Epithelioid osteosarcoma of the scapula.

Epithelioid and epithelial neoplasms of bone are rare. They include different epithelioid variants of vascular lesions, osteoblastoma, chondroblastoma and most importantly metastatic carcinoma. Up to now, only few cases of epithelioid osteosarcoma were described. In this case the authors report a 53-year-old patient presented with a medical history of chronic shoulder pain for 3 years. Magnetic resonance imaging (MRI and computed tomography (CT) showed a destructive, partially calcified osseous lesion of the scapula with expansion into the surrounding soft tissue, suggestive of a primary bone tumor. Histologically, the tumor consisted of epithelioid cells with expression of cytokeratine and the lesion was primarily diagnosed as metastatic carcinoma. With regard to the MRI morphology untypical for metastatic disease the histopathologic slides were re-evaluated and detection of tumor osteoid led to the diagnosis of epithelioid osteosarcoma. Chemotherapy was initiated, however follow-up imaging studies showed rapidly progressive disease of both primary tumor and lung metastases. In conclusion, epithelioid neoplasms of the bone are extremetumourly rare and must be distinguished from metastatic carcinoma. Despite the presence of cytokeratine positive cells a thorough histological evaluation is mandatory and osteoid detection is essential in order to establish the correct diagnosis and further treatment. Key words: osteosarcoma, epithelioid, aneurysmal bone cyst, chondrosarcoma, pathology, immunohistochemistry.

The many faces of chondrosarcoma of bone, own cases and review of the literature with an emphasis on radiology, pathology and treatment.

Chondrosarcoma is the third most frequent primary malignant tumor of bone, constituting up to 16% of the malignant osseous neoplasms. Up to date several genetic alterations and markers were described concerning the pathogenesis and the progression of the chondrosarcoma, which represents actually a heterogeneous group of different types including conventional intramedullary, clear cell, myxoid, mesenchymal, and dedifferentiated chondrosarcoma. The pathologic appearance varies, however, in general they grow with a lobulated pattern. Histologically the hyaline cartilage demonstrates high water content and typically enchondral ossification is apparent. Imaging reflect this while radiographic findings suggest the diagnosis when the typical "ring-and-arc" chondroid matrix mineralization, endosteal scalloping and soft-tissue extension were apparent. The CT is used for detecting the mineralization of the matrix, especially when it is subtle or when the lesion is located in complex areas. MRT is the method of choice to detect the high water content of these lesions with a high signal intensity with T2-weighting and its bone marrow extend. Surgical resection is the primary and preferred treatment modality for most individuals with localized disease. In selected cases of the Grad I conventional chondrosarcoma curettage should be discussed. Systemic chemotherapy may be considered in variant forms such as mesenchymal or dedifferentiated chondrosarcomas. In knowledge of the "many faces" of the primary chondrosarcoma individualized patient assessment and optimal clinical management is possible.

Differential diagnosis of primary malignant bone tumors in the spine and sacrum. The radiological and clinical spectrum: minireview.

Although primary malignant tumors of the spine and sacrum are described in all orthopedic textbooks, it is still remarkable how little attention is paid to differential diagnosis of persisting lower back pain and how to detect in special the underlying tumor disease. Chordoma, osteosarcoma, chondrosarcoma, plasmacytoma, lymphoma and Ewing's sarcoma, their radiological manifestation, age distribution and preferred location in the spine and sacrum are reviewed and discussed.

[Tumor-like lesions]. / Tumorähnliche Knochenläsionen.

Tumor-like bone lesions include various conditions of a non-neoplastic nature originating from or affecting the bone as solitary or sometimes multiple bone lesions. This review discusses the most important cystic tumor-like lesions, such as simple bone cyst, aneurysmal bone cyst, intraosseous ganglion, epidermoid cyst and subchondral cyst. MR is the most sensitive method of detection, and is the most accurate in depicting the extent of involvement of these lesions. MR signal characteristics may aid in differential diagnosis. Conventional radiographs are often more specific with respect to the underlying histopathology.

