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Journal of the College of Physicians... Nov 2021A 21-year male presenting with left testicular mass and retroperitoneal lymphadenopathy underwent radical orchiectomy; and his pathological examination showed a mixed...
A 21-year male presenting with left testicular mass and retroperitoneal lymphadenopathy underwent radical orchiectomy; and his pathological examination showed a mixed germ cell tumor composed of primitive neuroectodermal tumor mixed with mature teratoma. Six cycles of IE (ifosfamide, etoposide) and VAC (vincristine, doxorubicin, cyclophosphamide) chemotherapy were given after sperm preservation. He then underwent retroperitoneal lymph node dissection (RPLND). No tumor was detected in the removed lymph nodes, and all lymph nodes were reported as showing reactive changes. Key Words: Chemotherapy, Primitive neuroectodermal tumor, Surgery, Teratom, Testis.
Topics: Antineoplastic Combined Chemotherapy Protocols; Humans; Lymph Node Excision; Lymph Nodes; Male; Neoplasms, Germ Cell and Embryonal; Neuroectodermal Tumors, Primitive; Retroperitoneal Space; Teratoma; Testicular Neoplasms
PubMed: 34689500
DOI: 10.29271/jcpsp.2021.11.1362 -
American Journal of Surgery Oct 1981A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare...
A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare among blacks. Most patients presented with locally advanced disease. However, regional and distant metastases at the time of initial diagnosis are uncommon. Local recurrence at the primary site was very common, and this reflects either the conservative initial surgical treatment employed or the multicentric nature of the tumor. Cervical lymph node metastasis is present in less than 10 percent of patients at the time of diagnosis. Survival rates were better in patients with early stages of disease. From this study we conclude that the current management of these tumors should consist of a combination of radiation and surgery. Radical resection followed by postoperative radiotherapy appears to be the treatment combination of choice. The 5 year survival rate in our series was 52 percent.
Topics: Adolescent; Adult; Age Factors; Aged; Child; Humans; Middle Aged; Neuroectodermal Tumors, Primitive, Peripheral; Nose Neoplasms; Olfactory Mucosa; Retrospective Studies
PubMed: 7283046
DOI: 10.1016/0002-9610(81)90374-3 -
Developmental Dynamics : An Official... Dec 2008Establishment of left-right asymmetry in the mouse embryo depends on leftward laminar fluid flow in the node, which initiates a signaling cascade that is confined to the... (Review)
Review
Establishment of left-right asymmetry in the mouse embryo depends on leftward laminar fluid flow in the node, which initiates a signaling cascade that is confined to the left side of the embryo. Leftward fluid flow depends on two cellular processes: motility of the cilia that generate the flow and morphogenesis of the node, the structure where the cilia reside. Here, we provide an overview of the current understanding and unresolved questions about the regulation of ciliary motility and node structure. Analysis of mouse mutants has shown that the motile cilia must have a specific structure and length, and that they must point posteriorly to generate the necessary leftward fluid flow. However, the precise structure of the motile cilia is not clear and the mechanisms that position cilia on node cells have not been defined. The mouse node is a teardrop-shaped pit at the distal tip of the early embryo, but the morphogenetic events that create the mature node from cells derived from the primitive streak are only beginning to be characterized. Recent live imaging experiments support earlier scanning electron microscopy (SEM) studies and show that node assembly is a multi-step process in which clusters of node precursors appear on the embryo surface as overlying endoderm cells are removed. We present additional SEM and confocal microscopy studies that help define the transition stages during node morphogenesis. After the initiation of left-sided signaling, the notochordal plate, which is contiguous with the node, generates a barrier at the embryonic midline that restricts the cascade of gene expression to the left side of the embryo. The field is now poised to dissect the genetic and cellular mechanisms that create and organize the specialized cells of the node and midline that are essential for left-right asymmetry.
Topics: Animals; Body Patterning; Embryo, Mammalian; Gene Expression Regulation, Developmental; Mice; Morphogenesis; Notochord
PubMed: 18629866
DOI: 10.1002/dvdy.21598 -
Journal of Electrocardiology 2019Cardiac pacemaker cells, also named P-cells (pale cytoplasm, pacemaker, phylogenetically primitive), including cells of the sinoatrial node, are heterogeneous in size,... (Review)
Review
Cardiac pacemaker cells, also named P-cells (pale cytoplasm, pacemaker, phylogenetically primitive), including cells of the sinoatrial node, are heterogeneous in size, morphology, and electrophysiological characteristics. The exact extent to which these cells differ electrophysiologically in the human heart is unclear, yet it is critical for the understanding of normal cellular function. In this review, we describe major ionic currents and Ca clocks acting on Ca release in the sarcoplasmic reticulum. We also explain the external regulation of the heart rate controlled by the two branches of the autonomic (involuntary) nervous system: the sympathetic and the parasympathetic nervous system. Vagal stimulus causes bradycardia, rapid and short-duration modulation, and controls rapid responses, and increases heart rate variability. A typical example is constituted by phasic or respiratory sinus arrhythmia, characterized by pronounced vagal activity, more frequent in children and young individuals.
