RESUMEN
BACKGROUND: Primary hyperparathyroidism is rare in pediatric patients. Our study aim was to compare primary hyperparathyroidism in pediatric (<19 years) and young adult (19-29 years) patients. METHODS: A prospectively collected database from a single, high-volume institution was queried for all patients age <30 years who had initial parathyroidectomy for primary hyperparathyroidism yielding 126/4,546 (2.7%) primary hyperparathyroidism patients representing 39 pediatric and 87 young adult patients. Presenting symptoms, operative data, and postoperative course were compared for patients age 0-19 years and 20-29 years. RESULTS: Sporadic primary hyperparathyroidism was present in 81.7% and occurred less often in pediatric patients than young adult patients (74.4% vs 86.2%, P = .12). Among patients with hereditary primary hyperparathyroidism, multiple endocrine neoplasia type 1 was the most common type. Multiglandular disease was common in both pediatric (30.7%) and young adult (21.8%) patients. Following parathyroidectomy, 3 (2.3%) patients had permanent hypoparathyroidism and none had permanent recurrent laryngeal nerve paralysis. Biochemical cure at 6 months was equally likely in pediatric and young adult patients (97.1% vs 93.6%, P = .44) with comparable follow-up (78.4 months vs 69.1 months, P = .66) and rates of recurrent disease (5.9% vs 10.3%, P = .46). Recurrence was due to multiple endocrine neoplasia 1-related primary hyperparathyroidism in all cases. CONCLUSION: Although primary hyperparathyroidism is sporadic in most patients <19 years, they are more likely to have multiple endocrine neoplasia type 1-associated primary hyperparathyroidism (23%). Parathyroidectomy for primary hyperparathyroidism can be performed safely in pediatric patients with a high rate of cure. Follow-up for patients with hereditary disease is necessary.
Asunto(s)
Hiperparatiroidismo Primario/cirugía , Neoplasias de las Paratiroides/epidemiología , Neoplasias de las Paratiroides/cirugía , Paratiroidectomía/métodos , Adolescente , Adulto , Factores de Edad , Biopsia con Aguja , Niño , Bases de Datos Factuales , Femenino , Estudios de Seguimiento , Humanos , Hiperparatiroidismo Primario/diagnóstico por imagen , Hiperparatiroidismo Primario/epidemiología , Hiperparatiroidismo Primario/patología , Inmunohistoquímica , Incidencia , Estimación de Kaplan-Meier , Masculino , Neoplasias de las Paratiroides/diagnóstico por imagen , Neoplasias de las Paratiroides/patología , Paratiroidectomía/efectos adversos , Tomografía de Emisión de Positrones/métodos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/fisiopatología , Estudios Prospectivos , Recurrencia , Medición de Riesgo , Factores Sexuales , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Erectile dysfunction (ED) is a common diagnosis associated with age, hypertension, cardiovascular disease, and diabetes. Primary hyperparathyroidism (PHP) is also seen with these comorbidities, but its association with ED has yet to be studied. We evaluated the rate and resolution of impotence after curative surgery for PHP. METHODS: Prospectively collected data, including a self-reported questionnaire of symptoms, were reviewed for men who had curative parathyroid exploration for sporadic PHP from July 2010 to January 2014. Data were compared with an age-matched cohort of men who had thyroidectomy during the same period. RESULTS: Among 160 men with PHP and mean age of 60 years (range, 19-88), preoperative ED was reported by 13%, and this group was older than patients without ED (mean age, 70 vs 58 years, P < .01). Self-reported resolution of ED after parathyroidectomy occurred in 67% compared with 43% of patients in a thyroidectomy cohort. Preoperative mean arterial blood pressure was less in men with postoperative resolution of ED (96.6 vs 105.4 mm Hg, P = .03). Among 3 of 21 men on specific ED medications, 2 no longer required them postoperatively. CONCLUSION: Impotence is reported often by men undergoing parathyroidectomy for PHP. After curative surgery, 67% of those affected may self-report ED resolution, which may be more pronounced in those patients with a lesser preoperative mean arterial blood pressure.
