Document Type: Case Reports
Departmentof Pathology, Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India
Malignant peripheral nerve sheath tumor (MPNST) is a rare nerve sheath tumor derived from Schwann cells or pleuripotent cells of neural crest. Neurogenic tumors make about 10-20% of all mediastinal tumors. Incidence of MPNST is 0.001% in general population and 0.16% in patients with neurofibromatosis I (NF I). We report a case of 60 year female presenting with progressive cough and breathlessness for 2 years. The CECT revealed multiple focal enhancing lesions along inferior mediastinal pleural surface and along lateral pleural surface. A thoracotomy and tumor excision was done and MPNST was diagnosed on microscopy and immunohistochemistry. This case highlights that this unusual tumor may involve lung parenchyma. So this possibility should be kept in mind in patients with intrathoracic mass.
- Gryglicka B, Szkutnik IW, Milanowski J, Mandziuk S, Szatkowska LG, Surdyka D, et al. Malignant peripheral nerve sheath tumor associated with clear cell carcinoma- case report. Pneumonol Algerol Pol 2012;80(3):269-74.
- Chao BH, Stogner- Underwood KA, Kiev J, Smith TJ. Intrathoracic malignant peripheral nerve sheath tumor in neurofibromatosis I. J Clin Oncol 2008;26(13):2216-8.
- Kolarov V, Stanic J, Eri Z, Zvezdin B, Kojicic M, Hromis S. Intrathoracic malignant peripheral nerve sheath tumor with poor outcome: a case report. Bosnian J Bas Med Sci 2010;10(4):328-30.
- Enzinger F., Wiess S. Enzinger & Weiss.: Soft tissue tumors. New York: Mosby; 2008.
- Guo A, Liu A, Wei L, Song X. Malignant peripheral nerve sheath tumors: Differentiation patterns and immunohistochemical features - A mini-review and our new findings. J Cancer 2012;3:303-9.
- Kar M, Deo SVS, Shukla NK, Malik A, DattaGupta S, Mohanti BK, et al. Malignant peripheral nerve sheath tumors (MPNST): Clinicopathological study and treatment outcome of twenty four cases. World J Surg Oncol 2006;4:55.
- Patel VS, Louis JD, Oduntan O, Landolfo KP. Intrathoracic peripheral nerve sheath tumor in patients with neurofibromatosis type I (von Recklinghausen disease).J Thorac Cardiovasc Surg 2006;131(3):736-7.
- Gosk J, Zimmer K, Rutowski R. Peripheral nerve tumours: diagnostic and therapeutical basics. Folia Neuropathol 2004;42(1):31-5.
- Ariel IM. Tumors of the peripheral nervous system. CA Cancer J Clin 1983;33(5):282-99.
- Carli M, Ferrari A, Mattke A, Zanetti l, Casanova M, Bisogno G, et al. Pediatric malignant peripheral nerve sheath tumor: The Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 2005;23(33):8422-30.
- Hagel C, Zils U, Peiper M, Kluwe L, Gotthard S, Friedrich RE, et al. Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors. J Neurooncol 2007; 82(2):187-92.