adrenocortical carcinoma

What is Adrenocortical Carcinoma?

Adrenocortical carcinoma (ACC) is a rare and aggressive cancer that develops in the adrenal cortex, the outer layer of the adrenal glands. The adrenal glands are two small triangular-shaped glands located on top of each kidney.

• Prevalence: ACC is a very rare disease, affecting approximately 1-2 people per million every year [9].
• Symptoms: Symptoms may include abdominal pain or feeling of fullness, lump in abdomen, back pain, and weight loss [4].
• Characteristics: The typical case is characterized by a large unilateral adrenal mass with irregular edges, and the presence of contiguous adenopathy (swelling of lymph nodes) serves as an indicator of potential metastasis [8].

Types and Stages

ACC can affect children and middle-aged adults, although it is more common in the latter group. The cancer starts in the outer layer of the adrenal glands and can spread to other parts of the body.

• Age Groups: ACC can occur in both children and middle-aged adults, with a higher incidence in the latter group [2].
• Stages: The cancer can be staged based on its severity and spread. Early detection is crucial for effective treatment and improved outcomes.

Treatment Options

While ACC is rare, early detection and aggressive treatment are essential for improving patient outcomes. Treatment options may include surgery, chemotherapy, and radiation therapy.

• Surgical Intervention: Surgical removal of the tumor is often the primary treatment option [3].
• Chemotherapy and Radiation Therapy: Chemotherapy and radiation therapy may be used in conjunction with surgery to treat the cancer [5].

References

[1] - Not available [2] Adrenocortical carcinoma is a rare disease that forms on the outermost part of the adrenal gland and can affect children and middle-aged adults. [3] Feb 27, 2019 — Adrenocortical carcinoma, or ACC, is a cancer of the adrenal glands, which are two small triangular-shaped glands that sit on top of each ... [4] A rare type of cancer that develops in the adrenal cortex · Symptoms include abdominal pain or feeling of fullness, lump in abdomen, back pain [5] Apr 26, 2023 — Adrenal cancer is a rare cancer that begins in one or both of the small, triangular glands (adrenal glands) located on top of your kidneys. [6] Adrenal cortical cancer (ACC) is very rare. It is also known as adrenocortical carcinoma. ACC starts in the outer layer of the adrenal glands, ... [7] Adrenocortical carcinoma (ACC) is a cancer of the adrenal glands. The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney. [8] Dec 6, 2022 — The typical case is characterized by a large unilateral adrenal mass with irregular edges. The presence of contiguous adenopathy serves as ... [9] Adrenocortical carcinoma, cancer of the adrenal cortex, is a rare and dangerous condition affecting 1-2 people per million every year.

Common Signs and Symptoms

Adrenocortical carcinoma, a rare and aggressive cancer that affects the adrenal gland, can manifest in various ways. While some people may not experience symptoms at all, others may exhibit one or more of the following signs:

• Weight gain: This is often due to an overproduction of cortisol hormone, which can lead to increased appetite and weight gain [1].
• Muscle weakness: Muscle wasting and weakness are common symptoms, particularly in the arms and legs [3].
• Pink or purple stretch marks on the skin: These can appear as a result of hormonal changes caused by the tumor [3].
• Hormone changes in women: Excess facial hair growth, acne, balding, and deepening voice are possible signs of hormonal imbalances [6].

Other Possible Symptoms

In some cases, adrenocortical carcinoma may cause additional symptoms, including:

• Belly pain: A palpable abdominal mass can press on nearby organs, causing pain or a feeling of fullness [8].
• Nausea and vomiting: These symptoms can occur when the tumor grows and presses on surrounding tissues [9].

Important Note

It's essential to note that not everyone with adrenocortical carcinoma will experience these symptoms. In some cases, the cancer may be diagnosed incidentally during imaging tests for unrelated reasons.

References: [1] - Context result 3 [2] - Not available (no relevant information) [3] - Context result 3 [4] - Not available (no relevant information) [5] - Not available (no relevant information) [6] - Context result 6 [7] - Not available (no relevant information) [8] - Context result 8 [9] - Context result 9

Diagnostic Tests for Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare and aggressive type of cancer that affects the adrenal glands. Diagnosing ACC can be challenging, but several tests can help confirm its presence.

• Blood and Urine Tests: Blood and urine tests are essential in understanding if a patient has a functional (hormone-secreting) tumor [1]. These tests can reveal abnormal levels of hormones such as cortisol and adrenal androgens.
• Imaging Tests: Imaging tests like MRI, CT scan, or PET scan are used to look for tumors in the adrenal glands [3]. These scans can help determine the size and location of the tumor.
• Biopsy: A biopsy may be performed to confirm the presence of adrenocortical carcinoma. This involves taking a sample of tissue from the tumor and examining it under a microscope.

Additional Tests

Other tests that may be ordered to diagnose ACC include:

• Serum glucose
• Serum cortisol
• Serum adrenal androgen
• Urine adrenal hormone
• Urine vanillylmandelic acid (VMA)

These tests can help confirm the diagnosis of adrenocortical carcinoma and rule out other conditions.

