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f Cutan Pathol 1997: 24: 641-647 Cojiyright e Mniiksgaard 1997


Printed in Denmark - allrightsreseived
Journal of
Cutaneous Pathology
ISSN 0303-6987

Cutaneous epithelioid hemangioendothelioma,


epithelioid cell histiocytoma and Spitz nevus
Three separate epithelioid tumors in one patient

We report tbe unusual history of a yottng lady who, over a period Bettina G. Zelger,
of 9 years, developed 3 epithelioid neoplasms of different hisdo- Beatrix WambacherS
getietic origiti: a ctttatieotts epithelioid hemangioendothelioma, Hansjorg Steiner and
an epithelioid cell histiocytoma, and a Spitz nevus. Bernhard Zeiger^
To the best of our knowledge, such ati ttnustial combination of Departments of Patiiology and i Dermatology,
neoplasms bas not been reported previously atid raises tbe qttes- University of Innsbruck, Innsbruck, Austria
tion of at! underlying "epithelioid" reaction patterti respotisible
for the epithelioid appearance in all 3 tumors; the likelihood of
svtch a combination of' tumors occurring in a single patient would
be less than 1 in 1 billion.
Dr. Bettina Zelger, Department of Pathology,
Zelger BG, Wambacber B, Steiner H, Zelger B. Cutaneous epitlie-
University of Innsbruck, Mullersfr. 44,
lioid hemangioendothelioma, epithelioid cell histiocytoma and A-6020 Innsbruck, Austria
Spitz nevus. Three separate epithelioid tumors in one patient.
J Cutan Pathol 1997: 24: 641-647. © Munksgaard 1997. Accepted June 11,1997

Epithelioid differentiation is a rare, yet well-estab- vious history of trauma or insect bite and was ex-
lished cellular pbenomenon seen in a variety of tu- cised with the differential diagnosis of a hemangi-
mors (Table 1) (1-f f). Many of tbese tumors may oma. The local pathologist diagnosed a malignant
occur in a characteristic setting, so that patholo- melanoma, Clark IV, 2.0 mtii maximum tmiior
gists have become familiar with these problems thickness. Complete internal examination includ-
and/or constellations, e.g. Spitz (epitbelioid and itig routine laboratory values, chest x-ray, abdomi-
spindle cell) nevi (1), epithelioid cell bistiocytoma nal sonograpby, cerebral CT and bone scintigra-
(3) or angiolytTiphoid hyperplasia with eosi- pby were normal. Re-excision of tbe scar was per-
nophiha (5). Yet, in some types of lesions, where formed 2 weeks later with a 2-cm security margin
only a limited number of cases have been report- withovtt evidetice of tumor remnants. Her history
ed, e.g. epitbelioid malignant scbwannoma (8) was otherwise tmremarkable and tbe patient was
and (cutaneous) epithelioid leiomyosarcoma (9), in good getieral bealtb. Due to change of address
or where tbe clinicopathologic constellation fol- 2 years later, the patient came to our tumor chnic
lows no particular setting, e.g. epithelioid heman- where regular clinical (twice a year) as well as im-
gioendothelioma (12), there maybe considerable aging follow-ups (chest x-ray, abdominal sonogra-
diagnostic difficulties. pby once a year) remained inconspicuotis for
Tbe present case report describes such a patient more tban 6 years unfil May 1994. At this time -
with an unusual association of 3 epithelioid neo- the patient was nearly 33 years of age - a l-ctii-
plasms of different cellular lineage. large, moderately firm, tan paptile with some
prominent telangiectatic vessels had developed on
tbe left breast over a period of 3 months. Clinical-
Case report
ly, a basal cell carcinoma was suspected and tbe le-
A 25-year-old woman presented to her local der- sion excised witb small margins. Tbe diagnosis of
matologist with a reddish dermal tumor on the a cutaneous "epithelioid" hemangioendotbelio-
volar aspect of the left lower arm. The lesioti had ma (see below) was followed by re-excision with 2-
developed over the past 6 months without any pre- cm margins and complete internal work-up (rovi-
641
Zelger et al.

