31st Congress of the European Society of Pathology; Nice, France; 7th-11th September 2019.

Sunday, 8th September 2019 17.15-19.15.

Uranie-Calliope.

SS-03 slide seminar:

Case 5.

Roderick H.W. Simpson.

University of Calgary, Alberta, Canada.

Department of Laboratory Medicine & Pathology

Declaration of Conflict of Interest

• I have had no affiliation (financial or otherwise) with a pharmaceutical, medical device, and/or communications organization during the past two years.

31st Congress of the European Society of Pathology; Nice, France; 7th-11th September 2019.

Sunday, 8th September 2019 17.15-19.15.

Uranie-Calliope.

SS-03 slide seminar:

Case 5.

Roderick H.W. Simpson.

University of Calgary, Alberta, Canada.

Co-author: Dr. Martin Hyrcza, Univ of Calgary.

31st Congress of the European Society of Pathology; Nice, France; Sunday, 8th September 2019, 17.15-19.15.

Case no. 5. _________________________________________________________________________________________________________________________________________________________________________________________________________

Clinical History.

Male 73.

Long-standing history of congestion and rhinorroea, worse in cold weather.

No epistaxis.

Imaging: large right-sided nasal mass seems to arise from posterior septum.

Extends into nasopharynx.

Case 5: CT mass in right nasal cavity.

Case 5: Gross description.

Irregular rubbery mass weighing 10 grams and measuring 44 × 26 × 21 mm. The cut surface is solid and tan-coloured.

Case 5: Overview-1.

Case 5: Overview-2.

5 aspects:1. Cystic structures.

2. Basaloid areas.

3. Glandular areas.

4. Oncocytic and respiratory epithelial areas.

5. Stroma.

Case 5: Cystic structures-1.

Case 5: Cystic structures-2.

Case 5: Basaloid areas-1.

Case 5: Basaloid areas-2.

Case 5: Sero-mucinous glands-1.

Case 5: Sero-mucinous glands-2.

Case 5: Oncocytic area.

Case 5: Respiratory epithelial structure with cilia.

Case 5: Stroma, cellular with oedematous areas.

Case 5.

?What is your diagnosis?

Case 5.

?What is your diagnosis?

My impression at this stage was that the imaging suggested a hamartoma, but the histology did not

fit with either sero-mucinous or REAH (Respiratory Epithelial Adenomatoid

Hamartoma).

Will immunohistochemistry and special stains help?

Case 5: Secretions and rare goblet cells.PASD Mucicarmine

PASD Mucicarmine

Case 5: Immunohistochemistry-1.

CK7

DOG1 – mainly

seromucinous

glands.

CK19

GCDFP15 – mainly

seromucinous glands.

Case 5: Immunohistochemistry-2.p63 p40

CK5/6 S-100

Case 5: Immunohistochemistry-3, stroma.

SMMS Androgen Receptors

CD34CD10

Case 5: Immunohistochemistry Ki-67 – focally high.

Case 5: Immunohistochemistry.

The following investigations were negative:

IHC

CK20, CDX2, GATA3, Mammaglobin.

ER, PR, p16.

TTF-1, Synaptophysin.

FISH

MAML2

Case 5. Diagnosis.

Did immunohistochemistry and special stains help? Somewhat, but no definite answer.

My impression at this stage was that the overall appearance suggested a sinonasal hamartoma-like lesion, but histology and IHC still did not fit with

either sero-mucinous hamartoma or REAH.

It is probably a related benign lesion, but the focally high proliferation is a little worrying.

Case 5. Outcome.

No information on the website, but…

Had routine blood tests in June 2019, so presumably…

Well.

Respiratory Epithelial Adenomatoid Hamartoma (REAH).________________________________________________________________________________________________________________________________________________________________________________

Definition: “…a benign acquired overgrowth of indigenous glands of the sinonasal tract arising from the surface epithelium.” WHO 2017.

Sex: distinct male predominance.

Age: 3rd-9th decade.

Site: Most in posterior nasal septum.

REAH: proliferation of glands and ducts.

WHO 2005

Seromucinous Hamartoma.________________________________________________________________________________________________________________________________________________________________________________

Definition: “…a benign acquired overgrowth of indigenous seromucinous glands of the nasal cavity and paranasal sinuses.” WHO 2017.

Sex: male:female = 3:2.

Age: 14-85 (mean 56).

Site: Most in posterior nasal septum or nasopharynx.

Seromucinous Hamartoma.________________________________________________________________________________________________________________________________________________________________________________

WHO 2005.

WHO 2017.

IHC: CK19+, S-100+. Absence of basal-myoepithelial cells.

Scattered positivity for p63 – Huang Y-W et al 2018

Case 5: What next?

Toronto (Ilan Weinreb, Bayardo Perez-Ordoñez).

“The polyp is favoured to represent a seromucinoushamartoma. However, some of the features are unique and may represent an as yet unrecognized variant of the hamartoma spectrum.”

Also, Alena Skálová, Plzeň, Czech Republic.

Case 5: Summary.

• Clinically fits with a sinonasal hamartoma.

• Microscopically a benign or at worst low grade malignant proliferation

• BUT….microscopically does not really resemble REAH or Seromucinous Hamartoma.

Questions:

• Is it a true hamartoma or a benign neoplasm?

• What name should it be given?

Perhaps – Multiphenotypic sinonasal adenoma or hamartoma.

Lake Peyto, Banff National Park, Canada; May 2016.

Any Questions?

OR suggestions.