Extranodal NK/T cell lymphoma, nasal type（ENKTL） is a highly aggressive malignant tumor derived from NK cells. This article reports a case of ENKTL invading the larynx and digestive tract. The clinical clinical manifestations include hoarseness and intranasal masses.
摘要: 结外鼻型NK/T细胞淋巴瘤（extranodal NK/T cell lymphoma，nasal type，ENKTL）是NK细胞起源的高度侵袭性恶性肿瘤，本文报告1例侵及喉及消化道的ENKTL患者，临床表现为声音嘶哑、鼻腔内肿物。.

The nasal type of extranodal natural killer (NK)/T-cell lymphoma (ENKL) is an aggressive form of non-Hodgkin\'s lymphoma. Although ENKL is most commonly seen in the midline of the nose, face and Waldeyer\'s ring, it can also occur in the skin, gastrointestinal tract, soft tissues and other parts of the body. Severe ENKL cases are accompanied by hemophagocytosis, with clinical manifestations such as high fever, hepatosplenomegaly, and decreased blood cell count. ENKL at different locations exhibits similar histological features and immunophenotypes, such as a strong affinity for T cell markers CD2 and CD56, cytotoxic molecules, as well as a strong positive for EBER after in situ hybridization. Although indolent ENKL is extremely rare, we hereby present a case study of primary NK/T cell lymphoma in the spinal canal with the initial manifestation of a diffuse growth of small cells, and the survival and recurrence details after 11 years, accompanied by CD30-positive large cell transformation. The patient\'s condition after treatment has improved and is currently in good health.

Cardiac tumors range from benign to high grade malignancies. The incidence of cardiac involvement either by primary, or secondary tumors during autopsy is reported to be extremely low. Extranodal NK/T-cell lymphoma (ENKTL), nasal type is an unusual type of lymphoma. The skin is the second most common site of involvement after the respiratory tract. We present a case of a 63-year-old male, who was recently diagnosed with ENKTL, nasal type, who received chemotherapy, and died without any evident cause. The corpse was referred for routine medicolegal examination. Macroscopical determination of the cause of death was not feasible and subsequent histopathological examination revealed heart infiltration by ENKTL that was found in vivo in cutaneous lesions. Similar infiltrations existed in the pancreatic tissue. To the best of our knowledge, myocardial infiltration of ENKTL, inducing severe myocardial lesions that eventually caused death, is rare, with limited cases reported in the literature.

BACKGROUND: Extranodal NK/T-cell lymphoma (ENKTCL), nasal type is a very rare and aggressive non-Hodgkin lymphoma. Most commonly it occurs in the upper aerodigestive tract. But, it can also manifest at locations such as the skin, soft tissue, gastrointestinal tract (GI), lungs, testis, etc. These locations are designated as extranasal ENKTCL. The patients with the latter have often more adverse clinical features and poorer survival rate compared with nasal sites. We present a case of an 83-year-old patient with a primary ENKTCL, nasal type, with extranasal presentation in the right upper eyelid.
METHODS: Materials for the literature review was obtained by a comprehensive search on PubMed, which yielded 82 eligible cases with extranasal ENKTCL.
RESULTS: Sixty-eight cases (83 %) were localized as primary ENKTCL in the lungs (17), central nervous system (CNS) (14), testis (11), GI-tract (7), skin (6), orbit and intraocular tissue (4), pancreas (2), adrenal gland (2), breast (1), etc. 14 cases (17 %) presented as extended or disseminated diseases involving exclusively organs outside the upper aerodigestive tract. There was no systematic pattern of organ involvement in the extended/disseminated ENKTCL. 63 % of the patient with localized extranasal ENKTCL and about 50% of patients with extended/disseminated disease were reported to have died of the disease. Treatment strategies varied with no preferred option. Among the used treatment options were chemotherapy, radiotherapy, surgery, stem cell transplantation alone or in different combinations.
CONCLUSIONS: ENKTCL is a highly aggressive disease which may present in extranasal areas. Although the tumors respond to both chemotherapy and radiotherapy, durable complete remissions are very rare.

The present study reports a case of extranodal natural killer (NK)/T-cell lymphoma, nasal type, involving the skin. The clinical manifestations, pathological characteristics, treatment and prognosis of the case were analyzed to improve the clinical diagnosis and treatment for this disease. The patient was a 56-year-old male, presenting with dark red nodules and plaques that had been visible on the nose for half a year. Based on the skin lesions and histopathological and immunohistochemical examination results, the patient was diagnosed with extranodal NK/T-cell lymphoma, nasal type. This disease has unique histopathological and immunohistochemical features and a high malignancy. The condition tends to be misdiagnosed and has a poor prognosis, but seldom involves the skin. In the present case, only radiotherapy was performed, with no relapse occurring within 6 months.

L-asparaginase (L-Asp)-associated pancreatitis (AAP) occurs occasionally; however, this side-effect has predominantly been observed among pediatric patients. Usually, it is not life-threatening and generally responds to intensive medical therapy. The present study presents a rare case of lethal AAP in an adult. The patient was recently diagnosed with extranodal natural killer/T-cell lymphoma (ENKTL), nasal type, and the chronic hepatitis B virus (HBV) infection and was receiving L-Asp as part of a chemotherapy regimen. Severe acute pancreatitis occurred and the patient succumbed 72 h after completion of chemotherapy. The HBV infection and lipid disorders may have been potential risk factors for the development of severe acute pancreatitis in the patient.