Teratomy jsou nádory ze zárodečných buněk, které mohou obsahovat různě diferencované tkáně lidského organizmu. Výskyt teratomů v oblasti hlavy a krku je velmi raritní. Typicky se tyto nádory vyskytují ve vaječnících, varlatech či sakrokokcygeální oblasti. Následující kazuistika popisuje případ 22letého muže s narůstající rezistencí spodiny dutiny ústní, po jejíž exstirpaci byl histologicky prokázán teratom.

Teratomas are tumors derivated from germinal cells, which can contain various differentiated tissues of the human body. Appearance of teratomas in the head and neck area is very rare. Typically, these tumors are found in ovaries, testicles, and the sacrococcygeal area. Fol lowing a case report describing a 22 year old man with increasing resistance on the floor of the oral cavity, after whose excision a teratoma was histologically proven.

• MeSH
• Diagnosis, Differential MeSH
• Neoplasms, Germ Cell and Embryonal diagnostic imaging   diagnosis   classification   MeSH
• Humans MeSH
• Young Adult MeSH
• Head and Neck Neoplasms diagnostic imaging   diagnosis   classification   MeSH
• Teratoma * diagnostic imaging   diagnosis   classification   MeSH
• Mouth * pathology   MeSH
• Check Tag
• Humans MeSH
• Young Adult MeSH
• Male MeSH
• Publication type
• Case Reports MeSH

INTRODUCTION: Sacrococcygeal teratoma (SCT) is the most common congenital tumor. The incidence of malignant types is rare but increases with late detection or in case of relapse. Prenatal diagnosis is based on ultrasound examination and magnetic resonance imaging (MRI). Since this is a rare congenital anomaly, we should report all cases to improve prenatal diagnosis and postnatal management. MATERIAL AND METHODS: Retrospective analysis of sixteen cases of sacrococcygeal teratoma delivered and treated at the University Hospital Brno between 2005 and 2020. The following criteria were evaluated: gestational week of the primary diagnosis, exact description of ultrasound findings, pregnancy management, delivery mode, correlation of prenatal ultrasound with postnatal findings in the newborn, as well as the occurrence of early and late complications in newborns and children. RESULTS: Out of sixteen cases, seven cases (43.8%) were indicated for pregnancy termination based on ultrasound findings, the parent's decision, and an estimation of an adverse pregnancy outcome. In nine cases (56.2%), the pregnancy continued and was ended by delivery. In one case, there was an early postnatal death of a newborn after birth in the 25th week of gestation. In eight cases, live fetuses were born in which the tumor was surgically removed between day 1 and 14 months after birth. There was a strong correlation between the tumor description made by prenatal ultrasound diagnosis and related severe complications in newborns. The incidence of severe early and late complications in ongoing pregnancies was very low-only one case of infection in the surgical wound requiring reoperation (12.5%) was described. In two patients (25%), a transient stoma establishment was necessary for secondary ileus. One case of recurrence of the disease at two years of age occurred, requiring the administration of chemotherapy (12.5%), and one patient has mild persistent urinary incontinence. CONCLUSION: Sacrococcygeal teratoma is one of the rarest congenital malformations. A detailed prenatal ultrasound examination is essential to estimate the pregnancy prognosis. The most predictive ultrasound predictor of favorable early and late postnatal outcomes and long-term child development is the presence of cystic sacrococcygeal formation, the most common tumor type, and the absence of signs of cardiac failure due to fetal anemia. In these cases, with early surgical treatment provided, the incidence of severe complications and long-term consequences in children is very low, and parents should be informed during prenatal counseling. It is necessary to register all the SCT cases due to the rarity of this congenital anomaly for further statistical analysis of the importance of ultrasound markers.

• MeSH
• Adult MeSH
• Gestational Age MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Infant, Newborn MeSH
• Prognosis MeSH
• Retrospective Studies MeSH
• Sacrococcygeal Region * diagnostic imaging   MeSH
• Pregnancy MeSH
• Teratoma * diagnostic imaging   surgery   congenital   MeSH
• Ultrasonography, Prenatal * MeSH
• Pregnancy Outcome MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Infant, Newborn MeSH
• Pregnancy MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Review MeSH

Souhrn: Čisté testikulární teratomy jsou velice zřídkavými neseminomovými nádory ze zárodečných buněk (NSGCT – non-seminomatous germ cell tumor). Přestože mají omezenou chemosenzitivitu, je u pacientů s metastázami primárně doporučová na chemoterapie z důvodu možné přítomnosti jiné ho subtypu NSGCT v metastá zá ch, než je teratom. Cílem naší kazuistiky je odprezentovat pacienta s primárním postpubertálním teratomem a retroperitoneálními cystickými metastázami spolu s negativními sérovými nádorovými markery, což představuje mimořá dně vzácný klinický scénář. V takových případech je vysoce pravděpodobné , že cystické retroperitoneální metastázy budou tvořeny teratomem, proto je preferovanou lé čebnou strategií primá retroperitoneá lní lymfadenektomie před chemoterapií, která je u těchto nemocných neúčinná.

