[Gastroenteropancreatic neuroendocrine neoplasms-Heterogeneity, management and perspectives of treatment and research]. / Gastroenteropankreatische neuroendokrine Neoplasien ­ Heterogenität, Management und Perspektiven der Versorgung und Forschung.

The term neuroendocrine neoplasms (NEN) encompasses a molecularly and biologically very heterogeneous group of tumors, which have in common their origin in neuroendocrine cells. The also very heterogeneous subgroup of gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) is the best classified and investigated group. This article provides a systematic review of the current classification, diagnostics and treatment options of GEP-NEN. In order to achieve a better overview, it was consciously decided not to use an approach based on the primary localization. Instead, a thematic organization according to classification, clinical phenotype, diagnostics and treatment was chosen.

[Reports of prostate needle biopsies-what pathologists provide and urologists want]. / Befundbericht zu Prostatastanzbiopsien ­ was Pathologen liefern und Urologen wollen.

BACKGROUND: The prostate biopsy report is key for risk stratification of prostate cancer patients and subsequent therapeutic decision-making. However, due to the inclusion of a multitude of additional parameters its interpretation is becoming more challenging. OBJECTIVES: We aimed to determine how urologists currently interpret prostate biopsy reports, in particular how they consider different histopathological parameters for therapy decision-making. MATERIALS AND METHODS: A survey was sent to all urology practices in Germany with the help of the BDU (Berufsverband der Deutschen Urologen e. V.). In total, there were 106 complete responses that could be included for further analyses. RESULTS: Most urologists consider the number of positive cores and relative tumor burden (%) per core as crucial for the assessment of tumor extension. In case of targeted biopsies, the majority of urologists prefers a separate statement of positive cores per random biopsy scheme and per region of interest, respectively. The core with the highest Gleason score is mostly the basis for therapy decision-making (versus the overall Gleason score). Proportion of Gleason 4 pattern also seems to be critical for prostate cancer management. Only half of the urologists demand reporting of the new ISUP/WHO (International Society of Urological Pathology/World Health Organization) grade groups. Additional parameters claimed are Ki67, prostate-specific membrane antigen status, presence of intraductal or neuroendocrine component of the tumor. CONCLUSIONS: Our survey shows that there is no standardized reporting for prostate biopsies and that the interpretation of prostate biopsy reports varies among urologists. Further studies and guideline recommendations are necessary to establish a standardized reporting scheme for prostate biopsies.

[Tumor-induced osteomalacia caused by an FGF23-secreting myopericytoma : Case report and literature review]. / Tumorinduzierte Osteomalazie, verursacht durch ein FGF23-sezernierendes Myoperizytom : Literaturübersicht und Fallbericht.

BACKGROUND: The symptoms of muscle weakness, bone pain and fragility fractures can be an indication of osteomalacia. Phosphate is often not considered within osteologic parameters, decreased levels are therefore easily overseen. The additional test for fibroblast growth factor 23 (FGF23) as indicator for tumor-induced osteomalacia (TIO) is still largely unfamiliar. OBJECTIVE: By emphasizing the role of phosphate and furthermore FGF23 in bone metabolism illustrated by the long-term disease process of our clinical case we would like to introduce these parameters to a broader public. METHODS: We performed a literature search via PubMed and Google Scholar with the relevant key words and summarized the diagnostic and therapeutic information. The studies evaluated were mainly case reports. We present a case report of a 70-year-old patient with TIO and a myopericytoma and retrospectively analyzed the clinical case. The follow-up was 6 months. RESULTS: Our literature search found one case of TIO and evidence of FGF23 among 124 cases of myopericytomas in total. Over 300 cases of TIO are reported. In our case, we retrospectively found an FGF23-secreting myopericytoma in the phosphaturic mesenchymal tumors (PMT) group to be the cause of pseudarthrosis on the right humerus shaft and increasing disablement in a patient with osteomalacia. After surgical resection the patient was mobile again, and the osteologic parameters, especially phosphate, normalized from 0.21 to 1.52 mmol/l. CONCLUSION: Low phosphate levels are the decisive indication of TIO in our case. Therefore, we should always think of phosphate level control when dealing with osteomalacia. A hypophosphatemia and hyperphosphaturia should be recognized in time and be diagnostically verified. The additional FGF23 test (c-terminal and intact FGF23) should be considered.

Therapy susceptible germline-related BRCA 1-mutation in a case of metastasized mixed adeno-neuroendocrine carcinoma (MANEC) of the small bowel.

BACKGROUND: Adenocarcinomas or combined adeno-neuroendocrine carcinomas (MANEC) of small bowel usually have a dismal prognosis with limited systemic therapy options. This is the first description of a patient showing a germline-related BRCA1 mutated MANEC of his ileum. The tumor presented a susceptibility to a combined chemotherapy and the PARP1-inhibitor olaparib. CASE PRESENTATION: A 74-year old male patient presented with a metastasized MANEC of his ileum. Due to clinical symptoms his ileum-tumor and the single brain metastasis were removed. We verified the same pathogenic (class 5) BRCA1 mutation in different tumor locations. There was no known personal history of a previous malignant tumor. Nevertheless we identified his BRCA1 mutation as germline-related. A systemic treatment was started including Gemcitabine followed by selective internal radiotherapy (SIRT) to treat liver metastases and in the further course Capecitabine but this treatment finally failed after 9 months and all liver metastases showed progression. The treatment failure was the reason to induce an individualized therapeutic approach using combined chemotherapy of carboplatin, paclitaxel and the Poly (ADP-ribose) polymerase- (PARP)-inhibitor olaparib analogous to the treatment protocol of Oza et al. All liver metastases demonstrated with significant tumor regression after 3 months and could be removed. In his most current follow up from December 2017 (25 months after his primary diagnosis) the patient is in a very good general condition without evidence for further metastases. CONCLUSION: We present first evidence of a therapy susceptible germline-related BRCA1 mutation in small bowel adeno-neuroendocrine carcinoma (MANEC). Our findings offer a personalized treatment option. The germline background was unexpected in a 74-year old man with no previously known tumor burden. We should be aware of the familiar background in tumors of older patients as well.

First case report of a curative wedge resection in epithelial-myoepithelial carcinoma of the lung.

Epithelial-myoepithelial carcinoma is a well differentiated malignant neoplasm, which originates from the salivary glands. The primary pulmonary manifestation is rare-about 30 cases have been reported worldwide. In the literature, anatomical resection has been described as the standard surgical approach. In the presented case, a wedge resection was performed, with no evidence for tumor relapse in the follow-up reevaluation after 24 months. This is the first case report of a primary pulmonary epithelial-myoepithelial carcinoma that has been treated with a non-anatomical wedge resection and lymph node dissection as a curative approach.