Clinical Significance of Pheochromocytoma Size on the Timing and Extent of Surgery.

INTRODUCTION: Elevated metanephrine and catecholamine levels 3-fold upper limit of normal (ULN) are diagnostic for pheochromocytoma. We sought to determine whether size correlates with biochemical activity or symptoms which could guide timing of surgery. METHODS: Data from consecutive patients undergoing adrenalectomy for pheochromocytoma at our institution over a 10-year period were retrospectively collected. These included maximal lesion diameter on preoperative imaging, plasma/urine metanephrine and/or catecholamine levels, demographic variables and presence of typical paroxysmal symptoms. Receiver operating characteristic curves were used to assess predictive accuracy. RESULTS: Sixty-three patients were included in the analysis (41 females and 22 males). Median age was 56 (43, 69) years. Due to various referring practices, 31 patients had documented 24-h urine metanephrine, 26 had 24-h urine catecholamine, and 52 had fractionated plasma metanephrine levels available for review. Values were converted to fold change compared to ULN and the maximum of all measured values was used for logistic regression. Median tumor size was 3.40 (2.25, 4.55) cm in greatest dimension. Tumor size at which pheochromocytoma produced > 3-fold ULN was ≥2.3 cm (AUC of 0.84). Biochemical activity increased with doubling tumor size (odds ratio = 8, P = 0.0004) or ≥ 1 cm increase in tumor size (odds ratio = 3.03, P = 0.001). 40 patients had paroxysmal symptoms, but there was no significant correlation between tumor size/biochemical activity and symptoms. CONCLUSIONS: In our study, tumor size directly correlated with the degree of biochemical activity and pheochromocytomas ≥2.3 cm produced levels 3 times ULN. These findings may allow clinicians to adjust timing of operative intervention.

Medullary Thyroid Cancer: Single Institute Experience over Three Decades and Risk Factors for Recurrence.

CONTEXT: Medullary thyroid cancer has a historic recurrence rate up to 50%, and surgery remains the only cure. OBJECTIVE: This study aims to assess factors related to recurrence and metastatic spread in MTC. DESIGN: Retrospective chart review was performed from 1990-2023. Descriptive analysis and regression models were used for analysis. SETTING: Single specialized tertiary care referral center. PATIENTS: 68 patients with MTC, who underwent surgery, were included. MAIN OUTCOME MEASURE: Recurrence. RESULTS: Mean age at diagnosis was 54.9years(42.2-64.1), 65%(n=44) females. Lymph node and distant metastases were found in 24%(n=16) and 4%(n=3), respectively. RET mutations were present in 52%(n=35): MTC risk levels Highest 6%, High 7%, and Moderate 39%. Mean tumor size was 1.9cm(1.2-3.2) and mean preoperative calcitonin was 504.4pg/mL(133.2-1833.8). Total thyroidectomy(TT) was performed in 10 patients, TT+central neck dissection(CND) in 28, and TT+CND+lateral neck dissection(LND) in 25. On final pathology, 40% had positive central nodes and 25% had positive lateral nodes. Recurrence was 22%, median follow-up 4.7years(1.2-28.0). Male gender(HR=5.81, p=0.021), positive lateral neck nodes(HR 8.10, p=0.011) and high/highest MTC risk level RET mutations(HR 8.66, p=0.004) were significantly associated with recurrence. Preoperative calcitonin>2,175 pg/mL was a strong predictor for distant metastasis(AUC0.893) and a good predictor for lateral neck disease(AUC0.706). Extent of surgery was not significantly associated with recurrence(p=0.634). CONCLUSION: One of 4 patients undergoing surgery for MTC will recur. Risk factors associated with recurrence are male gender, lateral LN metastasis and high/highest MTC risk level mutations, but not necessarily surgery type. Preoperative calcitonin>2,175 pg/mL is suggestive of advanced disease and should prompt further evaluation.

Decision-Making in Surgery.

Surgeons face unique challenges in perioperative decision-making and communication with patients and families. In cardiothoracic surgery, the stakes are high, life and death decisions must be made quickly, and surgeons often lack a longstanding relationship with patients and families prior to intervention. This review considers specific challenges in the preoperative period followed by those faced postoperatively. While preoperative deliberation and informed consent focus on reaching a decision between 2 or more alternative approaches, the most vexing postoperative decisions often involve the patient's discontent with the best-case outcome or how to ensure goal-concordant care when complications arise. This review explores the preoperative ethical and legal requirement for informed consent by describing the contemporary preferred method, shared decision-making. We also present a framework to optimize surgeon communication and promote patient and family engagement in the setting of high-risk surgery for older patients with serious illness. In the postoperative period the family is often tasked with deciding what to do about major complications when the patient has lost decision-making capacity. We discuss several examples and offer strategies for surgeons to navigate these challenging situations. We also explore the concepts of clinical heroism and futility in relation to communicating with patients and families about the outcomes of surgery. Persistent ethical challenges in decision-making suggest that surgeons should improve their skills in communicating with patients to better engage with them, both before and after surgery.

Parathyroid Allotransplantation: Report of Outcomes in 3 Patients.

