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Biomedical subjects

F Bessho

Publications and source records attributed to F Bessho.

At least 91 records · Page 5Linked to original sources

11;14 translocation in childhood T-cell acute lymphoblastic leukemia.

No consistent chromosome abnormalities have been reported so far in T-cell lymphoma-leukemia. We report here two children suffering from T-cell acute lymphoblastic leukemia (ALL) with t(11;14)(q13;p11). Even though the breakpoints we claim are different from those in a recent report, we believe that their cases and ours have the same abnormality and that patients with this abnormality constitute a distinct subgroup of T-cell ALL positive for sheep erythrocyte receptor (E+) in children.

Child↗

Multiple primary malignancies in childhood cancer.

A total of 2,609 cases of childhood cancer (9,012 person-years), among which there were nine cases of second primary malignant neoplasms, were collected from six institutions. The expected number of second primary cancers was 0.88, and the observed/expected (O/E) ratio was 10.22. The estimated incidence of multiple primary cancers in children with primary cancers was 99.9 per 100,000. The tumor registry of Kanagawa Prefecture had 1,317 cases of childhood cancer with 3,326.4 person-years, among which six multiple primary cancer cases were reported. The O/E ratio was 18.4 with 180.3/100,000 person-years at risk in this series. A total of 51 cases of multiple primary cancer, including 20 synchronous cases, were reported by four major registries of childhood cancers. Frequent association with nervous tissue tumors was noted. In 17 cases the second tumor followed retinoblastoma, and most second tumors were related to radiation therapy. In the remaining 14 cases the second tumors varied; hematopoietic neoplasms in seven, thyroid carcinoma in three, and bone and connective tissue tumors in four.

Adolescent↗

Neuron-specific enolase in neuroblastoma and other pediatric tumors: a comparative nude mouse and clinical investigation.

Neuron-specific enolase (gamma-subunit isozyme) in extracts of human neuroblastomas and other tumors in children was measured by means of a radio-immunoassay in order to elucidate the rationale for clinical determination of its serum concentrations in patients. Surgical specimens as well as tumors xenotransplanted in nude mice were studied. The neuron-specific enolase (NSE) concentrations in extracts of xenografted and surgical neuroblastomas were 1,733.2 +/- 677.3 (range; 883-2,659) ng/mg protein and 1,735.3 +/- 941.1 (559-2,933) ng/mg protein, respectively, while other pediatric tumors examined showed much lower levels of NSE: 121.5 +/- 146.1 (22-374) ng/mg protein in xenografts and 17.7-610 ng/mg protein in surgical specimens. Sera of nude mice bearing human neuroblastomas also showed NSE concentrations ranging from 1,102 to 4,400 ng/ml, very much higher than those in sera of nude mice without tumors or with other pediatric tumors. These results tend to confirm the specificity of NSE for neuroblastoma, and the difference in NSE concentrations among five neuroblastoma xenografts is discussed in relation to their cytogenetic characteristics.

Animals↗

Stage IV-S neuroblastoma involving the liver and ectopic liver. Report of an unusual case.

Stage IV-S (special) neuroblastoma involving the liver and ectopic liver attached to the left adrenal gland of a 38-day-old girl was reported. At surgery, the ectopic liver with neuroblastoma metastases was excised, and the liver was biopsied. The histologic features of neuroblastoma involving hepatic tissue were quite similar in the liver and the ectopic liver. Eighteen months after surgery, the patient was doing well after treatment with less intensive chemotherapy. No similar case has been reported in the literature, and study of this case indicates that the hypothesis of fetal hematogenous metastasis proposed by Wieberdink does not suffice to explain the pathogenesis of extensive involvement of the liver in Stage IV-S neuroblastoma.

Adrenal Glands↗

[Treatment of advanced neuroblastoma with regimens containing cis-dichlorodiammineplatinum--effect and toxicity].

Ten children with advanced neuroblastoma were treated with regimens containing cis-dichlorodiammineplatinum (CDDP-regimens). Six cases had been refractory or had evidenced inadequate response to previous chemotherapy consisting of cyclophosphamide and adriamycin or vincristine, and radiotherapy. Clinical response was evaluable for 9 cases. Complete response was obtained in 2 cases, partial response in 2 cases, minor response in 2 cases, no change in 1 case and progressive disease in 2 cases. Six of these 9 cases had 2nd look surgery after several CDDP-regimens. Complete resection of tumor was possible in 1 case, subtotal resection in 4 cases and partial resection in 1 case. In an additional case whose clinical response was impossible because of absence of palpable tumor, the tumor had completely disappeared by the time of surgery. Three cases have survived for 5 months, 5 months and 28 months respectively without clinical evidence of tumor, 2 cases for 9 months and 12 months, respectively, with tumor. Four cases died of progressive tumor growth at 9 months, 10 months, 10 months, and 59 months, respectively. One case died of pneumonia at 10 months without clinical evidence of tumor. These results are far better than in our previous experience. Nephrotoxicity was observed in all cases, but it was tolerable in all cases including 3 heminephrectomized children. Hypomagnesemic, hypocalemic tetany was observed in 1 case. This complication was ameliorated by administration of magnesium gluconate. Mean +/- standard deviation of the lowest serum concentration of calcium and magnesium during this medication was 9.75 +/- 0.76 mg/dl and 1.39 +/- 0.44 mg/dl, respectively, against 7.56 +/- 1.64 mg/dl and 0.90 +/- 0.29 mg/dl without the medication, respectively. CDDP is concluded to be against neuroblastoma and its toxicity is tolerable. Magnesium gluconate administration is essential for prevention of tetany.

