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Key Clinical Message: In conus medullaris, mature teratomas are rare. We report a case of a 40‐year‐old man who presented with urinary incontinence, low back pain, and muscle weakness. Magnetic resonance imaging revealed a mass in conus medullaris (T11–L1), further confirmed as a mature teratoma by pathological examination. We identified 63 cases of conus medullaris teratoma over the past two decades by systematically analyzing the case reports. Findings demonstrated that most cases were diagnosed in the fourth decade of life, with the majority of cases (57.6%) being male. Lower back pain, radiating pain in the extremities, hypoesthesia, and urinary dysfunction are the most common clinical presentations among patients with teratoma of conus medullaris. Mature teratoma is the dominant pathologic subtype of teratomas in this region, comprising more than 95% of cases. Our case highlights the importance of considering spinal teratoma as a differential diagnosis in patients presenting with urinary incontinence and lumbar pain. [ABSTRACT FROM AUTHOR]

Key Clinical Massage: Autoimmune hemolytic anemia (AIHA) is a rare disease that is divided into primary and secondary types. In patients with AIHA we should consider mediastinal tumors as a serious cause. If such teratoma was found surgical removal of it is in order. Autoimmune hemolytic anemia (AIHA) is a rare disease that is divided into primary and secondary types. The secondary type usually appears as a complication to some diseases such as chronic inflammatory diseases, systemic lupus erythematosus (SLE), and malignancies. A 26 years‐old female with a chief complaint of weakness, fatigue, and exertional dyspnea is diagnosed with AIHA. Initially, medical steroid treatment appeared to be successful and the anemia improved, however, by weaning steroid dosage the disease relapsed. Eventually, surgical resection of the mediastinal teratoma lead to the hemolysis being treated successfully. In patients with AIHA we should consider mediastinal tumors as a serious cause. If such teratoma was found surgical removal of it is in order. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Extragonadal parasitic dermoid cysts are rare. Diagnosis of such extragonadal parasitic teratoma is often done intraoperatively during surgical exploration of abdominal mass. [ABSTRACT FROM AUTHOR]

Background: Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children. Even with intensive multimodal treatment, the prognosis of many types is guarded, and treatment-related toxicity is significant. Recent advances in molecular diagnostics allowed the discovery of novel entities and inter-tumor subgroups, with opportunities for improved risk-stratification and treatment approaches. Summary: Medulloblastomas separate into four distinct subgroups with distinct clinicopathologic characteristics, and data from recent clinical trials for newly diagnosed medulloblastoma support subgroup-specific treatment approaches. Atypical teratoid rhabdoid tumor (ATRT), embryonal tumor with multilayered rosettes (ETMR), and pineoblastoma, as well as other rare embryonal tumors, can be distinguished from histologically similar tumors by virtue of characteristic molecular findings, with DNA methylation analysis providing a strong adjunct in indeterminate cases. Methylation analysis can also allow further subgrouping of ATRT and pineoblastoma. Despite the dire need to improve outcomes for patients with these tumors, their rarity and lack of actionable targets lead to a paucity of clinical trials and novel therapeutics. Key Messages: (1) Embryonal tumors can be accurately diagnosed with pediatric-specific sequencing techniques. (2) Medulloblastoma risk stratification and treatment decisions should take into account molecular subgroups. (3) There is a dire need for a novel collaborative clinical trial design to improve outcomes is rare pediatric embryonal tumors. [ABSTRACT FROM AUTHOR]

To report a rare adult case of cystic teratoma of the neck. A 51-year-old male patient who had a neck mass for 20 years, and the mass enlarged with the symptom of neck compression in two weeks. The patient was performed a surgical operation after a definite diagnosis through a CT scan, pathology, and other necessary examinations. It is rare to find an adult case of cystic teratoma of the neck. The physician should explore rare cases with detailed imaging studies for early diagnosis and prevention of potentially fatal complications such as malignant transformation, airway obstruction, and dysphagia. [ABSTRACT FROM AUTHOR]

Background: Sacrococcygeal teratomas (SCTs) are the most common congenital neoplasm and often require resection soon after birth. There are rare reports of cardiac arrest during surgery due to manipulation of the tumor triggering secondary necrosis and hyperkalemia. Case presentation: This case describes a very preterm infant with a SCT who develops spontaneous preoperative tumor lysis syndrome (TLS). The medical team utilized rasburicase and the patient underwent total gross resection at 40 h of life. Conclusions: We emphasize the importance of the early recognition and management of tumor lysis syndrome in SCT with rasburicase, aggressive management of hyperkalemia and consideration of early resection of SCTs even in the case of a very premature infant. [ABSTRACT FROM AUTHOR]

Fetus in fetu is a rare congenital anomaly with fewer than 200 cases reported worldwide. It is a part of spectrum of anomalous embryogenesis. We report a case of an 18-month old boy with abdominal distension. Ultrasonography, X-ray, and computed tomography (CT) scan revealed a retroperitoneal mass suggestive of fetus in fetu. with evidence of organogenesis and a vertebral column, which differentiates it from an organized teratoma. The mass was resected in toto. [ABSTRACT FROM AUTHOR]

