scholarly journals Paediatric Nonfunctioning Adrenocortical Carcinoma with Extension up to Right-Side Heart: Cardiac Surgery Approach

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Federica Iezzi ◽  
Andrea Quarti ◽  
Chiara Surace ◽  
Marco Pozzi
Keyword(s):  
Cardiac Surgery ◽  
Adrenal Gland ◽  
Adjuvant Treatment ◽  
Adrenocortical Carcinoma ◽  
Rare Case ◽  
Cardiac Involvement ◽  
Adrenal Carcinoma ◽  
Rare Malignancy ◽  
The Right ◽  
Treatment Prognosis

Adrenocortical carcinoma is a rare malignancy. Due to late diagnosis and no adequate effective adjuvant treatment, prognosis remains poor. Only approximately 30% of these malignancies are confined to the adrenal gland when they are diagnosed, as these tumors tend to be found years after their genesis. Cardiac involvement of adrenal carcinoma is very rare. We report a rare case of a 7-year-old female with right adrenal cortical carcinoma, involving the right-side heart.

scholarly journals SUN-169 A Rare Case of Adrenocortical Carcinoma Presenting as Hyperaldosteronism Combined with Cushing’s Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Emma Punni ◽  
Jonea Lim
Keyword(s):  
Adrenal Gland ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Rare Case ◽  
Plasma Catecholamines ◽  
Cushing's Syndrome ◽  
Adrenal Crisis ◽  
Pathology Report ◽  
Dheas Level ◽  
The Right

Abstract Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Most ACC cases are hormonally functional. Commonly produced hormones are cortisol, followed by adrenal androgens. Aldosterone secretion is quite rare (< 2%) (1). Co-secretion of multiple hormones is further rare. Case Presentation: A 59 year old female presented with progressive worsening hypertension. Initial biochemical evaluation confirmed primary hyperaldosteronism. Her potassium was low at 2.9 mEq/L (N: 3.5-5.3 meq/L). Aldosterone level was elevated at 23 ng/dL (N: < or = 28 ng/mL), renin value was 0.90 ng/mL/h (N: 0.25-5.82 ng/mL/h). Aldosterone Renin Ratio was 25. CT abdomen with and without contrast showed 2 lesions within the right adrenal gland. The larger nodule was 3.7 x 2.7 x 4.9 cm with an absolute washout of 61%. A smaller nodule was 2.3x1.8 cm with an absolute washout of approximately 64%. Left adrenal gland was unremarkable. Further biochemical workup showed plasma catecholamines and metanephrines were normal. A random cortisol value was 22.8 mcg/dL (N: 3.0-16.0 mcg/dL) with a low ACTH level of 3.3 pg/mL (N: 7.2 - 63.3 pg/mL). Given suspicion for adrenal Cushing’s syndrome, we further obtained a 24 hour free urinary cortisol which was elevated at 84.1 mcg/24 h (N: 4-50 mcg/24 h). 8 am Cortisol after an overnight 1 mg Dexamethasone failed to suppress at 17.5 mcg/dL (n<1.8 mcg/dL). DHEAS level was low at 14.1 ug/dL (N: 29.4-220.5 ug/dL). The patient eventually underwent a right adrenalectomy. Post-operatively, her cortisol was suppressed at 1.9 mcg/dL (8 am ref range: 4-22 mcg/dL), and Hydrocortisone replacement dose was initiated. Surgical pathology report was consistent with adrenocortical carcinoma. The patient continues to follow-up with the endocrinology and oncology department for treatment. Conclusion: This case is particularly interesting given the co-secretion of both aldosterone and cortisol by an adrenocortical carcinoma which has been reported in only a few cases in literature. The case highlights the importance of completing a comprehensive biochemical workup pre-operatively in patients with suspicious adrenal mass. There should especially be a low threshold for initiating workup for cortisol hypersecretion as early intervention can help avoid an adrenal crisis in the post-operative period for such patients. A low DHEAS level should raise suspicion for cortisol hypersecretion in a patient with adrenal lesions. As ACTH is the primary stimulant of DHEA, the suppression of ACTH secretion in the setting of adrenal Cushing’s syndrome can contribute to a low DHEAS level. Reference 1.Else, T et al. Adrenocortical Carcinoma. Endocr Rev. 2014; 35(2):282-326.

