scholarly journals Cushing Syndrome Revealing an Adrenocortical Carcinoma

2018 ◽  
Vol 08 (02) ◽  
pp. 87-93
Author(s):  
Bintou Sanogo ◽  
Senkaye-Lagom Aimée Kissou ◽  
Zakari Nikiema ◽  
Makoura Barro ◽  
Djingri Lankouandé ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Cushing Syndrome

A 12-year-old Chinese girl with Cushing syndrome and virilization due to adrenocortical carcinoma

2011 ◽  
Vol 24 (3-4) ◽  
Author(s):  
Lap Ming Wong ◽  
Chak Ho Li ◽  
On Kei Angel Chan ◽  
Chi Chung Shek ◽  
Ngai Shan Kwong
Keyword(s):  
Adrenocortical Carcinoma ◽  
Cushing Syndrome ◽  
Old Chinese ◽  
Chinese Girl

scholarly journals Relacorilant With Pembrolizumab: A Phase 1b, Open-Label Study of a Selective Glucocorticoid Receptor Modulator Combined With a Checkpoint Inhibitor for Patients With Adrenocortical Carcinoma With Excess Glucocorticoid Production

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A94-A94
Author(s):  
Mouhammed Amir Habra ◽  
Gary D Hammer ◽  
Electron Kebebew ◽  
Francis P Worden ◽  
Nitya Raj ◽  
...  
Keyword(s):  
Glucocorticoid Receptor ◽  
Adrenocortical Carcinoma ◽  
Cushing Syndrome ◽  
Checkpoint Inhibitors ◽  
Combined Treatment ◽  
Survival Duration ◽  
Progression Rate ◽  
Late Night ◽  
Free Cortisol ◽  
Phase 1B

Abstract Adrenocortical carcinoma (ACC) is an aggressive cancer with poor response to chemotherapeutic and immunotherapeutic agents. About half of ACCs produce glucocorticoids (GC), and the presence of GC excess (hypercortisolism) is correlated with decreased survival in patients with ACC. The broad immunosuppressive effects of GC may contribute to the limited efficacy of immune checkpoint inhibitors, such as pembrolizumab, in these patients. Antagonism of the glucocorticoid receptor (GR) has the potential to increase immune-related transcripts, thus promoting tumor immune response in ACC with GC excess. To test this hypothesis, we introduce a phase 1b study evaluating the combined treatment of relacorilant (CORT125134, Corcept Therapeutics) with pembrolizumab in patients with advanced ACC and hypercortisolism (NCT04373265). Relacorilant is a selective (no activity at other steroid receptors), oral GR modulator in development for Cushing syndrome and, in combination with chemotherapy, for various solid tumors. In healthy subjects, prednisone causes rapid reductions in eosinophils, lymphocytes, and osteocalcin and rapid increases in neutrophils. Relacorilant ameliorates these effects. In a phase 2 study in patients with endogenous Cushing syndrome treated with relacorilant, improvements in the signs and symptoms of GC excess were seen. The primary objective of this study is to determine the safety and efficacy of the recommended regimen of relacorilant with pembrolizumab in patients with advanced ACC and hypercortisolism. Pembrolizumab infusion will occur on day 1 of each 21-day cycle, and relacorilant will be administered once daily, starting 3 days before the first pembrolizumab infusion. Relacorilant doses will be escalated in 100-mg increments (100 mg up to 400 mg, as tolerated). Patients will receive treatment until they experience disease progression or unacceptable toxicity. Approximately 20 adults with confirmed advanced, unresectable and/or metastatic ACC will be enrolled. GC excess must be documented by either ACTH <10 pg/mL and serum cortisol >1.8 µg/dL after dexamethasone suppression testing (DST), or the presence of two of the following criteria: elevated urinary free cortisol; high late-night salivary cortisol; and DST cortisol >1.8 µg/dL. Assessments will include safety, tolerability, and efficacy. Secondary objectives include a determination of the non-progression rate at 27 weeks, evaluation of progression-free survival, overall survival, duration of response, and an assessment of the effect of the combination on clinical manifestations of hypercortisolism. This will be the first clinical study to evaluate whether GR antagonism promotes tumor response in patients with ACC and GC excess treated with checkpoint inhibitors.