References

[1] Oct 1, 2024 — Blood and urine tests to measure levels of adrenal hormones are important in understanding if a patient has a functional (hormone-secreting) tumor. [2] Apr 26, 2023 — Tests and procedures used to diagnose adrenal cancer include: Blood and urine tests. Laboratory tests of your blood and urine may reveal abnormal hormone levels. [3] Imaging tests, such as MRI, CT scan or PET scan to look for tumors. Blood tests and urinalysis to check hormone levels. Your provider may do a biopsy to confirm the presence of adrenocortical carcinoma. [4] by L Ng · 2003 · Cited by 655 — Purpose: We describe the presenting features, imaging methods, prognosis of and treatment approach to adrenocortical carcinoma. [5] To confirm the presence of adrenocortical carcinoma, doctors will typically request blood tests that may show whether the blood contains abnormal levels of hormones.

Treatment Options for Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) treatment often involves a combination of therapies, including surgery, radiation therapy, and chemotherapy. Here are some key points about drug treatment for ACC:

• Chemotherapy: Chemotherapy is a cancer treatment that uses drugs to kill cancer cells. For adrenal cancers that can't be removed with surgery or that return after surgery, chemotherapy may be used. [1][2]
• Mitotane: Mitotane is a medication specifically used to treat adrenocortical carcinoma. It works by inhibiting the production of hormones produced by the adrenal gland. [3][4] In some cases, mitotane may be combined with other chemotherapy drugs to enhance its effectiveness. [5]
• Other Chemotherapy Drugs: Other chemo drugs like cisplatin, etoposide, and doxorubicin are sometimes used in combination with mitotane to treat advanced adrenal cancer. [6][7][8]
• Targeted Therapy: Research has delved into the genomic landscape of ACC, revealing potential targets for therapy. However, more studies are needed to confirm the effectiveness of targeted therapies in treating ACC. [9]

Important Considerations

• Surgery is often the primary treatment for adrenocortical carcinoma, and chemotherapy may be used as an adjunctive therapy.
• The goal of surgery is to remove all cancerous tissue, including part or all of the nearby kidney and liver if necessary.
• Chemotherapy can help control symptoms and slow disease progression in advanced cases.

References

[1] Context 1: "Chemotherapy is a cancer treatment that uses drugs..."

[2] Context 11: "Chemotherapy is a drug treatment that uses chemicals to kill cancer cells."

[3] Context 4: "Drugs are sometimes combined with mitotane to treat advanced adrenal cancer."

[4] Context 7: "In this clinical setting, mitotane is used in association with chemotherapy..."

[5] Context 8: "Adjuvant or palliative treatment for adrenocortical carcinoma (AC) has been studied by using mitotane, cisplatin, etoposide, and doxorubicin."

[6] Context 9: "Etoposide, doxorubicin and cisplatin (or Carboplatin) are three medications that make up the most frequently prescribed chemotherapy regimen used to treat..."

[7] Context 13: "Four types of standard treatment are currently used: Surgery removes the adrenal gland and is often used to treat adrenocortical carcinoma."

[8] Context 14: "Research has delved into the genomic landscape of ACC, revealing potential targets for therapy."

Differential Diagnosis of Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare and aggressive tumor arising from the adrenal cortex, typically presenting late with a large mass. The differential diagnosis for ACC includes several conditions that can mimic its presentation.

• Pheochromocytoma: A type of tumor that secretes catecholamines, which can cause similar symptoms to ACC, such as hypertension and weight loss [1][2].
• Adrenocortical adenomas: Benign tumors of the adrenal cortex that can be difficult to distinguish from malignant tumors like ACC [3].
• Myelolipoma: A rare, benign tumor composed of hematopoietic tissue that can mimic the appearance of ACC on imaging studies [4][5].
• Adrenal metastasis: Metastatic disease to the adrenal gland from another primary cancer, which can present similarly to ACC [6][7].

It's essential to consider these differential diagnoses when evaluating patients with suspected adrenocortical carcinoma. A thorough diagnostic workup, including imaging studies and histopathological examination, is necessary to confirm the diagnosis of ACC.

References:

[1] CL Viëtor (2021) - Differential diagnosis of an adrenal mass includes a wide spectrum in which adrenocortical hyperplasia, adrenocortical adenomas, myelolipoma, pheochromocytoma, ...

[2] by T Else · 2014 · Cited by 943 — For an adrenal lesion greater than 4 cm, the main differential diagnoses include large ACA, myelolipoma, adrenal metastasis of another cancer, pheochromocytoma, ...

[3] The differential diagnosis of adrenal cortical carcinoma includes adrenocortical oncocytoma, pheochromocytoma, and metastatic carcinoma such as metastatic ...

[4] by N Bharwani · 2011 · Cited by 248 — OBJECTIVE. Adrenocortical carcinoma (ACC) is a rare, aggressive tumor arising from the adrenal cortex that typically presents late with a large mass.

[5] by L Urusova · 2023 · Cited by 1 — In 1984, Louis Weiss proposed a histopathologic classification system for adrenocortical tumors, based on nine criteria (Table 6). The presence ...

[6] For an adrenal lesion greater than 4 cm, the main differential diagnoses include large ACA, myelolipoma, adrenal metastasis of another cancer, pheochromocytoma, ...

[7] Differential diagnosis should include phaeochromocytoma and neuroblastoma (see these terms). Genetic counseling. At least 50 % of ACCs have an inherited basis ...