RESUMO
The better understanding of the biological behavior of multiple endocrine neoplasia type 1 (MEN1) organ manifestations and the increase in clinical experience warrant a revision of previously published guidelines. Duodenopancreatic neuroendocrine neoplasias (DP-NENs) are still the second most common manifestation in MEN1 and, besides NENs of the thymus, remain a leading cause of death. DP-NENs are thus of main interest in the effort to reevaluate recommendations for their diagnosis and treatment. Especially over the last 2 years, more clinical experience has documented the follow-up of treated and untreated (natural-course) DP-NENs. It was the aim of the international consortium of experts in endocrinology, genetics, radiology, surgery, gastroenterology, and oncology to systematically review the literature and to present a consensus statement based on the highest levels of evidence. Reviewing the literature published over the past decade, the focus was on the diagnosis of F- and NF-DP-NENs within the MEN1 syndrome in an effort to further standardize and improve treatment and follow-up, as well as to establish a "logbook" for the diagnosis and treatment of DP-NENs. This shall help further reduce complications and improve long-term treatment results in these rare tumors. The following international consensus statement builds upon the previously published guidelines of 2001 and 2012 and attempts to supplement the recommendations issued by various national and international societies.
Assuntos
Consenso , Neoplasias Duodenais , Neoplasia Endócrina Múltipla Tipo 1 , Neoplasias Pancreáticas , Neoplasias Duodenais/diagnóstico , Neoplasias Duodenais/terapia , Humanos , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Neoplasia Endócrina Múltipla Tipo 1/terapia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapiaRESUMO
BACKGROUND: The role of lymphadenectomy in adrenocortical carcinoma resection is controversial. Therefore, we conducted a population-based study to assess the association between positive lymph nodes (LN) and survival. METHODS: The Surveillance, Epidemiology, and End Results set of cancer registries were utilized. The associations between positive lymph nodes and tumor size, grade and laterality were assessed. Cancer specific survival (CSS) trends and factors affecting survival were analyzed. RESULTS: A total of 2170 adult patients were identified; 60% underwent resection. Among those resected, LN were examined in 23% and were positive in 25% of patients with LN examined. Patients with positive LN tended to have smaller tumors compared to those with negative LN (12 ± 5 vs 15 ± 11 cm, p = 0.02). The rate of positive LN was higher in right ACC, p = 0.03. Median overall CSS was 21 months, with significant differences between resection (42 months) and no resection (4 months), p < 0.01. Median CSS did not change over time when comparing ACC patients who underwent surgery before 2000, 2000-2009, and 2010-2016. On multivariable analysis including resection group, advanced age, grades III and IV, regional and distant stage, in addition to positive LN were associated with worse survival, p < 0.05. CONCLUSION: Lymphadenectomy is infrequently performed during ACC resection, and when performed, regional LN involvement tends to be associated with worse survival. Neoplasm size and grade were not associated with LN involvement and therefore, do not inform lymphadenectomy need. Further studies are needed to assess the indications for, and value of lymphadenectomy in ACC.
Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/epidemiologia , Neoplasias do Córtex Suprarrenal/mortalidade , Neoplasias do Córtex Suprarrenal/patologia , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/epidemiologia , Carcinoma Adrenocortical/mortalidade , Carcinoma Adrenocortical/patologia , Carcinoma Adrenocortical/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Excisão de Linfonodo/estatística & dados numéricos , Linfonodos/patologia , Linfonodos/cirurgia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Retrospectivos , Programa de SEER/estatística & dados numéricos , Adulto JovemRESUMO
BACKGROUND: Bilateral adrenalectomy (BA) is a curative treatment for hypercortisolism in patients with corticotropin (ACTH)-dependent Cushing syndrome. Indications include refractory Cushing's disease (CD) following failed pituitary surgery, and occult or unresectable ectopic ACTH-producing neoplasms (EA). METHODS: This was a single-center, retrospective review (1995-2017) of patients undergoing BA for CD or EA. Demographics, laboratory findings, and intraoperative and postoperative variables were analyzed. RESULTS: Of 137 patients, 83 (61%) had CD and 54 (39%) had EA; 87% of CD patients were female versus 46% of EA patients (p < 0.0001). Mean age at diagnosis was 40 ± 15 years for CD and 49 ± 18 years for EA (p = 0.004). Preoperative serum cortisol concentrations were higher in the EA cohort (63 ± 40 µg/dL) versus the CD cohort (33 ± 19 µg/dL) [p < 0.001], with no significant differences in serum ACTH. Time from diagnosis until adrenalectomy was 54 ± 69 months for CD versus 4 ± 13 for EA (p = 0.002). Most patients underwent minimally invasive surgery (MIS; 86% CD vs. 75% EA; p = 0.19). There was no difference between the rates of conversion to an open approach, intraoperative blood loss, or operative time between groups, and no difference between complications in CD versus EA (p = 1.0). Five-year survival was significantly shorter among the EA cohort (30% for EA vs. 80% for CD; p < 0.001). CONCLUSION: Patients with EA presented with higher serum cortisol levels compared with patients with CD. EA patients were more likely to require intraoperative transfusion and postoperative intensive care. BA in patients with CD and EA can be performed safely in an MIS fashion, with similar morbidity; however, survival at 5 years was significantly less in the EA cohort.
