RET T244I Germline Variant Mutation in a Patient with Pancreatic Paraganglioma and Primary Hyperparathyroidism.

Introduction: Paragangliomas are rare neuroendocrine tumors that arise from chromaffin cells. Often termed extra-adrenal pheochromocytomas, these tumors vary with regards to their functionality, location, and malignant potential. Mutations in the RET proto-oncogene are associated with multiple endocrine neoplasia syndrome type 2 (MEN-2) and paragangliomas. The phenotypes of the individual mutations are documented to help determine prognosis. Case Presentation: We report a case of a 64-year-old man with a history of parathyroid adenoma who developed a pancreatic retroperitoneal paraganglioma. Despite having laboratory evidence of excess circulating catecholamines, the patient's only presenting symptom was hip pain. The patient underwent resection, and histologic findings were consistent with paraganglioma with lymph node metastasis. Genetic testing revealed a variant of uncertain significance within the RET gene [c.731C>T (p.T244I)]. Conclusions: Paragangliomas are rare extra-adrenal neuroendocrine tumors that can be associated with germline mutations. Our patient was diagnosed with a pancreatic paraganglioma associated with a RET T244I mutation. Identifying patients with germline mutations is important for documenting phenotypic presentations of RET gene variants of uncertain significance, which will allow physicians to provide proper management and surveillance of paragangliomas and other associated tumors.

Contemporary Experience of Posterior Retroperitoneoscopic Adrenalectomy in the US.

BACKGROUND: As the incidence of adrenalectomy increases steadily, so does the use of minimally invasive approaches like posterior retroperitoneoscopic adrenalectomy (PRA). To date, the largest studies of PRA have been from abroad, and we sought to provide a contemporary US update on the outcomes after PRA. METHODS: A retrospective chart review was conducted on all PRAs performed at a single tertiary care institution between 2013 and 2020. Patient demographic characteristics, indication for operation, operative details, and postoperative course were abstracted. Outcomes of interest included 30-day mortality, conversion to open or transabdominal approach, postoperative complication, and 30-day readmission. RESULTS: A total of 249 PRAs were performed between 2013 and 2020. The population was 54.2% women and mean (SD) age was 54.1 (14.1) years. Most lesions (60.6%) were left-sided, and the most common diagnosis was nonfunctioning adenoma (39.4%), followed by pheochromocytoma (21.3%) and aldosteronoma (16.6%). Mean (SD) tumor size was 3.2 cm (range 0.5 to 9.4 cm). Median operative length was 110 minutes (range 30 to 319 minutes). Overall, the complication rate was 6.4%. Nine patients (3.6%) had a minor postoperative complication (Clavien-Dindo I to III) and 5 patients (2.0%) had a major postoperative complication (Clavien-Dindo IV to V), including 1 mortality (0.4%). There were 2 conversions of approach (0.8%). The majority of patients (58.2%) were discharged on postoperative day 1, and 92.0% were discharged by postoperative day 3. The 30-day readmission rate was 1.6%. CONCLUSIONS: Current practice demonstrates that PRA is an extremely safe approach, with a complication rate < 7% and mortality rate < 1%. In addition, the vast majority of patients are able to return home in an expedient manner.

Diffuse large B-cell and follicular lymphoma presenting as a slowly growing compressive goiter: A case report and literature review.

INTRODUCTION: Neck ultrasonography with fine-needle aspiration cytology (FNAC) is the diagnostic modality of choice for clinicians who routinely work up a thyroid mass. Distinguishing chronic lymphocytic infiltration from a lymphoproliferative process with FNAC in patients with Hashimoto's thyroiditis presenting with a goiter can be particularly challenging. CASE DESCRIPTION: A 58 y.o. female with a history of a goiter showing interval growth and compressive symptoms over 18 months, was treated with a thyroid lobectomy. Surgical pathology demonstrated a thyroid lymphoma (TL) with mixed follicular and diffuse large B cell (DLBCL) components, not initially diagnosed by FNAC. Staging workup showed the involvement of chest lymph nodes only, consistent with Stage IIE disease. She was treated with combination chemotherapy and immunotherapy, followed by involved-field radiotherapy. DISCUSSION: TL often arises in a background of chronic lymphocytic thyroiditis which can make its histological diagnosis a challenge. The disease is heterogeneous in histological subtype and progression. CONCLUSION: While TL usually presents as a rapidly growing neck mass, indolent types can present as a slow growing mass with subsequent transformation. Patients may benefit from avoiding unnecessary diagnostic steps, including surgery, and potential delays in treatment by performing a core needle biopsy when a lymphoproliferative process cannot be excluded if FNAC was initially performed.

Lobectomy Compared to Total Thyroidectomy for Low-Risk Papillary Thyroid Cancer: A Systematic Review.

BACKGROUND: The 2015 American Thyroid Association (ATA) guidelines called for consideration of thyroid lobectomy (TL) as an acceptable surgical treatment for small and less aggressive papillary thyroid cancers (PTC) with no clinical evidence of metastasis or extrathyroidal extension. Optimal extent of surgery, however, remains controversial. METHODS: A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. PUBMED, EMBASE, Scopus, and Cochrane Library databases were searched to identify studies comparing TL to total thyroidectomy (TT) for low-risk PTC. Studies were grouped according to the major outcomes in the literature: survival and the need for completion thyroidectomy (CT). RESULTS: Overall survival for low-risk PTC patients who underwent TL was comparable to TT. Locoregional recurrence (LRR) rate following TL was less than 6% and salvaged with CT. The proportion of patients meeting the 2015 ATA guidelines selection criteria for TL who subsequently would need CT varied by study but averaged 34%. After excluding microscopic extrathyroidal extension and positive resection margin as indications for CT to facilitate radioactive iodine ablation, the estimated rate across the included studies was 11%. CONCLUSIONS: We performed a systematic review of outcomes following TL or CT for low-risk PTC according to 2015 ATA guidelines. Initial operative approach did not have a negative impact on overall survival. There is a paucity of high-quality data on this topic across the literature. Long-term follow-up studies on oncologic and patient-centered outcomes are essential.

Anterior-commissure laryngoscope extraction of esophageal coins in children using an apnea technique.

This is a case series with chart review of 59 consecutive pediatric patients with a diagnosis of cervical esophageal coin who underwent anterior-commissure laryngoscope (ACLA) extraction during apnea. The purpose of this study was (1) to evaluate the efficacy and safety of coin extraction and (2) to analyze foreign body features and intraoperative physiological parameters (apnea time, O2 saturation and end-tidal CO2 (ETCO2) of apnea, minimum O2 during procedure, and heart rate). The technique was completed in 94.9% of the sample. The mean of the length of apnea was 57.7 ± 25.2 seconds. The median minimum O2 saturation was 99.5% (minimum = 93, maximum = 100), and the median of the ETCO2 at the end of the procedure was 35.7 ± 4.8 mm Hg. Heart rates remained at baseline values during the procedure (P < .001). This technique represents an efficient and secure modality for treatment. If successful, the patient can be safely discharged after clearance from anesthesia and a swallowing trial.