[Clinico-morphological parallels in primary liver tumors in children].
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Biomedical subjects
Publications and source records attributed to M A Shabanov.
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Two cases of renal rhabdomyoid sarcoma with alveolar structures are described. Both children were under one year of age. In one of them the tumour was found at the age of two weeks and was combined with congenital anomaly of the calyx-pelvis system. The tumors were detected incidentally in both children who entered the hospital with the diagnosis of Wilms' tumour. Excretory urography revealed in one case the picture which somewhat differed from that typical for nephroblastoma. The histological and electron-microscopic examinations were performed in both cases. Differential diagnosis from rhabdomyosarcoma is discussed.
Combined morphological examinations of 55 primary tumors of the liver in children as well as livers of 7 human embryos and fetuses were carried out. The finding in hepatoblastoma tissue of the elements of hepatic diverticulum of endoderm persisting in the tumor tissue and comparison of the study results with the data on the embryogenesis and organogenesis of human liver suggest that primary hepatic tumors originate from the pluripotent endodermal germination layer. Islets of tissues not found in the normal liver as well as "osteoid" and squamous epithelium most likely result from cataplasia of "polygonal" myoepithelial-like cells of biliary epithelium in the period of synthesis of basal membrane substance. A classification of primary liver tumors in children taking into account the level of tumor anlage in the ontogenesis and based on the histogenetic principle is proposed.
Composite morphological studies established that "osteoid" may consist of various portions of finely granular nonparticular basic substance and fibrous structures resembling collagen fibers and randomly interwoven in different directions. This substance was found to be synthesized by myoepithelial-like cells contacting by desmosomes and desmosome-like contacts and having in their cytoplasm abundant bundles of microfibrils, developed granular endoplasmic reticulum, and Golgi zones. The regularities of histotopographic location of such islets suggest that polygonal cells of forming bile ducts subject to cataplasia participate in the synthesis of intercellular "osteoid-like" substance. The similarity between hepatoblastoma of the mixed type and pleomorphic adenoma of the salivary gland is indicated.
Mesenchymal hepatic hamartoma in 4 children aged 7 months, 13 months, 16 months and 8 years is described on the basis of histological, histochemical, and electron microscopic studies. It is suggested that hepatic hamartoma is a malformation consisting of excessive outgrowth of large intrahepatic bile ducts and their tubular-alveolar glands together with the surrounding connective tissue sheath.
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An observation of congenital hepatoblastoma of a mixed type in a baby dying on the 50th day of life is described. The boy was born with a metastasis of the tumour into the left superciliary area. The process of hepatoblastoma metastasising had begun in the intrauterine period. The main and metastatic nodes of the tumour consisted of epithelial and mesenchymal components. The multicomponent structure of the tumour attests to its origin from a pluripotent blastoma like Wilms tumour. In addition to the hepatoblastoma with disseminated metastases the boy had malformations in the liver and kidneys. The combination of hepatoblastoma with congenital malformations appears to be due to the effect on the fetus of some harmful factors in the first trimester of pregnancy (toxicosis, influenza).
Histological and electron microscopic studies of primary malignant liver tumors in children established the differences in the morphological composition of the epithelial and mixed variants of hepatoblastoma. The epithelial component is represented by embryonal and fetal cells at different levels of ultrastructural and functional differentiation. The mixed variant of hepatoblastoma is characterized by islets of stellate and lymphocyte-like cell capable of differentiation into epithelial and mesenchymal components. The latter does not have a regular composition and may include cells differentiating both into tissues with formation of myofibrils, collagen, glycosamine-glycanes, and hematogenically with formation of hematopoietic foci and vascular structures. Hepatocarcinomas in children are similar to those in adults. The results of the study attest to the origin of hepatoblastoma from a pluripotent source.
The review is based on the analysis of 17 national and 58 foreign publications dealing with the epidemiology, classification, age features, morphology, ultrastructure, histogenesis, complications and outcomes of primary malignant tumors of the liver in children. This analysis showed primary tumors of the liver in children to occupy the third place among neoplasia of the abdominal cavity and retroperitoneal space. There are no universally accepted terms for their designation and this prevents the development of a unique classification. Under 3 years, tumors develop predominantly from the embryonal tissue: hepatoblastomas of epithelial and mixed types frequently combined with congenital disorders and malformations of other organs. At more advanced age, hepatic carcinoma, a tumor of the adult type, is found. The review deals with the problems of histogenesis and classification of primary hepatic tumors in children. An opinion is given on the entodermo-mesodermal nature of embryonal hepatic tumors from the pluripotential blastema capable of malignization both into entodermal and mesodermal components. The prognosis of the disease is poor, 90% of children with hepatoblastoma and 80% with hepatocarcinoma die within one year after the diagnosis is established.,
Three different approaches to treatment of non-seminomal germinogenic testicular tumors (NSGTT) of stage I after orchidofuniculectomy: preventive retroperitoneal lymphadenectomy, preventive chemotherapy, expectant treatment. Recurrences, 5-year recurrence-free and overall survivals reached 17.4, 81.8 and 95.4%; 6.3, 93.8 and 100%; 33.3, 66.4 and 83.5%, respectively. Progression occurred more frequently in patients having invasion of tumor cells in lymphatic and blood vessels in the primary tumor. The authors conclude on preferable use of preventive chemotherapy after removal of the primary tumor.
Polymorphic alleles of CYP17 and CYP19, which are involved in estrogen biosynthesis, were tested for association with breast cancer (BC). Microsatellite (TTTA)n and 3-bp deletion of CYP19 and single-nucleotide polymorphism T27C of CYP17 were analyzed in 123 BC patients and 119 healthy women. Of the six (TTTA)n alleles observed, allele (TTTA)8 proved to be associated with BC (11.8% vs. 6.3%, P = 0.04). Genotype A2/A2 of CYP17 was also associated with BC (32.5% vs. 20.2%, P = 0.04). Risk of BC was especially high in the presence of both factors (7.3% vs. 0%, P < 0.01). Allele (TTTA)8 and genotype A2/A2 were assumed to be risk factors of BC.
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