Fibrosarcoma of bone. A demographic, clinical and histopathological study of all cases recorded in the Swedish cancer registry from 1958 to 1968.

The clinical records, radiographs and histopathological material of all forty-one patients recorded as suffering from fibrosarcoma of bone in the Swedish Cancer Registry for the years 1958 to 1968 have been analysed; in addition, four cases were found on histological review of a series of osteosarcomas. From this re-examination, twenty-four patients with genuine fibrosarcoma of bone were identified; twenty-two had primary neoplasms and two secondary. No sex or geographical differences were found. The tumours showed a prevalence for patients in the adult and older age groups. Half of the tumours arose from femoral or tibial metaphysial bone adjacent to a knee joint. All the tumours were of the medullary type. Almost one-third presented with a pathological fracture, and soft-tissue extension had occurred in all but three tumours. In contrast to previous reports, these tumours were more malignant than osteosarcomas and showed a five-year survival rate of only 4-2 per cent. In accessible sites, ablative surgery was used as the primary treatment, Fibrosarcoma of bone is a distinctive lesion and should be distinguished carefully from periosteal and soft-tissue fibrosarcomas because of differences in prognosis and treatment.     

Osteosarcoma: A Multifactorial Clinical and Histopathological Study with Special Regard to Therapy and Survival

A multifactorial analysis was performed on all 153 unequivocal cases of genuine osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968. Cases of so-called parosteal osteosarcoma, soft-tissue osteosarcoma and osteosarcoma secondary to Paget's disease of bone were not included. The osteosarcomas were subclassifiecl as follows: osteoblastic (69 per cent), chondroblastic (19 per cent) and fibroblastic (12 per cent). The overall 5-year survival rate was 22 per cent; 55 per cent for those who had undergone amputation above the joint proximal to the involved skeletal part, 22 per cent for those amputated on the involved skeletal part, 11 per cent for those treated with local extirpation of the tumor, and 1 per cent in cases in which the lesion was not radically removed. Tumors of the femur, humerus and scapula were as malignant as axial tumors. The former carried a 5-year survival rate of 13 per cent, regardless of whether the patients had been treated with exarticulation or amputation on the involved skeletal part. Patients with axial tumors showed a 5-year survival rate of 15 per cent. These survival data suggest that proximal amputation alone might suffice for lesions situated distally to the knee and elbow joints, while tumors in the humerus and femur should be treated with amputation combined with multicytostatic treatment or immunotherapy and axial tumors with local resection and multicytostatic or immunologic treatment.     

Osteosarcoma. A multifactorial clinical and histopathological study with special regard to therapy and survival.

A multifactorial analysis was performed on all 153 unequivocal cases of genuine osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968. Cases of so-called parosteal osteosarcoma, soft-tissue osteosarcoma and osteosarcoma secondary to Paget's disease of bone were not included. The osteosarcomas were subclassified as follows: osteoblastic (69 per cent), chondroblastic (19 per cent) and fibroblastic (12 per cent). The overall 5-year survival rate was 22 per cent; 55 per cent for those who had undergone amputation above the joint proximal to the involved skeletal part, 22 per cent for those amputated on the involved skeletal part, 11 per cent for those treated with local extirpation of the tumor, and 1 per cent in cases in which the lesion was not radically removed. Tumors of the femur, humerus and scapula were as malignant as axial tumors. The former carried a 5-year survival rate of 13 per cent, regardless of whether the patients had been treated with exarticulation or amputation on the involved skeletal part. Patients with axial tumors showed a 5-year survival rate of 15 per cent. These survival data suggest that proximal amputation alone might suffice for lesions situated distally to the knee and elbow joints, while tumors in the humerus and femur should be treated with amputation combined with multicytostatic treatment or immunotherapy and axial tumors with local resection and multicytostatic or immunologic treatment.     

In Vitro Diffusion of DYE Through the End-Plates and the Annulus Fibrosus of Human Lumbar Inter-Vertebral Discs

Of the 242 cases of osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968 only one was found to represent telangiectatic osteosarcoma. Another case was recently diagnosed in our department. The characteristic morphologic features of these neoplasms were anaplastic stroma, high mitotic activity, osteoid-formation, widely anastomosing blood spaces, and alkaline phosphatase activity. The experience gathered indicates that telangiectatic osteosarcoma constitutes a histopathologic variant of genuine osteosarcoma with a serious prognosis, necessitating the same kind of treatment as for the genuine tumour.     

