Chondroid Tumors: Review of Salient Imaging Features and Update on the WHO Classification

Chondrogenic tumors are typically well recognized on radiographs, but differentiation between benign and malignant cartilaginous lesions can be difficult both for the radiologist and for the pathologist. Diagnosis is based on a combination of clinical, radiological and histological findings. While treatment of benign lesions does not require surgery, the only curative treatment for chondrosarcoma is resection. This article (1) emphasizes the update of the WHO classification and its diagnostic and clinical effects; (2) describes the imaging features of the various types of cartilaginous tumors, highlighting findings that can help differentiate benign from malignant lesions; (3) presents differential diagnoses; and (4) provides pathologic correlation. We attempt to offer valuable clues in the approach to this vast entit

Neuroendokrine Neoplasien: Radiologische und Klinische Korrelation

The aim of this study was to prove that specific radiological findings and clinical- radiological scores can give physicians valuable information in order to better understand the clinical course and prognosis of neuroendocrine neoplasms (NEN). Mesenteric fibrosis (MF) is a radiological pathognomonic sign of midgut NEN. In Publication 1 a potential relationship of MF with carcinoid syndrome, urinary 5- hydroxyindoleacetic acid (5-HIAA), and carcinoid heart disease (CHD) was assessed. Imaging characteristics of lymphatic and hepatic metastases at diagnosis, 5-HIAA, functionality, and development of CHD were analyzed from a cohort of 81 patients. Independent predictors of MF were 5-HIAA ≥395 μmol/day, age, and largest lymphatic metastasis ≥24 mm (p<0.05). MF was associated with decreased time to development of CHD in functional midgut NENs (p<0.05). An association between MF and 5HIAA was found which suggests a linked pathophysiological mechanism, which might be similar to that of endocardial fibrosis in CHD. In Publication 2 Charité was part of a multicenter study which analyzed the combination of several risk factors to predict progression of disease (PD) in gastro- entero-pancreatic NEN. Multivariate analysis for PD was performed in a cohort of 256 patients. Independent risk factors for PD were proliferative index Ki67, radiologically assessed hepatic tumor burden, and presence of extra-abdominal metastases. A risk score formula was obtained which showed a superior accuracy to predict PD compared with grading system based in Ki67. The purpose in Publication 3 was to evaluate the correlation between clinical signs and computed tomography (CT) findings in everolimus-induced pneumonitis. From a cohort of 90 NEN patients 18 had pneumonitis. Clinical signs of pneumonitis were scored and pulmonary function tests (PFT) were evaluated if available. CT images were analyzed based on the severity of interstitial lung disease (ILD), the overall pneumonitis extent (PnE), and regarding presence of typical lung opacification patterns. There was no significant correlation between symptomatic score or PFT and ILD score or PnE. Cryptogenic organizing pneumonia pattern had significantly lower symptomatic scores than in case of other opacification patterns (p<0.05). Asymptomatic patients with specific pneumonitis CT findings should be closely monitored so that treatment can be timely initiated. In conclusion, radiological assessment of liver or lung affection were valuable tools in analyzing NEN. CT lung findings showed no clinical correlation in everolimus- induced pneumonitis. Hepatic tumor burden proved to be a relevant factor both for NEN prognosis and MF. MF and 5HIAA may play an important role in the pathophysiology of MF.Ziel der Arbeit war es zu beweisen, dass bestimmte radiologische Befunde und klinisch-radiologische Scores wertvolle Informationen liefern können, um den klinischen Verlauf und die Prognose neuroendokriner Neoplasien (NEN) besser zu verstehen. Mesenterialfibrose (MF) ist ein radiologisch pathognomonisches Zeichen für Mitteldarm-NEN. In der Publikation 1 wurde eine potenzielle Beziehung zwischen MF und Karzinoid-Syndrom, 5-Hydroxyindolessigsäure (5-HIAA) im Urin und Karzinoid- Herzkrankheit (CHD) untersucht. Die bildgebenden Eigenschaften von lymphatischen und hepatischen Metastasen bei Diagnose, 5-HIAA, Funktionalität und Entwicklung von CHD wurden aus einer Kohorte von 81 Patienten analysiert. Unabhängige Prädiktoren für MF waren 5-HIAA ≥ 395 μmol/Tag, Alter und größte lymphatische Metastase ≥ 24 mm (p <0,05). MF war in funktionellen Mitteldarm-NEN mit einer verkürzten Zeit bis zur Entwicklung von CHD assoziiert (p <0,05). Eine Assoziation zwischen MF und 5HIAA wurde gefunden, die auf einen verknüpften pathophysiologischen Mechanismus hinweist, der bei endokardialen Fibröse in CHD ähnlich sein könnte. In der Publikation 2 nahm Charité an einer multizentrischen Studie teil, in der die Kombination mehrerer Risikofaktoren analysiert wurde, um die Progredienz bei gastroentero-pankreatischem NEN vorherzusagen. Eine multivariate Analyse auf Progredienz wurde in einer Kohorte von 256 Patienten durchgeführt. Unabhängige Risikofaktoren für die Progredienz waren der Proliferationsindex Ki67, die radiologisch bewertete Belastung des Lebertumors und das Vorhandensein von extraabdominalen Metastasen. Es wurde eine Risiko-Score-Formel erhalten, die eine überlegene Genauigkeit zur Vorhersage der Progredienz im Vergleich zu einem auf Ki67 basierenden Bewertungssystem zeigte. Ziel der Publikation 3 war es klinischen Anzeichen und Computertomographie (CT) Befunde bei Everolimus-induzierter Pneumonitis zu korrelieren. 18 Patienten, aus einer Kohorte von 90 NEN-Patienten, hatten Pneumonitis. Klinische Anzeichen einer Pneumonitis wurden bewertet und Lungenfunktionstests (PFT) wurden analysiert, falls verfügbar. CT-Bilder wurden basierend auf der Schwere der interstitiellen Lungenerkrankung (ILE), dem Gesamtausmaß der Pneumonitis (PnE) und dem Vorhandensein typischer Lungentrübungsmuster analysiert. Es gab keine signifikante Korrelation zwischen dem symptomatischen Score oder dem PFT- und ILD-Score oder dem PnE. Das kryptogen organisierende Lungenentzündungsmuster hatte signifikant niedrigere symptomatische Score als bei anderen Trübungsmustern (p <0,05). Asymptomatische Patienten mit spezifischen Pneumonitis-CT-Befunden sollten engmaschig überwacht werden, damit die Behandlung rechtzeitig eingeleitet werden kann. Zusammenfassend war die radiologische Beurteilung der Leber- oder Lungenerkrankung ein wertvolles Instrument zur Analyse von NEN. CT-Lungenbefunde zeigten keine klinische Korrelation bei Everolimus-induzierter Pneumonitis. Die Belastung durch Lebertumoren erwies sich sowohl für die NEN-Prognose als auch für die MF als relevanter Faktor. MF und 5HIAA können eine wichtige Rolle in der Pathophysiologie von MF spielen

