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CASE REPORT
Ahead of print publication  

Paratesticular epithelioid sarcoma: A rare case


1 Department of Radiation Oncology, VMMC and Safdarjung Hospital, New Delhi, India
2 Department of Pathology, VMMC and Safdarjung Hospital, New Delhi, India

Date of Submission23-Jan-2022
Date of Decision25-Jan-2022
Date of Acceptance15-Feb-2022
Date of Web Publication24-Aug-2022

Correspondence Address:
Aashita ,
Department of Radiation Oncology, VMMC and Safdarjung Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jrcr.jrcr_8_22

  Abstract 

Primary soft-tissue sarcomas of the paratesticular region are uncommon tumors comprising 1% of all adult sarcomas. Paratesticular epithelioid sarcoma (ES) is a rare subtype. Here, we report the case of a 68-years-old male with scrotal swelling who underwent high inguinal exploration and right orchidectomy. Histopathology and immunohistochemistry revealed paratesticular ES. Very few cases of paratesticular ES have been reported so far in the literature. Clinical presentation, investigations, treatment interventions, and prognosis have been discussed. As it can be confused with other benign and malignant conditions, diagnosis is often made on histopathological evaluation.

Keywords: Case report, epithelioid sarcoma, paratesticular sarcoma, proximal variant



How to cite this URL:
Aashita, Sharma R, Yadav V, Divya T, Kaur N. Paratesticular epithelioid sarcoma: A rare case. J Radiat Cancer Res [Epub ahead of print] [cited 2022 Dec 4]. Available from: https://www.journalrcr.org/preprintarticle.asp?id=354443


  Background Top


Paratesticular tumors (PTTs) are less commonly encountered and account for 7%–10% of all intrascrotal tumors. PTT can originate from spermatic cord, epididymis, and testicular tunica. Seventy-five percent of cases originate from spermatic cord, however, usually the exact origin cannot be differentiated.[1] PTT can be benign or malignant. Although only 30% of paratesticular masses are malignant, 90% of them are sarcoma. Genitourinary sarcomas are 2.7% of all sarcomas but specifically paratesticular sarcoma (PTS) accounts to only 1% of all adult sarcoma.

PTS can occur at any age with a median age of 52 years. Ninety-two percent of cases present with localized disease, 5.9% with nodal involvement, and 1.9% with metastasis.[2]

Histopathologically, most of the PTS are rhabdomyosarcoma, leiomyosarcoma, or liposarcoma.[3] Epithelioid sarcoma (ES) has been rarely reported. Here, we report the fourth case of paratesticular ES in the literature to the best of our knowledge and have compared it to the previous reported cases [Table 1].
Table 1: Comparison of paratesticular epithelioid sarcoma cases reported in the literature

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  Case Report Top


A 68-year-old male with no known comorbidities presented with right scrotal swelling for 6 months, which was associated with pain for the last 2 months. On examination, it was a 10 cm × 10 cm well-defined hard growth in right scrotum with normal overlying skin. Penis was deviated to the left side. No inguinal or supraclavicular lymph node (LN) was palpable. Ultrasonography (USG) revealed a 10 cm × 12 cm right paratesticular mass pushing the right testis medially, with areas of necrosis and right epididymis not visualized separately. The right testis measured 47 mm × 27 mm × 26 mm and the left testis measured 45 mm × 37 mm × 30 mm. Serum lactate dehydrogenase was 404 U/mL, alpha-fetoprotein‒7.2, and β-human chorionic gonadotropin (β-HCG)‒0.12.

High inguinal exploration and right orchidectomy were done. Intraoperatively, there was a 12 cm × 8 cm right testicular mass densely adherent to cremasteric fascia and muscle with no LN palpable. Histopathological evaluation revealed an 8 cm × 7 cm × 4.5 cm size gray-white tumor pushing normal testis to one side. Sections showed tumor comprising of large round to polygonal cells arranged in sheets with areas of necrosis and extensive lymphoplasmacytic infiltration [Figure 1]a. Mitotic count of 1–2/high-power field was seen. Tumor was reaching up to the resected margin. On immunohistochemistry (IHC), tumor was positive for vimentin [Figure 1]b and focally positive for epithelial membrane antigen [Figure 1]c but negative for PLAP, β-HCG, OCT3/4, CD 34, CD68, CD117, Pan-CK [Figure 1]d, LCA, desmin, myogenin, myoD1, WT-1, calretinin, S-100, HMB-45, and DOG-1. Postoperative magnetic resonance imaging (MRI) revealed no residual disease.
Figure 1: (a) Tumor comprised round to over polygonal cells arranged in sheets with lymphoplasmacytic infiltration. (b) Tumor cells are positive for vimentin. (c) Tumor cells are positive for epithelial membrane antigen. (d) Tumor cells are negative for pan-CK. Scale bar -50μm

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Histology and IHC are suggestive of ES of paratesticular area. The patient has been planned for adjuvant chemotherapy with doxorubicin and ifosfamide followed by adjuvant radiation to postoperative site.


