Carcinoma adrenocortical en mujeres en edad fértil / Adrenocortical carcinoma in women of reproductive age

Resumen Los carcinomas adrenocorticales son tumores infre cuentes, habitualmente hiperfuncionantes y con una supervivencia global pobre. La edad frecuente de pre sentación se describe en adultos entre 40 a 60 años, con predominio en sexo femenino. Se presentan dos casos inusuales de carcinoma adrenal diagnosticados en mujeres en edad fértil. El primero de ellos se descubrió en el segundo trimestre de gestación, con un cuadro de hipercortisolismo y lesión adrenal localizada, que resolvió con resección completa hacia la semana 20. En el segundo, la paciente debutó con manifestaciones clínicas de virilización rápidamente progresiva, sien do el hiperandrogenismo puro el patrón bioquímico hallado. En ambos casos, a pesar de haberse realizado la resección completa, el Ki67 elevado como principal factor pronóstico condujo a categorizarlas como de "alto riesgo de recurrencia". Asimismo, se ha asociado a la gestación y al patrón secretor de glucocorticoides como factores adicionales de mayor riesgo de recurrencia. Este es particularmente elevado dentro de los dos primeros años posteriores al diagnóstico. Existe aún controversia sobre el uso de mitotane adyuvante en estos pacientes, y su inicio está recomendado hasta los tres meses del postquirúrgico. Sin embargo, la evidencia disponible no permite suponer la falta de eficacia si se utiliza fuera de ese período. Los limitantes, como fueron el curso de la gestación y el puerperio inmediato, así como la difi cultad para el acceso a la medicación en nuestro medio, impidieron el inicio precoz del tratamiento adyuvante en ambos casos, aunque surge la inquietud de si aún sería oportuna su instauración.

Abstract Adrenocortical carcinomas are rare tumors, usually hyperfunctioning, with poor overall survival. Frequent age of presentation is described in adults between 40 and 60 years of age, predominantly female. Two unusual cases of adrenal carcinoma diagnosed in young women are presented. The first one was discovered in the sec ond trimester of gestation, with signs and symptoms of hypercortisolism and localized adrenal lesion, which was resolved with complete resection by week 20 of pregnancy. In the second case, the patient begined with clinical manifestations of rapidly progressive virilization, the biochemical pattern being pure hyperandrogenism. In both cases, despite complete resection, the high Ki67 as the main prognostic factor leaded to categorization as "high risk of recurrence". In addition, pregnancy and glucocorticoid secretory pattern have been associated as additional risk factors of recurrence. This is particularly high within the first two years after diagnosis. There is controversy about the use of adjuvant mitotane in these patients, and the general recommendation is to be started no longer than 3 months after surgery. However, the available evidence does not suggest that its use is ineffective beyond that period. Limitations, such as the course of pregnancy and the immediate puerperium, as well as the difficulty of accessing this medication in our environment, prevented the early initiation of adjuvant treatment with mitotane in both cases, although there is still concern whether its administration would still be appropriate.

Atrial myxoma and associated Cushing syndrome: Carney complex / Mixoma auricular y síndrome de Cushing asociado: complejo de Carney

Abstract A 33-year-old woman with a history of high blood pressure since she was 8 years old, hypothyroidism, polycystic ovary syndrome, metabolic syndrome, multiple nevi, and a maternal family history of death at age 50 due to malignant high blood pressure and heart failure. Cushing's syndrome secondary to a secretory pituitary microadenoma was diagnosed, being the cause of secondary arterial hypertension, and ruling out other causes such as renal stenosis and coarctation of the aorta. A transthoracic and transesophageal echocardiogram was performed, which detected a left atrial myxoma. Given the presence of an atrial myxoma, Cushing's syndro me and polycystic ovary syndrome, a diagnosis of Carney Complex was made due to the presence of positive Stratakis criteria. The cardiac tumor was resected, and pathology confirmed that it was an atrial myxoma. She evolved clinically stable in outpatient controls in a 6-month follow-up. Resection of the pituitary microadenoma is planned as a curative treatment for Cushing's syndrome and arterial hypertension.