Asunto(s)
Disfunción Eréctil/cirugía , Hiperparatiroidismo Primario/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Disfunción Eréctil/etiología , Humanos , Hiperparatiroidismo Primario/complicaciones , Masculino , Persona de Mediana Edad , Paratiroidectomía , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVES: To correlate thyroid cancer genotype with histology and outcomes. BACKGROUND: The prognostic significance of molecular signature in thyroid cancer (TC) is undefined but can potentially change surgical management. METHODS: We reviewed a consecutive series of 1510 patients who had initial thyroidectomy for TC with routine testing for BRAF, RAS, RET/PTC, and PAX8/PPARG alterations. Histologic metastatic or recurrent TC was tracked for 6 or more months after oncologic thyroidectomy. RESULTS: Papillary thyroid cancer (PTC) was diagnosed in 97% of patients and poorly differentiated/anaplastic TC in 1.1%. Genetic alterations were detected in 1039 (70%); the most common mutations were BRAFV600E (644/1039, 62%), and RAS isoforms (323/1039, 31%). BRAFV600E-positive PTC was often conventional or tall cell variant (58%), with frequent extrathyroidal extension (51%) and lymph node metastasis (46%). Conversely, RAS-positive PTC was commonly follicular variant (87%), with infrequent extrathyroidal extension (4.6%) and lymph node metastasis (5.6%). BRAFV600E and RET/PTC-positive PTCs were histologically similar. Analogously, RAS and PAX8/PPARG-positive PTCs were histologically similar. Compared with RAS or PAX8/PPARG-positive TCs, BRAFV600E or RET/PTC-positive TCs were more often associated with stage III/IV disease (40% vs 15%, P < 0.001) and recurrence (10% vs 0.7%, P < 0.001; mean follow-up 33 ± 21 mo). Distant metastasis was highest in patients with RET/PTC-positive TC (10.8%, P = 0.02). CONCLUSIONS: In this large study of prospective mutation testing in unselected patients with TC, molecular signature was associated with distinctive phenotypes including cancers, with higher risks of both distant metastasis and early recurrence. Preoperative genotype provides valuable prognostic data to appropriately inform surgery.
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Carcinoma/genética , Carcinoma/mortalidad , Regulación Neoplásica de la Expresión Génica , Proteínas Proto-Oncogénicas/genética , Neoplasias de la Tiroides/genética , Neoplasias de la Tiroides/mortalidad , Adulto , Anciano , Carcinoma/patología , Carcinoma/cirugía , Carcinoma Papilar , Bases de Datos Factuales , Supervivencia sin Enfermedad , Femenino , Genotipo , Humanos , Masculino , Persona de Mediana Edad , Mutación , Invasividad Neoplásica , Estadificación de Neoplasias , Factor de Transcripción PAX8 , Factores de Transcripción Paired Box/genética , Fenotipo , Valor Predictivo de las Pruebas , Pronóstico , Proteínas Proto-Oncogénicas B-raf/genética , Proteínas Proto-Oncogénicas c-ret/genética , Estudios Retrospectivos , Análisis de Supervivencia , Cáncer Papilar Tiroideo , Neoplasias de la Tiroides/patología , Neoplasias de la Tiroides/cirugía , Tiroidectomía/métodos , Tiroidectomía/mortalidad , Resultado del TratamientoRESUMEN
INTRODUCTION: We aimed to determine influence of surgeon volume on (1) frequency of appropriate initial surgery for differentiated thyroid cancer (DTC) and (2) completeness of resection. METHODS: We reviewed all initial thyroidectomies (Tx; lobectomy and total) performed in a health system during 2011; surgeons were grouped by number of Tx cases per year. For patients with histologic DTC ≥ 1 cm, surgeon volume was correlated with initial extent of the operation, and markers of complete resection including uptake on I(123) prescan, thyrotropin-stimulated thyroglobulin levels, and I(131) dose administered. RESULTS: Of 1,249 patients who underwent Tx by 42 surgeons, 29% had DTC ≥ 1 cm without distant metastasis. At a threshold of ≥ 30 Tx per year, surgeons were more likely to perform initial total Tx for DTC ≥ 1 cm (P = .01), and initial resection was more complete as measured by all 3 quantitative markers. For patients with advanced stage disease, a threshold of ≥ 50 Tx per year was needed before observing improvements in I(123) uptake (P = .004). CONCLUSION: Surgeons who perform ≥ 30 Tx a year are more likely to undertake the appropriate initial operation and have more complete initial resection for DTC patients. Surgeon volume is an essential consideration in optimizing outcomes for DTC patients, and even higher thresholds (≥ 50 Tx/year) may be necessary for patients with advanced disease.