Fig. 1. a) Irregular plaque-like lesion of epilhelioid


hemangioendolhelioina. Note sheet-like infiltrate of brightly
eosinophilic (epithelioid) cells demarcated and hifiltrated by
lymphocytes, b) Higher magnification reveals "miniretiform"
lumina (arrows) and sparse stromal fibrosis (arrowhead), c)
High-power magnification shows dense epithelioid infiltrate
dissected by vessel with hobnail appearance of endothelial
cells. Note focal intracytoplasmic lumen formation (arrow).

tine laboratory values, chest x-ray, abdominal


sonograpby, cerebral CT and bone scintigraphy)
without any evidence of disease. Nine months lat-
er another reddish, moderately hard, exophytic
papule of 7-mm diameter developed on the right
shoulder, again without any previous evidence of
trauma or insect bite. Excision of the lesion, again
assumed to be a basal cell carcinoma, revealed an
epithelioid cell histiocytoma. Due to this unusual
combination of tumors the slides of the primary
melanocytic lesion from 1986 were requested and
revealed that the initial diagnosis of a malignant
melanoma had to be corrected to a Spitz nevus.
Since then the patient has remained well with no
further tumor recurrence after a follow-up of 2
years.

Material and methods


Slides and paraffm material of all 3 lesions (as well
as 2 re-excisions) were available to us. Serial sec-
tions were cut and mounted from formalin-fixed,
paraffin-embedded material of all biopsies. Rou-
tine H&E-stained sections were obtained in all cas-
es and supplemented by Perl's Prussian blue stain
for iron, Fontana-Masson stain for melanin as well
as orcein and van Gieson stains in some instances.
Immunohistochemically, a variable number of
markers was applied for each lesion including the
neurogenic and/or melanocytic markers SlOO
protein, NK1C3 (CD57) and HMB45, the histio-
cytic and/or dendritic markers Ki-Mlp, KPl
(CD68), HAM56 and factor Xllla, the vascular
markers JC70A (CD31), QBEndlO (CD34), factor
VIII and the lectin ulex europevis agglutinin, the
lymphocytic markers LCA (CD45), UCHLl
(CD45RO) and L26 (CD20), epithelial markers
for pankeratin, CAM 5.2, epithelial membrane an-
tigen and carcinoembryonic antigen, the interme-
diate filament markers vimentin and desmin, as
well as a variety of other markers such as for neu-
ron-specific enolase, smooth muscle-specific actin
and HHF 35.
The (routine) immunohistochemical technique
employed was a 3-step streptavidin-biotin complex
technique. Peroxidase was visualized with 3amino-

642
Cutaneous epithelioid hemangioendothelioma

9ethyl carbazole and finally counterstained with


Harris hematoxylin. Well-suited negative and posi-
tive controls were inckided in each staining proce-
dure. Moreover, positive internal controls were
present for most antibodies applied. Details of the
methods used have been described elsewhere
(13).
Ultrastructural studies were performed using
the block of formalin-fixed, paraffin-embedded
tissue of the vascular neoplasm. Selected tvunor
parts were punched out of the paraffin block, re-
embedded in Epon 812 and stained with uranyl
acetate/lead citrate in the usual way. Specimens '4''"-
were scanned with a goniometer-equipped Philips
EM 400 electron microscope operating at 80 KV.

