Advertisement

Der Pathologe

, Volume 24, Issue 6, pp 466–472 | Cite as

Das primäre synoviale Sarkom der Niere

Eine neue Entität im morphologischen Spektrum der spindelzellig differenzierten Nierentumoren
  • H. Moch
  • A. Wodzynski
  • L. Guillou
  • V. Nickeleit
Schwerpunkt: Uropathologie

Zusammenfassung

Wir berichten über 2 primäre synoviale Sarkome der Niere. Die Existenz dieses Tumortyps in der Niere wurde erst vor kurzem erkannt. Bisher wurden 20 Fälle publiziert. Wegen der primitiven „embryonalen“ Zellen und der auffälligen zystischen Strukturen wurden diese Tumoren ursprünglich als embryonale zystische Sarkome der Niere bezeichnet. Synoviale Sarkome der Niere treten meist bei 20- bis 50-jährigen Patienten auf. Eine Zusammenstellung aller publizierten Fälle lässt vermuten, dass das Auftreten von Lokalrezidiven nach Nephrektomie häufig ist. Makroskopisch handelt es sich bei den Primärtumoren meist um große (5–15 cm), teilweise nekrotische und häufig zystische Tumoren. Mikroskopisch sind synoviale Sarkome der Niere durch monomorphe, plumpe Spindelzellen charakterisiert. Die Zysten werden durch polygonale eosinophile Zellen ausgekleidet, die keine wesentlichen Zellatypien aufweisen. Die Spindelzellen exprimieren immunhistochemisch Vimentin, CD56, teilweise auch CD99 und EMA, sind aber negativ für Desmin, Aktin, S 100 und in unseren Fällen für Zytokeratin. Das Zystenepithel ist Zytokeratin-positiv. In beiden Fällen konnte die für synoviale Sarkome charakteristische SYT-SSX-Genfusion nachgewiesen werden. Primäre synoviale Nierensarkome stellen somit eine neue Tumorentität der Niere dar, die bei der Differenzialdiagnose von spindelzelligen Tumoren der Nieren berücksichtigt werden muss.

Schlüsselwörter

Niere SYT-SSX-Translokation Spindelzellen Differenzialdiagnose Embryonales zystisches Sarkom 

Abstract

We report 2 primary renal synovial sarcoma. These tumors were formerly designated as embryonal cystic sarcoma of the kidney. Most cases are diagnosed between the ages of 20 and 50 years. Some cases show local recurrence after nephrectomy. On gross examination, tumors are large, partially necrotic, and usually contain cysts. Microscopically, tumors are characterized by monomorphic plump spindle cells. The cysts are lined by mitotically inactive epithelial cells without striking cellular atypia. The spindle cells were immunoreactive for EMA, CD56, and sometimes for CD99. They were non-reactive for desmin, actin, S 100, and cytokeratins. The cyst epithelium is cytokeratin positive. The presence of a SYT-SSX gene fusion resulting from the t(X;18) characteristic for synovial sarcoma was demonstrated by reverse transcriptase polymerase chain reaction in both tumors. Primary renal synovial sarcoma is a distinctive tumor entity, which should be considered in renal tumors consisting of spindle cells.

Keywords

Synovial sarcoma Kidney Cystic neoplasms Renal call carcinoma SYT-SSX fusion 

Notes

Danksagung

Die Autoren danken Frau R. Epper und Frau M. Kasper für die Durchführung der Immunfärbungen. Dr. Jean Benhattar und G. Gallagher danken wir für die Durchführung und Interpretation der molekularen Untersuchungen zum Nachweis der SYT-SSX-Translokation.

