Case 4 Junya Fukuoka, MD. PhD. Nagasaki University Graduate School - - PowerPoint PPT Presentation

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Case 4 Junya Fukuoka, MD. PhD. Nagasaki University Graduate School - - PowerPoint PPT Presentation

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


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Case 4

Junya Fukuoka, MD. PhD. Nagasaki University Graduate School of Biomedical Sciences Kameda Medical Center

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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.
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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
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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)
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HNF4α TTF‐1 p40 CK7

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BRAF (V600E)

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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)
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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
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Molecular data of CMPT

  • BRAF V600E
  • KRAS G12D, G12V, G12C
  • EGFR ex19/20 uncommon mut
  • HRAS , ALK mutation (very few)
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IHC of CMPT

  • TTF1 mostly+
  • CK7+ / CK20‐ (may be focal+)
  • HNF4α (vary ++, +, ‐)
  • p40, p63+ basal cells (continuous or discontinuous)
  • p53 mostly wild type pattern.
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Then, how about this case?

  • 71 year old woman
  • Detected to have abnormal

shadow.

  • No symptom
  • Never smoker
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HNF4α

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p63

Invasive mucinous adenocarcinoma

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  • How to make judge for small biopsy?
  • How about frozen?

But the issue is…

NEED IMMUNO?? Do lobectomy + LN dissection?

  • r wedge only?

Mutation may not separate them!

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  • CMPT. Some are early IMA?

Miyai K et al. Pathol Int 2018

Well, may not be….

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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.

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Chang et al, AJSP 2018

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Chang et al, AJSP 2018

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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)

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Kameda Digital Pathology Lab Awaji Medical Center Nagasaki Univ International collaborators

Thank you for your attention