Teratomas In Children Research Articles

Diagnosis and treatment on the intracranial immature teratomas in children ( a report of 36 cases )

Objective To explore the diagnosis and combined treatments on the intracranial immature teratomas.Methods The clinical data of 40 intracranial immature teratomas were collected from Jan.2002 to Dec.2008,and 36 were followed up.According to the clinical symptoms,signs,serum tumor markers and radiology,the immature teratomas were diagnosed and the followed treated courses were different depending on the tumor volume and symptoms.Results All the 36 cases were operated and immature teratomas were confirmed by the histopathology.In the giant tumor group ( 26 cases,which the tumor diameter larger than 3 cm),the cases were divided into group A and group B.In the group A,the patients were in a relative good condition and the tumors didn' t invade the adjacent cerebral tissues,so,the operation was recommended first and the chemo - and radio - therapy were followed.In the group B,when the patients were in a poor condition and tumors invade the adjacent cerebral tissues obviously,chemotherapy were recommended first and when the tumors shrink,the operations were followed and then treated by the combined treatments.In the small tumor group ( 10 cases,which the diameter less than 3 cm),operations were recommended first and the combined treatments were followed.Conclusions Chemotherapy is firstly recommended on the intracranial immature teratomas,and when the tumors shrink and pseudo - membranes are formed,operations are followed.Postoperatively,combined treatments could obviously extend the patients' life. Key words: Intracranial immature teratomas; Surgical procedures,operative; Chemotherapy; Radiotherapy; Prognosis

Portal teratoma causing obstructive jaundice in children: a rarity

Teratoma in children is a common entity, usually occurring both in gonadal and extragonadal sites. Common extragonadal sites for teratoma in children are the sacrococcygeal region, retroperitoneum, and mediastinum. Various unusual extragonadal sites have been reported. However, teratoma in the hepatoduodenal ligament is a very rare occurrence. We herein report a case of a mature teratoma in the hepatoduodenal ligament in an 11-year-old child presenting with obstructive jaundice along with its surgical management and review of the literature.

Evaluation and Management of Ovarian Teratomas in Children and Adolescents: A Review of Eight Years Experience at Four Medical Centers in a Single Metropolitan Area

Depending on specialty service availability at a given hospital, a young female is managed for her suspected ovarian teratoma differently, in relation to imaging performed, tumor markers measured and surgical approach. The goal of this study is to illustrate different surgical specialists' management of ovarian teratomas in children and adolescents at urban community, county, pediatric and university hospitals in a single metropolitan area.

Head and Neck Teratomas in Children: A Case Series

Teratomas of the head and neck due to their obscure origin, bizarre microscopic appearance, unpredictable behaviour and often dramatic clinical presentation are a clinical surprise. This article focuses on pediatric head and neck teratomas and on their diversity and rarity and also reviews the recent terminology of this group of tumours.

Ovarian‐sparing surgery for ovarian teratoma in children

Ovarian teratoma (OT) is the most common ovarian neoplasm in children. Oophorectomy has been the standard treatment but may impair fertility. The aim of this study was to investigate the feasibility and outcome of ovarian-sparing surgery (OSS) for OT. We retrospectively studied all children treated for OT at a pediatric teaching hospital in Paris, France, between March 1992 and July 2006. OSS was performed when deemed technically feasible in patients who had no lymphadenopathy by preoperative imaging or surgical exploration, normal tumor marker levels, and calcifications on radiographs. We identified 30 patients, including 29 with unilateral OT and 1 with synchronous bilateral OT. Emergent surgery was performed in five patients, among whom four had ovarian torsion requiring oophorectomy and one underwent OSS. Of the 26 OTs in the 25 remaining patients, 10 were managed with OSS and 16 with oophorectomy. Subsequently, ultrasound monitoring detected OT development in the contralateral ovary in 4 (14%) patients, after a median of 3 years (range, 1-14 years); OSS was performed in all four cases. The patient with bilateral synchronous OT, managed by OSS initially, underwent unilateral oophorectomy 3 years later for a recurrence. Overall OSS was performed for 15 (42%) OTs. Our results suggest recommendations for preserving fertility whenever possible without compromising the oncological prognosis. In particular, OSS should be reserved for patients who meet all criteria for localized mature teratoma. Long-term follow-up is crucial.