Skeletal metastasis in tricholemmal carcinoma.

Tricholemmal carcinoma is an extremely rare cutaneous adnexal tumor, not exceeding 1-2 cm in diameter in some reported cases. The few reports describe only a greater histologic malignancy. Some cases of recurrences have been reported, and metastasis of the soft tissue was described once by Amaral et al in 1984. In the current patient with tricholemmal carcinoma of the right distal thigh, the primary tumor was unusually large. The first detected metastasis was localized in the right inguinal lymph nodes. Five years after diagnosis of tricholemmal carcinoma a metastasis of the left tibia and fibula were diagnosed. To our knowledge, an osseous metastasis in tricholemmal carcinoma has not been described. It is possible that a relationship between the extraordinary size and the osseous metastasis exists in the current patient.

Differential diagnosis of benign tumors and tumor-like lesions in the spine. Own cases and review of the literature.

Although benign tumors and tumor-like lesions of the spine are shown in every orthopedic teaching book, it is often surprising how little attention is paid to the differential diagnosis and diagnostic investigations, respectively, since surgical treatment and postoperative control depends on exact diagnosis. Clinically the importance of this fact can not be over-emphasized. The most common complaint is pain, either local or radicular in nature. Bone deviations are diagnosed radiologically. Different types of benign bone tumors and tumor-like lesions of the spine including osteochondroma, osteoblastoma, osteoid osteoma, aneurysmal bone cyst, eosinophilic granuloma, hemangioma, and giant cell tumor, their appearance relation to the age and location in the spine were reviewed and the common histologic subtypes described. Clinical, laboratory and histopathologic findings, radiologic inclusive MRT and scintigraphic features are evaluated. Diagnostic investigations including the invasive techniques of CT guided needle biopsy, Yamshidi needle biopsy and costotransversectomy for biopsy are shown. Treatment including radiation, chemotherapy and the surgical procedure as well as the postoperative treatment of patients with benign tumors and tumor-like lesions of the spine are discussed.

Angiogenesis and the ET-1/ETA receptor system: immunohistochemical expression analysis in bone metastases from patients with different primary tumors.

Angiogenesis is necessary for the growth of primary tumors and the formation of metastases. It is well known that vascular endothelial growth factor (VEGF) and its receptors play a major role in this process. To date, the formation of bone metastases has been poorly understood. Tumor cells must interact with the microenvironment of the bone and new blood vessels must spread. The ET/ET(A) (endothelin) receptor system seems to play an important role in this process. Specimens from metastatic bone lesions and non-malignant bone tissue were analyzed by histological and immunohistochemical staining. Sections were stained with antibodies against CD31, Flt-1, KDR, endothelin-1 (ET-1) and endothelin receptor A (ET(A)). Our studies show that there is an increased microvessel density (MVD) in metastatic bone lesions from different primary tumors in contrast to normal bone tissue. In nearly all tumor formations of the bone, ET-1 and its receptor ET(A) was found by immunohistochemistry. We also performed immunohistochemical staining for the VEGF-receptors Flt-1 and KDR. In conclusion, there is an increased vessel density in metastatic bone lesions in contrast to normal bone tissue. The ET/ET(A) system can be detected in nearly all bone specimens and is upregulated in metastatic bone lesions in contrast to healthy bone tissue.

Calvarial sclerosing osteomyelitis.

We report a 15-year-old boy who suffered from calvarial sclerosing osteomyelitis and presented with painful head swelling. X-rays of the skull revealed areas of irregular radiolucency. MR imaging and CT showed a well-demarcated intradiploic lesion with thickening of the skull extending from the frontal to the parietal calvarium with a low signal on T1-weighted images, strong but heterogeneous enhancement after gadolinium application and a mixed signal on T2-weighted images. Computer-navigated neurosurgery was planned, and the craniotomy defect was reconstructed by a preformed titanium implant. Sclerosing osteomyelitis of the calvarium has to be included in the differential diagnosis of osteolytic and sclerosing lesions of the skull coinciding with persistent swelling of the head.

