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1.
Zhonghua Bing Li Xue Za Zhi ; 53(1): 40-45, 2024 Jan 08.
Artigo em Chinês | MEDLINE | ID: mdl-38178745

RESUMO

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of adrenal cortical carcinoma (ACC) in children. Methods: Twenty-five children with ACC diagnosed in the Department of Pathology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China from March 2014 to August 2022 were retrospectively analyzed. The related literature was reviewed. Results: A total of 25 children with ACC were collected, including 11 males and 14 females, with a male to female ratio of 1.0∶1.3. The patient ages ranged from 8 months to 14 years (median, 4 years). Eighteen cases with clinical data had functional tumors (18/22, 81.8%) presenting with virilization or precocious puberty (15/18), symptoms related to hypercortisolism (8/18) or endocrine symptoms mixed with both (5/18), while 3 cases (3/22, 13.6%) had unknown clinical data. The clinical manifestations of four patients with nonfunctional tumors were an abdominal mass and/or abdominal pain, walking instability and others. Grossly, the average maximum diameter of the tumor was 9.4 cm. Most of the tumors were nodular and partially encapsuled. The cut surfaces were gray or gray brown, soft with hemorrhage. Histologically, the tumor cells were diffusely distributed, separated by a vascular-rich network. The tumor cells were large, with distinct nucleoli, abundant eosinophilic or clear cytoplasm, and round or oval nuclei. The mitotic index was high, and atypical mitoses were common. Necrosis, calcification, capsule invasion or/and venous invasion were present. In some cases, the tumor invaded the surrounding soft tissues or kidneys. Immunohistochemically, the tumor cells were diffusely positive for syn and SF1 and focally positive for α-inhibin, Melan A and Calretinin, but negative for CgA. Ki-67 proliferation index ranged from 2%-90%. TP53 gene status was examined in 7 cases, in which mutations were detected in 4 cases. Follow-up data was obtained in 21 patients, among whom 18 received chemotherapy and 3 received radiotherapy. Distant metastasis occurred in 13 patients. Median progression-free survival (PFS) was 11.2 months and median overall survival (OS) was 54.7 months. Patients aged less than 5 years had a better prognosis for OS (P<0.05) than the older ones (≥5 years), but a similar PFS (P>0.05). Male patients and Ki-67 proliferation index <15% had a better prognosis tendency for OS, but there was no statistically significant difference (P>0.05). Conclusions: ACC in children is a rare, often functional tumor associated with Li-Fraumeni genetic syndrome and has a poor prognosis. Diagnosis and differential diagnosis require a combination of morphological, phenotypic and clinical analysis.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Criança , Humanos , Masculino , Feminino , Lactente , Estudos Retrospectivos , Antígeno Ki-67 , China
3.
Yi Chuan Xue Bao ; 27(4): 344-50, 2000.
Artigo em Chinês | MEDLINE | ID: mdl-11147354

RESUMO

The character of red petals with a large basal spot was transferred from Gossypium bickii into G. hirsutum L., and the pure lines (HBRL) with the character were obtained by Liang Zhenglan in 1988. Results of genetic analysis for G. hirsutum pure line of red petals (R3bic) and large basal spot (R2bic) were reported in the present study. The large basal spot was dominant to the small basal-spot and spotlessness of G. hirsutum. R2bic was shown to be allelic to R2 of G. hirsutum, and R2bic, R2 and r2 were multiple alleles. R2bic was found to be linked with Lc1, and linkage between R2bic and Lc1 was estimated at 37.31 +/- 2.44 centimorgans, The red petal (R3bic) was epistatic dominance to the yellow petal (Y1) and cream petal (y1), and R3bic is closely linked with R2bic. It is proposed that the introgressive gene of the large basal spot from G. bickii into G. hirsutum be assigned gene symbol R2bic, and the red petals be given the gene symbol R3bic.


Assuntos
Genes de Plantas/genética , Ligação Genética/genética , Gossypium/genética , Cor
4.
Yi Chuan Xue Bao ; 27(11): 999-1005, 2000.
Artigo em Chinês | MEDLINE | ID: mdl-11209694

RESUMO

To study the genetic character of trispecific hybrid cultivar Shiyuan 321, we analyzed this cultivar and its parents by isozyme and RAPD techniques. The main results are as follows: (1) Esterase isozyme analysis showed that there was one specific band among cultivar Suiyuan 321 and its parents, G. thurberi and G. barbadense; (2) Six specific DNA fragments amplified by 4 primers were found among cultivar Shiyuan 321 and its parents G. barbadense, G. thurberi; (3) By comparing RAPD differences among Shiyuan 321 and its parents, it is indicated that the genetic heterogeneity of cultivar Shiyuan 321 is very high at its genomic DNA level. The above results proved that Shiyuan 321 is a trispecific cultivar from G. barbadense x G. thurberi x G. hirsutum as well as a new germplasm.


Assuntos
Esterases/metabolismo , Gossypium/genética , Isoenzimas/metabolismo , Técnica de Amplificação ao Acaso de DNA Polimórfico , Hibridização Genética
5.
Yi Chuan Xue Bao ; 27(12): 1094-9, 2000.
Artigo em Chinês | MEDLINE | ID: mdl-11209702

RESUMO

To study the effectiveness of Gossypium interspecific hybrid breeding on molecular level, the six germplasm lines from interspecific hybrids between G. hirsutum and G. bickii were analysed by the techniques of RAPD and isozyme electrophoresis. The main results were summarized as follows: (1) The basic zymograms of peroxidase isozyme of the six germplasm lines were similar to their G. hirsutum parent, but two out of the germplasm lines showed one specific band of the wild parent of G. bickii (pI 4.85); (2) The RAPD fingerprint showed distinct difference of genomic DNA among the germplasm lines; (3) The four specific DNA fragments of G. bickii were observed in RAPD patters of four out of the six germplasm lines.


Assuntos
Gossypium/genética , Isoenzimas/metabolismo , Peroxidases/metabolismo , Técnica de Amplificação ao Acaso de DNA Polimórfico , Hibridização Genética
6.
Yi Chuan Xue Bao ; 27(9): 810-6, 2000.
Artigo em Chinês | MEDLINE | ID: mdl-11132497

RESUMO

Data from a set of 5-location and 2-year experiments on 10 representative historical cotton varieties and the data of Huang-Huai Regional Cotton Trials from 1973 to 1996 were analyzed to estimate the effects of genetic improvement in agronomy properties, disease resistance and stability of cotton in Huang-Huai Region in China. The results indicated that a great genetic progress of earliness and disease resistance had been achieved by breeding programs since 1950's. The maturity was shortened 3-5 days; The rate of preforst yield was increased about 7 percentages. The problem of resistance to Fususium wilt has been solved and the resistance to Verticillum wilt was improving. Some progress in stability of cotton varieties also has been achieved by breeding programs since 1950.


Assuntos
Gossypium/genética , Doenças das Plantas/genética , China , Fatores de Tempo
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