Resumen Mujer de 33 años, con antecedentes de hipertensión arterial desde los 8 años, hipotiroidismo, síndrome de ovario poliquístico, síndrome metabólico, nevos múltiples y antecedente familiar materno de muerte a los 50 años por hipertensión arterial maligna e insuficiencia cardiaca. Se diagnosticó síndrome de Cushing secundario a un mi croadenoma hipofisario secretor, siendo la causa de la hipertensión arterial secundaria, y descartándose otras causas como estenosis renal y coartación de aorta. Se realizó u n ecocardiograma transtorácico y transesofágico que detectaron un mixoma auricular izquierdo. Ante la presencia de un mixoma auricular, síndrome de Cushing y síndrome de ovario poliquístico se llegó al diagnóstico de Complejo de Carney por la presencia de criterios de Stratakis positivos. Se realizó la resección del tumor cardiaco, y la anatomía patológica confirmó que se trataba de un mixoma auricular. Evolucionó clínicamente estable en controles ambulatorios en un seguimiento de 6 meses, y se planifica la resección del microadenoma hipofisario como tratamiento curativo del síndrome de Cushing y la hipertensión arterial.

Single-center experience with vascularized adrenal displacement for the treatment of primary bilateral macronodular adrenal hyperplasia / 现代泌尿外科杂志

【Objective】 To explore a new treatment of primary bilateral macronodular adrenal hyperplasia (PBMAH) and its efficacy. 【Methods】 Clinical data of 20 PBMAH patients treated in our hospital during Mar.2010 and Apr.2021 were retrospectively analyzed. All patients underwent laparoscopic subcutaneous displacement of vascularized adrenal. The clinical symptoms, plasma free cortisol, adrenocorticotrophic hormone (ACTH), and 24 h urinary free cortisol were regularly monitored after surgery. 【Results】 Of all 20 patients, 19 were followed up for 18 to 120 months (median 60 months). Three months after surgery, reexamination showed 1 patient had decreased plasma free cortisol and increased ACTH, but had no symptoms of low corticosteroids. After another 3 months, the plasma free cortisol and ACTH returned to normal. After 4 to 48 months, the parameters recovered in all patients and the clinical symptoms disappeared. 【Conclusion】 Laparoscopic vascularized adrenal displacement is a new and effective method for the treatment of PBMAH. It can alleviate the Cushing syndrome with no obvious adverse reactions.

Obstetric Clinical Analysis in 8 Cases With Cushing’s Syndrome / 中山大学学报(医学科学版)

ObjectiveTo summarize and analyze the clinical characteristics， diagnosis process， treatment process， and obstetric outcomes of pregnant women with Cushing's syndrome， helping to optimize pregnancy management. MethodsA retrospective study was conducted on 8 pregnant women with Cushing’s syndrome who were hospitalized in the First Affiliated Hospital， Sun Yat-sen University between January 2006 and August 2022. The clinical characteristics， management and obstetric outcomes were recorded. ResultsPreeclampsia was detected in 4 cases，pre-gestational diabetes mellitus in 2 cases， gestational diabetes mellitus in 5 cases， and hypokalemia in all 8 cases. Elevated serum cortisol， disappearance of day-night rhythm of cortisol， increased 24-hour urine cortisol and decrease in serum ACTH were found in 8 cases by laboratory examination. Furthermore， adrenal adenoma was detected in all 8 cases by ultrasonography or Magnetic Resonance Imaging. Three cases underwent laparoscopic adrenalectomy in the second trimester and 4 cases received surgery after delivery. The diagnosis of adrenal cortical adenoma was confirmed by pathological report. Six cases had preterm birth， while one patient delivered after 37 weeks of gestation and one patient suffered from spontaneous abortion. Among 7 cases of live birth， 6 patients underwent cesarean section and 1 patient had vaginal delivery. Of all newborns， 3 had low birth weight. One case had a birth defect. Four infants were transferred to the neonatal intensive care unit， and two infants died. One child was diagnosed with nephrotic syndrome at 2 years of age. ConclusionsCushing's syndrome is rare and high risk during pregnancy. It requires multidisciplinary diagnosis， treatment， and long-term follow-up. Drug therapy carries a risk of progression and requires intensive care during pregnancy， postpartum follow-up， and specialist treatment.

