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1.
Br J Surg ; 111(2)2024 Jan 31.
Artigo em Inglês | MEDLINE | ID: mdl-38306505

RESUMO

BACKGROUND: There is a surgeon volume-outcome effect in adrenal surgery but the threshold for high-volume surgeon remains controversial. This study aimed to determine predictors of high-risk adrenal operations and to explore whether these should be restricted to high-volume surgeons. METHODS: Patients undergoing adrenal surgery and registered in the United Kingdom Registry of Endocrine and Thyroid Surgery between 2004 and 2021 were analysed. Outcomes included postoperative complications, duration of hospital stay, and mortality. Factors included in multivariable analysis were age, sex, diagnosis, surgical approach, laterality, and surgeon volume. Patients with missing data were excluded. RESULTS: A total of 4464 of 6174 patients (72.3%) were analysed. Postoperative complications occurred in 418 patients (9.4%) and 14 (0.3%) died. Median duration of hospital stay was 3 (i.q.r. 2-5) days. Co-variables significantly associated with an increase or decrease in postoperative complications (P < 0.050) were age (OR 1.02, 95% c.i. 1.01 to 1.03), adrenal cancer (OR 1.64, 1.14 to 2.36), minimally invasive approach (OR 0.317, 0.248 to 0.405), bilateral surgery (OR 1.66, 1.03 to 2.69), and surgeon volume (OR 0.98, 0.96 to 0.99). An increase or decrease in mortality was associated with patient age (OR 1.08, 1.03 to 1.13), minimally invasive approach (OR 0.08, 0.02 to 0.27), and bilateral surgery (OR 6.93, 1.40 to 34.34). The incidence of postoperative complications was significantly lower above a threshold of 12 operations per year (P = 0.034) and 20 per year (P < 0.001), but not six per year (P = 0.540). Median duration of hospital stay was 2 days for surgeons doing over 20 operations per year, compared with 3 days for those undertaking fewer than 20, fewer than 12 or fewer than 6 operations per year. CONCLUSION: Increasing surgical volume is associated with shorter hospital stay and fewer complications. This analysis supports the case for centralization of surgery for adrenal cancer and bilateral tumours to higher-volume surgeons performing a minimum of 12 operations per year.


The adrenal glands are found in the fatty tissue at the back of the abdomen above each kidney, and produce steroid and adrenaline hormones. Surgery on tumours of the adrenal gland is uncommon compared with surgery for other tumours such as those of the breast, bowel, kidney, and lung. Research has shown that the more adrenal operations a surgeon undertakes per year, the better the overall outcomes for patients undergoing that type of surgery. In this study, the outcomes from adrenal operations recorded over 18 years in the national adrenal surgical registry by members of the British Association of Endocrine and Thyroid Surgeons were analysed. The results confirmed previous findings showing that postoperative complications and length of hospital stay were reduced for patients operated by surgeons who did more adrenal operations per year. Operations done by keyhole surgery had better outcomes. Operations done either in older patients, or for the rare adrenal cancer tumours had worse outcomes, as did operations in which both adrenal glands were removed. The authors recommended that all surgeons performing adrenal surgery should monitor the outcomes of their operations, ideally in a national registry, and discuss these with patients before surgery; and undertake a minimum of 6 adrenal operations per year, but a minimum of 12 per year if doing surgery for adrenal cancer or surgery to remove both adrenal glands.


Assuntos
Neoplasias das Glândulas Suprarrenais , Cirurgiões , Humanos , Glândula Tireoide/cirurgia , Reino Unido/epidemiologia , Sistema de Registros , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia
2.
BJS Open ; 7(4)2023 07 03.
Artigo em Inglês | MEDLINE | ID: mdl-37578027

RESUMO

BACKGROUND: Acute cholecystitis is one of the most common diagnoses presenting to emergency general surgery and is managed either operatively or conservatively. However, operative rates vary widely across the world. This real-world population analysis aimed to describe the current clinical management and outcomes of patients with acute cholecystitis across Scotland, UK. METHODS: This was a national cohort study using data obtained from Information Services Division, Scotland. All adult patients with the admission diagnostic code for acute cholecystitis were included. Data were used to identify all patients admitted to Scottish hospitals between 1997 and 2019 and outcomes tracked for inpatients or after discharge through the unique patient identifier. This was linked to death data, including date of death. RESULTS: A total of 47 558 patients were diagnosed with 58 824 episodes of acute cholecystitis (with 27.2 per cent of patients experiencing more than one episode) in 46 Scottish hospitals. Median age was 58 years (interquartile range (i.q.r.) 43-71), 64.4 per cent were female, and most (76.1 per cent) had no comorbidities. A total of 28 741 (60.4 per cent) patients had an operative intervention during the index admission. Patients who had an operation during their index admission had a lower risk of 90-day mortality compared with non-operative management (OR 0.62, 95% c.i. 0.55-0.70). CONCLUSION: In this study, 60 per cent of patients had an index cholecystectomy. Patients who underwent surgery had a better survival rate compared with those managed conservatively, further advocating for an operative approach in this cohort.