Impact of Change in Body Composition during Follow-Up on the Survival of GEP-NET

Background: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are heterogeneous rare diseases causing malnutrition and cachexia in which the study of body composition may have an impact in prognosis. Aim: Evaluation of muscle and fat tissues by computed tomography (CT) at the level of the third lumbar (L3 level) at diagnosis and at the end of follow-up in GET-NET patients and their relationships with clinical and biochemical variables as predictors of survival. Methodology: Ninety-eight GEP-NET patients were included. Clinical and biochemical parameters were evaluated. Total body, subcutaneous, visceral and total fat areas and very low-density, low-density, normal density, high-density, very high-density and total muscle areas were obtained from CT images. Results: Body composition measures and overall mortality correlated with age, ECOG (Eastern Cooperative Oncology Group performance status) metastases, lactate dehydrogenase (LDH), albumin and urea levels. Although there was no relationship between body composition variables at diagnosis and overall and specific mortality, an increase in low-density muscle and a decrease in normal-density muscle during follow-up were independently correlated to overall (p &lt;0.05) and tumor-cause mortality (p &lt; 0.05). Conclusion: Although body composition measures obtained by CT at diagnosis did not impact survival of GEP-NET patients, a loss of good quality muscle during follow-up was associated with an increased overall and tumor-related mortality. Nutritional status should therefore be supervised by nutrition specialists and an increase in good quality muscle could improve prognosis