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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 6  |  Issue : 3  |  Page : 108-110

Paratesticular desmoplastic small round cell tumor as an incidental finding during imaging of an obstructed inguinal hernia in an old-age patient


Department of Surgery, Al-Shiffa General Hospital, Basrah, Iraq

Date of Web Publication10-Sep-2018

Correspondence Address:
Dr. Sadq Ghaleb Kadem
Al-Shiffa General Hospital, Basrah
Iraq
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ssj.ssj_20_18

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  Abstract 

Desmoplastic small round cell tumor (DSRCT) is an extremely rare malignancy mainly located in the abdominal and pelvic peritoneal cavity. A few published studies have reported the involvement of testicular/paratesticular region. The present study describes an unusual case of paratesticular DSRCT that was discovered incidentally during imaging of an obstructed inguinal hernia in an old-age patient. After radical orchiectomy, the patient was administered treatment consisting of a multidrug combination chemotherapy and adjuvant radiotherapy and remained disease free during the 24-month followup period, with only one attachment of local tumor recurrence in the right inguinal region.

Keywords: Adjuvant chemoradiotherapy, desmoplastic small round cell tumor, immunohistochemistry, neoadjuvant chemotherapy, paratesticular


How to cite this article:
Kadem SG, Habash SM. Paratesticular desmoplastic small round cell tumor as an incidental finding during imaging of an obstructed inguinal hernia in an old-age patient. Saudi Surg J 2018;6:108-10

How to cite this URL:
Kadem SG, Habash SM. Paratesticular desmoplastic small round cell tumor as an incidental finding during imaging of an obstructed inguinal hernia in an old-age patient. Saudi Surg J [serial online] 2018 [cited 2021 Jul 30];6:108-10. Available from: https://www.saudisurgj.org/text.asp?2018/6/3/108/240911


  Introduction Top


Desmoplastic small round cell tumor (DSRCT) was first described as a distinct malignant serosa-related tumor by Gerald and Rosai in 1989.[1] It is a rare malignancy with less than 200 cases reported in the literature.[2] The majority of DSRCTs are located in the abdominal and pelvic peritoneal cavity, mainly affecting young male patients.[3] It is unusual for DSRCT to involve an extra-abdominal site such as the testicular/paratesticular region and, to the best of our knowledge, a few cases have been reported in the published studies.[4],[5],[6] The present study describes an unusual case of paratesticular DSRCT, discovered incidentally during imaging of a large obstructed right inguinal hernia in an old-age patient.


  Case Report Top


A 63-year-old male, a known case of long-standing, irreducible, right inguinal hernia, extending to the right hemiscrotum, presented to our emergency department complaining of severe pain over the site of a hernia, for few hours' duration. The condition was associated with nausea, vomiting, and constipation. There was no history of trauma or specific infection.

On examination, his body temperature was 37.6°C, the pulse rate was 100 beats per minute, and the blood pressure was 140/95 mmHg. The abdominal examination revealed mildly distended, soft abdomen with a tender, irreducible, right inguinal hernia, extending to the right hemiscrotum. His bowel sound was exaggerated. The blood investigations, including complete blood count, blood sugar, blood urea, and serum creatinine and general urine examination were normal. The scrotal ultrasound examination revealed right inguinal hernia containing an omentum and obstructed bowel loops that extend to the right hemiscrotum, and the right testis was contained within a solid heterogeneous mass (5 cm × 4 cm × 3 cm). The plain abdominal radiography revealed features consistent with small bowel obstruction.

An informed consent was taken for emergency repair of the obstructed right-sided inguinal hernia and right-sided radical orchiectomy.

On operation, the herniated small-bowel loop was viable; thus, we reduced it to the abdominal cavity, part of the herniated omentum was excised for histopathological examination, right-side radical orchiectomy was done, and the hernial defect was repaired without mesh. The postoperative course was smooth.

Regarding the histopathological examination of the excised specimen, the macroscopic examination revealed the following testis 4 cm × 5 cm × 6 cm and spermatic cord 10 cm × 2 cm × 1 cm, with a paratesticular mass attached to the tunica 5 cm × 4 cm × 3 cm; its cross section was gray and firm with foci of necrosis. The other part of the specimen consisted of a fatty mass measuring 14 cm × 10 cm × 6 cm (omentum).

The specimen was stained with hematoxylin and eosin. Microscopically, the tumors consisted of well-defined nests of small round blue cells, embedded in a densely fibrotic stroma [Figure 1]. The tumor cells have abnormal numerous mitotic figures [Figure 2].
Figure 1: Well-defined nests of small round blue cells separated by abundant desmoplastic stroma (hematoxylin and eosin stain, ×40)

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Figure 2: Numerous mitotic figures can be observed (×100)

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During the immunohistochemical examination, the tumor cells were positive for desmin (perinuclear dot like), vimentin, neuron-specific enolase (NSE), CD99, smooth muscle actin, cytokeratin, and Wilm's tumor 1 (WT1). These histological and immunohistochemical findings supported the diagnosis of a DSRCT.

