ICDO code97343

Extramedullary plasmacytoma can occur in the oral cavity and oropharynx, see Chapter 1 for details.

Langerhans cell histiocytosis

ICD-O code 9751/1

Oral involvement occurs in 10% of patients with Langerhans cell histiocytosis (LCH). 78% of these patients have eosinophilic granulomas clinically, while the rest have multifocal multisystem disease {1021}. Common oral symptoms include swelling, pain, gingivitis, loose teeth and ulceration. The majority of patients with intraoral lesions have intraosseous lesions in the jaw bone, more commonly in the mandible. The intraoral soft tissues may be secondarily affected, especially the gingiva, but the palate, floor of mouth, buccal mucosa and tonsil can also be involved {1021, 2043}. In a minority of patients with intraoral soft tissue involvement, there is no associated bony lesion {241,460,1731}. See chapter 7 for details.

Hodgkin lymphoma

Hodgkin lymphoma (HL) is predominantly a nodal-based disease, and primary extranodal presentation is very rare. When it presents in extranodal tissues, the Waldeyer ring, particularly the palatine tonsil, is a common site {1274,1756}. Most patients present with localized disease (stage I/II), with symptoms of chronic tonsillitis or tonsillar enlargement, with or without enlarged cervical lymph nodes. Other reported sites include the oropharynx {44,1756}, alveolar crest of mandible {1659}, and maxillary gingiva {2554}.

Most cases represent classical HL, as detailed in the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues {1197}, frequently of mixed cellularity subtype and showing strong association with Epstein-Barr virus (EBV) {1274}, although nodular lymphocyte predominant HL may also rarely present in the Waldeyer ring (palatine and lingual tonsils) {391,1274}.

Extramedullary myeloid sarcoma

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