Cutaneous clear cell myomelanocytic tumour: a new member of the growing family of perivascular epithelioid cell tumours (PEComas). Clinicopathological and immunohistochemical analysis of seven cases

Mentzel, Thomas; Reisshauer, S; Rütten, Arno; Hantschke, Markus; Soares‐de‐Almeida, Luís; Kutzner, Heinz

doi:10.1111/j.1365-2559.2005.02105.x

Cited by 108 publications

(90 citation statements)

References 21 publications

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“…In contrast to conventional atypical lipomatous tumor, staining for MDM2 and CDK4 is most often negative. This latter result is in line with more recent genetic data, 28,29 which have proposed that these tumors show either monosomy of chromosome 7 or, as suggested by Mentzel et al, 16 rearrangement of 13q. In our experience, the latter finding seems to be most accurate and this may be further supported by loss of Rb protein expression (Figure 8).…”

Section: Pecomasupporting

confidence: 89%

“…Some cases may show prominent stromal hyalinization, particularly in the retroperitoneum, and these are known as sclerosing PEComas. 15 Another distinct subset are PEComas arising in skin, which usually have clear cell morphology 16,17 (producing potential morphological overlap with balloon cell melanoma or a renal metastasis). The minority of cases which show marked atypia or pleomorphism, readily identified mitotic figures or necrosis are best regarded as malignant ( Figure 4b) and personal experience suggests that these lesions are often quite aggressive, with frequently rapid development of metastases.…”

Section: Pecomamentioning

confidence: 99%

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Recently characterized soft tissue tumors that bring biologic insight

Fletcher

2014

Modern Pathology

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Previously unrecognized but clinicopathologically (and often molecularly) distinct types of soft tissue tumor continue to be characterized, allowing wider recognition, more consistent application of diagnostic criteria, more reliable prediction of tumor behavior and enhancement of existing classification schemes. Examples of such 'entities' that have become much better understood over the past decade or so include deep 'benign' fibrous histiocytoma, hemosiderotic fibrolipomatous tumor, PEComa, spindle cell liposarcoma, myoepithelial tumors of soft tissue and spindle cell/sclerosing rhabdomyosarcoma. These tumor types, as well as the insights which they have engendered, are briefly reviewed here.

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Section: Pecomasupporting

confidence: 89%

Section: Pecomamentioning

confidence: 99%

Recently characterized soft tissue tumors that bring biologic insight

Fletcher

2014

Modern Pathology

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“…An occasional case may even have no features of malignancy and still show clinical aggressiveness [16]. No case described in the skin has behaved aggressively to date, suggesting that site may also determine behaviour in PEComas [9,23,30]. An occasional case may have frankly sarcomatous areas and mimic other sarcomas [1].…”

Section: Discussionmentioning

confidence: 98%

Perivascular epithelioid cell neoplasms (PEComas): four malignant cases expanding the histopathological spectrum and a description of a unique finding

et al. 2007

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Four cases of malignant PEComas were stained with smooth muscle actin, muscle specific actin, desmin, human melanoma black-45, melan-A, microphthalmia transcription factor, S100 and cyclin D1. One case was studied by electron microscopy (EM). Tumour locations were the thigh, elbow, retroperitoneum and bladder in association with a urachal cyst. There were two men and two women; the average age was 51.3 years, and the size ranged from 5.0-23.0 cm. In three cases, 50-95% of the tumour was composed of pleomorphic sarcomatous areas. All cases had at least focal clear-cell areas. One case showed a continuous single layer of perivascular clear cells remote from the tumour, transitioning to invasive nests and to PEComa. EM demonstrated these cells in apposition to and in direct contact with the abluminal surface of the basal lamina of the capillaries. We suggest the term "pecosis" for these areas. All cases were positive for two or more melanocytic markers and for at least one actin. S100 and desmin were focally positive in one case. Cyclin D1 was positive in 3:4 cases. Four cases of malignant PEComa are described with the existence of a unique lesion (pecosis) in one case. These tumours may manifest largely as sarcomas appearing to be undifferentiated and should be considered in their differential diagnosis.

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“…Histologically, PECs are characterized by an epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus, and an inconspicuous nucleolus, but they may have also spindleshaped and pleomorphic features [2,3,8,9,12 ]. Immunohistochemically, PECs are positive for melanocytic and myogenic markers, such as human melanoma black (HMB)-45, Melan-A, microphthalmia transcription factor, NKIC3, smooth muscle actin (SMA), calponin, muscle myosin, and, less commonly, desmin [2,3,8,18]. However, as for epithelial markers, PECs are exclusively negative [2,8,18].…”

Section: Introductionmentioning

confidence: 96%

Perivascular epithelioid cell tumor of the rib

et al. 2008

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We present a rare case of perivascular epithelioid cell tumor (PEComa) in the right 6th rib of a 28-year-old man. A plain computed tomography scan showed a round osteolytic lesion in the right 6th rib. The resected tissue contained a globular-shaped, soft tumor. Histologically, the tumor was rich in vasculature and exclusively composed of perivascular epithelioid cells with clear cytoplasm. Immunohistochemically, the tumor expressed diffusely a melanocyte marker, human melanoma black-45, and focally a myogenic marker, alpha-smooth muscle actin, but not an epithelial marker, AE1/AE3. Fontana-Masson-positive melanin pigments were present and c-kit receptor tyrosine kinase (CD117), involved in the development of melanocytes but not myogenic cells, was expressed in tumor cells. These findings indicate that the tumor is PEComa with some differentiation into melanocytes. Notably, owing to the unique location of the occurrence, the tumor occupied bone marrow tissues of the rib, resulting that the tumor has the potential for hematogenous metastasis. In spite of the lack of cells with severe atypia, necrosis, and numerous mitoses, tumor cells invaded into surrounding tissues and overexpressed cyclin D1. To the best of our knowledge, this is the first case report of PEComa arising from the rib with the signs of malignant potential.

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Cutaneous clear cell myomelanocytic tumour: a new member of the growing family of perivascular epithelioid cell tumours (PEComas). Clinicopathological and immunohistochemical analysis of seven cases

Abstract: With the presented series of cutaneous myomelanocytic tumours the clinicopathological spectrum of PEComas is expanded.

Cited by 108 publications

References 21 publications

Recently characterized soft tissue tumors that bring biologic insight

Recently characterized soft tissue tumors that bring biologic insight

Perivascular epithelioid cell neoplasms (PEComas): four malignant cases expanding the histopathological spectrum and a description of a unique finding

Perivascular epithelioid cell tumor of the rib

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