Primary Spinal Primitive Neuroectodermal Tumors Research Articles

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

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.

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

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.

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.

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.

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.

Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases

Primary spinal primitive neuroectodermal tumors (PNETs) are uncommon malignancies that are increasingly reported in the literature. Spinal PNETs, like their cranial counterparts, are aggressive tumors and patients with these tumors typically have short survival times despite maximal surgery, chemotherapy, and radiation. Because no standard management guidelines exist for treating these tumors, a multitude of therapeutic strategies have been employed with varying success. In this study the authors perform a comprehensive review of the literature on primary spinal PNETs and provide 2 new cases that highlight the salient features of their clinical management.

An unusual primitive neuroectodermal tumor in the thoracic epidural space

Primitive neuroectodermal tumor (PNET) is a generic term used to describe a group of histologically indistinguishable neoplasms, including cerebellar medulloblastomas, which are located at various sites in the central nervous system. Primary epidural PNETs are rare and few patients have been reported. We report a 15-year-old girl who presented with gradual onset, over 1 month, of upper back pain and bilateral lower leg weakness. A thoracic spine MRI showed a dumbbell-shaped epidural mass at T2–4 with right paraspinal and posterior mediastinal extension. Surgical resection of the epidural tumor for decompression was performed. The pathologic examination revealed a PNET. Primary spinal PNETs typically have a poor prognosis and optimal therapy has not yet been defined. Surgical resection, with the combination of chemo-radiotherapy or radiotherapy, leads to better outcomes. However, primary epidural PNETs may be classified as a subtype of spinal PNETs because they are free from intrathecal invasion. For these patients, surgery alone and surgery combined with radiotherapy or chemo-radiotherapy remain controversial. Our patient received surgery alone and, 1 year later, has experienced no local recurrence within the epidural space but the mediastinal part of the tumor has enlarged.

Papilledema and Visual Loss as the Presenting Signs of a Primary Spinal Primitive Neuroectodermal Tumor

We report a patient with a primary spinal primitive neuroectodermal tumor (PNET) who presented with papilledema and visual loss before the appearance of myelopathic symptoms. A 28-year-old woman had a 5-month history of intense headaches and progressive visual loss that affected her left eye more than her right eye. Best-corrected visual acuities were 20/50 in the right eye and 20/400 in the left eye. She was able to identify 11/11 Ishihara color plates with the right eye and 0/11 with the left eye. A relative afferent pupillary defect was noted in the left eye. Ophthalmoscopy revealed severe bilateral optic disc swelling associated with macular edema, retinal hemorrhages, and hard exudates (Fig. 1). Automated visual fields demonstrated severe constriction. Results of the neurological examination were normal.FIG. 1: Fundus photographs obtained at presentation show bilateral optic disc edema, hard exudates, and macular edema.Results of CT of the head and orbits and of brain MRI and magnetic resonance venography were normal. Lumbar puncture opening pressure was 630 mm H2O. There were 2 white blood cells (all mononuclear), 694 red blood cells, 500 mg/dL protein, and 16 mg/dL glucose. The elevated protein and decreased glucose levels raised concern for fungal or tuberculosis meningitis, although the patient did not have symptoms to suggest this diagnosis. Results of serum tests for syphilis, toxoplasmosis, HIV, hepatitis A, B, and C, tuberculosis, and Bartonella henselae were negative, as were results of cerebrospinal (CSF) fungal culture, India ink and KOH preparations, Gram stain, and acid fast bacilli (AFB) smear and culture. The patient was treated with 250 mg methylprednisolone intravenously every 6 hours for 5 days and 500 mg acetazolamide intravenously every 6 hours for 5 days with no improvement in visual function. A left optic nerve sheath fenestration was performed with stabilization of vision. Approximately 1 week after the optic nerve sheath fenestration was performed, the patient developed back pain and difficulty walking. Complete spine MRI showed 2 cervical intradural extramedullary masses at C3 and C7, 3 thoracic intramedullary enhancing masses at T4, T8, and T12-L1, and 2 cauda equina masses, one at the left S1 root and the other in the posterior lumbar vertebrae at L2 (Fig. 2). Chest and abdominopelvic MRI showed no abnormalities.FIG. 2: Three weeks after presentation, when the patient first reported back pain and difficulty walking, postcontrast T1 sagittal MRI shows intramedullary masses at C3 (A), C7 (A,B), T4 (B), T12 (C), and caudal equina (C), together with thickened, enhancing leptomeninges (C). Biopsy of the thoracic lesion showed findings consistent with primitive neuroectodermal tumor. (Courtesy of Liana Lopez, MD).The patient refused further medical treatment. Two months later, she returned to the hospital complaining of decreased movement and sensation of both lower extremities and worsening of vision. She subsequently underwent optic nerve sheath fenestration on the right side. A T5-T7 laminectomy with intramedullary biopsy showed a primitive neuroectodermal tumor (PNET). She refused further intervention and died 3 months later of respiratory failure. Papilledema associated with spinal cord tumors was first described by Taylor and Collier in 1901 (1). Since then the association of papilledema and spinal cord tumors has been well documented (2-12). However, papilledema and visual loss preceding myelopathic symptoms occurs much less frequently. There are 11 reported cases, including 1 in French and 1 in Spanish (13-19). In those patients, myelopathic signs developed between 1 and 7 months after the onset of visual symptoms. Five patients had hydrocephalus at presentation. All had elevated CSF protein with normal CSF glucose except for 1 patient with a low glucose level (19). The patient with the low CSF glucose level had a PNET but was treated for tuberculous meningitis before the correct diagnosis was made. We emphasize not only that visual symptoms from papilledema may be a presenting feature of a spinal cord tumor before myelopathic manifestations have appeared but also that spinal fluid analysis may show elevated protein and decreased glucose levels and thus be mistaken for signs of infectious meningitis unless proper spinal imaging is done. Cristobal Cruz Colon, MD Lilliam Díaz, MD Julio A. Rodríguez-Padilla, MD Luis Serrano, MD Department of Ophthalmology University of Puerto Rico Río Piedras, Puerto Rico [email protected]

