Presentation
Clinical presentation varies according to whether they are intra- or extra-axial. Intra-axial Intra-axial teratomas typically present either antenatally or in the newborn period. [radiopaedia.org]
We present a rare case of pineal germinoma treated with chemotherapy and radiotherapy. [cureus.com]
Clinical History An 18-year-old male presented to our hospital with headache, nausea and vomiting. Imaging Findings An 18-year-old male adolescent presented to our hospital with headache, nausea and vomiting. [eurorad.org]
Entire Body System
- Malnutrition
He was slightly pale and fatigued due to malnutrition, but his vital signs were normal. [jmedicalcasereports.biomedcentral.com]
Gastrointestinal
- Pelvic Mass
J Urol. 1998, 159:859-63. 10.1016/S0022-5347(01)63754-6 Nael A, Siaghani P, Wu WW, Nael K, Shane L, Romansky SG: Metastatic malignant ectomesenchymoma initially presenting as a pelvic mass: report of a case and review of literature. [cureus.com]
Skin
- Sweating
In addition, one tumor from a series of three childhood medullomyoblastomas contained a "teratoma", with mature tissue from all three germ layers including cartilage, hair follicles and sweat glands [5], and another cerebellar tumor from a boy aged 5 [path.upmc.edu]
Findings Histologically, teratomas are composed of tissues derived from the three germinal layers. [6], [21] The predominant germ cell types observed in orbital teratoma are surface ectoderm producing squamous epithelium-lined cyst, hair follicles, and sweat [eyewiki.aao.org]
Histopathology revealed that the resected mass had a composition based on mature adipose tissue, chondroid cartilage nests with some atypical cells (Figure 2B), and normal glandular tissue of intestinal and sweat glands (Figure 2C). [cureus.com]
Sebaceous and sweat glands were seen in a few cases. Cartilage with or without calcification, mature adipose tissue, fibrous tissue, mature smooth muscle, osteoid and glial tissue were seen in variable proportion [Figure 2]. [neurologyindia.com]
Eyes
- Vertical Gaze Palsy
Most patients present with hydrocephalus and/or Parinaud's syndrome (syndrome of vertical gaze palsy that can be associated with pupillary or oculomotor nerve paresis). [eurorad.org]
Psychiatrical
- Suggestibility
The hypothesis suggests that all germ cell tumors originate from primordial germ cells that migrated from the midline during development. [cureus.com]
Hyperintensity within the lesion, a finding suggestive of high cellularity. (No image.) Heterogeneous enhancement within the mass. Pineocytoma Hyperdense. Calcification may engulf the pineal. Demonstrates moderate homogeneous enhancement. [radcharts.org]
(WC) Fetus in fetu Grouped with mature teratoma, as it is considered a well-developed mature teratoma. [7] [8] It has been suggested they are distinct from teratomas. [9] They could be thought of as a parasitic twin. [librepathology.org]
The case suggests that PNETs have a more diverse repertoire of differentiation than previously recognized. REFERENCES: Anwer, U.E., T.W. Smith, U. DeGirolami, and H.A. Wilkinson, Medulloblastoma with cartilaginous differentiation. [path.upmc.edu]
Markedly elevated serum HCG/β-HCG levels are strongly suggestive of choriocarcinoma or mixed GCTs with choriocarcinoma elements. [ajnr.org]
Urogenital
- Sexual Dysfunction
Other possible symptoms of a tumor in the pineal region are visual disorders, endocrine abnormalities, sexual dysfunction, growth failure, puberty delay, ataxia, seizures, and behavioral changes. [jmedicalcasereports.biomedcentral.com]
- Urinary Retention
Case presentation A 35-year-old malnourished Asian white man was referred to our hospital with a main complaint of acute paralysis, accompanied by diplopia, tingling and numbness in the lower limbs, and urinary retention for the last 5 days. [jmedicalcasereports.biomedcentral.com]
Neurologic
- Headache
Clinical History An 18-year-old male presented to our hospital with headache, nausea and vomiting. Imaging Findings An 18-year-old male adolescent presented to our hospital with headache, nausea and vomiting. [eurorad.org]
These recurrent headaches typically begin in childhood or adolescence and may last from a few hours to a few days. People with familial hemiplegic migraine experience an aura that comes before the headache. [findzebra.com]
Case of the Week: April 9-April 16, 2008 14 year old with headache (Click for a larger image.) Case Details Heterogeneous mass in the pineal region with calcification and fat. Fat fluid levels in the lateral ventricle secondary to rupture. [pedsradiology.com]
Case report: This case report is that of a 9-year-old male who presented with frontal headache of eight days, with associated photophobia, nausea and vomiting, and diplopia. [pubmed.ncbi.nlm.nih.gov]
CASE REPORT: This case report is that of a 9-year-old male who presented with frontal headache of eight days, with associated photophobia, nausea and vomiting, and diplopia. [ncbi.nlm.nih.gov]
- Ataxia
One had ataxia, right leg weakness and dysarthria. Another developed left hemiparesis during a 2-week period and then recovered partially. [findzebra.com]
Other possible symptoms of a tumor in the pineal region are visual disorders, endocrine abnormalities, sexual dysfunction, growth failure, puberty delay, ataxia, seizures, and behavioral changes. [jmedicalcasereports.biomedcentral.com]
Cerebellar peduncle: Cerebellar efferent fibres: Ataxia and dysmetria. Inferior colliculus: rare: hearing dysfunction Rarity: Seeding: Seizure, Radiculomyelopathy, thalamic pain, Extrapyramidal (ganglia): rare synchronous lesion. [slideshare.net]
