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Purpose: To assess the diagnostic performance of mean apparent diffusion coefficient (mADC) in differentiating benign from malignant bone spine tumors, using histology as a reference standard. Conventional magnetic resonance imaging (MRI) sequences have good reliability in evaluating spinal bone tumors, although some features of benign and malignant cancers may overlap, making the differential diagnosis challenging., Materials and Methods: In all, 116 patients (62 males, 54 females; mean age 59.5 ± 14.1) with biopsy-proven spinal bone tumors were studied. Field strength/sequences: 1.5T MR system; T 1 -weighted turbo spin-echo (repetition time / echo time [TR/TE], 500/13 msec; number of excitations [NEX], 2; slice thickness, 4 mm), T 2 -weighted turbo spin-echo (TR/TE, 4100/102 msec; NEX, 2; slice thickness, 4 mm), short tau inversion recovery (TR/TE, 4800/89 msec; NEX, 2; slice thickness, 4 mm, IT, 140 msec), axial spin-echo echo-planar diffusion-weighted imaging (DWI) (TR/TE 5200/72 msec; slice thickness 5 mm; field of view, 300; interslice gap, 1.5 mm; NEX, 6; echo-planar imaging factor, 96; no parallel imaging) with b-values of 0 and 1000 s/mm², and 3D fat-suppressed T 1 -weighted gradient-recalled-echo (TR/TE, 500/13 msec; slice thickness, 4 mm) after administration of 0.2 ml/kg body weight gadolinum-diethylenetriamine pentaacetic acid. Two readers manually drew regions of interest on the solid portion of the lesion (hyperintense on T 2 -weighted images, hypointense on T 1 -weighted images, and enhanced after gadolinium administration on fat-suppressed T 1 -weighted images) to calculate mADC. Histology was used as the reference standard. Tumors were classified into malignant primary tumors (MPT), bone metastases (BM), or benign primary tumors (BPT). Statistical tests: Nonnormality of distribution was tested with the Shapiro-Wilk test. The Kruskal-Wallis and Mann-Whitney U-test with Bonferroni correction were used. Sensitivity and specificity of the mADC values for BM, MPT, and BPT were calculated. Approximate receiver operating characteristic curves were created. Interobserver reproducibility was evaluated using the intraclass correlation coefficient (ICC)., Results: The mADC values of MPT (n = 35), BM (n = 65), and BPT (n = 16) were 1.00 ± 0.32 (0.59-2.10) × 10 -3 mm 2 /s, 1.02 ± 0.25 (0.73-1.96) × 10 -3 mm 2 /s, 1.31 ± 0.36 (0.83-2.14) × 10 -3 mm 2 /s, respectively. The mADC was significantly different between BPT and all malignant lesions (BM+MPT) (P < 0.001), BM and BPT (P = 0.008), and MPT and BPT (P = 0.008). No difference was found between BM and MPT (P = 0.999). An mADC threshold of 0.952 × 10 -3 mm 2 /s yielded 81.3% sensitivity, 55.0% specificity. Accuracy was 76% (95% confidence interval [CI] = 63.9%-88.1%). Interobserver reproducibility was almost perfect (ICC = 0.916; 95% CI = 0.879-0.942)., Conclusion: DWI with mADC quantification is a reproducible tool to differentiate benign from malignant solid tumors with 76% accuracy. The mADC values of BPT were statistically higher than that of malignant tumors. However, the large overlap between cases may make mADC not helpful in a specific patient., Level of Evidence: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1034-1042., (© 2017 International Society for Magnetic Resonance in Medicine.)

A 65-year-old woman presented with back pain and a lytic destructive lesion of T-11. Fine-needle aspiration biopsy revealed a granulomatous reaction with unusual signet ring type cells and vacuolated histiocytes. Subsequent resection of the lesion revealed a fibrohistiocytic reaction with extensive amyloid deposition. Immunoperoxidase stains confirmed monotypic kappa light-chain staining. Ultrastructural examination of paraffin-embedded tissue confirmed extracellular amyloid deposition. A unique feature of this case was the finding of intracellular amyloid; the unusual signet ring cells were shown to be plasma cells with intracellular amyloid, and the vacuolated histiocytes also contained amyloid.

