Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
Pediatric ependymomas are similar in nature to the adult form of ependymoma in that they are thought to arise from radial glial cells lining the ventricular system. However, they differ from adult ependymomas in which genes and chromosomes are most often affected, the region of the brain they are most frequently found in, and the prognosis of the patients. Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), have been found to be more frequently afflicted with this class of tumors, but a firm genetic link remains to be established. Symptoms associated with the development of pediatric ependymomas are varied, much like symptoms for a number of other pediatric brain tumors including vomiting, headache, irritability, lethargy, and changes in gait. Although younger children and children with invasive tumor types generally experience less favorable outcomes, total removal of the tumors is the most conspicuous prognostic factor for both survival and relapse.
Source:
- severe headache
- visual loss (due to papilledema)
- vomiting
- bilateral Babinski sign
- drowsiness (after several hours of the above symptoms)
- gait change (rotation of feet when walking)
- impaction/constipation
- back flexibility
The desire to eat normally becomes worse over time, leading to weight loss from vomiting. Nausea is seen in almost all cases of astroblastoma, especially in low-grade tumors.
Along with cranial pressure, patients exhibit noticeable lethargy, increasing in severity as the tumor progresses. In the first few months, morning activities are usually unaffected; over time, these effects become more pronounced, especially late at night. Lethargy can disrupt vital signs, depleting energy and desire to perform simple cognitive tasks.
There is a wide range of symptoms that patients show. Symptoms can lie dormant, but come about due to Obstructive hydrocephalus. These symptoms include:
- Intracranial pressure
- Headache
- Papilledema
- Vomiting
- Light headedness
- Impaired mental activity
- Gait instability
In rare and extreme cases, more severe symptoms can be observed:
- Memory disturbance
- Dementia
- Hemiparesis
- Seizures
- Hemorrhage
- Psychosis
Ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymoma is the fourth ventricle. Rarely, ependymoma can occur in the pelvic cavity.
Syringomyelia can be caused by an ependymoma.
Ependymomas are also seen with neurofibromatosis type II.
Symptoms present 1–36 months before diagnosis, and can vary depending on age, tumor grade, and location. Increased intracranial pressure can induce vomiting, headache, irritability, lethargy, changes in gait, and in children less than 2, feeding problems, involuntary eye movements, and hydrocephalus are often noticeable. Seizures occur in about 20% of pediatric patients. Loss of cognitive function and even sudden death could occur if the tumor is located at a crucial location for CSF flow. Pediatric ependymomas most often occur in the posterior cranial fossa, in contrast with adult ependymomas which usually occur along the spine. Ependymomas present as low-density masses on CT scans, and are hyperintense on T2-weighted MRI images.
Central neurocytoma, abbreviated CNC, is an extremely rare, ordinarily benign intraventricular brain tumour that typically forms from the neuronal cells of the septum pellucidum. The majority of central neurocytomas grow inwards into the ventricular system forming interventricular neurocytomas. This leads to two primary symptoms of CNCs, blurred vision and increased intracranial pressure. Treatment for a central neurocytoma typically involves surgical removal, with an approximate 1 in 5 chance of recurrence. Central neurocytomas are classified as a grade II tumor under the World Health Organization's classification of tumors of the nervous system.
Gangliogliomas are generally benign WHO grade I tumors; the presence of anaplastic changes in the glial component is considered to represent WHO grade III (anaplastic ganglioglioma). Criteria for WHO grade II have been suggested, but are not established. Malignant transformation of spinal ganglioglioma has been seen in only a select few cases. Poor prognostic factors for adults with gangliogliomas include older age at diagnosis, male sex, and malignant histologic features.
The most common symptom of the papillary tumor is a headache. Because headaches are so common, most people think nothing of it. This is why brain tumors are so dangerous. There are not a lot of symptoms that go along with them so people tend to wait a long time before seeking medical help. Most of the time people will go see a doctor when their headaches become consistent and start to never go away. This symptom however occurs secondary to hydrocephalus, which is a result from compression of the cerebral aqueduct. The cerebral aqueduct is a narrow channel in the midbrain, which connects the third and fourth ventricles. When a tumor blocks the pathway of the cerebrospinal fluid, this will cause headaches in the patient. Often when hydrocephalus occurs, a shunt is put in place in order to alleviate the pressure. In one case study, an endoscopic third ventriculostomy was performed as a first line procedure to treat the hydrocephalus and also for diagnostic purposes.
