Patients with neuregulin 1 (NRG1) rearranged cancer are suitable for the theranostic approach and targeted therapy.

INTRODUCTION: Neuregulin 1 (NRG1) gene fusion was detected in a wide range of carcinomas. It is most frequently present in lung adenocarcinomas, especially in KRAS and BRAF wild-type cases. PURPOSE: We present a newly described diverse group of NRG1 rearranged carcinomas. The paper explains basic molecular principles associated with this oncogenic driver. It consists of ERBB3 (HER3) and ERBB2 (HER2) receptor activation with downstream activation of PIK and MAPK canonical pathways. The experience with new therapeutic modalities is summarized. CONCLUSIONS: So far, the global results of cytotoxic, immune and targeted therapies were dis-appointing. Further research (including two studies in Europe) is underway, developing new therapeutic strategies and examining this cancer bio-logy. In the meantime, it is possible to dia-gnose NRG1 rearranged carcinomas in the Czech Republic since mRNA next generation sequencing (NGS) analysis is readily available.

[Neoadjuvant Use of Isolated Limb Perfusion in Large Myxoid Liposarcoma of the Thigh: a Case Report]. / Neoadjuvantní uzití izolované perfuze koncetiny u objemného myxoidního liposarkomu stehna: kazuistika.

Large unresectable STS presents a therapeutic challenge. Several options are being explored to avoid amputation without compromising the oncological outcome. Neoadjuvant chemotherapy delivers inconsistent and rather unsatisfactory results, preoperative radiotherapy compromises healing, hence it can impede adjuvant systemic treatment. We present a case report of neoadjuvant use of isolated limb perfusion with TNF-alfa and Alkeran (Melphalan) in a patient with initially unresectable large myxoid liposarcoma of the thigh. We achieved 55% reduction in size of the tumor that allowed for wide resection with a safe margin. Pathology confirmed 99% tumor necrosis. The patient has a full function of his extremity and is disease-free at one year follow-up. ILP should be considered as a treatment option which, in selected cases, can contribute to limb sparing surgery. Key words: sarcoma, soft tissue, regional perfusion, chemotherapy, surgery, orthopedic, limb salvage.

[Bilateral Congenital Multiple Pigmented Vitreous Cysts in a Three-year-old Girl: Ten Years Follow up]. / Bilaterální kongenitální mnohocetné pigmentované vitreální cysty u tríleté dívky - deset let sledování.

The authors present a case report of a three-year-old female patient with bilateral multiple anterior vitreous cysts. During examination for intermittent esotropia of the right eye was diagnosed not only hypermetropia, astigmatism and anisometropia, but also pigmented changes in peripheral retrolental space of both eyes. Clinical examination under general anaesthesia revealed bilateral multiple pigmented immobile vitreous cysts. There were five almost spherical, translucent, but slightly pigmented cysts on its cover on the right eye and four similar on the left, but visible only with dilated pupils. A dilating of pupils was slow and required more mydriatics than in similar aged children. Follow up period is ten years now. Occlusion therapy of amblyopia was performed to nine years of age. Treatment of refractive error and esotropia with correction for hypermetropia, astigmatism and anisometropia continues. Best corrected visual acuity in thirteen-year-old girl is 1,0 in both eyes without any visual disturbances described by patient. Corrected visual acuity in each eye is 1,0, right eye with +3,5 D sph., -3,5D cyl., axis 175°, left eye with +7,5 D sph., -3,0 D cyl., axis 35°. Patient is otherwise healthy and without any mental deficit. Position of all cysts remains unchanged and stabile during the follow up period (with recommendation to avoid hits to the head for all time, mainly in sports). Formation and slow progression of partial cortical cataract in the area of contact of the lens and one cyst in inferonasal quadrant of the lens on the right eye is monitored. A lamellar retinal extrafoveal defect of posterior pole of the right eye was found by OCT imaging.Key words: vitreous cyst, idiopathic, congenital, bilateral, multiple, pigmented, immobile, hypermetropia, astigmatism, anisometropia, esotropia, cortical cataract, retinal lamellar extrafoveal defect, OCT.

[Current classification of malignant tumours in gynecological oncology--part II]. / Soucasná klasifikace zhoubných nádoru v onkogynekologii--cást II.