Assuntos
Colecistite Aguda , Gerenciamento Clínico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Colecistectomia/normas , Colecistectomia/estatística & dados numéricos , Colecistite Aguda/diagnóstico , Colecistite Aguda/mortalidade , Colecistite Aguda/cirurgia , Colecistite Aguda/terapia , Estudos de Coortes , Hospitalização/estatística & dados numéricos , Escócia , Idoso , Taxa de Sobrevida
3.
World J Surg ; 47(5): 1221-1230, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36593341

RESUMO

BACKGROUND: Outcomes in endocrine surgery have been shown to improve with surgeon volume. We aimed to study the effect of surgeon volume on morbidity following parathyroidectomy. METHODS: UKRETS data from 2004 to 2019 was studied. Parathyroidectomies for primary hyperparathyroidism with complete data were included. Exclusion criteria were age <18 or >80 years; surgeons contributing <10 cases overall; and length of stay >28 days. Multivariable analysis was performed. Primary outcome was persistent hypercalcaemia; secondary outcomes were haemorrhage, length of stay, need for re-admission, post-operative hypocalcaemia, and need for calcium/vitamin D supplements to maintain eucalcaemia at 6 months. RESULTS: 153 surgeons undertook mean 22.5 (median 17, range 2-115) parathyroidectomies/year. Persistent hypercalcaemia affected 4.8% (776/16140) overall; 5.7% (71/1242) in surgeons undertaking < 10 cases/year; 5.1% (3339/6617) for 10-30 cases/year; 5.0% (270/5397) for 30-50 cases; and 3.3% (96/2884) for >50 cases/year. High-volume (>50 parathyroidectomies/year) surgeons operated 23.4% (809/3464) of negative localisation cases compared to 16.4% (2074/12676) of positive localisation cases. Persistent hypercalcaemia was almost twice as common in image negative (7.9%) compared to image-positive (4%) cases. Persistent hypercalcaemia was significantly more likely to occur in the low volume (<10 parathyroidectomies/year) group than high volume (>50 parathyroidectomies/year), regardless of image positivity (p = 0.0006). Surgeon volume significantly reduced persistent hypercalcaemia on multivariable analysis (OR = 0.878, 95%CI 0.842-0.914, p < 0.001), along with age, sex, and positive localisation. BNE and re-operation significantly increased persistent hypercalcaemia. Post-operative hypocalcaemia occurred in 3.2% (509/16040) and was reduced with increasing surgeon volume (OR = 0.951, 95%CI 0.910-0.993, p < 0.001). Haemorrhage and length of stay were not significantly associated with surgeon volume. CONCLUSION: The incidence of persistent hypercalcaemia, post-operative hypocalcaemia, and persistent hypoparathyroidism decreased with increasing surgeon volume. The relative reduction in persistent hypercalcaemia with surgeon volume was similar in image negative and positive groups, but the absolute reduction was higher in image negative cases. Restricting image negative parathyroidectomy to high-volume surgeons could be considered.


Assuntos
Hipercalcemia , Hipocalcemia , Cirurgiões , Humanos , Idoso de 80 Anos ou mais , Paratireoidectomia/efeitos adversos , Paratireoidectomia/métodos , Glândula Tireoide , Hipocalcemia/epidemiologia , Hipocalcemia/etiologia , Hipercalcemia/epidemiologia , Hipercalcemia/etiologia , Incidência , Sistema de Registros , Reino Unido/epidemiologia
4.
Eur J Breast Health ; 17(1): 10-14, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33796825

