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Why are ovarian teratomas benign whilst teratomas of the testis are malignant?

A mechanism is suggested to account for the fact that most ovarian teratomas are benign (dermoid cysts) whereas testicular teratomas are almost always malignant. The mechanism advanced assumes that malignancy is cause by a recessive mutation, and then shows that the known facts of germ-cell development will produce the homozygous (malignant) state in the male but the heterozygous counterpart in the female. It is proposed that the benign nature of ovarian teratomas is a reflection of this heterozygous genotype.

Cell Differentiation↗

Isochromosome 1q as the sole chromosomal abnormality in two fetal teratomas. Possible trisomic or tetrasomic zygote rescue in fetal teratoma with an additional isochromosome 1q.

An isochromosome of the long arm of chromosome 1 leading to tetrasomy 1q was detected as the sole chromosomal aberration in two cases of fetal teratoma arising from the oral cavity. This type of teratoma is extremely rare and has seldom been investigated cytogenetically. Studies of DNA markers in the tumor, normal fetal skin, and parental cells demonstrated that in both cases the additional 1q material was of maternal origin. In one of the patients, the teratoma had maternal 1q marker alleles that were not found in the fetal body cells. This implies that the tumor was not derived in a direct way from the fetal body tissue; instead, the chromosomally-normal fetus might be the result of some trisomic or tetrasomic zygote rescue mechanism.

Alleles↗

Teratomas, neoplasia and differentiation: a biological overview. I. The natural history of teratomas.

In this paper I have attempted to outline the natural history of spontaneously occurring teratomas and associated tumours, but without introducing any preconceptions or assumptions as to their histogenesis or mechanisms of internal differentiation. The justification for this omission is that there is presently no direct evidence in respect of either process in human tumours of this type, with the exception of recent work by LINDER et al. (1975a, b). The various histopathological classifications of teratoid lesions have not been discussed in detail either, because for the most part they have been evolved to serve a specific prognostic purpose on either an empirical basis or on certain histogenetic assumptions. In either case there is an inevitable tendency to emphasize extremes of morphological appearance at the expense of possible continuities of structure and behaviour across the spectrum of teratoid lesions. The purpose of this paper, on the contrary, has been to discern features common to these tumours whenever and wherever they occur. Thus, human teratomas are uniquely puritissular lesions composed, potentially at least, of virtually every recognizable (and probably some as yet unrecognizable), type of embryonic, foetal and adult cell and tissue, together with, in some cases, frankly malignant cells of no obvious derivation or differentiation. In spite of the occasional appearance of structures resembling pre-somite embryos (embryoid bodies), there is no compelling evidence that human teratomatous histogenesis recapitulates embryogenesis in any strict fashion. Histologically, the overwhelming impression is of a more or less random association of cells and tissues at different stages of development and differentiation that proceed up to but not beyond organogenesis. Such teratomas are found at a number of well-defined sites, of which the sacrococcyx is the most prominent in infancy and the gonads during adult life. Both gonadal and extragonadal sites are also associated with apparently homogeneous neoplasms containing cells with features reminiscent of early stages of germinal differentiation which evoke, in many cases, a marked stromal and lymphocytic response, the so-called germinomas. In 'mixed' tumours, which are recognized in an increasing proportion of cases, there is karyological evidence of a conjoint rather than separate origin of teratomatous and germinomatous elements in at least some tumours, challenging a long held belief in their invariably separate character (e.g. HALLEY, 1963).

Animals↗

Teratoma in the orchiectomy specimen and volume of metastasis are predictors of retroperitoneal teratoma in low stage nonseminomatous testis cancer.

PURPOSE: We determined whether teratomatous elements in the orchiectomy specimen predict for teratoma in the retroperitoneum in patients who have not received chemotherapy. MATERIALS AND METHODS: We retrospectively reviewed the records of patients with clinical stages A, B and B2 nonseminoma who underwent retroperitoneal lymph node dissection. RESULTS: Teratomatous elements in the orchiectomy specimen predict for retroperitoneal teratoma. CONCLUSIONS: When deciding on treatment for low stage nonseminoma, the presence or absence of teratoma in the orchiectomy specimen should be considered to minimize double therapy.

