[Metastasis of the right colon mucinous adenocarcinoma to aortocaval and retropancreatic space: case report and literature review]. / Metastaz slizeobrazuyushchei adenokartsinomy pravoi poloviny obodochnoi kishki v aortokaval'noe i retropankreaticheskoe prostranstvo: obzor literatury i redkoe klinicheskoe nablyudenie.

Metastases of the right colon cancer to extra-regional lymph nodes are rarely observed. Available literature data cannot be a reliable guide to choose the optimal treatment strategy. Indeed, excision of extra-regional lymph nodes is a rare experience and its results are poorly represented. According to our clinical experience, surgical intervention following comprehensive examination may be radical in patients with right colon cancer if distant metastases are absent. Resection of extra-regional lymph nodes can be safely performed in these cases. We report a patient with the right colon cancer and lesion of extra-regional lymph nodes behind the pancreatic head, paracaval and paraaortic space, hepatoduodenal ligament. Standard laparoscopic right-sided hemicolectomy with D-3 lymph node dissection was accompanied by resection of a conglomerate of nodal metastases behind the pancreatic head and superficial resection of the pancreas. Extra-regional lymph node excision is a reasonable option for colon mucinous adenocarcinoma stage I-III. However, comprehensive preoperative examination is required. Technical difficulty of extra-regional lymph node excision it is not the reason for limitation of surgical intervention. However, safe and total resection requires an adequate surgical approach.

[Clinical and morphological analysis of Langerhans cell histiocytosis in a child of early age]. / Kliniko-morfologicheskii analiz sluchaya langergansokletochnogo gistiotsitoza u rebenka rannego vozrasta.

The authors characterize a rare variant of Langerhans cell histiocytosis in a child of early age. Multiple-system form including liver damage is characterized by a particularly severe course, especially in early childhood. The authors report clinical, morphological and immunohistochemical data of the patient aged 1 year and 11 months. It is shown that timely diagnosis of this disease should be carried out in close cooperation of surgeons and morphologists.

[Differential diagnostic value of the expression of the transcription factor PDX-1 in neuroendocrine and non-neuroendocrine tumors of the pancreas and other organs]. / Differentsial'no-diagnosticheskoe znachenie ékspressii transkriptsionnogo faktora PDX-1 v neiroéndokrinnykh i neneiroéndokrinnykh opukholiakh podzheludochnoi zhelezy i drugikh organov.

