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Fig. 7.8. Amyloidosis of the larynx. a Scattered reddish masses of ing. b The same field, green birefringence of the amyloid with po-amyloid are seen in the subepithelial stroma by Congo red stain- larised light

Grossly, the affected area of the larynx is swollen, sometimes polypoid, covered by an intact mucosa. On a cut surface, it is firm, pale, waxy, tan-yellow to red-grey [359]. Microscopically, H&E staining shows the deposition of an amorphous, eosinophilic, hyaline, extracellular substance in the subepithelial stroma, blood vessel walls, and along the basement membranes of seromuci-nous glands, with intact covering epithelium. The deposits may be discrete, or may appear as large rounded masses of variable size. They stain with Congo red and display green birefringence with polarised light; this property remains the diagnostic gold standard (Fig. 7.8). Immunohistochemical analysis must also be performed to determine the amyloid type [131].

Most patients can be successfully treated by a conserving surgical excision to preserve laryngeal function for as long as possible. In some patients, multiple procedures may be necessary, and recurrences may occur. A fatal outcome has been described in patients with progressive tra-cheobronchial involvement, but association with systemic amyloidosis is rare [59]. Clinical examination is advised to exclude the possibility of a systemic disease.

Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease, is an idiopathic, relatively rare benign lesion, based on a nodal and/or extranodal histiocytic proliferative disorder that usu ally resolves spontaneously. The most frequent clinical manifestation of the disease is cervical, bilateral and painless lymphadenopathy. However, extranod-al sites may also be involved and the head and neck region is one of the most commonly affected areas [381]. Extranodal disease may be the initial or sometimes the sole manifestation of the disease. Foucar and co-workers reported that 43% of patients had at least one site of extranodal location of SHML [118]. Within the head and neck, the nasal cavity, paranasal sinuses and orbit are commonly involved. Some cases of laryngeal lesions have been reported too [5, 11, 52, 66, 118, 207]. Typically, the SHML begins insidiously and progresses to a protracted course of the active stage, and ends with spontaneous remission. SHML occasionally appears with subsequent recurrence and serious consequences, occasionally even death, if vital organs are affected. Exclusive extranodal disease is more frequent in elderly persons [51]. Laryngeal SHML usually manifests as a circumferential narrowing or polypoid mucosal lesion of a tan-white to yellow appearance. Vocal cord involvement results in impaired mobility [51].

Histologically, the laryngeal mucosa is almost diffusely infiltrated by lymphocytes, plasma cells, neutrophils and clusters of histiocytes. Histiocytes are of various sizes, with a focally vacuolated pale to pink cytoplasm, but ill-defined borders. Their nuclei are round or oval, sometimes vesicular, with well-defined central nucleoli. Lympho- and granulophagocytosis is evident in the cytoplasm of these histiocytes; the phenomenon is termed emperipolesis. Immunohistochemical-

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