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This article provides a review of studies analyzing the etiology, diagnosis, and treatment of Peitz–Jeghers syndrome, as well as the risk of cancer of different locations in patients with this genetic disorder. We report a case of Peitz–Jeghers syndrome associated with breast cancer in young women in two generations (mother and daughter). Despite specific clinical manifestations (multiple hamartomatous polyps developing in childhood and ink-black mucous pigmentation in the woman and her immediate relatives), Peitz–Jeghers syndrome was diagnosed only after breast cancer development and consultation with oncologists. This confirms the need for informing both oncologists and general practitioners about this genetic disorder.

The retrospective study of clinical data, risk factors, and results of treatment of a large group of patients with primary systemic anaplastic large-cell lymphoma (ALCL) (n = 42) with the Т-/О phenotype is presented. Primary systemic ALCL occurred in young persons aged less than 30 years. A group of patients with its poor course (with complete remission (CR) being achieved) showed a trend for the prevalence of the signs that were a part of the standard (International Prognostic Index — IPI) factors and ones of poor prognosis, which were additionally analyzed by the authors. Significant differences in a group of patients with a good prognosis (with CR being achieved) were obtained only in the following indices: ALK protein expression (substantiating the further division and study of a homogenous group of patients), the stage of the disease, evaluation of the patients’ general condition by the ECOG scale, and the presence of B-symptoms.According to the data available in the literature, ALK-positive ALCL has a better prognosis than ALK-negative ALCL. The results given in this communication additionally confirm and extend these observations. There is evidence that, by using the IPI and the baseline prevalence of the disease, one may predict an outcome in the homogenous group of patients without CR being achieved after first-line therapy. Analysis of the findings has established no clinical risk factors associated with the baseline site of a tumor process, which influence long-term results, but this matter should be studied in further prospective investigations, by keeping in mind the location zones characteristic of ALCL.

In given article non-Hodgkin lymphomas epidemiology, main attempts of gastrointestinal tract (GIT) lymphomas sistematization, basic methods of stomach NHL diagnostics, such as radiologic and endoscopic are described. Also importance of modern endoscopic diagnostics methods is discussed: chromogastroscopy, magnifying and narrow spectral endoscopy. Morphological confirmation problems of neoplasm histologic structure using forceps biopsies during endoscopic examination are noted; possibilities of expanded procedures taking a sample, such as endoscopic resection mucous and submucosal layers are described. Possibilities and importance of endosonography both in diagnostics and in differentiation of NHL and a various malignant and non-malignant stomach pathology are in detail described.

Background. The role of neoadjuvant chemotherapy (NACT) in luminal HER2-negative breast cancer (BC) remains highly controversial due to the lack of reliable predictors of drug therapy efficacy.Objective: to evaluate the effectiveness of NACT in patients with aggressive luminal HER2-negative BC and to compare modern systems for assessing the pathomorphological response.Materials and methods. The tumor response to NACT regimens was assessed in 64 patients with aggressive luminal HER2-negative BC stage II–III. The median age of women was 46.5 years (range 31–76 years), 76.6 % had primary operable stages (cT1–3N0–1), locally advanced BC (cT4, cN2–3) – 23.4 % patients. The characteristics of BC were as follows: invasive ductal carcinoma (76.6 %), grade G2 and G3–54.7 % and 45.3 %, Ki-67 ranged from 20 % to 98 %, median 45 %. The ER expression level was low (1–10 %, ERlow) in 12.5 % and was more than 10 % in 87.5 % of cases. HER2 status corresponded to 0, 1+ and 2+ in the absence of gene amplification – in 50.0 %, 35.9 % and 14.1 % of patients, respectively. The rate of TILs 20 % was in 71.4 %, 10.7 % and 17.9 % of cases. After NACT with the inclusion of anthracyclines and taxanes ± platinum combinations (in BRCA mutated status), the patients underwent radical surgery (mastectomy or breast-conserving surgery) with an assessment of the pathological response.Results. 15.6 % of patients had a complete pathomorphological response (pCR) to treatment, which corresponded to the RCB-0 class and the pathomorphological stage ypT0N0. Residual tumor load with incomplete response was very significant – class RCB-I was noted in only 7.8 %, and RCB-II and RCB-III – in 39.1 % and 37.5 %, respectively. An increase in the size of the residual tumor and the number of affected lymph nodes were associated with an increase in the RCB class. Predictors of pCR achievement in luminal HER2-negative cancer were: grade G3, rare histological forms of BC (medullary, metaplastic), rate of TILs ≥30 %, low ER expression, and HER2 0 status.Conclusion. Assessment of Ki-67, tumor grade, ER and HER2 rate, and TILs before starting NACT will help identify a group of high sensitivity to chemotherapy and optimize the treatment strategy in aggressive luminal HER2-negative BC.

