Abstract
We present three patients with intrathoracic malignant neurogenic tumor. Two lesions showed no sign of invasion into adjacent structures, while the third lesion extended to the intraspinal canal with vertebral involvement. Although all three lesions were completely excised, each patient relapsed within 1 year of the initial treatment. One patient with local recurrence underwent radiation therapy, but the recurrent tumor continued to progress. Chemotherapy was subsequently performed. Two patients with distant metastases also received chemotherapy. Because there is no effective chemotherapeutic regimen for intrathoracic malignant neurogenic tumor, all three patients received high-dose chemotherapy followed by hematopoietic stem cell transplantation. Although the relapsed lesions temporarily regressed after treatment, all three patients showed disease recrudescence and ultimately died of their disease. A comparison of the intrathoracic malignant neurogenic tumors and the benign neurogenic tumors resected at our institution revealed no meaningful differences distinguishing malignant from benign neurogenic tumors prior to surgery.
Highlights
Neurogenic tumor is a common intrathoracic neoplasm, representing approximately 20% of all adult and 35% of all pediatric mediastinal neoplasms [1]
We report three cases of intrathoracic malignant neurogenic tumor (MNT) treated with surgery
Because the sample size was small and we did not have the data of MNT, we could not compare the F18-deoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT) findings between MNT and benign neurogenic tumors (BNTs)
Summary
Neurogenic tumor is a common intrathoracic neoplasm, representing approximately 20% of all adult and 35% of all pediatric mediastinal neoplasms [1]. Case 3 A posterior mediastinal tumor was detected on chest CT in a 17-year-old male without signs of neurofibromatosis type 1 Both chest CT and MRI revealed intraspinal canal extension (Figure 3A,B). High-dose chemotherapy comprising carboplatin (400 mg/m2 on days −7 and −6), etoposide (15 mg/kg on days −7, −6, −5, and −4), and melpharan (90 mg/m2 on days −7 and −6) was performed, followed by auto-PBSCT This treatment achieved a partial response, and for a time the patient remained stable with maintenance therapy of doxorubicin alone (50 mg/m2). Tumor regrowth was revealed 24 months postoperatively At this time, high-dose chemotherapy with flutamide (30 mg/m2 on days −7, −6, −5, −4, −3, and −2), melpharan (70 mg/m2 on days −7 and −6), and ATG (2.5 mg/kg on days −5, −4, −3, and −2) was performed, followed by allogeneic peripheral blood stem cell transplantation. Because the sample size was small and we did not have the data of MNT, we could not compare the FDG-PET/CT findings between MNT and BNT
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