[Psammous desmo-osteoblastoma of the right maxilla. Case report and differential diagnosis of a rare tumor]. / Psammöses Desmoosteoblastom des rechten Oberkiefers. Fallbericht und Differenzialdiagnose einer seltenen Tumorentität.

CASE REPORT: We report the case of a 9-year-old female patient with a moderate swelling of the right maxilla. A tumor was diagnosed by conventional X-ray within the region of tooth 17 in the right tuber maxillae. The histological diagnosis after biopsy of the lesion was "fibrous dysplasia Jaffe-Lichtenstein". One year later, the tumor was still growing, and computed tomography was carried out. The first histological diagnosis was confirmed by another biopsy; the differential diagnosis was "ossifying fibroma". In the following years, the tumor grew and expanded into the right orbit and maxilla. Four years after the first diagnosis the tumor was removed operatively. DIFFERENTIAL DIAGNOSIS OF THE OPERATIVE SPECIMEN: The histology showed round, partially trabecular or calcified bodies, known as psammoma-like ossicles. Therefore, in light of the clinical course and the radiographic appearance, the correct diagnosis was "psammous desmo-osteoblastoma". DISCUSSION: The psammous desmo-osteoblastoma and its differential diagnosis are discussed in this article.

[Soft tissue chondromatosis of the foot: diagnosis and therapy]. / Chondromatose der Weichteile am Fuss. Diagnose und Therapie.

Chondromas of the soft tissue are uncommon, benign tumors. They are most frequently found adjacent to periarticular tissues or tenosynovium with a predilection for the hands and feet. We report a case of a patient with a large, increasing tumor of the left foot. Because of subjective complaints and mechanical irritation the patient was scheduled for surgery. The preoperative radiological work-up showed popcorn like clusters of calcifications around the dorsum of the foot. The tumor mass was reduced using several incisions. Histology showed soft tissue chondromatosis of the foot without malignancy. Following the operative procedure, the patient was free of pain and able to wear normal shoes. This case illustrates the clinical and radiological characteristics as well as the surgical treatment of progressive soft tissue chondromatosis.

Multifocal osteoblastoma of the hand.

Osteoblastoma is a benign bone tumor with uncertain radiologic and typical histologic pattern that, in most cases, can be diagnosed without any problems. Usually, it is a solitary bone tumor. The case presented is a 9-year-old child with multiple osteoblastomas occurring in multiple bones of the right hand. The child had pain in his right hand for several weeks. On physical examination, no swelling or other symptoms were elicited. All lesions noted radiologically were treated by curettage, and in all the pattern of osteoblastoma was diagnosed.

Prognostic significance of nuclear DNA content in chondrosarcoma.

Chondrosarcoma is the second most frequent primary malignant tumor of bone. Many of these tumors represent histopathologic borderline cases. In this study, DNA ploidy status, 2c deviation index (2cDI), and DNA malignancy grade (DNA-MG; based on the variation of nuclear DNA content of tumor cells around the normal DNA [2c] peak) were examined for their diagnostic and prognostic value in comparison with conventional histopathologic grading. Twenty-two paraffin-embedded samples were available for histopathologic investigation and for quantitative cytophotometric DNA determination of Feulgen-stained nuclei. Clinicopathologic parameters and prognosis were analyzed over a maximum follow-up period of 252 months. Nineteen of 22 (86%) chondrosarcomas showed aneuploid DNA content. 2cDI (r =.58, P <.01) and DNA-MG (r =.58; P <.01) correlate with the histopathologic grading. Significant correlation between the 2cDI (P <.01) and DNA-MG (P <.025) and the overall survival was found. Ploidy did not influence the overall survival rate. In metastasis-free patients, the 2cDI and DNA-MG gave better prognostic information than conventional histopathologic grading. When patients developed metastasis, however, histopathologic grading was the prognostic parameter of choice. Cytometric DNA measurement provide additional objective information regarding the diagnosis and prognosis of chondrosarcomas, even more than that obtained by conventional histopathologic grading, and may be helpful in planning the treatment.

