ECCRINE ACROSPIROMA

A Clinicopatlzologic S t u d y
CMDR BERNETT
L. JOHKSON, JR., M C , USN,” AND ELSONB. HELWG,MD

Three hundred and nineteen examples of a histologically distinctive sweat

gland tumor, the acrospiroma, are presented. The tumor occurs as a single
mass in the skin and is nodular and solid or cystic. The color varies from that
of the surrounding skin to red or reddish blue, and the covering skin may be
smooth or thickened and verrucous. The tumors are rarely painful, and a
serous or hemorrhagic fluid may drain spontaneously from them. They may
recur but rarely undergo malignant change. Clinically, the tumors lack d i a g
nostic specificity, but they should be included in the differential diagnosis of
nodular and cystic lesions of the skin. Histologically, the acrospiroma is readily
differentiated from other sweat gland tumors, but frequently it is confused with
lesions of a metastatic renal cell carcinoma and sometimes with lesions of
squamous cell carcinoma. On the basis of histologic and histochemical studies,
we believe that the cells of this tumor mimic those of the eccrine sweat duct
arid have designated the entity as eccrine acrospiroma.

T t f Z l O R S O r S l V t A T GLAND ORIClN ARC FRE- I n 27G examples, the primary lesions were
quentlj confused with tumors arising in studied, in 7 both primary and benign recur-
other atlnexal structu~esand with metastatic rent lesions, and in 31 cases only the beriign
lesions. Ex en within the group of sweat gland recurrent lesions were examined. Fike ad-
tumors, thcie is confusion as to the classifi- ditional tumors that were regarded as malig-
catiorl . I , 2 .i -i nant were studied and will be presented at a
,I unique cutaneous tumor with laige cells later date. Follow-up information was avail-
and ductal structures has been reported vari- able o n 139 cases.
ously as: clear cell papillary cartinorna,lg clear Hematoxylin and eosin (I1 and E)-stained
cell myocpithelioma,l4 clear cell hidrade- sections were examined in all cases and se-
noIna,gj 18 carcinoma of the sweat gland,26 rial sections in selected cases. Several tumors
clear cell hidradenoina and hidradenocarci- were prepared by the following methods: pe-
norna,lO superficial hidradenoma,l2 large cell riodic acid-Schiff (PAS), with and without di-
sweat gland adenoma,ll basal cell carcinoma astase digestion; hlowry’s modification of the
of sweat gland origin,l dermal sweat duct Hale stain for acid mucopoly4accharides
t u ~ n o r , ~and ’ poros) ringoma.5 I n this paper, (AMP), with and without digestion with hy-
we report a clinicopathologic study of this aluronidase; Masson’s trichrome; aldehyde-
unique tumor, which we call eccrine acro- fuchsin at p H 1.7; hfajer’s mucicarmine;
spiroma. Snook’s reticulum; oil r e d - 0 for fat; Hart’s
elastica; Heidenhain’s iron-hematoxylin; tolui-
MATERIAL AND METHODS
dine blue at pH’s 1, 1.5 and 3; Prussian blue;
and Fontana. All stains were carried o u t as
Three hundred and nineteen cases of ac- indicated i n the Manual of Histologic and
rospiroma from the files of the Armed Forces Special Staining Technics.19
Institute of Pathology were selected €or study.
RESULTS
From the Dermal Pathology Branch arid the Reg-
istry of Dermal Pathology, Armed Forces Institute of CZin,icaZ data: Clinically, the acrospiroma
Pathologv, \Vashington, D.C. 20305. appeared as a single, nodular, solid, or cystic
* Presently Assistant Chief of the Dermatology Ser-
vice, l‘hiladclphia Xaval Hospital, Philadelphia, Pa. mass that sometimes protruded from the der-
Addiess for reprints: E. B. Helwig, M.D., Chief, mis (Figs. 1, 2). T h e skin over the tumors
Dermal Pathology Branch, Armed Forces Institute of
Pathology, Washington, D.C. 20305. varied from flesh color to red to blue and
Received for publication November 1 , 1968. sometimes was thickened and verrucous. The
64 1
6-12 Alarcli 1969
CANCER VOl. 23

