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Desmoplastic ameloblastoma featuring basal cell ameloblastoma: a case report.

The desmoplastic ameloblastoma is a histological variant of ameloblastoma. The neoplastic epithelial islands seen in desmoplastic ameloblastoma are small and ameloblastic cells are rare. Basal cell ameloblastoma is also a rare variant of ameloblastoma, in which the tumor is composed of more primitive cells and has even fewer features of peripheral palisading. This report describes the case of a 17-year-old female with an ameloblastoma in the right anterior maxilla. Orthopantomography and computed tomography showed a well-defined lesion in the right maxilla. A partial maxillectomy for tumor resection was performed under general anesthesia. Histologically, ameloblastic tumor cells were seen with dense collagenous stroma and the tumor cells showed primarily basal cell variants of ameloblastoma. After 7 years of follow-up, clinical and radiographic examinations have revealed no evidences of recurrence.

Adolescent↗

Evidence for the formation of metaplastic bone from pericytes in calcifying fibroblastic granuloma.

BACKGROUND: The calcifying fibroblastic granuloma (CFG) is a variant of epulis characterised histologically by calcified nodules and metaplastic bone formation and clinically by its pattern of frequent recurrence. Recent evidence suggests that calcified nodules and bone may form directly from non-haemopoetic pericytes in a variety of situations. This study aimed to test the hypothesis that metaplastic bone formation in CFG may be the result of a pericytic differentiation pathway. METHODS: A series of 16 cases of CFG meeting the criteria of Lee were reviewed and stained using markers which aid the identification of pericyte (smooth muscle actin, von Willebrand's factor, monoclonal antibody 3G5) and osteoblast (AML-3) lineages. RESULTS: The cells in mineralising areas of CFG resembled pericytes morphologically. Often, calcified nodules were observed within the vascular basement membrane and nodules frequently formed the nidus for reactive bone formation. The pericyte-like cells were positive for smooth muscle actin and AML-3 and were negative for von Willebrand's factor. CONCLUSIONS: These data provide circumstantial evidence for a pericytic lineage in CFG.

Actins↗

Unusual clinical presentation of Klebsiella pneumoniae induced endogenous endophthalmitis and xanthogranulomatous pyelonephritis in a non-nephrolithiasis and non-obstructive urinary tract.

The genitourinary tract is well recognized as a route through which bacteria could gain access to the blood circulation. Under some circumstances, metastatic infections may occur in distant organs, including endogenous endophthalmitis. Xanthogranulomatous pyelonephritis (XGP) is an unusual variant of chronic pyelonephritis. It most often occurs in middle-aged women who frequently have a history of recurrent urinary tract infections combined with obstruction and a kidney of poor function. We reported an unusual case of urinary tract infection in a non-nephrolithiasis and non-obstructive urinary tract complicated by Klebsiella pneumoniae endogenous endophthalmitis and developed XGP in two months.

Aged↗

Transpecific polymorphisms in an inversion linked esterase locus in Drosophila buzzatii.

Nucleotide variation was studied in a 1.1 kb section of the coding region of an Esterase gene (Est-A) that maps in the center of the segments rearranged by polymorphic inversions in the cactophilic Drosophila buzzatii. We examine 30 homozygous second-chromosome lines differing in gene arrangement and three D. koepferae isofemale lines as outgroups. Our data show that Est-A is a highly polymorphic gene at both synonymous and replacement sites. Significant departures from homogeneity in the distribution of the ratio of silent polymorphism to divergence predicted by the neutral theory reveals a local excess of silent polymorphism. This is consistent with the presence of two apparent narrow peaks of elevated silent polymorphism surrounding nonconservative amino acid substitutions. These polymorphisms as well as others at synonymous and nonsynonymous sites are shared with D. koepferae. We suggest that the presence of shared nucleotide polymorphisms is probably due to interspecific gene flow and/or balancing selection acting on replacement variants and/or to a decreased probability of loss of ancestral polymorphisms caused by linkage to an adaptive inversion polymorphism. Recurrent mutation and persistence of neutral ancestral polymorphisms cannot, however, be ruled out. The analysis of the distribution of nucleotide variation among the three chromosomal arrangements sampled reveals that derived arrangements (J and JZ(3)) are less polymorphic than the ancestral ST, and that the widely distributed ST and J arrangements are genetically differentiated. However, a significant number of polymorphisms are shared between arrangements, suggesting frequent exchange either from gene conversion or from double crossovers in heterokaryotypes. Finally, our present results in combination with data of sequence variation at the breakpoints of inversion J suggest that this old gene arrangement has risen in frequency in relatively recent times.

