A review was done of 120 cases of malignant peripheral nerve sheath tumor (MPNST) seen during a 71-year period. Of the 120 patients, 52 were males and 68 were females with a mean age at diagnosis of 35.3 years; 12 patients were younger than 20 years. The series included 62 (52%) patients with neurofibromatosis, 13 (11%) with postradiation sarcomas, and 19 (16%) with metaplastic foci. The incidence of MPNST arising in neurofibromatosis was 4.6% in the current series and 0.001% in the general clinic population. Tumors greater than 5 cm and the presence of neurofibromatosis adversely affected the prognosis (P less than 0.05). When both features were present, survival was greatly decreased. Patients with tumor in the extremities did better than those with head or neck lesions. Metaplastic foci or previous radiation at the tumor site did not alter the prognosis. Each tumor was graded 1 to 4 on the basis of cellularity, pleomorphism, mitotic index, and necrosis. No significant correlation was noted between survival and either grade or mitotic rate. Survival was improved when total rather than subtotal resection was done. This was most marked in patients with a small lesion, which may reflect the difficulty in adequately excising large tumors. Adjuvant radiation or chemotherapy did not appear to affect survival. The MPNST is an aggressive uncommon neoplasm, and large tumor size, the presence of neurofibromatosis, and total resection are the most important prognostic indicators.

Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases.

PURPOSEThis randomized, prospective study assesses the impact of postoperative external-beam radiation therapy on local recurrence (LR), overall survival (OS), and quality of life after limb-sparing resection of extremity sarcomas.PATIENTS AND METHODSPatients with extremity tumors and a limb-sparing surgical option were randomized to receive or not receive postoperative adjuvant external-beam radiotherapy. Patients with high-grade sarcomas received postoperative adjuvant chemotherapy whereas patients with low-grade sarcomas or locally aggressive nonmalignant tumors were randomized after surgery alone.RESULTSNinety-one patients with high-grade lesions were randomized; 47 to receive radiotherapy (XRT) and 44 to not receive XRT. With a median follow-up of 9.6 years, a highly significant decrease (P2 = .0028) in the probability of LR was seen with radiation, but no difference in OS was shown. Of 50 patients with low-grade lesions (24 randomized to resection alone and 26 to resection and postoperative XRT), th...

Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity.

The pathological features of 155 adult patients with soft-tissue sarcomas were studied retrospectively, in an attempt to set up a grading system for these tumors. As the first step, seven histological criteria (tumor differentiation, cellularity, importance of nuclear atypia, presence of malignant giant cells, mitosis count, pattern of tumor necrosis and presence of vascular emboli) were evaluated in a monofactorial analysis. Five of these (tumor differentiation, cellularity, mitosis count, tumor necrosis, and vascular emboli) were correlated with the advent of metastases and with survival. A multivariate analysis, using a Cox model, selected a minimal set of three factors (tumor differentiation, mitosis count, and tumor necrosis) the combination of which was necessary and sufficient to retain all the prognostic information. A grading system was elaborated, which turned out to be correlated with the advent of metastasis and with patients' survival. A second multivariate analysis introducing clinical prognostic features showed that the histological grade was the most important prognostic factor for soft-tissue sarcomas. Thus, this grading system appears to be highly interesting because of its prognostic value and the facility of its elaboration. However, its reproducibility should be tested.

Soft‐tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system

Percutaneous vertebroplasty with polymethylmethacrylate. Technique, indications, and results.

OBJECTIVE: To analyze treatment and survival of a large cohort of patients with retroperitoneal soft-tissue sarcomas (STS) treated and prospectively followed at a single institution. SUMMARY BACKGROUND DATA: Retroperitoneal STS are relatively uncommon and constitute a difficult management problem. Although surgical resection is often difficult or impossible, current chemotherapy is not effective and radiation is limited by toxicity to adjacent structures. Thus, complete surgical resection remains the most effective modality for selected primary and recurrent disease. METHODS: Five hundred patients with retroperitoneal STS were admitted and treated between July 1, 1982, and September 30, 1997, and prospectively followed. Patient, tumor, and treatment variables were analyzed for disease-specific and disease-free survival. Survival was determined with the Kaplan-Meier method. Statistical significance was evaluated using the logrank test for univariate influence and Cox model stepwise regression for multivariate influence. RESULTS: Two hundred seventy-eight patients (56%) had primary disease and 222 (44%) recurrent disease. Median follow-up was 28 months (range 1 to 172 months), 40 months for survivors. Median survival was 72 months for patients with primary disease, 28 months for those with local recurrence, and 10 months for those with metastasis. For patients with primary or locally recurrent tumors, unresectable disease, incomplete resection, and high-grade tumors significantly reduced survival time. CONCLUSIONS: In this study of patients with retroperitoneal STS, stage at presentation, high histologic grade, unresectable primary tumor, and positive gross margin are strongly associated with the tumor mortality rate. Patients approached with curative intent should undergo aggressive attempts at complete surgical resection. Incomplete resection should be undertaken only for symptom relief.

Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution.

