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Malignant Myopericytoma: Report of a New Case and Review of the Literature

Article  in  The American Journal of dermatopathology · February 2016

DOI: 10.1097/DAD.0000000000000463

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 EXTRAORDINARY CASE REPORT

Malignant Myopericytoma: Report of a New Case and

Review of the Literature
Agostini Patrick, MD,* Luís Soares-de-Almeida, PhD,† and Kutzner Heinz, PD, Dr med‡

5 malignant myopericytomas (MMPCTs) and showed that

Abstract: Malignant myopericytoma is a rare entity with only 8 they are associated with aggressive biological behavior. We
cases reported in the English literature. The authors report a case of performed a review of the English literature and found 3 addi-
a 65-year-old man with a slow-growing 8-cm nodule on the right tional cases described in the last 9 years.5–7 Finally, we report
arm. Marginal excision was performed, and a diagnosis of malignant a new case of MMPCT that shares the histological and
myopericytoma was made based on histopathologic and immuno- immunohistochemical findings as described by McMenamin
histochemical aspects. These tumors are characterized by a pro- and Fletcher.4
liferation of round-to-spindle cells of myoid appearance in
a concentric perivascular arrangement, along with malignant cyto-
logical findings. By immunochemistry, the cells were positive for CASE REPORT
smooth muscle actin and negative for desmin, cytokeratin AE1/AE3, We report a 65-year-old man who presented with a painless
S100 protein, Melan-A, p63, CD99, bcl-2, CD10, and STAT-6. No tumor, which had been growing slowly for a few months on the right
membranous expression of type IV collagen was observed. These arm. Physical examination revealed an 8-cm nodular tumor on
tumors are associated with aggressive biological behavior and most otherwise normal skin. His medical history was not significant. The
develops metastases. main clinical differential diagnosis was dermatofibrosarcoma protu-
berans. A surgical total excision was tried, and the specimen was
Key Words: malignant myopericytoma, perivascular neoplasm, my- sent for histopathologic examination. Macroscopic examination
oid, skin, soft tissue revealed a 7.6 · 6.0 cm nodule covered by a focally ulcerated epi-
dermis (Fig. 1). On section, the lesion occupied the entire dermis and
(Am J Dermatopathol 2016;38:307–311) the subcutis with gray-whitish appearance and pushing rather than
infiltrating borders. Microscopic examination showed a dermal–
hypodermal unencapsulated confluence of poorly defined nodules
INTRODUCTION around discrete hemangiopericytoma-like vascular spaces (Fig.
2A). Perivascular concentric growth of tumor cells was present
After Stout’s original description of hemangiopericytoma,1 (Fig. 2B). These nodules were composed of round-to-oval or short
it seems that a variety of “vascular” benign and malignant spindle-shaped cells with amphophilic or eosinophilic pale cyto-
tumors may have a hemangiopericytic pattern. Relationship plasm. The cells showed ill-defined borders, vesicular nuclei, and
between hemangiopericytoma and pericytes was frequently myoid features. Some areas of cells with central nuclei had glomus
not confirmed by ultrastructural and immunohistochemical
evaluation. In 1996, Requena et al2 proposed the term myo-
pericytoma as an alternate name for solitary myofibroma,
basing their argument on its myopericytic differentiation.
In 1998, Granter et al3 adopted the term myopericytoma
and argued that myopericytoma, myofibromatosis, solitary
myofibroma, and infantile hemangiopericytoma form a single
morphological spectrum of tumors that show differentiation
toward perivascular myoid cells/pericytes. In 2002, McMenamin
and Fletcher4 broadened this spectrum with a report of

From the *Dermatopathology Department, Centro Hospitalar do Algarve, Faro,

Portimão, Portugal; †Clínica Universitária de Dermatologia de Lisboa,
Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Portugal; and
‡Dermatophatologisches Gemeinschaftslabor, Friedrichshafen, Germany.
The authors declare no conflicts of interest.
Name of the institution(s) at which the research was conducted: Dermato-
pathology Department of Centro Hospitalar do Algarve (Faro-Portimão),
Portugal; Clínica Universitária de Dermatologia de Lisboa, Hospital de
Santa Maria, Centro Hospitalar Lisboa Norte, Portugal; and Dermatopha-
tologisches Gemeinschaftslabor, Friedrichshafen, Germany.
Reprints: Agostini Patrick, MD, Pathology Department, Centro Hospitalar do
Algarve (Faro-Portimão), Poço Seco 8500-338, Portugal (e-mail:
[email protected]). FIGURE 1. Malignant myopericytoma. Ex vivo photography
Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. of the cutaneous nodule.

