Skip to main content
SpringerLink
Log in

Entzündung, Malignität und Immunologie gastrointestinaler Spindelzelltumoren

Was gibt es abgesehen von GIST noch?

Inflammation, malignancy and immunology in gastrointestinal spindle cell tumors

What is beyond GIST?

  • Hauptreferate
  • Published:
Der Pathologe Aims and scope Submit manuscript

Zusammenfassung

Entzündungszellen sind bei einer Vielzahl mesenchymaler Tumoren im Gastrointestinaltrakt nachweisbar. Allerdings sind die Mechanismen für die Entstehung der Entzündung und die zugrundeliegenden Prozesse noch immer weitgehend unklar. Zu den klassischen Tumoren mit Entzündungsreaktionen zählen das Kaposi-Sarkom, entzündlich fibroide Tumoren, die sklerosierende Mesenteritis und der entzündlich-myofibroblastische Tumor. IgG4-positive fibroskleröse Krankheitsbilder haben unser Verständnis bzgl. Pathogenese und Behandlung entzündlicher Läsionen auch anderer anatomischer Lokalisationen tiefgreifend verändert. Die Entzündung wird zumindest bei einem Teil der Läsionen über virale Einflüsse (Kaposi-Sarkom) oder bakterielle Infektionen getriggert (IgG4-positive fibrosklerosierende Läsionen), während eine solche Wechselwirkung bei anderen Läsionen oft unklar ist. Es konnten Alterationen verschiedener Gene nachgewiesen werden (z. B. ALK-Rearrangements bei entzündlich myofibroblastischen Tumoren und PDGFRA-Mutationen in entzündlich fibromatösen Polypen sowie in einem Teil von gastrointestinalen Stromatumoren [GIST]). Bekannt ist, dass das entzündliche Milieu von GISTs sogar einen Einfluss auf die Prognose hat. Im Folgenden werden diagnostische Überlegungen für die Routine sowie der theoretische Hintergrund diskutiert.

Abstract

Many mesenchymal tumors and tumefactions associated with the gastrointestinal tract feature prominent inflammatory cells but the mechanisms for the inflammation and the processes themselves remain poorly understood. Such classic lesions include Kaposi sarcoma, inflammatory fibroid polyp, sclerosing mesenteritis and inflammatory myofibroblastic tumor but, more recently, the recognition of IgG4-related fibrosclerosing disease has resulted in modification of the views on pathogenesis and treatment of such inflammatory lesions in many anatomical sites. In some lesions the inflammation may reflect viral influences (Kaposi sarcoma) or a bacterial infectious trigger (IgG4-related fibrosclerosing disease) whereas in others such an interaction is unclear and alterations in various genes have been detected, such as anaplastic lymphoma receptor tyrosine kinase gene rearrangements in inflammatory myofibroblastic tumor and platelet-derived growth factor receptor alpha (PDGFRA) gene mutations in inflammatory fibroid polyp and some gastrointestinal stromal tumors (GIST). Even the inflammatory milieu of GISTs may have an impact on the outcome. This article discusses the practical diagnostic considerations as well as the theoretical background.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6

Literatur

  1. Huppmann AR, Orenstein JM (2010) Opportunistic disorders of the gastrointestinal tract in the age of highly active antiretroviral therapy. Hum Pathol 41(12):1777–1787 (PubMed PMID: 21078437)

  2. Braun M (1982) Classics in oncology. Idiopathic multiple pigmented sarcoma of the skin by Kaposi. CA Cancer J Clin 32(6):340–347 (PubMed PMID: 6812893)

  3. Chang Y, Cesarman E, Pessin MS et al (1994) Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi’s sarcoma. Science 266(5192):1865–1869 (PubMed PMID: 7997879)

  4. Horenstein MG, Moontasri NJ, Cesarman E (2008) The pathobiology of Kaposi’s sarcoma: advances since the onset of the AIDS epidemic. J Cutan Pathol 35(Suppl 2):40–44 (PubMed PMID: 18976418)

  5. Kao GF, Johnson FB, Sulica VI (1990) The nature of hyaline (eosinophilic) globules and vascular slits of Kaposi’s sarcoma. Am J Dermatopathol 12(3):256–267 (PubMed PMID: 1693818)

  6. Folpe AL, Chand EM, Goldblum JR, Weiss SW (2001) Expression of Fli-1, a nuclear transcription factor, distinguishes vascular neoplasms from potential mimics. Am J Surg Pathol 25(8):1061–1066 (PubMed PMID: 11474291)

  7. Folpe AL, Veikkola T, Valtola R, Weiss SW (2000) Vascular endothelial growth factor receptor-3 (VEGFR-3): a marker of vascular tumors with presumed lymphatic differentiation, including Kaposi’s sarcoma, kaposiform and Dabska-type hemangioendotheliomas, and a subset of angiosarcomas. Mod Pathol 13(2):180–185 (PubMed PMID: 10697276)

  8. Fukunaga M (2005) Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumours. Histopathology 46(4):396–402 (PubMed PMID: 15810951)

  9. Orchard GE, Wilson Jones E, Russell Jones R (1995) Immunocytochemistry in the diagnosis of Kaposi’s sarcoma and angiosarcoma. Br J Biomed Sci 52(1):35–49

    CAS  PubMed  Google Scholar 

  10. Hammock L, Reisenauer A, Wang W et al (2005) Latency-associated nuclear antigen expression and human herpesvirus-8 polymerase chain reaction in the evaluation of Kaposi sarcoma and other vascular tumors in HIV-positive patients. Mod Pathol 18(4):463–468 (PubMed PMID: 15578080)