Asunto(s)
Recurrencia Local de Neoplasia/patología , Neoplasias de la Tiroides/patología , Neoplasias de la Tiroides/cirugía , Tiroidectomía/métodos , Tiroidectomía/estadística & datos numéricos , Centros Médicos Académicos , Biopsia con Aguja , Competencia Clínica , Bases de Datos Factuales , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Invasividad Neoplásica/patología , Recurrencia Local de Neoplasia/mortalidad , Recurrencia Local de Neoplasia/cirugía , Estadificación de Neoplasias , Selección de Paciente , Estudios Retrospectivos , Medición de Riesgo , Cirujanos/estadística & datos numéricos , Análisis de Supervivencia , Neoplasias de la Tiroides/mortalidad , Tiroidectomía/efectos adversos , Resultado del Tratamiento , Recursos HumanosRESUMEN
BACKGROUND: Fibromyalgia (FM), an ill-defined symptom complex, is characterized by musculoskeletal pain, headache, depression, fatigue, and cognitive decline, symptoms also seen commonly in primary hyperparathyroidism (PHP). Prevalence of concurrent PHP and FM and response to parathyroidectomy (PTX) of those with both conditions are unknown. METHODS: We reviewed prospective data of 4,000 patients with sporadic PHP who had PTX from 1995 to 2013 examining perioperative symptoms and medication usage for those with diagnosed FM. Cure was defined by normocalcemia at ≥ 6 months. RESULTS: Of 2,184 patients, 80 (4%) had a prior diagnosis of FM. Of evaluable FM patients, 97.3% had definitive cure of PHP. After PTX, 89% had improvement in ≥ 1 symptom attributed to FM, with improved cognition/memory most common (80%). Improvement in ≥ 2, ≥ 3, and ≥ 4 FM symptoms was appreciated by 71%, 43%, and 25%, respectively. Quality of life and wellness improved in >50%. Postoperative use of drugs prescribed for FM often improved or resolved (narcotics, 77%; anti-inflammatories, 74%; "FM-specific medications," 33%; antidepressants, 30%); 21% discontinued all FM medications postoperatively. CONCLUSION: FM is common in patients operated on for sporadic PHP. Of those with both conditions, after PTX 89% appreciate symptom response and 77% and 21% had a decrease in or discontinuation or medications, respectively. Before diagnosing FM, providers should exclude PHP, which is surgically correctable.
