Immature Teratoma Research Articles

Clinical Approach to Neuroendocrine Neoplasm Associated With Ovarian Teratoma.

BackgroundNeuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and less than 5% of all neuroendocrine tumors. They may arise alone (as monodermal, specialized teratoma – ovarian carcinoid) or as a part of other ovarian lesion: cystic mature or immature teratomas. Due to the rarity and limited amount of such cases reported in the literature, there is no consensus on diagnostic and therapeutic procedures in this group of patients.Materials and MethodsThe group of 10 patients at the age of 19 to 77 years (mean 42.8 ± 17.9), diagnosed with unilateral NET within ovarian teratoma were analyzed. The histopathological type of tumor, progression free survival after surgical treatment and presence of hormonally active syndrome were assessed.Results70% (n=7) of patients was diagnosed with mature cystic teratomas containing NET component and 30% (n=3) with monodermal teratoma (strumal carcinoid). All cases of monodermal teratomas were found in women at premenopausal age. Determined Ki67 ranged from 2% to 9%. Ninety percent of lesions (n=9) stained positive for synaptophysin and chromogranin, while markers: CK20, CK7, TTF-1 and CDX2 were negative in all cases, which ruled out their metastatic nature. None of the patients presented with carcinoid syndrome. All followed-up patients remain progression-free, which confirms surgical intervention being a crucial and sufficient method of treatment.ConclusionsThe prognosis and clinical behavior of NETs associated with ovarian teratomas are good with long progression-free survival.

Adult Retroperitoneal Mature Cystic Teratoma Masquerading as Non-Endocrine Primary Adrenal Tumor

Teratomas belong to non-seminomatous germ cell tumors and originate from totipotent germ cells. They are mostly made up of tissues derived from all three layers, i.e., endoderm, mesoderm and ectoderm. They can be mature teratomas, which are benign or immature teratomas, having malignant potential. Extra-gonadal mature cystic teratomas are uncommon, particularly in retroperitoneum. Other sites are sacrococcygeal, mediastinal, and pineal regions. They are mostly detected in females in reproductive age group. In this case report, we discuss a female patient being worked up for persistent vomiting and food indigestion, initially managed on the line of acid peptic disease. She was found to have a large retroperitoneal mass in adrenal region producing mass effect on intestines. Later on, it was discovered to be mature cystic teratoma on histopathological evaluation. Key Words: Mature teratoma, Adrenal gland, Retroperitoneum.

WITHDRAWN: THE EFFECT OF NIVOLUMAB IN PEDIATRIC MALIGNANT TUMORS: A SINGLE CENTER EXPERIENCE WITH EIGHT PATIENTS

Objective The programmed cell death 1 (PD-1) receptor is an immune checkpoint receptor expressed by activated T cells. PD-1 inhibits the immune system by binding to its ligands expressed on tumor cells. Nivolumab and pembrolizumab are some of the monoclonal antibodies that inhibit the PD-1. We present our experience with eight cases which were treated with nivolumab for different malignant diseases in our clinic. Methodology A total of eight patients (5 girls, 3 boys) aged between 4 and 16 years were treated with nivolumab at 3mg/kg/dose approximately twice a week in Erciyes University Pediatric Hematology clinic between 2019-2021. Nivolumab treatments of seven patients were given median 11 (4-32) doses and discontinued due to progression at a median 6 months (2-17 months). Results Four patients were diagnosed with miss match repair syndrome in addition to their malignant disease. Non-Hodgkin lymphoma (two with T-cell and one with histiocyte-rich B-cell) in three patients, brain tumor (pons glioma, midline glioma, glioblastoma multiforme) in three patients, germ cell tumor (yolk salk, immature teratoma) in two patients, one patient had colon adenocarcinoma in addition to brain tumor. Except for nausea in a patient, no drug-related side effect was observed in patients. Conclusion Nivolumab was well tolerated in patients with CMMRD syndrome with some transient partial responses. There is a risk of developing secondary cancer in patients with CMMRD syndrome. Further studies are needed due to the limited use in children.

Molecular genetic evidence supporting diverse histogenic origins of germ cell tumors.