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Patrick et al Am J Dermatopathol  Volume 38, Number 4, April 2016

FIGURE 2. A, Malignant myoper-

icytoma. Tumor cells around discrete
hemangiopericytoma-like vascular
spaces (H&E, ·200). B, Malignant
myopericytoma. Perivascular concen-
tric growth of tumor cell (H&E, ·400).

appearance (Fig. 3A). Nuclear pleomorphism was widespread with immunohistochemical features described by McMenamin
large size, anisocariosis, multilobulation, macronucleoli, and giant and Fletcher,4 in the dermis and subcutis of the right arm.
pseudoinclusions (Fig. 3B). A mitotic count of 22 mitoses per 10 In Table 1, we present an overview of the clinical data, gross
high power fields (HPF) was identified. Necrosis “en masse,” lym- findings, treatment, and follow-up of these 9 cases. The larg-
phovascular invasion, and neural permeation were also observed
est series of 5 cases was published by McMenamin and
(Figs. 4A and B). Tumor cells stain diffusely for smooth muscle
actin (SMA) (Fig. 5) and calponin, and they were negative for des- Fletcher4 in 2002, and 3 others authors5–7 reported isolated
min, cytokeratin AE1/AE3, S100 protein, Melan-A, p63, CD99, cases. In this review, the median age of occurrence was 54.5
bcl-2, CD10, and STAT-6. No membranous expression of type IV years and the male:female ratio was 5:3. The tumors reported
collagen was observed. Stain for CD34 was positive for the normal had a wide anatomical distribution, arising subcutaneous/der-
endothelial cells and negative for tumor cells. The spectrum of his- mal or intramuscular in the extremities (n = 4), neck (n = 1),
tomorphological and immunohistochemical findings was congruent or deep-seated location, mediastinum (n = 1) with dissemi-
with a diagnosis of MMPCT. Staging studies were negative for nated metastatic disease at presentation, periampullary (n =
metastatic disease. 1), and left atrium (n = 1). Our case, like most, occurred in
a male and was located in the subcutaneous/dermal tissue of
an extremity. The presenting symptoms in 5 cases were
DISCUSSION a growing mass,4,5 painful (cases 2 and 3),4 or painless (cases
In 1998, Granter et al3 adopted the term myopericytoma 1 and 4).4 Macroscopically, the average size of the tumor was
to describe a benign tumor with concentric perivascular pro- 5.82 cm (1.5–13 cm). The size was not specified in 1 case.5
liferation of oval-to-spindle cells that show apparent differen- Most cases appeared well-defined but not encapsulated. Two
tiation toward perivascular myoid cells/pericytes. MMPCT cases were ill-defined.4,5 The color of the lesions varied
shows the same histological and immunohistochemical char- (white, gray purple, tan, or red). Microscopically, 6 cases
acteristics but exhibit malignant features of the tumor cells as showed a perivascular concentric growth accentuation.4,7 This
high cellularity, high mitotic index, pleomorphism, necrosis, striking tendency for concentric perivascular growth is not
and sometimes lymphovascular invasion and perineural infil- a feature in malignant glomus tumors.8,9 Vascular holes with
tration. MMPCT are exceedingly rare and only 8 cases have a staghorn pattern were present in 4 cases.4,7 Focal myxoid
been reported in the English literature. We report a new case stroma was described in 3 cases.4,6 A focal morphological
of MMPCT based on the histopathologic and overlap with malignant glomus tumor was reported.4 The

FIGURE 3. A, Malignant myoper-

icytoma. Areas of cells with glomus
appearance (H&E, ·400). B, Malig-
nant myopericytoma. Round-to-oval
cells with marked nuclear pleomor-
phism (H&E, ·400).