All definite cases of oncogenic osteomalacia have, until now, been classified as mesenchymal tumours. We report here a case of oncogenic osteomalacia caused by a spinal tumour. Microscopically, it resembled the mixed connective tissue variant of previously described phosphaturic tumours. Immunohistochemical studies, however, showed the tumour cells to be positive for low molecular weight cytokeratin (CAM 5.2), S100 protein, PGP 9.5, and synaptophysin. Electron microscopy demonstrated neurosecretory granules. The histopathological findings strongly suggest that this is a neuroendocrine tumour.

Altogether 20 chordomas were reported. The site distribution included 9 cases at the sacrococcygeal region, another 9 cases at the spheno-occipital region, 1 at the cervical vertebra, and another 1 at the lumbar vertebra. Histologic examination revealed that characteristic "physaliphorous cells" were easily identified in all the 19 cases. Tissue for immunohistochemistry study was available in 18 cases. Among them, tumor cells were found strongly positive to EMA, but negative for CEA. 16/18 cases also showed positive for keratin and S-100 protein. Totally, 2 cases were studied ultrastructurally and there were abundant RER and microfilaments seen in the cytoplasm of the tumor cells but only few surface microvilli detected. The epithelial nature of chordoma is strongly supported by the ultrastructural and immunohistochemical findings of these 20 cases.

The case is a 56-year-old woman who presented with cord compression from a lesion of the thoracic spine. Histologic analysis confirmed the diagnosis of a spindle cell sarcoma. Ultrastructural analysis showed features characteristic of a leiomyosarcoma. Subsequent discussion with the patient revealed a history of hysterectomy performed for fibroid uterus 5 years before the current presentation. Review of the previous surgical specimen confirmed the presence of a leiomyosarcoma originally interpreted as a large infarcted myoma.

A case of chronic myelogenous leukemia (CML) with penetrance of the Ph1 molecular alteration into the T-cell lineage of a paraspinal tumor is reported. The T-cell nature of the paraspinal tumor was documented by immunochemical study and genotyping. The Philadelphia chromosome was also detected by genotyping in the same tumor cell population. To the authors' knowledge, this is the first case of extramedullary T-cell blast crisis in CML demonstrated in an extranodal tumor. The clinical significance of detecting extramedullary blast crisis, and the theories for the scarcity of T-cell transformation in CML are discussed.

A tumor with the light microscopic and immunohistologic characteristics of chordoma in the sacrococcygeal region in a 48-year-old man was examined by electron microscopy and immunohistochemistry for tubulin. A large population of cells exhibited prominent parallel arrays of long, straight microtubules in the rough endoplasmic reticulum in addition to the well-described ultrastructural features of chordoma. Immunoreaction to tubulin recognized in the juxtanuclear regions of the large number of tumor cells was in accordance with the ultrastructural localization of the microtubular aggregates. This seems to be the first report of microtubular aggregations in rough endoplasmic reticulum in classic chordoma. Furthermore, the present electron microscopic and immunohistochemical findings suggest that tubulin is a constituent of the unusual structures.

An epidural neoplasm was removed from the right side of the third cervical vertebra of a Blue Tick Hound dog. By light microscopy the tumor was classified as a syncytial meningioma containing psammoma bodies that were histochemically positive for calcium salts, but negative for reticulum fibers and collagen. Ultrastructurally, the tumor cells had numerous processes and desmosomes. Cytoplasmic fibrils were sparse. Coated cytoplasmic vesicles frequently were fused with plasma membranes. The psammoma bodies were not within the cytoplasm, but were in spaces continous with the extracellular space. The bodies were composed of a medium electron-dense matrix; a crystalline, highly electron-dense center; and, sometimes, short fibrils embedded in the glanular matrix.

The plain radiographic and computed tomographic (CT) findings in two unusual cases of spinal Ewing sarcoma are reported. Radiographic features resembling neuroblastoma in one case and aneurysmal bone cyst in the other were present. These findings may be misleading and distinguishing characteristics in each case are discussed.

