Primary XXXX . Clinicopathologic study of 68 cases.

@entity1 @entity129 @entity5773 @entity66 @entity706 @entity5 @entity3521 @entity1282 @entity1893 @entity204
The clinical and pathologic findings were studied in 68 @entity1 with primary @entity706 ( @entity3521 ). The initial sites included: tonsil (51%; 9% bilateral), nasopharynx (35%), base of tongue (9%), and multiple areas (4%). The mean age was 58 years, with a male to female ratio of 1.1:1.0. @entity5773 presented with sore throat and @entity1282 , whereas @entity706 most commonly presented with nasal, auditory, and @entity129 . A neck mass was the sole presenting symptom in 12% of @entity1 . Work-up showed 42% Stage I, 47% Stage II, 4% Stage III, and 7% Stage IV. All were @entity1893 . Diffuse architecture predominated (71%) and 70% had a histiocytic or large cell morphology (diffuse histiocytic 51%, nodular histiocytic 19%). The National @entity5 Institute (NCI) Working Formulation grades were 13% low, 81% intermediate, and 6% high. In difficult cases, detection of monoclonal immunoglobulin, absence of keratin staining, and lack of epithelial features by electron microscopy were useful adjuncts aiding in diagnosis. Following complete remission, 28 @entity1 relapsed, 75% of these within 2 years (median, 10 months). Thirty-seven (54%) @entity1 died with disease (median survival, 27 months). Of these, 89% had disseminated @entity706 . Local-regional disease was the direct cause of @entity204 in 13%. Recurrence or persistence of disease in @entity3521 occurred in 9% of cases, most with initial clinical evidence of soft tissue extension. Stage I @entity1 had a significantly better median survival (67 months) than Stage II @entity1 (20 months) (P = 0.03). Prognostically favorable histologic parameters included lower NCI Working Formulation grade and follicular architecture. Ten @entity1 (15%) developed @entity66 , eight involving the gastrointestinal (GI) tract, and all died within 1 year of its occurrence. The findings indicate that stage and certain histologic parameters are important prognostic factors in @entity706 . The study confirms the association of primary @entity706 with @entity66 , particularly involving the GI tract, and emphasizes the poor prognosis of @entity1 in whom this occurs.