Albout SO^, of the patients with either a

primal y tumor or a benign recurrent tumor
noticed slow, or sometimes rapid, enlarge-
ment of the tumor. T h e association of trauma
with rapid growth of tlie tumor haa been re-
ported, but we were unable to establish such
a relationship. Trauma was reported in only
two of the lesions showing noticeable growth.
One of these patients injured the tumor by
a fall from a ladder; the other constantly
traumatiied his lesion by shaving.
In 31 (167;) of the patients the primary le-
sions oozed spontaneously, and in 7 (3.6%)
drainage occurred after trauma.
Pain on pressure occurred in 18.6z, of the
patients with primary tumors. Other symp-
toms noted were pruritis and binning (Ta-
ble 2).
Pathologic Features: T h e gross appearance
of the excised tumor varied. Generally, the
tumors appeared to be delineated from the
surrounding tissue b u t were not encapsulated.
Occasionally, the lesions were hemorrhagic,
but most were gray to tan. They were usually
firm and solid, but some contained cystic
foci filled with a serous to gelatinous, clear
Fic,. 1. Eccrine acrospiiomn, face (.\FIP Neg. 57- to hemorrhagic material. Several specimens
56921. had a sinus tract opening onto the surface of
the skin.
Histologically, the tumor was usually a cir-
blue and liliiisli-red lesions were often hem- cumscribed but not encapsulated (Figs. 3-9),
orrhagic. T h e diameter of the tumors ranged multilobular epithelial mass lying in the up-
from 0.5 to 10.5 cm, the median being 1.00 per or the middle dermis. It sometimes in-
cm. T h e tumors were present before excision volved and replaced the epidermis (Fig. 10)
for periods varying from 3 weeks to 64 years, and occasionally extended into the deeper
with a median duration of 2 years for the dermis and the subcutaneous fat (Fig. 11).
initial lesions and 1 year for recurrent le- T h e lobules were circular or irregular, and
sions. A recurrent lesion appears less likely to some had scalloped borders. I n several in-
be neglected by the patient than the initial stances, tumor nodules of identical cell type
gr0W~th. involved the epidermis and the dermis and
Tumors occurred on all areas of the body on serial section were noted to merge. T h e
(Table 1). epidermis covering most tumors was acantho-
T h e median age of the patients with pri- tic, but in a few instances it was atrophic and
mary tumors was 35 years, the range being occasionally was ulcerated.
from 3 to 93 years. T h e median age of those T h e tumor was composed of biphasic epi-
with recurrent tumors was 33 years. thelial cells supported on and surrounded by
About twice as many men as women had a variable amount of stroma. I n both phases
tumors in our series (1.94:1), but the popu- the cells were round, fusiform or polyhedral.
lation furnishing the lesions was predomi- I n one phase, the cytoplasm of the cell was
nantly male, from the military or Veterans eosinophilic (Fig. 12), solid, and finely gran-
Administration hospitals. Among the cases ular or finely vesicular, i n hematoxylin-and-
drawn from civilian contributors the pro- eosin-stained sections. T h e nucleus was oval
portion of women to men was 3:2. to round and had fine reticular chromatin
Eighty-five per cent of the patients were and a distinct nucleolus. T h e cell boundaries
Caucasian, 12% Negro, and 2% were of other were indistinct. Mitotic figures were infre-
races. quent i n the eosinophilic cell. I n tlie second
No. 3
FIG. 2. (Top) Eccrine acro-
spiroma, left knee. The tumor
is elevated, nodular and cystic
( A F I P Neg. 68-3038-1).
FIG. 3. (Bottom) Low magni-
fication of intracystic tumor
wen clinically in Fig. 2 (H and
E, x2; AFIP Neg. G8-3038-2).
phase the cell appeared as a clear cell (Fig. 13)
with little o r n o identifiable c)toplasm and
distinct cell walls. T h e nucleus was usually
small and eccentric, with an eccentrically
placed nucleolus. T h e chromatin was dense
and clumped. Mitotic figures were not seen
in this cell.
Transitions occurred from the eosinophilic
cell to the clear cell (Figs. 14 and 15). I n the
same tumor, some areas showed a gradual
transition, and others an abrupt change. T h e
number of clear cells in the tumor ranged
c i 13
from a h o s t loo(:; to less than 5%. Tumors
present for 2 years or longer had slightly
more clear cells.
I n most of the tumors the neoplastic cells
appeared in large, solid nests, sheets and lob-
ules without a particular pattern other than
their relation to ductlike structures (de-
scribed subsequently). In several tumors,
however, distinct patterns of the eosinophilic
cells were noted. One was a trabecular or
cribriform pattern in which irregular rows of
tumor cells were separated and surrounded
644 March 1969
CANCER Vol. 23

