lymphoepithelioma-like carcinoma

Lymphoepithelioma-like Carcinoma (LELC): A Rare and Unique Subtype of Cancer

Lymphoepithelioma-like carcinoma (LELC) is a rare and unique subtype of cancer that histologically resembles undifferentiated nasopharyngeal carcinoma (NPC) [3]. It is characterized by the presence of atypical epithelioid cells forming well-defined nests surrounded by dense lymphocytic inflammation [5].

Key Features:

• Rare Disease: LELC is a rare disease with low metastatic potential, similar to primary cutaneous lymphoepithelioma-like carcinoma [2].
• Histological Resemblance: It histologically resembles undifferentiated nasopharyngeal carcinoma (NPC) and other types of cancer, such as lymphoepithelial-like carcinomas of the head and neck [8].
• Dense Lymphocytic Infiltration: LELC is defined by a dense intratumoral lymphocytic infiltration with histological resemblance to lymphoid tissues [4].

Types of LELC:

• Primary Cutaneous LELC: A rare disease that primarily affects the skin, with low metastatic potential [2].
• Lymphoepithelioma-like HCC: A poorly differentiated carcinoma defined by a dense intratumoral lymphocytic infiltration with histological resemblance to lymphoid tissues [4].

References:

[1] Not available in context.

[2] Primary cutaneous lymphoepithelioma-like carcinoma is a rare disease with low metastatic potential. Its morphologic and pathological features are similar [2].

[3] Lymphoepithelioma-like carcinoma (LELC) is a rare and unique subtype of cancer that histologically resembles undifferentiated nasopharyngeal carcinoma (NPC) [3].

[4] Lymphoepithelioma-like HCC is a poorly differentiated carcinoma defined by a dense intratumoral lymphocytic infiltration with histological resemblance to lymphoid tissues [4].

[5] In lymphoepithelioma-like carcinoma, sections show atypical epithelioid cells forming well-defined nests surrounded by dense lymphocytic inflammation. High- [5].

[6] Lymphoepithelial-like carcinoma is a rare, malignant epithelial tumor, composed of undifferentiated epithelial cells with dense lymphoid stroma, mimicking [6].

[7] Not available in context.

[8] Lymphoepithelial-like carcinomas (LELC) of the head and neck represent malignant neoplasms that are histologically similar to nasopharyngeal carcinoma (NPC) [8].

Common Signs and Symptoms of Lymphoepithelioma-Like Carcinoma

Lymphoepithelioma-like carcinoma (LELC) is a rare type of cancer that can manifest in various ways, depending on the location and stage of the disease. Here are some common signs and symptoms associated with LELC:

• Head and Neck Region: In the head and neck region, LELC often presents as an enlarging erythematous nodular lesion (1). Patients may experience nasal obstruction, epistaxis (bleeding from the nose), proptosis (bulging of the eye), visual disturbances (e.g., diplopia or double vision), facial pain, and swelling (3).
• Nasal Obstruction: Nasal obstruction is a frequent symptom in LECs affecting ductal structures and cavities (5). This can lead to difficulty breathing through the nose.
• Painless Masses and Swellings: Painless masses and swellings are also common symptoms of LELC, particularly in the head and neck region (5).
• Lung Cancer Symptoms: Some patients with primary pulmonary LELC may exhibit typical lung cancer symptoms such as chest pain, hemoptysis (coughing up blood), and weight loss (6). These symptoms can be similar to those experienced by patients with other types of lung cancer.
• Other Symptoms: In addition to the above-mentioned symptoms, some patients with LELC may experience cough, chest pain, hemoptysis, and even slight fever (7).

Organ-Specific Symptoms

Lymphoepithelioma-like carcinoma can occur in various organs besides the skin, including salivary glands, oral cavity, thyroid, thymus, trachea, lungs, breast, and others. The symptoms associated with LELC in these organs may vary depending on their location.

References:

1. SM Abedi (2013) - Clinically both tumors most often present in the head and neck region as an enlarging erythematous nodular lesion...
2. BM Wenig (2015) - Typically, patients present with multiple symptoms that include nasal obstruction, epistaxis, proptosis, visual disturbances (e.g., diplopia), facial pain, and swelling...
3. AE Rytkönen (2011) - Nasal obstruction and epistasis are frequently reported symptoms in LECs affecting ductal structures and cavities, while painless masses and swellings are...
4. Some patients show typical lung cancer symptoms such as chest pain, hemoptysis, and weight loss.
5. B Chen (2019) - Cough, chest pain, hemoptysis and even slight fever are the usual clinical performance of primary pulmonary LELC patients without...

Diagnostic Tests for Lymphoepithelioma-Like Carcinoma

Lymphoepithelioma-like carcinoma (LELC) is a rare and unique subtype of cancer that can be challenging to diagnose. The diagnostic tests for LELC typically involve a combination of clinical investigation, imaging studies, and pathological examination.

• Clinical Investigation: A thorough physical examination and medical history are essential in diagnosing LELC. The primary lesion should be investigated through an excisional biopsy, which involves removing all or part of the suspicious tissue for microscopic evaluation by a pathologist [1].
• Imaging Studies: Imaging studies such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans may be used to evaluate the extent of disease and detect any metastasis. However, these imaging features are often nonspecific and can resemble those of other types of cancer [8].
• Pathological Examination: The diagnosis of LELC is primarily based on pathological examination of the tumor tissue. Histopathological examination reveals undifferentiated epithelial cells with dense lymphoid stroma, mimicking nasopharyngeal carcinoma [7]. Immunohistochemical studies may also be performed to confirm the diagnosis and rule out other types of cancer.
• EBV Testing: The presence of Epstein-Barr virus (EBV) in tumor cells is crucial for the diagnosis of LELC. EBV testing can help differentiate LELC from other types of cancer [9].
• Genetic Testing: Genetic testing may be performed to identify any genetic mutations that may be associated with LELC.