Background: Mature and immature teratomas are differentiated based on tumor markers and calcification or fat distribution. However, no study has objectively quantified the differences in calcification and fat distributions between these tumors. This study aimed to evaluate the diagnostic potential of CT-based textural analysis in differentiating between mature and immature teratomas in patients aged < 20 years. Materials and methods: Thirty-two patients with pathologically proven mature cystic (n = 28) and immature teratomas (n = 4) underwent transabdominal ultrasound and/or abdominal and pelvic CT before surgery. The diagnostic performance of CT for assessing imaging features, including subjective manual measurement and objective textural analysis of fat and calcification distributions in the tumors, was evaluated by two experienced readers. The histopathological results were used as the gold standard. The Mann–Whitney U test was used for statistical analysis. Results: We evaluated 32 patients (mean age, 14.5 years; age range, 6–19 years). The mean maximum diameter and number of calcifications of immature teratomas were significantly larger than those of mature cystic teratomas (p < 0.01). The mean number of fats of immature teratomas was significantly larger than that of mature cystic teratomas (p < 0.01); however, no significant difference in the maximum diameter of fats was observed. CT textural features for calcification distribution in the tumors showed that mature cystic teratomas had higher homogeneity and energy than immature teratomas. However, immature teratomas showed higher correlation, entropy, and dissimilarity than mature cystic teratomas among features derived from the gray-level co-occurrence matrix (GLCM) (p < 0.05). No significant differences were observed in the CT features of fats derived from GLCM. Conclusion: Our results demonstrate that calcification distribution on CT is a potential diagnostic biomarker to discriminate mature from immature teratomas, thus enabling optimal therapeutic selection for patients aged < 20 years. [ABSTRACT FROM AUTHOR]

Introduction: Teratomas, tumors derived from all 3 fetal germ cell layers, are rarely located in the head and neck region and extremely rare in the Eustachian tube area. Case report: The authors present the case of a 2 years old child with chronic ear discharge. Referral diagnosis was tumor or cyst of the temporal bone. Intraoperatively, a solid, hairy tumor located inside the Eustachain tube, with extension into the middle ear space was discovered and removed. Histology revealed a mature teratoma. Review of the literature showed reports of rare similar cases of Eustachian tube teratomas, differentiating themfrom hairy polyps of the rhynopharynx. The imaging of our patient, the surgical techique for removal of the tumor and long term follow-up are detailed. Conclusions: Teratomas located inside the Eustachian tube are exceptional. Their removal should be carefully planned in view of their long term anatomical sequelae and in order to avoid long term postoperative morbidity. [ABSTRACT FROM AUTHOR]

Introduction: Ovarian germ cell tumours are a heterogeneous group of neoplasms derived from primitive germ cells of the embryonic gonad, either directly or indirectly. They can be classified as benign and malignant, with slow and rapid growth and spread, respectively. Benign ovarian germ cell tumours are common, while malignant tumours are rare and account for about 2.6% of all ovarian malignancies. They are more common in the second and third decades of life and typically present with abdominal mass, pain, and elevated serum tumour markers, which aid in primary diagnosis and follow-up. Aim: To analyse the distribution of germ cell tumours in the ovary in relation to age, parity, mode of presentation, biochemical markers, histomorphological patterns, and immunohistochemical markers. Materials and Methods: This cross-sectional study was conducted at Department of Pathology, Sree Balaji Medical College, Hospital and Research Institute, Chromepet, Chennai, Tamil Nadu, India. The study involved 86 ovarian specimens, of which 25 were germ cell tumours. Complete clinical history, radiological findings, and pre-operative laboratory test values were recorded. The ovarian specimens were carefully examined for gross appearances, fixed in 10% neutral buffered formalin for 24-48 hours, and subjected to histopathological processing, routine and special staining, and immunohistochemical study after observing the different morphological patterns of the ovarian specimens received. Results: The age range of presentation was between 14 years and 58 years. Seventeen patients were parous (14 benign and 3 malignant), and eight (5 benign and 3 malignant) were nulliparous. Abdominal mass and abdominal pain were the most common modes of presentation. Out of 25 germ cell tumours, 19 were benign cystic mature teratomas, 2 were immature teratomas, 1 was a yolk sac tumour, 2 were dysgerminomas, and 1 was a carcinoid tumour, with 6 being malignant and 19 being benign tumours. Among the 6 malignant ovarian tumours, 5 cases had raised serum tumour markers {cancer antigen-125 (CA-125), Alpha-Fetoprotein (AFP)} pre-operatively, and the levels reduced and became normal after surgery. Among the 2 cases of immature teratoma, one was Grade-II and the other was Grade-III. For one case with mixed tumour components, CD-30 and a-fetoprotein immunohistochemical markers were performed, showing negative and positive results, respectively. Conclusion: Among the histopathological subtypes, benign cystic teratomas were the most common ovarian germ cell tumours in this study. Both benign and malignant tumours presented with abdominal pain and abdominal mass. Most of the tumours were diagnosed between the ages of 21 and 40 years. In this study, a-fetoprotein immunohistochemical marker showed strong positivity, confirming a single tumour component. [ABSTRACT FROM AUTHOR]

Ovarian immature teratoma is a common ovarian germ cell malignant tumor that occurs in adolescent females. Surgical resection is the main treatment, and postoperative adjuvant chemotherapy is required if necessary. This case is an immature differentiated teratoma in the left ovary found during cesarean section. After 3 courses of chemotherapy with BEP regimen, the liver teratoma metastasis occurred. The pathological results after the second tumor resection suggest that mature teratoma is considered as growing teratoma syndrome (GTS). During the course of follow-up, the liver lesions were suspicious and reappeared, but gradually disappeared. Follow -up for 1 year showed no recurrence. During the treatment of ovarian immature teratoma, GTS is rare, has a special pathogenesis, and is easily misdiagnosed as recurrence or progression of ovarian immature teratoma. Therefore, it is necessary to understand GTS and pay attention to it in clinical practice. [ABSTRACT FROM AUTHOR]

