A married 55-year-old male was admitted to the Third Affiliated Hospital of Soochow University in February 2017, because of “diagnosis of malignant lymphoma for about 12 years with high fever and a left neck mass present for about 3 weeks". Medical history showed the patient underwent sacral tumor resection at the Third Affiliated Hospital of Soochow University in April 2006, because of abdominal pain and right lower limb pain. The postoperative pathological diagnosis was NK/T cell lymphoma (stage II). After two courses of CHOP and MEOP regimen chemotherapy, the patient was remission. Four years later, In June 2010, the patient was admitted to the Third Affiliated Hospital of Soochow University for "high fever with body temperature of 39℃and shoulder pain for more than 1 month". CT displayed the mass involving the right adrenal gland and retroperitoneal lymph nodes, considered recurrence. The patient received ESHAP regimen chemotherapy on July 3rd 2010. On July 6th 2010, the patient developed high fever with body temperature of 40℃ and pain and discomfort, treated with meropenem for anti-infection. B-ultrasound showed left adrenal mass; the patient received ESHAP regimen chemotherapy again On July 31st 2010, and discharged after improvement and remission. In April 2013, the patient developed a lump behind the retroperitoneal left renal vein (Figure 1A), considered recurrence, and clinical remission after local radiotherapy. In February 2017, the patient with fever without obvious cause found swelling of lymph nodes in the left neck and lymph node biopsy after hospitalization (the Third Affiliated Hospital of Soochow University) (Figure 1B). Physical examination revealed the left neck lymph node of 2.5×2cm; other superficial lymph nodes were non-enlargement, no involvement in liver and spleen. Biopsy pathology showed NK/T cell lymphoma, nasal type, giving the GDP plus pembellase regimen chemotherapy, the patient's condition improved and discharged.
Figure 1 Computed tomography examination
A, T showed a small lymph node was behind the retroperitoneal left renal vein in April 2013; B, CT showed an enlarged lymph nodes was behind the retroperitoneal left renal vein, evenly enhanced, compared to the previous piece (in April 2013) significantly increased in February 2017
Pathological examination: The first biopsy tissue (July 2006), intra-spinal mass was off-white, about 5×5×3cm. Microscopic examinations microscopic examinations showed diffuse and small lymphoid cell hyperplasia, common cytoplasm, irregular nucleus, granular chromatin, unobvious nucleolus, frequent mitosis, focal necrosis (Figure 2A, 2B, 2C). According to the immunohistochemical staining, the tumor cells were positive for CD43, CD2, CD56, TIA, GrB, with positive in situ hybridization for EBER, negative for CD20, CD3, ALK, CD30, CD4, and CD8. The clinical and histopathological finding of this case was in accord with the extranodal NK/T cell lymphoma, nasal type. The second biopsy (left cervical lymph node with the same people, February 2017), lymph node, about 2.5×2×1.3cm.The section was off-white and tender. Microscopic examinations showed a diffuse proliferation of small and atypical lymphoid cells, admixed with large lymphoid cells (Figure 2D). The large lymphoid cells showed irregular nuclear borders and had amounts of cytoplasm, large nuclear, obvious nucleolus, part of nuclear folding, multiple mitotic figures, visible intravascular tumor suppository (Figure 2E). Small lymphocytes were uniform in size and regular in shape, less cytoplasm, nuclear folding, rarely mitotic figures (Figure 2F).
Figure 2: Tumor patterns, hematoxylin eosin staining.
A, necrotic tumor cell clusters (×5). B, tumor cells diffuse into pieces, infiltrating adipose tissue (×5). C, tumor cells are relatively uniform in size, nuclear round, oval, partial nuclear irregular, abundant cytoplasm (×10). D, a diffuse proliferation of small and atypical lymphoid cells, admixed with large lymphoid cells (×10).E, large nuclear, binuclear, multinuclear, visible intravascular tumor suppository (×20). F, Small lymphocytes were uniform in size and regular in shape, less cytoplasm, nuclear folding, rarely mitotic figures (×20). (A/B/C were the first biopsy organization in April 2006, and D/E/F were the biopsy organization in February 2017)
Histologically, extranodal NK/T-cell lymphoma was difficult to differentiate from metastatic carcinoma and intervariable large cell lymphoma. According to the immunohistochemical staining, the tumor cells were positive for CD3 (Fiugre 3A), CD30 (Figure 3B), CD43, CD2, CD56, TIA (Figure 3C), GrB (Figure 3D), negative for CD4, CD8, AE1/AE3, CD20, ALK, EMA. In situ hybridization for EBER was strongly positive in both specimens (Figure 3E). The proliferation index of Ki-67 was about 70% (Figure 3F). After inquiring medical history, the case was diagnosis to extranodal NK/T-cell lymphoma, nasal type, and recrudescence.
Figure 3: Assessment of CD3, CD30, TIA, GrB, EBER and Ki-67
A, positive CD3 staining in the tumor cells (×10). B, positive CD30 staining in large cells (×20). C, positive TIA-1 staining in of large cells (×20).D, positive GrB staining in large cells (×20). E, EBER positive of large cells (×20). F, the proliferation index of Ki-67 was about 70%(×20)（February 2017 biopsy organization)