Primary pulmonary lymphoepithelioma-like carcinoma

Abstract

Rationale: Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare subtype of non-small cell lung cancer (NSCLC). It is predominantly reported in East Asia and currently there is no standard treatment for this disease. We report a case of stage IV PPLELC that achieved pathological complete response (pCR) by neoadjuvant treatment. Patient concerns: The patient was a 46-year-old male who developed hemoptysis for about 20 ml of volume accompanied by cough and sputum after physical labor. Diagnoses: Contrast enhanced chest CT scanning showed occupation of left lower hilar area and left pleural effusion. Combined with medical history and auxiliary examination, the patient was formally diagnosed stage IV lymphoepithelioma-like carcinoma of the left lower lung (T3N0M1a pleura). Interventions: The patient was given Sintilimab combined with gemcitabine + nedaplatin chemotherapy (GP) regimen for four cycles with 3 weeks as a cycle, supplemented with antiemetics and stomach protection drugs to reduce chemotherapy-related side effects. Outcomes: After 4 cycles of treatment, the patient's left lung lesion has been markedly reduced and the left pleural effusion has also been significantly absorbed. Remarkably, surgical biopsies found no cancer cells in the lesion site and postoperative pathology showed complete pathological remission (pCR). Lessons: We reported a case of PPLELC that is sensitive to neoadjuvant treatment, showing excellent effectiveness and safety and achieving pCR.

Clinicopathological characteristics and cancer-specific prognosis of primary pulmonary lymphoepithelioma-like carcinoma: a population study of the US SEER database and a Chinese hospital

“…However, most PPLELC cases have no specific clinical manifestations at diagnosis, resulting in a common diagnosis of advanced tumor stages and a missed opportunity for curative resection. Literature reviews suggested effective chemotherapy and radiotherapy for late-stage disease (15,16). However, our research revealed that chemotherapy and radiotherapy were not correlated with the CSS of PPLELC.…”

Pathogenesis and therapeutic implications of EBV-associated epithelial cancers

“…On the other hand, no clear treatment guidelines for PPLELC have been outlined at present ( 34 ). In general, surgical treatment is the mainstay of treatment for early PPLELC, and treatment options like chemotherapy, radiotherapy are usually reserved for more advanced stages of PPLELC ( 45 ). Because PPLELC is uncommon, the adoption of standardized treatments has been challenging.…”

“…Because PPLELC is uncommon, the adoption of standardized treatments has been challenging. Existing treatment regimes are adapted from those used for stage IV NPC, which involves gemcitabine-based chemotherapy ( 45 ), taxanes ( 244 ), anti-angiogenic therapy ( 245 ), and anti-PD1/PD-L1 therapy ( 246 ). The favorable response of PPLELC to NPC treatments is evident of the therapeutic implications of molecular similarities between the two cancer types, and the substantial overlap in their molecular characteristics provides an explanation for the effective response of PPLELC to chemotherapies typically used in NPC.…”

“…It presents at a lower mean age of 51-55 years old and has a better prognosis than non-LELC lung cancers ( 42 ), with a higher incidence in non-smokers and Asian females ( 43 ). Since its first documentation ( 28 ), current understanding of the histological resemblance, geographical clustering and molecular similarities ( 36 ) between NPC and PPLELC have prompted increasing interest in the role of EBV in PPLELC ( 34 , 44 , 45 ). Currently, PPLELC is the most well studied LELC subtype and will be the primary focus when discussing LELCs.…”