Pancreatic cancer and hypercalcemia associated with von Recklinghausen's disease

Abstract

A 44-year-old man with von Recklinghausen's disease was admitted to our hospital presenting with left hypochondralgia. Computed tomography showed a large mass at the body and tail of the pancreas, with metastatic liver tumors. Endoscopic retrograde pancreatography revealed an obstruction of the main pancreatic duct at the body. In the course of the illness, the patient's serum calcium concentration increased gradually to 13.6 mg/dl, although bone scan with 99mtechnetium demonstrated no accumulation in the bones. Serum levels of parathyroid hormone related-protein and tumor necrosis factor were also elevated. Based on these findings, he was diagnosed as having pancreatic cancer with liver metastases and humoral hypercalcemia of malignancy associated with von Recklinghausen's disease. Postmortem examination revealed a solid tumor, measuring 6.0 x 6.0 x 8.0 cm, in the body and tail of the pancreas. Histologically, the tumor was moderately differentiated adenocarcinoma. The association of von Recklinghausen's disease with malignant neurogenic neoplasms is well established, whereas the association with non-neurogenic malignant neoplasm is considered to coincidental. Our current case suggests a possible relationship between von Recklinghausen's disease and pancreatic cancer.

Basic transcription factor 3 (BTF3) regulates transcription of tumor-associated genes in pancreatic cancer cells

“…58,59 Another factor, MRVI1, regulates cellular calcium release, 60 and is frequently disturbed in pancreatic endocrine tumors. 61,62 In addition, the nuclear transcription factor NFkB regulates the expression of a number of genes that are involved in tumorigenesis, and suppression of NFkB is associated with increased chemosensitivity as well as induction of apoptosis. 63,64 This indicates that BTF3 might influence cell growth through regulation of the expression of NFkB and its target genes in pancreatic cancer cells.…”

A case of numerous atypical adenomatous hyperplasia diagnosed by a video-assisted thoracoscopic lung biopsy

“…The pulmonary manifestations of NF-I previously reported are GGA, bibasilar reticular opacities, bullae, cysts, and emphysema in the upper lobe [10]. .Although there was a case of NF-1 with intense and widespread fibrosis and emphysematous change, which accompanied adenomatous hyperplasia in part, it was not diagnosed to be so-called AAH [11]. Therefore, the relationship between NF-1 and AAH still remains uncertain, as far as we could determine.…”

Adenosquamous pancreatic cancer producing parathyroid hormone-related protein

“…To our knowledge, only seven reports have described PTH-rP-mediated hypercalcemia associated with exocrine pancreatic cancer (five adenocarcinomas, one undifferentiated carcinoma, and one adenosquamous carcinoma). [15][16][17][18][19][20][21] and this is the first case directly demonstrating PTH-rP production from adenosquamous pancreatic cancer. In our patient, the plasma PTH-rP level was markedly elevated, and pathological examination confirmed the tumor cell production of PTH-rP by an immunohistochemical method.…”