Huge Mesenteric Teratoma Encasing the Superior Mesenteric Artery and Vein: Report of a Case

A 10-month-old male infant suffered from progressive abdominal distention and poor appetite for 2-months. Computed tomography revealed a mesenteric teratoma, 15 cm in diameter, extending from the subhepatic area to the pelvic cavity with the small intestine displaced caudally and to the left side of the pelvis. Both the superior mesenteric artery (SMA) and vein were encased within the tumor for the length of at least 5 cm. During operation, tumor was initially dissected through the plane beneath the elevated small bowel mesentery, and the procedure proved rather difficult as the SMA emerged from the tumor, which was accidentally divided. The small bowel turned pale immediately but became pink in color several minutes later. After excision of the tumor, the severed ends of the SMA and SMV were anastomosed smoothly. The color and peristalsis of the small bowel were normal after anastomosis. The patient was discharged on the 5th postoperative day.Mesenteric teratoma is an extremely rare tumor of the germ cells. Complete tumor resection is the main prognostic factor for survival in such cases. Therefore, an important factor for successful surgery is the exact preoperative visualization of the extent of the tumor and meticulous intra-operative assessment of the collateral vasculature. A huge mesenteric teratoma in children, encasing the root of the mesenteric vessels tightly, presents a management challenge to the surgeon. In this particular case, the reversibility of small bowel ischemia caused by accidental severance of the SMA is thought to be due to gradual development of abundant collateral circulation to the small bowel during prolonged compression of the SMA.

OLIGODENDROGLIOMA ARISING IN A MATURE CYSTIC OVARIAN TERATOMA IN A CHILD

In childhood mature cystic teratomas are the most common type of ovarian germ cell tumors. Tumors of neuroepithelial origin are extremely rare in mature teratomas. To the authors’ knowledge, this is the first case report oligodendroglioma arising in a mature cystic ovarian teratoma in children. A 13-year-old girl presented with a history of pelvic pain for 2 months. An ultrasound examination showed a monolateral multicystic, calcific ovarian mass, measuring approximately 6 × 5 cm, arising in the left over and adnexia, suggesting a teratoma. An exploratorative laparotomy and ooferectomy were performed. On histopatological examination, various samples from cystic areas had mature tissues from all 3 germ cell layers, including skin, bone, bronchial structures, and cerebellum. Sections from solid areas showed uniform population of monotonous, rounded cells with indistinct cytoplasm and having perinuclear halo (fried-egg appearance) on a fibrillary, neuropilic background. Microcalcifications as calcospherites were noted in the stroma. Based on the typical morphological features, the cased was diagnosed as mature cystic teratoma with low-grade oligodendroglioma. To the authors’ knowledge, there are a few reports in the literature of an oligodendroglioma arising in an ovarian teratoma in adults and this presented patient is the first case in childhood.

Intracranial immature teratomas in children (30 cases report)

Objective To investigate the clinical features,therapeutic strategies and prognosis of intraeranial immature teratomas in children.Methods A retrospective review of 30 children with immature teratomas pathologically confirmed by surgical resection was performed.26 cases were followed up.Results Semmand (or) cerebrospinal fluid tumor markers (AFP,β-HCG) were tested preoperatively in 19 cases,with positive result in 12 cases.At the initial operations,total,gross-total,sub-total and partial removal of the tumors were achieved in 15,7,6 and 2 cases respectively.Among the followed-up children,23 cases received adjuvant therapies.The half-year.1-year and 2-year survival rates were 80%.69% and 60% respectively.There was a significant correlation between the tumor resection extent and patient(s) survival.Conclusion The tumor markers are useful for diagnosis.Surgery is the therapeutic priority and total resection should be achieved ag far as possible.Postoperative radiotherapy and chemotherapy should be routine adjuvant treatment measures. Key words: Brain neoplasms; Immature teratoma; Teratoma; Germinoma; Child; Prognosis

