H. Katoh, Sabine Kajita, Mitsuo Yokota, Norihiko Sengoku, T. Sangai
We report a 65-year old female with advanced papillary thyroid carcinoma involving the critical vessels including common carotid artery (CCA). She initially refused surgery and lenvatinib (LEN) was used in neoadjuvant setting. Primary tumor effectively shrank by LEN without any critical adverse effect. Total thyroidectomy and modified neck dissection were curatively performed. Tumor invading into internal jugular vein exhibited remarkable response to LEN and was almost replaced with necrosis and fibrosis. CCA was surrounded by fibrosis but was smoothly dissected from tumor and preserved. The patient shows no sign of recurrence for 2 years after surgery at present. Neoadjuvant LEN treatment can be new option for locally advanced papillary thyroid carcinoma involving critical vessels, particularly CCA, to reduce risk of surgical morbidity.
{"title":"Neoadjuvant use of lenvatinib in locally advanced papillary thyroid carcinoma involving critical vessels","authors":"H. Katoh, Sabine Kajita, Mitsuo Yokota, Norihiko Sengoku, T. Sangai","doi":"10.2217/IJE-2020-0014","DOIUrl":"https://doi.org/10.2217/IJE-2020-0014","url":null,"abstract":"We report a 65-year old female with advanced papillary thyroid carcinoma involving the critical vessels including common carotid artery (CCA). She initially refused surgery and lenvatinib (LEN) was used in neoadjuvant setting. Primary tumor effectively shrank by LEN without any critical adverse effect. Total thyroidectomy and modified neck dissection were curatively performed. Tumor invading into internal jugular vein exhibited remarkable response to LEN and was almost replaced with necrosis and fibrosis. CCA was surrounded by fibrosis but was smoothly dissected from tumor and preserved. The patient shows no sign of recurrence for 2 years after surgery at present. Neoadjuvant LEN treatment can be new option for locally advanced papillary thyroid carcinoma involving critical vessels, particularly CCA, to reduce risk of surgical morbidity.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41892754","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A 43-year old woman demonstrated an intra-abdominal drop metastasis 6 years after initial presentation of stage-III adrenocortical carcinoma (ACC), previously treated with resection, adjuvant radiation and mitotane therapy. This recurrence was managed aggressively with complete excision, adjuvant radiation and mitotane. Imaging at 3.25-year follow-up showed no evidence of recurrence, a remarkable response given ACC’s poor prognosis and high recurrence rates. Management of oligometastatic ACC remains unclear and intra-abdominal drop metastases are particularly rare in ACC; both require further documentation. Aggressive management with adjuvant radiation and mitotane therapy may represent an effective and well-tolerated approach for improving local control for recurrent ACC, including drop metastases. Further research is required to codify potential benefits.
{"title":"Recurrence-free survival following aggressive multimodal treatment of an isolated recurrence of adrenocortical carcinoma","authors":"A. Magony, A. Mutsaers, B. Ahmad","doi":"10.2217/IJE-2020-0015","DOIUrl":"https://doi.org/10.2217/IJE-2020-0015","url":null,"abstract":"A 43-year old woman demonstrated an intra-abdominal drop metastasis 6 years after initial presentation of stage-III adrenocortical carcinoma (ACC), previously treated with resection, adjuvant radiation and mitotane therapy. This recurrence was managed aggressively with complete excision, adjuvant radiation and mitotane. Imaging at 3.25-year follow-up showed no evidence of recurrence, a remarkable response given ACC’s poor prognosis and high recurrence rates. Management of oligometastatic ACC remains unclear and intra-abdominal drop metastases are particularly rare in ACC; both require further documentation. Aggressive management with adjuvant radiation and mitotane therapy may represent an effective and well-tolerated approach for improving local control for recurrent ACC, including drop metastases. Further research is required to codify potential benefits.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Lombard-Bohas, C. Cao, J. Metges, Philippe Ruszniewski, Denis Smith, R. Guimbaud, C. Lepage, Ron Hollander, G. Goldstein, E. Wolin, Alexandre Santos, E. Baudin
Background: There is a lack of knowledge regarding the experience of patients with neuroendocrine tumors (NET) in France. Materials & methods: A patient survey that captured information on diagnosis, disease impact/management and awareness was conducted. Data of respondents from France were analyzed and compared with US data as a reference. Results: Key topics included delays in diagnosis, negative impact on quality of life, patient access to NET medical experts and treatments, and information on NET and treatments. Significant differences were observed between France and the USA regarding NET diagnosis. Conclusion: This survey highlights the considerable burden experienced by patients in France with NET and differences in patient experience between France and the USA that may result from different healthcare and social systems.
