OBJECTIVETo evaluate the diagnosis and treatment of hereditary renal cell carcinoma.

METHODSClinical data of 11 patients with hereditary renal cell carcinoma were analyzed retrospectively. Eight patients were male and 3 were female, age ranged from 32 to 67 (mean: age 48 years). Four cases were bilateral renal cell carcinoma, and 4 were multiple renal cell carcinoma. Two cases were diagnosed as Von Hippel-Lindau syndrome, 6 as familial clear cell renal cell cancer, and 3 as hereditary papillary renal carcinoma.

RESULTSTen patients performed nephron-sparing surgery and/or radical nephrectomy and 1 had no operation. The patients were followed up from 12 to 114 months. Tumor recurrence was observed in 4 patients, 1 patient died of tumor metastasis, and 2 died of other causes. Four patients survived free of tumor.

CONCLUSIONSHereditary renal carcinoma appears in the youth, and it is predominantly multiple and bilateral. Nephron-sparing surgery is the standard method of treatment for the patients.

Adult ; Aged ; Carcinoma, Renal Cell ; diagnosis ; genetics ; surgery ; Female ; Humans ; Kidney Neoplasms ; diagnosis ; genetics ; surgery ; Male ; Middle Aged ; Retrospective Studies

Epithelioid angiomyolipoma is a recently described rare variant of renal angiomyolipoma. It can occur in patients with or without tuberous sclerosis. We report the imaging findings of a case of epithelioid angiomyolipoma that showed the presence of fatty tissue undifferentiated from the typical angiomyolipoma at the beginning. After partial nephrectomy, tumour recurrence occurred two years later, presenting as completely solid tumours with no adipose tissue, and with invasion into the psoas muscle and left adrenal gland. Differentiation of this tumour from renal cell carcinoma is difficult. Both the radiologist and surgeon should be aware of the existence of this tumour and its potentially malignant behaviour.
Aged ; Angiomyolipoma ; diagnosis ; pathology ; surgery ; Carcinoma, Renal Cell ; diagnosis ; Diagnosis, Differential ; Female ; Humans ; Kidney ; pathology ; Kidney Neoplasms ; diagnosis ; pathology ; surgery ; Neoplasm Recurrence, Local ; pathology ; Tomography, X-Ray Computed

Adult ; Aged ; Aged, 80 and over ; Carcinoma, Renal Cell ; diagnosis ; diagnostic imaging ; surgery ; Female ; Humans ; Kidney Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Male ; Middle Aged ; Radiography ; Retrospective Studies ; Treatment Outcome

Adult ; Aged ; Carcinoma, Renal Cell ; diagnosis ; surgery ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; diagnosis ; surgery ; Male ; Middle Aged ; Nephrectomy ; methods ; Treatment Outcome

Carcinoma, Medullary ; pathology ; Carcinoma, Renal Cell ; diagnosis ; metabolism ; pathology ; radiotherapy ; surgery ; Diagnosis, Differential ; Humans ; Keratins ; metabolism ; Kidney Neoplasms ; diagnosis ; metabolism ; pathology ; radiotherapy ; surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasms, Squamous Cell ; pathology ; Nephrectomy ; Radiotherapy, Adjuvant

Adenocarcinoma, Clear Cell ; metabolism ; pathology ; surgery ; Adult ; Carcinoma ; metabolism ; pathology ; Carcinoma, Mucoepidermoid ; metabolism ; pathology ; Carcinoma, Renal Cell ; metabolism ; pathology ; secondary ; Diagnosis, Differential ; Humans ; Keratins ; metabolism ; Male ; Nasal Cavity ; Nose Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

Adenocarcinoma, Follicular ; Adult ; Carcinoid Tumor ; metabolism ; pathology ; Carcinoma, Renal Cell ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Keratin-7 ; metabolism ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Mucin-1 ; metabolism

