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PubMed2000

Burkitt's lymphoma in Greek adults. A study of the Hellenic co-operative oncology group.

Non-African Burkitt's lymphoma is a rare disease among adults without AIDS. Among 1352 Greek adult patients with non-Hodgkin's lymphoma, 24 cases (1.8%) were classified as Burkitt's (BL) or Burkitt-like (BLL) lymphoma. Eleven cases fulfilled the criteria of BL and 13 of BLL. No statistical differences were found in the general characteristics of the two groups at the time of diagnosis. Extranodal involvement was a common finding in both groups and bulky disease (>10 cm) was observed in almost one half of the patients. The majority of the patients were treated with intensive, although different, protocols. After induction treatment, complete remission (CR) was achieved in 14 patients (60.8%). CR was reached in all cases with stage I-II, while in stage IV the CR rate was 30.4%. The median overall survival was 27 months. The median survival for BL was 13 months compared to 27 months in the BLL group (P=0.34). The data of the present retrospective analysis, indicated that there were not significant clinical differences between BL and BLL variants. Since BLL is still a non-reproducible category in the REAL classification, all BL variants must be treated uniformly with intensive protocols.

Adolescent↗
PubMed1990

[The indications for and sequence of vitrectomy and scleroplastic operations in posttraumatic retinal detachment and vitreous body pathology (1)].

The paper presents a clinical analysis of surgical treatment of 378 patients (426 operations) with posttraumatic retinal detachment. A broadened variant of a classification of traumatic retinal detachment signs in associated pathology of the retina and vitreous body is proposed. Recommendations are worked out concerning the approach of surgical treatment of traumatic detachment with determination of indications and the sequence of vitrectomy and scleroplastic operations. It is advisable to assess the effectiveness of surgical intervention in this pathology in more delayed terms.

Adolescent↗
PubMed1994

[An international attempt for standardization on terminology and methodology in hemostasis and thrombosis].

Scientific and Standardization Committee (SSC) of International Thrombosis and Hemostasis has acted its activity since 1955 for establishing international concept on terminology, methodology in the fields of blood platelets, coagulation and fibrinolysis. Among the reports from 15 sub-committee in 1993 meeting, some clinically interesting topics are reported. In von Willebrand factor (vWF) Subcommittee, new classification of von Willebrand disease including new variant of VFW which shows defect on factor VIII binding capacity, is proposed. In Control of Anticoagulation Subcommittee, the necessity of coagulation monitoring during low molecular weight heparin (LMWH) administration was discussed. For prophylaxis use, monitoring is unnecessary except patients having renal failure or high-low body weight. For the treatment of venous thrombosis once or twice monitoring every 10 days would be necessary. In Lupus anticoagulant (LA)/phospholipid dependent antibodies Subcommittee, results of 3rd international survey for LA sent to 38 laboratories in 16 countries including Japan were reported. Most laboratory used APTT and dRVVT simultaneously as screening tests. Sensitivity and specificity of confirmatory test for LA are compared; the best one was Staclot LA and the second was DVVT.

Blood Coagulation Factors↗
PubMed1997

Early-onset benign occipital seizure susceptibility syndrome.

PURPOSE: Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. METHODS: This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting. RESULTS: One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year. CONCLUSIONS: Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.

Adolescent↗
PubMed2000

Burkitt's lymphoma: historical background and recent insights into classification and pathogenesis.

In this paper, the authors evaluate the historical evolution of the definition of Burkitt's lymphoma (BL) and of its clinicoepidemiological (endemic, sporadic, and acquired immunodeficiency syndrome-associated BL) and morphological variants. On the basis of the morphological, immunologic, genetic, and clinical characteristics of these tumors, the authors also emphasize the importance of precise disease definitions for biological and epidemiological studies. These principles were used in accordance with the Revised European-American classification of lymphoid neoplasms (REAL), which proposed that disease entities should be defined by a constellation of pathobiological and clinical features.

Burkitt Lymphoma↗
PubMed1988

Nonsalivary sinonasal adenocarcinoma.

Thirteen cases of primary non-salivary gland adenocarcinoma of the nasal cavity and paranasal sinuses were studied at UCLA over 20 years. All pathologic specimens were reviewed and those tumors that were histologically distinct from the more common salivary gland-derived tumors were included in the study. Three classifications were identified: well, moderately, and poorly differentiated adenocarcinoma. A distinct variant of sinonasal adenocarcinoma was the intestinal type. The clinical behavior of the latter resembled the well or moderately differentiated types, with behavior mainly predicted by the extent of the disease. These groups have prognostic significance, with the poorly differentiated group having the most virulent course. Nine of 13 tumors occurred in the ethmoid sinuses and all were aggressive locally. Only one case had distant metastases (nodal neck disease in a terminal case). Of five long-term survivors (median five-year follow-up), all had extensive surgical resections and three had full-course radiotherapy. The single most important factor in the treatment of these lesions is adequacy of surgical margins. Four of six patients with confirmed negative margins were cured despite extensive tumors. Three survivors had the cribriform plate taken and one required a combined intracranial/extracranial approach for tumor resection. There were no survivors in four patients treated with primary irradiation.

