Perirenal Perivascular Epithelioid Cell Tumor (PEcoma) with Pulmonary Micro Invasive Adenocarcinoma: A Case Report and Literature Review

Figures & data

Figure 1 (A) Chest CT detected a 13×4 mm nodule in the right upper lung; (B) abdominal MRI revealed a perirenal tumor, 18×28 mm in size, close to the hilum of the left kidney.

Figure 2 Positron emission tomography (PET)-CT whole body imaging. (A) A 15×10 mm shadow with ground glass density in the upper right lung was detected; (B) a neoplastic lesion with hypermetabolism close to the left renal hilum was shown.

Figure 3 Immunohistochemistry of the perirenal mass showed positive for Melan-A, cluster differentiation (CD)56, and TFE3, while negative for HMB45, Syn, S-100 and SOX10. Ki67 was present in approximately 1% of tumor cells.

Table 1 Characteristics of Prior Cases of Perirenal Perivascular Epithelioid Cell Tumor (PEComa)

Study (Publication Year)	Gender	Age	Region	Tumor Location	Tumor Size (cm)	Pathology	Immunohistochemical Study	Coexisting Tumor	Resectability	Other Treatments	Prognosis
Sven Gunia (2005)^Citation4	57	F	Germany	Below the left perinephric capsule with a tongue-like growth pattern towards the hilus of the kidney	5.3 x 5.0 x 3.0	Composed of epithelioid cells and spindle cells	HMB-45(+), Melan A(+), CD99(+), Ki67(+)<1%	NR	Yes	NA	No evidence of any local recurrence or of any distant metastases at the 8-month post-surgical follow-up No local recurrence or distant metastases at the 8-month post-surgical follow-up
Shotaro Nakanishi (2014)^Citation5	51	F	Japan	In the right perirenal space and multiple liver metastases	20.0 x 16.0 x 9.0	Composed of lightly eosinophilic cytoplasm, round to slightly ovoid nuclei, and small nucleoli	HMB-45(+), Melan-A (+)	NR	No	mTOR inhibitor and temsirolimus	DIe Died about five months after the start of molecular targeted therapy
Christos V Gkizas (2015)^Citation6	44	F	Greece	In close proximity to the left renal capsule	6.0 × 7.0 × 10.0	Composed of spindle and epithelioid cells	Vimentin(+), desmin(+), SMA(+), HMB45(+), CD56(+), CD99(+), NSE(+)	NR	Yes	NA	Uneventful recovery
Marian Danilewicz (2017)^Citation7	66	F	Poland	Close to the superior pole of the right kidney	1.5 × 1.0 x 0.6	Composed of polygonal clear cells or cells with eosinophilic cytoplasm with moderate epithelioid appearance	HMB-45 (+), vimentin (+), focally desmin (+), Ki-67(+) (8%), CD10(-), S-100(-)	Mucinous gallbladder cancer and pancreatic head tumor	Yes	NA	Uneventful recovery
Present case
Zhaoxun Li (2021)	43	M	China	Close to the hilum of the left kidney	4.0 x 2.0 x 1.0	Composed of cells with eosinophilic cytoplasm with moderate epithelioid appearance	Melan A (+), CD56(+), TFE3(+), HMB45(-), SMA(-), desmin(-), S-100(-), SOX10(-), Ki-67(+)≈1%	Pulmonary micro invasive adenocarcinoma	Yes	NA	Uneventful recovery

Abbreviations: F, female; M, male; NR, not reported; NA, no application; CD, cluster differentiation.

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