scholarly journals Autonomic dysfunction in postoperative primitive neuroectodermal tumor of heart

2021 ◽  
Vol 23 (2) ◽  
pp. 126-129
Author(s):  
Abhijith K Anil ◽  
Ilavarasi Vanidassane ◽  
Ritesh Netam ◽  
Deepam Pushpam ◽  
Sameer Bakhshi ◽  
...  
Keyword(s):  
Orthostatic Hypotension ◽  
Autonomic Dysfunction ◽  
Right Atrium ◽  
Primitive Neuroectodermal Tumor ◽  
Autonomic Function ◽  
Neuroectodermal Tumor ◽  
Chemotherapy Cycle ◽  
Visual Biofeedback ◽  
Function Test ◽  
The Right

We present a patient with a primitive neuroectodermal tumor arising from the right atrium who experienced multiple syncope episodes daily, which had first appeared 1 month after surgery. The symptoms continued to worsen over the course of chemotherapy, and the autonomic function test (AFT) was performed after the 14th chemotherapy cycle. The AFT revealed orthostatic hypotension and reduced baroreflex-dependent sympathetic reactivity. Physical counterpressure techniques were applied with a visual biofeedback intervention, and were found to be effective in reducing the syncope episodes.

Rare pulmonary primitive neuroectodermal tumor metastasizing to the right atrium: a case report

2012 ◽  
Vol 29 (4) ◽  
pp. 2649-2653 ◽  
Author(s):  
Liwei Mao ◽  
Hongmei Wang ◽  
Guoqing Xie ◽  
Na Zhang ◽  
Guoqing Liao
Keyword(s):  
Case Report ◽  
Right Atrium ◽  
Primitive Neuroectodermal Tumor ◽  
Neuroectodermal Tumor ◽  
The Right

scholarly journals Primitive Neuroectodermal Tumor of the Liver: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Eduardo Cambruzzi ◽  
Enilde Eloena Guerra ◽  
Hamilton Cardoso Hilgert ◽  
Herbert Jorge Schmitz ◽  
Vinícius Lopes Silva ◽  
...  
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Young Patients ◽  
Neuroectodermal Tumor ◽  
Solid Mass ◽  
Resected Liver ◽  
The Central Nervous System ◽  
Right Lobe ◽  
Upper Abdominal ◽  
The Right

Primary liver sarcomas represent a rare group of neoplasias, with angiosarcoma being the most common histological type. Primitive neuroectodermal tumor (PNET) represents a high malignant neoplasia that usually affects the central nervous system and soft tissues. An 18-year-old male patient was admitted with clinical complains of pain in the right upper abdominal quadrant. The clinical evaluation revealed a solid mass in the right hepatic lobe. On the gross examination of the resected liver specimen, the right lobe of the liver was replaced by a yellow-red solid mass measuring 21 cm in its largest dimension. On the histopathology, a tumor composed of small round blue cells with little cytoplasm and round nuclei was identified. The lesion revealed positive immunoexpression for vimentin and CD99 and negative immunostaining for desmin, CD45, cytokeratin, and neuroblastoma protein, suggesting, then, the diagnosis of PNET. Although it is an unusual tumor, it should be considered in the differential diagnosis of liver masses, especially in young patients.

scholarly journals A rare case of primitive neuroectodermal tumor of the right lung.

1996 ◽  
Vol 35 (5) ◽  
pp. 460-465 ◽  
Author(s):  
Mayumi UENO ◽  
Junzo FUJIYAMA ◽  
Tadashi IDEI ◽  
Kunishi KAWAMATA ◽  
Yukitoshi SATOH ◽  
...  
Keyword(s):  
Rare Case ◽  
Primitive Neuroectodermal Tumor ◽  
Neuroectodermal Tumor ◽  
The Right

scholarly journals Malignant Gastrointestinal Neuroectodermal Tumor in the Right Atrium: The First Case Report Presenting with an Extra-Gastrointestinal Mass

2021 ◽  
Author(s):  
Zhiwen Li ◽  
Xiaohong Pu ◽  
Yao Fu ◽  
Lin Li ◽  
Yuemei Xu ◽  
...  
Keyword(s):  
Case Report ◽  
Right Atrium ◽  
Tumor Location ◽  
Neuroectodermal Tumor ◽  
Case Presentation ◽  
First Case ◽  
Mitotic Figures ◽  
The Right ◽  
Whole Transcriptome

