The clinical case makes a textbook description of a medial temporal seizure. The aura of ascending epigastric sensation and olfactory alteration and the subsequent description of the seizure with alteration of the level of consciousness (disconnection from the environment) with oral and manual automatisms. The current classification is the 2017 ILAE classification that would describe this seizure as focal seizure with altered level of consciousness, but in the MIR they still use the old terminology (complex is what altered level of consciousness was called).

A 29-year-old woman with a history of febrile seizures and a heavy smoker. She consults for frequent, stereotyped episodes that begin with an ascending epigastric sensation and an unpleasant odor, followed by disconnection from the environment. Family members who are with her observe that she makes sucking movements and repeated opening and closing movements of the left hand. After two minutes the movements subside, but she has difficulty responding appropriately and only partially remembers what happened. Which of the following is the most likely diagnosis?

Transient ischemic attacks in right carotid territory.

[A case of painful seizure accompanying ictal paresis and homonymous hemianopia due to post-stroke epilepsy].

A 69-year-old female with an old infarct of the left parietotemporal lobe was admitted for the evaluation of suspected painful seizures accompanying ictal paresis. The painful seizure and ictal paresis involved her right extremities without convulsions, although intermittent tremulous movements were noted on the right upper extremity. She also showed right hemianopia during the seizure. Ictal scalp EEG demonstrated lateralized rhythmic sharply contoured delta activity intermingled with a large amount of spikes, sharp waves, and fast activity mainly on the posterior half of the left hemisphere. Ictal MRI showed restricted diffusion in the postcentral gyrus and dilatation of distal branches of the left middle cerebral artery (MCA). <sup99m</supTc-ECD SPECT revealed hyperperfusion on the left parietal cortex. Treatment with antiepileptic drugs successfully prevented seizure recurrence, then she was discharged home. On the follow-up SPECT after 1 month, the abnormal hyperperfusion disappeared. MRI demonstrated resolution of the restricted diffusion and the MCA dilatation. Taken together with the EEG abnormality and the transient abnormalities in SPECT and MRI, we concluded that her seizures were epileptic and that her painful seizures likely arise from the left primary somatosensory cortex. The mechanism of ictal paresis would be attributed to some disturbed functional architecture in the left primary motor cortex leading to loss of normal motor function through epileptic interference by ictal discharges. The same mechanism in the visual cortex could be assumed for her ictal hemianopia. Painful seizure and ictal paresis each is rarely encountered, even more so the combination thereof. These ictal manifestations might be difficult to differentiate from transient ischemic attack or postictal paresis, and thus EEG is essential to diagnose this treatable condition.

Focal Impaired Awareness Seizure -- History and Physical

As with other types of seizures, diagnosis of focal impaired awareness seizure is based on clinical history. Epilepsy is a clinical diagnosis. As with any other seizure, it is most important to make sure that the episode is truly a seizure. Obtaining a detailed history from the patient and family members is important. A detailed description of the spell, for example, the sequence of events, nature of onset, loss of consciousness, any motor or convulsive activity, bilateral involvement, tongue bite, incontinence, stare, automatisms, eye movements, postictal confusion, recovery, and duration of events are very important to elicit. It is important to elicit a detailed medical history to identify possible risk factors. The clinical semiology depends on the location of the seizure focus. A detailed neurological examination is important. Even subtle findings on the neurological exam can support the diagnosis of seizures. Also, certain lateralizing abnormalities on the neurological exam can help predict the epileptic focus. Some specific features associated with focal seizures can help in lateralizing the seizure origin to one hemisphere. These features provide a good clue for lateralization but can be falsely lateralizing. Dystonic limb posturing: Unilateral dystonic posturing is the most reliable lateralizing sign in temporal lobe automotor seizures. Unilateral hand/arm dystonic posturing in conjunction with automatisms of the opposite limb and head-turning is highly suggestive of temporal lobe onset contralateral to the dystonic upper extremity. Head version: The classical head version (defined as a tonic, unnatural, forced lateral gyratory head movement) is highly suggestive of seizure onset to the contralateral side. It can occur in both temporal and extratemporal seizures. Seizures spread to the premotor areas may be the cause. Automatisms: Oroalimentary automatisms like lip-smacking, chewing, or swallowing may occur without loss of consciousness at seizure onset in temporal lobe seizures with seizures confined to the amygdala and anterior hippocampus. Most automatisms are usually accompanied by impaired consciousness and have no lateralizing value, but preserved responsiveness during automatisms lateralize to the non-dominant hemisphere. Eye blinking or fluttering at seizure onset may indicate occipital lobe onset. Aversion of the eyes to the opposite side suggests seizures arising from the occipital region. Focal seizures with impaired consciousness arising from mesial frontal and orbitofrontal regions can cause bicycling or pedaling movements of the leg. Mimetic automatisms and crying have been reported in focal seizures with impaired consciousness arising from the non-dominant temporal lobe. Vomiting during seizure lateralizes to the non-dominant hemisphere. Sexual or genital automatisms (like pelvic thrusting and masturbation) can occur in focal seizures with impaired consciousness of frontal and temporal lobe origin. Postictal nose-wiping: Postictal nose-wiping that occurs within 60 seconds of the seizure occurs in about 50% to 85% of temporal lobe epilepsy patients and is performed with the ipsilateral hand in a majority of the cases. [1] [6]

