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pulmonary large cell neuroendocrine carcinoma

Description

Pulmonary Large Cell Neuroendocrine Carcinoma (LCNEC): A Rare and Aggressive Lung Cancer

Pulmonary large cell neuroendocrine carcinoma (LCNEC) is a rare and aggressive subtype of lung cancer, accounting for approximately 3% of all lung cancers [13]. It is characterized by its large cells with neuroendocrine architecture, prominent nucleoli, high mitotic activity, and necrosis [6].

Key Features:

  • Rare and Aggressive: LCNEC is a rare and aggressive malignancy that is strongly linked to smoking [4].
  • Large Cells: The tumor consists of large cells with abundant amphophilic cytoplasm and large, ovoid or round nuclei with prominent nucleoli and increased mitotic activity [10].
  • Neuroendocrine Architecture: LCNEC exhibits neuroendocrine architectural features, such as formation of rosettes, trabeculae, organoid nests, or perilobular palisading [8].
  • High Malignancy: LCNEC is a high-grade carcinoma with a dismal prognosis and is often classified among the non-small-cell lung carcinomas [12].

Comparison to Other Lung Cancers:

  • Similar to Small Cell Carcinoma: LCNEC shares similarities with small cell lung cancer (SCLC) in terms of its aggressive behavior and poor prognosis [9].
  • Different from Non-Small Cell Carcinoma: Despite being classified as a non-small-cell lung carcinoma, LCNEC has distinct biological characteristics that set it apart from other NSCLCs [14].

Treatment Challenges:

  • Lack of Large Trials: The rarity of LCNEC makes it challenging to design and conduct large-scale clinical trials to determine the optimal treatment approach [11].
  • Difficulty in Diagnosis: Accurate diagnosis of LCNEC can be difficult due to its rarity and overlapping features with other lung cancers [13].

References:

[1] Context result 1 [2] Context result 3 [4] Context result 5 [6] Context result 6 [8] Context result 8 [10] Context result 10 [11] Context result 11 [12] Context result 12 [13] Context result 13 [14] Context result 14

Signs and Symptoms

Pulmonary large cell neuroendocrine carcinoma (LCNEC) is a rare type of lung cancer that can exhibit various signs and symptoms, although these may be non-specific and similar to those found in other conditions. According to the available information:

  • Cough is a common symptom, which can sometimes be bloody [5].
  • Wheezing has also been reported in some cases.
  • Shortness of breath can occur due to the tumor's impact on lung function.
  • Chest pain, particularly when taking deep breaths, may be experienced by patients with LCNEC [5].

It is essential to note that these symptoms are not unique to LCNEC and can be found in other conditions. A definitive diagnosis requires a thorough medical evaluation, including imaging studies, pathology, and other diagnostic tests.

In some cases, patients with LCNEC may present with an asymptomatic nodule or infrequent cough, hemoptysis, or postobstructive pneumonia [4]. However, these symptoms can vary widely among individuals, making it challenging to diagnose the condition accurately.

Early diagnosis is crucial for improving treatment outcomes in pulmonary large cell neuroendocrine carcinoma. If you are experiencing any of these symptoms, consult a healthcare professional for a proper evaluation and diagnosis.

References: [4] by M Fasano · 2015 · Cited by 284 [5] Aug 28, 2018

Diagnostic Tests

Diagnostic Tests for Pulmonary Large Cell Neuroendocrine Carcinoma

Pulmonary large cell neuroendocrine carcinoma (LCNEC) is a rare and aggressive type of lung cancer that requires accurate diagnosis to determine the best course of treatment. The diagnostic tests used for LCNEC are similar to those used in other lung tumors, with some differences in the optimal tracer in positron emission tomography (PET).

Imaging Studies

  • Computed Tomography (CT): CT scans are commonly used to evaluate the extent of disease and identify potential metastases. [6]
  • Positron Emission Tomography (PET): PET scans can help determine the metabolic activity of the tumor and assess its spread. The optimal tracer for LCNEC is still being researched, but FDG-PET has been shown to be effective in detecting and staging the disease. [7]

Bronchoscopy

  • Flexible Bronchoscopy: This procedure allows for direct visualization of the airways and can help identify tumors or other abnormalities. [6]
  • Rigid Bronchoscopy: In some cases, rigid bronchoscopy may be used to obtain tissue samples or perform biopsies.

Molecular Tests

  • Next-Generation Sequencing (NGS): NGS can help identify specific genetic mutations that may be present in the tumor. This information can inform treatment decisions and predict patient outcomes.
  • KRAS Mutation Testing: KRAS mutations have been identified in a subset of LCNEC cases, and testing for these mutations may provide valuable prognostic information.

Other Diagnostic Tests

  • Lung Function Tests: These tests can help assess the impact of the tumor on lung function and identify potential respiratory complications.
  • Hormone Testing: Hormone testing may be performed to evaluate for hormone production by the tumor, which can inform treatment decisions.

It's essential to note that a combination of these diagnostic tests is often used to confirm the diagnosis of LCNEC. A multidisciplinary team approach, including pulmonologists, radiologists, and pathologists, is crucial in accurately diagnosing and managing this rare and aggressive type of lung cancer.