Assuntos
Neoplasias , Hipersecreção Hipofisária de ACTH , Adrenalectomia , Hormônio Adrenocorticotrópico , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Hipersecreção Hipofisária de ACTH/etiologia , Hipersecreção Hipofisária de ACTH/cirurgia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVE: Comprehensive data about patients with bilateral pheochromocytoma are limited. We aimed to describe the clinical presentation, genetic analysis, treatment and outcomes of patients with bilateral pheochromocytoma. DESIGN: A retrospective study at a tertiary care centre. PATIENTS: All patients with bilateral pheochromocytoma evaluated at Mayo Clinic in Rochester, Minnesota between January 1951 and December 2015. MEASUREMENTS: Tumour size, genetic testing, plasma/urine metanephrines and catecholamines. RESULTS: A total of 94 patients (51% women) were diagnosed with bilateral pheochromocytoma at a median age at first presentation of 31 years (range, 4-70). Bilateral disease was noted in 8.0% of pheochromocytoma patient overall and 37.5% of patients 18 years of younger. Most patients presented with synchronous tumours (80%). Median time to metachronous tumours was 4.5 years (range, 1-38). Genetic disease was identified in 75 (80%) patients, including MEN 2A (42.6%), VHL (19.1%), MEN 2B (9.6%) and NF1 (8.5%). Excess catecholamines were present in 97% of patients. Patients with synchronous pheochromocytoma commonly underwent simultaneous bilateral adrenalectomy (99%), and 18 (24%) had cortical-sparing surgery. Multicentric tumours were reported in 23 of 77 (30%) patients with available data. Recurrent disease was found in 9.6% of patients, and 8.5% developed metastatic disease. Median follow-up was 8.5 years. At the study conclusion, 4 patients had died due to pheochromocytoma or adrenalectomy. CONCLUSIONS: Bilateral pheochromocytoma occurred in 7.0% of adults with pheochromocytoma and 37.5% of paediatric patients. Genetic disease was identified in 80% of patients, predominantly MEN2A. Multicentric tumours were common, but most were still cured following adrenalectomy.
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Neoplasias das Glândulas Suprarrenais , Feocromocitoma , Doença de von Hippel-Lindau , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Adulto , Criança , Feminino , Seguimentos , Humanos , Masculino , Feocromocitoma/genética , Feocromocitoma/cirurgia , Estudos RetrospectivosRESUMO
OBJECTIVE: We aimed to describe clinical course of myelolipoma and to identify predictors of tumour growth and need for surgery. DESIGN: A retrospective study. PATIENTS: Consecutive patients with myelolipoma. RESULTS: A total of 321 myelolipomas (median size, 2.3 cm) were diagnosed in 305 patients at median age of 63 years (range, 25-87). Median follow-up was 54 months. Most myelolipomas were incidentally detected (86%), whereas 9% were discovered during cancer staging and 5% during workup of mass effect symptoms. Thirty-seven (12%) patients underwent adrenalectomy. Compared to myelolipomas <6 cm, tumours ≥6 cm were more likely to be bilateral (21% vs 3%, P < .0001), cause mass effect symptoms (32% vs 0%, P < .0001), have haemorrhagic changes (14% vs 1%, P < .0001) and undergo adrenalectomy (52% vs 5%, P < .0001). Among patients with ≥6 months of imaging follow-up, median size change was 0 mm (-10, 115) and median growth rate was 0 mm/y (-6, 14). Compared to <1 cm growth, ≥1 cm growth correlated with larger initial size (3.6 vs 2.3 cm, P = .02), haemorrhagic changes (12% vs 2%, P = .007) and adrenalectomy (35% vs 8%, P < .0001). CONCLUSIONS: Most myelolipomas are incidentally discovered on cross-sectional imaging. Myelolipomas ≥6 are more likely to cause mass effect symptoms, have haemorrhagic changes and undergo resection. Tumour growth ≥1 cm is associated with larger myelolipoma and haemorrhagic changes. Adrenalectomy should be considered in symptomatic patients with large tumours and when there is evidence of haemorrhage or tumour growth.
