Angiocentric T-Cell Lymphoma of the Tonsil: A Case Report

1996 ◽  
Vol 75 (5) ◽  
pp. 309-311 ◽  
Author(s):  
Lisa K. Helfend ◽  
Howard I. Finkle ◽  
Harvey M. Freedman

We present a case of an angiocentric T-cell lymphoma (ACTL) occurring in a 31-year-old woman with the Acquired Immunodeficiency Syndrome (AIDS). This patient had oropharyngeal pain, a non-healing superficially ulcerated right tonsil, and no obvious tumor mass. Initial tonsillar biopsy revealed a subtle, non-specific, perivascular lymphocytic infiltrate subjacent to an ulcerated and inflamed mucosal lining. A benign, although atypical, inflammatory process was suspected. The distinctive features in this case include the relationship of ACTL to the Epstein-Barr Virus (EBV), which is usually related to B-cell lymphoproliferative disorders, the unique non-midline location of the lesion, and the relationship of ACTL to AIDS. ACTL appears to represent a unifying pathologic entity encompassing formerly described angioinvasive lymphoid lesions including polymorphic reticulosis, midline lethal granuloma, and lymphomatoid granulomatosis. We present these subtle histopathologic features to alert otolaryngologists who may encounter these cases, especially in AIDS patients who present extensive diagnostic possibilities. To the best of our knowledge, this is the first case report of ACTL involving the tonsil.

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Yiting Li ◽  
Ivan Damjanov

Extranodal NK/T cell lymphoma is an uncommon malignancy usually involving the sinonasal area. We report an unusual case of extranodal NK/T cell lymphoma diagnosed in a 62-year-old Caucasian male who died of progressive cardiorespiratory failure but had no clinically detectable upper respiratory system lesions. The initial diagnosis was made cytologically on a sample of pericardial fluid that contained neoplastic lymphoid cells. These cells were positive for CD2, cytoplasmic CD3, and Epstein-Barr virus and negative for CD56. The diagnosis was confirmed at the autopsy, which disclosed lymphoma infiltrates in the myocardium, lungs, stomach, and pancreas. The death was caused by heart and lung failure due to uncontrollable arrhythmia and respiratory insufficiency due to the lymphoma infiltrates. To the best of our knowledge, this is the first case of extranodal NK/T cell lymphoma presenting with cardiopulmonary failure.


2021 ◽  
Vol 14 (3) ◽  
pp. e240995
Author(s):  
Constantin Manole ◽  
Eric Farrell ◽  
Emer Lang

Nasal-type Natural Killer/T-cell Lymphoma (NKTL) is a rare form of extranodal non-Hodgkin’s lymphoma, typically arising in the nasopharynx and displaying an aggressive and ultimately fatal clinical course. The disease is linked to Epstein-Barr virus infection and is endemic to Asia and South America, but extremely few cases have been reported in Europe. We present two cases of nasal NKTL unexpectedly diagnosed in elderly patients, following very different presentations to our otolaryngology service. The first case is that of a 73-year-old Irish man with recurrent nasal vestibulitis despite antibiotic treatment. The second case involves a 79-year-old Irish woman presenting with a large mass on the hard palate, found to invade into the floor of the nose. NKTL can pose diagnostic challenges, as the initial clinical presentation can be non-specific and overlap with other nasal conditions, leading to a delay in diagnosis. Biopsy with histopathological and immunohistochemistry analysis is required to establish the definitive diagnosis. Treatment involves multidisciplinary input from radiotherapy and medical oncologists. Clinicians must be aware of this disease and have an index of suspicion when dealing with persistent or aggressive nasal conditions.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S81-S81
Author(s):  
J Lanceta ◽  
W Xue ◽  
M Hurford ◽  
H Wu

Abstract Casestudy Epstein-Barr virus (EBV)-associated peripheral T-cell lymphomas are a group of aggressive neoplasms with a geographic predilection for South America and Asia, but are very rare in Western populations. Results We report a case of a 74-year-old Caucasian female who presented with pancytopenia and B symptoms with EBV-IgG detected on admission. Past medical history included: ITP, chronic urticaria, and recently diagnosed myelodysplastic syndrome (MDS) on bone marrow biopsy one month prior to admission. Excisional biopsies of an enlarged right neck lymph node (repeated within 6 months) and right axillary lymph node five years ago were negative for a lymphoproliferative disorder at the time. Repeated bone marrow biopsy, performed during the current admission, confirmed the diagnosis of MDS, with scattered T-cells without aberrant immunophenotype. Despite aggressive treatment from multiple specialties, the patient deteriorated and expired four weeks later from complications of MDS. At autopsy, there was diffuse lymphadenopathy involving the mediastinum, axilla, pelvis and peripancreatic fat. Lymph node sections demonstrated nodal architecture effacement by diffuse, vaguely nodular lymphoid infiltrates. Histologically, the infiltrates were composed of medium to large lymphocytes with round to slight irregular nuclei, rare Reed-Sternberg-like multinucleated cells, clumped chromatin, and indistinct nucleoli. Individual cell necrosis was abundant with mitotic figures readily identifiable. Immunohistochemistry revealed CD2+ CD3+ neoplastic T-cells that co-express MUM1 and a subset of CD30, while negative for CD4, CD5, CD8, CD56, ALK1, and TDT. EBV-encoded RNA in-situ hybridization was focally positive. The final postmortem diagnosis was peripheral T-cell lymphoma, not otherwise specified (NOS), with focal EBV positivity. Conclusion Co-existence of a de-novo MDS and non-Hodgkin lymphoma without any prior chemotherapeutic exposure is a highly unusual finding, although MDS-like presentations can occur with EBV-associated lymphomas. Peripheral T-cell lymphoma, NOS is an aggressive lymphoma and EBV positivity has been found correlated with a poor prognosis. This case demonstrates how postmortem examination remains an important tool in clinical- pathological correlation and highlights the potential pathogenetic role EBV plays in MDS and T-cell lymphoma.


Author(s):  
Sindhu Kilaru ◽  
Soumya Surath Panda ◽  
Sourav Mishra ◽  
Debahuti Mohapatra ◽  
Spoorthy Kolluri ◽  
...  

Extra-nodal Natural killer/T cell lymphoma (ENKTL) is a well-defined and highly aggressive form of NonHodgkin’s lymphoma with a scarcity of cases reported in literature. The most common primary site of involvement is the nasal cavity followed by skin and the gastrointestinal tract (GIT). Cutaneous involvement is a rarity. More than 95% of cases are usually in association with Epstein Barr Virus (EBV) infection. EBV negative ENKTL can be similar in clinical, pathological, and prognostic characteristics with EBV positive ENKTL. This malignancy is usually characterized by its poor prognosis irrespective of clinical stage and therapy. We describe here, a 58-year-old man presenting with multiple nodular lesions over legs and trunk, had an ileal perforation later, and was diagnosed as ENKTL on the ileal biopsy specimen. This case is being reported in view of the fulminant clinical course of the disease, simultaneous involvement of the GIT and skin without nasal or midline involvement, the usefulness of immunohistochemistry in arriving at a diagnosis, and EBV negativity which is quite rare in the Asian population.


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