Surgery for soft tissue sarcoma in the extremities. A multivariate analysis of the 6-26-year prognosis in 137 patients.

In 137 patients with soft tissue sarcomas in the extremities, the influence of patient and tumor characteristics and surgical procedures on prognosis was studied using a multivariate statistical analysis. The minimum follow-up time was 6 years, and no patient was lost to follow-up. Eighty-nine patients were referred with the tumor intact (primary series), while 48 were referred after biopsy or with local recurrence after previous surgery (secondary series). The frequency of amputation was 15 percent in the primary series and 48 percent in the secondary series. A local, function-preserving operation with a wide margin was performed in 37 patients without biopsy (clinical diagnosis alone). Local control of the disease was obtained in all but one of these patients without any adjunctive treatment. Independent, unfavorable factors concerning local recurrence were advancing age, open biopsy, and marginal surgery. Independent, unfavorable factors concerning survival were advancing age, increasing histologic malignancy grade, and ablative surgery. A tumor-related death was observed in 0, 29, 47, and 67 percent of the patients with tumors of malignancy grades I, II, III, and IV, respectively.

Surgical margin in soft tissue sarcoma. The Scandinavian Sarcoma Group experience.

Two-hundred and forty adult patients with a high-grade soft tissue sarcoma were treated surgically in 18 hospitals participating in the Scandinavian Sarcoma Group Protocol I. The patients were randomized to either postoperative doxorubicin or control; patients whose surgical margin was judged marginal also received radiotherapy. The outcome after different surgical margins was analyzed in 185 tumors of Grades III or IV in the extremities. The total cumulative local tumor control was 91 percent (168 of 185) after a median of 47 months. The cumulative local control rates in the surgical groups were: compartmental or wide amputation--37/37 (100 percent), compartmental local excision--23/24 (96 percent), wide local excision--77/84 (92 percent), marginal excision and radiotherapy--19/21 (90 percent), and marginal excision alone (reevaluated margin)--12/19 (63 percent, significantly lower than others). The risk of local recurrence was 13 times higher after marginal than after compartmental surgery (P = 0.02) and 3 times higher if the tumor was larger than 10 cm (P = 0.05). The treatment with doxorubicin did not influence the risk of local recurrence. The survival rates did not differ significantly in the groups.

Complete removal of vertebrae for extirpation of tumors. A 20-year experience.

Long-term results of complete removal of vertebrae with a minimum follow-up period of seven years are reported in 23 consecutive patients from March 1968 to January 1981. Seven patients were treated with vertebrectomy above the sacrum: three for a giant-cell tumor (T11, T12, and L1; T11; and L4), one for chondrosarcoma (one-half T6, T7, and one-half T8), one for chordoma (L3), one for plasmocytoma (L1), and one for a metastasis of renal carcinoma (L1). The latter two patients eventually died of generalized disease, whereas the other five patients have no evidence of tumor after seven to 20 years. In the six patients in whom the spine was reconstructed using corticocancellous iliac bone, a block-vertebra was created by the grafts and the adjacent vertebrae, allowing two women to complete one or more pregnancies successfully. Sixteen patients were treated with removal of sacral vertebrae (from one-half S3, S4, and S5 to all). None of the seven patients treated with adequate surgery for a sacral chordoma has had a local recurrence. Two women have given birth to children after sacral amputations, one performed for rhabdomyosarcoma and the other for a huge ganglioneuroma. A patient treated with a hemicorporectomy for chondrosarcoma 18 years ago has no evidence of tumor.

Primary leiomyosarcoma of bone. A clinical, radiographic, pathologic-anatomic, and prognostic study of 16 cases.