Assuntos
Neoplasias das Glândulas Suprarrenais , Mielolipoma , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Adulto , Idoso , Idoso de 80 Anos ou mais , Seguimentos , Humanos , Pessoa de Meia-Idade , Mielolipoma/diagnóstico , Mielolipoma/cirurgia , Estudos RetrospectivosRESUMO
BACKGROUND: To help control opioid overprescription, we conducted a large institutional, 3-site initiative to provide discharge prescribing guidelines for different procedures. Our aim is to refine institutional guidelines for parathyroidectomy. METHODS: Patients undergoing parathyroidectomy completed a 28-question survey about opioid consumption. Discharge opioid prescription amounts were converted into morphine milligram equivalents (MMEs) and reported as median and interquartile range (IQR). Consumption was dichotomized into top quartile MME users (Q4) versus standard users (Q1, Q3). Univariate analysis compared opioid consumption. RESULTS: A total of 91 patients were included; 90% were opioid-naive. While the median prescribed was 75 (IQR 75, 150) MME, the median consumed was 0 (IQR 0, 20). Top users reported higher pain scores [median (IQR): 2 (2, 4)] compared to standard users [1 (0, 3), P = 0.01]. However, there was no difference in opioid consumption between unilateral neck exploration, bilateral exploration, or thyroidectomy and parathyroidectomy, P = 0.11. There was no difference in opioid consumption by age, sex, or BMI (all P > 0.05). Of those receiving a prescription, 94.6% had left-over opioids at the time of survey, resulting in 82% of prescribed opioids being unused. CONCLUSIONS: Over half of patients undergoing parathyroidectomy did not consume any opioid, and very few needed more than 2 d of opioid. Moreover, most patients did not dispose the unused opioids, which put these pills at risk of diversion and misuse. Surgical approach did not change consumption, illustrating that these guidelines are applicable to thyroidectomy given the similarity between techniques. We recommend prescribing nonopioid analgesics for patients undergoing parathyroidectomy.
Assuntos
Analgésicos não Narcóticos/efeitos adversos , Analgésicos Opioides/uso terapêutico , Dor Pós-Operatória/tratamento farmacológico , Paratireoidectomia/efeitos adversos , Padrões de Prática Médica/normas , Adulto , Idoso , Idoso de 80 Anos ou mais , Prescrições de Medicamentos/normas , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Epidemia de Opioides/etiologia , Epidemia de Opioides/prevenção & controle , Medição da Dor , Dor Pós-Operatória/diagnóstico , Dor Pós-Operatória/etiologia , Guias de Prática Clínica como Assunto , Uso Indevido de Medicamentos sob Prescrição/prevenção & controle , Avaliação de Programas e Projetos de Saúde , Estudos Prospectivos , Estudos Retrospectivos , Tireoidectomia/efeitos adversosRESUMO
INTRODUCTION: Due to the rarity of malignant insulinoma, a lack of the literature describing factors affecting outcomes exists. Our aim was to review malignant insulinoma incidence, characteristics and survival trends. METHODS: We identified all patients with malignant insulinoma in the SEER registries from 1973 to 2015. Incidence, neoplasm characteristics and factors affecting cancer-specific survival (CSS) were described. RESULTS: A total of 121 patients were identified. The crude annual overall incidence was low (range 0.0-0.27 cases per million person years). The largest proportion had localized disease (40%), while 16% had regional disease, 39% distant metastatic disease, and stage was unreported in 5%. Most neoplasms were in the body/tail of the pancreas, followed by the head of the pancreas. Grade was reported in 40% of patients; only a single patient reported as having grade IV with the remainder all grades I/II. Surgical resection was performed in 64% of patients. Within surgical patients, the median primary neoplasm size was 1.8 cm. Regional lymph nodes were examined in 57.1% of surgical patients, while 34% of examined nodes were positive. The median CSS was 183 months. On multivariable analysis, surgical resection, male sex and absence of metastatic disease were associated with superior survival. CONCLUSION: While the greatest proportion of patients with malignant insulinoma present with localized disease, regional lymph node involvement was found in 34% of whose nodes were tested. Further studies are needed to assess the role of lymph node dissection in improving survival and preventing recurrence given the observed frequency of lymph node involvement.
