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: V Kaechele, J Vogelpohl, W Boeck, A Riecke, R Eisele, T Barth
• Journal: Zeitschrift fur Gastroenterologie (Z Gastroenterol) Vol. 53 Issue 7 Pg. 660-3 (Jul 2015) ISSN: 1439-7803 [Electronic] Germany
• PMID: 26167696 (Publication Type: Case Reports, Journal Article)
• Copyright: © Georg Thieme Verlag KG Stuttgart · New York.

Topics

• Antineoplastic Combined Chemotherapy Protocols (administration & dosage)
• Diagnosis, Differential
• Esophageal Neoplasms (drug therapy, pathology)
• Fatal Outcome
• Humans
• Liver Neoplasms (drug therapy, pathology, secondary)
• Male
• Middle Aged
• Rhabdoid Tumor (drug therapy, pathology, secondary)