Although rare, intrahepatic cholangiocarcinoma (ICC) is the second most common primary hepatic malignancy and the incidence of ICC has increased 14% per year in recent decades. Treatment of ICC remains difficult as most people present with advanced disease not amenable to curative-intent surgical resection. Even among patients with operable disease, margin-negative surgical resection can be difficult to achieve and the incidence of recurrence remains high. As such, there has been considerable interest in systemic chemotherapy and targeted therapy for ICC. Over the last decade, the understanding of the molecular and genetic foundations of ICC has reshaped treatment approaches and strategies. Next-generation sequencing has revealed that most ICC tumors have at least one targetable mutation. These advancements have led to multiple clinical trials to examine the safety and efficacy of novel therapeutics that target tumor-specific molecular and genetic aberrations. While these advancements have demonstrated survival benefit in early phase clinical trials, continued investigation in randomized larger-scale trials is needed to further define the potential clinical impact of such therapy.

Background The impact of tumor burden score (TBS) on conditional survival (CS) among patients undergoing curative-intent resection of hepatocellular carcinoma (HCC) has not been examined to date. Methods Patients who underwent liver resection of HCC between 2000 and 2017 were identified from a multiinstitutional database. The impact of TBS and other clinicopathologic factors on 3-year conditional survival (CS 3 ) was examined. Results Among 1,040 patients, 263 (25.3%) patients had low TBS, 668 (64.2%) had medium TBS and 109 (10.5%) had high TBS. TBS was strongly associated with OS; 5-year OS was 39.0% among patients with high TBS compared with 61.1% and 79.4% among patients with medium and low TBS, respectively (p \ 0.001). While actuarial survival decreased as time elapsed from resection, CS increased over time irrespective of TBS. The largest differences between 3-year actuarial survival and CS 3 were noted among patients with high TBS (5-years postoperatively; CS 3 : 78.7% vs. 3-year actuarial survival: 30.7%). The effect of adverse clinicopathologic factors including high TBS, poor/undifferentiated tumor grade, microvascular invasion, liver capsule involvement, and positive margins on prognosis decreased over time. Conclusions CS rates among patients who underwent resection for HCC increased as patients survived additional years, irrespective of TBS. CS estimates can be used to provide important dynamic information relative to the changing survival probability after resection of HCC.

Background: Renal cell carcinoma (RCC) commonly metastasizes, and unusual sites of spread are characteristic. However, metastatic RCC without an identified primary site is extremely rare, and only a few cases have been documented. We report the first case of clear cell RCC identified in retroperitoneal, supraclavicular, and mediastinal lymph nodes (LN) without a primary site in a kidney transplant patient.Case report: A 68-year-old patient presented with abdominal pain accompanied by weight loss, decreased appetite, and night sweats 6 months post female living donor kidney transplant. A CT scan revealed retroperitoneal, supraclavicular, and mediastinal lymphadenopathy. An excisional supraclavicular LN biopsy stained for markers consistent with RCC (pancytokertain, PAX8, CD10, and vimentin). Subsequent karyotyping revealed a female aneuploid cell line and raised suspicion for a donor derived RCC. A primary site for the RCC could not be identified on imaging of the native and allograft kidneys. The patient expired waiting for the pathology report, and an autopsy was performed to identify the origin of the RCC. Autopsy could not identify a primary site and cytologic studies indicated that the cell line was derived from the patient. Additional chromosomal abnormalities identified in the cytologic studies (loss of VHL, PBRM1, SETD2, BAP1, CDKN2A/B TP53) narrowed the diagnosis to clear cell RCC. Conclusion:Metastatic RCC can present without a primary site. IHC plays a vital role in making the diagnosis and cytologic studies can confirm the diagnosis and exclude the renal allograft as the origin in a kidney transplant patient.

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