Assuntos
Insulinoma , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Humanos , Insulinoma/diagnóstico , Insulinoma/epidemiologia , Insulinoma/patologia , Insulinoma/cirurgia , Excisão de Linfonodo , Metástase Linfática , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/epidemiologia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Prognóstico , Taxa de SobrevidaRESUMO
BACKGROUND: Adrenalectomy for non-neuroblastic pathologies in children is rare with limited data on outcomes. We reviewed our experience of adrenalectomy in this unique population. METHODS: Retrospective study of children (age ≤ 18) who underwent adrenalectomy with non-neuroblastic pathology from 1988 to 2018. Clinical and operative details of patients were abstracted. Outcomes included length of stay and 30-day postoperative morbidity. RESULTS: Forty children underwent 50 adrenalectomies (12 right-sided, 18 left-sided, 10 bilateral). Six patients (15%) presented with an incidental adrenal mass while 4 (10%) had masses found on screening for genetic mutations or prior malignancy. The remaining 30 (75%) presented with symptoms of hormonal excess. Nineteen patients (48%) underwent genetic evaluation and 15 (38%) had genetic predispositions. Diagnoses included 9 patients (23%) with pheochromocytoma, 8 (20%) with adrenocortical adenoma, 8 (20%) with adrenocortical carcinoma, 7 (18%) with adrenal hyperplasia, 2 (5%) with metastasis, and 6 (14%) with additional benign pathologies. Of 50 adrenalectomies, twenty-five (50%) were laparoscopic. Median hospital length of stay was 3 days (range 0-11). Post-operative morbidity rate was 17% with the most severe complication being Clavien-Dindo grade II. CONCLUSION: Adrenalectomy for non-neuroblastic pathology can be done with low morbidity. Its frequent association with genetic mutations and syndromes requires surgeons to have knowledge of appropriate pre-operative testing and post-operative surveillance.
Assuntos
Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/métodos , Laparoscopia/métodos , Feocromocitoma/cirurgia , Adolescente , Neoplasias das Glândulas Suprarrenais/diagnóstico , Criança , Feminino , Humanos , Tempo de Internação , Masculino , Feocromocitoma/diagnóstico , Período Pós-Operatório , Estudos RetrospectivosRESUMO
OBJECTIVE: To Identify predictors of recurrent disease following lateral neck dissection (LND) for papillary thyroid carcinoma (PTC). METHODS: A retrospective review of patients who underwent first-time LND for PTC at our institution (2000-2015) was performed. Medical records were examined for biopsy or pathologically proven lateral neck recurrence. Differences between the groups with and without recurrence were compared. All LNDs were then classified in to two groups: "comprehensive" (CND), involving levels IIa-Vb at minimum, or "selective", labelling less extensive dissection (SND). RESULTS: Four hundred nine patients underwent 467 LNDs. Surveillance data were available for 317 patients who underwent 362 LNDs (mean age 45 ± 16; range 18-88). The median follow-up was 64 ± 48 months (range 3-197). Recurrence was detected in 71 lateral necks (20%). The total number of lymph nodes was greater in the group without recurrence compared to those with recurrence (23 vs. 19, p = 0.02). Among patient demographics, radioactive iodine treatment, primary tumor characteristics and characteristics of nodal metastases, only an older patient age (mean 50 vs. 43 years) was associated with lateral neck recurrence (p < .01). CND was performed in 102 lateral necks and SND in 143 necks. There were 12 recurrences recorded in the CND group (12%) vs. 31 in the SND group (22%, p = .04). The majority of recurrences (70%) involved levels included in the original dissection. CONCLUSIONS: Younger patients, more extensive dissection and a higher total number of lymph nodes removed are associated with a lower incidence of lateral neck recurrence after LND for papillary thyroid carcinoma.
Assuntos
Carcinoma Papilar/cirurgia , Esvaziamento Cervical/métodos , Recidiva Local de Neoplasia/prevenção & controle , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia/efeitos adversos , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Papilar/patologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/etiologia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Neoplasias da Glândula Tireoide/patologiaRESUMO
BACKGROUND: Indocyanine green fluorescence angiography (ICGA) is a new adjunct that has been used in surgical procedures to assess blood flow. This study evaluated the utility of ICGA compared to visual inspection to predict parathyroid function, guide autotransplantation and potentially decrease permanent hypoparathyroidism. METHODS: This was a retrospective study of patients who underwent total or near-total thyroidectomy (T-NT) between January 2015 and March 2018. Patients with preoperative hyperparathyroidism and those undergoing reoperation were excluded. Patients who had ICGA were compared to T-NT patients without ICGA. Data were analyzed to assess the frequency of autotransplantation and incidence of hypoparathyroidism between groups. RESULTS: In total, 210 patients underwent T-NT: 86 with ICGA and 124 without. Autotransplantation was more common in the ICGA group at 36% compared to 12% in the control (p = 0.0001). There was no correlation with at least one normal parathyroid gland on ICGA and postoperative PTH levels (p = 0.75). There was a difference in having normal postoperative PTH when there were at least two normal parathyroid glands (n = 50) compared to patients with less than two normal ICGA glands (n = 36, p = 0.044). Visual assessment and ICGA assessment of vascularity were in agreement, 245/281 (87%). There were 19 glands (6.8%) that would have undergone autotransplant based on visual inspection that had adequate blood supply on ICGA. Transient hypoparathyroidism was present in 45 out of 124 controls (36%) and 32 out of 86 (37%) in the ICG group. CONCLUSIONS: ICGA is a novel technique that may improve the assessment of parathyroid gland blood supply compared to visual inspection. ICGA can guide more appropriate autotransplantation without compromising postoperative parathyroid function. At least two vascularized glands on ICGA may predict postoperative parathyroid gland function.