Idiopathic scoliosis is a disease of unknown etiology. It presents in growing children, progresses slowly with time, and is painless. Any variation from the usual presentation warrants investigation. Extra dural pressure, tethering of the cord, tumors, and progressive neurologic lesions must be excluded as causative factors.

We describe on a primary, subcutaneous sacrococcygeal ependymoma presenting in a young female patient. Detailed immunohistochemical and electron microscopic examinations were performed. Out of the 40 similar cases reported in literature this is the first in which the determination of DNA content was also used to predict biological behaviour. The tumour proved to be aneuploid with low proliferative capacity in spite of absent histological signs of malignancy. It is suggested that DNA determination may be helpful in establishing prognosis and that it may contribute to a better understanding of the biological behaviour of this tumour.

An epithelioid haemangioendothelioma arising in a 43-year-old Japanese woman is presented. The patient first complained of severe back pain and neurogenic disturbances due to compression fracture of the spinal bone. Radiographically the tumour masses showed osteolytic lesions without reactive new bone formation. Similar lesions were noted in other bones. Biopsy and surgical specimens showed the features of epithelioid haemangioendothelioma. A lymphocyte associated antigen, HLA-DR which has been regarded as a B lymphocyte marker was detected in the tumour cells. The importance of this finding was emphasized with respect to the identification or classification of vascular tumours.

A granular cell tumor in the spinal canal that presented with subarachnoid hemorrhage is reported. The tumor was totally excised. The pathogenic and clinical aspects are discussed.

The ultrastructure of three cases of osteoblastoma is described. The osteoblasts resemble normal osteoblasts with a few exceptions: irregular, indented nuclei, and occasional mitochondria with curved cristae and electron-lucent areas. The osteocytes and osteoclasts basically resemble their normal counterparts. There are also osteoprogenitor cells in different stages of maturation, some containing glycogen. The ultrastructure of an additional case histologically classified as aggressive osteoblastoma is described. It is essentially similar to the previous three cases of typical osteoblastoma. The only exception is the presence of osteoblasts with electron-lucent nuclei and less prominent organelles than the typical osteoblastoma cells have markedly indented and multilobed nuclei, dilated rough endoplasmic reticulum and lipid droplets. However, these differences from osteoblastoma cells are not pathognomonic. The final diagnosis of osteoblastic tumors rests at the light microscopy level.

Glycosaminoglycans of human chordoma and a metastasis were studied. The glycosaminoglycans were derived from the extracellular matrix and consisted of chondroitin 4- and 6-sulphate, keratan sulphate and hyaluronate. The ratio of chondroitin sulphate to keratan sulphate was much lower in the metastasis than in the primary. Proteoglycan extracted with 4M guanidinium chloride and purified by associative density-gradient centrifugation was assessed on Sepharose 2B before and after reduction and alkylation. The result of this experiment suggested that only a small proportion of the proteoglycans were aggregated.

A microscopic ectopic remnant of notochord and hyaline cartilage was an incidental autopsy finding within a vertebral body. Such ectopic notochordal vestiges have been hypothesized to be the origin of vertebral chordomas, but very few have ever been reported. Whereas ectopic notochord has been demonstrated regularly in the spheno-occipital region (in both embryos and adults) and in the sacrococcygeal region (in embryos), the occurrence of such tissue rests in other segments of the vertebral column is extremely rare. The possible relationship of ectopic vertebral notochordal rests to associated hyaline cartilage formation and to vertebral chordomas requires further investigations.

