[Disease picture of dysplasia epiphysealis hemimelica]. / Beitrag zum Krankheitsbild der Dysplasia epiphysealis hemimelica.

Dysplasia epiphysealis hemimelica - an epiphyseal developmental disturbance of the skeleton - is combined with exostose-like, tumor-simulating cartilaginous hypertrophy of bone tissue, mainly located at the epiphyses of the lower extremities and at the tarsal bones. In the case of multiple involvement the unilateral type prevails (hemimele type); within the epiphyses it is mostly the medial part which is undergoing changes. Rare sites are patella, head of the hip bone, shoulder joint, carpalia, more commonly affected are talus as well as other tarsal bones, distal femoral epiphysis and proximal and distal tibial epiphysis. Histologically, dysplasia epiphysealis hemimelica is identical to cartilaginous exostosis. Bone formation is taking place by enchondral ossification (epiphyseal osteochondroma). The ratio between male and female is 3:1. As a rule, this disease becomes manifest in early childhood or adolescence by indolent swellings of the joint region, restricted movement, axis shift, sometimes by differences in the length of the legs. Therapeutic guidelines are not unanimous. On one hand, correction osteotomy is not recommended until the completion of growth, while on the other hand excision of the cartilaginous hypertrophy in early childhood (before the age of 4) is postulated. Since there are less than 100 cases described in literature, another 8 cases of this extremely rare disease are to be presented here.

Biological characterization of human bone tumors. VII. Detection of malignancy in a giant cell tumor of bone by flow cytometric DNA-analysis.

The biological behavior of giant cell tumors of bone often cannot be definitively evaluated by light and electron microscopical criteria. As DNA aneuploidy was found to be a highly sensitive marker of malignant cells, we have analyzed this descriptive parameter in 4 giant cell tumors of bone by flow cytometry. Three of the four cases were non-metastasizing giant cell tumors, the fourth was a clinically malignant neoplasm presenting extensive visceral metastases four years after first diagnosis. Neither light- nor electron microscopical findings provided unequivocal criteria for defining the biological dignity of the four tumors. DNA aneuploidies were identified in the metastasizing case only, whereas the three clinically benign tumors showed a unimodal euploid DNA distribution. Thus flow cytometric DNA analysis is an additional diagnostic tool for the detection of malignancy in giant cell tumors of bone.

[Eosinophilic granuloma of the spine]. / Das eosinophile Granulom der Wirbelsäule.

The contribution to the clinical picture of eosinophilic granuloma of the spine. This tumor-like, osteolytic bone lesion presents both diagnostic and therapeutic problems. Solitary eosinophilic granuloma of the spine is not common. Compared to the multiple manifestation of eosinophilic granuloma, which seldom spares the spine, vertebral involvement is rare in cases of solitary eosinophilic granuloma (about 10 p.c.). Spinal involvement of this disease is mainly characterized by an undramatic, uneventful clinical course, even in cases of extensive osteolytic bone defects, varying laboratory findings as well as partial or complete collapse of a vertebra, mostly in form of a true vertebrae plana. Open biopsy is recommended instead of needle aspiration biopsy in solitary eosinophilic granuloma of the spine in order to exclude Ewing's sarcoma, neuroblastoma, or bony manifestations of leukemia. In cases of solitary eosinophilic granuloma various therapeutic methods have been tried. Some authors have registered good results by means of prolonged immobilization, similar to fracture treatment. Others have used radiation therapy in moderate doses. We suggest operative treatment of solitary eosinophilic granuloma. Our procedure comprises open biopsy, frozen section examination, and curettage of the affected vertebral body, taking care not to destroy the epiphyseal plate or the intervertebral disc. Vertebral body replacement is achieved by means of a bone graft, taken from the iliac crest. The original height of the vertebra is thus restored and immediate stability of the involved area guaranteed. Undisturbed bone growth of the end plated of the vertebral body can be observed. This technique is to prevent disturbances of spinal growth and permanent deformities.(ABSTRACT TRUNCATED AT 250 WORDS)

Biologic characterization of human bone tumors. II. Distribution of different collagen types in osteosarcoma--a combined histologic, immunofluorescence and electron microscopic study.

