HIV-infektio ja syöpä

English summaryPeer reviewe

Challenging Management of Plexiform Schwannoma and Plexiform Neurofibroma

Plexiform variants of neurofibromas and schwannomas are rare and typically arise in superficial soft tissues in the head and neck region. The treatment of these tumors is challenging and no generally accepted guidelines exist for their optimal management. The purpose of this study was to review the management and longterm prognosis of head and neck plexiform neurofibromas and schwannomas at 2 tertiary care academic hospitals in Finland over a 31-year period. The pathology files were searched for plexiform neurofibromas and schwannomas between the years 1990 and 2020. The case notes were reviewed for full management details. Two plexiform schwannomas and 6 plexiform neurofibromas were identified. Five of the 6 plexiform neurofibromas were managed operatively. All patients with a surgically managed plexiform neurofibroma underwent multiple operations. Sclerotherapy abolished 1 patient's cutaneous plexiform neurofibromas. The management of plexiform neurofibromas and plexiform schwannomas remains challenging. Sclerotherapy may offer a promising management option for cutaneous plexiform neurofibromas.Peer reviewe

IgG4-positive plasma cells in nonspecific sialadenitis and sialolithiasis

Chronic sclerosing sialadenitis is commonly regarded as a manifestation of IgG4-related disease. We previously found that a high IgG4 expression or IgG4-related disease could accompany nonspecific sialadenitis, whereas chronic sclerosing sialadenitis was not directly associated with IgG4-related disease. Our previous findings lead us to hypothesize that these inflammatory conditions of the submandibular gland signify a continuous progression of disease rather than different disease entities. We, therefore, aimed to determine the presence of IgG4-positivity and genuine IgG4-related disease in a cohort of 165 submandibular gland specimens from patients who underwent surgery due to chronic nonspecific sialadenitis or sialolithiasis. To do so, we re-evaluated histopathological features and divided samples into three groups: (A) nonspecific sialadenitis without known sialolithiasis, (B) sialadenitis with sialolithiasis, and (C) sialolithiasis without sialadenitis. We performed immunohistochemical staining for IgG4, IgG, and CD31, and assessed the Boston consensus statement criteria for IgG4-related disease in IgG4-positive samples. We also reviewed patient records and supplemented follow-up data with a questionnaire among patients with IgG4-positive samples. IgG4-positive plasma cells (range 1-344) were found in 131 samples. Among these, 19 samples were classified as IgG4-positive (>= 70 IgG4-positive plasma cells/high-power field). Two IgG4-positive samples were histologically highly suggestive of IgG4-related disease, but only one had a clinically confirmed diagnosis of IgG4-related disease. Our results indicate that patients with sialadenitis and sialolithiasis often present with IgG4-positive lymphoplasmacytic infiltrates, but exceedingly rarely present with genuine IgG4-related disease. In sialolithiasis without sialadenitis, IgG4-positive plasma cells are often absent or appear in small numbers. These results support our hypothesis of a continuum of disease, and indicate that progressive inflammation of the submandibular gland leads to the development of more specific pathological features over time.Peer reviewe

Preoperative evaluation and treatment consideration of parotid gland tumors

Background: The nature of parotid tumors often remains unknown preoperatively and final histopathology may reveal unexpected malignancy. Still, the use of fine-needle aspiration cytology (FNAC) and imaging varies in the management of these tumors. Methods: We evaluated the preoperative examinations and management of all 195 parotid gland tumors diagnosed within our catchment area of 1.6 million people during 2015. Results: Altogether 171 (88%) tumors were classified as true salivary gland neoplasms. FNAC showed no false malignant findings, but it was false benign in 5 (2.6%) cases. Preoperative MRI was utilized in 48 patients (25%). Twenty (10%) malignancies included 16 salivary gland carcinomas. Pleomorphic adenomas accounted for 52% of all adenomas. For 24 (40%) Warthin tumors, surgery was omitted. Conclusion: The proportion of malignancies was lower than generally presented. Our proposed guidelines include ultrasound-guided FNAC with certain limitations. MRI is warranted in selected cases, but seems unnecessary routinely.Peer reviewe

Pretreatment tumor sampling and prognostic factors in patients with soft-tissue sarcoma of the head and neck

Correction: DOI10.1007/s00405-021-07205-6 Early AccessDEC 2021Background 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.Peer reviewe