Results
Scanning magnification of the first basal cell carci-
noma-like lesion (with serial sections) seen at our
tumor clinic revealed an irregular plaque-like der-
mal infiltrate underlying an inconspicuous epider-
mis and a small grenz zone (Eig. la). The lesion
was moderately well circumscribed with a mostly
sheet-like infiltrate, but also showed foci with ar-
borizing blood vessels arranged in a retiform pat-
tern (reminiscent of normal rete testis). Tiny foci
of this pattern have been called (14) "minireti-
form" differentiation (Eig. lb). Higher magnifica-
tions of sheet-like areas (Fig. lc) revealed epithe-
lioid cells similar in size to keratinocytes with eosi-
nophilic cytoplasm, large nuclei with prominent
eosinophilic nucleoli and occasional intracytoplas-
mic lumina partially filled with erythrocytes. Reti-
form areas showed similar cellular characteristics
with hobnail appearance of endothelial cells (Fig.
lc). No atypia or atypical mitoses were detected in
the lesion. The lesion was infiltrated and demar-
cated by a very prominent lymphocytic infiltrate
and showed focal stromal fibrosis to sclerosis (Fig.
lb), but no pigment deposition (negative stains
for iron and melanin). Nearly all endothelial cells
showed strong labeling for CD34 with nearly equal
intensity of both the cytoplasm and the cell mem- Fig. 2. a) Immttnolabeling for CD34 outlines a sheet-like to ret-
brane (Fig. 2a); staining for CD31 (Eig. 2b) was iform area interspersed by numerous lymphocytes, b) High-
predominantly membranous, outlining the lumi- power magnification of epithelioid endothelial cells with focal
nal and abluminal sites of endothelial cells. In intracytoplasmic lumina positive for CD31 (Harris hematoxylin
cotuiterstain).
contrast, factor VIII and the lectin vilex europeus
agglutinin showed less prominent and consistent
labeling of vascular lumina. The lesion was also
strongly positive for neuron-specific enolase and pinocytosis, nrmierous Weibel-Palade bodies as
vimentin. All other markers, in particular epitheli- well as abluminal basement membrane formation.
al ones, were negative. Lymphocytes labeled for The epithelioid cell histiocytoma revealed an
LCA (CD45) and UCHLl (CD45R0), but were exophytic sheet-like tumor of the papillary dermis
negative with L26 (CD20). Electron microscopy with "collarette" formation of the epidermis later-
revealed large epithelioid cells with euchromatic al to the lesion. The infiltrate consisted of large
nuclei and prominent nucleolei, marked luminal epithelioid cells with scalloped, spidery cell exten-

643
Zelger et al.

Fig. 3. Epithelioid cell histiocytoma with sheet-like infiltrate of


characteristic scalloped cells.

sions embedded in a moderately loose fibrocolla-


genous stroma (Fig. 3). The cytoplasm was homo-
geneously eosinophilic, the nuclei large with
prominent eosinophilic nucleoli. Lymphohistio-
cytes were interspersed between the histiocytic in-
filtrate as well as at the periphery of the lesion.
The lesion was strongly positive for factor Xllla
and vimentin, weakly positive for Ki-Mlp and
HAM56, but negative for all other markers.
Re-examination of the melanocytic lesion re-
vealed a regular, exophytic papule with prominent Fig. 4. a) Silhouette of Spitz nevus widi hyperkeratosis, hyper-
hyperkeratosis, hypergranulosis and epidermal hy- granulosis, epidermal hyperplasia and homogeneous eosi-
perplasia (Fig. 4a), which showed signs of focal ir- nophilic infiltrate of b) nests of epithelioid and spindle-shaped
ritation by parakeratosis and serum exudate. Soli- nevus cells.
tary units and nests of spindle-shaped to epithelio-
id melanocytes were arranged along the basement
membrane and within the papillary dermis. Char-
acteristically, these cells showed large euchromatic - not including the pigmented spindle cell tumors
nuclei with prominent nucleoli, but no significant of Reed (15), which according to our experience
cellular atypia or hyperchromasia (Fig. 4b); below are 10 times more common -were collected. This
areas of irritation superficially located mitoses laboratory receives approximately 20,000 .skin
were present. Prominent ectatic capillaries were specimens per year in a medical catchment area of
seen at the bottom of the lesion as well as a moder- 1 million people (roughly 90% of all skin speci-
ate patchy lymphohistiocytic infiltrate. The lesion mens in this area). This means an incidence rate
was markedly positive for SlOO protein and NK1C3 of approximately 1 case per million people a year
(CD57), but showed only focal reactivity of for both, epithelioid cell histiocytoma and Spitz
melanocytes at the basement membrane for nevi, respectively. Due to the extreme rareness of
HMB45. Again, vimentin was positive, and all oth- cutaneous (as well as soft tissue) epithelioid he-
er markers negative. mangioendothelioma no definitive incidence rate
In a work-up of all soft tissue tumors sent to the is possible, but is certainly much less than the oth-
Dermatohistopathologic Laboratory of the De- er two entities.
partment of Dermatology, University of Innsbruck,
we found 15 epithelioid cell histiocytomas and 8
Discussion
epithelioid vascular tumors: 6 cases of angiolym-
phoid hyperplasia with eosinophilia, 1 subcutane- The present case report nicely documents how ep-
ous epithelioid hemangioendothelioma and 1 epi- ithelioid differentiation may cause considerable
thelioid angiosarcoma. Similarly, 22 Spitz nevi (1) diagnostic difficulties.

