Second-line immunotherapy combination with etoposide and capecitabine improves extracranial disease control in pulmonary choriocarcinomaNew treatment combinations show promise for rare lung cancer

Primary pulmonary choriocarcinoma (PPC) is an exceptionally rare and aggressive non-gestational trophoblastic malignancy for which no standard treatment has been established. We report a 61-year-old man with PPC and brain metastases who received intracranial radiotherapy and first-line albumin-bound paclitaxel plus cisplatin, followed by etoposide, capecitabine, and camrelizumab after disease progression. Targeted next-generation sequencing identified loss-of-function or deleterious alterations involving TP53, STK11, KEAP1, and SMARCA4. The second-line immunotherapy-based combination was associated with prolonged extracranial disease control and radiological improvement, although the independent contribution of immune checkpoint inhibition could not be isolated from concurrent chemotherapy and radiotherapy; the disease later progressed intracranially, and the patient ultimately died of intra-abdominal hemorrhage with hepatic metastases. A systematic review of English-language PubMed reports from 2001 to 2026 identified 45 previously reported PPC patients, underscoring the rarity, diagnostic difficulty, aggressive course, and limited evidence base for this disease. Together, this case and literature synthesis suggest that PPC may be clinically and molecularly distinct from gestational choriocarcinoma; however, because these molecular observations derive from a single patient, they should be regarded as hypothesis-generating rather than establishing a general molecular signature of PPC, and that multimodal treatment incorporating immune checkpoint inhibition warrants further investigation in collaborative rare-tumor cohorts.