Pure testicular teratomas are exceptionally rare among nonseminomatous germ cell tumors (NSGCT). Despite their limited chemosensitivity, upfront chemotherapy is recommended for metastatic patients due to the potential presence of another NSGCT subtype alongside teratoma in metastases. We present a case report of a patient with primary post-pubertal teratoma and retroperitoneal cystic metastases, along with negative serum tumor markers - an exceedingly rare clinical scenario. In such cases, cystic retroperitoneal metastases are highly likely to be formed by teratoma, making primary retroperitoneal lymph node dissection a preferred option over chemotherapy, which would be ineffective in such a patient.

• MeSH
• Adult MeSH
• Neoplasms, Germ Cell and Embryonal MeSH
• Humans MeSH
• Lymph Node Excision methods   MeSH
• Neoplasm Metastasis therapy   MeSH
• Teratoma * surgery   diagnosis   pathology   MeSH
• Testicular Neoplasms surgery   diagnosis   pathology   MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH

• MeSH
• Eye Abnormalities etiology   pathology   MeSH
• Dermoid Cyst * etiology   pathology   congenital   MeSH
• Humans MeSH
• Infant, Newborn MeSH
• Check Tag
• Humans MeSH
• Infant, Newborn MeSH
• Publication type
• Review MeSH

• MeSH
• Diagnosis, Differential MeSH
• Humans MeSH
• Oropharyngeal Neoplasms * surgery   diagnostic imaging   pathology   congenital   MeSH
• Infant, Newborn MeSH
• Oropharynx abnormalities   surgery   MeSH
• Prenatal Diagnosis MeSH
• Prognosis MeSH
• Teratoma * surgery   diagnostic imaging   pathology   congenital   MeSH
• Check Tag
• Humans MeSH
• Infant, Newborn MeSH
• Publication type
• Review MeSH

Sakrokokcygeální teratom patří mezi velmi vzácné vrozené vývojové vady. Prognóza je závislá na faktorech ovlivňujících již fetální vývoj. Diagnostika se opírá o ultrazvukové vyšetření v I. a II. trimestru těhotenství. Pokračuje snaha o identifikaci spolehlivých ultrazvukových markerů, které by prenatálně umožnily stanovení co nejpřesnější prognózy pro plod a usnadnily rodičům rozhodování o dalším osudu gravidity. K dispozici je pouze omezený počet studií s převahou kazuistických případů. Uvádíme literární přehled aktuálních informací týkajících se zejména diagnostiky sakrokokcygeálního teratomu, možností terapie a výskytu prenatálních i postnatálních komplikací teratomů v souvislosti s prenatálními nálezy. Ukazuje se, že v prognosticky příznivých případech dle prenatálního ultrazvukového vyšetření a při adekvátní chirurgické léčbě po porodu je postnatální prognóza této vrozené vývojové vady velmi dobrá.

: Sacrococcygeal teratoma is a rare congenital malformation, the prognosis depends on factors affecting foetal development. The diagnosis is based on ultrasound examination, especially the evaluation of the detailed morphology of the foetus in the 20th week of pregnancy. Therefore, it is crucial to keep looking for ultrasound markers that would prenatally determine the most accurate prognosis for the foetus. Now, we rely on a small number of studies with a predominance of case reports. We offer a literature review of the essential information concerning sacrococcygeal teratoma diagnostics, therapy, and complications of sacrococcygeal teratomas in connection with prenatal diagnosis. It turns out that in cases with a favourable prognosis according to prenatal ultrasound examination and adequate surgical treatment after childbirth, the prognosis of this congenital malformation is excellent.

