title: An autopsy case of tuberous sclerosis associated with primary pericardial mesothelioma.
abstract: Tuberous sclerosis (Bourneville-Pringle phacomatosis) has been known to be associated with cardiac rhabdomyoma, but apparently never previously with primary pericardial mesothelioma. We present an autopsy case of this condition in a 59-year-old man, who had been diagnosed as having tuberous sclerosis in view of the presence of facial sebaceous adenoma, mental retardation, intracranial calcification, cerebral ventricular dilatation and renal tumor. During the clinical course, characterized by heart failure due to cardiac tamponade, cardiac sarcoma was diagnosed by imaging techniques. Autopsy revealed biphasic-type primary pericardial mesothelioma. As to the tuberous sclerosis, atypical giant cells in the tubers of the cerebral cortex and the lateral ventricular wall were found, which were considered to be derived from neurons rather than glial cells on the basis of staining with Bodian, Holzer, and antibodies against NSE, GFA and S-100 protein. In old tubers protruding into the lateral ventricles, fibrous glias were present with dense calcospherite deposits, coinciding with the CT findings. The renal tumors were angiomyolipomas, which were present bilaterally and showed partially infiltrative growth, but seemed to have a benign nature because of the lack of metastasis and atypism of the leiomyocytes.
authors: 1st Department of Pathology, Shinshu University School of Medicine, Matsumoto, Japan.
authors: A
authors: A
authors: Naramoto
authors: N
authors: N
authors: Itoh
authors: M
authors: M
authors: Nakano
authors: H
authors: H
authors: Shigematsu
mesh_terms: D001344:Autopsy; D002531:Cerebellum / Q000473:pathology; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006338:Heart Neoplasms / Q000150:complications* / Q000473:pathology; D006391:Hemangioma / Q000473:pathology; D006651:Histocytochemistry; D006801:Humans; D007150:Immunohistochemistry; D007633:Keratins / Q000378:metabolism; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000473:pathology; D008067:Lipoma / Q000473:pathology; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism; D008654:Mesothelioma / Q000150:complications* / Q000473:pathology; D008875:Middle Aged; D018396:Mucin-1; D010496:Pericardium; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D009418:S100 Proteins / Q000378:metabolism; D014402:Tuberous Sclerosis / Q000150:complications* / Q000473:pathology
chemical_list: D005904:Glial Fibrillary Acidic Protein; D008562:Membrane Glycoproteins; D018396:Mucin-1; D009418:S100 Proteins; D007633:Keratins; D010751:Phosphopyruvate Hydratase