Complete Response to Immune Checkpoint Inhibition in a Platinum Resistant Primary Ovarian Cancer Patient With Lynch Syndrome: A Case Report and Review of the Literature.

BACKGROUND/AIM: Lynch syndrome (LS) is the secondary cause of hereditary ovarian cancer (OC). Germline mutations in the DNA-mismatch repair (MMR) genes cause tumorigenesis and a high immunogenicity. Recent studies showed a promising use of immunotherapy in MMR deficient (MMRd) tumors. This is a case report of a patient with LS-associated OC and a complete response to pembrolizumab. CASE REPORT: A 44-year-old patient was admitted to the hospital with lower abdominal pain. The patient's history showed LS with a germline mutation in the MSH2-gene. Initial diagnostics showed a pelvic tumor mass and a highly elevated cancer antigen 125. After debulking surgery, histopathological findings showed a high-grade serous OC with mutations in the MSH2 and MSH6 genes. Only 5 weeks after operation with no residual tumor mass, a quick and significant intraabdominal progression of the disease was diagnosed. Adjuvant therapy with carboplatin and paclitaxel in a weekly course did not lead to sustainable response. An anti-PD-L1 antibody therapy with pembrolizumab was initiated. After only two courses of therapy, the laboratory results and clinical status of the patient improved tremendously. Shortly after, a complete response was detected, and therapy is still ongoing. The patient remains tumor free for 21 months now. CONCLUSION: The significance of germline compared to somatic mutations has not yet been sufficiently investigated. To our knowledge, this is the first case with complete response to checkpoint inhibition in OC associated with LS. Regarding LS-associated OC, immune checkpoint inhibition is an efficient therapy in tumors nonresponsive to standard therapy.

Spectral CT Hybrid Images in the Diagnostic Evaluation of Hypervascular Abdominal Tumors-Potential Advantages in Clinical Routine.

BACKGROUND: This study aimed to investigate the use of spectral computed tomography (SCT) hybrid images combining virtual monoenergetic images (VMIs) and iodine maps (IMs) as a potentially efficient search series for routine clinical imaging in patients with hypervascular abdominal tumors. METHODS: A total of 69 patients with hypervascular abdominal tumors including neuroendocrine neoplasms (NENs, n = 48), renal cell carcinoma (RCC, n = 10), and primary hepatocellular carcinoma (HCC, n = 11) were analyzed retrospectively. Two radiological readers (blinded to clinical data) read three CT image sets (1st a reference set with 70 keV; 2nd a 50:50 hybrid 140 keV/40 keV set; 3rd a 50:50 hybrid 140 keV/IM set). They assessed images subjectively by rating several parameters including image contrast, visibility of suspicious lesions, and diagnostic confidence on five-point Likert scales. In addition, reading time was estimated. RESULTS: Median subjective Likert scores were highest for the 1st set, except for image contrast, for which the 2nd set was rated highest. Scores for diagnostic confidence, artifacts, noise, and visibility of suspicious lesions or small structures were significantly higher for the 1st set than for the 2nd or 3rd set (p < 0.001). Regarding image contrast, the 2nd set was rated significantly higher than the 3rd set (p < 0.001), while the median did not differ significantly compared with the 1st set. Agreement between the two readers was high for all sets. Estimated potential reading time was the same for hybrid and reference sets. CONCLUSIONS: Hybrid images have the potential to efficiently exploit the additional information provided by SCT in patients with hypervascular abdominal tumors. However, the use of rigid weighting did not significantly improve diagnostic performance in this study.

Angiosarcomas of Primary Gynecologic Origin - A Case Series and Review of the Literature.

BACKGROUND/AIM: Angiosarcoma of primary gynecologic origin is an extremely rare and highly malignant tumor of endothelial origin with a 5-year survival rate of less than 35%. To date, only 61 cases have been described in the literature. The aim of this study was to present more cases and discuss potential therapy options. CASE REPORT: The following case series presents three cases of gynecologic angiosarcomas that were under therapy at the Charité - University medicine of Berlin from June 2014 to February 2018. RESULTS: Two of the cases deal with primary angiosarcomas of the uterus whereas the third case was diagnosed after the suspicion of a recurrence of a poorly differentiated squamous cell carcinoma of the cervix uteri. In case one a 75-year old patient with initial postmenopausal bleeding and a tumor mass of the uterus is described. After surgery a hemangiosarcoma of the uterus was confirmed. After two months the patient presented with a presacral peritoneal sarcomatosis. Chemotherapy of weekly paclitaxel was administered. Case two deals with a patient presenting with abdominal pain. A uterine sarcoma with infiltration of the parametry and angiosarcomatosis peritonei was diagnosed during an emergency laparotomy because of spontaneous peritoneal bleeding. Moreover, osseous metastasis was found. The patient underwent weekly paclitaxel. Due to tumor progression, chemotherapy was changed to doxorubicin and olaratumab and radiotherapy was induced. The patient died 33 months after initial diagnosis. Case three describes a 34-year old patient with suspected local recurrence of cervical cancer with infiltration of the bladder. During TURB an angiosarcoma was found. Following laparoscopy revealed peritoneal metastasis. The patient underwent weekly paclitaxel followed by a paclitaxel and pazopanib maintainance therapy which showed a regression. Due to progression afterwards, chemotherapy was changed to gemcitabine and docetaxel and gemcitabine monotherapy. The patient died 33 months after initial diagnosis. CONCLUSION: Even though there is no evidence on standard treatment of this extremely rare and aggressive tumor entity of the female genital tract the patients showed the longest stability of disease during chemotherapy with weekly paclitaxel.