Acquired Jugular Vein Aneurysm

Venous malformations of the jugular veins are rare findings. Aneurysms and phlebectasias are the lesions most often reported. We report on an adult patient with an abruptly appearing large tumorous mass on the left side of the neck identified as a jugular vein aneurysm. Upon clinical examination with ultrasound, a lateral neck cyst was primarily suspected. Surgery revealed a saccular aneurysm in intimate connection with the internal jugular vein. Histology showed an organized hematoma inside the aneurysmal sac, which had a focally thinned muscular layer. The terminology and the treatment guidelines of venous dilatation lesions are discussed. For phlebectasias, conservative treatment is usually recommended, whereas for saccular aneurysms, surgical resection is the treatment of choice. While an exact classification based on etiology and pathophysiology is not possible, a more uniform taxonomy would clarify the guidelines for different therapeutic modalities for venous dilatation lesions

Human papillomavirus self-sampling with mRNA testing benefits routine screening

High risk human papillomavirus (hrHPV) based screening provides the possibility of vaginal self-sampling as a tool to increase screening attendance. In order to evaluate the impact and feasibility of opt-in self-sampling in the Finnish setting, we invited a randomized population of 5350 women not attending screening after age group invitation or after reminder, to attend HPV self-sampling-based screening in the autumn of 2018 in Helsinki. Out of those, 1282 (24.0%) expressed their interest and ordered the sampling package. Eventually 787 women (14.7% of the total invited population) took part in screening, 770 women by providing a vaginal sample within 2 months from invitation and 17 by providing a pap smear in the laboratory. Self-taken samples were collected in Aptima Multitest vials and tested using the Aptima HPV mRNA assay. A high proportion, 158/770 (20.5%) of the samples were positive in the Aptima HPV assay. One hundred and forty-one samples were further submitted to Aptima HPV Genotyping and extended genotyping by a Luminex based assay. Of those, 23 samples (16.3%) were HPV 16 positive and 7 (5.0%) were positive for HPV 18/45; extended genotyping revealed multiple high-risk and low-risk HPV genotypes. At follow-up seven cases of high-grade squamous intraepithelial lesion (HSIL) were diagnosed, which represents 4.4% of HPV positive women and 0.9% of screened women, whereas the rate was 0.5% in routine screening. Our findings suggest that self-sampling with HPV mRNA testing is a feasible approach to improve screening efficacy in a high-risk population among original nonattendees.Peer reviewe

Submandibular gland cancer : Specific features and treatment considerations

BackgroundIn the absence of unified treatment protocol, we evaluated the management and outcomes of submandibular gland cancers in an unselected patient series. MethodsWe included all patients with resected submandibular gland cancer treated at the Helsinki University Hospital from 2000 to 2010 with a 5-year minimum follow-up. ResultsTwenty-five patients with cancer represented 30% of submandibular gland neoplasms, and most were adenoid cystic carcinomas (ACCs; 56%). At presentation, 3 patients showed clinical signs of probable malignancy. Of 22 neck dissection specimens, 5 patients (20%) had metastases with an occult metastasis rate of 4%. Cancer recurred in 11 patients (44%), of which 7 (28%) were only at a distant site. The 5-year disease-specific survival (DSS) and overall survival (OS) rates were 76%, and disease-free survival (DFS) was 68%. ConclusionMost tumors were ACCs differing from the histological pattern of parotid gland cancers. Occult metastases were rare. The rarity of submandibular gland cancer, its variable histological pattern, and varying biological behavior warrant centralized management.Peer reviewe

Ear canal and middle-ear tumors : a single-institution series of 87 patients

Background Ear canal and middle ear tumors are rare and exhibit variability in histology and clinical manifestation. Surgical resection remains the treatment of choice, but individualized approach is needed to preserve function when possible. Aims/objectives To review the management and outcome of ear canal and middle ear tumors at an academic referral center. Materials and methods Helsinki University Hospital (HUS) patient files were searched for clinically and histologically confirmed ear canal and middle ear tumors over a 14-year period. The minimum follow-up time was 2 years. Results Eighty-seven patients with 88 tumors were identified. There were 20 (23%) benign external auditory canal (EAC), 36 (41%) benign middle ear space (MES), 29 (33%) malignant EAC, and 3 (3%) malignant MES tumors. Most (92%) tumors were managed with primary resection. Thirty-five percent of the operatively managed patients had a residual or a recurrent tumor. Conclusions and significance EAC and MES tumors show great diagnostic and histologic heterogeneity with need for individualized investigative and treatment approaches. In benign tumors, we advocate aggressive local surgical control without sacrificing vital structures. In malignant tumors, we recommend local surgical control with or without adjunct RT.Peer reviewe

