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2011
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Pseudomyogenic hemangioendothelioma: a distinctive, often
multicentric tumor with indolent behavior.Am J Surg Pathol. 2011
Feb;35(2):190-201.
A 1992 report
described 5 keratin-positive spindle cell neoplasms with multifocal
presentation in a single limb, which were proposed at that time to
be a variant of epithelioid sarcoma. This tumor type is not widely
recognized and is incompletely characterized. We examined 50 cases
of this distinctive tumor to evaluate histologic, immunophenotypic,
and clinical features. There was a 4.6:1 male predominance (mean
age, 31 y; 82% ≤40 y). Half of the patients presented with painful
nodules and the other half with painless nodules. Mean tumor size
was 1.9 cm (range, 0.3 to 5.5 cm). Tumors arose in the lower limb
(54%), the upper limb (24%), trunk (18%), or head and neck (4%).
Thirty-three (66%) were multifocal lesions (ranging from 2 to 15
lesions), including 32 cases with involvement of multiple tissue
planes. Of 205 total lesions, 64 (31%) involved the dermis, 42 (20%)
involved the subcutis, 70 (34%) lesions involved muscle, and 29
(14%) lesions involved bone; all the lesions had infiltrative
margins. The tumors were composed of loose fascicles and sheets of
plump spindle cells with vesicular nuclei, variably prominent
nucleoli, and abundant brightly eosinophilic cytoplasm, some with a
strikingly rhabdomyoblast-like appearance. In all cases, a minority
of cells were epithelioid. Twenty-seven tumors contained a prominent
neutrophilic inflammatory infiltrate. Most tumors showed only mild
nuclear atypia; 6 tumors contained foci of notably pleomorphic
cells. The median mitotic rate was 1 per 10 HPF (range, 1 to 10).
Seven tumors showed vascular invasion; 7 tumors had areas of
necrosis. By immunohistochemistry, all tumors were diffusely
positive for AE1/AE3 and FLI1; 22 of 47 tumors were variably
positive for CD31. Focal positivity was seen for CAM5.2 (21 of 35),
smooth muscle actin (14 of 42), epithelial membrane antigen (7 of 49
weak), and PAN-K (MNF116) (1 of 47). All were negative for CD34,
desmin, and S100 protein and showed intact INI1 expression.
Follow-up was available for 31 patients and ranged from 9 months to
17 years (mean, 4 y). Most lesions were treated by local excision.
Eighteen (58%) patients had local recurrence or developed additional
nodules in the same region, all but one, within 1 year of first
presentation. Eight patients had postoperative radiation therapy and
6 patients had chemotherapy. Four patients had amputations for
multifocal disease. One patient had a regional lymph node
metastasis, and, thus far, only 1 patient has developed distant
metastases (disseminated), 16 years after primary tumor excision. At
the time of the last follow-up, 27 patients were alive with no
evidence of the disease, 1 patient was alive with unknown disease
status, 2 patients were alive with recurrent disease, and 1 patient
died of the disease. In summary, we describe a distinctive type of
rarely metastasizing ("intermediate") tumor affecting mainly young
men and usually characterized by multifocality in different tissue
planes of a limb. Although sharing some features with epithelioid
sarcoma (skin/soft tissue of distal extremities, young adults,
keratin positive), it differs by having predominantly myoid-appearing
spindle cell morphology, expression of FLI1, common reactivity for
CD31, lack of epithelial membrane antigen, CD34, and PAN-K
expression, and intact INI1. The overall immunophenotypic findings
favor endothelial differentiation. Despite the ominous presentation,
follow-up thus far suggests an indolent clinical course with a small
risk of distant metastasis. Although the precise nosologic status of
this tumor type is uncertain, we propose the interim designation "pseudomyogenic
hemangioendothelioma." |
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The term '
haemangioendotheliomata ' was introduced by Mallory FB. (J Exp Med 1908;
10 : 575-593) to include all 'tumours arising from blood vessel
endothelium' and this term was not associated with the clinical behaviour
of the tumours.
