PEComa in a 12-year-old boy

Uddin, Zeeshan; Qureshi, Asim; Fatima, Samia; Kayani, Naila

doi:10.1136/bcr.04.2009.1735

Cited by 5 publications

(3 citation statements)

References 9 publications

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“…Malignant tumours such as renal‐cell carcinoma or renal medullary carcinoma can be detected in this age group 7 . This can explain the general attitude to perform an open radical nephrectomy, like we observed in all cases previously described 8–10 and in which histological diagnosis was only later refined as PEComas. This is the reason why we suggest to perform a percutaneous cutting needle biopsy (PCNB) in selected cases with the purpose to avoid the risk of an over‐treatment (benign tumours) or an under‐treatment (malignant tumours) either in terms of chemotherapy or to the extent of surgery.…”

Section: Discussionsupporting

confidence: 69%

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Silveri

Natali

Cobellis³

et al. 2020

Robotics Computer Surgery

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Background: Perivascular epithelioid cell tumours (PEComas) represent a rare group of mesenchymal tumours with an unpredictable outcome. They are usually observed in middle-aged women whilst they are very rare in children, with limited cases described. Due to the rare anatomical location in kidneys, a correct diagnosis and treatment is really challenging. Methods: We observed and treated a case of kidney's PEComa in a 14-year-old boy. Results: An individualized approach based on anatomical (3D CT-reconstructions) and histopathological (US-guided preliminary biopsy) features lead us to perform a successful robotic-sparing surgery enabling the preservation of two-thirds of the kidney involved. Conclusions: A meticulous preoperative planning in selected patients can lead to a minimally invasive approach even in some paediatric kidney's neoplasms.

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Section: Discussionsupporting

confidence: 69%

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Silveri

Natali

Cobellis³

et al. 2020

Robotics Computer Surgery

View full text Add to dashboard Cite

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“…PEComa tumours are more likely to affect women in the fourth and fifth decade of life, more specifically between the ages of 38 and 56,5 although cases have also been reported in children 9–11. They may be an incidental finding, which occurs in about 20% of cases, or present as pain or discomfort in the tumour area, weight loss, or other complaints related to the compression of adjacent structures 5.…”

Section: Discussionmentioning

confidence: 99%

Primary retroperitoneal PEComa: an incidental finding

et al. 2022

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Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumour, and primary retroperitoneal PEComa is rarer still. Although pulmonary lymphangioleiomyomatosis (LAM), angiomyolipomas and clear cell ‘sugar’ tumours of the lung are well described, relatively little is known about other members of the PEComa family. We describe a case of an asymptomatic retroperitoneal PEComa, lymphangioleiomyoma type, which appeared in a previously healthy middle-aged woman as an incidental finding, in a CT scan performed in the context of spontaneous pneumothorax. The patient underwent surgical excision of the tumour and the histopathological and immunohistochemical analysis of the surgical specimen made the definitive diagnosis. Although rare, reports of isolated retroperitoneal lymphangioleiomyoma and primary retroperitoneal PEComas NOS (not otherwise specified) are described in the literature, normally associated with pulmonary LAM. The patient’s pulmonary imaging was normal. Short-term re-examination did not detect any recurrence. We also provide a literature review of this rare group of tumours.

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“…It should be noted that before diagnosing melanoma in the liver, one should always consider the possibility of a perivascular epithelioid cell tumor (PECOMA), because PECOMAs can also be positive for S100, MART1, MITF, and HMB45. As a result, appropriate myoid markers such as smooth muscle actin and desmin along with detailed examination of the histology should allow for proper distinction between a PECOMA and a melanoma (42,43).…”

Section: Differential Diagnosismentioning

confidence: 99%

Undifferentiated Embryonal Sarcoma of the Liver in Adults

Huang

2021

Liver Cancer

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Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive malignancy that most commonly affects the pediatric age group. This tumor very rarely occurs in adults and, in such instances, can pose a considerable diagnostic challenge for the clinicians, radiologists, and pathologists involved. The clinical presentation is most often non-specific, and the radiology shows a solid and cystic liver mass which has a considerable differential on imaging. Especially in adult patients, UESL is a diagnosis of exclusion meaning that all other diagnostic possibilities must be excluded before the diagnosis of UESL can be confidently made. From a pathology perspective, this means careful examination of the histology along with a comprehensive panel of immunohistochemistry for almost all cases of newly diagnosed UESL. The prognosis of UESL used to be dismal, but with advances in treatment and the introduction of a multimodality approach, there has been considerable progress in improving outcomes and survival for patients with this aggressive tumor. This chapter outlines the clinical, radiological, and pathological features of UESL. An in-depth discussion is undertaken to describe the diagnostic approach and the differential diagnosis for this rare and challenging tumor.

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PEComa in a 12-year-old boy

Cited by 5 publications

References 9 publications

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Individualized robotic organ‐sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour

Primary retroperitoneal PEComa: an incidental finding

Undifferentiated Embryonal Sarcoma of the Liver in Adults

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