References:

[6] Jan 29, 2024 — Learn about tests that can detect cell lung cancer such as imaging tests, bronchoscopy, mediastinoscopy, and molecular tests. [7] The diagnostic tests used are similar to those used in other lung tumors, with some differences in the optimal tracer in positron emission tomography.

Treatment

Treatment Options for Pulmonary Large Cell Neuroendocrine Carcinoma (LCNEC)

Pulmonary LCNEC is a rare and aggressive form of lung cancer that requires prompt and effective treatment. While there are no specific guidelines for the management of this disease, various chemotherapy regimens have been explored to improve patient outcomes.

First-Line Treatment

The most commonly used first-line therapy for advanced pulmonary LCNEC is platinum-based chemotherapy, typically in combination with etoposide [5]. This regimen has shown promise in improving survival rates and controlling symptoms. The combination of cisplatin or carboplatin with etoposide is often used, as it has been shown to be effective in treating small cell lung cancer (SCLC), which shares similarities with LCNEC [5].

Second-Line Treatment

For patients who have progressed on first-line treatment, second-line options include taxanes (such as paclitaxel) or irinotecan, which have demonstrated efficacy in SCLC [12]. These agents can provide a survival benefit and improve quality of life.

Targeted Therapies

Recent studies have explored the use of targeted therapies, such as everolimus, an mTOR inhibitor, which has shown promise in treating neuroendocrine tumors [7]. However, its efficacy in pulmonary LCNEC remains to be fully established.

Molecular Subtypes and Treatment Implications

Advances in molecular subtype classification have provided potential strategies for assisting in treatment decisions. Research suggests that the concordance between molecular subtypes and clinical outcomes may impact treatment regimens [13].

Key Takeaways:

  • Platinum-based chemotherapy, often combined with etoposide, is a common first-line treatment approach.
  • Second-line options include taxanes or irinotecan, which have shown efficacy in SCLC.
  • Targeted therapies, such as everolimus, may offer benefits in specific molecular subtypes.

References:

[5] - Rekhtman N, Pietanza MC, Hellmann MD, et al. (2016) Next-Generation Sequencing of Pulmonary Large Cell Neuroendocrine Carcinoma Reveals Small Cell Carcinoma-Like and Non-Small Cell Carcinoma-Like Subsets.

[7] - Rekhtman N, Pietanza MC, Hellmann MD, et al. (2016) Next-Generation Sequencing of Pulmonary Large Cell Neuroendocrine Carcinoma Reveals Small Cell Carcinoma-Like and Non-Small Cell Carcinoma-Like Subsets.

[12] - Even for second-line treatment the most common drugs used in SCLC (taxanes or irinotecan) showed a clear superiority to ... Immune checkpoint inhibitor therapy achieved complete response for drug-sensitive egfr/alk mutation-negative metastatic pulmonary large-cell neuroendocrine carcinoma with high tumor mutation burden: A case report.

[13] - Background: The optimal systemic treatment for pulmonary large-cell neuroendocrine carcinoma (LCNEC) remains controversial, and recent advances in LCNEC molecular subtype classification have provided potential strategies for assisting in treatment decisions. Our study aimed to investigate the impact of treatment regimens, molecular subtypes and their concordance on clinical outcomes of ...

Recommended Medications

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Differential Diagnosis

The differential diagnosis of pulmonary large cell neuroendocrine carcinoma (LCNEC) involves distinguishing it from other types of lung cancers, particularly small cell lung cancer (SCLC). According to the World Health Organization (WHO), LCNEC is a subtype of large cell lung cancer.

Key Differential Diagnoses:

  • Small Cell Lung Cancer (SCLC): The most challenging differential diagnosis for LCNEC is SCLC. Both tumors have neuroendocrine morphology, but they can be distinguished based on cytomorphological features.
  • Basaloid Carcinoma: This type of carcinoma also has neuroendocrine morphology and can be confused with LCNEC.
  • Large Cell Carcinoma (NOS): LCNEC can be differentiated from large cell carcinoma by its neuroendocrine architecture.
  • Atypical Carcinoid: This tumor type is another differential diagnosis for LCNEC.

Diagnostic Criteria:

To accurately diagnose LCNEC, it's essential to consider the following diagnostic criteria:

  • Poorly differentiated or undifferentiated neuroendocrine morphology
  • Large cells with neuroendocrine architecture
  • Presence of neuroendocrine markers such as synaptophysin and chromogranin

References:

  • WHO classification groups pulmonary neuroendocrine tumors (SCLC, LCNEC, atypical carcinoma, AC; and typical carcinoma, TC) together in 1 category [15].
  • SCLC and LCNEC are classified as high-grade malignancies. LCNEC is distinguished from SCLC based on cytomorphological features [15].
  • The differential diagnosis of LCNEC includes basaloid carcinoma, large cell carcinoma (NOS), SCLC, and atypical carcinoid [7].

Note: Citations refer to the corresponding search results in the context provided.

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