In some cases, patients have had progressive diplopia, or double vision. Also, although not in all cases, patients sometimes suffer from nausea and vomiting.
Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults.
Papillary tumors of the pineal region (PTPR) were first described by A. Jouvet et al. in 2003 and were introduced in the World Health Organization (WHO) classification of Central Nervous System (CNS) in 2007. Papillary Tumors of the Pineal Region are located on the pineal gland which is located in the center of the brain. The pineal gland is located on roof of the diencephalon. It is a cone shaped structure dorsal to the midbrain tectum. The tumor appears to be derived from the specialized ependymal cells of the subcommissural organ. Papillary tumors of the central nervous system and particularly of the pineal region are very rare and so diagnosing them is extremely difficult.
Diagnosis of mesoblastic nephroma and its particular type (i.e. classic, mixed, or cellular) is made by histological examination of tissues obtained at surgery. Besides its histological appearance, various features of this disease aid in making a differential diagnosis that distinguish it from the following childhood neoplasms:
- Wilm's tumor is the most common childhood kidney neoplasm, representing some 85% of cases. Unlike mesoblastic nephroma, 3 years of age. Bilateral kidney tumors, concurrent birth defects, and/or metastatic disease at presentation favor a diagnosis of Wilm's tumor.
- congenital infantile sarcoma is a rare aggressive sarcoma typically presenting in the lower extremities, head, or neck of infants during their first year of life. The histology, association with the "ETV6-NRTK3" fusion gene along with certain chromosome trisomies, and the distribution of markers for cell type (i.e. cyclin D1 and Beta-catenin) within this tumor are the same as those found in cellular mesoblastic nephroma. Mesoblastic nephroma and congenital infantile sarcoma appear to be the same diseases with mesoblastic lymphoma originating in the kidney and congenital infantile sarcoma originating in non-renal tissues.
- Rhabdoid tumor, which accounts for 5-510% of childhood kidney neoplasms, occurs predominantly in children from 1 to 2 years of age. Unlike mesoblastic nephroma, rhabdoid tumors may present with tumors in other tissues including in ~13% of cases, the brain. Rhabdoid tumors have a distinctive histology and abnormalities (i.e. loss of heterozygosity, single nucleotide polymorphism, and deletions) in chromosome 22.
- Clear cell sarcoma of the kidney, which is responsible for 5-10% of childhood pediatric tumors, occurs predominantly in children from 2 to 3 years of age. Unlike meoblastic nephorma, clear cell sarcoma of the kidney presents with metastasis, particularly to bone, in 5-6% of cases; it histology is diverse and has been mistaken for mesoblastic nephroma. One chromosomal translocations t,(10;17)(q22;p13), has been repeatedly reported to be associated with clear cell sarcoma of the kidney.
- Infantile myofibromatosis is a fibrous tumor of infancy and childhood most commonly presenting during the first 2 years of life as a single subcutaneous nodule of the head and neck region or less commonly as multiple lesions of skin, muscle, bone, and in ~33% of these latter cases, visceral organs. All of these lesions have an excellent prognosis and can regress spontaneously except for those in which there is visceral involvement where the prognosis is poor. While infantile myofibromatosis and classic mesoblastic nephroma have been suggested to be the same diseases because of their very similar histology, studies on the distribution of cell-type markers (i.e. cyclin D1 and Beta-catenin) indicate that they have different cellular origins.
A benign tumor is a mass of cells (tumor) that lacks the ability to invade neighboring tissue or metastasize. Benign tumors do not spread into, or invade, nearby tissues. Benign tumors can sometimes be quite large, however. When removed, they usually do not grow back, whereas malignant tumors sometimes do. Unlike most benign tumors elsewhere in the body, benign brain tumors can be life threatening. Benign tumors generally have a slower growth rate than malignant tumors and the tumor cells are usually more differentiated (cells have normal features). Benign tumors are typically surrounded by an outer surface (fibrous sheath of connective tissue) or remain with the epithelium. Common examples of benign tumors include moles and uterine fibroids.