Topics: Autonomic Nervous System; Bradycardia; Child; Electrocardiography; Heart Rate; Humans; Sinoatrial Node
PubMed: 31654967
DOI: 10.1016/j.jelectrocard.2019.08.013 -
Proceedings of the National Academy of... Feb 2022In warm-blooded vertebrate embryos (mammals and birds), the axial tissues of the body form from a growth zone at the tail end, Hensen's node, which generates neural,...
In warm-blooded vertebrate embryos (mammals and birds), the axial tissues of the body form from a growth zone at the tail end, Hensen's node, which generates neural, mesodermal, and endodermal structures along the midline. While most cells only pass through this region, the node has been suggested to contain a small population of resident stem cells. However, it is unknown whether the rest of the node constitutes an instructive niche that specifies this self-renewal behavior. Here, we use heterotopic transplantation of groups and single cells and show that cells not destined to enter the node can become resident and self-renew. Long-term resident cells are restricted to the posterior part of the node and single-cell RNA-sequencing reveals that the majority of these resident cells preferentially express G2/M phase cell-cycle-related genes. These results provide strong evidence that the node functions as a niche to maintain self-renewal of axial progenitors.
Topics: Animals; Body Patterning; Chick Embryo; Endoderm; Gastrula; Mesoderm; Nervous System; Notochord; Organizers, Embryonic; Stem Cell Niche; Stem Cells
PubMed: 35101917
DOI: 10.1073/pnas.2108935119 -
World Journal of Surgical Oncology Apr 2020Gastrinoma is a rare form of neuroendocrine neoplasm. The presence of a primary lymph node localization of gastrinoma is a much debated and controversial topic in the... (Review)
Review
BACKGROUND
Gastrinoma is a rare form of neuroendocrine neoplasm. The presence of a primary lymph node localization of gastrinoma is a much debated and controversial topic in the literature, as regards whether these cases represent metastatic disease from an as yet unidentified primary tumor, or the de novo occurrence of a gastrinoma in a lymph node.
CASE PRESENTATION
We report the case of a 24-year-old male with intense epigastric pain treated at the beginning with high dose proton pump inhibitors. Further workup with CT and subsequent laparotomy revealed a single peripancreatic lymph node. Histological examination highlighted a well-differentiated neuroendocrine tumor.
CONCLUSION
This case underlines that the primitive lymph node gastrinoma is a distinct nosological entity with a precise location in the context of rare neuroendocrine tumors that should be considered when specific symptoms are associated with the identification of isolated lymph nodes, after excluding any possible primitive locations of neoplastic localization.
Topics: Abdominal Pain; Gastrinoma; Humans; Lymph Node Excision; Lymph Nodes; Lymphadenopathy; Male; Tomography, X-Ray Computed; Treatment Outcome; Young Adult
PubMed: 32345299
DOI: 10.1186/s12957-020-01860-5 -
Asia-Pacific Journal of Ophthalmology... 2018Retinoblastoma can present in 1 or both eyes and is the most common intraocular malignancy in childhood. It is typically initiated by biallelic mutation of the RB1 tumor... (Review)
Review
Retinoblastoma can present in 1 or both eyes and is the most common intraocular malignancy in childhood. It is typically initiated by biallelic mutation of the RB1 tumor suppressor gene, leading to malignant transformation of primitive retinal cells. The most common presentation is leukocoria, followed by strabismus. Heritable retinoblastoma accounts for 45% of all cases, with 80% being bilateral. Treatment and prognosis of retinoblastoma is dictated by the disease stage at initial presentation. The 8th Edition American Joint Committee on Cancer (AJCC) TNMH (tumor, node, metastasis, heritable trait) staging system defines evidence-based clinical and pathological staging for overall prognosis for eye(s) and child. Multiple treatment options are available in 2018 for retinoblastoma management with a multidisciplinary team, including pediatric ocular oncology, medical oncology, radiation oncology, genetics, nursing, and social work. Survival exceeds 95% when disease is diagnosed early and treated in centers specializing in retinoblastoma. However, survival rates are less than 50% with extraocular tumor dissemination. We summarize the epidemiology, genetics, prenatal screening, diagnosis, classification, investigations, and current therapeutic options in the management of retinoblastoma.
