Biomedical subjects
S I Hajdu
Publications and source records attributed to S I Hajdu.
Pediatric desmoid tumor: retrospective analysis of 63 cases.
PURPOSE: This study was conducted to evaluate clinical prognostic factors predictive of the probability of recurrence of desmoid tumor (DT). PATIENTS AND METHODS: Sixty-three patients with histologically confirmed diagnosis of DT were retrospectively studied. Median age at diagnosis was 13 years. Patient distribution by site was as follows: 61% extremities, 18% head and neck, 13% trunk (including 5% intraabdominal), and 8% multicentric lesions. All patients had partial or complete resections; 20 patients also received radiotherapy and/or chemotherapy. RESULTS: At a median follow-up time of 6 years since first treatment, the overall actuarial probability of having one or more recurrences was 75%. Age, sex, site, size, or number of previous recurrences had no significant impact on the likelihood of recurrence. The only factor associated with an increased proportion of recurrence-free patients was a negative margin of resection (70% v 15% with positive margins; P = .006). Of the four patients with more than 3 years follow-up since chemotherapy, two recurred, and of the 11 patients with the same follow-up after radiotherapy, four recurred, including two of five patients who received a dose of 50 Gy or more. No deaths directly related to tumor invasion were observed. CONCLUSION: A surgical approach aiming at clear margins is presently the best treatment option. When this cannot be accomplished without severe disfigurement or function impairment, partial resection is an acceptable alternative, but one associated with a high risk of regrowth. Whether adjuvant strategies should be used in this situation remains to be addressed.
Malpractice vs. benepractice.
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Tendosynovial sarcoma. Clinicopathologic features, treatment, and prognosis.
BACKGROUND: Clinicopathologic features, treatment, and results are reported for 95 tendosynovial sarcomas identified from a prospective sarcoma data base established at Memorial Sloan-Kettering Cancer Center (MSKCC) in 1982. METHODS: Eighty-five patients had definitive treatment of the primary tumor at MSKCC: Epithelial sarcomas were excluded. RESULTS: The local recurrence rate was 18%. Survival rates were not influenced by either the method of local treatment or the use of chemotherapy. The overall actuarial survival rate was 59% at 5 years (median, 92 months). For 25 patients who had resection of pulmonary metastases, the 5-year actuarial survival rate was 43% (median, 22 months) from the time of first thoracotomy. Sex, age, tumor site, and histologic subtype (monophasic versus biphasic) were not significant predictors of survival rates. Regional lymph node metastasis occurred in 3%. The incidence of distant metastasis was 49%, with an associated 5-year survival rate of 31% (median, 33 months). Tumor size was a highly significant prognostic feature, with 5-year survival rates of 86% for patients with tumors smaller than 5 cm and 22% for those with tumors larger than 10 cm (P less than 0.00001). CONCLUSIONS: Improvement of treatment results for tendosynovial sarcoma will require effective new systemic adjuvant therapeutic strategies.
Adult urological sarcoma.
From July 1982 to December 1989, 43 of 1,583 adults (2.7%) with soft tissue sarcoma admitted to the Memorial Sloan-Kettering Cancer Center (MSKCC) had tumors arising from the urinary tract and male genital tract (urological sarcoma). The most common site of origin of the tumor was paratesticular (14 patients), followed by the prostate/seminal vesicle (12), bladder (10) and kidney (7). The most common histological type was leiomyosarcoma (19 patients), followed by rhabdomyosarcoma (14), liposarcoma (5) and 5 other histological sarcoma categories (angiosarcoma, malignant fibrous histiocytoma, mesenchymoma and 2 undifferentiated sarcomas). Most of the tumors were high grade (86%) and more than half (56%) were greater than 5 cm. in diameter. A total of 9 patients (21%) presented with metastatic disease, 8 of whom had rhabdomyosarcoma. Complete resection with negative microscopic margins was possible in 58% of the patients. Actuarial relapse-free survival for all patients at 3 and 5 years was 55% and 40%, respectively. There were no significant differences in survival based on patient age, sex or histological tumor type. Favorable prognostic variables by univariate analysis included tumor diameter less than 5 cm., low histological grade, paratesticular or bladder tumor site and complete surgical resection. Application of the MSKCC sarcoma staging system, which is based on grade, size, depth and presence or absence of metastasis, was useful to predict survival. In our experience patients with stage 3 (high grade, greater than 5 cm., 15 patients) or stage 4 (metastatic disease, 9 patients) had a combined 3-year relapse-free survival rate of only 26% and they should be considered candidates for adjuvant treatment protocols.
Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity.