Suunielun syövät ja niiden riskitekijät

Tutkimuksen tarkoitus: Tarkoituksena oli tehdä kirjallisuuskatsaus suunielun syövistä, jossa tauti kuvaillaan pääpiirteittäin. Lisäksi viime vuosina Helsingissä hoidetuista suunielun syöpäpotilaista tehtiin analyysi, jonka tavoitteena oli analyysin perusteella kuvata HPV-positiivisen ja toisaalta HPVnegatiivisen suunielun syöpää sairastavan potilaan taudinkuva, riskitekijät, sekä levinneisyyteen vaikuttavat seikat. Tässä tutkimuksessa HPV-infektion yhteyttä suunielun syöpään on arviotu p16ilmentymisen perusteella, sillä HPV-infektioon liittyy usein p16-yliekspressio. Materiaalit ja menetelmät: Kirjallisuuskatsausta varten etsittiin PubMedistä artikkeleita aiheeseen liittyen, käyttäen esimerkiksi hakusanoja "HPV" ja "Oropharyngeal cancer". Analyysia varten kerätty potilasaineisto koostuu HUS Korva-, nenä- ja kurkkutautien klinikan tuumorimeetingeissä 1.3.2012 – 28.2.2014 diagnostisoiduista uusista suunielusyöpäpotilaista. Potilastietoja kerätään sähköisistä hoitokertomustiedoista, sekä HUSLAB Patologian laitoksen rekistereistä. Aineiston perusteella tehtiin kolme taulukkoa: potilaan kliiniset piirteet (esimerkiksi tupakointi, oireet) tuumorin p16 ilmentymisen mukaan, tuumorin ominaisuudet (esimerkiksi TNMluokitus) p16 ilmentymisen mukaan ja potilaan ensimmäinen suunniteltu hoito tuumorin p16 ilmentymisen mukaan. Tulokset: Potilaista (N=126) suurin osa oli miehiä ja kaikkien potilaiden keski-ikä oli 62.4 vuotta. P16-värjäys tehtiin 110:lle ja niistä positiivisia oli 80. Tupakoimattomilla potilailla todettiin ilmentyvän enemmän p16-postiivisia tuumoreita. Niillä potilailla, joilla alkoholin suurkulutusrajat täyttyivät, todettiin olevan vähemmän p16-positiivisia tuumoreita. P16-positiivisen tuloksen omaavilla potilailla enemmistöllä oireiden laatu oli patti kaulalla ja p16-negatiivisilla potilailla enemmistöllä oireena oli kipu. P16-negatiivisen tuumorin omaavilla potilailla etäpesäkkeiden esiintymistä alueellisissa imusolmukkeissa (N-luokitus) vaikutti olevan vähemmän kuin p16-positiivisen tuumorin omaavilla potilailla. Sekä p16-positiivisilla, että p16-negatiivisilla potilailla yleisin syövän gradus oli 3. Tuumorin yleisin lokaatio oli nielurisa sekä p16-positiivisilla, että p16negatiivisilla. P16-negatiivisia tuumoreita esiintyi toiseksi eniten pehmeässä suulaessa ja kielen tyvessä, sekä jonkin verran nielun takaseinämässä, kun p16-positiivisia tuumoreita esiintyi vain vähän pehmeässä suulaessa ja jonkin verran kielen tyvessä. P16-positiivisen tuumorin omaavat potilaat saivat enemmän kemosädehoitoa ja vähemmän leikkaushoitoa p16-negatiivisen tuumorin omaaviin potilaisiin verrattuna. Palliatiivista hoitoa sai 14% kaikista potilaista, p16-ilmentyminen ei näyttänyt olevan sidoksissa edelliseen. Johtopäätökset: Tutkimuksen perusteella voidaan sanoa, että HPV-lähtöinen syöpä käyttäytyy eri tavalla, kuin HPV-negatiivinen syöpä, ja sitä olisi mahdollisesti hyvä käsitellä ja hoitaa eri sairautena