Primary Cervical PNET mimicking as neurofibroma. A case report

Primitive Neuro Ectodermal tumor (PNET) are the lesions commonly occurring in intracranial locations, more often in children and young adult. These are aggressive and highly malignant tumors which may spread through CSF to the spinal cord. Occurrence of primary spinal PNET is rare. In the cord these can arise at any level and may be intramedullary, extramedullary or extradural in location. The appearance on MR studies may mimic a neurofibroma with intra and extradural components. The management includes surgical excision, radiotherapy and adjuvant chemotherapy. Despite good surgical excision, due to the aggressive nature of the tumor recurrence is likely and the life expectancy remains poor. An 18 year old right handed male presented with the complaint of pain in the neck and progressive weakness in both the upper and lower limbs more on left then right side of 4 month duration. Examination revealed wasting of the deltoid, supraspinatus and infraspinatus muscles on left side. Tone was increased in both lower limbs and the reflexes were brisk. Power was grade IV/V in RUL and RLL and III/V in LUL and LLL. Contrast MRI showed an extramedullary intradural mass of 3.4x1.2x1.7 cms in the spinal canal at C2 – C3 level compressing the cord and was extending to the C2 C3 neural foramen and thought to be neurofibroma with a major extradural component arising from the nerve root foramen. The patient was operated via C1-C4 laminectomy. Intra operative inspection revealed a large, vascular and firm tumor which had a major extradural component and was adherent to the left vertebral artery. The dura was opened and the intra and extradural components were excised. The intra foraminal portion of the tumor, which was adherent to the left vertebral was left. Histopathological examination revealed a highly cellular tumor which was infiltrating the surrounding meningeal and fibrocollagenous tissue with tumor cells arranged in small sheets suggestive of Primitive Neuro Ectodermal tumor (PNET). The patient did well after surgery and his power in upper and lower limbs improved to grade V on both the sides. A contrast CT scan of brain did not reveal any primary lesion in the brain thus ruling this out as a secondary lesion in the spine. The patient was advised adjuvant radiotherapy and chemotherapy, but was unable to take the treatment due to financial constraints. On follow up at the end of 4 months he was doing well. 6 months later he presented to us again with recurrence of progressive weakness in upper and lower limbs, as well as difficulty in breathing. A recurrence of the tumor was suspected and MRI of the cervical spine was repeated. This time it revealed two lesions; one at the previous (C2-3) level and the second lesion at the foramen magnum, which was compressing the cervico medullary junction anteriorly and encasing both vertebral arteries, which was a new development. The patient was re operated and both the lesions were excised by a posterior midline approach. The patient showed improvement post operatively with improvement in power and respiration. He was referred to the medical oncology department for chemotherapy. Primary Cervical PNET mimicking as neurofibroma. A case report

Primary primitive neuroectodermal tumor of the lumbar extradural space

Primary spinal primitive neuroectodermal tumors (PNETs) of the extradural space are very rare; only 10 cases have been reported in the English language literature. The histopathological diagnosis of primary spinal PNETs has been discussed for many years. These tumors have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach for these patients. The authors present a case of primary PNET located in the lumbar extradural space in a 13-year-old girl and report the clinical, radiological, histopathological, and surgical findings. They compare their findings with those from the other 10 cases reported in the literature and review relevant literature.

P.220 Extirpation of ossifying fibroma using the Le Fort I approach

Primary 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).There 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.The 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).Patients 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.

Primary primitive neuroectodermal tumor within the spinal epidural space: report of a case and review of the literature

Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare tumors and a have poor prognosis. In reviews of the literature, it is seen that primary intraspinal PNETs may arise at all levels of the spine and may be intramedullary, intradural-extramedullary, or epidural. Spinal epidural location of PNET is extremely rare and out of 22 cases of primary spinal PNETs reported to date, only two were epidural. Tumors within the epidural space of the spinal canal are most often metastatic neoplasms from different primary sites. Here we report a case of primary extradural PNET located in the thoracic spine in a 16-year-old boy and review the relevant literature.

Primary Intraspinal Primitive Neuroectodermal Tumor

Primary spinal primitive neuroectodermal tumor (PNET) is a rare condition, 18 cases of which have been reported in the literature. In general, this tumor is treated with surgery followed by radiotherapy and chemotherapy, but prognosis is still poor. An 18-year-old female patient with an intradural, extramedullary mass at L3-L5 levels is presented in this report. This is the first female patient with primary spinal PNET at lumbar region, second patient with spinal nerve root origin, and third one with intradural, extramedullary localization ever reported in the literature. After surgery, she was treated with craniospinal radiotherapy and four cycles of combination chemotherapy regimen consisting of vincristine, cyclophosphamide, doxorubicin alternated with ifosfamide, and VP-16. Currently, she is asymptomatic and alive at 25 months. The histopathologic, radiologic, and clinical findings of the patient are presented and relevant literature is reviewed.

Tumor neuroectodérmico primitivo de la cola de caballo. A propósito de un caso

Primitive neuroectodermal tumors are malignant neoplasms whose classification has been controversial. Spinal primitive neuroectodermal tumors are rare and at the cauda equina are even less common. We report the case of a 40 year-old man with a history of progressive back pain and gait difficulty. After the diagnosis of a primitive neuroectodermal tumor of the cauda equina, the patient presented seeding to the intracranial subarachnoid space followed by a poor outcome. We review the literature of primary spinal primitive neuroectodermal tumors.