The use of amputation in extremity soft tissue sarcoma has been decreasing at Memorial Sloan-Kettering Cancer Center (MSKCC) over the last 15 years. In an attempt to define the efficacy and future role of amputation in extremity soft tissue sarcoma, a prospective sarcoma database compiled at MSKCC from July 1982 to January 1990, consisting of 649 patients, was analyzed in a retrospective fashion. Ninety-two patients underwent amputation, and 557 had a limb-sparing procedure. Patients selected for amputation were those who had large (T greater than or equal to 5 cm) high-grade tumors that invaded major vascular or nervous structures. The amputation group achieved significantly better local control than the limb-sparing group (p = 0.007). No survival benefit could be demonstrated, however, in the groups selected for amputation (i.e., large, high-grade tumors) when compared with patients undergoing a limb-sparing procedure with similar tumors. Prevention of local recurrence by amputation also did not improve survival in this group compared with similar patients undergoing limb-sparing surgery who did develop a local recurrence. The group of patients with high-grade tumors 10 cm or larger who received chemotherapy did have a significant improvement in survival (p = 0.01) compared with a similar group of patients who did not receive chemotherapy, regardless of the type of operation. The prognosis of patients most likely to undergo an amputation for extremity soft tissue sarcoma (those with high-grade, large tumors) is not related to their local disease, but rather to the risk of distant metastases. Therefore, amputation in this cohort of patients can be recommended only when a limb-sparing procedure cannot achieve gross resection of tumor while still preserving a useful extremity, because amputation improves only local control and does not address distant disease. Further improvement in survival in this group of patients will be dependent on better systemic treatment for extremity soft tissue sarcoma, and not on more radical surgery.
Refinement of clinicopathologic staging for localized soft tissue sarcoma of the extremity: a study of 423 adults.
PURPOSE: The prognostic value of factors used in clinicopathologic staging of localized soft tissue sarcoma (STS) of the extremity were analyzed comprehensively. PATIENTS AND METHODS: Four hundred twenty-three patients with STS that was confined to the extremity were admitted to Memorial Sloan-Kettering Cancer Center from 1968 to 1978. Cox models for the hazards rates of tumor mortality, development of a distant metastasis, strictly local recurrence, and postmetastasis survival were developed. Tests of changes in the prognostic value of the important variables over time were performed, as well as an analysis of the effect of a local recurrence on the hazard rate of distant metastasis. RESULTS: Three unfavorable characteristics contained independent prognostic value for the rates of distant metastasis and tumor mortality: high grade (P less than .00001), deep location (P less than .0002), and size greater than or equal to 5 cm (P less than .007). Their Cox model coefficients did not differ significantly (P greater than or equal to .65); thus, a staging scheme based on the risk of ever developing a distant metastasis would assign equal prognostic weights to grade, depth, and size. The tumor grade effect during the initial 18 months was much larger in magnitude than those for depth and size, and its effect disappeared beyond that time (P = .0003). Thus, a staging scheme based on the risk of early metastatic spread would assign a distinctly larger prognostic weight to grade and lesser but equal weights to depth and size. There was no local recurrence effect on the rate of distant metastasis in the high-risk group (high grade, deep, and greater than or equal to 5 cm; P = .75), but there was a significant association among the remaining groups combined (P = .0039). The magnitude of this association actually increased according to the number of favorable characteristics presented (P = .0024). CONCLUSIONS: The refinement of clinicopathologic staging may depend on the choice of outcome variable: ultimate prognosis versus early metastatic spread. Additionally, the observed local recurrence effect may be explained by a tendency for some patients to acquire one or more unfavorable risk factors at the time of local recurrence.
Desmoid tumors of the chest wall. A locally recurrent problem.
Desmoid tumors, in general, are known for their propensity to recur. To evaluate treatment and results, we reviewed the records of 32 patients admitted to our institution with a chest wall desmoid tumor from 1948 to 1988. There were 13 men and 19 women whose ages ranged from 12 to 67 years (median 36 years). Four patients had Gardner's syndrome. Treatment was wide resection. Median follow-up was 110 months. The overall 5-year survival was 93%; 5-year disease-free survival was 71%. The 5-year local recurrence rate was 29%. Since death from disease is uncommon after resection, but local recurrence is common, we recommend aggressive wide resection in patients who have desmoid tumors of the chest wall when seen initially.
The changing role of amputation for soft tissue sarcoma of the extremity in adults.
