Rabdomiosarkomu un citu mīksto audu sarkomu klīniskās īpatnības, terapijas raksturojums un ārstēšanas iznākuma analīze bērniem laika periodā no 2004.-2018. gadam Latvijā - Goršantova, Viktorija (Latvijas Universitāte, 2020)
Ievads. Mīksto audu sarkomas ir heterogēna ļaundabīgu audzēju grupa, kas cēlusies no mezenhimāliem audiem un to iedala divas apakšgrupās: rabdomiosarkomas un nerabdomiosarkomas mīksto audu sarkomas. Kopumā mīksto audu sarkomas bērnu onkoloģijā ir sastopamas 4-8% gadījumu. Neskatoties uz to, ka mūsdienās tiek izstrādātas arvien efektīvākas ļaundabīgo audzēju diagnostikas un skrīninga metodes, mīksto audu audzēji tomēr visai bieži tiek atklāti vēlīnās stadijas un jau ar metastāzēm, kas nozīmē samazinātu pacienta dzīvildzi. Pētījuma mērķi. Apkopot un analizēt rabdomiosarkomu (RMS) un citu mīksto audu sarkomu slimības gaitas īpatnības, pielietotās ārstēšanas metodes un rezultātus bērniem Latvijā. Materiāli un metodes. Retrospektīvs pētījums, kurā tika iekļauti 47 pacienti, kuriem no 2004.gada līdz 2018.gadam tika diagnosticēts kāds no mīksto audu sarkomas veidiem. Katram pacientam tika noteikts: vecums, dzimums, audzēja histoloģiskais tips, metastāzes (diagnosticēšanas brīdī), RMS klīniskā stadija, primārā audzēja lokalizācija, simptomi, ārstēšanas īpatnības un ārstēšanas iznākums. Rezultāti. Pētījumā piedalījās 47 pacienti, no kuriem 25 (53,2%) bija zēni, bet 22(46,8%) – meitenes. Jaunākais pacients bija vienu mēnesi vecs, bet vecākais – 17 gadus vecs. RMS tika diagnosticētas 32 (68,1%) pacientiem, bet NRSTS (non-rhabdomyosarcoma soft tissue sarcomas) – 15 (31,9%). Salīdzinot divus RMS tipus, alveolāra rabdomiosarkoma (A-RMS) tika diagnosticēta septiņiem bērniem un to bieži atklāja vēlīni – III vai IV stadijā, turpretī embrionāla rabdomiosarkoma (E-RMS) tika diagnosticēta 21 bērnam (p<0,001) un to parasti atklāja agrīni – I vai II stadijā (p<0,024). Vidējais pacientu vecums A-RMS diagnosticēšanas brīdī ir četri gadi, bet E-RMS – septiņi gadi. Statistiski nozīmīga asociācija (p=0,003) bija starp A-RMS un E-RMS ārstēšanas iznākumiem. Starp A-RMS pacientiem 14 bērniem (67%) bija remisija un 6 bērniem (29%) bija exitus letalis. Kopumā mīksto audu diagnosticēšanas brīdī 21(45%) bērnam bija metastāzes, bet 26 (55%) bērniem – nebija. No 47 pacientiem ārstēšanā viens saņēma tikai ķīmijterapiju, 10 saņēma ķīmijterapiju un ķirurģisku ārstēšanu, 11 saņēma ķīmijterapiju un staru terapiju, bet 25 saņēma ķīmijterapiju, staru terapiju un ķirurģisku ārstēšanu. Piecu gadu dzīvildze bija 20 (43%) bērniem, dzīvildze mazāka par 5 gadiem arī bija 20 (42%) bērniem, bet par septiņiem (15%) bērniem nav informācijas.

Introduction. Soft tissue sarcomas are a group of heterogeneous malignancies that originate from mesenchymal tissue and are divided into two major subgroups: rhabdomyosarcomas and non-rhabdomyosarcomas soft tissue sarcomas. Soft tissue sarcomas are found in 4-8% of cases in paediatric oncology. Despite the fact that metods of tumor diagnosis and screening has improves in recent year, soft tissue tumors are still mostly detected at a late stage and with metastases already, which means reduced patient survival. Purpose. The aim of the study was to summarize and analyze factors such as the course of a disease, treatment methods, and treatment outcomes among children in Latvia, diagnosed with rhabdomyosarcoma (RMS) or other soft tissue sarcomas. Material and methods. A retrospective study of 47 patients included, which were diagnosed with any type of soft tissue sarcomas between 2004 and 2018. The following parameters were determined for each patient: age, gender, histological type of tumor, metastases (found at the moment of diagnosis), RMS clinical stage, location of the primary tumot, symptoms, treatment characteristics, and treatment outcome. Results. The study included 47 patients, of whom 25 (53,2%) were boys and 22 (46,8%) were girls. The youngest of patients was only 30 days old and the oldest one was 17 years old. RMS was diagnosed in 31 (68,1%) patients and NRSTS (non-rhabdomyosarcoma soft tissue sarcomas) in 15 (31,9%) patients. Comparing the two types of RMS, the alveolar rhabdomyosarcoma (A-RMS) type was diagnosed in seven children and was often detected in late-stage – III or IV, whereas embryonal rhabdomyosarcoma (E-RMS) type was diagnosed in 21 children (p<0,001) and was detected early in most cases – stage I or II (p<0,024). At the moment of diagnosis, the mean age was four years in patients with A-RMS and seven years in patients with E-RMS. There was statistically significant association (p=0,003) between the treatment outcomes of A-RMS and E-RMS. There was fatall outcome in children (86%) and no further information about one of the children (14%) among those with A-RMS, while 14 children (67%) experienced remission, yet there was a fatal outcome in children (29%) among those diagnosed with E-RMS. At the moment of diagnosis, metastases were found in 21(45%) children, but at the same – 26 (55%) children had no metastases. The treatment tactics of all 47 patients varied: one of them received chemotherapy alone, 10 received chemotherapy and surgery, 11 received chemotherapy and radiation therapy, and 25 received chemotherapy, radiation therapy, and surgery altogether. The survival rate was: five years in 20 (43%) children, less than 5 years also in 20(42%) children. There is no information available on the survival of the rest of children.
Clinical features, characteristics of therapy and treatment outcome analysis of rhabdomyosarcomas and ofter soft tissue sarcomas in children in period of 2004-2018 in Latvia
Keywords: Medicīna; mīksto audu sarkomas; RMS; E-RMS; A-RMS