Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings
Letter to the Editor

Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings

Wenying Chen1#^, Haimei Chen1#, Chang Zhao2, Sidong Xie1, Jie Qin1, Weimin Liu1

1Department of Radiology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China; 2Department of Pathology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China

#These authors contributed equally to this work.

^ORCID: 0000-0001-8661-5310.

Correspondence to: Weimin Liu, Master; Jie Qin, Doctor. Department of Radiology, The Third Affiliated Hospital of Sun Yat-sen University, No. 600, Tianhe Road, Shipai Street, Tianhe District, Guangzhou 510000, China. Email: liuwm8@mail.sysu.edu.cn; qinjie@mail.sysu.edu.cn.

Keywords: Desmoplastic small round cell tumor; Wilms tumor 1 (WT1); Ewing sarcoma region 1 (EWSR1); case description

Submitted Dec 03, 2022. Accepted for publication Apr 30, 2023. Published online May 29, 2023.

doi: 10.21037/qims-22-1344

Background

Desmoplastic small round cell tumor (DSRCT) is a rare, unique, and highly malignant tumor that tends to occur in children and adolescents. There is an obvious gender preference, with a male to female ratio reaching 5 to 1 (1,2). It occurs predominantly in the abdominal pelvic cavity, spreads along the peritoneal surface, and very rarely appears in the lungs (3), salivary glands (4), heart (5), colon (6), bones (7) and gonads (8,9). DSRCT has an occult onset and rapid disease progression and is susceptible to implantable spread and blood and lymphatic metastasis, which most often occurs in the liver, followed by the lungs, lymph nodes, and bones (10). In this study, we retrospectively analyzed the two cases of patients with DSRCT admitted to our hospital from October 2016 to October 2021 and referred to the relevant literature in recent years, with the aim of improving the understanding of their clinical and imaging characteristics.

Case presentation

Clinical history

Case 1

A 9-year-old boy touched a mass in his abdomen 2 weeks prior to attending The Third Affiliated hospital of Sun Yat-sen University but complained of no other discomfort. Multiphase contrast-enhanced computed tomography (CT; Toshiba CT Aquilion; 64 slices; slice thickness, 3 mm) of the abdomen revealed a large mass in the left kidney, measuring approximately 129 mm × 95 mm × 161 mm, with heterogeneous density and multiple calcifications within it, showing significant heterogeneous enhancement. There were also multiple metastases in the abdominal lymph nodes and the liver (Figure 1). Thereafter, the patient also underwent whole-body fluorine 18 fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT), which showed a large left renal mass with radioactive concentration and a maximum standardized uptake value (SUVmax) of approximately 10.2, which was considered malignant. Lymph node metastases were also found in the left supraclavicular fossa and upper mediastinum (Figure 2). During his hospitalization, the patient underwent a left-sided nephrectomy and microwave ablation of liver metastases, after which he underwent a series of chemotherapy treatments.

Figure 1 CT Results for case 1. (A) The image suggested a large mass in the left kidney compressing surrounding structures. The plain CT value of the solid component of the mass was about 36 HU, and the patchy calcification could be seen in the mass (right red arrow). In addition, the metastatic retroperitoneal lymph nodes were associated with calcification (left red arrow). (B) Extensive calcification and local necrosis were found in other layers of the mass (red arrow). (C) A contrast-enhanced scan showed uneven enhancement. The CT value after the enhanced scan was about 50–67 HU. (D) Multiple metastases in the liver (arrows). CT, computed tomography.
Figure 2 On PET-CT fusion images, the metabolic activity of the left kidney tumor (red arrows) was significantly increased, with an SUVmax of 10.2. In addition, multiple metastases in the liver and lymph node metastases around the lesion, abdominal cavity, mediastinum, and left supraclavicular fossa were detected, and radioactive concentrations were seen within them. PET, positron emission tomography; CT, computed tomography; SUVmax, maximum standardized uptake value.

Case 2

A 66-year-old man was admitted to hospital after a physical examination revealed an abdominal mass for more than 2 weeks. He underwent an abdominal CT examination, which showed multiple intra-abdominal masses that were poorly demarcated from the bowel and revealed multiple lymph node metastases between the mesentery, as well as metastases in the splenic hilum and liver (Figure 3). Subsequently, a puncture biopsy of the mass was performed. After a period of symptomatic management, the patient voluntarily requested to be discharged.

