Preferencje help
Widoczny [Schowaj] Abstrakt
Liczba wyników
2015 | 71 | 05 |

Tytuł artykułu

Patomorfologia i patogeneza bardzo rzadko wystepujących grup nowotworów


Warianty tytułu

Pathomorphology and pathogenesis of very rare groups of tumors

Języki publikacji



The paper describes the pathomorphology of very rare neoplastic syndromes in humans and animals, namely mucosa-associated lymphoid tumors (MALTomas), gastro-intestinal stromal tumors (GISTomas), perivascular endothelial tumors (PEComas), multiple endocrine neoplasias (MEN), and neuroendocrine tumors (NETs). Particular attention is devoted to MALTomas of a non-Hodkins lymphoma type, originating from the marginal zone of lymphoid tissue accumulations, as well as to “secondary” lymphomas, originating on the basis of an earlier development involving a chronic lymphocytic inflammatory process, frequently of the type of autoimmune reaction. This group includes classic lymphoma, arising with cooperation of Helicobacter pylori, thyroid lymphoma (in the autoimmune Hashimoto disease), and salivary gland lymphoma (in the autoimmune Sjögren’s syndrome). Interestingly, the abovementioned lymphomas are examples of neoplastic development based on local, rather than systemic, autoimmune processes. Very seldom lymphomas of the MALTomata type can be encountered in the skin, breast, brain, bronchi, thymus, bone marrow, uterine cervix and endometrium, auditory canal, testis, and lymph nodes that drain the alimentary tract. All non-Hodkin lymphomas are malignant and monoclonal with a very differentiated immunofenotype and with karyotypic lesions, which may injure bone marrow, thus leading to anaemia and haemorrhagic diathesis. In MALTomatas, one can note disturbances in function of affected organ due to the chromosomal aberration of the BCL-10 and iMALT1 genes and disturbances in proteins corresponding to them, the role of which is the formation of complexes that control cell death. GISTomata (GISTs) stem from precursor stem cells for myocytes, interstitial Cajal cells, and other mesenchymal cells of the alimentary tract. In such tu mors, mutations develop in the c-kit gene, causing a permanent activation of protein KIT, transmembrane type 3 tyrosine kinase, which promotes cell proliferation. As a rule, they represent non-malignant tumors; only occasionally developing pseudocapsules. Both non-Hodkin lymphomas and GISTomata can be classified as tumors of the mesenchymomata type or tumors originating from mesoderm and mesenchyme. PEComata represent another group of mesenchymal tumors, which includes the so-called clarocellular “sugar” tumor of the lungs, characterized by the expression of HMB-45, S-100 protein, and vimentin. Associations are also described between MEN I and MEN II syndromes and tumors originating from neuroendocrine cells, that is, NET type tumors (neuroendocrine tumors), currently included in the DNS (diffuse neuroendocrine system) – the previous APUD system. The markers of net type tumors include neurospecific enolase, synaptophysin, chromogranin A and B, and HVMAT-II (human vesicular monoamine transporter isoform – 2). In MEN I, MEN II syndromes and in fibromatosis, mutations develop in suppressor genes, that is, respectively in MEN I (MEN I), RET (MEN II), NF1 and NF2 (fibromatosis). In addition, the paper suggests new names for a group of tumors involving GALTomas (gut-associated lymphoid tissue) and for ESTomas (endometrial stromal tumors). It describes cases of borderline tumors, non-malignant carcinomas, for which it is difficult or impossible to establish a correct differential diagnosis. A mention is also made of two new suppressor genes of neoplastic development, that is, the alfa-Klotho gene and the BLM I gene, whose loss of function allows the cell to enter the pathway of neoplactic development. The role of the CDH1 gene is also presented as an example of an epigenetic factor in neoplasia.








Opis fizyczny



  • Zakład Patomorfologii i Weterynarii Sądowej, Katedra Patologii, Wydział Medycyny Weterynaryjnej, Uniwersytet Przyrodniczy we Wrocławiu, ul.Norwida 31, 50-345 Wrocław


