Nasal cavity, paranasal sinuses, nasopharynx
Sinonasal adenocarcinoma
Nonintestinal type
Editorial Board Member: Bin Xu, M.D., Ph.D.
Deputy Editor-in-Chief: Kelly Magliocca, D.D.S., M.P.H.
Last author update: 7 May 2024
Last staff update: 7 May 2024
Copyright: 2004-2024, PathologyOutlines.com, Inc.
PubMed Search: Nonintestinal type sinonasal adenocarcinoma
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Dutta R, Mannan R. Nonintestinal type. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalLGseromucinous.html. Accessed November 28th, 2024.
Definition / general
- Malignant tumor showing glandular differentiation, with presence of intraluminal mucin (in most cases)
Essential features
- Gland forming malignancy of the sinonasal tract
- Morphologically, lacks intestinal type features or characteristics of a salivary gland specific neoplasm
- Histologically divided into 2 grades: low grade (LG) and high grade (HG)
- Sinonasal renal cell-like adenocarcinoma (SRCLA) constitutes a definite subtype
- ~25% of LG sinonasal adenocarcinoma cases recur (Cancer 1982;50:312)
- HG sinonasal adenocarcinoma fares worse with reports of occasional metastases
Terminology
- Terminal tubulus adenocarcinoma
- Tubulopapillary low grade adenocarcinoma
- Low grade adenocarcinoma
- Seromucinous adenocarcinoma
ICD coding
- ICD-11
- 2C20.0 & XH74S1 - adenocarcinoma of nasal cavity & adenocarcinoma, NOS
- 2C22.0 & XH74S1 - adenocarcinoma of paranasal sinuses & adenocarcinoma, NOS
Epidemiology
- LG sinonasal adenocarcinoma is uncommon and does not exhibit any sex predilection
- HG sinonasal adenocarcinoma is rarer and affects men more frequently
- Both LG and HG sinonasal adenocarcinoma occur over a wide age range, with a peak in the sixth decade
Sites
- LG sinonasal adenocarcinoma arises most frequently in the middle turbinate (64%), followed by ethmoid sinus (20%) and other individual sinuses (Am J Surg Pathol 2009;33:401)
- ~50% of all HG sinonasal adenocarcinoma cases are locally advanced at presentation with involvement of multiple sites (Am J Surg Pathol 2011;35:971)
Pathophysiology
- Exact pathogenesis of HG sinonasal adenocarcinoma is still unknown
- Some LG sinonasal adenocarcinoma cases have been found to be enriched in ETV6::NTRK3 / ETV6::RET fusions and CTNNB1 mutations (Am J Surg Pathol 2017;41:1552, Case Rep Pathol 2018;2018:8741017)
Etiology
- Exact etiology of LG sinonasal adenocarcinoma is yet to be unveiled
- HG sinonasal adenocarcinoma may be related to exposure to carcinogens (such as wood dusts and other solvents) and occasionally also harbors association with high risk human papillomavirus (HPV) (Am J Surg Pathol 2011;35:971)
Clinical features
- Nasal obstruction is most common presenting symptom
- Patients harboring HG sinonasal adenocarcinoma additionally also present with epistaxis, pain, deformity and proptosis
Diagnosis
- Imaging followed by biopsy
Radiology description
- Nasopharyngeal endoscopy typically shows soft tissue mass
- Bone destruction can be seen in HG sinonasal adenocarcinoma
- Mucin producing adenocarcinoma shows hyperintensity on T2 weighted images and exhibits gradual enhancement on contrast enhanced T1 weighted images
- Adenocarcinoma without mucin production shows isointensity to hypointensity on T2 weighted images (J Clin Med 2017;6:116)
Radiology images
Images hosted on other servers:
Heterogeneously hyperintense lesion on T2 weighted image
Prognostic factors
- ~25% of LG sinonasal adenocarcinoma cases recur; 6% of patients die due to tumor related morbidity (Cancer 1982;50:312)
- Most patients of HG sinonasal adenocarcinoma die within 5 years, with evidence of local and distal metastasis
- Few reported cases of SRCLA have recurred or have evidence of metastasis (Head Neck Pathol 2008;2:75)
Case reports
- 29 year old woman presented with a round polypoid mass at the posterior end of nasal septum (Ear Nose Throat J 2022 Aug 10 [Epub ahead of print])
