PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Neoplasms, Germ Cell and Embryonal”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources

Tumors in Rubinstein-Taybi syndrome.

The 14 tumors reported in Rubinstein-Taybi syndrome since 1989, when added to the 22 previously reported, are beginning to show a pattern of neural and developmental tumors, especially of the head, which is malformed in the syndrome. Among the neoplasms were 12 of the nervous system: 2 each of oligodendroglioma, medulloblastoma, neuroblastoma, and benign meningioma, a pheochromocytoma, and 3 other benign tumors; 2 of nasopharyngeal rhabdomyosarcoma; and 1 each of leiomyosarcoma, seminoma, and embryonal carcinoma. Among the other benign tumors were an odontoma, a choristoma, a dermoid cyst, and 2 pilomatrixomas.

Adolescent↗

Mandibular and para-mandibular tumors in children. Report of 16 cases.

Sixteen cases of mandibular tumors or paramandibular soft tissue tumors with mandibular involvement are reported. These include such rare mandibular tumors or tumor-like conditions as melanotic progonoma, intraosseous haematoma secondary to von Willebrand's disease, post-irradiation osteosarcoma, monostotic eosinophilic granuloma, aneurysmal bone cyst and osseous hemangiopericytoma. Three cases of cherubism, one of fibrous dysplasia or aggressive fibromatosis and one of central giant cell reparative granuloma are also reported. The soft tissue tumors comprise round cell sarcoma, parotid adeno-carcinoma with generalised metastases, embryonal rhabdo-myo-sarcoma, neuro-fibro-sarcoma and congenital cystic hygroma. In all the cases the disease was well advanced when the patient presented for X-ray examination. The specific X-ray diagnosis of mandibular and paramandibular tumors in childhood is more difficult than that of similar tumors in other parts of the body.

Adolescent↗

Primitive neuroectodermal tumors after prophylactic central nervous system irradiation in children. Association with an activated K-ras gene.

Three patients had supratentorial malignant brain tumors 7 to 9 years after prophylactic central nervous system (CNS) treatment for acute lymphocytic leukemia or malignant T-cell lymphoma. Therapy was administered at the age of 3 to 8 years and included cranial irradiation (total dose, 1800 to 2400 cGy) and intrathecal methotrexate. The brain tumors had histologic and immunohistochemical features of primitive neuroectodermal tumors (PNET), including neuroblastic rosettes, rhythmic arrangement of tumor cells, and immunohistochemical expression of glial, and in one patient neuronal, marker proteins. Using polymerase chain reaction-mediated DNA amplification from paraffin-embedded tissues and subsequent DNA sequence analysis, an activating point mutation was detected in the K-ras protooncogene in one tumor. This mutation was a G to A transition in position 2 of codon 12, substituting aspartate (GAT) for glycine (GGT). This type of mutation has not been observed before in human brain tumors, but it is frequent in radiation-induced murine lymphomas. These observations suggest that PNET can be induced after completion of the embryonal and fetal development of the human CNS. Oncogene-activating point mutations may represent a pathogenetic mechanism involved in the genesis of radiation-induced brain tumors.

Aspartic Acid↗

Simultaneous occurrence of melanotic neuroectodermal tumor and brain heterotopia in the oropharynx.

A case is described of heterotopic brain tissue with simultaneous occurrence of melanotic neuroectodermal tumor in the oropharynx of a 6-week-old infant. The melanotic neuroectodermal tumor was embedded within the heterotopic glial tissue. This coexistence leads us to speculate that a defect causing a pinching-off of both neural crest cap and medullary epithelium of neural tube might have taken place at, or before, the 25-30 somite stage. The displaced embryonic structures subsequently differentiated into glial tissue, choroid plexus, and melanotic neuroectodermal tumor. This observation may be interpreted as further support in favor of a neural crest origin of juvenile melanotic neuroectodermal tumor.

Brain↗

Testicular seminoma occurring 8 years after treatment of a metastatic extragonadal germ cell tumor.

A 30-year-old man was admitted with a chief complaint of left-sided scrotal enlargement, and was diagnosed as having testicular seminoma after orchiectomy. Eight years earlier, he had been treated with chemotherapy for an extragonadal germ cell tumor, without orchiectomy, leading to complete remission. His histological diagnosis at that time was a germ cell tumor, composed of choriocarcinoma and embryonal carcinoma. He was followed up without testicular biopsy. Routine pretreatment testicular biopsy in patients with extragonadal germ cell tumor is controversial, but regular long-term follow up and information on the risk of developing a metachronous testicular tumor are needed after treatment of extragonadal germ cell tumors, even when there seems to be a partial or complete clinical response.

