Recurrent metastasis of progressive parachordoma of spermatic duct to different sites in a year: A rare case

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Yıldırım et al. Recurrent metastasis of parachordoma 377
J Clin Exp Invest www.jceionline.org Vol 4, No 3, September 2013
1 Turgut Özal University, School of Medicine, Department of Urology, Ankara, Turkey
2 Turgut Özal University, School of Medicine, Department of General Surgery, Ankara, Turkey
Correspondence: Hüseyin Sahin,
Turgut Özal Üniversitesi, Tıp Fakültesi, Genel Cerrahi AD, Emek, Ankara, Turkey Email: kazangap84@hotmail.com
Received: 28.04.2013, Accepted: 28.05.2013
Copyright © JCEI / Journal of Clinical and Experimental Invesgaons 2013, All rights reserved
JCEI / 2013; 4 (3): 377-379
Journal of Clinical and Experimental Invesgaons doi: 10.5799/ahinjs.01.2013.03.0307
CASE REPORT / OLGU SUNUMU
Recurrent metastasis of progressive parachordoma of spermac duct to dierent sites
in a year: A rare case
Progresif spermak kanal parakordomasının vücudun farklı kısımlarına bir yıl içinde
tekrarlayan metastazları: Nadir bir olgu
Mehmet Erol Yıldırım1, Hüseyin Şahin2, Hüseyin Badem1, Ersin Çimentepe1, Cenap Dener2
ÖZET
Bugüne kadar yaklaşık olarak 60 parakordoma vakası
bildirilmiştir. Parakordoma etyolojisi tam olarak bilinme-
yen nadir bir antitedir. Biz burada 5 ay içinde vücudun 2
farklı yerine metastaz yapan, agresif bir tümör olgumuzu
paylaşmak istedik. Sağ inguinal ağrı ile hastanemize baş-
vuran 49 yaşındaki erkek hastanın çekilen tüm abdomen
tomograsinde sağ inguinal subepidermal sahada 52x50
mm çapında kitle tespit edildi. Eksize edilen kitlenin pato-
lojik tanısı parakordoma olarak bildirildi. Operasyondan 4
ay sonra hasta ağrısız sağ testiküler kitle ile tekrar baş-
vurdu. Yapılan ultrasonograde sağ inguinal bölgede 3 ve
sağ testiste olmak üzere 4 adet kitle saptandı. Orşiektomi
ve kitlelerin eksizyonu sonrasında patoloji yine parakor-
doma olarak geldi. İkinci operasyondan 1 ay sonra hasta
sağ kalça ağrısı ile başvurdu ve yapılan manyeik rezo-
nans incelemede sağ femur başında kitle tespit edildi. Bi-
yopsi neticesinde parakordoma metastazı olması üzerine
radyoterapi alması için radyasyon onkolojisine konsülte
edildi. Parakordoma genelde nadir ve iyi huylu olmakla
birlikte, özellikle ilk sene içindeki metastaz ve rekürrensler
malign potansiyel açısından dikkate alınmalıdır. Özellikle
metastatik olgularda, tümörün en geniş şekilde rezeksiyo-
nunu takiben de radyoterapi önerilmektedir.
Anahtar kelimeler: Parakordoma, pelvik tümör, radyote-
rapi
ABSTRACT
Parachordoma is a rare entity with an unknown origin,
which reported nearly in 60 cases. We are reporting an
aggressive tumor that has two recurrences in 5 months
in different sites. A 49 year old male admitted with right
inguinal pain that underwent a computed tomography,
revealed a 52x50 mm tumor at the right inguinal subepi-
dermal region. After the resection, pathology of the mass
was parachordoma. Four months after the operation,
patient admitted with a solitary mass in the right testis.
