Testicular desmoplastic small round cell tumor: a case report and review of literature
World Journal of Surgical Oncology
Testicular desmoplastic small round cell tumor: a case report and review of literature
Gui-Ming Zhang 0 2
Yao Zhu 0
Hua-Lei Gan 1
Ding-Wei Ye 0
0 Department of Urology, Fudan University Shanghai Cancer Center , No.270, Dongan Rd, Shanghai 200032 , China
1 Department of Pathology, Fudan University Shanghai Cancer Center , Shanghai , China
2 Department of Oncology, Shanghai Medical College, Fudan University , Shanghai , China
Background: Desmoplastic small round cell tumor (DSRCT) is an uncommon and highly aggressive malignancy with undetermined histogenesis and poor prognosis. To date, no case of testicular DSRCT has been reported in the literature. Case: A 42-year-old Chinese man presented with painless swelling of his left testis and a painless palpable nodule in his left inguinal region. Computed tomography showed a solid mass in the left testis and multiple metastases in the body. Laboratory tests gave no abnormal results. Left radical orchiectomy was performed, and histopathological and molecular pathological examination showed typical features of DSRCT. Six cycles of chemotherapy were administrated after the operation, leading to partial remission. Postoperative 9-month follow-up indicated no progression.
Desmoplastic small round cell tumor; Testis; Immunohistochemistry; Chemotherapy
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Background
Desmoplastic small round cell tumor (DSRCT) is an
extremely rare malignancy with undetermined histogenic
origin. To date, about 300 cases have been reported in the
literature, since it was first described as a mesenchymal
entity with distinct clinicopathological features by Gerald
and Rosai in 1989 [1,2]. DSRCT is prone to develop in
adolescents and young adults, and the incidence in males
is more than three times that in females [3]. The majority
of DSRCTs originate from the abdominal cavity and pelvis,
thus the retroperitoneum, omentum, and mesentery are
often involved, in addition to multiple peritoneal tumors
[4]. Although rarer, invasion of extraperitoneal sites, such
as the central nervous system, nasal sinus, lung, bone
and soft tissues, kidney, ovary and paratesticular region,
has been documented [5-11]. However, no DSRCT derived
from testis has been reported to date. Herein, we report
a case of solid neoplasm in testis that was confirmed
pathologically to be testicular DSRCT.
Case presentation
A 42-year-old Chinese man was admitted to the
Department of Urology at our hospital, with an 8-month history
of painless swelling of his left testis and a 6-month history
of a painless palpable nodule in his left inguinal region. He
had no relevant personal or family history of malignancy.
On physical examination, an egg-size solid lump was
found in the left testis, and a palpable, slightly mobile,
solid nodule, 2 3 cm in size, in the left inguinal region.
Laboratory tests, including -fetoprotein, -human
chorionic gonadotropin, and lactate dehydrogenase, gave
no abnormal results. Computed tomography (CT) showed
a confined solid mass, 2 3 cm in size with homogenous
attenuation in the left testis, and multiple lesions in the
right diaphragmatic crus, retroperitoneal region, and
peritoneal and pelvic cavity, in the left inguinal region, and
in the near-bilateral iliac vessels, suggestive of multiple
metastases (Figure 1). In addition, the patients left renal
pelvis and ureter were dilated.
The preoperative diagnosis was left testicular tumor
with multiple metastases. Left radical orchiectomy was
carried out, and the cut surface of the tumor showed
fish-like changes. Histopathological examination of the
resected specimen revealed a malignant tumor composed
of well-defined nests of small round blue cells, which were
separated by abundant desmoplastic stroma (Figure 2).
The spermatic cord and epididymis were also involved.
Immunohistochemical evaluation exhibited the following:
Cytokeratin Pan (+), Epithelial Membrane Antigen (+),
Cytokeratin 7(), vimentin (+), desmin (+) (Figure 3),
Inhibin (/+), Chromogranin A (weakly +), synaptophysin
Figure 1 Computed tomography showed multiple lesions in left
inguinal region, and near-bilateral iliac vessels, suggestive of
multiple metastases.
(+/), CD56 (), Placental ALK. Phosphatase (/+),Wilms
Tumor-1 (WT-1) protein (), Myogenin (/+), MyoD1 ()
and Neuron-specific enolase (+). Molecular evidence of
t(11;22) (p13;q12) was detected by fluorescent in situ
hybridization (FISH). Based on the abovementioned
evidence, a diagnosis of testicular DSRCT was confirmed
(pT3 N2 M1 S0).
The patient was discharged on the fifth postoperative
day in a good condition. In the third postoperative week,
he was readmitted to the Department of Oncology at
our hospital and received the VAC multi-agent systemic
chemotherapy regimen consisting of vincristine (2 mg,
day 1), adriamycin (75 mg/m2, day 1) and
cyclophosphamide (1.2 g/m2, day 1). After six cycles of chemotherapy,
the patient was appraised as being in partial remission.
Postoperative 9-month follow-u (...truncated)