  • 1. Ashton-Key M., Biddolph S. C., Stein H.: Heterogenecity of bcl-2 expression in MALT lymphoma. Histopathology 1995, 26, 75-78.
  • 2. Bates A. W., Norton A. J., Baithun S. I.: Malignant lymphoma of the urinary bladder: a clinocopathological study of 11 cases. J. Clin. Pathol. 2000, 53,458-461.
  • 3. Bonetti F., Pea M., Martigoni G.: Clear cell (“sugar tumor”) of the lang is a lesion strictly related to angiomyolipoma – the concept of a family oflesions characterized by the presence of the perivascular epithelioid cell (PEC).Pathology 2003, 11, 156-160.
  • 4. Boon N. A., Colledge N. R., Walker B. R., Hunter J. A. A.: Choroby wewnętrzne Davidsona. T. 1, 2, 3. Elsevier Urban&Partner, Wrocław 2006.
  • 5. Changati R. S.: Recurring chromosomal abnormalities in non-Hodgkin’s lymphoma: histologic and clinical significance. Semin. Hematol. 2000, 37,396-405.
  • 6. Chaplin A., Conrad D. M., Tatlidi C., Jollimore J., Walsh N., Coverta A., Pasternak S.: Primary cutaneous PEComa. Am. J. Dermathology 2010, 32,310-312.
  • 7. Dabbs D. J.: Diagnostic immunohistochemistry. Churchill Lingviston Elsevier. Second Ed. 2006.
  • 8. Domagała W., Chosia M., Urasińska E.: Podstawy patologii. PZWL, Warszawa 2010.
  • 9. Farinha P., Gascoyne R.: Helicobacter pylori and MALT lymphoma. Gastroenterology 2005, 128, 1579-1605.
  • 10. Folpe A. L., Kwiatkowski D. J.: Perivascular epithelial cell neoplasms: pathology and pathogenesis. Hum. Pathol. 2010, 41, 1-15.
  • 11. Gaffey M., Milles S., Zarbo R.: Clear cell tumor of the lung: Immunohistochemical and ultrastructural evidence of melanogenesis. Am. J. Surg. Pathol. 1991, 15,644-653.
  • 12. Goldblum J. R.: Gastrointestinal stromal tumors. A review of characteristic morphologic, immunohistochemical and molecular genetic features. Am. J. clin. Pathol. 2002, 117, 49-61 (suppl.).
  • 13. Greenson J. K.: Gastrointestinal stromal tumors and other mesenchymal lesions of the gut. Mod. Pathol. 2003, 16, 366-375.
  • 14. Hormick J. L., Flechter C. D.: PEComa: what do we knows so far? Histopathology 2006, 48, 7-82.
  • 15. Isaacson P. G.: Gastrointestinal lymphomas of T- and B-cell types. Mod. Pathol. 1999, 12, 151-158.
  • 16. Kruger S., Schmidt H., Kausch I., Bohle A., Holzhausen H., Johannison R., Feller A.: Primitive neuroectodermal tumor (PNET) of the urinary bladder. Path. Res. Pract. 2003, 199, 751-754.
  • 17. Kruś S., Skrzypek-Fakhoury E. (red.): Patomorfologia kliniczna. PZWL, Warszawa 2007.
  • 18. Liu T. Y., Dei P. H., Kuno S. H., Lin C. W.: Early low grade MALToma rarely transforms into diffuse large cell lymphoma or progresses beyond the stomachand regional lymph nodes. J. Forms Med. Assoc. 2010, 6, 463-471.
  • 19. Mai K. T., Belanger E. C.: Perivascular epithelioid cell tumour (PEComa) of the soft tissue. Pathology 2006, 38, 415-420.
  • 20. Miettinen M.: Gastrointestinal stromal tumors. An immunohistochemical study of cellular differentiation. Am. J. Clin. Pathol. 1988, 89, 601-610.
  • 21. Miettinen M., El Rifai W., Sobin H. L.: Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum. Pathol. 2002, 33, 478-483.
  • 22. Miettinen M., Lasota J.: Gastrointestinal stromal tumors (GISTs): definition, occurance, pathology, differential diagnosis and molecular genetics. Pol. J.Pathol. 2003, 54, 3-24.
  • 23. Miettinen M., Sobin L. H., Sarlomo-Rikala M.: Immunohistochemical spectrum of GISTs at defferent sites and their differential diagnosis with a refereco toCD117 (KIT). Mod. Pathol. 2000, 13, 1194-1142.
  • 24. Milewicz A. (red.): Endokrynologia kliniczna. T. III. PTE, Warszawa 2012.