Stout AP (Ann Surg 1943; 118: 445-464) described the microscopic
appearances of malignant tumours of blood vessels and linked the term
hemangioendothelioma to malignant behaviour.
Later, Enzinger FM, Weiss SW (Soft tissue tumour, 2nd ed 1988: 533-544)
described hemangioendotheliomas as vascular tumours of intermediate,
borderline or low grade malignancy.
The term "hemangioendothelioma" has been used to designate various groups
of vascular tumours. Some of these entities are benign non- neoplastic
reactive lesions.
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EPITHELIOID HEMANGIOENDOTHELIOMA
Dermatopathology Case 98
Epithelioid
hemangioendothelioma: a vascular tumor often mistaken for a carcinoma.
Epithelioid hemangioendothelioma of skin and
soft tissues: clinicopathologic and immunohistochemical study of 30
cases.
Epithelioid
hemangioendothelioma is a malignant vascular tumour which
was described by Enzinger and Weiss in 1982.
The tumour occupies an
intermediate position in the spectrum of epithelioid vascular tumours
lying between the benign
epithelioid hemangioma and the more
aggressive
epithelioid angiosarcoma.
Site:
Soft tissue, lung ( formerly known as intravascular bronchioalveolar tumour), pleura, liver, peritoneum, bone, lymph
nodes and skin.
Age:
Middle-aged adults.
Microscopic features:
IMAGE
LINKS
:
Case Images :click here
Poorly defined and
infiltrative tumour, characterized by nests and cords of
spindle to epithelioid cells embedded in a hyaline, myxoid, chondroid
or collagenous stroma.
Diagnostic feature:
The cells show prominent cytoplasmic vacuoles. Red blood cells may be present within
some of these vacuoles (reminiscent of primitive vascular channels). These
vacuoles are mucin negative.
Other features that may be
present : Abundant osteoclast-like giant cells ;
dystrophic calcification; metaplastic ossification.
There is a low mitotic
activity and mild to moderate pleomorphism.
In
some cases there are solid nests of tumour cells displaying cytological atypia together with increased mitotic activity
(more than 1 per 10 HPF) and areas of necrosis. Cases with these
features are associated with bad prognosis.
Variant:
Epithelioid sarcoma-like
hemangioendothelioma. Am
J Surg Pathol.
2003;27(1):48-57
Immunohistochemistry: Tumour
cells express endothelial markers : CD31 is one of the most sensitive
immunohistochemical markers. von Willibrand factor is also
positive.
Some cases show positivity
for smooth muscle actin and cytokeratin. EMA is negative unlike other epithelial tumours.
S-100 is also negative.
The tumour has a good
overall prognosis but local recurrences and distant metastases can
develop.
Differential diagnosis :
Primary or metastatic
carcinoma ;
epithelioid sarcoma
(Positive for keratin, EMA and often CD34 ; Negative for CD31
or von Willibrand factor);
myxoid liposarcoma (branching
vascular pattern and small multivacuolated lipoblasts) ;
myxoid chondrosarcoma
(lobular architecture; absence of cytoplasmic vacuoles and
S-100 positive).
Characteristic morphological features
together with appropriate immuno- histochemical staining helps in
establishing the diagnosis.
Other Images:
Img1
(surgicalpathologyatlas.com):
Img2 (YaleRosen)
Abstracts:
Epithelioid hemangioendothelioma of the
suprasellar area (pdf file)
Primary pleural epithelioid
haemangioendothelioma with metastases to the skin. A case report and
literature review.J
Clin Pathol.
2005;58(1):107-9
Right upper quadrant pain and
fever in a 41-year-old man. Epithelioid hemangioendothelioma of the
liver with metastasis to porta hepatis lymph nodes and lung.Arch
Pathol Lab Med.
2005;129(5):e134-5.
Incidental in vivo detection
of an epithelioid hemangioendothelioma of the mitral valve.Pathol
Int. 2005;55(10):644-8
Cutaneous epithelioid
hemangioendothelioma.Ann
Dermatol Venereol.
