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Purpose To determine the effectiveness of carbon ion radiotherapy (CIRT) for localized primary sarcomas of the extremities in a prospective study. Patients and materials From April 2000 to May 2010, 17 (male/female: 12/5) patients with localized primary sarcoma of the extremities received CIRT. The median age was 53years (range: 14–87years). Nine patients had primary diseases and eight had recurrent diseases. Of the 17 patients, eight refused amputation, and the remaining nine refused surgical resection. Tumors were located in the upper limbs in four patients and lower limbs in 13. Histological diagnosis was osteosarcoma in three patients, liposarcoma in two, synovial sarcoma in two, rhabdomyosarcoma in two, pleomorphic sarcoma in two, and miscellaneous in six. The CIRT dose to the limb was 52.8GyE for one patient, 64GyE for three, 70.4GyE for 13 in 16 fixed fractions over 4weeks. Records were reviewed and outcomes including radiologic response, local control (progression-free), and survival were analyzed. Results The median follow-up was 37months (range: 11–97months). Radiological response rate was 65% (PR in 11, SD in 5, and PD in 1). The local control rate at 5years was 76%. The overall survival rate at 5years was 56%. Of the 17 patients, 10 survived without disease progression. Four patients had local recurrences, one was salvaged by repeated CIRT and the other three died due to systemic diseases. Distant failure was observed in six patients. One patient suffered from femoral fracture (grade 3) and received surgical fixation 27months after CIRT. No other severe reactions (grade 3) were observed. Conclusions CIRT is suggested to be an effective and safe treatment for patients who refuse surgery for localized primary sarcomas of the extremities.

The human glycine-N-acyltransferase (hGLYAT) gene and two related-genes (GLYATL1 and GLYATL2) were isolated. Human GLYAT, GLYATL1, and GLYATL2 cDNAs were isolated and shown to encode polypeptides of 295, 302, and 294 amino acids, respectively. GLYAT catalyzes glycine-N-acyltransfer reaction with benzoyl-CoA acting as a typical aralkyl transferase, while GLYATL1 catalyzed glutamine-N-acyltransfer reaction with phenylacetyl-CoA as an arylacetyl transferase. GLYAT was shown to be expressed specifically in the liver and kidney, and the cellular localization of GLYAT protein was restricted to the mitochondria. Interestingly, labeling using highly affinity purified anti-GLYAT antibody revealed that GLYAT expression was suppressed in all hepatocellular carcinomas, but not in other liver diseases. hGLYAT repression in cancerous cells in the liver was controlled at the transcriptional level. hGLYAT is a good candidate as a novel marker of hepatocellular carcinoma and may be a key molecule in the transition between differentiation and carcinogenesis of liver cells.

Objectives This study was carried out to detect vulnerable coronary plaques by color fluorescent angioscopy. Background Collagen fibers (CFs) mainly provide mechanical support to coronary plaques. Oxidized low-density lipoprotein (Ox-LDL) induces macrophage proliferation, which in turn destroy CFs while accumulating lipids. As such, demonstration of the absence of CFs, deposition of lipids, and the Ox-LDL may suggest plaque instability. Methods Fluorescence of the major components of the atherosclerotic plaques was examined by fluorescent microscopy using a 345-nm band-pass filter and 420-nm band-absorption filter (A-imaging). Fluorescence of Ox-LDL was examined using a 470-nm band-pass filter and 515-nm band-absorption filter (B-imaging) and Evans blue dye as an indicator. Fluorescence in 57 excised human coronary plaques was examined by A-imaging color fluorescent angioscopy. Oxidized LDL in 31 excised coronary plaques and in 12 plaques of 7 patients was investigated by B-imaging color fluorescent angioscopy. Results Collagen I, collagen IV, and calcium exhibited blue, light blue, and white autofluorescence, respectively. In the presence of beta-carotene which coexists with lipids in the vascular wall, collagen I and IV exhibited green, collagen III and V white, cholesterol yellow, cholesteryl esters orange fluorescence. Oxidized LDL exhibited reddish brown fluorescence in the presence of Evans blue dye. Therefore, coronary plaques exhibited blue, green, white-to-light blue, or yellow-to-orange fluorescence based on plaque composition. Histological examination revealed abundant CFs without lipids in blue plaques; CFs and lipids in green plaques; meager CFs and abundant lipids in white-to-light blue plaques; and the absence of CFs and deposition of lipids, calcium, and macrophage foam cells in the thin fibrous cap in yellow-to-orange plaques, indicating that the yellow-to-orange plaques were most vulnerable. Reddish brown fluorescence characteristic of Ox-LDL was observed in excised coronary plaques, as also in patients. Conclusions Color fluorescent angioscopy provides objective information related to coronary plaque composition and may help identify unstable plaques.