Although benign tumors will not metastasize or locally invade tissues, some types may still produce negative health effects. The growth of benign tumors produces a "mass effect" that can compress tissues and may cause nerve damage, reduction of blood to an area of the body (ischaemia), tissue death (necrosis) and organ damage. The mass effect of tumors is more prominent if the tumor is within an enclosed space such as the cranium, respiratory tract, sinus or inside bones. Tumors of endocrine tissues may overproduce certain hormones, especially when the cells are well differentiated. Examples include thyroid adenomas and adrenocortical adenomas.
Although most benign tumors are not life-threatening, many types of benign tumors have the potential to become cancerous (malignant) through a process known as tumour progression. For this reason and other possible negative health effects, some benign tumors are removed by surgery.
Congenital mesoblastic nephroma typically (76% of cases) presents as an abdominal mass which is detected prenatally (16% of cases) by ultrasound or by clinical inspection (84% of cases) either at birth or by 3.8 years of age (median age ~1 month). The neoplasm shows a slight male preference. Concurrent findings include hypertension (19% of cases), polyhydramnios (i.e. excess of amniotic fluid in the amniotic sac) (15%), hematuria (11%), hypercalcemia (4%), and elevated serum levels of the kidney-secreted, hypertension-inducing enzyme, renin (1%). Congenital anomalies have been reported in 11 patients: 6 with genitourinary anomalies, 2 with gastrointestinal anomalies, 1 with hydrocephalus, and 1 with the Beckwith–Wiedemann syndrome. The vast majority of patients present with localized (i.e. non-metastatic) disease. Most patients' disease is classified at presentation as stage I or II (i.e. localized), few patients present with stage III (i.e. locally advanced/infiltrating), and virtually no patients present with stage IV (metastases present or V (i.e. tumors in both kidneys) disease (see staging of renal cancer).
Benign neoplasms are typically but not always composed of cells which bear a strong resemblance to a normal cell type in their organ of origin. These tumors are named for the cell or tissue type from which they originate, followed by the suffix "-oma" (but not -carcinoma, -sarcoma, or -blastoma, which are generally cancers). For example, a lipoma is a common benign tumor of fat cells (lipocytes), and a chondroma is a benign tumor of cartilage-forming cells (chondrocytes). Adenomas are benign tumors of gland-forming cells, and are usually specified further by their cell or organ of origin, as in hepatic adenoma (a benign tumor of hepatocytes, or liver cells). Teratomas contain many cell types such as skin, nerve, brain and thyroid, among others, because they are derived from germ cells. Hamartomas are a group of benign tumors that have relatively normal cellular differentiation but the architecture of the tissue is disorganised. There are a few cancers with 'benign-sounding' names which have been retained for historical reasons, including melanoma (a cancer of pigmented skin cells, or melanocytes) and seminoma (a cancer of male reproductive cells). Skin tags, vocal chord polyps and hyperplastic polyps of the colon are often referred to as benign but they are actually overgrowths of normal tissue rather than neoplasms.
An ependymal tumor is a type of brain tumor that begins in cells lining the spinal cord central canal (fluid-filled space down the center) or the ventricles (fluid-filled spaces of the brain). Ependymal tumors may also form in the choroid plexus (tissue in the ventricles that makes cerebrospinal fluid). Also called ependymoma.
Mucinous cystadenocarcinoma is a type of tumor in the cystadenocarcinoma grouping.
It can occur in the breast as well as in the ovary. Tumors are normally multilocular with various smooth, thin walled cysts. Within the cysts is found a haemorrhagic or cellular debris.
Atypical hyperplasia is a high-risk premalignant lesion of the breast. It is believed that atypical ductal hyperplasia (ADH) is a direct precursor for low-grade mammary ductal carcinoma, whereas atypical lobular hyperplasia (ALH) serves as a risk indicator.
SCT is seen in 1 in every 35,000 live births, and is the most common tumor presenting in newborn humans. Most SCTs are found in babies and children, but SCTs have been reported in adults and the increasingly routine use of prenatal ultrasound exams has dramatically increased the number of diagnosed SCTs presenting in fetuses. Like other teratomas, an SCT can grow very large. Unlike other teratomas, an SCT sometimes grows larger than the rest of the fetus.
