ABSTRACT
Objective To summary the pathological and clinical characteristics,treatments and prognosis of malignant renal perivascular epithelioid cell tumor (PEComa).Methods Between August 2013 and June 2017,8 cases of malignant renal PEComa were analysed respectively.Of all the patients,there were 4 males and 4 females,aged 27-65 years with the average of 46.3 years old.Three cases were detected in routine examination occasionally,three cases complained of intermittent back pain with fever,one presented with swollen and painful right hand and right foot,and one case presented with hematuria.CT or MRI examinations indicated the malignant lesions before the surgery.Clinical staging was performed with 2 cases of T1N0M0,1 case of T1 N0M1,2 cases of T2N0M0,1 case of T3M0M0,and 2 cases of T4N0M1.Three cases underwent radical nephrectomy,1 case underwent radical nephrectomy plus renal artery embolization,2 cases underwent partial nephrectomy,and 2 cases underwent nephrectomy plus inferior vena cava tumor thrombectomy.One case was treated with ifosfamide plus epirubicin after operation due to multiple distant metastases and 1 case was treated with oxaliplatin before the sugery because of excessive tumor diameter.None of the rest received any adjuvant therapy.Results Postoperative histopathological examination showed multiple nodules in reddish gray and yellow color,with soft texture and partial incomplete capsule.Microscopically,there were large atypical cell components,some of which were spindle shaped,with disordered cell arrangement,some of which were associated with a large amount of necrotic tissue,and abundant light eosinophil cytoplasm.Tissue components can be found thick-wall blood vessels,smooth muscle-like cells,and adipose tissue.Immunohistochemistry showed 4 cases were positive of HMB-45,4 of Melan-A,7 of SAM.Seven cases were negative of CK,6 of S-100 75％ (6/8) and Ki67 (10％-70％).Two cases died of multiple metastases (lung,bone,liver),1 case survived with tumor recurrence,with a follow-up from 14 to 60 months (mean 29 months).Conclusions Primary malignant renal PEComa is rare in practice with relative large diameter.The diagnosis depends on pathological findings,and radical nephrectomy is the first choice of treatment.It is easy to recur and metastasize after the operation.
ABSTRACT
Objective To discuss the clinical diagnosis,treatment and prognosis of malignant melanoma of urinary system.Methods The clinical data of 5 patients with primary malignant melanoma of urinary system were retrospectively analyzed.There were 2 cases of primary melanona of the urethra,3 cases of primary malignant melanoma of the bladder.The diameter of the tumor ranged from 0.9 to 5.1 cm with an average of 3.1 cm.Results Two cases of urethral patients underwent radical resection of urethra.Among 3 cases of bladder,1 cases were in poor condition,and underwent laparoscopic partial cystectomy.In 1 young men,radical resection was refused and only transurethral resection of the bladder tumor was performed.Radical resection of bladder was done in 1 cases.Postoperative pathology showed that the tumor cells of 4 cases were fusiform under microscope,1 case was polygonal.5 cases showed melanin in the cytoplasm and diffuse proliferation of tumor cells,with obvious heterogeneity,cell proliferation index Ki-67 10％-30％.During the follow-up period of 7-30 months (median 19 months),3 patients died of metastasis.Conclusions Malignant melanona of urinary system is rare,with high malignancy and poor prognosis.Targeted therapy and immune therapy has become a new treatment option,which could improve the prognosis of patients.