scholarly journals Primitive Neuroectodermal Tumor/Ewing Sarcoma Presenting with Pulmonary Nodular Lesions

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Selvi Asker ◽  
Fuat Sayir ◽  
Gulay Bulut ◽  
Aysel Sunnetcioglu ◽  
Selami Ekin ◽  
...  

Primitive neuroectodermal tumors (PNETs) and Ewing sarcoma (EWS) belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. EWS-PNETs that arise in the lung parenchyma involvement are extremely rare in adults. A case of a 32-year-old male presenting with chest pain and diffuse pulmonary nodules on chest X-ray and diagnosed with Ewing sarcoma-PNETs will be presented here.

2020 ◽  
pp. 1-3
Author(s):  
Guzmán-Casta Jordi

Primitive Neuroectodermal Tumors (PNET) and Ewing Sarcoma (EWS) belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. Common locations for EWS-PNET include chest wall, pelvis, and extremities. EWSPNETs that arise in the lung parenchyma without chest wall involvement are extremely rare in adults. We report a case of EWS-PNET of the lung in an adult and review the distinct clinical, pathological, and molecular features of these tumors.


2001 ◽  
Vol 95 (2) ◽  
pp. 270-275 ◽  
Author(s):  
Robert J. Weil ◽  
Zhengping Zhuang ◽  
Svetlana Pack ◽  
Shimareet Kumar ◽  
Lee Helman ◽  
...  

✓ Molecular biological techniques have begun to transform modern medicine. These techniques have shown promise in the pathological diagnosis of difficult or uncommon tumors. Accurate molecular diagnosis of the small round-cell tumors, for example, is especially important because divergent therapies may be required to eradicate such disparate lesions as neuroblastoma, lymphoma, rhabdomyosarcoma, central primitive neuroectodermal tumors/medulloblastoma, or Ewing sarcoma (ES). The authors present an unusual case of a primary, extraosseous ES arising from the intramedullary spinal cord, in which molecular studies were required for specific diagnosis and therapeutic guidance.


2000 ◽  
Vol 114 (3) ◽  
pp. 345-353 ◽  
Author(s):  
D. Ashley Hill ◽  
John D. Pfeifer ◽  
Edith F. Marley ◽  
Louis P. Dehner ◽  
Peter A. Humphrey ◽  
...  

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 108-126 ◽  
Author(s):  
Maria Tsokos ◽  
Rita D. Alaggio ◽  
Louis P. Dehner ◽  
Paul S. Dickman

Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.


1998 ◽  
Vol 1 (4) ◽  
pp. 295-299 ◽  
Author(s):  
Maliheh Khoddami ◽  
Jeremy Squire ◽  
Maria Zielenska ◽  
Paul Thorner

Melanotic neuroectodermal tumor of infancy is a rare but well-recognized entity in pediatric pathology. However, the relationship of this tumor to other pediatric small cell tumors with neuroectodermal features (such as neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, and desmoplastic small round cell tumor) is undetermined. Molecular genetic studies of melanotic neuroectodermal tumor of infancy have not been reported. We studied three typical cases of mela-notic neuroectodermal tumor of infancy in an attempt to link this tumor to other small cell tumors with well-characterized molecular genetic changes. Tests performed included: detection of MYCN gene amplification and deletion of 1p (all 3 cases), and presence of the t(11;22)(q24;q12) and the t(11;22)(p13;q12) translocations (2 of 3 cases). None of these tests yielded positive results. Thus, there is no genetic basis at present to link melanotic neuroectodermal tumor of infancy to neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, or desmoplastic small round cell tumor.


2005 ◽  
Vol 4 (2) ◽  
pp. 63-65
Author(s):  
Veronica Varney ◽  
◽  
Mary Warren ◽  
M Palmer ◽  
◽  
...  

A 61 year old former paramedic presented to A&E complaining of palpitations. He was found to be in atrial fibrillation, which reverted spontaneously to sinus rhythm. A chest x-ray taken at that time showed multiple pulmonary nodules consistent with metastatic malignancy (Figure 1). In the past he had been treated with amiodarone 200mg daily for 6 years following a previous diagnosis of atrial fibrillation, which had been attributed to alcoholic cardiomyopathy. He had discontinued the drug 8 months earlier, after selfdiagnosing hypotension and bradycardia. A previous chest X-ray, taken before starting amiodarone, was normal.


2021 ◽  
Vol 49 ◽  
Author(s):  
Bruno Alberigi ◽  
Alexandre Bendas ◽  
Denise Do Vale Soares ◽  
Loide Machado ◽  
Ana Carolina Rodrigues ◽  
...  

