ANA AMELIA FIALHO DE OLIVEIRA HOFF
Projetos de Pesquisa
Unidades Organizacionais
Instituto do Câncer do Estado de São Paulo, Hospital das Clínicas, Faculdade de Medicina - Médico
12 resultados
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- Cytoreductive Surgery of the Primary Tumor in Metastatic Adrenocortical Carcinoma: Impact on Patients' Survival(2022) SROUGI, Victor; BANCOS, Irina; DAHER, Marilyne; LEE, Jeffrey E.; GRAHAM, Paul H.; KARAM, Jose A.; HENRIQUEZ, Andres; MCKENZIE, Travis J.; SADA, Alaa; BOURDEAU, Isabelle; POIRIER, Jonathan; VAIDYA, Anand; ABBONDANZA, Tiffany; KIERNAN, Colleen M.; RAO, Sarika N.; HAMIDI, Oksana; SACHITHANANDAN, Nirupa; HOFF, Ana O.; CHAMBO, Jose L.; ALMEIDA, Madson Q.; HABRA, Mouhammed Amir; V, Maria C. B. FragosoContext The role of cytoreduction of adrenocortical carcinoma (ACC) remains poorly understood. Objective To analyze the impact of cytoreductive surgery of the primary tumor in patients with metastatic ACC. Design and Setting We performed a multicentric, retrospective paired cohort study comparing the overall survival (OS) in patients with metastatic ACC who were treated either with cytoreductive surgery (CR group) or without cytoreductive surgery (no-CR group) of the primary tumor. Data were retrieved from 9 referral centers in the American-Australian-Asian Adrenal Alliance collaborative research group. Patients Patients aged >= 18 years with metastatic ACC at initial presentation who were treated between January 1, 1995, and May 31, 2019. Intervention Performance (or not) of cytoreductive surgery of the primary tumor. Main outcome and measures A propensity score match was done using age and the number of organs with metastasis (<= 2 or >2). The main outcome was OS, determined from the date of diagnosis until death or until last follow-up for living patients. Results Of 339 patients pooled, 239 were paired and included: 128 in the CR group and 111 in the no-CR group. The mean follow-up was 67 months. Patients in the no-CR group had greater risk of death than did patients in the CR group (hazard ratio [HR] = 3.18; 95% CI, 2.34-4.32). Independent predictors of survival included age (HR = 1.02; 95% CI, 1.00-1.03), hormone excess (HR = 2.56; 95% CI, 1.66-3.92), and local metastasis therapy (HR = 0.41; 95% CI, 0.47-0.65). Conclusion Cytoreductive surgery of the primary tumor in patients with metastatic ACC is associated with prolonged survival.
- Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy(2019) NEUMANN, Hartmut P. H.; TSOY, Uliana; BANCOS, Irina; AMODRU, Vincent; WALZ, Martin K.; TIROSH, Amit; KAUR, Ravinder Jeet; MCKENZIE, Travis; QI, Xiaoping; BANDGAR, Tushar; PETROV, Roman; YUKINA, Marina Y.; ROSLYAKOVA, Anna; HORST-SCHRIVERS, Anouk N. A. van der; BERENDS, Annika M. A.; HOFF, Ana O.; CASTRONEVES, Luciana Audi; FERRARA, Alfonso Massimiliano; RIZZATI, Silvia; MIAN, Caterina; DVORAKOVA, Sarka; HASSE-LAZAR, Kornelia; KVACHENYUK, Andrey; PECZKOWSKA, Mariola; LOLI, Paola; ERENLER, Feyza; KRAUSS, Tobias; ALMEIDA, Madson Q.; LIU, Longfei; ZHU, Feizhou; RECASENS, Monica; WOHLLK, Nelson; CORSSMIT, Eleonora P. M.; SHAFIGULLINA, Zulfiya; CALISSENDORFF, Jan; GROZINSKY-GLASBERG, Simona; KUNAVISARUT, Tada; SCHALIN-JANTTI, Camilla; CASTINETTI, Frederic; VLCEK, Petr; BELTSEVICH, Dmitry; I, Viacheslav Egorov; SCHIAVI, Francesca; LINKS, Thera P.; LECHAN, Ronald M.; BAUSCH, Birke; YOUNG JR., William F.; ENG, Charis; JAISWAL, Sanjeet Kumar; ZSCHIEDRICH, Stefan; V, Maria C. B. Fragoso; PEREIRA, Maria A. A.; LI, Minghao; COSTA, Josefina Biarnes; JUHLIN, Carl Christofer; GROSS, David; VIOLANTE, Alice H. D.; KOCJAN, Tomaz; NGEOW, Joanne; YOEL, Uri; FRAENKEL, Merav; SIMSIR, Ilgin Yildirim; UGURLU, M. Umit; ZIAGAKI, Athanasia; DIAZ, Luis Robles; KUDLAI, Inna Stepanovna; GIMM, Oliver; SCHERBAUM, Christina Rebecca; ABEBE-CAMPINO, Gadi; BARBON, Giovanni; TASCHIN, Elisa; MALINOC, Angelica; KHUDIAKOVA, Natalia Valeryevna; V, Nikita