Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Alaggio, Rita; Cecchetto, Giovanni; Martignoni, Guido; Bisogno, Gianni; Liu, Cheng; Sperlì, Domenico; d'Amore, Emauele S.G.; Dall’Igna, Patrizia

doi:10.1016/j.jpedsurg.2012.02.023

Cited by 22 publications

(19 citation statements)

References 48 publications

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“…The published cases seem to occur predominantly in middle-aged patients. However, the current patient is the youngest one and represents the first case involving the pediatric patient although less than 40 pediatric PEComas have been reported in other locations [23]. It is also noteworthy to mention that this patient present with a 3-year history of abdominal discomfort, implying that this lesion already existed during childhood.…”

Section: Discussionmentioning

confidence: 84%

Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Liu

Chen

et al. 2012

Diagn Pathol

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Perivascular epithelioid cell neoplasms (PEComas) of the urinary bladder are extremely rare and the published cases were comprised predominantly of middle-aged patients. Herein, the authors present the first urinary bladder PEComa occurring in an adolescent. This 16-year-old Chinese girl present with a 3-year history of abdominal discomfort and a solid mass was documented in the urinary bladder by ultrasonography. Two years later, at the age of 18, the patient underwent transurethral resection of the bladder tumor. Microscopically, the tumor was composed of spindled cells mixed with epithelioid cells. Immunohistochemically, the tumor were strongly positive for HMB45, smooth muscle actin, muscle-specific actin, and H-caldesmon. Fluorescence in situ hybridization analysis revealed no evidence of EWSR1 gene rearrangement. The patient had been in a good status without evidence of recurrence 13 months after surgery. Urinary bladder PEComa is an extremely rare neoplasm and seems occur predominantly in middle-aged patients. However, this peculiar lesion can develop in pediatric population and therefore it should be rigorously distinguished from their mimickers.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1870004378817301

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Section: Discussionmentioning

confidence: 84%

Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Liu

Chen

et al. 2012

Diagn Pathol

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“…The EAML is further divided into atypical epithelioid angiomyolipoma (AAML) and malignant renal epithelioid angiomyolipoma (MEAML). It is difficult to determine the presence of malignancy in renal PEComa, the suggested indicators are high index of mitotic activity, tumor size and presence of vascular invasion [1,8,10,14,15]. In our patient, the mitotic figure was rarely seen and there was no lymphovascular invasion suggesting that is was more likely not to be malignant.…”

Section: Discussionmentioning

confidence: 64%

“…PEComas include a variety of tumors e.g. angiomyolipomas, lymphangioleiomyomatosis, abdominopelvic sarcoma of perivascular epithelioid cells, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres and clear cell sugar tumor of the lung [8][9][10][11][12][13]. In 2006, Pan et al studied 8 cases of PEComas of the genitourinary tract (3 were renal in origin) and found that there was associated deletion of TSC2 locus on 16p [10].…”

Section: Discussionmentioning

confidence: 99%

“…Many of the literatures on PEComa focus on the histology and identification of the tumor with some assistance on clinicopathological prognostication but hardly any on standard protocol for treatment and management [1,10,15,16]. It is noted from literature that when malignant change occurs, the tumor tends to have variable response to chemotherapy and in some cases may relapse, requiring multiple surgeries [8,14].…”

Section: Discussionmentioning

confidence: 99%

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Renal perivascular epitheliod cell tumour (PEComa) in a child: Case report and review of literature

Da¹,

Al²,

Mr³

et al. 2015

Clin Case Rep Rev

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Perivascular epithelioid cell tumor (PEComa) is a rare entity with unpredictable clinical outcome. It is a group of mesenchymal tumors with presence of perivascular epithelioid cells (PEC), known to stain positively with melanosome markers HMB-45. They may arise from many unusual sites including the kidneys. Renal PEComa was previously groupedas angiomyolipoma and its existence is rare too. Pediatric PEComas are even rarer with less than 40 cases described worldwide and none involving the kidney before. We report a case of a 7 year old boy who presented with incidental findings of an abdominal mass, which confirmed to be a renal PEComa of the atypical epithelioid angiomyolipoma (AAML) type. The clinical management and review of literature for this interesting entity is discussed. Case presentationA previously well 7 years old boy was noted by his grandmother to have a bulge over the right side of the abdomen for one week duration. She also noticed that the boy had loss of appetite and less active. There was no complaint of abdominal pain, abnormal bowel habit or urinary symptoms such as hematuria. There was neither history of fever to suggest infection nor history of trauma. On examination, the boy appeared healthy and not syndromic. He was not pale or jaundice and his vital signs were stable. There was an obvious bulge on the right lumbar area. The bulge was a large non tender, 9 cm X 9 cm mass. The mass was ballotable suggesting that it was the right kidney. There was no other organomegaly. Blood investigations were done which included full blood count, renal profile, tumor markers for malignant germ cell tumour and teratoma and liver function test. All the blood investigations were normal. Radiological imaging was done concurrently; the abdominal ultrasound showed a mass arising from the right kidney, measuring 11.5 cm × 6.4 cm × 9.4 cm. It was a combined solid cystic mass with no calcifications seen. CT scan of the abdomen confirmed the findings and showed that the mass was exophytic in nature, localised with no invasion to surrounding structure. There was no caval involvement or aortocaval adenopathy. The other solid organs and lung bases were normal (Figure 1). The provisional diagnosis was a renal sarcoma with a differential diagnosis of renal cell carcinoma. An ultrasound guided percutaneous biopsy was performed which was reported as melanocytic angiomyolipoma. We proceeded to do open right nephro-ureterectomy; intraoperatively the tumor was quite vascularized, surgery went well without problems. We bisected the specimen to reveal an unusual looking tumor (Figure 2). Post operatively, the patient was well and discharged home at day 5. The pathological examination of the tumor revealed macroscopically the tumor was irregular, fragile, and blackishin color, located at the lower pole of the right kidney measuring 8.5 cm × 4.5 cm × 6.5 cm. The tumor extended into the renal pelvis and capsule but did not appear to breach the capsule. Microscopically, the tumor was composed of sheets and nests and per...

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“…In the present case, HMB-45 was expressed positively and diffusely in the epithelioid cells, while SMA and Desmin were expressed diffusely in the spindle cells. Though most PEComas are benign, approximately half of PEComas-NOS are malignant (5,6). As the number of reported cases is small, there are no consistent criteria for the diagnosis of benign or malignant PEComas.…”

Section: Discussionmentioning

confidence: 99%

Primary retroperitoneal perivascular epithelioid cell neoplasm: A case report

Liang

Chen

2015

Oncology Letters

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Abstract. Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumor, and primary retroperitoneal PEComa is rarer still. The present report discusses the case of a 51-year-old woman who was admitted to hospital following stiffness and discomfort in the upper right abdomen for longer than one month. Computed tomography identified a soft tissue mass in the rear right kidney, accompanied by clear cystic degeneration and bleeding. The borderline between the mass and the right kidney was not distinct. An enhanced scan revealed that the solid section of the mass was progressively enhanced. Following excision of the mass and the affected right kidney, the patient was pathologically diagnosed with retroperitoneal PEComa involving the right kidney. Short-term reexamination did not detect any recurrence.

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Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Cited by 22 publications

References 48 publications

Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Renal perivascular epitheliod cell tumour (PEComa) in a child: Case report and review of literature

Primary retroperitoneal perivascular epithelioid cell neoplasm: A case report

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