Assuntos
Angiofluoresceinografia , Verde de Indocianina , Glândulas Paratireoides/diagnóstico por imagem , Estudos de Casos e Controles , Corantes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Glândulas Paratireoides/transplante , Hormônio Paratireóideo/sangue , Estudos Retrospectivos , Tireoidectomia , Transplante Autólogo/estatística & dados numéricosRESUMO
INTRODUCTION: Patients undergoing unilateral adrenalectomy for primary aldosteronism (PA) may have a solitary adenoma, unilateral hyperplasia, or multiple adenomas on final pathology. This study investigated whether the underlying pathological diagnosis was associated with differences in clinical presentation and postoperative outcomes. METHODS: A retrospective cohort study of patients undergoing unilateral adrenalectomy for PA from 2004 to 2015 at our institution was performed. Baseline clinical and laboratory parameters, as well as postoperative biochemical and hypertension cure rates, were compared across the three aforementioned pathological groups. RESULTS: Of 206 patients who met criteria for inclusion, 152 (73.8%) had a single adenoma, 33 (16%) had unilateral hyperplasia, and 21 (10.2%) had multiple unilateral adenomas. Patients with unilateral hyperplasia were more likely to be male (81.2% vs 57.9%, P = .03), undergo left-sided adrenalectomy (78.8% vs 47.4%, P < .01), and had a lower median adrenal venous sampling lateralization index (9.8 vs 19.8, P = .04) compared to those with solitary, but not multiple unilateral adenomas. No differences were seen in age, duration of hypertension, preoperative plasma aldosterone levels, plasma renin activities, 24-h urinary aldosterone excretion, serum potassium concentrations, and the number of preoperative antihypertensive medications across all three pathological groups. All patients achieved biochemical cure following adrenalectomy, and no significant differences in the rates of hypertension cure or improvement were observed in comparisons across pathological subtype. CONCLUSIONS: Clinical presentation and postoperative outcomes are similar regardless of underlying pathology in patients with PA. Because one in four patients may harbor unilateral hyperplasia or multiple adenomas, total unilateral adrenalectomy should be performed as the operation of choice over adrenal-sparing approaches.
Assuntos
Adenoma/patologia , Neoplasias das Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/patologia , Adrenalectomia , Hiperaldosteronismo/cirurgia , Adenoma/complicações , Adenoma/cirurgia , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/cirurgia , Glândulas Suprarrenais/diagnóstico por imagem , Adulto , Aldosterona/sangue , Feminino , Humanos , Hiperaldosteronismo/etiologia , Hiperaldosteronismo/patologia , Hiperplasia/complicações , Hipertensão/etiologia , Masculino , Pessoa de Meia-Idade , Renina/sangue , Estudos RetrospectivosRESUMO
BACKGROUND: Criteria for diagnosing primary hyperparathyroidism (PHPT) include hypercalcemia in the presence of parathyroid hormone (PTH) levels that are either elevated (classic PHPT) or normal but non-suppressed. However, there is no standard definition of what constitutes normal non-suppressed levels, and data are lacking regarding the potential for surgical cure in these patients. METHODS: A retrospective review of patients undergoing parathyroidectomy for sporadic PHPT between 2012 and 2014 was performed. Patients with normal PTH were compared to classic PHPT patients to assess demographics, imaging, operative findings, and outcomes. RESULTS: In total, 332 patients met study criteria, and 60 (18%) had normal PTH levels. Negative sestamibi scans were seen more often with normal PTH levels (18.3 vs. 4.8%, p < 0.001). Patients with normal PTH were more likely to have ≥2 glands removed (26.7 vs. 14.3%, p = 0.02), and the specimens were more likely to be classified as only mildly hypercellular or normocellular (20 vs. 2.9%, p < 0.001). Average follow-up was 24 months (range 6-55). Cure rate was 88% in the normal PTH group, compared to 96% in classic PHPT (p = 0.02). Among patients with normal PTH, those with PTH ≤ 55 pg/mL had an 83% cure rate, whereas those with PTH 56-65 had a 96% cure rate (p = 0.12). CONCLUSIONS: Parathyroidectomy can have a high cure rate in the context of normal PTH levels despite an increased likelihood of negative imaging and multigland resection. Operative success is equivalent to classic PHPT when PTH levels are > 55 pg/mL.
