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5 Pericytic Perivascular Tumors
5 Pericytic Perivascular Tumors
CHAPTER 5
B
Fig. 5.04 Glomus tumour. A Tumour cells show con-
sistently strong immunoreactivity for smooth muscle
Fig. 5.02 Glomangioma. The lesion is composed of Fig. 5.03 Glomangioma, composed of dilated vascu- actin. B Ultrastructure showing prominent external
dilated vascular spaces, the walls of which contain lar spaces, the walls of which contain several layers lamina, pinocytotic vesicles and intracytoplasmic
several layers of glomus cells. of glomus cells. actin microfilaments.
In the first type, the malignant com- lignancy, but having at least one bundles of thin actin-like filaments with
ponent resembles a leiomyosarcoma or atypical feature other than nuclear dense bodies and occasional attach-
fibrosarcoma. In the second type, the pleomorphism should be diagnosed as ments plaques to the cytoplasmic mem-
malignant component retains an overall "glomus tumours of uncertain malignant brane and prominent external lamina
architectural similarity to benign glomus potential". {1449}.
tumour and consists of sheets of highly
malignant appearing round cells. Immunohistochemistry Genetics
Immunohistochemical demonstration of Glomus tumours of all types typically Multiple familial glomus tumours appear
smooth muscle actin and pericellular express smooth muscle actin and have to have an autosomal dominant pattern
type IV collagen is required for the abundant pericellular type IV collagen of inheritance {164,884,1363}. An asso-
diagnosis of this second type of production. H-caldesmon is also posi- ciation between subungual glomus
malignant glomus tumour, in the ab- tive. Other markers, including desmin, tumours and neurofibromatosis type I
sence of a clear-cut benign precursor. CD34, cytokeratin and S100 protein are has been reported {1109,1602,1867}.
Malignant glomus tumours are highly usually negative {697}. The gene for multiple inherited glomus
aggressive with metastases in appro- tumours has been linked to chromo-
ximately 40% of cases, resulting in the Ultrastructure some 1p21-22 {229,297}. The genetic
death of the patient {697}. Glomus Ultrastructurally glomus cells have short events underlying sporadic glomus
tumours not fulfilling criteria for ma- interdigitating cytoplasmic processes, tumours are not known.
A B
Fig. 5.05 Symplastic glomus tumour with prominent Fig. 5.06 A Malignant glomus tumour, spindle cell type. B Malignant glomus tumour, round cell type. Note the
nuclear atypia but without mitotic activity. brisk mitotic activity.
Synonyms
In the past, myopericytoma may have
been diagnosed as a solitary myofibro-
ma or "haemangiopericytoma."
Epidemiology
Myopericytoma arises most commonly in
mid adulthood; however, lesions can
arise at any age. Familial cases have not
been reported.
Sites of involvement
Myopericytoma generally arises in sub-
cutaneous tissue. There is a predilection A
for lesions to involve the distal extremi-
ties; however, tumours can also arise at
other sites, including the proximal
extremities and neck. It is likely that a
wider site distribution will be described
with increased recognition of this tumour.
Clinical features
Myopericytoma generally presents as a
painless, slow-growing subcutaneous
nodule that can be present for years.
Some lesions are painful. Myopericytoma
most commonly arises as a solitary
lesion but multiple lesions are not infre-
quent. Multiple lesions generally arise
metachronously and usually involve a
particular anatomic region such as a
foot.
Macroscopy B
Myopericytoma tends to be a well cir- Fig. 5.07 Myopericytoma. A Typical proliferation of tumour cells around blood vessels at the periphery of this
cumscribed nodule measuring less than poorly circumscribed example. B Prominent gaping thin-walled blood vessels (left) and formation of whorls of
2 cm in diameter. spindle cells.
Immunophenotype
The spindle cells in myopericytomas are
positive for smooth muscle actin (SMA). B
SMA staining is generally diffusely posi- Fig. 5.08 Myopericytoma. A Concentric perivascular growth pattern and foci of myxoid stroma. B A multilayered
tive, but can be only focally positive, gen- concentric proliferation of spindle cells with myoid features around blood vessels.
erally in a perivascular distribution.
Prognostic factors
Most myopericytomas do not recur fol-
lowing excision. Recurrence may be
related to poor circumscription of a
lesion. Sometimes it is difficult to know
whether a myopericytoma has recurred
Fig. 5.09 Myopericytoma. Marked immunoreactivity or whether a new lesion has developed in Fig. 5.10 Myopericytoma. A whorl of spindle cells in
for smooth muscle actin accentuates the perivascu- the same anatomic area. Very rare malig- myxoid stroma bulges into the lumen of a blood ves-
lar growth pattern. nant myopericytomas exist {1383}. sel reminiscent of myofibromatosis / myofibroma.
Myopericytoma 139