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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.

大動脈四尖弁は大動脈二尖弁と同様に大動脈弁機能不全の原因疾患となる先天性弁膜症である. 発生頻度は大動脈二尖弁より少なく稀な疾患である. 今回, 超音波検査により術前に大動脈弁四尖弁と診断し得た2症例を経験したので報告する. 症例1 : 63歳, 男性, ペースメーカー植込み術を受けている. 労作時呼吸困難を自覚するようになり入院した. 心エコー検査の結果, 重度の大動脈弁逆流を認め, 左冠尖と無冠尖の間に副尖を有する大動脈四尖弁であった. 手術で四尖が確認され大動脈弁置換術 (SJM 23 mm) を施行した. 組織像では軽度の線維増生と粘液変性が認められた. 症例2 : 58歳, 女性, 高血圧と気管支喘息のため近医で加療を受けていた. 労作時呼吸困難を自覚するようになり入院した. 心エコー検査の結果, 重度の大動脈弁逆流と大動脈弁狭窄の所見を認めたが, 大動脈弁形態は不明であった. 経食道心エコー検査を施行し, 左冠尖と右冠尖の間に副尖を有する大動脈四尖弁と判明した. 手術で四尖が確認され大動脈弁置換術 (SJM 17 mm) を施行した. 組織像では高度の線維増生と石灰化が認められた. 心エコー検査で大動脈弁膜症の原因を検索する際には大動脈四尖弁の可能性も考慮に入れ慎重に観察する必要がある.

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.

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.

Six new antibiotics, TMC-151 A-F (2–7) have been isolated from organic extracts of the fermentation broth of Gliocladium catenulatum Gilman & Abbott TC 1280, isolated from a soil sample. Their structures including the absolute stereochemistries, were determined by extensive analyses of NMR and X-ray crystallography, and degradation studies. TMC-151s are novel polyketides containing D-mannopyranoside and D-mannitol or D-arabitol. These antibiotics showed moderate cytotoxicity to several tumor cell lines.

Four new antibiotics, TMC-171A (2), B (3), C (4) and TMC-154 (5) have been isolated from the fermentation of fungal strains Gliocladium sp. TC 1304 and TC 1282, respectively. Spectroscopic and degradation studies have shown that TMC-171s and TMC-154 were new members of the TMC-151 class of antibiotics, unique polyketides modified with a D-mannose and a D-mannitol or a D-arabitol. These compounds showed moderate cytotoxicity to various tumor cell lines.

膣原発の明細胞腺癌を経験したので報告する.症例は不正性器出血, 排尿障害を主訴とした40歳の女性.膣前壁に易出血性の腫瘍が認められ, 病理学的検索が行われた.細胞診では, 腫瘍細胞はシート状あるいは乳頭状の集団として認められた.細胞質はライトグリーン淡染性で広く, 核には大小不同が目立ち, 微細穎粒状のクロマチン, 1個ないし数個の明瞭な核小体を有していた.裸核状の大型細胞の混在もみられた.明細胞腺癌が疑われたが, hobnail cellは明らかでなかった.組織学的には腫瘍細胞は淡明で比較的豊富な胞体と核小体の目立つ核をもち, 主として乳頭状, 部分的には管腔形成性あるいは充実性に増殖していた.乳頭状部分にはhobnail cellも見出された.胞体にはグリコーゲンと考えられるPAS染色陽性細穎粒が認められた.鼠径部リンパ節生検にて転移が確認されたが, 種々の画像検査, 膀胱鏡, 子宮および尿細胞診にて転移性腫瘍が否定され, 膣原発明細胞腺癌と診断された.

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.

Four new antibiotics, TMC-171A (2), B (3), C (4) and TMC-154 (5) have been isolated from the fermentation of fungal strains Gliocladium sp. TC 1304 and TC 1282, respectively. Spectroscopic and degradation studies have shown that TMC-171s and TMC-154 were new members of the TMC-151 class of antibiotics, unique polyketides modified with a D-mannose and a D-mannitol or a D-arabitol. These compounds showed moderate cytotoxicity to various tumor cell lines.

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.

The case is a 60-year-old woman who had complained of right coxalgia. At the first time, there was no fracture on roentgenograms of the hip. Significantly elevated erythrocyte sedimentation rate (ESR) level was found. After a month, the pathological fracture of the right femoral neck was fonud. The case was diagnosed as the multiple myeloma by open biopsy. And then the operation of endoprosthesis was performed. It is difficult to diagonose multiple myeloma without apparent fracture, but it is useful to examine bone scintgraphy and blood test.

最近我々の経験した肺原発悪性線維性組織球腫 (MFH) 4例について臨床病理学的検討を行った. 全例男性で高喫煙歴があった. 全例赤沈が亢進し, 血清鉄が低値を示した. 腫瘍マーカーでは, フェリチン, 免疫抑制酸性蛋白 (IAP) が高値を示した. 放射線治療を行った症例ではフェリチン, IAPが治療効果と関連して変動を示した. 2症例で白血球増多がみられ, サイトカインとの関連が示唆された. 術前の生検診断に際しては免疫組織化学染色が有用であり3例中2例でMFHと診断し得た. 予後は極めて不良で外科療法, 化学療法, 放射線療法にもかかわらず全例診断後1年以内に死亡した. 剖検では, 全例局所再発もしくは遠隔転移を認めた. 今後の集学的治療の確立が課題であろう.

