A. Franchi M. Santucci

B.M. Wenig

These are glandular malignancies of the slnonasal tract, excluding defined types of salivary gland carcinoma. Two main categories are recognized: (1) intestinal-type adenocarcinoma, and (2) non-intestinal-type adenocarcinoma, which can be further divided into low-grade and high-grade subtypes. Overall, adenocarcinomas and salivary-type carcinomas comprise 10-20% of all sinonasal primary malignant tumours.

Intestinal-type adenocarcinomas


A primary malignant glandular tumour of the nasal cavity and paranasal sinuses histologically resembling adenocarcinoma or adenoma of the intestines, or exceptionally normal small intestinal mucosa.

ICD-O code 8144/3


Colonic-type adenocarcinoma, enterictype adenocarcinoma.


The frequency of intestinal type adenocarcinomas (ITACs) among primary sinonasal malignancies is difficult to ascertain. Most series report a pronounced male predominance, possibly because of occupational exposure. Patients have ranged in age from 12 to 86 years at the time of diagnosis (mean 58 years) {124}.


The causal relationship of wood dust and leather dust with the development of sinonasal ITACs has been established by several epidemiological studies from different countries {1594}. In this setting, dust particle size is important because those smaller than 5 |jm reach the lower respiratory tract, while larger particles are accumulated in the nasal mucosa. However, the carcinogens involved in the onset of ITACs in wood workers and leather workers have not yet been clearly identified. Biologically active substances which can be present in wood and leather dusts include alkaloids, saponins, stilbenes, aldehydes, quinones, flavonoids, resins, oil, steroids, terpenes, fungal proteins, and tannins {1341}.

Association has also been reported for agricultural workers, food manufacturers, and motor-vehicle drivers among men, and for textile occupations among women {1443}.


ITACs Involve the ethmoid sinus, nasal cavities and maxillary sinus In approximately 40%, 27% and 20% of cases, respectively. In the nasal cavities, the inferior and middle turbinates are the sites of predilection. For larger destructive lesions it may be impossible to ascertain the exact site of origin. Advanced tumours tend to invade the orbit, the pterygopalatine and infratemporal fossae, and the cranial cavity. About 10% of cases show lymph node involvement at presentation {124,1341, 2234}.

Clinical features

Most patients present with unilateral nasal obstruction, rhinorrhea and epistaxis. Advanced tumours may cause pain, neurologic disturbances, exoph-thalmos and visual disturbances.


Computed tomography (CT) and magnetic resonance imaging (MRI) are used for diagnosis of early lesions, defining the extent of disease and detection of early recurrence. CT best shows sites of bone destruction, while MRI best delineates soft tissue extension {1537}.

Fig. 1.11 Intestinal-type adenocarcinoma A Well differentiated intestinal type adenocarcinoma shows a papillary growth pattern and occasional tubular glands. B Higher power view of a moderately differentiated intestinal type adenocarcinoma, showing glandular structures formed by cylindrical and goblet cells.

Fig. 1.11 Intestinal-type adenocarcinoma A Well differentiated intestinal type adenocarcinoma shows a papillary growth pattern and occasional tubular glands. B Higher power view of a moderately differentiated intestinal type adenocarcinoma, showing glandular structures formed by cylindrical and goblet cells.

Fig. 1.12 Intestinal-type adenocarcinoma A Mucinous intestinal-type adenocarcinoma invading bone. The tumour has an alveolar architecture and strands of neoplastic cells with clear mucus-containing cytoplasm are present within mucus pools. B Mucinous intestinal-type adenocarcinoma showing small glands and solid islands floating in abundant mucous substance. C Mucinous intestinal-type adenocarcinoma formed by signet ring cells.


ITACs present as an irregular exophytic pink or white mass bulging in the nasal cavity or paranasal sinus, often with a necrotic friable appearance. Some lesions are gelatinous.


Two classifications of ITACs have been proposed. Barnes divided these tumours into 5 categories: papillary, colonic, solid, mucinous and mixed. Kleinsasser and Schroeder divided ITACs into four categories: papillary tubular cylinder cell (PTCC) types I-III (I = well-differentiated,

II = moderately-differentiated, III = poorly-differentiated) {799,804,1333}, alveolar goblet type, signet-ring type and transitional type. Either classification is acceptable, but for simplicity the Barnes classification is preferred and will be the one utilized in this description. The most common histologic types seen in association with wood workers as well as in sporadic cases are the papillary and colonic types {124,1333}.

The papillary type (papillary tubular cylinder cell I or well-differentiated ade-nocarcinoma), which accounts for approximately 18% of cases, shows a predominance of papillary architecture with occasional tubular glands, minimal cytologic atypia, and rare mitotic figures. The colonic type (papillary tubular cylinder II or moderately-differentiated adeno-carcinoma), representing approximately 40% of cases, shows a predominance of tubulo-glandular architecture, rare papillae, increased nuclear pleomorphism and mitotic activity.

The solid type (papillary tubular cylinder

III or poorly-differentiated adenocarcino-ma), representing approximately 20% of cases, shows a loss of differentiation, characterized by solid and trabecular growth with isolated tubule formation, marked increase in number of smaller cuboidal cells with nuclear pleomor-phism, round vesicular nuclei, prominent nucleoli and increased mitotic figures. Analogous to colonic adenocarcinoma, some ITACs are predominantly comprised of abundant mucus and are classified as the mucinous type. The muci-nous type (alveolar goblet cell and signet ring) includes two growth patterns. In one pattern, there are solid clusters of cells, individual glands, signet ring cells, short papillary fronds with or without fibrovascular cores; mucin is predominantly intracellular and a mucomyxoid matrix may be present. The other pattern shows the presence of large, well-formed glands distended by mucus and extracellular mucin pools {799,804,1333}. In the latter type, pools of extracellular mucin are separated by thin connective tissue septa creating an alveolar type pattern. Predominantly cuboidal or goblet tumour cells are present in single layers at the periphery of mucus lakes.

Mucus extravasation may elicit an inflammatory response that can include multin-ucleated giant cells. {799}. The mixed type (transitional) is composed of an admixture of two or more of the previously defined patterns. Irrespective of the histologic type, ITACs histologically simulate normal intestinal mucosa and may include villi, Paneth cells, enterochromaffin cells and muscu-laris mucosae {1739}. In rare instances, the tumour is so well differentiated that it is composed of well-formed villi lined by columnar cells resembling normal resorptive cells; in some cases, bundles of smooth muscle cells resembling mus-cularis mucosae may also be identified under the villi.


ITACs are diffusely positive for epithelial markers including pancytokeratin, epithelial membrane antigen, B72.3, Ber-EP4, BRST-1, Leu-M1, and human milk fat globule (HMFG-2) {1687}. They show CK20 positivity (73%) and variable CK7

Table 1.1 Classification and survival of intestinal-type adenocarcinoma

Barnes {124}

Klesinsasser and Schroeder {1333}

3-year cumulative survival11





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