Teratomas In Childhood Research Articles

Oropharyngeal teratoma in a newborn: A case report

Background: Facial teratomas are rare congenital tumors in the craniocervical region. They constitute only 2-5% of all childhood teratomas. Case Presentation: This report presents a case of an oral teratoma in an infant, attached to the palate and extending sublingually on the right side, along with a cleft palate. The patient underwent successful surgical excision on the 17th day of life performed by a team of pediatric and maxillofacial surgeons. Conclusion: The case emphasizes the importance of interdisciplinary coordination for the management of facial teratomas, involving maxillofacial and pediatric surgeons, neonatologists, anesthesiologists, and obstetrics specialists.

Critical elements of pediatrics sacrococcygeal germ cell tumor surgery

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1,2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function. Herein we outline the critical elements of tumor resection and management of sacrococcygeal germ cell tumors with a focus on the technical aspects of this tumor across a range of presentations.

Case Report: Primary Liver Mature Teratoma in Childhood

We present the case of a male 2-year-old patient who underwent surgery for a 13-cm-long liver teratoma that caused elongation of the common hepatic duct and the portal vein. The boy had an abdominal mass and abdominal distension with pain of mild intensity. Abdominal computed tomography and abdominal nuclear magnetic resonance reported a predominantly cystic expansive liver lesion with some internal solid components. The case was evaluated and surgical intervention was decided, performing a right hepatectomy and biliodigestive derivation due to elongation and compromise of the common bile duct. Primary mature liver teratoma is a rare tumor in population with a higher incidence in children, so it should be suspected in all patients with solid liver tumors that do not present with high AFP values.

Incidence of metachronous contralateral mature ovarian teratoma in childhood and adolescence—a single-centre 20-year experience

BackgroundDespite complete resection of mature ovarian teratoma, there remains a risk of metachronous contralateral disease with implications for further surgery and fertility. Current estimates of this risk are wide and practice regarding surveillance varies. We aimed to identify the incidence of metachronous contralateral disease in girls presenting with unilateral mature ovarian teratoma and to describe current follow-up.MethodsRetrospective case note review was performed for all girls (< 17 years) undergoing surgery for histologically confirmed mature ovarian teratoma between 1998 and 2018. Data concerning initial hospital episode, follow-up, and further intervention were collected.ResultsForty-five girls were identified with a median age of 10 years (range 1–16). Salpingo-oophorectomy (47%) and oophorectomy (36%) were the commonest operations. Median follow-up was 2 years (range 0–16 years) with surveillance ultrasonography performed in 49%. One case of metachronous contralateral teratoma (2%) was detected at 1 year, requiring oophorectomy and ovarian tissue cryopreservation.ConclusionsThis series has demonstrated a lower incidence of metachronous contralateral mature ovarian teratoma compared to previously published data. Postoperative surveillance is variable, and the true natural history of this condition remains incompletely understood. Prospective, multicentre investigation at national or international level is required to improve the evidence upon which to base safe standards of care.

Malignant glioma arising from orbital teratoma in childhood

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小児奇形腫の検討 : 特に未熟,悪性奇形腫の診断と治療について

小児奇形腫の検討 : 特に未熟,悪性奇形腫の診断と治療について

Hepatic splenosis mimicking liver metastases in a patient with history of childhood immature teratoma.

BackgroundHepatic splenosis is rare condition, preceded by splenectomy or spleen trauma, the term refers to nodular implantation of normal splenic tissue in the liver. In patients with history of malignancy in particular, it can be mistaken for metastases and can lead to unnecessary diagnostic procedures or inappropriate treatment.Case reportTwenty-two-year old male was treated for immature teratoma linked to undescended right testicle after birth. On regular follow-up examinations no signs of disease relapse or long-term consequences were observed. He was presented with incidental finding of mature cystic teratoma after elective surgery for what appeared to be left-sided inguinal hernia. The tumour was most likely a metastasis of childhood teratoma. Origin within remaining left testicle was not found. Upon further imaging diagnostics, several intrahepatic lesions were revealed. Based on radiologic appearance they were suspicious to be metastases. The patient underwent two ultrasound guided fine-needle aspiration biopsies. Cytologic diagnosis was inconclusive. Histology of laparoscopically obtained tissue disclosed presence of normal splenic tissue and led to diagnosis of hepatic splenosis.ConclusionsThough hepatic splenosis is rare, it needs to be included in differential diagnosis of nodular hepatic lesions. Accurate interpretation of those lesions is crucial for appropriate management of the patient. If diagnosis eludes after cytologic diagnostics alone, laparoscopic excision of nodular lesion is warranted before considering more extensive liver resection.

