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Canadian Medical Association Journal Nov 1964Ataxia-telangiectasia is a syndrome of progressive cerebellar ataxia and other neurological manifestations associated with conjunctival and cutaneous telangiectases and...
Ataxia-telangiectasia is a syndrome of progressive cerebellar ataxia and other neurological manifestations associated with conjunctival and cutaneous telangiectases and with recurrent sino-pulmonary infections. Immunological and endocrine abnormalities occur. Two girls with this disease are described. The first had only minor respiratory infections; her serum proteins and immunity responses appeared normal. The second had recurrent pulmonary infections and bronchiectasis; she also exhibited sclerodermatous changes, poor development of secondary sexual characteristics with low urinary excretion of 17-ketosteroids, and lymphopenia. Autopsy at 17 years showed bilateral ovarian dysgerminomata and excessive cutaneous collagen as well as atrophy, and perhaps hypoplasia, of adrenals, thymus, spleen and lymphoid tissue (after steroid therapy). The cerebellum exhibited cortical degeneration. Both lungs were fibrotic with old and recent bronchopneumonia and bronchiectasis. The left lung was studied by injection of a latex preparation; no arteriovenous aneurysms were found, but the smaller pulmonary vessels showed some unusual morphological characteristics.
Topics: 17-Ketosteroids; Adolescent; Ataxia; Ataxia Telangiectasia; Atrophy; Blood Protein Electrophoresis; Bronchiectasis; Cerebellar Diseases; Child; Conjunctiva; Dysgerminoma; Eosinophils; Female; Genetics, Medical; Humans; Hypogonadism; Immunoelectrophoresis; Infant; Infant, Newborn; Leukocyte Count; Lymphocytes; Neoplasms, Germ Cell and Embryonal; Neurologic Manifestations; Ovarian Neoplasms; Pathology; Pulmonary Circulation; Pulmonary Fibrosis; Scleroderma, Systemic; Telangiectasis; Urine
PubMed: 14229760
DOI: No ID Found -
International Journal of Environmental... Jun 2023Nonepithelial ovarian cancers (NEOC) are a group of rare malignancies, including germ cell tumours (GCT) and sex cord-stromal tumours (SCST), along with small-cell... (Review)
Review
Nonepithelial ovarian cancers (NEOC) are a group of rare malignancies, including germ cell tumours (GCT) and sex cord-stromal tumours (SCST), along with small-cell carcinomas and sarcomas. GCTs represent 2-5% of ovarian cancers, with a yearly incidence of 4:100,000, and they usually affect young women and adolescents. Precursory germ cells of the ovary form the basis of GCT. They are histologically classified into primitive GCT, teratomas, and monodermal and somatic-type tumours associated with dermoid cysts. A primitive GCT can be either a yolk sac tumour (YST), dysgerminoma, or mixed germ cell neoplasm. Teratomas are either mature (benign) or immature (malignant). Given that malignant GCTs occur rarely compared to epithelial ovarian tumours (EOC), greater focus is required in their diagnosis and treatment. In this article, we review the epidemiology, clinical manifestations, diagnosis, and molecular biology, along with the management and therapeutic challenges.
Topics: Adolescent; Humans; Female; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Teratoma
PubMed: 37372675
DOI: 10.3390/ijerph20126089 -
Frontiers in Oncology 2023Malignant germ cell tumours are a group of rare cancers whose incidence peaks in late adolescence and early adulthood. Dysgerminomas of the ovary and seminomas of the...
Malignant germ cell tumours are a group of rare cancers whose incidence peaks in late adolescence and early adulthood. Dysgerminomas of the ovary and seminomas of the testis are analogous diseases, but seminomas have a 10-fold higher incidence. The two tumours are morphologically identical and are only differentiated by surrounding organ-specific tissue or testicular germ cell neoplasia . They share genetic features including and mutations, amplification of chromosome 12p, and expression of pluripotency markers (NANOG (Nanog homeobox), OCT3/4 (Octamer-binding transcription factor 3/4), and SAL4 (Spalt-like trascription factor 4)). Both histologies are exquisitely sensitive to platinum chemotherapy, and the combination of bleomycin, etoposide, and cisplatin (BEP) yields survival rates greater than 90%. However, BEP causes significant, lifelong toxicity (cardiovascular, renal, respiratory, and neurological) in these young patients with an expectation of cure. Here, we comprehensively review the biological features of dysgerminoma and seminoma to demonstrate that they are biologically analogous diseases. We present available clinical trial data supporting de-escalation of chemotherapy treatment. Finally, we propose that future trials should enrol men, women, and children to benefit all patients regardless of age or sex.
