An 86-year-old woman’s stool sample was positive for blood. Computed tomography (CT) showed wall thickening of the ascending colon at the hepatic flexure. Colonoscopy showed near-complete obturation ...by colon cancer. Since she was asymptomatic, elective surgery was planned. Laparoscopic right hemicolectomy was performed. Histopathological examination showed poorly differentiated carcinoma cells proliferating in a solid pattern with marked lymphocyte infiltration. The diagnosis was lymphoepithelioma-like carcinoma (LELC) associated with Epstein-Barr virus (EBV) infection; however, EBV-encoded small RNA–in situ hybridization was negative. Microsatellite instability was not assessed. The postoperative course was uneventful and she was discharged on the 15th postoperative day. She remains recurrence-free at 2 years after surgery. Past reports note that colorectal carcinomas with dense lymphoid stroma may be related to LELC or medullary carcinoma (MC). Gastrointestinal LELC is rare, with some reports on LELC of the esophagus and stomach. Reports on LELC of the large intestine are very rare. MC of the large intestine is relatively new concept, firstly described in the WHO Classification of Tumours of the Digestive System 3rd Edition in 2000. We herein present a case of lymphoepithelioma-like carcinoma of the ascending colon and relevant case reports about LELC and MC of the large intestine.
The relapse of acute lymphoblastic leukemia (ALL) usually involves the bone marrow, with the central nervous system being the most frequent extramedullary site. The relapse of ALL in the female ...genital organs, particularly the uterus, is markedly rare. We report such a patient who developed relapse in the bone marrow and uterus. The uterine lesion, which presented as abnormal uterine bleeding, consisted of a mass on MRI and proliferation of ALL cells on histology. MRI revealed a heterogeneous high-intensity mass (T2-WI/D-WI) with a diameter of 6.8 cm, a notable decrease in the apparent diffusion coefficient (ADC), and mild enhancement by contrast enhancement study. Histological findings of the uterine cervix demonstrated the infiltration of ALL. The patient achieved remission by allogeneic haplo-identical hematopoietic stem-cell transplantation, but died of complications of the transplantation. This case suggested that attention should be paid to the uterus as a site of extramedullary relapse. In addition, abnormal uterine bleeding, which is a common sign of hormonal imbalance and hormone replacement therapy after chemotherapy, may be an initial sign of extramedullary recurrence. To confirm uterine relapse as an intractable disease, the accumulation of more cases is required.
Adult T-cell leukemia/lymphoma (ATL) is caused by human T-cell leukemia virus type 1 (HTLV-1). In addition to HTLV-1 bZIP factor (HBZ), a leukemogenic antisense transcript of HTLV-1, abnormalities of ...genes involved in TCR-NF-κB signaling, such as CARD11, are detected in about 90% of patients. Utilizing mice expressing CD4
T cell-specific CARD11(E626K) and/or CD4
T cell-specific HBZ, namely CARD11(E626K)
mice, HBZ transgenic (Tg) mice, and CARD11(E626K)
;HBZ Tg double transgenic mice, we clarify these genes' pathogenetic effects. CARD11(E626K)
and HBZ Tg mice exhibit lymphocytic invasion to many organs, including the lungs, and double transgenic mice develop lymphoproliferative disease and increase CD4
T cells in vivo. CARD11(E626K) and HBZ cooperatively activate the non-canonical NF-κB pathway, IRF4 targets, BATF3/IRF4/HBZ transcriptional network, MYC targets, and E2F targets. Most KEGG and HALLMARK gene sets enriched in acute-type ATL are also enriched in double transgenic mice, indicating that these genes cooperatively contribute to ATL development.
The patient was a 63-year-old man. He was examined by a local doctor for dyspnea, where he was administered oral steroids and started on inhalation therapy for a diagnosis of bronchial asthma. ...However, his symptoms worsened and he was brought to our hospital. On admission, he was diagnosed with atrial fibrillation and acute heart failure. Upon administration of anticoagulants to prevent embolism in this patient with atrial fibrillation, the patient developed bloody stool. Colonoscopy revealed multiple diverticula in the hepatic flexure, along with ulcers and white coating localized to that area. Diverticular colitis was initially suspected, but vegetative amoebas were found in a tissue biopsy of the ulcers and he was diagnosed with amebic colitis. The ulcers healed with administration of metronidazole. We report this case in which amebic colitis developed in a man with asymptomatic Entamoeba histolytica infection from the use of steroids and anticoagulants, with the lesions localized in the area with multiple colon diverticula.
