PLEOMORPHIC TYPE OF PARATESTICULAR RHABDOMYOSARCOMA

IN YOUNG ADULTS - A CASE REPORT AT SANGLAH GENERAL

HOSPITAL
Dinatha, Gusti Ngurah Krisna*, Yudiana, I Wayan**, Oka, Anak Agung Gede**, Winarti, I Wayan***
*General Surgery Resident, Medical Faculty, Udayana University-Sanglah General Hospital, Denpasar-
Bali
**Subdivision of Urology, General Surgery Department, Medical Faculty, Udayana University-Sanglah
General Hospital, Denpasar-Bali
***Pathology Anatomic Department, Medical Faculty, Udayana University-Sanglah General Hospital,
Denpasar-Bali

ABSTRACT
Introduction. Paratesticular rhabdomyosarcoma is a rare malignant tumor, only 7-
10 % of all intrascrotal tumor and the mean age for these cases is 16-82 years old.
It is classified as embryonal, alveolar, pleomorphic, and mixed type. Embryonal
variant is the most common type and pleomorphic variant is the rarest type, occurs
mainly in adults and has a poor prognosis.
Case. 19-year-old male patient been reported who presented with left scrotal mass
and slight pain since 1 month, accompanied enlargement of right and left inguinal
lymph nodes. Testicle tumor markers (AFP and β-HCG) within normal limits, but
LDH was increasing. Scrotal ultrasonography showing a solid mass in the left
scrotum cavity (paratesticular) with the liquid component, suspiciously infiltrated a
small portion of the left testicle. He underwent left inguinal radical orchidectomy
with histopathological examination revealed pleomorphic rhabdomyosarcoma
origin from funicular spermaticus. Abdominal CT-Scan after surgery was done and
showing enlargement of paraaorta and inguinal lymph nodes with left
hydroureteronephrosis (IRSG group III-IV). Multi modality approach will be plan
to increasing of his survival rate with adjuvant chemotherapy and radiotherapy.

Conclusion. Pleomorphic paratesticular rhabdomyosarcoma is the rarest type and

aggressive tumor, and commonly in adult. Multi modality treatment was needed to
increase of survival rate and decrease of mortality rate.
Keywords : pleomorphic rhabdomyosarcoma, poor prognosis, orchidectomy
chemotherapy and radiotherapy.

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TUMOR PARATESTIKULAR RABDOMIOSARKOMA TIPE
PLEOMORFIK PADA DEWASA MUDA – LAPORAN KASUS DI RUMAH
SAKIT UMUM SANGLAH
Dinatha, Gusti Ngurah Krisna*, Yudiana, I Wayan**, Oka, Anak Agung Gede**, Winarti, I Wayan***
*Residen Bedah Umum, Fakultas Kedokteran, Universitas Udayana, Rumah Sakit Umum Pusat Sanglah,
Denpasar-Bali
**Subdivisi Urologi, Bagian Bedah Umum, Fakultas Kedokteran, Universitas Udayana, Rumah Sakit
Umum Pusat Sanglah, Denpasar-Bali
***Bagian Patologi Anatomi, Fakultas Kedokteran, Universitas Udayana, Rumah Sakit Umum Pusat
Sanglah, Denpasar-Bali

ABSTRAK
Latar belakang. Rabdomiosarkoma paratestikular adalah tumor ganas yang
langka, hanya 7-10% dari semua tumor intraskrotal dan usia rata-rata untuk kasus
ini adalah 16-82 tahun. Diklasifikasikan sebagai embrional, alveolar, pleomorfik,
dan tipe campuran. Varian embrional adalah jenis yang paling umum dan varian
pleomorfik adalah tipe yang paling langka, terutama pada orang dewasa dan
memiliki prognosis yang buruk.
Kasus. Pasien pria berusia 19 tahun dilaporkan datang dengan massa skrotum kiri
dan sedikit nyeri sejak 1 bulan, disertai pembesaran kelenjar getah bening inguinal
kanan dan kiri. Penanda tumor testis (AFP dan β-HCG) dalam batas normal, tetapi
LDH meningkat. Ultrasonografi skrotum menunjukkan massa padat di kavum
skrotum kiri (paratestikular) dengan komponen cairan, dicurigai menyusup ke
sebagian kecil testis. Dia menjalani operasi radikal orkidektomi inguinal kiri
dengan pemeriksaan histopatologi mengungkapkan suatu rabdomiosarkoma tipe
pleomorfik asal dari funikulus spermatikus. CT-Scan abdomen setelah operasi telah
dilakukan dan menunjukkan pembesaran kelenjar getah bening paraaorta dan
inguinal dengan hidroureteronephrosis kiri (kelompok IRSG III-IV). Pendekatan
multi modalitas akan direncanakan untuk meningkatkan tingkat kelangsungan
hidupnya dengan kemoterapi adjuvan dan radioterapi.
Kesimpulan. Rabdomiosarkoma paratestikular tipe pleomorfik adalah jenis yang
paling langka dan tumor agresif, dan umumnya pada orang dewasa. Perlakuan
multi modalitas diperlukan untuk meningkatkan tingkat kelangsungan hidup dan
menurunkan tingkat kematian.
Kata kunci : pleomorfik rabdomiosarkoma, prognosis buruk, orkidektomi
kemoterapi dan radioterapi.

