PANCREATIC CARCINOMA IN CHILDHOOD: Report of an Autopsy Case and a Review of the Literature

Clinical and histological findings of pancreatic carcinoma in a 6-year-old boy are reported. Gradual change of histological appearance of the tumor during his course of 3 years and elevation of serum alpha-1-fetoprotein (AFP) are documented. Two biopsy specimens showed immature histological appearance compatible with pancreatoblastoma, and autopsy material showed well-differentiated adenocarcinoma with distinct ductal and acinar differentiation. Electron microscopy demonstrated zymogen-like granules in the apical portion of the neoplastic cells. Immunoperoxidase method demonstrated AFP in the neoplastic cells in addition to alpha-1-antitrypsin. Literature of pancreatic tumor in the young was reviewed, and characteristics of this case were discussed. ACTA PATHOL. JPN. 35 : 1543–1554, 1985.     

Induction of suppressor T cells by intravenous administration of monoclonal anti-I-A antibody

Intravenously administered monoclonal anti-I-A antibodies successfully induced suppressor T (Ts) cells specific for alloantigen-specific delayed-type hypersensitivity (DTH) responses in mice. These Ts cells exerted their effects in the effector phase and had no H-2 restrictions. Phenotypic analysis revealed L3T4 antigens on their cell surface but failed to reveal Lyt-2 antigen. Ts cell activity was abrogated by a 30-min incubation with the anti-I-A antibodies used for Ts cell induction. Incubation in the anti-I-A-antibody-coated plate also abrogated the Ts cell activity. Since anti-I-A antibody is idiotypic for the I-A antigen, it is suggested that these Ts cells might express antiidiotypic receptors for I-A antigens. These findings are considered to be consistent with previous observations of hapten-specific systems, in which antiidiotypic Ts cells are inducible by idiotypic antibodies.     

An Autopsy Case of Renal Cell Carcinoma Associated with Extensive Peliosis Hepatis

An autopsy case of renal cell carcinoma with extensive peliosis hepatis is reported. The patient was a 34-year-old female, who had had a left nephrectomy for renal cell carcinoma but died of multiple metastases one year and 4 months after surgery, despite chemotherapy and interferon treatment. At autopsy, the liver was enlarged markedly with multiple metastatic nodules and the nonneoplastic hepatic parenchyma had a spongy appearance, due to diffusely scattered, blood-filled cystic lesions. Histological examination showed the oval to irregular shaped blood-filled spaces were lined by hepatic cell cords and located mainly in the periportal area. In addition, almost all of the sinusoids were dilated and communicated with the cystic blood-filled spaces, which also communicated directly with branches of the portal veins at various levels. Several interlobular portal branches were obstructed. The causative mechanism of peliosis hepatis has yet to be elucidated, although some causative conditions have been proposed. In this case, renal cell carcinoma may have caused the sinusoidal dilatation and the vascular changes in the portal areas, such as obstruction of terminal portal branches, may have contributed to its formation.     

Characterization of experimental rat nephroblastoma and its cell line.

Rat nephroblastoma (Wilms' tumor) was induced by transplacental administration of N-ethyl-nitrosourea (ENU). The induced renal tumors were histologically compatible with human nephroblastoma. A cultured cell line (ENU-T-1) established from a xenotransplant, showed similar morphological and biological features to cultured embryonal kidney cells. Introduction of normal human chromosome #11 (#11) bearing Wilms' tumor suppressor gene(s) (WT) suppressed colony-forming ability on soft agar plates (CFA) but tumorigenicity of ENU-T-1 was not affected. Whereas tumorigenicity of human nephroblastoma cell line, SK-NEP-1 was completely suppressed, CFA was unchanged. These facts indicated that pathogenetic mechanism is different between human and experimental rat nephroblastomas.     

Peripheral neuroectodermal tumors of the chest wall in childhood

A retrospective review of primary chest wall malignant tumors of childhood collected at the Children's Memorial Hospital of Chicago was undertaken. Among twelve instances of poorly differentiated neoplasms whose uniform, monotonous structure made accurate classification difficult or impossible by conventional histologic study, there were three tumors with features suggestive of neuroectodermal differentiation. Electron microscopic and immunohistologic findings further strengthened this interpretation, despite the fact that none of the patients had evidence of a primary neuroblastoma outside the chest wall. These results and a review of the pertinent literature support the conclusion that neuroectodermal neoplasms in childhood may present in peripheral somatic tissues with greater frequency than is commonly assumed. The importance of this distinction is discussed, particularly the need to distinguish these neoplasms from Ewing's sarcoma.     

