Autoimmune Encephalitis Associated with Thymoma

No abstract available.

Acute Atherosis of the Uterine Spiral Arteries: Clinicopathologic Implications

Acute atherosis is unique vascular changes of the placenta associated with poor placentation. It is characterized by subendothelial lipid-filled foam cells, fibrinoid necrosis of the arterial wall, perivascular lymphocytic infiltration, and it is histologically similar to early-stage atherosclerosis. Acute atherosis is rare in normal pregnancies, but is frequently observed in non- transformed spiral arteries in abnormal pregnancies, such as preeclampsia, small for gestational age (SGA), fetal death, spontaneous preterm labor and preterm premature rupture of membranes. In preeclampsia, spiral arteries fail to develop physiologic transformation and retain thick walls and a narrow lumen. Failure of physiologic transformation of spiral arteries is believed to be the main cause of uteroplacental ischemia, which can lead to the production of anti-angiogenic factors and induce endothelial dysfunction and eventually predispose the pregnancy to preeclampsia. Acute atherosis is more frequently observed in the spiral arteries of the decidua of the placenta (parietalis or basalis) than in the decidual or myometrial segments of the placental bed. The presence and deeper location of acute atherosis is associated with poorer pregnancy outcomes, more severe disease, earlier onset of preeclampsia, and a greater frequency of SGA neonates in patients with preeclampsia. Moreover, the idea that the presence of acute atherosis in the placenta may increase the risk of future cardiovascular disease in women with a history of preeclampsia is of growing concern. Therefore, placental examination is crucial for retrospective investigation of pregnancy complications and outcomes, and accurate placental pathology based on universal diagnostic criteria in patients with abnormal pregnancies is essential for clinicopathologic correlation.

Chronic Placental Inflammation in Twin Pregnancies

BACKGROUND: Chronic placental inflammation, such as villitis of unknown etiology (VUE) and chronic chorioamnionitis (CCA), is considered a placental manifestation of maternal anti-fetal rejection. The aim of this study is to investigate its frequency in twin pregnancies compared to singleton pregnancies. METHODS: Three hundred twin placentas and 1,270 singleton placentas were consecutively collected at a tertiary medical center in Seoul, Republic of Korea from 2009 to 2012. Hematoxylin and eosin sections of tissue samples (full-thickness placental disc and chorioamniotic membranes) were reviewed. RESULTS: Non-basal VUE was more frequent in twin placentas than in singleton placentas (6.0% vs 3.2%, p < .05). In preterm birth, CCA was found less frequently in twin placentas than in singleton placentas (9.6% vs 14.8%, p < .05), reaching its peak at an earlier gestational age in twin placentas (29-32 weeks) than in singleton placentas (33-36 weeks). CCA was more frequent in twin pregnancies with babies of a different sex than with those with the same sex (13.8% vs 6.9%, p=.052). Separate dichorionic diamniotic twin placentas were affected by chronic deciduitis more frequently than singleton placentas (16.9% vs 9.7%, p<.05). CONCLUSIONS: The higher frequency of non-basal VUE in twin placentas and of CCA in twin placentas with different fetal sex supports the hypothesis that the underlying pathophysiological mechanism is maternal anti-fetal rejection related to increased fetal antigens in twin pregnancies. The peak of CCA at an earlier gestational age in twin placentas than in singleton placentas suggests that CCA is influenced by placental maturation.

Inguinal Lipoblastoma Mimicking Recurrent Inguinal Hernia / 소아외과

Palpable inguinal mass in children should be differentiated from inguinal hernia, hydrocele, lymph node, and tumor. Though using ultrasonography, fatty tumor would be misdiagnosed as incarcerated inguinal hernia containing fatty component. We experienced the huge inguinal lipoblastoma in 5-year-old girl mimicking recurrent incarcerated hernia. Laparoscopic exploration revealed it was not incarcerated hernia but well demarcated bulging mass from abdominal wall. Mass was about 10x4x3 cm and extended from internal inguinal ring to saphenous opening. It was near total excised because of right external iliac vein injury. Pathologically, it was proven as lipoblastoma containing mature adipocyte with lipoblast and fibrous septa. Postoperatively, we noticed a segmental thrombotic occlusion of external iliac vein. After 1 year, she has no symptom related to occluded vessel. The remained lipoblastoma showed no interval change. Even lipoblastoma has a good prognosis with low recurrence rate, we need careful follow-up.

