“…According to Ba et al these tumors were hypodense, and osteolytic focal points were a sign that the tumor originated from the bone (15). Analogously to our radiological finding Xiao et al as the main CT characteristics of these tumors described an irregular form (83.3%), heterogeneity (66.7%) and hypodensity (94.4%) (16).…”
Peripheral primitive neuroectodermal tumors (pPNET) are a group of extremely rare, aggressive, malignant tumors that are most often found in the thorax (Askin tumor), abdomen, pelvis, extremities and less frequently in the head and neck. The most important prognostic factor is the stage of the tumor. Significant progress both in surgery and in neoadjuvant and adjuvant chemotherapy and radiotherapy, as well as the improvement in diagnosis by cytogenetic and immunohistochemical analysis, should improve the survival rate. We report a case of a 14-year-old girl, with ataxic gait, cardiopulmonary compensated, without respiratory symptoms, who was referred to our hospital for further examination and treatment of newly discovered tumor of the left hemithorax. After a detailed radiological and laboratory investigation, next step was an extensive thoraco-neurosurgical surgery. After histopathological, cytological and molecular analysis, a diagnosis of Askin tumor was made.
“…According to Ba et al these tumors were hypodense, and osteolytic focal points were a sign that the tumor originated from the bone (15). Analogously to our radiological finding Xiao et al as the main CT characteristics of these tumors described an irregular form (83.3%), heterogeneity (66.7%) and hypodensity (94.4%) (16).…”
Peripheral primitive neuroectodermal tumors (pPNET) are a group of extremely rare, aggressive, malignant tumors that are most often found in the thorax (Askin tumor), abdomen, pelvis, extremities and less frequently in the head and neck. The most important prognostic factor is the stage of the tumor. Significant progress both in surgery and in neoadjuvant and adjuvant chemotherapy and radiotherapy, as well as the improvement in diagnosis by cytogenetic and immunohistochemical analysis, should improve the survival rate. We report a case of a 14-year-old girl, with ataxic gait, cardiopulmonary compensated, without respiratory symptoms, who was referred to our hospital for further examination and treatment of newly discovered tumor of the left hemithorax. After a detailed radiological and laboratory investigation, next step was an extensive thoraco-neurosurgical surgery. After histopathological, cytological and molecular analysis, a diagnosis of Askin tumor was made.
“…Radiology may offer important information for the diagnosis and prognosis of pPNETs. As in the present case, pPNETs commonly present as large, ill-defined, aggressive, heterogeneous, hypodense masses, exhibiting heterogeneous enhancement with varying internal lower-density regions (11,12). Due to the high incidence of metastasis at presentation, a full metastatic workup is warranted in cases with suspected pPNET.…”
Abstract. Peripheral primitive neuroectodermal tumors (pPNETs) are a group of aggressive neoplasms that are most commonly encountered in pediatric patients and may be located in the abdomen, pelvis, thoracopulmonary region and, rarely, in the head and neck region. pPNETs in adults are extremely rare. The present study reports a case of pPNET located in the hip muscles with bone metastasis. The patient was a 44-year-old woman who complained of progressive pain and swelling with a mass near the left hip. Computed tomography (CT) and enhanced CT revealed a soft tissue mass lesion in the hip muscle group measuring 4.3x4.3x4.4 cm. The lesion was ill-defined, heterogeneous, exhibiting mild post-contrast enhancement. There was a large number of bent neovessels and several branches from the left internal iliac artery and deep femoral artery on enhanced CT scan. Triple-phase dynamic imaging with integrin α v β 3 -targeted 99m Tc-3P-RGD 2 as the radiotracer revealed increased blood perfusion and radiotracer aggregation in the large, ill-defined, heterogeneous, hypodense mass and adjacent bone. The patient was suspected of having pPNET with bone metastasis, which was confirmed by histological examination of a sample obtained by needle aspiration. Due to the high blood perfusion of primary pPNETs and high RGD uptake by the primary and metastatic lesions, chemoembolization and anti-angiogenic therapy were considered to be the optimal therapeutic choice. This also suggested that 177 Lu-labeled RGD has great potential for the targeted treatment of pPNETs with multiple metastases.
“…The imaging findings of PNET arising from cardium or other locations have been described in the previous reports[ 11 , 12 ]. On plain CT images, the majority of PNET lesions showed as ill-defined, irregularly shaped, hypodense masses accompanied with persistent moderate heterogeneous enhancement.…”
Section: Discussionmentioning
confidence: 87%
“…On plain CT images, the majority of PNET lesions showed as ill-defined, irregularly shaped, hypodense masses accompanied with persistent moderate heterogeneous enhancement. The cystic or necrotic changes were frequently observed in larger tumors, which may be due to the rapid growth and lack of blood supply[ 11 ]. On MRI images, the tumors generally showed iso- to hypointense T1-weighted signal and intermediate to hyperintense T2-weighted signal.…”
BACKGROUND
Primitive neuroectodermal tumors (PNETs) are rare, sporadic malignant tumors of the peripheral nervous system, bone, or soft tissues. However, to the best of our knowledge, only three cases of PNET in the pericardium have been reported in the English literature, and their magnetic resonance imaging findings have not previously been described.
CASE SUMMARY
A 3-year-old boy was hospitalized with a 1-wk history of recurrent vomiting and weakness. Detailed history-taking revealed no evidence of heart disease. Computed tomography demonstrated a soft tissue mass in the left pericardial cavity with heterogeneous contrast enhancement. The border between the mass and the heart was poorly defined. Thoracotomy revealed a mass invading the left ventricle, with a high risk of bleeding. The mass was considered inoperable. A biopsy was performed, and the histological and immunohistochemical findings confirmed the diagnosis of primary PNET of the pericardium. The patient received four cycles of standard chemotherapy. Chest magnetic resonance imaging 3 mo after the initiation of chemotherapy revealed that the tumor in the pericardium still existed, but its volume had slightly decreased. The patient was lost to follow-up, and the final outcome was therefore unknown.
CONCLUSION
Medical imaging plays an important role in defining the pericardial origin of PNET and understanding its characteristics. Magnetic resonance imaging can provide more information on the tumor than computed tomography and may thus aid therapeutic planning.
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