Abdominal Neoplasms↗

Acute nonlymphocytic leukemia showing abnormal nuclear lobulation.

An ultrastructural study of four cases of acute nonlymphocytic leukemia with abnormal nuclear lobulation was done. These cases were classified as M3 (Case 1), M3 variant (Cases 2 and 3), and M5, differentiated type (Case 4), on the basis of light microscopy according to the FAB classification. Ultrastructural observation of the leukemic cells of Cases 2 and 3 showed large bundles of fibrils in addition to abnormalities in the endoplasmic reticulum and granules, as had been reported in cases of acute promyelocytic leukemia. Large bundles of fibrils and abnormal nuclear lobulation also seemed to be characteristic of the M3 variant. However, the relationship between these structures was not identified. The exact nature of nuclear lobulation is not known, but seems to be related to some kind of intrinsic force and may be a presentation of the premature occurrence of lobulation intrinsic to the granulocyte or monocyte nucleus.

Adolescent↗

Weber syndrome caused by intracerebral hemorrhage in a hemophiliac boy.

A rare occurrence of the Weber syndrome in childhood is reported. The patient was a 7-year-old hemophiliac with recurrent intracerebral bleeding into the right cerebral peduncle and pontine base. Left hemiparesis involving the face and tongue developed and was later accompanied by right oculomotor nerve palsy.

Brain↗

Codon 201(Gly) polymorphic type of the DCC gene is related to disseminated neuroblastoma.

The deleted in colorectal carcinoma (DCC) gene is a potential tumor-suppressor gene on chromosome 18q21.3. The relatively high frequency of loss of heterozygosity (LOH) and loss of expression of this gene in neuroblastoma, especially in the advanced stages, imply the possibility of involvement of the DCC gene in progression of neuroblastoma. However, only few typical mutations have been identified in this gene, indicating that other possible mechanisms for the inactivation of this gene may exist. A polymorphic change (Arg to Gly) at DCC codon 201 is related to advanced colorectal carcinoma and increases in the tumors with absent DCC protein expression. In order to understand whether this change is associated with the development or progression of neuroblastoma, we investigated codon 201 polymorphism of the DCC gene in 102 primary neuroblastomas by polymerase chain reaction single-strand conformation polymorphism. We found no missense or nonsense mutations, but a polymorphic change from CGA (Arg) to GGA (Gly) at codon 201 resulting in three types of polymorphism: codon 201(Gly) type, codon 201(Arg/Gly) type, and codon 201(Arg) type. The codon 201(Gly) type occurred more frequently in disseminated (stages IV and IVs) neuroblastomas (72%) than in localized (stages I, II, and III) tumors (48%) (P=.035), and normal controls (38%) (P=.024). In addition, the codon 201(Gly) type was significantly more common in tumors found clinically (65%) than in those found by mass screening (35%) (P=.002). The results suggested that the codon 201(Gly) type of the DCC gene might be associated with a higher risk of disseminating neuroblastoma.

Adolescent↗

Microtubular aggregates within the rough endoplasmic reticulum of embryonal rhabdomyosarcoma cells: a case report.

This study presents a case of embryonal rhabdomyosarcoma (ERMS) of the forearm soft tissue in a 12-year-old female, in which microtubular aggregates in rough endoplasmic reticulum (rER) were noted ultrastructurally. Histologically, tumor cells consisted of typical rhabdomyoblastoid cells with abundant eosinophilic cytoplasm and relatively immature, small tumor cells. Ultrastructurally, two different types of tumor cells were also identified by light microscopy. More than half the tumor cells possessed the characteristic features of rhabdomyoblastic differentiation, such as abundant thick and thin filaments with Z-bands. The other tumor cells were less differentiated cells in which microtubular aggregates (MA) in rER were observed. MA in rER have been described in several nonepithelial tumors, including malignant melanoma, osteosarcoma, extraskeletal myxoid chondrosarcoma, and chordoma. ERMS is another example of mesenchymal tumor in which MA in rER are observed by electron microscopy. Considering the differential diagnosis among mesenchymal tumors, it is important to know that MA can be also observed in ERMS.

Actins↗