Introduction: Anaplastic large-cell lymphoma occurring in the nasal cavity is a rare disease. The latest World Health Organization (WHO) Classification recognizes three distinct subtypes: primary systemic anaplastic lymphoma kinase positive been our case , primary systemic anaplastic lymphoma kinase negative and primary cutaneous types. Through this case study, we focus on the clinical presentation, treatment and prognostic characteristics of this pathology. Case Presentation: We report the case of a patient aged 32 years, who presented for seven months a runny nose associated with swelling of the face on the left side, without peripheral lymphadenopathy or general signs. A Blondeau scanner objectified a total filling of the frontal and left maxillary sinus, and a filling of the left nasal cavity. Complete resection of the tumor was performed. Histological examination was in favor of anaplastic large T-cell lymphoma anaplastic lymphoma kinase positive. The patient was stage IE according to Ann Arbor classification, with an International Prog nostic Index estimated at one. Thus, the patient received six cycles of CHOP chemotherapy. Currently, he is in good loco-regional control with a decline of three months. Conclusion: The rarity of this case lies partly in the lympho matous localization in the nasal cavity, and secondly in the anaplastic histology . It poses a diagnostic problem. So, we conclude that in case of any symptom of nasal cavities, it is necessary to explore and possibly biopsy if tumor, before surgery, because lymphomas are chemosensitive disease.
M. E. Kadin and S. W. Morris, “The t(2;5) in Human Lymphomas,” Leukemia & Lymphoma, Vol. 29, No. 3-4, 1998, pp. 249-256. doi:10.3109/10428199809068562
H. Stein, D. Y. Mason, J. Gerdes, et al., “The Expression of the Hodgkin’s Diseaseassociated Antigen Ki-1 in Reactive and Neoplastic Lymphoid Tissue: Evidence That Reed-Sternberg Cells and Histiocytic Malignancies Are Derived from Activated Lymphoid Cells,” Blood, Vol. 66, No. 4, 1985, pp. 848-858.
N. L. Harris, E. S. Jaffe, H. Stein, et al., “A Revised European-American Classification of Lymphoid Neoplasms: A Proposal from the International Lymphoma Study Group,” Blood, Vol. 84, No. 5, 1994, pp. 1361-1392.
S. H. Swerdlow, E. Campo, N. L. Harris, E. S. Jaffe, S. A. Pileri, H. Stein, J. Thiele and J. W. Vardiman, “WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues,” IARC, Lyon, 2008.
K. J. Savage, N. L. Harris, J. M. Vose, et al., “ALK-Negative Anaplastic Large-Cell Lymphoma (ALCL) Is Clinically and Immunophenotypically Different from Both ALKPositive ALCL and Peripheral T-Cell Lymphoma, Not Otherwise Specified: Report from the International Peripheral T-Cell Lymphoma Project,” Blood, Vol. 111, No. 12, 2008, pp. 5496-5504. doi:10.1182/blood-2008-01-134270
F.-H. Wang, Y.-H. Li, J. Zeng, H.-L. Rao, Z.-J. Xia, X.-F. Sun, H.-Q. Huang, T.-Y. Lin, W.-Q. Jiang and Z.-Z. Guan, “Clinical Analysis of Primary Systemic Anaplastic Large Cell Lymphoma: A Report of 57 Cases,” Chinese Journal of Cancer, Vol. 28, No. 1, 2009.
D. A. Filippa, M. Ladanyi, N. Wollner, et al., “CD30 (Ki-1)-Positive Malignantlymphomas: Clinical, Immunophenotypic, Histologic, and Genetic Characteristics and Differences with Hodgkin’s Disease,” Blood, Vol. 87, No. 7, 1996, pp. 2905-2917.
B. Falini, S. Pileri, P. L. Zinzani, et al., “ALK + Lymphoma: Clinico-Pathological Findings and Outcome,” Blood, Vol. 93, No. 8, 1999, pp. 2697-2706.
M. E. Kadin and C. Carpenter, “Systemic and Primary Cutaneous Anaplasticlarge Cell Lymphomas,” Seminars in Hematology, Vol. 40, No. 3, 2003, pp. 244-256. doi:10.1016/S0037-1963(03)00138-0
J. T. Sandlund, C. H. Pui, V. M. Santana, et al., “Clinical Features and Treatment Outcome for Children with CD30+ Large-Cell Non-Hodgkin’s Lymphoma,” Journal of Clinical Oncology, Vol. 12, No. 5, 1994, pp. 895-898.