[The intervertebral disk in anatomy and pathology]. / Die Bandscheibe in Anatomie und Pathologie.

One of the most frequent and incapacitating medical and social problems of our times is back pain. In most patients the cause of the symptoms remains unknown, although some relevant clues have been discovered over the last few years: mechanical, nutritional, chemical, social, psychological and genetic factors. Pathoanatomic conditions that are generally accepted as related to the symptoms include disc herniation, fracture, infection, spinal stenosis, spondylarthropathy and spondylolisthesis. Diagnostic problems will only be solved by considering and synthesis of all findings.

Prognostic significance of DNA image cytophotometry for osteosarcoma.

OBJECTIVE: To investigate the prognostic significance of DNA image cytophotometric data. STUDY DESIGN: Twenty-six osteosarcomas in patients without lung metastases were investigated for several cytophotometric data. In 24 cases, these data were correlated with the clinical course of the patients to assess the prognostic value of nuclear DNA content in osteosarcomas. RESULTS: Of all osteosarcomas, 96% showed aneuploid DNA content. Patients with tumors having a 2c deviation index (2cDI) of 12.00, DNA malignancy grade (DNA-MG) of 2.0, a mean DNA content (MDC) of 4.95 c, DNA index (DI) of 1.75 or mean nuclear area (MNA) of 130 microns 2 had a significantly lower overall survival rate as compared to those with lower values (P < .05). CONCLUSION: Image cytophotometric features, such as 2cDI, DNA-MG, MDC, DI and MNA, are of prognostic value in patients with osteosarcoma and free of lung metastases.

Human articular cartilage: in vitro correlation of MRI and histologic findings.

The aim of our study was to correlate MRI with histologic findings in normal and degenerative cartilage. Twenty-two human knees derived from patients undergoing amputation were examined with 1.0- and 1. 5-T MR imaging units. Firstly, we optimized two fat-suppressed 3D gradient-echo sequences. In this pilot study two knees were examined with fast imaging with steady precession (FISP) sequences and fast low-angle shot (FLASH, SPGR) sequence by varying the flip angles (40, 60, 90 degrees) and combining each flip angle with different echo time (7, 10 or 11, 20 ms). We chose the sequences with the best visual contrast between the cartilage layers and the best measured contrast-to-noise ratio between cartilage and bone marrow. Therefore, we used a 3D FLASH fat-saturated sequence (TR/TE/flip angle = 50/11 ms/40 degrees) and a 3D FISP fat-saturated sequence (TR/TE/flip angle = 40/10 ms/40 degrees) for cartilage imaging in 22 human knees. The images were obtained at various angles of the patellar cartilage in relation to the main magnetic field (0, 55, 90 degrees). The MR appearances were classified into five categories: normal, intracartilaginous signal changes, diffuse thinning (cartilage thickness < 3 mm), superficial erosions, and cartilage ulcers. After imaging, the knees were examined macroscopically and photographed. In addition, we performed histologic studies using light microscopy with several different stainings, polarization, and dark field microscopy as well as electron microscopy. The structural characteristics with the cartilage lesions were correlated with the MR findings. We identified a hyperintense superficial zone in the MR image which did not correlate to the histologically identifiable superficial zone. The second lamina was hypointense on MRI and correlated to the bulk of the radial zone. The third (or deep) cartilage lamina in the MR image seemed to represent the combination of the lowest portion of the radial zone and the calcified cartilage. The width of the hypointense second zone correlated weakly to the accumulation of proteoglycans in the radial zone. The trilaminar MRI appearance of the cartilage was only visible when the cartilage was thicker than 2 mm. In cartilage degeneration, we found either a diffuse thinning of all layers or circumscribed lesions ("cartilage ulcer") of these cartilage layers in the MR images. Early cartilage degeneration was indicated by a signal loss in the superficial zone, correlating to the histologically proven damage of proteoglycans in the transitional and radial zone along with destruction of the superficial zone. We found a strong effect of cartilage rotation in the main magnetic field, too. A rotation of the cartilage structures caused considerable variation in the signal intensity of the second lamina. Cartilage segments in a 55 degreesangle to the magnetic main field had a homogeneous appearance, not a trilaminar appearance. The signal behavior of hyaline articular cartilage does not reflect the laminar histologic structure. Osteoarthrosis and cartilage degeneration are visible on MR images as intracartilaginous signal changes, superficial erosions, diffuse cartilage thinning, and cartilage ulceration.