  Discussion Top


ES is a rare aggressive type of soft-tissue sarcoma that can present as either of the two variants-distal and proximal. Distal type is the conventional form with an indolent superficial tumor presenting as slow-growing painless nodules in limbs. Proximal type usually presents in older age than distal variant is more aggressive and commonly found in pelvis, perineum, and genital tract. They can also be differentiated on cytology as distal variant has mixed proliferation of eosinophilic epithelioid and spindle cells with minimal nuclear atypia whereas proximal variant has large neoplastic cells with more pleomorphism and multinucleated cells.[4] Differential diagnosis include granulomatous inflammation, synovial sarcoma, melanoma, squamous cell carcinoma, and adenocarcinoma. More recurrence and metastasis have been reported for proximal variant.[5],[6]

Patients present with painless inguinoscrotal swelling. USG is highly sensitive investigation to differentiate between intratesticular and extratesticular malignancies. When USG gives equivocal results, it should be confirmed with MRI as it would help to clarify the local extent of disease as well as the status of retroperitoneal LN (RPLN).[8]

Treatment is adequate surgery by radical inguinal orchidectomy with high ligation of the spermatic cord.[9] Hemiscrotectomy with wide clear margins has also been recommended and is necessary if scrotal skin is involved. If retroperitoneal lymphadenopathy is present, then RPLN dissection is done. Incomplete surgery increases the chance of local recurrence and distant metastasis. In a series of 51 patients of PTS, only 9.8% of cases had undergone upfront hemiscrotectomy.[2],[10] Most of the cases are misinterpreted as a benign lesion, as it is only intraoperatively or after pathological report that suspicion/confirmation for malignancy occurs, hence, there is an increased chance of incomplete surgery and positive margins with need for re-excision. Twenty-one percent of patients subsequently present with metastasis. Thus, the most important and effective step for treating PTS is adequate surgery.[11] Radiation can be used preoperatively for extensive local disease or postoperatively for positive margins or local recurrence.[2] Role of adjuvant chemotherapy in PTS is yet to be fully established.[8] PTS of all grades are at high risk of local recurrence at a rate of 25%–37%.[12]

Unfavorable prognostic factors are metastasis at presentation, large tumor size, high tumor grade, incomplete resection, and positive margin.[13] As the recurrence is frequently seen and can occur early or late after completion of treatment, long-term follow-up has to be done in PTS and should include clinical examination and radiological investigation if needed.


  Conclusion Top


Due to the rarity of PTS, there are no standard recommendations. The presence of irreducible paratesticular mass should raise suspicion for malignancy which should be confirmed with necessary investigations followed by adequate surgery and adjuvant therapy. ES is rare and can be confused with various benign and malignant conditions. Histopathological examination and IHC are essential to make accurate diagnosis and thus decide for adjuvant treatment. Long-term follow-up of cases is recommended.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and accompanying images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Paul PA, Calton N, Arnestina S, Mammen KJ. Paratesticular tumors. A clinicopathological study from a single tertiary hospital in North India. Ann Diagn Pathol 2021;50:151658.  Back to cited text no. 1
    
2.
Goldberg H, Wong LM, Dickson B, Catton C, Yap SA, Alkasab T, et al. Long-term oncological outcomes of patients with paratesticular sarcoma. BJU Int 2019;124:801-10.  Back to cited text no. 2
    
3.
Matias M, Carvalho M, Xavier L, Teixeira JA. Paratesticular sarcomas: Two cases with different evolutions. BMJ Case Rep 2014;2014:bcr2014205808.  Back to cited text no. 3
    
4.
Stephen NS, Gochhait D, Dehuri P, Subramanian H, Goldar RS. Cytomorphology of proximal epithelioid sarcoma in the paratesticular location. Cytopathology 2018;29:386-9.  Back to cited text no. 4
    
5.
Cardillo M, Zakowski MF, Lin O. Fine-needle aspiration of epithelioid sarcoma: Cytology findings in nine cases. Cancer 2001;93:246-51.  Back to cited text no. 5
    
6.
Pol JN, Nisar Z, Phadke MD, Kadkol GA. Epithelioid sarcoma of paratesticular region: A case report of an unusual case. Indian J Pathol Microbiol 2019;62:306-9.  Back to cited text no. 6
  [Full text]  
7.
Onol F, Tanıdır Y, Kotiloğlu E, Bayramiçli M, Turhal S, Türkeri L. Proximal type epithelioid sarcoma of the scrotum: A source of diagnostic confusion that needs immediate attention. Eur Urol 2006;49:406-7.  Back to cited text no. 7
    
8.
Keenan RA, Nic An Riogh AU, Stroiescu A, Fuentes A, Heneghan J, Cullen IM, et al. Paratesticular sarcomas: A case series and literature review. Ther Adv Urol 2019;11:1756287218818029.  Back to cited text no. 8
    
9.
Floyd MS Jr., Omar AM. Letter to the editor re: Paratesticular sarcomas: A case series and literature review: Keenan, et al. Ther Adv Urol 2019;11:1756287219847098.  Back to cited text no. 9
    
10.
Ap Dafydd D, Messiou C, Thway K, Strauss DC, Nicol DL, Moskovic E. Paratesticular sarcoma: Typical presentation, imaging features, and clinical challenges. Urology 2017;100:163-8.  Back to cited text no. 10
    
11.
Dotan ZA, Tal R, Golijanin D, Snyder ME, Antonescu C, Brennan MF, et al. Adult genitourinary sarcoma: The 25-year Memorial Sloan-Kettering experience. J Urol 2006;176:2033-8.  Back to cited text no. 11
    
12.
Küçük Ü, Bayol Ü, Pala EE, Akman Ö, Divrik RT. Paratesticular sarcomas: Our case series. Turk J Urol 2013;39:84-9.  Back to cited text no. 12
    
13.
Gatto L, Del Gaudio M, Ravaioli M, Cescon M, Tonini V, Cervellera M, et al. Paratesticular mesenchymal malignancies: A single-center case series, clinical management, and review of literature. Integr Cancer Ther 2020;19:1534735419900554.  Back to cited text no. 13
    


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