El reto diagnóstico de la enfermedad de Cushing en pediatría: reporte de casos

Introducción: la enfermedad de Cushing (EC) se debe sospechar en el paciente pediátrico con retraso del crecimiento, aumento de peso y fenotipo característico. Se sugiere iniciar el protocolo diagnóstico, que consiste en: comprobación de hipercortisolemia, sitio de producción del cortisol e imágenes, para finalmente realizar el tratamiento quirúrgico curativo. Reporte de casos: se presentan los casos de tres pacientes con alteración de la velocidad de crecimiento, aumento de peso y fenotipo característico. Se realiza la secuencia diagnóstica para demostrar el hipercortisolismo, posteriormente se confirma EC y se realiza imagen por resonancia magnética nuclear (RMN) de hipófisis. Finalmente se lleva a cabo el tratamiento quirúrgico curativo. Conclusiones: la EC en pediatría sigue siendo un desafío diagnóstico. Un abordaje para su detección inicia con la sospecha clínica asociada a una adecuada interpretación de los estudios bioquímicos e imágenes, lo que permite realizar un diagnóstico oportuno y un tratamiento curativo definitivo.

Summary Introduction: Cushing's disease (CD) should always be suspected in a pediatric patient with growth delay, excessive weight gain and characteristic phenotype. It is suggested to start a diagnostic protocol sequence: verification of hypercortisolism, production site and images, to finally perform the definitive curative surgical treatment. Case reports: We report three CD patients with altered growth velocity, weight gain and characteristic phenotype. The diagnostic sequence was performed to demonstrate hypercortisolism. CD was subsequently confirmed, and a pituitary nuclear magnetic resonance (NMR) was performed. All patients underwent curative surgery. Conclusion: CD in pediatrics continues to be a diagnostic challenge for the clinician. An approach with high initial clinical suspicion, associated with paraclinical studies and obtaining the adequate images, are necessary to make an accurate diagnosis and a definitive curative treatment.

Preparation of ICD and overdenture in patients with Cushing's Syndrome: five-year follow-up / Preparo de PTI e overdenture em pacientes com Síndrome de Cushing: cinco anos de acompanhamento

The aim of this study is to report a clinical case of a patient with Cushing's syndrome, low self-esteem and need for oral rehabilitation. A 50-year-old female patient with hypercortisolism sought care at School of Dentistry of the Federal University of Minas Gerais. During anamnesis, the patient reported painful symptoms and mobility of the upper and lower teeth. On clinical examination, absence of many teeth were observed, periodontal disease and caries lesions. After discussion of the case, the planning involved the extraction of all teeth, and the preparation of Maxillary Immediate Complete Dentures (ICD's) and Immediate Mandibular Implant-retained Overdenture (IMIO). The treatment proposed allowed the restored aesthetics, phonetics and chewing(AU)

O objetivo desse estudo é relatar um caso clínico de uma paciente com síndrome de Cushing, baixa autoestima e necessidade de reabilitação oral. Paciente do sexo feminino, 50 anos de idade e com hipercortisolismo, procurou atendimento na Faculdade de Odontologia da Universidade Federal de Minas Gerais. Durante a anamnese, a paciente relatou sintomas dolorosos e mobilidade dos dentes superiores e inferiores. No exame clínico, foi observada a ausência de muitos dentes, doença periodontal e lesões cariosas. Após a discussão do caso, o planejamento envolveu a extração de todos os dentes, e o preparo de Prótese Total Imediata maxilar (PTI), e Overdenture Implantorretida Imediata mandibular. O tratamento proposto permitiu que restaurasse a estética, fonética e mastigação(AU)

Enfermedad de Cushing recurrente con hipercortisolismo catastrófico: reporte de caso y revisión de literatura / Catastrophic hypercortisolism and recurrence in Cushing´s disease: a case report and literature review / Doença de Cushing recorrente com hipercortisolismo catastrófico: relato de caso e revisão da literatura

Resumen: Presentamos un caso de una paciente femenina de 27 años, con síndrome de Cushing ACTH dependiente con hipercortisolismo severo, causado por un macroadenoma hipofisario recurrente y resistente pese a dos cirugías transesfenoidales, radioterapia y terapia médica. Dada la falla en las diferentes terapias se realiza una adrenalectomía bilateral como tratamiento definitivo. La paciente fallece en el posoperatorio por causa no clara. Si bien la adrenalectomía bilateral ha sido reportada como un tratamiento efectivo en pacientes con enfermedad de Cushing, se ha relacionado con una mortalidad significativa vinculada con la severidad del hipercortisolismo y las comorbilidades presentes. En este caso la adrenalectomía izquierda se tuvo que convertir a cielo abierto, asociada con mayor morbimortalidad.

