Chondroid tenosynovial giant cell tumor of the toe: A case report / 동물의과학연구지

Localized tenosynovial giant cell tumor (TGCT) usually occurs in the hand and foot regions. However, localized TGCT with extensive cartilaginous metaplasia is rare, especially in the tendon sheath of the toe. Here, we report a case of localized TGCT with cartilaginous metaplasia in a 57-year-old man. The tumor presented as a lobular mass measuring 2.2 cm in its greatest dimension and arose in the flexor digitorum tendon sheath of the right 2nd toe. Clinically, the mass was palpable 1 year ago and brought pain during walking. Microscopically, the mass was composed of focal conventional TGCT and cartilaginous components. The conventional TGCT areas consisted of mononuclear cells, multinucleated giant cells, and hemosiderin deposition. The chondroid areas were extensive and comprised more than 90% of the whole tumor. In this case, the mononuclear cells in the conventional TGCT areas showed focal immunohistochemical staining for podoplanin and S100 protein as well as diffuse staining for CD68, which is consistent with the staining pattern of conventional TGCT. The mononuclear cells in the chondroid areas were focal positive for podoplanin and diffuse positive for S100 protein. Chondroid metaplasia in diffuse TGCT has been reported in 10 cases involving the temporomandibular, elbow, and hip joints. However, there has been no report of a localized form of chondroid TGCT involving an extra-articular region.

A Case of Aloe-nduced Acute Interstitial Nephritis / 대한신장학회잡지

We report a case of aloe induced acute interstitial nephritis in a 66-year old man. He had been taken aloe as a healthy foodstuff for 6 weeks prior to admission. He complained poor oral intake and developed nonoliguric acute renal failure. Renal biopsy revealed focal tubular atrophy and interstitial infiltration of neutrophils, and lymphocytes. After discontinuation of aloe and high dose prednisolone therapy, acute renal failure of the patient improved and serum creatinine level decreased.

Neutrophilic Myositis without Cutaneous Involvement as the First Manifestation of Acute Myeloid Leukemia

Muscle involvement in acute febrile neutrophilic dermatosis is uncommon. Herein, we report a case of acute febrile neutrophilic myositis, without cutaneous involvement, as the first manifestation of acute myeloid leukemia. The patient was a 35-year-old male, referred due to painful swelling of the left upper arm and fever. The overlying skin looked normal, and a muscle biopsy revealed dense infiltrates, predominantly composed of mature neutrophils, edema and tissue necrosis. All culture reports were negative, and he was finally diagnosed as having acute febrile neutrophilic myositis, associated with acute myeloid leukemia. Corticosteroid treatment resulted in the progressive regression of the fever, myalgia and swelling.

A Case of Adenocarcinoma of the Larynx / 대한이비인후과학회지

The larynx is a rare site for an adenocarcinoma. This case presents adenocarcinoma of epiglottis, a rare malignant neoplasm of the larynx. A 74-year-old man was presented with throat discomfort that began 4 months ago and neck mass for 1 month ago. The laryngeal telescopic finding revealed centrally ulcerative fungating mass on the laryngeal surface of epiglottis. A biopsy of the neoplasm pathologically characterized the lesion as adenocarcinoma. An adenocarcinoma of the larynx is a highly lethal neoplasm and has worse prognosis than squamous cell carcinoma of the larynx. We treated with the conservative surgery and bilateral neck dissection. There is no evidence of recurrence at 18 months after the initial treatment.

Inflammatory Pseudotumor of the Paratesticular Area: A Case Report

Inflammatory pseudotumors of the paratesticular area are rare, and are often reported in the literature by various terms, e.g., proliferative funiculitis, inflammatory myofibroblastic tumor, pseudosarcomatous myofibroblastic proliferation and fibrous pseudotumor. This is one of the most common lesions of that region, and typically presents as a longstanding, painless scrotal mass. Here, we describe a 34 year-old man who has had a palpable scrotal mass for the past 10 years. The excised mass was composed of multiple conglomerated nodules, which had homogeneous rubbery cut surfaces. Histologically, each was a well circumscribed, but unencapsulated mass of hyalinized collagenous tissue interspersed with lymphoplasmacytic cells and lymphoid follicle formation. A small fraction of paucicellular spindle cells was positive for vimentin, smooth muscle actin and CD68. Ultrastructurally, abundant collagen fibrils were mixed with paucicellular spindle cells and inflammatory cells. These spindle cells had abundant rough endoplasmic reticula and myofilaments with focal densities, indicating myofibroblastic differentiation.