Chordomas are slowly growing malignant tumors arising from notochordal rests. They are occurring in adults (50 to 60 year old) and are mainly (85%) located in sacrococcygeal or spheno-occipital regions; other main localization is cervical spine. Chordomas are usually discovered in patients with pain or symptoms due to compression of surrounding viscera. Radiologically it is characterized by association of osteolysis and soft tissues opacity. On macroscopic examination tumoral tissue has mucoid appearance; under microscope it is made up with lobules of epithelial-appearing cells surrounded by acid mucosubstances. Tumorous cells contain glycogen and neutral mucosubstances. They are surrounded by argyrophilic rim due to pericellular condensation of intercellular matrix, well viewed on electron microscope examination. When their cytoplasm is filled with vacuoles, cells take up typical physaliphorous appearance. Chordomas cells express epithelial differentiation antigens (low molecular weight cytokeratins, EMA, CAM 52, HFM 62, even CEA), Vimentin and S-100 Protein: this triple positivity allow differentiation between chordomas and numerous others tumors. Correct treatment of chordoma is achieved with an initially complete excision. Local recurrences are frequent and sometimes inoperable: in this cases radiotherapy alone may be performed (70 grays). Sarcomas (fibroblastic or Malignant fibrous histiocytoma) may occur after radiotherapy or without it. Hematogenous metastasis occur in 10% to 15% of patients. Survival rate at five years is included between 50% and 75%. Chondroid chordoma is a special entity occurring in younger patients (35 year old) and located in spheno-occipital region. In addition to chordomas it contain chondroid (benign or malignant) islands. Mean survival rate (16 years) is far better than for chordoma or chondrosarcoma.

Tissue sections and records of 56 rats with chordoma, identified in the National Toxicology Program's (NTP) data base of approximately 115,000 rats, were examined to determine morphological characteristics, incidence, and aspects of biological behavior. Chordomas occurred in aged rats, originated predominantly in lumbosacral vertebrae, were highly malignant, occurred three times more often in male versus female rats, and commonly produced bilateral posterior paresis, paralysis, and/or distention of the colon and rectum.

The clinicopathological findings in a child with extraskeletal Ewing sarcoma are described. The patient complained of pain in the lower back and difficulty walking. An extraskeletal, epidural, friable tumor, 2-3 cm long was removed from the epidural space. It had no relationship with the bone structures. Light and electron microscopic examination of the tumor led to the diagnosis of Ewing sarcoma. The morphological aspects of this neoplasia and the problem of the differential diagnosis with other small cell tumors of the epidural space are discussed.

The Authors report four cases of spinal hemangiopericytomas. The diagnosis has been ascertained by electron microscopy. Despite the malignant nature, the association of surgery and radiotherapy is interesting in that it prolongs the survival rate. Clinical, neuroradiological, microscopic and ultrastructural features of intraspinal hemangiopericytomas are evaluated in the light of the review of the relevant literature.

The cytologic features of metastatic myxopapillary ependymoma were described. The cytologic material obtained from a metastatic subcutaneous nodule by the thin needle aspiration technique was examined by light and electron microscopy.

A case of neuroblastoma arising in an immature teratoma of the ovary in a 22-year-old woman is reported. Differentiation was grade 3 in the primary and metastases. Metastases to retroperitoneal, mediastinal, and supraclavicular lymph nodes and to bone were diagnosed 2 years after presentation of the primary tumor. Electron microscopic study demonstrated dense-core granules of the neurosecretory elements. Treatment with combination chemotherapy followed by radiotherapy resulted in complete remission which continues to the time of this report, more than 4 years after diagnosis of the primary tumor. Prolonged survival of metastasizing grade 3 immature teratoma is distinctly uncommon. The literature pertaining to this unusual tumor and to neuroblastoma of adults is reviewed.

A giant ganglioneuroma generating a progressive scoliosis in a 16-year-old girl is presented. The interval between the start of the orthopaedic treatment and the diagnosis of the true nature of the disease was more than 4 years, thus allowing the development of a giant partly intracanalicularly partly retroperitonealy expanding tumor mass. The report emphasizes and describes the combined neurosurgical, general surgical and orthopaedic surgical treatment and presents the results of light- and electron microsopical, immunohistochemical and quantitative neurochemical investigations of the resected tumor.

