A primitive neuroectodermal tumor (PNET) is an undifferentiated sarcoma that arises from neural crest cells [1]. These tumors develop in the central nervous system (CNS) or peripherally in soft tissues and bones. Specifically, a peripheral PNET involves organs such as the kidneys [2], bladder [3], female genital tract [4], myocardium, pancreas, retroperitoneum, chest wall, or lungs [5]. These undifferentiated tumors predominantly affect children and adolescents while exhibiting a slight predilection for males [6]. The prognosis of this neoplasm is poor as it is associated with a 5-year survival rate less than 25% [7].

The patient's manifestations will reflect the affected organ or system. For example, a tumor in the kidney or surrounding tissue may cause symptoms such as gross hematuria and flank pain while an adrenal gland malignancy can produce abdominal and flank pain [8]. Additionally, a tumor in the presacral space may result in foot pain and constipation [8]. Additionally, patients with a pulmonary PNET experience fever, cough, hemoptysis, dyspnea, and chest pain as well as symptoms based on involved neighboring structures [9]. Women with a cervical tumor will exhibit vaginal bleeding, vaginal discharge, lower abdominal pain and urinary features. These patients also have cervical lesions and an enlarged uterus [10].

As for individuals with CNS neuroectoderm tumors, the features will also be site-specific and may include headaches, emesis, visual changes, epistaxis, cranial neuropathies, lethargy, and other changes in mental status. The physical exam findings may include gait ataxia, papilledema, various palsies, and cerebellar features.

• Fever

  The patient was admitted with abdominal pain and fever and underwent emergency exploratory surgery with total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. [ncbi.nlm.nih.gov]

  Additionally, patients with a pulmonary PNET experience fever, cough, hemoptysis, dyspnea, and chest pain as well as symptoms based on involved neighboring structures. [symptoma.com]

  Systemic symptoms such as weight loss (14.5%) and fever (9.7%) may occur and must be differentiated from infectious etiologies [5]. [austinpublishinggroup.com]

  There was a brief episode of low-grade fever with constitutional symptoms two weeks prior to the current illness. Physical examination revealed jugular venous distention, prominent hepatomegaly, and ascites. [annalspc.com]

• Fatigue

  A 40-year-old male was admitted with complaints of nonproductive cough, exertional dyspnea, and fatigue since 4 months with a history of abdominal wall swelling which was excised and proven to be Ewing's sarcoma/primitive neuroectodermal tumor (PNET). [ncbi.nlm.nih.gov]

  Some common symptoms occur due to increased pressure within the brain, including: headache (generally upon awakening in the morning) vomiting fatigue /lethargy seizures Pineoblastomas (PNET in the pineal region, a tiny organ near the center of the brain [childrenshospital.org]

  Common symptoms include headache, nausea and vomiting, fatigue, lethargy, seizures, behavior or personality changes, unexplained weight loss or gain, difficulty looking upward and weakness on one side of the body. [danafarberbostonchildrens.org]

  Individuals with a medulloblastoma may present with: • Headaches which are often worse in the morning and get better during the day; • Nausea or vomiting in the morning; • Problems with motor skills, such as clumsiness or poor handwriting; • Tiredness/Fatigue [secure.ssa.gov]

• Difficulty Walking

  […] the optic disc at the back of the eye (papilloedema) In some cases, the tumour can spread to the spinal cord, causing another set of symptoms, such as back pain, an inability to control the bowels and bladder and difficulty walking. [thebraintumourcharity.org]

  walking If you are concerned about any changes your child experiences, please talk with your child’s doctor. [cancer.net]

  […] pain Difficulty walking Problems with bowel and bladder control Diagnosis The diagnosis of a Medulloblastoma/PNET is made by the Neurosurgeon taking a biopsy of the tumour. [rch.org.au]

  If the tumour spreads to the spinal cord, the signs and symptoms may include: back pain difficulty walking problems with bowel and bladder control. [royalmarsden.nhs.uk]

  Symptoms may include poor coordination, difficulty walking, and clumsiness (ataxia). Affected children may fall frequently and develop an unsteady, clumsy manner of walking (unsteady gait). [rarediseases.org]

• Dyspnea

  A 40-year-old male was admitted with complaints of nonproductive cough, exertional dyspnea, and fatigue since 4 months with a history of abdominal wall swelling which was excised and proven to be Ewing's sarcoma/primitive neuroectodermal tumor (PNET). [ncbi.nlm.nih.gov]

  Additionally, patients with a pulmonary PNET experience fever, cough, hemoptysis, dyspnea, and chest pain as well as symptoms based on involved neighboring structures. [symptoma.com]

  We describe the case of a 66-year-old no smoking woman admitted to our Hospital with dyspnea for tracheal compression, dysphagia, retrosternal pain and signs of initial extrinsic compression of superior vena cava (SVC). [thoracicmedicine.org]

  Department, Erzincan, Turkey 2 Suleyman Demirel University, Faculty of Medicine, Emergency Medicine Department, Isparta, Turkey A 63-year-old man presented to the emergency department with acute onset chest pain that began 3 h before arrival, without dyspnea [trjemergmed.com]

