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PubMed1997

Vascular endothelial growth factor expression in early stage ovarian carcinoma.

BACKGROUND: Tumor angiogenesis is essential for solid tumor growth. Yet, the importance of any particular factor in neoplastic proliferation is poorly defined. This study examines the clinical significance of increased expression of one of the angiogenic factors, vascular endothelial growth factor (VEGF), in early stage ovarian carcinoma. METHODS: Tumor specimens from 68 patients with International Federation of Gynecology and Obstetrics Stage I and II ovarian carcinoma were evaluated for VEGF expression. Antisense and corresponding sense (control) RNA probes were transcribed from the pCRII construct (Invitrogen, San Diego, CA), which contained human VEGF cDNA. The antisense probe was designed to include a highly conserved region of the VEGF coding sequence and thus detect all known variants. After in situ hybridization, sections were assessed for overexpression of VEGF. RESULTS: Twenty-nine of the tumor samples overexpressed VEGF, whereas 39 specimens did not. In patients whose tumors demonstrated elevated VEGF expression, 25% were without evidence of disease recurrence at last follow-up. In contrast, 75% of the patients whose tumors did not overexpress VEGF were without evidence of disease at last follow-up (P < 0.001). Median disease free survival for the VEGF positive group was 22 months, compared with > 108 months for the VEGF negative group (P < 0.001). When borderline tumors were excluded from the survival analysis, median disease free survival for the VEGF positive group was 18 months, compared with >120 months for the VEGF negative group (P < 0.001). Other possible prognostic variables had minimal impact on survival; these included age, stage, grade, cytology, and tumor size (P > 0.05). Assignment to a high risk group, as defined by the Gynecologic Oncology Group of the National Cancer Institute, was somewhat predictive of a shorter relapse free interval (P = 0.056). In a multivariate analysis, however, only elevated VEGF expression was associated with poorer survival. CONCLUSIONS: In this analysis, patients with early stage ovarian carcinoma with increased VEGF expression had a poorer prognosis. Further study of VEGF may ultimately lead to identification of patients with high risk lesions whose tumor biology portends a worse prognosis and who therefore may benefit from aggressive adjuvant therapy.

Adult↗
PubMedDate not supplied

Childhood lichen planus: a report of 23 cases.

Lichen planus (LP) in children is a rare entity. We report 23 cases of childhood LP seen over a period of 7 years. Ninety-six percent of the children were of Arab ancestry. There were 52% boys and 48% girls. Classic LP was the most common clinical variant (70%), followed by eruptive generalized LP (13%). A majority of the patients had mild, localized disease. Oral involvement was seen in 39% of patients. Topical steroids were the mainstay of treatment in most of the cases. Children with chronic and recurrent disease responded to dapsone therapy, whereas in those with eruptive and widespread disease, UVB phototherapy was found to be safe and effective. The present report highlights the salient clinical features, treatment, and course of LP in children in Kuwait compared to those reported in children of other countries as well as those of adults.

Administration, Topical↗
PubMed1991

Myoepithelial lesions of the breast. Myoepitheliosis, adenomyoepithelioma, and myoepithelial carcinoma.

The clinical and pathologic features of 31 breast lesions composed of a prominent proliferation of myoepithelial cells either admixed with epithelial cells or in pure form were studied. The lesions were divided into three categories: myoepitheliosis, adenomyoepithelioma, and malignant myoepithelioma (myoepithelial carcinoma); the latter is the only lesion composed purely of myoepithelial cells. Three multifocal, microscopic lesions located in the peripheral duct system were designated as myoepitheliosis. Twenty-seven solitary, grossly palpable, predominantly centrally located lesions qualified as adenomyoepithelioma. These were further subdivided into spindle-cell, tubular, and lobulated variants. Two lesions in the latter group had a carcinoma arising within them. Only one case, which was characterized by a solitary mass composed of an infiltrative spindle cell proliferation, qualified as malignant myoepithelioma (myoepithelial carcinoma). Two patients with adenomyoepithelioma developed recurrences; one tumor was of the tubular type, the other of the lobulated type. Both of these tumors had irregular margins. One of these patients had two recurrences and is currently well 8.5 years after the initial excision. The second patient developed a recurrence 8 months after initial excision; the recurrence presented as multiple nodules. One of the patients with myoepithelial carcinoma arising in an adenomyoepithelioma also developed a recurrence within 2.3 years. Her initial tumor was located in the axillary tail of the breast, and she had axillary node metastasis at the time of presentation. All remaining patients with follow-up are well without evidence of recurrence up to 17.3 years after the initial diagnosis (average follow-up, 6.1 years); one patient died of unrelated causes.

