Genital rhabdomyoma is very rare tumor that usually occurs in the vulvar of young women. Epididymis rhabdomyoma in a young man is extremely uncommon and has rarely been reported. Here, we report a case of epididymis rhabdomyoma of a 17-year-old man and review the literatures. VIRTUAL SLIDE: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/117762822469279

En-hua Wang

Jian Wang

Qing-chang Li

Qing-fu Zhang

Xu-yong Lin

Xue-shan Qiu

Yang Han

Yu-chen Han

Ze-liang Li

PubMed

Springer - Publisher Connector

Han et al. Diagnostic Pathology 2012, 7:47
http://www.diagnosticpathology.org/content/7/1/47CASE REPORT Open AccessEpididymis rhabdomyoma: A case report and
literature review
Yang Han1, Xue-shan Qiu1, Qing-chang Li1, Yu-chen Han1, Xu-yong Lin1, Qing-fu Zhang1, Jian Wang1,
En-hua Wang1* and Ze-liang Li2Abstract
Genital rhabdomyoma is very rare tumor that usually occurs in the vulvar of young women. Epididymis
rhabdomyoma in a young man is extremely uncommon and has rarely been reported. Here, we report a case of
epididymis rhabdomyoma of a 17-year-old man and review the literatures.
Virtual slide: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/
1177628224692794
Keywords: Rhabdomyoma, Epididymis, Epididymis rhabdomyoma immunohistochemistryBackground
Rhabdomyoma is an exceedingly rare benign tumor of
striated muscle. It can be divided into the following categor-
ies: cardiac rhabdomyomas and extracardiac rhabdomyo-
mas, which are relatively rare [1]. Genital rhabdomyoma is
even more uncommon, usually occurs in the vulva of young
women [2,3]. Epididymal rhabdomyoma is extremely rare
in the world and has rarely been reported [4,5]. Here, we re-
port a case of epididymis rhabdomyoma of a 17-year-old
man and review of the literatures.Clinical history
A 17-year-old Chinese man presented with a painless,
indurated, right testicular mass and no other associated
symptoms. The patient felt the mass increased slowly
during the past 2 years. He denied any history of trauma
to testes, systemic TB (tuberculosis), genital infections,
or STD (sexually transmitted diseases). The patient denied
his family history ever had male genital tumor, such as von
Hippel-Lindau disease (a risk factor of epididymal papillary
cystadenomas) or other genitourinary diseases [6]. During
the past 2 years, he tried a variety of non-surgical treat-
ments, including traditional Chinese medicine, but the mass
had no significant change. At first, his mother, a farmer,
believed sexual intercourse might reduce the mass, but one* Correspondence: wangeh@yahoo.cn
1Department of Pathology, College of Basic Medical Sciences and First
Affiliated Hospital of China Medical University, Shenyang, China
Full list of author information is available at the end of the article
© 2012 Han et al.; licensee BioMed Central Ltd
Attribution License (http://creativecommons.o
medium, provided the original work is properyear after married, the mass didn’t shrink. Physical examin-
ation showed a firm, nontender mass in the right scrotal
sac, measured about 1.5 cm×1.0 cm×1.0 cm and was con-
sidered originated from epididymis. The left scrotal sac was
unremarkable. The penis was uncircumcised. The rectal
examination was within normal limits. Urinalysis revealed
no significant abnormalities. Serum human chorionic go-
nadotropin (hCG) and a-fetoprotein (AFP) levels were not
elevated. Ultrasound revealed a slightly inhomogeneous
nodule in the head region of the right epididymis, about
1.51 cm×1.13 cm. Inguinal exploration with possible right
radical orchiectomy versus right epididymis tumor resection
was scheduled. Surgical findings: A 1.5 cm×1.0 cm×1.0 cm
firm mass involved the head of the right epididymis. There
was no obvious abnormal in the right testis and the tunica
vaginalis was intact. Intraoperative frozen section diagnosis
of the epididymal mass considered a benign tumor, possibly
leiomyoma (Figure 1, C). Conservative excision of the mass
with preservation of the right epididymis and testis was
performed. No adjuvant treatment was performed and the
patient is well, without recurrence 6 months after surgery.Pathology
GROSS
A firm mass with tan white fibrous capsule measured
1.5 cm×1.0 cm×1.0 cm in the head of epidymis (Figure 1,
A). The cut surface of the tumor was solid, tan-white, had a
faint fascicular pattern (Figure 1, B). No hemorrhage or ne-
crosis was noted.This is an Open Access article distributed under the terms of the Creative Commons
rg/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any
ly cited.
