A case of submandibular malignant rhabdoid tumor transformed from papillary thyroid carcinoma.

Malignant rhabdoid tumor (MRT) in the neck region is very rare. We report a case of MRT in a 60-year-old woman who had a history of papillary carcinoma of the thyroid gland 7 years previously. One year before admission, in 1995, thyroid carcinoma recurred, and the tumor contained a small undifferentiated region with rhabdoid features. The tumor in 1996 consisted of round to oval rhabdoid cells with abundant cytoplasm, and the growth pattern was diffuse and infiltrative, with no papillary structures. We therefore concluded that the lesion was MRT, transformed from papillary thyroid carcinoma.

Cytokeratin expression in squamous cell carcinoma of the lung and oral cavity: an immunohistochemical study with possible clinical relevance.

OBJECTIVE: Serologic identification of the fragment of cytokeratin 19 known as CYFRA 21-1 has been used for early detection of squamous cell carcinoma of the lung. The sensitivity of the CYFRA 21-1 assay in detecting oral cancers is lower than that in detecting lung cancers. To clarify the reason for this, we compared the cytokeratin expression in these cancers, with special reference to cytokeratin 19. STUDY DESIGN: Oral squamous cell carcinomas and lung squamous cell carcinomas were immunostained with cytokeratin 19, cytokeratin 10, and cytokeratin 13 antibodies. Staining intensity was scored on a graduated scale from 0 to 4. RESULTS: With respect to cytokeratin 19, the stainings of all lung cancers were scored as 4, which indicates a greater expression of cytokeratin 19 than is seen in oral cancers (p < 0.01). With an average cytokeratin 19 staining score of 1.67, oral cancers ranked lowest among the antibodies. Squamous cell carcinomas of the maxillary sinus arising from pseudostratified ciliated epithelium were highly expressive of cytokeratin 19. A marker for keratinizing cells (cytokeratin 10) and a marker for squamous cells (cytokeratin 13) were expressed more frequently and intensely in oral cancers (p < 0.01) than in lung cancers (p = 0.019). CONCLUSIONS: From the viewpoint of immunohistochemistry, cytokeratin 19 was found to be a tumor marker with low specificity and sensitivity in oral cancers. The staining results suggested that poor expression of cytokeratin 19 by oral squamous cell carcinoma may result in a low serum value of CYFRA 21-1 in patients with this condition.

Histological effects and predictive biomarkers of TPP induction chemotherapy for oral carcinoma.

The effects of an induction chemotherapy with THP-adriamycin, cisplatin, and peplomycin (TPP) were studied in 32 patients with operable oral cancer. The histological evaluation according to the Shimozato-Oboshi classification was Grade (G) IV in ten cases (31.3%), GIII in one case, and GIIb in four cases. Induction of apoptosis and differentiation-inducing effects, hyperkeratinization or bone formation, were observed in some cases. The overall clinical response rate and histological response rate were 63% and 47%, respectively. Grade III was obtained in seven metastatic lymph nodes of three patients. The expressions of PCNA, p53, and AgNORs before and after chemotherapy were studied. The prechemotherapeutic PCNA positive cell index (PI) of the highly responsive tumors (GIII, IV) was significantly lower than that of the poorly responsive tumors (G0-IIb) (P < 0.01). Similar results were obtained in the evaluation of p53 PI (P < 0.05), suggesting that PCNA and p53 are useful biomarkers for predicting the efficacy of TPP chemotherapy.

[Functional analysis of transforming growth factor-beta type II dominant negative receptor].

The transforming growth factor-beta (TGF-beta) is a multifunctional homodimeric protein with an apparent molecular weight of 25 KDa. TGF-beta transduces signals by forming heteromeric complexes of their type-I (T beta R-I) and type-II (T beta R-II) serin/threonine kinase receptors. TGF-beta binds first to T beta R-II receptor, and then the ligand in this complex is recognized by T beta R-I, resulting in formation of a heteromeric receptor complex composed of T beta R-I and T beta R-II. Once received, T beta R-I becomes phosphorylated in the GS domain by the associated constitutively active T beta R-II and transmits the downstream signal. It has been reported that formation of the heteromeric complex is indispensible at least in epithelial cells for growth inhibition and extracellular matrix production induced by TGF-beta. In this study, the functional role of T beta R-II for the TGF-beta-induced signals in osteoblastic cells was investigated by using a dominant negative type of T beta R-II mutant receptors (T beta RIIDNR). ROS 17/2.8 and MG 63 cells were found to express T beta R-I, T beta R-II, and T beta R-III, and their cell growth was inhibited by TGF-beta, whereas alkaline phosphatase activity was stimulated. Cells that were stably transfected with the T beta RIIDNR plasmid showed decreased response to TGF-beta during growth and alkaline phosphatase activity. These results indicate that the intracellular serine/threonine kinase domain of T beta R-II is essential for signal transduction of the TGF-beta-induced alkaline phosphatase activity as well as growth inhibition.

A case of pediatric cardiomyopathy with severely restrictive physiology.

A rare case of a 6-year-old male with idiopathic familial cardiomyopathy manifesting severely restrictive physiology is reported. The patient showed congestive heart failure with dilatation of both atria with a normal ventricular cavity. A square-root configuration was revealed in the ventricular pressure tracings. His elder brother had died of hypertrophic cardiomyopathy at the age of 3 years. Endomyocardial biopsy disclosed marked disorganization of muscle bundles with hypertrophy of the myocytes and interstitial fibrosis. The patient died suddenly during hospitalization. Autopsy revealed diffuse hypertrophy of both the ventricular walls and the ventricular septum with extensive myocardial disorganization and interstitial fibrosis. These advanced myopathic changes in the myocardium may have been related to the restrictive physiology in this case.

Treatment of cancer pain of the head and neck by continuous intravenous infusion of high-dose morphine: report of two cases.

Two pain-ridden patients in the terminal stage of a malignancy of the head and neck were treated with high-dose morphine which was administered as a continuous IV infusion along with a hyperalimentation solution using a pump. Good pain relief and a lessening of anxiety were achieved after failure to control pain in spite of the use of the analgesic ladder recommended by the WHO.

Surgical treatment of anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot.

An 8-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot, which was definitely diagnosed preoperatively, was operated on with success. Direct implantation of the left coronary artery into the aorta following division of the left coronary artery from the pulmonary artery and, concomitantly, total repair for tetralogy of Fallot using an external valved conduit were performed. Postoperative cineangiogram revealed a hemodynamically well-repaired intracardiac condition and anterograde filling of the left coronary artery, compared with retrograde left coronary flow from intercoronary collateral vessels preoperatively. To the best of our knowledge, there is not a previously published report of anomalous origin of the left coronary artery from the pulmonary artery associated with tetralogy of Fallot that was treated surgically with success.

Total one-stage repair of interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus.

Successful total repair in one stage was performed in a 3-year-old girl who had interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus. Surface-induced deep hypothermia and interrupted perfusion were used. The results of postoperative catheterization and angiocardiographic studies are analyzed, and the literature and results of previous surgical attempts at correction are reviewed.