PSTT has been established as a separate entity distinct from choriocarcinoma since 1983, because of its different morphological features, biologic behavior and response to chemotherapy. Most cases of PSTT have indolent clinical courses, but there have been several reports showing malignant behavior resulted in death of the patient. We report a case of PSTT showing vaginal metastatis. In biopsy specimen, it was difficult to make differential diagnosis from squamous cell carcinoma of uterine cervix due to uniform and mononuclear cytologic characteristics. Immunohistochemical stains for hPL and hCG were not typical for either PSTT or choriocarcinoma. We suggest that poorly differentiated PSTT may synthesize the lesser amount of hPL than typical PSTT and it may behave more aggressively.
Diagnosis, Differential ; Biopsy

No abstract available.
Down Syndrome* ; Humans ; Infant* ; Leukemia, Megakaryoblastic, Acute*

No abstract available.
Lipodystrophy*

BACKGROUND: CD34 is a 115 kD glycoprotein which is expressed on hematopoietic progenitor cells. It is also known as an immunohistochemical marker of endothelial cells. OBJECTIVE: This study was designed to investigate the patterns of CD34 expression on: (1) cutaneous benign and malignant vascular tumors and (2) on the mature and immature vessels of pyogenic grauloma and capillary hemangioma. METHOD: We performed immunoperoxidase staining using a monoclonal anti-CD34 antibody (QBEND/10) on formalin fixed, paraffin-embedded sections of 23 benign and malignant cutaneous vascular tumors. RESULTS: The results are summerized as follows: 1. In 3 cases of nevus flammeus and 6 cases of carvernous hemangioma, vascular endothelial cells of all hemangiomas showed CD34 expressions. In 5 cases of angiokeratoma, endothelial cells of hemangioma, did not express CD34. 2. In all 5 cases of pyogenic granulomas and one case of capillary hemangioma, endothelial cells of mature vessels, endothelial cells near the well-formed lumina and endothelial cells showing intracellular lumina showed strong positivity for CD34, wbile endothelial cells far from the lumina and endothelial cells without lumina formation mostly showed negative staining for CD34. 3. One cese of Kaposis sarcoma showed focall positivity for CD34 both in endothelial cells of the small, well-formed vessels and spindle cells. Two cases of angiosarcoma showed CD34 expression only in endothelial cells of well-formed, normal appearing vessels, whereas atypical endothelial cells of tumor vessels and spindle cells were negative for CD34. CONCLUSION: CD34 could be a marker for endothelium in mature, well-differentiated vascular structures and may serve as a marker of lumen formation or differentiation of endothelial cells.
Angiokeratoma ; Endothelial Cells ; Endothelium ; Formaldehyde ; Glycoproteins ; Granuloma, Pyogenic ; Hemangioma ; Hemangioma, Capillary ; Hemangiosarcoma ; Hematopoietic Stem Cells ; Negative Staining ; Port-Wine Stain ; Sarcoma, Kaposi

Achondrogenesis type 2 is a lethal form of congenital skeletal dysplasia characterized by severe short-limbed dwarfism, decreased vertebral ossification and normal ossification of the skull. We report an autopsy case of achondrogenesis type 2 in a female fetus terminated at 29 weeks of gestation. External morphology revealed a relatively large head, short upper and lower extremities, short neck, and distended abdomen. The x-ray finding showed normal calvarial ossification, hypoplastic ilium and unossified ischium, and metaphyseal flares of the femur and tibia. Histologically, chondrocytes were large and irregular with increased vascularity.
Abdomen ; Autopsy* ; Chondrocytes ; Dwarfism ; Female ; Femur ; Fetus ; Head ; Humans ; Ilium ; Ischium ; Lower Extremity ; Neck ; Pregnancy ; Skull ; Tibia

Porencephaly is a rare congenital anomaly characterized by a cavitary hole extending from the cerebral mantle to the ventricle of the brain and covered by leptomeningeal membrane. It is now generally believed that the porencephaly represents a fetal or neonatal destructive process of the neural tissue. Authors experienced an autopsy case of bilateral porencephaly in a 35-day-old Korean male infant who died of sepsis. The brain weighed 150 gms and morphologically well formed except for two large symmetric defects at the fronto-parietal region. These defects were characterized by cleft-like shape, covering the entire cerebral mantle with resultant direct communication between ventricular cavity and leptomeninges. Thin leptomeningeal trabeculae were covering the cavity. There was a smooth transition between ependyma and roof of the defects. There are focal, microscopic cerebral infarct and abscess without connection with the cavitary lesion. The remaining central nervous system was unremarkable.
Infant ; Male ; Female ; Infant, Newborn ; Humans

Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocypenia and eczema. Various defects in cell-mediated immunity and deficient antibody reponse to carbohydrate antigens have been described. We experienced a case of Wiskott-Aldrich Syndrome of 28 months old male patient. He has been suffered from multiple petechiae with bleeding, recurrent pyogenic infections and generalized eczema since 3 months of age. Immunological abnormalities are as follows: 1) Serum IgM was gradually decreased, while IgA and IgE were increased. 2) Antibody response against polysaccharide antigen (PRP) was not observed after 3 times of PRPT immunization. 3) CD4/CD8 ratio was reversed (0.6). 4) Proliferative response of mononuclear cells was significantly reduced, and CMI skin test also showed negative results. A brief review of literature was made.
Antibody Formation ; Child, Preschool ; Eczema ; Hemorrhage ; Humans ; Immunity, Cellular ; Immunization ; Immunoglobulin A ; Immunoglobulin E ; Immunoglobulin M ; Male ; Purpura ; Skin Tests ; Wiskott-Aldrich Syndrome*

No abstract available.
Cytomegalovirus Infections*

A 12-year-old boy developed cholestatic hepatitis with Steven-Johnson syndrome following the use of amoxicillin. The skin lesion and general condition were improved over 2 weeks, but jaundice was gradually aggrevated. We performed liver biopsy, on 30th hospital day, which showed cholestatic hepatitis. The patient improved gradually and liver function was normalized 5 months later.
Amoxicillin ; Biopsy ; Child ; Hepatitis* ; Humans ; Jaundice ; Liver ; Male ; Skin

Multiple primary tumors are defined as cases involving primary malignant tumors of different histologic origins in one person. The absolute number of reported cases of double primary malignant tumors has increased in recent years diagnostic procedures. Primary adenocarcinoma of duodenum is a rare disease and represents less than 0.5% of all gastrointestinal malignancies. Therefore, synchronous multiple primary cancers of the stomach and duodenum are very rare. Recently, we experienced a case of triple primary malignant tumors of different site originating from the stomach, duodenum and cervix in 71- year-old woman. She had had radiation therapy and chemotherapy for squamous cell carcinoma of the uterine cervix 8 years previously. We performed subtotal gastrectomy and pancreaticoduodenectomy with radical lymph node dissection. She remained healthy without any evidence of recurrence 12 months after the operation.
Adenocarcinoma ; Carcinoma, Squamous Cell ; Cervix Uteri ; Drug Therapy ; Duodenal Neoplasms* ; Duodenum ; Female ; Gastrectomy ; Humans ; Lymph Node Excision ; Pancreaticoduodenectomy ; Rare Diseases ; Recurrence ; Stomach ; Stomach Neoplasms* ; Uterine Cervical Neoplasms*