Retroperitoneal Immature Teratoma in a Neonate.
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ABSTRACT:
SUBMITTER: Kumar C
PROVIDER: S-EPMC5538607 | biostudies-other | 2017 Apr-Jun
REPOSITORIES: biostudies-other
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Project description: Not available
| S-EPMC10722056 | biostudies-literature
Project description:Teratomas are embryonal neoplasms composed of 2 or 3 germ layers. We present the case of a female neonate who had a rare, mature, skull base teratoma that was managed surgically by fetal surgery, neurosurgery, otolaryngology, and plastic surgery. Imaging revealed an anterior skull base teratoma with extension into the nasopharynx, resulting in gross enlargement of the right nostril and sinonasal cavity. An ex utero intrapartum treatment procedure was performed to establish an airway before placental support was removed. Subsequently, neurosurgery and ENT performed endonasal resection of the teratoma without intracranial involvement or cerebral spinal fluid leakage. Although originally not part of the surgical plan, plastic surgery was consulted and performed an advancement flap to repair the defect caused by the adhesion as well as reduce the substantially sized ala. After the resection, a 2-stage reconstruction was performed by the plastic and reconstructive surgeon. The first stage involved medial movement of the right ala and advancement lateral tissue to repair the nasal cleft. The second stage involved harvesting cartilage from the concha to reinforce the alar rim and further reducing nostril size by a Y-V advancement flap. The child is now 3 years of age and is without evidence of recurrence or neurological defects. She has an optimal, symmetrical cosmetic outcome of the nasolabial area. This rare case highlights the need for early diagnosis of these airway-obstructing masses to prepare for a safe delivery. Furthermore, a plastic surgery consult during the resection is indispensable to improved cosmetic outcomes.
| S-EPMC11903010 | biostudies-literature
Project description:The misdiagnosis of retroperitoneal teratoma as a gastric stromal tumor is rarely reported. There are numerous abdominal organs, and retroperitoneal tumors are easily concealed by organs situated more anteriorly on the abdomen; therefore, retroperitoneal tumors are easily misdiagnosed as human digestive system tumors by abdominal surgeons. We herein present the case of a 39-year-old female patient who was diagnosed with a gastric stromal tumor (GST) on preoperative examination, whereas the postoperative diagnosis was retroperitoneal mature cystic teratoma. Such cases are clinically rare. In the present case, the tumor was located in the retroperitoneum, near the lesser gastric curvature, without an obvious gap. The anterior surface of the tumor was concealed by the lesser gastric curvature and did not move significantly with breathing and changes in posture. In such cases, a preoperative misdiagnosis is very likely. The aim of the present study was to improve our understanding of the presentation of retroperitoneal teratomas, thereby gaining more clinical experience and hopefully reducing the rate of misdiagnosis.
| S-EPMC7179382 | biostudies-literature
Project description:Fetal cervical teratoma is a rare congenital neck tumor. Here, we report a case of a fetus with an anterior solid neck tumor that was confirmed to have an immature teratoma by histology. A duplication was found at chromosome 14q24.1-q24.3 of the fetus in chromosome microarray (CMA) and whole exome sequencing (WES), which was a copy number variation (CNV) and a probably new-onset. Ultrasound coupled with magnetic resonance imaging (MRI) can be considered to be a relatively reliable diagnostic tool, whereas ex-utero intrapartum therapy or resection of the tumor mass on placental support may improve the chances of the newborn's survival. Strangely, the same duplication occurred on her next fetus that was found with complex congenital heart malformations. CNV at chromosome 14q24.1-q24.3 needs to be paid more attention.
| S-EPMC9423720 | biostudies-literature
Project description:Mature cystic teratoma is one of the most common tumors of the ovaries, testis, mediastinum, and retroperitoneum; however, secondary retroperitoneum lesions are rare entities in adults. We report a case of a 22-year-old female who was previously diagnosed with a mature teratoma of left ovarian was hospitalized due to dull abdominal pain in right hypochondria. Radiological evaluation revealed a mass in the right upper abdominal and flank region with an extension from the posterior aspect of the duodenum, composed of greasy and cystic elements. A tumor was resected through the Kocher's laparotomy and the pathology report confirmed the diagnosis of a mature cystic teratoma with no evidence of malignancy or immature components.
| S-EPMC6439285 | biostudies-literature
Project description:Teratoma of the orbit is rare; very few cases have been published in literature. A case of orbital teratoma in a neonate is presented where the proptosis was massive enough to obscure the eyeball. Clinically, the diagnosis of teratoma was considered. As there was no possibility of salvaging the eye, exenteration of the orbit was done. Orbital teratomas are generally benign. Histopathological examination revealed the features of malignant teratoma.
