Kidney cancer (KC) is a common disease characterized by extreme heterogeneity. There are nine known monogenic diseases associated with a significantly elevated KC risk: von Hippel-Lindau disease, MET-associated papillary renal cancer, familial multiple leiomyomatosis and renal cell cancer, SDHx-associated familial pheochromocytoma/ paraganglioma, Birt-Hogg-Dube syndrome, tuberous sclerosis, Cowden syndrome, BAP1- and MITF-associated melanoma-KC predisposition. These syndromes differ in the degree of cancer risk, the quantity, growth and progression rates of associated precancerous lesions, the morphology, and clinical presentations of malignancy itself, and in the response to therapy. Identification of causative germline lesion allows planning the surveillance of a mutation carrier, choosing the right time and extent of surgery, and optimizing treatment regimen. Hereditary KC research often brings forward novel approaches to the management of sporadic “phenocopies” of hereditary syndromes, i.e. sporadic cancers with somatic mutations in similar genes. The main directions for further study of genetic factors of KC are to find novel KC genes, to study risk modifiers in carriers of highly penetrant mutations, to clarify the involvement of hereditary nephropathies in the occurrence of renal cancers.

Ovarian cancer (OC) is a heterogenous disease in terms of genetic mutations and tumor phenotypes and can be divided into I and II types. Type II high grade tumors are more common, accompanied by ascites, and are the main cause of cancer-related death in women. OC associated ascites is considered as valuable source of tumor material containing a wide range of dissolved components and cell populations. Over the past decades, the cellular and acellular components of ascites have been studied, but its effect on chemoresistance and the development of metastasis continues to be studied. This review describes the pathogenesis of ascites in OC, it’s cellular and acellular components, many of which are prognostic factors as well as markers of the effectiveness of anticancer therapy. Further study of the ascitic fluid composition in OC will help to identify not only prognostic factors, but also the points of application of targeted drugs and will improve the results of OC treatment.

In case of locally advanced soft tissue sarcomas of the extremities (STS), the large size of which does not allow resection with preservation of the limb, isolated regional perfusion (IRP) of the limb is considered as one of the effective treatment options.

Aim. To evaluate the long-term outcomes of IRP with melphalan under conditions of hyperthermia for treatment of locally advanced STS of the extremities.

Materials and methods. We conducted a prospective cohort clinical study that included 42 patients with STS of the extremities. All patients underwent IRP with melphalan, in one case – in combination with tumor necrosis factor alpha. Additional lymph node dissection was performed in 8 cases (19%). The 5-year overall survival was estimated by the Kaplan-Meier method.

Results. The median age of the patients was 50 years (from 24 to 80 years), women predominated – 32 (76%). STS of the upper limb was diagnosed in 14 patients (33%), of the lower limb in 28 (67%). G3 grade was verified in 34 (81%) patients, in the rest – G2. Most patients had a recurrent tumor – in 34 (81%) cases. All patients received previous treatment: surgery in combination with chemotherapy and / or radiation therapy – 36 (86%), chemotherapy alone – 2 (5%), surgery alone – 4 (9%) patients. Within 5 years, tumor recurrence developed in 20 (48%) patients, amputation was performed in 10 (24%) patients, distant metastases were diagnosed in 4 (10%) patients. The mean time to relapse after IPC was 9.3 ± 3.5 months. Overall survival at 1, 2, 3, 4, and 5 years was 98%, 88%, 86%, 74%, and 67%, respectively.

Conclusion. IRP under conditions of hyperthermia with melphalan is an effective method for the treatment of STS of the extremities: the overall 5-year survival rate is 67% with a low rate of amputations and metastasis.

Aim. To study the effectiveness of modified subtotal laryngectomy with the formation of a tracheopharyngeal shunt in patients with locally advanced cancer of the larynx and laryngopharynx.