2004 ;131 (8-9):818-21.
A clinicopathologic study on 9
cases of epithelioid hemangioendothelioma.
Zhonghua Bing Li Xue Za Zhi.
2001;30(3):177-9. |
|
Epithelioid hemangioendothelioma of the facial bone.J Craniofac Surg.
2010 Nov;21(6):1994-6.
The epithelioid
hemangioendothelioma is an uncommon vascular neoplasm. Numerous sites
of involvement are possible, but these tumors most commonly arise in
the soft tissues, liver, and lung. We report a case of epithelioid
hemangioendothelioma presenting as a single lesion on the nasal
portion of the maxilla.
Histologically,
the tumor within bone and skin exhibited cords and nests of plump,
epithelioid-appearing cells exhibiting rudimentary vascular
differentiation within a myxohyaline stroma. Immunohistochemical
reactivities for factor VIII-related antigen, CD31, CD34, and vimentin
were demonstrated.
Wide local
excision with close clinical follow-up seems to be the treatment of
choice for these tumors.
Intraoral epithelioid
hemangioendothelioma: a case report and review of the literature.Med
Oral Patol Oral Cir Bucal. 2010 Mar 1;15(2):e340-6.
The
epithelioid hemangioendothelioma (EH) is an uncommon angiocentric
neoplasm of borderline or intermediate malignant potential, between
the hemangioma and conventional angiosarcoma.
It is
characterized by the proliferation of endothelial cells with
epithelioid or histiocitóide morphology with vacuolated cytoplasm and
occasional eosinophilic spindle cells. Shows potential for local
recurrence as well as the ability to metastasize.
Rarely affects
the oral cavity, it have been described from 1975 until 2008 only 27
oral cases. Morphologically the EHs may be confused with other
lesions, from a hemangioma to a squamous cell carcinoma, and thus
immunohistochemical analysis is required.
This paper
reports the clinical and immunohistochemical characteristics of a case
of EH in the gingiva of the tooth 35 of a 17 years-old-white-female.
We present a
review of the clinicopathological and immunohistochemical
characteristics of the intraoral epithelioid hemangioendothelioma
cases previously reported. |
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KAPOSIFORM HEMANGIOENDOTHELIOMA
Dermatopathology Case 99
Kaposiform
hemangioendothelioma is a locally aggressive, non-metastasizing vascular tumour.
This lesion is commonly associated with Kasabach-Merritt syndrome and
lymphangiomatosis.
Site: Retroperitoneum, subcutaneous or deep soft tissue of upper
extremities, head and neck area.
Age:
Occurs as solitary tumour in infants (first decade of life)
.
Gross images 1(emedicine.medscape.com)
;
Gross Image2 (Dermatlas):
Microscopic features:
IMAGE LINKS
Microscopic images : click here
; Other images (Dermatlas).
Img1 ;
Img2 ;
Img3
Tumour consists of infiltrating lobulated nodules
of spindle shaped endothelial cells ; Nodules are
separated by thick fibrous bands ; Spindle cells form short
fascicles and line slit-like and round vessels ; Minimal cytological atypia
; Mitotic activity is low ; Epithelioid cells are
sometimes present ; Cells may contain hemosiderin pigment, hyaline globules and cytoplasmic
vacuoles ; Ectatic vessels are present in the periphery ; Scattered microthrombi within vascular spaces.
Immunohistochemistry:
The spindle cells stain positively for CD34 and focally for CD31. The pericytes
around the capillaries stain positively for smooth muscle actin.
Vascular Endothelial
Growth Factor Receptor-3 (VEGFR-3): A Marker of Vascular Tumors with
Presumed Lymphatic Differentiation, Including Kaposi's Sarcoma,
Kaposiform and Dabska-Type Hemangioendotheliomas, and a Subset of
Angiosarcomas.