Assuntos
Hiperparatireoidismo Primário/cirurgia , Paratireoidectomia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Hiperparatireoidismo Primário/metabolismo , Masculino , Pessoa de Meia-Idade , Hormônio Paratireóideo/metabolismo , Complicações Pós-Operatórias , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Contemporary guidelines for managing PTC advise an approach wherein primary tumor and regional metastases (RM) are completely resected at first surgery and radioiodine remnant ablation (RRA) is restricted to high-risk patients, policies our group has long endorsed. To assess our therapeutic efficacy, we studied 190 children and 4242 adults consecutively treated during 1936-2015. SUBJECTS AND METHODS: Mean follow-up durations for children and adults were 26.9 and 15.2 years, respectively. Bilateral lobar resection was performed in 86% of children and 88% of adults, followed by RRA in 30% of children and 29% of adults; neck nodes were excised in 86% of children and 66% of adults. Tumor recurrence (TR) and cause-specific mortality (CSM) details were taken from a computerized database. RESULTS: Children, when compared to adults, had larger primary tumors which more often were grossly invasive and incompletely resected. At presentation, children, as compared to adults, had more RM and distant metastases (DM). Thirty-year TR rates were no different in children than adults at any site. Thirty-year CSM rates were lower in children than adults (1.1 vs. 4.9%; p = 0.01). Comparing 1936-1975 (THEN) with 1976-2015 (NOW), 30-year CSM rates were similar in MACIS <6 children (p = 0.67) and adults (p = 0.08). However, MACIS <6 children and adults in 1976-2015 had significantly higher recurrence at local and regional, but not at distant, sites. MACIS 6+ adults, NOW, compared to THEN, had lower 30-year CSM rates (30 vs. 47%; p < 0.001), unassociated with decreased TR at any site. CONCLUSIONS: Children, despite presenting with more extensive PTC when compared to adults, have postoperative recurrences at similar frequency, typically coexist with DM and die of PTC less often. Since 1976, both children and adults with MACIS <6 PTC have a <1% chance at 30 years of CSM; adults with higher MACIS scores (6 or more) have a 30-year CSM rate of 30%.
Assuntos
Carcinoma Papilar/cirurgia , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia , Adulto , Fatores Etários , Carcinoma Papilar/mortalidade , Carcinoma Papilar/patologia , Carcinoma Papilar/radioterapia , Criança , Terapia Combinada , Feminino , Seguimentos , Humanos , Radioisótopos do Iodo/uso terapêutico , Masculino , Recidiva Local de Neoplasia , Probabilidade , Fatores de Risco , Câncer Papilífero da Tireoide , Neoplasias da Glândula Tireoide/mortalidade , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/radioterapia , Resultado do TratamentoRESUMO
BACKGROUND: Papillary thyroid carcinoma (PTC) is frequently associated with cervical lymph node metastases. Guidelines recommend performing ultrasound-guided fine-needle aspiration cytology (FNAC) for suspicious nodes to guide management. No specific recommendations are available for the use of FNA thyroglobulin assay (FNA-Tg). This study investigated the diagnostic value of performing FNAC and FNA-Tg. METHODS: Patient demographics, preoperative investigations, surgery, and lymph node pathology were collected for patients with PTC who underwent lateral neck lymphadenectomy and central compartment reexploration from January 2000 to July 2015. Sensitivities and accuracies were obtained. Patients with both diagnostic studies performed were compared using McNemar's test of paired proportion. Patient, imaging, and lymph node characteristics were correlated with test accuracy. RESULTS: The 480 patients in this study underwent 706 lateral neck dissections or central compartment reexploration. All the patients underwent preoperative neck ultrasound. Among these patients, FNAC alone was performed before 426 operations (60 %), FNAC with FNA-Tg before 105 operations (15 %), and surgery without biopsy for 175 patients (25 %). The sensitivity, positive predictive value, and accuracy were respectively 96, 95, 100 % for FNAC, 99, 97, and 97 % for FNA-Tg, and 95, 92, and 97 % for FNAC in combination with FNA-Tg. In the subgroup of patients who had both tests performed, the sensitivity of FNA-Tg was superior to that of FNAC (95 vs 87 %; p = 0.04). The addition of FNA-Tg to FNAC increased the detection of metastatic PTC by 13 %. CONCLUSIONS: For diagnosing cervical lymph node metastases in PTC, FNA-Tg is a valuable adjunct to FNAC. Its use should be considered to avoid missing metastatic disease in patients who may benefit from lymphadenectomy.