Background: Spinal atypical teratoid rhabdoid tumor (AT/RT) is an extremely rare tumor and represents less than 2% of all AT/RTs. Methods: Available medical literature on spinal AT/RT in English was retrieved from PubMed and comprehensively reviewed. Clinical presentation, diagnosis, management, prognosis, and outcome in patients with spinal AT/RT have been elucidated by citing a case of extradural AT/RT of the cervicodorsal spine. Results: The age at presentation is usually less than 3 years. The most common site is the cervicodorsal spine. The most frequent tumor location is intradural extramedullary. A contrast-enhanced magnetic resonance imaging (MRI) of the entire neuraxis is the imaging modality of choice. The incidence of leptomeningeal dissemination is high (15–30%). Histopathological examination shows an admixture of primitive neuroectodermal, mesenchymal, and epithelial elements along with rhabdoid cells. Loss of SMARCB1/INI1 is considered pathognomonic of AT/RT. Maximal safe resection of tumor is the initial management of choice. Thereafter focal radiotherapy for localized tumor or craniospinal irradiation for leptomeningeal dissemination should be considered. Post-operative intensive polychemotherapy including intrathecal and high-dose chemotherapy (with autologous stem cell rescue) is usually considered to optimize survival. Typically, the time to recurrence and overall survival are less than 6 and 12 months, respectively. However, with judicious multimodality management long-term survivors are increasingly being recognized. The illustrative patient was a 18-month-old girl diagnosed with extradural AT/RT of the cervicodorsal spine (C3-D1), who was managed with maximal safe resection of tumor, multiagent chemotherapy (ICE-ifosfamide, carboplatin, etoposide) and focal RT to the tumor bed—50.4 Gy/28 fractions/5.5 weeks. At the last follow-up visit, 30 months after surgery, she had complete clinicoradiological response. Conclusion: Multimodal treatment comprising maximal safe resection of tumor, multiagent chemotherapy (ICE), and focal RT can lead to successful outcome in patients with localized spinal AT/RT, under the age of 3 years. [ABSTRACT FROM AUTHOR]

Atypical teratoid rhabdoid tumors (ATRT) are rare and aggressive embryonal tumors of central nervous system that typically affect children younger than 3 years of age. Given the generally poor outcomes of patients with ATRT and the significant toxicities associated with conventional multi-modal therapies, there is an urgent need for more novel approaches to treat ATRT, one such approach being immunotherapy. The recent rise of large-scale, multicenter interdisciplinary studies has delineated several molecular and genetic characteristics unique to ATRT. This review aims to describe currently available data on the tumor immune microenvironment of ATRT and its specific subtypes and to summarize the emerging clinical and preclinical results of immunotherapy-based approaches. It will also highlight the evolving knowledge of epigenetics on immunomodulation in this epigenetically influenced tumor, which may help guide the development of effective immunotherapeutic approaches in the future. [ABSTRACT FROM AUTHOR]

Malignancies rarely occur in somatic parts of mature cystic teratoma of the ovary. Squamous cell carcinoma is the most common form of cancer that can develop in mature cystic teratoma. Other less frequent malignancies include melanoma, sarcoma, carcinoid, and germ cell neoplasms. Only three cases have been reported as papillary thyroid carcinoma arising in struma ovarii. We present a unique case of a 31-year-old female patient who presented with a left ovarian cyst and underwent conservative surgical management in the form of cystectomy. Histopathological examination confirmed the diagnosis of a tall cell subtype of papillary thyroid carcinoma arising from a small focus of thyroid tissue in a mature cystic teratoma of the ovary. The patient was followed up for 60 months with an uneventful clinical course. For a better understanding of such rare cancers, collaborative retrospective studies on large databases with other medical centers are required. [ABSTRACT FROM AUTHOR]

The use of peripheral blood hematopoietic stem cells for bone marrow reconstitution after myeloablative therapy is well established in children with malignant disorders. However, the peripheral blood hematopoietic stem cells collection in very low‐body weight (≤10 kg) children remains a significant challenge because of technical and clinical issues. A male newborn affected by atypical teratoid rhabdoid tumor, diagnosed prenatally, received two cycles of chemotherapy following surgical resection. After an interdisciplinary discussion, it was decided to intensify the treatment with high‐dose chemotherapy followed by autologous stem cell transplantation. After 7 days of G‐CSF administration the patient underwent hematopoietic progenitor cells‐apheresis collection. The procedure was performed in the pediatric intensive care unit, using two central venous catheters and Spectra Optia device. The cell collection procedure was completed in 200 min, during which time 3.9 total blood volumes were processed. During apheresis we did not observe electrolyte alterations. No adverse events were recorded during or immediately following the cell collection procedure. Our report describes the feasibility of performing large volume leukapheresis without complications in an extremely low‐body weight patient weighing 4.5 kg using the Spectra Optia apheresis device. No catheter‐related problems occurred, and apheresis was completed without any adverse event. In conclusion, we believe that very low‐body weight pediatric patients need a multidisciplinary approach to manage central venous access, hemodynamic monitoring, cell collection, prevention of metabolic complications to improve safety, feasibility, and efficiency of stem cell collection procedures. [ABSTRACT FROM AUTHOR]

Epilepsy is a common neurological disease. Systemic tumors are associated with an increased risk of epileptic events. Paraneoplastic encephalitis related to gonadal teratoma is frequently accompanied by seizures and life-threatening status epilepticus (SE). However, the risk of epilepsy in gonadal teratoma has not been studied. This study aims to investigate the relationship between epileptic events and gonadal teratoma. This retrospective cohort study used the Korean National Health Insurance (KNHI) database. The study population was divided into two study arms (ovarian teratoma vs. control and testicular teratoma vs. control) with 1:2 age and gender-matched control groups without a history of gonadal teratoma or other malignancy. Participants with other malignancies, neurologic disorders, and metastatic brain lesions were excluded. We observed the occurrence of epileptic events during the observation period (2013–2018) and investigated the risk of epileptic events in each gonadal teratoma group compared to controls. In addition, the influence of malignancy and tumor removal was investigated. The final analysis included 94,203 women with ovarian teratoma, 2314 men with testicular teratoma, and controls. Ovarian teratoma is associated with a higher risk of epilepsy without SE (HR, 1.244; 95% CI 1.112–1.391) and epilepsy with SE (HR, 2.012; 95% CI 1.220–3.318) compared to the control group. The risk of epilepsy without SE was higher in malignant ovarian teratoma (HR, 1.661; 95% CI 1.358–2.033) than in benign (HR, 1.172; 95% CI 1.037–1.324). Testicular teratoma did not show significant relations with epileptic events. The risk of epileptic events showed a tendency to decrease after removing the ovarian teratoma. This study found that ovarian teratoma is associated with a higher risk of epileptic events, especially in malignant tumors, whereas testicular teratoma did not show significant differences in epileptic events compared to the control group. This study adds to the current understanding of the association between gonadal teratoma and epileptic events. [ABSTRACT FROM AUTHOR]