• MeSH
• Humans MeSH
• Fetal Diseases surgery   diagnostic imaging   pathology   MeSH
• Fetus surgery   pathology   MeSH
• Prenatal Diagnosis * methods   MeSH
• Prognosis MeSH
• Sacrococcygeal Region pathology   MeSH
• Pregnancy MeSH
• Teratoma * surgery   diagnostic imaging   classification   pathology   MeSH
• Ultrasonography MeSH
• Check Tag
• Humans MeSH
• Pregnancy MeSH
• Publication type
• Review MeSH

Rhabdoid tumors are aggressive tumors that may arise in the kidney, soft tissue, central nervous system, or other organs. They are defined by SMARCB1 (INI1) or SMARCA4 alterations. Often, very young children are affected, and the prognosis is dismal. Four patients with primary atypical teratoid rhabdoid tumor (AT/RT, a rhabdoid tumor in the central nervous system) were treated by resection and high dose chemotherapy. Tazemetostat was introduced after completion of chemotherapy. Three patients have achieved an event free survival of 32, 34, and 30 months respectively. One progressed and died. His overall survival was 20 months. One patient was treated for a relapsed atypical teratoid rhabdoid tumor. The treatment combined metronomic therapy, radiotherapy, tazemetostat and immunotherapy. This patient died of disease progression, with an overall survival of 37 months. One patient was treated for a rhabdoid tumor of the ovary. Tazemetostat was given as maintenance after resection, chemotherapy, and radiotherapy, concomitantly with immunotherapy. Her event free survival is 44 months. Only approximately 40% of patients with rhabdoid tumors achieve long-term survival. Nearly all relapses occur within two years from diagnosis. The event free survival of four of the six patients in our cohort has exceeded this timepoint. Tazemetostat has been mostly tested as a single agent in the relapsed setting. We present promising results when applied as maintenance or add on in the first line treatment.

• MeSH
• Child MeSH
• DNA Helicases MeSH
• SMARCB1 Protein MeSH
• Nuclear Proteins MeSH
• Infant MeSH
• Humans MeSH
• Neoplasm Recurrence, Local drug therapy   MeSH
• Central Nervous System Neoplasms * pathology   MeSH
• Child, Preschool MeSH
• Rhabdoid Tumor * drug therapy   pathology   MeSH
• Teratoma * pathology   MeSH
• Transcription Factors MeSH
• Check Tag
• Child MeSH
• Infant MeSH
• Humans MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

• MeSH
• Asthma etiology   MeSH
• Depression diagnosis   psychology   therapy   MeSH
• Humans MeSH
• Adolescent MeSH
• Orchiectomy MeSH
• Sleep Paralysis * diagnosis   therapy   MeSH
• Teratoma surgery   diagnosis   MeSH
• Testicular Neoplasms * surgery   diagnosis   complications   MeSH
• Treatment Outcome MeSH
• Expert Testimony * MeSH
• Check Tag
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Publication type
• Case Reports MeSH

Spinal anaesthesia (SA) is one of the most prevalent types of anaesthetic procedures. There are very few reports of cord herniation through the site of spinal canal stenosis due to tumour. A 33-year-old female presented with acute paraparesis after spinal anaesthesia for caesarean section. Magnetic resonance imaging (MRI) revealed an intradural mass from posterior of T6 to T8-T9 interface. We operated the patient and after laminectomy of T6 to T9, dermoid tumour containing hairs was totally resected and cord was completely decompressed. After 6 months, the patient is without any neurological deficit. Puncturing the dura with cerebrospinal fluid (CSF) in the presence of an extramedullary mass could cause cord herniation through the blockade. In these cases, awareness about related signs even in absence of symptoms or complaints could help us to prevent post-SA neurological deficit.

• MeSH
• Cesarean Section MeSH
• Dermoid Cyst * diagnosis   surgery   pathology   MeSH
• Adult MeSH
• Hernia diagnosis   etiology   pathology   MeSH
• Thoracic Vertebrae pathology   surgery   MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Thoracic Neoplasms * pathology   MeSH
• Anesthesia, Spinal * MeSH
• Spinal Puncture MeSH
• Pregnancy MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Pregnancy MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH

• MeSH
• Diagnosis, Differential MeSH
• Infant MeSH
• Humans MeSH
• Magnetic Resonance Imaging methods   MeSH
• Orbital Neoplasms * surgery   diagnostic imaging   classification   MeSH
• Postoperative Period MeSH
• Teratoma * surgery   diagnostic imaging   diagnosis   pathology   MeSH
• Adipose Tissue pathology   MeSH
• Check Tag
• Infant MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Case Reports MeSH