Papillary Endothelial Hyperplasia of the Sella, Cavernous Sinus, Ethmoid and Sphenoid Sinus

Papillary endothelial hyperplasia(PEH), usually originates in extracranial soft tissue, is considered a reactive proliferation of endothlium and an unusual form of thrombus organization. However the occurance of PEH in the cranial cavity is very rare. The authors report a case of a PEH involving intracranial sella, parasella, cavernous sius, ethmoid and sphenoid sinus in an 11-year-old boy. The patient was presented with sudden right visual disturbance. Magnetic resonance image showed a well-enhanced mass in the right cavernous sinus extending into the ethmoid and sphenoid sinus. The cerebral angiography demonstrated a vigorous tumor staining supplied by both internal and external carotid arteries. The right fronto-temporal craniotomy and subtotal resection of the tumor were performed. The mass was comprised in a highly vascular red-black nodular tissue associated with a mixture of fresh and organizing thrombi. The histopathological examination revealed papillary structures lined by endothelium within vascular space with organized thrombus. While the patient was waiting for second operation in order to remove remaining small tumor in the ethmoid and sphenoid sinues, the lesion exhibited rapid regrowth and the size became to the preoperative size at three months after operation. At reoperation, gross total removal was accomplished by a combination of trans-cranial and trans-sphenoidal approaches. Postoperative radiotherapy was given.

Intracranial Endodermal Sinus Tumor

OBJECTIVE: Endodermal sinus tumor or yolk sac tumor is an uncommon malignant germ-cell neoplasm. This tumor was originally described as a germ cell tumor of the ovary or the testis. Intracranial endodermal sinus tumor is extremely rare and usually develop in the pineal or suprasellar regions. The authors evaluated the effect of adjuvant therapy(chemotherapy combined with radiotherapy) and radical removal of intracranial endodermal sinus tumors. MATERIAL AND METHODS: Between 1996 and 2001, four patients of intracranial endodermal sinus tumor were diagnosed with tumor marker(AFP) and biopsy. Three patients were treated with surgical removal and chemotherapy with cisplatin(20mg/m2), etoposide(100mg/m2) and bleomycin(15mg/m2) as well as external beam radiation therapy. We compared the management problems for these tumors. RESULT: In all three patients the tumor size and the level of tumor marker decresed during initial adjuvant therapy. However, Tumors showed regrowth with elevated AFP of serum and CSF possibly related to delayed chemotherapeutic treatment or inadequate administration of chemotherapeutic drugs due to severe bone marrow suppression. An additional chemotherapy and external radiation therapy were given, but tumors could not be controlled with leptomeningeal seeding. CONCLUSION: Radiotherapy is considered to be less effective. The combination chemotherapy with PVB(cisplatin, vinblastine, bleomycine) or PE(cisplatin, etoposide) is considered to be value in prolongation of the survival rate. But the role of chemotherapy in this tumor has not yet been clarified due to bone marrow suppression and drug resistance. Further study with large series of this tumor is necessary to establish the optimal management.