Survival and Prognosis of Patients with Pilocytic Astrocytoma: A Single-Center Study

BACKGROUND: Pilocytic astrocytoma (PA) is a brain tumor that is relatively more common in children and young adults. METHODS: We retrospectively reviewed the medical records of patients with PA treated at a single center between 1988 and 2018. RESULTS: We included 31 subjects with PA. The median age at diagnosis was 13.4 years, and the median follow-up duration was 9.9 years. The total PA group had a 10-year disease-specific survival (DSS) rate of 92.6% [95% confidence interval (CI), 82.6–100] and 10-year progression-free survival (PFS) rate of 52.8% (95% CI, 32.0–73.6). In patients aged <20 years, tumors were more likely to be located in sites in which gross total tumor resection (GTR) was impossible. No statistically significant difference in 10-year DSS was found between the GTR (100%) and non-GTR (89.7%; 95% CI, 76.2–100; p=0.374) groups. However, a statistically significant difference in 10-year PFS was found between the GTR (100%) and non-GTR groups (30.7%; 95% CI, 8.6–52.8; p=0.012). In the non-GTR group, no statistically significant difference in 10-year DSS was found between the patients who received immediate additional chemotherapy and/or radiotherapy (Add-Tx group, 92.9%; 95% CI, 79.4–100) and the non-Add-Tx group (83.3%; 95% CI, 53.5–100; p=0.577). No statistically significant difference in 10-year PFS was found between the Add-Tx group (28.9%; 95% CI, 1.7–56.1) and non-Add-Tx group (33.3%; 95% CI, 0–70.9; p=0.706). CONCLUSION: The PFS of the patients with PA in our study depended only on the degree of surgical excision associated with tumor location. This study is limited by its small number of patients and retrospective nature. A multicenter and prospective study is necessary to confirm these findings.

Amoebic Encephalitis Caused by Balamuthia mandrillaris

We present the case of a 71-year-old man who was diagnosed with amoebic encephalitis caused by Balamuthia mandrillaris. He had rheumatic arthritis for 30 years and had undergone continuous treatment with immunosuppressants. First, he complained of partial spasm from the left thigh to the left upper limb. Magnetic resonance imaging revealed multifocal enhancing nodules in the cortical and subcortical area of both cerebral hemispheres, which were suggestive of brain metastases. However, the patient developed fever with stuporous mentality and an open biopsy was performed immediately. Microscopically, numerous amoebic trophozoites, measuring 20 to 25 µm in size, with nuclei containing one to four nucleoli and some scattered cysts having a double-layered wall were noted in the background of hemorrhagic necrosis. Based on the microscopic findings, amoebic encephalitis caused by Balamuthia mandrillaris was diagnosed. The patient died on the 10th day after being admitted at the hospital. The diagnosis of amoebic encephalitis in the early stage is difficult for clinicians. Moreover, most cases undergo rapid deterioration, resulting in fatal consequences. In this report, we present the first case of B. mandrillaris amoebic encephalitis with fatal progression in a Korean patient.

Bile Granuloma Mimicking Peritoneal Seeding: A Case Report

Laparoscopic cholecystectomy is a widely used treatment method for most cholelithiasis and is a relatively safe procedure. Foreign body granulomatous reaction to bile or gallstone spillage during laparoscopic cholecystectomy has rarely been reported. We report a case of bile granuloma after laparoscopic cholecystectomy, which mimicked peritoneal seeding. A 59-year-old Korean man presented with right upper quadrant pain. He underwent laparoscopic cholecystectomy for acute cholecystitis with cholelithiasis. Pathologic examination revealed an incidental adenocarcinoma invading the lamina propria with acute cholecystitis and cholelithiasis. After 3 months, follow-up abdominal computed tomography revealed a subhepatic nodule, which showed hypermetabolism on positron emission tomography–computed tomography. Suspecting localized peritoneal seeding, wedge resection of the liver, wedge resection of the transverse colon, and omentectomy were performed. Pathologic examination of the resected specimens revealed multiple bile granulomas. Awareness of bile granuloma mimicking malignancy is noteworthy for patient management to reduce unnecessary procedure during postoperative surveillance.

