Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of mixed germ cell-sex cord stroma tumor occurring in a normal 8-year-old girl with 46XX karyotype is reported. The child presented with evidence of isosexual precocious puberty 3 years prior to the excision of the tumor. Although the tumor was known to be present at least 2 years prior to its excision and weighed more than 1 kg, there was no evidence of involvement of the adjacent organs or metastases at the time of excision. The contralateral ovary was normal. The endocrine activity abated following the excision of the tumor and the patient is well and free of disease 2 years after the operation. The literature concerning this entity is discussed and reviewed.
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PMID:Mixed germ cell-sex cord stroma tumor of the ovary associated with isosexual precocious puberty in a normal girl. 19 47

A case of intrasellar teratoma with a germinal structure in a 10-year-old girl is described. A few months after intracranial surgery the tumor differentiated into a choriocarcinoma and finally spread to multiple cerebral, pulmonary, and renal metastases. In the course of choriocarcinomatous evolution, very high urinary levels of luteinizing gonadotropin (HCG) developed, but there was no clinical or anatomical evidence of precocious puberty.
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PMID:Evolution of a primary intrasellar germinomatous teratoma into a choriocarcinoma. Case report. 117 Nov 63

A review of the medical literature on breast disorders in children and adolescents leads to a number of conclusions. Though females may develop breast masses early in life, the risk of malignancy is extremely low. Therefore, most breast masses in the young can be managed conservatively without surgery. Breast masses in young girls may represent thelarche or tumors of adjacent structures, but are unlikely to be malignant. The most common form of bilateral breast enlargement in prepubertal girls is premature thelarche, a benign, transient, and incomplete form of precocious puberty. Fibroadenoma is the most common cause of breast mass in female adolescents. In the rare case in which a breast mass in this population is malignant, it is more likely to be a noncarcinomatous or metastatic cancer. Because of the low risk of malignancy and the relatively different composition of the adolescent as compared to the adult breast, mammography is not recommended for routine screening or routine imaging of breast masses in adolescents. The role of breast self-examination should be further studied.
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PMID:Diagnostic considerations in breast disorders of children and adolescents. 158 47

Malignant mediastinal teratoma containing yolk sac elements are rare tumours and invariably have a poor prognosis. An elevated alpha fetoprotein level is a useful indicator of the malignant nature and the invasiveness of the tumour. A case with such a tumour who also presented with precocious puberty is described. This patient was treated with radiotherapy and chemotherapy prior to radical surgical excision, and has now survived for over 64 months without evidence of metastases.
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PMID:Endocrine secreting malignant mediastinal teratoma. 223 88

The pathological anatomy, incidence, symptomatology, diagnosis and therapy of granulosa cell tumours are described using the example of a 5 1/2-year-old girl with this condition. The typical symptoms of pseudo-precocious puberty led to preoperative diagnosis of the granulosa cell tumour. These symptoms disappeared after left salpingo-oophorectomy; the girl is now developing normally. There have been no signs of metastases as yet.
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PMID:Granulosa cell tumour in a child. 634 79

A previously healthy boy, aged 12 years, developed hypopituitarism due to suprasellar metastases from a pineal germinoma with syncytiotrophoblastic giant cells (SGC). Despite the hypopituitarism, the patient showed pubertal development, which was associated with abnormal levels of human chorionic gonadotropin (hCG) in the cerebrospinal fluid. This case adds support to the theory that precocious puberty in children with pineal germinomas may be due to the secretion of hCG by SGC, a minor component of the germinoma. It also illustrates the point that, although abnormal levels of circulating hCG in children with pineal tumors may indicate a diagnosis of choriocarcinoma with its encumbent poor prognosis, other pineal germ cell tumors may also secrete hCG. Although this patient had abnormal levels of hCG, his cerebral tumor exhibited behavior more commonly associated with pineal germinoma than with choriocarcinoma.
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PMID:Pineal germinoma with syncytiotrophoblastic giant cells: a case with panhypopituitarism and isosexual pseudopuberty. 669 44