Sixteen cases of typical highly malignant osteosarcoma were investigated by light, electron, and immunofluorescence microscopy to demonstrate the presence of collagen types I-III. It was shown that, in light-microscopically anaplastic areas of the tumor, collagen type III predominates, while only very few membranes of collagen type I are observed. Ultrastructurally, the cells are characterized by numerous free ribosomes in their cytoplasm and only a few membranes of granular endoplasmic reticulum (ER). In osteoblastic areas, collagen type I is increased, while type-III collagen is decreased. The cytoplasm of cells contains markedly more granular ER. An increasing mineralization of matrix is observed. In fibroblastic areas of the tumors, collagen types I and III are codistributed. Tumor cells have a fibroblast appearance with elongated nuclei and well developed granular ER. The chondroblastic areas, characterized by immature neoplastic cartilage, contain varying amounts of collagen type II. Chondroblast-like tumor cells have typical ring-shaped membranes of granular ER in their cytoplasm. The evidence of different collagen types in osteosarcomas lends additional support to the concept that a pluripotent mesenchymal cell is the stem cell of osteosarcomas.

Combined ultrastructural, histochemical, and autoradiographic study of osteosarcoma after preoperative chemotherapy according to the COSS 80 protocol.

Twelve osteosarcomas treated according to the COSS 80 protocol (preoperative chemotherapy, resection) were studied by light and electron microscopic, histochemical, and autoradiographic methods. Evidence of regressive and necrotic changes was found in many tumor cells, but the alterations were unspecific. Viable tumor cells of high malignancy were also observed regularly, often at the S phase. As the tumor regression continued, a strong reaction of the mononuclear phagocyte system was manifested by the presence of macrophages and giant cells.

Biologic characterization of human bone tumors. I. Ewing's sarcoma. A comparative electron and immunofluorescence microscopic study.

Six cases of Ewing's sarcoma were investigated by electron and immunofluorescence microscopy. A layer of basement membrane-like deposits was found between typical principal and secondary tumor cells. To clarify the nature of these ultrastructural deposits, antibodies against collagen type IV were applied to frozen sections of corresponding tumor tissue. This reaction revealed type IV collagen as a regular component of basement membranes in nonneoplastic tumor capillaries, but it was equally able to localize collagen type IV between single tumor cells in capillary-free areas. With the same method, factor-VIII-associated protein, predominantly found in endothelial cells, could be demonstrated in some tumor cells. These results demonstrate that, in addition to anaplastic cells, some tumor cells are found in Ewing's sarcoma that share certain differentiating features with the endothelial cell.

Benign fibrous histiocytoma of bone. Light- and electron-microscopic observations.

Case report of a patient with an unusual, rapidly growing bone tumor in the third and fourth cervical vertebrae. Histological and electron-microscopic investigations reveal a tumor composed of histiocytic cells, xanthomatous cells, giant cells of Touton type, and fibroblastic cells. No cellular features of malignancy are observed. From its cytologic appearance the tumor has to be classified as a benign fibrous histiocytoma. Regarding its ultrastructural features, the tumor may not be distinguished from non-ossifying fibroma of bone, but its clinical pattern shows obvious differences of localization and growth potential. the term "benign fibrous histiocytoma of bone" is proposed for these tumors which must be differentiated from non-ossifying fibroma.

Tumor cell types in osteosarcoma as revealed by electron microscopy. Implications for histogenesis and subclassification.