Background: If oxidized low-density lipoprotein (oxLDL), LDL, lysophosphatidylcholine (LPC) and apolipoprotein B (apoB) can be visualized simultaneously, their roles in the initiation, progression and destabilization of atherosclerotic plaques can be objectively evaluated. Methods and Results: (1) The fluorescence characteristic of each atherogenic substance was investigated by microscopy using a band-pass filter (470 nm) and a band-absorption filter (520 nm) with homidium bromide (Ho) and trypan blue (TB) as indicators. (2) 50 excised human coronary plaques were classified by their autofluorescence into green, greenish-yellow and yellow, and the localization of oxLDL, LDL, LPC and apoB were investigated by color fluorescence angioscopy (CFA). The plaque colors were white, yellow and glistening yellow by conventional angioscopy. (1) OxLDL and LDL exhibited golden fluorescence, whereas LPC and apoB exhibited red fluorescence. (2) By CFA, 16 of 19 greenish-yellow and 1 of 8 yellow plaques exhibited red and golden fluorescence in a mosaic pattern, indicating co-deposition of oxLDL/LDL and LPC/apoB; 3 greenish-yellow and 7 yellow plaques exhibited red fluorescence, indicating solitary deposition of apoB; 23 green plaques infrequently exhibited these fluorescence colors. Conclusions: OxLDL/LDL and LPC/apoB were successfully visualized as co-deposited in greenish-yellow autofluorescence plaques, but only LPC/apoB in yellow autofluorescence plaques. (Circ J 2010; 74: 2181-2186)

A 62-year-old female with primary leiomyosarcoma of the left femur is reported with a review of 21 cases reported in the literature. The resected specimen showed that the tumor extended from the femoral head to the diaphysis for 13 cm in length. The tumor showed mainly intramedullary proliferation, but extraosseous growth was also noted at the great trochanter. Microscopic examination revealed well differentiated leiomyosarcoma characterized by interlacing bundles of fusiform cells with eosinophilic cytoplasm and rod-shaped hyperchromatic nuclei. PAP stain of actin on the tumor cells was positive. On electron microscopy, microfilament of 6-8 nm in diameter, dense bodies, pinocytotic vesicles, marginal attachment plate, and basal lamina were noted. The patient died with pulmonary metastasis, 1 year and 7 months after the operation. An autopsy showed metastases in the right pelvic cavity and bilateral lungs, and confirmed the primary site to be the left femur.

A 57-year-old woman underwent modified radical mastectomy for cancer of the left breast (stage IIB) in February 2004. Invasive lobular carcinoma was diagnosed on histopathological examination. The patient received postoperative chemotherapy and endocrine therapy on an outpatient basis and was observed. In August 2005, anorexia developed. Blood chemical tests showed elevated levels of liver enzymes and bilirubin. Computed tomography (CT) of the abdomen revealed an enlarged duodenum and dilated intrahepatic biliary and pancreatic ducts. Upper gastrointestinal endoscopy showed edema of the duodenum. A biopsy yielded a diagnosis of poorly differentiated adenocarcinoma. Duodenal carcinoma was suspected, and a pancreatoduodenectomy was performed. Duodenal metastasis from invasive lobular carcinoma was diagnosed on postoperative histopathological examination. After surgery, the patient recovered uneventfully and was discharged from the hospital. In March 2006, bilateral hydronephrosis apparently caused by peritoneal metastasis developed, and she subsequently died. Invasive lobular carcinoma is characterized by the development of gastrointestinal metastases and is rarely detected before autopsy. We describe our experience with a patient in whom invasive lobular carcinoma of the breast with metastasis to the duodenal wall was definitively diagnosed on laparotomy.