Ivanov; PFEIFER, Marija; ZOVATO, Stefania; PLOECKINGER, Ursula; MAKAY, Ozer; GRINEVA, Elena; JARZAB, Barbara; JANUSZEWICZ, Andrzej; SHAH, Nalini; SEUFERT, Jochen; OPOCHER, Giuseppe; LARSSON, CatharinaIMPORTANCE Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management. OBJECTIVE To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence. DESIGN, SETTING, AND PARTICIPANTS This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019. EXPOSURES Total or cortical-sparing adrenalectomy. MAIN OUTCOMES AND MEASURES Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality. RESULTS Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutationswere detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients developed recurrent pheochromocytoma in the adrenal bed despite total adrenalectomy. In contrast, 33 patients (13%) treated with successful cortical-sparing adrenalectomy developed another pheochromocytoma within the remnant adrenal after a median (IQR) of 8 (4-13) years, all of which were successfully treated with another surgery. Cortical-sparing surgery was not associated with survival. Overall survivalwas associated with comorbidities unrelated to pheochromocytoma: of 63 patients who died, only 3 (5%) died of metastatic pheochromocytoma. CONCLUSIONS AND RELEVANCE Patients undergoing cortical-sparing adrenalectomy did not demonstrate decreased survival, despite development of recurrent pheochromocytoma in 13%. Cortical-sparing adrenalectomy should be considered in all patients with hereditary pheochromocytoma.
- Sorafenib for the Treatment of Progressive Metastatic Medullary Thyroid Cancer: Efficacy and Safety Analysis(2016) CASTRONEVES, Luciana Audi de; NEGRAO, Marcelo Vailati; FREITAS, Ricardo Miguel Costa de; PAPADIA, Carla; LIMA JR., Jose Viana; FUKUSHIMA, Julia T.; SIMAO, Eduardo Furquim; KULCSAR, Marco Aurelio Vamondes; TAVARES, Marcos Roberto; JORGE, Alexander Augusto de Lima; CASTRO, Gilberto de; HOFF, Paulo Marcelo; HOFF, Ana OliveiraBackground: Treatment of advanced medullary thyroid carcinoma (MTC) was recently improved with the approval of vandetanib and cabozantinib. However, there is still a need to explore sequential therapy with more than one tyrosine kinase inhibitor (TKI) and to explore alternative therapies when vandetanib and cabozantinib are not available. This study reports the authors' experience with sorafenib as a treatment for advanced MTC. Methods: This is a retrospective longitudinal study of 13 patients with progressive metastatic MTC treated with sorafenib 400mg twice daily between December 2011 and January 2015. The primary endpoints were to evaluate response and progression-free survival (PFS) in patients treated with sorafenib outside a clinical trial. The secondary endpoint was an assessment of the toxicity profile. One patient was excluded because of a serious allergic skin rash one week after starting sorafenib. Results: The analysis included 12 patients with metastatic MTC (median age 48 years), 10 with sporadic and 2 with hereditary disease. The median duration of treatment was 11 months, and the median follow-up was 15.5 months. At data cutoff, 2/12 (16%) patients were still on treatment for 16 and 34 months. According to Response Evaluation Criteria in Solid Tumors analysis, 10 (83.3%) patients showed stable disease, and two (16.6%) had progression of disease; no partial response was observed. The median PFS was nine months. However, three patients with extensive and rapidly progressive disease died within three months of sorafenib treatment. The median PFS excluding these three patients was 12 months. Adverse events (AE) occurred in nine (75%) patients. The main AEs were skin toxicity, weight loss, and fatigue. Five (41.6%) patients needed dose reduction, and one patient discontinued treatment because of toxicity. Conclusions: Treatment with sorafenib in progressive metastatic MTC is well tolerated and resulted in disease control and durable clinical benefit in 75% of patients. Sorafenib treatment could be considered when vandetanib and cabozantinib are not available or after failing these drugs.
