|Year : 2022 | Volume
| Issue : 2 | Page : 70-72
Multifocal myopericytomas: Rare soft tissue tumor over the sole
Bhavya Swarnkar1, Kanika Sahni1, Hena Khandakar2, Shipra Agarwal2
1 Department of Dermatology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||12-May-2022|
|Date of Decision||18-Jun-2022|
|Date of Acceptance||11-Jul-2022|
|Date of Web Publication||27-Oct-2022|
Department of Dermatology, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
Myopericytoma is a rare soft tissue tumour which orginates from pericytes. Histopathology shows nodular perivascular proliferation of cells with myoid differentiation. Myopericytomatosis and multifocal myopericytomas are the two uncommon variants of this entity. We report an uncommon case of multifocal myopericytomas over the sole.
Keywords: Multifocal, myopericytomas, soft tissue tumor, sole
|How to cite this article:|
Swarnkar B, Sahni K, Khandakar H, Agarwal S. Multifocal myopericytomas: Rare soft tissue tumor over the sole. Indian J Dermatopathol Diagn Dermatol 2022;9:70-2
|How to cite this URL:|
Swarnkar B, Sahni K, Khandakar H, Agarwal S. Multifocal myopericytomas: Rare soft tissue tumor over the sole. Indian J Dermatopathol Diagn Dermatol [serial online] 2022 [cited 2022 Nov 26];9:70-2. Available from: https://www.ijdpdd.com/text.asp?2022/9/2/70/359768
| Introduction|| |
Myopericytoma is a rare soft tissue tumour, originating from pericytes; pathologically characterized by nodular perivascular proliferation of fusiform cells with myoid differentiation, with a concentric perivascular growth pattern. Often the blood vessels take on a hemangiopericytoma-like “antler-horn” appearance.
The diagnosis is based on characteristic histomorphological, immunohistochemical, and ultrastructural findings.
| Case Report|| |
A female in her 30s presented with slowly progressive, mildly tender, skin-colored to reddish brown firm noduloplaques over the left sole for the past 9 years. Few of these nodules showed central callus-like thickening of overlying skin [Figure 1].
|Figure 1: Skin colored to reddish brown firm noduloplaques over left sole. Few of these nodules showed central callus-like thickening of overlying skin|
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There was no pus or blood discharge and no evident underlying bony defect.
A skin biopsy performed from one of the nodules showed a circumscribed unencapsulated tumor located in the dermis and subcutis, composed of plump spindle cells arranged around many small blood vessels, embedded in a collagenous stroma [Figure 2], [Figure 3]. Focal hyalinization was present. Occasional perilesional vessels also showed a concentric arrangement of similar spindle cells. There was no nuclear atypia or increased mitotic activity. The spindle cells were diffusely immunopositive for smooth muscle actin (SMA), [Figure 4] and focally positive for desmin and CD34. CD34 also highlighted the vascular channels. S-100, HMB45, and cytokeratin staining were negative.
|Figure 2: A circumscribed unencapsulated tumor located in the dermis and subcutis, composed of plump spindle cells arranged around many small blood vessels, embedded in a collagenous stroma. (HandE: 40x)|
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|Figure 4: The spindle cells were diffusely immunopositive for smooth muscle actin (SMA). (200x)|
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Ultrasound of the left foot showed multiple small sub-centimetric subcutaneous hypoechoic lesions over the plantar aspect. The lesions were hypovascular, with well-defined smooth margins and multiple small foci of calcification. There was no evidence of invasion into deeper tissues or muscles.
Based on the clinicopathological correlation, we made a diagnosis of multifocal myopericytomas.
Intralesional radiofrequency ablation was done for painful nodules, as she refused any major surgical procedure. A single session led to complete relief of symptoms within 3–4 weeks.
| Discussion|| |
Myopericytoma is a rare tumor of deep soft tissues, originating from pericytes and characterized by numerous thin wall blood vessels.
It is described as a circumscribed but unencapsulated perivascular proliferation of mesenchymal cells with pericytic differentiation, characterized by the radial and vertical growth of the cells forming a nodular growth. It is recognized, based on ultrastructural as well as immunohistochemical studies, as a tumor derived from perivascular myoid cells showing features of both glomus cells and smooth muscle cells. It consistently expresses myogenic markers like h-caldesmon, smooth muscle actin, and smooth muscle myosin heavy chain, while desmin has been reported to be rarely and only focally expressed, indicating a more immature smooth muscle origin.
The most common site of involvement is distal extremity (hand, ankle, knee, foot) as in our patient. It usually presents as slowly progressive, single or multiple asymptomatic skin-colored, violaceous, or whitish soft tissue nodules in adults. Sometimes, they can be painful or tender and larger lesions may ulcerate. Chronic scars and trauma have been reported as the triggering factors for the development of myopericytomas.,
The perivascular myomas represent a histogenetic continuum including three categories based on the predominant histological pattern: myofibromatosis, glomangiopericytoma, and myopericytoma.
Myofibromatosis is characterized by a distinct biphasic pattern consisting of fascicles of spindle cells resembling smooth muscle cells mixed with immature appearing zones with a hemangiopericytic pattern. Glomangiopericytomas demonstrate prominent large dilated vessels and lack of biphasic pattern and myoid nodules while a concentric proliferation of perivascular myoid cells is seen in myopericytomas. However, it should be understood that since all these tumors are perivascular myoid tumors they can show some overlap.
Histopathological differentials and differences are mentioned in [Table 1].
Multiple myopericytomas may develop in a particular anatomical location when they are labeled as multifocal myopericytomas.,, This subtype was also seen in our patient. Myopericytomatosis is a distinct subtype, where though localized in a particular anatomic region there is a diffusely infiltrative pattern with multiple discontiguous, mainly microscopic myopericytomatous nodules in dermis/subcutaneous tissue. Our patient did not show an infiltrative pattern but showed circumscribed unencapsulated single growth, hence we labeled it as multiple myopericytomas.
Myopericytomas usually follow a benign course with occasional recurrences post excision, and although rare, the malignant nature of deeper lesions has also been reported in the past.
This case is of interest due to the scarcity of such reports in the dermatologic literature. Dermatologists and dermatopathologists should be aware of this entity for proper evaluation and management of such patients.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]