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Everything you need to know about Computed Tomography (CT) & CT Scanning

Adrenal: Primary Adrenal Carcinoma Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Adrenal ❯ Primary Adrenal Carcinoma

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  • “Contemporary ACC predominantly presents as an incidental imaging finding, characterized by HU > 20 on nonenhanced CT but variable tumour size (20–196 mm). Malignancy cannot be ruled out by small tumour size only. The 5- year survival of 96% in ENSAT stage I–III compares favourably to previous studies.”
    Adrenocortical carcinoma: presentation and outcome of a contemporary patient series
    Kostiainen I et al.
    Endocrine (2019) 65:166–174
  • “Previously, 10–15% of ACCs have presented as incidental imaging findings, while reported prevalence of ACC among adrenal incidentalomas has varied between 1–11%. However, incidentally discovered asymptomatic adrenal masses are getting more common due to increasing use of imaging. We hypothesized that presentation and outcome of ACC may differ in modern compared to old series. We here report data on presentation, treatment and outcome in a contemporary series, including all patients from a single centre diagnosed in 2002 to 2018, and nationwide incidence of ACC in 2001–2015.”
    Adrenocortical carcinoma: presentation and outcome of a contemporary patient series
    Iiro Kostiainen I et al.
    Endocrine (2019) 65:166–174
  • “In conclusion, contemporary ACC predominantly presents as an incidental imaging finding, characterized by HU > 20 on nonenhanced CT but variable tumour size (20–196 mm). Malignancy cannot be ruled out by small tumour size only.”
    Adrenocortical carcinoma: presentation and outcome of a contemporary patient series
    Kostiainen I et al.
    Endocrine (2019) 65:166–174
  • “Adrenocortical carcinoma (ACC) is a rare, aggressive tumor arising from the adrenal cortex that typically presents late with a large mass. The increased use of cross-sectional imaging for unrelated reasons has led to a greater number of ACCs being detected incidentally at an earlier stage. Recognition of the typical clinical, biochemical, and imaging findings is imperative for rapid diagnosis, prompt intervention, and early use of the appropriate therapy. Cross-sectional imaging with CT and MRI is essential for determining the extent of local and distant tumor spread.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • “ACCs account for only 0.05–0.2% of all cancers or 1–2 patients per 1 million population per year. The age distribution of the affected population is bimodal, with an increased incidence in infants and children younger than 5 years old and in individuals in their fourth and fifth decades of life . A female preponderance has been noted, and women are more likely than men to present with more well-differentiated tumors that tend to be functional.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • Syndromes Associated with Primary Adrenal Carcinoma
    - Li-Fraumeni Cancer Syndrome: Li-Fraumeni cancer syndrome results in a familial susceptibility to a variety of cancers including adrenocortical tumors (carcinomas, adenomas), sarcomas, leukemias, breast, brain, lung, and laryngeal cancers because of a germline TP53 mutation.
    - Carney Complex: Carney complex consists of primary pigmented nodular adrenal dysplasia, cardiac myxomas, cutaneous myxomas, testicular tumors, and other en- docrine neoplasms.
    - Beckwith-Wiedemann syndrome is a congenital disorder characterized by pre- and postnatal overgrowth, macroglossia, and anterior abdominal wall defects (most commonly exomphalos).
  • Syndromes Associated with Primary Adrenal Carcinoma
    - Familial Adenomatous Polyposis Coli: Familial adenomatous polyposis coli causes multiple adenomatous polyps and cancer of the colon and rectum, thyroid tumors, hepatoblastoma, and adrenocortical tumors (carcinomas, adenomas).
    - Multiple Endocrine Neoplasia, Type 1: Multiple endocrine neoplasia, type 1, causes pituitary, parathyroid, and pancreatic tumors; adrenocortical adenomas or hyperplasia; and, very rarely, adrenocortical carcinomas.