Spontaneous CSF otorrhea is a rare condition, and about forty cases have been described in the literature to date. Here a case of spontaneous CSF otorrhea is reported.A 37-year-old man had suffered from recurrent bacterial meningitis and CSF otorrhea. Left mastoidectomy was carried out on May, 1989, by an otologist in the other hospital, but the origin of CSF leakage coudn't be identified. The patient was admitted to our hospital on January, 1990, to treat CSF otorrhea. Left temporal craniotomy was carried out. A dural defect was found in the left middle cranial fossa connecting to a bony defect at the left tegmen tympani. The defect was repaired by packing the tegmen tympani with fascia and fatty tissues. CSF otorrhea completely disappeared postoperatively.Spontaneous CSF otorrhea should be considered as a cause of recurrent bacterial meningitis. High resolution CT scan and MRI will be very useful in the diagnosis of this rare problem.

This paper proposes a technology for histopathological diagnostic support that utilizes the correlation-based features of histopathological tissues. In histopathological diagnosis, a clinical pathologist conducts a diagnosis of normal tissues and cancerous tissues. However, recently, the shortage of clinical pathologists is posing increasing burdens to meet the demands for such diagnoses, and this is causing serious social problems. In order to overcome this problem, we propose a technology of histopathological diagnostic support that uses higher-order local autocorrelation (HLAC) features. The proposed method can automatically screen tissue that is believed to be normal tissue to detect cancerous tissue as well as tissue that is suspected of being cancerous to detect abnormalities. Consequently, we can reduce the burden on clinical pathologists, allowing them to concentrate on diagnosing cancer.

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.

A case of mucoepidermoid carcinoma of the larynx is reported. The patient was a 63-year-old man who had suffered from sore throat for nine months. By physical examination, swelling in the right epiglottis and arytenoid was noted. Histopathological findings of the specimen showed a mucoepidermoid carcinoma (low grade malignancy). The patient had chemotherapy and radiotherapy. Among malignant tumors of the larynx, mucoepidermoid carcinoma seemed to be extremely rare. Only seven cases of the laryngeal mucoepidermoid carcinoma including this case have been reported in our country. A pertinent literature was reviewed in this paper.

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.

An 82 years old man with four large tumors on his left forearm visited our hospital in July 1991. At first these tumors were suspected to be malignant. Xray, bone-scintigram and angiogram of the forearm suggested malignant tumors. There was no evidence of tuberculosis on the chest Xray when first taken. Six months later the tumor developed, producing a fistula and the patient decided to be admitted for surgery. A diagnosis of tuberculosis was made and administration of INH RFP EB was effective. It is important to be aware that tuberculosis sometimes closely resembles malignant tumors. Therefore, if a malignant tumor is suspected biopsy is necessary to make a diagnosis, keeping tuberculosis in mind.

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 case of a sclerosing variant of osteosarcoma of a toe phalanx is reported in a 28-year-old man. This represents the first reported case of osteosarcoma of any kind at this site. This is based on a review of 4,214 cases of conventional osteosarcoma. The reason for the extraordinary rarity in toe or hand phalanges is unknown although osteosarcoma is the second most common primary tumor of bone. Since the neoplasm had minimal signs of cytologic anaplasia, it was originally mistaken for and treated as an osteoid osteoma. The lesion recurred and extended into soft tissues. Reevaluation revealed the tumor to be an osteosarcoma, sclerosing variant with "normalization" of nuclei. The lesions that this tumor should be distinguished from are osteoid osteoma and osteoblastoma.

An example of multicentric, skeletal, myxoid angioblastomas in a Japanese woman is reported. The disease was symptomatic at age 12 years and was characterized by slowly progressive, multiple, lytic bone defects. In addition the patient had juvenile hypertension, and, at age 20 years, had focal brain infarction. The primitive vascular nature of the process was supported by the following observations: occasional erythrocytes within cytoplasmic lumina and capillary-like cellular tubes; Weibel-Palade bodies, numerous pinocytotic vesicles, prominent microvilli, elaborate intercellular contacts, desmosomes, and numerous arrays of fine intracytoplasmic filaments by electron microscopy; and, in addition, Factor VIII positivity. The clinical findings in this case are more consistent with a multicentric, rather than a metastatic process. The name myxoid angioblastomatosis of bones is appropriate.

乳腺原発骨肉腫の発生頻度は比較的低く, 全乳腺悪性腫瘍の0.5～3.0%とされており, 本邦における既報告例も十数例を数えるに過ぎない.本症例は49歳・女性の左乳房下にみられた小腫瘤で, 化骨性筋炎に類し, 異型類骨組織, 異型軟骨組織および線維肉腫様組織が層状配列 (Zoning) を示していた.細胞診では偏心性の不正円形核, 陥入や切れ込みを有し, 粗穎粒状の分散クロマチンと核縁の肥厚が目立ち, 胞体限界の不整な異型骨芽細胞ど類円形核をもち, 核縁が菲薄で, 核質の明るい異型軟骨芽細胞を類別し得た. さらに, 長楕円形核を有する線維芽細胞様細胞および多核の異型巨細胞を認めた. これらの細胞は, 光顕および電顕的観察より得られた細胞学的特異性をおおむねよく反映しており, 塗抹標本における背景への配慮も診断上きわめて重要なことを示唆していた.