Adrenocorticotropic Hormone-Secreting Esthesioneuroblastoma with Ectopic Cushing's Syndrome

Esthesioneuroblastoma as a source of ectopic Cushing's syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. ⁶⁸Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Combined transcranial and endoscopic endonasal approach surgery was performed, and a diagnosis of esthesioneuroblastoma was given.

Secreción ectópica de ACTH: Presentación infrecuente de un estesioneuroblastoma / An Infrequent Presentation of Esthesioneuroblastoma with Ectopic ACTH Syndrome

Introducción: El estesioneuroblastoma es un tumor derivado de los neuroblastos del epitelio respiratorio neuroectodérmico. Representa entre el 3 al 6 % de los tumores intranasales. La secreción ectópica de Hormona Adrenocorticotrofina (ACTH) es la causa del 10 al 20 % de Síndrome de Cushing dependiente de ACTH, siendo el estesioneuroblastoma una rara causa de secreción ectópica de ACTH. Presentación del caso: Paciente de sexo femenino de 63 años que consulta por mareos, náuseas, debilidad muscular, y rinorrea de dos años de evolución tratada con corticoides tópicos en forma prolongada. Al examen físico mostraba fenotipo Cushing característico. El laboratorio confirmó valores de ACTH veinte veces por encima de los rangos normales. En IRM cerebral se evidencia lesión expansiva en región etmoidal con compromiso de órbita y base de cráneo. Se resuelve tratamiento quirúrgico ante la necesidad de descompresión tumoral y tratamiento médico para hipercortisolismo en el período pre y posquirúrgico. La anatomía patológica informó proliferación neoplásica maligna de origen neurogénico y las pruebas inmunohistoquímicas confirmaron positividad para ACTH. Comentario: El Síndrome de Cushing por secreción ectópica de ACTH a partir de estesioneuroblastoma es muy poco frecuente, reportándose solo once casos a la fecha en la literatura médica indexada. El tratamiento es complejo y requiere de equipo interdisciplinario. Rev Argent Endocrinol Metab 51:192-196, 2014 Los autores declaran no poseer conflictos de interés.

Introduction: Esthesioneuroblastoma is a tumor originating from the neuroblasts in the neuroectodermal olfactory epithelium. It accounts for 3 % to 6 % of nasal tumors. Adrenocorticotropic hormone (ACTH) ectopic secretion is the cause for 10 % to 20 % of ACTH-dependent Cushing Syndrome cases. Esthesioneuroblastoma constitutes a rare cause of ectopic ACTH secretion. Case presentation: A 63 year-old female patient presented with dizziness, nausea, muscle weakness, and rhinorrhea of two-year duration under long-term treatment with topical corticosteroids. On physical examination, she showed typical Cushingoid phenotype. Brain MRI revealed an expanding lesion in the ethmoid area involving the orbit and the cranial base. Sub­maxillary metastasis was also detected. Lab tests confirmed ACTH values 20-fold above normal. The patient underwent treatment for hypercortisolism prior to surgery. Surgical treatment was prompted by the need to decompress the tumor. Comment: Esthesioneuroblastoma is a rare tumor. Although approximately 1,000 cases have been published in the last 20 years, only eleven of them were due to ACTH secretion. Treatment is complex and requires an interdisciplinary team. Rev Argent Endocrinol Metab 51:192-196, 2014 No financial conflicts of interest exist.

Cushing's Syndrome Caused by an ACTH-Producing Neuroendocrine Carcinoma of the Gallbladder / 대한내과학회지

Neuroendocrine tumors of the gallbladder are rare, and typically found incidentally after a cholecystectomy. Few data are available on adrenocorticotrophic hormone (ACTH)-producing neuroendocrine tumors originating specifically from the gallbladder. We experienced the case of a patient with a gallbladder mass who presented with Cushing's syndrome, who was subsequently diagnosed as an ACTH-producing neuroendocrine carcinoma of the gallbladder. Despite being rare, hormone-producing neuroendocrine tumors should be part of the differential diagnosis in patients with Cushing's syndrome.

