A rare case of extramedullary spinal cord germinoma.

A rare case of spinal cord germinoma in a 20-year-old Japanese female is presented. The tumor was detected at the level of T11 and L3 by myelography. Histological findings of the resected tumor were identical to germinoma, which is characterized by polygonal tumor cells, lymphocytic infiltrate, and granuloma. Tumor cells and lymphocytes reacted with antibodies for placental alkaline phosphatase and CD45, respectively. A few multinucleated giant cells, negative immunohistochemically for human chorionic gonadotropin were also seen. The present case is the 10th case of spinal cord germinoma and is thought to be the first case of such a tumor located extramedullary. Diagnosis of such a rare tumor in an unusual location needs proper histological, ultrastructural and immunohistochemical evaluation.

Primitive neuroectodermal tumor in sinonasal region.

An elderly woman having a nasal tumor diagnosed as alveolar rhabdomyosarcoma was referred to our hospital. Histological reexamination of the patient revealed that the tumor was composed of lobules of compactly arranged small round atypical cells. The atypical cells contained large oval to round vesicular nuclei, and scanty cytoplasm showing PAS positive glycogen material. The tumor cells were immunohistochemically positive for NSE, S-100 protein and vimentin, but showed negative reaction for myoglobin, desmin, EMA, keratin, LCA, chromogranin and MIC2. Ultrastructurally, the tumor cells contained a few cluster of glycogen particles and less organellae and filaments, and there were no cytoplasmic processes, neurosecretory granules or neurofilaments. Pathological findings suggested primitive neuroectodermal tumor resembling extraskeletal Ewing's sarcoma, but negative reaction for MIC2 immunohistochemically could not ascertain the diagnosis. Location of the tumor and age of the patient of the present case were unusual.

Pigmented neuroectodermal tumor of infancy in the epididymis. A case report.

BACKGROUND: Pigmented neuroectodermal tumor of infancy (PNTI) is a rare pigmentary tumor. Cytologic study of this tumor had not been performed before. In the present study, cytology of such a tumor was done in addition to histology, electron microscopy and immunohistochemistry. CASE: A 5-month-old infant had a scrotal tumor. After tumorectomy, touch smear for cytology revealed small and large tumor cells. The small cells showed a high nuclear/cytoplasmic ratio, and the large cells contained pigment in their cytoplasm. Histologically, the small cells and large, pigmented cells showed a typical alveolar and tubular pattern, respectively. The small cells neuroblastlike and the large cells showed melanocytelike features ultrastructurally and immunohistochemically. CONCLUSION: Cytology has not been applied to the diagnoses of PNTI. In our opinion, fine needle aspiration cytology could be helpful in the preoperative diagnosis of this tumor.

A case report of carotid body tumor.

A case of left carotid body tumor is presented. A 47-year-old female patient consulted our otorhinolaryngological clinic with a neck swelling that had persisted for 5 months. On physical examination, a movable and pulsating hard mass measuring 2 x 2.5 cm in size was found on the left side of her neck, near the angle of the mandible. Computed tomography, magnetic resonance image and angiography all demonstrated a well-circumscribed tumor mass showing high vascularity and located at the bifurcation of the left carotid artery. The tumor involved the left carotid artery, but the patency of the artery was preserved. The patient showed satisfactory temporary balloon occlusion test results without neurological complications. Since the tumor was strongly adherent to the carotid arterial wall, the tumor was resected together with the carotid artery. Histologically, the tumor was composed of organoid clusters of round cells with eosinophilic granular cytoplasm. Involvement of tumor cells was seen to the adventitia of the carotid artery, but only slight cellular atypia was seen. Ultrastructurally, two types of cells were observed in the central and marginal portion of the tumor; these were round chief cells with few cytoplasmic neurosecretory granules and spindle-shaped sustentacular cells. Immunohistochemically, the chief cells and sustentacular cells showed positive reaction for neuron-specific enolase and S-100, respectively. The ultrastructural findings suggested the benign nature of the tumor.

Clinicopathologic features of resected primary adenosquamous carcinomas of the liver.

Four cases of resected adenosquamous carcinoma of the liver were clinicopathologically reviewed, together with immunohistochemical findings. Although no lymph node metastases were seen and a curative resection was achieved in all cases, two patients had recurrences in the peritoneum and distant organs such as the pericardium and pleura relatively soon after the operation. Of the remaining two cases, one patient died during the postoperative period and the other died of coexistent hilar cholangiocarcinoma. Together these findings suggest that this disease tends to spread locally and distantly in the early phase of tumor growth and shows aggressive biological behavior. In an immunohistochemical study, involucrin was a specific marker for the squamous component and CA19-9 was a marker for the adenomatous component.

Cardiac rhabdomyoma of a neonate: an autopsy case report.

