Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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Mature T Cell and NK Cell Neoplasms EXTRANODAL NK/T-CELL LYMPHOMA, NASAL TYPE Definition and Incidence Extranodal NK/T cell lymphoma, nasal type is a predominantly extranodal lymphoma characterised by a broad morphologic spectrum. The lymphoma typically presents as a locally destructive proliferative lesion. The disease is most common in Asia, Mexico, Central and South America. Males predominate and the median age of presentation is 50 to 55 years. These lymphomas have also been described in patients immunosuppressed following organ transplantation. ICD-O Code 9719/3 Clinical Presentation The commonest site is the nasal cavity. Identical neoplasms may be seen in other extranodal sites, including the nasopharynx, palate, skin, soft tissue, gastrointestinal tract and testis. Patients typically present with facial swelling and or mid-line facial destruction and the disease has an aggressive course. It is localised (stage I and II) in 80% at presentation but may disseminate to the skin, gastrointestinal tract, orbit, CNS or testis. Pathology and Genetics This lymphoma is described as an angiocentric and angiodestructive, proliferative lesion. Fibrinoid changes, coagulative necrosis and apoptotic bodies are common. There is a broad spectrum of tumour cell morphology. Cells may be small, medium, large or anaplastic. They may have irregular nuclei which may be elongated, and nucleoli are generally inconspicuous. Mitotic figures are easily found. There may be a prominent inflammatory infiltrate. Phenotype The most common phenotype is CD2+, CD3-, CD56+, CD7-, Granzyme +. Other T and NK cell antigens are usually negative, including CD4, CD5, CD8, CD16 and CD57. Genetics T-Cell receptor and immunoglobulin genes are in germline configuration in the majority of cases, although T-cell receptor gene rearrangement may be detected. EBV genome is detected in tumour tissue using in situ hybridization for EBV-encoded RNA. Staging As for other high grade lymphomas. Recommended Investigations As for other high grade lymphomas but should include in addition: a CT scan and MRI of nasal sinuses and brain. Lumbar puncture with cytology for malignant cells should also be performed. Prognostic Factors / Index The prognosis is variable, with some patients achieving complete responses to treatment, and others dying of progressive, disseminated disease. Extranodal involvement in nasal disease or disease occurring outside the nasal cavity is very aggressive and associated with a short survival. 45
Potential Pitfalls Treatment planning must involve both a radiation oncologist and a medical oncologist/haematologist. Treatment Localised disease is best treated with intensive radiation therapy which results in a complete remission in two-thirds of patients although local relapse occurs in 50% and 25% of patients and progress to disseminated disease. For late stage disease (stages III and IV) combined modality therapy with radiation and chemotherapy and CNS prophylaxis is recommended. Response Evaluation and Follow Up As for other aggressive lymphomas. 46
Page 1 and 2: Guidelines on Diag
Page 3 and 4: Guidelines on Diag
Page 5 and 6: Standards In Diagnosis of Lymphoma
Page 7 and 8: Standards in Staging of Lymphoma An
Page 9 and 10: Pathologic Diagnosis of Lymphoma Ge
Page 11 and 12: Hodgkin lymphoma and its differenti
Page 13 and 14: Potential diagnostic pitfalls for l
Page 15 and 16: Summary of the usual Immunostaining
Page 17 and 18: Mature B-Cell Neoplasms Mature B-Ce
Page 19 and 20: Immunophenotype The tumour cells ex
Page 21 and 22: Lymphoplasmacytic Lymphoma Definiti
Page 23 and 24: Splenic Marginal Zone Lymphoma Defi
Page 25 and 26: Extra-nodal Marginal Zone B-Cell Ly
Page 27 and 28: Persistent / progressive disease or
Page 29 and 30: Follicular Lymphoma Definition and
Page 31 and 32: Potential Pitfalls a. Failure to di
Page 33 and 34: Mantle Cell Lymphoma Definition and
Page 35 and 36: Diffuse Large B-Cell Lymphoma Defin
Page 37 and 38: Stage 1 (bulky) and stages II - IV
Page 39 and 40: Treatment Standard chemotherapy for
Page 41 and 42: Burkitt Lymphoma/Leukaemia Definiti
Page 43 and 44: CODOX-M, (3 cycles) for low risk di
Page 45 and 46: Potential pitfalls Failure to diffe
Page 47 and 48: Precursor T Cell Neoplasms PRECURSO
Page 49: Mature T-Cell and NK-Cell Neoplasms
Page 53 and 54: Recommended Investigations Evaluati
Page 55 and 56: Mycosis Fungoides and Sézary Syndr
Page 57 and 58: ■ ■ Lymph node biopsy if there
Page 59 and 60: Primary Cutaneous CD30-Positive T-C
Page 61 and 62: Angioimmunoblastic T-Cell Lymphoma
Page 63 and 64: Prognostic factors The IPI is of li
Page 65 and 66: Hodgkin Lymphoma (HL) Definition an
Page 67 and 68: Prognostic Factors / Index: i. Earl
Page 69 and 70: Immunodeficiency Associated Lymphop
Page 71 and 72: of cases and EBV in about 50%, but
Page 73 and 74: Common Issues in Lymphoma Managemen
Page 75 and 76: Tumour Lysis Syndrome Tumour Lysis
Page 77 and 78: Haematopoietic Recombinant Growth F
Page 79 and 80: Follow-Up Follow-up for patients wi
Page 81 and 82: Follow-up for patients with concern
Page 83 and 84: 77 Appendix
Page 85: Lower border: The lower of (i) 5 cm
Page 88 and 89: Glossary of Treatment Regimens Chlo
Page 90: 2nd Edition. May 2010. Supported by

Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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