Cerebral Amyloid Angiopathy: A Case Report

Symptomatic cerebral amyloid angiopathy (CAA) is characterized by lobar cerebral hemorrhage, transient neurological symptoms, and dementia with leukoencephalopathy in the absence of hypertension or coagulopathy. Contrary to its common appearance in English literature, pathologically confirmed CAA has rarely been reported in Korea. A 77-year-old normotensive, nondemented woman was admitted for a case of incidentally found intracerebral lobar hemorrhage at the left occipito-parietal area. On neurological examination, she displayed symptoms of a drowsy mentality and Gerstmann syndrome. Light-microscopically, medium to large cortical and meningeal vessels appeared to be thickened with amorphous glassy materials showing apple-green birefringence under polarized light on Congo red stain, which is consistent with CAA. The amyloid deposits were detected in the medium-sized vessels of the leptomeninges and cerebral cortex, but not in the capillaries and small arterioles in the white matter. Electron microscopy revealed amyloid fibrils and basal lamina duplication, indicating damaged vessels. Careful cortical biopsy during evacuating hematoma for histopathologic and ultrastructural examination is essential for diagnosis of CAA in case of clinical probability because the ignored sampling procedure and sampling error of patchy, segmental distribution of affected vessels might be ascribed to rare incidence of CAA in Korea.

Down Syndrome Associated with Testicular Seminoma: A Case Report

Individuals with Down syndrome have a susceptibility to neoplastic transformation, increased risk of chronic leukemia and central nervous system tumors. Recently, an increased number of cases of testicular germ cell tumors have been reported in individuals with Down syndrome, with more than forty cases in the literature. Here we report the first Korean case of seminoma with Down syndrome, in a 19-year-old institutionalized man who presented with painful scrotal swelling. Percutaneous needle biopsy showed histology of seminoma with invasion to the adjacent epididymis. Both testes were orthotopic. He underwent orchiectomy of the affected side, and the surgical staging was stage I seminoma with complete resection. We emphasize that the physician and nursing staff should be aware of the increased incidence of testicular seminoma in Down syndrome, because testicular seminoma might be misinterpreted as a scrotal infection with subsequent needle biopsy, which is contraindicated to avoid possible lymphatic metastasis.

Plexogenic Pulmonary Arteriopathy in Congenital Heart Disease: A Report of Two Cases

Hypertensive pulmonary vascular disease can develop in those cases of congenital cardiac shunt in which critical levels of pulmonary artery pressure and flow are reached and exceeded. We have experienced two cases of plexogenic arteriopathy in complex congenital heart disease and tried to evaluate of distribution of arterial lesions by total mapping of the explanted lung. Case 1 and 2 were 12-year-old boy and 36 year-old man. They were treated with combined heart-lung transplantation. Mapping of the both lungs was done, and graded according to Heath and Edward's grading scheme. The elastic pulmonary artery was tortuous, dilated and aortic configuration. Both lungs showed mostly grade 3. Plexiform lesion or veinlike branches of hypertrophied muscular arteries arosed in a lateral branch of a muscular artery that might be proximal to an area of occlusion. Comprising the right and left lung, the right was more severe than the left. By getting closer to the distal part, the grade tended to increase to 4 to 5. By analyzing the pulmonary lobe, severe pulmonary hypertension of grade 4 or 5 was comparatively disseminated throughout the right lung. On the other hand, in the left lung, the grade of the lower lobe was higher than that of the upper lobe, and within the upper lobe, there was a tendency for the grade of inferior segment to be higher than that of the corresponding apical segment.

Bilateral Renal Parenchymal Malacoplakia Presenting as Fever and Acute Renal Failure / 대한신장학회잡지

Malacoplakia is a rare, chronic inflammatory disorder characterized by abnormal macrophage function and cell infiltration into normal tissues. We report a 72 year old male with bilateral renal parenchymal malacoplakia who presented with high fever and acute renal failure. The patient had anemia, leukocytosis, thrombocytopenia and bilateral nephromegaly with reduced renal function. Blood and urine cultures showed no micro-organisms. A kidney biopsy revealed diffuse interstitial histiocytic infiltration with intracellular inclusion bodies(Michaelis-Gutmann body). He was treated with antibiotics, and bethanechol, ascorbic acid without improvement. Following treatment with an intravenous methylprednisolone pulse therapy, clinical improvement was made. Renal parenchymal malacoplakia should be considered in the differential diagnosis when presented with high fever, enlarged kidneys and acute renal failure.

