ICD-10: C74.0

Malignant neoplasm of cortex of adrenal gland

Additional Information

Description

The ICD-10 code C74.0 refers specifically to a malignant neoplasm of the cortex of the adrenal gland. This classification is part of the broader category of adrenal cancers, which can significantly impact patient health and treatment options. Below is a detailed overview of this condition, including its clinical description, incidence, and relevant coding information.

Clinical Description

Definition

C74.0 denotes a malignant tumor originating in the adrenal cortex, which is the outer layer of the adrenal glands located atop each kidney. These tumors can be classified as adrenal cortical carcinoma, which is a rare but aggressive form of cancer that can lead to various systemic effects due to hormone overproduction or local tumor growth.

Symptoms

Patients with adrenal cortical carcinoma may present with a range of symptoms, including:
- Hormonal Imbalances: Overproduction of hormones such as cortisol, aldosterone, or androgens can lead to conditions like Cushing's syndrome, Conn's syndrome, or virilization, respectively.
- Abdominal Pain: As the tumor grows, it may cause discomfort or pain in the abdominal area.
- Weight Changes: Unexplained weight gain or loss can occur, often related to hormonal changes.
- Fatigue and Weakness: General malaise and decreased energy levels are common complaints.

Diagnosis

Diagnosis typically involves imaging studies such as CT scans or MRIs to visualize the adrenal glands, along with biochemical tests to assess hormone levels. A biopsy may be performed to confirm malignancy.

Incidence and Mortality

Epidemiology

Adrenal cortical carcinoma is rare, with an estimated incidence of 0.5 to 2 cases per million people annually. It is more commonly diagnosed in adults, with a slight female predominance. The prognosis can vary significantly based on the stage at diagnosis, with early detection leading to better outcomes.

Mortality Rates

The mortality associated with adrenal cortical carcinoma is high, particularly if diagnosed at an advanced stage. The five-year survival rate can be as low as 15-30%, emphasizing the importance of early detection and intervention.

Coding Information

ICD-10-CM Code

The specific code C74.0 is used for billing and coding purposes in healthcare settings. It is essential for accurately documenting the diagnosis in medical records and for insurance reimbursement processes. The broader category C74 includes other types of adrenal neoplasms, but C74.0 specifically targets malignant tumors of the adrenal cortex.

  • C74.1: Malignant neoplasm of medulla of adrenal gland
  • C74.9: Malignant neoplasm of adrenal gland, unspecified

Importance of Accurate Coding

Accurate coding is crucial for effective patient management, research, and healthcare statistics. It helps in tracking the incidence of adrenal cancers and facilitates the allocation of resources for treatment and research.

Conclusion

The ICD-10 code C74.0 represents a significant health concern due to the aggressive nature of adrenal cortical carcinoma and its potential for severe systemic effects. Understanding the clinical presentation, incidence, and coding details is vital for healthcare professionals involved in the diagnosis and treatment of this condition. Early detection and appropriate management strategies are essential to improve patient outcomes and survival rates.

Clinical Information

The ICD-10 code C74.0 refers to a malignant neoplasm of the cortex of the adrenal gland, specifically indicating adrenal cortical carcinoma (ACC). This type of cancer arises from the adrenal cortex, which is responsible for producing hormones such as cortisol, aldosterone, and androgens. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for timely diagnosis and management.

Clinical Presentation

Adrenal cortical carcinoma often presents with a variety of symptoms that can be attributed to the tumor itself or the hormonal imbalances it causes. The clinical presentation may vary significantly among patients, but common features include:

  • Hormonal Symptoms: Depending on the type of hormones produced by the tumor, patients may experience symptoms related to excess hormone secretion. For instance:
  • Cushing's Syndrome: Caused by excess cortisol, leading to weight gain, hypertension, diabetes, and characteristic changes in fat distribution (e.g., moon facies, buffalo hump) [1].
  • Conn's Syndrome: Resulting from excess aldosterone, leading to hypertension and hypokalemia [2].
  • Virilization: Due to excess androgens, which can cause hirsutism, menstrual irregularities, and other signs of masculinization in women [3].

  • Local Symptoms: As the tumor grows, it may cause abdominal pain or discomfort, a palpable mass in the abdomen, or symptoms related to local invasion of surrounding structures [4].

