J Breast Cancer.  2009 Sep;12(3):223-226. 10.4048/jbc.2009.12.3.223.

Malignant Peripheral Nerve Sheath Tumor of the Breast in a Patient without Neurofibromatosis: A Case Report

• Yi JM
• Moon EJ
• Oh SJ
• Lee A
• Suh YJ
• Baek JM
• Choi SH
• Jung SS

• KMID: 2242016
• DOI: http://doi.org/10.4048/jbc.2009.12.3.223

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are malignant variants of peripheral nerve sheath tumors that develop at major or minor peripheral nerve branches or at the sheaths of peripheral nerve fibers. These tumors are derived from Schwann cells or pluripotent cells of a neural crest origin. Malignant tumors of the peripheral nerve sheath are most commonly seen in deeper soft tissues, and usually in the proximity of a nerve trunk. MPNSTs of the breast are very uncommon and they have rarely been reported on. We report here on a case of MPNST of the breast in a 59-year-old female who presented with a painless breast lump for two months. The excisional biopsy revealed a malignant peripheral nerve sheath tumor based on the microscopic findings and immunohistochemical staining. We performed wide excision of breast tissue around the biopsy site and thereafter the patient underwent radiation therapy. The patient remains well without signs of recurrence 1 year following surgery.

Keyword

Breast; Nerve sheath neoplasms

MeSH Terms

Biopsy
Breast
Female
Humans
Middle Aged
Nerve Sheath Neoplasms
Neural Crest
Peripheral Nerves
Recurrence
Schwann Cells

Figure

• 

  Figure 1 The mediolateral oblique view of mammography demonstrates an ovoid mass shadow just onto the pectoral muscle. The mass is well defined from the surrounding tissue and there is no calcification in the mass.

• 

  Figure 2 The ultrasonographic image of the right breast showed a well-demarcated mass. The mass is lobulated and ovoid, and it shows a hypoechoic mass density with posterior enhancement.

• 

  Figure 3 Microscopic finding of H&E staining. Note densely cellular areas alternating with less cellular areas and vague whorled structures (H&E stain, ×40).

• 

  Figure 4 Microscopic finding of H&E and immunohistochemical staining. (A) This area shows more dense fascicles of spindle cells (H&E stain, ×200). (B) S-100 protein shows focal weak staining to diffuse immunoreactive areas. These figures from areas of diffuse immunoreactive for S-100 protein (Immunohistochemical staining, ×200).

Reference

1. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumours: a clinicopathologic study of 120 cases. Cancer. 1986. 57:2006–2021.

2. Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumour: the clinical spectrum and outcome of treatment. Neurology. 2003. 61:696–698.

3. Anghileri M, Miceli R, Fiore M, Mariani L, Ferrari A, Mussi C, et al. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006. 107:1065–1074.

4. Bhattacharyya AK, Perrin R, Guha A. Peripheral nerve tumors: management strategies and molecular insights. J Neurooncol. 2004. 69:335–349.

5. Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res. 2002. 62:1573–1577.

6. Catania S, Pacifico E, Zurrida S, Cusumano F. Malignant schwannoma of the breast. Eur J Surg Oncol. 1992. 18:80–81.

7. Malas S, Krawitz HE, Sur RK, Uijs RR, Nayler SJ, Levin CV. Von Recklinghausen's disease associated with a primary malignant schwannoma of the breast. J Surg Oncol. 1995. 59:273–275.

8. Medina-Franco H, Gamboa-Dominguez A, de La Medina AR. Malignant peripheral nerve sheath tumor of the breast. Breast J. 2003. 9:332.

9. Dhingra KK, Mandal S, Roy S, Khurana N. Malignant peripheral nerve sheath tumor of the breast: case report. World J Surg Oncol. 2007. 5:142.

10. Hauser H, Beham A, Steindorfer P, Schmidt F, Smola MG. Malignant schwannoma of the breast. Langenbecks Arch Chir. 1995. 380:350–353.

11. Berrada R, Chahtane A, Lakhdar A, Elhanchi Z, Ferhati D, Baidada A, et al. Malignant schwannoma of the breast. A case report. J Gynecol Obstet Biol Reprod (Paris). 1998. 27:441–444.

12. Besznyák I, Dubecz S, Péter I. Malignant schwannoma of the breast. Orv Hetil. 1998. 139:137–139.

13. Thanapaisal C, Koonmee S, Siritunyaporn S. Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report. J Med Assoc Thai. 2006. 89:377–379.

14. Angelov L, Davis A, O'Sullivan B, Bell R, Guha A. Neurogenic sarcomas: experience at the University of Toronto. Neurosurgery. 1998. 43:56–64.

15. Sordillo PP, Helson L, Hajdu SI, Magill GB, Kosloff C, Golbey RB, et al. Malignant schwannoma: clinical characteristics, survival, and response to therapy. Cancer. 1981. 47:2503–2509.