{"title":"French patient-reported experience of diagnosis, management and burden of neuroendocrine tumors","authors":"C. Lombard-Bohas, C. Cao, J. Metges, Philippe Ruszniewski, Denis Smith, R. Guimbaud, C. Lepage, Ron Hollander, G. Goldstein, E. Wolin, Alexandre Santos, E. Baudin","doi":"10.2217/ije-2020-0013","DOIUrl":"https://doi.org/10.2217/ije-2020-0013","url":null,"abstract":"Background: There is a lack of knowledge regarding the experience of patients with neuroendocrine tumors (NET) in France. Materials & methods: A patient survey that captured information on diagnosis, disease impact/management and awareness was conducted. Data of respondents from France were analyzed and compared with US data as a reference. Results: Key topics included delays in diagnosis, negative impact on quality of life, patient access to NET medical experts and treatments, and information on NET and treatments. Significant differences were observed between France and the USA regarding NET diagnosis. Conclusion: This survey highlights the considerable burden experienced by patients in France with NET and differences in patient experience between France and the USA that may result from different healthcare and social systems.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47768633","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bad kits in the diagnosis of endocrine tumors","authors":"J. Rehfeld","doi":"10.2217/ije-2020-0003","DOIUrl":"https://doi.org/10.2217/ije-2020-0003","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47528314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rui Zheng-Pywell, A. Cherian, Macie Enman, Herbert Chen, D. Abraham
Aim: This study investigates if serum calcitonin or carcinoembryonic antigen (CEA) levels can differentiate between locoregional and metastatic medullary thyroid cancer. Methods: A single institution retrospective analysis was performed on 88 patients with medullary thyroid cancer between 2008 and 2014. Results: In M0disease, calcitonin (p < 0.001) and CEA (p = 0.003) significantly decreased postoperatively. Not only was the correlation significant between calcitonin and CEA preoperatively (r = 0.72; p < 0.001) and postoperatively (r = 0.68; p < 0.001), calcitonin could extrapolate CEA levels (p < 0.001). These findings were statistically insignificant in metastatic disease. Conclusion: Independently, calcitonin and CEA fail to differentiate between locoregional and metastatic disease. Both are essential for prognostication: loss of concordance is suspicious for metastatic disease. Hence, discordant CEA and calcitonin levels should be an indication to pursue additional imaging.
{"title":"Carcinoembryonic antigen should be concurrently checked with calcitonin to identify distant metastases in medullary thyroid cancer","authors":"Rui Zheng-Pywell, A. Cherian, Macie Enman, Herbert Chen, D. Abraham","doi":"10.2217/ije-2019-0016","DOIUrl":"https://doi.org/10.2217/ije-2019-0016","url":null,"abstract":"Aim: This study investigates if serum calcitonin or carcinoembryonic antigen (CEA) levels can differentiate between locoregional and metastatic medullary thyroid cancer. Methods: A single institution retrospective analysis was performed on 88 patients with medullary thyroid cancer between 2008 and 2014. Results: In M0disease, calcitonin (p < 0.001) and CEA (p = 0.003) significantly decreased postoperatively. Not only was the correlation significant between calcitonin and CEA preoperatively (r = 0.72; p < 0.001) and postoperatively (r = 0.68; p < 0.001), calcitonin could extrapolate CEA levels (p < 0.001). These findings were statistically insignificant in metastatic disease. Conclusion: Independently, calcitonin and CEA fail to differentiate between locoregional and metastatic disease. Both are essential for prognostication: loss of concordance is suspicious for metastatic disease. Hence, discordant CEA and calcitonin levels should be an indication to pursue additional imaging.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2019-0016","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46862057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pituitary adenomas are now described as pituitary neuroendocrine tumors (PitNets) [1]. A large proportion, approximately 22–54%, is clinically defined as nonfunctioning pituitary adenomas and will present with signs and symptoms of mass effect, rather than excessive hormone secretion. ‘Silent pituitary adenomas’ are tumors that express one or more of the anterior pituitary hormones or their transcription factors, which can be visualized by immunohistochemical analysis. Although, they do not secrete hormones at a clinically relevant level. Silent pituitary adenomas can be further sub categorized into totally silent or clinically silent tumors [2]. A null cell adenoma that is restricted to a primary anterior pituitary tumor, is hormone negative (determined by