Abstract: The study presents the case of a 27-year-old female patient with adrenocorticotropic hormone (ACTH) dependent Cushing's disease and severe hypercortisolism caused by recurrent pituitary macroadenoma that was resistant to treatment despite two transsphenoidal surgeries, radiotherapy and medical treatment. Upon failure of the different therapies a bilateral adrenalectomy was performed as the final treatment. The patient died in after surgery although the case of death was not clear. Despite bilateral adrenalectomy having been reports as an effective treatment in patients with Cushing's disease, it has been related to significant mortality rates in connection with the severity of hypercortisolism and existing comorbilities. In this case the left adrenalectomy ended up being an open surgery, which is associated to a higher mortality rate.

Resumo: Apresentamos o caso de uma paciente de 27 anos com síndrome de Cushing ACTH-dependente com hipercortisolismo grave causado por macroadenoma hipofisário, recorrente e resistente, apesar de haver sido submetida a duas cirurgias transesfenoidal, radioterapia e terapia medicamentosa. Diante do fracasso das diferentes terapias, foi realizada adrenalectomia bilateral como tratamento definitivo. A paciente faleceu no pós-operatório por causa não esclarecida. Embora a adrenalectomia bilateral tenha sido relatada como tratamento eficaz em pacientes com doença de Cushing, ela tem sido associada a mortalidade significativa relacionada à gravidade do hipercortisolismo e às comorbidades presentes. Neste caso, a adrenalectomia esquerda teve que ser convertida para cirurgia aberta, associada a maior morbimortalidade.

A case of subcutaneous sarcoidosis in a patient with Cushing's syndrome

Abstract A 41-year-old female visited our department complaining of asymptomatic subcutaneous nodules on the right forearm. She had been diagnosed as having Cushing syndrome due to an adrenal tumor 5-months previously. After she underwent surgery for the adrenal tumor, the subcutaneous nodules gradually increased in number. Physical examination showed ill-defined plate-like subcutaneous indurations on the bilateral lower extremities, buttocks, and right forearm. A biopsy of one of the subcutaneous indurations showed non-caseating epithelioid cell granulomas involving the hypodermis and subcutaneous tissues. The patient was diagnosed as having sarcoidosis based on the Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 criteria. Skin lesions decreased in size and had completely disappeared. Although the mechanism is unknown, there may be a possibility that the activity of sarcoidosis is suppressed by high cortisol concentrations due to Cushing syndrome.

Complejo de Carney: reporte de un caso y revisión de la literatura / Carney complex: a case report and literature review

Resumen El complejo de Carney es una enfermedad caracterizada por lesiones en la piel, tumores endocrinos, cardiacos, gonadales y en otros órganos, que está asociada con mutaciones del gen PRKAR1A. Presentamos el caso clínico de una paciente con varias de las manifestaciones más características de este síndrome. Finalmente, se hace una revisión de la literatura.

Abstract Carney complex is a disease characterized by skin lesions, endocrine, cardiac, gonadal and other organ tumors, associated with mutations of the PRKAR1A gene. We present the clinical case of a patient with several of the most characteristic manifestations of this syndrome. Finally, there will be a review of the literature.

Hipercortisolismo severo e hipocalemia recurrente / Severe hypercortisolism and recurring hypocalemia

RESUMEN El síndrome de Cushing endógeno deriva de un aumento crónico, inapropiado y sostenido de glucocorticoides principalmente como respuesta al exceso en las concentraciones séricas elevadas de la hormona adrenocorticotropa (ACTH) desde un tumor adenohipofisiario, enfermedad de Cushing; o producida de forma ectópica por tumores neuroendocrinos. El Cushing suprarrenal se origina por tumores de la corteza adrenal que producen de forma autónoma cortisol y es independiente de ACTH. El curso clínico, tratamiento, pronóstico y posibles complicaciones dependen de identificar de forma correcta la lesión desencadenante; situación que en múltiples ocasiones resulta en una experiencia retadora para los clínicos. Se presenta el caso de una mujer de 62 años, ingresada por síntomas constitucionales con hipocaliemia severa de difícil corrección e hipercortisolismo severo.

