[Rare hereditary syndromes associated with polyposis and the development of malignant tumors].

UNLABELLED: Familial adenomatous polyposis (FAP) and Peutz-Jeghers syndrome are genetic diseases characterized by gastrointestinal polyps, extraintestinal manifestations, and autosomal dominant inheritance. The carriers of these diseases from early childhood are at risk for neoplasias at different sites, which are symptomatic at various ages. AIM: to study the clinical organ-specific manifestations in patients with FAP and Peutz-Jeghers, genetics update and possibilities of diagnosis, monitoring, and treatment of these diseases. MATERIAL AND METHODS: The authors give the results of their examination and follow-up of children with FAP and Peutz-Jeghers hamartoma-polypous syndrome. In addition, current data from PubMed, Medline (including reviews, original articles and case reports) were used. RESULTS: The main clinical organ-specific signs of multiple tumors in FAP and Peutz-Jeghers syndrome are shown. Data on the assessment of a risk for malignant tumors at various sites in the affected patients and their family members at different ages are provided. Each of these syndromes has a dissimilar genetic foundation. FAP is caused by the germline mutations in the APC gene, Peutz-Jeghers syndrome is by the STK11 gene, which predispose individuals to specifically associated neoplasias and require different follow-up strategies. Information on a phenotype-genotype correlation may serve as a reference point for the possible severity and various manifestations of a disease. An update on the molecular pathogenesis of these diseases is considered. CONCLUSION: Molecular genetic testing of the genes associated with FAP and Peutz-Jeghers syndromes makes it possible to timely recognize family members at high risk, to plan therapeutic strategy and to affect the course of a disease. The joint participation of pediatricians, proctologists, oncologists, morphologists, geneticists, and molecular biologists is essential to timely recognize the carriers of the syndromes and a better prognosis in these patients.

[Calf blood hemodializate reduces neurotoxic effect of hydrogen peroxide on human neuroblastoma cells].

OBJECTIVE: Our aim was to study the effect of calf blood gemodializat on apoptosis and intracellular signaling pathways of neuroblastoma cells SK-N-SH human. METHODS: Apoptosis was recorded by fluorescent microscopy using Hoechst 33342. Necrosis cells was monitored by propidium iodide. The fluorescence of the cells was recorded on a fluorescence inverted microscope Keyence BZ8100 (Japan). Formation of reactive oxygen species (ROS) in the cells of SK-N-SH was determined using nitroblue tetrazolium by absorbance at 620 nm on a plate reader "Uniplan". RESULTS: When adding hydrogen peroxide to the background of the calf blood gemodializat been decreasing apoptosis of these cells with 43 to 17% relative to apoptosis in the presence of a hydrogen peroxide. Under these conditions, the calf blood gemodializat significantly reduced ROS formation in human neuroblastoma cells SK-N-SH by the action of hydrogen peroxide. In these cells, we investigated the influence of calf blood gemodializat on apoptosis and intracellular signaling pathway involving mitogen-activated protein kinase (p38MAPK), extracellular regulatory kinase (ERK), phosphatidylinositol 3-kinase (PI-3K) and-Jun-N-terminal kinase (JNK) using their selective inhibitors. CONCLUSION: It was shown that the mechanism of the protective effect of calf blood gemodializat against peroxide-induced apoptosis in SK-N-SH dominant role is played by p38 MAPK and PI-3K.

[Solid pseudopapillary tumors of the pancreas: clinical and morphological characteristics, specific features of their immunophenotype, and diagnostic problems].

OBJECTIVE: To analyze 60 cases of solid pseudopapillary tumors (SPTs) of the pancreas, to reveal their most characteristic clinical and morphological features, and to study their possible histogenesis. MATERIAL AND METHODS: Sixty cases of SPTs of the pancreas underwent clinical, morphological, and immunohistochemical (IHC) examinations; a comparison group consisted of 86 pancreatic tumors of other histogenesis. RESULTS: It has been shown for the first time that SPTs are characterized by the nuclear expression of claudin 3 and the cytoplasmic expression of claudin 7. It has been also ascertained that the aberrant perinuclear (dot-like) expression of CD99 is a unique feature of these tumors. CONCLUSION: SPTs of the pancreas are distinguished by a diversity of clinical manifestations and morphological features, but have a unique immunophenotype, which can differentiate them from other types of pancreatic tumors.

[Chromophobic carcinoma of the kidney with intracytoplasmic tubules].

The paper describes a rare case of chromophobic carcinoma (eosinophilic variant) of the kidney in a 72-year-old woman with the ultrastructural feature--intracellular tubules along with vesicular structures and mitochondria. Intracellular tubules are also seen during immunohistochemical study of the tumor.

[Interdigitating dendritic cell sarcoma].

