Multifocal retroperitoneal and pelvic PEComas mimicking liposarcoma: A case report and review of literature

Yim, Harold; Tang, Yee Lin; Tandon, Ankit

doi:10.1016/j.radcr.2021.06.020

Cited by 2 publications

(3 citation statements)

References 19 publications

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“…Some primary sites of PEComa include the retroperitoneum, uterus, vulva, gastrointestinal tract (oesophagus, stomach, duodenum, jejunum, ileum, colon, rectum), heart, breast, urinary bladder, abdominal wall, skin, mesentery, oral cavity, pancreas and liver. Women are more commonly affected than men, and these tumours are more frequently seen in the fourth and fifth decades of life 8. Imaging studies are not sufficiently sensitive to make the diagnosis of PEComas.…”

Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma

Siddiqi

Mesropyan

2022

BMJ Case Rep

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A young man in his 40s was evaluated in the emergency department for abdominal and right flank pain. A CT scan of the abdomen and pelvis showed a solid, well-circumscribed lesion measuring 7.1×8.1×5.4 cm, which was arising from the retroperitoneum and extending from the third portion of the duodenum towards the right kidney. A percutaneous core biopsy was obtained, demonstrating an atypical smooth muscle neoplasm suggestive of a low-grade leiomyosarcoma. The patient underwent surgery for an en-block resection of the mass and the final pathology confirmed a perivascular epithelioid cell neoplasm without significant pleomorphism, mitosis or necrosis. Our case adds to the small number of perivascular epithelioid cell tumour cases reported in the literature and we present it in order to increase our understanding of this tumour and to assist in its appropriate diagnosis and management.

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Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma

Siddiqi

Mesropyan

2022

BMJ Case Rep

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“…It is characterised by a copious amount of intratumoral fat density, a renal parenchymal defect, encapsulated margins, and enlarged/aneurysmal intralesional blood vessels [71]. Radiologic reviews of retroperitoneal tumours where benign AML is a consideration should be performed at a sarcoma referral centre, as AML can look similar to well-differentiated or dedifferentiated LPS or PEComa-NOS on imaging [Figure 1] [71][72][73]. This is due to the scarce or absent intratumoral fat of benign epithelioid AML on imaging.…”

Section: Differentiating Malignant Pecoma-nos From Benign Amlmentioning

confidence: 99%

“…Thus, tumours that cannot clearly be diagnosed as AML or PEComa-NOS on imaging require retroperitoneal coaxial core-needle biopsy for diagnosis [70]. Similarly, some LPS (especially well-differentiated LPS) may look similar to AML, necessitating biopsy and testing for fluorescence in situ hybridization amplification of the MDM2 and CDK4 genes for LPS diagnosis [71,73,74]. Importantly, even on biopsy, epithelioid AML resembles renal cell carcinoma, although immunohistochemistry can usually distinguish these entities.…”

Section: Differentiating Malignant Pecoma-nos From Benign Amlmentioning

confidence: 99%

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

et al. 2023

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With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as ‘ultra-rare’ sarcomas, with an incidence of ≤1–5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing’s sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes.

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Multifocal retroperitoneal and pelvic PEComas mimicking liposarcoma: A case report and review of literature

Cited by 2 publications

References 19 publications

Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma

Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

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