Human Pathology: Case Reports 11 (2018) 47–50

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Human Pathology: Case Reports

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Case Report

Low-grade serous carcinoma (mullerian/ovarian type) of the paratestis T

presenting as diffuse metastatic disease of unknown origin: Case report of an
uncommon tumor with an unusual presentation
Alexander Filatenkov (MD)1, Amanda Strickland (MD)1, Matthew Karpowicz (DO),
Franto Francis (MD PhD)
⁎

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA

ABSTRACT

Low-grade (papillary) serous carcinoma of ovarian type is rare in males and histologically identical to low-grade serous carcinomas in female patients. We present a
case of paratesticular low-grade serous carcinoma in a 42 year old male, with the highly unusual initial presentation as diffuse metastatic disease in the abdomen.
Imaging revealed a cystic lesion of the right testis/hemiscrotum. Biopsy of the abdominal metastasis and subsequent right orchiectomy specimen showed invasive
papillary serous carcinoma, low grade, with prominent psammomatous calcifications. Immunohistochemically, both tumors were positive for PAX8, WT-1, CA-125
and cytokeratin CK7, and negative for mesothelioma markers (calretinin, D2-40) and CD10. The main differential diagnoses for this tumor include mesothelioma of
tunica vaginalis, adenocarcinoma of rete testis/epididymis and metastases. Although rare as an initial presentation, ovarian type serous carcinoma of the paratestis
should be included in the differential diagnosis in males with abdominal metastases that show papillary architecture, psamommatous calcifications and PAX8
immunoreactivity.

1. Introduction immunohistochemical workup further identified the tumor as analo-

gous to low grade type of papillary serous carcinoma of the ovary.
Low-grade (papillary) serous carcinoma of ovarian type is rare in
the testis and paratestis. The tumor is histologically identical to serous 2. Materials and methods
carcinoma arising in the ovary, and uses the same descriptive nomen-
clature. There have been < 40 cases described in the literature, and the 2.1. Case report
majority have been locally confined tumors of either borderline type
with non-invasive histology, or showing focal invasion in a background A 42 year old undocumented male immigrant with no significant
of predominantly borderline histology. On follow-up, the majority of medical history presented to clinic at the county hospital, complaining
these tumors have not recurred after treatment, with very rare cases of constant, pressure like left flank abdominal pain. He also
showing distant metastasis on long term follow-up [2]. Most of these reported unintentional 20 lbs. weight loss over the previous six months
cases present as hydrocele and/or testicular swelling and the mean age and recently noticed a mass near his right testicle. No elevation of
of presentation for invasive tumors is 31 years (range: 16–42 years) serum markers for testicular germ cell tumors was detected on blood
[1–3]. testing.
We report what we believe is the first case of this rare tumor pre- Testicular ultrasound showed an extratesticular cystic structure
senting initially with widespread abdominal metastases. Because of the with peripheral nodularity on the right side, without apparent central
rarity of this tumor in males and absence of reported initial metastatic vascularity. At this point the differential diagnosis included posttrau-
presentation, pathologists need to be cognizant of this entity when matic/infectious hydrocele or a large exophytic epididymal cyst.
evaluating metastases of unknown origin. Once the entity is included in Computed tomography (CT) revealed multiple nodules in the chest/
one's differential diagnostic consideration, it is relatively easy to iden- pleura, partially calcified masses in the abdomen, low-density lesions in
tify because of the tumor's distinctive features (histomorphology, the liver and malignant ascites. Additionally, a partially calcified and
psammoma bodies, immunohistochemistry). In our case, cystic lesion was noted in the right hemiscrotum (Fig. 2A).

⁎
Corresponding author: Department of Pathology, 5201 Harry Hines Blvd., Dallas, Texas 75235, USA.
E-mail addresses: [email protected] (A. Filatenkov), [email protected] (A. Strickland),
[email protected] (M. Karpowicz), [email protected] (F. Francis).
1
These authors contributed equally to this work.

https://doi.org/10.1016/j.ehpc.2017.10.003
Received 10 August 2017; Received in revised form 11 October 2017; Accepted 19 October 2017
2214-3300/ © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/BY-NC-ND/4.0/).
A. Filatenkov, et al. Human Pathology: Case Reports 11 (2018) 47–50

Portions of the tunica vaginalis cyst lining away from the invasive
tumor showed serous borderline tumor morphology, i.e. complex
branching architecture and epithelial atypia, but without stromal in-
vasion (Fig. 3). The tumor appeared to arise multifocally in the para-
testicular region, from within the tunica vaginalis cyst and adjacent
testiculo-epididymal groove region. The tumor extended into the epi-
didymis, tunica albuginea and focally involved testicular parenchyma
and hilar fat. Tumor cells were immunoreactive for PAX8, WT-1, es-
trogen receptor, cytokeratin 7, Ber-Ep4 and CA-125 stains, and negative
for mesothelioma markers (calretinin & D240) as well as for CD10
immunostain, among other markers. Patchy to focal immunoreactivity
was seen with p53 immunostain, i.e. “wild type” of p53 staining pat-
tern, analogous to low grade type of papillary serous carcinoma in the
ovary. Overall, the histomorphology and immunoprofile of the tumor
were consistent with invasive, low-grade papillary serous carcinoma of
mullerian/ovarian type arising in the paratestis, and morphologically
similar to the lesion seen in the abdominal biopsy.

