Clinicopathologic and molecular features in cutaneous extranodal natural killer–/T-cell lymphoma, nasal type, with aggressive and indolent course

Fried, Isabella; Artl, Monika; Cota, Carlo; Müller, Hansgeorg; Bártolo, Elvira; Boi, Sebastiana; Chiarelli, Concetta; Vale, Esmeralda; Schmuth, Matthias; Wiesner, Thomas; Speicher, Michael R.; Cerroni, Lorenzo

doi:10.1016/j.jaad.2013.11.028

Cited by 15 publications

(9 citation statements)

References 20 publications

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“…103 Array CGH screens the entire genome for CNAs, but false-negative results occur when the percentage of tumour cells in the specimens is below 50%, e.g., due to tumour-infiltrating lymphocytes or other admixed non-neoplastic (e.g., stromal) cells. Most importantly, array CGH only detects CNAs, but no other genomic aberrations such as small indels, point mutations, and or balanced translocations.…”

Section: Diagnosis Of Spitz Tumoursmentioning

confidence: 99%

Genomic aberrations in spitzoid melanocytic tumours and their implications for diagnosis, prognosis and therapy

et al. 2016

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Summary Histopathological evaluation of melanocytic tumours usually allows reliable distinction of benign melanocytic naevi from melanoma. More difficult is the histopathological classification of Spitz tumours, a heterogeneous group of tumours composed of large epithelioid or spindle-shaped melanocytes. Spitz tumours are biologically distinct from conventional melanocytic naevi and melanoma, as exemplified by their distinct patterns of genetic aberrations. Whereas conventional naevi and melanoma often harbour BRAF mutations, NRAS mutations, or inactivation of NF1, Spitz tumours show HRAS mutations, inactivation of BAP1 (often combined with BRAF mutations), or genomic rearrangements involving the kinases ALK, ROS1, NTRK1, BRAF, RET, and MET. In Spitz naevi, which lack significant histological atypia, all of these mitogenic driver aberrations trigger rapid cell proliferation, but after an initial growth phase, various tumour suppressive mechanisms stably block further growth. In some tumours, additional genomic aberrations may abrogate various tumour suppressive mechanisms, such as cell-cycle arrest, telomere shortening, or DNA damage response. The melanocytes then start to grow in a less organised fashion, may spread to regional lymph nodes, and are termed atypical Spitz tumours. Upon acquisition of even more aberrations, which often activate additional oncogenic pathways or reduce and alter cell differentiation, the neoplastic cells become entirely malignant and may colonise and take over distant organs (spitzoid melanoma). The sequential acquisition of genomic aberrations suggests that Spitz tumours represent a continuous biological spectrum, rather than a dichotomy of benign versus malignant, and that tumours with ambiguous histological features (atypical Spitz tumours) might be best classified as low-grade melanocytic tumours. The number of genetic aberrations usually correlates with the degree of histological atypia and explains why existing ancillary genetic techniques, such as array comparative genomic hybridisation (CGH) or fluorescence in situ hybridisation (FISH), are capable of accurately classifying histologically benign and malignant Spitz tumours, but are not very helpful in the diagnosis of ambiguous melanocytic lesions. Nevertheless, we expect that progress in our understanding of tumour genomics and progression will refine the classification of melanocytic tumours in the near future. By integrating clinical, pathological, and genetic criteria, distinct tumour subsets will be defined within the heterogeneous group of Spitz tumours, which will eventually lead to improvements in diagnosis, prognosis and therapy.

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Section: Diagnosis Of Spitz Tumoursmentioning

confidence: 99%

Genomic aberrations in spitzoid melanocytic tumours and their implications for diagnosis, prognosis and therapy

et al. 2016

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“…Initially, 1998 records were retrieved from electronic databases and manual reference searches. After removing duplicates and the studies that did not meet the inclusion criteria, 61 articles (n = 271) were finally included (Figure ) 7,8,16,19,26–82 . After including 18 patients from the Asan Medical Center database, a total of 289 patients were identified.…”

Section: Resultsmentioning

confidence: 99%

Clinicopathological and prognostic study of primary cutaneous extranodal natural killer/T‐cell lymphoma, nasal type: A systematic review

Jung

Yang

Won

et al. 2021

The Journal of Dermatology

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Comprehensive studies of primary cutaneous extranodal natural killer/T‐cell lymphoma (PCENKTL) are scarce. The objectives of this study are to describe PCENKTL in terms of its clinical features, histopathology, immunophenotypes, and prognosis, and to analyze factors affecting patient survival outcomes. We searched four databases and include studies with extractable data. We also searched the Asan Medical Center database for cases of PCENKTL. We include a total of 289 patients. The mean age at diagnosis was 52.8 years and the female to male ratio was 1:1.2. The most common clinical morphology was a subcutaneous nodule, followed by ulceration. About half of the patients presented with disseminated skin lesions. The median overall survival was 12.0 months and the 5‐year survival rate was 22.0%. There was no correlation between the clinical morphology or the histopathological features of the skin lesions with the patient outcomes. Advanced TNM stage, a disseminated skin lesion, tumor location on the leg or trunk, the presence of B symptoms, and a high International Prognostic Index score were associated with a worse prognosis, and chemoradiotherapy was associated with a better survival outcome as compared with chemotherapy alone in univariable analyses. In multivariable analyses, only advanced TNM stage and tumor location on the leg were associated with a worse prognosis. In conclusion, PCENKTL is an aggressive cutaneous lymphoma and its prognosis is associated with TNM stage and tumor location.

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“…En cuanto a las alteraciones genéticas implicadas, las más frecuentes consisten en deleciones en 6q21 y 7p, y ganancia en 7q. La deleción de 6q21 se ha relacionado con la pérdida de expresión de genes supresores tumorales, como PRMD1 y FOXO3 5,6 .…”

Section: Caso Clínicounclassified

Linfoma de células NK extranodal tipo nasal: reporte de un caso exitoso asociado a trasplante de médula ósea

Gentile¹,

Abaca²,

Senarega³

et al. 2022

MCUT

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El linfoma NK extranodal tipo nasal es un linfoma no Hodgkin que suele evolucionar de manera rápida y agresiva, y se encuentra relacionado en general con la presencia del virus Epstein-Barr. La piel es el segundo sitio de afectación en frecuencia, generalmente por contigüidad. Existen diferentes estrategias terapéuticas según se trate de enfermedad localizada o avanzada. Presentamos el caso de un paciente con moderada respuesta a esquemas quimioterápicos y buena sobrevida luego de trasplante alogénico de médula ósea.

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Clinicopathologic and molecular features in cutaneous extranodal natural killer–/T-cell lymphoma, nasal type, with aggressive and indolent course

Cited by 15 publications

References 20 publications

Genomic aberrations in spitzoid melanocytic tumours and their implications for diagnosis, prognosis and therapy

Genomic aberrations in spitzoid melanocytic tumours and their implications for diagnosis, prognosis and therapy

Clinicopathological and prognostic study of primary cutaneous extranodal natural killer/T‐cell lymphoma, nasal type: A systematic review

Linfoma de células NK extranodal tipo nasal: reporte de un caso exitoso asociado a trasplante de médula ósea

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