No evidence of metastasis was identified on the postoperative clinical or imaging examinations. The patient was administered treatment consisting of multidrug combination chemotherapy regimen and remained disease free for about 6 months; after that, the patient developed local recurrence in the right inguinal region which was confirmed by histopathological examination after complete excision. Then, adjuvant radiotherapy has been added to the multidrug combination chemotherapy regimen. No evidence of another local recurrence or distant metastasis was identified on the clinical or imaging examinations during the 24-month followup period.


  Discussion Top


In this study, we described a case of localized paratesticular DSRCT that was diagnosed incidentally on imaging of an obstructed long-standing right-sided inguinal hernia in a 63-year-old patient. The usual presentation of the patient with a localized (DSRCT) in the testicular/paratesticular region has an age range of 17–43 years, complaining of a scrotal mass, swelling, and hydrocele, with variable pain.[4] Based on the above findings, our case may represent a typical age of presentation of this tumor, but the absence of typical symptoms and signs of paratesticular (DSRCT) in this patient may be due to the presence of the large long-standing inguinal hernia that extended to the right hemiscrotum.

On histopathological examination, DSRCT was composed of nests of small blue round cells within the desmoplastic stroma.[7] It should be differentiated from other small round tumor-like cells, Ewing's sarcoma, malignant mesothelioma, rhabdomyosarcoma, small cell carcinoma, WT, and lymphoma. A definite diagnosis may only be achieved with a demonstrated multidirectional differentiation and co-expression of epithelial, mesenchymal, and neural antigens in the same cell. The presence of perinuclear dot-like immunostaining with desmin strongly suggests a diagnosis of DSRCT.[8]

No validated staging system is in practice for (DSRCT); some centers use peritoneal carcinomatosis staging system (Peritoneal Cancer Index).[9] The 5-year survival of the patient with DSRCT is <20%; however, the primary extra-abdominal disease tends to be more localized, and patients with paratesticular tumors have a less disseminated disease and better prognosis.[4]

The survival rate may be improved using highdose multidrug combination chemotherapy followed by an aggressive surgical resection, radiotherapy, and myeloablative chemotherapy with stem cell rescue.[10]

In this study, our patient had a local tumor recurrence about 6 months after the surgical resection. This recurrence may be due to the fact that the patient did not receive any neoadjuvant chemotherapy due to the presence of obstructed inguinal hernia that necessitates emergency surgery and may be due to the delay in the addition of the adjuvant radiotherapy to the multidrug combination chemotherapy which was only added after surgical resection of local tumor recurrence.


  Conclusion Top


The paratesticular DSRCT is a rare malignant tumor, can occur in old-age patients, and tend to be localized with better prognosis. Multimodality therapies including neoadjuvant chemotherapy and then surgical resection followed by an adjuvant chemoradiotherapy may represent the best therapeutic strategy.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989;9:177-83.  Back to cited text no. 1
    
2.
Lettieri CK, Garcia-Filion P, Hingorani P. Incidence and outcomes of desmoplastic small round cell tumor: Results from the surveillance, epidemiology, and end results database. J Cancer Epidemiol 2014;2014:680126.  Back to cited text no. 2
    
3.
Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J, et al. Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 1991;15:499-513.  Back to cited text no. 3
    
4.
Cummings OW, Ulbright TM, Young RH, Dei Tos AP, Fletcher CD, Hull MT, et al. Desmoplastic small round cell tumors of the paratesticular region. A report of six cases. Am J Surg Pathol 1997;21:219-25.  Back to cited text no. 4
    
5.
Thuret R, Renaudin K, Leclere J, Battisti S, Bouchot O, Theodore C, et al. Uncommon malignancies: Case 3. Paratesticular desmoplastic small round-cell tumor. J Clin Oncol 2005;23:6253-5.  Back to cited text no. 5
    
6.
He L, Wen S, Hu X, Guo C, Yi C. Primary desmoplastic small round cell tumor of the testis: A case report and review of the literature. Oncol Lett 2013;6:565-7.  Back to cited text no. 6
    
7.
Stuart-Buttle C, Smart C, Pritchard S, Martin D, Welch I. Desmoplastic small round cell tumor: A review of literature and treatment options. Surg Oncol 2008;17:107-12.  Back to cited text no. 7
    
8.
Takekawa Y, Ugajin W, Koide H, Nishio S, Yamamoto T, Sawada T, et al. Pathologic, cytologic and immunohistochemical findings of an intra-abdominal desmoplastic small round cell tumor in a 15-year-old male. Pathol Int 2000;50:417-20.  Back to cited text no. 8
    
9.
Harmon RL, Sugarbaker PH. Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer. Int Semin Surg Oncol 2005;2:3.  Back to cited text no. 9
    
10.
Atallah V, Honore C, Orbach D, Helfre S, Ducassou A, Thomas L, et al. Role of adjuvant radiation therapy after surgery for abdominal desmoplastic small round cell tumors. Int J Radiat Oncol Biol Phys 2016;95:1244-53.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]


This article has been cited by
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