  When extra cranial metastases are present, hypercalcemia, thoracic or abdominal pain, dyspnea and bone nodule are the most prominent features. [scielo.br]

• Chest Pain

  We suggested that the mass caused the acute onset chest pain due to pushing the LIMA graft. It may cause blood flow deterioration from LIMA graft to cardiac vessel. [trjemergmed.com]

  The most common presentation of lung PNETs are cough, chest pain, dyspnoea, hemoptysis or even fever like other lung pathologies. [tlcr.amegroups.com]

  Additionally, patients with a pulmonary PNET experience fever, cough, hemoptysis, dyspnea, and chest pain as well as symptoms based on involved neighboring structures. [symptoma.com]

  Our case was a 16-year-old patient with symptoms consisting of chest pain and shortness of breath. [journals.lww.com]

  Case report On December 25, 2012, a 51-year-old female presented with intermittent chest pain that had been apparent for one year. The physical examination was unremarkable. [spandidos-publications.com]

• Hypertension

  Case Presentation A 46 year old woman with a history of hypertension, hyperlipidemia and nephrolithiasis presented to the emergency room with a 2–3 week history of intermittent bilateral lower quadrant abdominal pain and nausea. [austinpublishinggroup.com]

  During I-131 MIBG infusion, catecholamine discharge may cause hypertension and tachycardia in 20% of the patients [ 11 ]. [intechopen.com]

  The patient was a known case of hypertension. There was no other significant family or past history. Anterior rhinoscopy showed blood clots in middle meatus, with maxillary fullness. [jomfp.in]

• Short Arm

  […] lymphocyte, with scant cytoplasm and round nuclei are seen in ESFTs. [18] PNET is differentiated from other members by more neuronal differentiation. [9] Membrane staining for CD 99, transmembrane protein encoded by the MIC 2 pseudogene located in the short [faoj.org]

  Each chromosome has a short arm designated as “p” a long arm identified by the letter “q” and a narrowed region at which the two arms are joined (centromere). [rarediseases.org]

• Withdrawn

  FACS-sorted GFAP-GFP+ cells generated neurospheres when cultured at clonal density in the presence of growth factors, and individual clones were able to differentiate into neurons, astrocytes and oligodendrocytes when growth factors were withdrawn ( Supp [ncbi.nlm.nih.gov]

• Headache

  A 74-year-old woman presented with gradually progressive painless diminution of vision in both eyes alongwith headache for 6 months duration. Radiology demonstrated a clival mass as well as a suspected intracranial metastasis. [ncbi.nlm.nih.gov]

  Some common symptoms occur due to increased pressure within the brain, including: headache (generally upon awakening in the morning) vomiting fatigue /lethargy seizures Pineoblastomas (PNET in the pineal region, a tiny organ near the center of the brain [childrenshospital.org]

• Irritability

  Case presentation A tumor adjacent to the right kidney was detected by ultrasound coincidentally at a routine check-up in a 46-year-old woman with irritable bowel syndrome in her medical history. The patient had no clinical signs. [casesjournal.biomedcentral.com]

  A three month-old male child presented with a swelling arising from the oral cavity since 1½ months, difficulty in breast-feeding and irritability. [jiaps.com]

  Symptoms include: nausea and vomiting (most common) lethargy and irritability headaches clumsiness difficulty with tasks like handwriting gradual decline in school work changes in personality and behaviour abnormal gait (the way the child walks). [royalmarsden.nhs.uk]

  Some of the general symptoms of medulloblastoma include: Balance problems or clumsiness Changes in thinking ability Dizziness Double vision or other eye problems Tiredness and lack of energy (fatigue) Headaches Nausea and vomiting Hearing loss Irritability [cedars-sinai.org]

  Additional symptoms may include irritability, increased head size, and paralysis (paresis) of the muscles that help control eye movements (extraocular muscles). [rarediseases.org]

• Abnormal Gait

  Symptoms include: nausea and vomiting (most common) lethargy and irritability headaches clumsiness difficulty with tasks like handwriting gradual decline in school work changes in personality and behaviour abnormal gait (the way the child walks). [royalmarsden.nhs.uk]

  Deletion of ptc in GNPs results in medulloblastoma Although Math1-Cre/Ptc C/C mice were asymptomatic in early adulthood, by 8 weeks of age many animals began to display signs of illness, including domed head, hunched back, abnormal gait or decreased movement [ncbi.nlm.nih.gov]

• Somnolence

  Other common adverse effects being anemia, somnolence, peripheral neuropathy, headaches, constipation, paresthesias, mucositis, nausea and vomiting, immune compromise and bone marrow suppression, renal toxicities with electrolyte abnormalities, hepatotoxicity [intechopen.com]

Patients suspected to have a PNET warrant a full evaluation with history, a physical exam, and the appropriate studies.