- Assessment of Depression, Anxiety, Quality of Life, and Coping in Long-Standing Multiple Endocrine Neoplasia Type 2 Patients(2017) RODRIGUES, Karine C.; TOLEDO, Rodrigo A.; COUTINHO, Flavia L.; NUNES, Adriana B.; MACIEL, Rui M. B.; HOFF, Ana O.; TAVARES, Marcos C.; TOLEDO, Sergio P. A.; LOURENCO JR., Delmar M.Background: Data on psychological harm in multiple endocrine neoplasia type 2 (MEN2) are scarce. Objectives: The aim of this study was to assess anxiety, depression, quality of life, and coping in long-standing MEN2 patients. Patients and Methods: Patients were 43 adults (age >= 18 years) with clinical and genetic diagnosis of MEN2 and long-term follow-up (10.6 +/- 8.2 years; range 1-33 years). This was a cross-sectional study with qualitative and quantitative psychological assessment using semi-directed interviews and HADS, EORTC QLQ C30, and MINI-MAC scales. Adopting clinical criteria from 2015 ATA Guidelines on MEN2, biochemical cure (39%; 16/41), persistence/recurrence (61%; 25/41), and stable chronic disease (22/41) of medullary thyroid carcinoma (MTC) were scored. Pheochromocytoma affected 19 (44%) patients, with previous adrenalectomy in 17 of them. Results: Overall, anxiety (42%; mean score 11 +/- 2.9; range 8-18; anxiety is defined as a score >= 8) and depression (26%; mean score 11 +/- 3.8; range 8-20; depression is defined as a score >= 8) symptoms were frequent. Patients who transmitted RET mutations to a child had higher scores for weakness-discouragement/anxious preoccupation and lower scores for cognitive, emotional, and physical functioning (p < 0.05). Feelings of guilt were present in 35% of patients with mutation-positive children. Lower mean score values for depression and anxiety and higher scores for role, cognitive, and emotional functioning were noticed in 33 patients who were well-informed about their disease (p < 0.05). Fighting spirit was more frequently found in patients with multiple surgical procedures (p = 0.019) and controlled chronic adrenal insufficiency (p = 0.024). Patients with MEN2-elated stress-inducing factors had lower scores for fighting spirit and cognitive functioning and higher scores for insomnia and dyspnea (p < 0.05). Eleven patients required sustained psychotherapeutic treatment. Mean global health status was relatively good in MEN2 cases (68.1 +/- 22.3), and the cured group had higher physical functioning (p = 0.021). Conclusions: Psychological distress is likely chronic in MEN2 patients. This study identified diverse MEN2-related factors (degree of information on disease, mutation-positive children, number of surgeries, comorbidities, stress-inducing factors, and cure) interfering positively or negatively with the results of the psychometrics scales. The active investigation of these factors and the applied psychological assessment protocol are useful to identify MEN2 patients requiring psychological assistance.
- Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study(2019) CASTINETTI, Frederic; WAGUESPACK, Steven G.; MACHENS, Andreas; UCHINO, Shinya; HASSE-LAZAR, Kornelia; SANSO, Gabriella; ELSE, Tobias; DVORAKOVA, Sarka; QI, Xiao Ping; ELISEI, Rossella; MAIA, Ana Luisa; GLOD, John; LOURENCO JR., Delmar Muniz; VALDES, Nuria; MATHIESEN, Jes; WOHLLK, Nelson; BANDGAR, Tushar R.; DRUI, Delphine; KORBONITS, Marta; DRUCE, Maralyn R.; BRAIN, Caroline; KURZAWINSKI, Tom; PATOCS, Atila; BUGALHO, Maria Joao; LACROIX, Andre; CARON, Philippe; FAINSTEIN-DAY, Patricia; CHAZOT, Francoise Borson; KLEIN, Marc; LINKS, Thera P.; LETIZIA, Claudio; FUGAZZOLA, Laura; CHABRE, Olivier; CANU, Letizia; COHEN, Regis; TABARIN, Antoine; UROIC, Anita Spehar; MAITER, Dominique; LABOUREAU, Sandrine; MIAN, Caterina; PECZKOWSKA, Mariola; SEBAG, Frederic; BRUE, Thierry; MIREBEAU-PRUNIER, Delphine; LECLERC, Laurence; BAUSCH, Birke; BERDELOU, Amandine; SUKURAI, Akihiro; VLCEK, Petr; KRAJEWSKA, Jolanta; BARONTINI, Marta; VARGAS, Carla Vaz Ferreira; VALERIO, Laura; CEOLIN, Lucieli; AKSHINTALA, Srivandana; HOFF, Ana; GODBALLE, Christian; JARZAB, Barbara; JIMENEZ, Camilo; ENG, Charis; IMAI, Tsuneo; SCHLUMBERGER, Martin; GRUBBS, Elizabeth; DRALLE, Henning; NEUMANN, Hartmut P.; BAUDIN, EricBackground Multiple endocrine neoplasia type 2B is a rare syndrome caused mainly by Met918Thr germline RET mutation, and characterised by medullary thyroid carcinoma, phaeochromocytoma, and extra-endocrine features. Data are scarce on the natural history of multiple endocrine neoplasia type 2B. We aimed to advance understanding of the phenotype and natural history of multiple endocrine neoplasia type 2B, to increase awareness and improve detection. Methods This study was a retrospective, multicentre, international study in patients carrying the Met918Thr RET variant with no age restrictions. The study was done with registry data from 48 centres globally. Data from patients followed-up from 1970 to 2016 were retrieved from May 1, 2016, to May 31, 2018. Our primary objectives were to determine overall survival, and medullary thyroid carcinoma-specific survival based on whether the patient had undergone early thyroidectomy before the age of 1 year. We also assessed remission of medullary thyroid carcinoma, incidence and treatment of phaeochromocytoma, and the penetrance of extra-endocrine features. Findings 345 patients were included, of whom 338 (98%) had a thyroidectomy. 71 patients (21%) of the total cohort died at a median age of 25 years (range <1-59). Thyroidectomy was done before the age of 1 year in 20 patients, which led to long-term remission (ie, undetectable calcitonin level) in 15 (83%) of 18 individuals (2 patients died of causes unrelated to medullary thyroid carcinoma). Medullary thyroid carcinoma-specific survival curves did not show any significant difference between patients who had thyroidectomy before or after 1 year (comparison of survival curves by log-rank test: p=0.2; hazard ratio 0.35; 95% CI 0.07-1.74). However, there was a significant difference in remission status between patients who underwent thyroidectomy before and after the age of 1 year (p<0.0001). There was a significant difference in remission status between patients who underwent thyroidectomy before and after the age of 1 year (p<0.0001). In the other 318 patients who underwent thyroidectomy after 1 year of age, biochemical and structural remission was obtained in 47 (15%) of 318 individuals. Bilateral phaeochromocytoma was diagnosed in 156 (50%) of 313 patients by 28 years of age. Adrenal-sparing surgery was done in 31 patients: three (10%) of 31 patients had long-term recurrence, while normal adrenal function was obtained in 16 (62%) patients. All patients with available data (n=287) had at least one extra-endocrine feature, including 106 (56%) of 190 patients showing marfanoid body habitus, mucosal neuromas, and gastrointestinal signs. Interpretation Thyroidectomy done at no later than 1 year of age is associated with a high probability of cure. The reality is that the majority of children with the syndrome will be diagnosed after this recommended age. Adrenal-sparing surgery is feasible in multiple endocrine neoplasia type 2B and affords a good chance for normal adrenal function. To improve the prognosis of such patients, it is imperative that every health-care provider be aware of the extra-endocrine signs and the natural history of this rare syndrome. The implications of this research include increasing awareness of the extra-endocrine symptoms and also recommendations for thyroidectomy before the age of 1 year.