FIGURE 4. A, Malignant myoper-

icytoma. Lymphovascular invasion
(H&E, ·400). B, Malignant my-
opericytoma. Neural permeation
(H&E, ·400).

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Am J Dermatopathol  Volume 38, Number 4, April 2016 Malignant Myopericytoma

described. Necrosis was a feature in 5 cases.4,6,7 Vascular

invasion was a feature in 2 cases.4,6 Immunohistochemical
studies revealed that 7 cases were positive for SMA,4,5,7 in
1 case h-caldesmon was positive and SMA was not dis-
cussed.6 McMenamin and Fletcher4 interestingly described
a case where tumor cells were positive for SMA, and focally
for EMA and cytokeratin (AE1/AE3), the unique case in their
study with this immunohistochemical particularity. Desmin
expression was focally positive in case 3 of the McMenamin
series with a “dot-like” pattern of staining.
In view of the morphological findings, 2 pertinent
differential diagnoses are worth considering. Malignant
glomus tumor (glomangiosarcoma) is a closely related entity
within the pericytic family of tumors and can show morpho-
logic overlap. In contrast to MMPCTs, they do not show the
characteristic perivascular concentric multilayering of cells.
On occasion, there may be a residuum of precursor glomus
FIGURE 5. Malignant myopericytoma. Tumor cells stain dif- tumor around the malignant counterpart.8–11 It is now well-
fusely for smooth muscle actin (H&E, ·400). established by some authors that malignant glomus tumors
have an immunohistochemical membranous expression of
collagen type IV.9–11 No evidence of membranous expression
tumor cells were round, oval, or spindle shaped. Nuclear of collagen type IV was found in our case.
pleomorphism was seen with prominent nucleoli, hyperchro- Malignant solitary fibrous tumor is exceedingly rare;
matism, and multilobulation in most tumors. Mitoses were all cutaneous tumors reported so far have behaved in
numerous in all cases with an average of 27/10 HPF (12– a benign fashion. Typical histologic features for malignant
48/10 HPF), and atypical mitotic figures were frequently solitary fibrous tumors are small fibroblast-like cells

TABLE 1. Clinical Features of All Reported Cases of Malignant Myopericytoma

Presenting
Sex/ Symptoms and
Cases References Age Site Relevant History Gross Description Treatment Follow-up
1 McMenamin F/81 Soft tissue of the Rapidly growing 2.0-cm subcutaneous Marginal excision Liver metastasis 9
and Fletcher4 left side of neck painless mass mass mo after
of 2 mo history. diagnosis. Alive
Previous site of with disease at
a resected 24 mo
melanoma in situ
2 McMenamin M/46 Intermuscular Painful mass of 13.0-cm Marginal excision, Metastasis to the
and Fletcher4 mass of the left unstated intermuscular postoperative external heart, brain, liver,
posterior thigh duration mass, myxoid with beam radiation and bone after 6
central firm area mo. Dead of
disease at 7 mo
3 McMenamin M/19 Dermis/Subcutis Enlarging painful 4.0-cm infiltrative Below-knee amputation Metastatic disease
and Fletcher4 of the right foot mass. History of tumor in the and death within
heel multiple poorly dermis/subcutis 1 yr (exact time
circumscribed invading muscle of death not
glomus tumors and calcaneus bone available)
of the sole of
right foot · 6
yrs. External
Beam radiation 5
yrs earlier.
4 McMenamin F/80 Superficial mass Painless superficial 1.5-cm mass Marginal excision followed No metastasis.
and Fletcher4 of the left upper mass of unstated by wide excision Discharged after 8
arm duration mo of follow-up.
Patient as not
represented since.
Believed to be
disease free at
20 mo

(continued on next page )

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Patrick et al Am J Dermatopathol  Volume 38, Number 4, April 2016

TABLE 1. (Continued ) Clinical Features of All Reported Cases of Malignant Myopericytoma