Concurrence of Membranous Glomerulonephritis and Pulmonary Alveolar Proteinosis / 대한내과학회지

Membranous glomerulonephritis is one of the most common causes of nephrotic syndrome in adults. Pulmonary alveolar proteinosis (PAP) is a rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli. We describe a 61-year-old man with concurrent membranous glomerulonephritis and PAP, which is very rare; both are pathophysiologically related to an abnormal immune response. A patient came to hospital with leg edema but no respiratory symptoms. Chest X-ray and CT showed classical PAP findings, which are ground-glass opacities with interlobular septal thickening, in both lung fields. A bubbly whitish secretion retrieved via broncho-alveolar lavage showed neutrophils and lymphocytes as well as Periodic acid-Schiff-positive proteinaceous materials. A kidney biopsy revealed findings of membranous glomerulonephritis with irregular subepithelial deposits by electron microscopy. At 1 year after diagnosis, the membranous glomerulonephritis was well under control with steroids and mycophenolate mofetil but PAP became aggravated gradually and whole-lung lavage was needed.

Calcifying Aponeurotic Fibroma: Case Report with Radiographic and MR Features

Calcifying aponeurotic fibroma is a rare, benign fibroblastic tumor. The lesion has a propensity for local invasion and a high recurrent rate. Therefore, accurate preoperative diagnosis and complete excision are important to prevent the recurrence of the tumor after surgical removal. However, radiographic and magnetic resonance imaging findings of calcifying aponeurotic fibroma have been extremely rarely described in the radiology literature. Thus, we report a rare case of calcifying aponeurotic fibroma affecting the dorsal wrist in a 67-year-old man, describe radiographic and MR findings, and discuss the differential diagnosis of the tumor.

Primary malignant rhabdoid tumor of greater omentum in 10-year-old girl

Contrary to metastatic tumors of the omentum, primary tumors of the omentum are very rare. A 10-year-old girl presented with low abdominal pain. Imaging studies showed a multiseptated hemorrhagic tumor. The mass from the omentum was removed completely and confirmed as a malignant rhabdoid tumor. Despite aggressive chemotherapy, she died after 9 months due to disease progression. We report one case of primary malignant rhabdoid tumor of the omentum for the first time.

A Case of Organoid Nevus on the Knee / 대한피부과학회지

No abstract available.

An accessory limb with an imperforate anus

Congenital accessory limbs are very rare anomalies with many causative factors. We describe the case of a 1-day-old female neonate-born to a healthy, 27-year-old mother-who presented with an accessory limb (foot) attached to the buttock and an imperforate anus. We also provide a review of the relevant literature.

Multiple Papillary Fibroelastomas and Thrombus in the Left Heart

Cardiac papillary fibroelastomas (CPF) are benign cardiac tumors and usually discovered incidentally during echocardiography. This report describes the case of a 68-year-old man, referred to cardiology for multiple masses of the left ventricle and left atrium. The transthoracic echocardiography revealed multiple oscillating masses in the left ventricle and aortic valve, non-mobile mass in the left atrium with severe mitral stenosis and moderate aortic regurgitation. The patient underwent surgical resection of the masses with valve replacements. Histopathologic examination confirmed the diagnosis of CPF in the left ventricle and aortic valve, thrombus in the left atrium.

Uterine Adenomyosis Which Developed from Hypoplastic Uterus in Postmenopausal Woman with Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is characterized by vaginal agenesis with variable Mullerian duct abnormalities. We report here a case of uterine adenomyosis which developed from a hypoplastic uterus in a patient with MRKHS. A 55-year-old postmenopausal woman visited a university hospital for pelvic mass. She had underwent vaginoplasty via the McIndoe procedure for MRKHS at 15 years of age. Pelvic magnetic resonance imaging showed a 5.4 x 4.8 x 4.7 cm mass suspicious for a uterine myoma. She received total abdominal hysterectomy with bilateral salpingo-oophorectomy, and neither the cervix nor endometrium was found grossly in the surgical specimen. The final histologic diagnosis was uterine adenomyosis.

