International journal of surgery case reports · 2019
Mediastinal malignant triton tumor: A rare case series and review of literature.
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Chaudhry Ikram, Algazal Thabet, Cheema Ahsan, Al Faraj Aman, Al Malki Noor, Mutairi Hadi, Abbas Ahmed, Amr Samir
Аннотация
INTRODUCTION: In 1938, the Malignant Triton Tumor (MTT) was first explained by Mason.
CASE PRESENTATION: Case 1: A man aged 28 years presented with chest pain and difficulty in breathing since last five months, there was no history of cough fever or night sweats. Clinical examination was unremarkable. His routine hematological tests including tumor markers were within normal range, testicular ultrasound was normal. CT scan of thorax revealed a mass in the anterior mediastinum. CT guided biopsy revealed a malignant triton tumor. Case 2: A 30 years old man, nonsmoker presented with history of chest tightness and feeling pressure while kneeling down since last 3 months, otherwise fit and healthy. His routine hematological investigations including tumor markers were within normal range. A CT scan of thorax revealed a large mass in the right posterior mediastinum. CT guided biopsy showed malignant triton tumor. Case 3: A man aged 28 years presented with chest pain and difficulty in breathing since last five months, there was no history of cough fever or night sweats. Clinical examination was unremarkable. His routine hematological tests including tumor markers were within normal range, testicular ultrasound was normal. CT scan of thorax revealed a mass in the anterior mediastinum. CT guided biopsy revealed a malignant triton tumor.
DISCUSSION: Malignant peripheral nerve sheath tumors (MPNST) are uncommon sarcomatous tumors that are believed to be derived from Schwann cell or neighboring cells with perineurial differentiation. MTT is rarely reported in mediastinum, lung and heart (<10%) To the best of our knowledge, only few cases of MTT in the mediastinum have been reported in English literature, including, four were reported in the anterior mediastinum, three in the posterior mediastinum, one in the middle mediastinum and one between the ascending aorta and the main pulmonary artery. Most of the patients were young adults. We report three cases of rare mediastinal malignant triton tumors. They have been treated with palliative surgery/radical surgery +/- adjuvant therapy. The prognosis varied from a 3 month overall survival time to being alive at a 53 month follow-up period.
CONCLUSION: In conclusion we report three rare cases of mediastinal malignant triton tumor treated with radical surgical resection and post-operative radiotherapy, one patient developed lung metastasis, and two had late local recurrence. The malignant triton tumor is a lethal neoplasm which carries very poor prognosis particularly when they occur in the mediastinum because it's very difficult to obtain wider tumor free margin due to the nature of location site.
CASE PRESENTATION: Case 1: A man aged 28 years presented with chest pain and difficulty in breathing since last five months, there was no history of cough fever or night sweats. Clinical examination was unremarkable. His routine hematological tests including tumor markers were within normal range, testicular ultrasound was normal. CT scan of thorax revealed a mass in the anterior mediastinum. CT guided biopsy revealed a malignant triton tumor. Case 2: A 30 years old man, nonsmoker presented with history of chest tightness and feeling pressure while kneeling down since last 3 months, otherwise fit and healthy. His routine hematological investigations including tumor markers were within normal range. A CT scan of thorax revealed a large mass in the right posterior mediastinum. CT guided biopsy showed malignant triton tumor. Case 3: A man aged 28 years presented with chest pain and difficulty in breathing since last five months, there was no history of cough fever or night sweats. Clinical examination was unremarkable. His routine hematological tests including tumor markers were within normal range, testicular ultrasound was normal. CT scan of thorax revealed a mass in the anterior mediastinum. CT guided biopsy revealed a malignant triton tumor.
DISCUSSION: Malignant peripheral nerve sheath tumors (MPNST) are uncommon sarcomatous tumors that are believed to be derived from Schwann cell or neighboring cells with perineurial differentiation. MTT is rarely reported in mediastinum, lung and heart (<10%) To the best of our knowledge, only few cases of MTT in the mediastinum have been reported in English literature, including, four were reported in the anterior mediastinum, three in the posterior mediastinum, one in the middle mediastinum and one between the ascending aorta and the main pulmonary artery. Most of the patients were young adults. We report three cases of rare mediastinal malignant triton tumors. They have been treated with palliative surgery/radical surgery +/- adjuvant therapy. The prognosis varied from a 3 month overall survival time to being alive at a 53 month follow-up period.
CONCLUSION: In conclusion we report three rare cases of mediastinal malignant triton tumor treated with radical surgical resection and post-operative radiotherapy, one patient developed lung metastasis, and two had late local recurrence. The malignant triton tumor is a lethal neoplasm which carries very poor prognosis particularly when they occur in the mediastinum because it's very difficult to obtain wider tumor free margin due to the nature of location site.