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Nasal cavity, paranasal sinuses, nasopharynx

Sinonasal adenocarcinoma

Intestinal type

Authors: Vladyslav Ilchenko, M.D., Rifat Mannan, M.B.B.S., M.D.

Editorial Board Member: Bin Xu, M.D., Ph.D.

Deputy Editor-in-Chief: Kelly Magliocca, D.D.S., M.P.H.

Last author update: 23 May 2023

Last staff update: 27 February 2024

Copyright: 2004-2024, PathologyOutlines.com, Inc.

PubMed Search: Intestinal type adenocarcinoma nasal

Table of Contents

Cite this page: Ilchenko V, Mannan R. Intestinal type. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalintestinaladeno.html. Accessed December 27th, 2024.

Definition / general

Sinonasal intestinal type adenocarcinoma (ITAC) is an epithelial tumor of the nasal cavity and the paranasal sinuses, often related to professional exposure to organic dust (such as wood or leather) and histologically resembling intestinal adenocarcinoma (Head Neck 2016;38:1564)
ITACs can be present in 5 morphologic patterns: colonic, papillary, solid, mucinous and mixed (Head Neck Pathol 2017;11:295)

Essential features

Incidence is correlated with organic dust exposure (Cancers (Basel) 2021;13:5245)
ITAC is usually located in the olfactory mucosa of the ethmoid sinus (Cancers (Basel) 2021;13:5022)
A characteristic feature of this tumor is an increased production of intracellular or extracellular mucin, similar to mucinous adenocarcinoma of the intestinal tract (Cancers (Basel) 2021;13:5022)

Terminology

Colloid type adenocarcinoma, colonic type adenocarcinoma, enteric type adenocarcinoma (these terms are not recommended for use by WHO)

ICD coding

ICD-O: 8144/3 - intestinal type adenocarcinoma
ICD-11:
- 2C20.0 & XH0349 - adenocarcinoma of nasal cavity & adenocarcinoma, intestinal type
- 2C22.0 & XH0349 - adenocarcinoma of accessory sinuses & adenocarcinoma, intestinal type

Epidemiology

Male predominance (M:F ratio can vary from 4:1 to 21:1 in different geographical regions) with a median age of 60 - 65 years (Cancers (Basel) 2021;13:5245, Head Neck 2016;38:1564)
Strong correlation to professional exposure to organic dust, such as wood or leather (Head Neck 2016;38:1564)
Incidence of ITAC varies between geographical regions, presumably due to differences in dust exposure (Cancers (Basel) 2021;13:5245)
Reported average exposure period is ~40 years
In contrast to squamous cell carcinoma of a similar region, alcohol, smoking and human papillomavirus do not seem to be risk factors for the development of ITAC (Head Neck 2016;38:1564)

Sites

Most common location is the olfactory mucosa of the ethmoid sinus but can occur in the nasal cavity and maxillary antrum (Cancers (Basel) 2021;13:5022)
Cases related to industrial wood dust exposure show predilection for ethmoid sinus
Sporadic tumors are more common in maxillary antrum

Pathophysiology

ITAC is considered to develop through intestinal metaplasia of the sinonasal epithelium (Patholog Res Int 2011;2011:230147)
Wood dust does not seem to have direct mutagenic properties of its own but prolonged exposure to and irritation by wood dust particles can stimulate cellular turnover by inflammatory pathways
Chronic inflammation is recognized as an important mechanism in tumor initiation and progression (Head Neck 2016;38:1564)

Etiology

Exposure to organic dust (such as wood or leather) for several decades is the major risk factor (Head Neck 2016;38:1564)

Clinical features

Unilateral nasal obstruction, rhinorrhea, epistaxis are common presentations (Arch Craniofac Surg 2018;19:210)
Tumor tends to be very locally aggressive but rarely metastasizes (Arch Craniofac Surg 2018;19:210, Pathol Res Pract 2019;215:152432)
Advanced tumors may cause pain, neurologic disturbances, exophthalmos, visual impairment

Diagnosis

CT and MRI are used for diagnosing early lesions, defining disease extent and detecting early recurrences
Ultrasound guided fine needle aspiration plays an important role in confirming the diagnosis before treatment because it is frequently misdiagnosed as a salivary gland tumor (Arch Craniofac Surg 2018;19:210)

Radiology description

CT or MRI may reveal soft tissue mass in the ethmoid sinus, frontal sinus or other locations (Arch Craniofac Surg 2018;19:210)

Radiology images

Images hosted on other servers:

Frontoethmoidal sinus mass

Frontal sinus mass

Prognostic factors

Locally aggressive with ~50% local recurrence
Locoregional / distant metastasis occurs in 10 - 20% cases
Mean disease free survival is 32 months (Cancers (Basel) 2021;13:5022)
Papillary subtype is associated with better prognosis, while mucinous and solid subtypes carry poor prognosis (Cancers (Basel) 2021;13:5022)
Prognosis and response to the chemotherapy can be associated with genotoxic agents, such as TP53 mutations
- Significantly higher complete response rate and disease free survival rate were reported in patients carrying tumors with wild type TP53 compared to patients bearing mutated TP53 (Head Neck 2016;38:1564)
There is no significant prognostic difference between occupational and sporadic tumors

Case reports

36 year old man with ITAC that developed from inverted papilloma (J Clin Diagn Res 2016;10:ED12)
50 year old man with right nasal block, mass in right nose and frontal headache (J Clin Diagn Res 2014;8:149)
63 year old man with a rapidly growing soft mass on his glabellar region for 4 months (Arch Craniofac Surg 2018;19:210)

Treatment

Standard treatment is surgical resection, with a minimally invasive endoscopic approach when possible (Cancers (Basel) 2021;13:5022)
Due to the high recurrence rate of ITAC, postoperative radiation is commonly administered
- Neoadjuvant chemotherapy is used in advanced stage tumors (Cancers (Basel) 2021;13:5022)
Because local recurrences and metastases of these tumors occur frequently, palliative treatment is most often the only option (Head Neck 2016;38:1564)

Clinical images

Images hosted on other servers:

Mass on glabellar region

Wide excision of mass

76 days after surgery

Endoscopic resection

Gross description

Irregular exophytic tan-pink mass bulging into nasal cavity or paranasal sinuses, often with necrotic and friable appearance
Some lesions are gelatinous

Gross images

Contributed by Kelly Magliocca, D.D.S., M.P.H.