Sixteen cases of primary leiomyosarcoma of bone are described. The patients, 11 males and 5 females, ranged in age from 9 to 74 years. The annual incidence of this tumor in Sweden was calculated to be 0.09 cases per million. This figure was obtained by reviewing a Swedish series of spindle cell sarcomas of bone of which one quarter (11/44) were diagnosed by us as primary leiomyosarcoma. The diagnosis was based on light- and electron-microscopic examinations using the same criteria as for leiomyosarcoma of soft tissues. Thirteen tumors were located in a long bone of an extremity (nine close to the knee joint) and three in the central skeleton. Radiographically, all the tumors presented as a purely osteolytic lesion, and three patients had sustained a pathologic fracture. In four of six cases angiography suggested malignancy by revealing hypervascularity, irregular tortuous vessels, and diffuse contrast opacification. Contrast-enhanced computed tomography, performed in two cases, showed hypervascular areas within the tumors. Scintigraphy showed a marked increase in radionuclide uptake in all five cases studied. The clinical behavior indicates that primary leiomyosarcoma of bone is highly malignant. Eight patients had died of the tumor and, of the eight patients who were alive at follow-up, two had metastases, and one had been operated on three times for a cutaneous metastasis, which had recurred locally twice. The remaining five patients had been continuously free of disease for 6.5 to 12.3 years.

The diagnosis and prognosis of soft tissue tumors.

In this review, we present our experience on the clinical, radiographic, and morphologic diagnosis of soft tissue tumors gathered over a quarter of a century. Included is a brief presentation of our principles for surgical therapy and the results of multivariate statistical analysis performed to assess which clinical and morphologic variables determine prognosis.

Accessory soleus muscle. A clinical and radiographic presentation of eleven cases.

When an accessory soleus muscle is present, it consists of a soft-tissue mass bulging medially between the distal part of the tibia and the Achilles tendon. It usually inserts with a separate tendon on the calcaneus anteromedial to the Achilles insertion, and may be a cause of pain on exercise. One may suspect a soft-tissue tumor, such as lipoma, hemangioma, and even sarcoma, but the anomalous muscle has a typical appearance on plain radiographs, and the appearance on computed tomography is diagnostic. If the patient is asymptomatic, no therapy is required, but if pain or other discomfort is provoked by exercise, exploration with fasciotomy or excision of the accessory muscle is recommended, as was done in six of our eleven patients who were seen between 1968 and 1985.

Extra-abdominal desmoid tumors.

A follow-up study of 53 patients with extra-abdominal desmoid tumor is presented. Fine-needle aspiration biopsy seemed to be valuable in the differentiation between a desmoid tumor and a truly malignant soft tissue tumor. The angiographic pictures did not differ from those of other soft tissue tumors, but valuable topographic information was obtained from the angiography in the preoperative planning. The overall local recurrence rate was 17/44, and 15 of the recurrences occurred within the first 2 years. Complete removal of the affected muscle or muscle group is recommended. Local control can be achieved by non-ablative surgery even after multiple recurrences. Only one out of 53 patients died of the tumor disease. Radiotherapy can be attempted in extensive tumors, and amputation should be reserved for tumors which have failed to respond to this treatment.

Vascularization of peripheral neurilemomas: angiographic, computed tomographic, and histologic studies.

The vascularization of peripheral neurilemoma was studied in 17 patients, in eight by angiography and in 16 by computed tomography (CT) using contrast enhancement. Angiographically the degree of vascularity varied from scarcity to abundance of vessels. In two patients, arteries connected to the proximal and distal poles of the tumor could be identified as nutrient arteries of the affected nerve, and in three patients some of the veins draining the tumor followed the course of the nerve. All 16 tumors examined by CT showed lower density than skeletal muscle. They were well delineated and at least partially surrounded by adipose tissue. With contrast enhancement the density increased focally, reflecting the distribution of vessels in the tumors. Histologic examination showed that Antoni-A tissue was rich in small (below 15 micron) and medium-sized vessels, whereas large vessels (above 100 micron) were more frequently seen in Antoni-B tissue. Medium-sized and large vessels often tended to be arranged along the border between Antoni-A and Antoni-B tissues.

Scapulectomy for malignant tumor. Function and shoulder strength in five patients.