The role of amputation in soft tissue sarcoma of the extremity has decreased at Memorial Sloan-Kettering Cancer Center during the last 20 years. In an attempt to determine the reasons for this change in therapy, an analysis of two separate databases involving 1,057 patients compiled during the periods of 1968 to 1978 and 1982 to 1990 was performed. The patients requiring amputation for soft tissue sarcoma of the extremity in the two databases (n = 233) were compared in an attempt to determine any significant differences between the two time periods. The groups were specifically compared for differences in risk factors, indications for amputations and the effect a decreasing incidence of amputation in the 1982 to 1990 group had on local recurrence and overall survival between the two groups. Despite similarity of risk factors and indications for amputation, the decreased incidence of amputation during the 1982 to 1990 period was associated with a significant decrease in local recurrence after amputation and no significant change in overall survival compared with the 1968 to 1978 group. Absence of local recurrence was associated with significant improvement in survival. Possible reasons for the shift in therapy, as well as the present and future role of amputation in soft tissue sarcoma of the extremity, are discussed.
Conservative management of anal leiomyosarcoma.
Leiomyosarcomas of the large intestine are unusual neoplasms, comprising less than 0.1% of all malignancies of the colon and rectum. Six cases of leiomyosarcoma of the anus have been reported. The optimal treatment for this neoplasm is not known. The standard surgical approach is abdominoperineal resection. The authors report the seventh case of this rare neoplasm and outline its treatment using local excision and iridium 192 brachytherapy in an attempt to preserve the anal sphincter. In selected patients, conservative surgery followed by radiation therapy may be an alternative to radical surgery, with the goals of local control of the disease and anal sphincter preservation. However, more experience is needed before this approach could be recommended routinely.
A prospective randomized trial of adjuvant chemotherapy with bolus versus continuous infusion of doxorubicin in patients with high-grade extremity soft tissue sarcoma and an analysis of prognostic factors.
A prospective randomized trial was conducted to compare the cardiotoxic and therapeutic effects of doxorubicin (60 mg/m2 every 3 to 4 weeks) administered by bolus or 72-hour continuous infusion as adjuvant chemotherapy in 82 eligible patients after resection of high-grade soft tissue sarcoma of the extremity or superficial trunk. Cardiac toxicity, defined as a 10% or greater decrease in left ventricular ejection fraction as assessed by radionuclide cineangiography, was evaluated in 69 patients. Cardiotoxicity was seen in 61% of patients in the bolus treatment arm with the median doxorubicin dose of 420 mg/m2. Among patients who received continuous infusion, 42% had cardiotoxicity with a median dose of 540 mg/m2. The rate of cardiotoxicity as a function of the cumulative dose of doxorubicin was significantly higher in the bolus treatment arm (P = 0.0017). Two patients in each group had clinical congestive heart failure, with one cardiac death occurring in each. There was a trend toward a lower rate of metastasis (P = 0.19) and a significantly lower rate of death of disease (P = 0.036) for patients treated with the bolus dose. Cox model analysis identified three unfavorable characteristics for the rate of developing a distant metastasis: blood transfusion within 24 hours of operation (P less than 0.00001), tumor deep to the fascia and 5 cm or more in size (P = 0.0043), and a histologic subtype other than liposarcoma (P = 0.0002). The unfavorable effect of continuous infusion was not selected in the model (P = 0.16). Adjuvant chemotherapy for patients with soft tissue sarcoma is investigational. Furthermore, the impact of perioperative blood transfusion merits further study.
Extracranial primitive neuroectodermal tumors. The Memorial Sloan-Kettering Cancer Center experience.
The clinical data of 54 patients (57% males) with extracranial primitive neuroectodermal tumors (PNET) seen over a 20-year period at Memorial Sloan-Kettering Cancer Center were reviewed. The age at diagnosis ranged from 1 month to 81 years (median, 17 years). One PNET arose in a previously irradiated site. One patient had an unaffected identical twin. Primary sites were thoracopulmonary (n = 25), pelvis (n = 12), retroperitoneum or abdomen (n = 10), limbs (n = 5), neck (n = 1), and unknown (n = 1). At diagnosis, epidural extension was present in 13 patients with truncal primaries, and 11 patients had distant metastases. All of the latter died with disease. Progression-free survival (PFS) among the 43 patients with localized tumors (all greater than 5 cm) was 25% at 24 months. Two of 13 patients who relapsed after more than 12 months without therapy were long-term survivors. Patients with localized PNET who had resection of all gross disease within 3 months of diagnosis had a significantly longer PFS (P = 0.0003). Radiation therapy caused tumor shrinkage but was not curative of measurable disease. A dose-response effect was evident with the most commonly used drug, cyclophosphamide. Myeloablative regimens using melphalan (n = 8) or thiotepa (n = 1) with autologous bone marrow rescue were not clearly beneficial. The treatment results favor: (1) early surgical removal, (2) dose-intensive use of drugs active against PNET (especially cyclophosphamide), and (3) radiation therapy to ablate residual microscopic disease.
