Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India

Abstract

Maximal safe resection followed by craniospinal irradiation and systemic chemotherapy with 6-12 cycles of an alternating regimen of VAC and IE is a reasonable treatment strategy in patients with nonpineal SPNET.

Gamma Knife Surgery for Recurrent or Residual Supratentorial Primitive Neuroectodermal Tumors

“…Currently, the standard treatment strategy consists of a maximal safe resection followed by adjuvant radiotherapy and multiregimen systemic chemotherapy [2,12,13,15,16]. Despite these multimodal treatments, the recurrence of PNET is common: 30-40% in patients with PNET treated by conventional treatment [4].…”

“…Furthermore, recurrent tumors can be located in any area of the CNS via leptomeningeal seeding. In recurrent PNETs, additional resection and second-line chemotherapy can be considered according to the type of recurrence, but these salvage treatment options have shown only limited results [16]. The failure of conventional treatment to control local and distant recurrences indicates the need for additional therapies.…”

Keyhole surgery of pineal area tumors - personal experience in 22 patients

“…In adults, these tumors are rare. Most pineal tumors originate from the pineal gland – pineocytoma and pineoblastoma [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 24 ], some other tumors in this area are PNET, glial tumors, metastatic lesions, meningiomas, papillary tumors [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 ]. Other lesions such as central neurinoma [ 17 , 18 ] and cavernous angioma [ 13 ] are extremely rare.…”

“…The extensive variety of possible histological subtypes makes it imperative to establish a tissue diagnosis in patients with pineal region tumor. Management decisions regarding adjuvant therapy, prognosis, and follow-up strategies vary based on the histological diagnosis [ 2 , 4 , 5 , 24 ]. The patients were healthy after total removal of the benign tumors in our study.…”

“…These lesions are challenging to access due to the deep surgical field and the surrounding associated critical neurovascular structures. The most prevalent pineal tumors are pineocytomas, pineoblastomas and primitive neuroectodermal tumors (PNET) [ 3 , 4 , 5 , 6 , 7 , 8 ]. In addition, glioblastomas [ 9 , 10 ], metastatic tumors [ 11 , 12 ], vascular malformations [ 13 ], papillary tumors [ 14 ], meningiomas [ 5 , 16 ] and neurocytomas [ 17 , 18 ] can also occur.…”