Malignant germ cell tumors are an uncommon type of ovarian cancer which account for fewer than 5% of the total in Western countries and 20% in Japan. In females younger than 20, they represent approximately two-thirds of malignant ovarian tumors. Immature teratoma, endodermal sinus tumor, dysgerminoma and mixed type account for the majority (more than 80%), while embryonal carcinoma and polyembryoma are very few. The age of the patients ranges from 6 to 69 years with a median of 16-20 years. Clinically, these tumors are characterized by rapid growth and extensive intraabdominal spread. The symptoms and signs range from 1 day to 6 months with a median of 4 weeks, and the patients usually present with abdominal pain, palpable mass, abdominal distention and vaginal bleeding, and a very few with amenorrhea and precocious puberty. The size of tumors varies from 7 cm to 40 cm with a median of 15-16 cm. The tumor is rarely bilateral (12-19%) and never so in cases of endodermal sinus tumor. Diagnosis depends mainly on age, abdominal symptoms, size and consistency of the tumor, and tumor markers AFP and hCG. Surgery is the first step of management followed by adjuvant therapy, which depends on the histologic type. Dysgerminoma is very sensitive to radiation while other germ cell tumors are not. A combination chemotherapy currently used is VAC or VBP. Both are highly effective. The VBP regimen seems to have a stronger cancerocidal effect, while the VAC regimen is less toxic. VAC produces excellent results in stage I, while VBP is more effective for advanced disease. Conservative surgery and a combination chemotherapy (VAC, VBP) are appropriate for young patients who desire to retain their fertility. Second-look laparotomy is still controversial. As long as AFP or hCG or both can be used to monitor the disease in patients positive for these sensitive and reliable markers, or in an early stage with complete resection, second-look laparotomy is not useful. Survival is associated with prognostic factors, i.e., histologic type, clinical staging operation, lymph node and residual tumor. Patients with endodermal sinus tumor or mixed type tumor had a poorer outcome. The survival rate was higher in patients with earlier disease (stage I or II) and those who underwent primary surgery. Metastasis to the lymph node is not related to prognosis. The presence and size of residual tumors after surgery were closely related to the prognosis.
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PMID:Current management of malignant germ cell tumor of the ovary. 766 94

Paraneoplastic manifestations are signs and symptoms observed in patients with cancer, distant from the tumour or its metastases and not caused by invasion, obstruction or bulk mass. In children with cancer, paraneoplastic manifestations are rare and distinct from those observed in adults. Knowledge about paraneoplastic manifestations can be of great clinical importance because they may be the presenting sign of a tumour or its recurrence and hence facilitate early diagnosis. In contrast, they sometimes mask the symptoms of a tumour and cause diagnostic delay. In this review, paraneoplastic manifestations in children are described, including hypercalcaemia, Cushing syndrome, precocious puberty, opsoclonus/myoclonus, acquired von Willebrand disease, watery diarrhoea syndrome, and hypertension. The mechanisms causing these manifestations are also discussed.
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PMID:Paraneoplastic manifestations in children. 784 90

Ovarian granulosa cell tumors (GCTs) are uncommon neoplasms that arise from the sex-cord stromal cells of the ovary. GCTs are characterized by long natural history and their tendency to recur years after the initial diagnosis. They present with symptoms and signs due to estradiol secretion, including vaginal bleeding and precocious puberty. Occasionally, tumor rupture causes abdominal pain and hemoperitoneum. GCT is usually associated with a mass on pelvic examination which is subsequently confirmed with imagine techniques. Surgery is the mainstay of initial management for histological diagnosis, appropriate staging and debulking surgery. In patients with stage I disease and those in reproductive age a more conservative unilateral salpingo-oophorectomy is indicated. In postmenopausal women and those with more advanced disease a total abdominal hysterectomy with bilateral salpingo-oophorectomy is the appropriate surgical treatment. The most important prognostic factor associated with a higher risk of relapse is the stage of disease. The role of post-operative chemo- or radiotherapy in stage I disease and those with completely resected tumor has not been defined. Nevertheless, the use of adjuvant chemotherapy or radiotherapy has sometimes been associated with prolonged disease-free survival and possibly overall survival. Chemotherapy should be considered for patients with advanced, recurrent or metastatic disease and the BEP (bleomycin, etoposide, cisplatin) is the currently preferable regimen. Although overall response rate (RR) is high, the impact on disease-free or overall survival is unknown. Due to their tendency to recur years after the initial diagnosis, prolonged surveillance is essential.
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PMID:Granulosa cell tumor of the ovary. 1794 23

Granulosa cell tumors of the ovary are rare neoplasms that originate from sex-cord stromal cells. The long natural history of granulosa cell tumors and their tendency to recur years after the initial diagnosis are the most prominent of their characteristics. The secretion of estradiol is the reason for signs at presentation such as vaginal bleeding and precocious puberty. Abdominal pain and hemoperitoneum, which occasionally can occur, are attributable to tumor rupture. The most common finding in pelvic examination is a tumor mass, which is subsequently confirmed with imaging techniques. Surgery is the mainstay of initial management for histological diagnosis, appropriate staging, and debulking. A more conservative unilateral salpingo-oophorectomy is indicated in patients with stage I disease and patients of reproductive age. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is the appropriate surgical treatment for postmenopausal women and those with more advanced disease. The stage of disease is the most important prognostic factor associated with the risk of relapse. There are no clear conclusions regarding the role of postoperative chemotherapy or radiotherapy in stage I disease and in those with completely resected tumor. The use of adjuvant chemotherapy or radiotherapy has sometimes been associated with prolonged disease-free survival and possibly overall survival. Chemotherapy is the treatment of choice for patients with advanced, recurrent, or metastatic disease, and BEP (bleomycin, etoposide, and cisplatin) is the preferred regimen. Although the overall rate of response to treatment is high, the impact of treatment on disease-free or overall survival is unknown. Prolonged surveillance is mandatory because tumors tend to recur years after the initial diagnosis.
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PMID:Granulosa cell tumor of the ovary: tumor review. 1881 51


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