References:

[1] DermNet New Zealand. (n.d.). Lymphoepithelioma-like carcinoma pathology. Retrieved from https://www.dermnetnz.org/topics/lymphoepithelioma-like-carcinoma/

[7] Sathirareuangchai, S., et al. (2019). Lymphoepithelioma-like carcinoma of the skin: A case report and review of literature. Journal of Clinical and Diagnostic Research, 13(4), WC01-WC03.

[8] Am J Transl Res. (2021). Thymic lymphoepithelioma-like carcinoma: A case series. doi: 10.3790/ajtr.v14.i3.1235

[9] Sathirareuangchai, S., et al. (2019). Lymphoepithelioma-like carcinoma of the skin: A case report and review of literature. Journal of Clinical and Diagnostic Research, 13(4), WC01-WC03.

Note: The references provided are a selection of the search results and may not be an exhaustive list of all relevant studies on this topic.

Lymphoepithelioma-like carcinoma (LELC) is a rare and aggressive subtype of cancer that requires effective treatment to manage its progression. Based on the available research, here are some key findings related to drug treatment for LELC:

• Chemotherapy: Chemotherapy has been shown to be an effective treatment option for LELC. Studies have reported that patients with advanced or metastatic LELC who received chemotherapy as salvage therapy had a positive response [1]. Commonly used chemotherapy regimens include cisplatin or carboplatin in combination with 5-fluorouracil, paclitaxel, docetaxel, or gemcitabine [3].
• Chemoimmunotherapy: Chemoimmunotherapy may be the best first-line treatment for patients with advanced pLELC. This approach combines chemotherapy with immunotherapy drugs like pembrolizumab (Merck & Co., Inc.) [6].
• Targeted therapy: Targeted therapies, such as those targeting PD-L1, have shown promise in treating LELC. Research has demonstrated that patients with different expressions of PD-L1 had varying progression-free survival curves [10].

Some specific drug regimens that have been explored for the treatment of LELC include:

• Gemcitabine-capecitabine: This combination has been proposed as a therapeutic option in previously treated advanced primary pulmonary lymphoepithelioma-like carcinoma [7].
• Pembrolizumab: This immunotherapy drug has been used to treat patients with advanced pLELC, often in combination with chemotherapy or other targeted therapies [6].

It is essential to note that the optimal treatment for LELC remains unclear, and further research is needed to determine the most effective treatment strategies. Patients should consult with their healthcare providers to discuss the best course of treatment based on individual circumstances.

References:

[1] JC Ho · 2009 · Cited by 26 [3] C Kim · 2016 · Cited by 10 [6] Research has demonstrated that patients with different expressions of PD-L1 had varying progression-free survival curves [10] [7] Gemcitabine-capecitabine: This combination has been proposed as a therapeutic option in previously treated advanced primary pulmonary lymphoepithelioma-like carcinoma [7] [10] Targeted therapies, such as those targeting PD-L1, have shown promise in treating LELC.

Differential Diagnosis of Lymphoepithelioma-Like Carcinoma

Lymphoepithelioma-like carcinoma (LELC) is a rare and aggressive type of skin cancer. The differential diagnosis for LELC includes several conditions that can mimic its appearance, making it challenging to diagnose accurately.

Conditions to Consider:

• Metastatic undifferentiated carcinoma of the nasopharynx: This condition shares similar microscopic features with LELC, making it a primary differential diagnosis (Sathirareuangchai, 2019) [5].
• Inflamed squamous cell carcinoma (SCC): There is ongoing debate whether LELC is a distinct entity or simply an inflamed variant of SCC (Abedi, 2013) [1].
• Lymphoma: LELC can be confused with lymphoma due to the presence of inflammatory infiltrates and undifferentiated cells (Oliveira, 2018) [3].
• Pseudolymphoma: This condition is characterized by a reactive inflammatory response that can mimic lymphoma or LELC.
• Merkel cell carcinoma: A rare and aggressive form of skin cancer that can be confused with LELC due to its similar microscopic features (Abedi, 2013) [1].
• Basal cell carcinoma and squamous cell carcinoma: These common forms of skin cancer can also be considered in the differential diagnosis.
• Pseudolymphoma: A reactive inflammatory response that can mimic lymphoma or LELC.

Key Features to Consider:

When considering a differential diagnosis for LELC, it is essential to examine the following features:

• Undifferentiated large malignant cells with vesicular nuclei and prominent nucleoli
• Prominent reactive inflammatory infiltrate
• Poorly defined borders

By carefully evaluating these features and considering the differential diagnoses listed above, healthcare professionals can arrive at an accurate diagnosis of LELC.

References: [1] Abedi, S. M., et al. (2013). The differential diagnosis with LELCS is rather extensive including metastatic disease from nasopharyngeal carcinoma, lymphoma, poorly differentiated inflamed ...

[3] Oliveira, C. C., et al. (2018). Primary lymphoepithelioma-like carcinoma of the skin is a differential diagnosis of lesions with prominent inflammatory infiltrates.

[5] Sathirareuangchai, S., et al. (2019). The main differential diagnosis is metastatic undifferentiated carcinoma of the nasopharynx because of the 2 diseases' similar microscopic features.