Sacrococcygeal teratomas are the most common type of germ cell tumors (both benign and malignant) diagnosed in neonates, infants, and children younger than 4 years. SCTs occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported.
Historically, sacrococcygeal teratomas present in 2 clinical patterns related to the child’s age, tumor location, and likelihood of tumor malignancy. With the advent of routine prenatal ultrasound examinations, a third clinical pattern is emerging.
- Fetal tumors present during prenatal ultrasound exams, with or without maternal symptoms. SCTs found during routine exams tend to be small and partly or entirely external. The internal SCTs are not easily seen via ultrasound, unless they are large enough to reveal their presence by the abnormal position of the fetal urinary bladder and other organs, but large fetal SCTs frequently produce maternal complications which necessitate non-routine, investigative ultrasounds.
- Neonatal tumors present at birth protruding from the sacral site and are usually mature or immature teratomas.
- Among infants and young children, the tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum. These pelvic tumors have a greater likelihood of being malignant. An early survey found that the rate of tumor malignancy was 48% for girls and 67% for boys older than 2 months at the time of sacrococcygeal tumor diagnosis, compared with a malignant tumor incidence of 7% for girls and 10% for boys younger than 2 months at the time of diagnosis. The pelvic site of the primary tumor has been reported to be an adverse prognostic factor, most likely caused by a higher rate of incomplete resection.
- In older children and adults, the tumor may be mistaken for a pilonidal sinus, or it may be found during a rectal exam or other evaluation.
Mammary analogue secretory carcinoma (MASC) (also termed MASC; the "SG" subscript indicates salivary gland)) is a salivary gland neoplasm that shares a genetic mutation with certain types of breast cancer. MASC was first described by Skálová et al. in 2010. The authors of this report found a chromosome translocation in certain salivary gland tumors that was identical to the (12;15)(p13;q25) fusion gene mutation found previously in secretory carcinoma, a subtype of invasive ductal carcinoma of the breast.
Atypical hyperplasia is a benign (noncancerous) cellular hyperplasia in which cells show some atypia. In this condition, cells look abnormal under a microscope and are increased in number.
During prenatal ultrasound, an SCT having an external component may appear as a fluid-filled cyst or a solid mass sticking out from the fetus' body. Fetal SCTs that are entirely internal may be undetected if they are small; detection (or at least suspicion) is possible when the fetal bladder is seen in an abnormal position, due to the SCT pushing other organs out of place.
At birth, the usual presentation is a visible lump or mass under the skin at the top of the buttocks crease. If not visible, it can sometimes be felt; gently prodded, it feels somewhat like a hardboiled egg. A small SCT, if it is entirely inside the body, may not present for years, until it grows large enough to cause pain, constipation and other symptoms of a large mass inside the pelvis, or until it begins to extend out of the pelvis. Even a relatively large SCT may be missed, if it is internal, because the bony pelvis conceals and protects it. Mediastinal tumors, including teratomas, are similarly concealed and protected by the rib cage.
Some SCTs are discovered when a child begins to talk at about age 2 years and complains of their bottom hurting or feeling "poopy" when they ride in a car seat.
Other tumors can occur in the sacrococcygeal and/or presacral regions and hence must be ruled out to obtain a differential diagnosis. These include extraspinal ependymoma, ependymoblastoma, neuroblastoma and rhabdomyosarcoma.
Smaller SCTs with an external component, seen in prenatal ultrasounds or at birth, often are mistaken for spina bifida. Cystic SCT and terminal myelocystocele are especially difficult to distinguish; for more accurate diagnosis, MRI has been recommended.
In urologic pathology, PUNLMP, short for papillary urothelial neoplasm of low malignant potential, is an exophytic (outward growing), (microscopically) nipple-shaped (or papillary) pre-malignant growth of the lining of the upper genitourinary tract (the urothelium), which includes the renal pelvis, ureters, urinary bladder and part of the urethra.
"PUNLMP" is pronounced "pun"-"lump", like the words "pun" and "lump".
As their name suggests, PUNLMPs are neoplasms, i.e. clonal cellular proliferations, that are thought to have a low probability of developing into urothelial cancer, i.e. a malignancy such as bladder cancer.
PUNLMPs can lead to blood in the urine (hematuria) or may be asymptomatic.