Background: Pneumothorax is a clinical condition which can cause respiratory distress. It can have as its origin traumatic causes or even classified as spontaneous, mainly related to diseases of the lung parenchyma. Lipoid pneumonia is rarely described in dogs, and it is characterized by globules of lipid in the alveolar spaces. Endogenous lipoid pneumonia (EnLP) occurs when lesions on pulmonary cells release cholesterol and other lipids in the alveoli. There is no clinical approach established for EnLP in veterinary patients. The aim of this report is to describe a case of a young Maltese dog, with recurrent spontaneous pneumothorax in which EnLP was diagnosed Post mortem.Case: A 2-year-old sexually intact male Maltese dog was evaluated for restrictive dyspnea. Clinicopathologic findings included cyanotic, muffled chest auscultation with hypersonic thoracic percussion. Chest x-ray demonstrated an increase in pleuropulmonary radio transparency and a floating-looking heart, indicating pneumothorax. Complete blood counts andbiochemical panel results were normal. Dirofilaria immitis antigen test results were negative. Computed tomography demonstrated slightly hyper-expanded pulmonary fields, with slightly enlarged reticular marking with areas of mild multicentric panlobular emphysema and a fracture on the sixth left rib. The treatment was focused on improving the breathing pattern through sedation, supplementation with oxygen, and thoracentesis. Owing to the reserved prognosis of the case, the unknown etiology of the recurrent pneumothorax, and the clinical worsening of the patient, the owner opted for euthanasia. Necropsy displayed multiple, circular whitish areas in the lungs, distributed over the surface of all lobes. Histopathological examination revealed pulmonary tissue with the subpleural micronodular foci, multifocal to coalescent, with a moderate accumulation of foamy intra-alveolar macrophages, occasionally multinucleate, associated with cholesterol crystals compatible with endogenous lipid pneumonia. Discussion: The patient presented with clinical signs and physical examination characteristics of pneumothorax at the first visit. After the pneumothorax diagnosis, and clinical stabilization of the patient. No predisposing factor for the formation of the pneumothorax was identified as the radiography revealed only bronchitis and blood tests were normal, the patient was thus discharged after 24 h, with the recommendations for observing the breathing pattern. Initially, spontaneous pneumothorax was suspected. The antibiotics were administered since bacterial pneumonia, although not confirmed on chest x-ray, is the main cause of pneumothorax in dogs is lung parenchyma disease. With the worsening of the clinical condition of the patient, CT was performed and did not demonstrate any findings that would justify the presence of pneumothorax. Despite the placement of the chest tube for facilitating the management of thoracentesis, there was no stabilization of the condition, enhancing the frequency of centesis procedures, which led to the decision to euthanize. The microscopic examination of the pulmonary alterations was decisive for the diagnostic conclusion. The visualization of the accumulation of foamy intra-alveolar macrophages, occasionally multinucleate, associated with cholesterol crystals, was responsible for the diagnosis of EnLP. This condition is rarely described in dogs and as in the present report, it is a noninfectious inflammatory condition, characterized by intra- or extracellular globules of lipid in the alveolar spaces. In the present report, although it was not possible to determine the etiology of EnLP, we can conclude that although rare, it can affect dogs and can generate severe clinical repercussions.


2021 ◽  
pp. 55-56
Author(s):  
K Pratyusha ◽  
Satish Arakeri ◽  
Surekha Arakeri

Introduction: Extraskeletal Ewing sarcoma/PNET is a small round cell sarcoma showing gene fusions of EWSR1-FLI1. A 28-year-old male patient presented with right ank pain and low gr Case Report: ade fever since 15 days. On examination: a mass was palpable in the right hypochondrium. Provisional diagnosis of Liver abscess has been made. USG abdomen shows features cystic lesion in the liver with internal septation ?Liver abscess /Hydatid cyst. Intraoperatively, tumor was seen attached to upper pole of kidney. Since tumor was large, it was ruptured intraoperatively and debulking surgery has been done. Under microscopy, tumor was arranged in sheets with intervening stroma showing desmoplastic reaction. Perivascular pseudorosettes are seen. The diagnosis of malignant small round cell tumor has been given. On immunohistochemistry, tumor cells are positive for Vimentin, CD99, NKX2.2 , FLI1, Neurolaments, Synaptophysin with focal immunoreactivity for EMA, Pancytokeratin. Final diagnosis was EXTRASKELETAL EWING SARCOMA/PNET. Discussion: Extra-skeletal Ewing sarcoma/PNET is malignant soft tissue tumor seen in chest wall, thigh, paravertebal region etc. Retroperitoneum is a least common site. Most common presentation is swelling in the soft tissue with compressive symptoms. Histologically, it is composed of undifferentiated small round cells. Conclusion: Clinical examination and radiological ndings leads to ambiguous diagnosis in Ewing sarcoma/PNET. Hence proper histopathological study is essential for nal diagnosis.


2003 ◽  
Vol 89 (3) ◽  
pp. 324-327 ◽  
Author(s):  
Rodrigo Fresco ◽  
Lyber Saldombide ◽  
Lilians Suárez

Askin tumor, or malignant small round cell tumor of the thoracopulmonary region, is an extremely infrequent entity occurring primarily in children and adolescents. Its histopathologic and cytogenetic features suggest that it belongs to the family of Ewing's sarcoma and primitive neuroectodermal tumors. We report the case of a 43-year-old woman affected by an Askin tumor with bone metastases at diagnosis, presenting synchronously with a plasmacytoma. This is the first reported case of the simultaneous occurrence of an Askin tumor and a malignant hemopathy. The progression of the former and the remission of the plasmacytoma during chemotherapy were remarkable, since Askin tumor treatment shares drugs used for the treatment of plasma cell tumors. Given the infrequent presentation of these diseases in a young adult and the coexistence of two neoplasias characterized by typical chromosomal abnormalities, we consider the possibility of a genetic cancer susceptibility in our patient.


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