International Urology and Nephrology 36: 73–75, 2004.

© 2004 Kluwer Academic Publishers. Printed in the Netherlands.

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A case of testicular rhabdomyosarcoma

M. Erkan Erbay1 , Fatih Tarhan1 , Nagehan Özdemir Barışık2 & Uğur Kuyumcuoğlu1
1 Dr.
Lütfi Kırdar Training and Research Hospital Urology Clinics, İstanbul, Turkey; 2 Dr. Lütfi Kırdar Training and
Research Hospital Pathology Laboratories, İstanbul, Turkey

Abstract. Pure testicular rhabdomyosarcoma is a very rare tumor and 7 cases have been reported in literature. A
20-year-old male patient presented with a painless right testicular swelling who underwent inguinal orchiectomy
with suspicion of testicular malignancy. The case was regarded as an embryonal rhabdomyosarcoma according to
histopathologic and immunohistochemical findings. The case is presented and the relevant literature is reviewed
and discussed.

Key words: Rhabdomyosarcoma, Sarcoma, Testis

Introduction measuring 6 × 5, 5 × 5 cm. Tunica albuginea and

The majority of primary testicular tumors are germ
cell origin. Other rare primer testicular tumors include
lymphoma, gonadal stromal tumors, and sarcomas.
Scrotal rhabdomyosarcomas primarily origin from
paratesticular tissues and occur predominantly in
children and adolescent [1]. Primary intratesticular
sarcoma that is neither associated with germ cell ele-
ments nor retroperitoneal metastases is a unique subset
of intrascrotal sarcoma. Limited experience exists
about its natural history due to its rarity, and defini-
tive treatment recommendations including the role of
retroperitoneal lymphadenectomy are controversial.
Case report
A 20-year-old male patient presented with a painless
right testicular swelling for one month. Medical his-
tory was unremarkable. Physical examination revealed
a diffusely enlarged, non-tender, and hard right testis.
Sonogram showed a heterogeneous, 48 × 44 mm
intratesticular mass which was located in lower pole of
testis. Chest radiography, abdominal, and chest com-
puted tomography, alpha-fetoprotein, and B-human
chorionic gonadotropin were in normal limits. Right
inguinal orchiectomy was performed.
Macroscopic examination of testis showed that it
was enlarged and replaced by a whitish firm tumor,
rete testis were invaded by tumor. Microscopic exam-
ination of testis revealed a highly cellular tumor com-
posed of pleomorphic cells with round or oval shaped
hyperchromatic nuclei and clear cytoplasm, and addi-
tionally rhabdoid cells, and rhabdomyoblasts (Figure
1A, B, C).
The tumor was positive for smooth muscle actin,
vimentin, S-100 protein and muscle-specific actin
(Neomarkers USA), and negative for cytokeratine
(Neomarkers USA). Also tumor showed nonspecific
staining for placental alkaline phosphatase and alpha
fetoprotein (Neomarkers USA). The case regarded as
an embryonal rhabdomyosarcoma according to histo-
pathologic and immunohistochemical findings.
There was no evidence of metastasis in post-
operative thoracal and abdominal computerized tomo-
graphy. The patient regarded as a group I rhabdomy-
osarcoma and he had taken 54 weeks adjuvant chemo-
therapy composed of Vincristine and Actinomycin D.
Patient’s chemotherapeutic treatment completed and
he has been living disease free for 24 mounts in our
follow-up.
Discussion
Sarcomatous tumors of testis can be seen as a com-
ponent of germ cell neoplasm but occasionally they
can be seen as primary testicular sarcoma also. Since
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Figure 1. Microscopic examination revealed a highly cellular tumor composed of plcomorphic cells with round or oval shaped hyperchromatic
nuclei and clear cytoplasm (A HEx100), and additionally rhabdoid cells (B HEx400), and rhabdomyoblasts (C HEx400).
1960, there have been 19 cases of testicular sarcoma
reported in the literature. Histologically, these cases
included 4 leiomyosarcomas, 1 fibrosarcoma, 2 osteo-
sarcomas, 1 chondrosarcoma, 3 spindle cell sarcomas,
1 unclassified sarcoma and 7 rhabdomyosarcomas [2,
3, 4].
The origin of intratesticular sarcoma is unclear. It
is thought to originate from undifferentiated mesen-
chyme having the capacity for rhabdomyoblastic dif-
ferentiation, or perhaps from embryonal muscle tissue
that has been displaced during the early stages of tissue
development, but not from metaplasia of the con-
nective tissue or smooth muscle [1]. Theories about
sarcomatous component in germ cell tumors patho-
genesis include derivation of the tumor cells from
pluripotential germ cells and malignant transformation
from teratomatous elements [5].
The clinical presentation tends to include a short
history, usually only a few weeks of painless swelling
of the scrotum [1]. Mean patient age at presentation
was 32 years range 3 to 86). Tumor size averaged
6.8 cm (range 0.2 to 15 cm) and all were completely
contained within the tunica vaginalis [1, 2]. Serial
serologic evaluation with β-HCG and AFP, although
not specific for sarcoma, is useful to exclude serologic
relapse from undetected germ cell tumor metastases
[3].
Primary tumor must be investigated for the terato-
matous elements. Immunohistochemical markers were
critical in making the differential diagnosis of rhab-
domyosarcoma from the other primary mesenchymal
tumors and germ cell tumors which show rhabdomyo-
blastic differentiation [6]. Tumoral tissue was positive
for smooth muscle actin, muscle-specific actin, S-
100, and Vimentin and negative for cytokeratine and
non-specific for placental alkaline phosphatase and
alpha fetoprotein with immunohistochemical staining.
The lesion was diagnosed as primary rhabdomyosar-
coma without germ cell neoplasia according to these
findings.
Due to the rarity of primary testicular sarcomas,
treatment recommendations are difficult to establish.
All patients in the literature underwent initial orchiec-
tomy and two patients underwent retroperitoneal
lymph node dissection and combined with chemo-
therapy [2, 3]. The rate of lymph node metastases in
sarcomas is less than five percent so that routine ret-
roperitoneal lymph node dissection is not warranted
in pure testicular sarcoma unless the patient exhibits
suspicious, enlarged, or biopsy proven retroperitoneal
lymph nodes, but it has a role in debulking disease
if nodes persist after chemotherapy [1, 2]. Treatment
recommendation is unclear so it should be individual-
ized [2].