HER2-positive breast cancer is a unique subtype of the disease, not only in terms of aggressive biology, but also in terms of treatment options. Over the past 15 years, the strategy for treating early HER2-positive breast cancer has undergone a real evolution – from the absence of anti-HER2 therapy to the sequential introduction of adjuvant, neoadjuvant and post neoadjuvant approaches. This review describes key studies of systemic therapy for HER2-positive breast cancer stage I–III, which made it possible to establish clear priorities in the sequence of surgical and systemic steps, identify high risk groups which need of escalation of treatment, and determine the optimal anti-HER2 therapy for each steps, as well as de-escalation of stage I treatment without losing its effectiveness. The news from the latest cancer conferences (SABCS, ESMO, ASCO) on the impact of various biological markers on the effectiveness of anti-HER2 agents is presented. A clear concept of modern treatment of early HER2-positive breast cancer has been formed, allowing individualized approaches, and achieving better results of the therapy this aggressive biological subtype.

The objective: evaluation of effectiveness of the first-line therapy with rituximab of B-cell lymphoproliferative diseases in Russian clinical practice in the period from 2014 to 2017.Materials and methods. The EQUILIBRIUM post-registration multicenter study included 1000 patients aged 21 to 91 years old with a verified diagnosis of B-cell non-Hodgkin’s lymphoma, or chronic lymphocytic leukemia, who received at least 4 cycles of rituximab-containing therapy with Acellbia®. The group of aggressive non-Hodgkin’s lymphomas (aNHL), which is the subject of this article, included 295 patients with a median age of 55.9 years: diffuse B-large cell lymphoma – 87 %, primary mediastinal lymphoma – 11 %, Burkitt’s lymphoma – 1 %. Group characterized by the presence of aggressive clinical signs reflecting the poor prognosis: in the majority of patients, generalized stages were diagnosed (61 %), in half of the cases (50.2 %), extranodal localization of tumor foci was detected (in 32.4 % of patients there were 2 or more). The overwhelming majority of patients (84.5 %) received adequate treatment complying with national and international recommendations (R-CHOP, R-CHOEP and R-EPOCH, high-intensity NHL-BFM-R, R-HyperCVAD and R-MACOP-B regimes). The use of R-CVP, FCR, RB, Chl-R, R-monotherapy treatment programs (which received 15.5 % of patients) was considered inadequate for this category of patients.Results. According to the results of the final assessment, high therapy efficacy was established: the overall response exceeded 90 %, complete remission was achieved in most patients with aNHL (68.5 %), partial remission – in every 5th patient (21.8 %). With a median follow-up of 15 months, 16 (5.42 %) deaths and 34 (11.53 %) events were registered. Median of event-free survival and overall survival have not been achieved. Statistically significant differences depending on first-line therapy efficacy were found in overall survival (p = 0.00000) and eventfree survival (p = 0.00000), once again confirming that the main goal of aNHL treatment is to achieve complete remission.Conclusion. Available and compliant with national clinical guidelines treatment of aNHL patients with Russian bioanalogue of anti-CD20 monoclonal antibodies (Acellbia®) demonstrates high immediate efficacy and acceptable long-term results, comparable to a retrospective analysis of previous clinical studies of the original drug rituximab.