scholarly journals Androgen secreting giant adrenocortical carcinoma with no metastases: A case report and review of the literature

2015 ◽  
Vol 9 (9-10) ◽  
pp. 644
Author(s):  
Fatih Uruc ◽  
Ahmet Urkmez ◽  
Ozgur Haki Yuksel ◽  
Aytac Sahin ◽  
Ayhan Verit
Keyword(s):  
Abdominal Pain ◽  
Case Report ◽  
Rare Disease ◽  
Female Patient ◽  
Adrenocortical Carcinoma ◽  
Poor Prognosis ◽  
Cushing Syndrome ◽  
Review Of The Literature ◽  
En Bloc

Functional adrenocortical carcinoma (ACC) is a very rare disease with a poor prognosis. Over half (60%) of ACCs bigger than 6 cm synthesize hormones; hormone-secreting ACCs generally include virilization, feminization or Cushing syndrome. Besides, 82% of ACCs are metastatic at the time of diagnosis. While a 48-year-old female patient was examined for abdominal pain and flushing, we detected a non-metastasizing mass (23 × 18 × 16 cm) in the adrenal lodge. The mass was extracted en bloc during open exploration and its histopathology was reported as ACC. We review the literature and report the largest androgen-producing, clinically silent ACC mass cited in the literature so far.

Peripheral blood steroid levels in cushing syndrome due to adrenocortical carcinoma or adenoma

Urology ◽  
1983 ◽  
Vol 22 (6) ◽  
pp. 576-579 ◽  
Author(s):  
J.L. Gabrilove ◽  
E.K. Freiberg ◽  
G.L. Nicolis
Keyword(s):  
Adrenocortical Carcinoma ◽  
Peripheral Blood ◽  
Cushing Syndrome

scholarly journals Adrenal and Hepatic Venous Sampling in a Case of Aldosterone-Producing Adrenocortical Carcinoma with Hepatic Metastasis

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Toru Sugiyama ◽  
Yuriko Sasahara ◽  
Hironobu Sasano ◽  
Eri Hayakawa
Keyword(s):  
Liver Metastases ◽  
Hepatic Metastasis ◽  
Adrenocortical Carcinoma ◽  
Cushing Syndrome ◽  
Circadian Variation ◽  
Plasma Renin ◽  
Venous Sampling ◽  
Large Tumor ◽  
Plasma Aldosterone Concentration ◽  
Aldosterone Production

Background. Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignancy. ACCs often secrete adrenal steroid hormones including cortisol and androgens; however, aldosterone-producing ACC is very rare. Although adrenal production of aldosterone is assessed by adrenal venous sampling, the use of sampling from the relevant vein to assess aldosterone production from a tumor arising from ACC metastasis has not been previously reported. Case Presentation. We report the case of a 69-year-old Japanese man with aldosterone-producing ACC with hepatic metastasis. He presented with a history of treatment-resistant hypertension and hypokalemia. Endocrinological examination showed markedly increased plasma aldosterone concentration and suppressed plasma renin activity. Serum cortisol concentration was not suppressed by administration of dexamethasone 1 mg, and normal circadian variation of cortisol secretion was disrupted. Abdominal computed tomography showed a large tumor in the left adrenal gland and multiple tumors in the liver. Together, these results strongly suggested ACC with multiple liver metastases causing primary aldosteronism and subclinical Cushing syndrome. Adrenal and hepatic venous sampling showed markedly increased aldosterone concentration in the left adrenal vein but no increase in the hepatic vein, despite a pathological diagnosis of ACC with hepatic metastasis, with immunohistochemical investigation showing both primary and secondary tumors to have synthetic capability for aldosterone. The patient received mitotane but declined combination chemotherapy and died 2 months later. Conclusion. This is the first report of adrenal and hepatic venous sampling in a case of aldosterone-producing ACC with hepatic metastasis. The case suggests that hepatic venous sampling is unable to detect aldosterone production from liver metastases arising from ACC.