Mesial Temporal Lobe Epilepsy -- History and Physical -- Seizure Semiology

When there is preserved awareness, the patient can describe the occurrence of unusual sensations known as auras. Auras could be visceral, autonomic, gustatory, or affective symptoms. Patients commonly experience a rising epigastric sensation, nausea, and olfactory and/or gustatory hallucination. Psychic sensations can occur, such as depersonalization (out-of-body feeling), déjà vu (a feeling of familiarity), jamais vu (feeling of unfamiliarity), déjà entendu (hearing familiar sounds), or panoramic visions (a rapid recollection of episodes from the past). Dysphoric or euphoric feelings, fear, terror, anger, and other sensations can also occur. Often, the patients find the aura hard to describe. Autonomic features include piloerection, pallor/flushing, tachycardia, or pupillary dilatation. [24] With the loss of awareness, patients have a behavioral arrest and portray a blank staring facial appearance, which is followed by the development of oro/facial/alimentary automatisms such as lip-smacking, chewing, sucking, or swallowing, which is usually accompanied by ipsilateral gestural automatisms such as repetitive hand movements, picking and/or fidgeting behavior, disrobing and contralateral dystonic posturing of limbs. The presence of speech suggests non-dominant hemispheric seizure onset, but its absence is not a reliable lateralizing finding. Patients commonly have a period of postictal confusion following the episode. Less commonly, episodes may progress to generalized tonic-clonic seizures.

Temporal lobe epilepsy

These seizures tend to have a warning or aura before they occur, and when they occur they generally tend to last only 1–2 minutes. It is not uncommon for an individual to be tired or confused for up to 15 minutes after a seizure has occurred, although postictal confusion can last for hours or even days. Though they may not seem harmful, due to the fact that the individual does not normally seize, they can be extremely harmful if the individual is left alone around dangerous objects. For example, if a person with complex partial seizures is driving alone, this can cause them to run into the ditch, or worse, cause an accident involving multiple people. With this type, some people do not even realize they are having a seizure and most of the time their memory from right before or after the seizure is wiped. First-aid is only required if there has been an injury or if this is the first time a person has had a seizure.

Complex partial seizures.

A 24 year old woman with a past history of febrile convulsions presents with a history of several episodes of 'funny turns' over a period of six months. She states she 'feels weird' before each episode. Her partner describes that she becomes verbally unresponsive, licks her lips, and makes other strange movements with her mouth. They usually last several minutes, after which she appears quite drowsy for half an hour. She recalls nothing of the event itself. Examination findings are normal. You take blood tests and arrange for an electroencephalogram.

InternalMed_Harrison

Focal Seizures with Dyscognitive Features Focal seizures may also be accompanied by a transient impairment of the patient’s ability to maintain normal contact with the environment. The patient is unable to respond appropriately to visual or verbal commands during the seizure and has impaired recollection or awareness of the ictal phase. The seizures frequently begin with an aura (i.e., a focal seizure without cognitive disturbance) that is stereotypic for the patient. The start of the ictal phase is often a sudden behavioral arrest or motionless stare, which marks the onset of the period of impaired awareness. The behavioral arrest is usually accompanied by automatisms, which are involuntary, automatic behaviors that have a wide range of manifestations. Automatisms may consist of very basic behaviors such as chewing, lip smacking, swallowing, or “picking” movements of the hands, or more elaborate behaviors such as a display of emotion or running. The patient is typically confused

Neurology_Adams

In the category of genuine seizures, the diagnosis of temporal lobe epilepsy may be difficult to distinguish from imitators of epilepsy. These attacks are so variable and so often induce disturbances of behavior and psychic function—rather than overt interruptions or loss of consciousness—that they may be mistaken for temper tantrums in children, drug ingestion, hysteria, panic attacks, or acute psychosis. These seizures may include verbalizations that cannot be remembered, walking aimlessly, repetitive olfactory and gustatory hallucinations, stereotyped hand movements or automatism such as lip smacking. The nature of the patient’s report of a psychic experience is often helpful in distinguishing seizures from psychogenic events. In the former, patients attempt to focus with great effort on the description of the experience, although the term “indescribable” is often included in the report, whereas vague and imprecise descriptions of “something being wrong” or resorting to a friend or