Adult↗

Pediatric malignant ovarian tumors: a 43-year review.

Thirty-eight girls with malignant ovarian tumors were reviewed and studied during a 43-year period, 1945 to 1988 inclusive. The age range was 3 to 16 years. Eighteen girls were prepubertal and 16 were pubertal at diagnosis. Precocious puberty was noted in 4 children. The most common presenting symptoms and signs were pain, abdominal swelling, and pelvic mass. Emergency surgery for acute pain (? appendicitis) was needed in only 7 patients. Tumor size and cysts did not relate to histology or outcome. Tumors were classified histologically (germ cell, sex cord/stromal, epithelial tumors). Twenty-one (54%) of the patients survived 1 to 27 years (median, 8 years). Sixteen were left with ovarian tissue, 11 functioning. Diagnosis and treatment have been aided by better noninvasive radiological methods, tumor markers, newer and better chemotherapy, and pathological review and reclassification of these tumors as pediatric experience slowly increases. We can make at least four statements that differ from our past experience: (1) pure endodermal sinus tumor was previously confused histologically with embryonal carcinoma; (2) gonadoblastoma is associated with 45,X/46,Y, 45,X, or 46,Y karyotype and is known to be premalignant; (3) sex cord/stromal tumors are not necessarily malignant and metastatic behavior cannot be predicted from the histology; and (4) currently, epithelial tumors are classified as cystadenomas and are considered to be borderline malignancy. Current management should aim at both cure and preservation of fertility with conservative surgery whenever possible. The future must lie in chemotherapy.

Adolescent↗

Embryonal malignancies of unknown primary origin in children.

BACKGROUND: Little is known about embryonal malignancies of unknown primary origin in children. All such cases referred to a pediatric cancer center over a 30-year period were reviewed to delineate their clinical and prognostic features and to develop recommendations for evaluation and therapy. METHODS: Seventeen patients with embryonal malignancies of unknown primary origin were identified by reviewing the institutional solid tumor database. The medical records, imaging studies, laboratory records, and pathology reports for each patient were reviewed and tabulated. RESULTS: Pathology review confirmed the original histologic diagnosis in 15 of the cases. Final diagnoses after review were rhabdomyosarcoma (n = 9), neuroblastoma (n = 7), and Ewing's sarcoma (n = 1). At initial evaluation, an extensive array of radiologic studies revealed multiple abnormalities in 16 cases. Bone marrow sampling and urinary catecholamine analysis were valuable diagnostic tools. A primary site of origin was identified in five patients: by interval assessments during treatment in two cases, and at autopsy in three. Although the median survival was only 6 months, 3 patients are alive at 12, 15 and 17 years after diagnosis. CONCLUSIONS: An exhaustive search for a primary lesion appears unwarranted in children with disseminated embryonal malignancies. Tumor sampling should be adequate for the pathologic work-up to determine the definitive diagnosis. Although the chances of cure are slight, tumor specific therapy may improve the interval of disease control, the quality of life, and the possibility of long term survival.

Adolescent↗

[The synchronous appearance of multiple testicular epidermoid cysts and of a malignant mixed germinal tumor in the contralateral testis].

We describe the simultaneous appearance of multiple epidermoid cysts in the right and an adult teratoma containing embryonal carcinoma and choriocarcinoma in the left testis. No similar case has previously been described. Epidermoid cysts of the testis are rare, accounting for about 1% of all testicular tumors. Epidermoid cysts are now regarded as monoepidermally developed teratomas of germ cell origin. Testicular teratomas in adults, however, are always malignant. Because epidermoid cysts are rare tumors, primary therapy often consists in ablation of the testis. In the case described, excision of the epidermoid cyst protected the patient from complete castration. This case demonstrates the simultaneous appearance of a malignant and a benign testicular germ cell tumor.

Adult↗

Different transformation of mature teratoma in a patient with mixed germ cell tumor of the testis.

