Acute respiratory distress syndrome emerging after surgical debridement in a patient with extranodal natural killer/T cell lymphoma.

BACKGROUND: Extranodal natural killer/T cell lymphoma (ENKL) is a rare subtype of non-Hodgkin lymphoma, and lung involvement is extremely rare. The patients with pulmonary ENKL always presented unspecific symptoms of the respiratory system, such as cough with sputum and varying degrees of fever, while developing into acute respiratory distress (ARDS) was seldomly reported, especially promoted by the surgical procedure. CASE PRESENTATION: Here we describe a patient with nasal ENKL and most likely lung dissemination that was regarded as an infection at first. After nonresponse to a period of anti-infective therapy, this patient received surgical debridement. While the histopathology did not show the evidence of infection, but consistent with ENKL. The patient got refractory hypoxemia rapidly after surgery, with the LDH surging to a much higher level than before surgery. The ARDS was diagnosed, and he died on the 5th day after surgery. We postulate that ARDS was due to aggressive lymphoma proliferation promoted by the surgical procedure. CONCLUSIONS: Pulmonary ENKL developing into ARDS was scarce, and was likely attributed to the aggressive tumor cell proliferation after surgery in this case.

[A Case of Extranodal NK/T-Cell Lymphoma with Perforation of the Small Intestine].

A rare case of extranodal NK/T-cell lymphoma(ENKL)with small intestinal perforation is reported. A 92-year-old man was admitted for a loss of consciousness. Computed tomography(CT)scan revealed the presence of an intraperitoneal abscess that was drained. Two days later, the drained fluid changed to intestinal juice, and intestinal perforation was suspected. The patient underwent surgery which revealed a 1 cm perforation site in the ileum. A high fever continued after surgery, and malignant lymphoma was diagnosed from pathological findings; however, further treatment could not be performed. He died 24 days after the operation. Pathological dissection revealed metastasis of ENKL at the systemic lymph nodes.

Primary extranodal natural killer/T-cell nasal-type lymphoma of spine: A case report.

RATIONALE: Primary Extranodal Natural Killer/T-Cell Nasal-Type Lymphoma (ENKTCL) of spine is rarely reported. This case study presents a rare case of ENKTCL originating from the sixth thoracic vertebra. PATIENT CONCERNS: Here, we present a case of 49-year-old Asian male with chest and back pain. Physical examination revealed the myodynamia of both lower limbs decreased from IV degree to 0 degree, reflexes of both lower limbs and a large area of cutaneous sensation below xiphoid process disappeared in 3 days. DIAGNOSES: In immunophenotype analysis, CD2, CD3, CD7, and CD68 were positive and CD56 was suspiciously positive. Granzyme B and T-cell intracellular antigen (TIA-1) were also positive and in situ hybridization was positive for Epstein-Barr virus-encoded mRNA (EBER). Ki-67 was 60%+. Nuclide bone scan showed that the nuclide was unusually concentrated in the sixth thoracic vertebra which considered extremely active and slightly concentrated in the right sacrolilac joint. Magnetic resonance imaging detected an abnormal signal in the sixth thoracic vertebra with corresponding paravertebral and intraspinal occupying lesion. Based on the above features, a diagnosis of ENKTCL was made. INTERVENTIONS: This patient was treated with surgery and symptomatic supportive treatment. OUTCOMES: The myodynamia of patient's both lower limbs were elevated to I degree after the operation with chest and back pain partly relieved. However, the patient died about 3 months later. LESSONS: ENKTCL could originate from spine. Clinicians should be alert for early stage diagnose and distinguish it from some common spinal tumor such as neurofibroma and hemangioma.

Extranasal extranodal NK/T cell lymphoma: A post-transplantation lymphoproliferative disease.

Extranodal NK/T cell lymphoma (ENKTL) is a rare form of non-Hodgkin lymphoma. It mostly occurs in the upper respiratory tract. Cutaneous involvement can be seen among the extranasal ENKTLs. After solid organ and haematopoietic stem cell transplantation, post-transplantation lymphoproliferative disease because of immunosuppressive therapy is usually B cell-derived; T and NK/T cell-derived disease is rarely seen. A 43-year-old female patient who had renal transplantation 14 years ago presented with cutaneous ulceration and subcutaneous nodules located in the abdomen. The patient was diagnosed with ENKTL, nasal type. Although it is rare, ENKTL nasal type is a lymphoproliferative disease that should be considered in the differential diagnosis of ulcerated cutaneous tumoural lesions.

