| 1. |
- Brauckhoff, Michael, et al.
(författare)
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Premonitory symptoms preceding metastatic medullary thyroid cancer in MEN 2B : An exploratory analysis
- 2008
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Ingår i: SURGERY. - : Elsevier BV. - 0039-6060. ; 144:6, s. 1044-1050
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Tidskriftsartikel (refereegranskat)abstract
- Background. More than 90% of M918T carriers with multiple endocrine neoplasia type 2B (MEN 2B) harbor de novo mutations in the REarranged during Transfection (RET) protooncogene. DNA-based screening for RET germline mutations is rarely useful for early diagnosis, which thus is contingent on the clinical ascertainment of MEN 2B-specifitc symptoms as soon as they emerge. Little information exists about the presence of these symptoms in infancy.Methods. Detailed information was gathered regarding the development of MEN 2B-associated symptoms from the parents of 25 M918T RET carriers and 50 age- and sex-matched, controls with the use of a disease-specific questionnaire.Results. Until the end of the study, at a median age of 16 2 (range, 0.5-34.9 years), all 25 M918T RET carriers had developed medullary thyroid cancer. By that time, 96%, 91%, 71%, 75%, and 28% Of carriers displayed oral manifestations, ocular abnormalities, intestinal symptoms, musculoskeletal malformations, and pheochromocytoma, respectively. During the first year of life, fewer than 20% of carriers were found to express the typical MEN 2B phenotype, whereas 86% and 61% of these children, but none of the controls, were noted for their inability to cry tears and for constipation.Conclusion. Because the classic MEW 2B phenotype is rare during the first year of life, more emphasis should be placed on the more subtle features of the syndrome. Additional studies are needed to validate the usefulness of the symptoms "inability to cry" and "constipation" for earlier diagnosis of MEAT 2B.
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| 2. |
- Gimm, Oliver, et al.
(författare)
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Prognostic significance of disseminated tumor cells in the connective tissue of patients with medullary thyroid carcinoma.
- 2006
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Ingår i: World Journal of Surgery. - : Springer Science and Business Media LLC. - 0364-2313 .- 1432-2323. ; 30:5, s. 847-52
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Tidskriftsartikel (refereegranskat)abstract
- INTRODUCTION: Disseminated tumor cells in the connective tissue (CT-DTCs) do not have any connection to a primary tumor or the lymph nodes. They are identified quite often in patients with medullary thyroid carcinoma (MTC), but nothing is known regarding their prognostic significance.METHODS: Among 450 patients with MTC, 69 (15%) were identified as having CT-DTCs. A case-control group of patients without CT-DTCs was selected. The two groups were matched concerning TNM classification, age, heredity, and sex. Because many patients with CT-DTCs had extrathyroidal tumor extension (pT4 category), distant metastases (M1 category), or both, only 35 matched pairs could be identified. The TNM classification in both groups was as follows: pT1, n = 8; pT2, n = 15; pT3, n = 4; pT4, n = 8; pN0, n = 4; pN1, n = 31; M0, n = 30; M1, n = 5. The mean age was 46.8 +/- 17.0 years in the CT-DTC group and 44.4 +/- 15.0 years in the case-control group (NS).RESULTS: In both groups, 23 patients had sporadic MTC, and 12 patients had hereditary MTC. Neither mean basal preoperative nor postoperative calcitonin levels differed significantly between the two groups. In contrast, none of the patients with CT-DTCs was biochemically cured (normal calcitonin level after pentagastrin stimulation) compared to eight patients without CT-DTCs (P < 0.005). The two groups did not differ concerning other parameters (basal calcitonin level > 3000 pg/ml, more than 10 lymph node metastases, more than two involved locoregional lymph node compartments, mediastinal lymph node metastases) that have been reported to correlate with the lack of or almost (< 10%) lack of biochemical cure.CONCLUSIONS: In patients with MTC, disseminated tumor cells in the connective tissue correlate with advanced tumor stages and appear to be of prognostic significance.
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| 3. |
- Lorenz, Kerstin, et al.