Differential diagnosis : Kaposi's sarcoma
(rare in children; multicentric; prominent chronic inflammatory
infiltrate; lobular architecture is absent; dense fibrous bands are
not present);
infantile hemangioma
;
acquired tufted hemangioma
;
spindle cell
hemangioendothelioma
;
Angiosarcoma
(endothelial atypia, increased mitotic activity and dermal collagen
dissection by infiltrating tumour)
Abstracts:
D2-40 immunohistochemical
analysis of pediatric vascular tumors reveals positivity in kaposiform
hemangioendothelioma.
Mod Pathol. 2005;
[Epub ahead of print]
Thoracic kaposiform
hemangioendothelioma. Four consecutive cases with distinct outcome.An
Pediatr (Barc).
2005 Jul;63(1):72-6.
Kaposiform
hemangioendothelioma: a study of 33 cases emphasizing its pathologic,
immunophenotypic, and biologic uniqueness from juvenile hemangioma.Am
J Surg Pathol.
2004;28(5):559-68.
Retroperitoneal kaposiform
hemangioendothelioma with tufted angioma-like features in an infant
with Kasabach-Merritt syndrome.Pathol
Int. 2003;53(9):627-31.
|
| POLYMORPHOUS
HEMANGIOENDOTHELIOMA
Polymorphous
hemangioendotheliomas are rare, low-grade borderline malignant
vascular tumours of endothelial cell origin.
Site: The tumour most frequently involves lymph nodes,
but it can also affect extranodal locations like spinal cord.
Microscopic features:
Characterized by a variety of patterns within the same lesion.
- Solid areas with
sheets of polygonal cells with scanty cytoplasm and minimal atypia;
- Vascular
differentiation - cytoplasmic vacuoles, clefts, channels and papillary
structures;
- Angiomatous areas
with vascular spaces lined by endothelium with or without hobnail
appearance.
Immunohistochemistry:
UEA-1: positive ; von Willibrand factor: negative.
Differential diagnosis:
Angiosarcoma
(
absence of anaplasia and dissecting vascular channels in
polymorphous hemangioendothelioma)
Abstracts:
Polymorphous
hemangioendothelioma of the neck.
Ann
Diagn Pathol. 2003;7(3): 165-8.
Primary polymorphous hemangioendothelioma of the
spinal cord. Case report. J
Neurosurg. 2001;95(1 Suppl):93-5.
Polymorphous hemangioendothelioma. Ann
Thorac Surg. 1999;68(4):1396-7.
Polymorphous
hemangioendothelioma: a report of two cases, one affecting extranodal
soft tissues, and review of the literature.Am
J Surg Pathol.
1997;21(9): 1083-9.
Primary vascular tumors of
lymph nodes other than Kaposi's sarcoma. Analysis of 39 cases and
delineation of two new entities.Am
J Surg Pathol.
1992;16(4):335-50.
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Abstracts:
Hemangioendotheliomas-evolution of a concept of
a heterogeneous group of vascular neoplasms.Verh
Dtsch Ges Pathol.
1998;82:99-111
Cutaneous vascular
proliferations. Part III. Malignant neoplasms, other cutaneous
neoplasms with significant vascular component, and disorders
erroneously considered as vascular neoplasms.J
Am Acad Dermatol.
1998;38(2 Pt 1):143-75; quiz 176-8.
Eruptive epithelioid
hemangioendothelioma with spindle cells. Nosological place in the
spectrum of epithelioid vascular tumors.Ann
Pathol. 1999;19(4):312-5.
Epithelioid hemangio- endothelioma presenting
in the skin: a clinicopathologic study of eight cases.Am
J Dermatopathol. 1998;20(6):541-6.
A case of childhood epithelioid hemangio-
endothelioma.
J Am
Acad Dermatol. 2000 ;42(5 Pt 2):897-9.
Immunohistochemical study of
epithelioid hemangio endothelioma in the leg: a case report.Acta
Med Okayama. 1996;50(5)
:279-83.
Epithelioid hemangio-
endothelioma of the anterior mediastinum. Clinicopathologic, immuno
histochemical, and ultrastructural analysis of 12 cases.Am
J Surg Pathol. 1994
Sep;18(9):871-81.
