ABSTRACT
Melanoma is considered the most lethal skin cancer and its incidence has increased during the past decades. About 10 % of cases are classified as hereditary melanoma. Genetic predisposition usually manifests itself clinically as early onset and multiple cutaneous melanomas. Several genes have been identified as involved to melanoma susceptibility, some of them still with unknown clinical relevance. Beyond melanoma, the affected families are also more prone to develop other malignancies, such as pancreatic cancer. The identification of risk families and involved genes is of great importance, since different forms of oncological surveillance are recommended. However, well established guidelines to standardize both the selection of individuals and the genetic panel to be requested are still lacking. Given the importance of the genetic counseling and testing in the context of clinical suspicion of hereditary melanoma, this paper aims to review the literature regarding genetic panel indications worldwide.
Subject(s)
Melanoma , Neoplastic Syndromes, Hereditary , Pancreatic Neoplasms , Skin Neoplasms , Genetic Counseling , Genetic Predisposition to Disease , Humans , Melanoma/diagnosis , Melanoma/epidemiology , Melanoma/genetics , Skin Neoplasms/diagnosis , Skin Neoplasms/epidemiology , Skin Neoplasms/geneticsABSTRACT
Extranodal NK/T-cell lymphoma nasal type is a rare disease that mainly affects the nasal cavity and paranasal sinuses of males in the fifth decade of life. It has aggressive and locally destructive behaviour, and can be complicated by the hemophagocytic syndrome, conferring high lethality to the disease. This article describes a case of NK/T-cell lymphoma nasal type in a previously healthy patient, exemplifying its rapid and fulminant course.
Subject(s)
Lymphohistiocytosis, Hemophagocytic/pathology , Lymphoma, Extranodal NK-T-Cell/pathology , Nose Neoplasms/pathology , Epstein-Barr Virus Infections/complications , Fatal Outcome , Humans , Immunohistochemistry , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/virology , Lymphoma, Extranodal NK-T-Cell/diagnosis , Lymphoma, Extranodal NK-T-Cell/virology , Male , Middle Aged , Nose Neoplasms/diagnosis , Nose Neoplasms/virology , SyndromeABSTRACT
Abstract: Extranodal NK/T-cell lymphoma nasal type is a rare disease that mainly affects the nasal cavity and paranasal sinuses of males in the fifth decade of life. It has aggressive and locally destructive behaviour, and can be complicated by the hemophagocytic syndrome, conferring high lethality to the disease. This article describes a case of NK/T-cell lymphoma nasal type in a previously healthy patient, exemplifying its rapid and fulminant course.
