Desmoplastic small round cell tumor of the kidney with unusual imaging features

Eklund, Meryle J.; Cundiff, Caitlin A.; Shehata, Bahig M.; Alazraki, Adina

doi:10.1016/j.clinimag.2015.05.002

Cited by 13 publications

(10 citation statements)

References 8 publications

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“…Diffuse primary peritoneal disease accounts for the majority of cases; however, single-organ involvement may (rarely) occur. Eleven cases of renal DSRCT have been reported (78)(79)(80)(81)(82)(83)(84). With the exception of the initial case in a 41-year-old woman, all cases occurred in children, adolescents, or young adults aged 6-20 years.…”

Section: Desmoplastic Small Round Cell Tumormentioning

confidence: 99%

Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade:From the Radiologic Pathology Archives

2017

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Malignant renal tumors account for 7% of childhood cancers, and Wilms tumors are by far the most common-but not in older children and adolescents. Among individuals in the latter half of their 2nd decade of life, renal cell carcinoma (RCC) is more common than Wilms tumor. The histopathologic spectrum of RCCs in children differs from that in adults. The most common subtype of RCC in children and adolescents is Xp11.2 translocation RCC, which is distinguished by hyperattenuation at nonenhanced computed tomography, a defined capsule, and associated retroperitoneal lymphadenopathy. Papillary RCC is the second most common histologic subtype. It enhances less intensely compared with the adjacent renal parenchyma and has a propensity for calcification. Clear cell RCC is seen in patients with von Hippel-Lindau disease and is distinguished by its relatively hypervascular nature. Medullary carcinoma affects adolescents with the sickle cell trait and is characterized by an infiltrative growth pattern and extensive metastasis at presentation. Angiomyolipoma is seen in children with tuberous sclerosis complex and is often multifocal and hypervascular, with macroscopic fat. Metanephric tumors are central, circumscribed, and typically calcified. Lymphoma usually manifests as multifocal masses, but it may involve a solitary mass or infiltrative pattern. Extensive adenopathy and involvement of the gastrointestinal tract or other organs also may be seen. Primitive neuroectodermal tumor is an aggressive neoplasm that is typically quite large at diagnosis. Knowledge of the clinical, biologic, and histopathologic features of renal tumors in older children and adolescents and their effects on the imaging appearance can help the radiologist offer a useful preoperative differential diagnosis.

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Section: Desmoplastic Small Round Cell Tumormentioning

confidence: 99%

Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade:From the Radiologic Pathology Archives

2017

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“…DSRCT primary of the kidney was first described by Su, et al [4] in 2004 and since then only a total of 12 cases have been reported in the literature (Table 1) [ [5][6][7][8][9][10][11][12]. Herein, we present the thirteenth case of renal DSRCT that had variant histological features mimicking various types of other neoplasia.…”

Section: Introductionmentioning

confidence: 80%

“…Urogenital DSRCT may involve bladder, ureters, prostate and paratesticular structures [17]. Primary DSRCT of the kidney was first noted by Su, et al [4] in 2004 and 12 cases have been reported in the literature thereafter [5][6][7][8][9][10][11][12] albeit one of them has an inaccessible publication. Although male predilection is emphasized in abdominal DSRCTs, most of renal cases including ours have been female (F/M = 7/5).…”

