| Theme |
Evaluation of the Chemotherapy for the Unresectable Pancreatic Cancer |
| Title |
Management of Extraintestinal Manifestations in Familial Adenomatous Polyposis |
| Author |
Hidefumi Tsunozaki |
Department of Surgery, Kyoundo Hospital, Sasaki Foundation |
| Author |
Takeo Iwama |
Department of Surgery, Kyoundo Hospital, Sasaki Foundation |
| [ Summary ] |
A number of extra-gastrointestinal manifestations which are frequently associated with familial adenomatous polyposis (FAP) are discribed. In relation to these conditions, desmoid tumors are non-metastasising but aggressive forms of fibromatosis which occur in about 10 - 25 % of patients with FAP, usually after abdominal surgery. Close observation is one of the best ways to provide optimal treatment of desmoid tumors. Osteomas, including dental abnormalities and congenital hypertrophy of the retinal pigment epithelium (CHRPE) are harmless lesions but helpful in the diagnosis of FAP. Thyroid carcinoma occurs in 2 % of FAP cases and is usually limited to female patients. This condition displays the specific histological features of FAP. Pancreatic cancer and periampullary cancer are the most serious tumors, and are found in about 2 % of FAP patients. Early detection is essential for curative treatment. Adrenocortical tumors are usually found to be related to incidentaloma when examined with ultrasonography, CTs or MRIs. Large tumors which exceed 4 cm in size should be considered for surgical treatment. Brain tumors rarely occur in patients with FAP. This condition should be distinguished from Turcot syndrome. Other soft tissue tumors and gynecological malignancies are discussed. |