TABLE1. Anatomic Site of Acrospiromas

Primary Tumors
AFIP Literature AFIP Literature
,\natomic Site Number %
Scalp 24 8.6 14.9
Face and ear 29 10.4 14.9
Neck 10 3.6 2.1
Chest, anterior and posterior 37 13.3 17.0
Axilla 10 3.6 2.1
Abdomen 18 6.5 12.8
Back 20 7.2 8.5
Pubic area 2 0.7
Genital area 8 2.9
Buttocks 10 3.6
Inguinal area 6 2.2
Extremities
Arm 23 4 8.3 8.5
- - - -
Upper arm 4 1.4
Elbow 5 1.8
Forearm 8 2.9
Wrist 6 2.2
Hand 19 2 6.8 4.2
- - - -
Palm 4 1.4
Dorsum 3 1.1 2.1
Other 12 4.3 2.1
Leg 42 15.1 8.5
- -
Thigh 15 5.4 6.4
Knee 18 6.5
Lower 6 2.1 2.1
Ankle 3 1.1
Foot 20 3 7.2 6.4
- - -
Plantar and heel 12 2 4.3 4.3
Dorsum 3 1.1
5
Other
- -1 -
1.8
- 2.1
Total with data 278 47 100.0 100.0
___
TABLE
2. Incidence of Selected Symptoms and Signs in 226 Acrospironias
. _ ~ _ _
Number of Tumors yo of Tumors
Symptoms Primary Recurrent Primary Recurrent
Rate of growth
Slow and persistent 89 11 45.9 5.7
Rapid 14 3 7.2 1.5
Not known 33 5 17.0 2.6
Drainage
Spontaneous
Serous 25 3 12.9 1.5
Hemniorrhagic 6 1 3.1 0.5
Xssociated with trauma
Serous 2 2 1 .o 1 .o
Hemorrhagic 5 2 2.6 1 .o
Pain
Not further described 3 1.5
Spontaneous 11 1 5.7 0.5
On pressure 36 2 18.6 1.0
Due t o atmospheric pressure 1 0.5
Following exercise or motion 4 2.1
Other
Pruritis 14 1 7.2 1 .o
Burning 3 1.5
- - - -
Total with data 194 32 100.0 100.0
so. 3 ACROSPIKOMA* Johiuon a n d Helwig
ECCRINE 64 5
by a delicate stroina (Fig. 16). I n another, a
roset telike pattern with the eosinophilic cells
oriented about thin-walled blood 1 essels oc-
curred (Fig. 17). In a third arrangement,
patchy islands of large squanioid cells with
well-defined borders were present (Fig. 18).
Ductal str~ictureswere present in most tu-
mors, and they occurred in three variations
that sometimes overlapped. T h e first (Fig.
19) was a discrete, rounded duct with an in-
ner layer of cuboidal cells similar to the cells
of the inner layer of the eccrine sweat duct.
This form of ductal structure was similar to
those merging with the tumor from adjacent
existing sweat glands. Occasionally, this form
was larger and more dilated but otherwise
similar (Fig. 20). .\ cuticular border was

FIG. 6. (Top) EleIated nodular solid tumor with ex-

tensive epitlermal involvement (H and E, x9; AFIP
Seg. 54-12963).
FIG. 7. (Bottom) Recurrent nodular tumor deep in
dermis, with solid and cystic areas (H and E, X8;
AFIP Neg. 65-457).

rioted on the ductal luniinal cells (Fig. 21).

FIC. 4. (Top) Multilobular tumor incolving the epi-
dermis and extending deep into the dermis ( H and E,
xG: AFIP Neg. 68-2756).
FIG. 5 . (Bottom) Rlultilohulal intradermal tumor.
T h e base of the lesion is clrcurnscribed but not
encapsulated. Clear cells predominate (H and E,
x8; AFIP Neg. 65-459).
I n the second variation, a markedly dilated
and cystic structure with flat to cuboidal
lining cells was observed. T h e size of the
cysts varied within the same tumor, and oc-
casionally only a single, large intracystic tu-
mor was present (Figs. 3, 22). T h e lumens
contained an amorphous, eosinophilic, ho-
mogeneous material, and the larger lumens
in a few instances contained hemorrhagic
foci. Marked hemorrhage probably ac-
counted for the occasional rapid growth of a
lesion or for the seemingly sudden appear-
646 CANCERMarch 1969 Vol. 23

