Università di VERONA SCHWANNOMA DEL RENE M. Vergine, S. Gobbo, M. Brunelli, A. Eccher, D. Segala, M. Chiosi, F. Bonetti, F. Menestrina, L. Cheng, G. Martignoni Introduzione: Solo pochi casi di schwannoma del rene sono stati riportati. Autori, anno di pubblicazione Numero casi descritti Phillips & Baumrucker, 1955 1 Kuzmina, 1962 1 Fein, 1965 1 Bair, 1978 1 Steers, 1985 1 Somers, 1988 1 Kitagawa, 1990 1 Ma, 1990 1 Naslund, 1991 1 Romics, 1992 1 Ikeda, 1996 1 Singer, 1996 1 Alvarado-Cabrero, 2000 4 Tsurusaki, 2001 1 Singh, 2005 2 Tot: 19 casi Materiali e Metodi: Abbiamo riportato tre ulteriori casi di schwannoma renale con analisi immunoistochimica. Abbiamo inoltre eseguito una revisione sistematica della letteratura Analisi Immunoistochimica: S100 S100A1 NSE Neurofilamenti HMB45 Caso1 + Neg. 10% Neg Neg Caso2 + + 90% Neg Caso3 + Neg. 90% Neg MiTF AML CK AE1/3 CK 7 CD34 CD10 Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Neg Dati Clinici: Caso # Età Sesso Segni e Sintomi Lato Intervento chirurgico Follow-up 1 59 F Asintomatico S Nefrectomia Non Disponibili Nefrectomia Libero da malattia a 8 mesi di followup Nefrectomia Libero da malattia a 4 mesi di followup 2 3 27 35 F Reperto occasionale F Dolore addominale, nausea D S Dati patologici: Diametro Caso # 1 4,8 cm 8,5 cm 2 7 cm 3 Aspetto Macroscopico Sede Diagnosi Massa lobulata Ilo Schwannoma Massa fibrosomixomatosa Intraparenchimale, polo inferiore “Ancient” schwannoma Massa microcistica Ilo Schwannoma CASO 1 CASO 1 S100 CASO 2 CASO 2 S100 CASO 3 CASO 3 S100 REVISIONE DELLA LETTERATURA Author, Year of Publication Age Sex Symptoms and Signs Side Operation Gross Location Diagnosis Follow-up L Left nephrectomy 12-cm Encapsulated tumor Pelvis Schwannoma NA Phillips & Baumrucker, 1955 56 M Generalized malaise, weight loss, fever, flank pain, mild anemia Kuzmina, 1962 33 F Generalized malaise, weight loss, mild temperature, flank mass R Right nephrectomy Encapsulated, rubbery, pale gray; tumor Capsule Schwannoma NA F Congenital malforamtion, hydronephrosis, fever, flank pain, pyuria,palpable mass R Right nephrectomy 6-cm well encapsulated mass Pelvis Schwannoma a NED (~24-mo follow-up) R Right nephrectomy 7-cm mass Hilum Schwannoma NED (5-mo follow-up) Fein, 1965 51 Bair, 1978 56 M Hypertension, microhematuria, hypertensive renal disease Steers, 1985 50 F Microhematuria, palpable mass R Tumorectomy 9-cm multicystic mass Hilum Schwannoma NA Somers, 1988 55 F Incidental finding L Left nephrectomy 5.1-cm Well-defined solid lobular mass Intraparenchy mal upper pole Schwannoma NED (18-mo follow-up) L Left nephrectomy 2.8-cm Well-demarcated yellowish tumor, Hilum Schwannoma NA Kitagawa, 1990 51 M Upper abdominal pain and high fever;painful swelling of the scrotum Ma, 1990 67 M Epigastric pain R Right nephrectomy 8-cm Well-encapsulated, firm, yellowish Intraparenchy mal and pelvis Cellular schwannoma NED (1-y follow-up) Naslund, 1991 50 F Mild abdominal disconfort, weight loss, anemia L Left nephrectomy 14-cm mass Intraparenchy mal upper pole Malignant schwannoma DOD after 15-mo …CONTINUA Romics et al, 1992 52 M Back and flank pain, recurrent fever, anemia, leukocytosis Ikeda, 1996 89 M Abdominal pain R Right nephrectomy NA Pelvis Schwannoma NA Singer, 1996 70 F Asymptomatic, elevated CEA L Left nephrectomy, 6-cm well demarcated lobulated mass Hilum Schwannoma NED (18-mo follow-up) Alvarado-Cabrero et al, 2000 45 M Flank and abdominal pain L Left nephrectomy 16-cm mass Intraparench ymal “Ancient” schwannoma NED (5-y follow-up) 40 F Flank pain L Left nephrectomy 12.5-cm mass Intraparench ymal Cellular schwannoma NED (1-y follow-up) 84 M Found during work-up of cystitis R Right nephrectomy 4-cm mass Intraparench ymal Cellular schwannoma NED (4.5-y follow-up) 18 F Flank pain R Right nephrectomy 6.2-cm mass Intraparench ymal Cellular schwannoma NED (3.5-y follow-up) Tsurusaki, 2001 69 F Incidental finding L Tumorectomy Elastic white mass with necrosis Capsule Schwannoma NA Singh, 2005 40 M Renal colicky pain, vomiting L Left nephrectomy 3-cm firm mass Hilum Schwannoma NED (3-y follow-up) 35 M Flank pain, gross hematuria R Right nephrectomy NA Intraparench ymal and pelvis Schwannoma NED (2-y follow-up) Gobbo et al, 2008: case 1 35 F Abdominal pain, nausea L Left nephrectomy 7-cm encapsulated microcystic mass Hilum Schwannoma NED (4-mo follow-up) Gobbo et al, 2008: case 2 27 F Incidental finding during study for polycythemia R Right nephrectomy 8.5-cm myxomatous fibrous mass Intraparench ymal mid lower pole “Ancient” schwannoma NED (8-mo follow-up) Gobbo et al, 2008: case 3 59 F Asymptomatic L Left nephrectomy 4.8-cm lobulated mass Hilum Schwannoma NA R Right nephrectomy; mesenteric metastasectomy Large infiltrating mass with cystic necrotic degeneration Capsule Malignant schwannoma DOD after 3-mo Conclusioni: Lo schwannoma renale è raro, usualmente si localizza nella regione centrale del rene improntando l’ilo o la pelvi ed è curato chirurgicamente. Nella diagnosi differenziale devono essere presi in considerazione il carcinoma sarcomatoide e altri tumori a cellule fusate.