OBJECTIVE AND IMPORTANCE Peripheral primitive neuroectodermal tumor (pPNET) is a rare type of tumor, most commonly found in the limbs of children and young adults. The authors report an extremely rare case of pPNET located at the jugular foramen whose clinical course demonstrated rapid progression. CLINICAL PRESENTATION A 23-year-old man presented with a 2-month history of progressive hoarseness and dysphagia. The neuroradiological appearance of the lesion was a jugular foramen tumor. INTERVENTION The patient underwent a partial resection of the tumor through a far lateral suboccipital craniotomy. After surgery, the patient experienced an unexpected deterioration in consciousness. Magnetic resonance images on postoperative Day 18 revealed rapid and large expansion of the residual tumor into the posterior fossa. No adjuvant therapy was administered, and the patient died 6 weeks after diagnosis. CONCLUSION The pathological diagnosis of the surgical specimen was pPNET, according to the findings of hematoxylin and eosin and immunohistochemical stainings. To the best of our knowledge, this is the first reported case of pPNET at the jugular foramen.

We report a case of dilated cardiomyopathy with hyperthyroidism. A 28-year-old man was admitted because of congestive heart failure and atrial fibrillation, and was newly diagnosed as having hyperthyroidism. Despite administration of antithyroid medication, he developed recurrent congestive heart failure. An echocardiogram revealed a moderately dilated left ventricle with diffuse hypokinesis. Though his thyroid function normalized, the patient's cardiac dysfunction did not improve. Beta-blocker therapy was begun with subsequent improvement in clinical symptoms. This suggests that beta-blocker treatment may be effective in patients with atrial fibrillation associated with cardiomyopathy and hyperthyroidism.

A 46-year-old man with Crohn's disease was referred to our hospital. In 2007, abdominal pain accompanied by redness and swelling of the right lower quadrant developed. A small bowel series and computed tomography of the abdomen revealed a stricture in the terminal ileum, suggesting a penetration of the abdominal wall. He was transferred to the department of surgery, and the affected portion of the bowel was resected to eliminate the stricture. At laparotomy, the ileum 35 cm proximal to the ileocecal valve adhered to the abdominal wall in the right lower quadrant. The involved site of the ileal wall and a portion of the abdominal wall were resected. Postoperative microscopic examination revealed an invasion of the abdominal wall by an ileal adenocarcinoma; reoperation was therefore performed. Histopathological examination revealed an adenocarcinoma at the previously sutured site of the ileal wall, against a backdrop of Crohn's disease. The postoperative recovery was good, and the patient received chemotherapy. During follow-up, computed tomography and positron emission tomography demonstrated abnormal changes of the abdominal wall, suggesting a recurrence. He received radiotherapy of the abdominal wall. Although he had tentative regression, the patient died 1 year and 9 months after the first operation.

Lysophosphatidylcholine (LPC) is a proinflammatory and proatherogenic substance, and it plays an important role in the initiation, progression, and destabilization of atherosclerotic plaques. If LPC in the vascular wall is visualized in vivo, the mechanisms of atherosclerosis and the effects of medical and interventional therapies on atherosclerosis can be objectively evaluated. Therefore, this study was carried out to visualize LPC in human coronary plaques using a color fluorescence angioscopy (CFA) system. (1) The fluorescence characteristics of LPC were investigated by color fluorescence microscopy (CFM) using Trypan blue dye (TB) as an indicator. For fluorescence imaging, a combination of a band-pass filter (345 nm) and a band-absorption filter of 420 nm (A imaging), or a combination of a band-pass filter (470 nm) and a band-absorption filter of 520 nm (B imaging) was employed. (2) The fluorescence of LPC in the excised human coronary plaques was investigated by CFA and CFM scanning using the same filters as those in CFM. In the presence of TB, LPC exhibited a red fluorescence in both A and B imaging. This red fluorescence color in both A and B imaging was not observed for the other known major substances that constitute the atherosclerotic plaques. This red fluorescence color in both A and B imaging was detected by CFA in both white and yellow plaques that were classified by conventional angioscopy. This fluorescence color was found to be distributed in a web-like or diffuse configuration by CFM scanning. LPC in the human coronary plaques was successfully visualized by CFA using TB as an indicator.