Ectopic Adrenocorticotropic Hormone Syndrome Associated With Thymic Carcinoma in Old Age

A 70-year-old woman who developed a moon face was hospitalized. At admission, adrenocorticotropic hormone (ACTH) and serum cortisol levels were elevated. A high-dose dexamethasone suppression test demonstrated that cortisol secretion was uninhibited. Furthermore, chest computed tomography revealed an anterior mediastinal mass. On the basis of these results, ectopic ACTH syndrome was diagnosed and complete resection of the thymic tumor was performed. Histological examination revealed large cell neuroendocrine carcinoma of the thymus and positive immunoreactivity for ACTH. However, despite complete excision of the thymic tumor, ACTH and serum cortisol levels were high. Therefore, we investigated whether the lesion had recurred or metastasized; however, the results were inconclusive. Finally, laparoscopic bilateral adrenalectomy was performed to control the hypercortisolemia. After the surgery, the serum cortisol level was well-controlled and the general condition of the patient was good.

Ectopic ACTH Syndrome and Severe Hypokalemia Associated with Suspicious Pancreatic Malignancy: A Case Report / 대한신장학회지

A 72-year-old woman had a 1-month history of general weakness. Previously, after rectal cancer was treated with a low anterior resection, 12 cycles of adjuvant chemotherapy with 5-fluorouracil plus leucovorin was undergone. Follow-up examination showed no evidence of recurrence. However, she suffered from general weakness attributable to hypokalemia, which was refractory to potassium replacement therapy. After further work-up of the hypokalemia, Cushing's syndrome and ectopic adrenocorticotropic hormone (ACTH) syndrome were diagnosed from the laboratory findings that included a markedly elevated level of 24-hour urine-free cortisol. Several imaging studies revealed masses of the pancreas and other organs, which was more likely to be pancreatic cancer. This case is interesting in that cancerous lesions were identified during work-up of hypokalemia.

Six cases of ectopic ACTH syndrome / 中国实用内科杂志

1 000 nmol/L.We should search the cause of disease and perform tumor execution as soon as possible.The short-term prognosis is good.

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.

Ectopic ACTH Syndrome Due to Pheochromocytoma: A case report / 대한내분비외과학회지

A 28-year old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH at other hospital. However the operation was delayed due to poor general condition and abnormal liver function. She revealed severe hypercortisolism, hypokalemic metabolic alkalosis, glucose intolerance and showed rapid downhill course. She was transferred to our hospital for operation but septic complications were strarted already. She was not recovered in spite of desperate operation and died just after moribund discharge. ACTH producing pheochromocytoma is uncommon and literature review indicated high mortality rates due to improper preoperative diagnosis. Proper preoperative recognition and early surgical management is mandatory for better results.

2 Cases of Ectopic ACTH Syndrome due to Thymic Neuroendocrine Carcinoma Associated with Different Clinical Manifestations / 대한내분비학회지

Primary neuroendocrine carcinoma of the thymus (formerly known as thymic carcinoids) are unusual tumors that account for 5% of all anterior mediastinal neoplasms. Thymic neuroendocrine carcinomas exhibit a much more aggressive behavior than those originating from other foregut locations and are associated with the ectopic ACTH syndrome in 38% of cases. Patients having a thymic neuroendocrine carcinoma usually present with symptoms such as chest tightness, dyspnea, a palpable neck mass and sometimes a weight gain. We report on 2 patients with thymic neuroendocrine carcinoma who showed different clinical manifestations each other. One presented with a typical. Cushingoid appearance and the other had symptoms of fatigue and weight loss which is a common symptom of malignant tumors.

A Case of Ectopic ACTH Syndrome Caused by Thymic Carcinoid Tumor and Localized by PET / 대한내분비학회지

Ectopic ACTH syndrome is frequently caused by lung cancer and uncommonly by other tumors such as thymic carcinoid. For its treatment, early diagnosis and complete resection is irresponsible, but some cases are remained unlocalized in spite of all diagnostic modalities. Here we report a case of ectopic ACTH syndrome which was localized by PET but could not be localized by conventional technique. A tumor at thymic area was ACTH secreting thymic carcinoid which was operated but couldnt resect completly. Glucocorticoid hypersecretion was persisted with chemotherapy, radiotherapy, and ketoconazole treatment. Patient died of sepsis after 12 months of diagnosis.