In this case report, we describe a patient who developed metastatic liver cancer of unknown primary origin one year following the surgical removal of a retroperitoneal adenocarcinoma. The retroperitoneal adenocarcinoma is considered a malignant transformation of teratoma (MTT), given the patient's distant history of testicular tumor excised 25 years prior and treated with chemotherapy. Despite no primary tumor being identified, the leading primary hypothesis is that the liver metastasis stemmed from the resected retroperitoneal adenocarcinoma from one year prior. We theorize that the patient's cisplatinbased chemotherapy 25 years ago may have triggered the MTT, as documented in the existing literature. Using TEMPUS gene testing on both the retroperitoneal adenocarcinoma and the recently discovered liver metastasis, we identified several genes with variants of unknown significance (VUS) that could potentially be linked to cisplatin chemotherapy resistance. While we cannot conclude that this patient definitively underwent MTT, it remains the most plausible explanation. Future research should investigate both the validity of the genes we have uncovered with respect to cisplatin resistance, as well as other genes associated with cisplatin resistance to further understand the pathogenesis of cisplatin resistance for better prediction of treatment response. As the world of medicine shifts towards individualized therapies and precision medicine, reporting and analyzing genetic mutations derived from tumors remains imperative. Our case report aims to contribute to the growing database of defined mutations and underscores the immense potential of genetic analysis in directing personalized treatment options. [ABSTRACT FROM AUTHOR]

Since previous results demonstrated that miR-371a-3p parallels tumour differentiation, culminating with undetected levels in mature teratoma, our findings of a similar range of miR-371a-3p levels in CTT provide additional support to the hypothesis of an advanced maturation step towards teratoma for this entity. Cystic trophoblastic tumour (CTT) is an infrequent finding, first described in 2004.[1] CTT is most likely underdiagnosed, since it is often focal and mixed within teratoma components, most frequently found in the setting of postchemotherapy retroperitoneal lymph-node dissections (pcRPLND),[1] but also, albeit rarely, in metastatic chemo-naïve patients,[2] as part of primary testicular germ cell tumours (TGCT),[3] and recently in the setting of a primary central nervous system germ cell tumour.[4] Its pathogenesis is poorly understood, and two theories have been proposed: regression of choriocarcinoma under chemotherapy, explaining the event in pcRPLND cases, and spontaneous regression, justifying the event in untreated patients. MiR-371a-3p in cystic trophoblastic tumour of the testis: supporting a maturation phenomenon towards teratoma. [Extracted from the article]

OBJECTIVE The aim of this study was to describe the clinical, histological, and pathological characteristics in children with GCT and assess their outcome. METHODS This was a retrospective study conducted for children with germ cell tumors who were treated at the Pediatric Hematology-Oncology Center/Medical City for 3 years from January 1st, 2016 to December 31st, 2018, and their follow-up till June 30th, 2021. Thirty-four cases were identified. The total information was collected by using data from the medical record at our oncology registry and from registered archives in outpatient records. Follow-up of patients over an average period of more than 3 years was carried out either in person at the outpatient clinic or by phone call. The Statistical Package for the Social Sciences version 23 was used for analysis. RESULTS The initial age of presentation ranged from birth until 14 years. More than sixty percent presented below 4 years of age, and then after this, the age at diagnosis was near equally distributed in both 5-9 years and 10-14 years; 6 and 5 patients (17.6% and 14.7%, respectively). Females predominate in all age groups (76.5%) versus (23.5%) males with a male-to-female ratio of 1: 3.2. The main presenting symptom was fever in 13 (38.3%) patients. There were 11 (32.4%) patients who had testicular swelling, and 11 (32.4%) patients with abdominal distension. The duration of symptoms is more than 6 weeks in 19 (55.9%) cases. The common histological type is a yolk sac tumor in 18 (52.9%) patients. No patients presented with stage I, 9 patients with stage II, 6 (17.7%) patients with stage III, and 9 (26.5%) patients with stage IV. The duration of symptoms is more than 6 weeks in 19 (55.9%) cases. There were four benign cases. CONCLUSION The delayed diagnosis is one of the main obstacles in the management of this group. Risk assessment and staging were not conclusive in some cases due to the gaps between surgeons and oncologists. The upstaging gives better outcome in the overall survival compared to other studies at the same place. [ABSTRACT FROM AUTHOR]

Introduction: The aim of the study is to evaluate the incidence, frequency and histopathological pattern of the ovarian teratomas. Germ cell tumours represent early 25-30% of the ovarian neoplasms and it varies in respect to their clinical presentation and histopathology. Materials & methods: It is a retrospective & prospective study from the department of pathology at the tertiary health centre. From august 2012 to July 2015, retrieved from the recorded files & analysed. The tissues were fixed in 10% formalin and slides were made with routine hematoxylin & eosin stain. Results: Out of 54 cases, mature cystuc teratoma consitituted 51, 2 struma ovarii and immature teratomas of the ovary. Out of the 54 cases of teratoma, the most common histological type is the most common histological type is mature cystic teratoma. Conclusion: Benign tumours are common than malignant tumours. Mature cystic teratoma is common than immature teratoma. Most of them presented with abdominal pain, even though other presentable symptoms were abdominal lump, irregular bleeding & loss of weight. The most common age group affected was 20-30. Left side was affected more than left side. [ABSTRACT FROM AUTHOR]

