# Retroperitoneal Extragonadal Nonseminomatous Germ Cell Tumor with Synchronous Orbital Metastasis

Authors: Ali Fuat Atmaca; Serkan Altınova; Abdullah Erdem Canda; M. Fuat Ozcan; Suleyman Alıcı; Leyla Memıs; M. Derya Balbay
Journal: Advances in Urology
 (2009)

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## Abstract

A huge retroperitoneal tumor with a right orbital mass was detected and proved to be an extragonadal nonseminomatous germ cell tumor on biopsy. BEP chemotherapy caused some regression in orbital mass however no change in retroperitoneal tumor size as well as serum tumor marker levels occurred. Herein, we present a rarely seen entity of extragonadal retroperitoneal nonseminomatous germ cell tumor with synchronous orbital metastases and discuss its diagnosis and management.


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## Body

## 1. Introduction

Extragonadal germ cell tumors (EGGCT) account for approximately 2–5% of all germ cell tumors. They are mostly seen in the mediastinum and retroperitoneum. However, it can also be seen in less frequent locations such as sacrococygeal region, pineal region, prostate, orbita, liver, vagina, and gastrointestinal tract [1, 2].These lesions may grow up to extensive dimensions without any significant symptoms. The diagnosis is usually made in the third decade. Advanced local disease and distant metastases might exist at the
time of diagnosis in the majority of patients [3].


## 2. Case Report

A 31-year-old man complaining of bilateral lumber pain, fatigue was diagnosed with right hydronephrosis on ultrasonography (US), and a double J stent was placed into the right ureter at another hospital a
month ago. He then was referred to our institution for further evaluation. His
past medical history revealed bilateral nephrolithotomy in 1998 and right
nephrolithotomy in 1999. Physical examination of urogenital system was within
normal limits including the scrotal examination. On admission, there was proptosis
(eccentric) present displacing his right eye infero-nasally. His blood hemoglobulin
and leukocyte counts were 7.81 g/dL and 21 500, respectively. Blood chemistry
was within normal limits except elevated urea (49 mg/dL) and creatinine (2.1 mg/dL) levels. Serum tumor marker studies revealed an increased beta-human
chorionic gonadotropin (β HCG) level (37.8 mIU/mL) and an increased lactate dehydrogenase (LDH)
level (800 IU/L). Serum alpha fetoprotein (AFP) level was within normal limits.Abdominal doppler US has shown multiple
bilateral renal stones, grade 2 ectasia in the left kidney and multiple
irregular iso-hyperechoic hypervascular solid lesions in the right kidney.
Additionally, scrotal US examination revealed no abnormalities.On computerized tomography scans, multiple heavily
contrast-stained conglomaralated necrotic lymphadenopathies undifferentiated
from right kidney in paraaortic and paracaval regions and destruction of L2
vertebral body were observed (Figure1(a)). On magnetic resonance imaging, a
contrast stained mass of 23×20×18 mm in size displacing superior and
lateral orbital rectus muscles and also infiltrating into the frontal bone was
seen in the right orbita (Figure 1(b)).(a) CT of the
retroperitoneal mass. Note the vertebral body invasion (arrow). CT:
Computerized tomography. (b) T1-weighted MRI scans of the right orbital mass (arrow). MRI: Magnetic
resonance imaging.
(a)(b)Fine needle aspiration cytology from
retroperitoneal mass demonstrated a carcinoma associated with germ cell tumor
with positive staining for both AFP andβ-HCG on immunohistochemical evaluation
(Figures 2(a) and 2(b)).(a) Microscopic appearance
of tumor cells showing diffuse and strong positive immunohistochemical staining
for alpha fetoprotein. (b) Microscopic appearance of tumor cells showing diffuse and strong positive
immunohistochemical staining for beta human chorionic gonadotropin.
(a)(b)The diagnosis was made as primary nonseminomatous
extragonadal (retroperitoneal) germ cell tumor (EGGCT) with orbital metastasis.
Chemotherapy including cisplatin, etoposide, and bleomycine (BEP) was
administered. After completion of first course chemotherapy, symptoms subsided
with a significant reduction in proptosis. However, serum tumor marker levels
did not change. Afterwards, a very rapid progression of the disease has occurred
following initial regression of his symptoms. Dimensions of orbital lesion increased
significantly with concomitant increase in serum tumor marker levels including AFP
andβ-HCG. Unfortunately, the patient died after the second course of
chemotherapy.