Immunodeficiency-associated lymphoproliferative disorders (LPD) in rheumatoid arthritis are a rare, aggressive, and life-threatening clinical entity. We describe a 60-year-old man who had rheumatoid ...arthritis that was treated with methotrexate. Eight months after the treatment, the case was diagnosed as Epstein-Barr virus-negative LPD (diffuse large B-cell lymphoma) with abdominal bulky mass and clinical stage IVB at high risk in the international prognostic index. Immediate withdrawal of methotrexate led the patient to achieve complete remission, and 8 subsequent courses of rituximab treatment for the prevention of relapse kept the patient disease-free for 29 months. Our case suggests that these treatments may be an effective, safe, and feasible strategy for immunodeficiency-associated LPD in rheumatoid arthritis. J Clin Exp Hematopathol 52(3) : 193-198, 2012
A 33-year-old female presented with a rare synovial sarcoma manifesting as a painful 12 × 15 mm tumor in the median palmar carpus. Preoperative neurological examination detected only radiating ...spontaneous pain in her right radial palm and decreased right grasping power. Magnetic resonance (MR) imaging confirmed the presence of the tumor. The preoperative diagnosis was schwannoma originating from the right median nerve. Subcapsular removal of the tumor was performed for preservation of the nerve function. However, postoperative histological and immunohistochemical studies suggested synovial sarcoma originating from the median nerve. No systemic metastasis was detected and the residual tumor capsule was totally removed. Local radiation therapy of 40 Gy, 2 Gy per day, was administered. Fourteen months later, local recurrence was detected on MR imaging. Total removal of the recurrent tumor was performed. Synovial sarcoma was finally diagnosed by the identification of SYT-SSX1 fusion gene transcripts using reverse transcriptase-polymerase chain reaction with a frozen tumor tissue sample.
A 33-year-old female presented with a rare synovial sarcoma manifesting as a painful 12×15 mm tumor in the median palmar carpus. Preoperative neurological examination detected only radiating ...spontaneous pain in her right radial palm and decreased right grasping power. Magnetic resonance (MR) imaging confirmed the presence of the tumor. The preoperative diagnosis was schwannoma originating from the right median nerve. Subcapsular removal of the tumor was performed for preservation of the nerve function. However, postoperative histological and immunohistochemical studies suggested synovial sarcoma originating from the median nerve. No systemic metastasis was detected and the residual tumor capsule was totally removed. Local radiation therapy of 40 Gy, 2 Gy per day, was administered. Fourteen months later, local recurrence was detected on MR imaging. Total removal of the recurrent tumor was performed. Synovial sarcoma was finally diagnosed by the identification of SYT-SSX1 fusion gene transcripts using reverse transcriptase-polymerase chain reaction with a frozen tumor tissue sample.
The prognosis of aggressive adult T-cell leukemia/lymphoma (ATL) is poor, and allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative treatment. In order to identify favorable ...prognostic patients after intensive chemotherapy, and who therefore might not require upfront allo-HSCT, we aimed to improve risk stratification of aggressive ATL patients aged <70 years. The clinical risk factors and genetic mutations were incorporated into risk modeling for overall survival (OS). We generated the m7-ATLPI, a clinicogenetic risk model for OS, that included the ATL prognostic index (PI) (ATL-PI) risk category, and non-silent mutations in seven genes, namely TP53, IRF4, RHOA, PRKCB, CARD11, CCR7, and GATA3. In the training cohort of 99 patients, the m7-ATLPI identified a low-, intermediate-, and highrisk group with 2-year OS of 100%, 43%, and 19%, respectively (hazard ratio HR =5.46; P<0.0001). The m7-ATLPI achieved superior risk stratification compared to the current ATL-PI (C-index 0.92 vs. 0.85, respectively). In the validation cohort of 84 patients, the m7-ATLPI defined low-, intermediate-, and high-risk groups with a 2-year OS of 81%, 30%, and 0%, respectively (HR=2.33; P=0.0094), and the model again outperformed the ATL-PI (C-index 0.72 vs. 0.70, respectively). The simplified m7-ATLPI, which is easier to use in clinical practice, achieved superior risk stratification compared to the ATLPI, as did the original m7-ATLPI; the simplified version was calculated by summing the following: high-risk ATL-PI category (+10), low-risk ATL-PI category (-4), and non-silent mutations in TP53 (+4), IRF4 (+3), RHOA (+1), PRKCB (+1), CARD11 (+0.5), CCR7 (-2), and GATA3 (-3).
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