Long-term prognosis of BK virus-associated nephropathy in kidney transplant recipients

BACKGROUND: The long-term prognosis of BK virus-associated nephropathy (BKVAN) in kidney transplant recipients (KTRs) is uncertain. We evaluated the long-term prognosis in KTRs with BKVAN and the clinical significance of BKVAN on post-transplant clinical outcome. METHODS: We retrospectively analyzed the medical records of 582 patients who underwent kidney transplant (KT) between 2001 and 2014. We divided the patients into a BKVAN group (15 patients) diagnosed by allograft biopsy and a control group (356 patients). RESULTS: The incidence of BKVAN was 4.0%, and the mean follow-up duration was 93.1 ± 52.3 months. Median time from KT to BKVAN diagnosis was 5.9 months (interquartile range [IQR], 4.4–8.7). In the BKVAN group, 9 (60.0%) KTRs with combined acute rejection progressed to graft failure, and the median time from BKVAN diagnosis to graft failure was 36.2 months (IQR, 9.7–65.5). Death-censored graft survival rate and patient survival rate in the BKVAN group were significantly lower than those in the control group. BKVAN and rejection were independent risk factors for graft failure. In the subgroup analysis, death-censored graft survival rate of KTRs with BKVAN with acute rejection was significantly worst in comparison with similar patients without BKVAN regardless of acute rejection (P < 0.001). CONCLUSION: The long-term prognosis of BKVAN with acute rejection was very poor because of graft failure caused by inadequate treatment for acute rejection considering BKVAN. Therefore, we should carefully monitor the allograft status of KTRs through regular surveillance tests after treatment for BKVAN with acute rejection.

Nevoid Melanoma in the Forearm / 계명의대학술지

Nevoid melanoma is a very rare histological subtype of vertical growth phase melanoma. Histologically, it mimics benign nevus and thus may lead to an erroneous diagnosis. We report a case of nevoid melanoma arising in a 53-year-old American woman. High index of suspicion and evaluation of cytologic atypia with ancillary tests may help in establishing the diagnosis.

IMP3, a Promising Prognostic Marker in Clear Cell Renal Cell Carcinoma

BACKGROUND: Insulin-like growth factor II mRNA-binding protein 3 (IMP3) has been reported as a prognostic biomarker in various cancers. To validate IMP3 as a prognostic biomarker in renal cell carcinoma (RCC), we investigated the expression of IMP3, p53, and Ki-67, and their associations with clinicopathologic outcomes. METHODS: We studied 148 clear cell RCCs (CCRCCs) from patients who underwent radical nephrectomy. The expression levels of IMP3, p53, and Ki-67 were assessed by immunohistochemical staining and the clinical and pathologic parameters were retrospectively reviewed. RESULTS: Twenty-nine percent of CCRCCs expressed IMP3. Forty-one percent of IMP3-immunopositive tumors developed metastases, while only 11.4% of IMP3-negative tumors developed metastases (p<.001). A Kaplan-Meier curve showed that patients with IMP3-immunopositive tumors had lower metastasis-free survival and cancer-specific survival than did those with IMP3-immunonegative tumors (p<.001 and p<.001, respectively). Expression of high Ki-67 proliferation index was also associated with a higher metastatic rate. In the multivariate Cox regression analysis, pT stage and IMP3-positivity were independently associated with disease-specific survival. CONCLUSIONS: IMP3 is an independent prognostic biomarker for patients with CCRCC to predict metastasis and poor outcome.