Mediastinal masses can be challenging to the surgical team and anesthetic considerations vary according to the location, pathology, surgical approach, and patient comorbidities. We report the case of a 21 cm symptomatic intrathoracic teratoma in a postpartum patient with a history of poliomyelitis. Significant challenges were presented for anesthetic induction, potential extracorporeal membrane oxygenation, and the use of neuraxial pain techniques and neuromuscular blockade. This case report demonstrates techniques to safely manage a patient with a large symptomatic mediastinal mass and potential neuromuscular disease. [ABSTRACT FROM AUTHOR]

Objective: To investigate the relationship between mast cells (MCs) and different central nervous system (CNS) tumors. Methods: It was a comparative immunohistochemical study to investigate the presence, number, and location of MCs in pilocytic astrocytomas, glioblastoma, medulloblastoma, ependymoma, meningioma, and immature and mature teratomas. Means, medians, and standard deviation were obtained. Comparation in the number of MCs in the different tumors was carried out using the Mann-Whitney test (p < 0.05). Results: There was a significant difference in the number of MCs between pilocytic astrocytoma (... = 0), and mature teratoma (... = 65), in relation to the other neoplasms (... = 3-11.4). MCs were identified in meningeal lining in meningiomas, in the perivascular space in ependymomas and in the tumor stroma and perivascular space in the rest of the tumors analyzed. Conclusions: MCs appear to play different roles in CNS tumors. Its absence in pilocytic astrocytomas and its presence in glioblastomas suggests a role in the latter, probably related to angiogenesis. The maximum number of MCs was observed in mature teratomas, specifically in relation to stratified squamous epithelium, possibly in relation to trophic factors that contribute to epithelial renewal and maintenance. These findings call for future research to determine if MCs can be a therapeutic target or are important as a prognostic factor. [ABSTRACT FROM AUTHOR]

Background: Mature teratoma is a benign neoplasm, mostly composed of welldifferentiated derivations of almost two or three germ cell layers, while immature teratoma is a malignant neoplasm composed of immature neural and embryonic tissue. Immature teratoma in the context of ovarian endometrioma has not been reported yet. Case Presentation: A 34-year-old woman with primary infertility is reported in this study who suffered from immature teratoma associated with ovarian endometrioma. After several rounds of fertility treatment, the patient was referred for severe abdominal pain and underwent emergency surgery for the rupture of ovarian endometrioma. To preserve the ovary, the cyst was not resected in areas attached to the ovary. Some months later, the patient noticed a progressive abdominal enlargement. The sonographic evaluation revealed multiple solid-cystic lobulated masses on the abdominal wall and throughout the pelvic cavity. The histologic diagnosis was consistent with immature teratoma. The patient was treated with high-dose neoadjuvant chemotherapy and fertility-sparing surgery (FSS). The histologic evaluation of the extracted masses revealed teratoma maturation. Conclusion: This study reveals the importance of complete removal of endometrioma and highlights the role of neoadjuvant chemotherapy in fertility-sparing surgery and potentiating teratoma maturation. [ABSTRACT FROM AUTHOR]

Collision tumor (CT) is the coexistence of two adjacent but histologically distinct and nonadmixed tumor in the same tissue. We report a rare case of ovarian CT: mature teratoma (MT) with thecoma, as well as provide an extensive review of 47 published cases. Ovarian CTs occur in both pre- and postmenopausal women with nonspecific clinic-radiologic symptoms. Prognosis is dependent on the most aggressive component. All the cases, including the index case, were diagnosed post-operatively. Adequate sampling by pathologists and retrospective review and documentation of image features of histologically diagnosed cases by radiologists will enhance both proper and preoperative diagnosis of this lesion. This is the most extensive review on ovarian CTs to the best of our knowledge. [ABSTRACT FROM AUTHOR]

Teratoma is a common germ cell tumor that develops from germ cells with multiple differentiation potentials. It can be seen in multiple tissues and organs, and the gonad teratoma is the most common. It is rare to develop outside the gonad, especially in the rectovaginal septum. There are few reports of postoperative recurrence of gonad teratoma in literatures. We reported a case of recurrent mature teratoma of rectovaginal septum treated in the surgery department of our hospital. The clinical data were retrospectively analyzed, including clinical symptoms, signs and related auxiliary examinations. In addition, the domestic and foreign literatures on rectovaginal septum teratoma were summarized from the aspects of clinical characteristics, diagnosis, treatment and prognosis. The case report and literature review provide us a practical reference for the future clinical studies on extradadal teratoma. [ABSTRACT FROM AUTHOR]

Spinal intradural extramedullary teratomas are rare tumors arising from more than one germ cell layers and are more common in children than in older age groups. Teratomas are more commonly seen in intracranial compartment and are extremely rare in the spine. They are difficult to diagnose pre-operatively and the definitive diagnosis is usually done by histopathologic evaluation. Intraspinal teratomas should be considered in the differential diagnosis of any intradural extramedullary tumor of the spine. We reviewed the literature and reported a rare case of intradural extramedullary teratoma in an adolescent located in dorsolumbar spine without any history of prior spinal procedure. [ABSTRACT FROM AUTHOR]

