Hepatobiliary mucinous cystic neoplasms (MCN) harbor an invasive carcinoma in 16% of the cases, mainly tubular type, but occasionally sarcomatoid or undifferentiated, these entities being frankly ...rare.
We present the case of a liver MCN with an invasive component and sarcomatous degeneration.
The patient was treated surgically with subsequent adjuvant chemotherapy (capecitabine), presenting tumor progression after three months with peritoneal carcinomatosis and liver recurrence. The patient died due to liver failure 4 months after surgery.
MCN with an invasive component and sarcomatous degeneration are very rare, present advanced stages, show aggressive behavior, and have a poor prognosis.
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