Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin's lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.
Bone Marrow ; Central Nervous System ; Early Diagnosis ; Granuloma, Lethal Midline ; Incidence ; Inflammation ; Lung ; Lymphoma ; Lymphoma, Non-Hodgkin ; Mouth ; Parotid Gland ; Periodontitis ; Skin ; Spleen

Natural killer (NK)/T cell lymphoma is a lethal midline granuloma that produces necrotic and granulomatous lesions in the upper respiratory tract, especially in the nasal cavity. This tumor affects the nose and midface, but can also arise in the skin, gastrointestinal tract, testes, central nervous system, lungs, salivary glands, bone marrow, and larynx. Five to eight percent of extranodal lymphomas involve the orbit, and 1.3~2.0% of these present as primary orbital tumors. The skin can also be the primary site of NK/T cell lymphomas. Aside from affecting the nasal and facial skin, the tumor can involve the skin of the postauricular area, back, trunk, and upper and lower extremities. We present the case of a 37-year-old woman who had a nasal NK/T cell lymphoma with cutaneous and ocular involvement.
Adult ; Bone Marrow ; Central Nervous System ; Female ; Gastrointestinal Tract ; Granuloma, Lethal Midline ; Humans ; Larynx ; Lower Extremity ; Lung ; Lymphoma ; Nasal Cavity ; Nose ; Orbit ; Respiratory System ; Salivary Glands ; Skin ; Testis

To investigate the expression of survivin gene and its relationship with Epstin-Barr virus (EBV) infection in midline T-cell lymphoma (MTL), immunohistochemistry staining method was used to examine the expression of survivin and EBV-latent membrane protein (LMP-1) in the 41 cases. In situ hybridization (ISH) was used to detect EBV-encoded RNA (EBER1/2). The results showed that the expression of survivin was positive in 26 cases of midline T-cell lymphoma, but no positive was detected in 10 cases of reactive lymphoid tissues. The positive expression ratio of survivin was 12.5% in cases of MTL with low grade of malignancy, and was 75.76% in cases of MTL with middle and high grades of malignancy, the significant difference was found between these two groups (chi(2) = 8.55, P < 0.01). Positive expression ratios of EBER1/2 and LMP-1 were 70.73% and 41.46% respectively. Survivin expression was not significantly different between EBER1/2 positive and negative cases (P > 0.05). It is concluded that survivin expression is up-regulated in MTL, and survivin positive expression rate is associated with the degree of malignancy. Survivin may play a role in the pathogenesis of the MTL by influencing cell apoptosis. EBV infection is not significantly associated with survivin expression in the MTL.
Adaptor Proteins, Signal Transducing ; Adolescent ; Adult ; Aged ; Child ; Cytoskeletal Proteins ; Epstein-Barr Virus Infections ; metabolism ; pathology ; virology ; Female ; Granuloma, Lethal Midline ; metabolism ; pathology ; virology ; Humans ; Immunohistochemistry ; In Situ Hybridization ; Inhibitor of Apoptosis Proteins ; Intracellular Signaling Peptides and Proteins ; metabolism ; LIM Domain Proteins ; Lymphoma, T-Cell ; metabolism ; pathology ; virology ; Male ; Microtubule-Associated Proteins ; biosynthesis ; Middle Aged ; Neoplasm Proteins ; biosynthesis ; Nose Neoplasms ; metabolism ; pathology ; virology ; RNA, Viral ; genetics

Mature T-cell and natural killer-cell neoplasms account for 10 to 15% of all non-Hodgkin's lymphomas. Of the various subtypes of mature T-cell and NK-cell lymphomas, extranodal NK/T-cell lymphoma, nasal type (nasal type NK/T-L) is relatively more common among Asians including Koreans. Nasal type NK/T-L is an aggressive, Epstein-Barr virus-associated lymphoma with characteristic expression of NK-cell antigen CD56. In this report, we present an unusual case of EBV(+), CD56(-) NK/T-L of oropharynx which recurred in duodenum after the period of complete remission lasting for 10 years. A 58-year-old woman presented with 3 months history of abdominal pain. Gastroduodenoscopy showed the diffuse wall thickening with multiple ulcerations in bulb and proximal second portion of the duodenum. Pathological examination revealed the infiltration of atypical lymphocytes, which was positive for CD3, CD4, CD5, TIA-1, and EBV and was negative for CD15, CD20, and CD56, consistent with NK/T-L of mature T-cell origin. The past medical history included the presence of oropharyngeal mass 10 years earlier, which was diagnosed as polymorphic reticulosis. The mass resolved completely after the radiation therapy, and she remained free of the disease for 10 years. Upon review, the oropharyngeal biopsy showed an identical morphology and immunophenotype with duodenal lesion. In conclusion, we experienced an unusal case of NK/T-cell lymphoma, nasal type recured in the duodenum.
Abdominal Pain ; Asian Continental Ancestry Group ; Biopsy ; Duodenum ; Female ; Granuloma, Lethal Midline ; Herpesvirus 4, Human ; Humans ; Lymphocytes ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Middle Aged ; Oropharynx ; Recurrence* ; T-Lymphocytes ; Ulcer

