Anaplastic and poorly differentiated thyroid carcinoma: therapeutic strategies and treatment outcome of 52 consecutive patients.

Anaplastic thyroid carcinoma (ATC) is one of the most lethal malignancies; poorly differentiated thyroid carcinoma (PDTC) is a new diagnosis for rare aggressive thyroid tumours. Surgery is often considered the only chance for survival, but the benefit of surgery and subsequent multimodal therapy is unclear. We retrospectively analyzed the outcome of 44 ATC and 8 PDTC consecutive patients treated at Helsinki University Central Hospital between 1990 and 2008. All ATC and PDTC cases were re-examined and reclassified histologically. Median survival was only 3.1 months for ATC, but 3.7 years for PDTC. Most patients in both groups eventually died of cancer. ATC patients were older than PDTC patients (74 vs. 66 years). Nodal and distant metastases had a negative impact on survival (ATC; p = 0.038, p = 0.008). Long-term survivors in both groups were stage N0M0 at presentation. Multimodal therapy was successful for 9 (20%) ATC patients, and their median survival was the longest (11.6 months) among treatment groups. Most PDTC patients (88%) underwent total thyroidectomy followed by radioiodine ablation; the only 2 who received chemotherapy survived longest. Although ATC and PDTC are both aggressive thyroid carcinomas, multimodal therapy for both can provide a chance of prolonged survival in patients with locoregional disease.

Extraction and purification of DNA in rhizosphere soil samples for PCR-DGGE analysis of bacterial consortia.

Application of DNA fingerprinting methods enables the detection of diverse members of soil bacterial consortia, even including those bacteria not yet cultivated. However, extraction and purification of DNA from soil samples without bias is difficult. We compared five different DNA isolation methods and three purification methods for rhizosphere soil samples. Purified DNA extracts were amplified in PCR using universal bacterial primers and the PCR products were analysed with denaturing gradient gel electrophoresis (DGGE) for the visualisation of DNA bands representing dominant bacterial species. Both the isolation and purification methods affected the apparent bacterial community structure of the samples.

Impact of screening examinations on survival in familial adenomatous polyposis.

BACKGROUND: Prophylactic family screening and surgery has improved the outcome of patients with familial adenomatous polyposis (FAP) largely preventing deaths due to colorectal cancer. The present study compared the mortality rates and causes of death of FAP patients diagnosed by symptoms (probands) or by family screening (call-up). METHODS: The study comprised all 236 FAP patients registered in the Finnish Polyposis Registry until the end of June 1998. There were 116 probands and 120 call-up patients with a median age of 36.8 and 22.8 at diagnosis and median follow-up times of 6.3 and 9.9 years, respectively. Cumulative crude and relative survival estimates were calculated for each group and the causes of death were determined. RESULTS: The life expectancy was significantly better in the call-up group than in the probands after colectomy (P < 0.001). The survival rates of the call-up group equaled those expected for a comparable group in the general population up to 18 years after colectomy. The main cause of death was colorectal cancer accounting for 54 out of 68 deaths: four in the call-up group (all rectal stump cancer) and 50 in probands. Upper GI-tract cancer caused four deaths (periampullary cancer two, stomach cancer two) and two deaths were due to postoperative pulmonary embolism. CONCLUSION: The survival of FAP patients is significantly improved by prophylactic screening and surgery. Further improvement may be possible by using restorative proctocolectomy instead of colectomy and ileorectal anastomosis and by regular upper GI-tract endoscopic surveillance.

Management of duodenal adenomas in 98 patients with familial adenomatous polyposis.

