Gonadal and Germ Cell Neoplasms in Children

Oncology 1 Response »
Oct 262010

Malignant germ cell tumors (GCTs) and gonadal tumors are rare, with an incidence of 12 cases per million persons <20 yr of age. Most malignant tumors of the gonads in children are GCTs. The incidence varies according to age and sex.

Sacrococcygeal tumors occur predominantly in infant girls.

Testicular GCTs occur predominantly before age 4 yr and after puberty. Testicular GCTs occur much more often in whites than in blacks, whereas ovarian GCTs have a slight predominance in blacks.

Klinefelter syndrome is associated with an increased risk of mediastinal GCTs;

Down syndrome, undescended testes, infertility, testicular atrophy and inguinal hernias are associated with an increased risk of testicular cancer.

Clinical Manifestations And Diagnosis
The clinical presentation of germ cell neoplasms depends on location. Ovarian tumors often are quite large by the time they are diagnosed. Extragonadal GCTs occur in the midline, including the suprasellar region, pineal region, neck, mediastinum, and retroperitoneal and sacrococcygeal areas. Symptoms relate to mass effect, but the intracranial GCTs often present with anterior and posterior pituitary deficits.

The serum ?-fetoprotein (AFP) level is elevated with endodermal sinus tumors and may be minimally elevated with teratomas. Infants have higher levels of AFP, which fall to normal adult levels by about 8 mo; therefore, high AFP levels must be interpreted with caution in this age group. Elevation of the ? subunit of human chorionic gonadotropin (?-hCG) is seen with Continue reading »

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Osteosarcoma in Children

Musculoskeletal System, Oncology No Responses »
Aug 312010

EPIDEMIOLOGY.

The annual incidence of osteosarcoma in the USA is 5.6 cases per million children <15 yr of age. The highest risk period for development of osteosarcoma is during the adolescent growth spurt, suggesting an association between rapid bone growth and malignant transformation. Patients with osteosarcoma are taller than their peers of similar age.

PATHOGENESIS.

Although the cause of osteosarcoma is unknown, certain genetic or acquired conditions predispose patients to development of osteosarcoma.

Patients with hereditary retinoblastoma have a significantly increased risk of developing osteosarcoma. The sites of osteosarcoma in these patients initially were thought to be located only in previously irradiated areas, but more recently they have been shown to arise in sites far from the radiation field. Predisposition to development of osteosarcoma in these patients may be related to loss of heterozygosity of the RB gene.

Osteosarcoma also occurs in the Li-Fraumeni syndrome, which is a familial cancer syndrome associated with germline mutations of the p53 gene.

The pathologic diagnosis of osteosarcoma is made by demonstration of a highly malignant, pleomorphic, spindle cell neoplasm associated with the formation of malignant osteoid and bone. Continue reading »

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Brief Summary of Tumor Lysis Syndrome

Oncology No Responses »
Aug 172010
Tumor lysis syndrome consists of hyperuricemia, hyperphosphatemia, hyperkalemia, and hypocalcemia in a patient presenting with leukemia or lymphoma, especially those with a very high white blood cell count, Burkitt leukemia/lymphoma, or T-cell leukemia/lymphoma.
Cause:
Tumor lysis can occur prior to the onset of therapy due to spontaneous blast cell breakdown, but more typically occurs after initiating therapy. Tumor lysis syndrome has rarely been reported in children with other solid tumors. In order to prevent acute renal failure, it is essential that tumor lysis be treated aggressively.
Laboratory tests:
A patient with suspected tumor lysis syndrome should have the following laboratory studies performed at the time of presentation: CBC with manual differential, and chemistry panel (to include potassium, creatinine, calcium, phosphorus, and uric acid).
Therapy:
Therapy for tumor lysis syndrome includes IV hydration at 2 or more times Continue reading »
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