From January 1963 through December 1977, 300 adults with soft-tissue sarcomas were treated by a conservative surgical excision and postoperative radiotherapy. The absolute two- and five-year disease-free survival rates are 74% (222/300) and 61.3% (103/168), respectively. The five-year survival rate varies with: (1) anatomic site, e.g., 69.4% (75/108) for extremity lesions vs. 33% (5/15) for abdominal lesions; (2) histopathologic diagnosis, e.g., 86.4% (19/22) for fibrosarcoma vs. 50% (15/30) for neurofibrosarcoma; and (3) stage of the lesion. The overall local recurrence rate was 22.3% (67/300) and 27% (81/300) of the patients developed distant metastases. The incidence of lymph node metastases as an initial site of spread was only 2.7% (8/300); therefore, elective treatment of the regional lymphatics is not indicated. The 6.5% incidence of significant complications in extremities is low and might be further diminished by careful treatment planning. The combination of conservative surgery and postoperative radiation therapy maintains a functional limb in 84.5% (169/200) of patients with extremity lesions. This rate is comparable to the five-year survival rate attained with radical surgery.

Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas

The charts of 5,019 previously untreated patients with squamous cell carcinoma of the upper respiratory and digestive tracts who completed treatment for cure from January 1948 through August 1973, were reviewed. These patients had no evidence of distant metastases when initially evaluated. Five hundred and forty-six patients developed clinical evidence of distant metastases. The overall incidence of distant metastases was 10.9%, varying from 3.1% for vocal cord cancers to 28.1% for cancer of the nasopharynx. The lungs and bones were the most common first sites of metastases, accounting for 52% and 20.3% respectively, whereas metastases to the mediastinum (2.9%) were rare. Forty-eight percent of the metastases were detected within nine months after treatment and 80% were detected within two years. The rate of distant metastases increased with the stage (2% for Stage I to 19.5% for Stage IV). The rate also increased with the T and N classification; however, the N stage had greater influence on the rate of metastases than the T stage. The incidence of distant metastases was significantly higher when there was a recurrence above the clavicles (16.7%) than when there was no recurrence (7.9%, less than 0.001). In patients whose primary lesion was treated by radiotherapy or surgery alone, the incidence was essentialy the same. Patients receiving postoperative irradiation had double the incidence of the preoperative group (20.1% vs 9.9%--p less than .005); however, the sequence of modalities was not randomized.

An analysis of distant metastases from squamous cell carcinoma of the upper respiratory and digestive tracts.

Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMPC) are unusual solitary tumors of plasma cell origin. The clinical differences between these tumors and multiple myeloma are the subject of continued study. This review examines the radiotherapy experience at M. D. Anderson Hospital. Between 1948 and 1977, 12 patients with SPB and 12 patients with EMPC were treated with radiotherapy. Radiotherapeutic doses were most often (22/24) greater than 4,000 rads, at 200 rads per day. In order to qualify as solitary plasmacytoma a disease-free interval of 3 years was required. In the group of SPB, 5 patients of 9 were alive with no evidence of disease (NED) at 3 years, 4 of 5 at 5 years, and none of 4 at 10 years. In the EMPC group, 8 patients of 11 were NED at 3 and 5 years, and 6 of 9 at 10 years. Six patients with SPB developed multiple myeloma compared with only two with EMPC. The results confirm the better prognosis of EMPC and support the theory that SPB and EMPC are two different entities.

Solitary plasmacytoma of bone vs. extramedullary plasmacytoma and their relationship to multiple myeloma.

In 202 patients with rhabdomyosarcoma of the head and neck who registered in the first Intergroup Rhabdomyosarcoma Study, the primary lesions arose about the eye and orbit in 26%, in parameningeal sites in 46%, and in other head and neck areas in 28%. Histopathologically, 78% were embryonal-botryoid, 9% alveolar, 10% undifferentiated, and 3% extraosseous Ewing's types. Actual three-year relapse-free survival rates were calculated from data on 103 of these patients who were free of distant metastases at diagnosis and in whom follow-up had been completed for a three-year period. The actual relapse-free survival rates were 91% (21/23) for those with eye/orbit primaries, 46% (20/44) for those with parameningeal primaries, and 75% (27/36) for those with other head and neck sites affected. Among those with no clinical evidence of tumor activity at two years, 8% (6/75) had subsequent relapses.

Three-year relapse-free survival rates in childhood rhabdomyosarcoma of the head and neck: report from the Intergroup Rhabdomyosarcoma Study.

The experience at M. D. Anderson Hospital in the management of 59 patients with apparently localized Ewing's sarcoma from 1948 to May, 1972 is presented. Necropsy examination was performed in 18 cases. Disease-free survival times are compared between 19 patients treated by the combination of high-dose irradiation and intensive chemotherapy, and 40 patients treated by radiation therapy alone (25) or in combination with relatively less intensive drug therapy (15). Results favor the former group: local recurrence in 1 case of 19 compared to 19 of 40; and median time to first metastasis of >15 months compared to 8 months.

https://acsjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%28197407%2934%3A1%3C143%3A%3AAID-CNCR2820340121%3E3.0.CO%3B2-5

Robert D. Lindberg

Papers

Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas

An analysis of distant metastases from squamous cell carcinoma of the upper respiratory and digestive tracts.

Solitary plasmacytoma of bone vs. extramedullary plasmacytoma and their relationship to multiple myeloma.

Three-year relapse-free survival rates in childhood rhabdomyosarcoma of the head and neck: report from the Intergroup Rhabdomyosarcoma Study.

Localized Ewing's sarcoma--treatment and results.