A 63-year-old woman was referred to our hospital because of a right axillary nodule in 2004. Physical examination showed a spherical nodule measuring 0.5cm in diameter in the right axilla. No mass was palpable in either breast. Mammograms were normal. Ultrasonography revealed a subcutaneous hypoechoic mass 0.7mm in maximum diameter in the right axilla. The patient underwent an excisional biopsy. Histological examination revealed an invasive ductal carcinoma (scirrhous carcinoma) in ectopic breast tissue. The patient subsequently underwent a wide local excision of the tissue surrounding the biopsy scar, with axillary lymph node dissection. Histologically, no residual tumor or nodal metastasis was found. Postoperatively, she received endocrine therapy and remains well, without any evidence of recurrence 4 years 10 months after operation. Cancer of the ectopic breast tissue is rare, and most cases present as a solitary axillary mass. Long-term outcomes remain unclear. We present a case of breast carcinoma in the axillary ectopic mammary gland and summarize the clinical features of 94 cases, including ours, in Japan. We also compare long-term survival between ectopic breast cancer and usual breast cancer according to TNM T stage and lymph node metastasis.

Generally, it is considered that visceral fat brings insulin resistance and hyper-insulinemia, in the mechanisms of metabolic syndromes. However, whether hyperinsulinemia brings about accumulation of visceral fat is not clear. We followed a case of insulinoma that caused primary hyperinsulinemia, and measured the change in visceral fat and insulin resistance before and after surgical resection of the insulinoma. A 58-year-old woman was admitted to investigate the cause of spontaneous hypoglycemia. An oral glucose tolerance test (OGTT) showed hyperinsulinemia with a high basal level and a glucagon infusion test showed an abnormally high insulin level. Abdominal computed tomography (CT) scan showed an accumulation of visceral fat. Selective celiac angiography showed a pancreatic tumor shadow. Under a diagnosis of insulinoma, the pancreatic body and tail were removed. At 3 months after the operation, the visceral fat area had decreased from 132.6 to 64.2 cm(2). The fasting serum total cholesterol and triglyceride were also reduced. In addition, high-density lipoprotein cholesterol and preheparin serum lipoprotein lipase mass had increased. The midband on the polyacrylamide gel disc electrophoresis of lipoproteins, which appeared before operation, had disappeared completely. An OGTT showed a non-diabetic pattern after the operation. These results suggest that hyperinsulinemia might be one of the factors that enhance visceral adiposity and insulin resistance.

Primary carcinoid tumor of the testis only accounts for 0.2% of all carcinoid tumors in Japan. The clinicopathological features, including differences between Japanese and Europe/United States (Eur/US) series', which are known in ovarian carcinoids, have not yet been sufficiently recognized. In the present study, five testicular carcinoids from various clinicopathological viewpoints were analyzed, with a review of the literature. All 15 cases in Japan, including 10 previously reported, were insular carcinoids. Three of the 15 cases (20%) were associated with a teratoma. Serotonin production was confirmed in at least 10 cases (five cases in the present study and five cases in previous studies), but carcinoid syndrome was present in only two cases. Three of the 15 cases (20%) showed distant metastases, and the tumors that metastasized were larger than those that did not. These features were almost similar to those observed in the Eur/US series. Three pure carcinoids were also studied for the number of sex chromosomes by fluorescence in situ hybridization (FISH). They did not show a significant numerical abnormality of the X chromosome, which is common in testicular germ cell tumors. Therefore, the genetic background of pure carcinoids might be different from that of common germ cell tumors.

This report concerns a malignant glomus tumor, a rare soft tissue tumor that was examined immunohistochemically and ultrastructurally. It occurred in a 44-year-old male patient who had suffered from dull pain and stiffness in the right thigh for 10 months. Radiographic examination revealed a well-defined osteolytic lesion in the diaphysis of the right femur. Hypervascularity of the tumor was observed angiographically. Computed tomographic and magnetic resonance examinations showed an intramuscular mass invading the marrow space of the femur. Wide resection was performed after open biopsy. Histologically, round to polygonal tumor cells revealed a uniform appearance of round to ovoid nuclei with single large nucleoli and slightly eosinophilic cytoplasm, forming solid sheets of cells interrupted by vessels of varying size. A few mitotic figures and vascular invasion were observed. Immunohistochemically, vimentin and alpha-smooth muscle actin were stained intensely, and muscle actin was positive for tumor cells of the perivascular area. Tumor cells were negative for desmin, factor VIII-related antigen, S-100 protein, neurofilament, cytokeratin, and epithelial membrane antigen. Ultrastructurally, tumor cells were characterized by many cytoplasmic processes, pinocytotic vesicles, plasmalemmal dense plaques, and scattered microfilaments in the cytoplasm. Few cell junctions and focal basement membrane-like structures were observed. No recurrence or metastasis was noted 57 months after operation. This case was considered to be a malignant glomus tumor, that is, a glomangiosarcoma arising de novo.