Primary retroperitoneal teratomas in children: A single institution experience

This study aims to highlight the clinical features, investigations and treatment outcome of retroperitoneal teratomas (RPT) in children. A total of eight patients (six males and two females, age range between 6 months-10 years) of RPT admitted in the department of Paediatric Surgery, PGIMS, Rohtak, between 1996-2008, were studied. The patients were investigated with hematology, x-ray, ultrasound, and computerised tomography (CT) of abdomen and serum alpha-fetoprotein levels in pre and postoperative period. All patients underwent complete surgical resection. In one patient, the tumour had malignant component (yolk sac) and was given postoperative chemotherapy. Postoperative follow-up included serum alpha-fetoprotein in addition to clinical examination and radiological assessment to detect recurrences. The tumours were located on both sides in almost equal proportion (four on right, three on left, and one bilateral]. All tumours could be excised completely preserving the kidneys in all patients. But in one patient injury to inferior vena cava (IVC) occurred which was repaired successfully. Majority (7 out of 8) were histological benign, and in one yolk sac tumour was malignant component which needed chemotherapy. All children were on follow-up and one patient with malignancy lost to follow-up after three cycles of chemotherapy. In rest there was no tumour recurrence. RPT are rare paediatric neoplasms. As majority are benign, a complete excision preserving the kidneys, is usually curative. Serum alpha-fetoprotein is a reliable method of assessing recurrence. Malignancy in the tumour may warrant further chemotherapy.

Thyroid cervico-thoracic teratoma in a 14-month old child

Cervical teratoma (CT) is a rare disease which accounts for approximately three to five per cent of all teratomas in children. Airway compromise is the most serious postnatal complication of cervical teratoma. Prenatal diagnosis is crucial for early recognition of neck masses that could obstruct the airway. We present our experience in the management of a huge cervico-thoracic teratoma in a 14-month old Nigerian child.

Imaging of Ovarian Teratomas in Children: A 9-Year Review

Objective Germ cell tumours are the most common ovarian neoplasms in childhood and, of these, teratomas, whether mature or immature, are the most frequently found. Mature teratoma is a benign tumour, whereas the immature type, although also benign, has a more aggressive course, with a propensity to recurrence. A review of the literature revealed that there are some imaging features that may help to differentiate between these 2 types of teratoma, although no systematic comparison has been made. The objective of this study was to review imaging features of ovarian teratomas in children and to assess differentiating imaging features between the mature and immature types of ovarian teratoma. Methods Retrospective analysis of all patients who presented to our institution during a 9-year period (September 1999 to August 2008) with ovarian teratoma as confirmed on histology. Results Forty-one patients with pathologically proven ovarian teratoma were found. The patient ages ranged from 4–18 years at presentation (mean [standard deviation] age, 12.4 ± 3.4 years; median age, 13 years). Thirty patients (73.2%) were found to have mature ovarian teratoma, and 11 (26.8%) had immature teratoma. A component of endodermal sinus tumour was found in one of the immature teratomas. On ultrasonography, the appearance of the immature teratomas was purely solid in 3 (27.3%), mixed solid and cystic in 6 (54.5%), and predominantly cystic in 2 (18%). The mature ovarian teratomas demonstrated a predominantly cystic appearance in 22 (73.3%) and a mixed solid and cystic appearance in 8 (26.6%); there were no cases with a pure solid appearance. The prevalence of the more cystic appearance of the mature type showed significant statistical difference when compared with its prevalence in the immature type ( P = .0008, χ 2 test). Other imaging features, such as size, presence of fat, or calcifications, did not show a significant difference between the 2 types of teratoma. Conclusions The predominance of a cystic component and a pure solid component in ovarian teratoma are significant differentiating factors between the mature type and the more aggressive immature type of teratoma.

