Late Recurrence in Ovarian Dysgerminoma Presenting as a Primary Retroperitoneal Tumor: A Case Report and Review of the Literature.

Ovarian dysgerminoma is a rare type of germ cell tumor. The majority of patient relapses occur within 2 years of diagnosis. Here, we report the case of a 74-year-old woman with a history of ovarian dysgerminoma 39 years earlier. The patient visited the hospital presenting with heartburn. An abdominal computed tomography (CT) revealed a right retroperitoneal mass, and a primary retroperitoneal tumor was suspected. She underwent surgical resection of the retroperitoneal tumor. Histological examination confirmed a metastatic dysgerminoma to the retroperitoneum. Postoperative CT showed paraaortic and cervical lymph node metastases. The patient was treated with bleomycin, etoposide, and cisplatin chemotherapy. This case demonstrates the difficulties that may be encountered in the differential diagnosis of a retroperitoneal mass and underlines the necessity for understanding a patient's clinical history.

Primitive Neuroectodermal Tumors of the Female Genital Tract: A Morphologic, Immunohistochemical, and Molecular Study of 19 Cases.

Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET. The remaining 4 PNETs were composed entirely of undifferentiated small round blue cells and were classified as Ewing sarcoma/peripheral PNET. Eight PNETs were associated with another tumor type, including 5 ovarian mature cystic teratomas, 2 endometrial low-grade endometrioid carcinomas, and a uterine carcinosarcoma. By immunohistochemistry, 17 PNETs expressed at least 1 marker of neuronal differentiation, including synaptophysin, NSE, CD56, S100, and chromogranin in 10, 8, 14, 8, and 1 tumors, respectively. GFAP was positive in 4 PNETs, all of which were of central type. Membranous CD99 and nuclear Fli-1 staining was seen in 10 and 16 tumors, respectively, and concurrent expression of both markers was seen in both central and Ewing sarcoma/peripheral PNETs. All tumors expressed vimentin, whereas keratin cocktail (CAM5.2, AE1/AE3) staining was only focally present in 4 PNETs. Fluorescence in situ hybridization was successful in all cases and confirmed EWSR1 rearrangement in 2 of 4 tumors demonstrating morphologic features of Ewing sarcoma/peripheral PNET and concurrent CD99 and Fli-1 expression. In conclusion, central and Ewing sarcoma/peripheral PNETs may be encountered in the female genital tract with central PNETs being more common. Central PNETs show a spectrum of morphologic features that overlaps with CNS tumors but lack EWSR1 rearrangements. GFAP expression supports a morphologic impression of central PNET and is absent in Ewing sarcoma/peripheral PNET. Ewing sarcoma/peripheral PNETs lack morphologic features of CNS tumors.

Intravascular large B-cell lymphoma involving mainly the uterus: report of a case using liquid-based cytology of the endometrium.

BACKGROUND: Intravascular lymphoma is a rare subtype of extranodal lymphoma. Most instances of the disease are of B-cell lineage. Diagnosis is difficult because of its nonspecific clinical signs, and many cases are diagnosed at autopsy. Uterine involvement is rare, and it is commonly manifested as genital bleeding. In this case, the chief complaint was fever, which is also very rare. CASE REPORT: A 62-year-old woman presented with fever of unknown origin. Computed tomography revealed no localized lesion except for swelling of the right internal iliac nodes. A cytologic smear of the endometrium by liquid-based cytology demonstrated malignant cells. Based on the curettage material, the lesion was diagnosed as an undifferentiated malignant tumor. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic/paraaortic lymphadenectomy revealed widely scattered lymphoma cells of B-cell lineage mainly in the vascular lumina of the uterus, right ovary and lymph nodes. CONCLUSION: The final histologic type was established on the basis of the surgical material of hysterectomy. Diagnosis was difficult because of prominent cellular atypia and rare location of the tumor. Immunocytochemical examination of liquid-based samples can lead to a correct diagnosis of malignant lymphoma, even at the stage of endometrial cytologic examination.

Late spinal cord metastasis of fourth ventricle ependymoma appeared nineteen years after the initial treatment.