Background & Objective: Brain tumors are the most frequent solid tumors in children. High-grade tumors are more challenging in diagnosis. Atypical teratoid rhabdoid tumor (ATRT) may be mistaken for other high-grade brain tumors. Molecular genetic analysis of ATRT has shown deletion and mutation in the hSNF5/INI1 gene in most of the cases. The INI1 protein expression can be helpful for the accurate diagnosis. Methods: In this study, immunohistochemical staining (IHC) using INI1 antibody was performed to determine the possibility of ATRT misdiagnosis. Totally, 147 tumors including 6 ATRTs, 81 medulloblastomas, and 60 other CNS tumors were examined in children between 0 and 17 years old. Results: No nuclear staining was found in the six ATRT cases, while most of other CNS tumors demonstrated nuclear staining. Five cases were previously diagnosed with medulloblastoma, primitive neuroectodermal tumor (PNET), and anaplastic oligodendroglioma, while the diagnoses were changed to ATRT based on the reevaluation of the H&E slides and INI1 study. Additionally, two cases were recurrent tumors whose features were consistent with those of ATRT. The INI1 immunostaining was negative in these cases. Conclusion: INI1 was very helpful in distinguishing ATRT from its mimickers in challenging cases. All known ATRT cases in this study were immunonegative for INI1. Thus, INI1 is recommended to be used in the initial IHC panel for the high-grade brain tumors, especially in children under the age of three years, so that they can benefit from intensified therapeutic regimens. [ABSTRACT FROM AUTHOR]

Thanks to a conscientious work-up in the general practitioner's office and referral to goal-directed diagnostics, the benign thoracic tumor could be submitted to curative resection. Although the radiological and laboratory examinations made the benign entity of a mature teratoma in a young, postpubertal male highly probable, the clinic is usually nonspecific and makes classification difficult, so that a histo-pathological work-up and resection were mandatory in this case in order to obtain diagnostic certainty about the dignity and to allow for timely therapy according to the guidelines. [ABSTRACT FROM AUTHOR]

A 36-year-old pregnant woman with a prior history of depression and recent gunshot wounds presented with sudden deterioration in her mental status. Clinical examination revealed psychosis, hallucinations, and lack of orientation, with an otherwise normal neurological and cardiorespiratory examination. Computed tomographic scan of her head was normal, and she was diagnosed with acute psychosis and excited delirium. She did not respond to supraphysiologic dosages of antipsychotic therapy and needed physical restraints for combativeness and agitation. Her cerebrospinal fluid analysis was negative for an infectious etiology, but was positive for anti-N-methyl-D-aspartate receptor encephalitis antibodies. Abdominal imaging revealed a right-sided ovarian cyst. Subsequently she underwent right-sided oophorectomy. Postoperatively the patient continued to have intermittent episodes of agitation requiring antipsychotic medications. Later, she was safely transitioned to home care with family support. [ABSTRACT FROM AUTHOR]

Most abdominal masses in the pediatric population derive from the ovaries. Ovarian masses can occur in all ages, although their incidence, clinical presentation and histological distribution vary among different age groups. Children and adolescents may develop non-neoplastic ovarian lesions, such as functional cysts, endometrioma, torsion, abscess and lymphangioma as well as neoplasms, which are divided into germ cell, epithelial, sex-cord stromal and miscellaneous tumors. Germ cell tumors account for the majority of ovarian neoplasms in the pediatric population, while adults most frequently present with epithelial tumors. Mature teratoma is the most common ovarian neoplasm in children and adolescents, whereas dysgerminoma constitutes the most frequent ovarian malignancy. Clinical manifestations generally include abdominal pain, palpable mass, nausea/vomiting and endocrine alterations, such as menstrual abnormalities, precocious puberty and virilization. During the investigation of pediatric ovarian masses, the most important objective is to evaluate the likelihood of malignancy since the management of benign and malignant lesions is fundamentally different. The presence of solid components, large size and heterogenous appearance on transabdominal ultrasonography, magnetic resonance imaging and computed tomography indicate an increased risk of malignancy. Useful tumor markers that raise concern for ovarian cancer in children and adolescents include alpha-fetoprotein, lactate dehydrogenase, beta subunit of human chorionic gonadotropin, cancer antigen 125 and inhibin. However, their serum levels can neither confirm nor exclude malignancy. Management of pediatric ovarian masses needs to be curative and, when feasible, function-preserving and minimally invasive. Children and adolescents with an ovarian mass should be treated in specialized centers to avoid unnecessary oophorectomies and ensure the best possible outcome. [ABSTRACT FROM AUTHOR]

We present a case of ovarian torsion due to a nine-centimeter cystic ovarian teratoma diagnosed on transabdominal point-of-care ultrasound (POCUS). A 29-year-old female presented with abrupt onset of 10/10 left lower quadrant pain for one hour. She had a known left-sided ovarian cyst. Patient was normotensive, not tachycardic, and afebrile. Bimanual exam showed a seven-centimeter left, adnexal mass. POCUS revealed a nine-centimeter cystic structure in the left adnexa with a three-centimeter echogenic focus, consistent with a dermoid plug. A radiology-performed transvaginal ultrasound confirmed the left sided mass with absent blood flow. Emergent diagnostic laparoscopy showed the cyst was torsed twice and was found to contain adipose tissue and hair on pathologic evaluation consistent with a benign cystic teratoma. The patient did well postoperatively and was discharged the next day. Ovarian torsion is a gynecologic surgical emergency. The characteristic appearance of a cyst with “dermoid plug” in the appropriate clinical setting can lead to earlier diagnosis and treatment.

Despite their rapidly-expanding therapeutic potential, human pluripotent stem cell (hPSC)-derived cell therapies continue to have serious safety risks. Transplantation of hPSC-derived cell populations into preclinical models has generated teratomas (tumors arising from undifferentiated hPSCs), unwanted tissues, and other types of adverse events. Mitigating these risks is important to increase the safety of such therapies. Here we use genome editing to engineer a general platform to improve the safety of future hPSC-derived cell transplantation therapies. Specifically, we develop hPSC lines bearing two drug-inducible safeguards, which have distinct functionalities and address separate safety concerns. In vitro administration of one small molecule depletes undifferentiated hPSCs >106-fold, thus preventing teratoma formation in vivo. Administration of a second small molecule kills all hPSC-derived cell-types, thus providing an option to eliminate the entire hPSC-derived cell product in vivo if adverse events arise. These orthogonal safety switches address major safety concerns with pluripotent cell-derived therapies.