BACKGROUND AND STUDY AIMS: The advantage of endoscopic surveillance and treatment of duodenal polyposis is still unclear in familial adenomatous polyposis (FAP). The aim of this study was to evaluate the progression patterns of duodenal polyposis and the results of treatment. PATIENTS AND METHODS: In our institution, the 98 FAP patients included in the prospective follow-up study underwent at least one upper endoscopic examination, carried out with few exceptions, by a single surgeon endoscopist. The progression patterns of duodenal polyposis, the cumulative risk of severe duodenal polyposis and duodenal cancer as well as the results of surgical treatment were evaluated during a median follow-up of 11 years. RESULTS: Duodenal adenomas were detected in 78 patients corresponding to a cumulative lifetime incidence of 97%. The stage of adenomatosis progressed in 52 (73 %) of the 71 patients who underwent repeated endoscopies. The cumulative risks of stage IV adenomatosis and duodenal carcinoma were 30% and 4 %, respectively. Excisional treatment through open duodenotomy resulted in significant stage regression but was followed by new progression. In all patients the median interval for progression by one stage varied from 4 to 11 years. CONCLUSIONS: Duodenal adenomas almost invariably occur in FAP; endoscopic surveillance is thus warranted to anticipate severe progression and malignant transformation. Excisional surgical treatment can, however, give only transient stage reduction.

Fate of the rectal stump after colectomy and ileorectal anastomosis for familial adenomatous polyposis.

The justification of colectomy and ileorectal anastomosis as the primary treatment for familial adenomatous polyposis (FAP) remains questionable because of the rectal cancer risk. We estimated both the cancer risk and the need of rectal excision for benign polyposis in 100 FAP patients. We also evaluated the effects of sulindac therapy and the complications of polyp fulgurations during the follow-up time of the median of 10.6 years (2 to 29 years) after ileorectal anastomosis. There were 46 women and 54 men with a mean age of 32 years (17-67 years) at the operation. Forty-two patients were propositi and 15 had colon cancer primarily. Cumulative risk of rectal cancer and combined risk of cancer and rectal excision for other causes were estimated (Kaplan-Meier analysis) both from the date of surgery and from birth. Nine patients developed rectal cancer, while 12 others has the rectum excised for benign conditions. The cumulative rectal cancer risk was 4%, 5.6%, 7.9% and 25% at 5, 10, 15 and 20 years after the operation, respectively. Rectal excision rates were 7.3%, 13.7%, 23.6%, and 36.6%, correspondingly and finally 73.8%. Age-dependent rectal cancer risks were 3.9%, 12.8% and 25.7% at 40, 50 and 60 years, and the rectal excision rates 9.5%, 26.3% and 44%, respectively. Sulindac caused at least partial regression of rectal adenomas in 71% of patients without major adverse effects, but the long term effects of sulindac and impact on malignant transformation of rectal adenomas are not known. Our results favour proctocolectomy and ileonal anastomosis as the primary operation for FAP instead of colectomy and ileorectal anastomosis.

Occurrence of desmoid tumours in familial adenomatous polyposis and results of treatment.

Desmoids are of major concern in patients with familial adenomatous polyposis (FAP) besides the cancer risk. We estimated the risk for desmoid tumours and the results of their treatment in the Finnish Polyposis Registry. The analysis included 202 FAP patients, of whom 169 had undergone colectomy. Desmoids were observed in 29 cases: 15 in the mesentery, 10 in the abdominal wall and 4 in other sites. There were 12 male and 17 female patients with a mean age of 28.2 years (range 7 months to 59 years). The cumulative life-time risk was 21%; 1.5, 3.0, 8.9, 16 and 18% at ages of 10, 20, 30, 40 and 50 years, respectively. The risk of postcolectomy desmoids was 17% after ileorectal anastomosis and 12% after proctocolectomy. There were no deaths due to desmoids. One abdominal scar desmoid disappeared spontaneously and all the other abdominal wall desmoids could be excised without complications, but recurrence occurred in five (45%) cases. Excision was possible in only nine mesenteric desmoids (56%); in these cases recurrence was less common (two cases, 22%) but two others had life-threatening complications (bleeding, short bowel). We conclude that the desmoid problem concerns more than 20% of all FAP patients in long-term. Despite high recurrence rates and surgical hazards surgery remains a useful option for most desmoid tumours in FAP patients considering that other treatments are often ineffective or hazardous as well.