Spinal cord dissemination (metastasis) of a fourth ventricle ependymoma more than ten years after surgical resection is extremely rare. In this report, we present an unusual case of a fourth ventricle ependymoma with metastasis to the thoracic spinal cord 19 years after the initial therapy, but without local recurrence. A 37 year-old patient underwent gross total resection of a fourth ventricle ependymoma and postoperative radiation therapy to the posterior fossa. Computed tomography (CT) scanning and/or magnetic resonance (MR) imaging performed during follow up examinations, conducted annually for ten years after the therapy, revealed no evidence of local tumor recurrence. However, 19 years after the initial treatment, the patient complained of back pain and gait disturbances. MR imaging revealed an intradural extramedullary tumor at the Th2-5 levels. MR imaging of the brain revealed no local tumor recurrence or intracranial tumor dissemination. Cerebrospinal fluid cytology revealed no presence of tumor cells. Total resection of the spinal cord tumor was performed, and the tumor was diagnosed as an ependymoma. We describe the clinical features of this rare lesion and particularly emphasize the need for long-term follow up, for more than ten years after the initial treatment, in patients with fourth ventricle ependymoma.

Malignant fibrous histiocytoma of the thoracic spine manifesting as rapidly progressive compressive myelopathy.

A 75-year-old woman presented with an extremely rare case of malignant fibrous histiocytoma (MFH) arising in the thoracic spine, causing compression fracture and manifesting as rapidly progressive compressive myelopathy resulting in progressive gait disturbance and weakness of the bilateral legs. Computed tomography and magnetic resonance imaging demonstrated a solid enhanced intravertebral mass destroying the vertebral bodies at the T7 and T8 levels. Maximum resection of the lesion was performed through wide laminectomy from T7 through T9, followed by vertebroplasty using calcium phosphate paste and posterior fixation with a spinal fixation system. Histological examination showed proliferation of pleomorphic spindle-shaped cells having large irregular nuclei with hyperchromasia with storiform pattern and collagenous stroma, consistent with a diagnosis of MFH. This case illustrates the possibility of rapid maximum decompression surgery from the posterolateral side if MFH manifests as progressive transverse myelopathy.

Hypereosinophilic syndrome associated with occlusive coronary thrombosis and right ventricular thrombus.

A 44-year-old man with HES who developed a large thrombus in the right ventricle as well as multiple occlusive coronary thrombi died of cardiac failure. Autopsy showed that a large thrombus in the right ventricle was associated with eosinophilic endocarditis. In addition, an occlusive thrombus formed in the circumflex and right coronary arteries with eosinophilic infiltrate in the walls. The findings suggest a causal relationship between coronary thrombosis and eosinophilia. To the authors' knowledge this is the first report to document the clinical and histological findings of coronary thrombosis in a patient with HES.

Standardized uptake values for breast carcinomas assessed by fluorodeoxyglucose-positron emission tomography correlate with prognostic factors.

Several studies have revealed the diagnostic value of fluorodeoxyglucose-positron emission tomography for breast carcinomas. However, breast carcinomas display considerable variation in 18F-labeled 2-fluoro-2-deoxy-D-glucose uptake, and few papers have reported the clinical utility of the standardized uptake values (SUV). The purpose of this study is to investigate the relationship between SUV assessed by positron emission tomography (PET) and the clinicopathological characteristics of breast carcinoma. We reviewed 52 breast carcinomas of 45 patients presented at our department between January 2004 and July 2005. We compared the histopathological findings of the breast carcinomas with the preoperative SUV. Of the 52 breast carcinomas, 49 (94%) were detected by preoperative PET. A positive correlation was found between the SUV and tumor size (P < 0.01), histological grade (P < 0.01), the expression of the estrogen receptor (P < 0.001), progesterone receptor (P < 0.01), and p53 (P < 0.01). The number of metastatic axillary lymph nodes (r = 0.73; P < 0.0001) and the MIB-1 labeling rates (r = 0.5; P < 0.01) correlated with the SUV of the breast carcinomal. No relationship existed between the SUV and the following: histological tumor types (P = 0.07), human epidermal growth factor receptor-2 status (P = 0.10), and the presence of metastatic lymph nodes (P = 0.10). The SUV of the breast carcinomas correlate with several histopathological and immunohistochemical prognostic factors. We can obtain information on the degree of malignancy of the carcinoma and prognostic factors by preoperative PET examination.

Renal biopsy in elderly patients: a clinicopathological analysis.