The prognosis of primary testicular sarcomas was
considered to be generally poor, overall survival rate
being 68% at 1 year and 30% at 5 years. Prognosis
is now much improved with a multi-disciplinary
approach [1]. Intratesticular rhabdomyosarcomas had
better prognosis than paratesticular rhabdomyosar-
comas and two patients have survived for over 21 years
[1].
References
1. Stewart LH, Lioe TF, Johnston SR. Thirty year review of
intrascrotal rhabdomyosarcoma. Brit J Urol 1991; 68: 418–420.
2. Allaway M, Nseyo UO, Kandzari SJ. Primary testicular sar-
coma. J Urol 2000; 163: 1871.
3. Washecka RM, Mariani AJ, Zuna RE, Honda SA, Chong CDK.
75
Primary intratesticular sarcoma. Immunohistochemical ultra-
structural and DNA flow cytometric study of three cases with
a review of the literature. Cancer 1996; 77: 1524–1528.
4. Ali Y, Kehinde EO, Makar R, Al-Awadi KA, Anim JT. Leiomy-
osarcoma complicating chronic inflammation of the testis. Med
Princ Pract 2002; 11: 157–160.
5. Kaw YT, Cramer HM. Cytologic diagnosis of rhabdomyosar-
coma in a patient with germ cell tumor. Acta Cytol 1995; 39:
249–251.
6. Kahn GD. Rhabdomysarcoma mimiking acuta leukemia in an
adult. Report of a case with histologic, flow cytometric, cyto-
genetic, immunohiskemical, and ultrastructal studies. Arch
Pathol Lab Med 1998; 122: 375–378.
Address for correspondence: Dr. M. Erkan Erbay, Şekercioğlu
Sokak, Emlakbank Koşuyolu Konutları, C Blok D: 9 81020,
Koşuyolu-Kadıköy, İstanbul, Turkey
Phone: +90 216 428 51 36; Fax: +90 216 383 31 93
E-mail: erkanerbay@yahoo.com