A Carcinoid Tumor Arising from a Normal Kidney in a Young Man / 대한내과학회지

Carcinoid tumors are low-grade malignant tumors arising from neuroendocrine cells. Primary renal carcinoid tumor is very rare due to the absence of neuroendocrine cells in the kidney and ureter. Therefore, little is known about the management and prognosis of renal carcinoid. Here, we report a case of a primary renal carcinoid tumor arising from a normal kidney in a 21-year-old man. He presented with a left renal mass, which was found accidentally. Abdominal computed tomography (CT) showed a 5.5 x 5.0-cm cystic mass with calcification. We suspected a cystic renal cell carcinoma and performed a laparoscopic radical nephrectomy. However, the histology revealed a well-differentiated neuroendocrine tumor. We concluded that it was a primary renal carcinoid tumor with no distant metastasis and did not administer chemotherapy or radiation therapy. He is recurrence-free after 8 months.

A Case of Unusual Pituitary Apoplexy Presented as Aseptic Meningitis / 대한임상신경생리학회지

We encountered a case of pituitary apoplexy who presented with isolated headache and vomiting without visual disturbance or ophthalmoplegia. The cerebrospinal fluid examination was compatible with aseptic meningitis. A computed tomography revealed slightly high density in the pituitary fossa and suprasella area, but the signal change was very faint. Our case suggests that clinicians should take into account the possibility of pituitary apoplexy without visual disturbance or ophthalmoplegia, when aseptic meningitis is suspected.

An Unusual Case of Left Ventricular Free Wall Rupture Caused by a Silent Myocardial Infarction

Left ventricular free wall rupture (LVFWR) is a serious complication of myocardial infarction. It presents with a very high mortality rate and can be rescued by accurate diagnosis and emergency surgery. LVFWR can occur with sudden overt clinical symptoms or present insidiously. This report highlights the case of a man with no prior history of coronary artery disease, who presented with LVFWR and pericardial effusion that evolved to severe bacterial pericarditis.

Cytologic Features and Distribution of Primary Sites of Malignant Cells in Cerebrospinal Fluid / 대한세포병리학회지

Cytologic evaluation of cerebrospinal fluid(CSF) is an effective mean for diagnosing many disorders involving the central nervous systems(CNS). One of the most important reasons for cytologic examination of CSF is to detect metastatic or primary neoplasms of the CNS. We did a retrospective study of 1,438 CSF specimens obtained between 1992 and 1996. A total of 1,205 adult and 233 pediatric CSF specimens from 947 patients were accessed at the Department of Pathology of Seoul National University Hospital and Children's Hospital, respectively. Among 1,438 CSF cytology specimens, 169 cases(11.8%, 77 patients) including 135 adult cases(59 patients) and 34 pediatric cases(18 patients) were positive for malignant cells. Diagnoses included 60 metastatic carcinomas(adult, 60; pediatric, 0); 46 malignant lymphomas(adult, 44; pediatric, 2); 21 leukemias(adult, 20; pediatric, 1); 4 retinoblastomas(adult, 0; pediatric 4); 2 rhabdomyosarcomas(adult, 0; pediatric, 2); 1 multiple myeloma(adult, 1; pediatric, 0), and 35 primary CNS neoplasms(adult, 10; pediatric, 25). The most commonly identified metastatic carcinomas in adults were adenocarcinoma. Their primary sites were the lung, gastrointestinal tract, and breast in order of frequency. The most common primary CNS neoplasm in children was medulloblastoma.

Sarcomatoid Renal Cell Carcinoma; Special Reference to its Distinction from Carcinosarcoma

Sarcomatoid renal cell carcinoma is an uncommon tumor that has to be distinguished from renal carcinosarcoma. We have described three cases of sarcomatoid renal cell carcinoma showing different clinical and light microscopic features. An ultrastructural study of the tumor cells from the sarcomatoid area revealed frequent desmosomal junction, confirming the epithelial nature of the neoplasm. All three cases showed an aggressive clinical course and tended to invade adjacent organs or tissues. We believe that an histological and immunohistochemical examination in conjunction with an electron microscopic examination are necessary to diagnose sarcomatoid renal cell carcinoma.