RESUMO

OBJECTIVE: Phyllodes tumor (PT) is a rare entity accounting for 1% of breast neoplasms with a high propensity of recurrence. This study aimed to identify factors that are predictive of early recurrence in patients with PT. MATERIALS AND METHODS: This study reviewed clinical data of patients with PT (n=57) treated at our tertiary care referral center in South India between February 2010 and December 2019. The Pearson χ2 test was used to investigate the relationship between patient's clinical features and tumor histotypes. Survival curves were obtained using the Kaplan-Meier method based on the log-rank test. Multivariate Cox regression analyses were performed to identify predictors of early recurrence or local recurrence-free-interval (LRFI). RESULTS: The mean age was 38.3 [standard deviation (SD)=13.6] years, and the mean follow-up was 18 (SD=13.5) months. The median tumor size was 5 cm (interquartile range 3 and range: 3-22 cm). Moreover, 64.9% (n=37) of the tumors were benign, 21.1% (n=12) were borderline, and 14% (n=8) were malignant. Of the 57 patients, 17 (29.8%) developed local recurrence and one developed distant metastasis. Of the 17 patients, three were unwilling to undergo completion surgery. The median LRFI was 20 (range: 7-60) months. Multivariate cox regression analyses showed that mitotic rate >10/high power field [hazard ratio (HR) 0.147; p=0.04], stromal overgrowth (HR: 4.904; p=0.05), margin status (HR: 0.037; p<0.001), and preoperative neutrophil-to-lymphocyte ratio [(NLR), HR: 4.891; p=0.04)] were significant predictors of LRFI. CONCLUSION: A high mitotic rate, positive margin, stromal overgrowth, and NLR >3.5 were associated with early recurrence. These attributes mandate stringent follow-up, especially in a resource-limited setting.

5.
Biomark Med ; 15(5): 331-336, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33595341

RESUMO

Background: The optimum threshold of IOPTH decay remains a debate and numerous criteria have been described. In this study, we utilize a single-sample IOPTH taken 10 min post excision. Materials & methods: This 4-year query of a prospectively maintained database included primary hyperparathyroidism patients with pre-operative PTH done 1 week prior to surgery, and a 10-min post excision IOPTH value. Optimal cut-off for PTH and sensitivity/specificity were calculated. Results: A total of 93 patients had single-gland disease, of whom 79 (84.9%) were symptomatic. The 10-min post excision assay sensitivity in single-gland disease was 97.8% (50% fall), 95% (60% fall) and 83.9% (70% fall). Conclusion: A post excision single-shot IOPTH assay with a 50% fall offers a sensitivity of 97.8% in patients of primary hyperparathyroidism with single-gland benign disease.


Assuntos
Hiperparatireoidismo Primário/patologia , Hormônio Paratireóideo/metabolismo , Paratireoidectomia/métodos , Adulto , Idoso , Países em Desenvolvimento , Feminino , Humanos , Hiperparatireoidismo Primário/epidemiologia , Hiperparatireoidismo Primário/metabolismo , Hiperparatireoidismo Primário/cirurgia , Índia/epidemiologia , Cuidados Intraoperatórios , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Adulto Jovem
6.
Biomark Med ; 14(12): 1121-1126, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32969250

RESUMO

Background: We aimed to assess the predictive value of the absolute and relative intact parathormone (iPTH) decline levels as reliable markers of postoperative hypocalcemia. Materials & methods: iPTH levels were measured 4 h after surgery and the following morning after surgery (postoperative day 1). iPTH, absolute iPTH decline (ΔPTH) and relative iPTH decline (ΔPTH%) were calculated and correlated with symptomatic hypocalcemia. Results: Of the 95 patients, 20% of patients (n = 19) developed symptomatic hypocalcemia. The ΔPTH (U = 206; p < 0.001) and ΔPTH% (U = 127; p < 0.001) were significantly higher in patients with symptomatic hypocalcemia. A ΔPTH% of 20% (sensitivity of 84%; specificity of 91%); and an absolute iPTH decline of 3.75 pg/ml (sensitivity of 74%; specificity of 87%) were highly predictive of symptomatic hypocalcemia. Conclusion: Postoperative ΔPTH and ΔPTH% have the potential to be predictors of symptomatic hypocalcemia following thyroidectomy and could facilitate a safe early discharge.