## 3. Discussion

EGGCTs are rarely seen tumors with specific
biological and clinical characteristics. Symptoms depend on the location of the
tumor such as presence of a palpable mass, abdominal or back pain, dysphagia, and
edema in the limbs when the tumor is located in the retroperitoneum. Constitutional
symptoms such as fever and weight loss might accompany the disease. The
diagnosis is made histopathologically when seminomatous and nonseminomatous
elements of the tumor are seen on biopsy. Tumor markers are also expected to
increase if nonseminomatous elements are present [1, 2].In our patient, main symptoms were abdominal
and low back pain. Immunohistochemical evaluation of the biopsy for AFP and HCG
staining were positive therefore, the patient was diagnosed as having extragonadal
nonseminomatous germ cell tumor.Metastases to other tissues depend on the
localization and histological type of the primary tumor [4]. Lung metastasis
rate has been reported to be 27% in mediastinal nonseminomatous tumors whereas
it has been reported to be 49% in retroperitoneal tumors [4]. Additionally, abdominal
(34%), liver (25%), and cervical lymph node (18%) metastases might also be
present [4].Scrotal US could easily differentiate a
retroperitoneal EGGCT from primary testicular tumor metastases without routinely
performing testicular biopsy for differential diagnosis [2].The prognosis is excellent in cases with
seminomatous histology regardless of the localization of the EGGCT either in mediastinum
or in retroperitoneum. However, prognosis of nonseminomatous EGGCT is worse
than that of the seminomatous variant. Five-year-survival rates have been
reported to be 45% for mediastinal and 62% for retroperitoneal nonseminomatous
tumors [2, 4]. Unfortunately, the majority of patients (80%) have
nonseminomatous EGGCT thus a poor prognosis which is independent of the primary
location of the nonseminomatous EGGCT [2, 4].Management of patients with EGGCT is accomplished
according to the prognostic classification of International Germ Cell Cancer Collaboration Group [2]. Standard cisplatinum-based chemotherapy plus additional secondary
surgery in half of the patients is the recommended treatment strategy. Salvage
chemotherapy including high-dose chemotherapy does not have a significant
impact on long-term survival. Regarding seminomatous tumors, 3 courses of BEP chemotherapy
administration have been suggested for patients with good prognosis, and 4
courses of BEP chemotherapy have been recommended for patients with moderate
prognosis. In retroperitoneal nonseminatous tumors, 3 and 4 courses of BEP
chemotherapy regimen should be administered to patients with good or moderate
and poor prognostic criteria, respectively. In patients with mediastinal EGGCT,
4 courses of BEP chemotherapy are suggested [2].Secondary surgery is an integral part of the
treatment strategy in patients with EGGCT and is mandatory in cases with
residual mass, which is true for about 50% of the patient population [2].We intended to treat our patient with 4 courses
of BEP chemotherapy; however, we were able to administer only two courses. Unfortunately,
we lost the patient despite partial response with some regression in the
diameter of the orbital mass without any change in serum tumor marker levels.In our case, a right orbital mass accompanying
the retroperitoneal tumor which we think was metastasis rather than another
primary focus regressed after the administration of first course of BEP
chemotherapy. Because the volume of the orbital tumor mass was smaller than the
volume of the retroperitoneal tumor, we think that orbital tumor regressed to
some degree while the retroperitoneal mass remained unchanged without any
change in serum tumor marker levels.



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*Source: 419059-2009-01-29.xml*