A primigravida delivered a cyanosed female infant with a very low Apgar score. Cardiac anomaly of the fetus was detected at 32 weeks of gestation by ultrasonography. The baby died on the day of delivery. Autopsy revealed multiple tumor masses in the interventricular septum and ventricular walls. The tumor originating from the interventricular septum was the largest and measured 3.7 x 3 cm. Histologically, the tumor was composed of large polygonal glycogen-laden cells and 'spider-cells'. Eosinophilic giant histiocytic cells were also observed in the spleen. Ultrastructural features of the tumor cells correlated with those of typical cardiac muscle cells.

A true hermaphrodite with bilateral ovotestes: a case report.

A 20-month-old phenotypically male child with ambiguous external genitalia came to this hospital. Chromosomal and DNA analyses revealed 46/XX karyotype and absence of SRY gene, respectively. Two gonadal masses in the labioscrotal folds histologically showed both ovarian and testicular tissues. Clinicopathological features of the patient suggested true hermaphroditism with bilateral ovotestes.

Mixed gonadal dysgenesis with gonadoblastoma in a female with Y chromosome: case report.

A young female with primary amenorrhea and male karyotype (46/XY) is reported. Physical examination and clinical investigation of the patient suggested mixed gonadal dysgenesis (MGD). After gonadectomy, a tumor was found on the left side and histologically diagnosed gonadoblastoma. Postoperative decreased level of testosterone and estradiol reflected the activities of the tumor.

Plexiform leiomyoma of the esophagus: a peculiar gross variant simulating plexiform neurofibroma.

A plexiform variant of leiomyoma of the esophagus in a 51-year-old woman is reported. The patient was diagnosed with a tumor of the esophagus in an X-ray mass survey of the upper gastrointestinal tract. She was referred to the Ryukyu University Hospital for further examination. She appeared healthy with no complaints. Upper gastrointestinal series revealed an oval, well-defined filling defect in the lower esophagus just above the esophagogastric junction. Endoscopy revealed an undulating bulge covered with normal esophageal mucosa. Endoscopic ultrasonography showed a sharply demarcated hypoechoic mural tumor with internal linear pattern, with no evidence of penetration into the surrounding tissue. These findings were evaluated as consistent with a leiomyoma. Removing the tumor by enucleation was easily accomplished. Unexpectedly, on gross inspection, the tumor was a plexiform type, mimicking a plexiform neurofibroma. Light and electron microscopic examination and immunohistochemistry of the tumor tissue confirmed leiomyoma. Since the enucleation of the tumor, the patient has been free of recurrence and symptoms for 1.5 years at the time of this report.

An autopsy case of malignant lymphoma with Lyell's syndrome.

We present a case of fatal Lyell's syndrome which developed following a CT examination using omnipaque 3000 contrast medium. A 59-year-old man was suffering from malignant lymphoma. He was readmitted to this hospital due to relapse of fever and lymph node swelling. On the day of readmission, generalized erythema, purpura, and mucosal erosions developed after a CT examination. Steroids and chemotherapy were ineffective, and he expired approximately two weeks after admission. Drug-induced dermatopathy or leukemic cell infiltration in the skin was clinically suspected. Histological findings disclosed toxic epidermal necrolysis.

Effect of 20-methylcholanthrene on the development of atherosclerosis in LAP quails.

We studied the effect of 20-methylcholanthrene, a carcinogen, on atherosclerosis in the ascending aorta and brachiocephalic arteries of hyperlipidemic and atherosclerosis-prone (LAP) quail. A total of 66 quails were divided into 6 groups and fed the following diets: Group I, basal; Group II, basal+low dose of carcinogen; Group III, basal + high dose of carcinogen; Group IV, basal + 0.2% cholesterol; Group V, basal + 0.2% cholesterol + low dose of carcinogen; and Group VI, basal + 0.2% of cholesterol + high dose of carcinogen. The carcinogen was dissolved in corn oil at 2 mg/ml and 4 mg/ml as low and high doses respectively, and was given orally twice weekly. Marked elevation of the serum cholesterol level and significant lipid-rich aortic lesions were observed in all the cholesterol-fed groups after 12 weeks. Although the serum cholesterol level in Group VI was lower than that in Group IV, the severity of the atherosclerotic lesion was greater in the former than in the latter. An immunohistochemical study showed a positive reaction of DBA, PHA and OKM-1 with the lipid-containing cells of aortic intimal lesions.

A rare case of achalasia coexistent with sigmoid megacolon and associated with epilepsy.