  • General Symptoms: Patients may also present with nonspecific symptoms such as fatigue, weight loss, and anorexia, which are common in many malignancies [5].

Signs and Symptoms

The signs and symptoms of adrenal cortical carcinoma can be categorized based on hormonal activity and local effects:

Hormonal Activity

  • Cushing's Syndrome:
  • Central obesity
  • Skin changes (e.g., easy bruising, striae)
  • Osteoporosis
  • Muscle weakness
  • Hyperaldosteronism:
  • Persistent hypertension
  • Muscle cramps or weakness due to low potassium levels
  • Androgen Excess:
  • Hirsutism in women
  • Acne
  • Irregular menstrual cycles

Local Effects

  • Abdominal Mass: A palpable mass may be detected during physical examination.
  • Pain: Patients may report abdominal or flank pain due to tumor growth or invasion into adjacent structures.
  • Metastatic Symptoms: In advanced cases, symptoms may arise from metastases to other organs, such as the lungs or liver, leading to respiratory symptoms or jaundice [6].

Patient Characteristics

Certain demographic and clinical characteristics are associated with adrenal cortical carcinoma:

  • Age: ACC can occur at any age but is most commonly diagnosed in adults between the ages of 40 and 60 years [7].
  • Gender: There is a slight female predominance in the incidence of adrenal cortical carcinoma [8].
  • Comorbidities: Patients may have underlying conditions such as obesity, hypertension, or diabetes, which can complicate the clinical picture and management [9].
  • Genetic Factors: Some cases of ACC are associated with genetic syndromes, such as Li-Fraumeni syndrome or Beckwith-Wiedemann syndrome, which may influence patient characteristics and family history [10].

Conclusion

Adrenal cortical carcinoma (ICD-10 code C74.0) presents with a diverse array of clinical features, primarily driven by hormonal imbalances and local tumor effects. Recognizing the signs and symptoms associated with this malignancy is essential for early diagnosis and effective treatment. Given the complexity of the condition, a multidisciplinary approach involving endocrinologists, oncologists, and surgeons is often necessary to optimize patient outcomes. Further research into the genetic and molecular underpinnings of ACC may also enhance understanding and lead to improved therapeutic strategies in the future.

Approximate Synonyms

The ICD-10 code C74.0 specifically refers to a malignant neoplasm located in the cortex of the adrenal gland. This classification is part of the broader category of adrenal cancers, and there are several alternative names and related terms that can be associated with this condition. Below is a detailed overview of these terms.

Alternative Names for C74.0

  1. Adrenal Cortical Carcinoma: This is the most common alternative name for malignant tumors of the adrenal cortex. It emphasizes the cancerous nature of the tumor originating from the adrenal cortex.

  2. Adrenal Cortex Cancer: A straightforward term that describes cancer specifically arising from the adrenal cortex.

  3. Cortical Adrenal Carcinoma: This term highlights the specific area of the adrenal gland affected, focusing on the cortex.

  4. Adrenal Gland Cancer: A more general term that can refer to any cancer of the adrenal gland, but in the context of C74.0, it specifically pertains to malignancies of the cortex.

  5. Cortical Neoplasm: While this term can refer to any neoplasm in the cortex, when used in the context of the adrenal gland, it typically indicates a malignant growth.

  1. Adrenal Tumor: This term encompasses both benign and malignant tumors of the adrenal gland, but when discussing C74.0, it specifically refers to malignant tumors.

  2. Endocrine Tumor: Since the adrenal glands are part of the endocrine system, tumors in this area can be classified under endocrine tumors, although this term is broader and includes various types of tumors.

  3. Neoplasm of the Adrenal Gland: A general term that can refer to any neoplasm (benign or malignant) in the adrenal gland, but in the context of C74.0, it refers to malignant neoplasms.

  4. Adrenal Cortical Neoplasm: This term can refer to both benign and malignant neoplasms of the adrenal cortex, but in the context of C74.0, it is understood to refer to malignant cases.

