Limfomul malign primar osos

2 Septembrie, 2010 - 23:00

de Sharad Mathur,
Staff Physician, Department of Pathology, Kansas City VA Medical Center

Limfomul primar osos este o afectiune care trebuie separata de complicatiile scheletice in limfoamele sistemice. Primul este o forma rara de debut extranodal a limfoamelor ne-hodgkiniene si poate reprezenta o prioritate pentru medicul ortoped prin efectuarea biopsiei in cazurile leziunilor osoase pe fondul unui sarcom osos jogos casino online.

Limfoamele sistemice, prin definitie, sunt afectiuni in stadiul IV si de obicei nu ajung in atentia medicului ortoped, exceptie facand cazurile in care este nevoie de tratament pentru complicatii ca fracturile patologice Bonus Fara Depunere

Definitia limfomului primar osos continua sa fie controversata. Cei mai multi autori exclud ideea unei afectiuni diseminate sau recurente in care osul este unul din multele locuri in care boala s-ar putea dezvolta.

Pentru a diagnostica limfomul primar osos trebuie urmarite cateva criterii:
- identificarea histologica a limfomului in os
- leziunea osoasa singulara sau multiplele leziuni ale scheletului fara implicarea nodulilor limfatici sau a altor tesuturi limfoide
- nedezvoltarea unui nodul limfatic
- extensia tisulara moale de la leziunea osoasa este acceptata si dezvoltarea acesteia poate fi probata pentru a confirma limfomul malign.

Limfomul primar osos constituie 3% dintre tumorile primare osoase si 5% dintre limfoamele-extranodale (sau aproximativ 2% din totalul limfoamelor primare ne-hodgkiene).

Complicatiile secundare ale maduvei spinarii in limfom se vede la 5-15% dintre pacientii afectati de boala Hodgkin si la 30-53% dintre pacientii cu limfom ne-hodgkien. Mai mult de 50% dintre pacientii cu SIDA asociata bolii Hodgkin prezinta complicatii secundare ale maduvei osoase.

Barbatii sunt mult mai frecvent afectati decat femeile (raport de 1,8:8). Boala afecteaza toate varstele, desi limfomul este neobisnuit in copilarie. Cei mai multi pacienti se afla intre cea de-a cincea si a saptea decada a vietii lor.

Etiologia limfomului osos este necunoscuta. Agentii virali si imunosupresia sunt implicati in unele cazuri. Limfomul primar osos a fost identificat si in limfoproliferari post-transplant la pacientii imunosupresivi. Osul a fost studiat si ca locul limfomului primar la pacientii cu SIDA. Rar, pacienti cu boala Paget pot dezvolta limfom malign.

Anormalitatile moleculare si citogenice sunt implicate in patofiziologia mai multor limfoame. Cum era de asteptat acestea pot fi identificate in limfomul primar osos.

Cele mai frecvente anormalitati urmarite:
- t(14;18)(q32;q21) - supraexpresia proteinei Bcl-2, intalnita in 80-90% dintre limfoamele foliculare cu celule B si 20% dintre limfoamele difuze cu celule mari;
- t(8:14)(q24;q32) - supraexpresia proteinei Myc, intalnita in 80% dintre limfoamele Burkitt; diversele mutatii t(2;8)(p11;q24) sau t(8;22)(q24;q11) se mentin in 20% dintre cazuri;
- t(2;5)(p23;q35) - Formarea NPM-ALK - proteina de fuziune, se intalneste in cazul limfoamelor cu celule T;
- t(11;14)(q13;q32) - supraexpresia ciclinei D1, gasita in invelisul celulelor limfomului.

Cel mai frecvent simptom al prezentei unui limfom osos este durerea, prezenta la 60-100% dintre pacienti. Alte semne: umflatura dureroasa la palpare sau fracturile patologice. Pacientii de varsta pediatrica pot prezenta deficite functionale ale membrelor.

Simptomele sistemice, de exemplu, pierderea de greutate, febra, transpiratia nocturna, sunt intalnite la 10% dintre pacientii cu limfom osos.

Hipercalcemia este intalnita la unii pacienti cu varsta pediatrica si a fost asociata cu prognoze insuficiente.

A fost mentionat mai sus ca limfomul primar osos a fost asociat rareori cu SIDA, imunosupresia si cu boala Paget.

Indicatiile medicilor ortopezi pentru impunerea de masuri chirurgicale in cazul limfomului osos depinde de: diagnostic; tratamentul pentru fracturile patologice sau cele asupra carora planeaza suspiciunea de a fi patologice; decompresia canalului spinal compromis.

Procedurile diagnosticarii sunt indicate in mod frecvent in prezenta unei singure leziuni a osului cu diagnoza diferentiala inclusiv limfom si stadiile metastazice, mielom si sau sarcom primar.

In cele mai multe instante, punctia-biopsie este mult mai adecvata pentru diagnosticarea limfomului osos, dar subtipurile specifice pot necesita biopsie deschisa atunci cand se constata prezenta unei formatiuni tisulare pentru care punctia este o procedura mult prea fina.

Dialogul deschis intre chirurgul ortoped si patolog este crucial pentru a asigura toate informatiile despre acest nou tesut, inclusiv informatii privind stabilirea imunofenotipului si studiile moleculare.

Ori de cate ori extensia tisulara a leziunii osoase este vizibila la studii prebiopsice, o biopsie ar trebui facuta luand in considerare aceasta extensie pentru a evita lezarile ulterioare ale osului, care ar putea duce la fracturi patologice.

Similar, in afectiunea diseminata cu implicatii osoase, cum ar fi atunci cand exista limfoadenopatie, nodulii limfatici sunt de obicei cei mai accesibili si, de aceea, sunt cele mai bune locuri pentru biopsie.

In ultima instanta, biopsia osoasa poate fi evitata daca acele caracteristici radiologice sunt "compatibile" cu afectiunea diseminata.
La pacientii selectati care sunt inapti si refractari la tratament pentru a evita fracturile in timpul perioadei de supraveghere, stabilizarea profilactica ar fi indicata.

In primul rand, evaluarea amenintarii aparitiei unei fracturi patologice trebuie aplicata atat in cazul limfomului osos cat si leziunilor metastazice ale osului. Cand stabilizarea profilactica este considerata mai importanta decat radioterapia, dialogul cu medicul oncolog este esential pentru a se asigura ca implantul nu va impiedica cursul tratamentului.

Odata ce fractura patologica apare la osul care a dezvoltat limfomul, stabilizarea chirurgicala este indicata in majoritatea cazurilor, particular la extremitatile de jos, la oasele lungi ale extremitatilor superioare si fracturile coloanei vertebrale.

Atunci cand fixarea interna este necesara, dialogul cu medicul oncolog este necesara determinarea in ce fel aceasta fixare influenteaza optiunea radioterapeutica. Varsta, starea de sanatate si disponibilitatea de a indeplini rigorile tratamentului sunt consideratii importante de luat in seama.

Ocazional, limfomul se dezvolta la coloana vertebrala. in aceste cazuri, tumora se poate extinde prin canalul spinal si atunci cand extensia tisulara rezulta intr-un deficit neurologic progresiv, decompresia poate fi necesara.
Excizia chirurgicala arareori este indicata in tratamentul primar al limfomului osos.

Deoarece interventia chirurgicala este folosita numai dupa efectuarea biopsiei si pentru administrarea complicatiilor, nu exista contraindicatii la aceasta procedura.

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