  • 25. Navratil E., Gaulard P., Kanavaros P.: Expression of the bcl-2 protein in B cell lymphoma arising from mucosa associated lymphoid tissue. J. Clin. Pathol.1995, 48, 18-21.
  • 26. Niederkorn J. Y., Stein-Streilein J.: History and pathology of immune privilage. Ocular Immun. Inflammation 2010, 18, 19-23.
  • 27. Pamizo-Santos A., Sola I., de Alava E.: Angiomyolipoma and PEComa are immunoreactive for Myo D1 in cell cytoplasmic staining pattern. Appl.Immunohistoch. Mol. Morphol. 2003, 11, 156-160.
  • 28. Popielarski M., Woźniak K.: Helikaza Blooma „strażnik” genomu. Post. Biol. Kom. 2013, 2, 183-192.
  • 29. Ritter J. H., Milles S. E., Gaffey M. J.: Clear cell tumors of the alimentary tract and abdominal cavity. Semin. Diagn. Pathol. 1997, 14, 213-219.
  • 30. Roitt I., Brostoff J., Male D.: Immunologia. PZWL, Wyd. Med. Słowiński Verlag, Warszawa 2000.
  • 31. Sapieżyński R.: Nowotwory tkanki krwiotwórczej u psów i kotów. Część II. Chłoniaki u kotów – przyczyny, postacie kliniczne i rozpoznanie. Życie Wet. 2008, 83, 462-468.
  • 32. Shanker V.: Soft tissue tumors perivascular epitheliod cell family of tumors (PEComa) of soft tissue general. Path. Qutl. 2013, 15, 2-12.
  • 33. Silbernagl S., Lang F.: Taschenatlas Pathophysiologie. 3 ed. Georg Thieme Verlag K.G., Stuttgart, Germany 2009.
  • 34. Stachura J., Domagała W.: Patologia – znaczy słowo o chorobie. T. I, II. PAU, Kraków 2003.
  • 35. Szymczak A., Forma E.: Struktura i funkcja białka Klotho. Folia Med. Lodz. 2012, 39, 151-187.
  • 36. Thiery J. P.: Epithelial – mesynchymal transitions in tumour progression. Nat. Rev. Cancer 2002, 2, 442-454.
  • 37. Tucker T., Wolkenstein P., Revuz J., Zeller J., Friedman J. A.: Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology2005, 65, 205-211.
  • 38. Qi J., Zhu Y. Q., Luo J., Tao W. H.: Hypermethylation and expression regulation of secreted frizzled – related protein genes in colorectal tumor. World J.Gastroenterol. 2006, 12, 7113-7117.
  • 39. Usdin K., Grabczyk E.: DNA repeat expresions and human disease. Cell Mol. Lif Sci. 2000, 57, 914-924.
  • 40. Veldhoen N., Stewart J., Brown R., Milner J.: Mutations of the p53 gene in canine lymphoma and evidence form germ line p53 mutations ine the dog.Oncogene 1998, 16, 249-255.
  • 41. Watanable R., Nanko A., Fukuda S.: Lymphocytoma cutis due to perced earrings. J. Cutan Pathol. 2006, 33 (Suppl.), 9-16.
  • 42. Watson P., Wood K. M., Lodge A., Mcintosh G. G., Milton I., Piggott N. H.: Monoclonal antibodies recognizing CD5, CD10 and CD23 in formalin – fixed,paraffin – embedded tissue: production and assessment of their value in thediagnosis of small B-cell lymphoma. Histopath. 2000, 36, 145-150.
  • 43. Wells A. P., Franz C.: Medullary carcinoma of the thyroid. World J. Surg. 2000, 24, 952-962.
  • 44. Williamson S. R., Cheng L.: Perivascular epithelial cell tumor of the urinary bladder. J. Urol. 2011, 185, 1473-1474.
  • 45. Yang E., Korsmeyer S.: Molecular thanatopsis. A discourse on the BCL2 family and cell death (review). Blood 1996, 88, 1291-1298.
  • 46. Yong L. S., Dawson C. W., Eliopoulos A. G.: The expression and function of Epstein-Barr virus encoded latent proteins. J. Clin. Mol. Pathol. 2000, 53,238-247.
  • 47. Yoshida M.: Multiple viral strategies of HTLH-1 for dysregulation of cell growth control. Annu. Rev. Immunol. 2001, 19, 475-482.
  • 48. Zachary J. E., McGavin M. D.: Pathologic basis of veterinary disease. Elsevier Mosby St. Louis, Missouri 2012.

Typ dokumentu



Identyfikator YADDA

JavaScript jest wyłączony w Twojej przeglądarce internetowej. Włącz go, a następnie odśwież stronę, aby móc w pełni z niej korzystać.