PubMed: 37954076
DOI: 10.3389/fonc.2023.1271647 -
CA: a Cancer Journal For Clinicians 1986Early diagnosis is the most effective means of reducing the currently high mortality rate associated with ovarian cancer. The palpation of what appears to be a normal... (Review)
Review
Early diagnosis is the most effective means of reducing the currently high mortality rate associated with ovarian cancer. The palpation of what appears to be a normal size ovary in a premenopausal woman suggests an ovarian tumor in a postmenopausal woman. Ovarian cancer should be ruled out in any woman 40 years of age or older who has persistent, unexplained GI symptoms. Ninety percent of all ovarian tumors are of epithelial origin. Treatment consists of total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and appendectomy. Instillation of P32 is optional. In stages IIb, III, and IV tumors, chemotherapy is advised; in stages I and IIa, the use of prophylactic chemotherapy must be judged on an individual basis. In children, ovarian cancer that is beyond the localized stage is one of the most frustrating of all gynecologic diseases. Total surgical extirpation of disease is the only hope for cure; for now, early diagnosis is more chance than scientific method. Thanks to better public and professional education, ovarian cancer is now being diagnosed at an earlier stage. The earlier the diagnosis, the greater the chance for cure. It is becoming obvious that ovarian cancer is a disease of the GI tract, and physicians treating ovarian cancer should be prepared to deal with bowel-associated problems. The practice of tapping women with ascites for diagnosis as well as doing an exploration merely to obtain a biopsy should be discouraged. Unless the physician is prepared to carry out the optimal surgical approach for the patient, it is crucial that the patient be referred to either a center or to a physician who is actively engaged in the day-to-day care of cancer patients. With the combined use of all the available treatment methods, patients with ovarian cancer are now living longer and more comfortably. There is also indication that their long-term survival will be increased. The one message that is important for both patients and physicians is that the gloom and doom of the 1960s and 1970s can now be replaced by a spirit of optimism.
Topics: Adolescent; Adult; Age Factors; Aged; Antigens, Neoplasm; Black People; Carcinoembryonic Antigen; Carcinoma; Child; Chromosome Aberrations; Dysgerminoma; Female; Flow Cytometry; Humans; Immunotherapy; Menopause; Mesothelioma; Middle Aged; Neoplasm Metastasis; Neoplasm Staging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Pregnancy; Pregnancy Complications, Neoplastic; Sarcoma; White People
PubMed: 3011225
DOI: 10.3322/canjclin.36.3.149 -
Case Reports in Obstetrics and... 2023Persistent elevation in beta-human chorionic gonadotropin (-hCG) following a pregnancy is concerning for gestational trophoblastic neoplasia (GTN). However, the...
BACKGROUND
Persistent elevation in beta-human chorionic gonadotropin (-hCG) following a pregnancy is concerning for gestational trophoblastic neoplasia (GTN). However, the differential diagnosis should remain broad during the evaluation process.
CASE
A 34-year-old G3P3 presented with elevated -hCG four months after cesarean delivery with bilateral tubal ligation. The patient was treated with methotrexate for a presumed new ectopic pregnancy. Due to persistent -hCG elevation, she received actinomycin-D for GTN treatment. After completing chemotherapy, her -hCG increased. The patient underwent a laparoscopic hysterectomy with unplanned left oophorectomy due to its nodular appearance at the time of surgery. Pathology confirmed a dysgerminoma of the ovary and benign uterus.
CONCLUSION
Although dysgerminomas are uncommon, they should be considered when -hCG levels remain elevated despite therapies for more common pathologies.
PubMed: 36817070
DOI: 10.1155/2023/1901858 -
The Western Journal of Medicine Jul 1982
Review
Topics: Autoimmune Diseases; Bone Diseases; Cardiovascular Diseases; Central Nervous System Diseases; Diagnosis, Differential; Dysgerminoma; Estrogens; Female; Fertility; Growth Disorders; Humans; Male; Noonan Syndrome; Ovarian Neoplasms; Risk; Testicular Neoplasms; Turner Syndrome
PubMed: 6753342
DOI: No ID Found -
The International Journal of... Mar 1993Externalization of the visceral yolk sac, after fetectomy, induces the development of extra-embryonal fetal tumors in rodents. These tumors are either benign teratomas... (Review)
Review