Prognostic Significance and Nature of Rhabdoid Features in Renal Cell Carcinoma

BACKGROUND: Recent reports have indicated that renal cell carcinoma (RCC) with rhabdoid features follows an aggressive clinical course. We investigated the prognostic significance and nature of the rhabdoid component. METHODS: We retrospectively analyzed the incidence and clinicopathologic characteristics of RCC with rhabdoid features in 174 radical nephrectomy cases. The specimens were examined histologically and immunohistochemically. RESULTS: Twelve of the 174 RCC cases (6.9%) showed rhabdoid features. Histologically, all the tumors with rhabdoid features were of the clear cell type. The presence of rhabdoid features was significantly associated with higher Fuhrman's nuclear grade and higher pathologic tumor stage at presentation. Among the 12 patients who showed the rhabdoid component, nine (75%) developed metastasis and seven (58.3%) died of disease-related causes. The presence of rhabdoid features was independently associated with metastasis and disease-related mortality. The rhabdoid cells were positive for vimentin; variably positive for pan-cytokeratin, epithelial membrane antigen, and CD10; and negative for cytokeratin 7, smooth muscle actin, desmin, E-cadherin, and c-Kit. No case showed loss of integrase interactor-1; one was p53 positive, and five were insulin-like growth factor mRNA binding protein 3 positive. The Ki-67 labeling index was 1-25% (mean, 5.5%). CONCLUSIONS: The rhabdoid component is an independent prognostic factor for metastasis of RCC; therefore, identification of this component is critical.

Sporadic Cutaneous Keratocyst without Nevoid Basal Cell Carcinoma Syndrome: Report of 1 Case

A cutaneous keratocyst is very rare and is ordinarily associated with nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome. NBCCS is a rare autosomal-dominant disorder that results from the mutation in the patched homologue 1 (PTCH1) gene located on chromosome 9q22.3, with high penetrance and variable expressivity. NBCCS demonstrates multisystem manifestations such as multiple basal cell carcinomas in early age, jaw cysts and pits of the hands and feet. Cutaneous keratocysts are characteristically lined by festooned keratinized squamous epithelium with parakeratosis. The cystic wall contains neither granular cell layer nor skin appendages. To the best of our knowledge, only two cases of cutaneous keratocysts not associated with NBCCS have been reported to date. We report one another case of a histologically confirmed cutaneous keratocyst in a 50-year-old female without a family history and clinical features of NBCCS.

Brucella Prostatitis: A First Case Report Diagnosed in Korea

Brucellosis is a zoonosis caused by several species of Brucella. Brucellosis is usually an acute or sub-acute febrile illness that histologically develops granulomatous inflammation. Brucella prostatitis is a very rare complication and is usually accompanied by epididymo-orchitis. We now report a case of histologically proven granulomatous prostatitis due to Brucella without clinical evidence of epididymo-orchitis. A 61-year-old farmer presented with myalgia, low back pain, and fever. A needle biopsy of the prostate was performed due to symptoms of urinary frequency and high prostate specific antigen levels (17.3 ng/mL). Histologically, the prostate showed granulomatous inflammation without caseous necrosis. Polymerase chain reaction (PCR) studies of blood and prostatic tissue for Brucella were positive, while a PCR study for Mycobacterium tuberculosis was negative. The patient was treated with doxycycline and rifampin. A possibility of Brucella prostatitis should be considered in the differential diagnosis of granulomatous prostatitis or prostatitis of unknown origin associated with or without epididymo-orchitis.