Thrombocytopenic coagulopathy (Kasabach-Merritt
phenomenon) is associated with Kaposiform hemangioendothelioma and not
with common infantile hemangioma.Plast
Reconstr Surg.
1997;100(6):1377-86.
Kaposiform hemangio-
endothelioma of infancy and childhood. An aggressive neoplasm
associated with Kasabach-Merritt syndrome and lymphangiomatosis.Am
J Surg Pathol.
1993;17(4):321-8.
Kaposi-like infantile hemangioendothelioma. A
distinctive vascular neoplasm of the retroperitoneum.Am
J Surg Pathol.
1991;15(10):982-9.
Kaposiform hemangio- endothelioma: five patients
with cutaneous lesion and long follow-up.
Mod Pathol.
2001;14(11):1087-92.
Kaposiform hemangio-
endothelioma in adult. Report of a case with amianthoid-like fibrosis
and angiectases.Cesk
Patol. 2000;36(4):163-7.
Kaposiform hemangio-
endothelioma in adults. Clinicopathologic and immunohistochemical
analysis of three cases.
Am J Clin Pathol.1997;108(4):450-5.
Transformation between Kaposiform hemangio-
endothelioma and tufted angioma.
Dermatology.
2003;206(4):334-7.
Kaposiform hemangio- endothelioma of the
external auditory canal in an adult.
Head Neck.
2002;24(6):614-7.
| Vascular tumors
of the skin: A selective review.Seminars in Diagnostic
Pathology.Volume 21, Issue 3 , Pages 166-218, August
2004
Cutaneous vascular prolife rations
are a vast and complex spectrum. Many appear as hamartomas in
infancy; others are acquired neoplasms. Some vascular
proliferations are hyperplastic in nature, although they mimic
hemangiomas, i.e., neoplasms. The vast majority of the vascular
lesions are hemangiomas. Between the hemangiomas and frankly
angiosarcomas, there is a group of neoplasms that are
angiosarcomas, albeit ones of low grade histologically and,
probably, biologically. The term “hemangioendothelioma” has been
created to encompass these neoplasms.
Vascular proliferations are, fundamentally,
composed of endothelial cells. Some hemangiomas, however, contain
also abundant pericytic, smooth muscle, or interstitial
components, or a combination of them. These heterogeneous cellular
components are present usually in hemangiomas. Some of the newly
described vascular proliferations, however, are difficult to
differentiate from some of the angiosarcomas. Others are markers,
occasionally, of serious conditions such as Fabry’s Disease (angiokeratoma)
and POEM’s syndrome (glomeruloid hemangioma).
Kaposi’s sarcoma continues to be an enigma. The
demonstration of Herpes virus 8 in this condition raises doubt
about its neoplastic nature. The demonstration of endothelial
differentiation of its nodular lesions is tenuous and its true
nature remains unresolved.
While physicians have known about
post-mastectomy angiosarcomas from the origin of the radical
mastectomy, a new group of unusual vascular proliferations of the
mammary skin are being defined. These lesions arise in the setting
of breast-conserving surgical treatment with adjuvant radiation
therapy. The incubation period is usually 3 to 5 years, in
contrast with the 10, or more, in classical cases of
post-mastectomy angiosarcoma. These lesions usually are subtle,
both clinically and histologically, in contrast with the
“classical,” dramatic presentation of mammary angiosarcoma. The
spectrum of findings ranges from “simple” lymphangiectasia-like
vascular proliferations to unequivocal angiosarcomas. The
pathogenesis of these lesions remains a mystery.
There are very few clues that allow one to
separate hemangiomas from angiosarcomas. The presence of
heterologous cellular elements and, particularly, well-developed
smooth muscle components tends to favor a hemangioma. Similarly,
the presence of thrombosis usually supports hemangioma.
Nevertheless, there are no unequivocal or reliable individual
diagnostic criteria. A thorough knowledge of the different
conditions and their differential diagnoses eventually leads to
the proper diagnosis in most cases.
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