Telangiectatic Osteosarcoma

Of the 242 cases of osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968 only one was found to represent telangiectatic osteosarcoma. Another case was recently diagnosed in our department. The characteristic morphologic features of these neoplasms were anaplastic stroma, high mitotic activity, osteoid-formation, widely anastomosing blood spaces, and alkaline phosphatase activity. The experience gathered indicates that telangiectatic osteosarcoma constitutes a histopathologic variant of genuine osteosarcoma with a serious prognosis, necessitating the same kind of treatment as for the genuine tumour.     

Telangiectatic osteosarcoma.

Of the 242 cases of osteosarcoma recorded in the Swedish Cancer Registry for the years 1958 through 1968 only one was found to represent telangiectatic osteosarcoma. Another case was recently diagnosed in our department. The characteristic morphologic features of these neoplasms were anaplastic stroma, high mitotic activity, osteoid-formation, widely anastomosing blood spaces, and alkaline phosphatase activity. The experience gathered indicates that telangiectatic osteosarcoma constitutes a histopathologic variant of genuine osteosarcoma with a serious prognosis, necessitating the same kind of treatment as for the genuine tumour.     

Extra-osseous osteosarcoma: a clinical and histopathological study of four cases.

Four cases of extra-osseous osteosarcoma were found among 242 cases recorded as osteosarcoma in the Swedish Cancer Registry during the years 1958 to 1968. The tumours occurred in middle-aged and elderly patients. Three of the tumours were situated in the proximal part of the thigh and one in the scapular region. Histopathologically, all tumours were subclassified as osteoblastic osteosarcomas. The patients were treated by primary local excision which in one case was followed by a radical en bloc excision of the entire tumour bed. All cases subjected to simple excision died of metastatic disease five to twenty-four months after diagnosis. The patient treated by en bloc excision is alive and apparently free from disease fourteen years after diagnosis.     

The Van Nes tibial rotationplasty. A functionally viable reconstructive procedure in children who have a tumor of the distal end of the femur

Twelve patients who had a malignant tumor of the distal end of the femur were treated with a Van Nes tibial rotationplasty. The survival rates were comparable with those for above-the-knee amputees and patients who had an endoprosthetic replacement. The results of functional testing showed that these patients performed as well as those who had endoprosthetic replacement and better than those who had above-the-knee amputation. Rotationplasty is therefore a favorable alternative to amputation or endoprosthetic replacement, either as a primary or as a salvage procedure.     

Chondrosarcoma

Summary All cases of unequivocal chondrosarcoma recorded in the Swedish Cancer Registry for the years 1958 to 1968 were subjected to a multifactorial analysis. Of a total of 163 chondrosarcomas 25, 38 and 35% respectively were well, moderately and poorly differentiated, and 2% were of mesenchymal type. The moderately and poorly differentiated tumours characteristically exhibited general anaplasia, multiple often pale nuclei, distinct nucleoli, mitotic figures and occasionally spindle cell foci. The degree of cellularity was not of decisive value in differentiation.The prognosis was influenced by the extent and degree of vascularisation of the tumour and by the histopathological grading. The localisation of the tumour was of major importance in deciding the extent of operative treatment and prognosis. Primary radical removalen bloc by resection or ablative surgery was decisive for survival. Irrespective of the clinical and tumour variations, optimum treatment in individual cases involves complete removal of the tumour and, as far as possible, preservation of function.

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Résumé 163 cas de chondrosarcome repertoriés dans le Swedish Cancer Registry entre 1958 et 1968 ont été soumis à une analyse multifactorielle. 25, 38 et 35% étaient respectivement, fortement, modérément, et faiblement différenciés, et 2% étaient du type mésenchymateux. Les formes modérément et faiblement différenciées montraient habituellement une anaplasie généralisée, des noyaux multiples et souvent pâles, des nucléoles distincts, des images de mitoses et parfois des foyers de cellules fusiformes. Le degré de cellularité n'était pas en rapport avec la différenciation.Le pronostic dépend de la taille de la tumeur, de l'importance de sa vascularisation ainsi que de son stade histologique. Le siège de la tumeur est un élément primordial pour décider de l'importance de l'exérèse chirurgicale. La survie est liée à l'ablation primitive et radicale de la tumeur, par résection ou amputation. Indépendamment des particularités cliniques ou de celles de la tumeur, le meilleur traitement dans chaque cas consiste dans l'exérèse complète de la tumeur, en préservant la fonction dans la mesure du possible.