Fig. 1. CT guided needle biopsy of abdominal lesion revealed invasive carci- 2.2. Follow-up
noma with papillary architecture and prominent psammoma bodies in a dense
fibrous stroma. Following orchiectomy and diagnosis, the patient was scheduled for
clinic visit to discuss initiation of chemotherapy, but did not show up.
At two years following surgery, the patient had still not presented for
CT guided needle biopsy of an abdominal lesion was performed and
continuation of care.
revealed an invasive carcinoma with papillary architecture and pro-
minent psammoma bodies within dense fibrous stroma (Fig. 1). Im-
3. Discussion
munohistochemical staining of this tumor showed positivity for PAX-8,
WT-1 and cytokeratins CK7 and AE1/AE3, and non-reactivity with TTF-
In this case report, we report an uncommon tumor of the testis/
1, CDX2, PSA as well as D2-40 and calretinin stains. The case was
signed out as metastatic low-grade serous carcinoma with psammoma paratestis - ovarian-type serous carcinoma- with a highly unusual initial
presentation: widespread abdominal metastases. The majority of
bodies, with a note that immunohistochemistry argued against the
possibility of a lung, colon, prostate or pleural primary. ovarian type serous tumors of the paratestis/testis reported in the lit-
erature have been non- invasive, and none presented initially with
Because imaging suggested the likelihood of a paratesticular/testi-
widespread metastases. Serous carcinomas of ovarian type are capable
cular lesion, a right radical orchiectomy was performed shortly after the
of metastatic spread, but they typically present as testicular hydrocele,
biopsy (Fig. 2B). A 70.5 g orchiectomy specimen was received, com-
or testicular fullness or mass [1,4]. The mean age of presentation for
prising of the testis, epididymis and spermatic cord. On opening the
invasive tumors is 31 years (range: 16–42 years). Ovarian type epithe-
specimen, an approximately 4.0 cm cystic lesion filled with clear
lial tumors can be serous, mucinous, endometrioid, clear cell, transi-
transparent fluid was identified within the tunica vaginalis, in the tes-
tional, [5,6] or squamous [2,7–9], with serous tumors being most
ticulo-epididymal groove region. The cyst showed multiple small tan-
common. Serous tumors can in turn be benign, borderline, or malignant
yellow papillary excrescences and hard nodules, as well as a subjacent,
(carcinoma). It has been suggested that ovarian type serous tumors in
possibly contiguous 2.0 cm tan-brown mass. The rest of the testis was
the testis/paratestis arise from either mullerian metaplasia of tunica
tan-brown and spongy with unremarkable parenchyma.
vaginalis [11] or mullerian rests in paratesticular soft tissue or the
Microscopic examination of the specimen showed an invasive car-
appendix testis [9].
cinoma with prominent papillary features and abundant psammoma
The current tumor was cystic and showed prominent papillary ar-
bodies (Fig. 3). The neoplastic papillae were lined by moderately aty-
chitecture and psammomatous calcifications, and was associated with
pical, serous cuboidal and columnar cells. The mitotic count was low
serous borderline tumor. Tumor cells showed moderate nuclear atypia,
(< 3 per 10 high power fields) and no tumor necrosis was seen.
low mitotic count, absence of tumor necrosis and wild type p53

Fig. 2. A. Partially cystic, partially calcified mass noted in right hemiscrotum on CT. B. Gross exam showed 4.0 cm tunica vaginalis cyst with subjacent ~ 2.0 cm tan-
brown mass and yellow-tan nodules in the cyst wall. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of
this article.)

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A. Filatenkov, et al. Human Pathology: Case Reports 11 (2018) 47–50

Fig. 3. Microscopic examination show tumor with invasive growth (A), papillary architecture with prominent psammoma bodies (B), lined by moderately atypical
serous cells (C). Background cyst lining showed areas of borderline morphology (D), with inset showing higher magnification.

immunohistochemistry. Overall, the findings were analogous to low Conflict of interests

grade type of serous carcinoma (LGSC) described in the ovary. In
contrast, high grade ovarian serous carcinomas show significant nuclear None.
pleomorphism, frequent mitoses and tumor necrosis, and are confirmed
by diffuse and strong p53 immunostaining in tumor cells [12]. Author contributions
The main pathologic differential diagnoses for a paratesticular
tumor such as this include malignant mesothelioma of the tunica va- AF and AS were responsible for collecting and analyzing data, and
ginalis and adenocarcinoma of the epididymis/rete testis, besides ex- wring the manuscript. MK and FF were responsible for analyzing data,
cluding distant metastasis. Mesothelioma presents as diffuse or nodular writing and editing the manuscript.
thickening of the tunica vaginalis and is easily excluded by im-
munohistochemistry as it is immunoreactive with calretinin and D2-40 References
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