Literatur

  1. 1.
    Al-Rajhi N, Husain S, Coupland R et al. (1999) Primary pericardial synovial sarcoma: acase report and literature review. J Surg Oncol 70:194–198CrossRefPubMedGoogle Scholar
  2. 2.
    Argani P, Zakowski MF, Klimstra DS et al. (1998) Detection of the SYT-SSX chimeric RNA of synovial sarcoma in paraffin-embedded tissue and its application in problematic cases. Mod Pathol 11:65–71Google Scholar
  3. 3.
    Argani P, Faria PA, Epstein JI et al. (2000) Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. Am J Surg Pathol 24:1087–1096CrossRefGoogle Scholar
  4. 4.
    Bowers DL, Te A, Hibshoosh H, Sawczuk IS (1995) Renal hemangiopericytoma. Case report and review of the literature. Urol Int 55:162–166PubMedGoogle Scholar
  5. 5.
    Brett D, Whitehouse S, Antonson P et al. (1997) The SYT protein involved in the t(X;18) synovial sarcoma translocation is a transcriptional activator localised in nuclear bodies. Hum Mol Genet 6:155–164CrossRefGoogle Scholar
  6. 6.
    Cano JY, D’Altorio RA (1976) Renal liposarcoma: case report. J Urol 115:747–749Google Scholar
  7. 7.
    Casella R, Moch H, Rochlitz C et al. (2001) Metastatic primitive neuroectodermal tumor (PNET) of the kidney in adults: management of two patients. Eur Urol 39:613–617Google Scholar
  8. 8.
    Clark J, Rocques PJ, Crew AJ et al. (1994) Identification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcoma. Nat Genet 7:502–508PubMedGoogle Scholar
  9. 9.
    Crew AJ, Clark J, Fisher C et al. (1995) Fusion of SYT to two genes, SSX1 and SSX2, encoding proteins with homology to the Kruppel-associated box in human synovial sarcoma. Embo J 14:2333–2340Google Scholar
  10. 10.
    Delahunt B (1999) Sarcomatoid renal carcinoma: the final common dedifferentiation pathway of renal epithelial malignancies. Pathology 31:185–190CrossRefGoogle Scholar
  11. 11.
    Delahunt B, Beckwith JB, Eble JN et al. (1998) Cystic embryonal sarcoma of kidney: a case report. Cancer 82:2427–2433CrossRefPubMedGoogle Scholar
  12. 12.
    DeLong W, Grignon DJ, Eberwein P et al. 1993) Sarcomatoid renal cell carcinoma. An immunohistochemical study of 18 cases. Arch Pathol Lab Med 117:636–640Google Scholar
  13. 13.
    Devaney K, Vinh TN, Sweet DE (1993) Small cell osteosarcoma of bone: an immunohistochemical study with differential diagnostic considerations. Hum Pathol 24:1211–1225PubMedGoogle Scholar
  14. 14.
    Enzinger F, Weiss S (1995) Synovial sarcoma. In: Soft tissue tumors. Mosby, St. Louis/MO, pp 757–786Google Scholar
  15. 15.
    Farrow GM, Harrison EG Jr, Utz DC, ReMine WH (1968) Sarcomas and sarcomatoid and mixed malignant tumors of the kidney in adults. Cancer 22:545–550Google Scholar
  16. 16.
    Farrow GM, Harrison EG Jr, Utz DC (1968) Sarcomas and sarcomatoid and mixed malignant tumors of the kidney in adults. 3. Cancer 22:556–563PubMedGoogle Scholar
  17. 17.
    Fligman I, Lonardo F, Jhanwar SC et al. (1995) Molecular diagnosis of synovial sarcoma and characterization of a variant SYT-SSX2 fusion transcript. Am J Pathol 147:1592–1599PubMedGoogle Scholar
  18. 18.
    Folpe AL, Schmidt RA, Chapman D, Gown AM (1998) Poorly differentiated synovial sarcoma: immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors. Am J Surg Pathol 22:673–682CrossRefPubMedGoogle Scholar
  19. 19.
    Fritsch M, Epstein JI, Perlman EJ et al. (2000) Molecularly confirmed primary prostatic synovial sarcoma. Hum Pathol 31:246–250PubMedGoogle Scholar
  20. 20.
    Furman J, Murphy W, Jelsma P et al. (1996) Primary primitive neuroectodermal tumor of the kidney. Am J Clin Pathol 106:339–344PubMedGoogle Scholar
  21. 21.
    Gaertner E, Zeren EH, Fleming MV et al. (1996) Biphasic synovial sarcomas arising in the pleural cavity. A clinicopathologic study of five cases. Am J Surg Pathol 20:36–45CrossRefGoogle Scholar
  22. 22.
    Gelb AB, Simmons ML, Weidner N (1996) Solitary fibrous tumor involving the renal capsule. Am J Surg Pathol 20:1288–1295CrossRefGoogle Scholar
