A Nationwide Study of Multiple Endocrine Neoplasia Type 2A in Norway: Predictive and Prognostic Factors for the Clinical Course of Medullary Thyroid Carcinoma

Opsahl, Else Marie; Brauckhoff, Michael; Schlichting, Ellen; Helset, Kristin; Svartberg, Johan; Brauckhoff, Katrin; Mæhle, Lovise; Engebretsen, Lars; Sigstad, Eva; Grøholt, Krystyna; Akslen, Lars A.; Jørgensen, Lars; Varhaug, Jan Erik; Bjøro, Trine

doi:10.1089/thy.2015.0673

Cited by 36 publications

(41 citation statements)

References 188 publications

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“…Studies of hereditary MTC in Norway from overlapping time periods but with different objectives have been reported earlier [18, 19]. The present study aims to describe all patients with MTC (both sporadic and hereditary) in Norway from 1994 to 2016 and compare time-related trends in diagnostics and surgical treatment, including prognostic factors for biochemical cure and DSS.…”

Section: Introductionmentioning

confidence: 98%

Trends in Diagnostics, Surgical Treatment, and Prognostic Factors for Outcomes in Medullary Thyroid Carcinoma in Norway: A Nationwide Population-Based Study

Opsahl¹,

Akslen²,

Schlichting³

et al. 2018

Eur Thyroid J

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Background: Medullary thyroid carcinoma (MTC) is rare. Nationwide population-based studies are important to evaluate its clinical course. Objectives: To describe all patients with MTC in Norway during 1994–2016 and compare time-related trends in diagnostics and surgical treatment, including prognostic factors for biochemical cure and disease-specific survival (DSS). Methods: This retrospective population-based cohort study includes data for 228 out of 237 patients (96%) with MTC; 201 patients were surgically treated. Patients were identified in the 4 regional centers treating MTC and by the Cancer Registry of Norway. Data were collected from patients’ files. Trends were compared over 2 study periods. Results: MTC accounted for 4.2% of thyroid carcinomas. During the study periods, the incidence increased from 0.18 to 0.25: 100,000 per year, preoperative diagnostics improved with increased use of calcitonin, ultrasound, and fine-needle cytology (p = 0.010, p < 0,001, and p = 0.001), patients were diagnosed at an earlier tumor stage (p = 0.004), and more patients were cured (p = 0.002). Via multivariate analysis of patients with metastatic lymph nodes, independent prognostic factors for cure were: a low ratio of metastatic and total number of dissected lymph nodes (p = 0.021) and no extrathyroidal extension (p = 0.030). Independent prognostic factors for DSS were: no distant metastasis, a younger age, and a low ratio of metastatic and dissected lymph nodes (p = 0.005, p = 0.020, p = 0.022). Conclusions: Preoperative diagnostics have improved over time with increased therapeutic control. A low ratio of metastatic and dissected lymph nodes predicts better outcomes in patients with metastatic lymph nodes.

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Section: Introductionmentioning

confidence: 98%

Trends in Diagnostics, Surgical Treatment, and Prognostic Factors for Outcomes in Medullary Thyroid Carcinoma in Norway: A Nationwide Population-Based Study

Opsahl¹,

Akslen²,

Schlichting³

et al. 2018

Eur Thyroid J

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“…Este síndrome regularmente se acompaña de feocromocitoma y adenoma paratiroideo. Otras características que se pueden incluir son la enfermedad de Hirschsprung y el síndrome de Cushing 15 . Nosotros presentamos el caso de una paciente con el diagnóstico de síndrome de Sipple (neoplasia endocrina múltiple tipo IIA) asintomática con carcinoma medular de tiroides y feocromocitoma bilateral, con historia familiar de decesos por tumores de tiroides y suprarrenales, que fue tratada con fines curativos.…”

Section: Introductionunclassified

Neoplasia endocrina múltiple tipo IIa (síndrome de Sipple) de diagnóstico incidental con feocromocitoma bilateral y carcinoma medular de tiroides. Reporte de un caso