Figure 3 CT Results for case 2. (A,B) Irregular soft tissue masses (asterisks) were seen in the pelvic cavity, with uneven density and an indistinct boundary with the adjacent bowel. The plain CT value of the mass was about 24–29 HU, and the lesion showed moderate enhancement after the enhanced scan, with the CT value range of about 49–54 HU. (C) Coronal CT showed that the tumors (asterisk) were mainly located in the lower abdomen to the pelvic region. Multiple metastases were apparent in the liver, right diaphragm, and the mesenteric space (red arrows). CT, computed tomography.

Pathology

Two experienced pathologists (5 and 10 years of work experience) read the slides. The tumor sections of these 2 patients were grayish-yellow in color and soft to medium in texture. The pathological sections were stained with hematoxylin and eosin and observed under 100× and 200× microscopy, respectively. Microscopically, the small round tumor cells formed nests and sheets in the desmoplastic stroma, significant interstitial fibrous tissue hyperplasia with glassy degeneration, and occasionally chrysanthemum-like structures with visible patchy necrosis (Figure 4). Immunohistochemical results indicated that the tumor cells diffusely expressed cytokeratin (CK), vimentin, and desmin (paranuclear point), and epithelial membrane antigen (EMA; focal+) was positive in both cases, while neuron-specific enolase (NSE), MyoD1, myogenin, CD99, S100, CD3, CD20, and transducer-like enhancer of split 1 (TLE1) were all negative. In addition, genetic testing of the tumor was performed in case 1, which demonstrated a broken recombination of the Ewing sarcoma region 1 (EWSR1) gene and a positive fluorescence in situ hybridization (FISH) test. Overall, the final diagnosis was DSRCT.

Figure 4 HE staining was performed on pathological sections. (A) The tumor cells appeared distributed in a nested or sheet-like pattern with sparse cell cytoplasm (HE 100×). (B) Tumor cells with sparse cytoplasm, visible nuclei, and involvement of nerve bundles (HE 200×). HE, hematoxylin and eosin.

Follow-up

In case 1, the child began follow-up from July 16, 2021, which is continuing as of time of writing, and no definite tumor recurrence has been observed on abdominal CT examination for more than 1 year. Unfortunately, for case 2, no follow-up data have been obtained.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki (as revised in 2013). Verbal informed consent was obtained from the parent or legal guardian of the case 1 patient, and written informed consent was obtained from the case 2 patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Desmoplastic small round cell tumor (DSRCT), first discovered in 1989 by Gerald and Rosai, is a rare malignancy in the small round cell family (11). DSRCT often occurs in adolescents and young men, the peritoneal cavity is the most common site, and usually spreads along the peritoneal surface (12,13). Clinical symptoms are nonspecific, mainly manifest as abdominal pain, bloating, or palpable abdominal masses (10,12). Most (~90%) cases of DSRCT are associated with diffuse or multifocal large omental and peritoneal lesions. Diaphragmatic involvement and retroperitoneal involvement are seen in 40% to 50% of cases, mainly in the form of tumor implantation and lymph node metastases, with additional liver metastases being relatively common (>30%) (10). In case 2, the patient presented with multiple abdominal masses and combined abdominal lymph nodes and liver metastases, which are consistent with the literature mentioned above. Moreover, this was an older adult patient, an age group in which DSRCT is rarer. In recent years, a small number of studies have compared adolescents and adults (≥18 years old) with DSRCT, but no significant differences in clinical manifestations, treatment methods, or prognoses between the 2 groups were found (14,15). The primary renal DSRCT was first reported in 2004 by Su et al. (16). Thus far, 19 cases of primary renal DSRCT have been reported in the literature (13,17), so we report our case 1 as the 20th case (Table 1). When it occurs in visceral organs (such as the kidney), it is radiologically and pathologically similar to other malignancies, making its diagnosis markedly challenging.