Adenocarcinoma↗
PubMed1985

Cytogenetic study in six Spanish patients with Burkitt's lymphoma.

A cytogenetic study carried out on affected tissues of six children with Burkitt's lymphoma revealed five cases with a typical translocation (8q-; 14q+) and one with a variant t(2p-; 8q+). Additional cytogenetic variations were present in three cases. One had two acrocentric marker chromosomes comprised of material from chromosome #1 (1q21----q44) translocated to the Y gonosome. The other two cases had a cytogenetic alteration of chromosome #12. Cytogenetic studies carried out systematically in Burkitt's lymphoma could be a great help in possibly establishing differences in the biologic and clinical aspects of cases presenting with the t(8;14) versus cases with variant translocations, as this would allow for classification of these patients into groups which, at present, cannot be differentiated using other diagnostic methods.

Adolescent↗
PubMed1985

Small non-cleaved follicular center cell lymphoma: clinicopathologic comparison of Burkitt and non-Burkitt variants in Finnish material.

A retrospective clinical and histopathological analysis was made of patients with small non-cleaved-type malignant lymphoma according to the classification of Lukes-Collins. Nineteen cases were classified as the Burkitt type and 41 cases as the non-Burkitt type. The non-Burkitt type was more variable cytologically. A bimodal age distribution was seen in both subgroups. One half in both subtypes had an advanced disease (stage III-IV). Lymphadenopathy was slightly more common in the non-Burkitt type. No patient with non-Burkitt's lymphoma had mediastinal involvement (P less than 0.01). Liver and spleen were not involved by the tumor of the Burkitt type (P less than 0.005). Gastrointestinal involvement was seen in both types without differences. No statistical differences between the overall survivals of these subtypes were observed.

Adolescent↗
PubMed1991

[Central lesions in neurofibromatosis: clinical, MRI and histopathologic correlations. An attempted classification].

The National Institute of Health Consensus Panel on Neurofibromatosis (NF) recently recognized 2 distinct forms of NF (NF-1 and NF-2) and stated that variant forms may exist. We selected 30 patients who fulfilled the criteria of NF-1 or whose condition was consistent with NF-2. All patients showed pathological magnetic resonance images (MRI), and in 19 cases confirmation was obtained from histopathology. We established correlations between the site and nature of the lesions on the one hand and the diagnostic criteria of NF on the other hand, there by hoping to contribute to a better knowledge and classification of neurofibromatosis. Nineteen patients had only intraparenchymatous lesions of the central nervous system (CNS) and fulfilled the criteria of NF-1; histopathological examination demonstrated pilocytic astrocytoma in 8 cases. Eleven patients showed only extra-axial lesions; 8 of them had criteria suggestive of NF-2, except for familial history. Pathological examination revealed either acoustic, pluriradicular, peripheral or mixed schwannomas (7/8) or pluriradicular ganglioneuromas (1/8). Two patients had unilateral extra-axial pluriradicular cervical lesions and fulfilled the diagnostic criteria of NF-1; pathological examination revealed neurofibroma in both cases. One female patient had both intra- and extra-axial lesions that fulfilled the criteria of NF-1 and NF-2, suggesting the existence of a mixed form (NF-3).

Adolescent↗
PubMed1987

Hematopathological features of acute erythremia (morbus Di Guglielmo). A contribution to the classification and differential diagnosis of erythroid neoplasias.

Classification and differential diagnosis of erythroid neoplasias still are a matter of discussion. Eleven cases of primary acute erythremia were diagnosed between 1981 and 1984 at the Institute of Pathology, University of Kiel. Erythremia represented 0.5% of all hematological diagnoses and 1.0% of the myeloproliferative disorders. The male-to-female ratio was 1:1. Incidence peaked in the 7th decade. Evaluation of clinical data, of cytological and histological findings in blood and bone marrow, and of occasional immunophenotyping of blast cells (anti-glycophorin A+) revealed two variants of acute erythremia: a first, blastic one and a second, more differentiated form. Acute erythremia must be strictly distinguished from mixed erythroid/myeloid erythroleukemia and from secondary erythroid neoplasias, especially the erythremic 'blast crisis' of chronic myeloid leukemia or polycythemia vera rubra. Distinguishing the myelodysplastic variant of sideroblastic anemia from anerythremic acute erythremia can be extremely difficult. We discuss the differential diagnosis and classification of erythroid neoplasias based upon reproducible hematological criteria to facilitate the gathering and comparison of epidemiological and clinical data on these rare malignancies.

