Yoshiya Inoue

Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia.

Abstract Phosphaturic mesenchymal tumor is a rare neoplasm which causes osteomalacia or rickets. The tumor typically follows a benign clinical course. Even in the rare malignant cases, local recurrence and distant metastasis are uncommon. We report on an example of a malignant phosphaturic mesenchymal tumor which recurred several times over 16 years concurrently causing hypophosphatemia, bone pain, and osteomalacia. Following each surgery, symptoms and hypophosphatemia improved. The patient died of disease 17 years after the first surgery. Histologically, the initial tumor was composed of small spindle cells with clusters of giant cells, prominent blood vessels, poorly formed cartilaginous areas, and crystalline material. Cytological atypia was minimal. Following multiple recurrences, the tumor demonstrated areas of high-grade sarcoma exhibiting marked pleomorphism, numerous mitotic figures, and p53 overexpression. This case illustrates the potential lethality of incompletely removed phosphaturic mesenchymal tumors.

Margin-free spondylectomy for extended malignant spine tumors : Surgical technique and outcome of 13 cases

Study Design. Description of surgical technique and retrospective review of 13 cases. Objectives. To describe the surgical technique of margin-free spondylectomy and the outcome of 13 cases and to discuss the advantages and limitations of the procedure. Summary of Background Data. Recently, spondylectomy became a standard procedure by several pioneers. For extended malignant spine tumors involving pedicles or epidural space, however, performing an “en bloc” resection with a tumor-free margin remains a challenge. Methods. Our procedure consists of a combined anterior and posterior procedure with one or two stages. In the anterior procedure, tumor vertebrae are covered by the pleura or psoas muscles as a barrier. The posterior procedure includes decompression through the intact posterior elements, coverage of the tumor with all possible soft tissue barriers, and en bloc extirpation by rotating the tumor vertebrae around the spinal cord. We performed this procedure in 13 cases: 3 chondrosarcoma, 3 giant cell tumor, 1 osteosarcoma, 1 chordoma, and 5 metastases. Results. Neurologic status and pain improved in all cases except asymptomatic cases. There was no local recurrence, except in 2 cases (chondrosarcoma with extirpation of 5 vertebrae, chordoma with multiple previous surgeries). Two cases of chondrosarcoma were disease-free 14 years and 13 years after surgery, respectively. Conclusion. Although the best chance for a cure in extended malignant tumors of the spine is realized through wide resection, the procedure is not yet standardized. Margin-free spondylectomy is technically demanding, but the procedure can be used with a confidence as a more radical surgery for tumors extending to the epidural space and the unilateral pedicle. A key to success is the surgical technique, including a 360° dissection around the tumor vertebrae, instrumentation, and removal of the lesion with all possible soft tissues maintained intact to function as a barrier, like the dura mater.

HISTOCHEMICAL, IMMUNOHISTOCHEMICAL AND ULTRASTRUCTURAL INVESTIGATIONS OF GIANT CELL TUMORS OF BONE

The origin and characteristics of so‐called stromal cells (stromal cell) and the osteoclast‐like giant cell series of 19 cases of giant cell tumor (G.C.T.) of bone were studied. Immunohistochemically, two interesting cases were found. The stromal cells of one case were α‐1‐antitrypsin positive and those of the other case were α‐1‐antichymotrypsin positive. The histiocytic stromal cells of the latter case seemed to be surely neoplastic since they showed mild to moderate cell atypism. There were foci consisting of fibroblastic cells or osteoid and osteoblasts within the tumor. Those cells in the foci were apparently continuous with the surrounding stromal cells, and they were, therefore, also considered to be neoplastic. These findings strongly indicate that the stromal cells originate from the undifferentiated mesenchymal cells in the bone marrow and may differentiate to osteoblastic, fibroblastic, and histiocytic cells. All cells of these three series were not stained for a high stable form of acid phosphatase (SAPhase). SAPhase activity was demonstrated only in osteoclast‐like giant cells and some mononuclear cells, which are recently believed to be non‐neoplastic. Therefore, the cell atypia of SAPhase negative stromal cells is considered to have a prognostic value.

Accumulation of glycosphingolipids in spinal and sympathetic ganglia of a symptomatic heterozygote of Fabry's disease

Fabrys disease is an X-linked disorder of glycolipid catabolism. We have found a symptomatic heterozygous female with cardiomyopathy and severe pain in the extremities. We studied histochemically and biochemically the accumulated glycolipids in spinal and sympathetic ganglia of the patient. Histochemical examination demonstrated the marked glycolipid deposits that have been observed in heterozygous males in these ganglia. Gas-liquid chromatography (GLC) revealed that these accumulated glycolipids were characterized as globotriaosylceramide (Gb3cer) and galabiosylceramide (Ga2cer). In the heterozygous female, the accumulations of Gb3cer in spinal and sympathetic ganglia were, respectively, 34 and 48 times the amount in normal controls. This is the first report on quantitative and qualitative analyses of the accumulated glycolipids in spinal and sympathetic ganglia of a heterozygous carrier female.

Peripheral carcinoid tumor of the lung with focal melanin production

A case of carcinoid tumor of the lung with focal melanin production was encountered in a 56 year old Japanese woman. The tumor was found 16 years previously by mass survey chest X‐ray and had enlarged two‐fold in the intervening period. The tumor consisted of a variety of tumor cells showing a spindle, polygonal and pleomorphic appearance with abundant vasculature in the stroma. All tumor cells showed argyrophilia, together with a few showing argent‐affinity. Melanin‐containing tumor cells were also present in parts. Ultrastructurally, most tumor cells possessed various numbers of neurosecretory granules and a few of them contained granular type rnelanosomes. Tumor cells were connected with desmosomes and a few of them contained tonofilament‐like microfilaments. Only a few contained both neurosecretory granules and melanin. By immunohistochemistry, serotonin, metenkephalin and β‐endorphin positive cells were observed scattered throughout the tumor. A few tumor cells positive for tyrosine hydroxylase were also detected. Additionally, most tumor cells were positive for keratin. On the basis of these findings, the tumor of the current case is a pulmonary carcinoid tumor with focal melanin production.

CLEAR CELL CHONDROSARCOMAS ARISING FROM RARE SITES

Three cases are reported of clear cell chondrosarcoma arising from unusual sites: talus, rib and vertebra. Radio‐graphically, two tumors showed osteolytic features and the vertebral tumor showed osteoplastic change. Histologically, ell tumors consisted of clear cells that had a centrally piaced nucleus surrounded by clear cytoplasm, osteoclast‐like giant cells, am of conventional chondrosarcoma, and various amounts of reactive bone. The tumor in the talus was initially diagnosed as benign chondroblastoma, but it recurred. The rib tumor showed marked cystic formation stimulating aneurysmal bone cyst. The osteoplastic radio‐graphic feature in vertebral tumor was considered to be due to the abundant bone formation.

Malignant melanoma extending along the ulnar, median, and musculocutaneous nerves: A case report

We analyzed a case of malignant melanoma that resembled malignant peripheral nerve sheath tumor with marked neurotropism. The subungual tumor in the right ring finger extended along the ulnar nerve for a distance of 30 cm, as well as along the median and musculocutaneous nerves, with lymph nodal metastases. The tumor consisted of interlacing spindle-shaped cells with large nuclei and distinct nucleolei. Immunohistochemically, the tumor cells were diffusely positive for S-100 protein. Five years after forequarter amputation, the patient is alive without disease. Malignant melanoma has the potential of invading several major peripheral nerves and must be distinguished from malignant peripheral nerve sheath tumor, which rarely metastasizes to regional lymph nodes.