  • “ACCs are functional in approximately 60% of cases ,more commonly in children (? 85%) than in adults (15–30%). Unlike adrenal adenomas that predominantly secrete cortisol, ACCs secrete a variety of hormones including androgens, cortisol, estrogens, and aldosterone. In adult patients with functioning tumors, 30% present with Cushing syndrome, 20% with virilization, and 10–20% with a combination of the two. Feminization and hyperaldosteronism are much rarer, each accounting for approximately 2% of ACC cases.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • “Approximately 65–85% of ACCs in adults are nonfunctioning, and patients present with a large mass and symptoms related to mass effect (e.g., abdominal or flank pain in 55%) [7] or with a palpable mass (40–50%) [1, 7]. Some ACCs are discovered incidentally (0–25%) when they tend to be smaller.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • “Calcification, either microcalcification or coarse calcification, is seen on CT in approximately 30% of patients with ACC and is usually centrally located. Calcification is rare in adenomas, although it is present in approximately 10% of pheochromocytomas.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • “Metastases are frequently found at presentation: Regional and paraaortic lymph nodes (25–46%), lungs (45–97%), liver (48–96%), and bone (11–33%) are the common sites. Hepatic metastases tend to be hypervascular and are best seen on arterial phase imaging after IV contrast administration. 10% of pheochromocytomas.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • “FDG PET can identify some malignant adrenal masses by virtue of their increased metabolic activity; however, when FDG uptake is only modest, the likelihood of benign versus malignant is about equal. FDG PET combined with contrast-enhanced CT has a sensitivity of 100% and specificity of 87–97% for identifying malignant adrenal masses. The lower specificity is because a small number of adenomas and other benign lesions mimic malignancy.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • “Patients with unresectable stage IV ACC have a median survival of 3 months. When treated aggressively with surgery, patients with stage I and II tumors have an approximately 65% 5-year survival, whereas patients with stage III and IV disease have 40% and 10% 5-year survival, respectively . The overall 5-year survival rate for all patients with ACC is 38%. Recurrence and metastatic lesions mimic malignancy.”
    Adrenocortical Carcinoma: The Range of Appearances on CT and MRI
    Nishat Bharwani et al.
    AJR 2011; 196:W706–W714
  • Primary Adrenal Carcinoma: Differential Diagnosis
    - Large adenoma
    - Pheochromocytoma
    - Lymphoma
    - Metastases
    - Ganglioneuroma
    - Neuroblastoma
  • The causes of Conn syndrome and Cushing syndrome in the adrenal glands
    Cushings Syndrome
    - Adrenal adenoma
    - Adrenocortical carcinoma
    - Pheochromocytoma
    - Primary pigmented nodular adrenocortical disease (PPNAD)
    - Macronodular hyperplasia with marked adrenal enlargement (MHMAE)
  • The causes of Conn syndrome and Cushing syndrome in the adrenal glands
    Conn Syndrome
    - Adrenal adenoma (50-60%)
    - Adrenocortical carcinoma (1%)
    - Adrenal hyperplasia (bilateral idiopathic hyperplasia, primary adrenal hyperplasia)
  • “CS or hypercortisolism is classically described as the signs and symptoms associated with prolonged exposure to pathologically elevated cortisol levels, which represents hypercortisolism stemming from various causes other than a pituitary adenoma. CS can result from exogenous administration of glucocorticoids or endogenous overproduction of cortisol. The triggering pathways of CS can be divided into pituitary-dependent and pituitary-independent. Females are more likely to have CS than males. CS is most commonly caused by adrenal adenomas; other causes include adrenocortical carcinoma (ACC), pheochromocytomas.”
    CT and MRI of adrenal gland pathologies
    Fuqin Wang et al.
    Quant Imaging Med Surg. 2018 Sep; 8(8): 853–875.
  • “Conn syndrome, or primary aldosteronism, which is characterized by excessive spontaneous secretion of aldosterone from the adrenal glands, affects 6% of people with high blood pressure and can be either sporadic or familial. Conn syndrome is related to autonomous aldosterone production causing sodium retention, plasma renin suppression, hypertension, cardiovascular damage, and increased potassium excretion, leading to variable degrees of hypokalemia.”
    CT and MRI of adrenal gland pathologies
    Fuqin Wang et al.
    Quant Imaging Med Surg. 2018 Sep; 8(8): 853–875.