The paper presents a case of the extremely rare tumor--interdigitary dendritic cell sarcoma in a 47-year-old woman with buccal soft tissue lesion that has been immunohistochemically and electron microscopically verified.

[Unilateral renal tumor in a child: nephroblastoma concurrent with a rare form of (translocation?) renal-cell carcinoma].

The paper describes a rare case of a renal composite tumor in a two-year-old girl who has nephroblastoma and renal-cell carcinoma of alveolar-tubular structure with focal expression of pancytokeratin AE1/AE3, cytokeratins 7 and 20, EMA, vimentin, CD10, MELAN A, p 504 S, E-cadherin, most likely, carcinoma with Xp.11.2 translocation.

[Angiomyolipoma of the kidneys].

59 angiomyolipomas were studied: immunohistochemistry (IHC) was used in 24 cases, electron microscopy (EM) in 6 cases. 57 tumors had a typical structure, 2 did not contain fat component. Premelanosomes were found by EM in one tumor. Smooth muscle actin was found in all tumor cells. Involvement of lymph nodes was observed in 2 patients. Histogenesis of this tumor is discussed.

[Experience in treating Nephroblastomas]. / Nash opyt lecheniia nefroblastom.

The treatment of nephroblastomas which accounts for 72% of all malignant neoplasms in children is one of the topical problems of pediatric oncology. To detect the tumor at early stages of a tumorous process is one of the main conditions of successful treatment for nephroblastomas. The histological types of nephroblastoma, its international classifications and present-day treatment policy are given. A role of radiation treatment is shown at pre- and postoperative therapeutical stages. Drugs, their combinations, and the efficiency of polychemotherapy regimens in use are outlined. The importance of comprehensive examination of the patient to determine the extent of malignancy and to choose adequate antitumor treatment regimens is emphasized.

[Nephroblastoma with intestinal differentiation]. / Nefroblastoma s differentsirovkoi po kishechnomu tipu.

Available are two cases of Wilms's tumor with focal intestinal differentiation. Goblet- and endocrine-like cells as well as those similar to enterocytes were identified in one of the tumors upon electron microscopy. Squamous epithelial, cartilaginous cells, rabdomyoblasts presented in both cases. Problems of histogenesis and prognosis are under discussion.

[Mixed tumors of the kidney and lung]. / Opukholi smeshannogo stroeniia v pochke i legkom.

A renal tumor composed of fibrous and smooth-muscular tissues and containing focally disposed foam cells and tubular structures composed of cuboidal epithelium was found in a 2-year-old girl along with a similarly structured pulmonary tumor. The tumors were thought to be of a hamartoma nature and to have probably arisen from the mesonephros.

[A case of Wilms' tumor and neuroblastoma of the kidneys in a child]. / Nabliudenie opukholi Vil'msa i neiroblastomy pochek u rebenka.

Both kidneys in an 8-month-old boy contained tumours; Wilms' tumour was found in the right kidney and neuroblastoma in the left one. Nephroblastomatosis was observed in both kidneys. Multiple skin angiomas were observed after birth. Diagnosis of renal tumours was confirmed electron microscopically.

[Glomerulus-like structures in Wilms' tumor]. / Glomerulopodobnye struktury v opukholi Vil'msa.

Twenty-three cases of Wilms' tumour with numerous glomerulus-like structures (GLS) were studied. 74% children were admitted to the hospital with metastases or the latter developed within 2-3 months after a surgical operation. 5 out of the 8 children with bilateral Wilms' tumours had in the tumours an increased number of the GLS. There are some features which allow the GLS to be distinguished from the true renal corpuscles: the absence of capillaries and mesangium, structural polymorphism the presence of several pseudopapillae originating from different areas of the capsule. The possibility is shown to use these criteria in the differential diagnosis of renal sarcomas in children and sarcomatous variants of Wilms' tumour, in the determination of pseudocapsule infiltration and the degree of tumour tissue survival after the active therapy.

[Nephroblastoma: clinico-morphological characteristics and problems of classification]. / Nefroblastoma: kliniko-morfologicheskaia kharakteristika i voprosy klassifikatsii.

Clinical and morphological examinations were carried out in 159 children with nephroblastoma. A working histological classification of tumors with reference to prognosis is proposed. It includes typical (89 cases) and atypical (23 cases) variants of the tumor. Among typical tumors, those with predominance of nondifferentiated blastema are distinguished. The atypical tumors include tubular fetal rhabdomyomatous, cystic, sarcomatous (rhabdoid, clear cell) variants. A group of tumors with marked therapeutic pathomorphosis (27 cases) is distinguished among unclassified tumors. Unfavourable prognosis in tumors with predominance of undifferentiated blastema, sarcomatous variants, and tumors with marked therapeutic pathomorphosis is indicated. Attention is drawn to increased number of glomerulo-like structures and cell anaplasia as a possible unfavourable prognostic sign.