Abstract Background: Malignant gastrointestinal neuroectodermal tumor is an extremely rare soft tissue sarcoma which was firstly described in 2003 but until recently it had been designated as a definite new entity. According to the previous literatures, Malignant gastrointestinal neuroectodermal tumor was almost exclusively occurred in gastrointestinal tract.Case presentation: A 62-year-old male showed a mass in right atrium and the occupying mass was founded on the right ventricular diaphragm and involved the right atrium along the coronary sinus during the operation. Microscopically, the tumor characterized by the solid sheet and pseudopapillary focally architectures, and was composed of small to medium cells with round or oval nuclei, variable amount of eosinophilic or clear, and frequent mitotic figures. Immunohistochemically, the neoplastic cells were positive for S100 and SOX-10 but negative for HMB-45, A103 and CD99. EWSR1-AFTF1 rearrangement was detected by fluorescence in situ hybridization and further confirmed involving fusion of EWSR1 exon 8 with ATF1 exon 4 by whole transcriptome sequence analysis.Conclusions: This is the first case report of extra-gastrointestinal Malignant gastrointestinal neuroectodermal tumor that occurring in the right atrium，which remind of the new prospect of the tumor location.

scholarly journals Supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in adult

2014 ◽  
Vol 5 (02) ◽  
pp. 176-179 ◽  
Author(s):  
Bashar Abuzayed ◽  
Wiam Khreisat ◽  
William Maaiah ◽  
Saoud Agailat
Keyword(s):  
Female Patient ◽  
Intracranial Hemorrhage ◽  
Radiation Oncology ◽  
Primitive Neuroectodermal Tumor ◽  
Histopathological Examination ◽  
Neuroectodermal Tumor ◽  
Loss Of Consciousness ◽  
Postoperative Course ◽  
Supratentorial Primitive Neuroectodermal Tumor ◽  
The Right

ABSTRACTA 24-year-old female patient presented with complaints of nausea, vomiting and of loss of consciousness lasted for 15 minutes with left sided weakness. Neuroradiological evaluation revealed a hemorrhagic mass lesion in the right frontal lobe. The patient was operated and intraoperative findings showed a cortical-subcortical hematoma including hemorrhagic and disrupted tissue with a pathologic purple tissue on the periphery of the hematoma. Postoperative course was uneventful and postoperative histopathological examination revealed primitive neuroectodermal tumor. The patient was then referred to medical and radiation oncology clinics for further evaluation and treatment.

scholarly journals Primitive Neuroectodermal Tumor of Axilla in an Adult: A Rare Entity

2021 ◽  
Vol 3 (2) ◽  
pp. 1-2
Author(s):  
Shravan S Shetty ◽  
◽  
Vidhi Shah ◽  
Eshpuniyani Prriya Prriya ◽  
◽  
...  
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Subcutaneous Tissue ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Poorly Differentiated ◽  
The Right ◽  
Disease Free

Primitive neuroectodermal tumors (PNETs) are poorly differentiated small round cell neoplasms which mainly affect children and are not commonly seen in adults. Superficial primitive neuroectodermal tumors are rare and have a favourable prognosis compared to conventional deep seated tumors. We report a case of a 62 year old gentleman with a primitive neuroectodermal tumor arising from the subcutaneous tissue of the right axilla. He was treated with multimodal treatment including surgery and radiotherapy. He is alive and disease free at 2 year follow up.

scholarly journals Anti-PD-1 Immunotherapy Combined With Antiangiogenic Therapy as Salvage Therapy in a Patient With Refractory Metastatic Peripheral Primitive Neuroectodermal Tumor: A Case Report and Literature Review

2021 ◽  
Author(s):  
Qiaoyun Chen ◽  
Ronghui Jin ◽  
Xu Li ◽  
Qifan Wu ◽  
Gaili An
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Kinase Inhibitor ◽  
Treatment Plan ◽  
Antiangiogenic Therapy ◽  
Malignant Neoplasm ◽  
Neuroectodermal Tumor ◽  
Comprehensive Treatment ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
New Ideas ◽  
The Right

