Malignant extrarenal rhabdoid tumour (MERT) with liver metastases as a rare cause of an esophageal tumor in a 57 years old patient.

Tumors with a rhabdoid phenotype are aggressive neoplasms with a dismal prognosis. Malignant extrarenal rhabdoid tumor (MERT) of the esophagus is an extremely rare disease with so far only 6 cases reported. We report on a 57-year-old male patient with rhabdoid tumor situated in the esophagus with metastases to the liver and local lymph nodes. Assuming an undifferentiated esophageal adenocarcinoma a palliative chemotherapy with 5-FU/folinic acid, oxaliplatin, and docetaxel (FLOT) was initiated which was changed towards a combination of doxorubicin and ifosphamide as immunohistochemistry of the primary and the liver metastases revealed a rhabdoid tumor. This treatment with doxorubicin and ifosphamide resulted in a short clinical and radiological response which lasted only for 2 months. Due to the bad general condition at the time of progression no further chemotherapy was initiated. The patient died due to tumor progression 6 months after initial diagnosis which is consistent with other reports on malignant extrarenal rhabdoid tumors (median survival of metastatic disease less than 6 months). Thus, metastatic MERT represents a disease with a poor prognosis and no established standard therapy.

[Reflux-associated nutcracker oesophagus in a 49-year-old patient with non-cardiac chest pain (NCCP)]. / Reflux-assoziierter Nussknacker-Osophagus bei 49-jähriger Patientin mit nicht kardialem Thoraxschmerz (NCCP).

A 49-year-old woman was admitted to our department because of intermittent left-sided chest pain for the past 2 years. The patient reported an increasing frequency and duration of the symptoms which were independent from food intake, medication or physical exercises. A cardiac cause of the chest pain was excluded before admittance to our hospital. The initially performed upper endoscopy revealed a small oesophageal hiatal hernia but no signs of acute or chronic inflammation. Basal oesophageal manometry and pH monitoring confirmed the presence of a nutcracker oesophagus, defined by high-amplitude contractions of the distal oesophagus, in combination with a gastro-oesophageal reflux disease. The patient was treated with high-dose pantoprazole for 8 weeks before re-examination. Interestingly, the symptoms completely disappeared upon medication and the relief of the chest pain was accompanied by normalisation of the nutcracker oesophagus pattern. Thus, our observation provides evidence for a causative role of acid reflux in the pathophysiology of the nutcracker oesophagus and, in addition, suggests that effective acid suppression might be a useful therapeutic tool to deal with this painful motility disorder of the oesophagus.

[Intestinal wall vascularisation in Crohn's disease]. / Darmwandvaskularisation bei Morbus Crohn.

BACKGROUND AND AIMS: In a pilot study the semi-quantitative classification of intestinal wall vascularisation as proposed by Limberg was evaluated. PATIENTS AND METHODS: 20 patients with confirmed Crohn's disease and clinical activity (10 male, 10 female, mean age 30.0 +/- 7.72 years, range 21 - 49 years, mean time since onset of disease 4.6 years, range 0 - 15 years) were included. CDAI, CRP, ESR, and the blood count were evaluated. Two and six weeks after inclusion into the study these examinations were repeated. All patients were treated with anti-inflammatory drugs. The intestinal wall thickness was measured with ultrasound. The vascularisation following the Limberg classification and the number of blood vessels per square centimetre were assessed in the power-Doppler mode. RESULTS: The mean length of bowel segments with increased wall thickness (> 3 mm) at the beginning of the study was 20.3 cm (range 5 - 50 cm), the mean intestinal wall diameter 5.9 mm (range 4 - 9 mm). The mean density of blood vessels in the power-Doppler mode was 3.8 vessels/cm (2) (range 0 - 8 vessels/cm (2)), the median of Limberg levels was 2 (range 1 - 4). The density of blood vessels per cm (2) well correlated with the Limberg classification throughout the study (r = 0.2 at start; r = 0.94 at 1st follow-up; r = 0.91 at 2nd follow-up). CONCLUSION: The classification for measuring intestinal wall vascularisation semi-quantitatively (as proposed by Limberg) proved to be easily applicable in routine sonography. Besides the measurement of intestinal wall thickness, activity indices, clinical and laboratory parameters, it may constitute an additional means for evaluation of disease activity.