Ultrasonography revealed four different masses in the
right inguinal and right testicular region. Second pathol-
ogy results were also parachordoma. One month later the
second operation, patient again admitted to hospital with
right hip pain, had a solitary mass on the collum femoris
in the magnetic resonance imaging consulted for radio-
therapy. Parachordoma is a rare, benign entity that must
be closely followed up. The physician must be aware of
the malignancy especially in the rst year with existence
of recurrence and metastasis. Radiotherapy is recom-
mended after maximal resection of tumor especially in
metastatic cases. J Clin Exp Invest 2013; 4 (3): 377-379
Key words: Parachordoma, pelvic tumor, radiotherapy
INTRODUCTION
Parachordoma is a soft tissue tumor with an un-
known origin that proposed as mixed tumor and
myoepithelioma at 2002 by World Health Organiza-
tion [1]. Parachordoma was rst reported by Las-
kowski in 1959 as a peripheral chordoma and there
are about only 50-60 cases reported in the recent
literature [2]. This report presents the third case as
pelvic parachordoma with unusual aggressive be-
havior and two times metastasis at ve month pe-
riod.
CASE
A 49-year-old male admitted to urology clinic with a
painless growing mass in the right inguinal region
lasting for a month. He had no signicant medical
or surgical history. A three cm mass palpated at
the right inguinal region during physical examina-

Yıldırım et al. Recurrent metastasis of parachordoma
378
J Clin Exp Invest www.jceionline.org Vol 4, No 3, September 2013
tion. Ultrasonography (USG) revealed a 35x26 mm
heterogeneous semisolid mass at the right inguinal
region. A computed tomography (CT) scan showed
a lobular lesion of 52x50 mm, undistinguished
from the right spermatic cord, at the right side of
the midline upper to the symphisis pubis in the sub
epidermal fat tissue (Figure 1-A). Urology consulted
the patient to general surgery clinic. After the hys-
topathologic report of mixoid malign mesenchyme
tumor by the tru cut biopsy, we resected the inguinal
mass largely and repaired the inguinal region with
prolen mesh graft (Figure 1-B). Histopathology of
the resected mass was parachordoma with nega-
tive surgical margin and positive vascular invasion.
We decided to follow up the patient however there
was no tumor invasion at PET-CT scan. In the post-
operative fourth month, patient admitted with a solid
mass at right spermatic cord, superior to the right
testis. Doppler USG revealed a lobular lesion at
the right spermatic cord with increased blood ow,
two lobular lesions at the right inguinal region and
another solid mass in the sub epidermal fat tissue
nearby the right radix penis with increased blood
ow. These tumors resected with right orchiectomy
due to the preoperative parachordoma report of the
tru cut biopsy (Figure 1-C). Parachordoma reported
by pathology with negative surgical margin, positive
vascular invasion and necrosis widespread. In the
rst mount after the second operation the patient
admitted again with right hip pain. X-ray showed
nothing but magnetic resonance imaging showed a
2x1,5 cm T2 hyper intense lesion at the posterior
side of collum femoris. This lesion was hypo intense
on weighted image and enhancing homogeneously
in post contrast (Figure 1-D). A tru cut biopsy taken
with guidance of CT comprised malignant cells. We
consulted the patient to the department of radiation
oncology for radiotherapy with our close follow-up.
DISCUSSION
Parachordoma is an rare adult benign soft tissue
tumor, however recurrence and metastasis are
merely reported [3]. It is rst reported by Laskows-
ki in 1959 [4]. There is a slight male dominancy in
parachordoma [4]. The average age is about 34.4
years [range 4-86 years old], however 20% of the
patients are children in reported cases [2,5]. Our
patient was 49 years old male. The common local-
izations of the parachordoma are the extremities,
head, neck, torax, bones and rarely in the pelvis.
This is the third parachordoma case that has been
seen in the pelvis.