whorls without a lumen occurred in 11% of

the tumors and with a lumen in 7%.
Eccrine sweat glands were closely associ-
ated with 70y0 of the primary tumors, and
sweat ducts merged with the tumors in 10.6%
of the lesions. Frequently, the eccrine ducts
near the tumor showed proliferation of cells
similar to the cells seen within the tumor.
Occasional sweat glands were cystic and con-
tained a homogeneous material similar to
that seen within the ductal structures of the
tumors. T h e other adnexal structures ap-
peared to be normal except for deviation of
position caused by the growth of the tumor.
No connection between pilosebaceous struc-
tures and the tumor could be demonstrated.
The stroma of the tumor varied from deli-
cate septa to well-vascularized, often collage-
nous and sometimes hyalinized broad bands
of connective tissue (Fig. 24). This stroma
was contiguous with the surrounding stroma,
causing a scalloped appearance of the tumor
lobules. T h e amount of fibrous tissue was
greater in recurrent than in primary tumors.
Little or no inflammatory reaction was in-
cited in the tumors. When present, it con-
sisted of a scattering of predominantly peri-
vascular lymphocytes or of plasma cells about
the tumor lobules. I n the ulcerated lesions
the infiltrate included leukocytes.
FIG. 8. (-1op) Poorly circurnscribed intradermal tu- Melanin pigment, mostly in macrophages,
mor with irrcrrular lobules deep in dermis. Scattered
"
ductal stiuctures are present (H and E, ~ 1 3 % ;
was present in 2.5% of the primary tumors.
AFIP Neg. 54-11816). Focal calcification was found in S.lyoof the
FIG.9. (Bottom) Verruciform tumor with epidermal primary tumors-in the stroma and within
and derp dei ma1 involvement and maihed 'Irregular
extcnsions latcrally and deeply (H and E, x71/,; the epithelial masses that included the lum-
AEIP Neg. 54-12968). inal spaces.
Histochernical findings: Glycogen repre-
sented by PAS-positive diastase-digestible ma-
ance of a tumor after minor trauma. I n the terial was present to some extent in most
third form, there were whorls of two or three of the cells of the tumor but in greater quan-
concentric layers of squamous cells, usually tities in the clear cells (Figs. 25, 26). PAS-
around a lumen (Fig. 23). For the most part, positive diastase-resistant material was ob-
the squamous whorls were found in the tu- served within the luminal spaces, on the
mors with fewer clear cells and in most in- surface of the clear cells, and in granular form
stances were located at the periphery of the in the cells lining the ductal structures. T h e
lobules. T h e ducts within the squamous PAS-positive diastase-resistant material
whorls were eccentric and ellipsoidal, and the stained with the colloidal iron stain but was
lining cells sometimes contained basopliilic resistant to digestion by hyaluronidase and
granules. Lumens were not always identified responded negatively to the aldehyde-fuchsin
in the squamous whorls, but the circular ar- stain, probably indicating the presence of a
rangement of cells was always a feature of mucosubstance of the sialic acid type.8 Col-
this third type of ductal structure. T h e squa- loidal iron-positive, hyaluronidase-resistant
mow whorls resemble the intraepidermal ,granular material was also seen in the inter-
portion of the eccrine sweat duct, the acro- cellular spaces of the tumor. This material
syringium, and may represent the tumor's at- was not PAS positive. T h e stroma contained
tempt to form this s t r u c t ~ r e . ~Squamous
3 variable amounts of hyaluronic acid. Sections
so. 3

Fit;. 10. ('z'opj Partial 1-cplacc-

incilt o f ttrc epidermis by t i i i i i o i ~
c t . 1 1 ~ ( H ;11lt1 I:, X265; .lFIl'
s"6. ti.;-1 065).

FIG.11. iI$ottomj Kcsts o f

cobiriophilic arid clear t u m o r
cells cutcntling into deeprr 31-ca
o f tlcrrnis at base of lesion (H
;mtl F., ~ 2 2 0 ; AFIP Ncg. 65-
1O M ) .

