Metastases to the Nasal Cavity and Paranasal Sinuses
Definition
A malignant deposit emanating from a site outside the nose and paranasal sinuses. The majority derive from below the clavicle.
Etiology
Distant metastasis from sinonasal cancer is a relatively rare occurrence except in the terminal stages of the disease,1 but primaries from elsewhere in the body occasionally spread via the bloodstream into the sinonasal region and may be the first indication of the problem.
Incidence
Perls in 1872 first described such a lesion, which involved the sphenoid and arose from the bronchus.2 A thyroid secondary was reported by Von Eiselberg in 1893, and the first secondary from a renal tumor was reported by Albrecht in 1905.3,4 The frequency with which they occur is difficult to estimate with any accuracy but they appear to be exceptionally rare. This has been attributed to the paucity of bone marrow or lymph nodes in this area, which are thought to be prerequisites for metastases to occur.5 The mandible is a much commoner site than the maxilla (93:20 in Batsakis and McBurney′s study5). Hematogenous spread is thought to occur via the vertebral venous plexus or via (occult) lung metastases.6
In our series of 1,635 malignant sinonasal tumors, we have seen 10 cases (Table 17.1).
Site
The most frequent source of sinonasal metastases is renal cell carcinoma to the maxilla and ethmoid sinuses.7 It is estimated that 8% of renal carcinomas present with secondaries in the head and neck, of which half affect the sinonasal region. They made up 80% in Friedmann and Osborn′s series of sinonasal metastases, although they were around 42% of those reported in the past 140 years.8 Tumors from many other sites can also do this—lung, breast, thyroid, gastrointestinal tract, prostate, pancreas, adrenal gland, liver, and skin malignant melanoma (Table 17.2).
The most frequent site for metastases is the maxilla, followed by ethmoid, nasal cavity, and frontal with the sphenoid sinuses least affected. Occasionally the alveolar ridge and palate can be affected. Very rarely they can be bilateral.6,59,89
Diagnostic Features
Clinical Features
There are no particular features other than epistaxis (especially with renal tumors19), nasal obstruction, and pain, followed by a mass or facial swelling and various orbital and neurologic symptoms. These symptoms predate those from the primary in 50% of cases by up to 6 years90 but can also occur some time after the primary has been treated,20,91,92 between 10 months and 17 years later. In our group, half presented with the secondary and an unsuspected primary that manifested itself up to 8 months later, and in the other 5 the secondary appeared up to 9 years after the originating primary was diagnosed (Table 17.1). The deposit may be associated with cervical lymphadenopathy.89,93 In the literature a wide age range is reported of 17 months to 80 years and the metastatic deposit can be solitary in the sinonasal region or part of more systemic spread.
Imaging
Imaging shows a generally destructive process with bone erosion and spread into the orbit and anterior cranial fossa (Fig. 17.1a). For some, such as intestinal adenocarcinoma, areas of calcification may be seen. In the case of prostatic carcinoma (and sometimes breast), an osteosclerotic process may be seen as well as osteolytic lesions, and the deposit may be in the frontal bone adjacent to the sinus (Fig. 17.1b).
Once a metastasis is suspected, full-body screening is needed using CT of the thorax and abdomen and/or PETCT if available.
Histological Features and Differential Diagnosis
A low threshold for the possibility of a metastasis on the part of the histopathologist is the most important thing. In the case of renal carcinoma, the appearances are quite characteristic, with clusters of large clear vacuolated cells with a high glycogen content. The stroma is vascular, hence the epistaxis. Immunohistochemical confirmation of lipid and glycogen is useful. Cytoplasmic inclusions and microvilli can be seen on electron microscopy.
Resto et al94 looked for an immunohistochemical distinction between intestinal-type sinonasal adenocarcinoma and metastatic adenocarcinoma of intestinal origin. In a small number of cases they showed that tumors with CK7+, CK20+, MUC2+ immunophenotype are more likely to be primary sinonasal tumors, whereas tumors with the CK7−, CK20+, MUC2+ profile may be metastases and require further investigation for another primary.
Clear cell tumors may mimic acinic cell carcinoma.
Natural History
In unsuspected cases, the sinonasal lesion may be treated, only for the actual primary to declare itself later. We have had this experience on two occasions with pancreatic adenocarcinomas that declared themselves respectively 4 and 8 months after craniofacial resection in the early part of our series. This raises the question whether it is cost-effective to do full-body screening for all cases of adenocarcinoma not arising in a woodworker.
Treatment and Outcome
Treatment of a metastasis is usually palliative, but with modern surgical and medical oncology, surgical resection, be it endoscopic and even craniofacial resection, together with targeted chemoradiotherapy in addition to the treatment of the primary, may provide a useful period of survival as long as it is without significant morbidity.95 Having said this, in the past average survival with secondaries affecting the maxillary sinus was reported as 20 months90 and in most of the case reports no follow-up is given. Survival as short as 1 or 2 months is given after diagnosis of metastases from breast, stomach, and bladder, whereas longer periods of 24 months or more can be seen with kidney, bronchus, and adrenal gland. In our 10 cases, the outcome was uniformly poor with all dying of disease within a few months of diagnosis (Table 17.1).