ABSTRACT
von Hippel-Lindau (VHL) syndrome is an inherited neoplastic syndrome caused by abnormity of the VHL gene found on the short arm of the chromosome 3. We reported a case of VHL disease diagnosed by the detection of multiple pancreatic endocrine tumors and renal tumor 13 years after bilateral adrenalectomy. A 40-year-old man presented with multiple pancreas tumors (maximum size 42 mm in diameter) detected by screening abdominal ultrasonography. A 23 mm renal tumor was detected by contrast computed tomography scan at that time. His past history included left retinal angioma (age 15) and bilateral adrenal pheochromocytoma (age 27). VHL was diagnosed by genetic testing. Endoscopic ultrasound-guided fine-needle aspiration biopsy of the pancreas tumor was performed, and tumor was diagnosed as an endocrine tumor. After diagnosis, distal pancreatectomy (body-tail) was performed. This was a didactic case indicating that we should suspect VHL syndrome based on past history and family history and follow such cases up strictly.
Subject(s)
Adrenalectomy , Kidney Neoplasms/diagnosis , Neoplasms, Multiple Primary , Pancreatic Neoplasms/diagnosis , von Hippel-Lindau Disease/diagnosis , Adrenal Gland Neoplasms/surgery , Adult , Chromosomes, Human, Pair 3/genetics , Hemangioma , Humans , Male , Pancreatectomy , Pancreatic Neoplasms/surgery , Pheochromocytoma/surgery , Retinal Neoplasms , Time Factors , von Hippel-Lindau Disease/geneticsABSTRACT
From the EtOAc-soluble fraction of an MeOH extract of the stems of Tricalysia dubia, two new rearranged ent-kaurane derivatives, named tricalysiolides H and I, were isolated, together with five known rearranged ent-kauranes, i.e. tricalysiolides A-E, stigmast-4-en-6beta-ol-3-one, (+)-pinoresinol, scopoletin and syringaldehyde. Their structures were elucidated from spectroscopic evidence, and their cytotoxicity toward KB cells and P-gp inhibitory activity were assayed.
