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Journal of Zhejiang University....To determine the clinical, imaging, and histological features, and surgical resection modalities and outcomes of adult sacrococcygeal teratoma (SCT).
OBJECTIVE
To determine the clinical, imaging, and histological features, and surgical resection modalities and outcomes of adult sacrococcygeal teratoma (SCT).
METHODS
Adult patients with histopathologically diagnosed SCT were enrolled in our hospital between August 2010 and August 2018. Each patient's characteristics and clinical information were reviewed.
RESULTS
There were 8 patients in the study (2 males, 6 females) with a median age of 34 years (range, 18-67 years). The time to clinical symptoms was 14 d to 35 years, with a median time of 4 years. Six patients presented with symptoms of sacrococcygeal pain, and four with signs of sacrococcygeal mass and ulceration in the sacrococcygeal region. Six patients were evaluated using a combination of computed tomography (CT) and magnetic resonance imaging (MRI). All patients showed a presacral tumor with heterogeneous intensity on CT images. All patients underwent surgical treatment, including 6 parasacral, 1 transabdominal, and 1 combined anterior-posterior surgery cases. Seven patients were histopathologically diagnosed with benign mature SCT, and have shown no recurrence. One patient had malignant SCT, with recurrence at 84 months after surgery. After a second surgery, the patient had no recurrence within 6 months follow-up after re-resection.
CONCLUSIONS
Our retrospective study demonstrated: (1) adult SCT is difficult to diagnose because of a lack of typical clinical symptoms and signs; (2) a combination of CT and MRI examination is beneficial for preoperative diagnosis; (3) the choice of surgical approach and surgical resection modality depends on the size, location, and components of the tumor, which can be defined from preoperative CT and MRI evaluation; (4) most adult SCTs are benign; the surgical outcome for the malignant SCT patient was good after complete resection. Even for the patient with recurrent malignant SCT, the surgical outcome was good after re-resection.
Topics: Adolescent; Adult; Aged; Female; Humans; Magnetic Resonance Imaging; Male; Margins of Excision; Middle Aged; Neoplasm Recurrence, Local; Pain Measurement; Retrospective Studies; Sacrococcygeal Region; Teratoma; Tomography, X-Ray Computed; Treatment Outcome; Young Adult
PubMed: 31273964
DOI: 10.1631/jzus.B1800621 -
The Indian Journal of Radiology &... 2017Unilateral renal agenesis (URA) is a rare condition with a reported incidence of 0.93-1.8 per 1000 autopsies. It is commonly diagnosed as an incidental finding on...
Unilateral renal agenesis (URA) is a rare condition with a reported incidence of 0.93-1.8 per 1000 autopsies. It is commonly diagnosed as an incidental finding on imaging. URA is frequently associated with other genitourinary anomalies. Different associations have been described in both males and females, however, to our knowledge, it has not been reported with subseptate uterus (SSU) and sacrococcygeal teratoma (SCT) in the same individual. Here, we present a unique case of URA with SSU and SCT.
PubMed: 29089677
DOI: 10.4103/0971-3026.215577 -
Seminars in Pediatric Surgery Dec 2022
Topics: Child; Humans; Wilms Tumor; Hepatoblastoma; Liver Neoplasms; Kidney Neoplasms
PubMed: 36423516
DOI: 10.1016/j.sempedsurg.2022.151233 -
The FEBS Journal Nov 2020Antiepileptic/teratogen valproate (VPA) is a histone deacetylase inhibitor/epigenetic drug proposed for the antitumor therapy where it is generally crucial to target...