Primary adrenal extranasal NK/T cell lymphoma with subcutaneous involvement demonstrated on FDG PET/CT: A clinical case report.

RATIONALE: Primary adrenal non-Hodgkin lymphomas are predominant diffuse large B cell lymphoma with frequently bilateral adrenal involvement, but the occurrence of nasal type extranodal NK/T cell lymphoma is relatively rare. PATIENT CONCERNS: A 40-year-old woman complaining of left back pain for 2-month was admitted to our department. DIAGNOSIS: Based on the feature of enhanced computed tomography (CT) images which showed huge bilateral well-defined adrenal masses with heterogeneous enhancement, she was tentatively diagnosed as having primary adrenal malignancy. Postoperative pathology revealed the diagnosis of primary adrenal Epstein-Barr virus-associated nasal type extranodal NK/T-cell lymphoma. INTERVENTIONS: Then, she underwent F-fluoro-2-deoxy-D-glucose (F-FDG) positron emission tomography (PET)/CT examination for staging, which showed homogeneously increased FDG uptake in the right adrenal gland and left thigh subcutaneous lesion, as well as heterogeneous increased FDG uptake in the left adrenal gland region with no abnormal uptake in the nasal cavity. Subsequently, the patient has performed 7 cycles of gemcitabine, L-asparaginase, ifosfamide, dexamethasone, etoposide (GLIDE) regimen and autologous stem cell transplantation. OUTCOMES: Fortunately, the subsequent 2 follow-up FDG PET/CT scans within 1 year revealed complete resolution with no abnormal FDG uptake in the initially involved sites after 7 cycles of GLIDE chemotherapy and autologous stem cell transplantation. LESSONS: The enhanced CT and FDG PET/CT features of primary adrenal extranasal NK/T cell lymphoma are huge bilateral well-defined adrenal masses with heterogeneous enhancement, high FDG uptake, especially with subcutaneous involvement. And the awareness of this entity may help clinicians to differentiate it from other primary adrenal tumors and make reasonable therapeutic strategies. Besides, FDG PET/CT scan is very useful for the treatment follow-up of the primary adrenal extranasal NK/T cell lymphoma.

Primary cardiac natural killer/T-cell lymphoma, a very rare form of lymphoma.

Extranodal natural killer/T-cell lymphoma is a rare non-Hodgkin lymphoma that is divided into nasal, non-nasal, and aggressive/leukemia subtypes, according to anatomic origin and clinical manifestations, with each subtype carrying a different prognosis. We present a case of primary cardiac natural killer/T-cell lymphoma with extension to other organs in a 38-year-old man, to highlight the role of imaging in categorizing nasal versus non-nasal types. This distinction has relevant implications for patient care because the non-nasal type has a much lower survival rate.

Recurrent natural killer/T-cell lymphoma of the lacrimal sac and nasolacrimal duct in a 59-year-old Caucasian woman.

A 59-year-old Caucasian woman with past medical history significant for Natural Killer (NK)/T-cell lymphoma of the right nasal septum in remission for nine months presented after surveillance PET-CT imaging revealed increased metabolic activity in the right nasolacrimal duct. She also reported ipsilateral epiphora starting around this time. The lacrimal sac and nasolacrimal ductal mucosa were biopsied via an external approach. Pathologic evaluation revealed a proliferation of lymphoid cells with necrotic tissue. Immunohistochemical staining demonstrated predominantly CD3+, EBER+, and CD56+ cells indicating recurrent NK/T-cell lymphoma. This case describes an unusual presentation of recurrent NK/T-cell lymphoma involving the lacrimal excretory system in a Caucasian adult. Recurrent malignancy should be considered in the differential of any patient with a history of a lymphoproliferative disorder near the lacrimal drainage system who presents with new onset epiphora.

Rare case of low-grade extranodal NK/T-cell lymphoma, nasal type, arising in the setting of chronic rhinosinusitis and harboring a novel N-terminal KIT mutation.

