Yoshiya Shimao

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.

Validation of a newly proposed histopathological classification in Japanese patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis

BackgroundA new histopathological classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis was recently proposed. We evaluated the predictive value of this classification for renal outcome in Japanese patients.MethodsWe enrolled 122 patients with ANCA-associated glomerulonephritis diagnosed at several institutions in Japan between January 2000 and March 2010. Twenty patients were excluded because of observation durations of <1xa0year, and/or because their biopsy specimens contained <10 glomeruli. Renal biopsy specimens were categorized into four classes according to the proposed classification. We evaluated the predictive value of immunohistochemical staining for α-smooth muscle actin (SMA), Wilm’s tumor 1 (WT1), CD68, and cytokeratin for end-stage renal disease (ESRD).ResultsThe study population included 54 men and 48 women. Age, estimated glomerular filtration rate (eGFR), and proteinuria were 66.3u2009±u200911.3xa0years, 21.6xa0ml/min. and 1.10xa0g/24xa0h, respectively. Eighty-six patients were positive for myeloperoxidase-ANCA, five were positive for proteinase 3-ANCA, and 11 were negative for both antibodies. Median follow-up time was 41.0xa0months. Twenty-three patients (22.5%) developed ESRD during the follow-up period. Twelve patients died during follow up; 7/12 patients developed ESRD before death, and 5/12 patients died without ESRD. The incidence of ESRD increased with sequential categories: focal, 2/46 (4.3%); crescentic, 9/32 (28%); mixed, 8/18 (44%); and sclerotic, 4/6 (67%). The focal class had the best renal survival and the sclerotic class had the worst renal survival (pu2009<u20090.001). Kaplan-Meier renal survival analysis was similar to that of the new classification system proposal. In the multivariate analysis, the classification system tended to be a prognostic factor for ESRD (pu2009=u20090.0686, crescentic, mixed and sclerotic vs. focal, hazard ratio (HR) [95% confidence interval, CI]; 2.99 [0.61–22.7], 5.04 [1.11–36.4] and 9.93 [1.53–85.7], respectively). α-SMA-positivity also tended to be associated with ESRD (pu2009=u20090.1074).ConclusionThe new histopathological classification was associated with eGFR at 1xa0year and tended to be associated with ESRD in our Japanese cohort with ANCA-associated glomerulonephritis. α-SMA positivity might be an additional prognostic factor for ESRD.

Wegener’s granulomatosis detected initially by integrated 18F-fluorodeoxyglucose positron emission tomography/computed tomography

Early diagnosis is crucial for effective treatment of Wegener’s granulomatosis, although this disease shows only atypical symptoms in the primary stage. This report describes a patient suspected of having a malignancy based on integrated 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT), which showed increased uptake in pulmonary nodules and nasopharyngeal mucosa. Integrated PET/CT is therefore considered to be useful to confirm the distribution and determine the optimal site for biopsy.

Cerebral phaeohyphomycosis: case report.

Cerebral phaeohyphomycosis is a rare and frequently fatal disease. This disease is often caused by hematogenous spread of pathogens that are inoculated in the skin of the extremities after slight or minor trauma, and its mortality rate is rather high despite aggressive treatment. Our patient presented with headache and pyrexia. She was diagnosed with fungal meningitis and treated by systemic administration of voriconazole (VRCZ). However, after initial improvement, meningitis recurred. MRI of the brain showed multiple small masses in the cerebral hemisphere and she was thus referred to our Department of Neurosurgery. On admission, an examination showed that the masses were deeply located in the brain and were too small to be excised; therefore, treatment with systemic VRCZ and intrathecal amphotericin B was initially selected. However, the intracerebral masses continued to grow; therefore, they were surgically excised. Histological examination of the surgical specimens at that time identified the masses as granuloma caused by infection with Aspergillus niger. After the surgery, her general condition improved; therefore treatment with systemic and intrathecal antifungal agents were continued. However, the intracerebral masses recurred, and despite further aggressive surgical treatment and systemic and intrathecal antifungal administration, she died 43 months after the initial diagnosis. Autopsy examination showed that the cerebral lesions were phaeohyphomycotic granulomas. This paper describes the clinical presentation, histopathological results and treatment for this rare disease.

