Coxiella burnetii(C. burneii)was first recognized as the agent of Q fever in 1937. Q fever is an acute self-limited febrile illness. However, it manifests with several clinical symptoms depending upon the organs that are involved. The association of C. burnetii with human neoplasia has been rarely reported. We prospectively studied the 55 patients with fever of unknown origin, pneumonia, hepatosplenomegaly, lymphadenopathy, leukemia, lymphoma, and immunodeficiency and 14 persons who contacted the Q fever patients. The patient's sera were tested for antibodies specific for C. burnetii, using indirct fluorescent antibody techniques (IFA). 1) We serologically confirmed 23 C. burnetii infection. The 23 children with Q fever ranged in age from 0 to 15 years, with mean age of 4 years 11 months. Seventeen were boys and 6 were girls. 2) Characteristic symptoms and signs were fever (9/12 cases), rash (8/14 cases), hepatosplenomegaly (8/8 cases)and lymphadenopathy (14/27 cases). Five cases among 14 asymptomatic cases who contacted Q fever patients showed positive IFA test. One suffered from irregular uterine contraction, 4 weeks after contact with a Q fever patient. 3) There were no history of exposure to domestic animal carriers or contaminated dust, or drinking raw milk except one family. Three attending doctors and her father infected by a patient with Q fever. These suggested the person to person transmission of Q fever in a family and house staffs infected by a patient of Q fever. 4) Q fever (9 cases), acute lymphoblastic leukemia (2 cases), acute myelomonocytic leukemia (1 case), hairy cell leukemia (1case), Kawasaki disease (4 cases) and congenital dyserythropoietic anemia (1 case) showed positive IFA test. 5) Of 9 cases who suffered from lnly Q fever, 7 cases were confirmed hairy cell formation in their peripheral blood. One case was diagnosed as hairy cell leukemia after bone marrow study. Of 7 cases who showed hairy cells, all had hepatomegaly, 6 cases had lymphedenopathy and 5 cases showed splenomegaly. All except 1 case who was not followed cured after treatment. 6) We treated Q fever patients with rifampin and/or ciprofloxacin, and/or tetracyclin (over 8 year-old of age)for 2-4 weeks. One 25 month-old patient with hairy cell leukemia was treated with rifampin, ciprofloxacin and tetracyclin for 4 weeks, and rifampin for 8 months. A pregnant patient was administered with rifampin, and treated with rifampin and ciprofloxacin after delivery. We gave rifampin in one nweborn baby. In conclusion, we suggest that Q fever should be considered in the differential diagnosis of patients with FUO, hepatosplenomegaly and/or immunodeficiency.
Anemia, Dyserythropoietic, Congenital ; Animals, Domestic ; Antibodies ; Bone Marrow ; Child ; Child, Preschool ; Ciprofloxacin ; Coxiella burnetii* ; Coxiella* ; Diagnosis, Differential ; Drinking ; Dust ; Exanthema ; Fathers ; Female ; Fever ; Fever of Unknown Origin ; Fluorescent Antibody Technique ; Hepatomegaly ; Humans ; Internship and Residency ; Leukemia ; Leukemia, Hairy Cell ; Leukemia, Myelomonocytic, Acute ; Lymphatic Diseases ; Lymphoma ; Milk ; Mucocutaneous Lymph Node Syndrome ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prospective Studies ; Q Fever* ; Rifampin ; Splenomegaly ; Uterine Contraction

No abstract available.
Apgar Score ; Humans* ; Umbilical Cord*

The spindle and epithelioid cell nevus, is known also as Spitz's nevus or benign juvenile melanoma. The histologic picture of the lesion often closely resembles that of a malignant melanoma, but spindle and epithelioid cell nevus hehaves benignly. Criteria for accurate histological diagnosis of this nevus now exist. However, a difficult lesion occasionally brings one to the worrisome situation even now. We experienced four cases (three children and one adult) of spindle and epithelioid cell neveus with nodular or macular skin lesions. In children (7/F, 9/F, 4/M), all of the lesion show a solitary pinkish red or light brown nodular firm masses, 0.7 cm to 1.0 cm in greatest dimension, which are located at the left posterior thigh, posterior neck and right anterior neck region, respectively. In adult (36/F), the lesion shows a light brown, erythematous macule, 0.8 cm in greatest diameter, in the skin of the back. Microscopically, both spindle and epithelioid cells are found in all the four cases, but epithelioid cells predominate in children and spindle cells predominate in adult.
Child ; Adult ; Male ; Female ; Humans