Assuntos
Hipocalcemia/diagnóstico , Hipocalcemia/etiologia , Hormônio Paratireóideo/sangue , Hormônio Paratireóideo/deficiência , Tireoidectomia/efeitos adversos , Adulto , Biomarcadores/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/sangue , Fatores de Tempo
7.
J Burn Care Res ; 41(6): 1304-1305, 2020 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-32735668

RESUMO

Electrical injuries are among the most devastating of burn injuries. High-voltage electrical injuries result in extensive deep tissue damage and are associated with multiple complications, long-term morbidity, and a high mortality rate. We describe the case of a 16-year-old male cable operator who suffered high-voltage electric injury of left upper limb and anterior abdominal wall. Despite the exit wound causing transection of the ileum, the patient did not have any contamination of the peritoneal cavity as both ends of the bowel were sealed off due to electro-thermal energy. His management included multiple operative procedures, including intestinal resection and anastomosis, debridement, abdominal wall flap-based reconstruction, fasciotomies, split skin grafting, and left hand above elbow amputation.


Assuntos
Queimaduras por Corrente Elétrica/terapia , Intestino Delgado/lesões , Intestino Delgado/cirurgia , Adolescente , Amputação Cirúrgica , Traumatismos do Braço/cirurgia , Desbridamento , Fasciotomia , Humanos , Masculino , Procedimentos de Cirurgia Plástica , Transplante de Pele
8.
Indian J Endocrinol Metab ; 23(4): 486-490, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31741911

RESUMO

BACKGROUND AND AIMS: Pheochromocytomas (PCCs) and Paragangliomas (PGL) are rare catecholamine producing tumors that may present in sporadic or familial settings. Despite vast strides in understanding of PCC/PGL genetics in the last two decades, there is a dearth of information from India. The aim here is to study the prevalence of genetic mutations in Indian PCC/PGL patients. SETTINGS AND DESIGN: Tertiary care academic hospital; prospective study. METHODS: 50 histopathologically diagnosed PCC/PGL patients formed the study group. Clinical, biochemical, pathological attributes and outcomes were documented and the phenotype was compared to the genotype. Succinyl dehydrogenase (SDH), Re-Arranged during Transfection (RET), Von-Hippel-Lindau (VHL) and NeuroFibromatosis-1 (NF1) mutations were studied. Additionally, immunohistochemisty for SDHB was also done, and the results compared to mutational analysis of SDH by MLPA (Multiplex Ligation-dependent Probe Activation). STATISTICAL ANALYSIS: Independent samples t-test and Fisher's exact test were used as appropriate. P values ≤0.05 were considered statistically significant. RESULTS: The mean age was 34.3 years. Of the 50 patients, 27 were males and 23 females. 10 patients (20%) in all were detected to have a genetic mutation. 6 patients possessed a RET mutation, while two had VHL mutations. No patient presented with a NF1 mutation. 2 patients had a SDH mutation, and Immunohistochemistry for SDHB correlated with mutational analysis for these patients. CONCLUSIONS: The proportion of patients with a familial variant of PCC/PGL is more than what the historic "Rule of Ten" suggests. Our study found that one in five patients have a genetic mutation. PCC/PGL patients with genetic mutations not only require more stringent follow-up, but also screening of family members.

9.
World J Surg ; 43(11): 2651-2657, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31414166

RESUMO

BACKGROUND: Adequate postoperative analgesia, especially after major abdominal surgery is important for recovery, early mobility, and patient satisfaction. We aimed to study the effects of cryotherapy via an ice pack in the immediate postoperative period, for patients undergoing major abdominal operations. METHODS: This prospective study was conducted at our tertiary care referral center in a low-middle-income country setting. The preoperative patient characteristics, intra-operative variables, and postoperative outcomes were compared between two sets of patients. Cryotherapy was delivered via frozen gel packs for 24 h immediately following laparotomy. Pain relief was assessed with visual analog pain scores (VAS). Comparisons between groups were measured by Chi-square test, Fischer's exact test, or Mann-Whitney U test as appropriate. RESULTS: Sixty-eight patients were included in the study: 33 in the cryotherapy group and 35 in the non-cryotherapy group. Mean postoperative pain scores (VAS) were significantly lower in the cryotherapy group versus the control group (3.97 ± 0.6 vs. 4.9 ± 0.7 on postoperative day (POD) 1; p < 0.001, and 3 ± 0.5 vs. 09 ± 0.8 on POD2; p < 0.001). The median narcotic use in morphine equivalents was lesser in the cryotherapy group from POD 1-3 (66 (IQR-16) vs. 89 (IQR-17); p = 0.001). No significant difference was seen in the NSAID use between the groups. The cryotherapy group was also found to have a lesser incidence of surgical site infection (p = 0.03) and better lung function based on incentive spirometry (p = 0.01) and demonstrated earlier functional recovery based on their ability to perform the sit-to-stand test (p = 0.001). CONCLUSION: Ice packs are a simple, cost-effective adjuvant to standard postoperative pain management which reduce pain and narcotic use and promote early rehabilitation.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Crioterapia , Entorpecentes/uso terapêutico , Dor Pós-Operatória/terapia , Adulto , Feminino , Humanos , Gelo , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados em Cuidados de Saúde , Estudos Prospectivos
10.
World J Surg ; 42(5): 1391-1395, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29383426

