Hodgkins Lymphoma

ICD-O:9650/3

Although exceptionally rare in extranodal sites, primary Hodgkin's lymphoma does occur in Waldeyer's ring and nasopharynx. In a review of 659 upper respiratory tract lymphomas, 6 cases of Hodgkin's lymphoma in Waldeyer's ring were identified [35, 103]. The WHO divides Hodgkin's lymphomas into the nodular lymphocyte predominant Hodgkin's lymphoma and the classical Hodgkin's lymphoma (subdivided into lymphocyte-rich, nodular sclerosis, mixed cellularity and lymphocyte-depleted Hodgkin's lymphoma) [95]. Waldeyer's ring Hodgkin's lymphomas belong predominantly to the classical subtypes: seven primary cases of nodular lymphocyte-rich Hodgkin lymphoma were reported in two studies with a total of 27 patients [25, 179]. In another series with 16 patients with Waldeyer's ring Hodgkin's lymphoma, 50% were classified as mixed cellularity, 25% as nodular sclerosis, one case was a nodular lymphocyte predominant subtype and three were unclassified [103]. Epstein-Barr virus has been postulated to play a pathogenetic role in Hodgkin's lymphoma since it has been demonstrated in the majority of Hodgkin's lymphomas of Waldeyer's ring at a higher incidence than in nodal Hodgkin's lymphomas [81, 103]. In addition, patients often had a history of infectious mononucleosis. Therapy is local irradiation with or without chemotherapy.

ICD-O:9719/3

The extranodal NK/T-cell lymphoma, nasal type (or angiocentric T-cell lymphoma in the REAL classification; other historical names: lethal midline granuloma, malignant midline reticulosis, angiocentric im-munoproliferative lesion) shows a predilection for the nasal cavity, nasopharynx and palate, but also occurs in skin, soft tissues, gastrointestinal tract and testis. The denominator "nasal type" indicates that the nasal cavity is the most common and prototypic site of involvement (Chap. 2). Some cases can be accompanied by secondary lymph node involvement. Rapid systemic dissemination is common, but bone marrow involvement is very rare. NK/T-cell lymphoma is more common in Asia, Mexico and South America than in Europe and North America, and shows a strong association with Epstein-Barr virus [24, 65, 99, 135, 142, 148]. The prognosis of nasal NK/T-cell lymphoma is variable despite aggressive therapy. Some patients respond well and others die with disseminated disease. The prognostic influence of the cytological differentiation is unclear.

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