scholarly journals Atypical Carcinoid Tumor of the Mediastinum Presenting as Ectopic Cushing’s Syndrome

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A991-A992
Author(s):  
Luis Augusto Medina Mora ◽  
Raheel Sufian Siddiqui ◽  
Ian Landry ◽  
Kwan Cheng ◽  
David Michael Reich
Keyword(s):  
Lymph Nodes ◽  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Mediastinal Mass ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Anterior Mediastinal Mass ◽  
Atypical Carcinoid ◽  
Mediastinal Lymph Nodes ◽  
History Of

Abstract Introduction: Ectopic Cushing’s syndrome (ECS) refers to Cushing’s syndrome (CS) that results from extra-pituitary secretion of adrenocorticotropic hormone (ACTH). Neuroendocrine tumors (NETs) are a rare cause of ECS, and their presence in the anterior mediastinum only represents 2-4% of all cases. We report a rare case of ECS in a patient with an atypical mediastinal carcinoid tumor. Case Presentation: A 38-year-old male with a history of depression presented with generalized weakness, fatigue, and myalgia for one month. A one-week history of nausea and slight shortness of breath was reported. Physical exam and vital signs were unremarkable. Labs revealed hyperglycemia, hypokalemia, and metabolic alkalosis. A random serum cortisol was 47.7 mcg/dL (normal: 3-21 mcg/dL) and a 24-hour urine free cortisol was 7405 mcg/24 hours (normal: 3.5-45 mcg/24 hours). The morning serum cortisol after 8 mg dexamethasone at midnight, was 58.7 mcg/dL. Serum aldosterone and plasma renin activity were both undetectable. CT of the chest showed an anterior mediastinal mass measuring 9.0 x 10.3 x 6.1 cm accompanied by two large mediastinal lymph nodes. A PET CT confirmed the anterior mediastinal mass, which showed mild heterogeneous uptake and no evidence of metastatic disease. Core biopsy of mediastinal mass showed grade II neuroendocrine tumor/atypical carcinoid. Potassium, Spironolactone, Ketoconazole, and Metyrapone were used to control hypokalemia and hypercortisolemia. Trimethoprim-sulfamethoxazole (TMP-SMX) was started for Pneumocystis pneumonia prophylaxis. The patient underwent complete resection of the anterior mediastinal mass, thymectomy, and mediastinal lymph nodes dissection. Histopathology confirmed an atypical carcinoid in the mediastinal mass with negative margins. The rest of the thymus tissue and lymph nodes were negative for malignancy. ACTH and cortisol levels dropped significantly after surgery and the patient was discharged on oral hydrocortisone and TMP-SMX. At 4 weeks follow-up in the clinic, the patient was feeling much better and reported complete resolution of symptoms. Discussion: ECS caused by an atypical carcinoid tumor is an extremely rare finding. Mediastinal NETs tend to have a poor prognosis due to their metastatic tendency and recurrence rates. Due to limited evidence in the medical literature, there’s much uncertainty and controversy regarding the best treatment of NETs. Standardized guidelines for the management of these atypical tumors are not well established. The primary mode of treatment is surgical resection of the ACTH-secreting tumor. However, in cases of advanced and metastatic tumors, surgical resection might not be sufficient and medical therapy with adrenal enzyme synthesis inhibitors may be needed. The use of neoadjuvant chemotherapy and/or radiotherapy remains unclear.

scholarly journals ACTH-Producing Thymic Carcinoid: A Rare Cause of Cushing’s Syndrome

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A990-A990
Author(s):  
Lisette Patricia Rodriguez ◽  
Wende Michele Kozlow
Keyword(s):  
Cushing’S Syndrome ◽  
Mediastinal Mass ◽  
Cushing's Syndrome ◽  
Anterior Mediastinal Mass ◽  
Thymic Carcinoid ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
History Of

