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Coll. Antropol. 38 (2014) 2: 763–766
Case report
Leiomyosarcoma of the Spermatic Cord with Scalp
Metastasis: Case Report and Literature Review
[oip [oipi
1
, Majda Vu~i}
2,3
, Monika Ulamec
2,3
, Davor Tomas
2,3
, Bo`o Kru{lin
2,3
and Borislav Spaji}
1,3
1
University of Zagreb, University Hospital Centre »Sestre Milosrdnice«, Department of Urology, Zagreb, Croatia
2
University of Zagreb, University Hospital Centre »Sestre Milosrdnice«, Department of Pathology, Zagreb, Croatia
3
University of Zagreb, School of Medicine, Zagreb, Croatia
ABSTRACT
Herein we present 82-year-old man with leiomyosarcoma arising from the spermatic cord with scalp metastasis, five
years after primary surgical treatment. Complete surgical excision is required in such cases, as well as precise evaluation
of further therapy. Paratesticular leiomyosarcoma is a rare entity, malignant mesenchimal tumor of smooth muscle dif-
ferentiation. Although leiomyosarcomas of different localizations have well-known metastatic potential, cutaneous meta-
stases are extremely rare with only 16 cases described in the literature. To our knowledge there are no reported cases of
the paratesticular leiomyosarcoma metastatic to the skin. This article reviews the literature regarding paratesticular
leiomyosarcoma presentation, diagnosis and treatment.
Key words: spermatic cord tumors, leiomyosarcoma, metastases, skin
Introduction
Primary spermatic cord and paratesticular tumors
are rare yet clinically significant urologic lesions that af-
fect patients of all ages
1
. Their true incidence has actu-
ally never been established. Most tumors are benign,
usually lipomas. Approximately 25% of primary sper-
matic cord tumors are sarcomas originating from the
mesoderm layer of the embryo (Wolffian duct)
2
. Among
the paratesticular sarcomas, leiomyosarcoma is the sec-
ond most common sarcoma in frequency proceeded by
liposarcoma. Tumors in this region generally present as
asymptomatic, slow growing, firm and palpable para-
testicular masses. Radical inguinal orchiectomy and high
ligation of the cord is the standard primary surgical pro-
cedure. The extent of surrounding soft tissue excision is
required and the role of adjuvant radiotherapy remains
controversial. There is not much data in the literature
about this issue. Although leiomyosarcoma has well-
-known metastatic potential, skin metastases are re-
markably uncommon
3
. We report a leiomyosarcoma of
the spermatic cord in an 82-year-old man who developed
scalp cutaneous metastases five years after diagnosis of
primary tumor.
Case Report
82-year-old man presented with 9-month history of
enlarged inguinal region. Patient complained on inter-
mittent abdominal pain. He denied any history of trauma
and any lower urinary tract symptoms, 10 years ago pa-
tient operated on both sided groin hernias. Ultrasonogra-
phic scan revealed normal left testicle and extrates-
ticular, well circumscribed large mass of heterogeneous
echogenicity with prominent vascularity in the left groin
region. Computed tomography of abdomen and pelvis
with intravenous contrast revealed oval, solid, contrast
enhacement tumor mass with central calcifications (Fig-
ure 1a). Retroperitoneal and ingvinal lymph nodes were
not enlarged. Levels of a-fetoprotein and b human chori-
onic gonadotropin were within normal limits, as were re-
sults of routine blood investigations. Plain chest X-ray
was normal.
The physical examination of the scrotum and ingui-
num showed a large, oval, non tender, firm mass mea-
sured up to 10 cm in diametar. A radical left orhiedec-
tomy with high cord ligation and tumor extirpation was
performed. On gross examination the resected specimen
showed lobulated, oval tumor mass attached to sper
-
763
Received for publication January 7, 2014
matic cord that measured 12.5x8.5x6.5 cm (Figure 1b).