Antiepileptic/teratogen valproate (VPA) is a histone deacetylase inhibitor/epigenetic drug proposed for the antitumor therapy where it is generally crucial to target poorly or undifferentiated cells to prevent a recurrence. Transplanted rodent gastrulating embryos-proper (primitive streak and three germ layers) are the source of teratoma/teratocarcinoma tumors. Human primitive-streak remnants develop sacrococcygeal teratomas that may recur even when benign (well differentiated). To screen for unknown VPA impact on teratoma-type tumors, we used original 2-week embryo-derived teratoma in vitro biological system completed by a spent media metabolome analysis. Gastrulating 9.5-day-old rat embryos-proper were cultivated in Eagle's minimal essential medium (MEM) with 50% rat serum (controls) or with the addition of 2 mmVPA. Spent media metabolomes were analyzed by FTIR. Compared to controls, VPA acetylated histones; significantly diminished overall teratoma growth, impaired survival, increased the apoptotic index, and decreased proliferation index and incidence of differentiated tissues (e.g., neural tissue). Control teratomas continued to grow and differentiate for 14 days in isotransplants in vivo, but in vitro VPA-treated teratomas resorbed. Principal component analysis of FTIR results showed that spent media metabolomes formed well-separated clusters reflecting the treatment and day of cultivation. In metabolomes of VPA-treated teratomas, we found elevation of previously described histone acetylation biomarkers [amide I α-helix and A(CH )/A(CH )]) with apoptotic biomarkers within the amide I region for β-sheets, and unordered and CH vibrations of lipids. VPA may be proposed for therapy of the undifferentiated component of teratoma tumors and this biological system completed by metabolome analysis, for a faster dual screening of antitumor/embryotoxic agents.
Topics: Acetylation; Animals; Apoptosis; Cell Differentiation; Cell Proliferation; Embryo, Mammalian; Female; Histone Deacetylase Inhibitors; Histones; Male; Rats; Spectroscopy, Fourier Transform Infrared; Teratoma; Valproic Acid
PubMed: 32056377
DOI: 10.1111/febs.15248 -
Journal of Indian Association of... Jan 2012To review the experience with the diagnosis and management of extragonadal germ cell tumors (GCT) with a subset analysis of those with atypical features.
AIM
To review the experience with the diagnosis and management of extragonadal germ cell tumors (GCT) with a subset analysis of those with atypical features.
MATERIALS AND METHODS
A retrospective chart review of patients of extragonadal germ cell tumors between 2000 and 2010 was carried out.
RESULTS
Fifteen children aged 7 days to 15 years (median, 1.5 years) were included. Three had an antenatal diagnosis (one sacrococcygeal, one retrobulbar, one retroperitoneal tumor) and were operated in the neonatal period. The locations were distributed between the retrobulbar area (1), anterior neck-thyroid gland (1), mediastinum (4), abdominothoracic extending through the esophageal hiatus (1), retroperitoneal (4) and sacrococcygeal (4). On histological examination, five harbored immature elements while two were malignant; the latter children received postexcision adjuvant chemotherapy. There was no mortality. At a median follow-up of 4.5 years (6 months to 8 years), 14/15 have had an event-free survival. One immature mediastinal teratoma that recurred locally 7.5 years after the initial operation was excised and adjuvant chemotherapy instituted.
CONCLUSIONS
Extragonadal GCTs in children are uncommon and occasionally present with atypical clinical, radiological and histological features resulting in diagnostic and therapeutic dilemmas.
PubMed: 22279357
DOI: 10.4103/0971-9261.91079 -
Cancer Management and Research 2020The present study aims to discuss the clinical features, treatment, and prognosis of fetal sacrococcygeal teratomas (SCTs) to improve the standard of diagnosis and...
OBJECTIVE
The present study aims to discuss the clinical features, treatment, and prognosis of fetal sacrococcygeal teratomas (SCTs) to improve the standard of diagnosis and treatment.
METHODS
The clinical data of 15 pregnant females with fetal SCT, admitted to Fujian Maternity and Child Health Hospital from January 2013 to January 2020, were retrospectively analyzed with respect to clinical characteristics, imaging features, complications, treatment options, and pregnancy outcomes.
RESULTS
The 15 cases of fetal SCT were all detected by color ultrasonography. There were two cases of cystic tumors and 13 cases of solid cystic tumors. In terms of tumor blood supply, there was one case without blood flow signal, eight cases with little blood flow signal, and six cases with abundant blood flow. At the time of delivery, there were two cases with a tumor diameter less than 5 cm, five cases with a diameter of 5-10 cm, and eight cases with a diameter of more than 10 cm. In terms of tumor shape and location, there were two cases of type I, ten cases of type II, and three cases of type III. There were six cases with an increased fetal heart to chest ratio, four cases of fetal edema, three cases of placental edema, four cases of excessive amniotic fluid, one case of insufficient amniotic fluid, three cases of fetal distress, one case of stillbirth, two cases of gestational diabetes mellitus, two cases of mirror syndrome, and two cases of postpartum hemorrhage. According to the pathological diagnosis, there were seven cases of mature teratoma, seven cases of immature teratoma, and one case of mixed germ cell tumor. There were six cases of induced delivery, nine cases of cesarean section, one case of premature birth, and two cases of mild neonatal asphyxia.