  5. C74 Malignant Neoplasm of Adrenal Gland: This is a broader classification that includes all malignant neoplasms of the adrenal gland, with C74.0 specifically denoting those in the cortex.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C74.0 is crucial for accurate diagnosis, treatment planning, and communication among healthcare professionals. These terms help clarify the specific nature of the malignancy and its location within the adrenal gland, which is essential for effective management and research into adrenal cancers. If you have further questions or need more specific information, feel free to ask!

Diagnostic Criteria

The diagnosis of malignant neoplasm of the cortex of the adrenal gland, classified under ICD-10 code C74.0, involves a combination of clinical evaluation, imaging studies, and histopathological examination. Here’s a detailed overview of the criteria typically used for diagnosis:

Clinical Evaluation

  1. Symptoms: Patients may present with various symptoms that can indicate adrenal gland dysfunction or malignancy. Common symptoms include:
    - Abdominal pain or discomfort
    - Unexplained weight loss
    - Hormonal imbalances leading to conditions such as Cushing's syndrome (excess cortisol) or Conn's syndrome (excess aldosterone) [5].

  2. Medical History: A thorough medical history is essential, including any previous endocrine disorders, family history of adrenal tumors, or genetic syndromes associated with adrenal cancer, such as Li-Fraumeni syndrome or multiple endocrine neoplasia (MEN) [6].

Imaging Studies

  1. CT Scan or MRI: Imaging techniques like computed tomography (CT) or magnetic resonance imaging (MRI) are crucial for visualizing the adrenal glands. These imaging modalities help in:
    - Identifying the size, shape, and characteristics of the adrenal mass.
    - Differentiating between benign and malignant lesions based on features such as irregular borders, calcifications, and enhancement patterns [6].

  2. Functional Imaging: In some cases, functional imaging studies, such as PET scans, may be utilized to assess metabolic activity and help distinguish malignant tumors from benign adenomas [6].

Histopathological Examination

  1. Biopsy: A definitive diagnosis often requires a biopsy of the adrenal mass. This can be performed via:
    - Fine-needle aspiration (FNA) or core needle biopsy, which allows for cytological or histological examination of the tissue.
    - Surgical resection may be necessary for larger tumors or when malignancy is suspected [5].

  2. Microscopic Analysis: Pathological examination of the biopsy specimen is critical. Key features indicating malignancy include:
    - High cellularity with atypical cells
    - Mitotic figures
    - Necrosis within the tumor [6].

  3. Immunohistochemistry: Additional tests may be performed to assess specific markers that can help confirm the diagnosis of adrenal cortical carcinoma, such as the expression of steroidogenic enzymes [6].

Conclusion

The diagnosis of malignant neoplasm of the cortex of the adrenal gland (ICD-10 code C74.0) is a multifaceted process that integrates clinical assessment, imaging studies, and histopathological evaluation. Each step is crucial in ensuring an accurate diagnosis, which is essential for determining the appropriate treatment strategy. If you have further questions or need more specific information, feel free to ask!

Treatment Guidelines

The ICD-10 code C74.0 refers to a malignant neoplasm of the cortex of the adrenal gland, commonly known as adrenocortical carcinoma (ACC). This rare but aggressive cancer arises from the adrenal cortex, which is responsible for producing hormones such as cortisol and aldosterone. The treatment approaches for this condition typically involve a combination of surgical, medical, and supportive therapies.

Standard Treatment Approaches

1. Surgical Intervention

Surgery is often the primary treatment for localized adrenocortical carcinoma. The main surgical options include:

  • Adrenalectomy: This is the surgical removal of the affected adrenal gland. If the tumor is confined to one gland and has not spread, a complete adrenalectomy is usually performed. In some cases, surrounding tissues may also be removed to ensure complete excision of the tumor[2][3].

  • Laparoscopic Surgery: For smaller tumors, minimally invasive laparoscopic techniques may be employed, which can lead to quicker recovery times and less postoperative pain compared to open surgery[3].

2. Medical Management

For patients with advanced or metastatic adrenocortical carcinoma, medical therapies are crucial. These may include:

  • Chemotherapy: The use of chemotherapeutic agents can be considered, especially in cases where the cancer has spread beyond the adrenal gland. Common regimens may include drugs like mitotane, which is specifically used for ACC, often in combination with other agents[2][4].