- 35 year old African American man with von Hippel-Lindau (VHL) syndrome who developed SRCLA (Oral Oncol 2022;125:105705)
- 39 year old woman and 39 year old man with HG sinonasal adenocarcinoma exhibiting a distinct morphological and immunohistochemical phenotype and harboring ETV6::NTRK3 fusion (Virchows Arch 2023;483:187)
- 67 year old woman presented with a nasopharyngeal mass with an accompanying parapharyngeal mass (Curr Oncol 2017;24:e55)
Treatment
- No standardized treatment algorithm; however, surgery followed by radiation is a mainstay in management
- Tumor free resection margin is of utmost importance (World Neurosurg 2018;120:e962)
Clinical images
Images hosted on other servers:
Eustachian tube tumor
Gross description
- Variable; however, usually appears as a red, polypoidal mass or firm, raspberry-like growth
Microscopic (histologic) description
- LG sinonasal adenocarcinoma
- Papillary and glandular growth patterns (Cancer Manag Res 2021;13:1271)
- Complex papillary structures with fibrovascular cores
- Glandular (tubular) growth pattern has cribriform or back to back glands with little intervening stroma (Cancer 1982;50:312)
- Lining mucinous cuboidal to columnar epithelial cells have eosinophilic cytoplasm with uniform basally located rounded nuclei with finely granular chromatin
- Intraluminal mucin or material present
- Mitotic figures are rare
- Necrosis is absent
- Invasion into deeper soft tissue as well as into bone may be present
- Calcospherites and squamous morules may sometimes be seen (Case Rep Pathol 2018;2018:8741017)
- Papillary and glandular growth patterns (Cancer Manag Res 2021;13:1271)
- HG sinonasal adenocarcinoma
- Exhibits more diversity in histomorphological patterns (Am J Surg Pathol 2011;35:971)
- Predominately solid or nested growth pattern
- Dispersed glandular structures or small cysts with papilla projecting into them
- Cells have intracytoplasmic mucin
- Destructive infiltrative growth pattern with invasion into bone
- Brisk mitotic activity with foci of necrosis
- SRCLA
- Histological subtype reminiscent of renal cell carcinoma
- Tubulopapillary proliferation of tumor cells
- Predominately composed of polygonal cells, with clear or slightly eosinophilic cytoplasm (Head Neck Pathol 2008;2:75, Hum Pathol 2015;46:1598, Head Neck Pathol 2017;11:333)
- Tumor cells are glycogen rich, lacking mucin production
- Small, uniform, round nuclei with presence of intranuclear inclusions commonly
- Perineural or lymphovascular invasion, necrosis or severe pleomorphism are not seen
Microscopic (histologic) images
Contributed by Diana Bell, M.D. and Wai Szeto, M.D., M.S.
Low grade sinonasal adenocarcinoma
Tumor infiltrating
underlying stroma
Tubular growth pattern
High grade sinonasal adenocarcinoma
Nests of closely packed glands
Tumor necrosis
CK7
SOX10
Sinonasal renal cell-like adenocarcinoma
Tumor cells arranged as nests
Mimics renal cell carcinoma
Clear cytoplasm
CAIX
Positive stains
- PAS (intracytoplasmic, diastase resistant granules), mucin stains, such as mucicarmine (intracellular or luminal)
- Pancytokeratin (diffuse), CK7 (diffuse), p40 (focally, in few cases) (Virchows Arch 2003;443:152)
- Subset of cases can also express DOG1, SOX10 and S100 protein (Head Neck Pathol 2015;9:436)
- LG sinonasal adenocarcinoma: beta catenin (nuclear localization) (Case Rep Pathol 2018;2018:8741017)
- HG sinonasal adenocarcinoma: focal expression of neuroendocrine antigens, such as synaptophysin, chromogranin, CD56 (Am J Surg Pathol 2011;35:971)
- SRCLA: carbonic anhydrase IX (CAIX) and CD10 (diffuse) (Hum Pathol 2015;46:1598)
Negative stains
- CDX2, SATB2, villin, MUC2, CK20 (infrequently positive)
- HG sinonasal adenocarcinoma: beta catenin and mismatch repair (MMR) protein (wild type expression) (Mod Pathol 2005;18:315)
- SRCLA: PAX8 or renal cell carcinoma (RCC) antibody
Molecular / cytogenetics description
- No defined diagnostic molecular alterations to date
- Some LG sinonasal adenocarcinoma cases have been found to be enriched in ETV6::NTRK3 / ETV6::RET fusions or CTNNB1 mutations (Am J Surg Pathol 2017;41:1552, Case Rep Pathol 2018;2018:8741017)