Large suprasellar mass: answer

Teratomas are rare intracranial neoplasms that make up approximately 0.4% of all primary intracranial tumours [1]. They can be divided into extra-axial and intra-axial teratomas [2]. Extra-axial tumours usually arise in the suprasellar or pineal regions and typically present in childhood with a recent case series demonstrating a mean age of 14.5 years [3]. In contrast, intra-axial teratomas are the most common intracranial foetal tumour and present in the antenatal period with a mean gestational age at diagnosis of 27 weeks [4,5]. Intra-axial teratomas are more commonly supratentorial with a predilection for the frontal lobes [5]. Regardless of location, extragonadal teratomas are believed to develop from ectopic, totipotent primordial germ cells that have either been misplaced or undergone aberrant migration during early embryogenesis [2,6]. Because of this cellular origin, teratomas can contain diverse mixed tissues with up to 90% of childhood teratomas containing elements derived from all three embryonic layers (ectodermal, mesodermal and endodermal) [2]. This is in contrast to epidermoid and dermoid cysts which are both derived from ectodermal origin only [2]. Importantly, although dermoid cysts have sebaceous glands which produce liquid oily sebum appearing as fat density on CT scan and on MRI demonstrating T1 shortening, chemical shift artefact and signal attenuation on fat suppressed sequences, they do not contain solid adipose tissue as lipocytes are mesodermal in origin [2,7]. Similarly, dermoid cysts may contain semi-formed teeth but do not contain cartilage or bone [8]. It should be noted, however, that the absence of detectable fat or bone on imaging does not exclude the diagnosis of teratoma [9]. In this patient, the detection on CT scan of fatty tissue and bone within the mass is the key for diagnosis (Question: Fig. 1a–c). The presence of these tissues within a mixed cystic and solid enhancing mass is strongly suggestive of a pre-operative diagnosis of teratoma, as contrast enhancement is rarely seen in dermoid cysts [10]. MRI confirmed the presence of fat adjacent, but separate to, the right posterior clinoid process identified on CT scan as an area of intrinsic high T1 signal with surrounding chemical shift artefact on the T2 sequence (Question: Fig. 1d–f). Histology confirmed the radiological diagnosis of intracranial teratoma (Fig. 1).

Quality of life in adulthood after resection of a sacrococcygeal teratoma in childhood: a Dutch multicentre study

ObjectiveChildren treated for sacrococcygeal teratoma (SCT) may experience functional sequelae later in life. It is not known whether SCT and associated problems affect the patient's general quality of life (QoL)....

The long-term outcome of patients diagnosed with sacrococcygeal teratoma in childhood. A study of a national cohort

Background/PurposeThe improved survival of sacrococcygeal teratoma (SCT) has led to increased awareness of its long-term sequelae. Our aim was to assess the long-term outcome of a national cohort using detailed...

Visual Diagnosis: Nasal Congestion and Respiratory Distress in a 7-Day-Old Girl

Visual Diagnosis: Nasal Congestion and Respiratory Distress in a 7-Day-Old Girl

Teratoma inmaduro intramedular infantil

Teratomas in childhood are usually benign, the immature ones being the least frequent. They are intradural tumors, which account for 10% of all the spinal tumors in children. We present a case of a male whose lesion was detected when he was 2 years old with no sensory-motor deficit. Following the tumor excision, some sequels remained that required rehabilitation treatment: neurogenic bladder and lower limb paresis. The anatomy-pathology diagnosed an immature teratoma grade II/III located in D11-L3. There was also recurrence of the intradural lesions in spite of which the evolution was good. A bibliographic search was performed, without finding any case similar to our patient. The importance of a follow-up and multidisciplinary treatment for those lesions is demonstrated by this case. Thus, the role of the rehabilitation doctor is to act early to minimize the sequels and to the highest independence possible.

Pattern and outcome of childhood teratoma: a 10-year review.