  23. 23.
    Grignon DJ, McIsaac GP, Armstrong RF, Wyatt JK (1988) Primary rhabdomyosarcoma of the kidney. A light microscopic, immunohistochemical, and electron microscopic study. Cancer 62:2027–2032Google Scholar
  24. 24.
    Guillou L, Coindre J, Gallagher G et al. (2001) Detection of the synovial sarcoma translocation t(X;18) (SYT;SSX) in paraffin-embedded tissues using reverse transcriptase-polymerase chain reaction: a reliable and powerful diagnostic tool for pathologists. A molecular analysis of 221 mesenchymal tumors fixed in different fixatives. Hum Pathol 32:105–112CrossRefPubMedGoogle Scholar
  25. 25.
    Gupta NP, Singh BP, Raina V, Gupta SD (1995) Primitive neuroectodermal kidney tumor: 2 case reports and review of the literature. J Urol 153:1890–1892PubMedGoogle Scholar
  26. 26.
    Hasegawa T, Matsuno Y, Shimoda T et al. (1999) Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol 30:1464–1473PubMedGoogle Scholar
  27. 27.
    Helliwell TR, King AP, Raraty M et al. (1995) Biphasic synovial sarcoma in the small intestinal mesentery. Cancer 75:2862–2866PubMedGoogle Scholar
  28. 28.
    Hiraga H, Nojima T, Abe S et al. (1998) Diagnosis of synovial sarcoma with the reverse transcriptase-polymerase chain reaction: analyses of 84 soft tissue and bone tumors. Diagn Mol Pathol 7:102–110CrossRefPubMedGoogle Scholar
  29. 29.
    Hisaoka M, Hashimoto H, Iwamasa T et al. (1999) Primary synovial sarcoma of the lung: report of two cases confirmed by molecular detection of SYT-SSX fusion gene transcripts. Histopathology 34:205–210CrossRefGoogle Scholar
  30. 30.
    Iwasaki H, Ishiguro M, Ohjimi Y et al. (1999) Synovial sarcoma of the prostate with t(X;18)(p11.2;q11.2). Am J Surg Pathol 23:220–226CrossRefPubMedGoogle Scholar
  31. 31.
    Karn CM, Socinski MA, Fletcher JA et al. (1994) Cardiac synovial sarcoma with translocation (X;18) associated with asbestos exposure. Cancer 73:74–78PubMedGoogle Scholar
  32. 32.
    Kawai A, Woodruff J, Healey JH et al. (1998) SYT-SSX Gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 338:153–160Google Scholar
  33. 33.
    Kim DH, Sohn JH, Lee MC et al. (2000) Primary synovial sarcoma of the kidney. Am J Surg Pathol 24:1097–1104CrossRefPubMedGoogle Scholar
  34. 34.
    Kleinschmidt-DeMasters BK, Mierau GW, Sze CI et al. (1998) Unusual dural and skull-based mesenchymal neoplasms: a report of four cases. Hum Pathol 29:240–245PubMedGoogle Scholar
  35. 35.
    Lasota J, Jasinski M, Debiec-Rychter M et al. (1998) Detection of the SYT-SSX fusion transcripts in formaldehyde-fixed, paraffin-embedded tissue: a reverse transcription polymerase chain reaction amplification assay useful in the diagnosis of synovial sarcoma. Mod Pathol 11:626–633Google Scholar
  36. 36.
    Marley EF, Liapis H, Humphrey PA et al. (1997) Primitive neuroectodermal tumor of the kidney—another enigma: a pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol 21:354–359CrossRefGoogle Scholar
  37. 37.
    Mayes DC, Fechner RE, Gillenwater JY (1990) Renal liposarcoma. Am J Surg Pathol 14:268–273PubMedGoogle Scholar
  38. 38.
    Merchant SH, Mittal BV, Desai MS (1998) Haemangiopericytoma of kidney: a report of 2 cases. J Postgrad Med 44:78–80Google Scholar
  39. 39.
    Mezzelani A, Dagrada GP, Sozzi G et al. (2000) SYT-SSX Fusion transcripts and epithelial differentiation in synovial sarcoma. Diagn Mol Pathol 9:234–235CrossRefGoogle Scholar
  40. 40.
    Miettinen M, Limon J, Niezabitowski A, Lasota J (2001) Calretinin and other mesothelioma markers in synovial sarcoma: analysis of antigenic similarities and differences with malignant mesothelioma. Am J Surg Pathol 25:610–617CrossRefGoogle Scholar
  41. 41.
    Moch H, Gasser T, Amin MB et al. (2000) Prognostic utility of the recently recommended histologic classification and revised TNM staging system of renal cell carcinoma: a Swiss experience with 588 tumors. Cancer 89:604–614PubMedGoogle Scholar
  42. 42.