Bilateral Renal Parenchymal Malacoplakia Presenting as Fever and Acute Renal Failure / 대한신장학회잡지

Malacoplakia is a rare, chronic inflammatory disorder characterized by abnormal macrophage function and cell infiltration into normal tissues. We report a 72 year old male with bilateral renal parenchymal malacoplakia who presented with high fever and acute renal failure. The patient had anemia, leukocytosis, thrombocytopenia and bilateral nephromegaly with reduced renal function. Blood and urine cultures showed no micro-organisms. A kidney biopsy revealed diffuse interstitial histiocytic infiltration with intracellular inclusion bodies(Michaelis-Gutmann body). He was treated with antibiotics, and bethanechol, ascorbic acid without improvement. Following treatment with an intravenous methylprednisolone pulse therapy, clinical improvement was made. Renal parenchymal malacoplakia should be considered in the differential diagnosis when presented with high fever, enlarged kidneys and acute renal failure.

Effect of Enalapril or Lovastatin on Tubulointerstitial Injury Induced by Protein-overload Proteinuria in Rats / 대한신장학회잡지

BACKGROUND: Previous studies have demonstrated that enalapril or lovastatin seems to ameliorate the renal injury in several animal models with glomerulonephritis. The aim of this study was to examine whether enalapril or lovastatin was still beneficial in tubulointerstitial injury induced by protein-overload proteinuria in rats. METHODS: Enalapril(200mg/L in the drinking water) or lovastatin(16mg/kg, subcutaneously) was administered to uninephrectomized rats which received a daily intraperitoneal injections of bovine serum albumin(BSA, 1/100g body weight)(each n=6). Six rats were served as normal control. After 2 weeks, renal cortical pathologic findings, including immunohistochemistry for macrophage were examined and renal cortical osteopontin, MCP-1, endothelin-1, TGF-beta and procollagen alpha1(I) mRNA expression were examined by Northern blot analysis. RESULTS: Renal cortex in rats with protein-overload proteinuria showed infiltration of inflammatory cells including macrophages, tubular dilatation and atrophy. Renal cortical mRNA expression of osteopontin, MCP-1 and endothelin-1 were increased in rats with protein-overload proteinuria. There were no changes in TGF-beta and procollagen alpha1(I) mRNA expression. Enalapril decreased the macrophage infiltration significantly and inhibited the mRNA expression of osteopontin, MCP-1 and endothelin-1. However, lovastatin had no significant effects on the macrophage infiltration and cortical mRNA expression. CONCLUSION: Enalapril showed beneficial effects in tubulointerstitial injury induced by protein-overload proteiuria in rats by inhibition of macrophage infiltration and the cortical mRNA expression of osteopontin, MCP-1 and endothelin-1. However, lovastatin had no significant effects.

Effect of Enalapril or Lovastatin on Tubulointerstitial Injury Induced by Protein-overload Proteinuria in Rats / 대한신장학회잡지

BACKGROUND: Previous studies have demonstrated that enalapril or lovastatin seems to ameliorate the renal injury in several animal models with glomerulonephritis. The aim of this study was to examine whether enalapril or lovastatin was still beneficial in tubulointerstitial injury induced by protein-overload proteinuria in rats. METHODS: Enalapril(200mg/L in the drinking water) or lovastatin(16mg/kg, subcutaneously) was administered to uninephrectomized rats which received a daily intraperitoneal injections of bovine serum albumin(BSA, 1/100g body weight)(each n=6). Six rats were served as normal control. After 2 weeks, renal cortical pathologic findings, including immunohistochemistry for macrophage were examined and renal cortical osteopontin, MCP-1, endothelin-1, TGF-beta and procollagen alpha1(I) mRNA expression were examined by Northern blot analysis. RESULTS: Renal cortex in rats with protein-overload proteinuria showed infiltration of inflammatory cells including macrophages, tubular dilatation and atrophy. Renal cortical mRNA expression of osteopontin, MCP-1 and endothelin-1 were increased in rats with protein-overload proteinuria. There were no changes in TGF-beta and procollagen alpha1(I) mRNA expression. Enalapril decreased the macrophage infiltration significantly and inhibited the mRNA expression of osteopontin, MCP-1 and endothelin-1. However, lovastatin had no significant effects on the macrophage infiltration and cortical mRNA expression. CONCLUSION: Enalapril showed beneficial effects in tubulointerstitial injury induced by protein-overload proteiuria in rats by inhibition of macrophage infiltration and the cortical mRNA expression of osteopontin, MCP-1 and endothelin-1. However, lovastatin had no significant effects.

