Radiation-induced leiomyosarcoma.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M J Klein.
Explore the source record for details and available documents.
We evaluated the growth response of 20 childhood cancer survivors who received growth hormone (GH) replacement therapy (0.3 mg/kg/week) for at least 12 months. In all subjects, GH deficiency was associated with cranial irradiation and was documented with growth charts, bone age, and somatomedin C levels; at least one GH stimulation test was available for 14 children. Pretreatment overall growth velocity was 3.3 +/- 0.5 cm/year (mean +/- SE) over a 3-year period. After GH replacement, growth velocity was 8.6 +/- 0.6 cm/year during the first year (n = 20), 7.2 +/- 0.5 cm/year during the second year (n = 17), 5.9 +/- 0.6 cm/year during the third year (n = 11), and (6.1 +/- 0.6 cm/year during the fourth year (n = 7). Growth response, tabulated by age at onset of GH replacement, was compared with the response in GH-naive children with idiopathic GH deficiency (data obtained through the Genentech Inc. National Cooperative Growth Study Summary, September 1991); the growth velocity fell within the range described for idiopathic GH deficiency adjusted for either chronological or bone age. We conclude that children with GH deficiency after cranial irradiation for neoplastic diseases respond to GH replacement therapy as well as children with idiopathic GH deficiency.
In a prospective study, the correlation between serum levels of placental isoferritin (PLF) and outcome of pregnancy was determined in 56 pregnant women. Women with contractions before the 36th week of pregnancy showed significantly lower PLF values compared with women with later contractions (p < 0.01). Furthermore, a strong correlation of PLF levels with birth weight was observed. In 11 (79%) cases with a birth weight < 2,500 g (group A), PLF values were < 10 U/ml whereas only 14% (6 of 42) of women with babies with a birth weight > 2,500 g (group B) revealed PLF levels < 10 U/ml. Because it has been shown previously that PLF has immunosuppressive properties, the secretion of PLF by the placenta could be responsible for the inhibition of the immunoreactivity of the maternal lymphocytes against the embryo. The strong correlation of low PLF values with preterm contractions and/or low birth weight recommends the determination of this protein as a marker for monitoring women with high risk pregnancies.
A 43-year-old man presented with bubbly expansion and rarefaction of the body of the seventh cervical vertebra. The lesion involved the right side of the vertebral arch and had a soft-tissue component extending mainly anterior to the vertebra. Histopathologic examination of the specimen obtained by open biopsy revealed a giant cell tumour. Giant cell tumours of the spine, excluding the sacrum, are rare. Radiographically, they may be confused with metastatic carcinoma, plasmacytoma, lymphoma, chordoma and even benign lesions, particularly aneurysmal bone cyst and brown tumour of hyperparathyroidism. However, giant cell tumour occurs mainly in younger patients, involves the vertebral body selectively in most cases and has a bubbly appearance associated with rarefaction and expansion of the vertebral body, characteristics that may be helpful in the diagnosis.
1. The purpose of the present study was the detection at the cellular scale of the oxytocin (OT) receptors involved in the facilitatory effect of this neuropeptide on its own release during the milk ejection reflex. 2. OT binding sites were demonstrated in brain sections by using a highly selective 125I-labelled OT antagonist detected by film- and histoautoradiography. 3. Film autoradiographs revealed the presence of OT binding sites in the hypothalamic magnocellular (supraoptic, paraventricular and anterior commissural) nuclei in lactating rats, suckled or not. This detection was only possible after acute i.c.v. injection of OT antagonist which probably induced an upregulation of the OT binding sites to autoradiographically detectable levels. 4. Combined application of histoautoradiographic and immunohistochemical techniques showed that the OT binding sites were concentrated on OT magnocellular neurones. Labelling concerned cell bodies and dendrites but not the axons and endings in the pituitary neural lobe. 5. The presently detected somatodendritic autoreceptors on OT neurones probably mediate the facilitatory effect of OT on its own release during the milk ejection reflex.
An exceptionally rare case of periosteal Ewing's sarcoma involving the mid shaft of the right tibia was reported. In this case, MRI was the technique that confirmed the integrity of the bone marrow. The clinical, radiographic, and histopathologic features of Ewing's sarcoma have been discussed, as has the differential diagnosis of the periosteal type of this tumor.
A case of an elastofibroma in the right scapular region in a 56-year-old woman was reported. The clinical and pathological findings were discussed. The location of this lesion was uncommon. The common sites were mentioned. The differential diagnosis and magnetic resonance findings were also described.
An extremely rare case of multicentric periosteal osteoid osteoma in a child was presented. The lesions were found to be embedded in a narrow elliptical strip, suggesting the possibility that lesional tissue was stretched in a longitudinal fashion as a result of the rapid skeletal growth. In young patients with stretched periosteal reaction who complain of pain, multicentric osteoid osteoma should be suspected. These nidi can be obscured by the exuberant periosteal reaction. Accurate evaluation of the radiographic findings is very important.
Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell deposits. In one instance the mass destroyed the posterior cortex of the left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended into the soft tissues. While the incidence of neoplastic disorder such as lymphoproliferative disease appears to be more common in Gaucher disease patients than in the general population, lesions of benign etiology that mimic these aggressive processes should be considered in the differential diagnosis when cortical destruction with coexisting soft tissue most is found in these patients.