Figure 1 A and B, gross appearance of tumor. The mass with part of thin fibrous capsule (A) and the cut surface was solid, tan-white and had
a faint fascicular pattern (B). C, The frozen section, a little ice crystals in the tissue, HE × 400. D-G, Bland-appearing, round, spindled, elongated,
ribbon cells with abundant eosinophilic cytoplasm and eccentrically placed, oval nuclei rhabdomyocytes with cross-striations. No mitosis or atypia
is noted. Mononuclear cells infiltrate. D, HE × 100; E, HE × 200; and F&G, HE × 400.
Han et al. Diagnostic Pathology 2012, 7:47 Page 2 of 5
http://www.diagnosticpathology.org/content/7/1/47Histology and immunohistochemistry
The tumor was fixed in 10% formalin and embedded in
paraffin. Several 4-μm sections were cut from each paraffin
block. Hematoxylin-eosin (HE) and immunohistochemical
(IHC) stains were performed. IHC staining was performed
using the streptavidin-peroxidase system (Ultrasensitive;
MaiXin Inc., Fuzhou, China) according to the manufac-
turer's instruction. Commercially available prediluted
monoclonal antibodies against the following antigens were
employed: Actin (sm, smooth muscle) (1:200; Mouse mAb
(DE-B-5), Merck), CK (1:200; Mouse mAb (B311.1),
Merck), S-100 (1:200; Mouse mAb (1B2), Merck), CD34
(1:200; Mouse mAb (QBEnd/10), Merck), Vimentin (1:200;Mouse mAb (V-9), Merck), Desmin (1:200; Mouse mAb
(DE-B-5), Merck) and Ki-67 (1:200; MIB1, Dako). The im-
mune reactions were visualized with the use of DAB as the
chromogen (Sigma-Aldrich Co, St Louis, Mo, USA). All
internal and external controls worked appropriately.
Results
Microscopically, the mass showed a proliferation of rhabdo-
myocytes in a background of dense connective tissue.
Microscopic examination found multiple defined nests of
mature, round to fusiform,elongated, ribbon cells with
abundant eosinophilic cytoplasm and eccentrically placed,
oval nuclei. The nuclei were uniform and round. No
Han et al. Diagnostic Pathology 2012, 7:47 Page 3 of 5
http://www.diagnosticpathology.org/content/7/1/47pleomorphism, atypia, mitotic figures or pathological
mitotic were identified. Focal adipocyte collections were
noted among the dense fibrous connective and little
mucilage tissue. A scant, patchy mononuclear cellFigure 2 IHC staining of the tumor with strong staining of tumor cells
Negative for CK (A, ×200), CD34 (C, ×200), S-100 (D, ×200) and Actin (sm) (
vascular endothelial cells (C) and actin (sm), staining of vascular myoepitheinfiltrate, perhaps due to slight chronic inflammation
caused by tumor growth, was present throughout the
tumor (Figure 1, D-G). Immunohistochemical stains
showed these lesional cells to be negative for CK, CD34,for Vimentin (B, ×200) and Desmin (F, ×200; G&H, ×400).
E, ×200). Note the striate in the tumor cells (G&H), CD34, staining of
lial cells (E).
Han et al. Diagnostic Pathology 2012, 7:47 Page 4 of 5
http://www.diagnosticpathology.org/content/7/1/47S-100, Actin (sm), and positive for Vimentin and Desmin.
Ki-67, a proliferation marker, was positive in less than 1%
of lesional cells (Figure 2). The final diagnosis was right
epididymis fetal rhabdomyoma.Discusson
Rhabdomyomas are benign tumors of striated muscle
cells and generally divided into the following categories:
cardiac rhabdomyomas, which are relatively common,
and extracardiac rhabdomyomas (occurring outside of
the heart), which are rare (comprising only 2% of all
tumors with striated muscle differentiation) [2,3]. Based
on clinical manifestations and morphological characteristics,
the extracardiac forms of rhabdomyoma are subclassified
into 4 distinct types: (1) the fetal type, a rare form that
affects the head and neck region but occurs in both children
and adults. Within the fetal subtype, there is a wide
spectrum of histology reflecting the degree of differentiation
[7,8]; (2) the adult type, usually found in the head and neck
region of the older person and slowly growing, more than
40% recur. This type tumors can be multinodular, cells are
rounder, and there are crystalline cytoplasmic inclusions of
hypertrophic Z-band material [9-11]; (3) the genital type, a
tumor-like polypoid or cystic mass that has been described
commonly in the genital tract of middle-aged women with
a mean age of 42 years. This type resembled fetal rhabdo-
myomas in architecture but showed greater maturity of
the myocytes [4,12,13]; and (4) rhabdomyomatous mes-
enchymal hamartomas, a peculiar striated muscle pro-
liferation that occurs chiefly in the periorbital and
perianal region of infants and young children. The