Virilizing Adrenocortical Carcinoma Resected With Negative Margins Treated With Surgery Alone

2021 ◽  
pp. 000313482110508
Author(s):  
Tyler Fields ◽  
Kelsee Felux ◽  
Alejandro Chavarriaga ◽  
Philip Ramsay ◽  
Evan Weitman
Keyword(s):  
Menstrual Cycle ◽  
Adjuvant Treatment ◽  
Adrenocortical Carcinoma ◽  
Rare Case ◽  
Physical Exam ◽  
Rare Malignancy ◽  
Uncommon Tumor

Adrenocortical carcinoma is a rare malignancy. A virilizing adrenocortical carcinoma is even more unique of a diagnosis. In this report, we present a rare case of this uncommon tumor with an interesting presentation, clearly documented physical exam changes over a span of at least 8 years, and a technically challenging case. We also briefly review the management of adrenocortical carcinoma. The tumor was successfully resected with no planned adjuvant treatment at this time. The patient had recurrence of menstrual cycle post-operatively and required no steroid supplementation.

scholarly journals Virilization in a Girl with Adrenocortical Adenoma: A Case Report

2013 ◽  
Vol 6 (2) ◽  
pp. 70-72
Author(s):  
Tahniyah Haq ◽  
SM Ashrafuzzaman ◽  
Zafar A Latif
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Adrenal Gland ◽  
Ct Scan ◽  
Dehydroepiandrosterone Sulfate ◽  
Adrenocortical Adenoma ◽  
Adrenal Carcinoma ◽  
Mass Size ◽  
The Right ◽  
Androgen Levels

We present a case of Cushing’s syndrome and virilization in a 15 year old girl which was suspected to be due to an adrenal carcinoma. She presented with features of virilization in addition to those of hypercortisilism. Her high androgen levels especially dehydroepiandrosterone sulfate (DHEAS) were also in favor of an adrenal carcinoma. An unenhanced computerized tomography (CT) scan showed a mass (size: 5.3 cm) in the right adrenal gland with a soft tissue intensity of more than 10 HU which was suggestive of adrenal carcinoma. But, histopathology of the resected mass revealed a benign adrenocortical adenoma. DOI: http://dx.doi.org/10.3329/imcj.v6i2.14736 Ibrahim Med. Coll. J. 2012; 6(2): 70-72

scholarly journals Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome

2013 ◽  
Vol 3 ◽  
pp. 32 ◽  
Author(s):  
Santosh Kumar ◽  
Gautam R. Choudhary ◽  
Arawat Pushkarna
Keyword(s):  
Inferior Vena Cava ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Right Atrium ◽  
Inferior Vena ◽  
Vena Cava ◽  
Cushing's Syndrome ◽  
Curative Therapy ◽  
Rare Malignancy ◽  
The Right

Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Surgery is the only curative therapy available and overall 5-year survival for patients who undergo a complete resection is 32% to 48%. They are known to produce intravascular invasion and into the inferior vena cava (IVC) and in rare cases they may reach the right atrium. We report a case of functioning ACC extending into the inferior vena cava and right atrium in a female with Cushing's syndrome.

scholarly journals Oncocytic Variant of Adrenocortical Carcinoma: Case Report of a Rare Malignancy

2020 ◽  
pp. 1-4
Author(s):  
Sarath Sistla ◽  
Balamourougan Krishnaraj ◽  
Gomathi Shankar ◽  
Jigish Ruparelia ◽  
Prakriti Giri ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Surgical Resection ◽  
Adrenocortical Carcinoma ◽  
Histopathological Examination ◽  
Large Mass ◽  
Disease Stage ◽  
Surgical Specimen ◽  
Oncocytic Variant ◽  
Rare Malignancy ◽  
Oncocytic Neoplasm