Neurology_Adams

The motor components of a focal temporal lobe or limbic seizure, if they occur, arise during the later phase of the seizure and take the form of automatisms such as lip-smacking, chewing or swallowing movements, salivation, fumbling of the hands, or shuffling of the feet. Patients may walk around in a daze or act inappropriately. Complex acts that were initiated before the loss of consciousness—such as walking, chewing food, turning the pages of a book, or even driving—may continue. However, when asked a specific question or given a command, the patients are obviously out of contact with their surroundings. There may be no response at all, or the patient may look toward the examiner in a perplexed way or utter a few stereotyped phrases. The patient may walk repetitively in small circles (volvular epilepsy), run (epilepsia procursiva), or simply wander aimlessly, either as an ictal or postictal phenomenon (poriomania). These forms of seizure, according to some epileptologists, are

Focal seizure

Complex partial seizures A complex partial seizure is a seizure that is associated with unilateral cerebral hemisphere involvement and causes impairment of awareness or responsiveness, i.e. alteration of consciousness. Presentation Complex partial seizures are often preceded by an aura. The seizure aura is a focal aware seizure. The aura may manifest itself as a feeling of déjà vu, jamais vu, fear, euphoria or depersonalization. The aura might also occur as a visual disturbance, such as tunnel vision or a change in the perceived size of objects. Once consciousness is impaired, the person may display automatisms, such as lip smacking, chewing or swallowing. There may also be loss of memory (amnesia) surrounding the seizural event. The person may still be able to perform routine tasks such as walking, although such movements are not purposeful or planned. Witnesses may not recognize that anything is wrong. The person may or may not even realize that they experienced a seizure.

Focal Impaired Awareness Seizure -- Introduction -- Seizures of Parietal Lobe Region

Seizures from the parietal lobe may be difficult to diagnose because of their subjective nature. Positive and /or negative sensory features are common. Sensorimotor phenomenon and vestibular hallucinations suggest onset in the parietal lobe. Paresthesias, visual hallucinations, visual illusions, somatic illusions, and vertiginous features can occur. Seizures arising from the dominant hemisphere can cause receptive language impairment. Parietal lobe Focal impaired awareness seizures can have auras like epigastric sensations, visual hallucinations, panic attacks, and behavioral arrest. Often, there is an involvement of other lobes as the seizure spreads. When focal seizures from the parietal lobe spread and involve the temporal lobe, loss of consciousness and automatisms may occur.

Transient epileptic amnesia

Symptoms A person experiencing a TEA episode has very little short-term memory, so that there is profound difficulty remembering events in the past few minutes (anterograde amnesia), or of events in the hours before the onset of the attack, and even memories of important events in recent years may not be accessible during the amnestic event (retrograde amnesia). Some people report short-lived retrograde amnesia so deep that they do not recognize their home or family members, though personal identity is preserved. The amnestic attack has a sudden onset. Three-fourths of cases are reported upon awakening. In attacks that begin when an individual is fully alert, olfactory hallucinations or a "strange taste" or nausea have been reported. Somewhat less than half the cases include olfactory or gustatory hallucinations, and slightly more than a third involve motor automatisms. A quarter of attacks involve a brief period of unresponsiveness. Frequently, however, there is no warning.

Pharmacology_Katzung

Roger J. Porter, MD, & Michael A. Rogawski, MD, PhD was unchanged, and levetiracetam was gradually increased to 1000 mg bid. The patient had no significant adverse effects from this dosage. At age 21, she had a second tonic-clonic seizure while in college; further discussion with her room-mate at that time revealed a history of two recent episodes of 1–2 minutes of altered consciousness with lip smacking (focal impaired awareness seizure, formerly complex partial seizure). A repeat EEG showed occasional right temporal spikes. What is one possible strategy for controlling her present symptoms? A 23-year-old woman presents to the office for consultation regarding her antiseizure medications. Seven years ago, this otherwise healthy young woman had a tonic-clonic seizure at home. She was rushed to the emergency department, at which time she was alert but complained of headache. A consulting neurologist placed her on levetiracetam, 500 mg bid. Four days later, electroencephalography (EEG)

Focal Onset Seizure -- Introduction -- Focal Motor Seizures (Formerly Simple Partial Seizures, Complex Partial Seizures)

Focal motor seizures occur due to an epileptogenic lesion on the contralateral frontal lobe. They usually originate from the supplementary motor area. The excitatory focus is generally around the rolandic (motor) cortex. Temporal lobe seizures can also have motor symptoms. These symptoms include turning the head and neck to the opposite side and sometimes tonic contractions of the limbs and trunk. The motor manifestations of focal motor onset seizures can be characterized by tonic, clonic, atonic, myoclonic, hyperkinetic, epileptic spasms, and automatisms. [5] Motor manifestations of focal motor onset seizures include the following: Abnormal sustained contractions and posturing of the limbs characterize tonic movements. Clonic movements are characterized by repeated, short contractions of various muscle groups characterized by twitching movements or rhythmic jerking. Atonic movements are characterized by loss of tone in a limb.