Abstract Background: Thymic carcinoids are rare neoplasms that account for less than 5% of all thymic tumors. Approximately 25% of these tumors will result in Cushing’s syndrome due to ectopic ACTH secretion. These tumors can also be associated with MEN1 syndrome. This is a case report of a patient with history of macroprolactinoma now presenting with Cushing’s syndrome due to ectopic ACTH production from a thymic carcinoid tumor. Clinical Case: This is a 57 year old male with history of pituitary macroprolactinoma diagnosed in 2011, now status post transsphenoidal resection and external beam radiation therapy, with persistent hyperprolactinemia on cabergoline, who presented to our clinic for a routine follow up visit. Patient had already developed secondary hypogonadism and secondary hypothyroidism as a consequence of treatment for the macroprolactinoma. He complained of worsening fatigue and weight gain ongoing for several months. Laboratory studies revealed an hemoglobin A1c of 8.3% (nl < 5.7%), TSH 0.24 MIU/L (0.4-4.5 MIU/L), free T4 1.2 ng/dL (0.8-1.8 ng/dL), 8 AM cortisol 31.4 mcg/dL (4-22 mcg/dL), ACTH 185 pg/mL (6-50 pg/dL), prolactin 29.6 ng/mL (2-18 ng/mL), IGF-1 88 ng/mL (50-317 ng/mL). Follow up labs confirmed cushings syndrome: cortisol AM-DST 36.4 mcg/dL (< 2 mcg/dL), free urinary cortisol 291.9 mcg/24h (2-50 mcg/24h). Pituitary MRI showed empty sella turcica. Cortisol after an 8 mg DST 32.5 mcg/dL (< 5 mcg/dL). CT chest, abdomen and pelvis revealed an heterogeneously enhancing solid anterior mediastinal mass measuring 4.9 x 3.1 x 4.3 cm. Whole body OctreoScan showed a markedly hyperintense large mass adjacent to the right heart border measuring 47 x 32 mm. He was referred to cardiothoracic surgery and underwent a right video-assisted thoracic surgery with resection of the anterior mediastinal mass. Pathology revealed a thymic well-differentiated neuroendocrine tumor with strong cytoplasmic staining for ACTH. It was also positive for OSCAR, Cam5.2, synaptophysin, CD56, and S100. Ki67 stain was positive in fewer than 1% of tumor cells. Final diagnosis was carcinoid tumor. Conclusion: Cushing’s syndrome secondary to ectopic ACTH secretion from a thymic carcinoid is rare. The presence of two MEN1-associated tumors in this patient, macroprolactinoma and thymic carcinoid, is highly suggestive of a clinical diagnosis of MEN 1.

AB040. PS02.04: DOTATATE PET/CT in thymic atypical carcinoid tumor with secondary ACTH-dependent Cushing’s syndrome

Mediastinum ◽  
2017 ◽  
Vol 1 ◽  
pp. AB040-AB040
Author(s):  
John Agzarian ◽  
Hisham Qandeel ◽  
Irina Bancos ◽  
Geoffrey B. Johnson ◽  
Stephen C. Scharf ◽  
...  
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Atypical Carcinoid ◽  
Pet Ct

scholarly journals Atypical Carcinoid Tumor of the Mediastinum Presenting as Cushing’s Syndrome in an Otherwise Healthy Young Male

Cureus ◽  
2021 ◽  
Author(s):  
Ian Landry ◽  
Luis A Medina Mora ◽  
Raheel Siddiqui ◽  
Taisiya Tumarinson ◽  
David M Reich
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Young Male ◽  
Cushing's Syndrome ◽  
Atypical Carcinoid

scholarly journals Prevalence and echo features of mediastinal lymph nodes in EUS for non-malignant indications: a prospective study in a Southern European Population

2018 ◽  
Vol 06 (04) ◽  
pp. E432-E436
Author(s):  
Joana Carmo ◽  
Miguel Bispo ◽  
Susana Marques ◽  
Cristina Chagas
Keyword(s):  
Lymph Nodes ◽  
European Population ◽  
Significant Heterogeneity ◽  
Mediastinal Lymph Nodes ◽  
Single Center Study ◽  
History Of ◽  
Echo Features ◽  
Portuguese Study ◽  
A Prospective Study ◽  
Ct Studies

Abstract Background and study aims Significant heterogeneity in geographic distribution regarding the prevalence of mediastinal lymph nodes (MLN) has been documented in autopsy and computed tomography (CT) studies. Awareness of the local prevalence and characteristics of lymph nodes will be relevant when performing endoscopic ultrasonography (EUS) for staging of malignant neoplasias. The aims of this study were to document the prevalence and echo features of MLN in patients undergoing EUS for non-malignant extrathoracic disease and to identify predictive factors for the presence of MLN. Patients and methods A prospective single-center study was performed over 6 months. Mediastinal stations 9, 8, 7, 6, 5, 4 L and 2 were systematically evaluated using a linear echoendoscope in all patients undergoing EUS due to benign extrathoracic pathology and without history of oncologic disease. Demographic, clinical and EUS features of the lymph nodes were analysed. Results Seventy-five patients were included: male/female 32/43; mean age, 63 years. The majority of patients (72 %) had lymph nodes in at least one mediastinal station and 88 % of these were found in stations 7 or 4 L. Overall, 133 MLN were identified: 19 % were hypoechogenic, 6 % had a short-axis diameter > 10 mm, and 6 % were round. The prevalence of lymph nodes was higher in smokers (83 % vs 64 %, P = 0.024), with a higher average number of lymph nodes per patient in this group (2.1 vs 1.6; P = 0.017). By logistic regression analysis, none of the variables analyzed were independently associated with the presence of MLN. Conclusion This prospective Portuguese study documented a higher prevalence of MLN than previously reported in Northern Europe, in patients with no evidence of oncologic disease. This higher prevalence may negatively influence the specificity and positive predictive value for malignancy of MLN (N) staging by EUS.

