TY  - JOUR
A1  - Riedmeier, Maria
A1  - Decarolis, Boris
A1  - Haubitz, Imme
A1  - Müller, Sophie
A1  - Uttinger, Konstantin
A1  - Börner, Kevin
A1  - Reibetanz, Joachim
A1  - Wiegering, Armin
A1  - Härtel, Christoph
A1  - Schlegel, Paul-Gerhardt
A1  - Fassnacht, Martin
A1  - Wiegering, Verena
T1  - Adrenocortical carcinoma in childhood: a systematic review
JF  - Cancers
N2  - Adrenocortical tumors are rare in children. This systematic review summarizes the published evidence on pediatric adrenocortical carcinoma (ACC) to provide a basis for a better understanding of the disease, investigate new molecular biomarkers and therapeutic targets, and define which patients may benefit from a more aggressive therapeutic approach. We included 137 studies with 3680 ACC patients (~65% female) in our analysis. We found no randomized controlled trials, so this review mainly reflects retrospective data. Due to a specific mutation in the TP53 gene in ~80% of Brazilian patients, that cohort was analyzed separately from series from other countries. Hormone analysis was described in 2569 of the 2874 patients (89%). Most patients were diagnosed with localized disease, whereas 23% had metastasis at primary diagnosis. Only 72% of the patients achieved complete resection. In 334 children (23%), recurrent disease was reported: 81% — local recurrence, 19% (n = 65) — distant metastases at relapse. Patients < 4 years old had a different distribution of tumor stages and hormone activity and better overall survival (p < 0.001). Although therapeutic approaches are typically multimodal, no consensus is available on effective standard treatments for advanced ACC. Thus, knowledge regarding pediatric ACC is still scarce and international prospective studies are needed to implement standardized clinical stratifications and risk-adapted therapeutic strategies.
KW  - pediatric adrenocortical cancer
KW  - pediatric adrenocortical adenoma
KW  - pediatric adrenocortical tumor
KW  - prognostic factors
KW  - therapy
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-248507
SN  - 2072-6694
VL  - 13
IS  - 21
ER  - 
TY  - JOUR
A1  - Kelm, Matthias
A1  - Anger, Friedrich
A1  - Eichlinger, Robin
A1  - Brand, Markus
A1  - Kim, Mia
A1  - Reibetanz, Joachim
A1  - Krajinovic, Katica
A1  - Germer, Christoph-Thomas
A1  - Schlegel, Nicolas
A1  - Flemming, Sven
T1  - Early Ileocecal Resection Is an Effective Therapy in Isolated Crohn’s Disease
JF  - Journal of Clinical Medicine
N2  - Despite the increasing incidence and prevalence of Crohn’s Disease (CD), no curative options exist and treatment remains complex. While therapy has mainly focused on medical approaches in the past, growing evidence reveals that in cases of limited inflammation, surgery can suffice as an alternative primary treatment. We retrospectively assessed the disease course and outcomes of 103 patients with terminal Ileitis who underwent primary surgery (n = 29) or received primary medical treatment followed by surgery (n = 74). Primary endpoint was the need for immunosuppressive medication after surgical treatment (ileocecal resection, ICR) during a two-years follow-up. Rates for laparoscopic ICR were enhanced in case of early surgery, but no differences were seen for postoperative complications. In case of immunosuppressive medication, patients with ICR at an early state of disease needed significantly less anti-inflammatory medication during the two-year postoperative follow-up compared to patients who were primarily treated medically. Furthermore, in a subgroup analysis for patients with localized ileocecal disease manifestation, early surgery consistently resulted in a decreased amount of medical therapy postoperatively. In conclusion primary ICR is safe and effective in patients with limited CD, and the need for immunosuppressive medication during the postoperative follow-up is low compared to patients receiving surgery at a later stage of disease.
KW  - Crohn’s Disease
KW  - surgical therapy
KW  - ileocecal resection
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-228822
SN  - 2077-0383
VL  - 10
IS  - 4
ER  -