Browsing by Subject "http://www.yso.fi/onto/mesh/D016019"

Johdanto: Idiopaattinen keuhkofibroosi (IPF) on krooninen ja etenevä keuhkokudosta arpeuttava sairaus. Sairauden etiologia on vielä tuntematon, mutta myös geneettisillä muutoksilla arvioidaan olevan merkitystä. Uusien antifibroottisten lääkkeiden on osoitettu hidastavan IPF:n etenemistä, mutta ainoa ennustetta parantava hoitomuoto on yhä keuhkonsiirto. Tavoitteet: Tämän tutkielman tavoitteena oli saada lisää tietoa suomalaisista keuhkonsiirtoon päätyneistä IPF-potilaista sekä selvittää tekijöitä, jotka vaikuttavat heidän keuhkonsiirron jälkeiseen elossaoloonsa (survival). Toisena tavoitteena oli tutkia aiemmin sairastetun sytomegalovirusinfektion (CMV-status) vaikutusta keuhkonsiirron jälkeisten viremioiden määrään. Kolmantena tavoitteena oli selvittää potilaiden verestä ja keuhkokudoksesta eristettyjen nukleiinihapponäytteiden laatu ja käytettävyys myöhempiä geenitutkimuksia varten. Aineisto ja menetelmät: Tutkimusaineisto koostui 93:sta Suomessa vuosina 1991-2019 keuhkonsiirron saaneesta IPF-potilaasta ja heidän nukleiinihapponäytteistään (kudos-DNA, veri-DNA, kudos-RNA). Potilaiden tietoja vertailtiin eri vuosikymmenten välillä, ja elossaoloa analysoitiin Kaplan-Meier-menetelmän avulla. CMV-statuksen merkitystä viremioihin selvitettiin tilastollisin menetelmin, ja nukleiinihapponäytteiden laatua analysoitiin geelielektroforeesimenetelmien avulla. Tulokset: Potilaiden lukumäärä ja keski-ikä ovat kasvaneet seuranta-aikana. Valtaosa potilaista oli miehiä. Siirronjälkeiseen elossaoloon vaikutti selvimmin siirtovuosikymmen (Log Rank p=0,002). Iällä, sukupuolella ja verenkuvaparametreillä ei ollut merkitsevää yhteyttä elossaoloon, eikä CMV-statuksella ollut tilastollisesti merkitsevää yhteyttä viremioiden lukumäärän. Parafiiniin säilötyt RNA-näytteet olivat täysin hajonneita, mutta molemmat DNA-näytteet olivat sen verran eheitä, että näytteitä voidaan käyttää muun muassa mutaatioanalyyseissä. Verestä eristetyt DNA-näytteet olivat lähes eheitä. Johtopäätökset: IPF-potilaiden keuhkonsiirron jälkeinen elossaolo on pidentynyt vuosikymmenten saatossa. Nykymenetelmien avulla voidaan myös vanhoista arvokkaista kudosnäytteistä tehdä geneettisiä analyysejä, ja näin ollen niitä voidaan käyttää vielä hyväksi uusissa tutkimuksissa.

Abstract Background: Insufficient preoperative work-up and consequent intralesional or marginal resection of soft tissue sarcomas of the head and neck (STSHNs) is common. Methods: This retrospective cohort study comprised 63 patients with STSHN treated at the Helsinki University Hospital between 2005 and 2017. We assessed the effect of pretreatment tumor sampling on surgical margin status and need for supplemental surgery, as well as prognostic factors and survival. Results: The lack of representative pretreatment biopsy specimen was associated with unfavorable margin status. Primary surgery at a non-academic center was associated with need for supplemental surgery. The 3-year overall survival (OS) was 68%, disease-specific survival (DSS) 71%, and recurrence-free survival (RFS) 61%. Higher tumor grade and primary tumor size over 5 cm were associated with reduced DSS. Conclusions: Diagnosis and management of STSHNs should be centralized to experienced academic centers. Decision-making between needle biopsy, open biopsy, or upfront radical surgery depends on tumor location and size. Keywords: head and neck sarcoma, prognostic factors, pretreatment work-up, survival, single-center experience

Soft tissue sarcoma (STS) is a rare neoplasm consisting of approximately 50 different histologic subtypes treated similarly. The median patient age is 60 years, and there’s a slight male predominance. There are no known predisposing factors apart from ionizing radiation. STS is diagnosed with core needle biopsy of a suspicious lump, most commonly a palpable, painless mass. Surgery with adequate margins is the only curative treatment of STS. Adjuvant radiation therapy (RT) improves local control rates (LC) and is offered for patients with positive surgical margins. However, there is no consensus on which surgical margin is wide enough. Adjuvant chemotherapy yields rather poor survival benefit with significant risk of toxicity and is therefore offered for minority of the patients. Prognostic factors for local recurrence include patient age, tumor grade and surgical margin. Due to disease rarity, complex diagnostics, need for surgical treatment, adjuvant radiation therapy and critical evaluation of patient selection to receive chemotherapy, centralization of treatment is effective. Specialist centers adhere to treatment guidelines more strictly than non-tertiary centers. Adequate preoperative imaging methods are more often used, adequate surgical margins are more often achieved, patients undergo fewer operations, adequate adjuvant RT is more often offered and local control rates are better in specialist centers compared to non-tertiary centers. However, there is still a lack of adherence to referral policies with only 63 percent of STSs referred before surgical intervention. The aim of this study was to quality control treatment received in Soft Tissue Sarcoma Group at Helsinki University Hospital (HUH) during its first 25 years, with special interest in 1) adherence to treatment protocol, 2) metastases-free survival (MFS) and overall survival (OS) of STS patients and 3) treatment and survival of radiation-associated STS. We used a 1327-patient series from 1987-2012 consisting of patients referred to STS Group at HUH. Patients were treated according to treatment protocol based on 1987 Scandinavian Sarcoma Group recommendations. 1182 (79 %) patients had only primary tumor at presentation. Only 411 (35 %) patients referred for a primary tumor were referred untouched. However, the proportion increased by time from 13 to 47 percent. Of the 1115 patients treated with a curative intent, 680 (61 %) patients underwent only one procedure, proportion of which increased by time. Only 64 and 62 percent of patients with intralesional (tumor tissue in resection margin) and marginal margins (smallest margin under 2.5 cm) received adjuvant RT. Overall survival rates for patients treated with curative intent were 68 percent and 55 percent in 5-year and 10-year follow-up, respectively. No improvement in OS was recorded. 59 patients were referred for a radiation-associated sarcoma, and 52 of them were treated with a curative intent. Tumor appeared in the radiation field of an invasive breast cancer in 77 percent of the patients. Local control rates for patients treated with curative intent were 73 percent and 58 percent in 5-year and 10-year follow-up, respectively. Adherence to treatment protocol in STS group at HUH has improved over time in 1987-2012. To increase the percentage of patients referred for primary tumor without preceding biopsy, increasing soft tissue sarcoma knowledge in district hospitals is vital. All exceptions from treatment protocol should be discussed and reported in patient files.