Radiology-pathology conference: primary perinephric and renal extraosseous Ewing's sarcoma

Lazzara, Bryan M.; Scalcione, Luke R.; Garnet, Daniel J.; Geller, Matthew D.; Katz, Douglas S.

doi:10.1016/j.clinimag.2011.05.001

Cited by 8 publications

(5 citation statements)

References 13 publications

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“…1 The majority of PNETs present as bone sarcomas with only 25 per cent of PNETs arising principally from the soft tissue. 2 It is extremely rare to encounter adults with PNETs restricted to the soft tissue. This article reports the case of two individuals, ages 28 and 55 years, both of whom were found to have soft tissue PNETs.…”

Section: Adult Soft Tissue Ewing's Sarcoma/primitive Neuroectodermal Tumormentioning

confidence: 99%

“…3 The skeletal system is the most frequent site for a PNET to arise with only 25 per cent of PNETs occurring principally in the soft tissue. 2 Case reports have described peripheral PNETs of a wide variety of organs, but the vast majority of these reports have discussed tumor presentation and prognosis in the pediatric population. There is a relative paucity of literature focusing on adults.…”

Section: Adult Soft Tissue Ewing's Sarcoma/primitive Neuroectodermal Tumormentioning

confidence: 99%

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Adult Soft Tissue Ewing's Sarcoma/Primitive Neuroectodermal Tumor

Hanson

Ubert

Dyer

et al. 2013

The American Surgeon

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Brief Reports should be submitted online to www.editorialmanager.com/ amsurg. (See details online under ''Instructions for Authors''.) They should be no more than 4 double-spaced pages with no Abstract or sub-headings, with a maximum of four (4) references. If figures are included, they should be limited to two (2). The cost of printing color figures is the responsibility of the author.In general, authors of case reports should use the Brief Report format.

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Section: Adult Soft Tissue Ewing's Sarcoma/primitive Neuroectodermal Tumormentioning

confidence: 99%

Section: Adult Soft Tissue Ewing's Sarcoma/primitive Neuroectodermal Tumormentioning

confidence: 99%

Adult Soft Tissue Ewing's Sarcoma/Primitive Neuroectodermal Tumor

Hanson

Ubert

Dyer

et al. 2013

The American Surgeon

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“…3 Due to their rarity, little has been published specifically regarding sarcomas of the kidney and its supporting structures, with most of the literature consisting of case reports. [5][6][7][8][9][10][11][12][13][14][15][16][17] The aim of this study was to determine which sarcoma subtypes arise primarily within the perinephric soft tissue and renal hilar structures (ie not primary renal sarcomas), based on the data collected from our institution's cohort, as well as the clinical and pathologic characteristics of these tumors and their natural histories.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A Contemporary Clinicopathologic Analysis of Primary Sarcomas of the Perinephric Soft Tissue and Hilar Vessels Including a Subset Secondarily Involving the Kidney

et al. 2022

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A variety of primary malignant mesenchymal neoplasms can arise from the perinephric soft tissue and hilar vessels and potentially involve the kidney, mimicking primary renal tumors. A search was made at our institution for patients that underwent radical nephrectomy with associated perinephric or hilar sarcomas from 2010 to 2021. Twenty-six patients were identified. Mean patient age was 60 years (range: 34–83 years), with 16 (62%) females and 10 (38%) males. The mean tumor size was 21.6 cm (range: 8.1–36.5 cm). Among the perinephric/retroperitoneal sarcomas, 14/20 (70%) were dedifferentiated liposarcoma, 4/20 (20%) were well-differentiated liposarcoma, and 2/20 (10%) were leiomyosarcoma. There were 4 grade 1 (20%; all well-differentiated liposarcoma), 9 grade 2 (45%), and 7 grade 3 (35%) tumors. All 6 sarcomas arising from the renal vein/inferior vena cava were leiomyosarcoma: grade 2 in 1 (17%), grade 3 in 4 (67%), and ungraded (due to neoadjuvant therapy effect) in 1 (17%) patient. Four of the 26 (15%) tumors involved the ipsilateral kidney. All 4 tumors were grade 3 sarcomas. On follow-up, 8/26 (31%) patients developed local recurrence and/or metastasis. The mean time for recurrence was 22 months (range: 7–48 months). Two patients progressed with metastasis to the lungs, both of which were grade 3 leiomyosarcoma, and appeared 11 months after the initial diagnosis. Our data suggest that while local recurrence is prevalent with most subtypes of perinephric sarcomas, high-grade leiomyosarcoma has a distinct proclivity for distant metastasis, with the lungs being the most common site.

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“…El sarcoma extraóseo de Ewing corresponde a un grupo de neoplasias agresivas que afectan predilectamente a niños y adultos jóvenes, y escasamente a ancianos (Lazzara et al, 2012) No existe una etiología clara, pero factores genéticos y ambientales podrían predisponer al desarrollo del sarcoma.…”

Section: Introductionunclassified