    Muretto P, Lemma E, Grianti C et al. (1985) Inflammatory malignant fibrous histiocytoma of the kidney: an immunohistochemical and ultrastructural study. Tumori 71:147–153Google Scholar
  43. 43.
    Murphy WM, Beckwith JB, Farrow GM (1993) Tumors of the kidney, bladder, and related urinary structures. Armed Forces Institute of Pathology, Washington/DCGoogle Scholar
  44. 44.
    Naslund MJ, Dement S, Marshall FF (1991) Malignant renal schwannoma. Urology 38:477–479PubMedGoogle Scholar
  45. 45.
    Nicholson AG, Rigby M, Lincoln C et al. (1997) Synovial sarcoma of the heart. Histopathology 30:349–352PubMedGoogle Scholar
  46. 46.
    Nicholson AG, Goldstraw P, Fisher C (1998) Synovial sarcoma of the pleura and its differentiation from other primary pleural tumours: a clinicopathological and immunohistochemical review of three cases. Histopathology 33:508–513CrossRefPubMedGoogle Scholar
  47. 47.
    Nielsen GP, Shaw PA, Rosenberg AE et al. (1996) Synovial sarcoma of the vulva: a report of two cases. Mod Pathol 9:970–974PubMedGoogle Scholar
  48. 48.
    O’Connell JX, Browne WL, Gropper PT, Berean KW (1996) Intraneural biphasic synovial sarcoma: an alternative „glandular“ tumor of peripheral nerve. Mod Pathol 9:738–741PubMedGoogle Scholar
  49. 49.
    O’Malley FP, Grignon DJ, Shepherd RR, Harker LA (1991) Primary osteosarcoma of the kidney. Report of a case studied by immunohistochemistry, electron microscopy, and DNA flow cytometry. Arch Pathol Lab Med 115:1262–1265PubMedGoogle Scholar
  50. 50.
    Pantuck AJ, Barone JG, Amenta PS et al. (1996) Diagnosis and management of malignant perirenal schwannoma. Am Surg 62:1024–1027Google Scholar
  51. 51.
    Quezado M, Benjamin DR, Tsokos M (1997) EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney. Hum Pathol 28:767–771Google Scholar
  52. 52.
    Romics I, Bach D, Beutler W (1992) Malignant schwannoma of kidney capsule. Urology 40:453–455PubMedGoogle Scholar
  53. 53.
    Russo P, Brady MS, Conlon K et al. (1992) Adult urological sarcoma. J Urol 147:1032–1036PubMedGoogle Scholar
  54. 54.
    Shmookler BM (1982) Retroperitoneal synovial sarcoma. A report of four cases. Am J Clin Pathol 77:686–691PubMedGoogle Scholar
  55. 55.
    Störkel S, Eble JN, Adlakha K et al. (1997) Classification of renal cell carcinoma: Workgroup No. 1. Union Internationale Contre le Cancer (UICC) and the American Joint Committee on Cancer (AJCC). Cancer 80:987–989CrossRefPubMedGoogle Scholar
  56. 56.
    Tsuji S, Hisaoka M, Morimitsu Y et al. (1998) Detection of SYT-SSX fusion transcripts in synovial sarcoma by reverse transcription-polymerase chain reaction using archival paraffin-embedded tissues. Am J Pathol 153:1807–1812Google Scholar
  57. 57.
    Van de Rijn M, Barr FG, Collins MH et al. (1999) Absence of SYT-SSX fusion products in soft tissue tumors other than synovial sarcoma. Am J Clin Pathol 112:43–49PubMedGoogle Scholar
  58. 58.
    Vogelzang NJ, Fremgen AM, Guinan PD et al. (1993) Primary renal sarcoma in adults. A natural history and management study by the American Cancer Society, Illinois Division. Cancer 71:804–810PubMedGoogle Scholar
  59. 59.
    Wiener JS, Coppes MJ, Ritchey ML (1998) Current concepts in the biology and management of Wilms tumor. J Urol 159:1316–1325PubMedGoogle Scholar
  60. 60.
    Witkin GB, Miettinen M, Rosai J (1989) A biphasic tumor of the mediastinum with features of synovial sarcoma. A report of four cases. Am J Surg Pathol 13:490–499PubMedGoogle Scholar
  61. 61.
    Yurtsever H (1990) Primary osteosarcoma of the kidney. Case report and review of the literature. Pathologe 11:224–228PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • H. Moch
    • 1
    • 4
  • A. Wodzynski
    • 2
  • L. Guillou
    • 3
  • V. Nickeleit
    • 1
  1. 1.Institut für Pathologie der UniversitätBaselSchweiz
  2. 2.Kantonales Institut für PathologieBadenSchweiz
  3. 3.Institut Universitaire de PathologieLausanneSchweiz
  4. 4.Institut für PathologieUniversität BaselBaselSchweiz

Personalised recommendations