scholarly journals Efficacy of pasireotide in controlling severe hypercortisolism until cardiac transplantation

2017 ◽  
Vol 2017 ◽  
Author(s):  
Roberto Attanasio ◽  
Liana Cortesi ◽  
Daniela Gianola ◽  
Claudia Vettori ◽  
Fulvio Sileo ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Cushing’S Syndrome ◽  
Cardiac Transplantation ◽  
Serum Cortisol ◽  
Primary Treatment ◽  
Cushing's Syndrome ◽  
Line Treatment ◽  
First Line ◽  
First Line Treatment ◽  
Undergo Heart Transplantation

Summary Cushing’s syndrome is associated with increased morbidity and mortality. Although surgery is the first-line treatment, drugs can still play a role as an ancillary treatment to be employed while waiting for surgery, after unsuccessful operation or in patients unsuitable for surgery. We were asked to evaluate a 32-year-old male waiting for cardiac transplantation. Idiopathic hypokinetic cardiomyopathy had been diagnosed since 6 years. He was on treatment with multiple drugs, had a pacemaker, an implantable cardioverter and an external device for the support of systolic function. Physical examination showed severely impaired general status, signs of hypercortisolism and multiple vertebral compression fractures. We administered teriparatide, and the few evaluable parameters supported the diagnosis of ACTH-dependent hypercortisolism: serum cortisol was 24.2 µg/dL in the morning and 20.3 µg/dL after overnight 1 mg dexamethasone, urinary free cortisol (UFC) was 258 µg/24 h and ACTH 125 pg/mL. Pituitary CT was negative. Pasireotide 300 µg bid was administered and uptitrated to 600 µg bid. Treatment was well tolerated, achieving dramatic improvement of clinical picture with progressive normalization of serum cortisol and ACTH levels as well as UFC. After 4 months, the patient underwent successful heart transplantation. Many complications ensued and were overcome. Pituitary MRI was negative. On pasireotide 300 µg bid and prednisone 2.5 mg/day (as part of immunosuppressive therapy), morning serum cortisol and ACTH were 15.6 µg/dL and 54 pg/mL respectively, UFC was 37 µg/24 h, fasting glucose: 107 mg/dL and HbA1c: 6.5%. In conclusion, primary treatment with pasireotide achieved remission of hypercortisolism, thus allowing the patient to undergo heart transplantation. Learning points: Untreated Cushing’s syndrome is associated with ominous prognosis. First-line treatment is surgery (at pituitary or adrenal, according to disease localization). A few drugs are available to treat hypercortisolism. Pasireotide is a multi-ligand somatostatin analog approved for treatment of hypercortisolism. Primary treatment with pasireotide was effective in a patient with severe Cushing’s syndrome, allowing him to undergo heart transplantation.

Serum cortisol concentrations during low dose dexamethasone suppression test to screen for Cushing's syndrome

BMJ ◽  
1985 ◽  
Vol 290 (6462) ◽  
pp. 158-159
Author(s):  
L. Kennedy ◽  
D. Hadden ◽  
B. Atkinson ◽  
B Sheridan ◽  
H. Johnston
Keyword(s):  
Cushing’S Syndrome ◽  
Low Dose ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Dexamethasone Suppression Test ◽  
Suppression Test ◽  
Dexamethasone Suppression

scholarly journals Arterial thrombosis as primary presentation of endogenous Cushing’s syndrome

2019 ◽  
Vol 12 (2) ◽  
pp. bcr-2018-227491
Author(s):  
Vijay Alexander ◽  
Maria Koshy ◽  
Riddhi Dasgupta ◽  
Ronald Albert Carey
Keyword(s):  
Cushing’S Syndrome ◽  
Arterial Thrombosis ◽  
Clinical Manifestations ◽  
Dynamic Mri ◽  
Cushing's Syndrome ◽  
Positron Emission ◽  
History Of ◽  
Laboratory Investigations ◽  
Primary Presentation

Cushing’s syndrome is known to present with a characteristic set of clinical manifestations and complications, well described in literature. However, hypercoagulability remains an under recognised entity in Cushing’s syndrome. A 31-year-old woman from Southern India presented with history of fever, left upper quadrant pain and progressive breathing difficulty for 3 weeks. Clinical examination revealed discriminatory features of Cushing’s syndrome. Laboratory investigations showed biochemical features of endogenous ACTH-dependent Cushing’s syndrome. Imaging of the abdomen revealed splenic collection, left-sided empyema and extensive arterial thrombosis. Gadolinium enhanced dynamic MRI of the pituitary gland revealed no evidence of an adenoma while a Ga-68 DOTATATE positron emission tomography CT scan ruled out an ectopic Cushing’s. A diagnosis of endogenous Cushing’s syndrome causing a prothrombotic state with extensive arterial thrombosis was made. She was initiated on oral anticoagulation and oral ketoconazole for medical adrenal suppression. She subsequently underwent bilateral adrenalectomy and was well at follow-up.

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