Antenatal diagnosis of a third ventricular mass: fetus in fetu or teratoma?

BACKGROUND: Fetus in fetu (FIF) is a rare condition in medicine where an aberrant monozygotic twining results in inclusion of a degenerated fetus inside its twin bearer. Whereas FIF is generally defined as a fetiform vertebrate mass, teratoma is considered as a tumor composed of disorganized tissues derived from the three embryological layers. Recent data plead for a common origin and suggest a continuum between FIF and teratoma. CASE HISTORY: We describe the case of an intraventricular mass diagnosed by prenatal imaging and associated with triventricular hydrocephalus. Surgical removal of a non-vertebrate mass covered by normal skin was performed when the child was 15 months old. Now aged of 30 months, the child continues to develop normally. Histological data were in favor of a FIF. DISCUSSION: We discuss the diagnosis, pathogenesis, and prognosis in the light of data from the recent literature. CONCLUSIONS: Aberrant monozygotic twining leading to inclusion of a degenerate fetus inside its twin bearer results in FIF or teratomas. Surgical removal represents the main treatment. Intracranial FIF remains an extremely rare congenital condition, with a favorable oncological prognosis; the developmental prognosis appears conditioned by the absence of other malformation, the small size of the lesion, and the early management of intracranial hypertension.     

Posterior mediastinal teratomas

Eight benign posterior mediastinal teratomas have been found in the world's literature. Including our case, four teratomas were intimately involved with, and often required, partial resection of the major surrounding structures--aorta, chest wall, and esophagus. Preoperative evaluation of posterior mediastinal teratomas should include consideration of a barium swallow and/or aortography to rule out significant local involvement.     

Intramedullary cystic teratoid tumor of the cervical spinal cord in association with a teratoma of the ovary

A 15-year-old black female student came to the hospital because of disturbance of her gait. On examination, there was a dermal sinus in the skin overlying the spine of the seventh cervical vertebra, and myelography with computed tomography scan demonstrated a block to the flow of metrizamide contrast material at T-5 with cephalad extension of an intradural cystic lesion to the C-5 level. An x-ray examination of the abdomen at the time demonstrated a tooth-shaped radiodensity in the left hypogastrium. On exploration of the spine via C5-T5 laminectomy an intramedullary cystic teratoid tumor was found which was partially excised. At later exploration of the abdomen, a teratoma of the left ovary was removed. The pathology and natural history of teratomas and teratoid tumors is discussed along with surgical management of these tumors.     

Immature teratomas of childhood. Report of 21 cases

The benign or malignant nature and the biological behaviour of immature teratomas of childhood are difficult to predict. The age of the patient at diagnosis, the anatomical site of the tumour and the degree of immaturity are considered to be important prognostic parameters. In this study the pathological-anatomical findings and the most important clinical features of 21 patients with immature teratoma (including two with supposedly malignant monodermal teratoma with immature neuroectodermal structures) were evaluated. Significant results were: Twelve tumours occurred in infancy or infants who died in the perinatal period, the other nine tumours in children between the ages of 7 and 16 years. The most frequent anatomical sites were the ovary (6 tumours), sacrococcygeal region (4), testis (4) and mediastinum (3). In contrast to most of the tumours of other localizations, immature ovarian teratomas did not occur in children under 7 years of age (in four cases in association with gliomatosis peritonei). The immature tissue components of the tumours were mostly neuroectodermal structures. Eight tumour specimens showed grade 1, four grade 2 and nine grade 3 malignancy. Grade 3 tridermal teratomas chiefly occurred in young children, whereas two grade 3 monodermal tumours developed in older children. Immunohistochemical analysis of the neuroectodermal components showed that mature astrocytes contained glial fibrillary acid protein, whereas mature nerve cells, nerve fibres and a few groups of immature cells reacted with an antibody to neuron-specific enolase. Six of the 21 patients died; two were stillborn immature infants, two were premature infants, one died postoperatively and one died of metastatic disease. One patient with metastatic disease was alive. None of the 19 children with tridermal immature teratoma showed distant metastases. Metastatic disease was observed in only two patients with presumptive monodermal malignant teratoma. In early childhood the biological behaviour of immature teratomas is evidently similar to that of mature teratomas (provided that the tumour can be totally excised). In older children malignancy must be assumed when the tumour is located in the ovary and/or grade 3 immaturity is determined.     

