Search PubMed⌕ Search

Biomedical subjects

M Kujas

Publications and source records attributed to M Kujas.

At least 37 records · Page 2Linked to original sources
PubMed2000

Benign recurrence of a cerebellar pilocytic astrocytoma 45 years after gross total resection.

OBJECTIVE AND IMPORTANCE: Cerebellar pilocytic astrocytomas are common benign tumours of childhood and mostly easy to treat because of their location and conformation. Nevertheless, unpredictable recurrence as well as the lack of features to predict the rare malignant changes after first removal, still represent a challenging problem. CLINICAL PRESENTATION: A 54 years old man presented with a recurrence of a cerebellar pilocytic astrocytoma 45 years after complete removal. INTERVENTION: Gross total resection was performed. The pathological examination confirmed the same histological pattern as the first tumour. DISCUSSION: Current opinions about management and recurrence of pilocytic astrocytomas are discussed with a review of the literature.

Adolescent↗
PubMed1999

The antigonadotropic activity of a 19-nor-progesterone derivative is exerted both at the hypothalamic and pituitary levels in women.

We have previously shown in postmenopausal women that a 19-nor-progesterone derivative, nomegestrol acetate (NOMA) had a strong antigonadotropic activity and that this effect was not mediated via the androgen receptor. The aim of the present study was to further assess the action of this progestin on gonadotropin secretion in women. To demonstrate at which level of the hypothalamo-pituitary-ovarian axis the gonadotropin inhibition was exerted, 10 normally cycling (NC) women, 3 women with a gonadotropin-independent ovarian function [McCune-Albright (MCA) syndrome], and 5 women with functional hypothalamic amenorrhea (FHA) participated in the study. NC women were treated orally with 5 mg NOMA for 21 days, after one control cycle. Plasma estradiol (E2) and progesterone, LH, and FSH levels were measured during each cycle. A frequent sampling study (every 10 min for 4 h), followed by a classic GnRH test (100 microg, i.v.), was performed on day 11. Women with MCA were studied before, during NOMA, and after long-acting GnRH agonist administration. In women with FHA, pulsatile GnRH (20 microg s.c., every 90 min) was given for two cycles with or without NOMA (5 mg for 21 days). In all NC women, ovulation was suppressed by NOMA. Mean plasma LH levels, LH pulse frequency, and the LH response to exogenous GnRH were significantly decreased. In MCA, neither NOMA nor GnRH agonist modified multiple ovarian cysts on ultrasound or plasma E2, levels which remained elevated, ruling out a direct ovarian effect. In FHA, pulsatile GnRH administration recreated a normal ovulatory menstrual cycle. Addition of NOMA prevented the increase of plasma E2, decreased the amplitude of LH pulses, and prevented ovulation. In view of this unexpected action of NOMA at the pituitary level, seven samples of normal human female pituitaries were tested for the presence of progesterone receptor (PR) using a double labeling immunocytochemical technique. The presence of PR was detected in the seven human pituitary tissues. In addition, PR was found to be expressed only in gonadotroph cells. In conclusion, NOMA, a 19-nor-P derivative, has a potent antigonadotropic activity exerted at the hypothalamic level, inhibiting ovulation in NC women. In women with FHA, NOMA decreased the gonadotropin stimulation induced by pulsatile GnRH administration. According to the presence of PR in gonadotroph cells of normal human pituitaries, 19-nor-progesterone derivatives may also act on the gonadotropin secretion at the pituitary level.

Adolescent↗
PubMed1999

Bone giant cell tumour in neuropathological practice. A fifty year overview.

We report a case of a 29-year-old female patient who suffered from visual disturbance, resulting from a lesion in the sphenoid bone which, histologically, proved to be a giant cell tumour. Reviewing our laboratory practice over a 50 year period, only 7 cases of true giant cell tumour were found and they were in two major locations, i.e. the skull and vertebrae. These few cases led us to focus on the problem raised by the lack of histological patterns of malignancy. In agreement with Mazabraud's theory, it seems that a vertebral location is associated with a good prognosis and that gene mapping of chromosome 17 in relation to p53 mutations could be a valuable tool in the diagnosis of potential malignant behaviour.

Adult↗
PubMed1999

[Prognostic factors in the surgical management of acromegaly].