CT and MR Findings in Retinoblastoma: Correlation with Histopathologic Examination

PURPOSE: To evaluate histopathologic correlation of the component of the lesion on CT and MR of retinoblastomas. MATERIALS AND METHODS: Gross pathologic findings of 24 enucleated eyeballs in 24 patients with retinoblastomas were compared with preoperative CT (n=19) and MR (n=5) findings. In eight eyeballs, in which there were findings other than mass, histopathologic findings were reviewed and correlated with image findings. RESULTS: Retinal detachment and subretinal hemorrhage which were not detected on CT were demonstrated in two of eight eyeballs on histopathologic examination. In one eyeball, retinal detachment and subretinal effusion were detected on both CT and in a pathologic specimen. In two eyeballs with peripheral heterogeneous MR enhancement of the masses, tumor necrosis and calcification were demonstrated in the central non-enhancing portion of the mass. In two eyeballs, linear soft tissue along the retina apart from the main mass were revealed as tumor spread along the retinal surface. In one patient, retinal thickening on MR was presumed to be an MR artifact and no lesion was found in the gross specimen. CONCLUSION: On CT, retinal detachment and subretinal hemorrhage associated with retinoblastoma can mimic mass, and tumor spread along the retinal surface can be seen as a linear retinal lesion. On MR, tumor necrosis and calcification can be a cause of heterogeneous enhancement.

Congenital Cystic Disease of the Kidney overview and a classification

The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.

The Studies of bcl-2 Oncoprotein and Epstein-Barr Virus Expression in Malignant Lymphomas: Immunohistochemical and in situ hybridization analysis on 66 cases

Bcl-2 oncoprotein is being localized to mitochondria and interfering with programmed cell death (apoptosis) independent of promoting cell division in the lymphoid and nonlymphoid cells. The bcl-2 oncoprotein expression has been reported in follicular lymphomas as well as in diffuse non-Hodgkin's lymphoma, leukemia and a variable propotion of Hodgkin's lymphoma cases. Recent evidence suggests that some lymphomas protected from apoptosis is conferred through expression of Epstein-Barr virus(EBV) latent membrane protein which turn to cause upregulation of bcl-2. To define the role of the bcl-2 oncoprotein and EBV in lymphoid malignancy, we tried immunohistochemical studies with anti-bcl-2 antibody and In situ hybridization (ISH) with EBV-encoded small nuclear RNAs(EBER) in the paraffin embedded sections of 46 non-Hodgkin's lymphoma (NHL) cases and 20 Hodgkin's lymphoma (HL) cases. Bcl-2 oncoprotein expression was found in 37 of 46 cases (80%) of NHL with relatively strong cytoplasmic staining, and in 14 of 20 cases (70%) of HL with weak cytoplasmic staining in limited small numbers of RS, Hodgkin and lacunar cells. The widespread presence of bcl-2 oncogene in many different types of both NHL and HL supports that the extended cell survival through overexpression of bcl-2 gene protein may be a growth advantage of neoplastic lymphoid cells. In the ISH analysis for EBV, the presence of EBV was detected in 17 of 20 cases (85%) of HL, compared to 6 of 44 cases(13.6%) of NHL. It appears to be no direct correlation between overexpression of bcl-2 oncoprotein by neoplastic lymphoid cells and the presence of EBV in NHL but it seems to be a definite association between EBV and HL.

Histopathological Differences between Silicone Granuloma and Paraffinoma

During the past two decades, silicone (polydimethylsiloxane) has become one of the most extensively applied biomaterials. Although pure silicone is relatively inert and usually causes only minimal tissue reactions, it has been reported to evoke a definite foreign body reaction. We studied five cases of silicone-induced granulomas in various sites; two in the breast, one in the breast and axillary lymph nodes, one in the subcutis of the abdomen, back and extremities and one in the eyeball, to illustrate the salient histopathologic features of reactions to silicone with particular emphasis to its differences from paraffin granuloma. For this, 17 paraffinomas were also studied. Tissue reaction to silicone liquid and gel was characterized by numerous round to oval empty cystic vacuoles, mild to moderate fat necrosis, foreign body reaction, a variable degree of mononuclear inflammatory cell infiltration and mild focal fibrosis. The cystic spaces were relatively uniform and showed a snow-man like appearance. In contrast to the silicone granulomas, the paraffinomas, also refered to as sclerosing lipogranulomas showed diffuse sclerosis and frequent calcification around the cystic vacuoles. The cystic spaces in paraffinomas were swiss cheese-like configuration, and the content of the cystic spaces was dirty and frequently calcified. However, there were certain similarities between these two types of granulomas particularly in the early phases of the reaction, therefore, the history of silicone injection or implant, is sometimes critical to the diagnosis of silicone granuloma. Despite great technologic advances in the manufacturing of prostheses and medical equipment, droplets and/or particles of silicone still escape into the body tissues in a variety of ways; therefores, the pathologist should always wonder whether the histologic reaction observed is due to silicone or to some other foreign material including paraffin.