Isolation of three stem cell lines from human sacrococcygeal teratomas

Sacrococcygeal teratomas (SCTs) are benign tumours of the newborn with absolute indication for surgery directly after birth. We recently described the presence of stem cells positive for the stem cell markers nanog and Oct4 in SCTs. Here we report the isolation of three stem cell lines from three different SCTs. Cells were propagated in mesenchymal or in embryonic stem cell medium. Non-clonal homogeneous stem cell lines were obtained after two to three passages and characterized in vitro by immunocytochemistry, RT-PCR, western blot, FACS analysis, and metaphase spreads. The differentiation potential was tested in vitro and in vivo. The isolated cell lines, which we refer to as human sacrococcygeal teratoma stem cells (hSctSCs), express nanog, Oct4 and stella, and are negative for malignancy markers alpha-fetoprotein and carcinoembryonic antigen. They can be induced in vitro to express neuronal, osteogenic, and chondrogenic traits. After grafting in vivo, spontaneous integration into the neural crest of the chick embryo and teratoma formation in the nude mouse were obtained. Our results indicate that SCTs are derived from remnants of the epiblast-derived primitive streak, which in the human embryo normally regresses but forms teratomas in children affected with SCT. The hSctSCs therefore may be comparable to mouse epiblast-derived stem cells (EpiSCs) and share characteristic features with human embryonic stem (hES) cells. Thus, SCT tissue obtained after surgery appears to be a novel source for the generation of human stem cells without the ethical implications associated with hES cells.

Intracranial teratomas in children: the role and timing of surgical removal

In this study, the authors report their experience with the surgical treatment of intracranial teratomas with an emphasis on the indications for delayed resection after oncological treatment. The authors retrospectively reviewed the cases of 14 children with intracranial teratomas. The mean age at diagnosis was 10.5 years (range 2 days-18 years), and 11 patients were male. The final histological analysis revealed pure mature teratoma in 5 cases, mixed teratoma with germinoma in 3 cases, and nongerminomatous malignant germ cell tumor in 6 cases. Thirteen patients underwent tumor resection, and these patients were divided into 2 subgroups according to the timing of surgery. In Group A, 10 patients underwent resection as the primary treatment because no tumor markers were detected in 4 patients, a teratomatous component was revealed on biopsy sampling in 3 patients, and a large tumor volume in 3 patients. In Group B, 3 patients underwent removal of residual pure mature teratoma after oncological treatment. Seven of the 8 patients (87.5%) with pure mature teratomas or with mixed teratoma and germinoma are currently alive (mean follow-up of 9 years); the eighth patient died of postoperative meningitis. Two of the 6 patients (33%) with mixed nongerminomatous malignant germ cell tumors died of tumor progression regardless of the timing of surgery. The results of this study support the belief that microsurgical removal is the only effective treatment for intracranial teratomas. Surgery may be performed as the primary therapy when there is evidence of a noninvasive teratoma, and as a secondary therapy if there is only a partial response to neoadjuvant therapy or if progression is observed in mixed malignant germ cell tumors.

Pediatric Intramedullary Teratomas

Teratomas account for 3% of all childhood tumors, with the majority occurring in the sacrococcygeal region and in the ovary. Intradural spinal teratomas are extremely rare dysembryogenetic tumors. Spinal cord teratomas may be extradural, intradural or intramedullary. Intramedullary ones are the least frequently seen. We have extensively reviewed the literature for intramedullary spinal cord teratomas in children. Although an intramedullary teratoma of the conus medullaris in children is a rare entity, it should be considered in the differential diagnosis of masses involving the conus medullaris.