We reviewed the magnetic resonance (MR) appearances of 13 malignant fibrous histiocytomas (MFH) of soft tissue and correlated each with the respective lesion's histopathology. The MR images were evaluated for signal intensity on T1- and T2-weighted spin echo sequences, homogeneity of the lesion, presence of internal low signal septations, and margin definition. Histologic subtypes of MFH included storiform-pleomorphic, giant cell, myxoid, and inflammatory. We could not establish a correlation between MR appearance and histopathology. Instead, our series exhibited general features suggestive of malignant soft tissue neoplasms, namely poor margin definition, internal low signal septation, and heterogeneous high signal intensity on T2-weighted images.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The nature of the hormone(s) secreted by the pars tuberalis (PT) is still unknown. This pituitary lobe is mainly formed by specific glandular cells that differ in their ultrastructural features from the other adenohypophysial cell types. Data from the literature indicate the presence of thyroid-stimulating hormone immunoreactivity in the PT-specific cells of the rat and the Djungarian hamster but not of other species, including the mouse and guinea-pig. The PT also encloses variable numbers of pars distalis cells, essentially gonadotrophs that are mainly dispersed in its caudal area. We studied the expression of the glycoprotein hormone alpha-subunit in the PT of the rat, mouse and guinea-pig by in situ hybridization and immunocytochemistry. In situ hybridization, using an oligonucleotide probe complementary to rat cDNA sequence 196-237 revealed the expression of the alpha-subunit gene throughout the PT of the rat and the mouse; in the guinea-pig, the probe labelled no pituitary cells. Light- and electron-microscopic immunocytochemistry demonstrated alpha-subunit immunoreactivity in the secretory granules of the PT-specific cells in the three species examined. These cells did not react with a specific antibody against the beta-subunit of luteinizing hormone, an antibody that labelled scattered gonadotrophs. The present data suggest that hormone(s) produced by the PT-specific glandular cells are, at least partly, related to glycoprotein hormones.
A case of parosteal osteosarcoma arising in the radius was presented. The site is uncommon, being represented in about 1% of all reported cases of parosteal osteosarcoma. The clinical and radiographic characteristics of this lesion were described and the histological as well as the differential diagnosis discussed.
A case of juxtacortical chondromyxoid fibroma in a 31-year-old man was reported. Proof of the diagnosis was obtained by an open biopsy. The histopathology of chondromyxoid fibroma was discussed at length. The radiographic appearances and the differential diagnosis were also described.
Explore the source record for details and available documents.
BACKGROUND: Adrenocortical carcinoma is a rare and aggressive disease with a poor prognosis. Adjuvant mitotane administration has been suggested as a strategy that might improve the outcome of patients with localized disease. METHODS: The authors analyzed the clinical outcome of patients with localized or regional adrenocortical cancer. The study included 19 patients who were registered at M.D. Anderson Cancer Center during a 3-year period and who had localized or regional disease at the time of surgery. Of these, eight patients received mitotane postoperatively and continued the drug until their last contact or recurrence (Group A, adjuvant); five patients began taking mitotane after surgery but discontinued it after 2-12 months for reasons unrelated to the disease (Group P, postoperative); and six patients did not receive mitotane (Group N, no mitotane). All patients have been followed for at least 12 months. RESULTS: The treatment groups differed significantly in their time to recurrence; the disease-free interval was shortest in Group A (P = 0.0055, by log-rank test). There was no statistical difference in survival among the groups, but the profile remained unfavorable for Group A. The 2-year survival rate was 100% for Groups N and P but only 43% for Group A. Of the potentially confounding factors, gender, age, steroid hypersecretion, and tumor size, none had any influence on recurrence or survival rates. CONCLUSIONS: These findings do not support the conclusion that adjuvant mitotane is beneficial in patients with localized or regional adrenocortical cancer. Neither the disease-free interval nor survival was improved by the drug. The authors suggest that alternative therapeutic strategies be explored for the management of these patients.
BACKGROUND: The prognostic significance and optimal care of children with differentiated thyroid cancer and pulmonary metastases are not well established. METHODS: Of 209 patients younger than 25 years of age who were treated at University of Texas M. D. Anderson Cancer Center between 1960 and 1990 and for whom there was sufficient information, 19 (9%) had pulmonary metastases at presentation. RESULTS: All of these patients had regional lymphadenopathy at the time of diagnosis. All but two had intense, diffuse radioiodine uptake in the lungs; there were two false-negative scans immediately after surgical procedure caused by competing thyroid residual. The chest radiograph (CXR) was normal in 8 of 17 (42%) patients with abnormal radioiodine scans. After therapy with radioiodine (100-499 mCi), CXR appeared normal in 7 of 9 patients with initial abnormal radiographs (within 6-75 months). Radioiodine uptake by the lungs normalized in 3 of 8 patients with initially normal radiographs, and in 3 of 9 patients with initially abnormal radiographs. There have been no deaths in these 19 patients. CONCLUSION: Pulmonary metastases are not uncommon in children and young adults with differentiated thyroid cancer, especially those who have regional lymphadenopathy. The lung metastases almost always concentrate radioiodine diffusely and may be associated with a normal CXR in almost half of the patients. Pulmonary metastases may be overlooked unless near total thyroidectomy is followed by total body radioiodine scan (TBS) in all children and young adults who have regional lymphadenopathy of the neck.