ABSTRACT Endogenous Cushing syndrome derives from a chronic, inappropriate, and sustained increase in glucocorticoids, mainly in response to remarkably high serum concentrations of adrenocorticotropic hormone (ACTH) secreted from an adenohypophyseal tumor, Cushing's disease, or due to ectopic production by neuroendocrine tumors. Adrenal Cushing's disease is caused by tumors of the adrenal cortex that autonomously produce cortisol and this is independent from ACTH action. Clinical course, treatment, prognosis, and possible complications depend on correctly identifying the triggering lesion; this situation frequently becomes a challenging experience for clinicians. We present the case of a 62-year-old woman, admitted for constitutional symptoms with severe hypokalemia that was difficult to correct and severe hypercortisolism.

Hipertensión secundaria: ¿cuándo y cómo buscarla? / Secondary hypertension: when and how to look for it?

Resumen En la población hipertensa se detecta una causa subyacente en 5% a 10%. Identificar etiología y establecer un tratamiento específico reduce el riesgo de daño de órgano blanco y, en algunos casos, puede curar la enfermedad primaria y la hipertensión. Dada su baja prevalencia, no se recomienda realizar una búsqueda exhaustiva de causas secundarias en todos los pacientes hipertensos. En este artículo se analiza cuándo y cómo debe estudiarse una hipertensión secundaria.

Abstract In the hypertensive population, between 5-10%, an underlying cause is detected. Identifying the etiology and giving specific treatment reduces the risk of hypertension-mediated organ damage and, in some cases, can cure the disease and high blood pressure. Due to its low prevalence, an exhaustive search for secondary causes is not recommended in all patients with hypertension. This article will discuss when and how to study secondary hypertension.

Síndrome de Cushing por carcinoma adrenocortical en lactante de 5 meses

RESUMEN Introducción: Las causas adrenales del síndrome de Cushing como el carcinoma son las etiologías comunes en el niño, si bien su incidencia es baja. Objetivo: Describir los procedimientos diagnósticos y terapéuticos seguidos en el caso de una lactante de cinco meses con síndrome de Cushing como consecuencia de un carcinoma adrenocortical. Presentación del caso: Lactante femenina de cinco meses consultada por aumento excesivo de peso a partir del segundo mes de nacida a pesar de no alimentarse adecuadamente, además de presentar un retraso en el crecimiento. Se constató obesidad centrípeta y facie pletórica, ligero eritema facial y livedo reticularis, tensión arterial de 130/80 mmHg (>95 percentil). Presentaba agenesia de labios menores, sin vello axilar o pubiano. En el estudio de la función suprarrenal se encontró cifras elevadas de cortisol plasmático. En la tomografía computarizada abdominal se observó imagen hiperdensa compatible con tumor suprarrenal. Se diagnosticó síndrome de Cushing de etiología suprarrenal. Se le realizó adrenalectomía unilateral y se tomó muestra para biopsia posoperatoria y se emitió el diagnóstico de carcinoma adrenocortical. La paciente presenta una evolución desfavorable y finalmente fallece a consecuencia de un fallo multiorgánico al mes de realizada la cirugía. Conclusiones: Ante la sospecha clínica de síndrome de Cushing en la lactante se realizaron las determinaciones hormonales pertinentes y precisó la etiología mediante pruebas de imagen, confirmada por biopsia posquirúrgica. La cirugía constituye la terapia más adecuada ante un paciente con síndrome de Cushing de causa tumoral adrenal, aunque estos pacientes por lo general cursan con mal pronóstico.

