Spinal Primitive Neuroectodermal Tumor Research Articles

Primary spinal peripheral primitive neuroectodermal tumour: a report of 3 cases and review of the literature

Peripheral primitive neuroectodermal tumours (PNETs) are rare and highly malignant tumours in the spine, with a predilection for young adults. There are no standard guidelines for treating these tumours. Surgical resection combined with postoperative radiotherapy and chemotherapy is a common and effective treatment at present. Even so, survival time of patients with these tumours is still very short. In this study, we present three rare cases of thoracic epidural PNETs and review the literature.

Spinal Primitive Neuroectodermal Tumor (PNET) in a 64-year-old Male Treated with Surgery, Radiotherapy, and Chemotherapy: A Case Report

Primitive neuroectodermal tumors (PNETs) are a heterogeneous group of malignant neoplasms found primarily in childhood and early adulthood. In this paper, we described the case of a 64-year-old male with primary spinal PNET, successfully treated with surgery, craniospinal radiotherapy, and concurrent chemotherapy. This is the case of a 64-year-old male who presented with a 2-month history of bilateral lower extremity weakness and numbness associated with urinary and bowel incontinence. Work-up was done, and the spine's plain magnetic resonance imaging (MRI) revealed a heterogeneously enhancing intradural lesion with an extradural component at the right T9/T10 level, causing mild to moderate cord compression. The patient underwent laminectomy and gross total resection of the said tumor. Histology and immunohistochemistry were consistent with a primitive neuroectodermal tumor of the spine. The tumor recurred three months after the surgery, and the patient was then referred for radiation therapy with concurrent chemotherapy. Repeat spinal MRIs with three- to six-month intervals after treatment showed no tumor recurrence as of August 2021. Primary spinal PNETs are rarely found in adults, especially in the elderly. These tumors currently have no recommendations or guidelines regarding their management. Thus, most cases are presently being managed based on studies on children and central nervous system (CNS) PNETs. This paper presented a case of a successfully treated primary spinal PNET in the elderly. The management was primarily based on studies done on that of the pediatric population and CNS PNETs.

Primary spinal peripheral primitive neuroectodermal tumor with acute presentation: A case report and review of literature

Primary spinal primitive neuroectodermal tumors (PNET) is a rare occurrence and carries a poor prognosis. A 13-year old female patient acutely presented with pain in the thoracic region, bilateral lower limb weakness, bladder and bowel dysfunction. Clinically paraplegia with truncal weakness, lower limb deep tendon reflexes of both side were absent and planter reflexes equivocal bilaterally. Preoperative MRI of thoracic spine revealed D4-D6 extradural SOL. A D4-D5 Laminectomy and left Cortico transversectomy done. Pathological findings were consistent with PNET. The clinical, histopathological, and radiological findings of the patient are presented.

A Rare Case of Primary Spinal Primitive Neuroectodermal Tumor with Long-term Follow-up

A Rare Case of Primary Spinal Primitive Neuroectodermal Tumor with Long-term Follow-up

P11.13 Metachronous spinal primitive neuroectodermal tumor after treatment of a hemispheric anaplastic oligodendroglioma through p53 germline mutation

P11.13 Metachronous spinal primitive neuroectodermal tumor after treatment of a hemispheric anaplastic oligodendroglioma through p53 germline mutation

Spinal Primitive Neuroectodermal Tumor in Children – A rare case report and literature review

Primitive neuroectodectodermal tumor is a rare tumor in childhood. It is aggressive childhood malignancies and offer a significant challenge to treatment. An eleven year old female baby presented with fever, pain and neurologic manifestation. Initialy it was diagnose as TB vertebrae and treated accordingly but no improvment rather new metastatic swelling appear after few days. Diagnosis was made by MRI and local tissue biopsy and immunohistohistochemistry. As it is rare and highly invasssive malignancy with poor out come the case was presented along with literature review.J. Paediatr. Surg. Bangladesh 6(2): 64-67, 2015 (July)

Primary primitive neuroectodermal tumor of spinal cord

Primarily spinal primitive neuroectodermal tumors are rare neoplasm. A 28-year-old female presented with complaints of pain in lower back, radiating to both lower limbs. Magnetic resonance imaging scan of the lumbosacral spine showed an intradural extramedullary space-occupying lesion. The patient underwent L2–L5 laminectomy with excision of the lesion. Histopathology and immunohistochemistry reports confirmed the diagnosis of primitive neuroectodermal tumor while a thorough metastatic workup ruled out secondary to the spinal cord. The patient developed recurrence at local site within a month after surgery, even before the adjuvant treatment could be started. She is being treated with chemotherapy (human resources protocol).

Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome

ObjectivePrimary spinal primitive neuroectodermal tumors are extremely rare entities. The purpose of this study was to analyze the differences in clinical and imaging characteristics and outcomes between primary spinal central PNETs (cPNETs) and peripheral PNETs (pPNETs). MethodsThere were 25 consecutive patients with primary spinal primitive neuroectodermal tumors enrolled. The diagnosis was cPNET in 6 patients with negative CD99 expression and pPNET in 19 patients with positive CD99 expression. Gross total resection (GTR) was achieved in 12 patients, subtotal resection was performed in 9 patients, and partial resection was performed in 4 patients. Postoperative chemotherapy was given to 14 patients, and radiotherapy was given to 16 patients. ResultsThe age at diagnosis was significantly younger in the cPNET group (mean 12.8 years) compared with the pPNET group (mean 22.5 years) (P = 0.040); the 2 pathologies did not show a significant difference in prognosis. GTR (P = 0.041), radiotherapy (P = 0.008), and GTR with radiotherapy (P = 0.009) were significant factors leading to a higher 2-year survival rate. Kaplan-Meier analysis showed that radiotherapy (P < 0.001) and GTR with radiotherapy (P = 0.040) resulted in a longer median survival time. Patients who underwent GTR, chemotherapy, and radiotherapy all together had the highest 1-year (100.0%) and 2-year (71.4%) survival rates and the longest median survival time (32 months). ConclusionsPatients with spinal cPNETs were younger compared with patients with pPNETs. The prognosis of spinal cPNETs and pPNETs was poor with no significant difference between the entities. The most beneficial treatment modality is GTR combined with adjuvant radiotherapy and chemotherapy.

Thoracic spinal primitive neuroectodermal tumor with dural tail mimicking meningioma: a missed diagnosis

Thoracic spinal primitive neuroectodermal tumor with dural tail mimicking meningioma: a missed diagnosis

Clinical presentation and long-term outcome of primary spinal peripheral primitive neuroectodermal tumors.

Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare entities that predominantly occur in children and young adults. Few studies have reported more than three cases. There are no current optimum treatment strategies due to the paucity of data. Here, we present 13 patients (nine females and four males) with primary intraspinal pPNETs who were surgically treated from April 2008 to February 2014. Histopathologic findings revealed the expression of CD99 in all cases. Limb weakness was the most common initial symptom (11/13, 85%). The tumors were located mainly at the cervical level (6/13, 46%) and in the epidural space (10/13, 77%). The radiological diagnosis was neurinoma or meningioma in most cases (10/13, 77%). Gross total resection was achieved in 77% (10/13) of patients. During a mean follow-up of 25.5months, local relapse occurred in 8 (61.5%) patients and distant metastases occurred in 8 (61.5%) patients. The overall 1-year survival rate was 77% (10/13), and the overall 2-year survival rate was 54% (7/13). The 2-year survival rate was 57.1% in patients with adjuvant chemotherapy and 50% in those without chemotherapy. Gross total resection and adjuvant radiotherapy with or without chemotherapy demonstrated a longer survival period (1-year survival rate: 100%; 2-year survival rate: 86%). Our data showed that primary spinal pPNETs are extremely rare and aggressive tumors with a poor prognosis. Radical resection is advocated. Gross total resection combined with adjuvant radiation may help to significantly improve patient survival period. Chemotherapy may also help to slightly prolong patient life.

Primary spine tumor view spinal primitive neuroectodermal tumor;Case presantation

Primary spine tumor view spinal primitive neuroectodermal tumor;Case presantation

Spinal Primitive Neuroectodermal Tumor Mimicking as Chronic Inflammatory Demyelination Polyneuropathy

We report a young boy who presented with progressive weakness of lower extremities associated with areflexia and abnormal electrophysiological findings initially suggestive of chronic inflammatory demyelinating polyneuropathy. Initial lumbosacral spinal magnetic resonance imaging (MRI) showed thickened descending spinal nerve roots only. Immunomodulating therapy was given but with limited clinical response. Repeated spine magnetic resonance imaging showed cauda equina and also new spinal cord extramedullary contrast enhancement. The initial extensive investigations including open biopsy did not point to any specific diagnosis. Only through pursuing a repeated biopsy, the diagnosis of the spinal peripheral primitive neuroectodermal tumor was confirmed. This case highlights the diagnostic challenges of the spinal peripheral primitive neuroectodermal tumor that could have an initial chronic inflammatory demyelinating polyneuropathy-like presentation. The literature review confirms that this is a rare condition and cauda equina origin has only been reported in adults and teenagers, and this is the first reported case in a young child.