A case of achalasia coexistent with sigmoid megacolon in a 38-year-old man with known epilepsy is described. The patient was referred to the Ryukyu University Hospital with a 4-year history of dysphagia and heartburn and a 1-year history of abnormal bowel movement. On admission, upper gastrointestinal (GI) series demonstrated a dilated, tortuous thoracic esophagus with a flask-type configuration. Barium enema studies showed a dilated sigmoid colon from the rectosigmoid junction to the descending colon. Myotomy (modified Jekler-Lhotka's procedure) for achalasia and simple sigmoidectomy for sigmoid megacolon were carried out. The biopsied wall of the narrowed esophageal segment at operation showed decreased numbers of ganglion cells in Auerbach's plexus and atrophy of the muscle fibers. The resected dilated sigmoid colon revealed degeneration and markedly decreased numbers of ganglion cells in Auerbach's and Meissner's plexuses. The patient's postoperative course was uneventful and he has been doing well since surgery. The present case is very interesting and to our knowledge, such a case is rare in the literature. We believe that the abnormalities of the ganglion cells may be due to the same etiologic factor as the sigmoid megacolon. The association of the two pathologic processes is discussed, together with a brief review of the literature.

[Expression of PDGF-A and -B in human coronary atherosclerotic lesion: immunohistochemical and in situ hybridization study].

We studied the role of platelet-derived growth factor (PDGF) on the development of atherosclerosis of human coronary arteries of 63 autopsied cases. Smooth muscle cells in fibrocellular intimal thickening lesion showed no significant immunohistochemical reaction of antibodies for PDGF-A or PDGF-B or PDGF-receptor. In atherosclerotic lesions, foam cells derived from macrophages and smooth muscle cells showed intense immunohistochemical reaction with antibodies of PDGF-B and PDGF-receptor, but not with that of PDGF-A. By in situ hybridization, no significant signals of PDGF-A or PDGF-B or PDGF-receptor were demonstrated in proliferating intimal smooth muscle cells in fibrocellular intimal thickening lesions. Uncomplicated atherosclerotic lesions expressed significant amount of m-RNA of c-sis protooncogene and PDGF-B receptor in foam cells. However, no significant signals of PDGF-A were observed in uncomplicated atherosclerotic lesions. These results suggest that foam cells-producing PDGF-B play an important role for the progression of atherosclerotic lesions in human coronary arteries.

[An autopsy case of periarteritis nodosa associated with disseminated strongyloidiasis].

An autopsy case of periarteritis nodosa associated with disseminated strongyloidiasis in a 59-year-old woman is reported. The patient had unknown fever of 38 degrees C and marked impairment of renal function, for which pulse therapy of steroid was performed. Electromyogram showed myogenic pattern, and biopsy of the biceps presented necrotizing angitis of small arteries. Renal biopsy showed marked infiltration of lymphocytes in the stroma, and frequent hyalinosis and crescent formation of the glomeruli. Two months after admission, the patient died of respiratory failure associated with sepsis and disseminated intravascular coagulopathy. Postmortem examination disclosed strongyloides stercolaris and fungi in both lungs with extensive hemorrhage. Terminal ileum and ascending colon had multiple erosions, extensive hemorrhage, numerous strongyloides stercolaris, and frequent necrotizing angitis in the mucosa. Necrotizing angitis was also demonstrated in both kidneys.

Expression of platelet-derived growth factor and c-myc in atherosclerotic lesions in cholesterol-fed chickens: immunohistochemical and in situ hybridization study.

Immunohistochemical examination showed no significant expression of platelet-derived growth factor-A (PDGF-A), PDGF-B, PDGF receptors, or of c-myc in the thoracic and abdominal aortas of normal roosters. In cholesterol-fed roosters, intense immunohistochemical reaction for PDGF-B, PDGF receptor, and c-myc was seen in the lipid-rich thickened intimal lesions of the thoracic and abdominal aortas while no significant immunoreaction for PDGF-A was demonstrated in the same lesions. In accordance with immunohistochemical findings, in situ hybridization demonstrated a significant level of expression of PDGF-B, PDGF-A receptor, PDGF-B receptor, and c-myc genes in proliferating intimal cells of the thoracic and abdominal aortas. These results suggest that coordinate actions of PDGF-B and c-myc play an important role in proliferation of intimal cells in the developing atherosclerotic lesions in chickens.

[Cytofluorometric analysis of DNA content in proliferating cells of coronary arteriosclerotic lesions].

Cytofluorometric determination of DNA content was done on paraffin-embedded tissues of 19 cases of coronary arteriosclerotic lesions including fibrocellular intimal thickening lesions (FT) or atherosclerosis (AS). DNA distribution pattern of medial smooth muscle cells of coronary arteries with FT and AS was all diploid. The average proliferative index (PI) of both medial smooth muscle cells of coronary arteries with FT and AS was 4.8 +/- 0.6. DNA distribution patterns of intimal cells of coronary arteries with FT and AS were also diploid. The average PI of intimal cells of coronary arteries with FT and AS was 8.4 +/- 1.0 and 9.1 +/- 0.7, respectively. These results suggest that intimal cellular proliferation plays an important role in the development of atherosclerosis.