Meigs syndrome.

BACKGROUND: Meigs syndrome is defined by the presence of a benign ovarian tumor, ascites, and pleural effusion (predominantly on the right side). A characteristic sign of Meigs syndrome is the complete disappearance of exudate after surgical resection of the ovarian tumor. CASE REPORT: We present a case report of a 58-year-old patient admitted for an advanced ovarian tumor with pleural effusion, ascites, and tumor marker elevation typical for ovarian cancer. Cytological examination of ascites and pleural effusion was repeatedly negative for malignancy. Histopathological examination of the bio-psied tissue was concluded as low-grade mesenchymal neoplasia. The second opinion of histopathological examination was concluded as low grade fibroblastic pelvic tumor without the possibility of exact specification. Dia-gnoses of desmoid fibromatosis and low-grade fibromyxiod sarcoma (less likely) were considered. Surgical resection was indicated, and a large tumor with numerous adhesions to the uterus, bladder, and thin loops with a noticeably thickened peritoneum were perioperatively described. Histologically, left ovarian fibroma with productive peritonitis and sanguine-induced ascites was dia-gnosed. Due to the clinical findings and the result of the histopathological examination, the case was classified as Meigs syndrome. Two months after the surgery, the ascites and pleural effusion disappeared, and the tumor marker levels normalized. CONCLUSION: The present case report documents that it is always necessary to consider diseases other than those most likely at the outset, as the treatment algorithm and prognosis of these rare diseases may differ significantly.

Uncommon EGFR Mutations in Non-Small Cell Lung Cancer and Their Impact on the Treatment.

BACKGROUND: Epidermal growth factor receptor (EGFR) mutations play an important role in the pathogenesis of non-small cell lung cancer. Because these alterations are so-called targetable mutations, their identification is important in daily clinical practice. The diagnostic standard of EGFR mutations is currently based on polymerase chain reaction methods, particularly the quantitative real-time polymerase chain reaction. In recent years, new generation sequencing has become increasingly important. In patients with EGFR mutations, a significant improvement in therapeutic outcomes was achieved with the administration of targeted therapy using tyrosine kinase inhibitors. EGFR is composed of four domains: extracellular with a ligand binding site, a transmembrane domain, a cytoplasmic tyrosine kinase catalytic domain, and a C-terminal domain. The key structures of the tyrosine kinase domain responsible for signal activation and transmission are encoded within exons 18-21 on chromosome 7. EGFR mutations are highly heterogeneous. About 90% of EGFR mutations are deletions of exon 19 and point mutation L858R in exon 21. These are referred to as classic mutations. Approximately 10% of the total number of EGFR mutations is attributable to less frequent alterations in the EGFR gene. Due to the low incidence of non-small cell lung cancer with less frequent EGFR mutations, information on their predictive significance is still incomplete. Most of the data for the treatment of cases with uncommon mutations were gathered from retrospective analyses and evaluations of small cohorts. PURPOSE: The aim of this review is to summarise the current options for diagnosing and treating non-small cell lung cancer patients with uncommon EGFR mutations. This work was supported by the MEYS - NPS I - LO1413 and MH CR - DRO (MMCI, 00209805). The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 2. 6. 2019 Accepted: 26. 8. 2019.

[Reactive Lymphoid Hyperplasia of the Liver]. / Reaktivní lymfoidní hyperplazie jater.

CASE: Here, we present the case of a 50-year-old woman diagnosed with stage I hormone-dependent breast cancer. The patient underwent partial mastectomy followed by adjuvant radiotherapy and hormone treatment with tamoxifen. Three years later, she presented with a solitary liver lesion on MRI, which was highly suspicious of malignancy. However, several fine needle biopsies were performed, and histopathological examination revealed no signs of neoplasia. As a result of these alarming discrepancies, the multidisciplinary board recommended a diag-nostic laparotomy, which yielded a finding consistent with reactive lymphoid hyperplasia, a pseudolymphoma of the liver, on a background of incipient steatohepatitis. This rare condition is characterized by proliferation of non-neoplastic lymphocytes in extranodular sites, and is usually an incidental finding on imaging modalities in clinically asymptomatic patients, predominantly women. Lesions share some radiologic features with primary malignant liver diseases such as hepatocellular carcinoma or cholangiocarcinoma. Although the etiology remains unclear, reactive lymphoid hyperplasia is believed to be associated with some malignancies, including breast cancer, or inflammatory and autoimmune disorders. Reactive lymphoid hyperplasia usually progresses slowly, with some cases of spontaneous regression described in the literature. To the best of our knowledge, only 50 cases of hepatic reactive lymphoid hyperplasia have been reported so far.Key words: pseudolymphoma - hyperplasia - liver - lymphatic tissue The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.Submitted: 20. 3. 2017Accepted: 10. 4. 2017.