Table 1

Literature review of desmoplastic small round cell tumor of the kidney

Authors Cases (no.) Age (years) Sex History Treatment F-UP (mo) Outcome
Su et al. 1 41 Male None Radical nephrectomy 18 Alive with no evidence of disease
Egloff et al. 2 6 Female Gross hematuria after abdominal trauma Radical nephrectomy; adjuvant chemotherapy 9 Alive with no evidence of disease
Eaton and Cendron 3 7 Female Intermittent gross hematuria following a fall Radical nephrectomy 12 Alive with no evidence of disease
Collardeau-Frachon et al. 4 14 Female Gross hematuria, fever, abdominal mass Chemotherapy, radical nephrectomy 8 Alive with evidence of disease (lung and liver metastasis)
Wang et al. 5 7 Female Not stated Nephrectomy, chemotherapy 24 Alive with no evidence of disease
Wang et al. 6 6 Female Not stated Nephrectomy, chemotherapy 32 Pulmonary metastasis at 32 mo with subsequent chemotherapy and stem cell transplant; disease-free 12 mo later
Wang et al. 7 6 Female Not stated Nephrectomy, chemotherapy 22 Alive with no evidence of disease
Wang et al. 8 8 Male Not stated Nephrectomy, chemotherapy 20 Recurrent intra-abdominal and hepatic disease at 20 mo with subsequent chemotherapy; currently stable
Da Silva et al. 9 10 Male Abdominal pain, hematuria Radical nephrectomy, radiation therapy, chemotherapy 12 Alive with disease (liver, lung, and bone metastasis)
Janssens et al. 10 14 Female Pain, abdominal mass Clinical Dx: WT, RCC; no surgery Not stated Not stated
Popov et al. 11 14 Not stated Not stated Not stated Not stated Not stated
Tagarelli et al. 12 21 Female Not stated Nephrectomy, chemotherapy/radiation therapy 31 Alive with disease (peritoneal metastasis)
Rao et al. 13 20 Male Not stated Nephrectomy 24 Dead of disease
Eklund et al. 14 7 Male Gross hematuria, back pain Nephrectomy, chemotherapy Not stated Not stated
Walton and Flores 15 6 Male Pain related to facial bone metastasis Nephrectomy, chemotherapy Not stated Not stated
Baydar et al. 16 7 Female Abdominal pain Chemotherapy, nephrectomy 30 Dead of disease
Galliani et al. 17 15 Male Gross hematuria, mid-back to left flank pain Radical nephrectomy, chemotherapy/radiation therapy 48 Dead of disease
Liu and Ya 18 40 Female Cough, sputum, dyspnea Chemotherapy Not stated Not stated
Asadbeigi et al. 19 22 Male Right flank pain Radical nephrectomy Not stated Not stated
Present case 20 9 Male Palpable abdominal mass Nephrectomy, chemotherapy 14 Alive with disease (liver and spine metastasis)

mo, month; Dx, diagnosis; WT, Wilms tumor; RCC, renal cell carcinoma; F-UP, follow-up.

Conventional imaging modalities such as CT and magnetic resonance imaging (MRI) can provide good morphological information for DSRCT, while CT scanning is the most widely used and preferred diagnostic modality for initial diagnosis and follow-up evaluation (12,18). The characteristic imaging finding of DSRCT is multiple soft tissue masses in the abdomen and pelvis involving the omentum or mesentery without an exact organ of origin (1,2). The multiple larger nodular or lobulated masses often appear with heterogeneous density, similar to necrosis, cystic changes, and hemorrhage, displaying areas of nonenhancement. Punctate or amorphous calcifications may be observed in both the primary tumor and metastases (1,2,12,19). As described, numerous foci of calcification were observed on DSRCT of the left kidney in case 1 as well as within the intra-abdominal metastases. Weak contrast enhancement in some areas may be due to the fibrous component of the tumor (19). MRI helps to determine the extent of the disease (2). On MRI, DSRCT has been reported to exhibit inhomogeneous isointensity or hypointensity on T1-weighted images compared to skeletal muscle and inhomogeneous hyperintensity on T2-weighted images (2,7,12). Nonetheless, some solid components of tumor show relative hypointensity on T2-weighted images, possibly due to dense cellular and desmoplastic response (20).

PET-CT usually shows multiple intraperitoneal hypermetabolic masses with a variable SUVmax of the masses, often up to 12, indicating a strong hypermetabolic trend (10). PET-CT may be superior to other imaging modalities in detecting occult lesions and monitoring recurrence due to the advantages of functional imaging (1,12,21). The PET-CT scan of case 1 clearly revealed distant left supraclavicular fossa and mediastinal lymph node metastases in addition to CT-visible intra-abdominal metastases. This helped to determine the extent of the lesion and clinical staging.