pathologic subtype does not necessarily reflect the age
of the patient.
The differential diagnosis of an epididymal tumor
includes TB, spermatic granuloma, adenomatoid tumor (a
common tumor in this location), mesothelioma, papillary
cystadenoma (especially if the patient has a family history of
von Hippel-Lindau disease), and embryonal rhabdomyosar-
coma. The right epididymal tumor removed from the young
patient showed clinicopathologic features typical of the
genital variant of extracardiac rhabdomyoma. Additional
histologic considerations consist of other benign processes,
such as leiomyoma and fibromatosis; the presence of cross-
striations and IHC steins rule out leiomyoma or fibromato-
sis. Histologically, the fetal variant of rhabdomyoma shares
many features of the genital variant, and usually has a more
myxoid and less collagenous stroma with proliferation of
immature mesenchymal cells and is more cellular. Embry-
onal rhabdomyosarcoma should be considered in the differ-
ential diagnosis, in which one would expect to find
necrosis, increased mitotic activity, abnormal mitoses, nu-
clear atypia, pleomorphism, anaplasia and a high prolifera-
tion index, e.g. Ki67> 5% [14].Here, we report this quite rare tumor and provide
comprehensive figures, including gross appearance, HE
and IHC staining. This is the third epididymal rhabdo-
myoma described in the English literature [4,5]. The first
two cases are both 20 years old. They did not present
specific symptoms, and a tumor mass in left epididymis
(5.5 cm× 4.0 cm×2.5 cm and 5.0 cm×4.0 cm× 2.0 cm
respectively) was the only finding. The probable origin is
the cremaster muscle. Additionally, there are other six
rhabdomyomas in the male genitourinary tract described
in English, two in the spermatic cord (67-year-old man,
3.0 cm×2.0 cm×2.0 cm,17-year-oldman, 3.5 cm×2.4 cm×
2.1 cm, respectively) [10,15], one in the tunica vaginalis
(19-year-old man) [12], one in prostate (nineteen-year-old
white man) [16] and two paratesticular (10 months of in-
fant, 1.5 cm and 55-year-old man, 6.0 cm×5.5 cm×4.5 cm,
respectively) [17,18]. When faced with a pleomorphic
mesenchymal tumor in the male genitourinary tract,
pathologists should remember the diagnosis of rhabdo-
myoma. To our knowledge, rhabdomyomas exhibited
benign behavior and low recurrence rate in other sites.
Further documentation and follow-up of such cases will
help better define the biological behavior and prognosis
of the rhabdomyomas.
Competing interests
The authors declare that they have no competing interests.
Author details
1Department of Pathology, College of Basic Medical Sciences and First
Affiliated Hospital of China Medical University, Shenyang, China. 2Department
of Urology, First Affiliated Hospital of China Medical University, Shenyang,
China.
Authors’ contributions
YH analyzed the data, diagnosed and wrote the manuscript as a major
contributor. XQ, QL, YH, XL, QZ, JW contributed to diagnose and
management of the patient. EW carried out the histopathological evaluation
and helped to write manuscript. ZL performed the operation. All authors
have read and approved the final manuscript.
Received: 10 March 2012 Accepted: 20 April 2012
Published: 20 April 2012
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doi:10.1186/1746-1596-7-4710.1186/1746-1596-7-47
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Epididymis rhabdomyoma: A case report and literature review

A: Rhabdomyoma of the tunica vaginalis of the testis: a histologic, immunohistochemical, and ultrastructural study. Mod Pathol

AD: Rhabdomyoma of prostate. Urology

DM: Spermatic cord rhabdomyoma. Urology

FE: Epididymal rhabdomyoma: report of a case, including histologic and immunohistochemical findings. Arch Pathol Lab Med

Hyams VJ: Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study. Hum Pathol

IM: Epididymal rhabdomyoma.

In Myogenic tumors of soft tissue. Edited by

Jr: Tumors of skeletal muscle. Hum Pathol

MM: Epididymal cystadenomas in von Hippel-Lindau disease. Urology

Ortac R: Intrascrotal paratesticular rhabdomyoma: a case report. Acta Paediatr

Paratesticular rhabdomyoma.

Rhabdomyosarcoma of the head and neck: a clinicopathological and immunohistochemical analysis of 29 cases. Braz Dent J

Sant'Agnese PA: Extracardiac rhabdomyomas. Semin Diagn Pathol

SG: Paratesticular rhabdomyoma in a young adult: case study and review of the literature. J Pediatr Surg

WS, eds: Soft Tissue Tumors. 2nd edition. St Louis,

Epididymis rhabdomyoma: A case report and literature review

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