Systemic Disease Affecting Waldeyers Ring

Tangier's disease (familial hypo-alpha-lipoproteinemia) is an autosomal-recessive metabolic disease with a deficiency of high-density lipoproteins and extremely low levels of plasma cholesterol [85]. The deranged fat metabolism results in storage of cholesterol esters in the reticuloendothelial system and macrophages of the pha-ryngeal and gastrointestinal tract mucosa, but also in smooth muscle cells, pericytes and Schwann cells of pe ripheral nerves. Clinically, most patients are asymptomatic, but children with Tangier's disease have enlarged, hyperplastic palatine and pharyngeal tonsils with yellow-orange or yellow-grey discoloration. Histologically, large groups and accumulations of macrophages with foamy cytoplasm can be identified in the palatine and pharyngeal tonsils [6, 48].

Amyloidosis is usually a systemic disease of mul-tifactorial origin that may involve the head and neck area. Particularly the upper respiratory tract is commonly affected by amyloidosis with a symmetrical enlargement of the tongue. Small amounts of amyloid deposition in Waldeyer's ring have been described in plas-macytomas, nasopharyngeal carcinomas or tonsillitis. Isolated tumour-like involvement of the nasopharynx with and without immunoglobulin light chain restriction, of the entire Waldeyer's ring or the palatine tonsils without systemic disease is exceptionally rare [11, 45, 116, 156].

Involvement of the naso- and oropharynx by systemic s arcoidosis is well documented [127]. Unsuspected isolated sarcoidosis of the palatine and pharyn-geal tonsils in the absence of systemic disease is very rare [46, 126]. Histologically, the sarcoidosis granulomas are composed of densely packed epithelioid histiocytes and macrophages without central necrosis. Differential diagnoses include recurrent tonsillitis, which may feature giant cells and even foreign body giant cell granulomas. Another differential diagnosis consists of infections with mycobacterium tuberculosis and formation of caseating tuberculoid granulomas. The majority of patients with pulmonary tuberculosis have nasopharyngeal involvement, but isolated nasopharyngeal tuberculosis is rare [2, 161, 193].

Metastases from primary tumours outside the head and neck area to the naso- and oropharynx are exceptionally rare, because the nasopharyngeal and palatine tonsils have no afferent lymph vessels. Consequently, most metastatic tumours in tonsils represent haema-togenous deposits. Bilateral metastases to the palatine tonsils have been described for pancreatic carcinoma [131]. Other metastatic tumours to the palatine tonsil include carcinomas of the breast, lung, stomach, colon, prostate, skin and kidneys [173]. Renal cell carcinomas are known to metastasise to the nasopharynx [169].

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