Animals↗

Plexiform (multinodular) schwannoma. A tumor simulating the plexiform neurofibroma.

We describe a rare variant of schwannoma characterized by a interconnecting multinodular growth mimicking a plexiform neurofibroma. The schwannoma recurred twice. The second recurrence was not excised and has not increased in size for 1 1/2 years. The patient is alive and well, 3 years and 4 months after the first excision with no clinical evidence of metastasis.

Adult↗

Patients with myocardial infarction and normal coronary arteriogram.

Eighteen patients who survived an acute myocardial infarction were found to have a normal coronary arteriogram. Seven patients were younger than 35 years and six were female. The myocardial infarction was nontransmural in 11 cases. The mean follow-up was 21.6 months. Eleven patients developed residual chest pain at rest early after myocardial infarction. One, treated by beta-blockers, suffered a recurrent myocardial infarction. Eight became asymptomatic, and two improved under antispastic therapy. Another patient developed a severe form of variant angina three months after myocardial infarction; she died following plexectomy. Finally, two patients experienced rare episodes of angina at rest. The stress ECG was negative in all cases. Provocative test for spasm was positive in three out of nine patients. Diffuse narrowing associated with chest pain was demostrated in two patients at angiography. Thus, myocardial infarction and subsequent normal coronary angiogram are mainly found in young female patients, and infarction is often nontransmural. Clinical evidence of vasospastic phenomena and increased vasomotor tone are found in most patients. Whenever residual chest pain is controlled by antispastic therapy, the follow-up course seems benign.

Adult↗

CTL adoptive immunotherapy concurrently mediates tumor regression and tumor escape.

Tumor escape and recurrence are major impediments for successful immunotherapy. It is well-documented that the emergence of Ag-loss variants, as well as regulatory mechanisms suppressing T cell function, have been linked to inadequate antitumor activity. However, little is known regarding the role of Fas-mediated cytotoxicity by tumor-specific CD8(+) CTL in causing immune evasion of Fas resistant variants during adoptive immunotherapy. In this study, we made use of an adoptive transfer model of experimental lung metastasis using tumor-specific CTL as a relevant immune-based selective pressure, and wherein the Fas ligand pathway was involved in the antitumor response. Surviving tumor cells were recovered and examined for alterations in antigenic, functional, and biologic properties. We showed that diminished susceptibility to Fas-mediated cytotoxicity in vivo was an important determinant of tumor escape following CTL-based immunotherapy. Tumor escape variants (TEV) recovered from the lungs of CTL-treated mice exhibited more aggressive behavior in vivo. However, these TEV retained relevant MHC class I and tumor Ag expression and sensitivity to CTL via the perforin pathway but reduced susceptibility to Fas-mediated lysis. Moreover, TEV were significantly less responsive to eradication by CTL adoptive immunotherapy paradigms as a consequence of increased Fas resistance. Overall, we identified that Fas(low)-TEV emerged as a direct consequence of CTL-tumor interactions in vivo, and that such an altered neoplastic Fas phenotype compromised immunotherapy efficacy. Together, these findings may have important implications for both tumor progression and the design of immunotherapeutic interventions to confront these selective pressures or escape mechanisms.

Animals↗

[Improvement of postoperative radiotherapy in the complex treatment of stage II-III cervical cancer].