- Occasional BRAF mutations (Pathol Oncol Res 2014;20:571)
- No KRAS mutation
- SRCLA: a couple of cases reported in patients with von Hippel-Lindau (VHL) syndrome (Oral Oncol 2022;125:105705)
Sample pathology report
- Middle turbinate mass, biopsy:
- Invasive adenocarcinoma (low grade), nonintestinal type (see comment)
- Comment: The patient's clinical history of a mass in the middle turbinate is noted. Microscopic examination reveals complex, interwoven papillary architecture along with crowded back to back glands. The lining mucinous cuboidal to columnar epithelial cells have eosinophilic cytoplasm with uniform, basally located, rounded nuclei with finely granular chromatin. Occasional mitotic figures are present. Necrosis is absent. Lymphovascular or perineural invasion is not identified. On immunohistochemistry, the tumor cells are immunopositive for pancytokeratin and CK7 while negative for CK20, CDX2 and SATB2. The findings are consistent with low grade nonintestinal type sinonasal adenocarcinoma.
Differential diagnosis
- Sinonasal intestinal type adenocarcinoma:
- Metastatic papillary thyroid carcinoma:
- No surface epithelial dysplasia
- PAX8, TTF1 and thyroglobulin positive (Head Neck Pathol 2019;13:661)
- Sinonasal papilloma, oncocytic type:
- Noninvasive papillary epithelial neoplasm
- Mixed exophytic and endophytic growth patterns
- Multiple layers of cuboidal to columnar cells
- Abundant oncocytic cytoplasm, scattered admixed ciliated or mucous columnar cells
- Intraepithelial neutrophilic inflammation, microcysts with mucin or neutrophilic microabscesses are characteristic
- No epithelial dysplasia
- Respiratory epithelial adenomatoid hamartoma (REAH):
- Associated with allergy and inflammatory disorders
- Predilection for posterior nasal septum
- Lobular / polypoid growth pattern
- Proliferation of medium sized, elongated to rounded glands that expand from surface epithelium downward into the stroma
- Absence of complex glandular architecture, true invasion or dysplasia (Ear Nose Throat J 2021;100:495S)
Board review style question #1
The H&E images shown above depict a mass in the middle turbinate of a 45 year old man. Based on these images, what is the diagnosis?
- Respiratory epithelial adenomatoid hamartoma (REAH)
- Sinonasal nonintestinal type adenocarcinoma
- Sinonasal papilloma, oncocytic type
- Sinonasal renal cell-like adenocarcinoma
Board review style answer #1
B. Sinonasal nonintestinal type adenocarcinoma. The pictures show a tumor arranged as glands and interwoven papillae and infiltrating the underlying stroma. These features are consistent with a sinonasal nonintestinal type adenocarcinoma. Answers A and C are incorrect because these neoplasms lack true invasion into the underlying stroma. Answer D is incorrect because sinonasal renal cell-like adenocarcinoma is predominately composed of polygonal cells with clear cytoplasm, resembling a renal cell carcinoma.
Comment Here
Reference: Nonintestinal type sinonasal adenocarcinoma
Comment Here
Reference: Nonintestinal type sinonasal adenocarcinoma
Board review style question #2
Regarding sinonasal renal cell-like adenocarcinoma (SRCLA), which of the following statements is true?
- Carbonic anhydrase IX (CAIX) and CD10 are diffusely immunopositive
- PAX8 is immunopositive in this tumor
- Renal cell carcinoma (RCC) marker is immunopositive
- This tumor has high rate of metastasis
Board review style answer #2
A. Carbonic anhydrase IX (CAIX) and CD10 are diffusely immunopositive. Answers B and C are incorrect because unlike renal cell carcinomas, PAX8 (which is a transcription factor involved in development of the kidney) and RCC immunomarkers are immunonegative in SRCLA. Answer D is incorrect because no reported cases have metastases to date.
Comment Here
Reference: Nonintestinal type sinonasal adenocarcinoma
Comment Here
Reference: Nonintestinal type sinonasal adenocarcinoma
