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[Temporal nosography of primary headache].

The lack of certain etiopathogenetic elements and the numerous clinical-descriptive classifications of primary headaches has brought the research-workers to elaborate a chrono-pathological classification of pain in the primary headaches. So, they present a chrono-nosography and they suggest that the continuous form of primary headaches can be divided into two forms, one of these is associated with affective troubles. Then they emphasize the problem of classic migraine and its clinical variant as a trigger of vascular disease.

Headache↗

Rhabdomyosarcoma of infancy and childhood. Problems of morphologic classification.

The classification of the histologic types of rhabdomyosarcoma is based on poorly defined criteria. This has resulted in marked disparities in studies reported from different institutions, as well as difficulties in assessment of the clinical behavior of the different histologic types. A retrospective morphologic analysis of 36 consecutive cases of rhabdomyosarcoma of childhood was undertaken according to predefined and strict guidelines for diagnosis. Undeflecting adherence to such criteria identified the embryonal type as the most common form, and the alveolar variant as a distinct clinicopathologic entity with a much more aggressive course; it also resulted in a large proportion (approximately one-fourth) of sarcomas of undertermined histogenesis. In spite of either prolonged follow-up observation with repeated biopsies, autopsy study, or electron-microscopic study of tumor tissue, no evidence could be obtained to substantiate the rhabdomyogenic derivation of the latter group of neoplasms. Precise systematization of the morphology of these cases may be contingent upon careful inventory of their fine structural features; current classifications appear to have disregarded the morphologic heterogeneity of this group of tumors.

Adolescent↗

Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement.

Neuroblastic tumors comprise neuroblastoma (NB), ganglioneuroblastoma, and ganglioneuroma (GN). NBs have shown remarkable differentiation capabilities, which include spontaneous regression. In the last 3 decades, the prognosis for metastatic NB has improved significantly. The ability to distinguish prognostic subtypes based on clinical and biologic features allows for understanding of this disease process and development of management procedures. NBs are often asymptomatic and remain undetected until a large abdominal mass or metastasis is found. GN, the most differentiated variant, is a benign neoplasm with significant growth potential and ability to cause clinical complications. GN of the mandible has seldom been reported in the literature in English. We present a case of NB metastatic to the mandible exhibiting ganglioneuromatous differentiation. We discuss the current understanding of the biology, grading, classification, and prognostic implications of NB.

Abdominal Neoplasms↗

Ultrastructural classification of pituitary adenomas.

Forty-eight surgically removed pituitary adenomas have been investigated by electron microscopy. Distinct differences in fine structural appearances permitted their separation into 5 classes: 1) Growth hormone, (GH)-cell tumors; 2) Prolactin cell tumors; 3) Mixed adenomas composed of GH and prolactin cells; 4) Adrenocorticotrophic-Melanocyte stimulating hormone (ACTH-MSH) cell tumors; 5) Undifferentiated cell adenomas. Densely and sparsely granulated tumors were distinguished within classes 1, 2 and 4. Although these two forms appeared to represent well defined entities, they may be variants of the same tumor differing only in pace of hormone production and/or release. Number and size of secretory granules varied considerably among tumors composed of the same cell type indicating that pituitary tumor classification cannot be based solely on granule morphology. This classification takes into account morphogenesis with emphasis on clinical features and structure-function relationship.

Adenoma, Acidophil↗

[Pleuropulmonary blastoma in children: diagnosis and results of surgery and complex treatment].

This is a report on a retrospective study of experience had with complex treatment of pleuropulmonary blastoma in ten children aged 2-11 years, covering a 27-year period. In all patients diagnosis is made on the ground of clinical symptomatology, imaging methods and histological findings. All patients undergo surgery, as follows: tumor extirpation (n = 3), lobectomy (n = 2) and pneumonectomy (n = 5). Three deaths occur in the early postoperative period (30%). In the remainder postoperative chemotherapy is conducted. Histologically the solid variant (type 3 according to Dehner's classification) is predominant. There are three recurrences (42.8%) among the cases with simple tumor extirpation (2) and lobectomy (1), followed by successful pneumonectomy (two patients) and atypical pulmonary resection (one patient). At long-term follow-up, five patients (50%) are still alive for periods ranging from 1 to 11 years postoperatively. Pleuropulmonary blastoma is a surgical rarity in children. The most important factors for long-term survivorship are both radical surgery, and adequate chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗

[Viral encephalitis (etiology, pathogenesis, classification)].

The existing classifications of viral encephalitis are based on mixed, heterogenous principles. The author on the grounds of comparing modern data, related to the etiology, pathogenesis, predilectiveness, gives his suggestions as to a new classification of viral encephalitis. This classification is structured according to the pathogenetical principles, with a consideration of the different variants of the dynamics and polymorphism of separate nozological forms, depending upon the interrelationships of the viral and the immunity factors.