Acute Disease↗
PubMed2000

Evaluation of a specific nested PCR targeting domain III of the 23S rRNA gene of "Tropheryma whippelii" and proposal of a classification system for its molecular variants.

"Tropheryma whippelii"-associated infections are usually confirmed histopathologically by using light microscopy. PCR assays targeting the 16S rRNA gene (16S rDNA) of "T. whippelii" are increasingly being applied for this purpose. Compared to microscopic analysis, PCR seems to be more sensitive, as indicated by the fact that several cases of Whipple's disease with negative histopathological findings but positive PCR results have been reported. Considering the lack of pathognomonic clinical features for this disease and the fact that "T. whippelii" DNA has repeatedly been found in patients without clinical Whipple's disease, such PCR results should be confirmed by additional tests. We have, therefore, evaluated a "T. whippelii"-specific nested PCR targeting domain III of the 23S rDNA with 41 clinical specimens known to contain "T. whippelii" 16S rDNA. All of these specimens were also positive for "T. whippelii" 23S rDNA. The specificity of the test was shown by sequencing of the amplicons and by the absence of amplicons in 38 negative controls. We consider this PCR test to be a suitable tool for confirming the presence of "T. whippelii" DNA in specimens with inconclusive histopathological findings. The information derived from sequencing of the partial "T. whippelii" 23S rDNA was then combined with our recent data of the 16S-23S rDNA spacer region of this organism. Overall, four different rDNA types are recognized in our proposed classification system for molecular variants of "T. whippelii." This preliminary scheme may provide a basis for further epidemiological and clinical studies with "T. whippelii" and associated diseases.

Actinobacteria↗
PubMed1991

[Central nervous system lesions in neurofibromatosis. Clinical, MRI and histopathological correlations. A trial of classification].

The National Institute of Health Consensus Panel on Neurofibromatosis (NF) recently recognized 2 distinct forms of NF (NF-1 and NF-2) and stated that variant forms may exist. We selected 30 patients who fulfilled the criteria of NF-1 or whose condition was consistent with NF-2. All patients showed pathological magnetic resonance images (MRI), and in 19 cases confirmation was obtained from histopathology. We established correlations between the site and nature of the lesions on the one hand and the diagnostic criteria of NF on the other hand, there by hoping to contribute to a better knowledge and classification of neurofibromatosis. Nineteen patients had only intraparenchymatous lesions of the central nervous system (CNS) and fulfilled the criteria of NF-1; histopathological examination demonstrated pilocytic astrocytoma in 8 cases. Eleven patients showed only extra-axial lesions; 8 of them had criteria suggestive of NF-2, except for familial history. Pathological examination revealed either acoustic, pluriradicular of mixed schwannomas (7/8) or pluriradicular ganglioneuromas (1/8). Two patients had unilateral extra-axial pluriradicular cervical lesions and fulfilled the diagnostic criteria of NF-1; pathological examination revealed neurofibroma in both cases. One female patient had both intra- and extra-axial lesions that fulfilled the criteria of NF-1 and NF-2, suggesting the existence of a mixed form (NF-3).

Adolescent↗
PubMed2004

Carcinoma of lung with rhabdoid features.

Lung tumors with rhabdoid features, included as variants of large cell carcinoma in the 1999 World Health Organization classification of lung tumors, are rare and have an aggressive clinical course. We report 11 patients with primary lung tumors with rhabdoid features and review the literature on this uncommon tumor. We examined samples from 7 primary (6 resections, 1 biopsy) and 4 metastatic tumor samples. All specimens were stained with immunohistochemical stains for pancytokeratin (CK), cytokeratin 7 (CK7), cytokeratin (CK20), thyroid transcription factor-1 (TTF-1), and vimentin. The patients were 7 men and 4 women whose ages ranged from 35 to 70 years. Nine patients presented with respiratory symptoms, and 9 patients had a history of heavy smoking. One patient had TNM stage I tumor, 3 had stage III tumors, and 6 had stage IV tumors at presentation; tumor stage could not be determined in 1 patient. Histological examination of these tumors showed typical rhabdoid cells: large cells with abundant cytoplasm, a large eccentric nucleus with a central macronucleolus, and a rounded eosinophilic cytoplasmic inclusion that sometimes caused nuclear indentation. These cells constituted 10% to 90% of the tumor. The "parent" neoplasm was sarcomatoid carcinoma and adenocarcinoma in 4 cases each and was large cell undifferentiated carcinoma in 3 cases. Cytoplasmic staining in the rhabdoid cells was seen in 9 of 11 cases for CK, in 4 of 10 cases for CK7, and in all 11 cases for vimentin. Nuclear staining for TTF-1 in the rhabdoid cells was absent in all 11 cases, and cytoplasmic staining for CK20 was negative in the rhabdoid cells in all 10 cases studied. Of the 9 patients with available follow-up information, 8 died of disease, and 1 is alive with no evidence of disease 20 months after the initial diagnosis. We conclude that rhabdoid features can occur in a variety of lung tumors, including sarcomatoid carcinoma. Recognizing these lesions is important because of their possibly aggressive clinical course.