Background: Teratomas are neoplasms which originate from pluripotent stem cells. They are composed of a wide variety of tissues foreign to the organ or anatomic site in which they arose. This study sought to determine the clinical manifestations and outcome of treatment of childhood teratomas. Method: A retrospective analysis of the case files of children treated for teratoma at the University of Benin Teaching Hospital Benin City, Nigeria between January 1999 and December 2008 was done. Results: Fifty-three children aged between 5 days and 16 years, comprising 15 males and 38 females with male:female ratio of 2:5 were treated for teratoma; gonadal 27(50.9%) and extragonadal 26 (49.1%). Ovarian, 23 (43.4%) and sacrococcygeal, 17 (32.1%) sites were frequently involved. Other sites included testicular, 4 (7.5%), retroperitoneal, 4 (7.5%) and renal, 2 (3.8%), while posteriormediastinal, cervical and breast involvement were 1 (1.9%) each. Despite late presentation, no frankly malignant primary teratoma was diagnosed. Benign cystic teratomas with malignant elementscomprising yolk sac tumour and immature neuroepithelial elements (mixed germ cells tumour) were the major histological types. Complete tumour resection that included total coccygectomy, oophorectomyand orchidectomy, plus combination chemotherapy using vincristine, actinomycin, and cyclophosphamide was curative in 25 (47.2%) children. Eighteen (34%) cases of recurrence were all malignant. They presented very late with rapid disease progression that resulted in 10 (18.8%) deaths during the five years of follow-up. Conclusion: Although all primary teratoma were benign, recurrent malignant tumours with rapid progression to carcinomatosis, multiple organs failure and deaths were common in childhood teratoma. We advocate close follow-up of all children with teratoma to detect and commence early treatment.

Pattern and outcome of childhood teratoma: A 10-year review

Pattern and outcome of childhood teratoma: A 10-year review

Adenocarcinoma Originating from a Mature Teratoma of the Testis

Malignant transformation in testicular teratomas has been reported very rarely in the literature. Although testicular teratomas in childhood are regarded as benign neoplasms, these tumors, if left untreated until advanced ages, may present the risk of malignant transformation. We report a case of differentiated adenocarcinoma originating from colonic glands in primary testicular teratoma.

Ex-Utero intrapartum procedure for congenital high airway obstruction syndrome in a neonate: First case in Alexandria

<b>Introduction:</b> Large fetal neck masses can present a major challenge for securing an airway at birth with associated risks of hypoxia, brain injury and death. Teratomas of the oropharynx are rare, presenting 3% of teratomas in childhood, and are treated by surgical excision. If respiratory distress accompanies the lesion, priority must be given to the securing of the airway.<b> Case History:</b> We present a case of an infant who was diagnosed antenatally as having a huge oropharyngeal teratoma. The anaesthetic, surgical and neonatology teams were ready to perform surgical excision depending on the placental circulation immediately after securing the airway. The tumour weighed 1591 g and was 20 x 22 x 12 cm. The patient was a male and weighed 715 g. Histopathology showed Grade II teratoma. <b> Conclusion:</b> Large fetal neck masses can present a major challenge for securing an airway at birth with associated risks of hypoxia, brain injury and death. A multidisciplinary team approach combined with an accurate prenatal diagnosis obtained through fetal ultrasound is the key to a successful outcome. Ex utero intrapartum treatment (EXIT) that is based on the placental blood during intubation, tracheostomy or surgical excision is the standard procedure.

Pathology and Molecular Biology of Teratomas in Childhood and Adolescence

The biologic behaviour of teratomas depends on various interdependent clinical and epidemiologic variables such as the age at diagnosis, sex, tumor site, histology which all correlate to different cytogenetic and molecular biologic aberrations. Thus, testicular teratomas of infancy are generally benign. Accordingly, prepubertal teratomas show no cytogenetic or molecular genetic aberrations. In contrast, postpubertal testicular teratomas can present as clinically malignant tumors and may show complex cytogenetic aberrations such as the isochromosome 12p, which is pathognomonic of malignant germ cell tumors. Notably, teratomas of both age groups show an at least partial erasure of the genomic imprinting, correlating with their origin from primordial germ cells. The Kiel Pediatric Tumor Registry includes 541 teratoma specimens, and among these, the most frequent tumor sites (in descending order) are: the sacrococcygeal region (33.8 %), the ovaries (31.2 %) and the testes (10.5 %). Rare localizations include the mediastinum, the retroperitoneum, the head and neck region as well as the central nervous system. The WHO classification of germ cell tumors distinguishes mature and immature teratomas as well as teratomas with malignant transformation. In immature teratomas, primitive neuroectodermal structures predominate. According to the grading system (Gonzalez-Crussi, 1982), mature teratomas (G0) are more frequent (54.5 %) than immature teratomas (G1-G3, 45.5 %). Only 7.8 % of all teratomas show the highest grade of immaturity (G3). The frequency of additional microscopic foci of malignant yolk sac tumor correlates with the grade of immaturity. In sacrococcygeal teratomas, the yolk sac tumor microfoci may give rise to a malignant relapse after incomplete resection. The rare teratomas with malignant transformation contain components with "conventional" somatic type malignancy such as leukaemia, carcinoma or sarcoma. Here, molecular genetic analysis has demonstrated the origin of the somatic malignancy from a malignant transformation within the germ cell tumor with retention of the cytogenetic changes characteristic of malignant germ cell tumors.