Humans↗

The growing teratoma syndrome secondary to immature teratoma of the ovary.

The growing teratoma syndrome (GTS) secondary to a testicular nonseminomatous germ cell tumor has been well described since the first case was reported in 1982. In addition, radiological findings of male GTS have also reported in several studies; however, its occurrence secondary to an ovarian germ cell tumor is extremely rare. We experienced a 5-year-old girl who had GTS secondary to immature teratoma of the right ovary. In this article we present radiological findings of female GTS with clinical and pathological correlation.

Child, Preschool↗

Immature teratoma of the ovary with a neural component ("solid" teratoma). A clinicopathologic study of 20 cases.

Twenty cases of immature teratoma of the ovary with a neural component are analyzed. A plea is made for use of the nomenclature adopted from the new World Health Organization classification of ovarian tumors, the past confusion over terminology and histogenesis of this rare tumor is discussed. All the primary tumors in the present series contained at least some immature tissues (predominantly of neural origin) and were thus graded from 1 to 3 according to the criteria of Thurlbeck and Scully. No grade 0 tumors ("benign solid teratomas") were identified. We believe that thorough sectioning almost always insures the identification of immature elements. The prognosis was closely related to the histologic grade, but correlated poorly withthe clinical stage, the latter being influenced by the common finding (25 per cent of the cases in this series) of peritoneal implants composed exclusively of mature glial tissue, which is associated with a benign clinical evolution. This phenomenon of maturation or differentiation appears to be the rule rather than the exception in this tumor, since implants are usually of better or equal differentiation when compared with their primary tumors and older patients tend to have lower grade tumors than younger patients. Since the majority of patients with this tumor are young, primary surgical therapy should be conservative, unilateral salpingooophorectomy often being sufficient. Spontaneous or operative rupture of the tumor capsule carries an increased risk of subsequent dissemination. We have noted impressive clinical responses in patients with disseminated tumors of a high histologic grade after treatment with triple chemotherapy (vincristine, actinomycin D, and cyclophosphamide) but do not recommend adjuvant therapy in patients with only grade 0 implants.

Adolescent↗

Cytogenetics of primary testicular nonseminoma, residual mature teratoma, and growing teratoma lesion in individual patients.

Residual mature teratoma (RMT) is often left behind when metastases of primary nonseminomatous germ cell tumors (NSs) are treated with chemotherapy. RMT is composed of fully differentiated somatic tissue. A growing teratoma (GTE) lesion may occur after (incomplete) resection of RMT. To shed light on tumor progression or the mechanism(s) of therapy related differentiation we investigated the chromosomal pattern of the primary NSs and RMTs in twelve patients, of the primary NS, RMT, and GTE lesion in one patient, and of the RMT and GTE lesion in two patients. Although several chromosomal differences are observed between the RMT and NSs and between the GTE and RMTs in the same patient, we obtained no evidence that specific chromosomal alteration(s) play a role in metastasis or differentiation.

Disease Progression↗

Well-differentiated adenocarcinoma arising from mature cystic teratoma of the mediastinum (teratoma with malignant transformation). Report of a surgical case.

A surgical case of teratoma with malignant transformation (TMT) of the mediastinum in a 66-year-old man is reported. The unilocular, cystic, mediastinal mass, which had been seen in a chest radiograph 7 years earlier, was extirpated. The mass was histologically revealed to be a mature cystic teratoma containing skin and respiratory epithelial tissues, with some areas of well-differentiated adenocarcinoma. Surgical cases of TMT in the mediastinum, which have not received any preoperative chemotherapy or radiation therapy, are rare. By reviewing the previous literature on TMT in various organs, the authors distinguished two types of TMT, chemotherapy- or radiotherapy-induced TMT and naturally occurring TMT. The pathogenesis of these two types are discussed.

Adenocarcinoma↗

Prenatal diagnosis of intracranial teratoma. Prolonged survival after resection of a malignant teratoma diagnosed prenatally by ultrasound: a case report and literature review.