Neurology_Adams

the description of the experience, although the term “indescribable” is often included in the report, whereas vague and imprecise descriptions of “something being wrong” or resorting to a friend or family member to describe the event usually implicates a psychogenic seizure. We place emphasis on amnesia for the events of at least part of the seizure as an important criterion for the diagnosis of temporal lobe epilepsy. Hysterical fugues can cause substantial difficulty in diagnosis. They may be recognized by the loss of personal identity and by episodes that are longer than typical of seizures, sometimes up to a few days.

Focal Impaired Awareness Seizure -- Introduction -- Seizures of Temporal Lobe Origin

These are the most common type of focal impaired awareness seizures. Stereotyped automatisms occur in about 40% to 80% of patients with temporal lobe epilepsies. Seizures with predominantly oral and manual automatism and some other motor manifestations are highly suggestive of temporal lobe origin. About 60% of temporal lobe seizures have a secondary generalization. Gradual recovery after several minutes of confusion occurs postictally in most patients; however, in some patients, automatic behavior like running or walking about, the non-directed violent behavior may occur. Temporal lobe focal impaired seizures can have features similar to frontal seizures, but temporal lobe focal impaired seizures typically have slower onset and progression and more pronounced confusion. Certain features can help in localizing the seizure onset to one hemisphere. Ictal vomiting, ictal speech, urinary urge, and automatisms with intact consciousness suggest seizure onset in the non-dominant hemisphere, and speech disturbance postictally is suggestive of seizure onset in the dominant hemisphere. Upper limb dystonia lateralizes seizure to the opposite hemisphere.

Focal seizure

A focal impaired awareness seizure affects a larger part of the hemisphere and the person may lose consciousness. If a focal seizure spreads from one hemisphere to the other side of the brain, this will give rise to a focal to bilateral seizure. The person will become unconscious and may experience a tonic clonic seizure. When people have multiple focal seizures they generally have a condition known as temporal lobe epilepsy. (A generalized seizure is one that involves both sides of the brain from the onset.) Simple partial seizures Simple partial seizures are seizures which affect only a small region of the brain, often the temporal lobes or structures found there, such as the hippocampi. People who have focal aware seizures remain conscious. Focal aware seizures often precede larger focal impaired awareness seizures, where the abnormal electrical activity spreads to a larger area of the brain. This can result in a tonic-clonic seizure. Presentation

EEG Localization Related Epilepsies -- Clinical Significance -- Temporal Lobe Epilepsy (TLE)

Seizure semiology: Auras: Visceral sensations and fear are associated with mesial temporal lobe epilepsy, while auditory and vertiginous auras have been associated with the neocortical temporal lobe epilepsy. Neocortical temporal lobe epilepsy: At seizure onset, patients with neocortical TLE are more likely to describe any type of hallucination or illusion. Automatisms and dystonic posturing are not found in neocortical TLE. [32] [35] Mesial temporal epilepsy: Patients present with a behavioral arrest that is observed as a blank facial expression along with loss of awareness. This change is followed by oral, facial, or alimentary automatisms such as lip-smacking, chewing, sucking, or swallowing or is accompanied by ipsilateral automatisms such as repetitive hand movements, picking, or fidgeting behavior, and contralateral abnormal posturing of limbs. Patients commonly have a period of postictal confusion. Rarely, this progresses to secondary generalization. [36]

[Amnesic presentations of the compulsive obsessional confusions (about 3 patients appearing in a consultation of memory)].

