Neuroblastoma, an embryonal cancer of the autonomic nervous system, is the most common cancer diagnosed during the first year of life. Although neuroblastoma accounts for disproportionately high morbidity and mortality among childhood cancers, it has one of the highest rates of spontaneous and complete regression. The author discusses recent advances in our understanding of neuroblastoma.

/pdf/recent-advances-in-neuroblastoma-40yd8bqy2g.pdf

Recent advances in neuroblastoma.

Our forefathers in pathology, on observing cancer tissue under the microscope in the mid-19th century, noticed the similarity between embryonic tissue and cancer, and suggested that tumors arise from embryo-like cells [1] , [2] [Recherches dur le Traitement du Cancer, etc. Paris. (1829); Editoral Archiv fuer pathologische Anatomie und Physiologie und fuer klinische Medizin 8 (1855) 23]. The concept that adult tissues contain embryonic remnants that generally lie dormant, but that could be activated to become cancer was later formalized by Cohnheim [3] , [4] [Path. Anat. Physiol. Klin. Med. 40 (1867) 1–79; Virchows Arch. 65 (1875) 64] and Durante [5] [Arch. Memori ed Osservazioni di Chirugia Practica 11 (1874) 217–226], as the “embryonal rest” theory of cancer. An updated version of the embryonal rest theory of cancer is that cancers arise from tissue stem cells in adults. Analysis of the cellular origin of carcinomas of different organs indicates that there is, in each instance, a determined stem cell required for normal tissue renewal that is the most likely cell of origin of carcinomas [6] [Lab. Investig. 70 (1994) 6–22]. In the present review, the nature of normal stem cells (embryonal, germinal and somatic) is presented and their relationships to cancer are further expanded. Cell signaling pathways shared by embryonic cells and cancer cells suggest a possible link between embryonic cells and cancer cells. Wilm’s tumors (nephroblastomas) and neuroblastomas are presented as possible tumors of embryonic rests in children. Teratocarcinoma is used as the classic example of the totipotent cancer stem cell which can be influenced by its environment to differentiate into a mature adult cell. The observation that “promotion” of an epidermal cancer may be accomplished months or even years after the initial exposure to carcinogen (“initiation”), implies that the original carcinogenic event occurs in a long-lived epithelial stem cell population. The cellular events during hepatocarcinogenesis illustrate that cancers may arise from cells at various stages of differentiation in the hepatocyte lineage. Examples of genetic mutations in epithelial and hematopoietic cancers show how specific alterations in gene expression may be manifested as maturation arrest of a cell lineage at a specific stage of differentation. Understanding the signals that control normal development may eventually lead us to insights in treating cancer by inducing its differentiation (differentiation therapy). Retinoid acid (RA) induced differentiation therapy has acquired a therapeutic niche in treatment of acute promyelocytic leukemia and the ability of RA to prevent cancer is currently under examination.

Stem cell origin of cancer and differentiation therapy

Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma are tumors of the sympathetic nervous system that arise from primitive sympathogonia and are referred to collectively as neuroblastic tumors. They arise wherever sympathetic tissue exists and may be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis. The three tumors differ in their degree of cellular and extracellular maturation; immature tumors tend to be aggressive and occur in younger patients (median age, just under 2 years), whereas mature tumors occur in older children (median age, approximately 7 years) and tend to behave in a benign fashion. The most benign tumor is the ganglioneuroma, which is composed of gangliocytes and mature stroma. Ganglioneuroblastoma is composed of both mature gangliocytes and immature neuroblasts and has intermediate malignant potential. Neuroblastoma is the most immature, undifferentiated, and malignant tumor of the three. Neuroblastoma, however, may have a relatively benign course, even when metastatic. Thus, these neuroblastic tumors vary widely in their biologic behavior. Features such as DNA content, tumor proto-oncogenes, and catecholamine synthesis influence prognosis, and their presence or absence aids in categorizing patients as high, intermediate, or low risk. Treatment consists of surgery and, usually, chemotherapy. Despite recent advances in treatment, including bone marrow transplantation, neuroblastoma remains a relatively lethal tumor, accounting for 10% of pediatric cancers but 15% of cancer deaths in children.

Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.

Neuroblastoma, the most common extracranial solid tumor of childhood, is thought to originate from undifferentiated neural crest cells. Amplification of the MYC family member, MYCN, is found in ∼25% of cases and correlates with high-risk disease and poor prognosis. Currently, amplification of MYCN remains the best-characterized genetic marker of risk in neuroblastoma. This article reviews roles for MYCN in neuroblastoma and highlights recent identification of other driver mutations. Strategies to target MYCN at the level of protein stability and transcription are also reviewed.

/pdf/neuroblastoma-and-mycn-1gz3s440we.pdf

Neuroblastoma and MYCN

MANY EXPLANATIONS have been advanced for the low thrombocyte (platelet) count in primary or idiopathic thrombocytopenic purpura, but so far all theories lack substantial proof. The disease is somewhat commoner in females and occurs more frequently in the first half of life, although it has been seen in persons of all age groups. A familial history of easy bruising and prolonged bleeding is frequently mentioned in case histories, but few hematological data are offered to support the supposition that thrombocytopenia has occurred in other members of the family. Reports of transitory thrombocytopenia in infants of women with thrombocytopenic purpura are of particular interest, since these cases suggest that an agent producing the thrombocytopenia is transmissible through the placenta.1Both increased destruction and decreased formation of thrombocytes have been advanced as causes of primary thrombocytopenia. Since there is no technic comparable to the reticulocyte count for estimating the rate of

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=intemed&articleId=555362&pdfFileName=archinte_87_1_005.pdf

Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology.

The Histogenesis Of The Blood Platelets

~/[ycetoma is a chronic inflammatory process, most commonly affect-hag the tissues of the foot~ in which suppurative nodular swellings, sinus formation, and enlargement and distortion of the parts are prominent features. The bones may become involved and undergo a rarefying osteitis. The affection is characterized by the presence in the diseased tissue and in the discharge from sinuses, of peculiar granules, usually not more than one millimetre in diameter, but sometimes larger. These granules in certain cases are of a black color, of irregular shape, and are hard and rather brittle. In general they resemble grains of gunpowder. In other cases the granules are whitish, greyish or yellowish in color and are of a soft or cheesy consistency. They have been compared to fish-roe in appearance. A very few cases are also recorded in which the granules were of a red color. There is no good evidence to show that more than one kind of granules are ever present in any one case of the disease. On account of the fact that at least two very different kinds of granules are found associated with the lesions, two varieties or forms of the disease are recognized: the " me]anoid" or black variety, in which the granules are black, and the " ochroid" or pale variety, in which the granules are white to yellow in color. A. Vandyke Carter, who was the first to show that mycetoma was a special pathological process and to name the disease itself and its varieties, considered the granules to be the cause of the affection and regarded them as representing stages in the life history of one and the same fungus. Carter claimed that he had cultivated this fungus and that it grew as a pink

/pdf/a-case-of-mycetoma-madura-foot-14cyxzseod.pdf

A case of mycetoma (madura foot).

Late labelling of iso-x chromosome

Anti-Tumor Activity of IPI-504, a Novel Hsp90 Inhibitor in Multiple Myeloma.

The glanders nodule in the class of cases studied by us is in no sense analogous to the miliary tubercle in its histogenesis, and our studies afford no support to Baumgarten's views. The primary effect of the bacillus of glanders on a tissue we found to be not a production of epithelioid cells, which undergo necrosis and invasion by leucocytes, as happens in the cases in which the bacillus of tuberculosis is concerned, but to be the production of primary necrosis of the tissue, followed by inflammatory exudation, often of a suppurative character. Degenerative changes rapidly ensue in the inflammatory products. These conclusions are in harmony with the observations of Tedeschi, above referred to.

https://rupress.org/jem/article-pdf/1/4/577/1126232/577.pdf

The histologigal lesions of acute glanders in man and of experimental glanders in the guinea-pig

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