     

Incidence and prognosis in early onset breast cancer

The aim of this study was to assess the incidence and prognosis in early onset breast cancer. Age-adjusted incidence and death rate for the 5394 Swedish women diagnosed with breast cancer under the age of 40 between 1960 and 1996 was studied using data from the Swedish Cancer Registry and Swedish Death Cause Registry. A total of 107 consecutive young patients with invasive breast cancer undergoing surgery during 1980-1993 in the Southeast Swedish health care region were retrospectively followed up and their cancers reviewed and graded blindly. The median follow-up time was 11.2 years. The applicability of the Nottingham Prognostic Index (NPI) as a prognostic tool was investigated. Grade, age, node status, tumour size, S-phase fraction and steroid receptor content were related to survival univariately and multivariately in a Cox proportional hazard analysis. The incidence of early onset breast cancer has increased moderately and the survival rate has not improved during the last 35 years. When young women are diagnosed with breast cancer their tumours are larger, their lymph nodes more often involved, and the median grade higher than in older with 64% having grade 3 tumours. Lymph node status was the strongest sole prognostic indicator but the use of NPI gave more accurate prognostic information than node status alone.     

Tibial turn-up for long distal femoral bone loss

Since 1990, seven patients have been treated by the authors with the tibial turn-up procedure. They have ranged in age from 8 to 37 years; four were skeletally mature adults and three were children. All seven patients were faced with a clinical situation that required surgical removal of a long portion of their affected distal femur. Three of the adult patients initially were treated for osteosarcoma with long distal femoral resections and allograft arthrodesis of the knee. The allografts ultimately failed, two because of aseptic failure and one because of infection. One patient required distal femoral removal for chronic osteomyelitis and pathologic fracture. Of the three children who were treated, two had turn-ups after long resection of the distal femur for bone malignancy, and one had a secondary turn-up after failure of a long distal femoral endoprosthesis. The technique uses the normal ipsilateral tibia as a vascularized pedicle graft to restore femoral length. The ultimate result, even after very high above knee resection, is a long above knee amputation stump. The followup of the patients in the current study ranged from 2 to 8 years. All patients achieved healing and were able to wear above knee prostheses. The tibial turn-up is an effective procedure that results in a long functional above knee amputation stump even after very high above knee resections.     

Limb salvage surgery for primary bone sarcoma of the lower extremities: Long-term consequences of endoprosthetic reconstructions

Background: Adjuvant chemotherapy and endoprosthetic replacement for bone sarcomas of the lower extremity is well established. The specific long-term consequences of these endoprosthetic reconstructions for the patient's affected limb are unknown. Method: The oncologic results and the survival of the endoprostheses were reviewed in 32 patients with primary bone sarcoma of the femur or proximal tibia. There were 26 high-grade sarcomas, and 6 low-grade sarcomas. A proximal femoral endoprosthesis was used for reconstruction in 4 patients, a total or push-through femoral endoprosthesis in 11 patients, a distal femoral endoprosthesis in 15 patients, and a proximal tibial endoprosthesis in two patients. Results: Median survival was 10 years (range, 1.1 to 18.9 years) for patients with high-grade sarcoma, and 8.1 years (range, 7.1 to 10 years) for patients with low-grade sarcomas. Distant metastases developed in seven patients (22%), all with stage IIB sarcoma, with concomitant local recurrence in 3 patients (9%). Five-year overall and disease-free survival rates for high-grade sarcomas were 81% and 73%, respectively. The overall endoprosthetic survival rate was 87% at 5 years, 80% at 10 years, and 56% at 15 years. Median follow-up of the original endoprostheses was 8.3 years (range, 0.6 to 18.7 years). Endoprosthesis-related complications occurred in 13 patients (41%); most complications were mechanical failures. The highest complication rate was found in distal femoral replacements (60%); amputation was necessary in both patients treated with a proximal tibial endoprosthesis. Five endoprostheses (16%) were revised. An amputation of the involved limb was performed in four patients (13%): in two patients because of local recurrence and in the other two patients because of infection. For patients alive at follow-up, the median functional Enneking evaluation score was 22 points (range, 12 to 28 points), with the highest functional scores in patients with a distal femoral endoprosthesis, and the lowest functional scores in patients with total or push-through femoral replacements. Conclusion: Endoprosthetic reconstructions gave satisfying functional results in most patients after long-term survival. However, the proximal tibial and distal femoral endoprosthesis are particularly at risk for long-term endoprosthetic complications requiring additional surgical procedures.Presented at the Ninth International Symposium of the International Society of Limb Salvage (ISOLS), New York, September 11, 1997.     