Actins↗
PubMed2002

A longitudinal analysis of T-cell epitope-coding regions of hepatitis C virus after liver transplantation.

BACKGROUND: Hepatitis C virus (HCV)-related liver failure is the single leading indication for orthotopic liver transplantation (OLT) worldwide. The mechanisms that underlie the observed differences in natural history of HCV recurrence remain poorly understood. We have previously demonstrated that differential T-cell responses correlate with histologic severity after OLT. We hypothesized that amino acid substitutions within critical T-cell epitopes could lead to increased severity of HCV disease. METHODS: We determined the peptide sequences from sequential serum-derived viral RNA by reverse transcription and direct polymerase chain reaction sequence analysis from 32 HCV genotype 1-infected patients with well-characterized outcomes after liver transplantation. Serum samples were analyzed for HCV sequence the day of OLT and at least one time point post-OLT. To construct evolutionary relationships among the different patient samples, phylogenetic analyses of core and NS3 sequences were performed using a matrix fed into a neighbor-joining tree algorithm. RESULTS: The phylogenetic analyses revealed remarkable conservation within a given individual and no significant differences when comparing patients with severe versus mild recurrence. Accordingly, the synonymous mutation rate was consistently greater than the nonsynonymous substitution rate. The nine epitopic regions analyzed were also preserved so that, with the exception of one patient with mild recurrence, none of the patients demonstrated a shift in viral peptide sequence. CONCLUSIONS: HCV core and NS3 viral peptide sequences are identical before and after OLT in most patients, suggesting that the prevalent sequence is preserved in most cases, and viral variants are competent to establish infection after OLT. Although these results do not support viral mutation as a dominant pathogenic mechanism after OLT, other viral regions need to be analyzed.

Adult↗
PubMed1987

Pancytopenia secondary to primary malignant lymphoma of bone marrow as the first hematologic manifestation of the Estren-Dameshek variant of Fanconi's anemia.

We describe a 4-month-old girl with Estren-Dameshek variant of Fanconi's anemia (FA) presenting with a pancytopenia secondary to extranodal bone marrow malignant lymphoma as the first hematologic manifestation of the syndrome. Although she was phenotypically normal, her cultured lymphocytes showed an increased spontaneous chromosome breakage, which was enhanced by diepoxybutane (DEB). Cytogenetic studies showed that 7 of her 12 relatives, including both parents, were heterozygote carriers of the FA gene. This confirms the diagnosis of FA of the Estren-Dameshek variant. A complete hematologic remission was attained within 28 days, and she remained in continuous bone marrow remission till the age of 14.5 months, when she developed acute gastroenteritis and died. No unusual sensitivity to chemotherapy was experienced, though leukopenia and recurrent infections proved troublesome. FA has an incidence many times higher in the Orange Free State region of South Africa than reported elsewhere in the world.

Anemia, Aplastic↗
PubMed2006

Cytology of chordoid meningioma: a series of five cases with emphasis on differential diagnoses.

BACKGROUND: Chordoid meningioma is a rare meningioma variant characterised by epithelioid cord-like tumour cells in a myxoid stroma. It is classified as grade II (World Health Organization) tumours, as they have a tendency to behave more aggressively than traditional meningiomas and have a greater likelihood of recurrence. AIMS: To report the features of intraoperative imprint smears of five cases of chordoid meningioma. METHODS: The intraoperative squash smears were reviewed for cellularity, cellular atypia, mitotic figure, cytoplasmic vacuolation, intranuclear inclusion, presence of a cohesive cord of tumour cells, whorl-like structure, psammoma bodies, chronic inflammatory cells (lymphocytes and plasma cells), background mucin and necrosis. RESULTS: All cases were of moderate to high cellularity, with cohesive cords of bland tumour cells possessing uniformly round nuclei with smooth nuclear outline, stippled chromatin and small nucleoli, with cytoplasmic vacuolation and chronic inflammatory cells in the background. Intranuclear inclusions (80%) and whorl-like structures (60%) were also common. Necrotic background, psammoma bodies or mitotic figures were consistently absent. CONCLUSIONS: The cytological features of chordoid mengiomas are distinctive, and intraoperative imprint diagnosis is feasible.