- LIBRETTO-531: a phase III study of selpercatinib in multikinase inhibitor-naive RET-mutant medullary thyroid cancer(2022) WIRTH, Lori J.; BROSE, Marcia S.; ELISEI, Rossella; CAPDEVILA, Jaume; HOFF, Ana O.; I, Mimi Hu; TAHARA, Makoto; ROBINSON, Bruce; GAO, Ming; XIA, Meng; MAEDA, Patricia; SHERMAN, EricSelpercatinib is a first-in-class, highly selective and potent, central nervous system-active RET kinase inhibitor. In the phase I/II trial, selpercatinib demonstrated clinically meaningful antitumor activity with manageable toxicity in heavily pre-treated and treatment-naive patients with RET-mutant medullary thyroid cancer (MTC). LIBRETTO-531 (NCT04211337) is a multicenter, open-label, randomized, controlled, phase III trial comparing selpercatinib to cabozantinib or vandetanib in patients with advanced/metastatic RET-mutant MTC. The primary objective is to compare progression-free survival (per RECIST 1.1) by blinded independent central review of patients with progressive, advanced, multikinase inhibitor-naive, RET-mutant MTC treated with selpercatinib versus cabozantinib or vandetanib. Key secondary objectives are to compare other efficacy outcomes (per RECIST 1.1) and tolerability of selpercatinib versus cabozantinib or vandetanib. Plain language summary: Selpercatinib (also known by the brand name Retevmo (R)/Retsevmo (R)) is a new treatment available in multiple countries for people with advanced or metastatic RET-mutant medullary thyroid cancer (MTC). Thyroid cancer starts in your thyroid gland and may spread or metastasize to other parts of the body, including lungs, bones, and occasionally the brain, which means the cancer is likely to be advanced. Advanced thyroid cancer can be driven by a gene in your body, one of which is RET. This is a summary of the LIBRETTO-531 study which compares selpercatinib, which is a strong and selective inhibitor of RET, with two approved drugs, cabozantinib and vandetanib. Patients with advanced or metastatic RET-mutant MTC who have not already received treatment with kinase inhibitors are being enrolled. This trial will evaluate how long people during and after treatment live with the disease without it getting worse. Selpercatinib may affect both healthy cells and tumor cells, which can result in side effects, which will also be evaluated in this study. This study is active and currently recruiting new patients.