OBJECTIVE: Review of new staging systems for gynaecological cancers and their impact on prognosis and planning treatment. DESIGN: Review article. SETTING: Department of Gynaecology and Obstetrics, First Faculty of Medicine and University Hospital Na Bulovce, Charles University, Prague; Department of Radiotherapeutic Oncology, First Faculty of Medicine and University Hospital Na Bulovce, Charles University, Prague; Department of Pathology, University Hospital Na Bulovce, Prague. RESULTS: Every staging system should have 3 basic characteristics: it must be valid, reliable, and practical. Over the years, these staging classifications--with the exception of cervical cancer and gestational trophoblastic neoplasia--have shifted from a clinical to a surgical-pathological basis. Changes based on new findings were proposed in 2008 by the FIGO Committee on Gynecologic Oncology, approved in September 2008 by the FIGO Executive Board, and published in 2009. The greatest changes were made in the new staging system for carcinoma of the vulva and others in the new staging systems for carcinoma of the cervix and carcinoma of the endometrium. A new stanging system was also created for uterina sarcomas, based on the criteria used in other soft tissue sarcomas. A clinical staging system for carcinoma of cervix continues because surgical staging cannot be employed worldwide (especially in third world countries). Stage 0 has been deleted from the staging of all tumours, since it is pre-invasive lesion and it is not an invasive tumour. In the revised staging system for carcinoma of the endometrium, four fundamental changes have occurred, which will be discussed. Carcinosarcoma is still staged identically to carcinoma of the endometrium. A completely new staging system was created for adenosarcomas, along with an almost identical staging system for leiomyosarcoma and endometrial stromal sarcoma. The staging system for carcinoma of ovary and Fallopian tube remains without changes. CONCLUSION: Since medical research and practice in the field of oncology have shown explosive growth, the staging of some of the gynaecological cancers did not give a good spread of prognostic groupings. Therefore, revised FIGO and TNM staging system has been structured to represent major prognostic factors in predicting patients' outcomes and lending order to the complex dynamic behavior of gynaecological cancers. The purpose of good staging system is to offer a classification of the extent of gynaecological cancer in order to provide a method of conveying one's clinical experience to others for the comparison of treatment methods.

[Current classification of malignant tumours in gynecological oncology--part I]. / Soucasná klasifikace zhoubných nádoru v onkogynekologii--cást I.

OBJECTIVE: Review of new staging systems for gynaecological cancers and their impact on prognosis and planning treatment. DESIGN: Review article. SETTING: Department of Gynaecology and Obstetrics, First Faculty of Medicine and University Hospital Na Bulovce, Charles University, Prague. Department of Radiotherapeutic Oncology, First Faculty of Medicine and University Hospital Na Bulovce, Charles University, Prague. Department of Pathology, University Hospital Na Bulovce, Prague. RESULTS: The main objectives of any good staging system--essential to an evidence-based approach to cancer--include planning treatment, providing an assessement of prognosis and the evaluation of the results of treatment. With this approach, the exchange of relevant information between oncological centers is facilitated, thus disseminating knowledge and stimulating research in other parts of the world. A good staging system must have three basic characteristics: validity, reliability, and practicality. The first staging system for gynaecological cancers appeared around the turn of the 20th century and was applied to carcinoma of the cervix uteri. Classifications for the other gynaecological malignancies were not created until the 1950s. Over the years, these staging classifications--with the exception of cervical cancer and gestational trophoblastic neoplasia--have shifted from a clinical to a surgical-pathological basis. Some changes, brought about through new findings, were approved by the FIGO in 2008 and published in 2009. The greatest changes were made in the new staging system for carcinoma of the vulva, while others were made in the new staging systems for carcinoma of the cervix and carcinoma of the endometrium. A new stanging system was also created for uterine sarcomas, based on the criteria used in other soft tissue sarcomas. CONCLUSION: As a result of the explosion of medical research in the field of oncology, the staging of some of the gynaecological cancers became outdated and did not give a good spread of prognostic groupings. Therefore, revised FIGO and TNM staging system has been structured to represent major prognostic factors in predicting patients' outcomes and lending order to the complex dynamic behavior of gynaecological cancers. The purpose of a good staging system is to offer a classification of the extent of gynaecological cancer, in order to provide a method of conveying one's clinical experience to others for the comparison of different treatment methods.

[Diagnostic pitfalls of HIV-associated Kaposi's sarcoma]. / Uskalí diagnostiky Kaposiho sarkomu sdruzeného s HIV infekcí.

Kaposi's sarcoma was one of the very first diseases which indicated the advent of the AIDS pandemic. Despite the marked fall in its occurrence thanks to the introduction of the cART, Kaposi's sarcoma remains the most frequent tumour in HIV-positive patients and still represents a major diagnostic and therapeutic problem. Particularly in the early stages both the macroscopic and histopathological picture of Kaposi's sarcoma may be very atypical, which can cause diagnostic difficulties right at the time when an early therapy may be most successful. In order to improve both the diagnostics and therapy of Kaposi's sarcoma, close collaboration between physicians taking care of HIV-positive patients--mainly infectologists, dermatologists and pathologists, is necessary.