Tumor is characterized with painless and slow
progression, but our patient admitted to us three
times with pain in six-month period. It is well circum-
scribed and pseudo capsulated with a thin brous
tissue. It is usually 2-7 cm in size but can grove up
Figure 1. A) Ab-
dominal CT image
of the right inguinal
mass before the
rst operation; B)
mass resected at
the rst operation;
C) mass and right
testis resected at
the second opera-
tion; D) MR image
of the lesion at the
right collum femoris

Yıldırım et al. Recurrent metastasis of parachordoma 379
J Clin Exp Invest www.jceionline.org Vol 4, No 3, September 2013
to 12 cm [4]. Patient’s rst tumor size was 7 cm and
second was 3 cm respectively. It is curable with re-
section but sometimes recurrences and metastasis
may occur from the tumor negative margins. Even
its slow growth and locally invasive pattern, mortal-
ity with recurrence and metastasis in a year can
be seen rarely [4]. There are only two deaths from
metastatic parachordoma in the literature; however,
some authors called parachordomas as low-grade
sarcomas [7].
Our patient presented with left inguinal pain
and we have seen two recurrences and distant me-
tastasis in a 5 months period. First recurrence was 4
months after the rst surgery at the same area and
the second recurrence was at the collum femoris.
Although the difculty of resection of femur metas-
tasis we decided to treat the patient with the pelvic
radiotherapy. Recurrence reported at 3, 6 and 12
months by Niezabitowski, Carstens and Ishida re-
spectively [8,9]. There is not a long follow up espe-
cially about pelvic metastatic parachordoma in the
literature. Adjuvant radiotherapy is usually proposed
after maximal resection of the tumor [10]. After com-
paring with other forms of radiation therapy, they ad-
vocated proton beam therapy as a better tool used
to increase the dose delivered to the tumor while
elegantly sparing dosing to adjacent critical normal
structures.
Parachordoma is a rare entity that can affect
any site of the body. İmaging can direct us to the tu-
mor but exact diagnose could only be made by the
pathologist. A correct diagnosis has denite thera-
peutic implications. Multidisciplinary diagnose must
be done. Radiotherapy should be kept in mind for
the metastatic cases at the rst year of diagnose.
REFERENCES
1. Weiss SW, Goldblum JR: Enzinger & Weiss’s Soft Tis-
sue Tumors. 5th ed., Philadelphia, Mosby Elsevier,
2008,1109-1116.
2. Tirabosco R, Mangham DC, Rosenberg AE, et al.
Brachyury expression in extra-axial skeletal and soft
tissue chordomas: a marker that distinguishes chor-
doma from mixed tumor/myoepithelioma/parachordo-
ma in soft tissue. Am J Surg Pathol 2008;32:572-580.
3. Sangueza OP, White CR Jr. Parachordoma. Am J Der-
matopathol 1994;16:185-188.
4. Karakaya YA, Özekinci S, Büyükbayram H, Mızrak B.
Parachordoma: A Recurrent Case and Review of the
Literature. Turk Patoloji Derg 2011;27:173-176.
5. Lococo F, Cesario A, Meacci E, et al. Pulmonary
metastases from parachordoma Ann Thorac Surg
2009;88:9-10.
6. Dabska M. Parachordoma: A new clinicopathologic en-
tity. Cancer 1977;40:1958-1992.
7. Clabeaux J, Hojnowski L, Valente A, Damron TA. Case
report: parachordoma of soft tissues of the arm. Clin
Orthop Relat Res 2008;466:1251-1256.
8. Fisher C, Miettinen M. Parachordoma: a clinicopatho-
logic and immunohistochemical study of four cases of
an unusual soft tissue neoplasm. Ann Diagn Pathol
1997;1:3-10
9. Ishida T, Oda H, Oka T, et al. Parachordoma: an ultra-
structural and mmunohistochemical study. Virchows
Arch A Pathol Anat Histopathol 1993;422:239-245.
10. Nguyen QN, Chang EL. Emerging role of proton beam
radiation therapy for chordoma and chondrosarcoma
of the skull base. Curr Oncol Rep 2008;10:338-343.

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