotherapy was administered to one patient with localized disease and to six patients with metastatic disease. There were no local recurrences. Treatment of metastatic tumors involved chemotherapy in seven patients, metastectomy in three patients, and radiation in two patients. All instances of the metastatic disease either recurred or progressed. Overall survival was 75% at 5 years and 40% at 10 years with a mean survival of 6.5 years, despite the high number of patients with metastatic disease. Current treatment results in good local control of primary tumors, but poor control of metastatic tumors. New approaches to treatment of metastatic alveolar soft part sarcoma must be investigated and applied. LEVEL OF EVIDENCE: Therapeutic study, Level IV-1 (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗

Paget's sarcoma: a historical and outcome review.

UNLABELLED: Paget's sarcoma is a rare entity that principally occurs in elderly people with extensive Paget's disease. The primary sites of occurrence are the pelvis, proximal femur, proximal humerus, and calvarium. The lesions are highly malignant and the patients frequently are found to have metastases at the time of discovery. The survival rate reported in the literature generally is very poor. We were able to locate 16 patients with Paget's sarcoma in the Dr. Henry L. Jaffe Pathology Collection treated from 1942 to 1967. These were added to 27 patients treated for the disease by the Harvard Orthopaedic Oncology Group from 1972 to 2001. Three were giant cell tumors, which are occasionally found in patients with florid Paget's disease but are not malignant. Of the remaining 40 patients, outcome data were available on 35 patients. The rate of Musculoskeletal Tumor Society Stage III tumors was very high (35%) and the rate of survival was 14% at approximately 2.5 years, without differences between the two series. In view of the remarkable concordance in the poor survival figures for patients treated between 1946 and 1967 and those treated between 1972 and 2001 it appears that little progress has occurred in the treatment of Paget's sarcoma over the years, which is in sharp contrast with the current statistics for standard osteosarcoma. LEVEL OF EVIDENCE: Prognostic study, Level IV (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Adult↗

Articular cartilage and osteoarthritis.

Articular cartilage, which makes possible the painless, low-friction movement of synovial joints, consists of a sparsely distributed population of highly specialized cells called chondrocytes that are embedded within a matrix and provide articular cartilage with remarkable mechanical properties. Chondrocytes form the tissue matrix macromolecular framework from three classes of molecules: collagens, proteoglycans, and noncollagenous proteins. The matrix protects the cells from injury resulting from normal joint use, determines the types and concentrations of molecules that reach the cells, acts as a mechanical signal transducer for the cells, and helps maintain the chondrocyte phenotype. Throughout life, articular cartilage undergoes internal remodeling as the cells replace matrix macromolecules lost through degradation. Aging decreases the ability of chondrocytes to maintain and restore articular cartilage and thereby increases the risk of degeneration of the articular cartilage surface. Progressive degeneration of articular cartilage leads to joint pain and dysfunction that is clinically identified as osteoarthritis. Investigation regarding the pathogenesis of posttraumatic osteoarthritis, the form of osteoarthritis that develops following joint injury, is helping to explain the development and progression of joint degeneration.

Aged↗

Giant-cell tumor of the distal forearm.

PURPOSE: Many authorities express concern that giant-cell tumors of the distal forearm are more frequently recurrent and difficult to treat chiefly because of the proximity to the carpus and the resultant diminished range of motion in the hand and forearm. We have studied the results from our institution for 49 patients with giant-cell tumors of the distal forearm treated from 2 to 28 years (mean, 14 +/- 7 years) and compared the results for different methods of treatment. METHODS: Through the computer database 49 patients with giant-cell tumors of the distal forearm (46 of the radius, 3 of the ulna) were identified. By using material from patient visits, chart review, and when necessary telephone interviews it was possible to gather demographic and outcome data for the 49 patients. For the radial lesions, 15 of the patients had a marginal resection of the distal radius along with the periosteum and ligamentous structures and implantation of cadaveric allografts. Twenty-six patients had intralesional curettage and insertion of polymethylmethacrylate (PMMA) and 5 had curettage and autograft insertion. The 3 patients with ulnar lesions were treated with Darrach resections. RESULTS: There were no deaths, infections, metastases, or amputations. In addition to the 49 original surgeries, the patients required 41 additional surgical procedures, 17 of which were for recurrent disease. The greatest numbers of recurrences were in the patients who underwent curettage with autograft or PMMA implantation. Only 2 recurrences were in patients who had a marginal resection and implantation of cadaveric allografts. The overall results for the patients showed that many had mostly minor complaints referable to function or pain and that only 18 of the 49 patients were asymptomatic. CONCLUSIONS: Although the patients with distal forearm giant-cell tumors have had a difficult course in terms of local recurrence and subsequent surgeries required for treatment failures, the ultimate outcomes for both allograft transplantation and curettage and insertion of PMMA are satisfactory. The patients with complete distal radial allografts had a better record for prevention of recurrence than the patients treated with curettage and PMMA insertion but the percentages of currently asymptomatic patients are approximately the same for both series (40% [6/15], 35% [9/26]). These data support the concept that marginal resection and complete distal radial allograft implantation should be used for patients with tumors that have destroyed much of the bone and have extensive soft tissue components and that curettage and PMMA insertion should be reserved for patients where the structural alteration of the bone is minimal.

Adolescent↗