  • "Addison disease, or primary adrenal insufficiency, is a systemic disease caused by hypoadrenocortical hypofunction. Addison disease has many causes, the most common of which is autoimmune adrenalitis, and other causes include tuberculosis, malignant tumor, infection, hemorrhage, HIV and certain genetic conditions. The incidence of Addison disease is 0.6/100,000 of population per year. Patients may experience weight loss, weakness, fatigue, gastrointestinal upset, orthostatic hypotension and pigmentation of skin. Dehydration, shock, hyperkalemia, and hyponatremia occurred in patients with adrenal crisis.”
    CT and MRI of adrenal gland pathologies
    Fuqin Wang et al.
    Quant Imaging Med Surg. 2018 Sep; 8(8): 853–875.
  • Addisons Disease: Causes
    - autoimmune adrenalitis
    - tuberculosis
    - malignant tumor
    - infection,
    - hemorrhage,
    - HIV certain genetic conditions.
  • "Tumors were inhomogeneous at nonenhanced CT, particularly masses larger than 6 cm, owing to the presence of necrosis. After contrast material infusion, adrenocortical carcinoma enhances heterogeneously, often peripherally, with a thin rim of enhancing capsule seen in some cases. Studies of lesion washout have reported that adrenocortical carcinoma typically has an RPW of less than 40%, with specific measures reported in Table 4 .However, the large size and heterogeneity are more reliable indicators of the diagnosis than are washout values, which vary depending on which part of the mass is sampled. In 19%–33% of cases, calcifi- cations have been identified, more commonly microcalcifications.”
    Adrenal Mass Imaging with Multidetector CT: Pathologic Conditions, Pearls, and Pitfalls
    Pamela T. Johnson, MD • Karen M. Horton, MD • Elliot K. Fishman MD
    RadioGraphics 2009; 29:1333–135
  • “Invasion of the IVC is a well-known complication of adrenocortical carcinoma. Patients with IVC involvement may present with hormonal syndromes, constitutional symptoms, abdominal pain, lower extremity edema, or pulmonary embolism. In one series of 15 adrenocortical carcinomas that invaded the IVC, 12 were on the right side. Also presented in that article was a summary of the literature, which revealed that the highest level of venous extension in 51% of patients was the suprahepatic IVC among those for whom this information was provided. When masses arising in the region.”
    Adrenal Mass Imaging with Multidetector CT: Pathologic Conditions, Pearls, and Pitfalls
    Pamela T. Johnson, MD • Karen M. Horton, MD • Elliot K. Fishman MD
    RadioGraphics 2009; 29:1333–135
  • “Cortical carcinoma is an uncommon malignant adrenal tumor, with a bimodal peak of incidence in childhood and middle age. For unclear reasons, it may be functioning especially in females, who also show more frequently an association with endocrine syndromes including Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Carney complex, congenital adrenal hyperplasia, and MEN-1. Non-functioning lesions are asymptomatic as long as they become so large to determine compression and dislocation of adjacent structures.”
    Imaging features of adrenal masses
    Albano D et al.
    Insights into Imaging (2019) 10:1
  • “On CT, adrenal carcinoma shows a heterogeneous appearance due to necrosis, calcifications, and hemorrhage. After intravenous contrast injection, it demonstrates heterogeneous and mainly peripheral enhancement [66]. As previously reported, the RPW of the carcinoma tends to be less than 40%. Invasion of adjacent structures such as kidney, inferior vena cava, and splenic vessels are common as well as liver metastasis and retroperitoneal lymph nodal locations.”
    Imaging features of adrenal masses
    Albano D et al.