1796 CASE SERIES OF PRIMARY PRIMITIVE NEUROECTODERMAL TUMORS OF THE KIDNEY

INTRODUCTION AND OBJECTIVES: Primitive neuroectodermal tumor (PNET) of the kidney is a rare disease often characterized by a young age of onset and aggressive clinical course. Because there are few case series and the optimal management is not well defined, we reviewed the combined experience of two large tertiary centers in the diagnosis and management of pts with renal PNET. METHODS: Following IRB approval at the University of Pittsburgh and Memorial Sloan-Kettering Cancer Center, we conducted a retrospective review of pts with surgically managed renal PNET treated from 1998?2012. RESULTS: There were 5 female and 3 male pts diagnosed at a median of 38 years (21 – 71 years). All pts had symptoms at presentation including abdominal pain (87.5%), anorexia (67.5%), and hematuria (50%). An abdominal mass was palpable in 50%. At presentation, 2 pts had metastatic disease, 3 had an IVC thrombus, and 2 had a renal vein thrombus. One pt had a history of a spinal PNET treated six years prior. The average radiographic size of the renal masses was 13.5cm (8.3-23cm) and 6 were left sided. All pts underwent radical nephrectomy and 50% had concurrent resections of the pancreas (3), spleen (2), colon (1), diaphragm (1), and/or psoas (1). Lymph nodes were positive in 25%, negative in 12.5%, and not reported in 67.5%. Three pts underwent percutaneous biopsy and the diagnosis of PNET was made in all 3 cases. One pt who had preoperative chemotherapy had complete tumor necrosis on final pathology. FISH analysis demonstrated EWS-FLI-1 and EWSR1 rearrangements in 2 and 3 pts, respectively. Four pts received chemotherapy prior to and 4 after surgery based on guidelines for the Ewing’s family of tumors. Radiation was performed for adjuvant (n 2) and palliative purposes (n 1). Median follow up was 27 mths (2.7 – 175 mths). Two pts died of disease at a median of 22.5 mths after diagnosis. Kaplan Meier estimates for 3 and 5 year overall survival were 62.5%, respectively. CONCLUSIONS: Renal PNET is a rare tumor that should be considered in younger pts presenting with a large renal mass associated with an IVC thrombus. The diagnosis can be made by percutaneous biopsy but more commonly it is established after nephrectomy. Treatment is multi-modal and chemotherapy is based on the Ewing’s family of tumors guidelines. Pts who present with metastatic disease have a poor overall prognosis (Fig 1). Source of Funding: Steven Hanson Family Kidney Cancer Research Fund.

Intracranial metastasis from primary spinal primitive neuroectodermal tumor

Primary spinal primitive neuroectodermal tumors (PNET) are rare tumors, with only 94 cases reported till date. Metastasis to brain from a spinal PNET is even rarer. In the present report, we evaluate the pathology and treatment of solitary intracranial metastasis from spinal PNET in a 22-year-old female who presented with headache and left hemiparesis and was diagnosed to have right parietal parasagittal tumor. She has been previously diagnosed to have cervicothoracic primary spinal PNET, and was treated by surgery, radiotherapy, and chemotherapy seven years back. The intracranial tumor has been removed and pathological examination confirmed as PNET. She received radiotherapy and chemotherapy with ifosfamide and etoposide, following surgery for the right parietal PNET. At 20 months follow-up, patient is stable and has no recurrence of the disease. Critical review of reported cases of primary spinal PNET metastsising to brain was done.

Imaging Characteristics of Primary Spinal Primitive Neuroectodermal Tumors

Primary spinal primitive neuroectodermal tumors (PNETs) are rare and classified into peripheral and central types because the treatment strategy and outcome are different. This study describes five cases of primary spinal peripheral type PNETs. Magnetic resonance imaging showed large extradural masses in the cervical and thoracic spine with bony invasion and paraspinal extension in four cases and a transdural mass in the sacral spinal canal in one case. We also briefly summarize the imaging characteristics of 36 peripheral type and six central type primary spinal PNETs in the literature.