Adult↗
PubMed2003

A family with seizures and minor features of tuberous sclerosis and a novel TSC2 mutation.

The authors studied nine members of a family that demonstrated a limited form of tuberous sclerosis complex (TSC). Cutaneous findings were limited to hypopigmented macules in four patients. Five family members had recurrent seizures, and three of these had migrational defects of the cerebral mantle. Mutational analysis of TSC2 indicated the presence of the novel missense change 3106T-->C, 1036S-->P in all family members with seizures. The findings suggest that this mild variant form of TSC is due to a novel TSC2 mutation.

Adolescent↗
PubMed1985

CT evaluation of the pelvis after hemipelvectomy.

From examination of 69 pelvic CT scans from 25 patients, the role of CT after hemipelvectomy is examined. A brief description of the various types of hemipelvectomy, including simple, extended, compound, and modified, is presented. The new variants of internal and modified internal hemipelvectomy are also included. The normal post-surgical changes in the musculoskeletal structures and pelvic viscera are described and illustrated. Postoperative abnormalities detected included hematoma, inflammatory changes, incisional hernia, ureteral obstruction, adenopathy, and local recurrence. An example of the usefulness of CT in CT-guided biopsy and CT-guided thermocouple placement is provided. CT of the pelvis is advocated as the method of choice to follow these patients, because of its ability to detect postoperative recurrence and complications.

Adolescent↗
PubMed2001

Medulloblastomas of the cerebellopontine angle.

It is unusual for a medulloblastoma to occur in the cerebellopontine angle (CPA). Four of the total 63 cases of medulloblastoma managed in our department between 1988 to 1998, presented with a mass in the cerebellopontine angle. There were two children amongst these four patients, aged eight and nine years. The other two patients were young adults. Three patients had primary CPA medulloblastoma, while the fourth case had a recurrence in this region, following the excision of the primary vermian tumour. All the four cases were managed by gross total microsurgical excision and craniospinal irradiation. One of the two children, developed subfrontal recurrence after 1 year and 8 months of initial treatment, while the other child developed a large recurrence at primary site, within three months of post-operative irradiation. However, the adult patient developed CPA recurrence and subsequent cervical intramedullary metastasis within two and a half years of diagnosis. These three cases succumbed to their disease, between three months to thirty months of their initial management. However the fourth patient, a 20 years female with desmoplastic variant, was well at two and a half years of follow up.

Adult↗
PubMed1990

[A study of the efficacy of new methods of laboratory diagnosis of pseudotuberculosis].

A total of 188 patients with sporadic pseudotuberculosis were examined with the use of new rapid methods for serologic indication, i.e. enzyme immunoassay, antibody neutralization test. Combined examinations of the patients' blood serum, urine, saliva, and coprofiltrate samples have detected Y. pseudotuberculosis (serologic variant 1) antigens in 175 (93.1%) patients. Coprofiltrate and saliva examinations were found the most effective if carried out over the febrile period and 5 days after it. The methods' efficacies were still high when the patients were examined during disease recurrence. Enzyme immunoassay has proved to be the most sensitive and specific technique.

Adolescent↗
PubMed1989

[Immediate and late results of selective proximal vagotomy in pyloroduodenal ulcers].

Selective proximal vagotomy (SPV) was performed in 435 patients whose age ranged from 16 to 80. The isolated variant of SPV was applied in 130 and SPV with a stomach draining operation was conducted in 305 patients; 320 patients were examined after the operation in follow-up periods of 7 years and more. Postoperative lethality was 0.6%. A recurrent ulcer was revealed in 4.3% of patients. The causes of the recurrence are indicated. The results were found to be excellent and good in 76%, satisfactory in 16.9%, and poor in 7.1% of the examined patients.

Adolescent↗
PubMed1996

Basal cell adenocarcinoma of minor salivary and seromucous glands of the head and neck region.