Change in size, number and morphology of the nucleoli in human hearts as a result of hyperfunction.

In the present study we examined light-microscopically the nuclei of 16 hearts (7 normal hearts, 7 hearts with hypertrophy and 2 hearts with atrophy) for the size, number and morphology of their nucleoli. The size of the nucleoli, defined as the total area of all nucleoli per nucleus, was an early and sensitive indication of a beginning hypertrophy of the myocardium. It increased in parallel to the total heart weight and to the total area of the nucleus of the cell, but initially the size of the nucleoli changed earlier than the other parameters. We found an increase of the ratio in the total area of the nucleoli per nucleus to the total area of the nucleus at the beginning of clinical hyperfunction. This ratio normalised during chronic hyperfunction. The number of the nucleoli also increased during hyperfunction, but it did not exceed the number of nucleolus organiser regions (NOR) given by the number of chromosomes. Nevertheless, we found numbers of nucleoli higher than 10 because, the number of NORs increases during polyploidization. Regarding the morphology of the nucleoli in hearts with hypertrophy, we found a predomination of the nucleoli with a highly branched nucleolonemal structure as an indication of an increased RNA synthesis.

Comparison of MR sequences in quantifying in vitro cartilage degeneration in osteoarthritis of the knee.

Magnetic resonance imaging of amputated human knees was performed to determine optimal sequences for depicting articular cartilage. 24 knees were examined with eight different sequences in a 1.0 T imager. Each cartilage lesion was graded from 1 to 4 (Outerbridge staging system). The results of each sequence were compared with the macroscopic findings and statistically tested against each other. The FLASH sequence (TR = 50 ms) with combination of flip angle of 40 degrees and echo time of 10 ms and the FISP sequence (TR = 40 ms) with combination of flip angle of 40 degrees and echo time of 11 ms were best for depicting cartilage structure and internal detail. There was no significant difference between fat-saturated three-dimensional FLASH (FS-3D-FLASH) and FS-3D-FISP (p = 0.05). These FS-3D sequences were significantly better than sequences without fat saturation (p = 0.05). There was no significant difference between magnetization transfer (MT) 3D-FLASH, MT-3D-FISP and 3D-FISP. All 3D sequences showed significantly (p = 0.05) better results than spin echo or fast spin echo sequences. The T1 weighted SE pulse sequence was significantly (p = 0.005) better than the T2 weighted TSE sequence. Fast T2 weighted spin echo was not suitable for early and accurate detection of cartilage lesions.

[Regeneration of the heart muscle after myocardial infarct]. / Regeneration des Herzmuskels nach Myokardinfarkt.

Myocardial infarction is characterized by the loss of contractile cardiomyocytes. This results in an increased load on the spared myocardium. The aim of this article is to describe the response of the non-affected myocardium after infarction. Human hearts were investigated by microscopical, biochemical and cytophotometrical methods. The number of nuclei was calculated from the numerical ratio of connective tissue nuclei/myocyte nuclei, the values of the total DNA amount and the percentage proportion of the various ploidy classes in the myocyte nuclei. After myocardial infarction the DNA concentration as well as the DNA content was increased. Different DNA distribution pattern could be found in infarcted hearts. Percentage proportion of higher ploid nuclei and aneuploid nuclei was increased. Number of connective tissue nuclei was increased in infarcted hearts. And, in spite fibrosis of the necrotic area, number of myocyte nuclei did not change significantly. Beside scarring of the necrotic tissue, hypertrophy and hyperplasia of cardiomyocytes occur after myocardial infarction and may compensate the loss of contractile fibres to guarantee myocardial function.