644
Cutaneous epithelioid hemangioendothelioma

Table 1. Clinicopathologic characteristics of epitheiioid neopiasms

Entity (reference) Sex Age Prediiection Clinicopathoiogic criteria


(Femaie-to- (years)
maie ratio)

Spitz/epithelioid and spindie 1:1 0-5 Face, (distal) Solitary (rareiy muitipie) reddish tumor(s) cilnicaiiy simulating a hemangioma,
ceil nevus (1) extremities pyogenic granuioma etc., histologicaiiy a maiignant meianoma ("juveniie malignant
melanoma")

Epithelioid neurcfibroma (2) 1:1 20-40 None Epithelioid celis in iesion otherwise similar to ordinary neurofibroma

Epitheiioid celi histiocytoma 4:1 20-40 (Lower) Soiitary, moderateiy firm exophytic papuie to nodule (5 to 10 mm diameter),
(3) extremities histologicaiiy with epidermai coiiarette and characteristic scaiioped celis positive
for factor Xiiia

Epitheiioid ("bizarre") 1:2 40-60 Stomach Weil-demarcated noduie with sh6et-iii<e, vacuoiated-to-ciear epithelioid ceils with
ieiomyoma; blunt-ended nuciei; consistent smooth muscle actin, variabie desmin reactivity
ieiomyobiastoma (4)

Angioiymphoid hyperpiasia 2:1 , 30-50 Scalp, in Soiitary (to muitipie) piaque-iii<e anglomatous lesion(s) with prominent epitheiioid
with eosinophiiia (5); particuiar endotheiial celis, intracytoplasmic blebs and lymphocytic stromai response often
epitheiioid (6) or histiocytoid periauricular rich in eosinophiis
hemangioma (7)

Epithelioid maiignant 1:1 30-50 Superficial/deep Often originating from iarge nerve (fascicle) or neurofibroma: geographical
schwannoma (8) soft tissue of appearance with ceiiuiar and myxoid areas, sheets of epitheiioid celis with
extremities prominent nucleoii; variabie S100 and NSE reactivity; uitrastructurai schwannian
(inciuding hip differentiation.
and shouider)

Epitheiioid ieiomyosarcoma 3:1 60-80 Deep soft Large (>20 cm) muitilobulated tumors with iong fascicies of epitheiioid ceils with
(9) tissue of cigar-like nuciei; desmin reactivity; frequent iiver and iung metastases
retroperitoneum

Epitheiioid angiosarcoma 1:4 >60 Deep soft Sheets of epitheiioid celis wifh focai intracytopiasmic lumina; positive for vascuiar
(10) tissue of lower and (often) epitheiiai markers; can be misinterpreted as metastasis ot carcinoma
extremities
Epitheiioid sarcoma (11) 1:2 10-35 Distai Multinoduiar sheet-like proiiferation of monomorphous epitheiioid (to spindie-
extremities shaped) ceils often with centrai necrosis ('geographic pattern'); coexpression of
pankeratin, EMA and vimentin; can be misinterpreted as granuloma annuiare