Presenting
Sex/ Symptoms and
Cases References Age Site Relevant History Gross Description Treatment Follow-up
5 McMenamin F/67 Mass in the Short history of 10.0-cm mass Excision of 1 cutaneous Metastasis disease
and Fletcher4 superior superior vena abutting the lung metastatic deposit to the skin and
mediastinum cava syndrome. and lower pole of subcutis. Dead
Large enlarged thyroid of respiratory
mediastinal mass (goiter) failure within 1
found on mo
imaging.
Multiple
cutaneous and
subcutaneous
metastases
developed
rapidly
6 Mentzel et al5 M/61 Lower leg Subcutaneous mass Ill-defined mass Marginal excision, recurrence Free disease at 3
of unstated (size not specified) after 12 mo treated by yrs
duration wide excision with
negative margins
7 Ramdial et al6 M/30 Periampullary Obstructive 5.0-cm circumscribed Biopsy and Liver metastasis 8
periampullary pseudocapsulated pancreaticoduodenectomy mo after
mass with mass 2 mo later diagnosis
jaundice that
indented the
duodenum and
bulged into the
common bile
duct lumen in
a HIV patient
8 Mainville et al7 M/52 Floor of the left Sudden decrease in 5.3-cm rubbery atrial Marginal excision of cardiac Alive with
atrial wall left vision. mass with focal mass. Positive margins. metastatic
Work-up hemorrhage and Excision of metastatic brain disease at 8 mo
revealed right necrosis tumor followed by ɣ-knife
occipital mass radiotherapy. Laminectomy
(metastasis) and and vertebral fixation
left atrial mass.
Vertebral and
liver metastases
discovered 3 mo
later
9 Our case M/65 Dermis/subcutis of Enlarging painless 8.0-cm infiltrative Marginal excision of the mass. Free of disease
the right arm mass of unstated tumor in the Positive margins after 5 mo
duration dermis/subcutis.
Unencapsulated,
with pushing
borders. Tan-white
cut section with
areas of
hemorrhage and
necrosis

arranged in the well-characterized “patternless pattern” of Three patients died after developed metastasis4 and 5 were
architecture, hemangiopericytoma-like vessels and areas alive,4–7 3 of the 5 with metastatic disease.4,6,7
with dense collagen along with malignant cytological fea- In summary, we have reported a new case of the rare
tures, lymphovascular invasion, and neurotropism. In con- entity MMPCT and review the 8 cases published in the
trast to MMPCTs, they do not show the characteristic English literature. A careful examination of a proliferation
perivascular concentric multilayering of cells. By immu- of round-to-spindle cells with myoid appearance in
nochemistry, these tumors express Vimentin, CD 34, a concentric perivascular arrangement along with malig-
CD99, bcl-2, and STAT-6. In contrast to MMPCTs, they nant cytological findings, and positive immunophenotype
do not show expression for SMA. for the SMA, will facilitate a prompt diagnosis and the
The therapeutic options were individually decided. initiation of appropriate therapy. MMPCTs showed
Marginal excision is the major option when metastases are aggressive biological behavior with metastases occurring
excluded after proper staging and was practiced in 5 cases.4,5,7 within the first year of evolution. Only 2 cases were free of

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Am J Dermatopathol  Volume 38, Number 4, April 2016 Malignant Myopericytoma

metastatic disease, remarkably one of these cases showed 5. Mentzel T, Dei Tos AP, Sapi Z, et al. Myopericytoma of skin and soft
a focal positivity for cytokeratin (AE1/AE3).4 The follow- tissues. Am J Surg Pathol. 2006;30:104–113.
6. Ramdial PK, Sing Y, Deonarain J, et al. Periampullary Epstein-Barr
up period for our patient is as yet short-term; the patient is virus–associated myopericytoma. Hum Pathol. 2011;42:1348–1354.
free of disease after 5 months. It is clear in this study, 7. Mainville GN, Satoskar AA, Hans Iwenofu O. Primary malignant myoper-
however, that metastatic disease can appear later and icytoma of the left atrium—a tumor of aggressive biological behavior:
a long-term follow-up period is strongly recommended. report of the first case and review of literature. Appl Immunohistochem
Mol Morphol. 2012. [Epub Ahead of print].
8. Gaertner EM, Steinberg DM, Huber M, et al. Pulmonary and mediastinal
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