Function and muscular strength were studied in five patients who had undergone either total or subtotal scapulectomy for malignant tumor. We found a striking difference in the functional impairment between the two patients with total and the three patients with subtotal scapulectomy. The latter three patients were able to handle light objects with their hands over their heads, and reached or nearly reached the horizontal plane in flexion and abduction of the shoulder. The two patients with total scapulectomy had a flexion and abduction range between 40 and 50 degrees. The mean isometric muscle strength in flexion relative to the non-operated side was 17 and 37 per cent for patients with total and subtotal scapulectomy, respectively, and the abduction strength 14 and 44 per cent, respectively. After scapulectomy, a reasonably good function can be expected, especially if it is possible to preserve the glenoid fossa and/or the acromion.

Atypical hemangioendothelioma of venous origin. A clinicopathologic, angiographic, immunohistochemical, and ultrastructural study of two endothelial tumors within the concept of histiocytoid hemangioma.

Two patients, a 62-year-old man and a 50-year-old woman, both with deep-seated atypical endothelial tumors within the wide concept of histiocytoid hemangioma, are reported. In case 1, the tumor involved the brachial vein, and, in case 2, a medium-sized vein of the anterior neck. In both cases the involved vein was occluded. Angiography in case 1 suggested a tumor that was enclosed by the same fibrous sheath, the conjunctiva vasorum, that enclosed the occluded vein and its concomitant artery. Both tumors were solid, without conspicuous vascular differentiation by light microscopy. Such differentiation, however, was evident from the electron-microscopic examination, which showed tumor cells with endothelial features forming primitive vascular structures. Positive lectin histochemistry (Ulex Europeus I) and positive immunohistochemistry for factor-VIII-related antigen, actin, and vimentin also gave strong support for the endothelial differentiation of the tumor cells. Immunohistochemical studies of markers for histiocytic (alpha 1-antitrypsin, ferritin, lysozyme), epithelial (cytokeratin, epithelial membrane antigen), and neuroectodermal (S-100 protein) and skeletal muscle (myoglobin) differentiation were negative. At follow-up, after 7 years and 2 years, respectively, there were no signs of local recurrence or metastasis.

Shoulder function after deltoid muscle removal.

Postoperative function and muscular strength of the shoulder joint were studied in five patients who had undergone extirpation of the deltoid muscle because of a soft tissue tumor. No patient had significant functional impairment but all had a slightly limited range of active abduction and flexion. The strength reduction did not exceed 40 per cent in any position.

Musculoskeletal tumor surgery in Göteborg.

Careful preoperative planning is of great importance for successful surgical treatment of soft-tissue sarcomas. All means available should be used for determining as exactly as possible the location and extension of the tumor. Based on the observation that malignant soft-tissue tumors usually respect preformed fibrous barriers until a late stage, special surgical techniques have been devised. Some muscles in the body are dispensable and can be removed without significant loss of function. For assessing the prognosis after surgical treatment of soft-tissue sarcomas, a nonparametric multivariate analysis and a long follow-up period are essential. Seven cases of total spondylectomy (removal of one or several entire vertebrae), three cases of extended hemipelvectomy (parts of the adjacent spine included in the specimen), and one case of lumbosacral amputation (hemicorporectomy) are briefly reviewed with recent follow-up data.

Leiomyosarcomas of venous origin in the extremities. A correlated clinical, roentgenologic, and morphologic study with diagnostic and surgical implications.

This article presents a correlated clinical, roentgenologic, and morphologic study of six cases of leiomyosarcoma of large veins in the extremities. Growth of the tumor within the lumen of the affected vein was demonstrated by phlebography in one case and by angiography and computed tomography in one case. In all six cases, such growth was demonstrated by dissection of the surgical specimen. In four cases, arteriography showed both neovascularity at the site of the lesion and compression of the artery accompanying the affected vein. The compression apparently was caused by entrapment of the artery within the same preformed fibrous sheath (conjunctiva vasorum) that enclosed the tumor and the vein from which it had arisen. The histologic diagnosis was based on the light microscopic appearance in all cases and was supported by electron microscopic findings in three cases by the demonstration of ultrastructural features of leiomyoblasts. All six tumors were light microscopically of high-grade malignancy. Five of the patients died of metastatic tumor disease and one is alive with lung metastases. The information obtained by a careful physical examination combined with phlebography, arteriography, and computed tomography may be helpful in the clinical diagnosis and the planning of adequate surgical treatment.