Paneth cell-rich carcinoma is essentially an adenocarcinoma with a predominance of Paneth cells. A 60-year-old male patient was admitted with a history of abdominal distension for several months. Endoscopic examination revealed a large ulceroinfiltrative tumor involving most of the areas of the stomach. The biopsy of the lesion confirmed poorly differentiated adenocarcinoma and total gastrectomy was followed. The submitted total stomach contained a diffuse infiltrative Borrmann type IV mass with ulceration, 8.0 3.5 cm, at the body along the lesser curvature. Microscopically, it was composed of Paneth cell differentiated cancer cells and poorly differentiated tubular adenocarcinoma cells. The Paneth cell differentiation was characterized by cytoplasmic coarse eosinophilic granules, which were PAS-positive and positive reaction for lysozyme. Electron microscopic examination showed numerous, spherical, electron-dense, homogeneous granules corresponding to those in Paneth cells as well as mucin granules in the signet-ring cells, and various intermediate forms in some cancer cells, which might be immature in the Paneth cell lineage.
Adenocarcinoma ; Biopsy ; Cell Differentiation ; Cell Lineage ; Cytoplasm ; Eosinophils ; Gastrectomy ; Humans ; Male ; Middle Aged ; Mucins ; Muramidase ; Paneth Cells ; Stomach* ; Ulcer

Nucleolar organizer regions (NORs) are loops of DNA which occur in the nucleoli of cells which possess ribosomal RNA genes. The numbers and areas of NORs have been thought to be related to cellular activities. We aimed to investigate the direct relationship between the parameters of AgNORs and cellular proliferative activity using immunohistochemical method with the monoclonal antibody Ki-67, which demonstrates proliferating nuclei. The sequential technique for the simultaneous Ki-67 immunostaining and NOR staining was applied to the same slides of a series of non-Hodgkin's lymphomas (NHL) of the low, intermediate, and high grade type. The number of AgNOR per cell was counted and mean NOR percentage nuclear area (NPNA) was measured by morphometry in both the Ki-67 positive and Ki-67 negative nuclei. The increased immunoreactivity for Ki-67 was found in the high grade than in the low grade non-Hodgkin's lymphoma. This was reflected in the two areas of the palatine tonsils and lymph nodes, the positive cell counts being higher in the follicle center nuclei than in those in the interfollicular compartment. In general the numbers and NPNA of AgNORs were higher in the Ki-67 positive nuclei than in those lacking the antigen in malignant lymphomas as well as in control. The AgNORs numbers and NPNA in controls were the highest in the Ki-67 positive cells in the follicular area and the lowest in the Ki-67 negative cells in the perifollicular area. In malignant lymphomas the numbers and NPNA of AgNORs tended to increase in proportion to their grade in both the Ki-67 positive and negative cells. The numbers of AgNORs of the high grade and the NPNA of the intermediate and the high grade were significantly higher in Ki-67 positive cells than in Ki-67 negative ones. With this double staining method it was now possible to confirm that numbers and NPNA of AgNORs were directly related to the cellular proliferative activity. In maligant lymphoma, among the several parameters of the AgNORs, NPNA in Ki-67 positive cells is proposed to be the most useful marker in determining the prognosis of the patient.
Cell Count ; DNA ; Genes, rRNA ; Humans ; Lymph Nodes ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Nucleolus Organizer Region* ; Palatine Tonsil ; Prognosis

No abstract available.