睾丸畸胎瘤核仁组成区嗜银蛋白定量研究

应用银染色技术对1O例睾丸正常组织和13例睾丸良恶性畸胎瘤细胞进行了核仁组成区(NORs)研究,结果发现:正常睾丸及畸胎瘤的核仁组成区嗜银蛋白(AgNORs)颗粒呈圆形,规则,边界清楚,大小均匀,位于胞核编中成边缘部位;畸胎癌的AgNORs颗粒呈圆形或卵圆形,欠规则,边界欠清,大小较不一致,偏中或散在分布于核内.AgNORs计数正常组为1.56±0.17,畸胎瘤组为2.4O±0.26,畸胎癌组为5.24±0.36.各组间有显著性差(P<0.01).AgNORs计数在良恶性畸胎瘤间有分离.认为AgNORs技术对睾丸良恶性畸胎的鉴别是有意义的.     

Neonatal Intracerebral Teratoma

A neonate, who presented with a large head but no neurological abnormalities, was diagnosed by ultrasound and CT scan as having a brain tumor. The pathological diagnosis was teratoma with matured tissues. The literature is briefly reviewed and the clinical manifestations and histological classification are documented.     

Videothoracoscopic resection of anterior mediastinal teratoma in a child

This report describes a case of anterior mediastinal teratoma in a 10-year-old girl, which was successfully resected thoracoscopically. Received: 4 October 1996/Accepted: 23 January 1997     

Primary intracranial germ cell tumours

Summary A histological study has been made of a retrospective series of 17 primary intracranial germ cell tumours found in a collection of 3550 intracranial neoplasms (incidence of 0.48%). All, except for two differentiated teratomas (one extracerebral in a neonate and another in the lateral ventricle), were situated in the midline in persons aged 5 to 37 years (13 males, 4 females). 12 tumours were located in or originated from the (para)pineal region, two of them also invaded the hypothalamus, while three germinomas occupied the retrochiasmal (supra/intrasellar) region without pineal involvement. There were 11 rather pure tumours (7 germinomas, 4 teratomas of various differentiation) and six mixed neoplasms (2 germinomas with teratoid areas, 3 embryonal carcinomas containing elements of endodermal sinus tumour, choriocarcinoma and germinoma, and one teratocarcinoma with endodermal sinuses). Only one case showed prominent features of endodermal sinus tumour, but characteristic elements of this type were present in four other mixed tumours. All germinomas and germinomatous parts of mixed neoplasms showed an inflammatory reaction of varying intensity, in 6 cases associated with multinucleated giant cells, which may be related to the prognosis of these tumours (one patient with hypothalamic germinoma is alive 6 years after radiotherapy). The close structural similarities between the various types of intracranial and gonadal dysgerminomas and their frequent combination within the same tumour support the concept of a common histogenesis of germ cell tumours regardless of their site of origin. Difficulties of classification may arise from the rather frequent occurrence of mixed germ cell neoplasms.Dedicated to E. Frauchiger, on the occasion of his 70th anniversary.     

Orbital teratoma in the newborn A case report

A case of congenital orbital teratoma is presented with illustrations and discussion of typical radiographic features which may facilitate preoperative diagnosis and treatment.     

Prenatal ultrasonic diagnosis of epignathus.

This case demonstrated a mass seen on an ultrasound scan performed prior to amniocentesis to determine fetal maturity. It emphasizes the importance of scanning the entire contents of the amniotic sac rather than simply looking at the portion for which the examination was ordered. It is important to recognize such lesions to prepare for adequate management of the airway in the neonate, to prepare for surgical excision as soon as feasible, and to prepare the family for the birth of a baby with a congenital abnormality.