BACKGROUND: Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT), is a rare aggressive subtype of non-Hodgkin lymphoma characterized by angioinvasion, angiodestruction, necrosis and strong association with Epstein-Barr virus (EBV). ENKTCL-NT occurs worldwide and is more prevalent in Asian and the Native American populations of Mexico, Central and South America. It represents approximately 10% of all peripheral T-cell lymphomas worldwide. The aim of this report is to present a rare case of ENKTCL-NT with an unusually indolent clinical course and low-grade histopathologic features. CASE PRESENTATION: A 71-year-old Asian woman with a long-standing history of seasonal rhinosinusitis presented with persistent nasal congestion, cough, and fever unresponsive to antihistamines and antibiotics. Histopathological evaluation of a polypoid nasal mass revealed an atypical infiltrate with predominantly small lymphoid cells that were CD2+, surface CD3-, cytoplasmic CD3+, CD5(dim)+, CD7(dim)+, cytotoxic markers (granzyme B and perforin)+, EBER+ and CD56-. The Ki-67 proliferative index was very low (< 1%). T-cell receptor gamma gene rearrangement studies were positive for a monoclonal rearrangement, and sequencing studies identified a novel KIT mutation (p. K167 M, c. 500 A > T). A diagnosis of low-grade ENKTCL-NT was rendered. CONCLUSIONS: Our case of ENKTCL-NT is unusual due to (1) an indolent clinical course (2) low-grade histopathologic features including a low proliferative index (3) lack of CD56 expression and (4) a novel KIT mutation. This case raises awareness of the existence of a subset of cases of ENKTCL-NT that can potentially be misdiagnosed as a reactive process, particularly in patients with recurrent chronic rhinosinusitis.

Breakthrough Candida guilliermondii (Meyerozyma guilliermondii) fungemia after cord blood transplantation for extranodal NK-cell lymphoma with azole prophylaxis.

Fluconazole (FLCZ) is an azole antifungal agent and it has shown excellent clinical activities in suppressing fungemia with Candida albicans after hematopoietic stem cell transplantation. Increased administration of prophylactic FLCZ seems to have given rise to the relatively higher incidence of more resistant Candida non-albicans infection. We present a case with a rare breakthrough fungemia with C. guilliermondii after cord blood transplantation for Extranodal NK cell Lymphoma, nasal type (ENKL), during antifungal prophylaxis with FLCZ. High level of caution is needed for the breakthrough, especially after long-term azole administration.

[The clinical observation of NK/T-cell lymphoma of the nasal type]. / Klinicheskoe nabliudenie NK/T-kletochnoi limfomy nazal'nogo tipa.

The objective of the present work was to present the results of the clinical analysis of the patient presenting with natural killer (NK)/T-cell lymphoma of the nasal type. We undertook the analysis of the medical documentation concerning the case of interest. It was shown that the development of progressive perforation of the nasal septum and the pronounced destructive changes in the intranasal and adjacent structures following the endonasal surgical interventions made necessary differential diagnostics between the condition under consideration and certain latent disorders (such as Wegener's granulomatosis, leprosy, syphilis, leishmaniasis, dirofilariasis tuberculosis, etc.). The study has demonstrated that the negative results of the analysis imply the necessity of special attention to the possibility of development of oncological diseases including hematological disorders (e.g. NK/T-cell lymphoma) and the repeat careful follow-up examination of the patients by the experienced experts.

[A case of nasal NK/T-cell lymphoma].

A 29-year-old male patient with extranodal NK/T-cell lymphoma, a nasal type lymphoma with involvement of skin as the first symptom, was reported. The patient presented with swelling in the left side of the nose and suffered intermittent fever for 1 month. The fester in the oral mucosa and skin under the left nostril and redness, and the swelling on the orbit of the left eye lasted for 1 week. Physical examination showed that the left side of nose was swelling, and the skin below the left nostril was anabrotic and crusted. There were different ulcers in his jaws and buccal mucosa. Bilateral eyelid was redness and swelling, especially in the left side. Binocular conjunctival was congestive. The diagnosis of extranodal NK/T-cell lymphoma (nasal type) was confirmed by biopsy and immunohistochemistry.

Epiphora as first presenting sign of primary extranodal NK/T-cell lymphoma of the lacrimal sac.