Germ cell tumors (GCTs) originate during the histogenesis of primordial germ cells to mature gametes. Previous studies identified five histogenic mechanisms in ovarian mature teratomas (type I: failure of meiosis I; type II: failure of meiosis II; type III: duplication of the genome of a mature gamete; type IV: no meiosis; and type V: fusion of two different ova), but those of other GCTs remain elusive. In this study, we analyzed 84 GCTs of various pathologic types to identify the histogenesis using single-nucleotide polymorphism array by analyzing copy-neutral loss of heterozygosity (CN-LOH) and copy number alterations (CNAs). We detected types I and II in ovarian teratomas, type III in ovarian teratomas and yolk sac tumors (YSTs), and type IV in all GCT types. The GCTs with multiple-type histogenesis (I-IV) (ovarian mature/immature teratomas and YST) show meiotic CN-LOH with scant CNAs. Type IV-only GCTs are either with mitotic CN-LOH and abundant CNAs (seminoma, dysgerminoma, testicular mixed GCTs) or with scant CNAs and no CN-LOH (pediatric testicular and mediastinal teratomas). The development sequences of CN-LOH and CNA are different between the multiple type (I-IV) GCTs and type IV-only GCTs. We analyzed two different histologic areas in eight GCTs (one mature teratoma with a mucin-secreting adenoma, two immature teratomas, and five mixed GCTs). We found that GCTs (mature teratoma, immature teratoma, and mixed GCT) showed different genomic alterations between histologic areas, suggesting that genomic differences within a GCT could accompany histologic differentiation. Of note, we found evidence for collision tumors in a mixed GCT. Our data indicate that GCTs may have various histogenesis and intratumoral genomic differences, which might provide important information for the identification of GCTs, especially for those with different histologic areas. © 2021 The Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

High-Grade Sarcoma Arising in the Background of Growing Teratoma Syndrome; A Rare Case

Abstract Introduction/Objective Growing teratoma syndrome is a rare condition seen in patients with a history of immature teratomas or mixed germ cell tumor status post-treatment. It usually occurs within the first two years of diagnosis but rarely can be seen years later. Methods/Case Report We report a case of a 48-year-old female with a previous history of ovarian malignancy in adolescence status post-TAH/BSO and chemotherapy, now presenting with a 23 cm multi cystic septate mass with some solid components in the subdiaphragmatic area, extending into the liver on imaging. Nodular implants along the surface of the right hepatic lobe and multiple peritoneal implants were seen. Histology of peritoneal implants showed fibro adipose tissue admixed with rare scattered glandular elements, epithelial fragments, and mature neuroglial tissue, consistent with mature teratoma. Liver biopsy revealed short fascicles of spindled, epithelioid and rhabdoid tumor cells admixed with foci of the myxoid stroma. Scattered moderate cytologic atypia, atypical mitosis, and necrosis were appreciated. Tumor cells showed strong and diffuse reactivity to vimentin while being negative for epithelial, neural/mesenchymal, mesothelial, sex cord-stromal, and germ cell differentiation markers. Differential diagnostic considerations included spindle cell transformation of a germ cell tumor or high-grade sarcoma, not otherwise specified arising in the background of growing teratoma syndrome. FISH testing for isochromosome 12p was negative excluding sarcomatoid germ cell tumor. Results (if a Case Study enter NA) NA Conclusion This case study highlights the importance of considering the rare complication of high-grade sarcoma arising in the background of growing teratoma syndrome.

12p gain is predominantly observed in non-germinomatous germ cell tumors and identifies an unfavorable subgroup of central nervous system germ cell tumors.

