Nasal cavity, paranasal sinuses, nasopharynx
Sinonasal adenocarcinoma
Intestinal type
Last author update: 1 October 2013
Last staff update: 21 March 2023 (update in progress)
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PubMed Search: Intestinal type adenocarcinoma nasal
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Diagnosis | Prognostic factors | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Mannan R, Yuan S. Intestinal type. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalintestinaladeno.html. Accessed March 21st, 2023.
Definition / general
- Primary sinonasal epithelial malignancy that histologically resembles intestinal adenoma or adenocarcinoma
- #2 most common type of sinonasal adenocarcinoma after adenoid cystic carcinoma
Terminology
- ITAC is also known as colonic type adenocarcinoma or enteric type adenocarcinoma
Epidemiology
- Male predominance (M:F ratio is 4:1)
- Tends to affect older patients (mean 60 years)
- Strong association with chronic exposure to hardwood dust (Cancer 1984;54:482) and leather dust (Acta Otorhinolaryngol Ital 2004;24:199)
- In woodworking industry, incidence is 1,000 times higher than general population (Am J Surg Pathol 1986;10:192)
- Reported average exposure period is about 40 years
Sites
- Ethmoid sinus is most common location (40%), followed by nasal cavity (25%) and maxillary antrum (20%) (Head Neck Pathol 2007;1:38)
- Cases related to industrial wood dust exposure show predilection for ethmoid sinus
- Sporadic tumors are more common in maxillary antrum
- In nasal cavities, inferior and middle turbinates are the preferred sites
Clinical features
- Unilateral nasal obstruction, rhinorrhea, epistaxis are common presentations
- 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
Prognostic factors
- Locally aggressive with ~50% local recurrence
- Locoregional / distant metastasis occurs in 10 - 20% cases
- 5 year cumulative survival rate is ~40%
- Histologic subtype bears prognostic significance
- Papillary subtype is associated with 80% survival at 5 years, while mucinous and solid subtypes carry poor prognosis (Hum Pathol 1999;30:1140)
- HRAS mutation, chromogranin expression and HER2 / c-erbB-2 expression are associated with aggressive behavior (Cancer 1999;86:255)
- There is no significant prognostic difference between occupational and sporadic tumors
Treatment
- Adequate surgical removal
- Postoperative radiotherapy for advanced cases
Gross description
- Irregular exophytic tan, pink mass bulging into nasal cavity or paranasal sinuses, often with necrotic and friable appearance
- Some lesions are gelatinous
Microscopic (histologic) description
- Two major histologic classifications for ITAC:
- Barnes classification recognizes 5 categories:
- Papillary
- Colonic
- Solid
- Mucinous
- Mixed (Am J Surg Pathol 1986;10:192)
- Kleinasser and Schroeder subdivides ITAC into 4 categories:
- Papillary tubular cylinder cell types I - III (I - well differentiated, II - moderately differentiated and III - poorly differentiated)
- Alveolar goblet type
- Signet ring type
- Transitional type (Arch Otorhinolaryngol 1988;245:1)
- Barnes classification recognizes 5 categories:
- Papillary and colonic types are most common histologic types
- Papillary type: predominantly papillary growth pattern with tubular elements
- Colonic type: tubuloglandular architecture with minor papillary elements; neoplastic cells have palisaded hyperchromatic nuclei and a few goblet cells
- Solid type: poorly differentiated; trabecular and solid proliferation of neoplastic cells
- Mucinous: mucin laden neoplastic glands or tumor cell clusters within pools of extracellular mucin
- Mixed: variable admixture of two or more subtypes
- Goblet cells, Paneth cells and argentaffin cells may be found in all subtypes
- Exceptionally well differentiated ITAC may resemble normal small intestinal mucosa with well formed villi and muscularis mucosae
Microscopic (histologic) images
Contributed by Beverly Wang, M.D.
Colonic type
Focal CK7+
Diffuse nuclear CDX2+
Images hosted on other servers:
Colonic and papillary types
Colonic type
Papillary pattern
Papillary tubular cylinder cell I
Papillary tubular cylinder cell II
Mucinous pattern
Alveolar goblet cell
Various subtypes
CK7-
Various stains
Other diagnoses for comparison:
Intestinal metaplasia of nasal mucosa
Low grade nonintestinal adenocarcinoma
Tubulopapillary low grade (nonintestinal) adenocarcinoma
Positive stains
- Consistently positive for CK20 (73%), CDX2, villin, MUC2 (J Clin Pathol 2004;57:932)
- Variably positive for CK7 and CEA
- Chromogranin+ cells may be present, scattered or in clusters
Molecular / cytogenetics description
- Frequent KRAS and p53 mutations
- Tumors with occupational exposure to wood dust show p53, p14 ARF, p16 INK4a gene deregulation
- EGFR alterations also reported (Cell Oncol (Dordr) 2012;35:443)
Differential diagnosis
- Low grade sinonasal adenocarcinoma: usually not associated with wood dust exposure; "clean" background, may have papillary / tubular architecture; more glandular, less papillary, few columnar / goblet cells
- 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
