title: Malignant extrarenal rhabdoid tumour (MERT) with liver metastases as a rare cause of an esophageal tumor in a 57 years old patient.
abstract: 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.
authors.affiliation: HOP Ulm, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Kaechele
authors.affiliation: Gastroenterology, General Hospital, Blaubeuren, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Vogelpohl
authors.affiliation: Private Practice for Gastroenterology, Ulm, Germany.
authors.forename: W
authors.initials: W
authors.lastname: Boeck
authors.affiliation: Hematology and Oncology, German Army Forces Hospital, Ulm, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Riecke
authors.affiliation: Gastroenterology, General Hospital, Blaubeuren, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Eisele
authors.affiliation: Pathology, University of Ulm, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Barth
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004938:Esophageal Neoplasms / Q000188:drug therapy* / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000556:secondary*; D008297:Male; D008875:Middle Aged; D018335:Rhabdoid Tumor / Q000188:drug therapy / Q000473:pathology* / Q000556:secondary*