Adrenocortical carcinoma is a rare cancer. Oncocytic tumors of the adrenal gland are rarer. Most Oncocytic Adrenal Neoplasms are benign and carry favourable prognosis. They are classified as oncocytoma, oncocytic neoplasm of uncertain malignant potential and oncocytic adrenal carcinoma. The malignant nature of oncocytic neoplasm of adrenal gland can only be confirmed on histopathology. We report a case of a 55-year-old male with newly diagnosed hypertension being evaluated for left adrenal mass concerning for adrenocortical carcinoma. Open radical left adrenalectomy and nephrectomy was done and histopathology confirmed oncocytic variant of adrenocortical carcinoma based on Lin-Weiss-Bisceglia scoring system which has been developed particularly for oncocytic type of tumor. Though rare, oncocytic neoplasm has to be considered as one of the differential diagnoses of adrenocortical mass, especially those presenting as a large mass because malignant oncocytic neoplasm of adrenal gland as large as 23cm have been reported. Imaging modalities like ultrasonography, computed tomography or magnetic resonance imaging, though useful in evaluating an adrenocortical mass, cannot predict malignant nature of an oncocytic neoplasm. Diagnosis of adrenocortical carcinoma is therefore reliably made only after histopathological examination of the surgical specimen. Surgical resection in those presenting with nonmetastatic resectable disease remains the mainstay of ACC treatment. Oncocytic ACC compared with conventional ACCs matched for age, gender, disease stage and status of surgical resection, shows significant better overall survival thus representing more indolent variant of an aggressive and often fatal disease.

scholarly journals Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma

2010 ◽  
Vol 54 (4) ◽  
pp. 419-424 ◽  
Author(s):  
Lívia Mara Mermejo ◽  
Jorge Elias Junior ◽  
Fabiano Pinto Saggioro ◽  
Silvio Tucci Junior ◽  
Margaret de Castro ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Adrenal Gland ◽  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Surgical Excision ◽  
Abdominal Ultrasound ◽  
Hydroxylase Deficiency ◽  
Adrenal Myelolipoma ◽  
The Right ◽  
21 Hydroxylase Deficiency

The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

Concurrent adrenocortical carcinoma and Conn's adenoma in a man with primary hyperaldosteronism. In vivo and in vitro studies

1992 ◽  
Vol 127 (2) ◽  
pp. 189-192 ◽  
Author(s):  
Y Touitou ◽  
A Boissonnas ◽  
A Bogdan ◽  
A Auzéby
Keyword(s):  
Adrenal Gland ◽  
Adrenocortical Carcinoma ◽  
Primary Aldosteronism ◽  
Unusual Case ◽  
In Vitro Studies ◽  
In Vitro Biosynthesis ◽  
The Right ◽  
Tomography Scan

This is a report of a rare and unusual case of adrenal pathology. A patient presented with clinical and biological signs of primary aldosteronism and computed body tomography scan led to our suspecting the presence of a left adrenocortical carcinoma. The in vitro studies performed on the resected tumour showed very low synthesis of mineralocorticoids and glucocorticoids. The patient could not be re-examined until 15 months later, when he still suffered hypertension; another tomography scan revealed a mass on the right adrenal gland. The studies performed on this second tumour confirmed the diagnosis of Conn's adenoma: active in vitro biosynthesis of 18-hydroxy-corticosterone and aldosterone from exogenous tritiated precursors.

Virilising adrenocortical carcinoma

2021 ◽  
Vol 14 (6) ◽  
pp. e242895
Author(s):  
Diogo Nunes Correia ◽  
Inês Redondo de Carvalho ◽  
Jeenal Assuani Mangi
Keyword(s):  
Adrenocortical Carcinoma ◽  
Median Overall Survival ◽  
Elevated Serum ◽  
Imaging Study ◽  
Palpable Mass ◽  
Hepatic Invasion ◽  
Serum Androgens ◽  
Rare Malignancy ◽  
The Right