Immature teratoma is a subtype of malignant germ cell tumor of the ovary that occurs most commonly in the first three decades of life, frequently with bilateral ovarian disease. Despite being the second most common malignant germ cell tumor of the ovary, little is known about its genetic underpinnings. Here we performed multiregion whole-exome sequencing to interrogate the genetic zygosity, clonal relationship, DNA copy number, and mutational status of 52 pathologically distinct tumor components from ten females with ovarian immature teratomas, with bilateral tumors present in five cases and peritoneal dissemination in seven cases. We found that ovarian immature teratomas are genetically characterized by 2N near-diploid genomes with extensive loss of heterozygosity and an absence of genes harboring recurrent somatic mutations or known oncogenic variants. All components within a single ovarian tumor (immature teratoma, mature teratoma with different histologic patterns of differentiation, and yolk sac tumor) were found to harbor an identical pattern of loss of heterozygosity across the genome, indicating a shared clonal origin. In contrast, the four analyzed bilateral teratomas showed distinct patterns of zygosity changes in the right versus left sided tumors, indicating independent clonal origins. All disseminated teratoma components within the peritoneum (including gliomatosis peritonei) shared a clonal pattern of loss of heterozygosity with either the right or left primary ovarian tumor. The observed genomic loss of heterozygosity patterns indicate that diverse meiotic errors contribute to the formation of ovarian immature teratomas, with 11 out of the 15 genetically distinct clones determined to result from nondisjunction errors during meiosis I or II. Overall, these findings suggest that copy-neutral loss of heterozygosity resulting from meiotic abnormalities may be sufficient to generate ovarian immature teratomas from germ cells.

PurposeAtypical teratoid/rhabdoid tumor (AT/RT) is an aggressive, early-childhood brain tumor without standard effective treatment. To our knowledge, we conducted the first AT/RT-specific cooperative group trial, ACNS0333, to examine the efficacy and safety of intensive postoperative chemotherapy and focal radiation to treat AT/RT.Patients and methodsPatients from birth to 22 years of age with AT/RT were eligible. After surgery, they received 2 courses of multiagent chemotherapy, followed by 3 courses of high-dose chemotherapy with peripheral blood stem cell rescue and involved-field radiation therapy. Timing of radiation was based on patient age and disease location and extent. Central testing of tumor and blood for SMARCB1 status was mandated. Tumor molecular subclassification was performed retrospectively. The primary analysis was event-free survival (EFS) for patients < 36 months of age compared with a cooperative groups' historical cohort. Although accrual was based on the therapeutic question, potential prognostic factors, including age, tumor location, M stage, surgical resection, order of therapy, germline status, and molecular subtype, were explored.ResultsOf 65 evaluable patients, 54 were < 36 months of age. ACNS0333 therapy significantly reduced the risk of EFS events in patients < 36 months of age compared with the historical cohort (P < .0005; hazard rate, 0.43; 95% CI, 0.28 to 0.66). Four-year EFS and overall survival for the entire cohort were 37% (95% CI, 25% to 49%) and 43% (95% CI, 31% to 55%), respectively. Timing of radiation did not affect survival, and 91% of relapses occurred by 2 years from enrollment. Treatment-related deaths occurred in 4 patients.ConclusionThe ACNS0333 regimen dramatically improved survival compared with historical therapies for patients with AT/RT. Clinical characteristics and molecular subgrouping suggest prognostic differences. ACNS0333 results lay a foundation on which to build future studies and incorporate testing of new therapeutic agents.

Objetivos: reportar el caso de una gestante con diagnóstico de feto con teratoma nasofaríngeo inmaduro y realizar una revisión describiendo el pronóstico de esta patología. Materiales y métodos: se reporta el caso de una gestante de 27 años, atendida en la Unidad de Ginecología y Obstétrica de un centro de referencia en Bogotá (Colombia), por hallazgo durante el control prenatal de un feto con evidencia de polihidramnios secundario a teratoma nasofaríngeo. Se realizó una búsqueda bibliográfica en las bases de datos Medline vía PubMed, Scopus, SciELO y ScienceDirect, restringiendo tipo de idioma (inglés y español) y por fecha de publicación (enero de 2001 a enero de 2021). Se incluyeron reportes de caso y series de casos que abarcaran el pronóstico de esta patología. Resultados: la búsqueda recuperó 168 títulos, de los cuales 55 cumplieron con los criterios de inclusión. En total se reportó el resultado perinatal de 58 fetos con diagnóstico de teratoma nasofaríngeo inmaduro detectado en etapa prenatal. En los casos identificados, la mortalidad perinatal fue del 25,4 % y el porcentaje de óbito fue cercano al 3,6 %. Conclusiones: el teratoma nasofaríngeo inmaduro es una patología poco frecuente. La literatura disponible sugiere que el pronóstico fetal depende del grado de compromiso de las estructuras intracraneales y de la posibilidad de resección de la lesión. Se requieren más estudios que evalúen el pronóstico de los fetos con teratoma nasofaríngeo inmaduro.