As the numbers of aging patients with manifestations of renal disease increase, the elderly must frequently undergo renal biopsies. This study examined the characteristics of clinicopathological correlations in elderly patients. Medical and clinical records from renal biopsies registered in two hospitals between January 2000 and December 2004 were reviewed. Among 406 patients (female: male 224/182; age 43.9 +/- 18.8 years, mean +/- SD) who underwent renal biopsies, 61 (15.1%) who were aged 65 years and older (female: male, 29/32; age 72.8 +/- 5.2 years) were selected. The elderly usually underwent percutaneous renal biopsies for renal diseases such as nephrotic syndrome (43%) and acute or rapidly progressive renal failure (A/RPRF, 39%). Focal/segmental glomerulosclerosis (23%), minimal change disease (19%), and membranous nephropathy (15%) are frequently diagnosed based on biopsy specimens from patients with nephrotic syndrome. Among patients presenting with A/RPRF, 17 (71%) and 4 (17%) had pauci-immune, MPO-ANCA positive, crescentic glomerulonephritis and interstitial nephritis, respectively, and benefited from therapeutic intervention. Histopathological and pre-biopsy clinical diagnoses differed in nine (15%) patients. The complication rate after biopsy was low (3%). Primary glomerular diseases presenting with nephrotic syndrome and primary crescentic glomerulonephritis associated with rapidly progressive renal failure were the most frequently diagnosed among the elderly who underwent renal biopsy. Percutaneous renal biopsy provides clinically useful information about the elderly because clinical presentation and the predicted diagnosis sometimes vary.

Melanotic schwannoma of the pancreas: report of a case.

We report a case of pancreatic melanotic schwannoma mimicking an epithelial cystic neoplasm of the pancreas. A 67-year-old Japanese woman underwent routine ultrasonography, which showed a large cystic mass in the head of the pancreas. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) showed an inhomogeneous, round cystic mass, which was encapsulated, in the head of the pancreas. We performed pyrolus-preserving pancreatoduodenectomy under the tentative diagnosis of a cystic neoplasm of the pancreas. The histopathologic diagnosis was benign melanotic schwannoma. The patient is now well after 43 months of follow-up. We describe the CT and MRI findings in detail, and review the literature on previously reported cases of pancreatic schwannoma.

Role of mRNA expression of transcription factors in glucocorticoid sensitivity of peripheral blood mononuclear cells and disease state in rheumatoid arthritis.

OBJECTIVE: To investigate the mechanisms underlying glucocorticoid (GC) resistance in rheumatoid arthritis (RA), we evaluated the suppressive effects of prednisolone (PSL) or methylprednisolone (MPSL) on the blastogenesis of peripheral blood mononuclear cells (PBMC). We also measured the expression of mRNA for transcription factors [GC receptor-alpha (GRalpha) and activator protein-1] known to be involved in the exertion of GC effects. METHODS: Twenty-six patients with RA and 17 healthy subjects were studied. IC50 of PSL and MPSL on the blastogenesis of PBMC stimulated with concanavalin A in vitro was estimated. Transcripts for GRalpha, c-fos, c-jun, and GAPDH genes in PBMC were quantitatively determined by real-time RT-PCR procedures. RESULTS: The amount of c-fos transcript in PBMC from RA patients was significantly high compared to the healthy subjects (p = 0.001). However, no difference was found in the amounts of mRNA of other transcription factors between the patients and healthy subjects. When PSL or MPSL IC50 in patients were directly correlated with patients' characteristics in RA, the duration of disease showed a significant positive correlation with PSL IC50 (p = 0.035). However, no significant association of PSL or MPSL IC50 with GRalpha, c-fos, or c-jun mRNA expression determined by RT-PCR was observed. Additionally, there were significant correlations between the amount of GRalpha mRNA and inflammatory indices such as erythrocyte sedimentation rate (p < 0.001) and C-reactive protein (p < 0.05) in the RA patients. CONCLUSION: Chronic exposure to inflammation in RA suggests a decrease in the GC sensitivity of peripheral lymphocytes. Although c-fos and GRalpha transcripts in PBMC have been implicated in the pathology of RA, the amount of expression of these factors may not be critical for the development of GC insensitivity in the PBMC in RA.