Tissue analysis

A biopsy should be obtained whether through a needle or surgical excision. Subsequently, the specimen should be analyzed through diagnostic studies such as light microscopy, immunohistochemistry, and cytogenetic testing. Specifically, light microscopy reveals the characteristic appearance of small round cell tumors [11]. A PNET will stain positively for markers such as CD99, CD 56, NSE, and vimentin [11]. Furthermore, genetic analysis reveals a balanced translocation t(11;22)(q24;q12), which is a hallmark feature of this tumor [12].

Imaging

Computed tomography (CT) and magnetic resonance imaging (MRI) are among the techniques used to identify the location, extent, and degree of tumor involvement. Note that advanced disease is common at the time of presentation and hence the patient should be evaluated for potential metastasis. The clinician should perform a bone marrow biopsy, a chest CT scan, a chest radiograph, and possibly a positron emission tomography (PET) scan and technetium 99m bone study.

In CNS cases, the preferred imaging modality is the brain MRI although a brain CT scan takes precedence in emergencies. Note that a lumbar puncture is not performed if there is a mass lesion.

Other

To differentiate between a PNET and a neuroblastoma, urinary catecholamines and their metabolites should be obtained. These tests are negative in patients with PNET.

Traditional surgery, radiotherapy, and chemotherapy are used for the treatment of PNET, but are usually ineffective. There was a rare case of a 17 year-old man diagnoses with primary pulmonary PNET. [ncbi.nlm.nih.gov]

Leptomeningeal and/or subarachnoid seeding are common, necessitating imaging and treatment of the whole neuraxis. [radiopaedia.org]

Supratentorial PNET's generally have a poorer prognosis than medulloblastomas, with a low 5-year survival rate. [radiopaedia.org]

The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first case of a PNET in the ventricles with good prognosis. [ncbi.nlm.nih.gov]

< 10 years (EFS 69%); 10 - 17 years (EFS 74%) Unfavorable prognosis: ≥ 18 years (EFS 44%) Site Favorable prognosis: distal extremity (EFS 74%); proximal extremity (EFS 62%) Unfavorable prognosis: pelvis (EFS 50%) Size Favorable prognosis: < 8 cm greatest [pathologyoutlines.com]

Publication types, MeSH terms, Substances Publication types Case Reports Review MeSH terms 12E7 Antigen Abdominal Pain/etiology Adolescent Adrenal Gland Neoplasms/complications Adrenal Gland Neoplasms/diagnosis* Adrenal Gland Neoplasms/genetics Adrenal [ncbi.nlm.nih.gov]

Updated, reorganized, and revised throughout, this highly lauded three-volume reference provides an interdisciplinary approach to the diagnosis, treatment, and management of head and neck diseases, including the incidence, etiology, clinical presentation [books.google.ro]

3 months to 8 years) but also reported in adults Most common in maxilla of infants with 2:1 female preponderance (see Mandible - Maxilla Chapter ) Also reported in long bones ( Cancer 1983;52:661 ), uterus, skin and brain Epididymis is uncommon site Etiology [pathologyoutlines.com]

Systemic symptoms such as weight loss (14.5%) and fever (9.7%) may occur and must be differentiated from infectious etiologies [5]. [austinpublishinggroup.com]

Consequently, we explore the Surveillance, Epidemiology, and End Results (SEER) database to explore the epidemiology of DNT. [ncbi.nlm.nih.gov]

Leukaemia Research Fund, Centre for Clinical Epidemiology University of Leeds Leeds UK 2. Cancer Epidemiology Unit Imperial Cancer Research Fund Oxford UK [springerlink.com]

Pathophysiology Large, solid tumors: Cerebral supratentorial PNETs are often large at the time of initial diagnosis. On gross examination they are solid with areas of hemorrhage, calcification, necrosis, and cysts. [ispn.guide]

We reviewed the pathophysiology of the vasospasm that occurs after tumors resection, and due to the rarity of case, we speculated on the possibility that specific histological and molecular features of the tumor could have contributed to the delayed and [ncbi.nlm.nih.gov]

Further studies on molecular biology and the gene expression of the PF - PNET may shed light on the biological properties of the tumors that metastasize and the comprehension of the pathophysiology may guide new forms of treatment. REFERENCES 1. [scielo.br]

All of that changed after the 2016 World Health Organization (WHO) reclassification. [3] Pathophysiology In the brain, medulloblastoma most often arises in the posterior fossa as shown in the image below. [emedicine.medscape.com]

The high under staging rate may have prevented patients from selecting definitive radiotherapy after surgery. [ncbi.nlm.nih.gov]

His research interests include prevention of head and neck cancers, aetiology of oral cancer in young patients, and development of simple, low-cost treatment strategies for head and neck cancers suitable for countries with limited resources. [books.google.it]

Consistently, the treatment with Cxcl3 completely prevents the growth of medulloblastoma lesions in a Shh-type mouse model of medulloblastoma. Thus, CXCL3 is a target for medulloblastoma therapy. [en.wikipedia.org]

Knowing what these side effects are can help your care team prepare for and, in some cases, prevent these symptoms from occurring. [danafarberbostonchildrens.org]

Página 310 - A randomized trial comparing ticlopidine hydrochloride with aspirin for the prevention of stroke in high-risk patients. ‎ [books.google.es]

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