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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 -
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 -
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 -
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 -
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 -
Cancer Biology & Therapy Aug 2018Dysgerminoma is an uncommon malignant tumor arising from the germ cells of the ovary. Its association with pregnancy is extremely rare; the incidence is about 0.2-1 per... (Review)
Review
BACKGROUND
Dysgerminoma is an uncommon malignant tumor arising from the germ cells of the ovary. Its association with pregnancy is extremely rare; the incidence is about 0.2-1 per 100,000 pregnancies. Because of its infrequency, there are few recommendations regarding its management in pregnancy; therefore, it is important to discuss and summarize the treatment strategy.
CASE
We presented a case of a 23-year-old pregnant woman with a large dysgerminoma originated from the right ovary, which had the unusual coincidence of being associated with an abdominal desmoid tumor simultaneously. We did not find any similar cases published in the PubMed database after 1947. A cesarean section was performed at 34 + 6 weeks gestation secondary to her abdominal pain worsening. The patient delivered a healthy boy and had fertility-preserving surgery, followed by 6 cycles of chemotherapy. This case is compared with 21 other reported cases of pure ovarian dysgerminoma in the literature to evaluate the clinical characteristics, feto-maternal compromise, treatment, long-term survival, and fertility outcome.
CONCLUSION
The treatment strategy in women with ovarian dysgerminoma should be discussed and structured on an individual basis. If pregnancy is desired, surgical intervention undertaken in the second trimester seems to be the first choice. When chemotherapy is indicated, unless delivery can be accomplished within a few weeks of diagnosis, it should not necessarily be delayed until after delivery. Good reproductive function and high survival rate can be achieved in patients treated with conservative surgery and adjuvant chemotherapy.
Topics: Adult; Biomarkers, Tumor; Biopsy; Combined Modality Therapy; Dysgerminoma; Female; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Ovarian Neoplasms; Pregnancy; Pregnancy Complications, Neoplastic; Treatment Outcome
PubMed: 29580145
DOI: 10.1080/15384047.2018.1450118 -
Medicine Oct 2020Dysgerminoma is an extraordinarily rare neoplasm arising from the malignant germ cells of the ovary. Early antenatal diagnosis and proper management of the neoplasm to... (Review)
Review
RATIONALE
Dysgerminoma is an extraordinarily rare neoplasm arising from the malignant germ cells of the ovary. Early antenatal diagnosis and proper management of the neoplasm to improve maternal-neonatal results are the considerable challenges facing the gyne-oncologist. We summarize the clinical features and discuss treatment strategies of the ovary dysgerminoma (OD). Besides, we also review the literature on OD in PubMed, Web of Science Core Collection, Library of Congress, and LISTA from 1939 to 2019 to evaluate its clinical characteristics, feto-maternal compromise, management, and fertility outcome.
PATIENT CONCERNS
A 25-year-old pregnant woman reported lower abdominal pain and vomiting.
DIAGNOSIS
The patient was diagnosed as right OD.
INTERVENTIONS
She received a cesarean section due to severe abdominal pain, delivered a healthy girl at 38 C 4 weeks of gestation, and accepted fertility-preserving surgery. However, the patient refused chemotherapy postoperatively.
OUTCOMES
The patient was followed up 42 days, 3 months, and 6 months after surgery, and no tumor recurrence was observed.
LESSONS
OD has non-specificity characteristics, including age, symptoms, image date, and tumor marks. However, these abnormal indicators may provide some evidence for accurate antenatal diagnosis. The management strategies should be considered comprehensively on an individual basis, and fertility-preserving surgery should be carried out in the second trimester if further pregnancy is desired. Adjuvant chemotherapy needs to be applied to the treatment of OD patients with The International Federation of Gynecology and Obstetrics (FIGO) stages II, III, and IV and timely chemotherapy is suggested if there are several weeks before the expected date of delivery. The overall prognosis of OD patients is excellent.
Topics: Adult; Cesarean Section; Dysgerminoma; Female; Humans; Ovarian Neoplasms; Pregnancy; Pregnancy Outcome
PubMed: 33031254
DOI: 10.1097/MD.0000000000021214 -
JNMA; Journal of the Nepal Medical... Nov 2022Dysgerminoma is the most common malignant germ cell tumour of the ovary. Abdominal pain, abdominal distention, and the presence of a palpable mass are common symptoms at...
UNLABELLED
Dysgerminoma is the most common malignant germ cell tumour of the ovary. Abdominal pain, abdominal distention, and the presence of a palpable mass are common symptoms at presentation. This is usually detected in youth, before the age of 20 years. Ovarian or adnexal tumours are very rare in patients below the age of 18 years, most of them being functional cysts, only 10% being malignant. Here is a rare case of an 8 years old girl with dysgerminoma who underwent right-sided salpingo-oophorectomy for unilateral involvement with conservation of fertility and now the patient is on chemotherapy as the tumour metastasized to the pre-aortic lymph node.
KEYWORDS
case reports; dysgerminoma; metastasis; paediatrics.
Topics: Child; Female; Humans; Abdominal Pain; Dysgerminoma; Lymph Nodes; Ovarian Neoplasms
PubMed: 36705173
DOI: 10.31729/jnma.7894 -
SAGE Open Medical Case Reports 2022We report on the case of a patient with dysgerminoma, a rare germ cell tumor, which showed hypercalcemia with an elevation of 1α,25-dihydroxycholecalciferol...
We report on the case of a patient with dysgerminoma, a rare germ cell tumor, which showed hypercalcemia with an elevation of 1α,25-dihydroxycholecalciferol (calcitriol). A 27-year-old nulliparous woman presented with hypercalcemia during the examination of a right ovarian tumor with an elevation of calcitriol, lactate dehydrogenase, and alkaline phosphatase. Fractional excretion of calcium was elevated, and intact parathyroid hormone was suppressed. After undergoing right salpingo-oophorectomy, the patient's serum calcium and calcitriol returned to the normal range within a week. A literature search was conducted on the topic by reviewing databases for dysgerminoma showing hypercalcemia. We identified 14 patients from the literature and performed a pooled analysis, including the results of our case. However, most cases lack data that can help investigate the potential association between parathyroid hormone, parathyroid hormone-related protein, calcitriol, and phosphorus in hypercalcemia. Thus, more case reports that include additional information are required to fully elucidate the mechanism of hypercalcemia associated with dysgerminoma.
PubMed: 35070316
DOI: 10.1177/2050313X211068562