Clinical Trials as Topic↗

Palmar crease variants and their clinical significance: a study of newborns at risk.

An analysis of palmar crease variants was carried out in a group of "at risk" newborns, without any evident congenital anomalies. This group consisted of 108 prematures, 74 infants who were small for gestational age, 62 newborns with history of gestational complications, and 46 newborns with a history of intrauterine methadone exposure. A system of classification was developed based on observations of 500 normal newborns as control subjects, 466 normal mothers, and 200 normal children. The palmar crease variants can be divided into four main groups, schematically presented as normal variants, simian crease and its variants, Sydney line and its variants, and another group of unusual variants which do not fit into the other groups. A study of these groups revealed that familial components, race, sex, and age are factors that can influence the expression of palmar crease patterns. There is an increased frequency of abnormal creases in each of the groups of "at risk" newborns. Moreover, there is an apparent association of interrupted transrerse creases and intrauterine methadone exposure.

Adult↗

[Mastocytosis, classification, biological diagnosis and therapy].

In mast cell (MC) disorders (mastocytosis), clinical symptoms are caused by the release of chemical mediators from MCs, the pathologic infiltration of neoplastic MCs in tissues, or both. Cutaneous mastocytosis is a benign disease in which MC infiltration is confined to the skin. In pediatric cases cutaneous mastocytosis might regress spontaneously. Systemic mastocytosis (SM) is more frequently diagnosed in adults and is a persistent (clonal) disease of bone marrow-derived myelomastocytic progenitors. The somatic c-kit mutation D816V is found in the majority of such patients. The natural clinical course in SM is variable. Whereas most patients remain at the indolent stage for many years, some have aggressive SM (ASM) at diagnosis. Other patients have an associated clonal hematologic none MC lineage disease (AHNMD). MC leukemia (MCL) is a rare disease variant characterized by circulating MCs and fatal disease progression. Two important diagnostic clues in SM are an increased serum tryptase level and the presence of abnormal mast cells in the bone marrow. The current review provides an overview of mastocytosis and its subvariants, the new classification of these diseases, a practical guide for the biological diagnosis and advances and future directions in therapy of these pathologies.

Bone Marrow Examination↗

Molecular biology of opioid analgesia.

Opioids provide excellent pain relief in most patients. Yet the responses of patients to individual opioids can vary markedly, even among the mu opioids. Understanding this variability would greatly enhance our ability to treat patients appropriately. Classical pharmacological studies have long implied the existence of multiple subtypes of mu opioid receptors. More recently, a number of variants of the cloned mu opioid receptor have been described. These variants all show the same selectivity for mu opioids, confirming their classification as mu opioid receptors. Yet, they differ in their functional activation by opioids as well as in their localization within cells and regions in the brain. These multiple mu opioid receptors may help explain the range of responses seen clinically among patients for the various opioid drugs.

Analgesics, Opioid↗

Mycosis fungoides d'embleé: CD30-negative cutaneous large T-cell lymphoma.

The d'embleé variant of mycosis fungoides initially described patients with a rapid onset of tumors without progression through patch- and plaque-stage disease. We report a case of a patient with the clinical presentation of mycosis fungoides d'embleé and correlate the histologic and immunophenotypic data with those of a more updated classification scheme.

Antineoplastic Combined Chemotherapy Protocols↗

The WHO classification of tumors of the nervous system.

The new World Health Organization (WHO) classification of nervous system tumors, published in 2000, emerged from a 1999 international consensus conference of neuropathologists. New entities include chordoid glioma of the third ventricle, cerebellar liponeurocytoma, atypical teratoid/rhabdoid tumor, and perineurioma. Several histological variants were added, including tanycytic ependymoma, large cell medulloblastoma, and rhabdoid meningioma. The WHO grading scheme was updated and, for meningiomas, extensively revised. In recognition of the emerging role of molecular diagnostic approaches to tumor classification, genetic profiles have been emphasized, as in the distinct subtypes of glioblastoma and the already clinically useful 1p and 19q markers for oligodendroglioma and 22q/INI1 for atypical teratoid/rhabdoid tumors. In accord with the new WHO Blue Book series, the actual classification is accompanied by extensive descriptions and illustrations of clinicopathological characteristics of each tumor type, including molecular genetic features, predictive factors, and separate chapters on inherited tumor syndromes. The 2000 WHO classification of nervous system tumors aims at being used and implemented by the neuro-oncology and biomedical research communities worldwide.

Humans↗

French American British (FAB) morphological classification of childhood lymphoblastic leukaemia and its clinical importance.