A 44-year-old male was referred with a left supraclavicular lymphadenopathy. A biopsy of the lymph node showed metastatic embryonal carcinoma. Tumor markers were present at high levels: alpha-fetoprotein 253.9 ng/mL, beta-human chorionic gonadotrophin 62 ng/mL. Computed tomography (CT) showed retroperitoneal adenopathy. High orchiectomy was done. The patient was treated with three cycles of etoposide plus cisplatin, achieved normalization of the serum tumor markers and underwent retroperitoneal lymph node dissection. Pathological findings of multiple lymph nodes showed teratomatous glands without viable cells. At follow-ups performed every 3 months, tumor markers remained within normal limits and no evidence of recurrence was observed. Eight years after first admission a CT scan revealed a cystic tumor 1 cm in diameter in the para-aortic region. The cystic tumor continued to slowly grow, expanding by 1 cm in diameter per year without elevation of tumor markers. The para-aortic tumor had grown to 4 cm in diameter and a left supraclavicular lymphadennopathy recurred. A resection of the supraclavicular cystic tumor showed mucinous cystadenocarcinoma, but a cystic tumor in the para-aortic region revealed mature teratoma. Here we report a case of mature teratoma with metastases at supraclavicular and para-aortic lymph nodes which had different transformations in spite of both regions consisting of cystic tumors.

Adult↗

A mixed tumor of the omentum majus in an adult with discussion related to hepatoblastoma.

A mixed tumor of the omentum majus found at autopsy of a 28-year-old man with a huge liver tumor is described. The primary tumor was identified in the omentum majus by macroscopic, histologic and electron-=microscopic observations. The tumor was finally diagnosed as a mixed tumor which resembled a hepatoblastoma consisting of immature and embryonal types of epithelial component and of mesodermal tumor including hemangioma, osteoid and myxomatous tissues in the primary lesion. Widespread multiple metastases of the epithelial component mainly to the liver, and partially in the lungs, diaphragm and lymph nodes were noted. The levels of alpha-fetoprotein and carcinoembryonic antigen were within normal limits, but the epithelial component showed a resemblance to primitive hepatocytes. This tumor was discussed related to hepatoblastoma in an adult, with the features compatible with description of the same tumors reported previously in the literature.

Adult↗

True malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor.

A 24-year-old Japanese man was admitted due to bloody phlegm in May 2002. A diagnosis of mediastinal germ cell tumor, mixed type involving seminoma, immature teratoma and embryonal carcinoma, was made by transthoracic needle biopsy. Three months later, his complete blood counts revealed pancytopenia with high fever. Examination of bone marrow revealed increased atypical large histiocytes (5.6%) with hemophagocytosis, and thus, hemophagocytic syndrome related to germ cell tumor was diagnosed. In addition, chromosomal analysis of the bone marrow cells revealed a 47, XY, +9 genotype. Chemotherapies for germ cell tumor and hemophagocytic syndrome were performed without any improvement, and he died of diffuse alveolar damage. Autopsy revealed diffuse infiltration of immature histiocytes with hemophagocytosis in the liver, spleen and bone marrow. The atypical histiocytes were positive for CD68 and lysozyme and negative for lymphoid markers, and the diagnosis of true malignant histiocytosis associated with mediastinal germ cell tumor was made. The rare chromosomal abnormality of trisomy 9, a marker for benzene-related leukemia, was seen in the present case without apparent benzene exposure.

Adult↗

Right intracavitary cardiac tumors. Surgical management.

The surgical treatment of 6 patients affected by right cardiac tumors is reported. Three of them showed a right atrial myxoma, 1 metastases from an occult embryonal carcinoma and 1 a leiomyosarcoma of the pulmonary artery. While clinical diagnosis was not reliable, the echocardiographic examination was able to assess the site of the tumor, supplying useful information for the anatomy and surgery in 5 cases out of 6; in one case, the diagnosis was reached intraoperatively. All the patients survived the surgical treatment: while those with atrial myxoma recovered completely, the others with malignant tumors died from metastases 5 to 10 months postoperatively. The importance of early diagnosis is stressed together with the role of surgery, which permits the recovery of cases with benign tumors and a longer survival of cases with malignant tumors.

Adult↗

The correlation of neoplastic vulnerability with central neuroepithelial cytogeny and glioma differentiation.