Thus, first, the Spitz nevus was misdiagnosed as Obviously, this was neither a common form of
malignant melanoma, pi'obably favored by the ad- angioma (16) nor a classical angiosarcoma (17).
ditional changes of the lesion due to irritation. The epithelioid features with focal hobnail appear-
This phenomenon is well established in the litera- ance suggested tTvo differential diagnoses: angioi-
ture and numerous, similarly problematic cases ymphoid hyperplasia with eosinophilia (5), also
have over the years clearly justified the separation known as "epithelioid" (6) or "histiocytoid heman-
of Spitz nevi as a distinct clinicopathologic entity gioma" (7), and retiform hemangioendothelioma
(1). (14). Eor angioiymphoid hyperplasia with eosi-
Secondly, influenced by the patient's history, nophilia, which usually occurs on the periauricular
the clinically basal cell carcinoma-like lesion on scalp of females in their 4th to 5th decade (Table
the breast was originally suspected to be an unusu- 1), the clinical presentation would have been rath-
al metastasis from the malignant melanoma. Yet, er unusual. Moreover, the sheet-like appearance of
when all immtniohistochemical markers thereof epithelioid cells in most parts of the lesion as well as
were negative (despite good internal controls), a the complete absence of eosinophiis did not match
second run with a broad panel of markers re- with this diagnosis. For retiform hemangioendothe-
vealed the vascular origin of this lesion. Only then lioma (Table 2), tlie characteristic features were too
did the intracytoplasmic lumina became obvious. focal and subtle to explain the whole lesion. Other
Further ultrastructural studies then confirmed the vascular neoplasms such as microvenular hemangi-
vascular origin, yet the exact classification re- oma (18) or targetoid hemosiderotic hemangioma
mained another difficult task/challenge. (19) could be excluded by the prominent epithelio-

645
Zelger et al.

Tabie 2. Clinicopathoiogic characteristics of hemangioendotheiiomas

Entity (reference) Sex Age Predilection Clinicopathologic criteria Prognosis and pecuiarities
(Femaie-to- (years)
maie ratio)

Spindle ceii 1:1.5 10-30 Distai Soiitary to muitipie, biuish, painful Vascuiar malformation, occasionaiiy associated with
hemangio- extremities tumors aiong pre-existing vesseis, congenital lymphedema, Maffucci- or Klippei-
endotheiioma (25) coexistence of cavernous and Kaposi- Trenaunay-syndrome; recurrences
iike areas

Kaposiform 1:1 Child- Retroperito Soiitary, multilobular, septated tumors 50% mortaiity by consumption coaguiopathy
hemangio- hood neum similar to nodular stage of Kaposi (Kasabach-Merritt-syndrome); presumably only a
endotheiioma (26) sarcoma without intracytoplasmic, variant of (benign) oeiiuiar hemangioma ("juveniie,
eosinophiiic giobuies intantiie hemangioendotheiioma")

Poiymorphous 1:1 20-40 Lymph Polymorphous mixture of capiilary Extremeiy rare with oniy a few cases in the worid
hemangio- nodes hemangiopericytoma-like areas beside iiterature; probably only a variant of (benign) ceiiuiar
endothelioma (27) cavernous tumor parts as weii as hemangioma ("juveniie, infantiie
strands and nests of epitheiioid ceils in hemangioendothelioma") with variabie differentiation
fibroscierotic stroma

Retiform hemangio- 1:1 10-40 Extremities Piaque-like iesion with long arborizing Frequent recurrences, rare metastases (iymph node);
endotheiioma(14) ("retiform") vessels, prominent, presumably one component of maiignanf
partiaiiy epitheioid endotheliai celis, endovascuiar papiiiary angioendothelioma (Dabsi<a-
papiiiary formation and prominent tumor)
iymphocytic infiitration

Epitheiioid 1:1 10-80 Deep soft Soiitary, moderateiy painful fumor, in Variabie course, often protracted for years with 10%
hemangio- tissue 50% assooiated with a iarge vessei, recurrences, 30% metastases (mostly to iymph
endotheiioma(12) showing strands and nests of nodes, rareiy to internai organs) and 10-20% tumor-
epitheioid tumor celis in a myxoid-to- associated mortaiity; overiap with epithelioid
fibrotic stroma, intracytoplasmic angiosarcoma, inciuding misinterpretation as
iumina with erythrocytes metastasis of carcinoma

