Department of Pathology, Hospital San Gerardo, University of Milan-Bicocca, Monza, Italy.
Anticancer Res. 2010 Jul;30(7):3009-14.
Oral amyloidosis is a rare and debilitating disease that, whether primary or secondary, may severely impact the quality of a patient's life. The study investigated the characteristics of amyloid deposition in the tongue from the clinical and histopathological profiles.
Biopsy specimens were received from five patients: 2 female, 3 male. All biopsies were taken from the tongue, and all had amyloid deposition in the subepithelial connective tissue, conclusive for a diagnosis of amyloidosis. All patients showed macroglossia and difficulty in eating and impairment of speech.
In three cases there was no evidence of systemic involvement or associated disease; these were characterized as localized amyloidosis of the tongue. The other two cases revealed multisystemic involvement. Histologically, the disease was diagnosed through specific staining with Congo red, which examined under polarized light revealed the amyloid deposits as apple-green birefringence.
The findings show the tongue to be the site most frequently affected in forms of localised amyloidosis, and that a tongue biopsy possess a highly diagnostic value for amyloidosis. There is still no consensus regarding the management of lingual amyloidosis, although numerous therapies have been proposed, including surgical excision and pharmacological treatment. However lesions often persist or recur. The prognosis is uncertain, owing to the rarity of the condition, requiring regular follow-up and monitoring.
口腔淀粉样变是一种罕见且使人虚弱的疾病,无论是原发性还是继发性,都可能严重影响患者的生活质量。本研究从临床和组织病理学特征方面调查了舌部淀粉样沉积物的特征。
共接收了五名患者的活检标本:2 名女性,3 名男性。所有活检均取自舌部,所有标本的上皮下结缔组织中均有淀粉样沉积物,可明确诊断为淀粉样变。所有患者均表现为巨舌症,伴有吞咽困难和言语障碍。
其中三例无系统性受累或相关疾病的证据,这些患者被诊断为舌部局限性淀粉样变。另外两例则显示多系统受累。组织学上,通过刚果红的特异性染色诊断该病,在偏振光下检查显示淀粉样沉积物呈苹果绿双折射。
研究结果表明,舌部是局限性淀粉样变最常受累的部位,舌活检对淀粉样变具有高度诊断价值。尽管已经提出了许多治疗方法,包括手术切除和药物治疗,但对于舌部淀粉样变的治疗仍未达成共识。然而,病变往往持续存在或复发。由于该病罕见,预后不确定,需要定期随访和监测。