ABSTRACT
BACKGROUND: Histologic prognostic factors have been described for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). This study examines histologic and immunophenotypic variants in a clinical trial for pediatric NLPHL. PROCEDURE: One hundred sixty-eight cases of localized NLPHL were examined for histologic variants, CD30 and immunoglobulin D (IgD) expression, and outcome. Histologic types were scored categorically as 0 = 0, 1 ≤ 25%, and 2 > 25% of the sample. RESULTS: Fifty-eight (35.1%) cases showed only typical nodular with or without serpiginous histology (types A and B). The remainder showed mixtures of histologies. The numbers of patients with score 2 are 85 (50.6%) type A, 21 (12.5%) type B, 46 (27.4%) with extranodular large B cells (type C), 3 with T-cell-rich nodular pattern (type D), 55 (32.7%) with diffuse T-cell-rich (type E) pattern, and 2 (1.2%) with diffuse B-cell pattern (type F). Higher level of types C (P = 0.048) and D (P = 0.033) resulted in lower event-free survival (EFS). Cytoplasmic IgD was found in 65 of 130 tested (50%), did not significantly associate with EFS but positively correlated with types C and E histology (P < 0.0001) and negatively correlated with types A (P = 0.0003) and B (P = 0.006). Seventeen (10%) expressed CD30, with no adverse effect. CONCLUSIONS: Variant histology is common in pediatric NLPHL, especially types C and E, which are associated with IgD expression. Type C variant histology and possibly type D are associated with decreased EFS, but neither IgD nor CD30 are adverse features. Variant histology may warrant increased surveillance, but did not affect overall survival.
Subject(s)
B-Lymphocytes , Gene Expression Regulation, Neoplastic , Hodgkin Disease , Immunoglobulin D/biosynthesis , Ki-1 Antigen/biosynthesis , T-Lymphocytes , Adolescent , B-Lymphocytes/metabolism , B-Lymphocytes/pathology , Child , Child, Preschool , Disease-Free Survival , Female , Hodgkin Disease/metabolism , Hodgkin Disease/mortality , Hodgkin Disease/pathology , Humans , Immunohistochemistry , Infant , Infant, Newborn , Male , Survival Rate , T-Lymphocytes/metabolism , T-Lymphocytes/pathologyABSTRACT
Midline destructive lesions of the face have multiple possible etiologies. The majority of these cases are found to be due to an extranodal lymphoma of natural killer/T-cell-type non-Hodgkins lymphoma (NKTL). Unfortunately, diagnosis is often delayed. With variable presenting complaints, including nonspecific issues like chronic rhinosinusitis or nasal congestion, initial treatments are aimed at these presumed diagnoses. Only as the lesion progresses do overt signs of destruction occur. As with our patient, who was initially treated for presumed infection and abscess, final diagnosis often does not occur until several months, and several antibiotic courses, from initial presentation. As such, it is important for this rare entity to remain in the clinician's differential diagnosis for nasal lesion.
ABSTRACT
Primary intracranial meningeal sarcoma is a rare neurological malignancy without strong evidence-based treatment guidelines. The authors describe a boy with primary meningeal sarcoma who symptomatically presented at 10 months of age and was treated with primary resection. The patient had multifocal recurrence approximately 2 years later. Given the location and rapid progression of the disease, the boy was treated with gamma knife surgery. He had a complete radiographic response 3 years posttreatment. He attends school full time and enjoys good quality of life. Based on local control and response to radiosurgery, the authors suggest that multifocal meningeal sarcomas not amenable to resection can be effectively managed with stereotactic radiosurgery.