Much attention has been recently given to the prognosis of extranodal non-Hodgkin’s lymphomas (NHL). All currently available prognostic scales fail to fully characterize the prognosis of primary NHL of the eye and its accessory apparatus.The purpose of this study was to identify prognostic factors for vision and life in patients with primary NHL of the organ of vision. Retrospective and prospective studies of the specific features of the disease in 94 patients with primary NHL of the organ of vision revealed some factors influencing its prognosis.

Non-Hodgkin's lymphomas are extremely rare among all tumors of female reproductive system. Diagnostic mistakes and inadequate therapeu- tic tactics in these diseases are results of usual absence of alertness of gynecologists. The aims are to analyze reasons of diagnostic mistakes in patients with non-Hodgkin's lymphomas of female reproductive system and to discover definitive clinical and morphological characteristics of female reproductive system lymphoid tumors. During the period between 1989 and 2006, 305 cases of primary extranodal non-Hodgkin's lym- phomas were detected; female reproductive system was affected in 7% of patients (totally 40 patients), which were included in investigated group. In the whole analyzed group of women (n=40, median age 43 yrs, range 17-84 yrs), patients with primary lesion of female reproductive system had median age of 40 yrs and with secondary involvement - 46 yrs. Most of patients were fertile (60%, n=24). Such tumors was localized in breast in 40% of cases (n=16), in ovaries - 20% (n=8), in uterine corpus - 12,5% (n=5), in uterine cervix - 15% (n=6), and in vagina - remaining 12,5% (n=5). Average time from diagnosis to beginning of the treatment was 7,5 months. As a result, the onset of specific therapy was delayed in 65% cases (n=26) and 50% (n=20) underwent unneeded surgery. Diagnostic mistakes lead to inadequate treatment. Extranodal non-Hodgkin’s lymphomas of female reproductive system, both primary and secondary, are rare pathology. Primary lesion is more typical for older women, sec- ondary is mainly affecting younger women (in reproductive period). Chemotherapy response and prognosis are better in primary cases.

The purpose of the study was to define the factors influencing the development of a recurrence depending on the volume of surgical intervention and to investigate the possibility of performing organ-saving surgery (OSS) for centric breast cancer.The study used the clinical findings of 200 patients treated at the Department of Radiosurgery, N.N. Blokhin Russian Cancer Research Center in 1996 to 2006. According to the volume of surgical intervention, the patients were divided into 2 groups: 1) 106 patients who had undergone radical mastectomy; 2) 94 patients who had undergone radical resection. According to our data, the disease progressed in 32 (16%) cases. There were 12 local recurrences: in 8 (7.5%) and 4 (4.2%) cases after radical mastectomy and radical resection, respec- tively. The findings suggest that OSS may be performed after carefully patient selection, with all contraindications being kept in mind.