To determine the prognostic factors of surgical cure of growth hormone secreting pituitary adenomas, we have studied 48 patients operated between 1982 and 1992. We compared the features of the cured group (n = 18) with the non cured group (n = 30). We found that the factors of poor prognostic were: young age, growth hormone level before surgery over 50 ng/ml, invasive adenomas, monohormonal status in immunocytochemistry, and lack of decrease in postoperative growth hormone level during the glucose tolerance test.

Acromegaly↗
PubMed1999

[Intracranial cholesterol granulomas. Report of 2 cases].

Two cases of cholesterolic granuloma, one intraorbital and the other of the petrous apex, are reported. The xanthogranulomatous and/or cystic process localized in the intracranial cavities or in the brain parenchyma are discussed. A simple topographical and histological classification is attempted in answer to a rather ambiguous terminology.

Adult↗
PubMed1998

Perichiasmatic granuloma occuring after radical mastoidectomy: MR findings.

A case of chronic chemical meningitis occurring after a radical mastoidectomy is reported. Imaging and surgical findings were suggestive of a dissemination of cholesteatoma debris within the subarachnoid spaces. Chemical meningitis has been described in epidermoid and dermoid cyst rupture. This report illustrates that clinicians should be aware of this possible complication. Skull base imaging is mandatory before considering the diagnosis of idiopathic meningitis. Only treatment of the abnormal communication between cerebrospinal fluid and middle ear may eradicate the origin of this rare meningitis.

Adult↗
PubMed1998

Ganglioglioma of the pineal region: case report and review of the literature.

BACKGROUND: Ganglioglioma is a lesion rarely encountered in the pineal region. Only six cases have been published to our knowledge. METHODS AND RESULTS: We present the case of a 38-year-old male suffering from a pineal ganglioglioma that was totally removed by surgery. Histological diagnosis was made by light microscopy using immunostaining for synaptophysin. We review the few radiological and pathological data available in the literature concerning this very rare lesion for which surgery alone provides excellent results. CONCLUSION: The possibility of long term remission obtained by surgery alone and the lack of specificity of clinical and radiological features of ganglioglioma are additional reasons for an aggressive surgical management of pineal tumors when tumor markers and cytopathological examination of the cerebrospinal fluid are not contributive.

Adult↗
PubMed1998

Primary central nervous system lymphomas in 72 immunocompetent patients: pathologic findings and clinical correlations. Groupe Ouest Est d'étude des Leucénies et Autres Maladies du Sang (GOELAMS)

We reviewed 72 primary central nervous system lymphomas occurring in immunocompetent patients. The cases were reviewed for clinical data, histology, immunophenotype, bcl-2 and p53 expression, and Epstein-Barr virus association. Follow-up was available for 40 patients included in the Groupe Ouest Est d'étude des Leucénies et Autres Maladies du Sang (GOELAMS) lymphomes cérébraux primitifs (LCP 88) trial. Each diagnosis, requiring a consensus among at least 3 pathologists, was performed according to the recent Revised European-American Lymphoma classification and equivalents in the updated Kiel classification. Tumors were predominantly classified as diffuse large B-cell lymphomas. There were 3 T-cell lymphomas and 1 Hodgkin lymphoma. The proteins bcl-2 and p53 were expressed in 35% and 16% of the tested cases, respectively. Epstein-Barr virus was not found by in situ hybridization except in the case classfied as a cerebral localization of Hodgkin disease. No significant association was found between subtypes, bcl-2 or p53 expression, and patient survival. From the standpoint of their biologic characteristics, primary central nervous system lymphomas are very similar to systemic diffuse large B-cell lymphomas. In contrast to AIDS-related primary central nervous system lymphomas, primary central nervous system lymphomas are rarely associated with Epstein-Barr virus and in immunocompetent patients they express bcl-2 at a relatively low rate.

Adult↗
PubMed1998

Characterization of human lutropin carboxyl-terminus isoforms.