scholarly journals MON-LB061 Cushing Syndrome Due to an Adrenocortical Carcinoma in a Baby with Atypical Beckwith-Wiedemann Syndrome

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Abdullah Bereket ◽  
Mehmet Eltan ◽  
Kivilcim Cerit ◽  
Sare Betül Kaygusuz ◽  
Esra Ates ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Cushing Syndrome

scholarly journals SUN-198 Metastatic Progression of Adrenocortical Carcinoma: Phenotypic Transformation to ACTH-Independent Cushing’s Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Javier Ocampo ◽  
Maria Gracia Luzuriaga ◽  
Alejandro Raul Ayala
Keyword(s):  
Weight Gain ◽  
Metastatic Disease ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Cushing Syndrome ◽  
Cushing's Syndrome ◽  
Metastatic Progression ◽  
Phenotypic Transformation ◽  
Rare Malignancy

Abstract Background: Adrenocortical carcinoma (ACC) is a rare malignancy. Around 60% of adrenal carcinomas are functioning tumors and may present with distinctive phenotypes (1). Very rarely, as the disease progresses, changes in secretory patterns may be observed. Clinical Case: A 39-year-old female presented to the hospital with a 1-month history of abdominal pain associated with hirsutism, weight gain, acne, and amenorrhea. Computed tomography of the abdomen showed a 7 cm left adrenal mass with areas of necrosis as well as presumptive metastatic disease involving the liver and lungs. Biopsy of the adrenal mass confirmed the diagnosis of adrenocortical carcinoma. Initial laboratories were compatible with hyperandrogenemia (increased 24-hour urine 17-ketosteroid level of 106.3 mg/24 hr (n 6.0-15.0) and mildly elevated testosterone of 65 ng/dL (n<45)). Aldosterone, renin, and metanephrines levels were normal. The patient underwent adrenalectomy after 1 month and was placed on hydrocortisone replacement. Follow-up biochemical testing showed a decrease in 17-ketosteroids level to 24.7 mg/24hr. DHEAS and testosterone were persistently elevated. Following discontinuation of hydrocortisone, her 24-hour urine cortisol was normal at 18 ug/24hr, (n 6-24) and ACTH was suppressed at 1.8 pg/mL (n 7-63). She was started on mitotane therapy shortly after. Three months after initiation of treatment, she was admitted for a pulmonary embolism. At that time, she had clear signs of hypercortisolism, such as weight gain, hyperglycemia, easy bruising as well as purple striae. Progressive metastatic disease with enlarged lung and liver masses was observed in imaging. On this occasion, her morning cortisol was elevated at 35 ug/dL and ACTH was suppressed at 1 pg/mL. Th hyperandrogenemia was more evident this time with DHEAS of 778 ug/dl. Steroid supplementation was discontinued, and ketoconazole was started. An elevated 24-hour urinary cortisol (2085 ug/24hr) confirmed the diagnosis of Cushing Syndrome. Given the progression of disease while on mitotane, the patient was started on chemotherapy with etoposide, doxorubicin, and cisplatin. Conclusion: This case represents an unusual example of the phenotypic transformation of adrenal cancer resulting in new-onset ACTH-independent Cushing’s syndrome while the patient was on treatment. Awareness of such changes in secretory pattern are important to guide therapy and minimize morbidity associated with hypercortisolism. Reference: (1) Puglisi S, Perotti P, Pia A, Reimondo G, Terzolo M. Adrenocortical Carcinoma with Hypercortisolism. Endocrinol Metab Clin North Am. 2018 Jun;47(2):395-407.

Adrenocortical Carcinoma Presenting with Cushing Syndrome

2014 ◽  
Vol 191 (3) ◽  
pp. 800-801 ◽  
Author(s):  
Nidhi Sharma ◽  
Brian R. Herts
Keyword(s):  
Adrenocortical Carcinoma ◽  
Cushing Syndrome
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