Cardiac Primitive Neuroectodermal Tumor Presenting As Acute Coronary Syndrome

Rajappa, Senthil; Gundeti, Sadashivudu; Varadpande, Lalit; Bethune, Norman; Rao, Satish; Digumarti, Raghunadharao

doi:10.1200/jco.2006.09.2346

Cited by 7 publications

(16 citation statements)

References 6 publications

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“…To our knowledge, there have been 9 reported cases of primary cardiac PNETs. Arterial and ventricular locations are the common sites [ 6 ]. So far, only three cases of PNETs in the pericardium has been reported [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

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Primitive neuroectodermal tumor of the pericardium: a case report and literature review

Wang

Xiao

et al. 2021

BMC Cardiovasc Disord

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Background The primitive neuroectodermal tumors (PNETs) are a family of highly malignant tumors with a multidirectional differential potential. The tumors are characterized by aggressive small round tumor cells that originate from the spinal cord of the central and sympathetic nervous systems. Cases involving the pericardium are extremely rare. Herein, we present a case of peripheral primitive neuroectodermal tumor (pPNET) that originated in the pericardium. Case presentation A 23-year-old woman presented with cough and progressive dyspnea for 1 month, followed by eyelid and facial edema for 10 days, without any apparent cause. Significantly elevated tumor markers were detected in her blood. A cardiac ultrasound revealed a 74 mm × 61 mm spherical mass that was attached to the left pericardium, as well as massive pericardial effusion. Positron emission tomography-CT (PET-CT) showed focal hypermetabolism in the left pericardium. Via histopathology and immunohistochemistry, the spherical mass was identified as PNETS. The patient was successfully treated with a combination of surgical resection via thoracotomy and postoperative chemotherapy, and she was disease-free for 7 years at follow-up. Unfortunately, at 7 years after the treatment, the patient’s pPNET recurred. Positron emission tomography-MRI (PET-MRI) and 64-slice coronary CTA revealed that the aorta and multiple coronary arteries were involved. Subsequently, the patient refused a heart transplant and voluntarily left the hospital. Conclusions This paper reports on a rare and recurrent case of PNET in the parietal pericardium. With respect to the different biologic characteristics and prognoses of pPNETs (compared to other known pericardium tumors), it is essential to consider this entity as a differential diagnosis in pericardium tumors.

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Section: Discussionmentioning

confidence: 99%

“…Transthoracic echocardiography is the first choice for the initial diagnosis. CT and MRI scans play a main role in diagnosis and evaluation of the local invasion and distant metastasis [ 6 ]. Consistently with previous reports [ 1 ], the tumor in this present study on CT/MR image was rounded masses with well-defined margins.…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor of the pericardium: a case report and literature review

Wang

Xiao

et al. 2021

BMC Cardiovasc Disord

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“…The natural history of cardiac tumors has been revolutionized by the prevalent use of echocardiography and advanced surgical techniques ranging from simple resection of intrapericardial tumors to cardiac transplantation for unresectable tumors with the aid of extracorporeal circulation [5]. Cardiac tumors are metastatic at presentation in 80% of cases and primary cardiac tumors constitute only 0.001%-0.28% of autopsy series [2,3,6]. Among the common primary tumors, atrial myxoma constitutes 70%-77% [5,7].…”

Section: Discussionmentioning

confidence: 99%

“…Cardiac PNET is an extremely rare tumor, with only 6 cases reported in literature to date since it was first described by Charney, et al in 1996 [1]. Cardiac PNETs have poor prognosis with extensive metastasis at presentation, and have been felt to warrant aggressive treatment with chemotherapy and radiotherapy, with limited surgical options [2][3][4]. Though recurrences have been commonly reported, there have been two cases described in which complete resection of tumor in patients without extracardiac or metastatic spread was shown to be beneficial, without recurrence in subsequent short follow up.…”

Section: Introductionmentioning

confidence: 99%

Cardiac Primitive Neuroectodermal Tumor (PNET) with Metastasis at Presentation

Jawahar¹

2017

Ann Hematol Oncol

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Primitive neuroectodermal tumors (PNET) are rare tumors and PNET of cardiac origin are extremely rare, with only 6 cases reported since 1996. Cardiac PNET cases have been associated with poor prognosis with extensive metastasis at presentation, and warrant aggressive treatment.We present a 19-year-old male patient who presented with headache and falls; workup demonstrated cerebellar lesions which were resected and noted to be metastatic PNET, and his preoperative transthoracic echocardiography showed an irregular cardiac mass protruding into the interventricular septum which was later identified as the primary malignant mass. Despite the mass being of the cardiac primary origin, he did not have cardiac symptoms until 15 months after his initial symptoms.

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“… a From Lee et al [28] and Rizzieri et al [30] b From Lacasce et al [31], Abramson et al [36] c From Magrath et al [25] d From Dunleavy et al [33] e From Thomas et al [32] BM bone marrow; CALGB Cancer and Leukemia Group B; CI continuous infusion; CNS central nervous system; CODOX-M cyclophosphamide, vincristine, doxorubicin, methotrexate; CTX cyclophosphamide; EF ejection fraction; G-CSF granulocyte colony-stimulating factor; h hours; IV intravenous; IVAC ifosfamide, etoposide, cytarabine; MTX methotrexate; PO by mouth; subQ subcutaneous; XRT radiation therapy …”

Section: Tablementioning

confidence: 99%

Burkitt Lymphoma and Atypical Burkitt or Burkitt-like Lymphoma: Should These be Treated as Different Diseases?

Thomas

O’Brien

Faderl

et al. 2010

Curr Hematol Malig Rep

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Burkitt lymphoma (BL) is a mature B-cell non-Hodgkin lymphoma with an aggressive clinical course. Since the advent of short, intensive, multiagent chemoimmunotherapy regimens, it has carried a favorable prognosis. BL has been rather well characterized, whereas the other lymphomas morphologically resembling it are more heterogeneous. The cases classified as atypical BL/Burkitt-like lymphoma by the 2001 World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissue were thought to represent a continuum between BL and diffuse large B-cell lymphoma (DLBCL). The optimal therapeutic strategy for this provisional entity was not definitively established. However, recent incorporation of molecular genetic data into the 2008 WHO Classification has allowed further refinements with significant therapeutic implications, including the designation of a new provisional entity, “B-cell lymphoma, unclassifiable, with features intermediate between BL and DLBCL.” This review presents a comprehensive overview of the previously designated provisional entity of atypical BL/BLL in conjunction with a detailed comparison with BL and DLBCL.

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Cardiac Primitive Neuroectodermal Tumor Presenting As Acute Coronary Syndrome

Cited by 7 publications

References 6 publications

Primitive neuroectodermal tumor of the pericardium: a case report and literature review

Primitive neuroectodermal tumor of the pericardium: a case report and literature review

Cardiac Primitive Neuroectodermal Tumor (PNET) with Metastasis at Presentation

Burkitt Lymphoma and Atypical Burkitt or Burkitt-like Lymphoma: Should These be Treated as Different Diseases?

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