A 28-year-old Caucasian female presented with a six-month history of epiphora of the right eye. Diagnostic lacrimal syringing revealed obstruction of the right nasolacrimal duct. During external dacryocystorhinostomy (DCR), the lacrimal sac had an unusual papillary appearance. Frozen sections suggested malignant lymphoid cells. Pathologic examination revealed extranodal NK/T-cell lymphoma, nasal type. Imaging revealed a mass lesion centered on the lacrimal sac. The authors describe the youngest Caucasian patient with a primary NK/T-cell lymphoma of the lacrimal sac, as well as the first case presenting with epiphora as the sole presenting symptom, reported in the literature. Extranodal NK/T-cell lymphomas rarely occur in the orbit, and are known to be rapidly growing, aggressive lesions. Despite chemotherapy, the patient succumbed to her disease twelve months after diagnosis. Lacrimal surgeons should maintain a high index of suspicion in all cases of primary nasolacrimal duct obstruction. Pre- or intra-operative clinical suspicion of malignancy in the lacrimal sac necessitates biopsy.

Nasal NK/T-cell lymphoma: A tragic case.

INTRODUCTION: Nasal NK/T-cell lymphoma is a rare clinicopathological entity, formerly called midline lethal granuloma. Following progress in histology and the routine use of immunohistochemistry, nasal NK/T-cell lymphoma was recognized as a distinct entity by WHO in 2001. CASE REPORT: The authors report the case of a 22-year-old, insulin-dependent diabetic woman, who presented with mid-facial inflammatory swelling following facial trauma, initially diagnosed and treated as cellulitis of the face. The subsequent course was rapidly progressive and fatal, with the development of midline destructive disease. Histological examinations concluded on NK/T-cell lymphoma. DISCUSSION: The various differential diagnoses of NK/T-cell lymphoma include gangrenous cellulitis, invasive mycotic rhinosinusitis, Wegener's granulomatosis, actinomycosis, and facial T-cell lymphoma. The clinical presentation of this case was atypical, resulting in delayed diagnosis and treatment. Treatment is based on radiotherapy and chemotherapy, but the prognosis remains very poor even when treatment is rapidly initiated.

[A Case of Extranodal Nasal Type, NK／T Cell Lymphoma in the Ileum with Perforated Peritonitis during Chemotherapy].

A 65-year-old woman complaining of melena and a high fever was admitted to our hospital. Colonoscopy revealed a large tumor with a wide ulcer in the terminal ileum, and nasal type, extranodal NK/T cell lymphoma was suspected via biopsy. Owing to rapid progression of hepatocellular damage with hepatomegaly and splenomegaly, chemotherapy with CHOP was initiated immediately. Two days later, the patient developed panperitonitis, and emergency laparotomy was performed. The tumor in the terminal ileum widely adhered to the bladder, where a deep perforated ulcer was found, for which palliative ileocecal resection was performed. Enlargement of the residual tumor caused an abdominal abscess, intestinal obstruction, and hepatic failure, and the patient died 75 days after surgery. In summary, we initiated chemotherapy to control the deteriorating systemic condition of the patient. However, nasal type, extranodal NK/T cell lymphoma is a rapidly progressing lymphoma occasionally accompanied by perforation of the tumor. Surgery preceding chemotherapy should be considered an alternative treatment.

Extranodal lymphomas of head and neck with emphasis on NK/T-cell lymphoma, nasal type.

PURPOSE: To determine the occurrence, clinical course and outcome of our patients with natural killer/T-cell lymphoma (NKTCL), nasal type. The nasal type of natural killer/T-cell lymphoma (NKTCL), is rare in the European population but is more frequent in Asia, Central and South America where it accounts for 15-20% of all non-Hodgkin lymphomas (NHL). This type of lymphoma is characterized by tissue necrosis and destruction of the nasal cavity and upper aero-digestive tract. The disease is closely associated with Epstein-Barr virus (EBV) infection and has an aggressive clinical course, poor clinical outcome and high resistance to chemotherapy. PATIENTS: 63 patients with NHL were treated in our department between 2006 and 2011. Eleven patients were diagnosed with extranodal lymphoma, 6 in the area of head and neck. Three of them belonged to NKTCL, nasal type group (4.7%), 1 was diffuse large B cell lymphoma and 2 were marginal zone lymphomas. CONCLUSION: Two out of 3 patients with nasal type NKTCL, had extremely aggressive disease resistant to applied chemotherapy (anthracycline containing regimen), while 1 patient remains in complete remission 6 years after diagnosis.