Clinical data on the management of squamous-cell cervical carcinoma stage II-III FIGO are presented. At stage I, treatment included neoadjuvant polychemotherapy (NACT)--cisplatin 100 mg/m2, carboplatin 450 mg/m2 in conjunction with 5-FU 250 mg/m2 or paclitaxel 135 mg/m2. According to protocol, two identical courses of NACT were given, at 21-28 day interval, followed by extended extirpation of the uterus (Wertheim). Immediate results and complications caused by preoperative radiotherapy, important for postoperative one, were evaluated, concepts and strategies for postoperative intracavitary and distant radiotherapy worked out. To optimize radiotherapy, dosage was reduced by 10% or daily single target dose of intracavitary and distant irradiation split per 24 hrs, indications for preoperative radiotherapy variants were extended, and local dosage escalation in areas of lymphocyst or hematoma renounced. Overall survival was 85%, recurrence-free--77.5%.

Adult↗

[Criteria of aggressive rheumatoid arthritis course in children].

AIM: To ascertain criteria of aggressive course of juvenile rheumatoid arthritis (JRA) during 6-12 months since the disease onset, to measure the rate of destruction progression, to formulate indications for conduction of early immunosuppressive therapy. MATERIALS AND METHODS: The study included 30 patients (21 girls and 9 boys) aged 4-15 years with classic JRA. 25 children (76.2%) had systemic, 5 children--articular disease (23.8%). RESULTS: 50% and 20% of the examinees got the disease before they reached the age of 5 years and in puberty, respectively. Severe systemic disease in the debute was registered in 83.3% of patients, 16.7% developed primarily articular JRA. For 1 year the disease transformed into generalized and polyarticular in 43.3% and 56.7% of patients, respectively. Its activity reached the third degree in all the patients. IgG levels exceeded the age standards 1.5 times. Articular dysfunction occurred in 100% of the cases. Destruction in the joints was recorded in 37% and 92.5% of patients after 1 and 2 years of the disease, respectively. CONCLUSION: Early or in prepuberty, puberty onset of JRA, systemic variants of the debute, debute by classical seropositive RA without systemic symptoms, fast appearance of symmetric, generalized joint lesions, recurrent course with high ESR, C-reactive protein, IgG, growing joint functional insufficiency within the first year of the disease may severe criteria of aggressive course of RA in children. RESULTS: The presence of the above markers in RA patients is indication for administration of the disease-controlling drugs within the first 12 months before development of anatomic destruction and the patients' invalidation.

Adolescent↗

Early invasive cervical adenocarcinoma: its potential for nodal metastasis or recurrence.

OBJECTIVE: To investigate the potential for nodal spread or recurrence in patients with early invasive cervical adenocarcinoma. The possible application of the International Federation of Gynecology and Obstetrics (FIGO) classification (1994) to this variant was also examined. DESIGN: Retrospective observational study. SETTING: Gynaecological oncology division of Cancer Institute Hospital, Japan. POPULATION: 302 patients with FIGO Stage 0-IIB cervical adenocarcinoma treated surgically at the Cancer Institute Hospital. METHODS: Clinicopathological analysis was performed on 47 patients with early invasive cervical adenocarcinoma in whom the depth of stromal invasion was 5 mm or less. All patients underwent radical hysterectomy and pelvic lymphadenectomy. RESULTS: In 30 patients with a depth of tumour invasion of 3 mm or less, no lymph node metastasis was found, while two patients developed recurrence; one had a depth of invasion of 3 mm and a horizontal tumour spread of 3 mm, and the other had horizontal spread of more than 7 mm. In 17 patients with a depth of invasion from 3 to 5 mm, there was also no lymph node metastasis, but two patients developed recurrence; one had horizontal tumour spread of 7 mm or less, and the other had horizontal spread of more than 7 mm. CONCLUSIONS: Early invasive cervical adenocarcinoma with a depth of invasion of 3 mm or less and a horizontal spread of 7 mm or less has little potential for nodal metastasis or recurrence. It seems possible that the FIGO definition (1994) of early cervical cancer may be applicable in its present form to early cervical adenocarcinoma.