半骨盆截肢后用废弃自体骨重建骨盆承重功能的解剖学与生物力学研究

目的 探讨对骨盆肿瘤行半骨盆截肢后,应用废弃自体骨重建坐骨结节和骨盆环以恢复骨盆承重功能的可行性.方法 带双侧下肢骨的10具骨盆标本,测量坐骨结节至正中矢状面和经骶骨耳状面中心冠状面的垂直距离、坐骨结节至同侧的骶骨耳状面和耻骨联合的距离.测量股骨髁和胫骨平台的最大径,对股骨和胫骨进行CT扫描并纵向剖开,观察骨质分布情况.根据解剖测量结果,选择4具成人尸体标本,应用电子万能试验机对完整骨盆和重建标本进行生物力学检测,比较两者在0-500 N的垂直加压下的稳定性.结果 坐骨结节至正中矢状面和经耳状面中心至冠状面的垂直距离分别为(4.52±0.48)cm和(3.31±0.57)cm.坐骨结节至同侧骶骨耳状面和耻骨联合的距离分别为(11.75±6.19)cm和(15.72±5.19)cm.股骨髁最大斜径在外后至内前方向平均为(7.93±0.44)cm,胫骨平台的最大径在冠状面上,平均为(7.19±0.61)cm.股骨髁和胫骨平台主要为松质骨,股骨髁松质骨平均纵向长度为(6.24±0.22)cm,胫骨上端平均纵向长度为(5.64±0.18)cm.在生物力学试验中,重建骨盆的抗压缩能力较完整骨盆弱,但在载荷去除后,重建骨盆和完整骨盆均可恢复原来的状态,说明重建骨盆和完整骨盆同样为弹性良好的刚体结构,具有较好的即刻稳定性.结论 半骨盆截肢后,可应用废弃股骨或胫骨重建坐骨结节和骨盆环,为患者坐立和装配假肢提供力学支持点,以降低半骨盆截肢者的残废程度.     

Treatment of tibial hemimelia without amputation: preliminary report

Treatment of tibial hemimelia, traditionally, is by amputation. This is not acceptable in our community. Hence we treated our cases without amputation. We treated two cases of type Ia and four cases of type II tibial hemimelia. The age at operation ranged from 3.5 to 13 years For type Ia cases, we applied the Ilizarov external fixator to the femur, fibula and foot to centralize the fibula between the femoral condyles and talus using gradual distraction. The second step was the Brown procedure. Then the fixator was reapplied to correct the deformities. For type II, synostosis of the tibia and fibula was performed followed by differential lengthening. Then we overlengthened the femur. After follow-up for 2-5.5 years, all patients showed improved function and were satisfied. The tibial lengthening ranged from 6 to 8.5 cm, and femoral lengthening ranged from 5 to 7 cm.     

A rotating-hinge knee replacement for malignant tumors of the femur and tibia

We evaluated the 2- to 7-year results of a rotating-hinge knee replacement after excision of malignant tumors of the knee joint. There were 25 distal femoral and 7 proximal tibial replacements. The 5-year prosthetic survival for distal femoral replacements was 88%, compared with 58% for proximal tibial replacements. Seven patients underwent prosthetic exchange: 1 for aseptic loosening, 2 for wound slough and perioperative infection, and 4 for articulating component failure. One patient underwent above-knee amputation owing to skin necrosis. The median functional scores at the latest follow-up were 27 by the International Society of Limb Salvage evaluation system and 80 by the Hospital for Special Surgery Knee Score system. This implant is a promising choice for joint reconstruction after excision of tumors at the knee joint.     

