Abstract Introduction Kaposi's Sarcoma (SK) is a vascular tumor that manifests clinically with single or multiple skin lesions and in the most aggressive forms also involves the mucous membranes, lymphnodes, and visceral organs. Over the years, four different clinical-epidemiological forms have been identified; classical, endemic, epidemic, and iatrogenic. Data from the RARECARENet project report an incidence rate of KS in Europe of 0.28 per 100,000 persons/year. The incidence increases with age: 0.18/100,000 from 0 to 44 years; 0.25/100,000 from 45 to 64 years; 0.69/100,000 from 65 years and older. The incidence in males is four times higher than in females. The risk factors for the development of KS reflect the epidemiology of the disease, and the two main promoters of the neoplasm are: HHV-8 infection and immunosuppression status. However, each clinical-epidemiological subtype of KS has peculiar risk factors. KS is diagnosed by histopathological and immunohistochemical analysis of the lesion. Treatment of this neoplasm is very heterogeneous, and the therapeutic strategies can be local or systemic. Follow-up of KS depends on the clinical-epidemiologic subtype, the extent of the disease, and the treatment chosen. Aim of the study Our study aimed to describe the characteristics of patients with Sarcoma di Kaposi who came to the observation of the Azienda Ospedaliera Universitaria di Padova (AOPD) and the Istituto Oncologico Veneto (IOV) between 1993 and 2022. The goal was to describe the demographic and clinico-pathological characteristics of patients, disease features, and the diagnostic-therapeutic pathway, through the creation of a unique database with the aim of better characterizing the disease, expanding knowledge, and providing better patient care. Materials and Methods This work is a retrospective cohort study. Clinical characteristics of patients, neoplasm, immunohistochemical data and treatment were extracted from a local database. Statistical analysis was performed using R 4.1 (R Foundation for Statistical Computing,Vienna, Austria) Results Considering the four clinical-epidemiological types of SK, there were 37 cases of classical SK (43.02%), 29 cases of epidemic SK (33.72%), 17 cases of iatrogenic SK (19.77%) and 3 cases of endemic SK (3.49%). The most common anatomical site appeared to be the lower limbs (51.16%). 67 patients (77.91%) had exclusively cutaneous lesions. 86.05% of patients were found to have comorbidities, including autoimmune (19.77%) and neoplastic (25.58%) diseases. 61.63% of patients had a single treatment, while in 38.37% of cases it was necessary to use several treatments combined in various ways. Treatment approaches were local and systemic. A lasting response (for at least 2 years) was achieved in about 65% of patients. Relapses occurred in about 22% of cases. The median follow-up is 5.8 years while the median interval for disease recurrence is 2.7 years. 17 patients died but none from SK. Conclusions The complexity of these patients suggests the need to create an integrated multidisciplinary team that can take dedicated care of the patient with KS. The dermatologist could assume the role of reference and mediator among the various specialists in order to provide better patient care.
Riassunto Introduzione Il Sarcoma di Kaposi è un tumore di origine vascolare che si manifesta clinicamente con lesioni cutanee singole o multiple e nelle forme più aggressive coinvolge anche le mucose, i linfonodi e gli organi viscerali. Negli anni sono state identificate quattro differenti forme clinico-epidemiologiche di SK: classica, endemica, epidemica e iatrogena. I dati del progetto RARECARENet riportano un tasso di incidenza in Europa pari a 0,28 su 100.000 persone /anno. L'incidenza aumenta con l'età: 0,18/100.000 da 0 a 44 anni; 0,25/100.000 da 45 a 64 anni; 0,69/100.000 da 65 anni e oltre. L'incidenza nei maschi è quattro volte maggiore rispetto alle femmine. I fattori di rischio principali sono: l’infezione da HHV-8 e lo stato di immunosoppressione. Tuttavia, ogni sottotipo clinico-epidemiologico presenta dei peculiari fattori di rischio. La diagnosi si basa sulla analisi istopatologica e immunoistochimica. Il trattamento è molto eterogeno e le strategie terapeutiche possono essere locali o sistemiche. Il follow-up dipende dal sottotipo clinico-epidemiologico, dall’estensione della malattia e dal trattamento scelto. Scopo dello studio Lo scopo di questo studio è stato quello di descrivere le caratteristiche dei pazienti affetti da Sarcoma di Kaposi giunti all’osservazione del nostro centro tra il 1993 e il 2022. L’obiettivo primario è stato la creazione di un database aggiornato al fine di raccogliere i dati dei pazienti in modo completo ed omogeneo. L’obiettivo secondario è stato quello di descrivere le caratteristiche demografiche e clinico-patologiche dei pazienti, le caratteristiche della malattia e il percorso diagnostico-terapeutico al fine di caratterizzare al meglio dal punto di vista clinico-scientifico questa rara neoplasia e fornire una migliore cura del paziente. Materiali e metodi Questo lavoro è uno studio retrospettivo di coorte che ha incluso 86 pazienti affetti da Sarcoma di Kaposi trattati presso l’Azienda Ospedaliera Universitaria di Padova (AOPD) e l’Istituto Oncologico Veneto (IOV) tra il 1993 e il 2022. L'analisi statistica è stata effettuata utilizzando R 4.1 (R Foundation for Statistical Computing,Vienna, Austria) Risultati Considerando i quattro tipi clinico-epidemiologici di Sarcoma di Kaposi sono stati registrati: 37 casi della forma classica (43,02%), 29 casi della forma epidemica (33,72%), 17 casi della forma iatrogena (19,77%) e 3 casi della forma endemica (3,49%). Il sito anatomico più coinvolto è rappresentato dagli arti inferiori (51,16%). 