CORDOMA DEL CLIVUS PDF

I tumori del clivus possono essere benigni o maligni; essi possono essere classificati come cordomi o condrosarcoma. I cordomi sono tumori rari, aggressivi a lenta crescita, e localmente distruttivi che originano dal notocorda, una struttura che embrionale che guida la crescita delle ossa craniche e della colonna vertebrale. Normalmente, i residui della notocorda formano parte dei dischi intervertebrali. Un cordoma si verifica quando parte delle cellule supplementari della notocorda restano racchiuse nelle ossa in via di sviluppo. Questi tumori rari sono lesioni benigne a lenta crescita, ma possono invadere le strutture vicine e tendono a recidivare dopo il trattamento, possono distruggere il tessuto circostante, e possono diffondersi in altre parti del corpo.

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Imaging differential diagnosis Epidemiology Chordomas can occur at any age but are usually seen in adults years. Those located in the spheno-occipital region most commonly occur in patients years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group peak around 50 years They are commonly found in Caucasians 3. Pathology Macroscopically, chordomas present as firm masses.

Fluid and gelatinous mucoid substance associated with recent and old hemorrhage and necrotic areas are found within these tumors. In some patients, calcification and sequestered bone fragments are found as well. The variety of these components may explain the signal heterogeneity observed on MRI. Microscopically, chordomas are characterized by physaliphorous cells. These tumors are often poorly marginated and microscopic distal extension of tumor cells likely explains the frequency of recurrences.

Three subtypes are recognized 11,14, conventional chordoma most common chondroid chordoma best prognosis dedifferentiated chordoma least common, worst prognosis True malignant forms of chordomas occasionally have areas of typical chordoma as well as undifferentiated areas, most often fibrosarcoma. The prognosis in such cases is poor. In this location, a male predilection has been reported M:F ratio of , and the tumor may be particularly large at presentation Chordoma is the most common primary malignant sacral tumor 9, Typically the mass projects posteriorly at midline, indenting the pons; this characteristic appearance has been termed the so-called thumb sign.

In contrast to sacrococcygeal tumors, there is no recognized gender difference. Vertebral bodies Chordomas of the vertebral bodies are rare, but nonetheless, after lymphoproliferative tumors, are the second most common primary malignancy of the spine in adults 2.

They most commonly involve cervical particularly C2 , followed by lumbar, and then the thoracic spine. They often extend across the intervertebral disc space, involving more than one vertebral segment. They may extend into the epidural space compressing the spinal cord, or along the nerve roots enlarging the neural foramen. CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion.

MRI provides excellent anatomical delineation of adjacent structures and is able to characterize the signal of the lesion usually allowing for a confident preoperative diagnosis. MRI is, however, limited in its ability to evaluate calcification and the precise involvement of skull base osteolysis less well than CT, especially for skull base foramina.

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CHE FARE DI FRONTE ALLA DIAGNOSI DI CORDOMA CLIVUS

Inserisci la tua email per non perderti i nuovi articoli! Quelli intracranici si localizzano alla base cranica, soprattutto nella regione del clivus, e vengono quindi definiti col nome di cordoma clivus altre sedi possono essere il dorso della sella, la fossa cranica posteriore. Spesso invadono anche il tessuto osseo adiacente. Questi i freddi dati standard offerti dalla chirurgia oncologica americana. Non esistono statistiche aggiornate su chi sfugge al sistema medico e decide di non farsi operare. Una ventina di anni fa risultava, da statistiche mediche, che la sopravvivenza media sui tumori in generale, a partire dalla diagnosi, fosse di 3 anni per chi si operava e di 11 anni per chi si rifiutava.

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