Mature and Immature Extracranial Teratomas in Children: The UK Children's Cancer Study Group Experience

The purpose of this article is to describe the features, treatment, and risk factors for relapse of children with mature teratoma (MT) and immature teratoma (IT) to assist future treatment plans. Patients were younger than 16 years of age and referred to the UK Children's Cancer Study Group centers with biopsy-proven extracranial MT and IT and no prior chemotherapy. Complete excision, with the coccyx in sacrococcygeal patients, and follow-up, including serum alpha-fetoprotein monitoring for early detection of malignant yolk sac tumor (YST) recurrence, were recommended. Carboplatin, etoposide, and bleomycin (JEB) were given for YST relapse, whereas relapsed MT and IT were treated at clinicians' discretion, usually surgically. Pathology was reviewed and treatments, outcome, and prognostic features assessed. There were 351 patients, 227 with MT, 124 with IT. Tumor sites were: testis (n = 53), ovary (n = 130), sacrococcygeal region (n = 98), thorax (n = 23), and other (n = 47). Surgical resection was incomplete in 26% of MT and 40% of IT patients; 5-year event-free survival was 92.2% and 85.9%, respectively, and 5-year overall survival was 99% and 95.1%. Poorer outcome occurred with incomplete resection, tumor rupture, nongonadal site (particularly sacrococcygeal), young age, higher stage and grade, and gliomatosis peritonei, but not with cyst fluid aspiration/spillage, tumor enucleation, nodal gliomatosis, or microfoci of YST in the tumor (Heifetz lesions). JEB was effective for YST recurrence, but not for MT or IT. Treatment remains primarily surgical, with JEB chemotherapy for YST relapse. No definite response followed JEB for pure MT and IT. Adjuvant chemotherapy after surgery for sacrococcygeal patients is not advocated.

Clinicopathologic and prognostic study of pediatric immature teratoma

To study the clinicopathologic features and biologic behavior of pediatric immature teratoma. The clinical data, pathologic features, immunohistochemical findings (for cyclin D1, P27 and Ki-67) and follow-up information of 39 cases of pediatric immature teratoma were analyzed. Amongst the 39 cases studied, 12 arose in the sacrococcygeal region, 12 in testis, 5 in retroperitoneum, 4 in ovary, 4 in abdomen and 2 in mediastinum. Histologically, 16 cases were of grade 1, 8 cases of grade 2 and 15 cases of grade 3. Seven of the cases contained foci of yolk sac tumor. Immature neuroepithelial features used in histologic grading included the presence of primitive neural tubules, immature rosettes, undifferentiated neuroblastoma cells and primitive neuroectodermal structures. Immunohistochemical study showed that cyclin D1 was positive in 3 cases of grade 1 tumors, 4 cases of grade 2 tumors and 9 cases of grade 3 tumors. The positivity rates for p27 were 8, 3 and 6 cases respectively, while those for Ki-67 were 3, 4 and 13 cases respectively. Follow-up data were available in 30 cases. Three of them, including 2 cases with histologic grade 3 (with or without yolk sac tumor component), recurred after operation. The expression of cyclin D1 and Ki-67 is a useful adjunct in histologic grading. On the other hand, p27 overexpression shows little correlation with tumor grade. The prognosis of immature teratoma in children is different from that in adults. Sacrococcygeal immature teratoma occurring in patients younger than 1 year old and with low histologic grade do not require postoperative chemotherapy if the tumor is completely excised. Similarly, for testicular immature teratoma occurring in patients below 1 year of age, regardless of tumor grading, need no adjunctive therapy. On the other hand, ovarian immature teratoma with high histologic grade requires postoperative chemotherapy, regardless of age of the patients. The presence of microscopic foci of yolk sac tumor is a useful predictor of recurrence in pediatric immature teratoma.