BACKGROUND: Hypoparathyroidism is a relatively rare endocrine disorder defined as inadequate parathyroid hormone (PTH) secretion leading to a clinical syndrome characterized by hyperphosphatemia and hypocalcemia. This condition has high morbidity; patients present with a heterogeneous range of emotional, mental, and physical symptoms. We present our experience with PTH transplantation, using parathyroid glands surgically removed in the setting of secondary hyperparathyroidism, with a description of the clinical course, immunosuppressive management, and surgical technique. METHODS: Between 2017 and 2021, 3 patients underwent parathyroid allotransplantation at the University of Illinois at Chicago. The 2 outcomes of interest were (1) symptomatic relief and improvement in calcium levels and (2) time to graft failure, defined as the presence of undetectable PTH levels. RESULTS: All 3 patients experienced dramatic improvement in their debilitating symptoms, even though 2 patients required repeated PTH transplantation procedures. One patient had a remarkable course with symptom resolution, normalization of PTH levels, and a great reduction in calcium supplementation. CONCLUSION: The use of hyperplastic glands from patients with secondary hyperparathyroidism undergoing 4-gland parathyroidectomy with autotransplantation represents an important source. However, a uniform definition of graft viability and prospective studies with long follow-ups are needed to address how much parathyroid tissue is optimally transplanted and the need for immunosuppression. Most patients affected by hypoparathyroidism are successfully managed by medical treatment; however, some do not respond to therapy and present debilitating symptoms related to hypocalcemia. This subgroup may benefit from parathyroid allotransplantation. Our 3 patients had remarkable improvement in their symptoms with the adoption of hyperplastic glands. Two out of 3 patients required multiple procedures to sustain symptom control.

Severe Hypercalcemia Due to Primary Hyperparathyroidism and Heterozygous Pathogenic Variant of CYP24A1.

Pathogenic variants of CYP24A1 are associated with hypercalcemia due to disruptions in the ability of 24-hydroxylase to break down 1,25-dihydroxyvitamin D (1,25-DHVD). A case involving a heterozygous pathogenic variant of CYP24A1 and primary hyperparathyroidism leading to severe hypercalcemia has not been previously reported. A 23-year-old woman presented with fatigue and was found to be hypercalcemic at 13.8 mg/dL [reference range, 8.4-10.2 pg/mL]. Her parathyroid hormone (PTH) was 62 pg/mL [reference range, 19-88 pg/mL] and 1,25-DHVD was elevated to 242.7 pg/mL [reference range, 18-72 pg/mL]. Other laboratory workup was unrevealing. She had a bone scan, whole body CT scan, and thyroid ultrasound that were normal. Her 25-hydroxy-vitamin D to 24,25-dihydroxy-vitamin D ratio was elevated at 25.18 (normal, < 25). Because of concern for primary hyperparathyroidism, she was referred to an endocrine surgeon and underwent a parathyroidectomy with the removal of a 3.5-gram adenoma. Pathology showed a parafibromin-deficient parathyroid neoplasm. Genetic testing demonstrated a heterozygous pathogenic variant in CYP24A1. Three weeks after surgery, PTH was 14 pg/mL (1.48 pmol/L), calcium and 1,25-DHVD normalized. In summary, we report a case where a patient with severe symptomatic hypercalcemia was found to have primary hyperparathyroidism exacerbated by an underlying heterozygous pathogenic variant in CYP24A1.

When Guideline-Concordant Standardized Care Results in Healthcare Disparities.

AbstractClinical red blood cell transfusion guidelines have been widely adopted in clinical practice, resulting in standardized transfusion practices in hospitalized patients with anemia. Standardization of transfusion practice has been welcomed by clinicians and health systems as a mechanism for reducing unnecessary, harmful, and costly practice variation that results in healthcare disparities. However, overzealously applied guidelines can have deleterious consequences for individual patients, ultimately resulting in and/or exacerbating healthcare disparities, rather than resolving them. This article provides empirical examples of the adverse consequences from the well-meaning attempt to standardize transfusion practice based on clinical practice guidelines and discusses the ethical implications of standardized transfusion practice.

Preoperative Evaluation of Thyroid Cancer: A Review of Current Best Practices.

OBJECTIVE: The incidence of thyroid cancer has significantly increased in recent decades. Although most thyroid cancers are small and carry an excellent prognosis, a subset of patients present with advanced thyroid cancer, which is associated with increased rates of morbidity and mortality. The management of thyroid cancer requires a thoughtful individualized approach to optimize oncologic outcomes and minimize morbidity associated with treatment. Because endocrinologists usually play a key role in the initial diagnosis and evaluation of thyroid cancers, a thorough understanding of the critical components of the preoperative evaluation facilitates the development of a timely and comprehensive management plan. The following review outlines considerations in the preoperative evaluation of patients with thyroid cancer. METHODS: A clinical review based on current literature was generated by a multidisciplinary author panel. RESULTS: A review of considerations in the preoperative evaluation of thyroid cancer is provided. The topic areas include initial clinical evaluation, imaging modalities, cytologic evaluation, and the evolving role of mutational testing. Special considerations in the management of advanced thyroid cancer are discussed. CONCLUSION: Thorough and thoughtful preoperative evaluation is critical for formulating an appropriate treatment strategy in the management of thyroid cancer.

IgG4-related sclerosing thyroiditis (Riedel-Struma): a review of clinicopathological features and management.

We present a thorough review of the literature on Riedel thyroiditis (RT) with emphasis on aetiology, diagnosis and management, using the PubMed, Sinomed, and China National Knowledge Infrastructure databases. Although the exact aetiology of RT remains obscure, the histopathological features are consistent with a localized form of IgG4-related systemic disease (IgG4-RSD). Nevertheless, IgG4-RSD as a systemic fibroinflammatory disorder per se rarely affects the thyroid in the context of multiorgan manifestations. The initial diagnosis of RT is based on clinical history and imaging, but confirmation by histopathological examination is mandatory. In contrast to the historical surgical approach, glucocorticosteroid therapy is currently considered first line therapy, in line with the RT currently being viewed as a manifestation of, or analogous to, IgG4-RSD. For disease relapse, immunomodulatory agents (azathioprine, methotrexate, rituximab) can be used.