As part of the Medical Research Council Leukaemia Trial UKALL VIII, 738 unselected children with acute lymphoblastic leukaemia (ALL) had the morphology of their marrow blast cells reviewed by a panel of three haematologists. Ninety four (13%) showed appearances classifiable as type L2 by the French American and British (FAB) cooperative group's criteria, five (0.7%) were typed L3, and the remaining 639 (86%) as L1. Disregarding the patients classified as L3, those with the L2 variant showed an inferior disease free survival to that of the remainder (p less than 0.01), and more of them failed to remit after receiving "standard" remission induction treatment (p less than 0.01). They included an excess of older children (p less than 0.01) with less profound marrow failure at diagnosis, and fewer of them expressed the common ALL antigen (p = 0.05). There was no association between L2 morphology and the diagnostic white cell count, sex, or the presence of a mediastinal mass. These findings confirm earlier reports that FAB L2 ALL is associated with a poor prognosis and that it occurs more commonly in older children. The high remission failure rate is a recent observation and indicates that alternative early treatment may be appropriate for such patients.

Actuarial Analysis↗

[Erysipelas. Clinicopathological classification and terminology].

Erysipelas and necrotizing fasciitis are the words used to qualify 2 well-defined aspects of acute microbial dermohypodermal infection. According to the literature a third word, "cellulitis" is used to describe a large variety of inflammatory conditions of soft tissues, either infectious or not. The authors who advocate using this word consider either that an infectious cellulitis is a clinical variant differing from erysipelas or fasciitis, or that it describes the whole spectrum of acute microbial infections of soft tissues, including these 2 entities. This term, whatever its meaning, has no anatomical or histopathological validation. It should be definitively deleted and replaced by "dermohypodermal infection".

Erysipelas↗

[Reflex-motor asymmetries in clinical neurology (mechanisms, differentiation, and clinical evaluation)].

The neurological status of almost 7,000 patients aged 18 to 20 years, as well as of normal subjects under the conditions of long-time stay in bed was examined. In a considerable number of observations, reflector motor asymmetries pointing to prevailing insufficiently of the left hemisphere were revealed. A classification of the asymmetries is suggested and cliniconeurological characteristics of the variants of genital and acquired asymmetries are presented. Criteria for differentiating residual and actual, functional, morphological, and mixed asymmetries are offered, and the role of the reflector motor asymmetries in diagnosing diseases and evaluating the patients' working capacity is determined.

Adolescent↗

Acute monocytic leukemias.

Normal monocytes and macrophages are characterized by peculiar ultrastructural and cytochemical features and, in addition, show characteristic membrane properties, a variety of special functional capacities, and important secretory activities. Almost all these cytological features can also be detected in leukemic monocytic cells, and it is quite conceivable that these peculiar features may influence or determine the clinical syndrome associated with the leukemic accumulation of monocytic cells. The morphological identification of monocytic leukemias is a very intriguing diagnostic problem and some controversies still exist concerning their cytological classification. For clinical as well as scientific purposes the diagnosis of monocytic leukemias should rely on the demonstration of specific monocytic features of the leukemic cells. Clinical findings frequently associated with acute monocytic leukemia include increased frequency of leukemic tissue infiltrations as well as increased tendency to hypokalemia and - especially in the "immature" variants - to disorders of hemostasis.

Cell Adhesion↗

Renal vasculitis.

The term 'vasculitides' encompasses a group of inflammatory disorders which may affect the kidney by damaging its blood supply. Although the kidney may be affected by many types of systemic vasculitis, renal involvement is particularly frequent in some forms of systemic necrotizing vasculitis which are considered primary, such as Wegener's granulomatosis and microscopic polyarteritis, including its 'renal-limited' variant. Renal vasculitis can occur at any age, but is seen particularly frequently in middle-aged and elderly subjects, in whom clinical presentation and prognosis are significantly worse. In addition to the specific problems related to age, this review focuses on a few aspects that are still the subjects of debate: classification, the role of ANCA, renal pathology and the treatment of renal vasculitis.

Age Factors↗

Follicular neoplasms of the thyroid: view, biases, and experiences.

The authors review the group of thyroid tumors characterized by a follicular growth pattern; these include follicular adenoma, follicular carcinoma, and the follicular variant of papillary carcinoma. Most of these lesions can be diagnosed with ease, but a subgroup has generated recent controversy in the literature. The authors present their views based on their experience with the cytologic and histologic diagnosis of these tumors and propose a scheme to assist in their classification and appropriate clinical management.

Adenocarcinoma, Follicular↗

The hypereosinophilic syndrome revisited.

Clinical and biological features of patients with the idiopathic hypereosinophilic syndrome (HES) are heterogeneous. Recent evidence suggests at least two distinct underlying hematological disorders involving myeloid and lymphoid cells, respectively. We therefore suggest that the term idiopathic should be abandoned in the classification of HES. This review defines the "myeloproliferative" and "lymphocytic" variants of the HES and addresses the management of each variant, focusing on diagnosis and treatment of the newly identified lymphocytic variant.

Cytokines↗