RESUMO

BACKGROUND: The current standard-of-care for surgical staging of the axilla in clinically node-negative (N0) early breast cancers is sentinel lymph node biopsy (SLNB), which requires expensive radiopharmaceuticals for efficacious results. In-house produced low-cost radiopharmaceuticals may be the solution and have shown efficacy in earlier observational/pilot studies. We compared SLNB using in-house prepared radiopharmaceutical (99mTc-Antimony-colloid) versus commercially marketed radiopharmaceutical (99mTc-Sulphur-colloid) in this prospective randomized study. STUDY DESIGN: 78 clinically N0 early breast cancer patients (T1/2, N0 stages), undergoing primary surgery were prospectively randomized 1:1 into two groups; to receive SLNB using methylene blue, and either 99mTc-Antimony colloid (Group-1) or  99mTc-Sulphur colloid (Group-2). Completion axillary dissection was done in all (validation SLNB). SLNB indices were compared between the groups. RESULTS: The groups were comparable with regard to age, stage, tumour size, hormone receptors and HER2neu status. Cost of the in-house prepared 99mTc-antimony colloid was 16-times lesser compared to 99mTc-sulphur colloid. SLN identification rates (IR) in Groups 1 and 2 were 100 and 97.4% respectively, (p > 0.05). False negative rates (FNR) in Group 1 and 2 were 6.3% (1/16 patients) and 7.7% (1/13 patients), respectively, (p > 0.05). There were no major allergic reactions in either group. CONCLUSION: In this prospective randomized trial on early breast cancer patients, accuracy of SLNB was comparable using in-house prepared, 99mTc-antimony colloid and commercially marketed 99mTc-sulphur colloid as radiopharmaceutical, while 99mTc-antimony colloid was much cheaper than 99mTc-sulphur colloid.


Assuntos
Neoplasias da Mama/diagnóstico por imagem , Carcinoma Ductal de Mama/diagnóstico por imagem , Compostos Radiofarmacêuticos , Biópsia de Linfonodo Sentinela/métodos , Antimônio , Neoplasias da Mama/patologia , Carcinoma Ductal de Mama/patologia , Feminino , Humanos , Indicadores e Reagentes , Azul de Metileno , Pessoa de Meia-Idade , Estudos Prospectivos , Cintilografia , Enxofre , Compostos de Tecnécio
12.
World J Surg ; 40(7): 1583-9, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26324160

RESUMO

INTRODUCTION: Sentinel lymph node biopsy (SLNB) is the standard of care for staging N0 primary early breast cancers (EBC). Patients in developing countries mostly present with large (LOBC) or locally advanced cancers (LABC) and are treated with neo-adjuvant chemotherapy (NACT). Accuracy of SLNB in staging stage III N0 and post-NACT N0 patients is uncertain. This prospective validation study on LOBC/LABC patients compared the accuracy of SLNB between primary versus post-NACT surgery. MATERIALS AND METHODS: Fifty T3/T4, N0 patients undergoing primary surgery (Group I) and 70 LOBC/LABC (index stage) treated with NACT and N0 at the time of surgery (Group II) were inducted. Validation SLNB was performed using low-cost methylene-blue and (99m)Tc-Antimony colloid. SLN identification (IR) and false-negative (FNR) rates were compared between the groups. Sub-group analysis was done in Group II per index tumor and nodal stage to identify factors predicting SLN IR and FNR in post-NACT patients. SLN IR and FNR in both groups were compared with those in previously published SLN validation study and meta-analysis in EBC. RESULTS: Using combination of blue-dye and radio-colloid, post-NACT SLN IR and FNR (82.9, 13.5 %) were far inferior to T3/T4 primary surgery group (94, 7.7 %; p values 0.034, 0.041) and in EBC. SLN IR using blue-dye alone was dismally low in post-NACT LABCs. Factors predicting unidentified post-NACT SLN and false-negative SLNB included young age, LVI, skin infiltration, extra-nodal spread or N2a stage, and UOQ tumors. CONCLUSIONS: Accuracy of SLNB in T3, N0 tumors undergoing primary surgery is comparable to that of SLNB for N0 EBC. In post-NACT patients, SLNB IR are lower and FNR are higher. Factors predictive of non-identification and false-negative SLNB include pre-NACT skin involvement (T4b), N2a stage or extra-nodal invasion and LVI, and to a lesser extent, young age and UOQ location of the tumor.