Sertoli-Leydig cell tumors of the ovary are rare, accounting for no more than 0.5% of all ovarian neoplasms. These tumors occur predominantly in women 10 to 30 years of age. Heterologous elements are found in over 20% of the moderately and poorly differentiated Sertoli- Leydig cell tumors. The most common element is gastrointestinal mucin-secreting epithelium, found in approximately 20% of all cases. Approximately 4% of Sertoli-Leydig cell tumors contain immature skeletal muscle and/or cartilaginous elements. The origin of these neoplasms is regarded as neometaplasia of the mesodermal neoplastic element. While the degree of differentiation of Sertoli-Leydig cell tumors is probably of greater importance in determining prognosis than the presence of heterologous components, recent evidence does point to an unfavorable effect on clinical outcome when heterologous mesenchymal components such as cartilage and striated muscle are present. We experienced two cases of ovarian heterologous Sertoli-Leydig cell tumors with gastrointestinal type epithelium and striated skeletal muscle in 20 and 21 year-old females, respectively. One case involved a patient who had complained of amenorrhea for 3 years while the other case involved a patient who had complained of left lower abdominal tenderness and amenorrhea for one and one half years. It was determined by ultrasonography of the pelvis that the two patients suffered from right and left ovarian masses. Both patients underwent adnexectomy. These tumors were large with partly solid and partly cystic areas. Based on light microscopic examination one case was classified as an intermediate Sertoli-Leydig cell tumor with heterologous gastrointestinal type epithelium, while the other case was classified as a poorly differentiated Sertoli-Leydig cell tumor with heterologous striated skeletal muscle.
Female ; Humans

Expression of the nm23 and E-cadherin proteins has been studied in a number of tumors. Reduced expression of the nm23 and E-cadherin proteins seems to be associated with metastasis or disease progression in some tumors, including breast carcinoma. To assess the role of nm23 and E-cadherin in tumor differentiation and metastasis of breast carcinoma, immunohistochemical staining for the nm23 and E-cadherin proteins was performed in paraffin embedded tumor samples from 86 breast carcinomas. The results were as follows: 1) Expression of the nm23 protein in breast carcinoma was strong positive in 32 cases (37.2%), weak positive in 26 cases (30.2%), and negative in 28 cases (32.6%) of the cases. Expression of the nm23 protein in breast carcinoma decreased according to histological grade and lymph node metastasis, but was not statistically significant. 2) Expression of the E-cadherin protein in breast carcinoma was strong positive in 50 cases (58.1%), weak positive in 24 cases (27.9%), and negative in 12 cases (14%) of the cases. Expression of the E-cadherin protein in breast carcinoma decreased according to histological grade and lymph node metastasis, but was not statistically significant. 3) There was a statistically significant correlation between the expression of the nm23 protein and the E-cadherin protein in breast carcinoma (p<0.05). These results suggest that the expression of the nm23 and E-cadherin proteins is related to tumor differentiation, and may also be an useful prognostic factor in breast carcinoma.
Breast Neoplasms* ; Breast* ; Cadherins* ; Disease Progression ; Lymph Nodes ; Neoplasm Metastasis ; Paraffin

We present a case of mucous gland adenoma arising from the main bronchus of the lower lobe of the left lung in terms of clinicopathologic, immunohistochemical, and ultrastructural aspects as well as review of related literatures. The patient, a 31-year-old female, was admitted to Catholic University Medical College Hospital with complaints of coughing and purulent sputum for about seven years. The chest CT showed a severely calcified tumor in the left lower lobe of the lung. Grossly, the calcified tumor arising from the main bronchus protruded into the lumen and showed bronchiectasis of the lower lobe and atelectasis of the upper lobe of the lung. The tumor was pale brown-gray and sharply circumscribed and showed some small cystic spaces filled with mucoid material. Microscopically, most of the tumor showed dystrophic calcification. The growth pattern of the tumor is composed of cysts, tubules, and glands lined by cytologically bland columnar, cuboidal, or flattened mucus secreting cells. Electron micrograph of tumor cells showed some round or oval mucous granules measuring 0.5-1.8 micrometer.
Adenoma* ; Adult ; Bronchi* ; Bronchiectasis ; Cough ; Female ; Humans ; Lung ; Mucus ; Pulmonary Atelectasis ; Sputum ; Tomography, X-Ray Computed

Intracranial teratomas which were first described by Maier in 1861 are uncommon. Those presenting at birth are very rare and in our knowledge no case has been reported in Korea. In November, 1988, we experience a case of congenital intracrainal teratoma which replaced almost all cerebral tissue, filled out the oral cavity, and was protruded from the mouth. A female fetus was artificially delivered by a 25-year-old primigravida at 22 weeks of gestation, because of marked hydramnios and fetal hydrocephalus which were detected by prenatal ultrasonography. Microscopically, tissues from all three germ layers, including a lot of neuroepithelim and primitive mesenchymal tissue, were shown. A brief review of the literature was done.
Female ; Humans