Macroscopic appearance

Images hosted on other servers:

Open biopsy masses

Microscopic (histologic) description

ITACs can be present in 5 morphologic patterns: colonic, papillary, solid, mucinous and mixed (Head Neck Pathol 2017;11:295)
Papillary pattern: predominantly papillary growth with tubular elements
Colonic pattern: tubuloglandular architecture with minor papillary elements; neoplastic cells have palisaded hyperchromatic nuclei and a few goblet cells
Solid pattern: poorly differentiated; trabecular and solid proliferation of neoplastic cells
Mucinous pattern: mucin laden neoplastic glands or tumor cell clusters within pools of extracellular mucin
Mixed pattern: variable admixture of 2 or more subtypes
Goblet cells, Paneth cells and argentaffin cells may be found in all patterns
Exceptionally well differentiated ITAC may resemble normal small intestinal mucosa with well formed villi and muscularis mucosae

Microscopic (histologic) images

Contributed by Vladyslav Ilchenko, M.D., Diana Bell, M.D. and Kelly Magliocca, D.D.S., M.P.H.

Complex glands

Papillary pattern with goblet cells

Tubuloglandular architecture

Complex colonic type glands and stroma

Mucinous type

CK20

CDX2

Positive stains

Consistently positive for CK20, CDX2, villin, MUC2, SATB2 (Head Neck Pathol 2022;16:670)
Variably positive for CK7 and CEA
Chromogranin+ cells may be present, scattered or in clusters

Molecular / cytogenetics description

Frequent KRAS and p53 mutations (Head Neck Pathol 2022;16:670, Head Neck 2016;38:1564)
Tumors with occupational exposure to wood dust show p53, p14 ARF, p16 INK4a gene deregulation
EGFR alterations also reported (Cancers (Basel) 2021;13:5022)
Mutations in the genes in Wnt, MAPK and PI3K pathways were reported in 20%, 20% and 24% of cases, respectively (Cancers (Basel) 2021;13:5022)

Videos

Update on sinonasal carcinoma

Malignant tumors of the paranasal sinuses

Sample pathology report

Ethmoid sinus, biopsy:
- Invasive adenocarcinoma, intestinal type (see comment)
- Comment: The patient's clinical history of a mass in the right ethmoid sinus is noted. Morphology and immunophenotypic features (positive for CK7, CK20, villin and MUC2) are most consistent with intestinal type sinonasal adenocarcinoma. However, a metastasis from elsewhere, particularly the digestive tract, cannot be absolutely ruled out. Clinical and imaging correlation is suggested.

Differential diagnosis

Nonintestinal sinonasal adenocarcinoma:
- Usually not associated with wood dust exposure
- Clean background, may have papillary / tubular architecture
- More glandular, less papillary, few columnar / goblet cells
- Typically negative for intestinal markers, such as CDX2 and SATB2
Metastasis from colonic adenocarcinoma:
- Rare but most important differential
- Usually CEA+, CK7-, chromogranin-
- Clinical features and colonoscopy are helpful
Papillary sinusitis:
- May have abundant mucinous material but has short and blunt papillae with clean background
- Thick and hyalinized basement membrane
- Ciliated surface cells
- Prominent eosinophils and no significant cytological atypia

Board review style question #1

A 70 year old man presented with a mass in the right nose and a frontal headache. A representative image of the mass is shown in the image above. Which of the following statements is true?

Chemotherapy is the standard treatment
Cigarette smoking is the number one risk factor for this tumor
The tumor can be locally aggressive but rarely metastasizes
The tumor is most commonly located in the nasal cavity

Board review style answer #1

C. The tumor can be locally aggressive but rarely metastasizes. The image depicts intestinal type adenocarcinoma, with colonic type morphology. These tumors are known to be locally aggressive but metastasis is very uncommon (answer C). Ethmoid sinus is the most common location, not nasal cavity (answer D). Exposure to organic wood dust is considered as a strong risk factor. Unlike squamous cell carcinoma, cigarette smoking or human papillomavirus is not etiologically linked to ITAC (answer B). Surgical resection is the standard treatment, not chemotherapy (answer A).

Comment Here

Reference: Intestinal type adenocarcinoma

Board review style question #2

Which of the following statements is true for intestinal type sinonasal adenocarcinoma?

Signet ring cell morphology is the most common histologic pattern
Tumor cells have uniform nuclei with salt and pepper chromatin
Tumor may be positive for EBV
Tumor usually demonstrates positive staining for CK20, villin or SATB2

Board review style answer #2

D. Tumor usually demonstrates positive staining for CK20, villin or SATB2. ITAC shows intestinal phenotype and shows immunoreactivity for CK20, villin and SATB2 (answer D). There is no association with EBV (answer C). Common histologic subtypes include colonic, papillary, solid, mucinous and mixed patterns. Signet ring cell morphology is uncommon (answer A). Salt and pepper nuclear chromatin is characteristically observed in neuroendocrine tumors and not in ITAC (answer B).

Comment Here

Reference: Intestinal type adenocarcinoma

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