[…] and ataxia, are present preoperatively and become transiently worse postoperatively before significantly improving or resolving completely. [emedicine.medscape.com]
Teratoma in a 56-year-old man with worsening confusion and ataxia. [ncbi.nlm.nih.gov]
- Nystagmus
Parinaud’s syndrome is present in half of such patients, causing paralysis of upward gaze, while paralysis of downward gaze is less frequent, similarly to convergence disorder and/or convergence–retraction nystagmus [1, 3]. [jmedicalcasereports.biomedcentral.com]
Both showed upward gaze palsy and horizontal nystagmus with lateral gaze. One showed abnormal intracranial calcification on plain skull film. One presented at age 13 years with a 7-month history of polydipsia, polyuria and loss of appetite. [findzebra.com]
Convergence or retraction nystagmus (60-70%): Co-contraction of all the horizontal extraocular muscles occurs, and the eyes are pulled into the orbit. Light near pupillary dissociation (Argyll Robertson's pupil) Midbrain: Sylvian aqueduct syndrome [slideshare.net]
- Neurologic Manifestation
manifestations secondary to anomalies of the odontoid process. [findzebra.com]
- Paresthesia
There were paresthesias in 4 of the 8 patients; and focal neurological deficits, in 2 of the 8 patients. ( [Table 1] - Cases 1-8 are spinal and cases 9-14 are intracranial). Notable male predominance was seen in tumors of the pineal region. [neurologyindia.com]
Treatment
SRS is increasingly being used to treat pineal region tumors, either as an additional therapy after conventional treatments or as a primary treatment. [wikidoc.org]
Treatment of germ cell tumours Treatment for intracranial germ cell tumours depends on the type of tumour and its location and spread. [royalmarsden.nhs.uk]
[…] or treatment according to protocol is not intended Participation within a different trial for treatment of germ cell tumours and/or concurrent treatment within any other clinical trial. [kinderkrebsinfo.de]
No adjuvant treatment was given. A complete neurological recovery was observed after the surgical procedure. Follow-up at 2 years did not show any recurrence. [karger.com]
OBJECTIVE: The treatment of intracranial mixed germ cell tumors presents a unique challenge, since eradication of malignant tumor by radiation and/or chemotherapy may spare the benign tumor component. [ncbi.nlm.nih.gov]
Prognosis
Location and size determine prognosis: intra-axial (fetal) lesions: stillbirth occurs relatively frequently, and difficulty with vaginal delivery (if attempted) can occur due to enlarged cranial circumference 3 extra-axial lesions: largely depends on [radiopaedia.org]
Estimation of Prognosis: i. Good prognosis: Germinoma, Mature teratoma, Pinealocytoma ii. [slideshare.net]
The prognosis of immature teratoma is less favorable than that of mature teratoma, with a 5-year survival rate of around 70% ( 9 ). [pubs.rsna.org]
Etiology
Etiology Childs Nerv Syst 2021 Feb;37(2):671-676. Epub 2020 Jun 25 doi: 10.1007/s00381-020-04742-z. PMID: 32588176 Liu W, Raynald, Tian Y, Gong J, Ma Z, Ma'ruf L, Li C Acta Neurol Belg 2021 Dec;121(6):1535-1542. [ncbi.nlm.nih.gov]
Epidemiology
They can be divided into two broad categories, intra- and extra-axial, which differ in epidemiology and clinical presentation. Another method of classifying an intracranial teratoma is as mature, immature and mature with malignant transformation. [radiopaedia.org]
[…] influences, or from primordial germ cells that aim toward the pineal gland, even though other theories have also been proposed. [6] Orbital teratomas reported so far have been unilateral, and tumors cited as bilateral seem to have been limbal dermoids. [7] Epidemiology [eyewiki.aao.org]
Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990-1994. Neuro Oncol. 1999;1(1):14–25. [PMC free article] [PubMed] [Google Scholar] 8. [ncbi.nlm.nih.gov]
Pathophysiology
Fat Cystic spaces due to mucus production or other exocrine products Soft-tissue from any part of the body Calcification, including teeth Pathophysiology On microscopic histopathological analysis, pineal teratoma is characterized by cells originating [wikidoc.org]
Pathophysiology Normal pineal gland: The pineal gland is composed of pineocytes (95%) and supporting glial cells (5%) ( 22, 34 ). Pineocytes are the principal parenchymal neurons of the pineal gland and function as neuroendocrine transducers. [ispn.guide]
References Mavridis IN, Pyrgelis ES, Agapiou E, Meliou M: Pineal region tumors: pathophysiological mechanisms of presenting symptoms. Am J Transl Res. 2021, 13:5758-66. Favero G, Bonomini F, Rezzani R: Pineal gland tumors: a review. [cureus.com]
In this condition, the teratomas may contain B cells with NMDA-receptor specificities. [25] After surgery, a risk exists of regrowth in place, or in nearby organs. [26] Pathophysiology [ edit ] Teratomas belong to a class of tumors known as nonseminomatous [en.wikipedia.org]
Prevention
We believe that more vigilant observation and examination should be provided to patients with pineal teratoma to detect any new lesions and prevent them from becoming dangerous. Availability of data and materials Not applicable. [jmedicalcasereports.biomedcentral.com]
Prevention of recurrence does not require en bloc resection of surrounding tissues. Chemotherapy [ edit ] For malignant teratomas, usually, surgery is followed by chemotherapy. [en.wikipedia.org]
Also, the evidence for melatonin deficiency is recognized as a predictive factor to prevent post- pinealectomy syndrome. The absence of production of melatonin, as after pinealectomy, causes a “Jet-lag- like ” syndrome. [slideshare.net]