Basal cell adenocarcinoma of salivary glands is an uncommon and recently described entity occurring almost exclusively at the major salivary glands. This report provides an overview of the clinicopathologic profile of this neoplasm by including the personal experience on the clinical features, microscopic and ultrastructural characteristics, proliferation activity, and DNA tumor patterns of 12 lesions occurring at the minor salivary glands of the head and neck region, where basal cell adenocarcinoma is probably an underecognized entity, previously reported under different designations. Basal cell adenocarcinoma predominates at the seventh decade without sex preference. The tumors affecting the minor salivary glands occur most frequently at the oral cavity (jugal mucosa, palate) and the upper respiratory tract. The prevalent histologic tumor pattern is represented by solid neoplastic aggregates with a peripheral cell palisading arrangement frequently delineated by basement membrane-like material. The neoplastic clusters are formed by two cell populations: the small dark cell type (that predominates) and a large cell type. Necrosis, either of the comedo or the apoptotic type, is a frequent finding. Perineural growth occurs in 50% of the cases and vascular permeation in 25%. Immunohistochemistry identifies a dual differentiation with a reactivity pattern indicative of ductal epithelial and myoepithelial differentiation, which can be confirmed by electron microscopy. The differential diagnosis of the neoplasm includes its benign counterpart, the basal cell adenoma, solid variant of adenoid cystic carcinoma, undifferentiated carcinoma, and basaloid squamous carcinoma. The tumors recur more frequently than lesions originating in major salivary glands. Mortality is associated with the anatomic site of the lesion, advanced stage, residual neoplasia at surgery, and tumor recurrence. The importance of recognizing basal cell adenocarcinoma outside major salivary glands is related to the clinical behavior of the neoplasm that seems to be less indolent than that of tumors arising in major salivary glands.

Adenocarcinoma↗
PubMed1996

Giant polypoid basal cell carcinoma.

Basal cell carcinomas may attain giant proportions due primarily to recurrence and neglect. Giant basal cell carcinomas (5 cm or more in diameter) are of four clinical subtypes: noduloulcerative, morpheaform, superficial, and polypoid. We report a patient with a typical polypoid lesion of fifteen years' duration on his shoulder. The polypoid variant differs from other giant basal cell carcinomas in several important ways: the polypoid lesions appear on the torso or extremity, rather than the head or neck, as beefy-red, friable, exophytic masses for which the patient typically has had no previous treatment; the histologic type tends to be nonaggressive; and finally, the lesions are amenable to surgical cure with low metastatic potential.

Adult↗
PubMed1997

[Initial experience in the diagnosis of thrombophilia due to activated protein C resistance].

The authors describe the first in Russia cases of thrombophilia caused by factor Va resistance to activated protein C. This abnormality was diagnosed in 6 of 25 patients (24%) with recurrent early thrombosis. The diagnosis was conducted according to a modified method implying the addition of protac (activator of protein C) to normal platelet poor plasma (PPP) free of factor V containing 100% of protein C. Protac dose was adjusted to increase the activated partial thromboplastin time (APTT) 2.4-2.8-fold in mixing PPP of the examinee with plasma containing activated protein C. The indices were estimated indicative of insufficient prolongation of APTT in response to activated protein C. Of 6 patients, 2 had apparent and 4 strong resistance to activated protein C. Treatment policy in this variant of thrombophilia is discussed.

Adolescent↗
PubMed1999

Malignant fibrous histiocytoma of the pancreas: a case report with genetic analysis.

BACKGROUND: Malignant fibrous histiocytoma (MFH) is the most common type of soft tissue sarcoma in adults; it occurs frequently in the extremities, the trunk, or retroperitoneal tissues. MFH rarely is detected in digestive organs, such as the liver or stomach. METHODS: The authors report a patient with MFH of the pancreas who was treated with surgery alone. The tumor was studied for genetic alterations in the p53, p16ink4a, and DPC4 tumor suppressor genes as well as the K-ras oncogene by immunohistochemistry, single strand conformation variant (SSCV) analysis, and direct DNA sequencing. RESULTS: The authors believe that this is the 13th report of primary pancreatic MFH in the world literature and the first genetic analysis of this rare tumor. The patient is alive with no evidence of recurrence 34 months after surgery. Immunohistochemistry revealed no abnormal accumulation of the p53 protein and normal nuclear p16 expression. Mutation analysis of the p53, p16, DPC4, and K-ras genes showed only a polymorphism at codon 72 of the p53 gene and no mutations in any of the genes. CONCLUSIONS: Genotypically, MFH of the pancreas is clearly different from other malignant pancreatic tumors, which further supports the hypothesis that this tumor is a rare but distinct entity.