Disorders or complaints of memory are a frequent cause of consultation in depression, major anxiety and psychiatry disease with personality disorders. We report 3 patients with obsessive compulsive disorder (OCD), without diagnosis and treatment, examined in a specialized memory consultation. They always had OCD with cognitive checking. Diagnosis of transient global amnesia and temporal complex seizure were discussed in 2 cases. Psychometric impairment only was observed in first free recall of a verbal memory task and was no specific. Behavioural during testing seemed to be very important to analyse. First, a 49-year-old man consulted because he had stereotyped transient amnesia lasted one minute, 2 or 3 times a week, since 6 months. He was a teacher. Transient amnesia always occurred during lessons. Suddenly he didn't know where he was or what he was speaking about. Episodes lasted one minute. After them, he had no confusion and no difficulty in concentration but intense anxiety. In an another hand, when he was in his car, after lessons, he could forget where he was during some minutes. CT scan and EEG were normal. Neuropsychological tests only objectived impairment in first free recall of Grober and Buschke's words. Patient explained that he could not prevent to check responses. He told us checking obsessive compulsive disorder during since long time ago. We discussed clear differences which existed between seizure and ruminations or preoccupations. Secondly, a 55-year-old woman was afraid of her memory performances. She was medical secretary and had no problem in her work but she would like a memory consultation to reassure herself. She was neither depressed nor anxious. She presented curious production in fluency task. She had to produce as many animals's names as possible: she could say 35 names which was an excellent performance but only in alphabetic order! Neuropsychological tests objectived impairment in her first free recall of Grober and Buschke's words. She tried in her first free recall to remember words in alphabetic order. She explained how she was bound to range everything in alphabetic order! She had a lot of rituals. She thought that she had an obsessive compulsive disorder but never consulted about this. The observation illustrated suspiscions about memory operations which could be observed in patients group with obsessive compulsive disorders. Finally, a 62-year-old man told us that he had presented a transient global amnesia during 4 hours. He had an important appointment and was upset about that. He didn't go to it and wandered in his flat. He always asked the same questions and forgot everything. He had no neurological deficit. He was anxious, sad and cried several times. He perfectly remembered the episod and thought that he had a panic attack! Verbal memory tests only objectived difficulties in his first free recall of Grober and Buschke words as the two others patients. He had a story of obsessive compulsive disorder with checking and rituals. In this observation, we discussed clear differences which existed between panic attacks and global transient amnesia. We analyzed patterns of neuropsychological performances which illustrated clinical features of obsessive compulsive disorder. These three patients impaired in their first free recall of verbal memory task. It is not a specific result. We observed during psychometric evaluation, strategic processing which impaired episodic memory: patients tried to check their performances. Memory complaints only were observed in checking obsessive compulsive disorder. It is a difficulty or a doubt about memory capacities. Difficulties could be due to particular cognitive processes who pertubate normal memory capacities.

Clinical and EEG features of complex partial seizures of extratemporal origin.

Complex partial seizures (CPSs) of extratemporal origin are frequently misdiagnosed. Common reasons for diagnostic errors are failure to recognize the epileptic cause of the attacks or to appreciate localizing clinical seizure characteristics and nonspecific or misleading scalp EEG findings. This report describes clinical characteristics of two types of extratemporal CPSs, frontal and occipital. Frontal lobe CPSs are brief, frequent attacks that begin and end suddenly. They are dominated by complex, semi-purposeful motor automatisms, and vocalization and sexual automatisms are common. Their often bizarre appearance frequently leads to a misdiagnosis of hysteria. Occipital lobe CPSs are characterized primarily by their spread pattern, which can follow various paths. They may mimic temporal lobe CPSs, sensorimotor partial seizures, or supplementary motor partial seizures. Scalp EEGs are frequently misleading. The initial clinical symptoms are the most important clue to correct diagnosis and include elemental visual symptoms, visual loss, eye pulling or movement sensations in the absence of detectable movement, rapid forced blinking or eye flutter, and contralateral eye deviation.

Rare Phenomenon of Limb-shaking TIA, Resolved With Intracranial Wingspan Stenting.

Illustrates the importance of differentiating limb-shaking transient ischemic attack (LS-TIA) from focal seizures and carefully selecting patients for intracranial stenting. LS-TIA is associated with severe carotid stenosis or occlusion, often precipitated by cerebral hypoperfusion. A case study of 313 patients with symptomatic intracranial artery stenosis/occlusion reported 11% with LS-TIA. In our literature search, we did not find any other cases of successful treatment of LS-TIA with an intracranial Wingspan stent. A 66-year-old woman with a history of atrial fibrillation on anticoagulation, hypertension, hyperlipidemia and left middle cerebral artery (MCA) stroke followed by a left internal carotid artery (ICA) endarterectomy presented with transient repetitive involuntary movements (TRIMs) over her right upper and lower limbs. She described episodes of numbness followed by TRIMs over the right side of her body lasting for 1 to 2 minutes. TRIMs occurred only while standing and usually resolved on sitting or lying down. Conventional angiogram showed severe stenosis of left supraclinoid ICA and proximal MCA. Brain single photon emission computed tomography scan showed impaired vascular reserve in the left MCA territory. An ambulatory electroencephalogram captured TRIMs without epileptiform discharges. She continued to have symptoms with no improvement over a period of 3 months on maximal medical management. She underwent successful endovascular treatment with the Wingspan stent system in the left M1 segment and terminal ICA. Following the revascularization procedure, her symptoms resolved. It is vitally important to differentiate LS-TIA from focal seizures. This is the first documented case report of complete resolution of LS-TIA symptoms following an intracranial Wingspan stenting.

Recurrent diarrhea as a manifestation of temporal lobe epilepsy.