Ultrastructural studies in 26 osteosarcomas of high grade malignancy which were in diverse locations and of varied histological types revealed seven different tumor cell types. They were characterized by their features as follows: 1) anaplastic cells of malignant blast structure: 2) osteoblastic cells -- some of them with dot-like intranuclear bodies; 3) osteocyte-like cells surrounded by mineralized matrix; 4) fibroblast-like cells; 5) cells of myofibroblastic differentiation; 6) chondroblast-like cells in chondroblastic areas, and even 7) angioblastic cells that may be differentiated from the angioblastic and endothelial structures of capillaries. Histogenetically, osteosarcoma may be derived from stromal mesenchymal cells with a potential for differentiation into these seven tumor cell types, any tumor including, however, the osteoblastic and the osteocyte-like cells with tumor osteoid. This matrix serves as the specific criterion for identifying a tumor as "osteosarcoma", but almost every osteosarcoma of high grade malignancy will show these seven tumor cell types. The predominance of one or the other cell in the population may provide the basic information for achieving a cytologic subclassification of osteosarcoma in order to obtain relevant morphologic criteria in terms of prognosis.

Ultrastructure of telangiectatic osteosarcoma.

Recent investigations have shown that telangiectatic osteosarcoma has a poorer prognosis than other osteosarcomas. To elucidate the histogenesis of TOS two cases were investigated on the electron microscopic level. The results show that besides anaplastic, osteoblast-like, and fibroblast-like tumor cells angiosarcomatous components can be observed in this malignant bone tumor, which are characterized by endothelial cells with pinocytotic vesicles, tight intercellular junctions, fine fibrils, and so-called Weibel-Palade bodies in their cytoplasm. From these results, it is concluded that telangiectatic osteosarcoma is derived from multipotent mesenchymal cells with potential differentiation into various directions, such as osteoblast-like cells, and fibroblast-like cells.

[Contribution to capsular hemangioma of the knee joint (author's transl)]. / Beitrag zum Hämangiom der Kniegelenkkapsel.

Hemangioma of the knee joint capsule is rare. Although there are exact descriptions of the lesion to be found in the world literature, it is relatively unfamiliar among surgeons. Some characteristic symptoms as painful swelling, hemarthrosis, disappearance of swelling upon limb elevation, muscular atrophy, limitation of the knee motion, occasionally increased leg length or locking in circumscribed tumors and x-ray findings as osteolytic changes, periostal new bone formation and bony atrophy lead to the diagnosis. Phleboliths are pathognomonic. Another very characteristic case is added to the about 160 cases described in the world literature. Arthrography is helpful only in rare instances. Arteriography is critizised differently, but more frequently it is thought to be of diagnostic value. Arthroscopy does not lead any further in diffuse hemangioma. The treatment of choice is radical excision of the tumor whenever possible. In order to avoid secondary arthrotic changes early surgical intervention is favourable.

[Treatment and prognosis of osteosarcoma in childhood (author's transl)]. / Therapie und Prognose der kindlichen Osteosarkoms

126 osteosarcoma under the age of 15 years are followed up in an interclinical study. There is a steady increase of tumor incidence towards the 15th year with a slight male preponderance. The main sites of the tumor are the distal femur followed by the proximal tibia and humerus. Evidence of multiple metastases is most often present in the lungs, less often in the skeleton. Nearly always metastases became evident within 2 1/2 years after diagnosis (98%). Therefore the 2 1/2-year disease--free survival rate seems to be sufficient for prognostic evaluation. The over all 2 1/2-year survival rate was 17,5%. Though the different methods of treatment are not statistically valid, the best results can be expected after early amputation. By radiotherapy alone no cure has been achieved. Survivals were seen at any age and at any site of long bones (distal femur, proximal tibia, humerus, femur, distal radius). Prognosis of osteosarcoma in the childhood is similar to that of the adult group.

Surgical treatment of solitary bone cysts.

A special method of subperiosteal segmental resection is described as an operative treatment for solitary bone cysts in long bones. This procedure exploits the high osteogenetic potency of the periosteum in adolescence. An autogenous or homologous fibula bone graft locked between the ends of the cyst serves to maintain the length of the bone fragments and acts as a guiding rod for continued subperiosteal new bone formation. There is full reconstruction of bone stability and function at both the cyst region and the donor region (fibula).