Assuntos
Carcinoma Papilar/metabolismo , Linfonodos/metabolismo , Linfonodos/patologia , Tireoglobulina/metabolismo , Neoplasias da Glândula Tireoide/metabolismo , Adulto , Biópsia por Agulha Fina , Carcinoma Papilar/diagnóstico , Carcinoma Papilar/secundário , Feminino , Humanos , Biópsia Guiada por Imagem , Linfonodos/cirurgia , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Pescoço , Esvaziamento Cervical , Valor Preditivo dos Testes , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/patologia , UltrassonografiaRESUMO
INTRODUCTION: Pheochromocytoma and paraganglioma (PPGL) are rare neoplasms; about 10% are malignant. Literature regarding possible benefit from resection is extremely limited. METHODS: A 20 year review of all patients undergoing surgery for malignant PPGL at the Mayo Clinic Rochester Campus between 1994 and June 2014 was performed. RESULTS: We identified 34 patients undergoing surgery for malignant PPGL. Median follow up was 6 and 5 years survival was 90% (median 11 years). Complete resection (R0) was achieved in 14 patients (41%). Median disease-free survival was 4.6 years for patients with R0 resection (up to 12 years). Only eight patients (23%) were disease-free on last follow up. Elevated preoperative fractionated metanephrines or catecholamines were documented in 23 patients (68%); these normalized in 13 of 23 patients (56%) postoperatively-with symptom relief in 15 of 18 preoperatively symptomatic patients (79%). Among 23 patients with hormone-producing tumors, significant reduction in number of antihypertensive medications was also noted postoperatively; 11 patients have remained off all antihypertensives, 6 required 1 medication, 1 required 2, while 5 required full blockade with phenoxybenzamine and a beta-adrenergic blocker. CONCLUSION: Surgery plays a significant role in the management of selected malignant PPGL. Resection can be effective in normalizing or significantly reducing levels of catecholamines and metanephrines, and can improve hormone-related symptoms and hypertension. Surgical resection, either complete or incomplete, is associated with durable survival despite a high rate of tumor recurrence.
Assuntos
Neoplasias das Glândulas Suprarrenais/cirurgia , Paraganglioma/cirurgia , Feocromocitoma/cirurgia , Adolescente , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Paraganglioma/patologia , Feocromocitoma/patologia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Adulto JovemRESUMO
OBJECTIVE: Individuals with neurofibromatosis type 1 (NF1) are at an increased risk of developing a pheochromocytoma or paraganglioma (PHEO/PGL). However, the best case detection strategy is unknown. Our objectives were to describe the prevalence, clinical presentation and outcomes of PHEO/PGL associated with NF1 and formulate case detection testing recommendations for PHEO/PGL. DESIGN: A retrospective cohort study from 1959 to 2015, Tertiary medical centre. PATIENTS AND MEASUREMENTS: We studied 41 patients with NF1 and PHEO/PGL who were identified using the PHEO/PGL and NF1 databases: 3289 and 1415 patients, respectively. Our main outcome measures were prevalence of PHEO/PGL in NF1 and occurrence of bilateral, recurrent, or metastatic disease and method of PHEO/PGL detection (symptoms vs incidental vs biochemical case detection testing). RESULTS: The prevalence of PHEO/PGL in patients with NF1 was 2·9%. The 41 patients included 23 men (56%) and 18 women. The median age at diagnosis was 41·0 years (range 14-67). The median tumour size was 3·4 cm (range 0·8-9·5). Bilateral PHEO was identified in 17% (n = 7) of patients, all women. Metastatic or recurrent disease occurred in 7·3% (n = 3). In the last 25 years, PHEO/PGL was diagnosed after incidental finding on computed imaging in 31% of patients (n = 11). Only three patients (7·3%) had PHEO/PGL discovered because of biochemical case detection testing. CONCLUSION: We recommend patients with NF1 have biochemical case detection testing for PHEO/PGL every 3 years starting at age 10 to 14 years. Biochemical case detection testing should also be carried out prior to elective surgical procedures and conception.
Assuntos
Neurofibromatose 1/complicações , Paraganglioma/etiologia , Feocromocitoma/etiologia , Adolescente , Adrenalectomia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Minnesota/epidemiologia , Paraganglioma/epidemiologia , Feocromocitoma/epidemiologia , Feocromocitoma/cirurgia , Prevalência , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Pheochromocytoma with synchronous ipsilateral adrenal cortical adenoma (PSCA) may present with mixed clinical, biochemical, and radiological features characteristic to each neoplasm subtype. METHODS: All patients with a pathological diagnosis of pheochromocytoma were evaluated for an ipsilateral cortical adenoma from 1994 through 2015. Retrospectively extracted data included indications for adrenalectomy, diagnostic workup (biochemical and radiographic), operative characteristics, pathological findings, and postoperative complications. RESULTS: Sixteen of 413 patients (4%) undergoing adrenalectomy for pheochromocytoma had a PSCA. Median patient age was 57.7 years (IQR 50.1, 63.1); 50% were male. On imaging, 75% of the adrenal neoplasms were found incidentally and only 50% were reported to have a synchronous ipsilateral neoplasm based on imaging findings. Clinically important cortical hormone secretion was diagnosed in 38% of these patients; 25% had glucocorticoid secretory autonomy; and 13% had primary aldosteronism. CONCLUSION: Physicians should be aware that adrenal neoplasms with mixed diagnostic findings may represent PSCA. Evaluation should be performed on this co-occurrence to prevent perioperative complications from resection of an unexpected secretory cortical neoplasm.