A woman with temporal lobe epilepsy manifesting with repeated episodes of sudden diarrhea and loss of consciousness is reported. A 63-year-old, right-handed female presented with chief complaints of sudden diarrhea and loss of consciousness for almost three decades. The first attack occurred in her 30s, and similar attacks repeated several times in a year. Her attacks comprised abrupt abdominal discomfort, diarrhea, sudden emergence of old memories relating to when she had played with her brother in her childhood, and loss of consciousness during defecation. She had no convulsion or automatism and fully recovered in a few minutes. Every time she was transferred to emergency hospital by ambulance, she had examinations such as blood test, head computed tomography, electrocardiogram, abdominal ultrasound, and electroencephalography (EEG), but no specific diagnosis was made. On admission to our hospital, vital signs, neurological examination, and blood tests did not show abnormal findings. During long-term video-EEG monitoring for 40 h, she had no habitual event. Interictal EEG showed intermittent irregular delta waves and sharp regional transients in the left anterio-midtemporal area. Sharp transients were not as outstanding from background activities as to be defined as epileptiform discharges, but they were reproducible in morphology and distribution and appeared not only in sleep but also in wakefulness. Brain magnetic resonance imaging was unremarkable. Single-photon emission computed tomography showed a decrease of blood flow in the left frontal and temporal lobes. Wechsler Adult Intelligence Scale-III showed a decline of verbal comprehension. We concluded that the patient was suffering from partial epilepsy originating from the left temporal lobe. Carbamazepine markedly improved her seizures. Temporal lobe epilepsy can manifest with diverse autonomic symptoms and signs. Abdominal sensations often herald the onset of epileptic seizures. Among them is an uncommon syndrome called abdominal epilepsy in which gastrointestinal complaints are the primary or the sole manifestation of epileptic seizures. In patients who present with diarrhea and other autonomic symptoms otherwise unexplained, a possible diagnosis of epilepsy should be considered.

Transient epileptic amnesia

TEA is a form of focal seizure, which is "the most common variety of adult-onset epilepsy" as opposed to the stereotypical tonic-clonic or grand mal seizure in which affected persons suffer a loss of consciousness and convulse. Diagnostic criteria for the disorder were adopted in the 2007 study of 50 case emphasized clinical features that distinguish TEA from transient global amnesia (TGA), with which TEA is often compared: A history of recurrent witnessed episodes of transient amnesia. In some people, they are very infrequent (less than one per year) whereas others experience them as often as once a week. The episodes are usually very similar to each other. Cognitive functions other than memory are judged to be intact during typical episodes by a reliable witness. In addition to the complex learned behaviors cited above by the TIME organization, reports include people with TEA sight-reading music and playing piano, translating languages, driving or sailing a yacht in the open sea.

Seizure types

Focal seizures (Older term: partial seizures) Simple partial seizures – consciousness is not impaired With motor signs With sensory symptoms With autonomic symptoms or signs With psychic symptoms Complex partial seizures – consciousness is impaired (*Note: impaired does not necessarily mean [fully] lost) (Older terms: temporal lobe or psychomotor seizures) Simple partial onset, followed by impairment of consciousness With impairment of consciousness at onset Partial seizures evolving to secondarily generalized seizures Simple partial seizures evolving to generalized seizures Complex partial seizures evolving to generalized seizures Simple partial seizures evolving to complex partial seizures evolving to generalized seizures Generalized seizures Absence seizures (Older term: petit mal, meaning 'small, or smaller, bad') Myoclonic seizures Clonic seizures Generalized tonic–clonic seizures (Older term: grand mal, meaning 'great, or greater, bad') Atonic seizures

Pharmacoresistant partial-onset epilepsy misdiagnosed as panic disorder: a case report.

The clinical differentiation between simple partial epileptic seizures of temporal lobe origin and panic attacks is often difficult on clinical grounds alone, because both conditions are characterized by common symptomatology which includes the feeling of fear, autonomic system dysfunction, disorientation and alternation of the level of consciousness when these conditions evolve clinically. The symptoms stem from common pathophysiologic and anatomic substrates of these two conditions, localized in the limbic system, especially the amygdala. We present the case of a young woman who had a febrile seizure in childhood and subsequent episodes of fear accompanied by tremor and possible alteration of consciousness followed by headache. These spells were diagnosed as panic attacks during her teenage years and she was given Clobazam in order to suppress them. The patient responded well for several years without attacks but her symptoms reappeared following discontinuation of her medication in order to conceive. At that time a detailed history was taken from her spouse and further clinical evaluation raised the suspicion of seizures especially due to the fact that her spells were characterized by alteration of consciousness; she was therefore referred for additional investigations which included admission to a monitoring unit for epilepsy. Long-term video-EEG recording revealed the presence of simple partial seizures with secondary generalization confirming the clinical impression. She was subsequently treated with antiepileptic medications; however the patient's condition worsened to the point where she became pharmacoresistant having failed several antiepileptic drug trials in monotherapy or combination. An MRI scan of the brain revealed the presence of right-sided mesial temporal sclerosis, a known consequence of febrile seizures. Her seizures were nocturnal tonic-clonic and gradually worsened to the point of occurring during most nights. She was therefore referred for a presurgical evaluation which confirmed that the epileptic focus was associated with the area of mesial temporal sclerosis. The epileptic focus was successfully removed from the right anterior temporal lobe and since then she remained free of seizures whereas, in addition, the presumed symptoms of panic attacks also resolved. This case indicates the occasional difficulty in diagnosing simple partial seizures and how it may be confused with psychiatric conditions. Therefore, the treating physician, especially the psychiatrist, should remain vigilant when treating cases of panic attacks, especially when they present with either atypical symptomatology, such as the case described, or when they do not respond to appropriately chosen treatment; such cases may warrant referral for further investigation.

