Lymph node metastases are a poor prognostic factor for patients with malignant tumors of the paranasal sinuses. The incidence of these metastases is low, particularly in ethmoid sinus tumors. A prophylactic treatment of the neck in patients with N0 tumors (surgery or radiotherapy) might be considered in T2 squamous cell carcinoma of the maxillary sinus and in undifferentiated carcinoma of the ethmoid sinus.

Intestinal-type adenocarcinoma (ITAC) of the nasal cavity and paranasal sinuses is an uncommon tumor associated with occupational exposure to dusts of different origin. Few investigations addressed molecular alterations in ITAC mainly focused on TP53, K-ras and H-ras gene mutations. The occurrence of TP53, p14 ARF Intestinal-type adenocarcinoma (ITAC) of the nasal cavity and paranasal sinuses is an uncommon, professional-related tumor characterized by high local aggressiveness and ominous outcome. 1 ITAC encompasses a neoplasm group showing a range of microscopic features spanning from tumors indistinguishable from typical colonic adenocarcinoma to colloid or signet-ring cell carcinoma of the colon. 2 Surgery is, and still remains, the treatment of choice for this tumor although it has been complemented recently by primary chemotherapy. 3 ITAC clearly predominates among males and exhibits an extreme gender distribution likely to be related to an occupational exposure. Several epidemiologic studies pointed out the association of ITAC with professional exposure primarily to wood or leather dust. 4 -8 Dusts of different origin are other potential risk factors for sinonasal adenocarcinomas, including textile, 9,10 cereal or cement dust. 11 The close relationship between professional exposure and ITAC strongly suggests that dusts and chemical elements may be implicated as etiological agents in the tumorigenesis of this tumor. Investigations into the genotoxic action of several substances present in the wood or used by wood-and leather-workers showed the combined genotoxic effects of dusts and chemicals. 7,8 A variety of chemical carcinogens cause mutations in human tumors by forming covalent adducts that increase the probability of errors during DNA replication. 12 Some carcinogenic agents may target oncogenes and tumor suppressor genes producing specific types and locations of DNA alterations. These carcinogen-induced mutational spectra are influenced by the specific DNA sequence. 13 Previous studies showed that methylated CpG dinucleotides may represent preferential targets for mutagens. 14,15 A strong and selective formation of both adducts and mutations involving CpG islands of the TP53 gene is frequent in human tumors 16,17 and an association between DNA methylation and exposure to carcinogens, as tobacco 18 and vinyl chloride 19 has been reported recently.In addition, loss of heterozygosity (LOH) at chromosomal loci encoding the TP53, p14 ARF and p16 INK4a genes represents a further molecular event related to carcinogenic exposure, as found in tobacco smoke-associated larynx cancer. 20 Moreover, TP53 mutations and p16INK4a deletion or hypermethylation correlate with early genetic changes in malignancy development of the Barrett's esophagus 21,22 and frequently occur as late events in colorectal adenocarcinoma. [23][24][25] Few investigators addressed molecular alterations in ITACs. In the assumption that morphologic resemblance with colorectal cancer might reflect equivalent genetic alterations, Wu et al. 26 ...

Ethmoid malignant tumours are rare, but nearly all at least approach or involve the lamina cribrosa. An anterior craniofacial resection is almost always mandatory for a radical resection. While almost everything has been written about technical details, few studies reported meaningful analysis about prognostic factors and long-term results, for a series of reasons: the infrequency of these tumours, the variety of histologies, small patients cohorts presented by each author, a medley of untreated and pre-treated patients, the lack of a universally accepted classification. We perform a review of the literature in the light of our experience of 330 anterior craniofacial resections for ethmoid malignant tumours. We present our classification of ethmoid malignant tumours (called INT, Istituto Nazionale Tumori). It turned out to be more prognostic than AJCC-UICC classification.

Both the 1997 and 2002 AJCC-UICC classifications seemed to have limited prognostic value. By contrast, the INT classification satisfied one of the main goals of tumor staging, demonstrating the progressive worsening of prognosis with different tumor classes.

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Genetic testing is the appropriate procedure in MEN 2A syndrome for the early diagnosis of medullary carcinoma even at a preclinical stage. Prophylactic total thyroidectomy represents the standard preventive and therapeutic surgical approach in the treatment of medullary thyroid carcinoma in MEN 2A syndrome. Our experience has confirmed the presence of CCH and medullary thyroid carcinoma even in clinically negative patients, in agreement with reports in the literature.