Biomedical subjects

H Mankin

Publications and source records attributed to H Mankin.

11 recordsLinked to original sources

PubMed2001

Familial occurrence of adiposis dolorosa.

Adiposis dolorosa is a rare condition characterized by multiple painful lipomas. We describe two typically affected siblings who were found to have at least 5 affected first-degree relatives in two generations. Manifestations were remarkably variable, ranging from complete disability to solitary asymptomatic tumors. Review of histopathologic findings did not reveal any features that might distinguish the tumors from common sporadic lipomas. Mutational analysis excluded the 8344 A to G mitochondrial mutation seen in other patients with multiple lipomas. Adiposis dolorosa may be an extreme manifestation of the more common condition of familial multiple lipomas. Further work is needed to define the genetic basis of these conditions.

Adiposis Dolorosa↗

PubMed2000

Total knee arthroplasty in patients who have massive osteoarticular allografts.

The authors treated 24 patients with total knee arthroplasty who had a massive allograft used to reconstruct the knee and who later had instability, degeneration, or a fracture near the articular surface of the graft develop. Patients then were followed up for a minimum of 2 years and a mean of 8.2 years. Overall, 96% of the patients retained a functional limb, although 46% underwent revision surgery, and an additional 12% had some other major complication. Statistical analysis showed a significant negative effect of chemotherapy on revision-free survival of the prosthesis. Patients with high-grade tumors were at significantly greater risk of fracture of the allograft-prosthesis composite. Certain technical factors were identified that seemed to predispose the allograft-prosthesis reconstructions to early failure. Total knee arthroplasty can be used to treat patients with complications of massive osteoarticular allografts and may prolong the functional life of an otherwise successful limb salvage reconstruction.

Adolescent↗

PubMed1999

Long-term results of combined modality therapy in primary bone lymphomas.

PURPOSE: To report the Massachusetts General Hospital experience in the management of patients with primary bone lymphoma (PBL) treated with combined modality therapy (CMT). METHODS AND MATERIALS: Records from 37 eligible patients were reviewed. Two patients were treated with complete resection of the tumor, while 35 patients underwent radiation therapy with a median total dose of 54 Gy (range 38.35-66.5). All patients received combination chemotherapy, which contained doxorubicin in 33 cases. We compared the current data with our previous experience in patients treated with local measures only. RESULTS: Actuarial disease-free survival (DFS) at 5 and 10 years is 78% and 73%, respectively, while overall survival (OS) is 91% and 87%, respectively. No local failures were seen. Pathologic fracture at presentation influenced DFS (p = 0.005) and OS (p = 0.017) adversely. OS was compromised in patients older than 60 years (p = 0.059) and DFS in patients with pelvic primaries (p = 0.015). CMT was associated with improved DFS (p = 0.0008) and OS p = 0.0001) compared to our historical controls. Ten patients (27%) developed complications requiring orthopedic procedures following completion of therapy at a median of 25.5 months (range 4-228). CONCLUSION: Patients with PBL have a favorable outcome with CMT, which appears superior to radiation therapy alone. Late complications can be seen, especially in weight-bearing bones.

Adolescent↗

PubMed1994

Quality of life in osteosarcoma survivors.

Charts of 89 osteosarcoma survivors from Massachusetts General Hospital and The Children's Hospital/Dana Farber Cancer Center, who had received primary treatment more than 1 year previously and had no evidence of disease, were reviewed. Sixty-two patients, mean 12 years from diagnosis, agreed to structured interviews. Rates of psychopathology did not differ significantly from the general population. High distress was noted in 13%. Twenty-three normal progeny had been born postchemotherapy to eight women and the wives of five male patients. One pregnancy was complicated by doxorubicin-induced cardiac toxicity. Only two with previous childhood tumors believed themselves infertile. All felt the effort to save the limb was worthwhile. In most, ongoing pain was mild; phantom pain and neuralgia common. Most survivors were in good mental and physical health with the capacity to bear children.

Adolescent↗

PubMed1993

Gorham's massive osteolysis: the role of radiation therapy and a review of the literature.

PURPOSE: This paper reviews the natural history and management of patients with Gorham's disease and presents four cases treated at The Massachusetts General Hospital since 1965. Gorham's disease is characterized by localized endothelial proliferation which results in destruction and resorption of bone. The etiology is undefined. There is no evidence of a malignant, neuropathic, or infectious component. This disease is progressive in most patients, but in occasional instances the process has been noted to be self-limited. The principal treatment modalities are surgery and radiation therapy. METHODS AND MATERIAL: Since 1965, four patients with Gorham's Disease have been treated at the Massachusetts General Hospital. Three received definitive radiation therapy in doses ranging from 31.5 to 45 Gy. The fourth patient underwent surgery primarily. RESULTS: Three patients are currently alive and fully functional with no evidence of disease at last follow-up. The fourth patient died of progressive disease despite treatment with both radiation therapy and surgery. CONCLUSION: The prognosis for patients with Gorham's disease is generally good unless vital structures are involved. Due to the rarity of this entity, there is no standard therapy. Definitive radiation therapy in moderate doses (40-45 Gy in 2 Gy fractions) appears to result in a good outcome and few long-term complications.

Adult↗

PubMed1993

Preoperative radiation, surgical margins, and local control of extremity sarcomas of soft tissues.

The relationship between status of the surgical margin and local control of soft tissue sarcoma of the extremities by preoperative radiation therapy has been assessed in 132 consecutive patients from 1974 to 1988. The 5-year actuarial local control rate was 94% for all patients; the rates were 97% and 82% for the 104 tumors with negative margins and the 28 tumors with positive margins, respectively. The local control rate was independent of the extent of the negative margin. For primary sarcomas, the local control rates were 96%, 97%, and 100%, respectively, for margins that were negative at < or = 1 mm or > 1 mm, or the specimens were negative for tumor; for positive margins the result was 83%. The overall local control rate was lower (not significant) for recurrent than for primary sarcomas: 88% vs. 94%. On the other hand, when stratifying by margin status, no difference was observed in local control results, i.e.: (1) 97% and 91% for primary and recurrent tumors with negative margins, respectively, and (2) 83% and 80% for primary and recurrent tumors with positive margins, respectively. Local control was not significantly higher in those patients who were treated by surgical resection (S), radiation (RT), and re-excision of the previous tumor bed than in those whose radiation therapy was started after the biopsy and followed by one surgical resection. For primary sarcomas that were resected with negative margins the results were: 100% of 15 patients treated by S-RT-S and 96% of 75 patients treated by RT-S.

Actuarial Analysis↗

PubMed1990