Central nervous system (CNS) germ cell tumors (GCTs) are neoplasms predominantly arising in pediatric and young adult populations. While germinomas generally respond to chemotherapy and radiation, non-germinomatous GCTs (NGGCTs) require more intensive treatment. This study aimed to determine whether 12p gain could predict the prognosis of CNS GCTs. Eighty-two CNS GCTs were included in this study. The 12p gain was defined by an additional 12p in the background of potential polyploidy or polysomy. Cases were analyzed using an Illumina methylation 450K array for copy number investigations and validated by fluorescence in situ hybridization (FISH). A 12p gain was found in 25-out-of-82 cases (30%) and was more frequent in NGGCTs (12% of germinoma cases and 50% of NGGCT cases), particularly in cases with malignant components, such as immature teratoma, yolk sac tumor, choriocarcinoma, and embryonal carcinoma. 12p gain and KIT mutation were mutually exclusive events. The presence of 12p gain correlated with shorter progression-free (PFS) and overall survival (OS) (10-year OS: 59% vs. 94%, with and without 12p gain, respectively, P = 0.0002), even with histology and tumor markers incorporated in the multivariate analysis. Among NGGCTs, 12p gain still had prognostic significance for PFS and OS (10-year OS: 47% vs. 90%, respectively, P = 0.02). The 12p copy number status was shared among histological components in mixed GCTs. 12p gain may predict the presence of malignant components of NGGCTs, and poor prognosis of the patients. It may be associated with early tumorigenesis of CNS GCT.

Fat-containing pelvic lesions in females.

Pelvic tumors are common in females and have a broad differential diagnosis. The clinical management of pelvic tumors varies widely-from observation to surgical resection-and imaging plays a pivotal role in diagnosis and clinical decision-making in these cases. In particular, imaging can help determine the organ of origin and tissue content of these tumors, which are the most important steps to narrowing the differential diagnosis. Fat has a characteristic appearance and is often easily identified on ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). The amount and distribution of intralesional fat varies in different types of tumors. Macroscopic intralesional fat is often easily recognized by its hyperechoic appearance on US and low attenuation on CT similar to subcutaneous fat. On MRI, macroscopic fat is hyperintense on T1-weighted (T1W) images, with characteristic signal loss on fat-saturated sequences and India-ink artifact on opposed-phase T1W images. Macroscopic fat is the hallmark of teratomas, which are the most common ovarian neoplasms. Uterine lipoleiomyomas, peritoneal loose bodies, intraperitoneal and extraperitoneal primary lipomatous tumors such as lipoma and liposarcomas, and extra-adrenal myelolipomas are other pelvic masses distinguished by the presence of macroscopic fat. However, the imaging diagnosis of pelvic masses containing minimal or microscopic fat, such as immature ovarian teratomas, steroid cell ovarian neoplasms, and extramedullary hematopoiesis, can present a diagnostic challenge owing to their nonspecific appearance on US or CT. Obtaining MRI with in-phase and opposed-phase dual-echo T1W sequences and depicting chemical shift artifacts can be helpful in distinguishing these lesions.

A Case of Cervical Teratoma in an Infant.

Cervical teratoma is a rare form of teratoma in neonates and is an unusual cause of cervical masses in them. Teratomas are unusual tumors derived from all 3 germs cell layers: endoderm, mesoderm, and ectoderm, with varying proportions. The cervical teratoma is a rare entity. Its prognosis mostly depends on the risk of neonatal respiratory distress, its extension and potential malignancy. Surgical management must be as complete as possible to avoid recurrences and malignant transformation. We report a case of a cervical immature teratoma in an infant with total excision and cure. No recurrence has been reported. The aim of our study is to review the diagnosis, management and outcomes of congenital cervical teratomas. Cervical teratoma although uncommon should be considered in the differential diagnosis of neck masses in neonates. Teratomas are rare tumors derived from all three germ cell layers affecting the neck in 3% of all cases. An early complete surgical approach to congenital cervical teratomas allows good results with low rates of complication and recurrence.

Immature Teratoma and Teratoma Associated with Associated with Malignancies, Clinicopathological and Survival Outcome at AHPGIC

Immature Teratoma and Teratoma Associated with Associated with Malignancies, Clinicopathological and Survival Outcome at AHPGIC

A Newborn With a Mass on the Brain.

A Newborn With a Mass on the Brain.

A 28-week-old fetus with an orbital immature teratoma

A teratoma is a tumor comprising three embryonic germ layers. Teratomas are classified as mature and immature teratomas based on their histological differentiation. Few teratomas, particularly immature teratomas, occur in the orbital cavity. We report a case of a 28-week-old fetus with an orbital immature teratoma. The parents terminated the pregnancy because of the rapid growth and unknown nature of the tumor. Owing to its relatively low incidence, no standard treatment for orbital teratomas has been devised. Generally, to preserve vision and for aesthetic reasons, surgery should be performed as soon as possible.