Materials and methods. We conducted a cohort study on the use of combined treatment (surgical and radiation/ polychemotherapy) in 25 patients: 24 men, 1 woman; median age – 56 years. The subtotal laryngectomy technique that we developed and used included the preservation of one arytenoid cartilage, the posterior third of the vocal fold, and the recurrent nerve on the side not affected by the tumor. We studied postoperative complications, the length and diameter of the tracheopharyngeal shunt, the functioning of the arytenoid cartilage, the frequency of voice recovery and its quality, and overall and relapse-free survival.

Results. In the postoperative period, 8 (32%) patients developed a pharyngocutaneous fistula; tracheal stenosis after tracheostomy was observed in 8 (32%) cases. The length of the tracheopharyngeal shunt varied from 16.4 to 25.6 mm, in all cases its course was tortuous, the diameter of the preserved part of the vocal fold varied from 1.1 to 1.5 mm. 24 (96%) patients demonstrated the functioning of the arytenoid cartilage and its work as a valve. Voice function recovered in 24 (96%) patients. Fundamental frequency was 155.3 ± 20.9 Hz, maximum phonation time of the vowel “A” – 3.5 ± 1.0 s, duration of pauses in phrase – 0.24 ± 0.03 s, speech rate – 99.8 ± 8.4 words/ min, number of words in phrase 6.5 ± 0.3. The average value of the Acoustic Voice Quality Index was 6.74, which indicates dysphonia (for the Russian language, the threshold value for dysphonia is 4.86), but this did not interfere with patients’ communication at home and on the phone. Overall and relapse-free survival at 1, 2, and 3 years were 100%, 89%, 78% and 86%, 78%, 72%, respectively.

Conclusion. The method of operation proposed by us contributes to the restoration of voice function in the majority of patients with oncological outcomes comparable to total laryngectomy.

In colorectal cancer (CRC) in addition to the most common mutations in the second exon of KRAS, there are rarer mutations that are not well understood as prognostic markers.

Description of cases. We describe two clinical cases of moderately differentiated adenocarcinoma (low-grade, G2), which manifested a complicated course in a 67-year-old woman – intestinal obstruction (case 1) and in a 62-year-old man – paratumorous abscess (case 2). Both cases were characterized by the development of regional and distant metastases (liver, brain) and a short relapse-free period. We found rare sporadic mutations of KRAS A146V and A59G with microsatellite stability and no mutation in the V600 BRAF site.

Discussion. In the presented case 1, the KRAS A146V mutation was detected, the frequency of its detection in our practice of testing 2103 cases of CRC was 0.48%. Case 2 shows the extremely rare A59G mutation. Among the patients with CRC we studied, we found mutations in codon 59 in only two, which amounted to 0.095%. Both rare mutations represent separate molecular subgroups of CRC, characterized by an unfavorable clinical outcome and requiring the development of special treatment regimens.

3D printing has opened new opportunities for the development of personalized systems for prosthetics of extensive chest wall defects after radical surgical interventions for malignant tumors. However, risk factors for an adverse outcome of such operations have not yet been identified.

Clinical cases. A 65-year-old man with primary chondrosarcoma of the V rib, underwent surgical resection of three ribs, plasty with local tissues, and an individual 3D printed titanium implant was installed on the sternum and IV-VI ribs. Follow up for 26 months showed no complaints or signs of recurrence. A 52-year-old woman with radiation-induced soft tissue sarcoma of the chest wall, that developed 9 years after radiation for breast cancer, underwent resection of four ribs and pectoral muscles and a 3D printed titanium implant was installed on the sternum and II-V ribs. The operation was complicated by the marginal necrosis of the soft tissue flap and infection of the endoprosthesis, which required removal of the metal structure and reconstruction using TRAM flap. After 9 months, a local recurrence of the tumor was diagnosed.

Discussion. As a factor of a positive outcome in a man should be noted a thick layer of subcutaneous fat with muscle tissue, due to which plastic surgery was performed, covering the defect without tension. An extensive resection of the pectoral muscles in a woman created a tissue deficit. Another factor of an unfavorable outcome can be considered the radio-induced nature of the sarcoma. Further research is needed to improve the strategy for selecting patients with malignant tumors of the chest wall for prosthetics.