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.

Long-term outcome after bilateral adrenalectomy in Cushing's disease with focus on Nelson's syndrome

ABSTRACT Objective We analyzed the clinical, biochemical, and imaging findings of adrenalectomized patients with Cushing's disease (CD) in order to compare the characteristics of those who developed Nelson's syndrome (NS) versus those who did not develop this complication (NNS), aiming to identify possible predictive factors for its occurrence. Subjects and methods We performed a retrospective review of the clinical records of a group of patients with CD who underwent TBA between 1974 and 2011. Results Out of 179 patients with CD, 13 (7.3%) underwent TBA. NS occurred in 6 of them (46%) after a mean of 24 months from the total bilateral adrenalectomy (TBA). Age at diagnosis, duration of Cushing's syndrome (CS) until TBA, and steroid replacement doses were similar in both groups. Initial urinary cortisol levels (24-hour urinary free cortisol [UFC]) were significantly higher in the NS group than in the NNS group (p = 0.009). Four patients in the NS group and three of those in the NNS group received radiotherapy before TBA (p = 0.26). Three patients in the NS group presented residual tumors before TBA, compared with none in the NNS group (p = 0.04). At 1 year after TBA, the median ACTH level was 476 ng/L (240-1500 ng/L) in the NS group and 81 ng/L (48-330 ng/L) in the NNS group (p = 0.0007). Conclusion In conclusion, a residual tumor before TBA, higher 24-hour UFC at diagnosis, and increasing ACTH levels within 1 year after TBA emerged as predictive factors of development of NS.

Surgical Outcomes of Bilateral Adrenalectomy / 대한내분비외과학회지

PURPOSE: Because the main drawback of bilateral adrenalectomy is permanent adrenal insufficiency and the subsequent risks of life-long steroid use, adrenal preserving partial adrenalectomy is being accepted as its alternative. The aim of this study is to investigate the indications for bilateral adrenalectomy and to assess the postoperative outcomes and steroid replacement according to operative methods. METHODS: From May 1996 through July 2013, a total of 25 patients who underwent bilateral adrenalectomy in our institution were reviewed retrospectively. Surgical outcomes were compared between total and partial adrenalectomy, and postoperative steroid hormone replacement were examined according to the volume of remnant adrenal gland. RESULTS: The median follow-up duration of 25 patients was 55.8 months. The most common indication for bilateral adrenalectomy was bilateral pheochromocytoma (n=16), which was associated with genetic mutation of RET or VHL gene in 11 cases. Cushing's syndrome (n=8) and hyperaldosteronism (n=1) were another indications of bilateral adrenalectomy. Total adrenalectomy was performed in 8 patients and adrenal preserving partial adrenalectomy in 17 patients. Among the 17 patients, only 5 patients needed adrenal hormone replacement and 2 patients had a recurrence at remnant adrenal tissue. CONCLUSION: Adrenal preserving partial adrenalectomy might be a better option for bilateral adrenal tumor than total adrenalectomy because it can reduce complications associated with adrenal insufficiency and recurrence of the disease is not common.

Adrenalectomía laparoscópica bilateral sincrónica en feocromocitoma bilateral / Bilateral laparoscopic synchronous adrenalectomy in bilateral pheochromocytoma: experience in 8 patients

Aim: To evaluate 8 cases of bilateral pheochromocytoma and laparoscopic synchronous treatment. Patients and Methods: From May 1999 to May 2010, 8 patients with bilateral pheochromocytoma were found. A complete preoperative workup was done. Results: We perform 8 transperitoneal laparoscopic bilateral synchronous adrenalectomy. There was no open converted procedure. Mean operative time was 184.37 min. (range 95 to 300 min). Mean hospital stay was 3.8 days (range 3 to 5 days). Mean blood loss was 191.25 ml (range 0 to 500 min). In 7 cases the histopathological result was of pheochromocytoma and 1 as a malignant pheochomocytoma based on its histology. Conclusions: With very well establish diagnostic disease, an appropriate anesthetic strategy and as surgical skills, laparoscopic bilateral synchronic adrenalectomy is a feasible and safe surgical technique for bilateral pheochromocytomas.

Introducción: La incidencia del feocromocitoma varía entre un 0,005 y un 0,1 por ciento y de estos sólo un 10 por ciento se presenta en forma bilateral. El objetivo de este trabajo es presentar nuestra experiencia en adrenalectomía laparoscópica bilateral sincrónica en pacientes con diagnóstico de feocromocitoma bilateral. Se revisa la literatura y se analizan nuestros resultados. Material y Métodos: Se realiza una revisión retrospectiva de todos los pacientes sometidos a una adrenelectomía laparoscópica bilateral sincrónica entre mayo de 1999 y mayo de 2010, con diagnóstico de un feocromocitoma bilateral. Todos los pacientes fueron estudiados con medición de catecolaminas plasmáticas y urinarias y estudio por imágenes. Resultados: Se realizaron 8 adrenelectomías laparoscópicas bilaterales sincrónicas. El tiempo promedio de cirugía fue de 184 min (rango 95-300 min). La estadía intrahospitalaria media fue de 3,8 días (rango de 3-5 días). El promedio de sangrado fue de 83 ml (rango 0-500 ml). El diagnóstico histopatológico fue de feocromocitoma en todas las muestras, en un caso se diagnosticó un feocromocitoma maligno. No hubo conversión a cirugía abierta. Conclusiones: La adrenelectomía laparoscópica bilateral sincrónica para feocromocitomas bilaterales es un procedimiento seguro y de baja morbilidad, realizada por un cirujano con experiencia en cirugía laparoscópica. Es necesario tener previamente el diagnóstico de certeza para el manejo anestésico y postquirúrgico adecuado para estos pacientes.