Cut surface of tumor was gray, firm and whorled. The tu-
mor mass was completely located outside the tunica
albuginea, not infiltrative to testis or epididymis. Micro-
scopic examination revealed a tumor composed of slen-
der, elongated tumor cells arranged in interlacing fasci-
cles. Tumor cells showed marked nuclear pleomorphism
and up to 10 mitoses per 10-high power fields, without
necrosis (Figure 1c). The testis and epididymis were free
of tumor. Immunohistochemistry revealed positive stain-
ing of tumor cells for smooth muscle actin (SMA) (Figure
1d) and negative for S100. The histology and immuno-
histochemical findings were diagnostic of leiomyosar-
coma. Postoperative course was uneventful and the pa-
tient received no adjuvant therapy. During regular follow
up, 5 years after initial presentation, clinical examina-
tion revealed nodule measuring up to 2 cm in diameter,
on the skull skin. Patient underwent excision biopsy and
microscopic examination showed a well circumscribed
dermal nodule, on high-power it was cellular tumor com-
posed of atypical spindled cells with a woven, lobulated
histological pattern and a high mitotic ratio. Further
immunohistochemical staining was positive for SMA and
confirmed the diagnosis of metastatic leiomyosarcoma.
One month later staging computer tomography of the
chest and abdomen showed multiple lung metastases.
Regarding patient’s age he didn’t receive any adjacent
chemo or radiotherapy and after 3 months is still alive.
Discussion
Leiomyosarcoma is a rare testicular and paratesti
-
cular neoplasm, with about 110 cases reported
4
. There
have been other reports of leiomyosarcoma in the genito-
urinary system, including urethra, renal capsule and
epididymis. These tumors have a higher incidence after
the sixth decade of life
4
. Although the exact origin is un-
known, it is speculated that they most likely originate
from the smooth muscle of different cord structure areas,
such as the vas deferens, canal wall, blood vessels and
cremasteric muscle
5
. Some authors suggested that leio-
myosarcomas of spermatic cord arise as a result of malig-
nant degeneration from previously existing benign leio-
myomatous tumors
5,6
. The role of hormonal stimulation
has also been linked to leiomyosarcoma carcinogenesis.
Interestingly, some spermatic cord leiomyosarcomas
have been found to produce b human chorionic gonado-
tropin causing a paraneoplastic syndrome
6,7
.
Most spermatic cord malignancies originate just be-
low the external inguinal ring and grow as scrotal rather
than inguinal masses. Since they may appear as scrotal
masses, the preoperative clinical diagnosis may be chal-
lenging given that it may be difficult to determine the exact
location of the tumor. Paratesticular sarcomas usually
have a heterogeneous pattern and are seen as hyper-
vascular tumors on Doppler sonography
8
. Computed to-
mography scan and magnetic resonance imaging may be
helpful in refining tumor location, morphologic features,
and tissue characteristics, as well as determining the ex-
tent of the mass into the neighboring tissues. Computed
tomography is helpful in distinguishing a primary sper
-
matic cord tumor from a retroperitoneal process extend
-
ing into the scrotum
8–10
.
Leiomyosarcoma of the cord structures are known to
spread by local invasion, lymphatic dissemination and
[. [oipi et al.: Leiomyosarcoma of the Spermatic Cord, Coll. Antropol. 38 (2014) 2: 763–766
764
Fig. 1. a) CT scan, paratesticular, relatively well circumscribed tumor, up to 9 cm. b) Macroscopic view of the funicular
tumor. c) Microscopically, sheets and cords of polymorphous spindle shaped cells, (100xHE). d) Immunohistochemically
tumor cells are positive for smooth muscule actin (40xSMA).
hematogenous metastases. The route of lymphatic spread
involves the external iliac, hypogastric, common iliac and
paraaortal nodes. Lung is the most common metastatic
site and is almost always involved in metastatic disease.
The curative treatment of choice is radical orchiectomy
with high cord ligation and wide excision of surrounding
soft tissue structures within the inguinal canal. Patients
with inadequately resected tumor should undergo re-
operative procedure for wide inguinal re-excision. It has
been estimated that approximately 50% of these tumors
recur loco-regionally following definitive surgery. Patho-
logic features that convey a higher risk of local recur-
rence include large tumor size, inguinal location, narrow
or positive margins and prior intralesional surgery. Loco-
-regional relapse may occur in the cord, scrotum, or adja-
cent pelvis, with or without involvement of the regional
lymph nodes
1–6
.
