Örjan Berlin

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.

Monitoring referral and treatment in soft tissue sarcoma: Study based on 1,851 patients from the Scandinavian Sarcoma Group Register

This report is based on 1.851 adult patients with soft tissue sarcoma (STS) of the extremities or trunk wall diagnosed between 1986 and 1997 and reported from all tertiary referral centers in Norway and Sweden. The median age at diagnosis was 65 years and the male-to-female ratio was 1.1:1. One third of the tumors were subcutaneous, one third deep, intramuscular and one third deep, extramuscular.The median size was 7 (1-35) cm and 75% were high grade (III-IV). Metastases at presentation were diagnosed in 8% of the patients. Two thirds of STS patients were referred before surgery and the referral practices have improved during the study. The preoperative morphologic diagnosis was made with fine-needle aspiration cytology in 81%, core-needle biopsy in 9% and incisional biopsy in 10%. The frequency of amputations has decreased from 15% in 1986-88 to 9% in 1995-1997. A wide surgical margin was achieved in 77% of subcutaneous and 60% of deep-seated lesions. Overall, 24% of operated STS patients had adjuvant radiotherapy. The use of such therapy at sarcoma centers increased from 20% 1986-88 to 30% in 1995-97. Followup has been reported in 96% of the patients. The cumulative local recurrence rate was 0.20 at 5 years and 0.24 at 10 years. The 5-year metastasis-free survival rate was 0.70.

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 ultrastrucctural 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.

Clinical course of nonvisceral soft tissue leiomyosarcoma in 225 patients from the Scandinavian Sarcoma Group.

Leiomyosarcoma of nonvisceral soft tissues is an uncommon malignant tumor; thus, only small numbers of cases have been reported. This study was based on a large series of patients from the Scandinavian Sarcoma Group Register acquired during a 15‐year period (from 1986 to 2001). Follow‐up information was available for all patients.

Primary leiomyosarcoma of bone: A study of five cases

Five canes of leiomyosarcoma of bone are described. The patients, 3 men and 2 women, ranged in age from 24 to 74 years. Four of the five tumors were located in the distal half of the femur. Radiographically, all tumors presented as purely osteolytic lesions. Angiography, performed in 3 cases, revealed hypervascularity and the presence of irregular, tortuous vessels at the site of the lesion in 1, but not in 2 others. Histologically, the five tumors showed features characteristic of leiomyosarcomas. The most prominent features were blunt‐ended, cigar‐like, chromatin‐rich nuclei in elongated acidophilic cells arranged in bundles which intersected each other at wide angles. The importance of trichrome stains, such as van Gieson stain, for the recognition of leiomyosarcomas is emphasized. Electron microscopic examination, performed in 3 cases, showed as the most prominent features spindle‐shaped cells in parallel arrangement containing abundant myofilaments with elongated densities, pinocytotic vesicles, and basal lamina structures.

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.

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.

Consequences of local recurrence of soft tissue sarcoma : 205 patients from the Scandinavian Sarcoma Group Register

From the Scandinavian Sarcoma Group Register, information on 1,224 surgically-treated patients with soft tissue sarcoma (STS) of the extremity or trunk wall, diagnosed between 1987 and 1995, was collected. 205 patients, one third of whom were referred to a center with a local recurrence, had a total of 284 local recurrences. This analysis describes the treatment for these local recurrences, complications and risk of further recurrences. 169 patients were surgically treated for their first local recurrence. An intralesional or marginal margin was achieved in 110 of these patients, 59 of whom were also given radiotherapy. 54 of the 169 patients had a second local recurrence. The second local recurrence rate was 0.50 if the first local recurrence had been treated with only surgery with a marginal margin, compared to 0.28 if treated with surgery with a marginal margin and radiotherapy or with a wide margin (p = 0.0008). In extremity STS, the crude amputation rate for local recurrences was 0.22 (31 of 142)--i.e., higher than for primary tumors 0.09 (96 of 1065) (p < 0.0001). A high local recurrence rate after treatment outside of sarcoma centers has earlier been shown. We conclude that the consequences of local recurrence in terms of morbidity and costs justifies referral of STS patients for multidisciplinary evaluation and multimodality treatment.

Primary leiomyosarcoma of bone

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.

Implant Survival, Adverse Events, and Bone Remodeling of Osseointegrated Percutaneous Implants for Transhumeral Amputees

BackgroundOsseointegrated percutaneous implants provide direct anchorage of the limb prosthesis to the residual limb. These implants have been used for the rehabilitation of transhumeral amputees in Sweden since 1995 using a two-stage surgical approach with a 6-month interval between the stages, but results on implant survival, adverse events, and radiologic signs of osseointegration and adaptive bone remodeling in transhumeral amputees treated with this method are still lacking.Questions/purposesThis study reports on 2- and 5-year implant survival, adverse events, and radiologic signs of osseointegration and bone remodeling in transhumeral amputees treated with osseointegrated prostheses.MethodsBetween 1995 and 2010, we performed 18 primary osseointegrated percutaneous implants and two implant revisions in 18 transhumeral amputees; of those, 16 patients were available for followup at a minimum of 2 years (median, 8 years; range, 2–19 years). These include all transhumeral amputees who have received osseointegrated prostheses and represented approximately 20% of the all transhumeral amputees we evaluated for potential osseointegration during that time; general indications for this approach included transhumeral amputation resulting from trauma or tumor, inability to wear or severe problems wearing a conventional socket prosthesis, eg, very short residual limb, and compliant patients. Medical charts and plain radiographs were retrospectively evaluated.ResultsThe 2- and 5-year implant survival rates were 83% and 80%, respectively. Two primary and one revised implant failed and were removed because of early loosening. A fourth implant was partially removed because of ipsilateral shoulder osteoarthritis and subsequent arthrodesis. The most common adverse event was superficial infection of the skin penetration site (15 infections in five patients) followed by skin reactions of the skin penetration site (eight), incomplete fracture at the first surgery (eight), defective bony canal at the second surgery (three), avascular skin flap necrosis (three), and one deep implant infection. The most common radiologic finding was proximal trabecular buttressing (10 of 20 implants) followed by endosteal bone resorption and cancellization (seven of 20), cortical thinning (five of 20), and distal bone resorption (three of 20).ConclusionsThe implant system presented a survivorship of 83% at 5 years and a 38% 5-year incidence of infectious complications related to the skin penetration site that were easily managed with nonoperative treatment, which make it a potentially attractive alternative to conventional socket arm prostheses. Osseointegrated arm prostheses have so far only been used in transhumeral amputations resulting from either trauma or tumor. Their use has not been tested and is therefore not recommended in transhumeral amputations resulting from vascular disease. This method could theoretically be superior to socket prostheses, especially in transhumeral amputees with very short residual humerus in which the suspension of a conventional prosthesis is difficult. Comparative studies are needed to support its potential superiority. Moreover, the radiological findings in this study need to be followed over time because some of them are of uncertain long-term clinical relevance.Level of Evidence Level IV, case series. See Guidelines for Authors for a complete description of levels of evidence.