Topics: Child; DNA, Neoplasm; Genes, Retinoblastoma; Genetic Testing; Humans; Mutation; Ophthalmologists; Retinal Neoplasms; Retinoblastoma
PubMed: 29737052
DOI: 10.22608/APO.201870 -
Advances in Surgery Sep 2023Well-differentiated nonfunctional pancreatic neuroendocrine tumors are often indolent neoplasms without lymph node (LN) metastasis at diagnosis. Patients with PNETs that... (Review)
Review
Well-differentiated nonfunctional pancreatic neuroendocrine tumors are often indolent neoplasms without lymph node (LN) metastasis at diagnosis. Patients with PNETs that are functional or >2 cm should have surgical resection as per the standard of care. However, in appropriately selected patients with NF PNETs <2 cm who are at low risk of LN metastasis, the extent of surgery and lymphadenectomy could be limited.
Topics: Humans; Lymph Node Excision; Pancreatectomy; Pancreatic Neoplasms; Neuroectodermal Tumors, Primitive; Lymph Nodes; Neuroendocrine Tumors; Retrospective Studies
PubMed: 37536861
DOI: 10.1016/j.yasu.2023.06.001 -
Archives of Iranian Medicine Apr 2015Primitive neuroectodermal tumors are fairly rare in uterus. A case of uterine body primitive neuroectodermal tumor in a 32-year-old Iranian woman is presented. The...
Primitive neuroectodermal tumors are fairly rare in uterus. A case of uterine body primitive neuroectodermal tumor in a 32-year-old Iranian woman is presented. The patient was admitted with abdominal pain and fever and underwent emergency exploratory surgery with total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Posterior wall of the uterus was necrotic and ruptured and a huge tumor disrupted the uterine body. The tumor was strongly positive for CD99, NSE, and chromogranin; No reaction was seen for CD10, CD45 and myogenin. To the best of our knowledge, this is the first report of an uterine body primitive neuroectodermal tumor and the second report of uterine primitive neuroectodermal tumor from Iran.
Topics: Adult; Biomarkers; Female; Humans; Hysterectomy; Magnetic Resonance Imaging; Neuroectodermal Tumors, Primitive; Ovariectomy; Uterine Neoplasms; Uterus
PubMed: 25841950
DOI: No ID Found -
Zic3 is required in the migrating primitive streak for node morphogenesis and left-right patterning.Human Molecular Genetics May 2013In humans, loss-of-function mutations in ZIC3 cause isolated cardiovascular malformations and X-linked heterotaxy, a disorder with abnormal left-right asymmetry of...
In humans, loss-of-function mutations in ZIC3 cause isolated cardiovascular malformations and X-linked heterotaxy, a disorder with abnormal left-right asymmetry of organs. Zic3 null mice recapitulate the human heterotaxy phenotype but also have early gastrulation defects, axial patterning defects and neural tube defects complicating an assessment of the role of Zic3 in cardiac development. Zic3 is expressed ubiquitously during critical stages of left-right patterning but its later expression in the developing heart remains controversial and the molecular mechanism(s) by which it causes heterotaxy are unknown. To define the temporal and spatial requirements, for Zic3 in left-right patterning, we generated conditional Zic3 mice and Zic3-LacZ-BAC reporter mice. The latter provide compelling evidence that Zic3 is expressed in the mouse node and absent in the heart. Conditional deletion using T-Cre identifies a requirement for Zic3 in the primitive streak and migrating mesoderm for proper left-right patterning and cardiac development. In contrast, Zic3 is not required in heart progenitors or the cardiac compartment. In addition, the data demonstrate abnormal node morphogenesis in Zic3 null mice and identify similar node dysplasia when Zic3 was specifically deleted from the migrating mesoderm and primitive streak. These results define the temporal and spatial requirements for Zic3 in node morphogenesis, left-right patterning and cardiac development and suggest the possibility that a requirement for Zic3 in node ultrastructure underlies its role in heterotaxy and laterality disorders.
Topics: Animals; Dextrocardia; Gene Deletion; Gene Expression Regulation, Developmental; Genetic Diseases, X-Linked; Heart; Heterotaxy Syndrome; Homeodomain Proteins; Humans; Mice; Mice, Mutant Strains; Organogenesis; Transcription Factors
PubMed: 23303524
DOI: 10.1093/hmg/ddt001