stained with aldehyde-fuchsin at pH 1.7 gave
variable results. When positive, the material
was found in granular form in the intercel-
lular spaces and in the stroma. Specimens
stained with toluidine blue at pH 1.5 and 3.5
were negative for metachromasia, indicating
that there were no strongly acid rnucopoly-
saccharides present. Prussian blue and acid-
fast stains25 were consistently negative. Speci-
mens stained with the iron-hematoxylin
method did not reveal myofibrils in the tu-
mor cells. Mucicarmine- and colloidal iron-
positive material was noted in several tumors
(Fig. 27).5 I t was focal in distribution and
frequent in the cells lining the larger ductal
spaces. Lennoxl3 also noted mucin in sweat
gland tumors.
Reticular tissue was abundant in the inter-
nal stroma of the tumor as well as condensed
about the tumor lobules. Elastic tissue in the
stroma was not increased but sometimes ap-
peared to be compact about the tumor, prob-
ably from compression. T h e tumor cells did
not contain fat in oil-red-0 preparations.
648 March 1969
CANCER
T h e histocliemical reactions in the normal-
appearing etcrine sweat glaiicls in the dermis
were similar to those noted in the tumor
(Table 3).
Rm-tirmit tumors: Thirty-eight of the 319
tumors were recurrent. I n 7, both the pri-
mary and the recurrent tumors were avail-
able for study; and in 31, only the recurrent
lesion.
T h e recurrent and the primary tumors
were essentially similar, histologically and
Vol. 23
FIG. 12. (Top) Eosinophilic
cells. T h e cytoplasm is granu-
lar, and the cell outlines are
indistinct (H and E, x600; AFIP
Scg. 68-2758).
FIG. 13. (Bottom) Clear cells
showing scanty cytoplasm and
eccentric nuclcus (H and E,
x600, AFIP Neg. 65-1061).
lii~tocliemically.T h e recurlent tumor was us-
ually located deeper in the dermis than was
the primary, as would be expected, since in-
adequate excision was probably the major
cause of recurrence. Recurrent tumors were
neither more aggressive nor more atypical
than the primary tumors and did not differ
in cell type or in the frequency of mitotic
figures. I n some instances, the recurrent tu-
mors were more characteristic than the pri-
mary lesion.
No. 3 ECCRINE -
ACROSPIROMAJohnsoii aizd Hcliciig liI!)

WITH REPORIS
COMPARISON IN THE
LITERATURE
Approximately 74 tumors of sweat gland
origin with clear cells have been reported in
the American literature.
Liu15 in 1949 reported on the histogenesis
o f clear cell papillary carcinoma occurring in
2 male and 2 female patients. RIetastatic le-
sions did not occur. Structures Liu iclentified
as immature hair shafts within the tumor are
probably ductal structures within ~i Iiorl5 of
squamous cells. This type of ductal struc-
ture is thought to represent a mimicry of the
iiitraepidermal portion ol the sweat duct.
Liu theorized that, on the basis of the abun-
dance of glycogen in the tumor and in the
hair mot sheath, these tumors weie of hair
follicle origin. Glycogen also occurs in sweat
glands2n 2 2 and sweat gland tuinots; this ma-
terial was present in the tiiniors of oiir re-
port.
Leier and Castleman14 in 1952 reported on
10 cases they called clear cell m\oepitheli-

I ic 14. (Top) Area ot tumm

wltli inostly cle'ir cclls arid o n l ~
,I frv eosiuophilic cell5 iH a n d
k., x l l i : A r I P hcg. 54-16619).

1.s. 15. (12ottum) Area of t u -

mor with mostly foci ot cosino-
pllilic cclls a n d clear cells (H
ant1 E, X185; AFIP S C ~68- .
60X).
 March 196'3
CANCER
oma. More of their patients were male than
female. None of their tumors recurred or
metastasized. No lesions were reported on the
palms or the soles, in contrast with the dis-
tribution in our group. Three of their tu-
mors showed an epidermal connection, but
they believed that the tumors arose from the
myoepithelial cells of the sweat gland. We
could not confirm this origin in our cases.
Horny cells (squamous whorls), glandular
structures and cysts were also noted by these
investigators. They concluded that the cysts
were the result of a degenerative process, but
Vol. 23
FIG. 16. (Top) Cribriform pat-
tern of eosinophilic cells. Nests
of cells are suirounded and
separated b y a delicate stroma
(H anti E, X l G ; AFIP Neg.
68-6089).
FIG. 17. (Bottom) Rosette-like
pattern of eosinophilic cells.
Cells appear to be oriented
about a thin-walled blood ves-
sel (H and E, ~ 4 4 0 ;AFIP Neg.
68-6083).
in our opinion the cysts represent dilated
ductlike structures. Glycogen was noted in
their tumors.
Stout and Cooley26 reported on 11 tumors,
6 of which were sweat gland carcinomas, but
from their descriptions and photographs
these did not resemble the acrospiroma or
other reported clear cell tumors of the sweat
gland. Of their remaining 5 tumors, only 1
(with clear cells) fits our criteria. This tumor
attained a very large size and led to amputa-
tion of an extremity. A 9-year follow-up of
this patient showed n o evidence of meta-
static spread. These authors called their tu-
mors “squamous cell epitheliomas of sweat
glands with clear cells.” They did not specu-
late as to their histogenesis. No histochemical
procedures were done.
Keasbey and Hadley” reported the largest
series of clear cell tumors. I n their review of
235 sweat gland tumors, 50 were of the clear
cell variety, and of this group 3 metastasized
and 4 recurred after surgical excision. Two
of the latter four lesions recurred locally and
were uncontrollable and resistant to massive
x-ray. T h e 38 benign tumors reported by
these authors are apparently similar to those
studied by us. ‘CVithin their group, they noted
some histologically atypical tumors, but on
subsequent study and follou~-upinformation,
evidence of malignancy was not established.
Their group of malignant tiimois grew re-
lentlessly and attained large size. One tumor
metastasized to nodes, bone a i d pleura.
These metastatic lesions resembled the pri-
niary lesions in general but did not tend to
develop tubules.
Helwig,s in discussing the et crine spiiade-
noma tlestrilxd 11) Kei sting m c 1 hiniself,11