An important role in the differentiation of tissues in different organs is played by transforming factors (TFs); pancreatic and duodenal homebox 1 (PDX-1) is one of the earliest factors for pancreatic cells. Many malignant tumors, including neuroendocrine tumors (NETs), are similar in structure, and therefore the actual problem of oncomorphology is to search for narrow-specific markers and TFs. AIM: to comparatively analyze and assess the value of the expression of the TF PDX-1 in NETs and non-NETs of different localization and histogenetic origin. MATERIAL AND METHODS: Anti-PDX-1 antibodies were used to study 528 tumors divided into 3 groups: Group 1 included 394 NETs, among them there were those of the pancreas (n=173), stomach (n=46), bowel (n=65), lung (n=40), thymus (n=8), kidney (n=6), Merkel's cell carcinomas (n=14), NETs of the breast (n=3), larynx (n=2), trachea (n=2), bladder (n=1), and metastatic NETs (n=34) of unknown primary site; Group 2 consisted of 16 tumors, of them there were paragangliomas (n=6), medullary thyroid cancers (MTC) (n=6) and adrenal pheochromocytomas (APCC) (n=4); Group 3 comprised 118 non-NETs, among them there were tumors of the pancreas (n=54), stomach (n=26), bowel (n=17), lung (n=11), breast (n=3), kidney (n=4), adrenal glands (n=2), and bladder (n=1). RESULTS: PDX-1 was positive in 75.1% (130/173) of pancreatic NETs, all insulinomas (50/50), gastrinomas (11/11), somatostatinomas (3/3), ACTH-producing tumors (2/2); PDX-1 was positive in the non-functioning pancreatic NETs, all PPomas (19/19), 76.1% (35/46) of NETs without the hormone detected, 50% (2/4) of calcitoninomas, and 21.1% (8/38) of silent glucagonomas. PDX-1 was positive in 32.4% (11/34) of carcinoids and 50% (6/12) of neuroendocrine carcinomas, all duodenal NETs (18/18), 90% (9/10) of rectal carcinoids and 30.8% (4/13) colonic carcinoids, 37.5% (3/8) of thymic/mediastinal carcinoids, 66.7% (4/6) of kidney carcinoids, and 37.5% (9/24) of metastatic NETs of unknown primary site. PDX-1 was negative in all carcinoids of the colon and sigmoid (0/5), ileum and jejunum (0/24), lung (0/40), trachea (0/2), larynx (0/2), Merkel's cell carcinoma (0/14), breast (0/3), bladder (0/1), as well as MTC (0/6), APCC (0/4), and paragangliomas (0/6). PDX-1-positive non-NETs included 81.8% (18/22) of adenocarcinomas (AC) and all serous cystic, mucinous cystic, intraductal and acinar cell tumors of the pancreas (4/4, 3/3, 2/2, and 3/3), 57.1% of AC (8/14) and 83.3% of signet ring cell carcinomas of the stomach (10/12), 56.2% AC of the bowel (9/17), bladder cancer (1/1). PDX-1 was negative in all anaplastic cancers (0/2) and solid pseudopapillary tumors of the pancreas (0/20), cancers of the lung (0/11), kidney (0/4), breast (0/3), and adrenal glands (0/2). CONCLUSION: The expression of PDX-1 is very specific for most digestive tract NETs and non-NETs. Pancreatic ductal and acinar cell tumors and gastric signet ring cell carcinomas are most commonly PDX-1-positive. Most tumors that do not originate from the digestive tract have a PDX-1 negative immunophenotype.

[The morphological and molecular genetic characteristics of nephroblastomas in immature ovarian teratoma in adult patients]. / Morfologicheskaia i molekuliarno-geneticheskaia kharakteristika nefroblastomy v nezreloi teratome iaichnikov u vzroslykh bol'nykh.

The paper describes 2 cases of immature ovarian teratoma with elements of nephroblastoma (ICD-0 code 9080/3) in patients aged 61 and 70 years. Microscopic examination revealed that both cases had blastemal cells with scant cytoplasm and basophil nuclei sticking together. Epidermal, glandular, rhabdomyoblastic, chondroid, bone, neuroectodermal, and histiocytic components were determined. Papillary and glomeruloid structures and primitive tubules were immured in the sarcomatous stroma. Immunohistochemical studies showed a strong reaction with Wilms tumor 1 (WT1), paired box gene (PAX-2), cytokeratin 7, desmin, smooth muscle actin, and α-1 antitrypsin. The recurrent tumors displayed a 1.8- to 6.1-fold increase in the level of p53. At the same time, the molecular genetic study revealed p53 gene mutation. In both cases, serous ovarian cystadenocarcinoma was misdiagnosed, ineffective chemotherapy was performed, and a recurrence occurred. The literature review revealed only 8 such cases.

[The forensic medical estimation of the biological age of the corpse based on the morphological changes in the skin]. / Sudebno-meditsinskaia otsenka biologicheskogo vozrasta trupa po morfologicheskim izmeneniiam kozhi.

The objective of the present work was the immunohistochemical evaluation of the age-related dynamics of apoptosis-associated proteins contained in the human skin epidermis. We studied the samples of skin tissues obtained during the forensic medical expertises of 100 corpses of the subjects who died in the consequence of acute blood loss in the absence of injuries or pathological changes in the skin (56 men and 44 women at the age varying from 5 to 85 years). The results of this study give evidence that the histoimmunological investigations of the skin tissues can be carried out with the use of the mmunohistochemical Ki67, bcl-2, and p53 markers for the more accurate determination of the biological age of the deceased subjects. The quantitative characteristics thus obtained serve as reliable indicators of the chronic changes in the skin and can be used as the components of the combined diagnostic procedures.