Background: Mature teratoma is a germinal cell tumor that is commonly found in ovaries and testis. Mature teratoma in the lung region is a rare case. According to the literature there are only 67 cases worldwide starting from 1939 to 2007. There were one cases in Dr. Soetomo general hospital during January 2013 – December 2017. Objective: Mature teratoma in the lung region is a rare case. Presenting in a case report would be beneficial for pathology database Case Presentation: A 19 years old man came to hospital with shortness of breath within 3 months ago. It was followed with hemoptysis, trichoptysis and coughing out tooth. CT Scan examination showed an irregular walled cavity with semisolid component and calcification along with a description of "air cresent sign". In thoracotomy tumor excision obtained multilobulated mass size 8.5x6,8x5,6 cm, yellowish-gray white color, and firm consistency. There were also hair and tooth components. The microscopic representation shows pieces of lung tissue with tumor growth coated with squamous epithel with a stroma containing adnexal skin glands, fatty tissue, cartilage tissue, and serous glands (asinar). Discussion: Mature teratoma of the lung is one of the rare tumors, with symptoms of chest pain, hemoptysis, and trichoptysis. Mature teratoma of the lung is generally cystic and multiloculated, but can also be solid, encapsulated. On microscopic examination, shows a picture of various components of ectoderm, mesoderm and endoderm. Conclusion: Mature teratoma of the lung is a rare tumor. Diagnosis was based from clinical findings, radiologic examination, and confirmed with histopathology and immunohistochemistry.

Foetus-in-fetu (FIF) is a condition where one of the twin foetuses is found developing inside the other. Differentiating this condition from fetiform teratoma (FT), in which the teratoma becomes highly differentiated to take a foetus-like appearance, often becomes arduous. Unfamiliarity with the situation and the presence of bone and hair within such lesions can potentially mislead the surgeon to diagnose it as a dermoid cyst erroneously. Suboptimal surgery can complicate things further when it comes to redoing operations and the final histopathological reporting. We report this case of a partially removed cystic retroperitoneal mass in a 14-year-old male, posing a significant surgical challenge due to dense adhesions to the vital retroperitoneal structures, such as common iliac vessels and ureter. We also faced similar diagnostic dilemmas during histopathology due to the tissues removed during the previous surgery. Based on gross and microscopic features, the possibility of a FIF or FT was rendered.

Teratomas are germ cell tumors occurring usually in gonadal organs. They are neoplasms composed of one or more of the three germ layers. Extragonadal teratomas are uncommonly reported in the literature. The liver is an extremely rare site for teratoma, constituting < 1% of all teratomas. The majority of the liver teratomas are found in the pediatric population with only a dozen cases reported in adults to the best of our knowledge. We present a case of a 27-year-old male with a history of abdominal pain of 5 months duration. CT scan revealed liver mass suggesting teratoma. The patient underwent cholecystectomy and segmental liver resection. Histopathology revealed mature cystic teratoma. Complete resection remains the best treatment option. Teratomas of the liver are extremely rare, constituting < 1% of all teratomas. An extensive review of the literature yielded < 50 cases of primary hepatic teratomas with only a dozen cases in adults and only two cases in males.

Fetus in fetu was first identified in the early 19th century by Meckel. It is a rare congenital anomaly which occurs from anomalous embryogenesis in which a malformed parasitic twin is found within the body of the normally developed host. In most cases, the parasitic twin is anencephalic and usually contains a vertebral column and budding limbs. The upper limbs are less developed than lower limbs, and are usually located in the abdomen of the autosite. Diagnosis is often made preoperatively with ultrasonography, plain radiography, computed tomography, or magnetic resonance imaging. Histopathological findings confirm the diagnosis and the recommended treatment is complete surgical excision of mass. We report a case of 15-month-old baby boy of Nepalese origin referred to our hospital with mass in abdomen diagnosed during neonatal period.

Mature mediastinal teratomas are infrequent, slow growing, and often asymptomatic. Traditionally, they present in the fourth decade or earlier and are found in the anterior mediastinum. These teratomas are often found incidentally on imaging, and surgical resection can be a challenge due to the size and location of the tumor; however, complete surgical excision is usually curative. Our case represents an infrequent benign giant (25 cm × 18 cm × 12 cm) mature intrathoracic mediastinal teratoma with uncommon clinical presentation of congestive heart failure and cardiac cachexia. The rare location of the tumor in the mid posterior mediastinum, very large teratoma, clinical presentation with right ventricular failure and cardiac cachexia, preoperative diagnostic dilemma, perioperative surgical challenge, and management marks this case unique.

Abstract Objectives To investigate the utility of a novel serum miRNA biomarker panel to distinguish teratoma from nonmalignant necrotic/fibrotic tissues or nonviable tumours in patients with NSGCT undergoing post‐chemotherapy consolidation surgery. Patients and methods We prospectively collected pre‐surgical serum samples from 22 consecutive testicular NSGCT patients with residual NSGCT after chemotherapy undergoing post‐chemotherapy consolidation surgery. We measured serum miRNA expression of four microRNAs (miRNA‐375, miRNA‐200a‐3p, miRNA‐200a‐5p and miRNA‐200b‐3p) and compared with pathologic findings at time of surgery. Receiver operating characteristic (ROC) curves were performed to assess the ability of these miRNA to differentiate between teratoma and necrosis or viable malignancy. Results Twenty‐two patients with NSGCT were split into two groups based on pathology at time of post‐chemotherapy consolidation surgery (teratoma group vs. necrosis/fibrosis/viable tumour group, i.e., NFVT). Patients with teratoma were older at diagnosis compared with those patients with NFVT (median age 28.7 vs. 23.9). Patients with NFVT were more likely to have embryonal carcinoma in their primary tumour (81.8% vs. 27.3%; p = 0.01). The majority of patients in both groups were stage III (63.6% vs. 72.7%). In this analysis, none of the miRNAs had good sensitivity or specificity to predict teratoma. There was no significant association between the expression levels of the miRNAs and the presence of teratoma. There was no statistically significant correlation between any of the miRNAs and teratoma size. Conclusion This novel miRNA panel (miRNA‐375, miRNA‐200a‐3p, miRNA‐200a‐5p and miRNA‐200b‐3p) did not distinguish teratoma from nonmalignant necrotic/fibrotic tissues or nonviable tumours in patients with NSGCT undergoing post‐chemotherapy consolidation surgery.