FIG. 18. (Top) Squamoid pat-

tern. Patchy areas of squamoid
cells are present (H and E, X200;
4FIP Ncg. 68-7983).

FIG.19. (Bottom) Acrospiroma

with discrete rounded duct with
cells resembling those of an ec-
crine swcat gland (H and E,
xGOO; AFIP Neg. 65-1060).
 March 1969
CAKCER
noted that they also recognized an aclenoma
of sweat gland of the “large cell type” and
regarded it as originating from an eccrine
sweat duct. T h e cell of this tumor was much
larger than the cell of the eccrine spirade-
noma. Squamous differentiation, ductlike
structures and clear cells with glycogen in the
cytoplasm were observed.
Lundl* reported on 2 female patients with
benign “clear cell hidradenoma.” These le-
sions recurred locally and were identical with
those in our report.
A report of clear cell tumor by Kerstinglo
Val. 23
FIG. 20. (Top) Elongated, di-
lated and irregular ductal struc-
tures within thc tumor ( H and
E, x40; AFIP Neg. 651149).
FIG.21. (Bottom) Dilated duc-
tal structure with cuticular bor-
der (H and E, xGO0; AFIP Neg.
65-1058).
recorded under the title of “Clear Cell Hi-
clradenoma and Hidradenocarcinoma” de-
scribed 2 patients with 5 tumors. He con-
sidered that these tumors were derived from
sweat glands and could find no evidence for
myoepithelial cell or hair origin. Through
his review of the literature, he recognized the
existence of a malignant form that can me-
tastasize, but no malignant tumors were in-
cluded in his series. His cases were similar to
those in this report.
T h e eccrine poroma described by Pinkus
et al.24 in all probability represents a sub-
so.3
ECCRINEACROSPIROMAJolriisoii aizcl Helzuig
group of the eccrine acrospiroma. I n the orig-
inal study of the poromx, 5 cases were pre-
sentcd. All the tumors were located on the
plantar surface of the foot, and histologically
all were within or connected to the epider-
mis. I-Iistochemically, the tumors contained
abundant gljc-ogen. O u r examples of acro-
spiroma occurring on the plantar surface of
the feet and the palms of the hands appeared
to be similar to the lesions of eccrine poroma
described by Pinkus.
Ilashimoto, DiBella and Levi4 reported 2
cases of a clear cell tumor (“clear cell hid-
radenoma”) on which they performed his-
Fir.. 22. (Top) Intracystic t u
inor with cuticular border (H
a n d E, x95; AFIP Neg. 65-1062).
FIG.23. (Bottom) Small lumens
within whorls of squamous cells
forming a ductal structure (H
a n d E, ~ 6 0 0 ;AFIP Neg. 65-
1057).
-
653
tochemical and enz) matic studies. They now
beliexe that this tumor is of eccrine origin
and shows different histologic variations, de-
pending upon the level of involvement. Find-
ings from enzymatic studies were similar to
those of the eccrine sweat gland and duct.
T h e histologic observations in their report
are similar to ours.
Tvinkelmann and i\IcLeod2i presented 3
cases of “dermal duct tumors.” These tumors
are histologically and histochemically similar