Aged↗
PubMed1980

A study of the cellularity and ultrastructure of congenital mesoblastic nephroma.

This study reported 10 cases of congenital mesoblastic nephroma (CMN) from Children's Hospital of Pittsburgh: five were the typical fibromatous lesion, two were the cellular variant, and three had both components. Six cases occurred in infants less than one week of age. The degree of cellularity had no correlation with patient or tumor size. Three- to 10-year follow-ups of nine patients showed neither local recurrence nor metastasis. One infant died of generalized bleeding post nephrectomy. Ultrastructural study of seven cases showed mesenchymal cells with varying degrees of fibroblastic and/or myofibroblastic differentiation.

Culture Techniques↗
PubMed1994

Carcinomatous meningitis in patients with breast cancer. An aggressive disease variant.

BACKGROUND: Carcinomatous meningitis is a rare and often devastating complication in patients with breast cancer, and the treatment is controversial. METHODS: A retrospective analysis of 35 patients with carcinomatous meningitis from breast cancer was performed to define the biology of the disease and to guide treatment. RESULTS: An aggressive variant of breast cancer was revealed: meningeal metastasis complicates less than 3.5% of cases of metastatic breast carcinoma. Sixty-seven percent of these patients had tumors that were lobular or combined lobular/ductal histology; the median intervals from primary treatment to disease recurrence and from recurrence to death were 10.9 and 15 months, respectively. The median survival after diagnosis of carcinomatous meningitis was 77 days. The most significant prognostic factor was the Karnofsky performance status (KP) at presentation of meningeal disease. Patients with a KP greater or equal to 70 survived a median of 313 days, whereas those with a KP of 60 or less survived for a median of 36 days (P = 0.0002). In addition, there was a trend suggesting that the response 2 weeks after treatment was initiated, correlated with survival. CONCLUSIONS: Carcinomatous meningitis from breast carcinoma is an aggressive metastatic complication with a poor prognosis. The authors suggest that patients with a poor KP (< 70) should be treated symptomatically and those with a good KP (> or = 70) should receive more aggressive treatment. The patients' survival in this study compared well with other reports, and yet, only one patient was treated with intraventricular chemotherapy. Therefore, these data question the superiority of intraventricular treatment versus other modalities.

Adult↗
PubMed2003

[Inflammatory paradental cyst. Report of 6 cases].

BACKGROUND: The inflammatory paradental cyst has been described as an entity in the WHO classification of odontogenic tumors and cysts (1992). It is mainly located at mandibular molars, in particular third molars of the lower jaw. Radiologically, involved molars show a circumscribed, mostly half-moon shaped translucency distal or distobuccal to the involved tooth. Patients frequently report episodes of infection (pericoronitis). The histological findings are identical to those of inflammatory radicular cysts. The inflammatory paradental cyst has been described infrequently in the international literature. There are no reports available in German. AIM OF THE STUDY AND CASES: The aim of the present study was to present six of our own cases of inflammatory paradental cysts. Five men and one woman with an average age of 29.5 years were affected. In two cases paradental cysts occurred bilaterally. Three patients reported recurrent previous infections (pericoronitis). Radiologically, the typical translucency with clear demarcation distal to the third molars was observed. All of the third molars were vertically retained. Histologically, the inflammatory paradental cysts showed features identical to those of radicular cysts. The inflammatory paradental cyst is a clear indication for osteotomy of lower wisdom teeth. Postoperative complications or recurrences of the inflammatory paradental cysts have not been described. DISCUSSION: A correct clinical, radiological, and histopathological diagnosis of paradental cysts is mandatory, and more reports are needed in order to compile more information about relative frequency and pathogenesis of this cyst variant.

Adult↗