Retroperitoneal lymph node dissection is not regu-
larly recommended for patients with spermatic cord leio-
myosarcoma. Retroperitoneal lymphadenectomy is rec-
ommended when there is preoperative evidence of retro-
peritoneal lymph node metastases. Adjuvant treatments,
such as radiotherapy and chemotherapy, have shown lit-
tle efficacy, except in the management of patients with lo-
cal recurrence
10
. Some data suggest that patients who
underwent postsurgical radiotherapy have better prog-
nosis
11
. Our patient underwent radical orchidectomy with
high cord ligation, no further treatment was given. Long-
-term follow up is recommended due to high recurrence
rates. Patients may also present with signs and symp-
toms of distant metastases 15 years or more after first
resection
4,10,11
.
Sarcoma metastases to the skin are rare, in published
literature series reported prevalence was between 1%
and 2.6%. Skin metastases are usually late events in sar-
coma clinical progression and carry a poor prognosis.
Lung metastases may occur in patient either simulta-
neously or within a short period after skin metastases.
The majority of sarcoma patients developed skin me-
tastases distant to the site of primary origin which is in
contrast to many types of carcinomas where regional
skin metastases are common presentation. The scalp is
frequently involved anatomic site, perhaps because of
the highly vascular nature and immobility of this re
-
gion
11
. In the English-spoken literature only 16 cases of
leiomyosarcoma metastatic to the skin have been repor-
ted
3
. The uterus is the overall most common site of origin
metastasizing to the skin. Moreover, primary tumors of
the genitourinary system account for more than half of
the cases. Other sites of primary tumors include gastro-
intestinal tract, heart, breast and the retroperitoneum.
Only one patient in the reported series developed a sin-
gle, isolated skin metastasis. The mean reported interval
between the time of diagnosis of primary leiomyosar-
coma and the development of cutaneous metastases is 3
years
11–13
. To our knowledge there are no reported cases
of the paratesticular leiomyosarcoma metastatic to the
skin.
Prognosis is highly variable, more recently a five-year
survival of 50–80% has been reported, possibly reflecting
the advances in diagnosis and management of these
tumors
13–15
. The wide range in the five-year survival rate
might be due to the variations in tumor stage and grade
at the time of diagnosis as well as the diversity of thera-
pies involved. A recent review disclosed a mean survival
of 138 months for patients with leiomyosarcoma, but the
mean survival after the occurrence of cutaneous meta-
stases in the present series was just 10 months
16
.
Conclusion
Although leiomyosarcoma is a rare neoplasm, it can
present as an extremely large primary tumor. It seems to
have low metastatic potential but metastases may be
seen many years after the primary tumor. Management
is largely surgical. The need for adjuvant therapy is in
question; reports of spermatic cord leiomyosarcoma pro-
vide limited data on postoperative recurrence.
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[. [oipi et al.: Leiomyosarcoma of the Spermatic Cord, Coll. Antropol. 38 (2014) 2: 763–766
765
B. Spaji}
University of Zagreb, University Hospital Centre »Sestre Milosrdnice«, Department of Urology, Vinogradska 29,
10000 Zagreb, Croatia
e-mail: borislav.spajic@kbcsm.hr
LEIOMIOSARKOM SJEMENOG SNOPA S METASTAZOM U KO@I LUBANJE:
PRIKAZ SLU^AJA I PREGLED LITERATURE
SA@ETAK
Leiomiosarkom paratestikularnog tkiva je veoma rijedak maligni mezenhimalni tumor koji pokazuje glatkomi{i}nu
diferencijaciju. Iako leiomiosarkomi razli~itih sijela imaju dobro poznatu sposobnost metastaziranja, ko`ne metastaze
su izrazito rijetke, sa samo 16 opisanih slu~ajeva u literaturi. Do sada nije opisan nijedan slu~aj metastaze leiomio-
sarkoma sjemenog snopa s metastazama u ko`u. Donosimo opis 82-godi{njeg bolesnika s bezbolnom masom u lijevom
hemiskrotumu, leiomiosarkom sjemenog snopa s metastatskom bolesti koja se manifestirala nakon pet godina kao
ko`ni tumor te zatim i metastazama u plu}a. U takvim slu~ajevima potrebno je potpuno kirur{ko odstranjenje tumor-
ske mase te precizna procjena odre|ivanja daljnje terapije. U ovom radu donosimo prikaz literaturnih navoda koji se
bave opisanom problematikom prezentacije, dijagnoze i lije~enja paratestikularnih leiomiosarkoma.
[. [oipi et al.: Leiomyosarcoma of the Spermatic Cord, Coll. Antropol. 38 (2014) 2: 763–766
766