[The morphological and molecular genetic characteristics of sarcomatoid chromophobe renal cell carcinoma]. / Morfologicheskaia i molekuliarno-geneticheskaia kharakteristika sarkomatoidnoi khromofobnoi pochechno-kletochnoi kartsinomy.

The paper describes a case of chromophobe renal cell carcinoma growing into the muscular layer of the descending colon and with metastases in 4 lymph nodes of paranephral tissue in a 66-year-old woman. The tumor had a zonal structure with an alternation of epithelioid and sarcomatoid structural sites and with the signs of grades I, II and III according to the grading system by Paner and et al. (2010). The sarcomatoid renal component occupied about 70.0% of the tumor. There was a pronounced immunohistochemical reaction with VEGF-A (5 scores), a high Ki-67 proliferation index (70%), and a large number of tumor cells with nuclear p53 expression (85%) in the areas with minimal differentiation and sarcomatoid elements (Grade III). These signs can serve as criteria for the aggressive behavior of the tumor. A large volume of the sarcomatoid carcinoma component and a strong reaction with VEGF-A are indications for targeted therapy with anti-VEGF drugs.

[Solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal amyloidosis of the lung and Rosai-Dorfman disease]. / Solitarnaia épitelioidnaia gemangioéndotelioma v sochetanii s noduliarnym parenkhimatoznym amiloidozom legkogo i bolezn'iu Rozai-Dorfmana.

The paper describes a case of solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal AL amyloidosis of the lung and Rosai-Dorfman disease in a 70-year-old woman. The core of the tumor was represented by bone tissue with dendriform ossification, as well as by amyloid that showed green apple birefringence at polarized light microscopy. The peripheral portions of the tumor and the myxohyaline stroma exhibited slit-like structures, epithelioid and fusiform cells with small cytoplasmic vacuoles. These cells expressed CD31, CD34, factor VIII, and cytokeratins 7 and 18. The Ki-67 proliferation index was 10%. S100- and CD68-positive histiocytes with the phenomenon of emperipolesis were revealed in the tumor and in the lymph nodes of the mediastinum and lung hilum. There was a positive reaction to immunoglobulin lambda light chains in the lymphocytic infiltration around amyloid clumps. The frequency of epithelioid hemangioendothelioma was less than 1 case per million people annually. We found only one case of its concurrence with pulmonary amyloidosis in the English-language literature. No relationship could be revealed between this tumor and Rosai-Dorfman disease.

[Disseminated combined small cell carcinoma with graft involvement after kidney transplantation from a deceased donor to two patients]. / Disseminirovannyi kombinirovannyi melkokletochnyi rak s porazheniem transplantatov posle peresadki pochek ot umershego donora dvum bol'nym.

The paper describes cases of disseminated small-cell carcinoma after kidney transplantation from a deceased donor to two patients. Microscopic examination showed that the kidney graft tumor consisted of tightly packed small rounded cells with hyperchromatic nuclei and a narrow cytoplasmic rim with invisible nucleoli. The mitotic index was 25-40/2 mm2. Azzopardi's phenomenon and crush artifact were detected in the tumor. Giant cell and large cell components were 30-40% of the area of sections. Immunohistochemical examination revealed the expression of synaptophysin, chromogranin A, CD56, TTF-1, HMWK, СК7, СК18, and Ki-67 (80% of tumor cells). Histological findings and immunophenotype in both cases led to the conclusion about combined small cell carcinoma with renal graft involvement. Both patients died from tumor dissemination 9 and 11 months after transplantations. In reviewing the literature, the authors found only one such observation.

[Eosinophilic granuloma of the parietal bone of an adult patient with BRAF mutation]. / Éozinofil'naia granulema temennoi kosti s mutatsiei gena BRAF u vzroslogo patsienta.

