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Cutaneous Lymphomas
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Emily Correia, Shalini Krishnasamy, Neda Nikbakht
Clinical presentation: Extranodal NK/T-cell lymphoma, nasal type is commonly seen in Asia, Central America, and South America and predominantly affects adult males. Typical presentation involves multiple plaques or tumors on the trunk and extremities, or a destructive midfacial tumor previously called lethal midline granuloma. Fever, malaise, weight loss, and other systemic symptoms may be seen, along with HLH and ulceration of lesions.
Test Paper 5
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
CT scanning is likely to reveal a common mucosal thickening similar to that encountered in chronic sinusitis. However, the presence of bony erosions of the sinonasal wall is very suspect of the diagnosis of a granulomatous disease. Bone erosion commonly affects the lamina papyracea (orbital wall), inter sino-nasal wall and nasal septum. Bone destruction usually involves the nasal septum and then extends to the sinonasal wall, destroying the turbinates. Differential diagnoses including traumatic lesions (accidental, iatrogenic) or toxic lesions (cocaine abuse, chromium salts) should be considered. Isolated septal perforation should suggest the diagnosis of GP. There are no specific imaging findings to distinguish GP from ‘lethal midline granuloma’, but the destructive lesions seem to be more extensive in lethal midline granuloma.
Nasal Cavity and Paranasal Sinus Cancer
Published in Dongyou Liu, Tumors and Cancers, 2017
Clinical presentation of esthesioneuroblastoma (or olfactory neuroblastoma) is usually secondary to nasal stuffiness and rhinorrhoea or epistaxis. Large tumor extending into the intracranial compartment (25%–30% at diagnosis) may cause anosmia. Lethal midline granuloma is associated with rhinorrhea, epistaxis, nasal stuffiness, obstruction, and pain.
Recurrent natural killer/T-cell lymphoma of the lacrimal sac and nasolacrimal duct in a 59-year-old Caucasian woman
Published in Orbit, 2019
Daniel L. Jones, Suzanne W. van Landingham, John-Paul J. Yu, Mark J. Lucarelli
Extra-nodal natural killer (NK)/T-cell lymphoma is an aggressive hematologic malignancy with cells predominantly of NK-cell origin. It typically occurs in the nose, and rarely appears in the ocular adnexa.1 Historically, when it occurred in the central face, it was included in the category “lethal midline granuloma,” along with several other inflammatory and neoplastic entities.2,3 Most ocular adnexal lymphoma cases are extra-nodal marginal zone (or mucosa-associated lymphoid tissue [MALT]) lymphoma,4,5 while very few are of NK/T-cell type.2,5,6 To the best of our knowledge, only six documented cases of NK/T-cell lymphoma involving the lacrimal sac and/or nasolacrimal duct have been published in the literature,7–11 and only one of these cases may have been a recurrence.11 We report an unusual case of an extra-nodal NK/T-cell lymphoma recurring in the lacrimal sac and nasolacrimal duct in a Caucasian adult.
Non-Hodgkin’s lymphoma of the oral cavity and maxillofacial region: a pathologist viewpoint
Published in Expert Review of Hematology, 2018
Mahmoud Rezk Abdelwahed Hussein
EBV tests are usually positive in NKTCL [88,89]. Nagata and his colleagues were the first to establish two EBV-positive cell lines (SNK-6 and SNT-8 cell lines) from primary nasal NKTCL using high-dose recombinant interleukin 2. The primary tumors had essentially similar clinicopathologic features. The SNK-6 cell line had an NK-cell immunophenotype (CD3- CD4- CD8- CD19- CD56 + T-cell receptor (TCR) alpha/beta- TCR gamma/delta-) and unrearranged TCR and immunoglobulin heavy-chain genes. The SNT-8 immunophenotype was CD3+ CD4- CD8- CD19- CD56+ TCR alpha/beta- TCR gamma/delta+ and rearranged TCR beta-, gamma- and delta-chain genes [92]. Harabuchi et al. reported the presence of EBV-DNA and EBV oncogenic proteins in this tumor. The tumor cells produce several autocrine cytokines such as interleukin (IL) −9, IL-10, and interferon-γ-inducible protein-10. The serum EBV-DNA copy number is used as a prognostic tumor biomarker. Therapeutic modalities with favorable outcome include combined arterial infusion chemotherapy, from the superficial temporal artery and radiotherapy [84]. Harabuchi et al. examined five cases of lethal midline granuloma. The patients had high titers of antibodies to EBV. On histology, the lesions proved to be peripheral T-cell lymphomas. Molecular analysis (using southern blotting and in-vitro hybridization) revealed EBV-DNA and EBV-determined nuclear antigen. Immunofluorescence and northern blotting analyses revealed the expression of T-cell surface markers, EBNA2 gene, and latent membrane protein gene in the neoplastic cells[93].
Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas
Published in Expert Review of Hematology, 2019
Eric Tse, Rex Au-Yeung, Yok-Lam Kwong
Before the advent of modern immunophenotyping, NK-cell malignancies have been known by different names. Lethal midline granuloma referred to destructive midline facial tumors that led relentlessly to death [4]. As the polymorphic infiltrate comprised atypical lymphoid cells and inflammatory cells, the tumor was later described morphologically as polymorphic reticulosis. With the improvement in immunohistochemistry, the detection of cytoplasmic CD3ε led to the classification of the tumor as a T-cell lymphoma. Owing to a propensity of the neoplastic cells to invade and destroy blood vessels, the lymphoma was classified as an angiocentric T-cell lymphoma in the REAL classification [5].