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Fibromialgia/tratamiento farmacológico , Fibromialgia/epidemiología , Hiperparatiroidismo Primario/epidemiología , Hiperparatiroidismo Primario/cirugía , Dolor Postoperatorio/tratamiento farmacológico , Adulto , Anciano , Anciano de 80 o más Años , Distribución de Chi-Cuadrado , Estudios de Cohortes , Comorbilidad , Bases de Datos Factuales , Femenino , Fibromialgia/diagnóstico , Estudios de Seguimiento , Humanos , Hiperparatiroidismo Primario/diagnóstico , Masculino , Persona de Mediana Edad , Dimensión del Dolor , Dolor Postoperatorio/fisiopatología , Paratiroidectomía/efectos adversos , Paratiroidectomía/métodos , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
OBJECTIVE: To test whether a clinical algorithm using routine cytological molecular testing (MT) promotes initial total thyroidectomy (TT) for clinically significant thyroid cancer (sTC) and/or correctly limits surgery to lobectomy when appropriate. BACKGROUND: Either TT or lobectomy is often needed to diagnose differentiated thyroid cancer. Determining the correct extent of initial thyroidectomy is challenging. METHODS: After implementing an algorithm for prospective MT of in-house fine-needle aspiration biopsy specimens, we conducted a single-institution cohort study of all patients (N = 671) with nonmalignant cytology who had thyroidectomy between October 2010 and March 2012, cytological diagnosis using 2008 Bethesda criteria, and 1 or more indications for thyroidectomy by 2009 American Thyroid Association guidelines. sTC was defined by histological differentiated thyroid cancer of 1 cm or more and/or lymph node metastasis. Cohort 2 patients did not have MT or had unevaluable results. In cohort 1, MT for a multigene mutation panel was performed for nonbenign cytology, and positive MT results indicated initial TT. RESULTS: MT guidance was associated with a higher incidence of sTC after TT (P = 0.006) and a lower rate of sTC after lobectomy (P = 0.03). Without MT results, patients with indeterminate (follicular lesion of undetermined significance/follicular or oncocytic neoplasm) cytology who received initial lobectomy were 2.5 times more likely to require 2-stage surgery for histological sTC (P < 0.001). In the 501 patients with non-sTC for whom lobectomy was the appropriate extent of surgery, lobectomy was correctly performed more often with routine preoperative MT (P = 0.001). CONCLUSIONS: Fine-needle aspiration biopsy MT for BRAF, RAS, PAX8-PPARγ, and RET-PTC expedites optimal initial surgery for differentiated thyroid cancer, facilitating succinct definitive management for patients with thyroid nodules.
Asunto(s)
Algoritmos , Biopsia con Aguja Fina/métodos , Guías de Práctica Clínica como Asunto , Glándula Tiroides/patología , Neoplasias de la Tiroides/patología , Nódulo Tiroideo/patología , Tiroidectomía/métodos , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Reproducibilidad de los Resultados , Estudios Retrospectivos , Glándula Tiroides/cirugía , Neoplasias de la Tiroides/cirugía , Nódulo Tiroideo/cirugíaRESUMEN
BACKGROUND: In thyroid nodule fine-needle aspiration (FNA) cytology, the atypia of undetermined significance or follicular lesion of undetermined significance (AUS/FLUS) category has a 5-15% malignancy risk that increases to 85-99% when mutation testing for BRAF, RAS, RET/PTC, or PAX8/PPARγ is positive. However, negative testing does not exclude malignancy. The study objective was to identify clinical and imaging features that predict cancer in mutation-negative AUS/FLUS thyroid nodules. METHODS: All patients were reviewed (April 2007 to April 2009) who had AUS/FLUS cytology, negative prospective molecular testing of FNA, and histopathology. RESULTS: Of the 230 nodules, 12 (5.2%) were malignant in 11 of 190 patients, and known clinical risk factors for thyroid cancer did not predict malignancy. On preoperative imaging, ≥1 suspicious ultrasound feature was identified in 33% of nodules and occurred regardless of histology (P = .23). Malignant mutation-negative AUS/FLUS nodules were larger than benign nodules (mean maximum diameter, 33.6 vs 24.0 mm; P = .007). On multivariate analysis, nodule size remained an independent predictor of malignancy (odds ratio, 1.043; P = .018). We observed no malignancies in 88 mutation-negative AUS/FLUS nodules <18.5 mm. CONCLUSION: Size is an independent predictor of malignancy in mutation-negative AUS/FLUS nodules and the risk increased 4.3% with every millimeter increase in nodule size. Selected patients with small, mutation-negative AUS/FLUS thyroid nodules may be managed with ultrasound surveillance in lieu of thyroidectomy.