Primitive neuroectodermal tumors (PNET) are highly malignant group of neoplasm of soft tissue that affects both children and adults. These malignant tumors are small round cells with neuroectodermal origin affecting soft tissues and bones rapidly. PNET was first described by Stout in 1918 and were thought to rise from the nerves. Peripheral primitive neuroectodermal tumors (pPNETs) are classified in the Ewing family of tumors (EFTs). Both these tumors have similar genetic alterations with different manifestations, and so are indistinguishably referred in the medical literature. Soft tissue tumors are associated prominently with peripheral primitive neuroectodermal tumors, while Ewing sarcoma is associated with bone tumor. These primitive neuroectodermal tumors have various clinical and pathological characteristics similar to many other types of small and round tumors. Hence, Batsakis et al in 1996 classified PNET family into three types based on the origin of tumor. The three types of PNET are CNS primitive neuroectodermal tumors (derived from the central nervous system), neuroblastoma (derived from autonomic nervous system), and peripheral primitive neuroectodermal tumors (originate from tissues other than autonomic or central nervous system).
Multidisciplinary therapeutic options are utilized for the treatment of PNET including surgery, chemotherapy, radiation therapy, and ventriculoperitoneal shunt. Surgery is carried out primarily for diagnosis and then for tumor removal. Removal of tumor is usually possible if it is supratentorial, and is less frequent in case of spine or pineal region. Radiation therapy is recommended if the tumor is not completely eradicated or if it reappears. Radiation therapy is followed by chemotherapy in order to interfere with growth or reproduction of the cancer. Ventriculoperitoneal shunt is a bypass, wherein a tubing is placed in the brain area in order to drain excess cerebrospinal fluid to space into the abdomen. Endoscopic third ventriculostomy is necessary in case of hydrocephalus (swelling of the brain).
The malignant nature of tumor is a major driver of the primitive neuroectodermal tumors treatment market owing to the recommended use of combination of therapies to combat aggressive nature of tumor. Recent advances in molecular diagnosis and cancer therapeutic combination treatments is expected to boost the growth of the primitive neuroectodermal tumors treatment market during the forecast period. Threat of drug failure, severe side effects of therapeutics, and high cost of research & development of better therapeutic options are expected to restrain the global primitive neuroectodermal tumors treatment market during the forecast period. However, rise in government funds and increase in awareness among the population about fatal diseases in emerging nations are expected to support the growth of the global primitive neuroectodermal tumors treatment market.
The global primitive neuroectodermal tumors treatment market can be segmented based on tumor type, treatment type, distribution channel, and region. In terms of tumor type, the market can be categorized into CNS primitive neuroectodermal tumors, neuroblastoma, and peripheral primitive neuroectodermal tumors. Based on treatment type, the global primitive neuroectodermal tumors treatment market can be classified into surgery, chemotherapy, radiation therapy, ventriculoperitoneal shunt, and others. In terms of distribution channel, the market can be divided into hospital pharmacies, drug stores, online pharmacies, and others. Based on region, the global primitive neuroectodermal tumors treatment market can be categorized into North America, Latin America, Asia Pacific, Europe, and Middle East & Africa.
Major players dominating the global primitive neuroectodermal tumors treatment market include Eli Lilly and Company, Novartis AG, Pfizer, Sanofi, GlaxoSmithKline plc, and Merck & Company.
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