Adenocarcinoma↗

Randomized trial of lamivudine versus hepatitis B immunoglobulin for long-term prophylaxis of hepatitis B recurrence after liver transplantation.

BACKGROUND/AIMS: The long-term prophylaxis of hepatitis B after liver transplantation requires further optimization. In a randomized trial we investigated a regimen where the initially given hepatitis B immunoglobulin (HBIg) is replaced by long-term lamivudine treatment. METHODS: Twenty-four liver transplant recipients (all HBsAg-positive/HBV DNA-negative before transplantation), who had received HBIg for at least 6 months without HBV recurrence, were randomized to receive lamivudine (n = 12) or HBIg (n = 12) for 52 weeks. The efficacy criteria involved seronegativity for HBsAg and undetectable HBsAg/ HBcAg in the liver. RESULTS: Twenty-one of 24 patients completed the study without hepatitis B virus (HBV) recurrence (11 on HBIg, ten on lamivudine), while three patients became HBsAg-positive. Amongst those without HBV recurrence HBV DNA was detectable only by polymerase chain reaction, intermittently in serum and lymphocytes, and in liver specimens from six of eight patients receiving HBIg and five of seven receiving lamivudine. YMDD variant was found in four cases with no viral antigen expression. Eight patients continued lamivudine after the study and during an additional 6-22 months remained HBsAg-negative with normal graft function. CONCLUSIONS: Substitution of HBIg with lamivudine is effective for prevention of HBV recurrence in low-risk liver transplant recipients and offers a convenient and cost-effective alternative for long-term HBV prophylaxis.

Adult↗

Endocrinocarcinomas (carcinoids and their variants) of the duodenum. An evaluation of 927 cases.

This study was undertaken to supplement our previous analysis of 635 duodenal carcinoids to give both wider and different viewpoints on a larger series of duodenal endocrinocarcinomas, and to provide up-to-date clinicopathologic information regarding these neoplasms. A total of 927 cases of such neoplasms of the duodenum were collected from the Niigata Registry for gut-pancreatic endocrinomas, consisting of the carcinoid group with 897 cases, 857 typical carcinoids and 40 atypical varieties, and the variant group with 30 remaining endocrinocarcinomas expressed by various terminology. Significant statistical differences between these two groups were evident in various aspects: in sites of tumor growth, aggressiveness of invasion to the extra-duodenal structures, rates and sites (liver, lymph nodes and bone) of metastases, average tumor-size, immunohistochemistry of gastrin and serotonin, rates of recurrence, five-year survival rates and others. In addition, a close similarity between these two groups was noted in several aspects of histology, histochemistry and immunohistochemistry, rendering the variant group in a member of the carcinoid family. Regarding the carcinoid group in comparison with that in other organs may be summarized as follows: a reasonable male/female ratio of 1.39, a reasonable average age of 55.9 years, a high typical/atypical ratio of 21.4, a relatively small average tumor-size of 17.7 mm, a relatively low metastasis rate of 27.4%, a high histologic B-type dominance of 75.5%, a high postoperative survival rate of 83.3% and a reasonable incidence of the carcinoid syndrome of 3.1%.

Adolescent↗

Esophageal carcinoma. The unusual variants.

The clinical behavior and response to therapy of rare histologic variants of esophageal carcinoma are unclear. To evaluate the results of therapy in this group the records of 29 patients treated between 1949 and 1991 with primary rare histologic variants of esophageal carcinoma were retrospectively reviewed. This group represented 1.2% of 2454 cases of esophageal carcinoma treated between 1949 and 1991 and included mucoepidermoid (n = 14), small-cell (n = 12), adenoid cystic (n = 2), and carcinosarcoma (n = 1) carcinomas. Treatment for localized disease consisted of esophagectomy in five of seven patients with mucoepidermoid carcinoma, two of six patients with small-cell carcinoma, two of two patients with adenoid cystic carcinoma, and one of one patient with carcinosarcoma. Patients with stage IV mucoepidermoid carcinoma were treated predominately with radiation therapy (5/7). The majority of small cell carcinomas were treated with multiagent chemotherapy (10/12). The 1- and 3-year disease-specific survivals were 54% and 9% for mucoepidermoid carcinoma (median survival, 5 months) and 16% and 0% for small-cell carcinoma (median survival, 7 months), respectively. Patients with stage III mucoepidermoid carcinoma (median survival, 20.5 months) compared with those with stage III small-cell carcinoma (median survival, 6.2 months) had a significantly longer duration of survival (p < 0.05). Distant disease was present in 86% of patients in whom recurrence developed after esophagectomy Esophagectomy is standard therapy for localized carcinomas of the esophagus. Small-cell carcinoma appears to be a more aggressive variant of carcinoma and is most commonly treated with chemotherapy.