immunohistochemistry) and does not express any of the pituitary transcription factors. The clinical picture of pituitary adenomas reflects a continuum between functional adenomas and ‘totally silent’ adenomas. The functional status of the tumor can change throughout the disease course [3]. Somatotropic tumors are growth hormone (GH) producing tumors. These account for approximately 20% of surgically treated pituitary tumors and more than 95% of cases of acromegaly. Very rare cases of acromegaly are due to an excess of the GH-releasing hormone (GHRH). This may be associated with neuroendocrine tumors of lung, pancreas, medullary thyroid cancer, pheochromocytomas and hypothalamic gangliocytomas. Ectopic production of GH has been reported from rare cases of pancreatic neuroendocrine tumor or lymphoma [4,5]. Many familial syndromes have been reported to predispose to acromegaly or gigantism. These include multiple MEN1 and MEN4, familial isolated pituitary adenoma and Carney complex. A sporadic germline mosaic disorder McCune-Albright disease can also result in GH excess [6]. A rare genetic syndrome, X-linked acrogigantism has been described in early-onset childhood gigantism [7]. In familial cases, onset is in young age with florid presentation due to high GH levels. The response to medical treatment is poor [7]. Silent somatotroph adenomas lack clinical and biological signs of acromegaly but are GH-immunoreactive tumors. They represent approximately 2–4% of all pituitary adenomas in surgical series. Patients with truly silent somatotroph adenomas have normal preoperative GH and IGF-1 levels; some cases can be clinically silent but demonstrate a lack of GH suppression and elevated IGF-1 levels (whispering adenomas) [8]. Growth hormone excess can be due to pure somatotroph adenomas and these can be densely granulated (DGSA) or sparsely granulated (SGSA). DGSA are present in 30–50% of acromegaly cases. The cells are eosinophilic and are strongly as well as diffusely positive for GH and α-subunits. They resemble normal somatotrophes. DGSA is usually present in older patients and are slow-growing lesions. Patients have typical features of acromegaly and high levels of GH and IGF-1 and imaging demonst
{"title":"Somatotroph adenomas: histological subtypes and predicted response to treatment","authors":"G. Bano","doi":"10.2217/ije-2020-0002","DOIUrl":"https://doi.org/10.2217/ije-2020-0002","url":null,"abstract":"Pituitary adenomas are now described as pituitary neuroendocrine tumors (PitNets) [1]. A large proportion, approximately 22–54%, is clinically defined as nonfunctioning pituitary adenomas and will present with signs and symptoms of mass effect, rather than excessive hormone secretion. ‘Silent pituitary adenomas’ are tumors that express one or more of the anterior pituitary hormones or their transcription factors, which can be visualized by immunohistochemical analysis. Although, they do not secrete hormones at a clinically relevant level. Silent pituitary adenomas can be further sub categorized into totally silent or clinically silent tumors [2]. A null cell adenoma that is restricted to a primary anterior pituitary tumor, is hormone negative (determined by immunohistochemistry) and does not express any of the pituitary transcription factors. The clinical picture of pituitary adenomas reflects a continuum between functional adenomas and ‘totally silent’ adenomas. The functional status of the tumor can change throughout the disease course [3]. Somatotropic tumors are growth hormone (GH) producing tumors. These account for approximately 20% of surgically treated pituitary tumors and more than 95% of cases of acromegaly. Very rare cases of acromegaly are due to an excess of the GH-releasing hormone (GHRH). This may be associated with neuroendocrine tumors of lung, pancreas, medullary thyroid cancer, pheochromocytomas and hypothalamic gangliocytomas. Ectopic production of GH has been reported from rare cases of pancreatic neuroendocrine tumor or lymphoma [4,5]. Many familial syndromes have been reported to predispose to acromegaly or gigantism. These include multiple MEN1 and MEN4, familial isolated pituitary adenoma and Carney complex. A sporadic germline mosaic disorder McCune-Albright disease can also result in GH excess [6]. A rare genetic syndrome, X-linked acrogigantism has been described in early-onset childhood gigantism [7]. In familial cases, onset is in young age with florid presentation due to high GH levels. The response to medical treatment is poor [7]. Silent somatotroph adenomas lack clinical and biological signs of acromegaly but are GH-immunoreactive tumors. They represent approximately 2–4% of all pituitary adenomas in surgical series. Patients with truly silent somatotroph adenomas have normal preoperative GH and IGF-1 levels; some cases can be clinically silent but demonstrate a lack of GH suppression and elevated IGF-1 