  11. Vanek J (1949) Gastric submucosal granuloma with eosinophilic infiltration. Am J Pathol 25:397–411

    CAS  PubMed Central  PubMed  Google Scholar 

  12. Helwig E, Ranier A (1953) Inflammatory fibroid polyps of the stomach. Surg Gynecol Obstets 96:355–367

    Google Scholar 

  13. Liu TC, Lin MT, Montgomery EA, Singhi AD (2013) Inflammatory fibroid polyps of the gastrointestinal tract: spectrum of clinical, morphologic, and immunohistochemistry features. Am J Surg Pathol 37(4):586–592 (PubMed PMID: 23426127)

  14. Allibone RO, Nanson JK, Anthony PP (1992) Multiple and recurrent inflammatory fibroid polyps in a Devon family (‚Devon polyposis syndrome‘): an update. Gut 33(7):1004–1005 (PubMed PMID: 1644320)

  15. Anthony PP, Morris DS, Vowles KD (1984) Multiple and recurrent inflammatory fibroid polyps in three generations of a Devon family: a new syndrome. Gut 25(8):854–862 (PubMed PMID: 6745724)

  16. Schildhaus HU, Cavlar T, Binot E et al (2008) Inflammatory fibroid polyps harbour mutations in the platelet-derived growth factor receptor alpha (PDGFRA) gene. J Pathol 216(2):176–182 (PubMed PMID: 18686281. Epub 2008/08/08. eng)

  17. Hasegawa T, Yang P, Kagawa N et al (1997) CD34 expression by inflammatory fibroid polyps of the stomach. Mod Pathol 10(5):451–456 (PubMed PMID: 9160309)

  18. Pantanowitz L, Antonioli DA, Pinkus GS et al (2004) Inflammatory fibroid polyps of the gastrointestinal tract: evidence for a dendritic cell origin. Am J Surg Pathol 28(1):107–114 (PubMed PMID: 14707872)

  19. Montgomery EA, Meis JM (1991) Nodular fasciitis. Its morphologic spectrum and immunohistochemical profile. Am J Surg Pathol 15(10):942–948

    Article  CAS  PubMed  Google Scholar 

  20. Voltaggio L, Murray R, Lasota J, Miettinen M (2012) Gastric schwannoma: a clinicopathologic study of 51 cases and critical review of the literature. Hum Pathol 43(5):650–659 (PubMed PMID: 22137423. Pubmed Central PMCID: 3305846)

    Google Scholar 

  21. Rodriguez E, Tellschow S, Steinberg DM, Montgomery E (2014) Cytologic findings of gastric schwannoma: A case report. Diagn Cytopathol 42(2):177–180 (PubMed PMID: 24436245)

  22. Fanburg-Smith JC, Majidi M, Miettinen M (2006) Keratin expression in schwannoma; a study of 115 retroperitoneal and 22 peripheral schwannomas. Mod Pathol 19(1):115–121 (PubMed PMID: 16357842, Epub 2005/12/17. eng)

  23. Fletcher C, Unni K, Mertens FE (2002) World Health Organization classification of tumours. In: Sohin L (Hrsg) Pathology and genetics of tumours of soft tissue and bone. IACR Press, Lyon

  24. Fletcher C, Bridge J, Hogendoorn PC, Mertens FE (2013) WHO classification of tumours of soft tissue and bone. WHO Press, Geneva

  25. Chen TS, Montgomery EA (2008) Are tumefactive lesions classified as sclerosing mesenteritis a subset of IgG4-related sclerosing disorders? J Clin Pathol 61(10):1093–1097 (PubMed PMID: 18682417, Epub 2008/08/07. eng)

  26. Deshpande V, Zen Y, Chan JK et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25(9):1181–1192 (PubMed PMID: 22596100)

  27. Frulloni L, Lunardi C, Simone R et al (2009) Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med 361(22):2135–2142 (PubMed PMID: 19940298)

  28. Montgomery EA, Voltaggio L (2012) Biopsy interpretation of the gastrointestinal tract mucosa, vol 2: neoplastic. Wolters Kluwer, Philadelphia, S 104

Download references

Einhaltung ethischer Richtlinien

Interessenkonflikt. E. Montgomery, L. Voltaggio, M. Vieth geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

Author information

Authors and Affiliations

  1. Pathology, Oncology, and Orthopedic Surgery, Johns Hopkins Medical Institutions Baltimore, Weinberg Building Room 2242, 401 North Broadway, 21224, Baltimore, USA

    E. Montgomery MD

  2. Pathology, Johns Hopkins Medical Institutions, Baltimore, USA

    L. Voltaggio

  3. Institut für Pathologie, Klinikum Bayreuth, Bayreuth, Deutschland

    M. Vieth

Authors
  1. E. Montgomery MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. L. Voltaggio
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Vieth
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to E. Montgomery MD.

Additional information

The supplement this article is part of is not sponsored by the industry.

Der Abstract des Beitrags wurde erstpubliziert in Der Pathologe Supplement 1, 2014: 17

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Montgomery, E., Voltaggio, L. & Vieth, M. Entzündung, Malignität und Immunologie gastrointestinaler Spindelzelltumoren. Pathologe 35 (Suppl 2), 207–213 (2014). https://doi.org/10.1007/s00292-014-1978-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00292-014-1978-z

Schlüsselwörter

Keywords

Search

Navigation