Case 27 Male 42. Painless, static, well-circumscribed, subcutaneous nodule right lower leg,?lipoma. The best diagnosis is: A. Angiosarcoma B. Haemangiopericytoma C.Myopericytoma D.Myofibroma E. Angioleiomyoma
SMA H-Caldesmon Calponin CD31 Desmin
Case 27: Myopericytoma Perivascular neoplasm Recently delineated entity Shows a haemangiopericytoma (HPC) like vascular pattern Formed of myopericytes- short oval spindle cells Precursor-? Myofibroblast, pericyte or both. Dray MS, McCarthy SW, Palmer AA, Bonar SF, Stalley PD, Marjoniemi V, Millar E, Scolyer RA. Myopericytoma: A unifying term for a spectrum of tumours that show overlapping features with myofibroma. A review of 14 cases. J Clin Pathol 2006;59;67-73
Clinical Features Middle aged adults mainly 5 th decade M > F Extremities, most commonly lower followed by upper limbs and head and neck Dermal or subcutaneous firm nodule usually < 2cm. Single/ multiple Rarely occur in deep soft tissue. An association with HIV/ AIDS.
Histological Features Mostly well circumscribed Rarely entirely intravascular Oval to spindle shaped cells Concentric perivascular growth pattern Cells typically spinning off from vessel wall
Differential Diagnosis Angiosarcoma (or haemangioendothelioma) Angiosarcoma of skin 4 types Post radiotherapy Lymphoedema associated Sun damaged skin of head and neck of elderly Sporadic - rare
Differential Diagnosis Angiosarcoma (or haemangioendothelioma) infiltrative growth pattern atypia of endothelial cells mulltilayering mitoses
Differential Diagnosis Haemangiopericytoma: in skin now a mixture of entities
Overlapping histological features shown by myofibroma, glomus tumours, angioleiomyoma, myopericytoma myofibroma Myofibroma Glomus tumour Myofibroma Myofibroma angioleiomyoma Myofibroma myopericytoma
Differential Diagnosis Angioleiomyoma Fascicles of mature smooth muscle cell bundles and thick walled vessels with fibrosing change in the vessel wall Cigar - shaped nuclei abundant brightly eosinophilic cytoplasm IHC- SMA and Desmin positive
Myofibroma Well defined, multinodular tumour showing a biphasic or zonal appearance Broad sweeping fascicles or nodules of spindle cells with eosinophilic cytoplasm and cigar shaped nuclei 2 nd pattern- more cellular areas of primitive round to spindle cells around HPC like vascular spaces. Usually lack the characteristic concentric cell arrangement Positive for SMA but usually lack h- caldesmon Mentzel T, Dei Tos AP, Sapi Z, Kutzner H. Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol. 2006;30:104 113.
Glomus tumour Round to oval cells with distinct cell borders Abundant cytoplasm Arranged in an eccentric fashion around small vessels Lack an HPC- like vascular pattern Glomangioma- vascular component prominent and tend to be cavernous
Angioleiomyoma Myofibroma Glomus Tumour Glomangioma
Does Immunohistochemistry help? Tumour SMA h-caldesmon Desmin Calponin Myopericytoma ++ ++ - + Myofibroma ++ - - - Angioleiomyoma ++ ++ ++ ++ Glomus tumour ++ ++ - +/-
Management and Prognosis: myopericytoma Adequate surgical excision is curative 10-20% recurrence rate probably representing multifocality Majority follow a benign course Malignancy is very rare- high mitotic rate, increased cellularity, pleomorphism and necrosis Calonje E. Vascular tumours: tumour and tumour like condition of blood vessels and lymphatics.in: Elder DE, Elenitsas R, Johnson BL Jr, Murphy GF, Xu X,editors. Lever s Histopathology of the Skin.10 th ed. Philadelphia.Lippincott-Raven 2008:1049-1051.
Case 27 Summary Myopericytoma describe a spectrum of tumours showing a haemangiopericytoma-like vascular pattern composed of perivascular myoid cells Show a spectrum of growth pattern that overlap with myofibroma, angioleiomyoma and glomangioma Majority follows a benign course
Case 27 Male 42. Painless, static, well-circumscribed, subcutaneous nodule right lower leg,?lipoma. The best diagnosis is: A. Angiosarcoma B. Haemangiopericytoma C.Myopericytoma D.Myofibroma E. Angioleiomyoma
References Mentzel T, Dei Tos AP, Sapi Z, Kutzner H. Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol. 2006;30:104 113. Dray MS, McCarthy SW, Palmer AA, Bonar SF, Stalley PD, Marjoniemi V, Millar E, Scolyer RA. Myopericytoma: A unifying term for a spectrum of tumours that show overlapping features with myofibroma. A review of 14 cases. J Clin Pathol 2006;59;67-73 Calonje E. Vascular tumours: tumour and tumour like condition of blood vessels and lymphatics.in: Elder DE, Elenitsas R, Johnson BL Jr, Murphy GF, Xu X,editors. Lever s Histopathology of the Skin.10 th ed. Philadelphia.Lippincott-Raven 2008:1049-1051. Granter SR, Badizadegan K, Fletcher CDM. Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumours showing a perivascular myoid differentiation. Am J Surg Pathol.1998;22:513-525. Acknowledgement- Dr Thusitha Somarathna Honorary FY3 Gloucestershire Hospitals NHS Foundation Trust