Human lutropin (hLH) exhibits both carbohydrate and peptidic heterogeneities that affect its biological potency and the duration of its activity in vivo. Peptidic changes within the hLH beta-subunit are characterized as intrachain proteolytic nicking and carboxyl terminus heterogeneity. To date, the carboxyl terminus of hLHbeta appears to end at either position Gln114 or Gly117, as determined by sequencing of purified subunit. Furthermore, the complementary DNA for hLHbeta predicts a protein containing an additional peptidic stretch, which would make the beta-subunit 121 residues long. This extension may be responsible for the particular intracellular behavior of hLHbeta. To investigate the carboxyl terminus polymorphism of natural hLHbeta, monoclonal antipeptide antibodies were raised against a synthetic peptide mimicking the 104-119 portion of hLHbeta. One antibody, designated LHP09, was found to specifically react with the recombinant hLHbeta ending at position hLHbeta[Leu119] but not with other recombinant forms ending at [Ser116], [Phe120] or [Leu121]. Immunochemical analysis of hLH, either pituitary or urinary in origin, indicated that only pituitary hLH contains a Leu119-ending form of hLHbeta. Finally, immunohistochemical detection was performed using LHP09 and showed specific staining of a normal adult pituitary gland. These observations support the in vivo existence of intrapituitary molecular forms of hLHbeta ending at various positions between Gln114 and Leu121.

Amino Acid Sequence↗
PubMed1998

Prevalence of lymphocytic infiltrate in 1400 pituitary adenomas.

To evaluate the prevalence of lymphocytic infiltrate in a large series of pituitary adenomas, we retrospectively studied tumor tissues from 1400 patients. Based on immunocytochemical data, tumors were classified as PRL (n=411), multihormonal (n=310), immunonegative (n=275), ACTH (n=166), GH (n=137), alpha subunit (n=44), FSH and/or LH, (n=42), and TSH (n=15) adenomas. The lymphocytic infiltrate was diagnosed on histological examination and investigated by immunostaining with anti LCA (human leucocyte common antigen), anti CD45RO (human T cell) and anti CD20 (human B cell) antibodies. Lymphocytic infiltrate was present in 40 adenomas (2.9%), 26 females and 14 males, aged 18 to 77 years (mean+/-SD, 37+/-14 years). The tumors were 19 PRL, 8 multihormonal, 4 GH, 4 alpha subunit, 3 ACTH, and 2 immunonegative adenomas. In PRL adenomas, the sex ratio (female/male) and the age were similar in patients with and without lymphocytic infiltrate (2.8 vs. 4.6 and 29+/-6 years vs. 32+/-11 years, respectively). The frequency of lymphocytic infiltrate was similar in PRL, GH, ACTH and multihormonal adenomas, but lymphocytic infiltrate was significantly more frequent in PRL adenoma than in immunonegative adenoma, and in alpha subunit adenoma than in immunonegative, ACTH and multihormonal adenomas. The lymphocytic cells were almost exclusively T cells. We conclude that lymphocytic infiltrates are rare in pituitary adenomas. Their frequency is not statistically different in major categories of secreting adenomas (PRL, GH, ACTH, multihormonal). Their pathophysiological significance remains to be established.

Adenoma↗
PubMed1997

Intramedullary neurenteric cyst without any associated malformation. One case evaluated by RMI and electron microscopic study.

A 46 years old woman presented with several years history of low back pain. For five years she suffered from weakness of the left lower limb and three years later she experienced an episode of right foot weakness. She suffered too from occasional urinary urgency. The examination showed decreased power and diminished sensory perception in the left leg. On myelography, a block at L2 level was observed. RMI evaluation showed an intramedullary cyst in the anterior part of the spinal cord without any enhancement of its wall by the Gadolinium. At operation a thin-wall cyst was found containing clear fluid. After a biopsy of the wall, a cystosubarachnoid shunt was performed. Histological examination of the surgical sample showed a simple cuboidal epithelium lying on collagen fibrills. Electron microscopic studies showed ciliated cells with a clearly-visible basement membrane. The diagnosis of neurenteric cyst was confirmed. In the postoperative course the patient complained about sensory loss of the legs and the perineal area. Six months later, she exhibited a sensory disturbance of the feet and the right sacral area, a motor deficit of the distal left leg without urinary disturbance. Neurenteric cysts are dysraphic lesions which can be observed without other abnormalities. They are usually extramedullary and the intramedullary forms are very rare: among 5 cases reported in the literature, one has been evaluated by RMI. In the absence of enhancement by the Gadolinium, the other possible diagnosis seems an ependymal cyst. Contrary to extramedullary forms the postoperative course of intramedullary neurenteric cysts are not always eventful. Because the cyst wall cannot be removed, repeated RMI are desirable in the follow-up.