levels (whispering adenomas) [8]. Growth hormone excess can be due to pure somatotroph adenomas and these can be densely granulated (DGSA) or sparsely granulated (SGSA). DGSA are present in 30–50% of acromegaly cases. The cells are eosinophilic and are strongly as well as diffusely positive for GH and α-subunits. They resemble normal somatotrophes. DGSA is usually present in older patients and are slow-growing lesions. Patients have typical features of acromegaly and high levels of GH and IGF-1 and imaging demonst","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2020-0002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42628177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aim: Hyperfunctioning intrathyroidal parathyroid glands are rare and often result in thyroid lobectomy. This study examines the utility of radioguided surgery to guide enucleation of intrathyroidal parathyroids. Methods: Between December 2002 and March 2018, 2291 patients underwent parathyroidectomy by one surgeon for primary hyperparathyroidism. A total of 74 (3%) patients had an ectopic intrathyroidal parathyroid gland and underwent radioguided. Results: All of intrathyroidal parathyroid glands were localized with the gamma probe. In vivo radionuclide counts were above 120% of the background in all but three patients. All intrathyroidal parathyroids were enucleated with the guidance of the gamma probe. Conclusion: Radioguided surgery is useful for intraoperative identification of hyperfunctioning, intrathyroidal parathyroid glands. This technique allows for enucleation of the abnormal parathyroid gland, avoiding thyroid lobectomy and preserving healthy thyroid parenchyma.
{"title":"Preventing blind thyroid lobectomy in patients with intrathyroidal hyperfunctioning parathyroid glands with radioguided enucleation","authors":"S. Dream, Brenessa Lindeman, Herbert Chen","doi":"10.2217/ije-2019-0013","DOIUrl":"https://doi.org/10.2217/ije-2019-0013","url":null,"abstract":"Aim: Hyperfunctioning intrathyroidal parathyroid glands are rare and often result in thyroid lobectomy. This study examines the utility of radioguided surgery to guide enucleation of intrathyroidal parathyroids. Methods: Between December 2002 and March 2018, 2291 patients underwent parathyroidectomy by one surgeon for primary hyperparathyroidism. A total of 74 (3%) patients had an ectopic intrathyroidal parathyroid gland and underwent radioguided. Results: All of intrathyroidal parathyroid glands were localized with the gamma probe. In vivo radionuclide counts were above 120% of the background in all but three patients. All intrathyroidal parathyroids were enucleated with the guidance of the gamma probe. Conclusion: Radioguided surgery is useful for intraoperative identification of hyperfunctioning, intrathyroidal parathyroid glands. This technique allows for enucleation of the abnormal parathyroid gland, avoiding thyroid lobectomy and preserving healthy thyroid parenchyma.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2019-0013","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48038269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Welcome to Volume 7 of the International Journal of Endocrine Oncology","authors":"Lauren Woolfe","doi":"10.2217/ije-2020-0001","DOIUrl":"https://doi.org/10.2217/ije-2020-0001","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2020-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2020-0001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47025482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dr Hammer is the cofounder of Millendo Therapeutics (NASDAQ, MLND, MI, USA) and the founder of Vasaragen (MI, USA), two biotechnology companies focused on rare endocrine diseases. Hammer is also an employee of the University of Michigan.
{"title":"An interview with Gary D Hammer, MD, PhD","authors":"G. Hammer","doi":"10.2217/ije-2019-0017","DOIUrl":"https://doi.org/10.2217/ije-2019-0017","url":null,"abstract":"Dr Hammer is the cofounder of Millendo Therapeutics (NASDAQ, MLND, MI, USA) and the founder of Vasaragen (MI, USA), two biotechnology companies focused on rare endocrine diseases. Hammer is also an employee of the University of Michigan.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2019-0017","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47202612","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Therapy with tyrosine kinase inhibitor lenvatinib in radioactive iodine-naive advanced differentiated thyroid cancer","authors":"J. Sukumar, W. Moore, S. Khan","doi":"10.2217/ije-2019-0007","DOIUrl":"https://doi.org/10.2217/ije-2019-0007","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2019-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2019-0007","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43183667","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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