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Rare primary pulmonary mucoepidermoid carcinoma shows low Ki-67 and CRTC1-MAML2 fusionRare Lung Tumor Case Reveals Key Gene Fusion

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Key Takeaway
Recognize PMEC as a rare entity; this single case cannot guide diagnosis or prognosis.

This case report describes a single patient with primary pulmonary mucoepidermoid carcinoma (PMEC), a rare thoracic malignancy. The patient was a 61-year-old male with a 20-year smoking history. The report focuses on the diagnostic workup and molecular profile rather than on treatment or outcomes.

Immunohistochemistry showed CK7 (+), P63 (-), P40 (-), CD117 (focal+), TTF-1 (-), NapsinA (-), and Ki-67 (7%+). Molecular testing identified a CRTC1-MAML2 gene fusion and found no classic driver gene mutations such as EGFR or KRAS. PD-L1 expression was negative, and tumor mutational burden was low.

The authors suggest the findings are consistent with a low-grade PMEC and a relatively favorable prognosis based on the low Ki-67 and positive MAML2 fusion. However, this interpretation comes from a single case, and no follow-up, treatment, or outcome data are reported.

The main limitation is the single case report design, which precludes any assessment of diagnostic accuracy, prognosis, or treatment response across patients. No safety data, adverse events, or tolerability information are reported. Funding and conflicts of interest are not reported.

For clinicians, this report may raise awareness of PMEC and its molecular features, but it does not provide evidence to guide management. Diagnostic and prognostic decisions should not be based on this single case.

This is a single case report of a 61-year-old man with a 20-year smoking history who was diagnosed with primary pulmonary mucoepidermoid carcinoma (PMEC), a rare type of lung cancer. The report describes his tumor's features in detail.

Doctors found that the tumor had a CRTC1-MAML2 gene fusion, which is a genetic change often seen in this type of cancer. They also found no common driver mutations like EGFR or KRAS. The tumor did not have PD-L1 expression, and its mutational burden was low. The Ki-67 level was 7%, which is a marker of how fast cells are dividing.

Because this is only one case, the findings cannot be generalized to all patients with PMEC. The report suggests the tumor may be low-grade with a relatively favorable prognosis, but this is based on a single patient. No safety or treatment information was reported.

Readers should understand that this is early, limited evidence. It may help doctors recognize this rare cancer, but it does not change treatment guidelines or provide proof of what works best. Anyone with questions about their own diagnosis should talk to their doctor.

What this means for you:
A single case of rare lung cancer showed a specific gene fusion and low-grade features, but more research is needed.

Common questions

What is primary pulmonary mucoepidermoid carcinoma?

It is a rare type of lung cancer. This case report describes one man with this diagnosis. The tumor had a CRTC1-MAML2 gene fusion and low Ki-67, which may suggest a low-grade tumor. Because it is so rare, most information comes from individual cases like this one.

Is this cancer hereditary?

The report does not say whether this cancer runs in families. It only describes one patient. The CRTC1-MAML2 fusion is a genetic change found in the tumor itself, not necessarily an inherited one. Talk to your doctor if you have concerns about family risk.

What does a low Ki-67 mean?

Ki-67 is a marker that shows how fast cells are dividing. In this case, it was 7%, which is considered low. The report suggests this may indicate a low-grade tumor with a relatively favorable prognosis. However, this is based on a single patient, so it cannot be applied to everyone.

Does this change how this cancer is treated?

No. This is a single case report, so it does not provide evidence that any treatment works better. It may help doctors recognize the disease, but treatment decisions should be based on guidelines and discussions with your care team. Always consult your doctor about your own situation.

Study Details

Study typeSystematic review
EvidenceLevel 1
PublishedOct 2026
View Original Abstract ↓
Primary pulmonary mucoepidermoid carcinoma(PMEC) is a rare salivary gland-type malignancy, accounting for fewer than 0.2% of all primary lung tumors. We report herein a 61-year-old male patient with PMEC who was admitted for a space-occupying lesion in the left upper lobe and had a 20-year smoking history. Contrast-enhanced chest CT and positron emission tomography-computed tomography (PET-CT) revealed a central space-occupying lesion in the left hilum with distal obstructive inflammation. Flexible bronchoscopy revealed a nodular mass in the left main bronchus. Biopsy histopathology combined with immunohistochemistry confirmed the diagnosis of PMEC. Immunohistochemistry showed CK7 (+), P63 (-), P40 (-), CD117 (focal+), TTF-1 (-), NapsinA (-), and Ki-67 (7%+). Molecular testing identified a CRTC1-MAML2 gene fusion, whereas no classic driver gene mutations (e.g., EGFR or KRAS) were detected. PD-L1 expression was negative, and the tumor mutational burden (TMB) was low. The low Ki-67 index and positive MAML2 fusion in this case suggest a low-grade PMEC with a relatively favorable prognosis. The present study discusses the clinicopathological features, imaging findings, molecular diagnosis, and therapeutic advances of PMEC, along with a review of the relevant literature, aiming to increase clinical awareness of this rare disease.
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