Assuntos
Corticosteroides/metabolismo , Neoplasias do Córtex Suprarrenal/cirurgia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adenoma Adrenocortical/cirurgia , Neoplasias Primárias Múltiplas/cirurgia , Feocromocitoma/cirurgia , Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/metabolismo , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/metabolismo , Adrenalectomia/efeitos adversos , Adenoma Adrenocortical/diagnóstico por imagem , Adenoma Adrenocortical/metabolismo , Adulto , Idoso , Feminino , Glucocorticoides/metabolismo , Humanos , Hiperaldosteronismo/etiologia , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/diagnóstico por imagem , Neoplasias Primárias Múltiplas/metabolismo , Feocromocitoma/diagnóstico por imagem , Feocromocitoma/metabolismo , Complicações Pós-Operatórias/cirurgia , Estudos RetrospectivosRESUMO
Patients with multiple endocrine neoplasia type 1 (MEN1) are commonly evaluated for clinical manifestations of this syndrome with the rationale that early diagnosis and adequate treatment will result in improved survival and quality of life. Thymic and bronchial carcinoid tumours are uncommon but important manifestations of MEN1. Current practice guidelines recommend evaluation with computed tomography or magnetic resonance imaging scan of the chest every 1-2 years to detect these neoplasms. However, the certainty that patients will be better off (increased survival or quality of life) as a result of this case detection strategy is based on evidence at moderate-high risk of bias that yields only imprecise results of indirect relevance to these patients. In order to improve the care that patients with MEN1 receive, co-ordinated efforts from different stakeholders are required so that large, prospective, multicentre studies evaluating patient important outcomes are carried out.
Assuntos
Neoplasias Brônquicas/diagnóstico , Tumor Carcinoide/diagnóstico , Detecção Precoce de Câncer/métodos , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Neoplasias do Timo/diagnóstico , Neoplasias Brônquicas/complicações , Tumor Carcinoide/complicações , Humanos , Imageamento por Ressonância Magnética , Neoplasia Endócrina Múltipla Tipo 1/complicações , Qualidade de Vida , Fatores de Risco , Neoplasias do Timo/complicações , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: To report the prevalence of parathyroid carcinoma (PC) in patients with multiple endocrine neoplasia type 1 (MEN1) and review of the literature. BACKGROUND: Primary hyperparathyroidism (PHP) is the most common manifestation of MEN1. The occurrence of PC in patients with MEN1 is rare and the literature regarding the clinical manifestations - including the prevalence of the disease - is scarce. CONTEXT: Single tertiary care centre experience from 1977 to 2013. DESIGN: Electronic search of the medical records to identify a cohort of patients with MEN1. Literature review based on current case reports. PATIENTS: Single case of PC in a cohort of 348 patients with MEN1. Ten cases of PC in patients with MEN1 reported in the literature. MEASUREMENT: Clinical features of PC in patients with MEN1. RESULTS: The prevalence of PC in 348 patients with MEN1 was found to be 0·28% (95% CI, 0-1·4%). Based on the current published cases of PC in patients with MEN1, 54·5% were women, mean age at diagnosis was 48·3 years, and the serum PTH concentrations at least four times the upper limit of the reference range in 73% of the cases. CONCLUSION: PC in patients with MEN 1 is rare with a prevalence of 0·28%, and the clinical features are similar to PC in patients without MEN1.
RESUMO
INTRODUCTION: Technological advances have brought about robotic single-site (RSS) cholecystectomy and hysterectomy. The application of RSS to additional procedures requires careful assessment of the surgeon learning curve, the technological limitations, patient selection criteria and associated outcomes. METHODS: Patient demographics, BMI, surgical indications, adrenal size, OR times, length of stay, postoperative pain and complications were assessed. RESULTS: Thirty-three patients underwent RSS-A by a single surgeon with 53 % being male, mean age 54 ± 16 years and mean BMI of 32.7. There were 18 left, 10 right, and 5 bilateral procedures for a total of 38 adrenal glands removed (mean tumor size 3.2 cm). There were 5 conversions to a laparoscopic approach, and two to open approach. The necessity for conversion was not associated with age, BMI, tumor size, surgical side or pathology (p > 0.05). The patients who underwent successful unilateral RSS-A had a profile of mean age 55, BMI 31, tumor size 3 cm, and a mean operative time of 118 ± 25.8 min. Pain scores were <4 (10 point scale) in 67 % of patients. 74 % of patients were discharged on POD 1 and 96 % were discharged by POD 2. An assessment of the quartile learning curve for the unilateral RSS-A showed operative times decreased from a mean of 124 to 103 min after 21 cases (p = 0.05). CONCLUSION: Patients with functioning and non-functioning tumors, along with those with obesity can safely be treated with RSS-A. The surgeon learning curve was associated with shortened operative times and not increased complication rates.