Section: Discussionmentioning

confidence: 99%

“…Exitus at 2nd year Rao, et al, [ 10 ] Case 10 7 / M Gross hematuria, microscopic hematuria and intermittent back pain 3 years priorly Left Polypoid mass confined to the renal collecting system, extending into proximal and mid ureter, no involvement of renal parenchyma Spindled and polygonal tumor cells, rare rosettes, low mitotic rate CD99 (+), Vimentin (+), Desmin (focal+), Actin (focal+), WT1 (focal+), PAX2 (+); PAX8 (−) FISH showing EWSR1 rearrangement, karyotyping showing t(11;22) (p13;q12). Surgery + CT + RT Alive (NED) (duration unknown) Eklund et al, [ 11 ] Case 11 6 / M Facial swelling and pain, headache, decreased oral intake Right 5.7 × 5.5 × 4.7 cm mass with large areas of central necrosis, invading hilar soft tissues Sheets of poorly differentiated round cells, no desmoplastic stroma Bcl-2 (+), CD99 (+), desmin (+), vimentin (+), CD56 (+), and FLI-1 (+), WT1 (−), Synaptophysin (−), SMA (−), Myogenin (−), Myo-D1 (−), CD31 (−), CD34 (−), Napsin (−) RT-PCR showing EWSR1-WT1 fusion No information Metastatic disease (multiple, bone and lungs) at presentation. No further follow-up information Walton, et al, [ 12 ] Case 12 8 / F Abdominal pain Left 11x9x7 cm mass with renal pelvis, perirenal fat tissue and adrenal gland invasion Nests, cords, sheets of small round cells within desmoplastic stroma; frequent rosette-like structures, psedopapillary appearance and frequent rhabdoid cells.…”

Section: Introductionmentioning

confidence: 99%

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Desmoplastic small round cell tumor of the kidney: a case report

Baydar¹,

Armutlu²,

Aydın³

et al. 2020

Diagn Pathol

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Background: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm seen in children and young adults, usually manifested by involvement of abdominal serosa. Here, we present an unusual case of primary DSRCT of kidney. Case presentation: The patient was an 8-year-old girl with a large renal mass which was confused with primitive neuroectodermal tumor (PNET) in the needle biopsy. The tumor had a variegated histology revealing frequent pseudo-rosette formations, pseudopapillary architecture, rhabdoid, clear or pleomorphic cells in addition to typical small round cell morphology and desmoplasia. It showed immunohistochemical features of DSRCT, and EWSR1 rearrangement. Conclusions: Proffering this diagnosis is particularly difficult for tumors of viscera because of the incognizance of the entity in these locations. Moreover, DSRCT is a great mimicker and may get easily confused with more common kidney malignancies of childhood such as Wilms tumor, PNET/EWS, rhabdoid tumor, clear cell sarcoma, and other small round cell tumors as well as renal cell carcinomas. The distinction is critical as the accurate therapeutic approach will require correct diagnosis.

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“…Although the imaging characteristics of desmoplastic small round cell tumor have rarely been described with a renal primary tumor, several case reports describe multiple appearances. Eklund et al (6) described an indolent case in a 7-year-old boy with disease entirely confined to the renal collecting system. Additional cases have shown disease confined entirely to the renal parenchyma, with imaging showing a hypoattenuating mass relative to normal enhancing renal parenchyma with punctate calcifications, features similar to those found in this patient (5).…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation

Walton¹,

Flores²

2016

RadioGraphics

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Editor's Note.-RadioGraphics continues to publish radiologic-pathologic case material selected from the American Institute for Radiologic Pathology (AIRP) "best case" presentations. The AIRP conducts a 4-week Radiologic Pathology Correlation Course, which is offered five times per year. On the penultimate day of the course, the best case presentation is held at the American Film Institute Silver Theater and Cultural Center in Silver Spring, Md. The AIRP faculty identifies the best cases, from each organ system, brought by the resident attendees. One or more of the best cases from each of the five courses are then solicited for publication in RadioGraphics. These cases emphasize the importance of radiologic-pathologic correlation in the imaging evaluation and diagnosis of diseases encountered at the institute and its predecessor, the Armed Forces Institute of Pathology (AFIP).

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Desmoplastic small round cell tumor of the kidney with unusual imaging features

Cited by 13 publications

References 8 publications

Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade:From the Radiologic Pathology Archives

Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade:From the Radiologic Pathology Archives

Desmoplastic small round cell tumor of the kidney: a case report

Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation

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