Externalization of the visceral yolk sac, after fetectomy, induces the development of extra-embryonal fetal tumors in rodents. These tumors are either benign teratomas that appear 3 to 4 weeks after the displacement of the yolk sac or malignant tumors, i.e. yolk sac carcinomas. The latter appear 4 to 8 months after the surgery. If however, Mouse Sarcoma Virus (MSV) is injected in the placentas at the time of fetectomy (day 12 of pregnancy) the malignant tumors develop much earlier (2 to 3 months after surgery) and some display characteristics of embryonal carcinoma. Whether virus induced or not, the yolk sac carcinomas that develop from the displaced visceral yolk sac possess the same morphological and biological characteristics. They are composed of both parietal and visceral yolk sac structures and sometimes trophoblast. The tumors metastasize, grow in ascites form and kill their host. They are readily transplantable in syngeneic rats and grow in tissue culture as an epithelial-like sheet of cells. On the other hand, the benign teratomas are composed of various well differentiated adult tissues. In these tissues, derivatives of all three germ layers are observed. Numerous experiments prove that the stem cells for these various adult tissues are not germ cells. Instead the stem cells are multipotential cells that arise in the displaced yolk sac by a process of dedifferentiation. These poorly differentiated cells originate from the endoderm of the displaced visceral yolk sac. By redifferentiation they give rise to the various adult tissues characteristic for benign teratomas. The multipotential poorly differentiated cells are also likely to be the target cells for malignant transformation. Malignant transformation of these cells, whether induced by a virus or spontaneously occurring in the displaced yolk sac, leads not only to the development of yolk sac carcinomas and eventually embryonal carcinoma but also, although rarely, to choriocarcinoma. The latter tumor is transplantable in allogeneic hosts. It is hormonally active since it secretes lactogen and progesterone. The extra-embryonal fetal tumors and in particular the rat yolk sac carcinomas and choriocarcinoma proved to be a good source for the detection of oncofetal antigens. At least two different oncofetal endodermal antigens were detected with monoclonal antibodies (mab) made after immunization with yolk sac carcinoma. Another mab, made against choriocarcinoma, was found to react specifically with the cytotrophoblast both in the normal placenta and in the tumor. No other placental cells showed a positive reaction.
Topics: Animals; Antigens, Neoplasm; Cell Differentiation; Choriocarcinoma; Dysgerminoma; Female; Immunohistochemistry; Mice; Rats; Sarcoma Viruses, Murine; Teratoma; Tumor Cells, Cultured
PubMed: 8389575
DOI: No ID Found -
Diagnostic and Interventional Radiology... Jan 2023Ovarian dysgerminoma (OD) is a rare germ cell tumor accounting for 1%-2% of all malignant ovarian tumors and is generally associated with a good prognosis. The condition...
Ovarian dysgerminoma (OD) is a rare germ cell tumor accounting for 1%-2% of all malignant ovarian tumors and is generally associated with a good prognosis. The condition is more frequent in young women and can arise in dysgenetic gonads that contain gonadoblastomas. While the definitive diagnosis of OD is only possible histologically, certain radiological features can provide facilitating clues. A large, unilateral, solid, lobulated ovarian tumor with markedly enhancing septa should raise the suspicion of OD in young women. Serum lactate dehydrogenase is characteristically elevated in this tumor type and can complement its diagnosis and postoperative follow-up; however, it is a nonspecific marker. Moreover, knowing the mimickers of OD is essential to optimizing the radiological image interpretation and allowing for adequate management and timely treatment. Therefore, in this article, the radiological and clinical-pathologic features of ODs were reviewed to allow radiologists to become familiarized with them and narrow the diagnostic possibilities when facing this type of tumor.
Topics: Female; Humans; Dysgerminoma; Ovarian Neoplasms; Neoplasms, Germ Cell and Embryonal; Radiography
PubMed: 36959710
DOI: 10.5152/dir.2022.21317 -
Journal of the National Medical... Sep 1990From 1969 to 1984, 58 patients with a diagnosis of testicular seminoma were seen and treated at the University of Kansas Medical Center. The median age was 34 years...
From 1969 to 1984, 58 patients with a diagnosis of testicular seminoma were seen and treated at the University of Kansas Medical Center. The median age was 34 years (range of 20 to 62 years). The American Joint Committee on Cancer Staging System was followed: stage I (34 patients); stage II (6); stage III (8); and stage IV (10). Forty-two patients had typical seminoma, and 16 had anaplastic histology. Nine patients had elevated B subunit of human chorionic gonadotropin, and nine had a history of cryptorchidism. Fifty-six patients received radiation treatment, and seven received chemotherapy with or without radiation. The median follow-up was 7 years (range 3 to 16 years). The overall disease-free (absolute) survival according to stage was: stage I, 91% (100%); stage II, 66% (80%); stage III, 75% (85%); and stage IV, 50% (50%). There were no late complications. The survival for patients with anaplastic histology or with elevated B subunit of human chorionic gonadotropin was not significantly different from that of typical seminoma.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Dysgerminoma; Humans; Kansas; Male; Middle Aged; Orchiectomy; Radiotherapy, High-Energy; Survival Rate; Testicular Neoplasms
PubMed: 2120460
DOI: No ID Found -
Postgraduate Medical Journal Jun 1967
Topics: Adolescent; Adult; Child; Child, Preschool; Dysgerminoma; Female; Humans; Hypogonadism; Infant; Infant, Newborn; Middle Aged; Ovarian Neoplasms
PubMed: 6043689
DOI: 10.1136/pgmj.43.500.400