ABSTRACT Introduction: The adrenal causes of Cushing's syndrome such as carcinoma are the common etiologies in the child, although their incidence is low. Objective: Describe the diagnostic and therapeutic procedures followed in the case of a five-month-old infant with Cushing's syndrome as a result of adrenocortical carcinoma. Case presentation: Five-month-old female infant consulted due to excessive weight gain from the second month of birth despite not eating properly, in addition to presenting a stunted growth. Centripetal and facial plethora obesity, slight facial erythema and livedo reticularis, blood pressure of 130/80 mmHg (>95 percentile) were found. She had agenesis of labia minora, without axillary or pubic hair. In the study of adrenal function, elevated plasma cortisol levels were found. Abdominal CT scan showed hyperdense image compatible with adrenal tumor. Cushing's syndrome of adrenal etiology was diagnosed. Unilateral adrenalectomy was performed and a sample was taken for postoperative biopsy and the diagnosis of adrenocortical carcinoma was made. The patient presented an unfavorable evolution and she finally died as a result of a multi-organ failure a month after the surgery. Conclusions: Given the clinical suspicion of Cushing's syndrome in the infant, the pertinent hormonal determinations were made and the etiology was specified by imaging tests, confirmed by post-surgical biopsy. Surgery is the most appropriate therapy for a patient with Cushing's syndrome with an adrenal tumor cause, although these patients usually have a bad prognosis.

Unusual Presentation of a Thyroid Malignancy: A Case Report

Medullary thyroid carcinoma is a rare malignancy. We report a case of Medullary thyroid carcinoma in a 56-year-old male patient. Patient presented with lower limb paresis and severe hypokalemia. Cushing’s syndrome is a rare complication of Medullary thyroid carcinoma [MTC] and is due to ectopic Adrenocorticotropic [ACTH] secretion by tumor cells. Cushing’s syndrome presents a challenging diagnostic and management issue in patients with MTC. Entire clinical history, laboratory investigations, microscopic pictures are discussed in detail.

Analysis of clinical characteristics of 985 patients with Cushing's syndrome treated with adrenal surgery in a single center / 中华泌尿外科杂志

Objective:To investigate the etiological spectrum, clinical features, and surgical treatment of patients with Cushing's syndrome (CS) who underwent adrenal surgery.Methods:From August 2002 to August 2022, the clinical data of 985 patients with Cushing's syndrome who underwent surgical treatment in the department of urology, Peking Union Medical College Hospital were retrospectively analyzed. There were 210 males and 775 females. The average age was 43.33±13.49 years old. The age of males was older than that of females (45.53±14.39 vs. 42.68±13.16 years, P=0.016). The principle of preoperative surgical method selection for patients in this group was described as follow. For adrenocorticotropin (ACTH) independent CS, adrenal tumor resection was considered for unilateral solitary lesions and unilateral adrenalectomy was considered for unilateral multiple lesions. For bilateral lesions, the larger tumor was removed first, and the contralateral operation was decided according to the follow-up results. Patients with suspicion of cortical cancer are subjected to R0 resection, and open surgery was performed if the tumor diameter is≥6 cm. The clinical characteristics of CS patients were summarized. The clinical symptom characters, etiology spectrum and the corresponding selection principles of surgical methods were analyzed. Results:Among the 985 cases, ACTH-independent CS accounted for 92.8% (914/985), and ACTH-dependent CS accounted for 7.2% (71/985). According to the postoperative pathological results, ACTH-independent CS was the most common, among which adrenal adenoma was the most common 75.94% (748/985), PBMAH 11.37% (112/985), Cushing's disease 4.26% (42/985), PPNAD 3.25% (32/985), EAS 2.94 (29/985), cortical carcinoma 2.23% (22/985). In terms of clinical manifestations, full moon face, bloody face, buffalo back, central obesity and weight gain were more common. The incidence of the above single symptoms was >30%. In terms of hormone secretion, the cortisol level of ACTH-dependent CS patients was significantly higher than that of ACTH-independent CS ( P<0.001). Cortisol [(16.61±6.78) μg/dl] and 24h-UFC [103.65 (59.83, 175.70) μg/24h] in patients with subclinical cortical adenoma were lower than those in other types of patients ( P<0.001). Among the patients with bilateral adenomas, 4 patients underwent simultaneous resection of bilateral adrenal tumors due to severe CS symptoms, and the remaining 74 patients underwent two-stage simple tumor resection, total adrenalectomy or subtotal adrenalectomy according to the size and number of tumors. Of the 22 patients with cortical carcinoma, 2 patients underwent R0 resection after neoadjuvant therapy with mitotane after being diagnosed by needle biopsy due to local infiltration of the tumor. In patients with EAS and Cushing's disease, 62.07% (18/29) and 23.81% (10/42) were treated with simultaneous bilateral target gland resection due to severe CS symptoms, respectively. In 112 cases of PBMAH and 32 cases of PPNAD, the initial treatment was unilateral adrenalectomy or subtotal adrenalectomy, and the follow-up was continued to decide whether to perform contralateral adrenalectomy or subtotal adrenalectomy. Conclusions:The etiological spectrum of CS patients admitted to the urology department is different from that of the overall CS, and is dominated by adrenal disease, and Cushing's disease is rare. Due to the different etiological spectrum, the patient's symptom spectrum is different, and difficulty in squatting and fracture are rare. The main treatment for unilateral adrenal disease is tumor resection or unilateral adrenalectomy. For ACTH-dependent CS in bilateral lesions, bilateral adrenalectomy is recommended, and hormone supplementation is recommended after surgery. For ACTH-independent CS, unilateral adrenalectomy is recommended first, and individualized treatment plans are formulated based on the results of follow-up.