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Introduction.
Primary paratesticular tumors are rare, only accounting for 7% to 10% of all intrascrotal
tumors. The mean age in these cases is 50 to 60 ranging from 16 to 82 years old. All of these
cases have been reported separately and there is no evidence of a hereditary disorder causing
them. There is no known environmental cause to these cases due to their rarity. These tumors are
usually symptomatic, large, and rapid growth tumors. Paratesticular sarcomas constituting a
major proportion of these tumors, particularly in the elderly. The paratesticular region consists of
mesothelial, various epithelial and mesenchymal cells and may therefore give rise to a number of
tumors with various behaviors. Defining the association between the paratesticular mass and the
testicular, and differentiation between benign and malignant masses using radiology is
challenging, therefore the mass is usually considered to be malignant and radical orchidectomy
with high ligation is performed.
Rhabdomyosarcoma (RMS) is the most frequent malignant tumor of paratesticular origin.
It is classified as embryonal, alveolar, pleomorphic, and mixed type. Embryonal variant is mostly
frequent in childhood and has the best prognosis. Alveolar variant occurs mainly in adolescent
and young adults. Pleomorphic subtype is the rarest type, occurs mainly in adults and has a poor
prognosis. Paratesticular rhabdomyosarcoma can develop from mesenchymal elements of the
spermatic cord, the epididymis and the testicular envelopes, resulting in development of a
painless scrotal mass. The clinical presentation includes a short history of painless swelling of
the scrotum in a child or a young adult.
LDH levels were shown to be a marker of retroperitoneal disease in the present group of
men. None of the men with retroperitoneal metastasis had normal LDH levels, and all men with
elevated LDH levels showed retroperitoneal evidence of disease at CT-Scan. LDH was also a
poor prognostic marker in the present series, as all patient with elevated LDH at presentation
died from disease after a mean period of 10 months. This finding is similar to that reported for
soft tissue sarcomas.
Clinical diagnosis of tumors of the spermatic cord can be difficult. Differentiating
between a hernia and a spermatic cord tumor may be possible only at exploration. In general,
these lesions should be approached through an inguinal incision. The cord should be occluded at
the internal ring and frozen sections obtained. If malignancy is diagnosed, attention should be
directed toward performing wide local excision to avoid local recurrence. Staging of disease is
similar to that of testicular tumors. For rhabdomyosarcoma, RPLND (Retroperitoneal Lymph
Node Dissection) should be performed with adjuvant radiotherapy and chemotherapy. The value
of RPLND for the other malignant spermatic cord tumors remains to be determined. Prognosis
relates to the histologic status, stage, and site of disease.

Case.
19-year-old male patient presented to our urology outpatient clinic with paratesticular
pleomorphic rhabdomyosarcoma. He had undergone initially an orchidectomy. Previously patient
complained swelling in the left testicle and slight pain, accompanied by pain in the left waist.
Voiding is within normal limits. Examination of general status is within normal limits.

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Enlargement of right and left inguinal lymph nodes, with pain on left costovertebral angle and
scarring from previous surgery in left inguinal was obtained on physical examination. From the
results of scrotal ultrasonography before prompt surgery, there was mass lobulated with fluid
components on the left scrotum. The partial limit of the lobulated solid component seems
difficult to distinguish from the left testicle. The left testicle appears to be squeezed by a solid
mass in the left scrotal cavity There was a homogenous parenchymal echo and visible
vascularization increases within the solid lesion, the impression of nodules in the left testicle.
Left epididymis was difficult to assess, with suspicion of left inguinal lymphadenopathy
(hypoechoic ovoid-shaped lesions in inguinal left 1.45 x 0.7 cm).
Testicular tumor marker (AFP and β-HCG) are within normal limits, and LDH still
increase after orchidectomy. Ct-scan abdomen with contast showed a hypodens mass in the right
testicle (3 x 4 cm), right and left scrotal hydrocele, enlarged lymph nodes in paraaorta and
inguinal right and left, with left moderate hydroureteronephrosis, with suspect of metastatic
process in the 3rd lumbar vertebrae. Histopathological examination of resected spermatic
funicular showed infiltration of intravenous tumor cells, with the impression of a sarcoma tends
to be a pleomorphic rhabdomyosarcoma which invade into the testicle tissue. Due to histologic
subtype of the tumor, adjuvant treatment was indicated. The patient accepted the possible
adverse effects of the treatment comprising infertility.