[Papillary tumors of the kidneys in children]. / Papilliarnye opukholi pochek u detei.

Five rare papillary renal tumors (2 tubular adenomas and 3 epithelial nephromas) found in children (4 of them girls) 7 months to 4 years old, are reported. Four of the tumors occurred in the right kidney. Micro- and ultrastructural characteristics of tumors of this type are described for the first time, and their histogenesis and diagnosis are discussed.

[Mesoblastic nephroma]. / Mezoblasticheskaia nefroma.

Three cases of the "cellular" variant of mesoblastic nephroma are reported. A feature of one of them was prolonged (7.5 months) spontaneous development of the tumor which presented a peculiar morphologic appearance. Diagnostic differentiation of mesoblastic nephroma from Wilm's tumor and sarcomas is discussed. Results of an electron-microscopic study of one of the tumors are given.

[Malignant epithelioid schwannoma of the kidney]. / Zlokachestvennaia épitelioidnaia shvannoma pochki.

Malignant epithelioid renal schwannoma was recorded in a 16-year-old youth. Examined light-optically and electron-microscopically, it showed signs of multiprofile differentiation involving elements of a malignant schwannoma, neurofibroma, undifferentiated epithelioid component, photoreceptors, cells with neuroendocrine granules, Vater-Pacini corpuscles.

[Secondary tumors of the heart (based on autopsy materials in the Oncology Research Center of the Academy of Medical Sciences of the USSR over the period of 1960-1977)]. / Vtorichnye opukholi serdtsa (po materialam autopsii v Onkologicheskom nauchnom tsentre AMN SSSR za 1960-1977 gg.).

The autopsy material of the USSR AMS Cancer Research Center for 1960--1977 was studied. Among 3327 patients dying of malignant tumours, secondary tumour involvement of the heart was revealed in 170 (5.1%) cases, in 119 patients the tumour metastasized into the heart, in 51 grew into the pericardium. Most frequently, tumour involvement of the heart occurred in lung cancer, systemic diseases of the hemopoietic tissue, melanoma, and mammary gland carcinoma. Growth of the tumour into the heart, as a rule, was found in systemic diseases and lung cancer. Melanoma, renal carcinoma, and carcinoma of the oral cavity were characterized by small- and large-nodular forms of metastasis. The tumour involvement was most frequently found in the pericardium followed by the myocardium. In outgrowths of the tumour, the outer layer of the pericardium and in small-nodular metastases the visceral layer (epicardium) were affected most frequently. Hemorrhagic pericarditis was frequently observed in tumour alterations of the heart. Metastases and outgrowths into the heart were always associated with progression of the tumour.

[1 of the variants of Wilms' tumor]. / Ob odnom iz variantov opukholi Vil'msa.

A peculiar variant of Wilms' tumor was studied. Out of 77 cases of nephroblastoma 2 observations were selected in which the tumor consisted of loosely arranged oval and polygonal cells with clear nuclei. Occasional formed tubules were found in the tumor. The cells had anastomosing processes, few organoids, microfibrils. A nonepithelial nature of the tumor is assumed. A trend for primary metastasis of the tumor into bones was observed. Because of the difficulties and rare occurrence of such tumors, their histology and differential criteria are presented at the light and ultrastructural levels. Indication of this variant in the diagnosis may also suggest the possibility of metastatic affection of the skeleton.

[Clear-cell variant of nephroblastoma (clinico-morphological characteristics of 9 cases)]. / Svetlokletochnyi variant nefroblastomy (kliniko-morfologicheskaia kharakteristika 9 nabliudenii).

Nine post-mortem cases included 5 boys and 4 girls at the age from 1 1/2 to 13 years. The ratio right/left kidney was 6 to 3; average tumour weight 780 g, higher than in typical nephroblastoma. Bone metastases were found in 6 cases, the therapy was inefficient. Light-microscopic examination revealed structural polymorphism (typical for nephroblastoma areas, fibroses, hyalinosis, myxomatosis), smooth muscles in 2 cases. Electron microscopy of clear cells revealed fibrillar component developed at a various degree, vacuoles, pynocytosis vesicles, large nuclei with fine structure of chromatin. The results indicate the clear-cell variant of nephroblastoma is a sarcomatoid tumour with characteristic structure and uncommon clinical behaviour which makes necessary a special diagnostic and therapeutical approach.

[Melanocytic schwannoma]. / Melanotsitarnaia shvannoma.

One observation of melanocytic schwannoma in a woman aged 53 is described. The tumour localized in the subcutaneous fat of the crus and looked like an encapsulated node. Electron-microscopic study indicated the signs of schwannoma and the presence of premelanosomes in the tumour cells. Polymorphism of the cells and the increase in mitotic figures was suggesting of a malignant character of the tumour. The differential diagnosis with melanoma should be based on clinical, light-optical and ultrastructural data.