[Clinicopathologic features of intestinal natural killer/T-cell lymphoma].

OBJECTIVE: To study the clinicopathologic features, diagnosis and differential diagnosis of intestinal natural killer (NK)/T-cell lymphoma. METHODS: The clinical features, histopathology, immunohistochemical findings and follow-up data of 14 cases of intestinal NK/T-cell lymphoma were retrospectively reviewed. RESULTS: The male-to-female ratio was 9:5. The medium age of patients was 45 years. The sites of involvement included small intestine (6 cases), colon (6 cases) or both (2 cases). The main clinical manifestations were an abdominal mass, other gastrointestinal symptoms such as abdominal pain, as well as systemic symptoms such as fever and cachexia. Intestinal perforation complicated by acute peritonitis might occur in advanced disease. Histologically, the intestinal wall showed full-thickness infiltration by medium-sized atypical lymphoid cells with pleomorphic nuclei, prominent inflammatory background, angiocentric/angiodestructive growth pattern and coagulative necrosis. Immunohistochemical study showed that the tumor cells were positive for CD3ε, CD43, CD56, granzyme B and perforin. They were negative for CD20, CD79α and MPO. In-situ hybridization for Epstein-Barr virus encoded RNA (EBER) showed negative signals. A high proliferative index was demonstrated by Ki-67 immunostaining. Follow-up data of 8 cases were available, with duration of follow up ranging from 0.5 to 36 months. Five patients died within 20 months. CONCLUSIONS: Extranodal NK/T-cell lymphoma, nasal-type primarily involving intestine is rare and tends to carry an aggressive clinical course. The relatively non-specific clinical manifestations of intestinal NK/T-cell lymphoma may result in misdiagnosis in some cases. A comprehensive evaluation of clinical manifestations, pathologic features and immunohistochemical findings is essential for definitive diagnosis.

Synchronous adenocarcinoma and extranodal natural killer/T-cell lymphoma of the colon: a case report and literature review.

Extranodal natural killer/T-cell lymphoma (ENKTL) is a distinct subtype of non-Hodgkin's lymphoma and is rare in the colon. Synchronous adenocarcinoma and ENKTL of the colon has not been reported in the literature. In the present study, we report a 63-year-old male who suffered from intermittent bloody stools for 2 mo. He did not have fever, body weight loss or night sweat. Endoscopic and imaging studies revealed a 4.5-cm ulcerative mass in the ascending colon and a 3.0-cm polypoid, easy bleeding mass in the sigmoid colon, respectively. Thought to have double carcinoma of the colon, he received simultaneous right hemicolectomy and sigmoidectomy. The pathological diagnosis was a synchronous ENKTL (ascending colon) and adenocarcinoma (sigmoid colon). The literature on synchronous adenocarcinoma and malignant lymphoma of the colon was also reviewed.

First report of primary pancreatic natural killer/T-cell nasal type lymphoma.

BACKGROUND: Primary pancreatic lymphoma (PPL) is an extremely rare form of extranodal malignant lymphoma and pancreatic tumour. Natural killer/T-cell lymphoma is an aggressive rare form extranodal lymphoma with a predilection for the nasal cavity/nasopharynx, it can arise in other organs such as skin, testicles, spleen, adrenal, or GI tract, but the initial presentation of our patient in the pancreas is unreported. CASE PRESENTATION: We present a case of primary pancreatic natural killer/T-cell nasal type lymphoma in a 62-year-old man. The presenting symptoms were non-specific only for upper abdominal pain and weight loss. Imaging techniques showed the lesion was located in the head of pancreas. Computed tomography (CT) scanning and otorhinolaryngology examination were negative for nasopharyngeal lymphoma. The initial concern was for pancreatic tumor and the patient underwent pancreaticoduodenectomy. The diagnosis of primary pancreatic natural killer/T-cell nasal type lymphoma was established as the combination of NK-lineage antigens (TIA-1, granzyme B, CD56) with EBV-expression. CONCLUSIONS: This is the first case of primary pancreatic natural killer (NK)/T-cell nasal type lymphoma. PPL, although a rare pathologic entity, should be considered in the differential diagnosis for a large homogeneous mass with extrapancreatic extension in the head especially in those of normal serum CA 19-9 level.