Title: Myopericytoma -A Perivascular Myoid Cell Tumor with Review of Literature

Authors: Kalpana Mangal, Bhawna Bansal, Alka Mittal

 DOI:  https://dx.doi.org/10.18535/jmscr/v5i7.213

Abstract

Myopericytomas are rare perivascular neoplasms, often mistaken as solitary myofibroma or other PMC (perivascularmyoid cell)/PEC (perivascular epitheloid cell) tumors, due to their histomorphological overlapping. Their most common site is superficial soft tissues of extremities particularly distal portions of lower limb followed by head and neck in adults. Men are more commonly affected than women. The recent concept of phenotypic recognition as PMC and PEC phenotype has changed the concept of angiogenic tumors. Here we are reporting a case of sub cutaneous myopericytoma of sternal region in a 21yr male with complete review of literature. The patient had noticed a well defined nodularity of 1.5 x 1.2cm in midsternal region. Total excision was performed. On gross examination it was unencapsulated, well circumscribed reddish brown nodule with firm consistency. Cut surface grey brown homogeneous. Histologically it comprised of numerous thin walled vessels, proliferating spindled to oval cells having eosinophilic cytoplasm, blend to mildly pleomorphic nuclei, arranged in concentric perivascular pattern like hemangiopericytoma and focal glomoid nodular proliferation. Immunohistochemically tumor cells were positive for SMA (Smooth Muscle Actin), calponin and Bcl2, negative for cytokeratins, ,CD-31,CD-34,S-100 ,melanosome, CD-99 & KIT.  MIB score was 25-30 %, p53 was mildly positive. Mostly Myopericytomas  usually behave in a benign fashion , Local recurrences & metastasis may occur in atypical & malignant neoplasms. The patient is doing well without recurrence in thirty months after surgery.

Keywords: Myopericytoma (MPC), Myofibroma (MF), Hemangiopericytoma (HPC), PEC, PMC, IHC

References

  1. Eduardocalonje, Christopher D M Fletcher .Pericytictumors . In : Fletcher Diagnostic histopathology of tumors 4th Edition volume 1,2013;(42,43)
  2. Armah , H.B. and Parwani , A.V. Perivascularepitheloid cell tumor. Arch Pathol Lab Med. 2009; 133.648-654
  3. Mentzel T, Dei Tos AP, SapiZ ,Kutzner H 2006 Myopericytoma of skin and soft tissues: Clinicopathologic and immuno-histochemical study of 54 cases. Am J Surg Pathol 30:104-113
  4. Eduardo calonje , Christopher D. M. Fletcher. Vascular tumors. In : Fletcher Diagnostic histopathology of tumors 4th Edition volume 1 , 2013 ; (78,79)
  5. Fletcher CD, Unni KK, Mertens F, editors. World Health Organisation  classification of tumors. Pathology and Genetics of Tumors of Soft Tissue and Bone. Lyon , France : IARC press ; 2002. P138-9.
  6. Terada T. Myopericytoma of low grade malignancy in the oral cavity. Rare tumors 2012;4:e9
  7. MC Menamin ME, Fletcher CD. Malignant myopericytoma : Expanding the spectrum of tumors with myopericytic differentiation. Histopatholgy 2002; 41:450-60.
  8. Lau PP, Wong OK, Lui PC, Cheung OY, Ho LC, Wong WC, etal. Myopericytoma in patients with AIDS : A new class of Epstein - Barr virus - associated tumor. Am J Surg Pathology 2009;33:1666-72.
  9. Dictor M, Elner A, Andersson T, etal :Myofibromatosis like hemangiope-ricytoma metastasizing as differentiated vascular smooth muscle and myosarcoma: Myopericytes as a subset of ‘myofib-roblasts’. Am J SurgPathol1992; 16: 1239.
  10. Vasenwala SM , Afroz N , Ansari HA, Khan AH , Basari R, Rehman S. Myopericytoma of lip : A rare lesion in an unusual location . Indian J pathol Microbiol 2015 ; 58 : 211-3.
  11. Martignoni,G., Pea , M., Regheilin, D., Zamboni , G., and Bonetti , F. PEComa : The past , the present and the future. Virchows Arch.2008;452 :119-132.
  12. Dray , M. S., Mc Carthy, S.W., Palmer , A.A., Bonar, S.F., Stalley, P.D., Marjoniemi, V. et al. Myopericytoma : a unifying term for a spectrum of tumors that show overlapping features with myofibroma. A review of 14 cases. J Clin Pathol. 2006 ;59 : 67-73
  13. Scott , R.S. , Blank , K.L., Proffer L.H., Kraus,E.W., and Heim – Hall , J. Perivascularmyoma of myopericytoma and myofibromatosis – type arising in a chronic scar. J Cutan Pathol .2006 : 33 :231-235.
  14. Laga, A. C. ,Tajirian , A. L.,Islam , M.N., Bhattachaya, I., cohen, D. M., Plamondon, C.J. etal. Myopericytoma : report of 2 cases associated with trauma . J Cutanpathol. 2008;35 : 866-870.
  15. Mc Menamin ME, Calonje E: Intravascular myopericytoma . J Cutan Pathol 2002; 29:557-561.
  16. Mimami ,Y.,Shiomi,T., and Manaba , T. Perivascularmyoma : case report and immunohistochemical and ultrastructural studies . Pathol Int. 2002;52 : 69 -74.
  17. Terada ,T. Minute myopericytoma of the neck : a case report with literature review and differential diagnosis. Patholoncol Res. 2010;16 :613-616.
  18. Park H J, Lee DR , Park MY ,etal : A case of intravascular myopericytoma . J ClinPathol2010 ; 63 : 847-848.
  19. Ko JY, ChoiWJ, Kang HS, etal: Intrava-scular myopericytoma : an interesting case of a long standing large, painful subcuta-neous tumor. PatholInt2011 ;61 : 161-164.
  20. Woollard AC, Southgate C , Blair JW : Intravascular myopericytoma of the superficial palmar arch. J Hand SurgEur 2007 ;32:475.
  21. Tadashi Treada . Myopericytoma of soft tissue ( thigh) . Human Pathology : Case Reports (2016) 3, 37 - 40.

Corresponding Author

Dr Kalpana Mangal

204, Sidha Apartment, Devi Marg, Bani Park, Jaipur, Rajasthan, India, 302016

Tel No.:  9887056209, Email: This email address is being protected from spambots. You need JavaScript enabled to view it.