[Extraoseus Ewings Sarcoma, Primary Affection of Uterine Cervix--Case Report]. / Extraoseální Ewinguv sarkom, primární postizení delozního cípku--kazuistika.

BACKGROUND: Ewing's sarcoma is usually diagnosed in adolescents and young adults, peak of incidence is around 15 years of age. Primary localization is mostly in the skeleton of long bones and chest wall. Primary extraosseous involvement rarely occurs, incidence increases with age. CASE: We present a case report of a 57-year-old patient with locally advanced tumors of the cervix, clinical stage IIB. Due to histological and molecular genetic examination revealing EWS -ERG fusion gene, Ewing's sarcoma was diagnosed. CT revealed pathological pelvic lymphadenopathy and multiple pulmonary bilateral methastases, scintigraphy did not prove any affection of skeleton. The patient underwent a two-stage intensive chemotherapy regimens VIDE (vincristine, ifosfamide, doxorubicin, etoposide) and VAI (vincristine, actinomycin D, ifosfamide). During the second phase, concomitant radiotherapy of pelvis was aplied. According to PET/CT, complete remission was achieved. Whole-lung irradiation was applied in consolidation of the result. CONCLUSION: Primary Ewing's sarcoma of the cervix is an extremely rare disease. To our knowledge, only 12 cases was presented until this time. The average age at time of dia-gnosis was 35 years. Unlike the previous reports, we initially diagnosed distant metastases. The treatment was led according to the protocol Ewing 2008 designed for primary skeletal Ewing's sarcoma. Currently, 18 months after the therapy, the patient is without signs of disease. However, long-term follow-up is necessary.

[Trabectedin registry]. / Lékový registr--trabectedin.

BACKGROUNDS: The Yondelis (trabectedin) project was initiated in January 2011 with the aim to obtain basic epidemiological information on patients with soft tissue sarcomas, standard treatment procedures, and results of trabectedin therapy in routine clinical practice. Expert patronage is provided by the Czech Society for Oncology, CzMA JEP. The project covers a representative sample of Comprehensive Cancer Care centres established to provide systematic treatment to patients with soft tissue sarcoma. PATIENTS AND METHODS: 45 patients diagnosed with soft tissue sarcoma were retrospectively included in the database. Median age at the initiation of trabectedin therapy was 51 years (23-72 years). Leiomyosarcoma was the most frequent tumour (35.6%), synovial sarcoma occurred in 13.3% of patients; liposarcoma, peripheral nerve sheath tumours and unspecified sarcomas contributed 6.7% each. 62.2% of sarcomas were larger than 5 cm. Trabectedin was administered in a dose of 1.5 mg/m2 once in 3 weeks. 40% of patients received trabectedin as the 2nd line treatment, 35.6% as the 3rd line, and 34.4% as the 4th line. RESULTS: Median number of administered cycles was 4 (1-10 cycles). Neutropenia (28.9% of patients) and elevated liver enzymes (26.7% of patients) were the most frequent adverse affects. 73.8% of patients terminated the therapy due to disease progression. Treatment response was recorded in 6.6% of patients (complete and partial remission), stable disease in 26.7%, and progression in 53.3%. Median overall survival (95% CI) was 11.7 months (9.6; 13.8), median progression-free survival (95% CI) was 3 months (2.4; 3.6). CONCLUSION: Expert cancer societies have recently recommended trabectedin as the 2nd line palliative treatment for soft tissue sarcomas with documented effectiveness, particularly in liposarcomas and leiomyosarcomas, and good safety profile.