    Insights into Imaging (2019) 10:1
  • “ACC is a rare, highly malignant tumor with a reported annual incidence of 1 cases per million in the united states according to the Surveillance, Epidemiology, and End Results- registry (SEER) database for ACC cases identified from 1974 to 2014. The previous reported annual incidence was 0.5–2 cases per million. Females are affected more commonly than males. The reported female-to-male ratio is 1.34:1, and most cases are unilateral. A bimodal age distribution has been described for ACC with one peak occurring in the first decade and a second peak later in the fifth decade of life. However, this bimodal presentation has been questioned with a recent study suggesting a predominantly unimodal distribution with a median age of diagnosis of 55 years.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • Primary Adrenal Carcinoma: Presentation
    - Hormonal abnormality (i.e. Cushings)(40-60%)
    - Flank or back pain (30%)
    - Incidental finding on CT (10-15%)
  • “The most common clinical presentation for functioning ACC is Cushing’s syndrome, characterized by symptoms related to excess corticosteroid synthesis including truncal obesity, diabetes, hypertension, easy bruising, and menstrual cycle irregularities. In females, virilization resulting from excess androgens may accompany signs of excess cortisol. A small percentage of male patients, present with signs of estrogen excess, such as gynecomastia, breast tenderness, decreased libido, and testicular atrophy.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “Although the majority of ACCs develop sporadically, many cases arise in association with various familial cancer syndromes including Li–Fraumeni, Beckwith–Wiedemann, and Lynch syndromes. Several genetic alterations have been noted to play an important role in the pathogenesis of ACC. Most of the drivers for the pathogenesis of ACC are related to mutations or downregu- lation of tumor suppressor genes, overexpression of certain growth factors, chromosomal aberrations, and dysregulation of certain important signaling pathways.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “Ki67 is among the most important markers in differentiating benign from malignant adrenal lesions. Lesions with Ki67 index greater than 5% are likely to be malignant. Moreover, Ki67 positivity is considered the single most important predictor for local recurrence after complete R0 surgical resection of ACC. Tumors with less than 10% Ki67 have significantly better clinical outcomes than those with greater than 10% Ki67.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “ACCs are typically large, with roughly 70% of tumors larger than 6 cm at the time of diagnosis. Size of the adrenal tumor, pattern of contrast enhancement, and degree of heter- ogeneity by CT are all important predictors of the malignant potential of the adrenal lesion. ACC is typically heterogeneous by CT and displays mixed intratumoral attenuation. An attenuation value of more than 10 HU on non-contrast CT has high sensitivity for detecting malignancy (93%), but a specificity of only 71–73%. Contrast enhancement is heterogeneous and may be increased peripherally due to central necrosis. ACC characteristically displays less washout of contrast than benign adrenal adenomas (absolute washout < 60% and relative washout < 40%). However, the use of adrenal washout charac- teristics should be reserved for homogenous well-defined masses.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “ACCs are typically large, with roughly 70% of tumors larger than 6 cm at the time of diagnosis. Size of the adrenal tumor, pattern of contrast enhancement, and degree of heterogeneity by CT are all important predictors of the malignant potential of the adrenal lesion. ACC is typically heterogeneous by CT and displays mixed intratumoral attenuation. An attenuation value of more than 10 HU on non-contrast CT has high sensitivity for detecting malignancy (93%), but a specificity of only 71–73%. Contrast enhancement is heterogeneous and may be increased peripherally due to central necrosis.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “An irregular tumor margin is a sign of aggressiveness; however, its absence is not a reliable sign of benignity. A thin rim of well-defined enhancement commonly detected around ACC likely represents the tumor capsule. Invasion of periadrenal fat and the surrounding organs or vasculature are other specific features of malignancy. IVC invasion is common at the time of diagnosis, and therefore, it is recommended that CT images extend to the level of the right atrium to exclude right atrial thrombus. Metastases are also relatively common at time of presentation, and the most common sites are in the liver, lung, bone, and retroperitoneal lymph nodes.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “An irregular tumor margin is a sign of aggressiveness; however, its absence is not a reliable sign of benignity. A thin rim of well-defined enhancement commonly detected around ACC likely represents the tumor capsule. Invasion of periadrenal fat and the surrounding organs or vasculature are other specific features of malignancy. IVC invasion is common at the time of diagnosis, and therefore, it is recommended that CT images extend to the level of the right atrium to exclude right atrial thrombus.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “Although the presence of macroscopic fat is most commonly seen in benign myelolipoma, ACC may show areas of macroscopic fat as well. Other imaging and biochemical features should be kept in consideration to differentiate this rare manifestation of ACC from myelolipoma, as previously discussed. With any adrenal lesion, the appropriate workup depends on a variety of factors. Incorporating clinical, imaging, and biochemical data allow the formulation of a proposed systematic approach.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • Mimics of Adrenal Carcinoma
    - Pheochromocytoma
    - Adrenal adenoma
    - Adrenal metastases
    - Adrenal lymphoma
    - Ganglioneuroma and ganglioneuroblastoma
    - Adrenal hemorrhage and pseudocyst
    - Adrenal hemangioma
  • "Due to the high recurrence rate of ACC, close follow-up is recommended even after complete resection. Follow-up with abdominal CT or MRI and CT of the chest every 3 months for the first 2 years is recommended. Additional monitoring of steroid hormones level is essential, especially in hormonally active tumors to detect early recurrence. After 2 years of follow-up, the interval may be increased to every 6 months for 5 years. Approximately 90% of cases recur during the first five years after resection, so there is no clear recommendation for patient surveillance after 5 years. Annual follow-up for another 5 years might be adapted according to the clinical situation and the judgment of the physician.”
    Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management
    Ayahallah A. Ahmed et al.
    Abdominal Radiology 2020 (in press)
  • “The proven low rates of adrenocortical cancer in lesions smaller than 6 cm and the ever-increasing ability to characterize incidentalomas radiographically should give clinicians pause to reconsider whether resecting most lesions larger than 4 cm is warranted. However, it remains true that risk of adrenocortical malignancy significantly increases for lesions larger than or equal to 6 cm. Unless the radiographic signature is unquestionably benign, such as is the case with myelolipoma and cyst, these lesions still warrant resection.”

    Radiographic Evaluation of Nonfunctioning Adrenal Neoplasms
    Mazzaglia PJ
    Surg Clin N Am 94 (2014) 625–642
  • “Although it is established that 5% to 20% of adrenal incidentalomas will show slow growth of 1 cm or more over an average of 4 years, these are almost always benign Also, the advances made in imaging quality and evaluation enable involved clinicians to be more confident in their assessments of the likelihood of benignity for lesions in the 4 to 6 cm range. Therefore, a reasonable approach to lesions smaller than 4 cm that are nonfunctional and clearly meet imaging criteria of a benign lipid-rich adenoma is to not perform further imaging,as well as limit repeat biochemical evaluation to patients who are hypertensive or develop symptoms and signs associated with cortisol, catecholamine, or aldosterone excess."

    Radiographic Evaluation of Nonfunctioning Adrenal Neoplasms
    Mazzaglia PJ
    Surg Clin N Am 94 (2014) 625–642 
  • “Lesions between 4 and 6 cm that carry slightly higher malignancy rates should be excised if imaging cannot confidently characterize them as benign. However, if imaging suggests a lipid-rich adenoma, a nonoperative approach may be pursued and repeat adrenal imaging may be obtained in 6 to 12 months. Lesions larger than or equal to 6 cm should be resected unless there is unequivocal imaging evidence of a benign nature. If radiographic features are suggestive of possible adrenocortical cancer, strong consideration should be given to open adrenalectomy instead of an open approach.”

    Radiographic Evaluation of Nonfunctioning Adrenal Neoplasms
    Mazzaglia PJ
    Surg Clin N Am 94 (2014) 625–642 
  • Adrenocortical Carcinoma
    1. 1st and 4th decades of life
    2. 55% are functional
    - Cushing syndrome
    - Feminization
    - Virilization
    - Mixed Cushing/virilization
    3. Hypertension common with functional
  • Adrenocortical Carcinoma
    1. Mean 10 cm…up to 25 cm
    - larger less likely to be functional
    2. Inhomogeneous precontrast
    3. Heterogeneous enhancement
    4. Presence of necrosis
    5. Calcification in minority
  • Adrenocortical Carcinoma
    1. Compression of adjacent organs
    2. IVC invasion
    - Adrenocortical carcinoma
    - Renal cell carcinoma
    - Hepatocellular carcinoma
  • Adrenocortical Carcinoma
    -Mean 10 cm…up to 25 cm
    -larger less likely to be functional
    -Inhomogeneous precontrast
    -Heterogeneous enhancement
    -Presence of necrosis
    -Calcification in minority
  • Adrenocortical Carcinoma

    Compression of adjacent organs

    IVC invasion
    -Adrenocortical carcinoma
    -Renal cell carcinoma
    -Hepatocellular carcinoma
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