CONCLUSION
Fetal SCT was generally diagnosed by prenatal ultrasonography. The tumor blood supply, growth rate, size, nature of the tumor, clinical type, pathology, and maternal-fetal complications are all closely correlated with the prognosis. The timing and manner of the termination of pregnancy should be determined on the basis of the pregnant female, the fetus, and the tumor.
PubMed: 33380826
DOI: 10.2147/CMAR.S287682 -
African Journal of Paediatric Surgery :... 2022Parasitic conjoined twin is a rare but well-known entity with unclear embryopathogenesis. Abnormal conjoined twinning can result in an externally attached parasitic twin...
BACKGROUND
Parasitic conjoined twin is a rare but well-known entity with unclear embryopathogenesis. Abnormal conjoined twinning can result in an externally attached parasitic twin (PT), an enclosed foetus in foetu, or a mature teratoma. The treatment requires complete excision and reconstruction of local anatomy which is not always straightforward.
MATERIALS AND METHODS
PT cases presenting over 12 years were analysed. Patients with complete data, histopathological diagnosis and follow-up were included in the study. During follow-up, specific complications and related investigations were considered.
RESULTS
A total of five patients at four different sites were identified: two retroperitoneal foetus in foetu and three externally attached PTs which were located in the lumbar region, sacrococcygeal area and on the lower anterior abdominal wall. All patients underwent complete surgical excision. In foetus in foetu cases, the blood supply was directly from the aorta with a short stump while the three externally located ones required meticulous and careful dissection with the reconstruction of local anatomy.
CONCLUSION
Parasitic conjoined twinning can present at different sites and surgical challenges vary accordingly. For surface lesions, reconstruction may be as complicated as excision. Prognosis can be affected by the excellence of anatomical restoration. Long-term follow-up is essential to address problems specific to the site of lesion and method of surgical reconstruction.
Topics: Abdomen; Humans; Retroperitoneal Space; Teratoma; Twins, Conjoined
PubMed: 36018202
DOI: 10.4103/ajps.ajps_78_21 -
Modern Pathology : An Official Journal... Apr 2014The biological behavior of teratomas is highly variable, and morphologic features alone are insufficient to predict their clinical course. Prognostic factors that...
The biological behavior of teratomas is highly variable, and morphologic features alone are insufficient to predict their clinical course. Prognostic factors that influence behavior include the following: patient sex, age, anatomic site, coincident neoplasm, and cytogenetic abnormalities. Gonadal teratomas have been well-characterized; postpubertal testicular teratomas are commonly associated with isochromosome 12p (i12p) and considered to nearly always carry a potential for malignant behavior, whereas ovarian and prepubertal testicular teratomas are i12p negative and predominantly benign in behavior. For extragonadal sites, such as sacrum and coccyx, clinical characteristics and i12p status are yet to be adequately characterized. As part of this study, we identified 19 sacrococcygeal teratomas in our surgical pathology archives from 1990 to 2012. Clinical records and slides were reviewed to confirm the original diagnosis. Gains in chromosome 12p, including i12p status were assessed in representative paraffin sections by fluorescence in situ hybridization. Our cases included 16 mature sacrococcygeal teratomas (11 prepubertal and 5 postpubertal) and three immature saccrococygeal teratomas (all prepubertal). Among mature teratomas, the average tumor size was larger in adults compared with prepubertal patients. A higher number of adult cases were recurrences (80% vs 21%), but only pediatric recurrences were managed with postoperative chemotherapy. All examined tumors were negative for i12p. 100% survival was documented in our cohort with a median follow-up of 6 years. We present a large series of sacrococcygeal teratomas and the first series to examine postpubertal adults at this anatomic site. All tumors lacked chromosome 12p gains, including i12p. Both pre- and postpubertal sacrococcygeal teratomas had a favorable outcome regardless of age or sex.