A patient with atonic seizures mimicking transient ischemic attacks.

A focal atonic seizure is a partial seizure in which the ictal manifestation consists of paresis of the extremities or muscles on one side of the body, and this phenomenon can easily be misdiagnosed as a transient ischemic attack. An 86-year-old woman visited our hospital complaining of transient right upper extremity weakness lasting for 10 min following an unusual sensation in her chest accompanied by palpitations. On the third hospital day, she again complained of right arm weakness, which progressed to jerky movements of her right extremity accompanied by facial twitching and then generalized into a tonic-clonic seizure. The EEG displayed several interictal spikes in the contralateral temporal area, and the ictal SPECT, analyzed using the SISCOM system, showed an increased signal in both the contralateral superior parietal area and the mesial frontal area. In this case, the patient was diagnosed with focal atonic seizures as the cause of the monolimb weakness, which had been initially misdiagnosed aas transient ischemic attacks. In cases in which a patient presents with monolimb paresis, physicians should consider the possibility of an atonic seizure as the cause.

[Ictal semiology of temporal partial complex seizures: usefulness for localizing and lateralizing the origin of the attacks].

To review signs and symptoms associated with temporal complex partial seizures (CPS) and their utility in the localization and lateralization of seizure onset. CPS are particularly resistant to the standard antiepileptic drugs. Since surgical treatment is a therapeutical alternative in patients with intractable seizures, localization and lateralization of seizure origin are the principal aims in the preoperative assessment. Video-EEG monitorization has made possible characterization of ictal behaviour and correlation with cerebral regions generating the epileptic discharge. Therefore, ictal semiology has increased its importance in the localization and lateralization of seizures. The utility of auras, automatisms, motor manifestations, speech disturbances and autonomic features have been reviewed in relation to this approach. Viscerosensorial and experiential auras have been associated with temporal lobe epilepsy. The automatisms are not exclusive of temporal seizures and may be observed in frontal epilepsy and parietal and occipital seizures with spreading to temporal structures. There is not agreement in relation to head turning and version, therefore, this clinical sign should be used in correlation to other clinical manifestations. Distonic posturing, comprehensible ictal speech and postictal dysphasia appear to be the most reliable clinical signs in the lateralisation of temporal lobe seizures.

Comorbid seizure reduction after pallidothalamic tractotomy for movement disorders: Revival of Jinnai's Forel-H-tomy.

Forel-H-tomy for intractable epilepsy was introduced by Dennosuke Jinnai in the 1960s. Recently, Forel-H-tomy was renamed to "pallidothalamic tractotomy" and revived for the treatment of Parkinson's disease and dystonia. Two of our patients with movement disorders and comorbid epilepsy experienced significant seizure reduction after pallidothalamic tractotomy, demonstrating the efficacy of this method. The first was a 29-year-old woman who had temporal lobe epilepsy with focal impaired awareness seizure once every three months and an aura 10-20 times daily, even with four antiseizure medicines. For the treatment of hand dyskinesia, she underwent left pallidothalamic tractotomy and her right-hand dyskinesia significantly improved. Fourteen months later, she had experienced no focal impaired awareness seizure and the aura decreased to one to three times per month. The second case was that of a 15-year-old boy diagnosed with progressive myoclonic epilepsy, who developed generalized tonic-clonic seizure, which manifested once every month, despite treatment with five antiseizure medicines. After surgery, myoclonic movements in his right hand slightly improved. A one-year follow-up revealed that he had not experienced a generalized tonic-clonic seizure. The lesion locations in the two cases were close to the vicinity of Jinnai's Forel-H-tomy. Forel's field H deserves reconsideration as a treatment target for intractable epilepsy.

A particular evolution of two cases of somatosensory spike epilepsy.

"Somatosensory spike epilepsy" is still little known, except in highly specialized places, because of two factors: 1) it is rare (the incidence in our experience is one case in 1,000 new EEGs); 2) the evoked spikes, that constitute the first sign for the evolution of the syndrome, are not systematically sought, and many months can pass before finding one "positive" cases. The evolution of a "positive" case towards epilepsy is represented by a 15% possibility. In this report we present two unique cases of this syndrome; they are of interest because they constitute the first such cases in the literature and also because of the neurophysiological problems that they reveal. The cases are represented by an eight year old girl and by an eleven year old boy, who suffered from the typical form of "sensitive spike epilepsy" characterized by its classical evolution in four stages, with a clinical phase of about one year. Two years after the cessation of previous seizures, they presented a new "bouffee" of partial motor seizures. The EEG abnormalities were in the parietal parasagittal region the first time, while they were situated in the temporoparietal region in the second time. The somatosensory spikes were evoked from the foot the first time and from the fingers the second time. In the second case the trigger zones were only on the left side of the body.