Diagnosis, Differential ; Granuloma, Lethal Midline ; diagnosis ; therapy ; Humans ; Infant ; Male

BACKGROUND AND OBJECTIVES: Polymorphic reticulosis (PMR), a type of lethal midline granuloma, has characteristics of necrosis, angiocentricity, and angiogenesis, which is also known as nasal T-cell lymphoma. In this study, we classified PMR and nasal lymphoma using immunohistochemical staining and investigated the clinical characteristics of nasal malignant lymphoma including survival rates with treatment modalities. MATERIALS AND METHODS: Twenty-six patients previously diagnosed with PMR or nasal lymphoma from May 1992 to April 1997 were included in this study. We performed immunohistochemical staining with CD3, CD56 and CD79a for classification and reviewed the patients' clinical characteristics and survival rates, retrospectively. RESULTS: Of twenty-six patients, twenty-five patients were classified as having angiocentric lymphoma and only one patient with B-cell lymphoma. Higher mortality rates were observed in patients receiving chemotherapy alone than in those receiving chemotherapy and radiation therapy and in advanced stage. CONCLUSIONS: Early detection and combined treatment could improve the survival rate of patients with nasal malignant lymphomas.
Classification* ; Drug Therapy ; Granuloma, Lethal Midline ; Humans ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, T-Cell ; Mortality ; Necrosis ; Nose ; Retrospective Studies ; Survival Rate

BACKGROUND AND OBJECTIVES: The histologic features of polymorphic reticulosis are identical to those of sinonasal "angiocentric immunoproliferative lesions" (AILs) which are characterized by angiocentric and angioinvasive lymphoid infiltrates with a wide range of cytologic atypia. 1) This report was conducted to further define the clinicopathological nature of this lesion and evaluate the important prognostic factors and therapeutic strategies. MATERIALS AND METHODS: The clinical features and therapeutic outcomes of 22 patients with AILs were reviewed. Histologic grading was performed by using previously reported criteria (Grade I-III).2) To clarify the cell origin of AIL, immunohistochemical studies were performed in 21 cases. RESULTS: On the slide review, 10 cases were Grade II and 12 cases were grade III which showed characteristic histologic features similar to those of peripheral T-cell lymphoma. On immunohistochemical studies, neoplastic cells of all patients had a T-cell phenotype. The difference in primary treatment results according to the histologic grade, clinical staging and therapeutic modalities was not significant statistically. The overall 5 year survival rates were 58%. CONCLUSION: Our results suggest strongly that quite a significant portion of AIL may be in fact T-cell lymphoma. The only significant prognostic factor that predicts better survival is the achievement of initial complete response.
Granuloma, Lethal Midline ; Head* ; Humans ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Neck* ; Phenotype ; Survival Rate ; T-Lymphocytes