Topics: Adult; Biomarkers, Tumor; Child, Preschool; Chromosomes, Human, Pair 12; Female; Genetic Predisposition to Disease; Humans; In Situ Hybridization, Fluorescence; Infant; Infant, Newborn; Isochromosomes; Male; Middle Aged; Phenotype; Sacrum; Spinal Neoplasms; Survival Analysis; Teratoma; Treatment Outcome
PubMed: 24051698
DOI: 10.1038/modpathol.2013.171 -
Cureus Mar 2023Sacrococcygeal masses encompass a diverse range of pathologies. Prenatal ultrasound facilitates early detection of congenital sacrococcygeal masses. We present the case...
Sacrococcygeal masses encompass a diverse range of pathologies. Prenatal ultrasound facilitates early detection of congenital sacrococcygeal masses. We present the case of a newborn of a 22-year-old woman who was identified to have a sacrococcygeal mass by prenatal ultrasound that was initially diagnosed as sacrococcygeal teratoma. On examination after delivery, a large midline mass in the sacrococcygeal region was observed, which was globular in shape and had smooth, thin skin with bluish discoloration. Magnetic resonance imaging revealed a cystic lesion that protruded through a caudal sacral defect, consistent with a sacrococcygeal meningocele. The patient underwent surgical repair of the meningocele without any intraoperative complications and had preserved motor function in the lower extremities after the procedure. This case underscores the challenge of distinguishing sacrococcygeal teratoma from meningocele based on clinical presentation and prenatal ultrasound findings. An accurate preoperative diagnosis is essential for effective surgical planning.
PubMed: 37090346
DOI: 10.7759/cureus.36485 -
Modern Pathology : An Official Journal... Feb 2021Mature ovarian teratoma is considered to be a parthenogenetic tumor that arises from a single oocyte/ovum. Conversely, complete hydatidiform mole (CHM) is androgenetic...
Mature ovarian teratoma is considered to be a parthenogenetic tumor that arises from a single oocyte/ovum. Conversely, complete hydatidiform mole (CHM) is androgenetic in origin: classic CHM arises from a single or two sperm. Since mature ovarian teratoma and CHM have only maternal and paternal genomes, respectively, their genome imprinting is theoretically reverse, but this has yet to be investigated. Genome imprinting in struma ovarii, a special form of mature teratoma, remains unclear. Although a mature teratoma can rarely arise in extragonadal sites, its genome imprinting, as well as cell origin, is poorly understood. One of the most important mechanisms of genome imprinting is DNA methylation. To investigate the methylation profile of imprinted genes, we performed methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) of 21 imprinting control region (ICRs) of 9 imprinted genes/gene clusters in formalin-fixed, paraffin-embedded samples obtained from 12 mature ovarian teratomas, 6 struma ovarii, 10 CHMs, and 7 extragonadal (1 sacrococcygeal, 6 mediastinal) mature teratomas of females. In mature ovarian teratomas, ICRs of maternally and paternally imprinted genes showed high and low levels of methylation, respectively, and this pattern was almost reverse in CHMs. In CHMs, however, some ICRs showed aberrant methylation. The methylation profile of struma ovarii was comparable to that of mature ovarian teratomas, except for an adenomatous tumor. In extragonadal mature teratomas, the methylation pattern was somatic or irregular. In conclusion, mature ovarian teratomas/struma ovarii, CHMs, and extragonadal mature teratomas showed distinct methylation profiles of imprinted genes. Ovarian teratomas and CHMs are most likely to inherit their methylation profiles from their ancestral germ cells, although some aberrant methylation suggests a relaxation of imprinting in CHMs and a subset of struma ovarii. Extragonadal mature teratomas may carry a methylation profile of misplaced primordial germ cells or possibly somatic cells that have been reprogrammed in vivo.
Topics: DNA Methylation; Female; Genomic Imprinting; Humans; Hydatidiform Mole; Ovarian Neoplasms; Pregnancy; Teratoma; Uterine Neoplasms
PubMed: 32873866
DOI: 10.1038/s41379-020-00668-8