(författare)
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Selective arterial chemoembolization for hepatic metastases from medullary thyroid carcinoma
- 2005
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Ingår i: Surgery. - : Elsevier. - 0039-6060 .- 1532-7361. ; 138:6, s. 986-993
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Hepatic metastases from medullary thyroid carcinoma (MTC) may impair quality of life by hypercalcitonemia-associated diarrhea and pain. In this prospective study, the effect of selective arterial chemoembolization (SACE) was evaluated.METHODS: Eleven patients with hepatic metastases from MTC received 1 to 9 courses of SACE using epirubicine. Symptomatic, biochemical, and morphologic responses on SACE were recorded.RESULTS: Symptomatic response was observed in all symptomatic patients. However, biochemical and radiologic response occurred only in 6 patients. Liver function was not affected by SACE. One patient with unexpected concurrent pheochromocytoma metastases died after the first course. Development of side effects in the course was observed in 8 patients but were only World Health Organization grade 1. Patients' satisfaction with SACE was excellent. Long-term follow-up found 7 patients alive (1-72 months). Three patients died with tumor 6, 12, and 24 months after SACE, respectively.CONCLUSION: SACE provided good symptom palliation for the majority of patients with hepatic metastases from MTC. However, transient remission or stabilization of hepatic metastases resulted in only 60%. Further studies using a randomized protocol are required.
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| 4. |
- Lorenz, Kerstin, et al.
(författare)
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Total parathyroidectomy without autotransplantation for renal hyperparathyroidism : experience with a qPTH-controlled protocol.
- 2006
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Ingår i: World Journal of Surgery. - : Springer Science and Business Media LLC. - 0364-2313 .- 1432-2323. ; 30:5, s. 743-51
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Controversy regarding the optimal surgical treatment for secondary hyperparathyroidism (sHPT) continues. Subtotal parathyroidectomy (PTX) with a small remnant and total parathyroidectomy with autotransplantation prevail, although impaired by considerable recurrence rates. Concerns about postoperative management and long-term supplementation prevent broader acceptance of total parathyroidectomy without autotransplantation.MATERIALS AND METHODS: The standardized surgical procedure with intraoperative PTH assessment (qPTH) included cervical thymectomy, histological proof of four parathyroid specimens and obligatory cryopreservation of parathyroid tissue in all 23 patients undergoing total PTX without autotransplantation. Whenever qPTH did not normalize, complete cervical exploration of ectopic sites was performed. Another 64 patients with subtotal PTX for sHPT served as comparison for the postoperative course.RESULTS: There were 13 primary and 10 completion (5 persistent, 5 recurrent sHPT) total PTX with 14 concurrent thyroid resections performed. Mean preoperative PTH was 1.351 pg/ml (12-72 pg/ml) and serum calcium was 2.5 mmol/l (2.25-2.5 mmol/l). PTH showed intraoperative normalization in 15 patients and a 50% PTH reduction from preoperative values in all. Postoperative course was not significantly different from the subtotal PTX group and showed PTH within the normal range for 5 patients (4 < 35 pg/ml), 7 with PTH < 12 pg/ml, and 4 without measurable PTH. In 4 patients PTH did not normalize postoperatively. Serum calcium levels were below normal in all patients: < 2.25 mmol/l in 9, < 2.00 mmol/l in 7, and <1.8 mmol/l in 6 patients. Only 1 patient required intermittent early postoperative i.v. calcium supplementation, 6 patients received oral calcium and vitamin D supplement for low calcium levels, but no severe hypocalcemic symptoms were encountered. Mean postoperative hospital stay was 5 days. No recurrent laryngeal nerve palsies were encountered. Complications were two cervical bleedings following postoperative hemodialysis requiring evacuation.CONCLUSIONS: Total PTX without autotransplantation proves to be an equally safe and successful procedure for sHPT as subtotal PTX or total PTX with autotransplantation. Measurable PTH after total PTX as demonstrated in this study, supports the idea of uncontrollable isolated cell nests that are inevitably prone to stimulated growth with time. Therefore, total PTX is superior with regard to prevention of recurrence. Adequate supplementation with calcium and vitamin D, often necessary after subtotal PTX to suppress inadequate PTH and protect from recurrence, will prevent severe hypocalcemia and with the modern aluminium-diminishing dialysis regimen, development of adynamic bone disease appears less likely than feared. If necessary, cryopreserved parathyroid tissue can be autotransplanted on demand.
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