OC03.06: Origin of acoustic shadowing as a discriminatory tool in determining the nature of neoplastic adnexal masses

Acoustic shadowing is considered a predictor of benignity in adnexal masses but may also be seen in malignant ones. We aimed to investigate whether the origin of the shadow can help determine the nature of a mass. This was a retrospective multicentre study of neoplastic adnexal masses with known histopathology, which showed acoustic shadowing on preoperative sonography. Two examiners reviewed the images and video clips, and reached consensus. The study included 100 adnexal masses with 25 different histopathologic diagnoses. More than 80% of the masses with shadowing from cystic contents (e.g. dermoid) or solid tissue because of inherent structural features (e.g. fibroma) were benign. When shadowing from solid areas arose in calcific foci (e.g. immature teratoma) or along fibrovascular tissue (e.g. serous carcinoma), 80-90% were malignant. Shadows from the margins of locules, or intracystic septae imaged perpendicularly or obliquely appeared non-informative. There was one case of shadowing from calcific foci in the walls of a mucinous cystadenoma (figure 1). Acoustic shadowing in an adnexal mass can have different connotations depending on the structure causing the attenuation. Of particular importance are shadows along the fibrovascular structures in solid tissue, which may result in malignant masses being erroneously classified as ‘likely benign’. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Ex Utero Intrapartum Treatment procedure for congenital cervical germ cell tumor

Congenital cervical germ cell tumors are a rare location for extragonadal germ cell tumors that may lead to acute airway obstruction. We present the unique case and successful resection of an immature cervical teratoma. Urgent Ex Utero Intrapartum Treatment (EXIT) procedure was performed via Cesarean section with subsequent endotracheal intubation to maintain the airway. Successful surgical resection of the mass at day six of life was performed showing histologically immature teratoma along with gliomatosis in surrounding lymph node tissue. The patient recovered well with no abnormalities on follow up at 14 months.

VP24.02: Severely enlarged fetal head due to intracranial immature teratoma: a case report

VP24.02: Severely enlarged fetal head due to intracranial immature teratoma: a case report

Multidisciplinary management of ovarian germ cell tumours-a single institutional study from India.

BackgroundOvarian germ cell tumours constitute a heterogeneous group of neoplasm with malignant potential being seen in 5% of cases. There is limited data on treatment outcomes of patients with malignant ovarian germ cell tumours (MOGCT). Here, we present our hospital audit of patients with MOGCT.Material and methodsThis is a retrospective data review of patients with MOGCT treated between May 2011 and December 2019. Patients were treated with staging laparotomy and adjuvant chemotherapy, wherever applicable. Surveillance was allowed for those at low risk for recurrence. Clinicopathologic features and treatment details were recorded, and survival analysis was performed.ResultsSixty-five patients with a median age of 25 years (range: 11–52 years) were treated during the study period. The most common histology was immature teratoma in 35.3% of cases. International Federation of Gynecology and Obstetrics stage IC was the most common stage of presentation (47%). Surveillance was advised for 12.3% of cases. Systemic therapy was given in 51 (78%) patients. At a median follow-up of 46 months (range: 1–109 months), the median progression-free survival (PFS) was not reached. Five-year PFS was 79.3% (95% CI: 65.8–88). The most common toxicity was febrile neutropenia (22%) among those who received systemic therapy.ConclusionImmature teratoma was the most common histology in our series. The majority presented in the early stage. MOGCT is a highly curable disease with surgery and systemic therapy.

Ex Utero Intrapartum Treatment (EXIT) Into the World: A Multidisciplinary Approach and Outcomes in a Malaysia Tertiary Centre.

