Malignant peripheral neuroectodermal tumors.A retrospective analysis of 42 patients

Abstract

The clinical presentation of the disease and the results of treatment in 42 patients with malignant peripheral neuroectodermal tumors (MPNT) entered into the Cooperative Ewing's, soft tissue, and neuroblastoma trials of the German Society of Pediatric Oncology were retrospectively analyzed. Within the Ewing's sarcoma trial, patients with chest wall lesions were particularly analysed for MPNT features. The period of observation ranged from 15 to 86 months; the median relapse-free time was 24 months. There were 28 male and 14 female patients, the median age of patients was 15 years (range, 9 months-23 years). Thirty-two patients had l o c a l i i disease (MO), and ten patients presented with primary metastases (Ml). The predominant location of the tumors was the thoracopulmonary region, followed by the extremities, the abdominal/pelvic, and head and neck region. Thirty-one of 42 tumors invohed the adjacent bone. The disease-free survival according to Kaplan-Meier life-table analysis was 56% f 11% for Stage MO patients at 3 years. Nine of ten patients with M1 disease showed progression of their disease. Most patients had combined modality treatment with surgery, chemotherapy and radiation therapy. Best results were obtained with extensive surgery. Radiation doses ranged from 20 to 60 Gy and could not be correlated with the outcome of the disease. Most recurrences occurred at the site of the primary tumor. In patients with primary chemotherapy after biopsy-proven diagnosis, the responsiveness of this disease to chemotherapy could be demonstrated. Combination chemotherapy containing anthracyclines and high doses of alkylating agents appeared superior. Cancer 61:349-357, 1988. ALIGNANT PERIPHERAL neuroectodermal tumors M (MPNT) form a distinct entity from other malignant small round cell tumors such as neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, and lymphoma and were first distinguished by their light and electron microscopic features. Their neuroectodermal origin was then confirmed by immunohistochemical rnethod~.~" Cytogenetic studies demonstrated a special relation of MPNT to Ewing's sarcoma.'-1° The MPNT arise in soft tissue and bone.

Peripheral primitive neuroectodermal tumor of head-neck region: our experience

“…The estimated incidence of PNET is 2.9 per million per year. In most large series PNET usually presents in the second decade of life with slight male preponderance [2,3,4]. But in our series, we found cases of PNET in 9 to 40 years age group with female preponderance.…”

“…The most common sites of metastases from PNET include lung, bone and bone marrow [7]. In a large series, the rate of metastases range from 20-31% with long-term survival rate of less than 25% [2,3,4]. In our series, we also found bone metastasis in one out of four cases of PNET.…”

Locally Advanced Askin’s Tumour in a Child—a Rare Case Report and Review of the Literature

“…The accepted protocol for the management of this tumour is neoadjuvant chemotherapy followed by surgical excision of the tumor followed by post operative chemotherapy with or without radiotherapy [1][2][3][4]. The neo-adjuvant chemotherapy leads to better local control of the disease, less extensive surgery and treats the distant microscopic metastasis.…”

“…Askin's tumor is a primitive neuroectodermal tumor (PNET) of the thoracopulmonary region described by Askin [1]. It develops from the soft tissues of the chest wall, particularly in the paravertebral region.…”

Peripheral Primitive Neuroectodermal Tumor Involving the Paravertebral and Retroperitoneal Regions.

“…Tumorsconsisting of small round cells of neuroectodermal origin are classified as central and peripheral PNET.The term peripheral PNETis used to describe a group of tumors that originate from peripheral nerve tracts (1,(4)(5)(6) reported. Peripheral PNETprobably includes the tumors described as malignant tumors of the thoracopulmonaryregion in childhoodand adolescence as a uniqueclinicopathologic entity (Askin tumor) (7)(8)(9).…”