M. Herling, G. Z. Rassidakis, D. Jones, et al., “Absence of Epstein-Barr Virus Inanaplastic Large Cell Lymphoma: A Study of 64 Cases Classified According to World Health Organization Criteria,” Human Pathology, Vol. 35, No. 4, 2004, pp. 455-459. doi:10.1016/j.humpath.2003.10.013
S. Noorali, S. Pervez, N. Yaqoob, et al., “Prevalence and Characterization of Anaplastic Large Cell Lymphoma and Its Association with Epstein-Barrvirus in Pakistani Patients,” Pathology—Research and Practice, Vol. 200, No. 10, 2004, pp. 669-679. doi:10.1016/j.prp.2004.08.004
V. Costes-Martineau, C. Delfour, S. Obled, et al., “Anaplastic Lymphomakinase (ALK) Protein Expressing Lymphoma after Liver Transplantation: Case Report and Literature Review,” Journal of Clinical Pathology, Vol. 55, No. 11, 2002, pp. 868-871. doi:10.1136/jcp.55.11.868
R. D. Gascoyne, L. Lamant, J. I. Martin-Subero, et al., “ALK-Positive Diffuse Large B-Cell Lymphoma Is Associated with Clathrin-ALK Rearrangements: Report of 6 Cases,” Blood, Vol. 102, No. 7, 2003, pp. 2568-2573. doi:10.1182/blood-2003-03-0786
H. Ruchatz, A. M. Coluccia, P. Stano, et al., “Constitutive Activation of Jak2 Contributes to Proliferation and Resistance to Apoptosis in NPM/ALK Transformed Cells,” Experimental Hematology, Vol. 31, No. 4, 2003, pp. 309-315. doi:10.1016/S0301-472X(03)00007-9
R. L. Ten Berge, J. J. Oudejans, G. J. Ossenkoppele, et al., “ALK Expression in Extranodal Anaplastic Large Cell Lymphoma Favours Systemic Diseasewith (Primary) Nodal Involvement and a Good Prognosis and Occurs before Dissemination,” Journal of Clinical Pathology, Vol. 53, No. 6, 2000, pp. 445-450. doi:10.1136/jcp.53.6.445
R. D. Gascoyne, P. Aoun, D. Wu, et al., “Prognostic Significance of Anaplastic Lymphoma Kinase (ALK) Protein Expression in Adults with Anaplasticlarge Cell Lymphoma,” Blood, Vol. 93, 1999, pp. 3913-3921.
L. Lamant, A. de Reyniès, M. M. Duplantier, et al., “GeneExpression Profiling of Systemic Anaplastic Large-Cell Lymphoma Reveals Differences Based on ALK Status and Two Distinct Morphologic ALK+ Subtypes,” Blood, Vol. 109, No. 5, 2007, pp. 2156-2164. doi:10.1182/blood-2006-06-028969
M. Shiota, S. Nakamura, R. Ichinohasama, et al., “Anaplastic Large Cell Lymphomas Expressing the Novel Chimeric Protein p80NPM/ALK: A Distinct Clinic Opathologic Entity,” Blood, Vol. 86, No. 5, 1995, pp. 1954-1960.
K. J. Savage, N. L. Harris, J. M. Vose, et al., “ALK-Negative Anaplastic Large-Cell Lymphoma (ALCL) Is Clinically and Immunophenotypically Different from Both ALK-Positive ALCL and Peripheral T-Cell Lymphoma, Not Otherwise Specified: Report from the International Peripheral T-Cell Lymphoma Project,” Blood, Vol. 111, No. 12, 2008, pp. 5496-5504. doi:10.1182/blood-2008-01-134270
P. L. Zinzani, M. Martelli, M. Magagnoli, et al., “Anaplastic Large Cell Lymphoma Hodgkin’s-Like: A Randomized Trial of ABVD versus Macop-B with and without Radiation Therapy,” Blood, Vol. 92, No. 3, 1998, pp. 790-794.
H. Tilly, P. Gaulard, E. Lepage, et al., “Primary Anaplastic Large-Cell Lymphomain Adults: Clinical Presentation, Immunophenotype, and Outcome,” Blood, Vol. 90, No. 9, 1997, pp. 3727-3734.
R. I. Fisher, E. R. Gaynor, S. Dahlberg, et al., “Comparison of a Standard Regimen (CHOP) with Three Intensive Chemotherapy Regimens for Advanced Non-Hodgkin’s Lymphoma,” The New England Journal of Medicine, Vol. 328, No. 14, 1993, pp. 1002-1006. doi:10.1056/NEJM199304083281404
K. Seidemann, M. Tiemann, M. Schrappe, et al., “ShortPulse B-Non-Hodgkin Lymphoma-Type Chemotherapy Is Efficacious Treatment for Pediatric Anaplastic Large Cell Lymphoma: A Report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90,” Blood, Vol. 97, No. 12, 2001, pp. 3699-3706. doi:10.1182/blood.V97.12.3699
S. R. Park, J. Y. Baek, D. W. Kim, et al., “Primary Systemic Anaplastic Large Cell Lymphoma in a Single Korean Institution: Clinical Characteristics and Treatment Outcome,” Journal of Korean Medical Science, Vol. 21, No. 4, 2006, pp. 633-638. doi:10.3346/jkms.2006.21.4.633
T. Mori, T. Takimoto, N. Katano, et al., “Recurrent Childhood Anaplastic Large Cell Lymphoma: A Retrospective Analysis of Registered Cases in Japan,” British Journal of Haematology, Vol. 132, No. 5, 2006, pp. 594-597. doi:10.1111/j.1365-2141.2005.05910.x
Jagasia, M., Morgan, D., Goodman, S., et al. (2004) Histology impacts the outcome of peripheral T-cell lymphomas after high dose chemotherapy and stem cell transplant. Leukemia & Lymphoma, 45, 2261-2267