Figure 1 : Clinical photo of patient after surgery

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 Figure 2 : Photo of patient during surgery

Figure 3 : Scrotal ultrasonography before surgery

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Figure 4 : Thorax photo, cor and pulmo within normal limits

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Figure 5 : Ct-scan abdomen with contrast

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 Figure 6 : Tumor mass outside the testicle

Figure 7 : An intravenous invasion of spermatic funiculus

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Discussion.
The paratesticular region is the commonest site for the development of RMS. Clinically
RMS present as an intrascrotal mass, hard, usually large,sometimes reaching up to the external
inguinal ring and compressing the testicular and epididymis. Pain is associated with the tumor in
only 7 % of cases. It’s incidence is similar in Africo-American and Caucasian and appears to be
lower in Asian population. A hydrocele can be occasionally present in adults explaining the
frequent misdiagnostic of paratesticular rhabdomyosarcoma. An association with genetically
transmitted disease exists rhabdomyosarcoma occurring more frequently in particular with von
Recklinghausen disease, Li-fraumeni syndrome, Beckwith-Wiedemann, Costello, and Noonan
syndromes.
Paratesticular rhabdomyosarcoma (PTR) usually arises in the spermatic cord but it can
compress or invade neighboring structures such as the epididymis or testicle, and may be very
extensive. All known rhabdomyosarcoma histological subtypes are described, however the
commonest subtype of PTR is embryonal rather than pleomorphic or alveolar. Although electron
microscopy can help to establish the diagnosis by showing actin and myosin filaments and Z-
bands, immunohistochemistry is much more sensitive. Antibodies to myogenin and MyoD1, as
well as desmin and smooth muscle actin, help to differentiate rhabdomyosarcoma from other
small, round cell tumors of childhood such as neuroblastoma, Ewing sarcoma, or lymphoma, and
from other paratesticular sarcoma in adults.
Spread of the tumor is mostly by lymphatics to the iliac and paraaortic nodes, but
hematogenous spread are uncommon at presentation, occurring in less 5 % of patients, mainly in
the liver, bone marrow, and lung. The most common site of recurrence after treatment for early-
stage disease include the groin, retroperitoneal lymph nodes, lung, bone, bone marrow. Work up
investigations at diagnosis includes physical examination, full blood count, AFP, and β-HCG
estimation (to differentiate germ cell cancer), chest x-ray, bilateral bone marrow smears and
biopsies, abdominal and chest computed tomography (CT) scan, and bone scan. Staging of
partesticular rhabdomyosarcoma can be done according to both the tumor-nodus metastase
classification and the IRSG (The Intergroup Rhabdomyosarcoma Study Group). Multiagent
chemotherapy is an important component of the multidisciplinary approach in the treatment of
these tumors. Various combination and dose schedules have been used in different protocols in
Europe and North America. Overall, regimens have been progressively reduced in intensity and
duration over the years. The active agents include vincristine, dactinomycin, cyclophosphamide,
ifosfamide, and doxorubicin.
Paratesticular sarcoma are rare, there is no standard treatment. In the localized disease,
treatment strategies include radical high inguinal orchidectomy, RPLND, chemotherapy and
radiotherapy. Lymph node dissection was found to improve the 5-year overall survival rate from
64 % to 86 % (p<0,01), and was recommended by the authors for children > 10 years and adults.
In this case the patient will have adjuvant chemotherapy with VAC (vincristine, actinomycin-D /
doxorubicin, cyclophosphamide), and will be evaluated continuously. The prognosis of
paratesticular rhabdomyosarcoma is extremely poor especially pleomorphic type. Patient in the

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IRGS had a 5-year survival rate of 22,2 %, furthermore, age seems to be a prognostic factor with
a worse prognosis in adults patients than children.

Conclusion.
Paratesticular rhabdomyosarcoma is rare and aggressive tumor. In the absence of
protocols designed specifically for adult patients, it is necessary to follow therapeutic guidelines
in pediatric protocols. Patient will have adjuvant chemotherapy and radiotherapy, and will be
evaluated further.

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