Fetal airway obstruction that present during birth are challenging to manage. Cervical and thoracic mass may cause significant airway compression that leads to irreversible fetal morbidity and mortality. With the current advancement in prenatal diagnosis and assessment for surgical planning, EXIT has become an accepted, recognized and preferred option for safe delivery in complex head and neck masses. Ex utero intrapartum treatment (EXIT) is a life-saving procedure that provides time to establish an airway while maintaining uteroplacental circulation. It requires flawless coordination amongst multidisciplinary team to ensure immediate and safe baby deliveries. Retrospective review of EXIT procedures performed from the initial establishment of pediatric otolaryngology service in this centre starting from January 2019 to December 2020. Based on the prenatal diagnosis, five EXIT cases were performed in this centre within 2years. 4 cases were diagnosed as lymphatic malformation and 1 immature teratoma with cervical and thoracic compression. 4 babies successfully delivered to airway without any complications with 1 death resulting from disseminated intravascular coagulopathy after EXIT. This review of five EXIT procedures aims to bring forth the different outcomes, preoperative planning and sharing our experience as a tertiary and newly established centre. Early prenatal diagnosis, good collaboration and experience among multidisciplinary team ensures good long term outcomes.

Cystectomy of Ovarian Borderline and Malignant Tumors for Fertility Sparing: Outcome of Seventeen Cases

Background: Here we present a retrospective study of 17 cases in which the ovary on the affected side was spared in fertility-sparing surgery (FSS) during treatment for ovarian borderline malignant or malignant tumor. We determine that cystectomy is a suitable treatment for ovarian borderline tumors. Methods: A retrospective observation study was conducted at Saiseikai Fukuoka General Hospital in Japan between April 2009 and September 2020. Our hospital experienced 89 cases of FSS during treatment for ovarian borderline or malignant tumor. Of those, there were 17 cases in which the ovary on the affected side was spared. We examined recurrent and pregnant cases by stage, preoperative diagnosis, intraoperative pathological diagnosis, postoperative pathological diagnosis, and adjuvant therapy. Result: Of the 17, 12 cases were borderline malignant tumor, 4 were immature teratoma grade 1 (G1), and 1 case was endometrioid adenocarcinoma G1. Rapid intraoperative pathological diagnosis was conducted in 9 of the cases, and there were 6 in which surgical method was chosen based on the aforementioned results. Laparoscopic surgery was performed in 2 cases in which tumors were deemed benign via preoperative diagnosis, 2 cases of mature teratoma, and 2 in which borderline ovarian tumor was suspected. One (1) case of paraovarian cystecomy in a patient with history of multiple cesarean sections turned out to be serous borderline tumor. Postoperative treatment took place in only 1 case: endometrioid adenocarcinoma. There were 2 cases of recurrence, and 4 cases were eventually able to become pregnant naturally post-surgery. These pregnant cases included 1 in which serous borderline tumor recurred and we performed both cystectomy and lymphadenectomy, and one in which chemotherapy was performed after cyst enucleation for endometrioid adenocarcinoma G1. Conclusion: At present, there is no clear policy for FSS in cases such as stage Ib in which there are bilateral tumors. Accordingly, in the current study a radiologist was consulted for preoperative diagnosis, and surgical method was chosen with a view towards possible borderline malignancy or malignancy. In cases where fertility preservation of the affected ovary is a high priority, it is crucial to clearly explain the possibility of recurrence to the patient. We also stress the importance of detailed consultation among the surgical team during rapid intraoperal frozen section pathological examination for making the appropriate decision to ensure fertility preservation mid-surgery.

Gastric teratomas in paediatric population: A case series from a tertiary care centre of Northern India

