|Ahead of print publication
Paratesticular epithelioid sarcoma: A rare case
Aashita1, Rajiv Sharma1, Vikas Yadav1, T Divya1, Navpreet Kaur2
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 Submission||23-Jan-2022|
|Date of Decision||25-Jan-2022|
|Date of Acceptance||15-Feb-2022|
|Date of Web Publication||24-Aug-2022|
Department of Radiation Oncology, VMMC and Safdarjung Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
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
| Background|| |
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. 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.
Histopathologically, most of the PTS are rhabdomyosarcoma, leiomyosarcoma, or liposarcoma. 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|| |
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|| |
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. Differential diagnosis include granulomatous inflammation, synovial sarcoma, melanoma, squamous cell carcinoma, and adenocarcinoma. More recurrence and metastasis have been reported for proximal variant.,
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).
Treatment is adequate surgery by radical inguinal orchidectomy with high ligation of the spermatic cord. 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., 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. Radiation can be used preoperatively for extensive local disease or postoperatively for positive margins or local recurrence. Role of adjuvant chemotherapy in PTS is yet to be fully established. PTS of all grades are at high risk of local recurrence at a rate of 25%–37%.
Unfavorable prognostic factors are metastasis at presentation, large tumor size, high tumor grade, incomplete resection, and positive margin. 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|| |
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
Conflicts of interest
There are no conflicts of interest.
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