The paper describes a case of eosinophilic granuloma of the parietal bone in a 32-year-old man. Histological examination revealed a large number of bean-shaped Langerhans cell histiocytes with lobed nuclei and nuclear grooves. The histiocytes alternated with the foci of obvious eosinophilic infiltration and with eosinophilic microabscesses. There were osteoclast-like multinucleated giant cells, bone resorption, and numerous bone rods covered with osteoblast chains. The histiocytes expressed CD1α, langerin, CD68, S100, and p53 (in 90.0% of the tumor cells). The Ki-67 proliferation index was 18.0%. A molecular genetic study identified BRAFV600E mutation (nucleotide substitution s.1799 T>A (p.V600E) in the heterozygous state). Clinical and morphological data and the results of molecular genetic studies led to the conclusion that there was eosinophilic granuloma of the right parietal bone (the unifocal form of Langerhans cell histiocytosis (LCH), type I, group A1, with the monoossal nature of lesion and with BRAFV600E mutation). In adults, this disease is extremely rare (2-5 cases of LCH per million people, bone loss in the fourth decade of life in 2.5% of the patients).

[BRAF-positive paucicellular variant of anaplastic carcinoma in the presence of tall cell variant papillary thyroid cancer]. / BRAF-pozitivnyi malokletochnyi variant anaplasticheskoi kartsinomy na fone papilliarnogo raka shchitovidnoi zhelezy iz vysokikh kletok.

To paper describes a case of paucicellular anaplastic cancer in the presence of tall cell variant papillary thyroid carcinoma. Microscopic examination showed that the differentiated component of the tumor was composed of papillary structures with tall cells, the height of which exceeded 3-4 times the width. Its anaplastic component consisted of fibrous tissue with occasional spindle-shaped cells and focal lymphocytic infiltration to the extent of 70%. The spindle-shaped cells expressed cytokeratins, ß-catenin, p53, and vimentin. The tumor cells and lymphocytes showed an association with Epstein-Barr virus. Molecular genetic study of the tumor revealed the following mutations: BRAF p.Val600Glu (p.V600e was), HRAS p.His27His (p.H27H), PIK3CA p.Glu545Lys (p.E545K), TP53 p.Arg248Gln (p.R248Q).

[The forensic medical evaluation of traumatic and spontaneous ruptures of the organs affected by the tumours]. / Sudebno-meditsinskaia otsenka travmaticheskikh i spontannykh razryvov organov pri porazhenii opukhol'iu.

The present article was designed to report the results of the analysis of the cases of traumatic and spontaneous ruptures of the organs affected by the tumours based on the original observations and the literature data. It is shown that the probability of the tumour rupture depends on its histological type, localization, the size, and the distance from the capsule of the affected organ, the degree of involvement of the major blood vessels, the severity of the necrotic changes, the presence of cysts in the neoplasm, and the regimens of radio- and chemotherapy. Moreover, the rupture can be facilitated by anticoagulation therapy, intake or oral contraceptives, pregnancy, concomitant diseases, alcoholic intoxication, splenomegaly, and hypocoagulation resulting from dissemination of the neoplastic process or the metastatic lesions of the liver. Even a minimal injury to the skin can provoke the tumour rupture associated with the fatal hemorrhage. A delayed rupture within a few hours or days is possible.

[Parosteal osseous lipoma of the temporal bone concurrent with involvement of the parotid salivary gland]. / Parosteal'naya ossifitsiruyushchaya lipoma visochnoi kosti v sochetanii s porazheniem okoloushnoi slyunnoi zhelezy.

The paper describes a case of parosteal lipoma of the temporal bone, by simultaneously involving the parotid salivary gland. Computed tomography performed in a 48-year-old man showed that in the area of the posterior edge of the glenoid fossa of the right temporal bone there was a massive (1.5×1.7×0.9-cm) exostosis extending down from the skull base. The exostosis was surrounded by a 3.5×3.6×5.3 soft tissue mass that was located in the projection of the right parotid salivary gland with a capsule and minor calcifications. Morphological examination determined a sessile exostosis-like mass in the area of the periosteum of the temporal bone. Foci of desmal and enchondral ossification (types III and IV according to the classification of Miller et al.) could be seen in the adjacent fatty tissue. The tumor underwent necrotic and dystrophic changes (Milgram's Stage 3). Temporal bone involvement was accompanied by osseous sialolipoma of the right parotid salivary gland. In this case, the fatty component accounted for 60-80% of salivary gland volume. Terminal secretory segments, ducts, and bone trabeculae with calcification foci and hematopoietic elements were uniformly distributed among the fatty tissue. None similar case has been found in the literature.