Ectopic adrenocorticotropic hormone syndrome originating from retroperitoneal paraganglioma: one case report and literature review / 中国医师杂志

Objective:To present the clinical characteristics and treatment on patients with ectopic adrenocorticotropic hormone(ACTH) syndrome (EAS) caused by the retroperitoneal paraganglioma.Methods:The clinical data of a case of EAS caused by retroperitoneal paraganglioma were analyzed retrospectively, and the related literature at home and abroad was reviewed.Results:The 53-year-old female patient was admitted to endocrinology department due to a fifteen-year history of hypertension, accompanied by fatigue for three months, headache and dizziness for one month. The laboratory data demonstrated severe hypokalemia, high level of serum and urinary cortisol, while the ACTH level remained unsuppressed. The 24 h urinary vanillyl mandelic acid (VMA) and serum free methoxyepinephrine (MNs) level were elevated. The abdominal computed tomographic scan suggested a retroperitoneal mass next to the abdominal aorta. After the retroperitoneal tumor resection was performed, immunohistochemical staining of the tumor revealed Syn (+ ), CgA (+ ), ACTH (focal + ). By the retrospective analysis of 22 similar cases from 16 papers and the case summarized above, we found that most patients with EAS caused by the paraganglioma could demonstrate the typical clinical features of Cushing′s syndrome, while lack of the manifestation of paraganglioma. Therefore, preoperative preparations for paraganglioma were usually neglected.Conclusions:Ectopic ACTH syndrome (EAS) originating from paraganglioma is very rare. To improve the diagnosis rate, examination for catecholamine, MNs and 24 h urinary VMA before surgery in patients with EAS is suggested. Considering surgical resection as the optimal treatment, comprehensive preoperative preparations for both paraganglioma and Cushing′s syndrome are significant. A genetic test for pheochromocytoma/ paraganglioma and lifelong postoperative follow-up are also recommend.

Analysis of etiology distribution and clinical manifestation in bilateral adrenal lesions / 中华内分泌外科杂志

Objective:To investigate the etiologies and clinical characteristics of bilateral adrenal lesions.Methods:The clinical data of 143 patients with bilateral adrenal lesions hospitalized in the First Affiliated Hospital of Chongqing Medical University from Jan. 2013 to Mar. 2018 were collected and analyzed.Results:140 patients were retained for final analysis. 79 were men, and 61 were women. The age was (51.53±13.93) years. Regarding the etiologies, there were primary aldosteronism ( n=44, 31.43%) , Cushing’s syndrome ( n=27, 19.29%) , non-functional lesions ( n=23, 16.43%) , adrenal tuberculosis ( n=17, 12.14%) , pheochromocytoma ( n=11, 7.86%) , congenital adrenal hyperplasia ( n=5, 3.57%) , adrenal metastases ( n=5, 3.57%) , and adrenal lymphoma ( n=4, 2.86) . These patients were classified into the following groups according to the mass size: ≤2 cm, 2-4 cm and ≥4 cm. The highest proportion of primary aldosteronism (62.79%) , Cushing’s syndrome (46.15%) and pheochromocytoma (31.25%) was observed in the ≤2 cm, 2-4 cm and ≥4 cm groups, respectively. The mass sizes of primary aldosteronism, Cushing’s syndrome and pheochromocytoma were compared, with pheochromocytoma the largest, followed by Cushing’s syndrome, non-functional lesion, and primary aldosteronism. Conclusions:For patients with bilateral adrenal lesions in our hospital, primary aldosteronism and Cushing’s syndrome are more common than non-functional lesion. Mass size is of great value in the diagnosis of endocrinological etiology, as well as distinguishing malignant tumors from the benign ones. The imaging phenotype is helpful to determine tumor types.