Surgical removal of bone and muscle metastases of renal cancer.

Fifteen men and six women with renal cancer underwent surgical removal of metastatic lesions in bone (19 patients) or muscle (two patients). The operation was carried out 2 years before nephrectomy/renal resection in two patients, on the same occasion in four, and 1-196 months after in 15. Surgical interventions of various kinds were undertaken, resulting in the loss of a lower limb in seven patients and an upper limb in one. The observed 5-year survival was 4 out of 10. Six patients were alive at follow-up, five of them without evidence of disease. Eight of the remaining 15 patients died of an unrelated disease (five without evidence of tumor); the other seven patients died of metastatic tumor disease. Local recurrence was diagnosed, and removed, in two patients. The results compare favourably with reports on surgically removed pulmonary metastases of renal cancer and seem to justify an aggressive attitude towards solitary bone and muscle metastases of renal cancer.

A multivariate analysis of the prognosis after surgical treatment of malignant soft-tissue tumors.

Ninety-seven patients who had received their primary and definitive surgical treatment for a soft-tissue sarcoma during the years 1956--1976 were studied with respect to local recurrence, metastasis, and survival. The aim of the treatment was to eradicate the tumor while preserving good function. The influence of host and tumor properties and different diagnostic and surgical procedures on the prognosis was studied by means of nonparametric multivariate analysis. Ninety-four percent of the tumors were located in the extremities. Malignant fibrous histiocytoma was the most common histologic type. A four-grade scale of histologic malignancy was used. Eighty-eight percent of the tumors were Grade III or IV. In 85% of the patients with an extremity lesion, local extirpation was carried out. The overall local recurrence rate was 21.7% and in 76 patients who underwent an adequate surgical procedure it was 6.6%. The overall five-year survival rate was 59%. The survival depended on the local control of the primary tumor, which was related to the adequacy of the surgical procedure and to the histologic grade of malignancy. No patient with a Grade I or II tumor died. The five-year survival rate for patients with Grade III tumors was 68% and for patients with Grade IV tumors 47%.

Function after removal of various hip and thigh muscles for extirpation of tumors.

Forty-six patients who had undergone excision of one or more well defined hip and/or thigh muscles because of a soft-tissue tumor or a tumoriform lesion were investigated with respect to the function of the operated limb and the isometric and isokinetic strength of the affected motion or motions, relative to the non-operated side (percentage). Hip flexion: Loss of the iliopsoas caused slight impairment of function. The flexion strength decreased with increasing flexion of the hip joint. Loss of the rectus femoris reduced the isometric strength by 37 and the isokinetic strength by 17 per cent. Hip abduction: The strength reduction was only about 50 per cent and the impairment of function only slight or moderate even in patients with extensive loss of abductor muscles. Hip adduction: Removal of all three prime adductors (longus, brevis, magnus) caused a strength reduction of about 70 per cent but the impairment of function was only slight or moderate. Hip extension: Loss of the gluteus maximus caused only a small strength reduction and no impairment or only slight impairment of function. Significant strength reduction was only seen when all hamstrings had been removed. Knee extension: Loss of one, two, and three of the quadriceps muscles reduced the isometric strength by 22, 33, and 55 per cent, respectively. The isokinetic strength was reduced somewhat more. The strength reduction usually had to exceed 50 per cent to cause more than slight impairment of function. Knee flexion: Loss of the semitendinosus, the biceps femoris, and all the hamstrings reduced the isometric strength by 24, 28, and 67 per cent, respectively. The isokinetic strength was reduced somewhat less. Loss of one of the hamstrings usually caused no impairment of function whereas loss of all three resulted in moderate impairment of function.