The vulnerability of neuroepithelial cells in the central nervous system (CNS) to neoplastic transformation results from the interaction of several factors: the existence of a reserve population of stem cells, the capability of differentiated cells to reenter the kinetic cycle, the number of replicating cells at risk at a particular time, the length of time during which a particular cell population remains in the cycle, the state of differentiation and the further differentiation potential of that population, and the steps of differentiation that are achieved in successive cell generations. This concept explains many aspects of CNS tumor incidence and the relationship of central neuroepithelial embryonal tumors to tumors of adult cell type. The incidence of different types of central neuroepithelial tumors can be correlated with the width of the window of neoplastic vulnerability. Examples illustrating the existence of only a narrow window include such rare tumors as medulloepitheliomas, cerebral neuroblastomas, gangliogliomas and ependymoblastomas. By contrast, cerebellar medulloblastomas, astrocytomas, mixed astrocytomas and oligodendrogliomas, and glioblastomas exemplify instances in which a relatively wider window of vulnerability exists in the light of cellular neuro-ontogeny and of the capacity of glial cells for postnatal replication. The relationship that may occasionally be established between the development of a glioma and the production of cellular gliosis such as may follow brain injury or accompany multiple sclerosis can also be viewed in the light of that concept. Increasing awareness is needed concerning the development of postradiation gliomas, in particular after the apparently successful treatment of acute lymphocytic leukemia.

Astrocytoma↗

Bilateral asynchronous mixed tumors of the parotid gland.

Bilateral occurrence of benign mixed tumors of the salivary glands is rare. Simultaneous involvement of the submandibular and parotid glands has been described. Multicentric foci in the parotid gland has also been reported. Only four cases of bilateral mixed tumors, occurring simultaneously and confined to the parotid glands, have been reported. We report the first known cases of bilateral asynchronous benign mixed tumors of the parotid and review the pertinent literature.

Female↗

Klinefelter's syndrome associated with testicular microlithiasis and mediastinal germ-cell neoplasm.

Klinefelter's syndrome is a genetic disorder of male sexual differentiation characterized by an XXY karyotype. Although considered a benign condition, it is associated with several types of malignancies, including mediastinal germ-cell neoplasm. In addition, Klinefelter's syndrome has been rarely associated with testicular microlithiasis. Whereas patients with Klinefelter's syndrome have an increased incidence of extragonadal germ-cell neoplasms, patients with testicular microlithiasis have a predisposition to testicular germ-cell neoplasms. To our knowledge, an extragonadal germ-cell neoplasm has not been previously described in association with testicular microlithiasis. We present a patient with 2 unusual conditions, both of which are independently associated with Klinefelter's syndrome: mediastinal germ-cell neoplasm and testicular microlithiasis.

Adolescent↗

The growing teratoma syndrome in a nongerminomatous germ cell tumor of the pineal gland: a case report and review.

BACKGROUND: The growing teratoma syndrome is a recognized complication of metastatic nonseminomatous germ cell tumors of the testis and is managed surgically. It may also occur in intracranial nongerminomatous germ cell tumors. METHODS: The authors performed an English language computer search using the EMBASE data base (from January 1980 to December 1996) for pineal tumors, read all abstracts, and then selected all articles pertaining to germ cell tumors at this site. RESULTS: The case history of a 19-year-old male who presented with a pineal nongerminomatous germ cell tumor, which was treated with chemotherapy, is reported. Despite normalization of raised tumor marker levels, the pineal mass enlarged during chemotherapy. This was excised and proved to be a mature teratoma. A review of the literature regarding this complication of intracranial germ cell tumors is also presented. CONCLUSIONS: The authors believe this to be the first reported case of growing teratoma syndrome in the pineal gland of an adult patient, two previously reported cases occurred in children. The authors conclude that the pineal gland is an unusual but important site in which to recognize the growing teratoma syndrome.

Adult↗

The international classification of childhood cancer.

The International Classification of Childhood Cancer (ICCC) updates the widely used Birch and Marsden classification scheme. ICCC is based on the second edition of the International Classification of Diseases for Oncology (ICD-O-2). The purpose of the new classification is to accommodate important changes in recognition of different types of neoplasms, while preserving continuity with the original classification. The grouping of neoplasms into 12 main diagnostic groups is maintained. The major changes are: (1) intracranial and intraspinal germ-cell tumours now constitute a separate subgroup within germ-cell tumours; (2) histiocytosis X (Langerhans-cell histiocytosis) is excluded from ICCC; (3) Kaposi's sarcoma is a separate subgroup within soft-tissue sarcomas; (4) skin carcinoma is a separate subgroup within epithelial neoplasms; (5) "other specified" and "unspecified" neoplasms are now usually separate sub-categories within the main diagnostic groups. Draft copies of the ICCC were distributed to some 200 professionals with interest and expertise in the field and their comments are considered in this final version. This classification will be used for presentation of data in the second volume of the IARC Scientific Publication "International Incidence of Childhood Cancer." A computer programme for automated classification of childhood tumours coded according to ICD-O-1 or ICD-O-2 is now available from IARC.

Bone Neoplasms↗