[Aortic aneurysm in Erdheim's idiopathic cystic medial necrosis in autopsy and forensic medical practice]. / Anevrizma aorty pri idiopaticheskom kistoznom medionekroze Erdgeima v patologo-anatomicheskoi i sudebno-meditsinskoi praktike.

UNLABELLED: To date, the modern Russian literature has not covered morphological studies of aortic aneurysm in Erdheim's idiopathic cystic medial necrosis on autopsy and surgical materials, by using immunohistochemical studies, and it has not estimated the magnitude of pathohistochemical changes in the aortic media either. AIM: to conduct a morphological examination of aortic aneurysm in Erdheim's idiopathic cystic medial necrosis, by using the material of pathology and forensic medical departments. MATERIAL AND METHODS: 41 surgical samples from patients with idiopathic cystic medial necrosis of the ascending aorta and autopsy samples from 17 sudden deaths from aortic rupture were examined. The aortic wall was histologically and histochemically studied. Immunohistochemical examination was done using antibodies to smooth muscle actin, collagens types I and III, elastin, CD3 (T lymphocytes), CD20 (B lymphocytes), CD45 (leukocyte common antigen), CD68 (macrophages), apoptotic marker p53, tumor necrosis factor-α (TNF-α), and intercellular adhesion molecule-1 (ICAM-1). RESULTS: Morphological examination showed lamellar unit destruction, fibrosis, medial necrosis, and elastic fiber fragmentation. These signs were assessed by their degree. The immunohistochemical examination of collagens types I and III determined fascicles of randomly positioned fibers. The multidirectional orientation of smooth muscle cells was confirmed by the expression of smooth muscle actin. There was an obvious expression of the apoptotic marker p53 in the smooth muscle cells of the aortic media and in the aortic adventitia. The expression of TNF-α was revealed in the cells of an inflammatory infiltrate in the aneurysm wall and that of ICAM-1 was found in the aortic endothelium, vasa vasorum, and in the cells of an inflammatory infiltrate in the media and adventitia. The paper proposes a design of postmortem and forensic medical diagnosis, which reflects the components of pathogenesis and tanatogenesis in Erdheim's idiopathic cystic medial necrosis and which allows ICD-10 diagnosis coding. CONCLUSION: The main histopathological signs of aortic aneurysm in Erdheim's idiopathic cystic medial necrosis were presented; structural changes in the primary components of the aortic wall were characterized; the cells of an inflammatory infiltrate were investigated; and the formulation of autopsy and forensic medical diagnoses was proposed.

[Von Hippel-Lindau disease type 2-related pancreatic neuroendocrine tumor and adrenal myelolipoma].

The paper describes a case of von Hippel--Lindau-related pancreatic neuroendocrine tumor and adrenal myelolipoma in a 44-year-old woman. The pancreatic tumor and a left retroperitoneal mass were removed in the women in July 2014 and May 2015. Histological examination of the pancreatic tumor revealed that the latter consisted of clear cells forming tubular and tubercular structures showing the expression of chromogranin A, synaptophysin, and cytokeratins 18 and 19 and a negative response to CD10 and RCC. The adrenal medullary mass presented as clear-cell alveolar structures with inclusions of adipose tissue mixed with erythroid, myeloid, and lymphoid cells. The clear-cell component of the adrenal gland expressed neuroendocrine markers with a negative response to cytokeratins, CD10, and RCC. Molecular genetic examination yielded a signal corresponding to two copies of the VHL gene. No deletions or amplifications of the gene were detected. Cases of von Hippel--Lindau disease concurrent with adrenal pheochromocytoma and myelolipoma and simultaneous pancreatic involvement were not found in the literature.