- Defining Radioiodine-Refractory Differentiated Thyroid Cancer: Efficacy and Safety of Lenvatinib by Radioiodine-Refractory Criteria in the SELECT Trial(2017) KIYOTA, Naomi; ROBINSON, Bruce; SHAH, Manisha; HOFF, Ana O.; TAYLOR, Matthew H.; LI, Di; DUTCUS, Corina E.; LEE, Eun Kyung; KIM, Sung-Bae; TAHARA, MakotoBackground: While there is a clear consensus for defining radioiodine-refractory differentiated thyroid cancer (RR-DTC), it is unknown whether these criteria are equally valid for determining when radioiodine (RAI) therapy is no longer beneficial and systemic treatment should be considered. Lenvatinib, a multikinase inhibitor, significantly prolonged progression-free survival (PFS) compared to placebo in a Phase 3 trial in RR-DTC (SE-LECT; hazard ratio [HR]: 0.21 [99% confidence interval (CI) 0.14-0.31]; p < 0.001). This sub-analysis compared clinical outcomes of lenvatinib-treated patients in SELECT stratified by RR-DTC inclusion criteria. Methods: In SELECT, patients with measurable RR-DTC and radiologic evidence of disease progression <= 13 months prior to study entry were randomized 2: 1 to lenvatinib (24 mg/day; 28-day cycle) or placebo. In this analysis, patients were stratified based on the following RR-DTC inclusion criteria: no RAI uptake, disease progression within 12 months of RAI therapy despite RAI avidity at the time of treatment, and extensive (>600 mCi) cumulative RAI exposure. All had disease progression as an inclusion criterion for SELECT. Results: Of 392 patients (261 lenvatinib; 131 placebo) enrolled, 275, 235, and 73 patients met the inclusion criteria for no RAI uptake, disease progression despite RAI avidity, and extensive RAI exposure, respectively. There was significant overlap between the patient groups, with 167 (42.6%) patients meeting more than one inclusion criterion. Lenvatinib improved median PFS compared to placebo in all groups (""no RAI uptake"": lenvatinib not quantifiable [NQ; CI 14.8-NQ] vs. placebo, 3.7 months [CI 2.5-5.3]; ""disease progression despite RAI avidity"": lenvatinib 16.5 months [CI 12.8-NQ] vs. placebo, 3.7 months [CI 1.9-5.4]; ""extensive RAI exposure"": lenvatinib 18.7 months [CI 10.7-NQ] vs. placebo, 3.6 months [CI 1.9-5.5]). Objective response rates were 71.8%, 60.0%, and 56.0% for patients with no RAI uptake, disease progression despite RAI avidity, and extensive RAI exposure, respectively. Lenvatinib-related adverse events were similar across groups. Conclusions: Comparable efficacy and safety profiles were observed in lenvatinib-treated patients regardless of RR-DTC criteria, possibly because of a large overlap among patients fulfilling each criterion. However, differing definitions for RR-DTC may be equally valid because both lenvatinib and placebo arms exhibited similar PFS outcomes across groups.
- Adjuvant radiotherapy for the primary treatment of adrenocortical carcinoma: are we offering the best?(2017) SROUGI, Victor; BESSA JUNIOR, Jose; TANNO, Fabio Y.; FERREIRA, Amanda M.; HOFF, Ana O.; BEZERRA, Joao E.; ALMEIDA, Cristiane M.; ALMEIDA, Madson Q.; MENDONCA, Berenice B.; NAHAS, William C.; CHAMBO, Jose L.; SROUGI, Miguel; FRAGOSO, Maria C. B. V.Purpose: To evaluate the role of ARDT after surgical resection of ACC. Materials and Methods: Records of patients from our institutional ACC database were retrospectively assessed. A paired comparison analysis was used to evaluate the oncological outcomes between patients treated with surgery followed by ARDT or surgery only (control). The endpoints were LRFS, RFS, and OS. A systematic review of the literature and metaanalysis was also performed to evaluate local recurrence of ACC when ARDT was used. Results: Ten patients were included in each Group. The median follow-up times were 32 months and 35 months for the ARDT and control Groups, respectively. The results for LRFS (p= 0.11), RFS (p= 0.92), and OS (p= 0.47) were similar among subsets. The mean time to present with local recurrence was significantly longer in the ARDT group compared with the control Group (419 +/- 206 days vs. 181 +/- 86 days, respectively; p= 0.03). ARDT was well tolerated by the patients; there were no reports of late toxicity. The meta-analysis, which included four retrospective series, revealed that ARDT had a protective effect on LRFS (HR= 0.4; CI= 0.17-0.94). Conclusions: ARDT may reduce the chance and prolong the time to ACC local recurrence. However, there were no benefits for disease recurrence control or overall survival for patients who underwent this complementary therapy.