PNET Spine

Spinal primitive neuroectodermal tumor (PNET) is rare. We present clinical, radiologic profile and treatment outcome of 15 spinal PNET patients from June 2003 to March 2010 treated with chemoradiotherapy. Median duration of backache was 6.5 months; all had features of myelopathy and/or radiculopathy; 5/15 (33.3%) patients were diagnosed initially as spinal tuberculosis. The event-free survival (EFS) was 24.73% at a median follow-up of 22 months. Complete functional recovery to treatment significantly predicted better EFS; 4 patients discontinued treatment because of poor functional recovery. It is important to recognize spinal PNET early to prevent permanent neurological damage, which in turn would improve compliance, quality of life, and perhaps EFS.

Intramedullary Spinal Cord Primitive Neuroectodermal Tumor Presenting With Hydrocephalus

Spinal primitive neuroectodermal tumors are exceedingly rare. Herewith, we present the first case of an intramedullary spinal cord tumor associated with hydrocephalus in a 2-month-old boy that presented with left hemiparesis. The patient had been diagnosed on prenatal ultrasound with enlarged ventricular system. At his current admission, a brain magnetic resonance imaging (MRI) revealed hydrocephalus and an intramedullary lesion extending from the second cervical to the first thoracic vertebrae. Dissemination of the tumor was revealed intracranially and in the spinal canal. After a ventriculoperitoneal shunt placement a radical resection of the tumor was performed, however some small tumor remnants could not be safely removed. Postoperative there was no neurologic deterioration. The tumor was diagnosed as a central nervous system primitive neuroectodermal tumor (World Health Organization grade IV). Spinal intramedullary primitive neuroectodermal tumors are extremely rare. In such rare tumors, multiinstitutional studies are needed for treatment guidelines to be established.

Primary spinal intramedullary primitive neuroectodermal tumor

Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual. The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery. The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

Primary spinal extradural Ewing′s sarcoma (primitive neuroectodermal tumor): Report of a case and meta-analysis of the reported cases in the literature

Background:Primary spinal primitive neuroectodermal tumors (PNET) and/or spinal extraskeletal Ewing's sarcoma family tumors (ESET) are rare lesions appearing in the spinal extradural space. One hundred forty-one primary spinal PNETs, including 29 intramedullary lesions, have been reported in the literature. Encountering a case of primary epidural EES/peripheral PNET (pPNET) in sacral level, which is the fifth one occurring at this level in the literature, we have tried to conduct a meta-analysis of the reported cases.Case Description:A 44-year-old lady with epidural EES/pPNET is reported here. She was once operated for L5/S1 herniated disc, which did not ameliorate her symptoms. The clinical, imaging, surgical, and histopathologic characteristics of our case are presented and wide search of the literature is also done. All the reports were level 3 or less evidences and most of the series had missing parts. 106 cases of primary intraspinal (extradural/extramedullary-intradural) EES/pPNET and 29 cases of primary intramedullary PNET (CNS-PNET) have been reported in the literature. The most common clinical presentation in both entities was muscle weakness proportionate to the tumor location. Distant metastasis occurred in 38 of 99 (38%) cases of primary intraspinal EES/pPNET, while the rate of metastasis was 48% in patients with PNETs occurring in the intramedullary region (P > 0.05). One-year survival rate of the patients who underwent chemo-radiation after total or subtotal resection was better than those who did not receive chemotherapy or radiotherapy, or did not have total or subtotal resection. However, this difference was not repeated in 2-year survival rate in any of the tumor groups.Conclusion:It seems that total or subtotal removal of the tumor and adjuvant chemo- and radiation therapy can improve the outcome in these patients.

Intramedullary PNET of the Spine

Primitive neuroectodermal tumors (PNET) originating within the spinal cord are extraordinarily rare. We report a female who presented at age 21 with diffuse involvement of the lower spinal cord. After biopsy and successful treatment with radiation and chemotherapy, she recurred 10 years later with disease in her cerebellum. She was reinduced with chemotherapy and subsequently received high-dose chemotherapy with autologous stem cell support. She is alive and free of disease 11 years after her initial presentation. This represents the longest survival ever documented for a primary spinal PNET.