PURPOSE: To evaluate the CT finidngs of malignant nasal cavity tumors. MATERIALS AND METHODS: Retrospective analysis was performed on 20 patients with pathologically-proven malignant nasal cavity tumors. Using CT, we analysed their location, extent of bone destruction and of involvement of adjacent structures, and enhancing pattern. RESULTS: A total of 20 cases included nine squamous cell carcinomas, three olfactory neuroblastomas, three lymphomas, two polymorphic reticulosis, one adenoid cystic carcinoma, one undifferentiated carcinoma and one metastasis from renal cell carcinoma. All cases except one adenoid cystic carcinoma and one squamous cell carcinoma revealed bone destruction or erosion. Aggressive bone destruction and irregular enhancement were seen in eight cases of squamous cell carcinoma, seven cases of which showed involvement of the adjacent paranasal sinuses, nasopharynx, and orbit. Olfactory neuroblastomas were centered in the superior nasal cavity and the adjacent ethmoid sinus, and had erosion or destruction of the cribriform plate had occurred. Lymphomas showed bilateral involvement, with uniform contrast enhancement. Polymorphic reticuloses showed perforation or erosion of the nasal septum with bilateral involvement of the nasal cavity. CONCLUSION: The location, presence of bone destruction, involvement of adjacent structures, and enhancement pattern of tumor on CT can be helpful for the differential diagnosis of malignant nasal cavity tumors.
Carcinoma ; Carcinoma, Adenoid Cystic ; Carcinoma, Renal Cell ; Carcinoma, Squamous Cell ; Diagnosis, Differential ; Esthesioneuroblastoma, Olfactory ; Ethmoid Bone ; Ethmoid Sinus ; Granuloma, Lethal Midline ; Humans ; Lymphoma ; Nasal Cavity* ; Nasal Septum ; Nasopharynx ; Neoplasm Metastasis ; Orbit ; Paranasal Sinuses ; Retrospective Studies

Angiocentric lymphoma is a lymphoma like lesion which has been known as a lethal midline granuloma, lymphomatoid granulomatosis and polymorphic reticulosis. It involves predominantly lungs, and other extranodal sites such as upper respiratory tract, skin, kidneys, peripheral nervous system and CNS. The authors experienced a case of angiocentric lymphoma which was misdiagnosed as orbital cellulitis because there were persistent epiphora, erythematous swelling in dacryocyst area and diffuse edema in upper and lower eyelids which occurred after functional endoscopic sinus surgery. This case was diagnosised as angiocentric lymphoma through multiple tissue biopsies. This disease is rare, especially in orbit and eyelid. It leads to the destruction and inflammation of tissue. To avoid misdiagnosis to orbital cellulitis, accurate biopsy is needed with the interchange of clinical informations with pathologist. We report this case with the review of literatures.
Biopsy ; Diagnosis ; Diagnostic Errors ; Edema ; Eyelids ; Granuloma, Lethal Midline ; Inflammation ; Kidney ; Lacrimal Apparatus Diseases ; Lung ; Lymphoma* ; Lymphomatoid Granulomatosis ; Orbit ; Orbital Cellulitis ; Peripheral Nervous System ; Respiratory System ; Skin

During the period from January, 1975, to June, 1989, one hundred patients with histopathologically proven polymorphic reticulosis in the upper respiratory tract were treated with radiation therapy and the analysis of treatment results was undertaken. One hundred patients (69 males, 31 females) with a mean age of 46 years (range 12-79 years) were presented. Nasal cavity was the most frequent site of involvement(56%), and 44 cases had multifocal sites of involvement. The incidence of cervical lymph node metastasis at initial diagnosis was 24%. Staging was determined by Ann-Arbor classification, retrospectively. The number of patients of stage IE, IIE, IIIE and IVE were 35, 60, 1, and 4, respectively. The overall 5 year actuarial survival rates were 38.4%. The difference in 5 year survival rates between patients with stage IE and IIE, with solitary and multiple, with CR and PR after irradiation were significant statistically. For the analysis of failure patterns, failure sites include the following: local failure alone(30/55=54.6%), systemic failure alone(9/55=16.4%), both local and systemic failure(16/55=29.0%). Retrograde slide review was available in 29 cases of PMR with respect to histopathologic bases, and immunohistochemical studies were performed using MTI and DACO-UCHL-1 as T-cell markers, MB2 as a B-cell marker and alpha-1-antichymotrypsin as a histiocytic markers. All that 29 cases showed characteristic histologic features similar to those of peripheral T-cell lymphoma and showed positive reactio to the T-cell marker. These findings suggest strongly that quite a significant portion of PMR may be in fact T-cell lymphoma.
B-Lymphocytes ; Classification ; Diagnosis ; Granuloma, Lethal Midline* ; Humans ; Incidence ; Lymph Nodes ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Male ; Nasal Cavity ; Neoplasm Metastasis ; Respiratory System ; Retrospective Studies ; Survival Rate ; T-Lymphocytes