Adenocarcinoma↗
PubMed1984

A case of malignant myxoma (myxosarcoma) of the maxilla.

A centrally located maxillary myxoma with malignant histologic appearance and aggressive clinical course is reported in a 40-yr-old man. The gelatinous polypoid tumor mass was diagnosed as a myxoma in the first biopsy. The tumor recurred rapidly (within 3 wk) eroding the bony structures of the maxillary sinus and the hard palate and infiltrating the adjacent soft tissues. In repeated biopsy (as well as reassessment of the first biopsy) the tumor was found to be composed of plump stellate cells, some of which were pleomorphic with atypical and bizarre mitotic figures. The amorphic myxoid matrix contained acid mucopolysaccharides and was completely devoid of lipids. Electron microscopy demonstrated that the tumor cells were identical to fibroblasts, which is consistent with the appearance of myxoma cells. When radiotherapy was unsuccessful, we removed the left maxilla with orbital excenteration and reconstructed the jaw. Three years later the patient died accidentally. At autopsy, no recurrence of myxoma or distant metastases were found. Due to the malignant histologic appearance and the aggressive clinical course, this tumor can be called an odontogenic myxosarcoma and should be added to the WHO classification as a malignant variant of odontogenic myxoma.

Adult↗
PubMed1989

Leprosy in children: correlation of clinical, histopathological, bacteriological and immunological parameters.

The study of leprosy in children has indicated an incidence of 10% amongst leprosy patients attending the clinic. The duration of the disease was usually less than 2 years. The expression of leprosy in this group was either a macule and/or a plaque. Classification was, therefore, different and conforms to indeterminate (I), borderline tuberculoid (BT) and borderline (BB) leprosy. Only occasionally were other clinical variants seen. The bacteriology was largely unproductive by slit-skin smears. The lepromin (Mitsuda) responses were positive in BT and unpredictable in BB patients. Epicutaneous responses to sensitization with dinitrochlorobenzene (DNCB) paralleled responses to lepromin. Microscopic pathology was of very little help. The correlation of these parameters was only 50-60% indicating that the diagnosis of leprosy should primarily be based on clinical features.

Adolescent↗
PubMed2000

[The relationship between clinical types of postinfarction angina and stenosis of ischemia-related artery].

OBJECTIVE: To approach the pathogenesis of postinfarction angina and set up its treatment guidelines. METHODS: 67 patients with postinfarction angina undergoing coronary angiography during the hospitalization were studied. RESULTS: (1) According to the clinical classification of angina pectoris, spontaneous angina is the most common type (43.9%); mixed angina is second (25.7%), effort angina and variant angina are relatively low frequency (16.7% & 13.6%). (2) Stenosis >or= 90% in the ischemia-related artery accounted for the anginal symptoms constituted 100%, 82.8%, 72.7% and 44.4% of the cases with mixed angina, spontaneous angina, effort angina and variant angina respectively. (3) Postinfarction angina caused by non infarction-related artery was relatively less frequent (8.9%). CONCLUSIONS: Severe residual stenosis of infarction-related artery is the main pathologic factor on the pathogenesis of postinfarction angina, so early interventional therapy or bypass surgery should be taken.

Adult↗
PubMed1981

[Carcinoma of the major duodenal papilla].

The analysis of 134 postmortem cases and 65 clinical observations of carcinoma of the major duodenal papilla (MDP) has revealed different variants of development of carcinoma of MDP. The classification by the TNM system is made. In 34% of cases carcinoma was found to spread beyond the duodenum limit. Papillectomy should not be considered radical intervention. Average survival time after papillectomy was 17 months, after pancreatoduodenal resection it was 34 months. Fibroduodenoscopy, laparoscopic cholecystocholangiography in combination with relaxation duodenography was used for early diagnosis of carcinoma of MDP. Laparoscopic investigation was always accomplished by laparoscopic cholecystostomy which permitted avoiding hepatic insufficiency in the postoperative period.

Adult↗