A unique case of gliomatosis peritonei after resection of a retroperitoneal immature teratoma in an infant.
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ABSTRACT:
SUBMITTER: Liu S
PROVIDER: S-EPMC10722056 | biostudies-literature | 2023 Dec
REPOSITORIES: biostudies-literature
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A unique case of gliomatosis peritonei after resection of a retroperitoneal immature teratoma in an infant.
Liu Shuyi S Gao Qiu Q Hu Yuelin Y
Quantitative imaging in medicine and surgery 20231025 12
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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.
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Project description:We report a rare case of large immature retroperitoneal teratoma in a neonate. The diagnostic and therapeutic challenges of dealing with such a case have been discussed and the relevant literature reviewed.
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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:BackgroundGiant mediastinal tumors in the pediatric population can pose unique challenges for resection such as cardiovascular collapse on induction of anesthesia and injury to surrounding structures that may be compressed, displaced, or invaded by the mass. Principles that must be borne in mind during removal of giant mediastinal masses include: appropriate cross-sectional imaging to define extent of mass; airway control during induction of anesthesia; a multidisciplinary collaborative approach including cardiothoracic surgery; preparation for urgent sternotomy; plan for peripheral cannulation to institute cardiopulmonary bypass if needed; preservation of neurovasculature structures during dissection; complete resection whenever possible. While complete resection is desirable and results in an excellent prognosis, it may not be achievable especially if the tumor encases coronary arteries, and it is acceptable to leave small amounts of tumor behind.Case descriptionHere we present a case describing surgical management of a giant mediastinal teratoma in a two-month-old female. The patient was found to have a large mediastinal mass during workup for cough and noisy breathing. She underwent preoperative echocardiogram demonstrating normal cardiac function followed by uncomplicated, open resection of the mass.ConclusionsGiant mediastinal tumors give rise to unique challenges for resection in small infants. The principles of airway control, preparation for urgent sternotomy, preparation for peripheral cardiopulmonary bypass cannulation, and preservation of neurovasculature during dissection must be borne in mind.
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Project description:Gastric teratoma is a rare tumor, accounting for less than 1 % of all teratomas in infants & children. To date, only about 102 cases have been reported in the literature. A 10 month old infant was brought with a history of upper abdominal mass which was otherwise asymptomatic. On evaluation it was diagnosed as gastric teratoma. On laparotomy the mass was found to be originating from lesser curvature of stomach. Mass was excised and histopathologically it was a mature cystic teratoma. No recurrence after 18 months of follow-up.
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Project description:BACKGROUND:Hydrops fetalis as well as abdominal compartment syndrome (ACS) are conditions that are associated with high mortality rates. A rare case of immature gastric teratoma causing fetal hydrops and subsequent ACS is presented. The related pathophysiologic mechanisms are discussed, and the importance of timely recognition and appropriate interventions are highlighted. CASE PRESENTATION:The male patient was born preterm, weighing 3.9?kg., by Cesarean section. Prior prenatal ultrasounds were normal, but a scan done just before delivery had findings indicating polyhydramnios, fetal ascites, and meconium peritonitis. Upon delivery, the patient had respiratory distress, anasarca and a massively distended abdomen. Resuscitation measures, including ventilatory support, were instituted. Imaging studies showed ascites as well as a large, complex intra-abdominal lesion with calcifications. In the succeeding hours, anuria persisted, anasarca worsened, the abdomen became more distended, and inotrope requirements increased. The occurrence of ACS, from what was presumed to be a retroperitoneal teratoma, was therefore considered. Laparotomy was done on the 28th hour of life, with en bloc excision of a massive tumor and attached section of the greater curvature of the stomach. Passage of urine occurred intra-operatively, and the patient was soon after weaned off inotropes and ventilator support. The histopathologic result was immature gastric teratoma. No chemotherapy was given, and the patient's serum AFP is at normal levels 15?months following surgery. CONCLUSION:The presence of a massive intra-abdominal lesion can result in the pathophysiologic continuum of hydrops fetalis and neonatal ACS. The early recognition of such an association can enable appropriate expectant management of similarly affected neonates, including emergent decompression laparotomy.
| S-EPMC7184682 | biostudies-literature
Project description:BackgroundPseudomyxoma peritonei is a rare disease condition mainly caused by primary mucinous tumors from the appendix and rarely from the ovary, such as when mucinous ovarian tumors arise from within a teratoma. Molecular analyses of pseudomyxoma from the appendix showed that KRAS and GNAS pathogenic variants are common genetic features of pseudomyxoma peritonei. However, the origin of the tumors is difficult to be identified via genetic variants alone. This study presents a case of pseudomyxoma peritonei of ovarian origin, which was diagnosed by comprehensive genomic profiling with ploidy analysis in a series of primary, recurrent, and autopsy tumor specimens.Case presentationA 40-year-old woman was diagnosed with Stage IC2 mucinous ovarian tumor of borderline malignancy with mature cystic teratoma, upon clinical pathology. Immunohistochemical analysis suggested that the mucinous tumor was derived from the intestinal component of an ovarian teratoma. Three years later, intraperitoneal recurrence was detected, which subsequently progressed to pseudomyxoma peritonei. Genomic analysis detected KRAS (G12D), GNAS (R201C), and FBXW7 (R367*) variants in the primary tumor. In addition, the tumor showed aneuploidy with loss of heterozygosity (LOH) in all its chromosomes, which suggested that the primary ovarian tumor was derived from germ cells. Existence of one Barr body suggested the existence of uniparental disomy of the tumors throughout the genome, instead of a haploid genotype. All three pathogenic variants remained positive in the initial recurrent tumor, as well as in the paired DNA from the whole blood in pseudomyxoma peritonei. The pathogenic variant of KRAS (G12D) was also identified in the autopsy specimen of the appendix by droplet digital polymerase chain reaction.ConclusionsThis study pathologically and genetically confirmed that the primary ovarian borderline tumor was derived from the intestinal component of an ovarian teratoma, and that the subsequent pseudomyxoma peritonei progressed from the primary ovarian tumor. Integrative genomic analysis was useful to identify cellular origin of tumors, as well as to precisely interpret the process of disease progression.
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