Citations

Summary We report the case of a male patient with papillary thyroid cancer, familial thoracic aortic aneurysm and dissection, and a variation in the MYH11 gene. Because of considerable tumor bulk in the neck that was not resectable, the patient underwent partial resection at age 14 years. Since then, the patient has received only suppressive thyroid hormone therapy. He is now 71 years old, which is 57 years after the initial resection. The patient received care at our institution from July 2009 to August 2019, during which we documented the stability of multiple calcified masses in the neck. Follow-up examinations at another institution from September 2019 to April 2023 also confirmed the stability of the masses. The underlying cause of this unusually long indolent course of the disease is unclear. Whether extensive tumor calcifications or the MYH11 sequence variation contributed to the disease course is also uncertain. Learning points Papillary thyroid cancer with neck metastases may, in some cases, be stable and remain asymptomatic for decades. If locoregional stability of papillary thyroid cancer is documented for many years, observation may be preferable to extensive neck surgery in selected cases. This is the first report of an MYH11 gene alteration and thoracic aortic aneurysm in a patient with papillary thyroid cancer with indolent neck metastases. Future studies of MYH11 gene alterations in thyroid carcinoma are needed.

AbstractLymphangioleiomyomatosis (LAM) is a rare disease with variable presentations. The neoplastic cells in LAM demonstrate a unique and diagnostically important “myomelanocytic” phenotype. Cytologic reports of LAM are infrequent and have not in the past emphasized the floating island pattern in which circumscribed aggregates of lesional cells are rimmed by appliques of flattened endothelium. This case illustrates the cytology of LAM and emphasizes that the floating island cytoarchitectural pattern more classically associated with entities such as hepatocellular carcinomas may be seen in cytological preparations of LAM at unanticipated body sites.

The published literature on cervicovaginal cytology includes fewer than ten reported cases of Cokeromyces recurvatus identified in Pap test samples. We report a unique case of an asymptomatic 27-year-old female with persistent gynecologic tract colonization by C. recurvatus in which distinctive fungal microorganisms were identified in three samples collected over three consecutive years.

Abstract ALK-positive histiocytosis is a rare subtype of histiocytic neoplasm first described in 2008 in 3 infants with multisystemic disease involving the liver and hematopoietic system. This entity has subsequently been documented in case reports and series to occupy a wider clinicopathologic spectrum with recurrent KIF5B-ALK fusions. The full clinicopathologic and molecular spectra of ALK-positive histiocytosis remain, however, poorly characterized. Here, we describe the largest study of ALK-positive histiocytosis to date, with detailed clinicopathologic data of 39 cases, including 37 cases with confirmed ALK rearrangements. The clinical spectrum comprised distinct clinical phenotypic groups: infants with multisystemic disease with liver and hematopoietic involvement, as originally described (Group 1A: 6/39), other patients with multisystemic disease (Group 1B: 10/39), and patients with single-system disease (Group 2: 23/39). Nineteen patients of the entire cohort (49%) had neurologic involvement (7 and 12 from Groups 1B and 2, respectively). Histology included classic xanthogranuloma features in almost one-third of cases, whereas the majority displayed a more densely cellular, monomorphic appearance without lipidized histiocytes but sometimes more spindled or epithelioid morphology. Neoplastic histiocytes were positive for macrophage markers and often conferred strong expression of phosphorylated extracellular signal-regulated kinase, confirming MAPK pathway activation. KIF5B-ALK fusions were detected in 27 patients, whereas CLTC-ALK, TPM3-ALK, TFG-ALK, EML4-ALK, and DCTN1-ALK fusions were identified in single cases. Robust and durable responses were observed in 11/11 patients treated with ALK inhibition, 10 with neurologic involvement. This study presents the existing clinicopathologic and molecular landscape of ALK-positive histiocytosis and provides guidance for the clinical management of this emerging histiocytic entity.

Abstract In 2016, we established the year-long Society of Behavioral Medicine (SBM) Mid-Career Leadership Institute. Individuals are often selected for leadership positions without intentional training in needed leadership skills, including strategic planning, building collaborative teams, goal setting, negotiation, and communication. The purpose of the Leadership Institute is to: (a) provide opportunities for mid-career professionals to build and sustain their leadership capacity; (b) create cohorts of connected fellows in behavioral medicine fields, disciplines, and institutions, who can support one another throughout their professional careers; and (c) enhance specific skills needed to navigate the challenges of mid-career. Over the first 4 years of the Institute, 139 fellows have participated, representing 35 states. Most of the fellows hold PhDs (93%) as a terminal degree. This special issue is dedicated to the leadership experiences of fellows, faculty, senior SBM mentors, peer mentors, and executive coaches.

Abstract In 2015, the Society of Behavioral Medicine (SBM) created the Mid-Career Leadership Institute. The initial model that motivated the creation of the institute came from my participation in the Hedwig van Ameringen Executive Leadership in Academic Medicine (ELAM) program, specifically designed to enhance the leadership skills of senior female faculty in the health sciences. My participation in ELAM was transformative. It led me to run for president of SBM in 2014, to consider how behavioral scientists could similarly benefit from mid-career leadership training, and ultimately to work with the SBM Executive Director, the SBM Leadership Institute Steering Committee, and leadership training professionals to launch the Leadership Institute in 2016. The overarching goal of the SBM Leadership Institute is to train mid-career behavioral scientists engaged in academic and health care settings to develop essential skills needed to navigate the unique challenges of mid-career. Central to this goal is a focus on integrating diversity, equity, and inclusion into their emerging leadership roles through participation in training exercises, lectures, and dynamic group discussions. To optimize the Leadership Institute experience, opportunities were developed, including mentoring from SBM seasoned members, peer mentoring through the creation of “learning communities,” career coaching, and additional networking opportunities. The purpose of this brief commentary is to summarize lessons learned by highlighting program evaluation, describing key changes over the initial 4 years, and sharing future plans for leadership training.

Abstract We present a case report of a 25 year-old man with MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes) syndrome, who died suddenly and unexpectedly from diabetic ketoacidosis. This case report illustrates why it is important for medical examiners to be familiar with the clinical and autopsy features of MELAS syndrome and to be aware of the common complications, which may lead to sudden unexpected death.