Clinical outcomes of Asymptomatic Urinary Abnormalities in Adults / 대한신장학회지

PURPOSE: Urinalysis is one of the best methods for early detection of renal disease and recent wide- spread use of mass screening led to increasing prevalence of asymptomatic urinary abnormalities. Usually, primary chronic glomerulonephritis first presents with asymptomatic urinary abnormalities and chronic glomerulonephritis commonly causes end-stage renal disease. However, clinical outcome of asymptomatic urinary abnormalities in adults is not well known. METHODS: Between Jan 1995 to Aug 2009, 333 patients with asymptomatic urinary abnormalities who underwent percutaneous renal biopsy were enrolled. A retrospective study was performed to clarify the prognostic factors and the long-term renal outcome of this disease. RESULTS: According to clinical manifestation, there were 79 (23.7%) of isolated microscopic hematuria, 30 (9.0%) of isolated proteinuria and 224 (67.3%) of mixed hematuria and proteinuria. The patients were significantly younger in case with microscopic hematuria. Group with microscopic hematuria had significantly shorter follow up period (p=0.013). In pathologic diagnosis, IgA nephropathy was most common with 244 patients (73.3%). The proteinuria group and mixed group showed significantly higher rate of progression to chronic renal failure than the microscopic hematuria group (p=0.015). The group that 24-hour proteinuria was more than 0.5 g/day showed significantly higher progression rate to chronic renal failure (p<0.000). Using univariate regression analysis, 3 risk factors for progression to chronic renal failure were identified: age, serum creatinine, 24-hour total urine protein. In multivariate regression analysis, only 24-hour proteinuria was the independent prognostic factor for progression to chronic renal failure. CONCLUSION: IgA nephropathy is the most common cause of asymptomatic urinary abnormalities in adults. The group of proteinuria has higher progression rate to chronic renal failure than other groups. Over 0.5 gm of 24-hour proteinuria is a significant risk factor for progression to chronic renal failure in multivariate regression analysis.

De Novo Light Chain Deposition Disease in Long-term Survived Renal Allograft / 대한신장학회지

Light chain deposition disease (LCDD) is characterized by the deposition of abnormal immunoglobulin light chains in many organs, including kidney. It is usually associated with multiple myeloma or other lymphoproliferative disorders. Myeloma usually occurs in old age and may develop after renal transplantation thus being categorized as posttransplant lymphoproliferative disease (PTLD). Renal LCDD usually presents with variable degree of proteinuria and renal insufficiency. The diagnosis of LCDD depends on histologic findings with detection of monoclonal immunoglobulin light chain. Histologically, it is characterized by nodular glomerulosclerosis. We report the first case of de novo LCDD associated with myeloma after renal transplantation in Korea. With advancing renal transplantation and increasing old aged renal recipients, myeloma or LCDD should be included in the differential diagnoses of renal recipient patients with deteriorating renal function.

Efficacy of Mycophenolate Mofetil in the Treatment of Refractory Membranous Nephropathy and Focal Segmental Glomerulosclerosis / 대한신장학회지

PURPOSE: This study was planned to determine the efficacy and safety of mycophenolate mofetil (MMF) as a rescue treatment in patients with membranous nephropathy (MN) and focal segmental glomerulosclerosis (FSGS) who were not responsive to standard therapy with steroid and immunosuppressive regimen. METHODS: We planned a prospective, non-randomized study from Oct. 2002 to Aug. 2009, including biopsy-proven MN or FSGS patients in Keimyung university Dongsan hospital. MMF was initiated at 0.5-0.75 g twice daily, and advanced as appropriate or as tolerated to 0.75-1 g twice daily. RESULTS: 14 cases with MN and 5 cases with FSGS was enrolled. The mean age of patients was 51.7+/-12.3 years, and mean treatment duration was 14.4+/-6.5 months. Five patients (26.4%) went into complete remission and the seven (36.8%) into partial remission. The mean value of 24hr total urine protein over the follow-up 6 months' period declined significantly from 7.6+/-6.2 g in pre-treatment, to 4.1+/-3.2 g in 3 months, and 3.1+/-2.1 g in 6 months (p=0.011). The mean 24hr total urine protein decreased from 7.5+/-6.3 g in pre-MMF to 1.9+/-1.8 g in post-MMF (p=0.001). The mean serum albumin rose from 3.2+/-0.8 g/dL in pre-MMF to 3.9+/-0.5 g/dL in post-MMF (p=0.001). There were no significant changes in mean value for WBC, hemoglobin, serum creatinine, and total cholesterol. Side effects of MMF were infrequent and generally mild. CONCLUSION: MMF appears effective in 63% of patients with MN and FSGS who are resistant to other forms of treatment. Studies with more cases and multicenter controlled trials are required to establish the role and standards of MMF in these disorders.

