Case 4 Junya Fukuoka, MD. PhD. Nagasaki University Graduate School of Biomedical Sciences Kameda Medical Center
Case 4: Pulmonary nodule • 50 year old woman detected to have abnormal shadow in her regular health screening. • Never smoking history. • No familial history of lung disease. • Left upper lobe mass. • Lobectomy was performed.
Pathological features by H&E • Single nodule with abundant mucin production • Focal papillary structure • Focal inflammatory changes • Focal ciliated epithelia • Minimal cellular atypia • No mitosis • Multi‐focal basal cell hyperplasia
Differential diagnosis (H&E): • Early invasive mucinous adenocarcinoma (IMA) • Inflammatory scar with abundant bronchiolar metaplasia • Glandular papilloma • Mixed squamous and glandular papilloma • Ciliated muconodular papillary tumor (CMPT) • Metastatic carcinoma from GI tract (esp panc)
HNF4α TTF‐1 CK7 p40
BRAF (V600E)
Differential diagnosis: • Early invasive mucinous adenocarcinoma (IMA) • Inflammatory scar with abundant bronchiolar metaplasia • Glandular papilloma • Mixed squamous and glandular papilloma • Ciliated muconodular papillary tumor (CMPT)
Ciliated muconodular papillary tumor (CMPT) • Recently proposed disease. • First report is 2002 (Japanese) by Ishikawa et al. • Nearly 40 cases reported, mostly from Asia. • Harboring frequent mutation • No recurrent occur but does not have long term follow up data • Little association with bronchial wall • Female ≒ male • Little association with smoking • Solitary nodule (1‐2cm) >> multiple
Molecular data of CMPT • BRAF V600E • KRAS G12D, G12V, G12C • EGFR ex19/20 uncommon mut • HRAS , ALK mutation (very few)
IHC of CMPT • TTF1 mostly+ • CK7+ / CK20‐ (may be focal+) • HNF4α (vary ++, +, ‐) • p40, p63+ basal cells (continuous or discontinuous) • p53 mostly wild type pattern.
Then, how about this case? • 71 year old woman • Detected to have abnormal shadow. • No symptom • Never smoker
HNF4α
p63 Invasive mucinous adenocarcinoma
But the issue is… • How to make judge for small biopsy? • How about frozen? Do lobectomy + LN dissection? NEED IMMUNO?? or wedge only? Mutation may not separate them!
CMPT. Some are early IMA? Miyai K et al. Pathol Int 2018 Well, may not be….
However!!! Recent Publication from MSKCC • The new concept of “ Bronchial Adenoma ” for all similar conditions. • There are several cases not completely fit to the criteria of CMPT. (lack mucous, cilia, papillary structure…) • But share most of molecular abnormality and clinical pictures.
Chang et al, AJSP 2018
Chang et al, AJSP 2018
Take Home Message: • CMPT is a newly recognized lung neoplasm • Diagnosis by small biopsy is challenging • Frozen to judge segmentectomy vs. lobectomy is difficult • Key is a presence of basal cells. (Do p63/p40) • May better to be included in “Bronchial adenoma”(?) • Mutation is frequent and gene analysis may not distinguish BA(CMPT) and IMA (except BRAF)
Nagasaki Univ Awaji Medical Center Thank you for your attention International Kameda Digital collaborators Pathology Lab
Recommend
More recommend