  • Targeted Therapy: Newer treatments targeting specific pathways involved in cancer growth are being explored. For instance, drugs that inhibit the mTOR pathway or angiogenesis may be beneficial in some cases[4].

  • Hormonal Therapy: Since ACC can produce excess hormones, managing these hormonal imbalances is essential. Medications may be used to control symptoms related to hormone overproduction, such as hypertension or hyperglycemia[3].

3. Radiation Therapy

Radiation therapy may be utilized in specific scenarios, particularly for patients who are not surgical candidates or for those with residual disease post-surgery. It can help reduce tumor size or manage symptoms related to metastasis[5].

4. Clinical Trials

Given the rarity of adrenocortical carcinoma, participation in clinical trials may be an option for patients. These trials often explore new treatment modalities, including novel chemotherapeutic agents, immunotherapy, and combination therapies that may offer additional benefits over standard treatments[4][5].

5. Supportive Care

Supportive care is vital in managing symptoms and improving the quality of life for patients with ACC. This may include:

  • Palliative Care: For advanced disease, palliative care focuses on symptom management and support for both patients and their families[3].

  • Nutritional Support: Patients may require dietary adjustments to manage side effects from treatments or hormonal imbalances[2].

Conclusion

The management of malignant neoplasms of the adrenal cortex, specifically adrenocortical carcinoma, involves a multidisciplinary approach tailored to the individual patient's disease stage and overall health. Surgical resection remains the cornerstone of treatment for localized disease, while advanced cases may necessitate a combination of chemotherapy, targeted therapies, and supportive care. Ongoing research and clinical trials continue to evolve the treatment landscape, offering hope for improved outcomes in this challenging malignancy.

Related Information

Description

  • Malignant neoplasm of adrenal cortex
  • Outer layer of adrenal glands affected
  • Rare but aggressive form of cancer
  • Hormone overproduction or local growth
  • Symptoms include hormonal imbalances and pain
  • Diagnosis involves imaging and biochemical tests
  • High mortality rate if diagnosed late

Clinical Information

  • Malignant neoplasm of adrenal cortex
  • Arises from adrenal cortical cells
  • Hormonal imbalances cause various symptoms
  • Excess cortisol causes Cushing's Syndrome
  • Excess aldosterone causes Conn's Syndrome
  • Excess androgens cause virilization in women
  • Abdominal pain or discomfort due to tumor growth
  • Palpable mass in abdomen
  • Nonspecific symptoms like fatigue and weight loss
  • Central obesity from Cushing's Syndrome
  • Skin changes from Cushing's Syndrome
  • Osteoporosis from Cushing's Syndrome
  • Muscle weakness from Cushing's Syndrome
  • Persistent hypertension from hyperaldosteronism
  • Low potassium levels from hyperaldosteronism
  • Hirsutism in women due to androgen excess
  • Acne from androgen excess
  • Irregular menstrual cycles from androgen excess
  • Abdominal or flank pain due to tumor growth
  • Metastatic symptoms from lung or liver metastases

Approximate Synonyms

  • Adrenal Cortical Carcinoma
  • Adrenal Cortex Cancer
  • Cortical Adrenal Carcinoma
  • Adrenal Gland Cancer
  • Cortical Neoplasm
  • Adrenal Tumor
  • Endocrine Tumor
  • Neoplasm of the Adrenal Gland
  • Adrenal Cortical Neoplasm
  • C74 Malignant Neoplasm of Adrenal Gland

Diagnostic Criteria

  • Abdominal pain or discomfort
  • Unexplained weight loss
  • Hormonal imbalances
  • Irregular borders on imaging
  • Calcifications on imaging
  • Enhancement patterns on imaging
  • High cellularity with atypical cells
  • Mitotic figures
  • Necrosis within the tumor

Treatment Guidelines

  • Surgical resection is primary treatment for localized disease
  • Adrenalectomy may be complete or partial
  • Laparoscopic surgery is minimally invasive option
  • Chemotherapy uses agents like mitotane in combination
  • Targeted therapy inhibits mTOR pathway or angiogenesis
  • Hormonal therapy controls hormone overproduction symptoms
  • Radiation therapy reduces tumor size or manages symptoms
  • Clinical trials explore new treatment modalities and combinations
  • Supportive care includes palliative care and nutritional support

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