Prospective Study of 17 Cases with Ectopic ACTH Syndrome / 上海第二医科大学学报

Objective To discuss the optimal clinical diagnosis and treatment of ectopic ACTH syndrome with occult tumors. Methods Clinical features, imaging examinations and treatment of 17 patients with ectopic ACTH syndrome were described and compared. Results All patients illustrated the typical clinical features of Cushing’s syndrome. They had hypokalemic alkalosis, elevated serum cortisol and plasma ACTH levels. In the high-dose dexamethasone suppression tests, most patients failed to suppress serum cortisol and 24-hour urinary cortisol. CT and MRI are useful imaging modalities to localize the ACTH-secreting tumor in patients with ectopic ACTH syndrome. The patients with overt ACTH-secreting tumors had surgical curative resection soon after diagnosis. Among patients with occult ACTH-secreting tumors, three underwent subtotal bilateral adrenalectomy, two underwent right adrenalectomy, four received inhibitor of steroidogenesis aminoglutethimide. Their hypercortisolism was controlled. Conclusion Surgical curative resection is the optimal treatment of ectopic ACTH syndrome with overt ACTH-secreting tumor. Bilateral adrenalectomy, right adrenal ectomy or chemotherapy to control hypercortisolism is an available treatment of ectopic ACTH syndrome with occult ACTH-secreting tumors.

A Case of Bilateral Macronodular Adrenocortical Hyperplasia Accompanied by Hyperresponsiveness to Vasopressin / 대한내분비학회지

Cushing's syndrome associated with nodular adrenal hyperplasia glands is divided into 4 main categories: adrenal adenoma, adrenal carcinoma, primary pigmented nodular adrenal dysplasia and macronodular adrenal hyperplasia(MAH). The mechanism of bilateral MAH, when ACTH is suppressed, was previously unknown, and referred to as being "autonomous". Recently, several reports have shown MAH to be under the control of ectopic or eutopic membrane hormone. Here, a case of Cushing's syndrome, caused by bilateral MAH, is reported. A 62-year-old woman presented with Cushingoid features, hypertension and diabetes mellitus. In her case, abnormal adrenal stimulation of cortisol secretion in response to exogenous vasopression stimulation was shown. Her urine free cortisol was 726.0microgram/dL, which was not suppressed after administration of high-dose dexamethasone. Her plasma cortisol level was elevated, but without circadian rhythm. ACTH was undetectable. An abdomen CT scan demonstrated bilaterally enlarged multinodular adrenal glands. A Sella MRI revealed no alteration of the pituitary gland. The patient underwent a laparoscopic bilateral adrenalectomy. Histological examination revealed bilateral macronodular hyperplasia. After having recovered, the patient showed progressive regression of the Cushingoid status.

A Case of Pulmonary Cryptococcosis Followed by Pulmonary Tuberculosis Developed after Bilateral Adrenalectomy / 결핵

Cryptococcosis is a systemic infection caused by the yeast-like fungus Cryptococcus neoformans. Respiratory tract is the usual portal of infection but relatively few cases of pulmonary cryptococcosis have been reported. Recently the incidence of the disease are increasing because the perception to seek the disease is increasing and immunocompromised hosts are more widespread with introduction of immune-suppressants, steroids and advent of AIDS. Because of nonspecific pattern of clinical manifestation, radiologic findings, differentiating the other diseases is essential and it needs fungal culture and biopsy. We describe a patient in the state of bilateral adrenalectomy due to bilateral ACTH-independent macronodular hyperplasia who developed pulmonary cryptococcosis and was later infected with pulmonary tuberculosis.

Anesthetic Experience with a Case of Bilateral Adrenalectomy for Pheochromocytoma / 대한마취과학회지

An anesthetic experience with bilateral adrenalectomy for pheochromocytoma in an 26-year-old male has been reported. The patient was treated with phenoxybenzamine and propranol for about five weeks His blood pressure was 160/100 mmHg, pulse rate was 92 beats/min. Following induetion of an anesthesia with intravenous diazepam, thiopental sodium, morphine, and sodium nitroprusside infusion, vecuronium was administered intravenously, and endotracheal intubation was performed. Anesthesia was maintained with nitrous oxide, oxide (50%~50%) and isoflurane (0.5~3%) using a semiclosed circle system. For the control of blood pressure during surgical manipulation, we used sodium nitroprusside and phentolamine infusion. After tumor resection, blood pressure was controlled by intravenous Hartmans solution and whole blood. The patient tolerated anesthesia and surgery well despite the episodic hemodynamic changes.