Pulmonary hypertension associated with portal hypertension in childhood. Case report of a 6-year-old child and review of the literature

Pulmonary hypertension is a rare complication of portal hypertension. Reports of childhood cases especially rare. This report describes an autopsy case of a 6-year-old boy with congenital biliary atresia followed by liver cirrhosis in whom severe hypertensive pulmonary arterial changes, including medial hypertrophy, intimal fibrosis and plexiform lesions were demonstrated. Fresh and organizing fibrin-platelet thrombi as well as probable organized thrombi with recanalization were occasionally found in the pulmonary vasculature, but it was thought that they had probably been formed locally as a late complication rather than being of thromboembolic origin. Retrospectively, the chest roentgenograms had revealed abnormalities suggestive of pulmonary hypertension since infancy, but the patient showed no apparent symptoms of it during life. Previously reported childhood cases of pulmonary hypertension associated with portal hypertension were briefly reviewed. Although the mechanism is presently not known, it is suggested that patients with portal hypertension, even in early childhood, are at risk of developing this unusual complication.     

Ewing's sarcoma family of tumor arising in the adrenal gland--possible diagnostic pitfall in pediatric pathology: histologic, immunohistochemical, ultrastructural, and molecular study

We present an adrenal Ewing's sarcoma family of tumor (ESFT) arising in an 11-year-old Japanese boy. Although intensive chemoradiotherapy and radical surgery were performed, the patient died of obstinate disease 1 year and 3 months after the initial presentation. The primary site (adrenal gland) with radiologic findings (with foci of calcification), high titer of serum neuron specific enolase, and sheets of monotonous primitive rounded cells on histology mostly favored neuroblastoma. However, a diagnosis of ESFT was confirmed by immunohistochemical profile, including MIC2-positivity and molecular study disclosing EWS-FLI1 chimera gene verified by direct sequencing. Recognition of adrenal ESFT and use of newly developed diagnostic techniques are required for differential diagnosis of undifferentiated small round cell tumor of the adrenal gland.     

Stimulation of cellular growth and adhesion to fibronectin and vitronectin in culture and tumorigenicity in nude mice by overexpression of trypsinogen in human gastric cancer cells

It has previously been reported that the trypsinogen gene is expressed in various human cancers. To inves-tigate the possible role of trypsin in tumor malignancy, trypsinogen-1 cDNA was introduced into the human gastric carcinoma cell line MKN-1. The overexpression of trypsinogen-1 in MKN-1 cells stimulated cellular growth and adhesion to fibronectin and vitronectin when the trypsinogen activator enterokinase was added into the culture. Enterokinase treatment of the conditioned medium of the MKN-1 transfectants partially converted the proforms of gelatinases B and A to their apparent active forms. When the MKN-1 transfec-tants expressing trypsinogen-1 were intraperitoneally transplanted into nude mice, the mice frequently produced tumors in the colon, spleen and liver. However, the mice implanted with control MKN-1 cells produced no tumors. These results strongly suggest that tumor-derived trypsin contributes to the dissemi-nated growth of some types of cancer cells including gastric cancer. ©Lippincott Williams & Wilkins     

CLOVERLEAF SKULL SYNDROME An Autopsy Case and Review of Literatures

An autopsy case of cloverleaf skull deformity associated with hydrocephalus, systemic skeletal malformation including facial dysostosis, fused elbow, syndactyly of the toes, odd digits and striking anomaly of tracheal cartilage is presented. Extra-skeletal abnormalities included covered anus, dermal sinus and the absence of corpus callosum and the septum pellucidum. Severe basal skull deformity appeared to be a primary morphologic alteration associated with premature closure of the specific sutures, which terminally resulted in life-threatening hydrocephalus. Histological investigation showed the abnormalities of endochondral ossification in the cartilage at the epi- and meta-physis of the fused elbows. In addition, electron microscopical study revealed unusual fat droplet-containing chondrocytes even in the resting and multiplicative phase. Association of the tracheal anomaly with this syndrome was disclosed in this case. Generalized bone and cartilage abnormalities not only in the skeletal system but also in the internal organs strongly suggest that this disorder involves generalized osteocartilagenous system. The present case makes a total of 14 cases of the cloverleaf skull syndrome reported in the Japanese literatures to date. Major clinical and pathologic findings of these cases were summerized.