id appearance of tumor cells as well as the absence Enzinger (12), the case of Malane etal. (21) shows
of characteristic features such as collagen dissection great similarities to the 3 cases of epithelioid angi-
by small capillaries and/or complete absence of osarcoma described by Marrogi et al. (10). These
dermal hemosiderin deposits, respectively. Thus, difficulties in exact diagnosis underline the bor-
dealing with an epithelioid vascular neoplasm with derline character of epithelioid hemangioen-
irregular shape and of uncertain behavior, we made dothelioma with overlap to both benign and ma-
the diagnosis of an epithelioid hemangioendotheli- lignant lesions.
oma (Table 2), a borderline vascular lesion (12). Moreover, when recognizing the focal similari-
Characteristically, epithelioid hemangioen- ties of our lesion to retiform hemangioendothelio-
dothelioma occurs in deep soft tissue (12). To the ma (14), another vascular variant of intermediate
best of our knowledge, only 4 cases of cutaneous biologic behavior, the situation becomes even
epithelioid hemangioendotheiiomas have been more complex. One might speculate that our le-
reported (20—23). Illustrations of these lesions re- sion represents a very early variant of a vascular
veal rather heterogeneous histologic features, neoplasm with biphasic, i.e. epithelioid and reti-
which cast some doubt on the diagnoses. Thus, form, differentiation. Indeed, such combination
from the clinical and histologic photographs pro- and/or overlap between various types of heman-
vided, we would have favored an "old", i.e. partial- gioendothelioma, e.g. retiform and spindle cell;
ly fibrotic, pyogenic granuloma and a sclerosing retiform, spindle cell and epithelioid; retiform
hemangioma, a vascular-rich variant of fibrohistio- and polymorphous, was mentioned by Calonje et
cytic tissue response (24), as in the cases of Resnik al. (14) in their description of retiform heman-
et al. (22) and Fitzpatrick et al. (20), respectively. gioendothelioma. Table 2 briefly summarizes the
While the case of Tyring et al. (23) certainly main characteristics of these borderline vascular
matches best the original description of Weiss and entities (12, 14, 25-27). Early recognition of our

646
Cutaneous epithelioid hemangioendothelioma

neoplasm was due to a patient sensitized by the 11. Chase DR, Enzinger FM. Epithelioid sarcoma: diagnosis,
previous history of a malignant melanoma to even prognostic indicators and treatment. Am J Surg Pathol
minimal changes in her skin. 1985:9:241.
Thirdly, the diagnosis of an epithelioid cell histi- 12. Weiss SW, Enzinger FM. Epithelioid hemangioendotheli-
oma: a vascular ttinior often mistaken for a carcinoma.
ocytoma was comparatively easy, yet unusual, espe-
Cancer 1982: 50: 970.
cially when the misdiagnosis of the Spitz nevus be- 13. Zelger B, Statidacher Ch, Orchard G, Wilsonjones E,
came obvious. The likelihood of such a combina- Btirgdoi f WHC. Solitaiy and generalized variants of spin-
tion of tumors occurring in a single patient would dle cell xanthogranuloma (Progressive nodtilar histiocyto-
be less than 1 in 1 billion. This calctilation results sis). Histopatholog)' 1995: 27: 11.
from the incidence rates of the single tumors. One 14. Calonje E, Fletcher CDM, Wilson Jones E, Rosai J. Reti-
might therefore spectilate on a genetic or meta- form hemangioendothelioma. A distinctive form of low-
bolic basis favoring an "epithelioid" cell response grade angiosarcoma delineated in a series of 15 cases. Am
or cell type. Unfortunately, we did not have J Surg Pathol 1994: 18: 115.
enough material for respective cytogenetic studies 15. Smith NS. The pigmented spindle cell tumor of Reed: an
of our lesions. underdiagnoscd lesion. Semin Diagn Pathol 1987: 4: 75.
16. Enzinger FM, Weiss SW. Benign ttimors and Itimorlike le-
In summary, this case report describes a young
sions of blood vessels. In: Enzinger FM, Weiss SW, eds. Soft
lady who, over a period of 9 years, developed 3 tissue tumors (3rd edn). St. Louis, MO, USA: CV Mosby
separate cutaneous tumors of different histio- Co, 1995: 579.
genetic origin with the unifying feature of epithe- 17. Rosai J, Sumner HW, Kostianovsky M, Perez-Mesa C. Angi-
lioid differentiation. osarcoma of the skin. A clinicopathologic and fine struc-
tural study. Hum Padiol 1976: 7: 83.
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647