骨膜骨肉瘤与高度恶性表面骨肉瘤

目的报告表面骨肉瘤的两种少见亚型,即骨膜骨肉瘤和高度恶性表面骨肉瘤的诊治经验。方法回顾 5例骨膜骨肉瘤和 4例高度恶性表面骨肉瘤患者的影像学和病理学特点,并对临床治疗结果进行分析。 5例骨膜骨肉瘤患者,男 1例,女 4例;年龄 28～ 42岁,平均 35岁;肿瘤位于胫骨上段者 4例,股骨下段者 1例。 4例高度恶性表面骨肉瘤患者,男 3例,女 1例;年龄 17～ 23岁,平均 19.25岁; 4例患者肿瘤均位于股骨下段。结果 5例骨膜骨肉瘤均予以广泛切除,除 1例因局部复发行截肢术,现无瘤生存 1年 3个月外,余 4例已无瘤生存 3～ 9年,平均 5年 9个月。 4例高度恶性表面骨肉瘤患者,虽经积极综合治疗,但仅 1例无瘤生存 7年; 1例术后 2年 5个月复发而截肢,现无瘤生存 1年 9个月; 1例于术后 2年 4个月死于肺转移;另 1例在确诊后 3个月死亡。结论骨膜骨肉瘤和高度恶性表面骨肉瘤各具鲜明的影像学和病理学特点,骨膜骨肉瘤恶性程度较低,应采用以局部广泛切除为主的手术治疗,预后相对较好;而高度恶性表面骨肉瘤的生物学行为则与经典的髓内骨肉瘤相似,预后较差,必须采用手术与化疗相结合的综合治疗方法。     

Iliofemoral fusion for proximal femoral focal deficiency

Iliofemoral fusion, as performed for proximal femoral focal deficiency, is designed so that the knee, acting at the level of the triradiate cartilage, will act as the hip. This procedure was indicated in four of our patients to promote fitting of a prosthesis. The results after follow-up of more than five years are reported. Two of the patients also had a Van Nes rotationplasty and two had a Syme amputation. The Syme amputation produced better results. Distal epiphyseodesis of the ipsilateral femur also was performed in three of the four patients to minimize the anterior prominence of the knee. Two patients required a closing wedge osteotomy of the distal part of the femur to improve the alignment. All four patients walked well as functional above-the-knee amputees.     

Distal tibial artery thrombectomy in acute limb ischemia

During the past two years, in cases of infrapopliteal embolic occlusion not retrievable through the femoral approach, we have used distal tibial thrombectomy in preference to a direct approach via the popliteal artery. Distal tibial thrombectomy was done in seven extremities in six patients with excellent results. Only one extremity required subsequent amputation. There were no mortalities. This represents a limb salvage rate of 85%.     

The shapes of the tibial and femoral articular surfaces in relation to tibiofemoral movement

We report a study of the shapes of the tibial and femoral articular surfaces in sagittal, frontal and coronal planes which was performed on cadaver knees using two techniques, MRI and computer interpolation of sections of the articular surfaces acquired by a three-dimensional digitiser. The findings using MRI, confirmed in a previous study by dissection, were the same as those using the digitiser. Thus both methods appear to be valid anatomical tools. The tibial and femoral articular surfaces can be divided into anterior segments, contacting from 0 degrees to 20 +/- 10 degrees of flexion, and posterior segments, contacting from 20 +/- 10 degrees to 120 degrees of flexion. The medial and lateral compartments are asymmetrical, particularly anteriorly. Posteromedially, the femur is spherical and is located in a conforming, but partly deficient, tibial socket. Posterolaterally, it is circular only in the sagittal section and the tibia is flat centrally, sloping downwards both anteriorly and posteriorly to receive the meniscal horns. Anteromedially, the femur is convex with a sagittal radius larger than that posteriorly, while the tibia is flat sloping upwards and forwards. Anterolaterally, both the femoral and tibial surfaces are largely deficient. These shapes suggest that medially the femur can rotate on the tibia through three axes intersecting in the middle of the femoral sphere, but that the sphere can only translate anteroposteriorly and even then to a limited extent. Laterally, the femur can freely translate anteroposteriorly, but can only rotate around a transverse axis for that part of the arc, i.e., near extension, during which it comes into contact with the tibia through its flattened distal/medial surface as against its spherical posterior surface.     

Ten-year survival among patients with supratentorial astrocytomas grade III and IV

The Swedish Cancer Registry, in a linkage with the Swedish Registry of Statistics, was used to trace all patients operated on in Southern Sweden for histopathologically verified malignant primary central nervous system tumors during the years 1958 to 1975. Patients surviving 10 years after the operation were identified, and their original surgical specimens were reexamined. Six (0.5%) of 1147 patients were shown to have survived for between 12 and 28 years after surgery for supratentorial astrocytomas grade III and IV. All six patients are in good condition, without signs of tumor recurrence. The only obvious common denominator for the survivors is their youth at operation. Three patients were 7 to 11 years old at surgery, and none was older than 38 years. It is concluded that a cure may be possible in exceptional patients with the "incurable" astrocytoma grade III and IV.