We are reporting a case of an infant with an intracranial malignant teratoma which was diagnosed prenatally by ultrasound at 37 weeks of gestational age. After a cesarean delivery, the resection of the tumor was performed at 24 h of age. This infant is currently the oldest reported survivor that carries this prenatal diagnosis. He is also the first reported infant with surgical intervention for an intracranial malignant teratoma diagnosed prenatally.

Adrenocorticotropic Hormone↗

A complicated case of metastatic teratoma. Growing teratoma syndrome and cerebral metastasis.

All patients presenting with metastatic teratoma should be regarded as potentially curable and this case demonstrates the multiple treatment modalities which are often needed in the management of such patients. Increasing experience with cisplatin based combination chemotherapy has led to the development of prognostic factors which are used to determine the intensity of treatment given to individual patients. Surgical intervention plays a very important role in the management of residual disease at the completion of chemotherapy. Recognition of the growing teratoma syndrome and the importance of early surgical excision is illustrated by this case. Isolated CNS relapse may occur because the CNS may act as a sanctuary site in patients receiving systemic chemotherapy, but does not preclude long term disease free survival.

Antineoplastic Combined Chemotherapy Protocols↗

Systemic staging of supratentorial extra-axial brain tumors in children. Craniopharyngiomas, atypical teratoma and teratoid tumors of the suprasellar region (germinomas), and intracranial teratomas.

A staging system has been proposed for the following tumors: craniopharyngiomas, germinomas, and intracranial teratomas. Various aspects of the specific pathologic and growth characteristics of the tumor determines such categorization and subclassification of the tumors. A brief analysis of the clinical aspects in reference to classification is presented. The suggested systems will need to be tested in clinical studies to determine their validity.

Brain Neoplasms↗

Adult teratoma of the testis metastasizing as adult teratoma: case report and review of literature.

A case of adult teratoma of the testis in a 19-year-old white man is presented, The tumor metastasized to the retroperitoneal lymph nodes and the metastases were also well differentiated, benign-appearing tissues. This case suggests that even the benign-appearing components of these tumors have malignant potential. Because these tumors are malignant, they should be treated by radical orchiectomy and radical retroperitoneal dissection.

Adult↗

The origin of extragonadal teratoma: case report of an immature teratoma occurring in a prenatal brain.

We describe a massive congenital intracranial teratoma (MCIT), which had a normal chromosome banding pattern 46,XY karyotype and a normal diploid DNA histogram, and which produced a variety of carcinoembryonic antigens. The volume density of primitive neural components (primitive neural tubes, small undifferentiated neuroepithelial cells, immature glial fibers and pigment cell components without neurofibrillar differentiation) was estimated to be 45%. We discuss the histogenesis, pathobiology and cell cycle kinetics.

Adult↗

Length of follow-up in teratomas--results of a modification of Einhorn's regime in testicular teratomas.

A modification of Einhorn's original chemotherapy schedule was used to treat 40 patients with metastatic testicular teratomas. Each cycle of chemotherapy consisted of cisplatin (100 mg/m2), bleomycin (30 mg X 3) and vinblastine (5 mg/m2 X 2). Four patients failed to achieve a complete response and died with advanced disease. There was one treatment death. Only two of 14 patients who had residual masses resected still had active tumour. The 2-year actuarial disease free survival for patients in first remission was 85%, but this fell to 77% at 5 years as 3 patients relapsed (34, 36, 37 months). The results are comparable with other series but are associated with less toxicity. The need for continued follow-up is demonstrated in view of relapses occurring after 2 years.

Aged↗

[Sacrococcygeal teratomas. Apropos of a case of teratoma weighing 4200 g].

We report a clinical case of a large sacro-coccygeal teratoma diagnosed antenatally by ultra-sound at 35 weeks gestation. A review of the literature shows that their outcome depends upon the size of the tumor, its degree of maturity, whether its location is pelvic or not, the presence of any associated malformations, prematurity, atraumatic delivery and upon prompt and complete removal of the tumor.

Adult↗