An Overview of Testicular Germ Cell Tumors

More than 90% of testicular neoplasms originate from germ cells. Testicular germ cell tumors (GCTs) are a heterogeneous group of neoplasms with diverse histopathology and clinical behavior. To help the readers distinguish various subtypes of GCTs, to highlight the clinical manifestations and pathologic features of these tumors, and to review several newly developed immunohistochemical markers for GCTs. Review of the pertinent literature and our experience. The etiology of GCTs is largely unknown. Cytogenetic studies suggest a different pathogenesis for each group of infantile/prepubertal GCTs, postpubertal GCTs, and spermatocytic seminoma. Unclassified intratubular germ cell neoplasia is the precursor of all GCTs, excluding spermatocytic seminoma and infantile/prepubertal GCTs. Seminoma, the most common GCT in adults, does not occur before 5 years of age. Spermatocytic seminoma, a tumor of elderly men, typically has an indolent clinical behavior, but rarely it undergoes sarcomatous transformation associated with an aggressive behavior. Embryonal carcinoma is the most common component in mixed GCTs. Eighty percent or more of embryonal carcinoma component and vascular invasion are recognized predictors of occult metastasis for clinical stage I mixed GCTs. Most patients with prepubertal yolk sac tumor, the most common pediatric GCT, have stage I disease at presentation. Most choriocarcinomas present with metastatic symptoms because of the propensity for rapid hematogenous dissemination. Teratomas in children regardless of maturity and dermoid cysts in adults are benign; in contrast, teratomas in adults have a malignant behavior. With appropriate therapy, the majority of testicular GCTs are curable.

Mixed Nonseminomatous Germ Cell Tumor Presenting as a Subcutaneous Tissue Mass

Extragonadal germ cell tumors most commonly arise in the midline of the retroperitoneum or the mediastinum. Primary tumors involving the skin are very rare. Only one case of malignant primary germ cell tumor located in the skin has been reported. We present the case of a 44-year-old white man with a primary subcutaneous mixed nonseminomatous germ cell tumor. This man had a long-lasting subcutaneous lump of the breast, which became painful. Surgery revealed 3 juxtaposed nodules. Microscopic examination showed a mixed germ cell tumor with a 90% immature teratoma component and a 10% embryonal carcinoma component. Testicular ultrasound and computed tomography of the chest, abdomen, pelvis, and brain were normal. Serum human chorionic gonadotrophin, beta-human chorionic gonadotrophin, alpha-fetoprotein, and lactate dehydrogenase were within normal ranges. A further surgical excision was performed. The patient is presently alive with no evidence of disease after a follow-up of 7 years. Review of the literature indicates that primary cutaneous extragonadal germ cell tumors usually occur as cutaneous or subcutaneous solitary nodules or as ulcerated lesions. They mainly consist of mature teratomas in children. Only 2 cases have been reported in adults.

Primary Thyroid Teratomas in Children

Cervical teratomas are uncommon neoplasms, although the commonest neck tumors in newborns and infants. Presence of associated thyroid tissue often causes speculation as to the site of origin, ie, arising from within thyroid, adjacent soft tissue with secondary involvement of thyroid, or as innate part of a cervical teratoma. Twenty-eight cases of cervical teratomas were identified over 75 years, including 11 cases containing associated thyroid tissue. Clinical history, treatment, and follow-up were reviewed and the neoplasms analyzed regarding location, size, degree of maturity, and relative arrangement of thyroid and other tissues. All thyroid teratomas were congenital, measured 3.5 to 13.5 cm in diameter (median size, 6.9 cm), and were resected. Follow-up ranged from 1 to 45 years (median, 17 years) without recurrent disease in any patient. Neuroglial tissue predominated in 10 of 11 tumors. Intimate admixture of thyroid and other tissues with or without surrounding fibrous pseudocapsule was present in 8 cases, suggesting thyroid as origin. Histologic immaturity in congenital thyroid teratomas is not the harbinger of adverse behavior as seen in adolescents and adults. Intimate intermingling of thyroid tissue with teratoma and presence of a pseudocapsule seem to be the most significant criteria for establishing thyroid as origin.

Immature Gastric Teratoma in a Neonate

Gastric teratoma (GT) is an exceeding rare lesion seen most often in male infants, comprising less than of all teratomas in children. A 2-day-old male child was preoperatively diagnosed to have gastric teratoma on ultrasonogram and abdominal computed tomography (CT). On laparotomy, Dumbbell- shape GT was located on lesser curvature of stomach and complete excision of the tumor was performed. The histological examination revealed immature teratoma of the stomach. The child is well 10 months after surgery.