Marked hypocomplementemia and tubulointerstitial nephritis in a male patient with Sjögren's syndrome.

We report a case of marked hypocomplementemia and tubulointerstitial nephritis associated with Sjögren's syndrome (SS) in a male patient. Renal biopsy revealed tubulointerstitial nephritis but did not identify specific immune deposits of the tubulo-interstitium. After steroid therapy, the renal failure and hypocomplementemia diminished. Hypocomplementemia without cryoglobulinemia is not commonly observed in SS patients, and hypocomplementemic tubulointerstitial nephritis was strongly suspected. Hypocomplementemic tubulointerstitial nephritis is rare; only one case has been described in the literature. In our case and the previous case, the patients were elderly men, and they had some similar clinical characteristics. Idiopathic hypocomplementemic tubulointerstitial nephritis resembling our case has been reported. These facts suggest that hypocomplementemic tubulointerstitial nephritis may occur in patients with SS, and such cases may not be as rare as once thought because it might be appropriate to include them in the category of idiopathic cases. Such a syndrome should be included in the differential diagnosis of hypocomplementemia.

Thrombotic thrombocytopenic purpura complicating Sjögren's syndrome with crescentic glomerulonephritis and membranous nephritis.

The association of either thrombotic thrombocytopenic purpura (TTP) or crescentic glomerulonephritis with Sjögren's syndrome is rare. We report a case of TTP appearing after the diagnosis of SjOgren's syndrome with crescentic glomerulonephritis and membranous nephropathy. Circulating immune complex was detected, and immune complex deposits were shown along the capillary walls of renal biopsy specimens. Despite steroid pulse therapy and plasma exchange therapy, the patient died. The etiology of TTP is unclear. This case is important when considering the etiology of TTP related to autoimmune disease.

Adrenal function as assessed by low-dose adrenocorticotropin hormone test before and after switching from inhaled beclomethasone dipropionate to inhaled fluticasone propionate.

Low-dose adrenocorticotropin hormone (ACTH) tests (0.5 microg/L 73 m2) were done before and after switching from inhaled beclomethasone dipropionate to inhaled fluticasone propionate in 12 patients 33-77 years old who had mild-to-severe asthma to compare the effects of these drugs on adrenal function. Low-dose ACTH tests were performed after the subjects had received inhaled beclomethasone dipropionate (200-900 microg/day) for at least 12 wk. Treatment was then switched to inhaled fluticasone propionate (200-600 microg/day) for at least 12 wk, and a second low-dose ACTH test was done. Pulmonary function was assessed on the basis of peak expiratory flow rate (PEFR, % of predicted value). After switching treatment, the daily dose of inhaled corticosteroid decreased by about 40%. Basal serum cortisol and ACTH levels were similar with both treatments. The adrenal response, as assessed by incremental rise in the serum cortisol level (peak minus basal) after ACTH challenge, improved significantly (5.6-7.9 microg/dL, p < 0.01) after switching to fluticasone. All three patients who had lower serum cortisol levels during beclomethasone treatment than during fluticasone treatment showed improvement in both the peak cortisol level and the incremental rise in cortisol. Mean morning and evening PEFRs significantly increased after switching from beclomethasone to fluticasone (morning: 71.2 to 76.0%, p < 0.01; evening: 67.3 to 72.1%, both p < 0.05). The diurnal variation of PEFR significantly decreased from 10.9% to 8.3% after switching treatment (p < 0.01). We conclude that switching from beclomethasone to fluticasone reduces the risk of adrenal dysfunction associated with inhaled steroids and improves pulmonary function.

Large cell neuroendocrine carcinoma of the uterine cervix: a clinicopathological study of six cases.

Six cases of cervical large cell neuroendocrine carcinomas (LCNEC) were found among 972 patients (0.6%) with invasive cervical carcinoma. The patients, who were from 27 to 51 (mean 38) years of age, presented with vaginal bleeding or an abnormal Papanicolaou smear. Five tumors were stage Ib and one was IIa. All patients underwent radical hysterectomy and received adjuvant chemotherapy and pelvic radiotherapy. Four patients died of tumor 6 to 19 months (mean 14 months) postoperatively. On histologic examination, the tumor cells were arranged in an organoid growth pattern and were larger than those of typical small cell carcinoma. Glandular differentiation was present in one case. Mitotic figures ranged from 15 to 45 (mean 29) per 10 high-power fields. Prominent vascular invasion and necrosis was seen in all of the tumors. Each tumor was immunoreactive for chromogranin A and/or synaptophysin. The results of this study confirm the aggressive nature of cervical LCNECs. The recognition of LCNECs is necessary to establish the most effective treatment for these aggressive tumors.

