ONCOLGY CONFERENCE 3-28-2011

CNS MENINGEAL SARCOMAS --RARE

MENINGEAL HEMANGIOPERICYTOMA

INTRADURAL CAUDA EQUINA EWING SARCOMA

1938 CUSHING TERMED A SPECIFIC MENINGEAL TUMOR AS AN ANGIOBLASTIC MENINGIOMA.  SHORTLY THEREAFTER  A SOFT TISSUE SARCOMA, HEMANGIOPERICYTOMA, WAS RECOGNIZED AS HISTOLOGICALLY IDENTICAL TO THE ANGIOBLASTIC MENINGIOMA.

MENINGEAL SARCOMAS ARE GENETICALLY DISTINCT FROM MENINGIOMAS. 

THIS IS A MALIGNANT PRIMARY NEOPLASM OF THE MENINGES.

THESE TUMORS ARE ASYMPTOMATIC FOR SHORTER PERIODS OF TIME DUE TO THE RAPID TUMOR GROWTH.  THESE TUMORS BEHAVE LIKE A SOFT TISSUE SARCOMA.  THERE IS A SLIGHT MALE PREPONDERANCE AND A TENDENCY TO OCCUR AROUND THE AGE OF 40.

MANAGEMENT IS DIFFICULT AND AGGRESSIVE SURGERY IF POSSIBLE IS THE TREATMENT OF CHOICE.  COMPLETE REMOVAL AT THE TIME OF THE FIRST SURGERY  IS BEST AS RECURRENCE IS BASED ON THE EXTENT OF RESECTION. 

OBTAINING TUMOR FREE MARGINS IN THE BRAIN OR SPINAL CORD CAN CAUSE SIGNIFICANT NEUROLOGICAL DEFICITS AND THE QUALITY OF LIFE HAS TO BE CONSIDERED.

SKULL AND DURAL RECONSTRUCTION ARE REQUIRED.

THESE TUMORS HAVE A RELENTLESS TENDENCY TO RECUR EVEN AFTER COMPLETE SURGICAL RESECTION.  THE  5  AND 10 AND 15 YEAR RECURRENCE RATES ARE 65% 76% AND 87%.

THE RATE OF METASTASIS AT 5,10, AND 15 YEARS ARE 13%, 33%, AND 64%.

INTRADURAL CAUDA EQUINA EWING SARCOMA

EWING SARCOMAS ARE EXTREMELY RARE BUT USUALLY DESCRIBED AS FOUND IN THE EPIDURAL SPACE PARTICULARLY IN THE SPINE OR INTRAPARENCHYMAL IN THE BRAIN

ORIGIN CNS SARCOMAS: MENIGEAL SARCOMA THAT ARISES DENOVO

OR AFTER RADIATION THERAPY (SUCH AS FOR A PITUITARY TUMOR)

OR CLASSIFIED AS A MALIGNANT MENINGIOMA

OR AS A GLIOSARCOMA (NEUROECTODERMALLY DERIVED GLIOBLASTOMA)

OR SCALP AND SKULL NEOPLASM WITH INTRACRANIAL EXTENSION OR INTRACRANIAL METASTASES

ALMOST ALL CNS SARCOMAS ARISE FROM THE DURA BUT INFREQUENTLY INTRACRANIAL INTRAPARENCHYMAL SARCOMAS ARISE FROM THE PARENCHYMA OF THE BRAIN  SUCH AS  MESENCHYMAL TISSUE i.e.. BLOOD VESSELS, CHOROID PLEXUS

FIBROSARCOMA AND SARCOMATOUS DEGENERATION OF A MENINGIOMA MAY BE DIFFICULT TO DISTINGUISH BETWEEN BUT MIGHT BE IDENTIFIED BY STAINS AS A MENINGIOMA USUALLY STAINS FOR EPITHELIAL MEMBRANE ANTIGEN BUT DOES NOT EXCLUDE IT OR THE PATIENT HAD A PRIOR HISTORY OF A MENINGIOMA RESECTION OR FINDINGS OR WHORLS OR PSAMMOMA BODIES.

TREATMENT OPTIONS INCLUDE SURGERY  BUT THE SUPPORTING ROLES OF RADIOTHERAPY AND CHEMOTHERAPY ARE UNCLEAR.

SURGICAL GOAL IS COMPLETE SURGICAL EXCISION. BUT TOTAL EXCISION IS DIFFICULT WITHOUT THE SACRIFICE OR FUNCTIONING BRAIN OR CNS TISSUE AND VITAL ASSOCIATED BLOOD VESSELS.

MEDIAN SURVIVAL IS LESS THAN 24 MONTHS.  IN CHILDREN MENINGEAL SARCOMATOSIS IS FATAL IN A FEW WEEKS.