| S-EPMC5525488 | biostudies-other
Project description:Coexistent growing teratoma syndrome (GTS) and gliomatosis peritonei (GP) arising during chemotherapy of ovarian immature teratoma (IMT) is extremely rare and can be misdiagnosed as recurrent or progressive disease. We present a 33-year-old woman diagnosed with GTS with synchronous GP during chemotherapy of IMT. She underwent ovarian cystectomy due to ovarian immature teratoma and chemotherapy were administered. The α-fetoprotein (AFP) concentration decreased from 28.7 ng/mL to normal after the second cycle. Four days after the third cycle of chemotherapy, ultrasound and CT revealed an 8-cm mass with negative tumor markers in the pouch of Douglas. An exploratory laparotomy was conducted, and a smooth round cystic-solid 8-cm mass was noted in the pouch of Douglas. Extensive peritoneal seeding glial nodules were also observed on the surface of the uterus, peritoneum, and omentum. The patient underwent a partial omentectomy, intact resection of the tumor, and resection of most of the glial nodules. Postoperative pathology demonstrated a pure mature cystic teratoma component in the mass, as well as diffuse GP involving the uterine serosa, peritoneum, and omentum; this diagnosis of GTS with synchorous GP should be considered in IMT patients with mass newly identified during chemotherapy while tumor markers are normal after treatment.
| S-EPMC9497723 | biostudies-literature
Project description: Not available
| S-EPMC10722040 | biostudies-literature
Project description:ObjectiveImmature teratomas are rare malignant ovarian germ cell tumours, typically diagnosed in young women, where fertility-sparing surgery is the treatment of choice. The role of adjuvant chemotherapy in stage I disease remains controversial. We evaluated the impact of surveillance versus chemotherapy on the recurrence rate in stage I immature teratomas.MethodsWe collected a single centre retrospective series of patients with stage I immature teratomas treated with fertility-sparing surgery at San Gerardo Hospital, Monza, Italy, between 1980 and 2019. Potential risk factors for recurrence were investigated by multivariate logistic regression.ResultsOf the 74 patients included, 12% (9/74) received chemotherapy, while 88% (65/74) underwent surveillance. Median follow-up was 188 months. No difference in recurrence was found in stage IA/IB and IC immature teratomas [10% (6/60) vs. 28.6% (4/14) (P=0.087)], grade 1, grade 2, and grade 3 [7.1% (2/28) vs. 14.3% (4/28) vs. 22.2% (4/18) (p=0.39)], and surveillance versus chemotherapy groups [13.9% (9/65) vs. 11.1% (1/9)) (p = 1.00)]. In univariate analysis, the postoperative approach had no impact on recurrence. The 5-year disease-free survival was 87% and 90% in the surveillance and chemotherapy groups, respectively; the overall survival was 100% in both cohorts.ConclusionsOur results support the feasibility of surveillance in stage I immature teratomas. Adjuvant chemotherapy may be reserved for relapses. However, the potential benefit of chemotherapy should be discussed, especially for high-risk tumours. Prospective series are warranted to confirm our findings.What is already known on this topicTo date, no consensus has been reached regarding the role of adjuvant chemotherapy in stage I immature teratomas of the ovary. Some studies suggest that only surveillance is an acceptable choice. However, guidelines are not conclusive on this topic.What this study addsNo difference in terms of recurrence was observed between the surveillance and the adjuvant chemotherapy group. All patients who relapsed were successfully cured with no disease-related deaths.How this study might affect research practice or policyAdjuvant chemotherapy should be appropriately discussed with patients. However, it may be reserved for relapse according to our data.
| S-EPMC10869612 | biostudies-literature
Project description:Ovarian immature teratomas (OITs) are malignant tumors originating from the ovarian germ cells that mainly occur during the first 30 y of a female's life. Early age of onset strongly suggests the presence of susceptibility gene mutations for the disease yet to be discovered. Whole exon sequencing was used to screen pathogenic mutations from pedigrees with OITs. A rare missense germline mutation (C262T) in the first exon of the BMP15 gene was identified. In silico calculation suggested that the mutation could impair the formation of mature peptides. In vitro experiments on cell lines confirmed that the mutation caused an 84.7% reduction in the secretion of mature BMP15. Clinical samples from OIT patients also showed a similar pattern of decrease in the BMP15 expression. In the transgenic mouse model, the spontaneous parthenogenetic activation significantly increased in oocytes carrying the T allele. Remarkably, a mouse carrying the T allele developed the phenotype of OIT. Oocyte-specific RNA sequencing revealed that abnormal activation of the H-Ras/MAPK pathway might contribute to the development of OIT. BMP15 was identified as a pathogenic gene for OIT which improved our understanding of the etiology of OIT and provided a potential biomarker for genetic screening of this disorder.
| S-EPMC10927523 | biostudies-literature