Robles-Pérez¹,

Córdova-López²,

Hernández-González³

et al. 2022

GAMO

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Gaceta Mexicana de Oncologia. 2017;16(5):295-298 Resumen La neoplasia endocrina múltiple tipo IIA, conocida también como síndrome de Sipple, es una entidad rara, de difícil sospecha diagnóstica, potencialmente mortal y ocasionada por abmutaciones en el protooncogén RET. La importancia de su diagnóstico y de la determinación de esta mutación en el paciente y en sus familiares consanguíneos radica en evitar la aparición de cáncer medular de tiroides mediante la extirpación profiláctica de esta glándula y el seguimiento ante la aparición de feocromocitoma o adenoma paratiroideo. Nosotros presentamos el caso de una paciente asintomática con mutación TGC634AGC-Cys634Arg, en la cual se diagnosticó carcinoma medular de tiroides, feocromocitoma bilateral, historia familiar de decesos por tumores de tiroides y suprarrenales, que fue tratada con fines curativos y en la que el diagnóstico se sospechó mediante estudios de tamizaje. (creativecommons.org/licenses/ by-nc-nd/4.0/). abstract Múltiple endocrine neoplasia IIA, also know as sipple syndrome, is a rare entity, difficult to diagnose and potentially fatal, caused by RET proto-oncogene mutations. The importance of diagnosis and determination of this mutation in the patient and their consanguineous relatives lies in avoiding the appearance of medullary thyroid cancer by prophylactic thyroidectomy and the follow up before development of pheochromocytoma and/or parathyroid adenoma. We present the case of an asymptomatic patient with a tail gut cyst 634 AGC (Cys 634 Arg) mutation who was diagnosed with medullary thyroid carcinoma, bilateral pheocromocytoma, family history of deaths from thyroid and adrenal tumors who was treated for curative purposes and in whom the diagnosis was suspected through screening studies. PalaBRaS ClaVENeoplasia endócrina múltiple tipo IIa; Carcinoma medular de tiroides; Feocromocitoma; Protooncogén RET KEy woRDSMultiple endocrine neoplasia IIa; Medullary thyroid carcinoma; Pheochromocytom; Proto-ongogen-RET

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“…This syndrome is regularly accompanied by pheochromocytoma and parathyroid adenoma. Other characteristics that may be included are Hirschsprung's disease and Cushing syndrome 15 .…”

Section: Introductionmentioning

confidence: 99%

Incidentally-Diagnosed Multiple Endocrine Neoplasia IIA (Sipple Syndrome) with Bilateral Pheochromocytoma and Medullary Thyroid Carcinoma

Robles-Pérez¹,

Córdova-López²,

Hernández-González³

et al. 2022

GAMO

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Multiple endocrine neoplasia IIA, also known as Sipple syndrome, is a rare entity that is difficult to diagnose and potentially fatal and is caused by RET proto-oncogene mutations. The importance of its diagnosis and determination of this mutation in the patient and his/her consanguineous relatives lies in avoiding the appearance of medullary thyroid cancer by means of prophylactic thyroidectomy and follow-up at the appearance of pheochromocytoma or parathyroid adenoma. We present the case of a female asymptomatic patient with the TGC34AGC-Cys634Arg mutation who was diagnosed with medullary thyroid carcinoma and bilateral pheochromocytoma, had a family history of thyroid and adrenal tumor-related deaths, was treated for curative purposes, and in whom the diagnosis was suspected by screening studies. (creativecommons.org/licenses/by-nc-nd/4.0/).

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A Nationwide Study of Multiple Endocrine Neoplasia Type 2A in Norway: Predictive and Prognostic Factors for the Clinical Course of Medullary Thyroid Carcinoma

Abstract: Preoperative basal calcitonin alone can serve as an indicator for optimal timing and the extent of thyroid surgery for MEN 2A patients that could be considered safe. The results are consistent with previously reported data.

Cited by 36 publications

References 188 publications

Trends in Diagnostics, Surgical Treatment, and Prognostic Factors for Outcomes in Medullary Thyroid Carcinoma in Norway: A Nationwide Population-Based Study

Trends in Diagnostics, Surgical Treatment, and Prognostic Factors for Outcomes in Medullary Thyroid Carcinoma in Norway: A Nationwide Population-Based Study

Neoplasia endocrina múltiple tipo IIa (síndrome de Sipple) de diagnóstico incidental con feocromocitoma bilateral y carcinoma medular de tiroides. Reporte de un caso

Incidentally-Diagnosed Multiple Endocrine Neoplasia IIA (Sipple Syndrome) with Bilateral Pheochromocytoma and Medullary Thyroid Carcinoma

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