Adrenocortical carcinoma (ACC) is a rare malignancy, with an estimated annual incidence of 0.7–2 cases per million and a median overall survival of 3–4 years. Hormone-secreting ACCs represent most cases; of these, only a small minority presents with virilisation alone. Early diagnosis is key to increase the chances of a better outcome. Here, we report a case of a 41-year-old woman who presented with menstrual irregularities, hirsutism and virilising symptoms, associated with abdominal discomfort and constitutional symptoms. On physical examination, there was a palpable mass in the right upper quadrant. Laboratory workup revealed elevated serum androgens. The imaging study showed a 163×110×122 cm right adrenal mass with features consistent with ACC and suggested potential hepatic invasion. Our patient underwent surgical resection, and the histopathological findings confirmed the diagnosis. She was referred to a specialised centre for follow-up and adjuvant therapy.

scholarly journals Pathobiology and genetics of adrenocortical carcinoma

2019 ◽  
Vol 62 (2) ◽  
pp. R105-R119 ◽  
Author(s):  
James F H Pittaway ◽  
Leonardo Guasti
Keyword(s):  
Adrenal Cortex ◽  
Adjuvant Treatment ◽  
Adrenocortical Carcinoma ◽  
Genetic Alterations ◽  
High Recurrence Rate ◽  
Self Renewal ◽  
Steroidogenic Cells ◽  
Rare Malignancy ◽  
Definition Of ◽  
Stem Cell Hierarchy

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence worldwide of 0.7–2.0 cases/million/year. Initial staging is the most important factor in determining prognosis. If diagnosed early, complete surgical resection +/− adjuvant treatment can lead to 5-year survival of up to 80%. However, often it is diagnosed late and in advanced disease, 5-year survival is <15% with a high recurrence rate even after radical surgery. The mainstay of adjuvant treatment is with the drug mitotane. Mitotane has a specific cytotoxic effect on steroidogenic cells of the adrenal cortex, but despite this, progression through treatment is common. Developments in genetic analysis in the form of next-generation sequencing, aided by bioinformatics, have enabled high-throughput molecular characterisation of these tumours. This, in addition to a better appreciation of the processes of physiological, homeostatic self-renewal of the adrenal cortex, has furthered our understanding of the pathogenesis of this malignancy. In this review, we have detailed the pathobiology and genetic alterations in adrenocortical carcinoma by integrating current understanding of homeostasis and self-renewal in the normal adrenal cortex with molecular profiling of tumours from recent genetic analyses. Improved understanding of the mechanisms involved in self-renewal and stem cell hierarchy in normal human adrenal cortices, together with the identification of cell populations likely to be co-opted by oncogenic mutations, will enable further progress in the definition of the molecular pathways involved in the pathogenesis of ACC. The combination of these advances eventually will lead to the development of novel, effective and personalised strategies to eradicate molecularly annotated ACCs.

scholarly journals Adrenocortical Carcinoma Discovered with Point-of-care Ultrasound

2021 ◽  
Vol 5 (4) ◽  
pp. 482-484
Author(s):  
Mark McIntyre ◽  
Michael Prats
Keyword(s):  
Lower Extremity ◽  
Adrenocortical Carcinoma ◽  
Point Of Care ◽  
Cystic Mass ◽  
Obstructive Nephropathy ◽  
Point Of Care Ultrasound ◽  
Lower Extremity Edema ◽  
Important Diagnostic Tool ◽  
Rare Malignancy ◽  
The Right

Case Presentation: A 34-year-old woman presented to the emergency department with bilateral lower extremity edema and shortness of breath. She had been seen by her primary care provider. Lab work and a follow-up with endocrinology had been unrevealing. Using point-of-care ultrasound we identified a cystic mass in the right upper quadrant prompting further imaging. Discussion: Abdominal and pelvic computed tomography confirmed a mass in the right posterior liver, which was later identified as an adrenocortical carcinoma. Ultrasound is an important diagnostic tool in the setting of lower extremity edema and can be used to assess for heart failure, liver failure, obstructive nephropathy, venous thrombosis, and soft tissue infection. In this case, ultrasound helped expedite the diagnosis and treatment of a rare malignancy.

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