Recurrence-free survival following aggressive multimodal treatment of an isolated recurrence of adrenocortical carcinoma

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.

Neoadjuvant use of lenvatinib in locally advanced papillary thyroid carcinoma involving critical vessels

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.

French patient-reported experience of diagnosis, management and burden of neuroendocrine tumors

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.

Carcinoembryonic antigen should be concurrently checked with calcitonin to identify distant metastases in medullary thyroid cancer

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.

Preventing blind thyroid lobectomy in patients with intrathyroidal hyperfunctioning parathyroid glands with radioguided enucleation

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.

Gary D Hammer on the improvement of patient care in endocrine neoplasia

Dr Gary D 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.