Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum

Shimoyama, Takehiko; Yoshiya, Katsuo; Yamato, Yasushi; Koike, Teruaki; Honma, Keiichi

doi:10.1007/s11748-008-0358-0

Cited by 10 publications

(12 citation statements)

References 9 publications

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“…To the best of our knowledge, only 5 patients with MPNST of the anterior mediastinum have been reported to date, including our patient (Table I) (5)(6)(7)(8). Of all the reported cases, our patient is the only one to have a tumor derived from the phrenic nerve.…”

Section: Discussionmentioning

confidence: 73%

Malignant peripheral nerve sheath tumor in the anterior mediastinum: A case report

Koezuka

Hata

Sato

et al. 2014

Molecular and Clinical Oncology

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Abstract. Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, accounting for only 5% of all malignant soft-tissue sarcomas. This is the case report of a 28-year-old male patient with a MPNST located in the anterior mediastinum, originating from the phrenic nerve. The patient presented with an abnormal shadow on chest radiography and a 10-cm mass in the right anterior mediastinum was identified on computed tomography and magnetic resonance imaging. The patient subsequently underwent surgical resection. The tumor originated from the right phrenic nerve and was tightly adherent to the middle and lower lobes of the right lung, the pericardium and the diaphragm. The tumor was completely resected, along with partial resection of the adherent tissues. The histological diagnosis was spindle cell sarcoma and the final diagnosis was MPNST of the anterior mediastinum, based on the characteristic microscopic appearance indicating the nervous origin of the tumor and on the intraoperative findings. The patient remains free of recurrence 1 year after surgery. IntroductionMalignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, accounting for only 5% of all malignant soft-tissue sarcomas (1,2). The most common sites of occurrence are the torso, extremities and head and neck, whereas MPNSTs are extremely rarely located in the mediastinum (3). This is the case report of a patient with an MPNST in the anterior mediastinum, originating from the right phrenic nerve. Case reportA 28-year-old man underwent screening chest radiography and was found to have an anterior mediastinal mass. There was no abnormal shadow on a chest radiograph obtained the previous year. The patient was asymptomatic, had no family history of neurofibromatosis type 1 (NF1) and his medical history included a right pneumothorax that was treated surgically, with pleurodesis, performed 3 years prior. The results of blood testing were unremarkable. A chest radiograph revealed a well-defined tumor, 10 cm in size, in the right anterior mediastinum (Fig. 1A). Contrast-enhanced computed tomography of the chest revealed a heterogeneous mass with rim enhancement; the mass compressed the diaphragm and the right atrium (Fig. 1B). Magnetic resonance imaging revealed an anterior mediastinal mass with heterogeneous high intensity on T1 and T2-weighted images. The area of high intensity persisted on fat saturation imaging and was therefore suspected to represent hemorrhage.Considering the rapid growth of the lesion, its location and the possibility of malignancy, surgical resection of the tumor was recommended. Surgical access was obtained via a right posterolateral thoracotomy over the sixth rib. The tumor was located in the interlobular space, appearing to originate from the right phrenic nerve and was densely adherent to the middle and lower lobes of the right lung, the pericardium and the diaphragm. The tumor was completely resected and partial resection of the pericardium, right phrenic nerve, diaphragm and the middle and lower lobes of the r...

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Section: Discussionmentioning

confidence: 73%

Malignant peripheral nerve sheath tumor in the anterior mediastinum: A case report

Koezuka

Hata

Sato

et al. 2014

Molecular and Clinical Oncology

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“…Unfortunately, although our patients’ relapsed lesions temporarily regressed after HSCT treatment, all three patients showed disease progression within 1 year of treatment and died of their disease. Previous reports have also demonstrated a poor prognosis with intrathoracic MNT [ 3 , 4 , 13 , 14 ]. Although complete resection is a necessary and potentially curative therapeutic modality for intrathoracic MNT, the prognosis following resection was unsatisfactory in our cases.…”

Section: Discussionmentioning

confidence: 94%

Primary intrathoracic malignant neurogenic tumor: report of three cases and comparison with benign neurogenic tumors resected at our institution

et al. 2015

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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.

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“…Prognostic factors are tumor size, grade of malignancy, speed of clinical evolution, degree of cellular pleomorphism and mitotic activity. Prognosis is usually poor, especially in large invasive tumors, those related to NF1, and those with high-grade histologic features [8,[12][13][14][15][16].…”

Section: Discussionmentioning

confidence: 99%

“…Chemotherapy can be used as adjuvant treatment, but its efficacy has not been proven. It can be useful for metastatic disease or in neoadjuvant setting for large and unresectable tumors [ 8 ]. In this report, an unusual case of MPNST arising from phrenic nerve is described.…”

Section: Introductionmentioning

confidence: 99%

Successful Management of Intrathoracic Phrenic Malignant Peripheral Nerve Sheath Tumor by Multimodal Treatment

Rais¹,

Maidi²,

Rais³

2020

J Med Cases

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Malignant peripheral nerve sheath tumors (MPNSTs) are rare and can be associated with type 1 neurofibromatosis. They are rarely located at the mediastinum, most often at the posterior mediastinal compartment or the paravertebral gutters. MPNSTs of the anterior mediastinum arising from the phrenic nerve are exceptional. Their prognosis is poor due to the high potential of local tumor recurrence and metastasis. Complete surgical resection remains the only radical treatment. However, the outcomes after this treatment alone cannot be considered satisfactory. Preoperative chemotherapy could be an interesting therapeutic option for unresectable tumors. We report here the case of a 77-year-old woman with an MPNST located at the anterior mediastinum arising from the phrenic nerve. This tumor was successfully managed with multimodal therapy combining preoperative chemotherapy, complete surgery and adjuvant radiation.

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Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum

Cited by 10 publications

References 9 publications

Malignant peripheral nerve sheath tumor in the anterior mediastinum: A case report

Malignant peripheral nerve sheath tumor in the anterior mediastinum: A case report

Primary intrathoracic malignant neurogenic tumor: report of three cases and comparison with benign neurogenic tumors resected at our institution

Successful Management of Intrathoracic Phrenic Malignant Peripheral Nerve Sheath Tumor by Multimodal Treatment

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