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 19xa0years after the initial therapy, but without local recurrence. A 37xa0year-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, 19xa0years 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.

Clinical features and treatment outcomes of isolated secondary central nervous system lymphomas in Miyazaki Prefecture

BackgroundSecondary central nervous system lymphoma (SCNSL) without extra-central nervous system (CNS) involvement is characterized by isolated secondary CNS relapse in malignant lymphoma patients. SCNSL is a rare disease, and no standard treatment has yet been established.Patients and methodsTo elucidate the clinical characteristics and outcomes of SCNSL, we retrospectively analyzed 12 patients (median age 67xa0years) in Miyazaki prefecture for the last 5xa0years.ResultsThe initial histological diagnoses of the patients were diffuse large B-cell lymphoma (DLBCL), mantle-cell lymphoma, and adult T-cell lymphoma in 9, 2, and 1 patient, respectively. We focused on analysis of the 9 SCNSL cases originating from DLBCL. The locations of CNS relapse were the cerebral hemisphere, basal ganglia, and cerebellum in 7, 1, and 1 patient, respectively. Three patients were treated with high-dose methotrexate (HD-MTX) therapy; 4 with whole-brain radiation therapy (WBRTX); and 1 with both HD-MTX and WBRTX. The remaining patients were treated with rituximab. Partial remission was achieved in 6 out of 9 patients (67%); the other 3 patients (33%) did not respond to therapy. Median survival of the 9 patients with CNS relapse was 253xa0days; 6 of the 9 patients survived for more than 6xa0months. As of March 2011, 2 HD-MTX group patients but none of the WBRTX group patients were alive.ConclusionsIn this retrospective study, 6 of 9 patients with SCNSL originating from DLBCL survived for more than 6xa0months. Both HD-MTX and WBRTX had clinical benefits in the treatment of SCNSL.

Successful treatment of non-Hodgkin’s lymphoma with rituximab and dose-adjusted CHOP therapy in a patient with concomitant end-stage renal disease requiring haemodialysis

Although malignancy is a fatal complication of end-stage renal disease (ESRD) requiring haemodialysis, successful treatment of haematological malignancies has been rarely reported. We describe the case of a 64-year-old man who presented with non-Hodgkin’s lymphoma (NHL; clinical stage, IVB) concomitant with ESRD. Before chemotherapy, haemodialysis was initiated, and one course of dose-adjusted CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy followed by eight courses of rituximab therapy were administered according to the performance status and degree of organ dysfunction. Consequently, the patient was disease free for 27 months. Thus, rituximab plus CHOP combination therapy was effective for NHL concomitant with ESRD.

Complete response to second-line chemotherapy with sunitinib of a gastrointestinal stromal tumor: A case report

Gastrointestinal stromal tumors (GISTs) are a type of sarcoma, and the most common mesenchymal tumor of the gastrointestinal tract. Systemic chemotherapy is recommended for unresectable or metastatic GISTs. Imatinib is an oral multitargeted receptor tyrosine kinase inhibitor that is effective as adjuvant chemotherapy for primary high-risk cases, and as palliative chemotherapy for unresectable or metastatic cases. For imatinib-resistant cases, second-line chemotherapy with sunitinib is recommended due to significantly longer median progression-free survival and higher response rates compared with a placebo. A 54-year-old woman presented with persistent upper abdominal pain and anorexia. An upper gastrointestinal endoscopy and computed tomography revealed a submucosal tumor of the stomach with no apparent metastases. The patient underwent total radical gastrectomy, and was diagnosed histologically with high-risk GIST for recurrence, therefore, the patient received adjuvant chemotherapy with imatinib. However, multiple liver and lymph node metastases were detected, and the patient received sunitinib therapy. After four cycles of sunitinib, the liver and lymph node metastases disappeared, and a complete response (CR) was achieved. To date, there have been no cases of CR in the prospective clinical trials examining the effects of sunitinib, or in case reports worldwide. Therefore, this is a very rare case report of a patient with metastatic GISTs who achieved CR with sunitinib as second-line chemotherapy.