Franc Gruberov tumor pankreasa

[Gandra]

Htela sam da pitam za ovaj tip tumora pankreasa, mojoj bratanici je operisan, sami lekari su se zacudili kad je stigao PH nalaz a na internetu nisam nasla nista o tome osim da je benigne prirode.Interesuje me da li daje recidive?Ako neko moze da mi objasni o kakvom se tumoru radi?Unapred zahvalna!!!

[dr ivica]

Verovatno osim patologa i retkih profesora niko drugi od lekara ne bi mogao ni da Vam kaze nista vise bez pomoci interneta.

Detaljno o tumoru:

Solid pseudopapillary tumor


Aka papillary and solid epithelial neoplasm, papillary-cystic neoplasm, Gruber-Frantz Tumor
1-2% of non-endocrine pancreatic neoplasms

Young (mean 30-35 years), 90% women, often non-white, favorable prognosis (95% cured after resection), low grade

Metastases (seen in 10%) to liver or peritoneum are associated with venous invasion, high nuclear grade and

necrosis; patients usually survive even with metastases

Not truly papillary or truly cystic

Low proliferative neoplasms with evidence of dysregulation of activating cyclins and inhibitory proteins; may derive from pluripotent indifferent stem cells capable of endocrine and exocrine differentiation

Symptoms: abdominal pain / mass

Treatment: wide excision

Poor prognostic factors: venous invasion, high nuclear grade, “necrobiotic nests”

Gross: large (mean 9 cm), hemorrhagic, necrotic, usually encapsulated, rarely multifocal (Archives 1991;115:958)

Gross images: image1, image2

Micro: cellular (resembles islet cell tumor and ependymomas); pseudopapillae with hyalinized fibrovascular cores lined by several layers of bland fragile epithelial cells with nuclear grooves, indistinct nucleoli, few mitoses; may have PAS+ hyaline globules, foam cells, clusters of lipid/cholesterol crystals surrounded by foreign-body giant cells; fibrovascular core contains mucinous changes; tumor friable but not necrotic; may have pseudocystic areas

“Pseudopapillae” due to solid nests minus cells degenerating away from the small vessels; look like rosettes in cross section; tumor cells infiltrate without any stromal reaction

Micro images: image1, image2

Positive stains: vimentin, chymotrypsin, trypsin, estrogen and progesterone receptors, CD10, CD56 (intense, diffuse, AJSP 2000;24:1361), focal neuroendocrine markers; hyaline globules are PAS+ (for alpha-1-antitrypsin), variable alpha-1-antitrypsin

Negative stains: chromogranin, CEA, acinar and ductal markers (keratin may be patchy)

EM: large electron dense granules with complex internal membranous and granular inclusions and alpha-1-antitrypsin

Molecular: most tumors diploid; case report of unbalanced chromosome translocation der(17)t(13;17)(q14;p11) resulting in loss of 13q14-->qter and 17p11-->pter; tumor had cellular pleomorphism, tumor giant cells, tetraploid population but was otherwise typical, AJSP 1996;20:339

DD: pancreatic endocrine tumor (diffuse and strong endocrine immunoreactivity), adrenal cortical tumors (positive for inhibin, vimentin, keratin)

References: Mod Path 2001;14: 47, Archives 2002;126:985 (case report), AJSP 1987;11:85, Archives 2001;125:971 (case report), Archives 1995;119:268 (case report, liver metastases), Archives 1984;108:723

preuzeto sa sajta:
http://www.pathologyoutlines.com/pancreas.html


Samo da ukazem na nesto sto nije od znacaja za bolest i pacijenta koga pominjete, ali jeste kolegama, pa i pacijentima:

U medicini, kao i u drugim naukama, ima previse sindroma, bolesti, promena, metoda, itd.. - nazvanih po autorima koji su ih otkrili prvi primenili, opisali... Jedna od najboljih lista ovih zasluznih autora je ovde:

http://www.whonamedit.com/

Ako neceg/nekog nema na ovoj listi, onda je zaista rec o ili jako retkoj pojavi, ili akademskom izivljavanju onih koji koriste namerno imena retkih autora, samo da bi ispali pametniji - posebno u dopisivanju sa kolegama koji nisu iz iste branse.

U kontekstu prethodnog; patolog je lepo mogao da napise:

"Solidna pseudopapilarna epitelna neoplazija benigne prirode, Gruber-Frantz tumor"

Posebno je bitno da ne okrecemo red imena u nazivim, a kao i da nazive pocnemo da pisemo u originalu, a ne kako se mole svidi i kako ko ume da procita.

S postovanjem.

[Gandra]

Hvala vam puno na odgovoru i trudu oko razjasnjenja.