Abstract Background: Peripheral primitive neuroectodermal tumor (pPNET) is a relatively rare malignant neoplasm that usually occurs in children and young adults,associated with poor prognosis. However, standard treatment for refractory pPNET has not been determined.Case presentation: A case of a 38-year-old woman with pPNET on the right shoulder and back,she had gained up to seven-and-a-half years suvival undergoing these comprehensive treatment including surgery, radiotherapy, chemotherapy, antiangiogenic treatment. However, the tumor eventually progressed after receiving multiline treatment.With the patient's strong desire to receive immunotherapy, we finally adjusted the treatment plan to " anti-angiogenic tyrosine kinase inhibitor Lenvatinib combined with PD-1 inhibitor Toripalimab" for 2 cycles.Unfortunately,she developed grade 2-3 immune pneumonia after treatment.Conclusions: To our knowledge, this is the first report of immunotherapy combined with antiangiogenic therapy in pPNET,which provides new ideas for the treatment of refractory pPNET.

A case of peripheral primitive neuroectodermal tumor of the ovary

2004 ◽  
Vol 14 (2) ◽  
pp. 370-372 ◽  
Author(s):  
K.-J. Kim ◽  
B.-W. Jang ◽  
S.-K. Lee ◽  
B.-K. Kim ◽  
S.-L. Nam
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Case Reports ◽  
The Body ◽  
Neuroectodermal Tumor ◽  
Pelvic Cavity ◽  
Age Group ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Children And Young Adults ◽  
The Right

Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family. PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults. It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues. There have been a small number of case reports of PNET arising in the ovary. We presented a case of PNET arising in the right ovary of an 18-year-old woman. The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging. We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycink, Cyclophosphamide/Doxorubicin (VACA). She died after 10 months due to septic shock.

scholarly journals Impaired cortical autonomic responses during sympathetic activation in neurogenic orthostatic hypotension characterized by postganglionic autonomic dysfunction

2018 ◽  
Vol 125 (4) ◽  
pp. 1210-1217 ◽  
Author(s):  
Jacquie Baker ◽  
Justin R. Paturel ◽  
Kurt Kimpinski
Keyword(s):  
Orthostatic Hypotension ◽  
Autonomic Dysfunction ◽  
Valsalva Maneuver ◽  
Late Phase ◽  
Brain Regions ◽  
Neurogenic Orthostatic Hypotension ◽  
Sympathetic Activation ◽  
The Right ◽  
Cardinal Feature ◽  
Phase Iv

Neurogenic orthostatic hypotension (NOH) is a cardinal feature of autonomic dysfunction. The cortical autonomic network (CAN) is a network of brain regions associated with autonomic function. Therefore, our objective was to investigate whether impairment of CAN structures is involved in the pathophysiology of NOH. Fifteen controls (63 ± 13 yr) and 15 NOH patients (67 ± 6 yr; P = 0.2) with peripheral autonomic dysfunction completed standard tests of parasympathetic [deep breathing (DB)] and sympathetic [Valsalva maneuver (VM)] activation during a functional MRI. Blood-oxygen-level dependent (BOLD) contrasts were obtained and contrasted. Compared with controls, patients had significantly smaller heart rate responses to DB (control: 15.23 ± 9.6 vs. NOH: 5.7 ± 2.1) and Valsalva ratios (control: 2.1 ± 0.47 vs. NOH: 1.2 ± 0.1; P < 0.001). NOH patients had absent adrenergic phases (late phase II and phase IV) during VM as per a qualitative analysis. During VM, controls had greater activation in the right hippocampus (T-value: 8.03), left posterior cingulate (TL: 7.6), and bilateral thalamus (TR: 7.41, TL: 8.45; P < 0.05). During phase IV, controls had greater activation in the right hippocampus (TR: 5.78l P < 0.05). Following subtraction analysis, no significant differences were evident during DB. In conclusion, NOH patients have significantly less CAN activation during sympathetic, but not parasympathetic, activation. Impaired CANs associated with sympathetic activation may be involved in the pathophysiology of NOH. NEW & NOTEWORTHY Neurogenic orthostatic hypotension (NOH) is a cardinal feature of autonomic dysfunction characterized by failure of reflexive sympathetic activation. Our result reveal that patients with autonomic dysfunction caused by postganglionic sympathetic impairment also have impaired activation of structures within the cortical autonomic network. Impaired activation is evident during a test of sympathetic, but not parasympathetic, activation. Impaired cortical autonomic networks associated with sympathetic activation may be involved in the pathophysiology of NOH.

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