PC associates with severe primary hyperparathyroidism and must be suspected if calcium ≥1.77 mmol/l. The prevalence of CDC73 germline mutations in PC and APA in Finland is 6%. PC has distinct histopathological characteristics and its incidence has increased over the past decades.

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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.

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.

All patients with primary hyperparathyroidism should undergo localization studies before reoperation, but it is not known which method is most accurate. The purpose of this prospective study was to compare the performance of planar scintigraphy with 123 I/ 99m Tc-sestamibi, 99m Tc-sestamibi SPECT (SPECT/CT), 11 C-methionine PET/CT, and selective venous sampling (SVS) in persistent primary hyperparathyroidism. Methods: Twenty-one patients referred for reoperation of persistent hyperparathyroidism were included and investigated with 123 I/ 99m Tc-sestamibi, SPECT/CT (n 5 19), 11 C-methionine PET/CT, and SVS (n 5 18) before reoperation. All patients had been operated on 1-2 times previously because of hyperparathyroidism. The results of the localization studies were compared with operative findings, histology, and biochemical cure. Results: Eighteen (86%) of 21 patients were biochemically cured. Nineteen parathyroid glands (9 adenomas, 1 atypical adenoma, and 9 hyperplastic glands) were removed from 17 patients, and 1 patient who was biochemically cured had an unclear histology result. The accuracy for localizing a pathologic parathyroid gland to the correct side of the neck was 59% (95% confidence interval [CI], 36%-79%) for 123 I/ 99m Tc-sestamibi, 19% (95% CI, 5%-42%) for SPECT/CT, 65% (95% CI, 43%-84%) for 11 C-methionine PET/CT, and 40% (95% CI, 19%-65%) for SVS (P , 0.01 for 123 I/ 99m Tc-sestamibi vs. SPECT/ CT). The corresponding accuracy for the correct quadrant or more specific site was 48% (95% CI, 27%-69%) for 123 I/ 99m Tc-sestamibi, 14% (95% CI, 3%-36%) for SPECT/CT, 61% (95% CI, 39%-80%) for 11 C-methionine PET/CT, and 25% (95% CI, 9%-49%) for SVS (P , 0.02 for 123 I/ 99m Tc-sestamibi vs. SPECT/CT). In the 3 patients not cured, preoperative 123 I/ 99m Tc-sestamibi and SPECT/CT remained negative, SVS was false predictive in all, and 11 C-methionine PET/CT in 1. 11 C-methionine PET/CT accurately revealed the pathologic gland in 4 of 8 (50%) patients with a negative 123 I/ 99m Tc-sestamibi scan result, all of whom were biochemically cured after reoperation. Conclusion: Planar scintigraphy with 123 I/ 99m Tc-sestamibi performs well in complicated primary hyperparathyroidism and is recommended as first-line imaging before reoperation. 11 C-methionine PET/CT provides valuable additional information if 123 I/ 99m Tc-sestamibi scan results remain negative. 99m Tc-sestamibi SPECT/CT and SVS provide no additional information, compared with the combined results of 123 I/ 99m Tcsestamibi and 11 C-methionine PET/CT imaging.Key Words: primary hyperparathyroidism; persistent; reoperation; 123 I/ 99m Tc-sestamibi; SPECT-CT; 11 C-methionine PET/CT; selective venous sampling; PTH Nucl Med 2013; 54:739-747 DOI: 10.2967/jnumed.112.109561 Pri mary hyperparathyroidism is a common endocrine disease, with a peak incidence in women aged 50-60 y (1). It is explained by a single parathyroid adenoma in 80%-85% of patients, double adenomas in about 4%, and parathyroid hyperplasia in 15%-20% of cases. Parathyroid carcinoma is...

Objective: Endocrine Society guidelines recommend adrenal venous sampling (AVS) in primary aldosteronism (PA) if adrenalectomy is considered. We tested whether functional imaging of adrenal cortex with 11C-metomidate (11C-MTO) could offer a noninvasive alternative to AVS in the subtype classification of PA. Design: We prospectively recruited 58 patients with confirmed PA who were eligible for adrenal surgery. Methods: Subjects underwent AVS and 11C-MTO positron emission tomography (PET) without dexamethasone pretreatment in random order. The lateralization of 11C-MTO-PET and adrenal computed tomography (CT) were compared with AVS in all subjects and in a prespecified adrenalectomy subgroup in which the diagnosis was confirmed with immunohistochemical staining for CYP11B2. Results: In the whole study population, the concordance of AVS and 11C-MTO-PET was 51% and did not differ from that of AVS and adrenal CT (53%). The concordance of AVS and 11C-MTO-PET was 55% in unilateral and 44% in bilateral PA. In receiver operating characteristics analysis, the maximum standardized uptake value ratio of 1.16 in 11C-MTO-PET had an AUC of 0.507 (P=n.s.) to predict allocation to adrenalectomy or medical therapy with sensitivity of 55% and specificity of 44%. In the prespecified adrenalectomy subgroup, AVS and 11C-MTO-PET were concordant in 10 of 19 subjects with CYP11B2-positive adenoma and in 6 of 10 with CYP11B2-positivity without an adenoma. Conclusions: The concordance of 11C-MTO-PET with AVS was clinically suboptimal, and did not outperform adrenal CT. In a subgroup with CYP11B2-positive adenoma, 11C-MTO-PET identified 53% of cases. 11C-MTO-PET appeared to be inferior to AVS for subtype classification of PA.

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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.