to the acrospiroma, and we believe that theirs
is not a separate entity but just one of the
variations of the acrospiroma.
6.54 M a r c h 1969
CANCER
T h e acrospiroma is an eccrine ductal tu-
mor that may mimic any segment or seg-
ments of the duct. T h e predominant pattern
of the tumor will depend on the area of ori-
gin and the potentiality of the cells in that
area of the duct, or of the cells from which
the duct is derived.
DISCUSSION
T h e eccrine acrospiroma lacks specific di-
agnostic features clinically, but the structure
and the cells are histologically distinctive. It
is usually a nonencapsulated multilobular
tumor in the dermis that in many instances
also involves the epidermis and occasionally
Vol. 23
FIG.24. (Top) Tumor showing
hyalinized stroma (H and E,
X185; AFIP Neg. 68-6082).
FIG. 25. (Bottom) T h e tumor
cells are strongly PAS positive,
and the material (glycogen) is
digested with diastase (PAS, ~ 6 ;
AFII’ Neg. 684093).
the subdermis. One phase of the biphasic cell
pattern comprising the tumor appears as a
clear cell that is oval, fusiform or polygonal,
with scant to absent cytoplasm. T h e nucleus
of the clear cell is small, eccentric and con-
tains a nucleolus. I n the other phase the cell
is of similar shape but has an eosinophilic,
solid and finely granular or finely vesicular
cytoplasm. T h e nucleus of the eosinophilic
cell is vesicular and has a reticular chroma-
tin pattern and a prominent nucleolus. T h e
predominance of either type of cell within
a given tumor is variable. T h e eosinophilic
type of cell tends to predominate in the tu-
mor of less than 2 years’ duration, and the
So. 3 ECCRINE
XCKOSPIROZ~A
* Joliiisoii
clear cell in tumors of more than 2 years' du-
ration. Ductal structures may be seen within
the tumor in three forms: (1) resembling ec-
cline sweat ducts; ( 2 ) large, dilated and cys-
tic; and (3) occurring within whorls of squa-
mom cells.
Tlie majoricy of the acrospiromas are be-
nign. Malignant forms occur and have the
same general configuration and pattern as do
tlie benign tumors, but they also present ple-
omorphic cellular features that we belie\ e
are recogriiirable and distinctive.
Tlie acrosprroma has been ariousl\ ie-
ported as arising from the epithelium of the
niitl Hcliuig G.55
hair follicles or from the ni) oepithelial cell.
IVe believe that tlie histologic ant1 tlic his-
tochemical changes noted in the t uniors i n
our study support our peniire that [lie tu-
mor arises from or mimics the stiricture of
the eccrine sweat duct. Tlie ductal wuctures
present in the tumor resemble the intraepi-
dermal zone,17. 2 2 , "3 the dermal, or both zones
of the eccrine sweat duct. T h e cell t)Iies of
the tumor and of d i e etcrine hlve'it t l u c t are
similar. I n sevei a1 instances. a cuticuhr border
'rva~seen on the ductal lurriinal cells of the
.\
tumor. similar border occ irr5 on the luniinal
tells of the ecciine cluct.20 l\'itliin the luniinal