A retrospective analysis of all paediatric patients diagnosed and treated for gastric teratomas in our hospital over the past 20 years was carried out (1997–2017). A database of the clinic-pathological characteristics of these patients was developed. In all, 7 patients were identified. All the patients underwent resection and were followed up every 6 months. The investigations performed included routine blood investigations, X-ray, alpha fetoproteins (AFP), beta HCG levels and a CT scan of the abdomen. There were seven patients with gastric teratomas and all of them were male except one female. Their ages ranged from 1 day to 4 years. Six (85.7%) patients presented during infancy. The manifestations were as follows: a palpable abdominal mass followed by anemia, respiratory distress, abdominal distension, gastric outlet obstruction with recurrent vomiting, and abdominal pain, anorexia, and melena. Histopathology was mature type in five (71.4%) cases, and immature Grades 1 and 2 in one (14.2%) each. Complications were seen in four (57.1%) cases, and there was one (14.2%) mortality in our series. Recurrence was seen in two of our cases. The preoperative diagnosis of Gastric teratoma may be challenging, but with recent advances in imaging an accurate diagnosis and staging is possible. Complete surgical excision is curative in all cases, with close observation and follow-up. • Gastric teratoma is an exceptionally rare embryonic neoplasm occurring in neonatal period and infancy. • Due to the paucity of the number of cases, the natural history of the disease is not fully understood and its preoperative diagnosis remains elusive. • Complete surgical excision is curative in all cases, with close observation and follow-up being recommended in Grade 2 and 3 immature teratomas. • The detection of immature component carries a prognostic implication and hence, extensive sampling is essential. • Postoperatively, the prognosis is excellent. • Long term follow-up with AFP and beta hCG depending on the type is the standard protocol to be adopted.

Mature Cystic Teratoma of the Ovary; Clinicopathological Evaluation of 143 Cases in our Series, 5 Years Experience

OBJECTIVE: Mature cystic teratoma, also known as a dermoid cyst, is the most common germ cell tumor of the ovary. In this retrospective study, it is aimed to evaluate clinicopathological findings of the patients who were operated on due to the preliminary diagnosis of dermoid cyst. STUDY DESIGN: Between May 2013 and May 2018, the findings of a total of 143 patients who were operated on with a preliminary diagnosis of dermoid cyst in our institution were analyzed retrospectively. In addition to demographic characteristics such as age, parity number and tumor size, lateralization, preoperative tumor markers, surgical procedure, presence of other pathological findings, and the rate of malignant transformation were analyzed. RESULTS: The median age of patients was 35.4 years and the ratio of nulliparous, primiparous, and multiparous patients was 46.9%, 25.2%, and 28%, respectively. The median tumor diameter was 6.7 cm, 50.3% of which were right-sided, 46.9% were left-sided and the remaining 2.8% were bilateral. Intraoperative torsion was found in 15 (10.5%) cases. The treatment consisted of 60.1% cystectomy by laparoscopy, 3.5% unilateral salpingo-oophorectomy by laparoscopy, 9.8% unilateral salpingo-oophorectomy by laparotomy, while the rate of 16.1% cases underwent hysterectomy with bilateral salpingo-oophorectomy by laparotomy, 10.5% of the cases, cysts were excised during cesarean section. As the final pathology report revealed, of 143 patients, immature teratoma was observed in five cases (3.5%), in one of them (0.7%) mixed germ cell tumor and in three of them (2.1%) borderline mucinous cancer, squamous cell carcinoma, and carcinoid tumor on mature cystic teratoma base were observed. CONCLUSION: Torsion is the most common complication with mature cystic teratoma. The size of the tumor is an important prognostic factor in terms of the malignant transformation of teratoma. Fertility sparing surgery with a minimally invasive surgical approach is available for fertility patients diagnosed with Mature cystic teratoma.

Malignant Germ cell Tumor of Ovary: An Unusual Combination and An Irony of Fate

Malignant mixed ovarian germ cell tumors are very rare malignant ovarian germ cell tumors, with a very high degree of malignancy. They are aggressive cancers affecting young adolescent girls. The commonest combination reported in literature is dysgerminoma and endodermal sinus tumors. Fertility sparing surgery followed by combination chemotherapy is the treatment of choice. The objectives of this case report are the advanced age of presentation, unusual histopathological combination, and the irony of infertility treatment. We report a rare case of 40-year-old woman with Malignant mixed germ cell tumor comprising of components of endodermal sinus tumor, immature teratoma, dysgerminoma and embryonal carcinoma. Patient was on hormonal therapy for the cause of infertility, presented with complains of lower abdominal pain, constipation and incomplete evacuation of bladder for 2months. Transvaginal ultrasonography and CT scan showed a large multicystic space occupying lesion with predominant solid component in midline abdominopelvic location. Tumor markers i.e., AFP, Prolactin and CA125 were raised. Total abdominal hysterectomy and bilateral salpingoophorectomy, pelvic and retroperitoneal node resection, peritonectomy and omentectomy was performed.