Assuntos
Neoplasias da Mama/patologia , Biópsia de Linfonodo Sentinela , Adulto , Idoso , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/terapia , Quimioterapia Adjuvante , Reações Falso-Negativas , Feminino , Humanos , Pessoa de Meia-Idade , Terapia Neoadjuvante , Estadiamento de Neoplasias , Estudos Prospectivos
14.
World J Surg ; 40(3): 690-6, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26438242

RESUMO

INTRODUCTION: Pheochromocytoma (PCC) manifests in up to 50% of MEN2 patients. We correlated the clinico-pathological features of MEN2-associated PCC (MEN-PCC) with RET mutations and compared them with non-MEN adrenal-PCCs. METHODS: In this retrospective single institution study on a large PCC database (n = 208, 1997-2014) 24 MEN-PCC patients with known RET mutations were reviewed. Excluding 7 with incomplete data, the study cohort of 17 MEN-PCC patients from 11 kindreds (M:F::7:10) was identified. Clinical, biochemical, pathological attributes, and outcomes in the MEN-PCC group were correlated with the genotype, and further compared with non-MEN, apparently sporadic adrenal-PCCs (n = 132, excluding 37 extra-adrenal and 15 VHL/NF1/SDH-associated PCC). RESULTS: Components of MEN2 encountered included MTC in 13(76.5%), Marfanoid habitus in 2, and PHPT, cutaneous lichen amyloidosis and mucosal neuromas in 1 patient each. In 11(64.7%), PCC was the first detected MEN2 component (Symptomatic:8, Incidentaloma:3). Four (23.5%) were normotensive; 8(47.1%) had bilateral PCC (7 synchronous, 1 metachronous). Surgery for PCC included laparoscopic adrenalectomy in 12; and cortical-sparing adrenalectomy in 2 of 8 bilateral PCC patients. Mean MEN-PCC tumor size was 6.9 ± 3.9 cm, and 6(35%) had additional adrenal medullary hyperplasia. Four different genotypes were encountered, commonest involving codon 634, others being 804 and 918. Mean age in MEN-PCC (27.7 ± 12.2 years) was lower than non-MEN PCC (39.4 ± 15.7, p = 0.018). Proportion of pediatric patients (35.3% in MEN-PCC vs. 12.9% in non-MEN-PCC, p = 0.007), bilateral tumors (47.1% in MEN-PCC, 4.5% in non-MEN-PCC, p < 0.001), and adrenal medullary hyperplasia (35.2% in MEN-PCC, 0.7% in non-MEN-PCC, p < 0.001) were different. Median 24-hour urinary metanephrines was significantly higher in index MEN-PCC patients, than non-MEN-PCC (634 vs. 214 mcg/24 h, p value = 0.006), but was non-significantly higher in non-index MEN-PCC patients. Mean tumor sizes were comparable in the two groups. None of MEN-PCC patients had malignant PCC, compared to 7(5.3%) in non-MEN-PCC. CONCLUSIONS: In this cohort of MEN-PCC from India, the commonest causative RET mutations for MEN-PCC involved codon 634. MEN-PCC patients were younger, and more frequently had bilateral PCC than non-MEN disease. MEN-PCC patients in India are diagnosed with large tumors and extremely high catecholamine/metanephrine levels.


Assuntos
Neoplasias das Glândulas Suprarrenais/genética , Glândulas Suprarrenais/patologia , DNA de Neoplasias/genética , Neoplasia Endócrina Múltipla Tipo 2a/genética , Mutação , Feocromocitoma/genética , Proteínas Proto-Oncogênicas c-ret/genética , Adolescente , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/metabolismo , Adulto , Códon , Análise Mutacional de DNA , Feminino , Estudos de Associação Genética , Genótipo , Humanos , Incidência , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Mitógenos , Neoplasia Endócrina Múltipla Tipo 2a/patologia , Neoplasia Endócrina Múltipla Tipo 2a/cirurgia , Feocromocitoma/epidemiologia , Feocromocitoma/patologia , Estudos Retrospectivos , Adulto Jovem
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