- Potential role of sorafenib as neoadjuvant therapy in unresectable papillary thyroid cancer(2018) DANILOVIC, Debora L. S.; CASTRO JR., Gilberto; ROITBERG, Felipe S. R.; VANDERLEI, Felipe A. B.; BONANI, Fernanda A.; FREITAS, Ricardo M. C.; COURA-FILHO, George B.; CAMARGO, Rosalinda Y.; KULCSAR, Marco A.; MARUI, Suemi; HOFF, Ana O.Total thyroidectomy, radioiodine (RAI) therapy, and TSH suppression are the mainstay treatment for differentiated thyroid carcinomas (DTCs). Treatments for metastatic disease include surgery, external-beam radiotherapy, RAI, and kinase inhibitors for progressive iodine-refractory disease. Unresectable locoregional disease remains a challenge, as standard therapy with RAI becomes unfeasible. We report a case of a young patient who presented with unresectable papillary thyroid carcinoma (PTC), and treatment with sorafenib allowed total thyroidectomy and RAI therapy. A 20-year-old male presented with severe respiratory distress due to an enlarging cervical mass. Imaging studies revealed an enlarged multinodular thyroid gland, extensive cervical adenopathy, severe tracheal stenosis, and pulmonary micronodules. He required an urgent surgical intervention and underwent tracheostomy and partial left neck dissection, as the disease was deemed unresectable; pathology revealed PTC. Treatment with sorafenib was initiated, resulting in significant tumor reduction allowing near total thyroidectomy and bilateral neck dissection. Postoperatively, the patient underwent radiotherapy for residual tracheal lesion, followed by RAI therapy for avid cervical and pulmonary disease. The patient's disease remains stable 4 years after diagnosis. Sorafenib has been approved for progressive RAI-refractory metastatic DTCs. In this case report, we describe a patient with locally advanced PTC in whom treatment with sorafenib provided sufficient tumor reduction to allow thyroidectomy and RAI therapy, suggesting a potential role of sorafenib as an induction therapy of unresectable DTC.
- AACE/ACE DISEASE STATE CLINICAL REVIEW: PANCREATIC NEUROENDOCRINE INCIDENTALOMAS(2015) HERRERA, Miguel F.; AKERSTROM, Goran; ANGELOS, Peter; GRANT, Clive S.; HOFF, Ana O.; PANTOJA, Juan Pablo; PEREZ-JOHNSTON, Rocio; SAHANI, Dushyant V.; WONG, Richard J.; RANDOLPH, GregoryIncidental detection of pancreatic neuroendocrine tumors (PNETs) has substantially increased over the last decade due to widespread use of advanced imaging studies. Reliable initial imaging-based characterization is crucial for the differential diagnosis from other exocrine neoplasms and to determine the appropriate management plan. Measurements of chromogranin A, pancreatic polypeptide, and calcitonin are recommended for the biochemical evaluation. A thorough medical history needs to be performed to rule out multiple endocrine neoplasia (MEN) type 1. The European Neuroendocrine Tumor Society (ENETS)/Tumor Node Metastasis (TNM) staging system together with a grading based on the Ki-67 proliferation index and mitotic counts has proven to give more appropriate prognostic information than the World Health Organization (WHO)/American Joint Committee on Cancer (AJCC) TNM staging but may still fail to safely differentiate benign from malignant lesions. Poorly differentiated PNETs generally present with metastases and are rarely amenable for resection. Well-or intermediately differentiated tumors >= 2 cm with imaging evidence of malignancy or with a Ki-67 > 2% should be resected. It has been suggested that non-MEN related, nonfunctioning, and asymptomatic PNETs < 2 cm with a Ki-67 index >= 2% carry a low risk of metastasis and may be observed in the absence of clinical or radiologic criteria of malignancy or progression, especially in older patients. However, because metastases may occur with long delay with smaller PNETS, physicians should consider patient age, lesion location, and the risks of operation, and patients not undergoing surgery need to be closely followed closely.