Adult↗

Parathyroid carcinoma in a patient with non-secretory pituitary tumor: a variant of multiple endocrine neoplasia type-I?

We report a case of pituitary adenoma in association with parathyroid carcinoma as an unusual combination of multiple endocrine neoplasia (MEN). A 48-year-old man had a trans-sphenoidal hypophysectomy and transcranial partial removal of a recurrent pituitary chromophobe adenoma followed by a course of radiotherapy in 1980. Four years later, he developed hypercalcemic crisis from a parathyroid carcinoma with invasion to the adjacent thyroid gland and skeletal muscle. A hemithyroidectomy and resection of the left lower parathyroid gland was performed. Three years later, he had local recurrence and anterior chest wall metastasis accompanied by hypercalcemia. After resection of the remnant and metastatic lesion, eucalcemia was achieved. There was no family history of endocrine tumors. This case illustrates the rare association of a malignant parathyroid tumor and a chromophobe adenoma of the pituitary as a variant of MEN syndrome.

Adenoma, Chromophobe↗

Chromosomal abnormalities in adult non-endemic Burkitt's lymphoma and leukemia: 22 new reports and a review of 148 cases from the literature.

Tumour cell karyotypes from patients with Burkitt lymphoma (BL) or Burkitt's type leukemia (ALL3) were studied for correlation with survival, bone marrow and cerebral spinal fluid involvement (CSF), human immunodeficiency virus (HIV) serology, and for recurrent cytogenetic abnormalities. The records of 22 patients with BL from our institution and of 148 cases of BL and ALL3 reported in the literature with karyotypes were evaluated for clinical and cytological features. Overall survival was only 28 per cent and 88 per cent of deaths occurred within the first nine months after diagnosis. Those who survived at least 18 months were unlikely to relapse. Age and gender did not significantly affect survival. Patients presenting with advanced Ann Arbor stage, bone marrow or CSF involvement had lower survival rates. The association of translocations involving chromosome band 8q24 with this disease is confirmed. Sixty-two per cent of karyotypes had t(8;14)(q24;q32) translocations; the recognized variant translocations t(8;22)(q24;q11) and t(2;8)(p12;q24) affected 12 per cent and 9 per cent respectively. Seventeen per cent had abnormal karyotypes but no classic translocation. Patients with variant translocations had the poorest survival rates, and those with the classic t(8;14)(q24;q32) did the best. Despite a small sample size, the variant translocation t(8;22)(q24;q11) appeared to occur at an increased frequency in the patients with AIDS. In the entire group, recurrent involvement of chromosome regions 1q2, 6q11-14 and 17p1 suggests that alteration of genes at these loci, B Cell Growth Factor (BCGF) at 1q2 and p53 on 17p, may contribute to the development and progression of this tumour. Similarly, the frequent trisomies of chromosomes 7, 8, 12 and 18 may indicate an effect on tumour cell growth due to increased gene dosage. Trisomy 12 was found in eight tumours, five from patients with AIDS, suggesting that chromosome 12 has a site or gene whose allelic dosage is selected for in AIDS related lymphoma cells. Cytogenetic studies of adult Burkitt lymphoma and leukemia suggest several likely loci for gene alterations that in conjunction with myc translocations can lead to tumorigenesis.

Acquired Immunodeficiency Syndrome↗