A Case of Pleomorphic-Type Anaplastic Carcinoma of the Pancreas with Rapidly Progressive and Fatal Cardiac Metastasis

一例快速进展并导致致命性心脏转移的胰腺多形性未分化癌

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Abstract

INTRODUCTION: Cardiac metastasis from pancreatic ductal adenocarcinoma (PDAC) is extremely rare. Pleomorphic-type anaplastic carcinoma of the pancreas (PACP) is an uncommon, highly aggressive PDAC variant with lymphatic and hematogenous spread and poor prognosis. We report, to our knowledge, the first PACP case with antemortem diagnosis of cardiac metastasis and a rapidly fatal course. CASE PRESENTATION: A 75-year-old man presented with left hypochondrial pain. CT showed a 4-cm, gradually and heterogeneously enhancing pancreatic tail mass without distant metastasis. Gadolinium-enhanced MRI demonstrated peripheral progressive enhancement with internal non-enhancing areas, suggesting a non-conventional PDAC. Ultrasound-guided fine needle aspiration (EUS-FNA), nevertheless, yielded mainly scattered atypical epithelial cells with a small cohesive columnar component, and adenocarcinoma was diagnosed; further subclassification was not feasible on the limited cytologic material. Because the tumor was radiologically resectable, neoadjuvant gemcitabine plus S-1 was administered. Follow-up CT showed enlargement to 5.5 cm, new invasion of the spleen and gastric wall, and peripheral progressive enhancement-previously demonstrated on MRI-now appreciable on CT, while distant disease remained absent. Distal pancreatectomy with splenectomy, regional lymphadenectomy, and partial gastrectomy was performed. The resected specimen revealed a whitish solid tail tumor with focal hemorrhage/necrosis; histology confirmed PACP. Adjuvant S-1 was initiated 1 month postoperatively. Three months after surgery, CA19-9 increased despite no recurrence on CT. One month later he presented with exertional dyspnea and complete atrioventricular block. Echocardiography and CT showed an approximately 8-cm interatrial mass and a left-ventricular wall lesion. Retrospective review of CT performed 1 month earlier identified a 2-cm atrial nodule at the same site, indicating very rapid intracardiac growth. He was transferred to a cardiovascular center for planned pacemaker implantation, and heart-failure therapy was initiated first. During preparation he developed sudden cardiac arrest and died. Postmortem myocardial biopsy confirmed PACP metastasis. CONCLUSIONS: Clinicians need to recognize that PACP can metastasize to the heart and incorporate this risk into routine follow-up, maintaining vigilance for imaging features suggestive of cardiac involvement and for new-onset cardiac symptoms or conduction disturbances; when cardiac metastasis is suspected, prompt evaluation and intervention are warranted.

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