Repeated fracture-full moon face-perioral spotted pigmentation / 中华内分泌代谢杂志

Carney complex(CNC) is a rare disease in endocrinology, with osteochondromyxoma accounting for only 1% of it. This article details the diagnosis and treatment of a young female patient with recurrent fracture, full moon face and perioral spotted pigmentation. Combined with clinical manifestations, auxiliary examination and pathology, Carney complex with osteochondromyxoma was diagnosed. Multiple bone deformities in this case should be differentiated from McCune-Albright syndrome and ectopic ACTH secreting tumor with multiple bone metastases. The report of this case is helpful to improve the understanding of osteochondromyxoma in endocrinology, radiology, orthopedics and other disciplines, so as to avoid missed diagnosis and misdiagnosis.

Síndrome de Cushing secundario a administración tópica decorticoesteroides / Cushing Syndrome due to topical corticosteroid misuse

Los corticoesteroides tópicos son drogas muy comunes, frecuentemente utilizadas en patologías dermatológicas. Su mal uso puede causar efectos sistémicos, como el síndrome de Cushing y la supresión del eje hipotalámico ­ hipofisiario ­ adrenal. Presentamos un caso de un lactante menor de siete meses quien desarrolla un síndrome de Cushing secundario al uso de Clobetasol por una dermatitis en el área del pañal, por tiempo prolongado, sin prescripción médica. Al examen físico se evidencia obesidad a predominio central, con fascie de luna llena, hipertricosis en región frontal, telangiectasias aisladas en mejillas y cuello de búfalo. Los paraclínicos demuestran una hipercolesterolemia, hipertrigliceridemia, elevación de las transaminasas y cortisol sérico en la mañana disminuido. Se concluye que se debe informar a los padres de los efectos adversos sistémicos de los esteroides tópicos y se sugiere evitar en pacientes pediátrico(AU)

Topical corticosteroids are very common drugs used in the treatment of inflammatory skin diseases. Prolonged ormisuse of them may cause systemic adverse effects, including Cushing syndrome and hypothalamic-pituitary-adrenal axissuppression. We present a case of a seven months old maleinfant who developed iatrogenic Cushing syndrome after diaperdermatitis treatment through misuse of Clobetasol, withoutdoctor's prescription. We observe redness and a moon face, abuffalo hump, central obesity and hirsutism. Laboratory values revealed hypercholesterolemia, hypertriglyceridemia, elevationin liver enzymes and low early morning cortisol. To conclude,parents must be informed by physicians about the adverse effect of steroids and the should be avoided in very young infant(AU)

Efectos adversos de la terapia con glucocorticoides / Adverse effects of glucocorticoid therapy

RESUMEN Los glucocorticoides son medicamentos ampliamente usados para el manejo de muchas enfermedades crónicas; a pesar de esto, están asociados con múltiples efectos adversos que pueden afectar diferentes órganos. En la presente revisión se hará una descripción de los efectos derivados del uso de estos sobre el eje hipotálamo-hipófisis-adrenales y los diferentes sistemas, haciendo énfasis en el sistema cardiovascular y óseo.

SUMMARY Glucocorticoids are widely used medications for the management of many chronic diseases, but they are associated with multiple adverse effects that can affect different organs. In the present review, a description will be made of the effects derived from the use of these medications on the hypothalamic-pituitary-adrenal axis and other systems, with emphasis on the cardiovascular system and bone.