67 pazienti (77,91%) presentavano lesioni esclusivamente cutanee. Dal punto di vista delle comorbidità l’86,05% dei pazienti risultavano affetti da altre patologie, tra cui patologie autoimmuni (19,77%) e neoplastiche (25,58%). Il 61,63% dei pazienti ha effettuato un unico trattamento, mentre nel 38,37% dei casi è stato necessario ricorrere a più trattamenti combinati in vario modo tra loro. Gli approcci di cura sono stati di tipo locale e sistemico. Una risposta duratura (per almeno 2 anni) è stata ottenuta in circa 65% dei pazienti. Le ricadute si sono verificate in circa il 22% dei casi. La mediana del follow up è stata di 5,8 anni mentre la mediana dell’intervallo per la ricomparsa di malattia è 2,7 anni. 17 pazienti sono deceduti ma nessuno a causa del Sarcoma di Kaposi. Conclusioni Il dato più interessante che è emerso riguarda le caratteristiche di complessità di questi pazienti che suggeriscono la necessità di creare un gruppo multidisciplinare integrato che possa prendere in carico in maniera dedicata il paziente con SK. Il dermatologo potrebbe quindi assumere un ruolo di riferimento e mediatore tra i vari specialisti al fine di fornire una migliore cura al paziente.
SARCOMA DI KAPOSI: VALUTAZIONE DELLE CARATTERISTICHE CLINICO-PATOLOGICHE, DELLE STRATEGIE TERAPEUTICHE E DEI FATTORI PROGNOSTICI IN UNA CASISTICA RETROSPETTIVA MONOCENTRICA.
NERJAKU, FITNETE
2022/2023
Abstract
Abstract Introduction Kaposi's Sarcoma (SK) is a vascular tumor that manifests clinically with single or multiple skin lesions and in the most aggressive forms also involves the mucous membranes, lymphnodes, and visceral organs. Over the years, four different clinical-epidemiological forms have been identified; classical, endemic, epidemic, and iatrogenic. Data from the RARECARENet project report an incidence rate of KS in Europe of 0.28 per 100,000 persons/year. The incidence increases with age: 0.18/100,000 from 0 to 44 years; 0.25/100,000 from 45 to 64 years; 0.69/100,000 from 65 years and older. The incidence in males is four times higher than in females. The risk factors for the development of KS reflect the epidemiology of the disease, and the two main promoters of the neoplasm are: HHV-8 infection and immunosuppression status. However, each clinical-epidemiological subtype of KS has peculiar risk factors. KS is diagnosed by histopathological and immunohistochemical analysis of the lesion. Treatment of this neoplasm is very heterogeneous, and the therapeutic strategies can be local or systemic. Follow-up of KS depends on the clinical-epidemiologic subtype, the extent of the disease, and the treatment chosen. Aim of the study Our study aimed to describe the characteristics of patients with Sarcoma di Kaposi who came to the observation of the Azienda Ospedaliera Universitaria di Padova (AOPD) and the Istituto Oncologico Veneto (IOV) between 1993 and 2022. The goal was to describe the demographic and clinico-pathological characteristics of patients, disease features, and the diagnostic-therapeutic pathway, through the creation of a unique database with the aim of better characterizing the disease, expanding knowledge, and providing better patient care. Materials and Methods This work is a retrospective cohort study. Clinical characteristics of patients, neoplasm, immunohistochemical data and treatment were extracted from a local database. Statistical analysis was performed using R 4.1 (R Foundation for Statistical Computing,Vienna, Austria) Results Considering the four clinical-epidemiological types of SK, there were 37 cases of classical SK (43.02%), 29 cases of epidemic SK (33.72%), 17 cases of iatrogenic SK (19.77%) and 3 cases of endemic SK (3.49%). The most common anatomical site appeared to be the lower limbs (51.16%). 67 patients (77.91%) had exclusively cutaneous lesions. 86.05% of patients were found to have comorbidities, including autoimmune (19.77%) and neoplastic (25.58%) diseases. 61.63% of patients had a single treatment, while in 38.37% of cases it was necessary to use several treatments combined in various ways. Treatment approaches were local and systemic. A lasting response (for at least 2 years) was achieved in about 65% of patients. Relapses occurred in about 22% of cases. The median follow-up is 5.8 years while the median interval for disease recurrence is 2.7 years. 17 patients died but none from SK. Conclusions The complexity of these patients suggests the need to create an integrated multidisciplinary team that can take dedicated care of the patient with KS. The dermatologist could assume the role of reference and mediator among the various specialists in order to provide better patient care.File | Dimensione | Formato | |
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https://hdl.handle.net/20.500.12608/47425