The BCIRG 001 study has shown that a combination of Taxotere, doxorubicin, and cyclophosphomide (TAC regimen) is more effec- tive than the standard FAC (5-fluorouracil, doxorubicin, cyclophosphomide) regimen in adjuvant chemotherapy of operable breast cancer (BC) with mestastases to regional lymph nodes. With higher efficacy, TAC regimen was more toxic: the incidence of febrile neu- tropenia was 24.7%. According to the current EORTC and ASCO guidelines, the use of granulocyte colony-stimulating factors (G-CSF) for primary prophylaxis is indicated when the risk of febrile neutropenia is ≥20%. This study was designed to evaluate the safety of TAC regimen with G-CSF primary prophylaxis in adjuvant chemotherapy (CT) of BC .Patients with operable BC (T1—3N1M0) and Karnofsky performance status 80% received adjuvant TAC regimen after radical surgery: Taxotere 75 mg/m2, doxorubicin 50 mg/m2, and cyclophosphomide 500 mg/m2. G-CSFs were administered in the standard doses for 5—7 days starting from cycle 1 of CT, for primary prophylaxis of febrile neutropenia.One hundred and one patients have been treated since 2006. Mean age was 47.5 years (range 25—67 years). Almost half (48.5%) of the patients had Stage IIIa disease, Stages IIa and IIb had 18.8 and 27.7% of patients, respectively.The tumor was estrogen receptor-positive in 57.4% of the patients and progesterone receptor-positive in 62.4%. Overexpression of Her- 2/neu receptor was documented in 49.5% of cases. The mean number of cycles per patient was 5.8. Ninety-one (90.1%) patients have received a complete course of 6 TAC cycles. The duration of prophylactic use of lenograstim or filgrastim during one CT cycle was 5.9 and 5.6 days, respectively.Episodes of febrile neutropenia were observed at 19 (3.2%) CT cycles in 9 (8.9%) patients. Neutropenic infections were recorded at 4 (0.78%) cycles in 3 (3%) patients.Thus, the use of GCSF substantially reduces the incidence of TAC-associated febrile neutropenia and infectious complications and ensures a safe and complete course of effective adjuvant CT for the vast majority (90.1%) of patients. The data presented suggest that there is a need for primary prophylaxis with G-CSF in all BC patients receiving adjuvant TAC.

The paper gives the results of studying the genes of epidermal growth factor receptor (EGFR) and topoisomerase IIα (TOP IIα) in patients with triple-negative breast cancer (BC). It is suggested that the lack of EGFR gene amplification might serve as a factor of good prognosis. TOP IIα gene amplification usually occurs in the tumors responsive to chemotherapy including anthracycline drugs. The altered status of the above genes should be interpreted in the context of aneusomy of chromosomes 7 and 17, on which they are located. No association was found between the pattern of EGFR and TOP IIα gene abnormalities and the basaloid phenotype of BC.

In the present review has described the historical stages of systemic therapy of breast cancer with the use of taxanes, and has given a detailed description of drugs (paclitaxel and docetaxel). The role of docetaxel (Taxotere) in reducing the recurrences and death from cancer risk has shown. The major randomized trials has described; was studied the effectiveness of combination with docetaxel (Taxotere) in the adjuvant treatment of early breast cancer with or without lymph nodes involvement. The important role of docetaxel (Taxotere) in the adjuvant treatment of HER2-positive breast cancer was shown.

The results of treatment were studied in 95 patients with local recurrences of uterine sarcomas, who had been treated at the N.N. Blokhin Russian Cancer Research Center in 1972 to 2010. Two patient groups were comparatively analyzed after surgical and conservative (chemo- and radiotherapy) treatments. Overall survival was found to be significantly higher in the group of patients who had undergone surgical treatment.

Clinical, morphologic and immunohistochemical data of 51 patients with stage T2N1-3M0 breast cancer treated in the N.N. Blokhin Russian Cancer Research Center of Russian Academy of Medical Sciences in 2002 was analyzed. Expression of Pgp 170 was found in 19 patients. Relationship between immunophenotype of stage T2N1-3M0 breast cancer cells and main clinical and morphologic fea- tures, treatment provided and expression of multiple drug resistance gene MDR1.

Taking into account the fact that the treatment of advanced ovarian cancer includes cytoreductive surgery and chemotherapy, it is important to take advantage of contemporary highly effective agents. In 2003 the Regional program for optimization of ovarian cancer treatment in Cancer centers of Russia was initiated: 100 patients from 22 Cancer dispensaries received combination chemotherapy with Taxol at the dose of 175 mg/m2 as 3 hours intravenous infusion, then Carboplatin at the dose to obtain AUC=5,0 (TCb scheme). As a result of this multi-center study, the effectiveness of TCb scheme as first line chemotherapy was confirmed. The scheme can be successfully implemented in Cancer Centers.