Female↗
PubMed1997

Idiopathic granulomatous hypophysitis: clinical and imaging features.

Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery.

Adenoma↗
PubMed1997

Craniopharyngiomas in two consanguineous siblings: case report.

OBJECTIVE AND IMPORTANCE: We describe a double case of craniopharyngioma in consanguineous siblings, suggesting the disease is sometimes genetic. CLINICAL PRESENTATION: Two typical adamantine craniopharyngiomas were observed in two consanguineous siblings. The brother and the sister, whose parents were first cousins, developed the tumors at the same age. INTERVENTION: The male patient was operated on using a frontopterional approach, and the tumor was completely resected. The patient remained free from recurrence 9 years after surgery. His older sister died after tumor removal was attempted at another institution. CONCLUSION: To our knowledge, such a connection has never been reported in the literature. It suggests that craniopharyngioma, which is usually sporadic, can also be transmitted in an autosomal recessive manner.

Adolescent↗
PubMed1997

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel-Lindau disease. Case report.

This report describes a patient with von Hippel-Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.

Adenoma↗
PubMed1997

Familial aggregation of malignant melanoma/dysplastic naevi and tumours of the nervous system: an original syndrome of tumour proneness.

A five-generation family is here reported in which several members developed malignant melanoma, dysplastic naevi, astrocytoma in all grades, benign or malignant schwannoma, neurofibroma, or meningioma in a single instance. Significant cosegregation of skin and nervous tumours, preclusion of allelism to type 1 neurofibromatosis and phenotypic departure from known syndromes of hereditary proneness to cancer make one suggest an original familial predisposition to both malignant melanoma and central/peripheral nervous tumours.

Adult↗
PubMed1997

[Chondrosarcoma of the temporomandibular joint. Apropos of a case and review of the literature].

Chondrosarcomas of the temporo-mandibular joint are exceptional and have been previously reported in the literature in only seven cases. A case located in the temporal bone with extension in the infratemporal fossa and the cranial base is presented. Treatment consisted in a wide surgical resection combined with postoperative radiotherapy. The patient is alive with a follow-up of more than two years.

Adult↗
PubMed1997

Detection rate and intratumoral virus load of human herpesvirus-8 in immunodeficiency-related B-cell lymphoid malignancies.

Human herpesvirus-8 (HHV-8), associated with Kaposi's sarcoma, primary effusion lymphoma, and Castleman's disease, has been found in circulating B-cells and might have a causative role in B-cell malignancies associated with immunodeficiency syndromes. We determined the rate of detection and intratumoral virus load of HHV-8 by means of a semiquantitative approach in post-transplant lymphoproliferative diseases (PTLDs), AIDS-related non-Hodgkin's lymphomas (NHLs), including both Burkitt's lymphomas (BLs) and large cell lymphomas (LCLs), as well as in control groups consisting of follicular hyperplasias (FHs) and HIV-negative LCLs. HHV-8 sequences were detected at a similar rate in HIV-negative PTLDs (24%), HIV-negative LCLs (22%) and HIV-negative FHs (17%). The detection rate was significantly higher in HIV-positive BLs (73%), HIV-positive LCLs (67%), and HIV-positive FHs (65%) supporting the view of an epidemiological link between HHV-8 and HIV infections. The viral load was 10(2) genome copies per cell in the single case of primary effusion lymphoma included in the LCL group while it was 10(-3) copy per cell (median value; range: 10(-4)-10(-1)) in all the other HHV-8-positive samples. No significant difference of viral load was found according to HIV status. The virus loads of PTLDs and HIV-positive LCLs were significantly higher than those observed in HIV-positive BLs and FHs, suggesting, to some extent, that the degree of immunodeficiency may influence HHV-8 replication. However, with the exception of the single case of primary effusion lymphoma studied, the low intratumoral load of HHV-8 strongly argues against a direct causative agent of the virus in the occurrence of PTLDs and AIDS-related NHLs.

Case-Control Studies↗