Combined surgical resection and iridium 192 implantation for locally advanced and recurrent desmoid tumors.
Thirty-eight histologically confirmed desmoid tumors were treated with conservative surgical resection and interstitial iridium 192. Patients included in this series constituted a poor prognostic group. Seventy five percent (75%) had previous recurrences of their disease and 50% had tumors 8 cm or greater in size. In addition, 16% of the patients had gross residual disease after surgical excision and 79% had positive or close margins of resection. With a median follow-up of 5.5 years, the 5-year actuarial in-field local control rate was 75% and the overall local control rate was 66%. Four of the 12 local recurrences developed at the periphery of the implant volume and four patients failed at least 3 cm or more beyond the treated volume. After salvage therapy, the 5-year actuarial local control rate was 95%. Two patients required amputation for recurrent disease. Thirty of 34 patients remain free of disease with an excellent functional result achieved. The authors conclude that surgical resection and postoperative interstitial iridium 192 is effective therapy for patients with desmoid tumors. Despite the fact that most patients in this series had bulky, recurrent tumors, excellent long-term local control and limb function were achieved.
Prognostic factors in primary retroperitoneal soft-tissue sarcomas.
We analyzed independent treatment variables (age, sex, signs and symptoms, site, size, histopathologic findings, grade, and clinical presentation) and treatment-dependent variables (resectability, type of operation, surgical margins, surgical boundaries, microscopic margins, adjuvant radiotherapy, and adjuvant chemotherapy) in 80 patients with primary retroperitoneal soft-tissue sarcomas admitted from 1982 through 1988. Both univariate and multivariate analysis of survival and disease-free survival were performed. The major factor in survival outcome was the ability to completely resect the lesion. When the 62 patients who underwent complete resection were examined, the only independent prognostic factor for both survival and disease-free survival was grade. We conclude that completeness of resection and grade of the lesion are primary determinants of survival. Once all tumor is macroscopically removed, no advantage could be demonstrated by more extensive surgical resection or current adjuvant therapy.
Vascular soft-tissue sarcomas. An analysis of tumor-related mortality.
Between 1982 and 1990, 69 adult patients were admitted to Memorial Sloan-Kettering Cancer Center with a diagnosis of angiosarcoma, lymphangiosarcoma, or malignant hemangiopericytoma. The existing literature regarding sarcomas focuses on individual histologic conditions or site-specific activity and includes pediatric patients, which makes estimates of survival difficult. We describe the clinical course of all vascular sarcomas, the survival without distant recurrence of patients with vascular sarcomas, and the overall survival of patients with vascular sarcomas. Using Cox's stepwise regression model, histologic characteristics of the tumors, tumor grade, tumor size, and other factors were assessed to determine their prognostic significance. Noncurative treatment and the presence of metastases were the only two factors influencing survival. Tumor grade approached significance. Disease recurrence was common among curatively treated patients (37% of such patients), and more than half of these recurrences involved distant sites. The survival of these patients depends on complete surgical resection and is independent of individual histologic characteristics of the tumor.
The role of multimodality therapy in soft-tissue sarcoma.
Soft-tissue sarcomas are uncommon malignancies. The development during a period of 8 years, in one institution, of a prospective data base incorporating more than 1600 patients with these tumors is described. The most common sites for occurrence are the extremities, but they can occur in any of the soft tissues of the body. Liposarcoma and leiomyosarcoma are the most common histopathologic conditions identified. Prognostic factors for both recurrence and survival include site, histopathology, size, grade, and adequacy of resection. A prospective randomized trial of the use of adjuvant radiation by the brachytherapy technique in extremity lesions has shown a decrease in local recurrence, but no impact on survival. Eligible patients not randomized to the trial show no difference in local recurrence or survival, regardless of whether they received adjuvant radiation.
Hemorrhage simulating tumor growth in malignant fibrous histiocytoma at MR imaging.
During the course of preoperative chemotherapy for treatment of malignant fibrous histiocytoma of the lower extremity, the mass in three patients was found to be enlarged at physical examination. Magnetic resonance (MR) imaging demonstrated, and subsequent pathologic examination of resected specimens proved, that the enlargement was caused by extensive hemorrhage within the masses, rather than by tumor growth. MR imaging can demonstrate this phenomenon well, particularly on T1-weighted images.