Prognostic value of histologic grading of ovarian carcinomas.

Histologic grading of ovarian carcinomas has prognostic and therapeutic relevance, but although several grading systems have been proposed, no universal grading system has been established. Silverberg's group has recently proposed a simple histologic grading system of ovarian carcinomas. We studied its prognostic value in 70 patients with invasive ovarian carcinomas and compared it with that of histologic typing and clinical staging. Kaplan-Meier survival curves showed the following 5-year survival rate using the Silverberg grading system: grade I (n=21) 91%, grade II (n=20) 64%, grade III (n=29) 38% (p<0.001). Multivariate analysis indicated that the histologic grade, the clinical stage, and clear cell histologic type were significant prognostic factors. The Silverberg histologic grade correlated well with prognosis for all histologic types of ovarian carcinomas except for clear cell carcinoma. It is simple, reproducible, and provides useful prognostic information.

Expression of c-fos, rather than c-jun or glucocorticoid-receptor mRNA, correlates with decreased glucocorticoid response of peripheral blood mononuclear cells in asthma.

Resolution of the molecular mechanism(s) underlying glucocorticoid (GC) resistance is an important clinical problem when performing individualized GC therapy according to the GC response of peripheral cells in asthma. In order to investigate the mechanism(s) underlying the individual differences of lymphocyte GC response, we examined the relationship between lymphocyte sensitivity to GC in vitro and the expression of mRNAs for GC receptor (GR) alpha, GRbeta, c-fos and c-jun, which are reported to be implicated in the regulation of the pharmacological effects of GCs in asthma patients. Twenty-seven patients with bronchial asthma and 14 healthy subjects were included in the study. IC50s of prednisolone and methylprednisolone on blastogenesis of peripheral blood mononuclear cells (PBMCs) stimulated with concanavalin A in vitro were estimated. Transcripts for GRalpha, c-fos, c-jun and beta-actin genes in PBMCs were quantitatively determined by reverse transcription-competitive polymerase chain reaction (RT-cPCR) procedures. GRbeta mRNA expression was examined with an RT-PCR technique. A statistically significant positive correlation was observed between the IC50s for prednisolone (p <0.002) or methylprednisolone (p <0.001) and expression of c-fos mRNA in PBMCs of asthma patients (n = 27). Thus, the increased expression of c-fos mRNA correlated with the decreased responses of PBMCs to prednisolone and methylprednisolone in vitro. In contrast, the expression of GRalpha and c-jun mRNAs did not correlate with the IC50 for prednisolone and methylprednisolone in asthma patients. In addition, no statistically significant difference in IC50s of GCs between asthma patients with PBMCs exhibiting GRbeta mRNA and those without GRbeta mRNA expression was observed. The increased expression of c-fos mRNA suggests to attenuate PBMC response to GCs, which may contribute to progression of GC resistance in asthma. On the other hand, c-jun and GC receptor mRNA expression appears to have less influence on poor GC-response establishment.

[A case of migrainous infarction accompanying idiopathic thrombocytopenic purpura].

We reported a 31 year-old man with repeated episodes of migraine at a frequency of about once a week on and after January, 2000. In January 2001, scintillating scotoma and pulsating headache appeared followed by left hemianopsia. His platelet count decreased to 80,000/microliter and high intensity areas were observed in the right occipital lobe and hippocampal gyrus on the FLAIR image of brain MRI. Subsequently performed brain MRA and vertebral angiography revealed segmental stenosis and obstruction in the right posterior cerebral artery. Under the diagnosis of migrainous infarction, sodium ozagrel and lomerizine hydrochloride were administered. Idiopathic thrombocytopenic purpura was additionally diagnosed based on the decreased platelet count which was then treated with predonisolone. After these treatment, his migraine attack disappeared. In this patient, platelet destruction due to idiopathic thrombocytopinic purpura and subsequent release of serotonin seemed to have involved in the occurrence of migrainous infarction.