Ectopic Pituitary Adenoma within the Sphenoid Sinus

A 69-year-old woman presented with right abducent nerve paresis caused by an ectopic pituitary adenoma invading the posterior wall of the sphenoid sinus. The tumor was removed via transsphenoidal approach. The histological diagnosis was invasive pituitary adenoma with bony destruction. The symptom was improved without complication. The authors present a rare case of ectopic pituitary adenoma with a literature review.

A Case of Primary Biliary Cirrhosis in Association with Sjorgen's Syndrome Developing Features of Systemic Lupus Erythematosus / 대한류마티스학회지

Primary biliary cirrhosis (PBC)is a chronic inflammatory disease characterized by fibrous obliteration of intrahepatic bile ductules.Although the etiopathogenesis of PBC remains unclear,several studies suggest that a disordered immune response may be involved.This disease is commonly associated with various autoimmune diseases,such as Sjogren's syndrome,scleroderma, rheumatoid arthritis,Hashimoto's thyroiditis,polymyositis,and pernicious anemia.More than 80%of cases with PBC are accompanied by at least one autoimmune disorder,and 40%by two or more. Sjogren's syndrome,defined by dry eyes and xerostomia,may be found in 69 to 81%of PBC patients. However, concurrent coexistence of PBC and systemic lupus erythematosus (SLE)has been rarely described in the literature.We report a 46-year-old female with PBC and Sjogren's syndrome who also satisfied a classification criteria of SLE.

The Prognostic Value of DNA Flow Cytometry in Patients with Early Primary Oral Cavity and Oropharyngeal Squamous Cell Carcinoma / 대한이비인후과학회지

BACKGROUNDS AND OBJECTIVE: It has been known that tumor size, regional neck metastasis state and tumor thickness are the prognostic factors of oral cavity or oropharyngeal cancer. Additionally, DNA flow cytometry has also been reported to be one of the pronosic factors. We would like to evaluate the prognostic value of DNA flow cytometry in early oral cavity or oropharyngeal squamous cell carcinoma. MATERIALS AND METHODS: We analyzed the relation between tumor thickness or neck metastasis and survival rate in 59 patients with early oral cavity or oropharyngeal cancer. Among those patients, DNA flow cytometry was performed in 28 cases and the prognostic value of the parameters of DNA flow cytometry was analyzed. RESULTS: Overall, a 2-year survival rate of the patients was 90.1%. Neither tumor thickness nor neck metastasis state was related to the overall survival rate. Tumor thickness was not related to neck metastasis state, either. Of the parameters of the DNA flow cytometry, only the S phase fraction of aneuploidy was related to the overall survival rate (p=0.0288). Also the total proportion of aneuploidy was weakly related to the state of neck metastasis (p=0.0518). CONCLUSION: These results indicate that DNA flow cytometry can be used as a good complementary factor for predicting the overall survival or neck metastasis in patients with early primary oral cavity or oropharyngeal squamous cell carcinoma.

A Case of Renal Sarcoidosis Presented with Hypercalcemia and Acute Renal Failure / 대한신장학회잡지

We report a case of renal sarcoidosis accompanied by hypercalcemia and renal insufficiency. A 52-year-old woman presented to hospital with nausea, pruritus, general weakness and weight loss. Laboratory tests revealed a serum creatinine was 3.6mg/dL, calcium ll.lmg/dL(ionized calcium 5.6mg/dL), hemoglobin 10.2g/dL, parathyroid hormone <3.0pg/mL, 24 hour urinary protein 1,127mg/day and 24 hour urinary calcium 489mg/day. Chest X-ray showed normal and both kidneys were within normal size with mild increased echo-genicity on renal ultrasound. Renal biopsy showed interstitial nephritis with mononuclear cell infiltration and mild interstitial fibrosis. Non-caseating granuloma with epitheloid and giant cells were seen. Following corticosteroid therapy, renal function and hypercalcemia were dramatically improved. Within 1 month, calcium level was normal and the creatinine level fell to 2.0mg/dL and subsequently remained stable while corticosteroid was progressively tapered off. She has been followed up for 11 months with normal seum calcium level and the creatinine level of 1.9mg/dL.