The diagnosis of DSRCT relies on pathology and immunohistochemistry. Histopathological examination demonstrates nests, cords, and sheets of small round cells embedded in the desmoplastic stroma. Immunological phenotyping shows the multidirectional differentiation of tumor cells, which can express a variety of immunophenotypes including epithelial (EMA and keratin), neural (NSE), and mesenchymal (vimentin and desmin) markers (20,22). The human Wilms tumor gene (WTI) was originally discovered as a tumor-suppressor gene, and it may play an important role in angiogenesis, the occurrence of metastasis, and the suppression of immune responses. Recent studies have found that WTI has potential carcinogenic effects in a variety of malignant tumors. DSRCT is characterized by chromosomal translocations that result in gene fusion involving EWSR1 and Wilms tumor 1 (WT1) (13,23). Therefore, the EWS-WT1 fusion gene has high sensitivity and specificity for the diagnosis of DSRCT (24). The molecular test results of case 1 demonstrated a broken recombination in the EWSR1 gene, which helped to confirm the diagnosis of DSRCT.

The differential diagnosis of DSRCT is challenging, as diffusely spreading tumors such as desmoid tumors, malignant peritoneal mesothelioma, lymphoma, peritoneal sarcoma, gastrointestinal mesenchymal tumors, and Castleman disease can mimic DSRCT (25). However, they generally develop at an older age (19). In addition, malignant mesothelioma may involve a history of asbestos exposure; tends to occur in the pleura, peritoneum, and greater omentum; and is often associated with thoraco-abdominal fluid. Lymphoma is often associated with greater retroperitoneal lymph node enlargement but rarely calcification; meanwhile, desmoid tumor is a benign proliferation of fibrous tissue of the musculotendinous membrane that occurs mainly in women (26,27). Renal DSRCT in children needs to be differentiated from other renal malignancies, including Wilms tumor, rhabdoid tumor, and clear cell sarcoma. Clear cell sarcoma and rhabdoid tumor show almost no calcification. Although <15% of Wilms tumors in children may have calcifications, these calcifications are usually curvilinear in appearance and can be distinguished from the patchy and speckled calcifications found in DSRCT (28). In addition, the incidence of Wilms tumor and renal clear cell sarcoma peaks between 2 and 3 years of age, and rhabdoid tumors occur in infancy; however, in the bulk of the reports on pediatric renal DSRCT, this occurs above 6 years old, and thus this epidemiological feature can also assist in identification (29,30).

At present, there is no standard treatment protocol for DSRCT, and complete surgical resection of DSRCT is crucial when technically feasible (13). For most patients in advanced stages, effective systemic chemotherapy helps in systemic control (31). Other studies have shown that hyperthermic intraperitoneal chemotherapy (HIPEC) after cytoreductive surgery (CRS) is an effective local control method and can improve the survival rate of patients with DSRCT (32,33). Despite advances in multimodal therapy, patients with DSRCT have a poor prognosis, with about 60–70% dying from the disease within 2–3 years (34,35).

Conclusion

DSRCT is a rare disease commonly seen in children or adolescents with certain radiological features. By integrating morphology, immunohistochemistry, and molecular genetics, DSRCT can be accurately diagnosed. Despite aggressive treatment, the outcome remains poor. Clinical vigilance, identification, and multimodal therapy are required to improve patient prognosis.

Acknowledgments

Funding: This work was supported by National Natural Science Foundation Cultivation Project of the Third Affiliated Hospital of Sun Yat-sen University (No. 2021GZRPYMS06) and Five-Five Project of the Third Affiliated Hospital of Sun Yet-Sen University (No. 2023WW605).

Footnote

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-22-1344/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Verbal informed consent was obtained from the parent or legal guardian of the case 1 patient, and written informed consent was obtained from the case 2 patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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Cite this article as: Chen W, Chen H, Zhao C, Xie S, Qin J, Liu W. Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings. Quant Imaging Med Surg 2023;13(7):4762-4769. doi: 10.21037/qims-22-1344

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