Background Leiomyosarcoma occurring as a primary cardiac tumor has been known as an extremely rare condition. Previous studies of leiomyosarcoma with rhabdomyoblastic differentiation have conducted to those arisen from another site, and they indicated a poorer prognosis of this tumor. Case presentation A 69-year-old woman was referred to our hospital for an operation concerning umbilical hernia. Subsequent imaging examinations before an operation indicated the presence of primary cardiac malignant tumor due to its atypical shape. And then, it was surgically removed. Histopathologically, tumor cells consisted of two different types: spindle and polyhedral cells. Immunohistochemically, it is interesting to note that 2.1% of spindle cells and 23.1% of polyhedral cells showed positive reactivity for myogenin. Furthermore, we performed double-immunostaining for alpha-smooth muscle actin (SMA) and myogenin. The rates of alpha-SMA and myogenin double negative, alpha-SMA single positive, myogenin single positive, and alpha-SMA and myogenin double positive in spindle cells were estimated as 69.1%, 28.8%, 1.1% and 1.0%, respectively. In contrast, the rates in polyhedral cells were estimated as 76.9%, 0.0%, 23.1%, and 0.0%, respectively. Conclusion Our immunohistochemical evaluation suggested that rhabdomyoblastic differentiation in leiomyosarcoma might be generated not only by de novo generation from mesenchymal cells. To the best of our knowledge, this is the first case of primary cardiac leiomyosarcoma with partial rhabdomyoblastic differentiation.

A case of bilateral renal cell carcinoma with multilocular cysts is described. A 42-year-old female was admitted to our hospital for the purpose of further examination or right renal cystic mass discovered incidentally by ultrasonography. After she was examined by CT-scan and angiography, right nephrectomy was performed under the diagnosis of right renal cancer. Histological diagnosis was multilocular cystic renal cell carcinoma. One year later a small cystic legion was found in the left kidney. Under the diagnosis of left renal cancer, left partial nephrectomy was performed. Histological diagnosis was multilocular cystic renal cell carcinoma same as the right kidney. Multilocular renal cyst is classified into several classes by its style of development. This case of bilateral renal cell carcinoma which grew to be multilocular cysts is the first case in Japan as far as we can search.

A case of true malignant mixed tumor of the submandibular gland is reported. The submandibular tumor, occurring in a 52-year-old man, started to grow rapidly after a long history without any change in size. Surgical resection was carried out and the resected tumor measured 5.5 cm with a cut surface showing mixed solid structures. Microscopically, the tumor had both carcinomatous and sarcomatous elements, the former consisting of poorly differentiated adenocarcinoma with squamous cell differentiation and the latter consisting of osteosarcoma with chondrosarcomatous and fibrosarcomatous elements. A remnant of benign pleomorphic adenoma could also be identified. Immunohistochemical study demonstrated keratin and epithelial membrane antigen in the carcinoma cells and vimentin in all elements of the osteosarcoma. It is assumed from these clinical and histological findings that the tumor had transformed from a pre-existing benign pleomorphic adenoma. Acta Pathol Jpn 40: 137–142, 1990.

A boy 15 months old with diffuse infantile fibromatosis in the thigh is reported. A rapidly growing tumor in the right thigh was the only clinical manifestation, suggesting a malignant soft tissue tumor. Macroscopically, the lesion in the medial vastus muscle with an illdefined border gave a pepper and salt appearance. Histologically, the lesion was characterized by an abnormal proliferation of fibroblasts accompanied by some fibrosis, infiltration of chronic inflammatory cells, formation of lymphoid follicles, proliferation of capillaries and the presence of a small number of mature fat cells. No evidence of recurrence is found at present one and half years after operation.

A case of a 19-year-old man with a right parasellar chondroma examined with computed tomography (CT) and nuclear magnetic resonance (NMR) scan is reported. The literature about CT and NMR scan findings of intracranial chondroma is reviewed. Typical CT findings are a round, high density mass with calcification, that is only slightly enhanced by contrast medium.