FIG. 26. (Top) Higher power

vicw of PAS-positive material
(glycogm) in the tunior cells
shown in Fig. 25. T h e clear cells
noted i n hcrnntosylin and eosin
pcparations show the greatest
concentralion of glycogen (P2\S,
~'350;AFII' Neg. 68-6096).

FIG. 27. (Bottom) Scattered

cell9 rontaining mucus among
tells lining a cystic ductlike
\pace (rnnricarmine, ~ 2 1 0 AFIP
;
Ueg. 684090).
656 March 1969
CANCER
3 . Histochemical Findings of the
TABLE
Eccrine Sweat Gland and Acrospiroma
Xcrospiroma Eccrinc s a e a t gland
-
PAS
No digestion +++t ti
Digestion* f rt
AMP
No digestion
Digestion+
++
++ ++
++
Aldehyde-fuchsin
pH 1.7 F F
illucicarniine =I= rt
Toluidine blue
pH 1.5 - -
pH 3 - =I=
~ this type of tumor, e.g., peripheral palisading
* Digestion with diastase.
t Digestion with hyaluronidase.
spaces a homogeneous or secretory material is
seen that is similar histochemically and mi-
croscopically to that noted in the eccrine sweat
ducts in tlie adjacent areas. Evidence for sweat
duct secretory activity has been previously
docuniented.1~ On serial sectioning, eccrine
sweat ducts from glands in the area of the
tumor merged with the tumor; other adnexal
structures showed n o connection arid were
pushed aside by the tumor. T h e acrospiroma
never shows peripheral palisading of the cells
or connection with follicular structures. In-
traepidermal acrospiroma has been noted and
occurs at the site of the epidermal portion oE
the eccrine sweat duct, or acrosyringium. Evi-
dence to support origin from other deriva-
tions, sutli as the presence of iron in the lu-
minal cell7 (apocrine origin), fat (sebaceous
origin) and niyofibrils (myoepithelial cell ori-
gin) were not found in any of the tumors
studied. T h e histocl~emicalreactions oE the
acrospiroma are similar to those of the ec-
crine sweat gland.
As has been shown, the acrospiroma pre-
sents distinctive and diagnostic histologic
features. T h e clinical identification of the tu-
mor is difficult but should be considered with
other cystic or nodular lesions of the skin.
Hemorrhagic acrospiromas can simulate blue
nevi and melanomas, but these can easily be
differentiated histologically. T h e tumors can
be confused with lymphangiomas and he-
mangiomas when they spontaneously dis-
charge serous or hemorrhagic fluid, but the
duration and the clinical and histologic ap-
pearance of the lesions also easily distinguish
the conditions.
Acrospiroma is occasionally misinterpreted
as a squamous cell carcinoma, especially i n
Vol. 23
those examples in which the cytoplasm of the
cells is eosinophilic and the tumor also in-
volves the epidermis. I n addition, some tu-
mors show squamoid cells, b u t these tumors
in most instances include areas in which the
cells are arranged in whorls about a lumen or
potential lumen. An irregular pattern of ple-
oinorphic squamous cells, as seen in squa-
mous cell carcinoma, is never present.
Histologically, the acrospiroma is most of-
ten confused with other appendageal tumors
of the skin. Since this tumor is not of follicu-
lar origin, it presents none of the features of
of cells, basaloid proliferation and pilar kera-
tin formation. Other tumors of sweat gland
origin may present a more difficult task in
differentiation. T h e eccrine cylindroma is
multilobular with small, discrete cylinders
and cords of basophilic epithelial cells, often
surrounded by a dense eosinophilic hyaline
membrane, especially in the older tumoi 7.3
Mjalin may also be present within the cords
of tumor cells. Within the lobules are small
ductlike structures that may o r may not con-
tain an eosinophilic material. This tumor
lacks the characteristic clear cells and tlie pat-
tertis of ductal structures seen in acrospir-
oma. T h e eccrine spiradenoma is usually a
single dermal nodule with two distinct cell
i\pes. Both types of cells are smaller than the
tell< of the acrospiroma and both have scanty,
pale cytoplasm.11 They resemble the cells of
tlie secretory segment of the eccrine sweat
gland. Large clear cells and well-defined ductal
slructures are not seen.
T h e acrospiroma and the chondroid syrin-
gorna are similar i n that ductal structures are
seen i n both.? I n the chondroid syringoma
tlie chondroid matrix is the main distinguish-
ing feature; histochemically, it is rich in sul-
fated acid mucopolysaccharides. This type of
matrix is never seen in the acrospiroma.
It is most important to distinguish acro-
spiroma from metastatic renal cell carcinoma.2
Clinically, both lesions present as a nodular
growth, but the metastatic renal cell carci-
noma is more frequently hemorrhagic. Histo-
logically, the lesions are confusingly similar.
T h e metastatic renal cell carcinoma is usually
unilobular, and the acrospiroma is usually
multilobular. I n the renal cell carcinoma
marked vascularity, particularly hemorrhage,
is likely to be present throughout the tumor
and in the glandlike spaces, but this is not a
characteristic of the acrospiroma. T h e distinc-
Ao. 3 ECCRIXEACROSPIROMA Johnson and Helwig
tive ductal structures in the acrospiroma are
not seen in the metastatic renal cell carci-
noma. T h e renal cell carcinoma is often com-
posed of clear cells arranged in a glandular
and papillary pattern, whereas the acrospiroma
almost a1wa)s shows a mixture of two cell
types without a papillary pattern. Histochem-
i d l y , the metastatic renal cell carcinoma fre-
quently contains hemosiderin and fat but
never mucin. ‘The acrospiroma rarely contains
hemosiderin, never contains fat and may con-
tain mucin. Both lesions may contain glycogen.
Follow-up data were received for 120
(42.4y0)of the 283 patients for whom sections
of primary benign acrospiromas were sub-
mitted. One hundred and seven, or about
90% of the patients with follow-up data, were
alive at last contact; 73 of these 107 were
alive and well, 23 were living but had other
complaints, and 11 patients had recurrences
oE their acrospiromas. Thirteen, or 10.8%, of
the 120 patients had died, 8 from other
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657
causes. No autopsy information was avail-
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T h e tumor is designated an eccrine acro-
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