Solitary Polypoid Ganglioneuroma of the Cecum

A ganglioneuroma is a very rare neoplasm in the gastrointestinal tract and consists of ganglion cells, nerve fibers, and supporting cells. A gastrointestinal ganglioneuroma is occasionally related to inherited diseases, like neurofibromatosis type I and multiple endocrine neoplasm type 2b. We have experienced a case of a solitary polypoid ganglioneuroma in the cecum of a patient with no history of inherited diseases. The patient was a 56-yr-old male who had suffered from dyspepsia for a year. On the colonoscopic examination, a sessile polyp, measuring 0.7 x 0.7 cm in greatest dimensions, was discovered and eliminated. The remaining large intestine was unremarkable. Microscopically, the polyp was composed of isolated or nested ganglion cells admixed with a proliferation of spindle cells in the mucosa and the submucosa. The background showed interspersed cystic glands in an expanded lamina propria. Immunohistochemically, the ganglion cells were positive for NSE and NeuN while the spindle cells demonstrated a positive response to S-100 protein. Since a ganglioneuroma has a benign nature, complete resection is the treatment of choice.

A case of TSH-secreting pituitary adenoma with acromegaly / 대한내과학회지

Growth hormone (GH) and thyrotropin (TSH)-secreting pituitary adenomas are very rare and account for only 0.5% of all pituitary adenomas. We report a case of a GH/TSH-secreting pituitary adenoma in a 53-year-old male patient. He presented with symptoms of thyrotoxicosis, clinical features of acromegaly, and diabetes mellitus. The laboratory examinations showed high serum levels of free T4, TSH, and free alpha-subunit. Additionally, serum levels of GH and insulin-like growth factor (IGF-1) wereincreased. GH was not suppressed below 1 microgram/L by an oral 75 g glucose loading test, and TSH was not stimulated by thyrotropin- releasing hormone. A sellar MRI showed a large lobulated mass on the pituitary gland, so transcranial surgery was performed. Immunohistochemical staining showed anti-GH and anti-TSH positive tumor cells in the cytoplasm. Serum GH, IGF-1, free T4, and TSH levels normalized after surgery.

Altered Expression of beta3 Integrin on Sclerotic Aortic Valves in a Hypercholesterolemic Rabbit Model / 대한흉부외과학회지

BACKGROUND: Although aortic valve sclerosis causes no significant hemodynamic alterations, it is associated with an increased risk of cardiovascular death and myocardial infarction. However, the role of beta3 integrin in aortic valve sclerosis remains unclear. MATERIAL AND METHOD: Twenty male New Zealand rabbits were divided into two groups. Group 1 rabbits (n=10) received a normal chow diet, while group 2 (n=10) rabbits received a diet containing 1% cholesterol for 12 weeks. After the rabbits were euthanized, their aortic valves and ascending aortas were excised for analysis. RESULT: Total serum cholesterol (2,148.3+/-1,012.5 mg/dL versus 53.7+/-31.8 mg/dL, p<0.05), triglyceride (240.4+/-218.3 mg/dL versus 31.6+/-6.4 mg/dL, p<0.05), and low density lipoprotein (LDL)-cholesterol (2,065.3+/-960.9 mg/dL versus 29.1+/-30.9 mg/dL, p<0.05) levels were significantly higher in the cholesterol diet group compared with the normal diet group. Myofibroblasts and macrophages were more highly expressed in the aortic valve leaflets of rabbits in the cholesterol diet group than of those in the normal diet group. A real-time polymerase chain reaction revealed decreased beta3 integrin mRNA levels in the hypercholesterolemic aortic valves and aortas. CONCLUSION: The present study shows that hypercholesterolemia induces aortic valve sclerosis. These findings suggest that alterations in beta3 integrin may play a role in the development of aortic valve sclerosis.