Primary nasopharyngeal Hodgkin's disease: case report and literature review.

INTRODUCTION: Primary Hodgkin's disease of the nasopharynx is a rare and uncommon event. It has a relatively favorable prognosis and represents less than 1% of all documented cases of Hodgkin's disease. CASE PRESENTATION: A 40-year-old Arabic man presented initially with bilateral nasal obstruction, which was then followed by a significant involvement of his bilateral cervical lymph nodes. His nasopharyngeal biopsy together with immunohistochemistry analysis showed negative expressions of CD15, CD20 and CD3, but positive expressions of CD30 and epithelial membrane antigen. This confirmed the diagnosis of nasopharyngeal Hodgkin's disease of a mixed cellularity subtype. The disease was at stage IIEA. Our patient received four cycles of chemotherapy, which yielded a 75% response. This was followed by irradiation of his Waldeyer's ring and supraclavicular lymph nodes. He remains in good local control after 30 months of follow-up. CONCLUSION: The literature review and our case report discuss the optimal management of this rare and atypical localization of Hodgkin's disease, which should be differentiated from lymphoproliferations associated with Epstein-Barr virus and non-Hodgkin's lymphoma.

Analyses of the prognostic significance of the Epstein-Barr virus transactivator ZEBRA protein and diagnostic value of its two synthetic peptides in nasopharyngeal carcinoma.

BACKGROUND: Although numerous serological studies have determined the diagnostic and prognostic values of Epstein-Barr virus (EBV) antibodies in adult patients with nasopharyngeal carcinoma (NPC), little data about the anti-EBV immune response in children with NPC is available. OBJECTIVES: To examine the diagnostic value of IgG antibodies against BamHI Z Epstein-Barr replication activator (ZEBRA) protein and two related synthetic peptides (Zp125 and Zp130). To compare the prognostic value of IgA antibodies against early antigens (EA) and viral capsid antigen (VCA), and IgG antibodies against ZEBRA protein, of Moroccan children treated for NPC with their prognostic value for young and adult NPC patients. STUDY DESIGN: Sera were collected from 255 newly diagnosed Moroccan NPC patients and 226 healthy donors. IgA antibody against VCA and EA was measured by immunofluorescence assays. IgG antibody against ZEBRA, Zp125, and Zp130 was measured by ELISA. RESULTS: No significant difference in the detection of IgG-Zp125 and Zp130 antibodies was observed in children with NPC. IgG-Zp130 were detected less frequently than IgG-Zp125 in young and adult patients, as compared to children. High specificity of IgG-Zp125 and -Zp130 antibodies was found in the three age groups. A decrease in IgG-ZEBRA was observed in patients with NPC in clinical remission, whereas patients with NPC who died or developed metastases maintained or had an increase in these titers. CONCLUSION: IgG-ZEBRA is a better diagnostic and post-therapeutic prognostic marker in children with NPC, who showed very low titers of IgA -VCA and -EA.

[Bilateral primary adrenal lymphoma]. / Lymphome surrénalien primitif bilatéral.

The adrenal gland is a rare site of non-Hodgkin's lymphoma, as only about 70 cases have been reported in the literature, usually with bilateral involvement. Most tumours have a high grade histology, almost always with the B phenotype. Medical imaging is nonspecific and biopsy remains the most reliable diagnostic method. Chemotherapy is the treatment of choice, but the prognosis remains poor in the majority of cases, although long-term survivals have been described. The authors report a case of bilateral high-grade lymphoma of the adrenal glands in a 31-year-old patient presenting with acute adrenal insufficiency. Imaging demonstrated large bilateral adrenal masses, and surgical biopsy of the adrenal gland and staging confirmed the diagnosis of bilateral primary adrenal lymphoma. After corticosteroid replacement therapy, treatment consisted of primary CHOP chemotherapy administered for 9 cycles, followed by external beam radiotherapy delivered at a dose of 40 Gy. After 3 months of follow-up, the patient was still alive, with partial response, with an overall survival of 15 months.