A 62-year-old housewife had a metastasis of granular cell ameloblastoma to the third thoracic vertebra. She had suffered at least four local recurrences during the 38-year duration of the right mandibular tumor and was treated by posterior decompression of the spinal cord with instrumentation. Histologically, the granular cells appeared to be of epithelial origin. Ultrastructurally, the granules were identified as lysosomes, including some that were autophagic. Of 45 reported cases of granular cell ameloblastoma, there have been only three cases with pathologically confirmed metastasis. Their characteristics are long duration of the tumor, multiple local recurrences, and predominance in blacks.

A case of malignant rhabdoid tumor of the spine and a description of its CT findings are presented. The CT demonstrated a heterogeneous contrast enhancing epidural mass associated with severe bony destruction of the adjacent vertebra and cord compression.

Spinal cord compression was induced in Fischer rats by percutaneous inoculation of 10(6) cells of malignant fibrous histiocytoma anterior to the T13 vertebral body. Paraplegia and incontinence occurred in all animals after 14-27 days (median, 23 +/- 3.0 days). Autonomic dysfunction and a measurable increase in tumor volume were documented with the use of computer tomography. The tumor penetrated the vertebral bone, invaded the epidural space, and gradually compressed the lumbar spinal segments. Electron-microscopic examination revealed dilated intermyelin spaces containing exuded homogenous material and extravasated leukocytes and erythrocytes. Myelin breakdown was accompanied by the presence of lipid-laden macrophages. Sequential recording of somatosensory evoked potentials (SEP) revealed a progressive increase in the latency of the cervical responses, which preceded the onset of clinical signs. In the presence of paraplegia, spinal cord conductivity was abolished. The levels of the prostaglandins TXB2, 6-keto-PGF1 alpha, and PGE2 were measured in the compressed and remote spinal cord segments during the presymptomatic and symptomatic periods. Only PGE2 was significantly elevated (P less than 0.001) in the paraplegic rats, all along the spinal cord segments. A significant increase in water content was measured in the compressed lumbar segments in the presymptomatic period, and when paralysis set in it was increased in the adjacent low thoracic area as well. Tissue specific gravity was significantly increased only in paraplegic rats in the compressed (P less than 0.01) and the adjacent low thoracic areas (P less than 0.05) but no significant change occurred during the presymptomatic period. Multiple mechanisms play a role in the pathogenesis of neurologic symptoms in neoplastic spinal cord compression.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

The cytologic, cytochemical, immunocytochemical and ultrastructural findings on the aspirated material are presented for the case of a 57-year-old man with sacrococcygeal chordoma diagnosed by fine needle aspiration biopsy. Cytologically, two types of cellular elements were differentiated: medium-sized cells with few cytoplasmic vacuoles and classic physaliferous cells. Both types showed marked cytoplasmic positivity for keratin and S-100 protein; the absence of nuclear positivity in the physaliferous cells was notable. Ultrastructural study demonstrated the existence of true intracytoplasmic vacuoles and frequent rough endoplasmic reticulum-mitochondria complexes. The cytologic differential diagnosis with chondrosarcoma, myxoid liposarcoma, ependymoma and metastases of mucosecretory carcinomas is reviewed.

A 72-year-old man had developed amiodarone neuropathy. He was found, at biopsy, to have a fascicular neuroma of his right sural nerve, unassociated with his underlying neuropathy, apparently due to blunt trauma, as electroneurographic needling of this nerve could safely be ruled out by the patient and his physicians. Such fascicular neuromas, which may remain without sensory deficits, may develop at an unknown frequency, and may only be uncovered by biopsy--or autopsy--in a coincidental neuropathic process.

The electron microscopic study of a sacral chordoma, the cells of which contain variable amounts of glycogen, allowed to characterize different stages in the evolution of the tumoral cells. The young cells contain almost no glycogen ; progressively it becomes more abundant and fills up almost the whole cytoplasm. At the end of the evolution glycogen disappears while the cell becomes vacuolated. This suggests that enzymatic lysis of this polysaccharide could be the cause of the vacuolation which finally produces the physaliferous cell. Comparison between the cells of the chordoma and those of the normal notochord shows some morphological similarities in the development of these cells. These different data may be useful for a classification of the various types of chordomas.