Targeted therapy in Xp11 translocation renal cell carcinoma.

BACKGROUND: Translocation renal cell carcinoma (TRCC) is a rare form of RCC affecting mostly children and young adults with the occurrence of only 1-5% of all renal cell carcinomas. These carcinomas are associated with different translocations on a short arm of chromosome X in the region 11.2, which results in genetic modification of the p arm containing the transcription factor E3 gene. METHODS: Herein we report a case of a patient who was dia-gnosed with TRCC with c-Met overexpression and was treated with multiple targeted therapy agents and immunotherapy. CASE: A 28-year old woman without a significant past medical history underwent left sided total nephrectomy for TRCC. Seven months later, she developed systemic relapse and was treated with multiple lines of targeted therapy including sunitinib, everolimus, sorafenib, crizotinib, and pazopanib as well as with anti-PD-L1 antibody nivolumab, with stable disease as a best response. The most pronounced disease stabilization was achieved with sorafenib, which lasted 18 months. The patient died 81 months after initial dia-gnosis and 74 months from the dia-gnosis of metastatic disease. CONCLUSION: Improved survival observed in our patient could be related to the effectivity of tyrosine-kinase inhibitors, but notm-TOR inhibitors, even though disease stabilisation was observed as a best response. Identification of new treatment targets are warranted in this rare disease.

Undifferentiated Carcinoma of the Pancreas - a Case Report.

BACKGROUND: Undifferentiated carcinoma of pancreas (pancreatic cancer - PC) is a rare subtype of malignant PC. It was thought to be a sarcoma of the pancreas due to its typical morphological pattern. There are three histomorphological variants: anaplastic, sarcomatoid, and carcinosarcoma. There is also a separate category: undifferentiated pancreatic carcinoma with osteoclast-like giant cells. In contrast to ductal adenocarcinoma of the pancreas, undifferentiated carcinoma of pancreas is characterized by more aggressive behavior, other predilection localization of tumor and different predilection for localization of tumor and metastasis, a larger primary tumor, and different symptomatology at the time of diagnosis. CASE: We present the case of a patient who was diagnosed with undifferentiated PC and was treated at the National Cancer Institute in Bratislava. We provide information about the clinical, radiological, and histomorphological characteristics of the disease, along with the diagnostic and therapeutic approach and a brief review of the literature. RESULTS: The patient was diagnosed with inoperable locally advanced disease and was treated with first line chemotherapy comprising gemcitabine and cisplatin, followed by second line treatment with FOLFIRINOX. No response was achieved; on the contrary, we observed progression of the disease and deterioration in the patients condition. Overall survival was 4.5 months from the time of diagnosis. CONCLUSION: The only appropriate therapeutic approach to this highly malignant disease is most likely “en-bloc” resection, which is possible only at the early stage of the disease. At present, no curative chemotherapy or radiotherapy regimen exists. The dominant features of undifferentiated PC described in the literature are aggressive behavior, an unfavorable prognosis, and chemo-refractoriness. Key words: pancreatic cancer - prognosis - chemotherapy 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: 4. 6. 2018 Accepted: 25. 10. 2018.