We report the rare case of a patient with advanced renal cell carcinoma (RCC) who initially presented to the hospital with symptoms of cardiac failure. Preoperative cardiac studies did not reveal any underlying ischemia. After resection of a large 14-cm left renal tumor, cardiac function was noted to improve dramatically. We discuss this case of concomitant RCC and nonischemic cardiomyopathy.
Asian Continental Ancestry Group ; Carcinoma, Renal Cell/*diagnosis/surgery ; Cardiomyopathies/*diagnosis ; Female ; Heart Failure/*etiology ; Humans ; Middle Aged ; Paraneoplastic Syndromes/complications ; Thalassemia/complications

The inflammatory myofibroblastic tumor (IMT), also knowns as inflammatory pseuduotumor, is a soft tissue lesion of unknown etiology. In the urogenital tract, IMT mainly affects the urinary bladder or prostate, but rarely the kidney. It has been considered as a nonneoplastic reactive inflammatory lesion, but nowadays, it is regarded as a neoplasm due to its high recurrence rate and metastasis. We describe a case of a 61-yr-old woman that had originally been misdiagnosed as renal cell carcinoma, which was pathologically revealed to be an IMT.
Actins/metabolism ; Carcinoma, Renal Cell/diagnosis/pathology ; Diagnosis, Differential ; *Diagnostic Errors ; Female ; Granuloma, Plasma Cell/*diagnosis/pathology/surgery ; Humans ; Kidney Neoplasms/*diagnosis/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Tomography, X-Ray Computed ; Vimentin/metabolism

Objective To investigate the clinicopathological features and prognosis of chromophobe renal cell carcinoma(ChRCC). Methods The clinical and pathological data of 126 patients with ChRCC treated in Cancer Hospital of Chinese Academy of Medical Sciences were retrospectively analyzed. Results The patients included 64 males and 62 females,with the age of 22-80 years(median of 52 years).The tumor was located on the right side in 70 cases and on the left side in 56 cases.Ultrasound,CT or magnetic resonance imaging(MRI)were performed.Of the 110 cases receiving ultrasound examination,63,23,13,10,and 1 cases showed hypoecho,hyperecho,isoecho,uneven or mixed echo,and dark hypoecho,respectively.Color Doppler flow imaging showed no blood flow signal in 42 cases and low blood flow signal in 60 cases out of 68 cases with blood flow signal.Among the 54 cases receiving CT,50 cases showed equal density or low density and 4 cases showed high density with clear boundary.The enhanced scanning showed mild to moderate uniform or non-uniform reinforcement,mostly below the renal parenchyma,and still showed reinforcement in the delayed period.Among the 97 cases receiving MRI,96 cases showed hypo-or isointense signals and 1 case showed hyperintense signal in T1 weighted images;71 cases showed hyper-or isointense signals and 26 cases showed hypo-or isointense signals in T2 weighted images;93 cases showed hyperintense signals with obvious limited diffusion and 4 cases showed unobvious limited diffusion in diffusion weighted images.Mild to moderate uniform or non-uniform reinforcement was observed in most of the enhanced scans.All the 126 patients underwent surgical treatment,including 64 cases of nephron sparing surgery and 62 cases of radical surgery.Pathological examinations confirmed ChRCC for all the patients,including 91 cases of T1N0M0,15 cases of T2N0M0,and 20 cases of T3N0M0.The immunohistochemical assay demonstrated the positive expression rate of 48.2%(54/112)for CD10,92.3%(96/104)for CD117,8.0%(9/112)for vimentin,85.6%(95/111)for CK7,and 97.6%(83/85)for colloidal iron.Conclusions ChRCC is less common,with low level of malignancy and good prognosis.Since the clinical symptoms of ChRCC are not typical,MRI is an important means of imaging differential diagnosis,and the disease can be confirmed depending on pathological diagnosis.Surgery is the preferred treatment method,and currently there is no standard treatment regimen for metastatic patients.
Adult ; Aged ; Aged, 80 and over ; Carcinoma, Renal Cell/diagnostic imaging* ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Kidney Neoplasms/surgery* ; Male ; Middle Aged ; Retrospective Studies ; Young Adult