INTRODUCTIONPeutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamartomatous polyposis syndrome. Morbidity arises from polyp-related complications and increased risks of malignancy. We report on PJS patients registered in the Singapore Polyposis Registry, identified principal causes of morbidity and appraised current management strategies. A followup protocol based on recent literature has been proposed.

MATERIALS AND METHODSA search of a prospectively collected database in the Singapore Polyposis Registry was made. Only patients who fulfilled the diagnostic criteria of PJS were included. The clinical records were retrieved for review. Information on affected family members was obtained from the Registry's pedigree records.

RESULTSSeven unrelated patients fulfilled the criteria of having PJS. Principal causes of morbidity include recurrent bouts of abdominal colic, episodes of intestinal obstruction, gastrointestinal bleeding and the need for repeated laparotomies. Six out of 7 patients had initial presentation with acute intestinal obstruction requiring emergency laparotomy. Management was mostly problem-oriented and marked inter-surgeon variation with regard to cancer screening and genetic counselling was observed.

CONCLUSIONPatients with PJS suffer gastrointestinal complications from polyposis and are at increased risks for developing cancers. A move towards surveillance and planned comprehensive care may reduce the morbidity of the condition. A protocol driven approach conducted in the setting of a Polyposis Registry is ideally suited to facilitate such care.

Adolescent ; Adult ; Child ; Cohort Studies ; Critical Pathways ; Disease Management ; Female ; Gastrointestinal Diseases ; complications ; epidemiology ; Humans ; Male ; Peutz-Jeghers Syndrome ; complications ; therapy ; Population Surveillance ; Prevalence ; Registries ; Singapore ; epidemiology ; Young Adult

Aged, 80 and over ; Axilla ; Breast Neoplasms ; epidemiology ; surgery ; Comorbidity ; Diabetes Mellitus ; epidemiology ; Dyslipidemias ; epidemiology ; Female ; Humans ; Hypertension ; epidemiology ; Length of Stay ; Lymph Node Excision ; Mastectomy ; Myocardial Infarction ; epidemiology ; Myocardial Ischemia ; epidemiology ; Operative Time ; Postoperative Complications ; epidemiology ; Postoperative Hemorrhage ; epidemiology ; Seroma ; epidemiology ; Singapore ; epidemiology ; Surgical Wound Dehiscence ; epidemiology ; Surgical Wound Infection ; epidemiology ; Venous Thrombosis ; epidemiology

INTRODUCTIONThe extent of lymph nodes (LNs) metastasis is a major determinant for the staging and the most reliable adverse prognostic factor. Primary tumours can induce lymphatics and vasculature reorganisations within sentinel LN before the arrival of cancer cells and these key blood vessels are identified as high endothelial venules (HEV). The alterations of HEV in the presence of cancer, coupled with the increased proliferation rate of the endothelial cells, results in a functional shift of HEV from immune response mediator to blood flow carrier. We aim to evaluate tumour-induced vascularisation in regional LN of cancer patients by studying the morphological and functional alterations of HEV and its correlation to clinico-pathological features.

MATERIALS AND METHODSThis multi-centre study with a prospective database identified 65 consecutive patients with tongue squamous cell carcinoma (SCC) who underwent primary surgical treatment from 2001 to 2005. Immunohistochemical staining for HEV and image analysis were performed and analysed with correlation to the patients' clinico-pathological features.

RESULTSThe total number of HEV is significantly associated to disease-free interval when controlling for the group (P = 0.022) as well as combining both groups as one cohort (P = 0.023). There is also a similar association comparing the HEV parameters to overall survival.

CONCLUSIONOur results suggest that HEV possibly plays a key role in the pathogenesis of lymphatic and subsequent distant metastases and may provide the missing link in cancer metastasis. Confirmation of this hypothesis would offer a novel therapeutic approach to preventing metastasis by blocking the remodeling processes of HEV in LN.

Awards and Prizes ; Biomarkers ; Databases, Factual ; Endothelial Cells ; physiology ; General Surgery ; Humans ; Lymph Nodes ; blood supply ; pathology ; Neoplasm Metastasis ; diagnosis ; Neovascularization, Pathologic ; pathology ; Prospective Studies ; Venules ; pathology