Two Cases of Thin Basement Membrane Nephropathy presented with Minimal Change Nephrotic Syndrome

Thin basement membrane nephropathy(TBMN) is defined histologically as follows: 1) By light rnicroscopy only minor abnormalities are detected in the glomeruli at most minor mesangial widening. 2) By electron microscopy, diffuse thinning of glomerular basement rnembrane is demonstrated. 3) By immunofluorescence, absence of immunoglobulins and complement components is demonstrated. 4) Alport's syndrome and systemic diseases that may affect the glomerular structure have been excluded. TBMN presented frequently with recurrent or persistent microscopic hematuria. Massive proteinuria such as in nephrotic syndrome rarely occurs in TBMN. We reported two cases of TBMN presented with typical minimal change nephrotic syndrome.

A Case of Idiopathic Fibrillary Glomerulonephritis / 대한신장학회잡지

A 17-year-old girl was admitted to our hospital due to mild generalized edema. Laboratory tests revealed a serum creatinine was 0.7mg/dL, protein/albumin 6.7/3.5g/dL, cholesterol 190mg/dL, hemoglobin 10.0g/dL, and 24 hour urinary protein 4,40mg/day. Chest X-ray and renal ultrasound were normal. There were no clinical or serologic evidences of paraproteinemia, cryoglobulinemia, light chain disease or systemic lupus erythematosus. Renal biopsy showed membranoproliferative glomerulonephritis-like pattern with lobular accentuation, hypercellularity and diffuse GBM thickening by light microscope. Congo red staining was negative. Granular IgG and C3 deposits were found along the glomerular capillary wall and mesangium by immunofluorescence microscope. Ultrastructurally, abundant subendothelial and mesangial fibrillary deposits were found associated with thickening and wrinkling of GBM. These fibrils, measured about 20-30nm in diameter, were nonbranching and randomly arranged without either periodicity or an organized structure. These findings were compatible with those of fibrillary glomerulonephritis. Thus we report a case of idiopathic fibrillary glomerulonephritis, which is a rare cause of nephrotic syndrome.

Clinical Significance and Prognostic Factors of IgA Nephropathy Developed in Renal Allograft / 대한신장학회잡지

Recently IgA nephropathy(IgAN) in transplanted kidney have been reported a more grave prognosis of the disease, which account for progressive allograft loss as 12-16%. But, the studies for the clinical significance and the prognostic factors of IgAN developed in transplanted kidney were not sufficient. We analyzed the frequency and the prognostic factors of IgAN developed in patients who underwent Kidney transplantation(KT) between January 1990 and October 1998. Total 231 renal biopsies was done. IgAN was diagnosed in 31 cases, and it was 13.4% of total cases and 24.4% of cases that acute rejection(AR) were excluded. After the 3 patients of IgAN was excluded due to combined AR, 28 patients were followed. The allograft dysfunction, which defined as serum creatinine at last follow-up was above 2.0 mg/dL, was observed in 10 patients(35.7%) and 4 patients(14.3%) of them had graft failure and restarted hemodialysis. The serum crcatinine and creatinine clearance at renal biopsy were significantly correlated with allograft dysfunction, also interstitial fibrosis, tubular atrophy and vascular fibrous intima1 thickening showed similar correlation with prognosis. In conclusion, when allograft dysfunction was developed, IgAN in transplanted kidney should be considered and if the evidences of elevated serurn creatinine and decresed creatinine clearence at biopsy, severe interstitial fibrosis and tubular atrophy, severe vascular fibrous intimal thickening were present, more closer follow-up and careful treatment should be taken.

Flow cytometric immunophenotyping in fine-needle aspiration of lymph nodes

Fine-needle aspiration (FNA) of lymph nodes has been regarded as a useful method in the diagnosis of lymphadenopathy. However, this procedure has been shown to be of limited value in the diagnosis of low or intermediate grade malignant lymphomas in some studies. Immunophenotyping is an essential adjunct to cytomorphology for the diagnosis of lymphoma by FNA. Immunophenotyping using flow cytometry (FCM) is rapid, objective and reliable. Using FCM, multiparametric analysis of 33 FNA materials from lymph nodes was performed and profiles of surface markers of lymphoid cells were assessed. In reactive hyperplasia, patterns of cell surface markers were quite variable, but disclosed polyclonality. Most of the B-cell lymphomas showed immunophenotypes for B-cell lineages with their kappa: lambda or lambda: kappa ratio being over 3:1. In T-cell lymphomas, T-cell surface markers were predominantly expressed as well. In conclusion, our results suggest that immunophenotyping of lymph node aspirates is a valuable diagnostic adjunct for lymphoproliferative disorders, particularly in B-cell lymphomas because immunophenotyping can be easily and adequately performed by FCM.