Imbalances of chromosome arm 1p in pediatric and adult germ cell tumors are caused by true allelic loss: A combined comparative genomic hybridization and microsatellite analysis

Previous studies on childhood germ cell tumors (GCTs) report highly variable frequencies of losses at chromosome arm 1p. Since deletions at 1p portend a poor prognosis in other embryonal tumors, this study aims to clarify the question of the frequency of true allelic loss at 1p and whether it constitutes a prognostic parameter. We analyzed 13 GCTs from different gonadal and extragonadal sites of children (4 teratomas, 9 malignant GCTs) and 18 GCTs of adolescents and adults (3 teratomas; 15 malignant GCTs) using automated microsatellite analysis with 23 polymorphic markers and chromosomal "high resolution" comparative genomic hybridization (HR-CGH). With this combined approach, we detected loss of heterozygosity (LOH) at 1p in 8/9 childhood malignant GCTs with concordant data from HR-CGH and microsatellite analyses. In contrast, LOH at 1p was not detected in childhood teratomas (0/4) and constituted a rare event in GCTs of adolescence and adulthood (3/18). The commonly deleted region was located at distal 1p36-pter, with a proximal boundary between the markers D1S450 and D1S2870. These data unequivocally demonstrate that deletion at 1p is common in childhood GCTs and results in allelic loss. This observation argues for the presence of a classical tumor suppressor at distal 1p. Considering the high frequency of LOH at 1p and the overall favorable prognosis of childhood GCTs, a prognostic impact of LOH at 1p in childhood GCTs appears unlikely. However, since two postpubertal tumors with LOH at 1p progressed, a prognostic relevance in this age group seems possible, warranting a prospective evaluation.

Fusion of the Tumor-Suppressor Gene CHEK2 and the Gene for the Regulatory Subunit B of Protein Phosphatase 2 PPP2R2A in Childhood Teratoma

We characterized the molecular genetic consequences of a balanced chromosome translocation t(8;22)(p21; q12), which occurred as the sole cytogenetic aberration in short-term cultured cells from an intrathoracic mature teratoma in a 15-year-old girl. Fluorescence in situ hybridization and reverse transcription- polymerase chain reaction disclosed that t(8;22) resulted in the fusion of the genes PPP2R2A and CHEK2, with an inserted fragment belonging to class I endogenous retrovirus-related sequences at the junction. Sequencing of the two genes did not reveal any additional mutation. None of the three detected PPP2R2A/CHEK2 fusion transcripts resulted in an in-frame PPP2R2A/CHEK2 chimerical open reading frame; however, in all of them, the known open reading frame of CHEK2 was preserved. Thus, promoter swapping leading to deregulated CHEK2 expression would be the most likely oncogenic mechanism. Whereas inactivating mutations of CHEK2 previously have been described in a variety of sporadic tumors and in inherited cancer-predisposing syndromes, PPP2R2A, encoding a regulatory subunit of the multimeric enzyme phosphatase 2, has not been directly implicated in tumorigenesis. Our findings suggest that deregulation of CHEK2 and/or PPP2R2A is of pathogenetic importance in at least a subset of germ cell tumors.

Congenital cervical teratomas

Congenital cervical teratomas are rare, representing 3% of teratomas in childhood. Although mostly benign, they are associated with a high mortality rate due to respiratory distress and require immediate surgical excision. The diagnosis is usually suggested on the physical examination. The imaging investigation is essential for the proper diagnosis and preoperative planning.We present the imaging characteristics of cervical teratomas identified in five neonates over a 10-year-period. The masses were surgically resected. Histopathology revealed the presence of congenital teratomas (three immature, two mature). Two neonates died soon after surgery, while the remaining were discharged from the hospital without symptoms.