InternalMed_Harrison

Focal seizures can spread to involve both cerebral hemispheres and produce a generalized seizure, usually of the tonic-clonic variety (discussed below). This evolution is observed frequently following focal seizures arising from a focus in the frontal lobe, but may also be associated with focal seizures occurring elsewhere in the brain. A focal seizure that evolves into a generalized seizure is often difficult to distinguish from a primary generalized-onset tonic-clonic seizure, because bystanders tend to emphasize the more dramatic, generalized convulsive phase of the seizure and overlook the more subtle, focal symptoms present at onset. In some cases, the focal onset of the seizure becomes apparent only when a careful history identifies a preceding aura. Often, however, the focal onset is not clinically evident and may be established only through careful EEG analysis. Nonetheless, distinguishing between these two entities is extremely important, because there may be substantial

[A case of left dorsomedial thalamic infarction with unilateral schizophrenia-like auditory hallucinations].

We report a case of a right-handed, 73-year-old woman with auditory hallucinations lateralized to the right ear. A brain MRI revealed a small infarction in the left dorsomedial nucleus (DM) of the thalamus. The patient did not have either psychiatric or neurological prior history, and had otherwise been treated for ischemic heart disease, hypertension, and hyperlipidemia for 10 years. Two months prior to admission, she had become forgetful, and had lost her wallet several times. She concurrently began to experience auditory hallucinations in which she heard the voices of her acquaintances, or "the gods". She frequently monologized and wandered about outside following the contents of the hallucinations. Therefore, she was admitted to our institution. On admission, no apparent abnormalities were revealed by physical examinations or blood analyses. She was alert and had no aphasic symptoms. Except for memory disturbances, no neurological symptoms, including no hearing loss, were found. A brain MRI showed a small localized infarction in the left DM, but EEG findings were normal. The patient had prominent anterograde memory deficits: she hardly remembered what she had done the very same day, or the names of the doctor and hospital. She also demonstrated a retrograde amnesia of the past decade or two: she showed difficulty recalling either personal history or social events that occurred during this era. Wechsler Adult Intelligence Scale-Revised (WAIS-R) revealed a total IQ of 75 (verbal IQ 77; performance IQ 77). The verbal hallucinations continued with frequent occurrence even after admission. They included voices telling her about misfortunes, such as death or sickness, of her relatives. These turned into threats and commands, such as "I'm gonna kill ya. I attack you from behind. You, do not eat!" In addition, she occasionally experienced "third person auditory hallucination", in which several men were discussing the plan to kill her. As is characteristic of this type of case, the hallucinations always appeared in only her right ear. They did not occur in the other modalities (e.g. as a visual one). She was convinced that the hallucinations were real and looked frightened while they were happening. Whereas the anterograde amnesia continued for 6 months after admission, the retrograde amnesia gradually improved within 2 or 3 months after admission, although a partial amnesia on the past decade eventually turned out to persistent. On the other hand, the hallucinations did not ameliorate satisfactorily with risperidone (3-6 mg/day), but on augmentation with olanzapine (5-20 mg/day), they lessened gradually and almost disappeared within 6 months. She also slowly developed symptoms similar to those of frontal lobe syndrome, i.e., aspontaneity and apathy. In conclusion, our case indicates the importance of DM on memory function. It is noteworthy that schizophrenia-like hallucinations developed in the case. Localized neuronal deficits evoked by infarction in the left DM probably caused the schizophrenia-like hallucinations; the lateralization phenomenon further indicates the involvement of specific neuronal mechanisms in the mediation of the hallucinations. According to the knowledge of the functional anatomy of the DM and the lateralization phenomenon of auditory hallucinations, it is possible that the neuronal loop, comprised of the prefrontal cortex and thalamus, designated as "basal ganglia-thalamocortical circuits", in addition to the left temporal cortex, plays an important role in the development of the hallucinations in this case. This possibility might also shed light on the neurological basis of schizophrenia.

[A case of frontal lobe epilepsy presenting with recurrent speech arrest].

We report a 55-year-old right-handed man with frontal lobe epilepsy manifesting recurrent speech arrest. He was known to have hypertension, hypertriglyceridemia, and gout. In the three days prior to admission, he had episodes of sudden inability to talk. These episodes lasted 10 to 30 seconds and recurred ten to twenty times a day. On admission, speech comprehension and other mental functions were normal, as were findings on neurologic examination. During the period of speech arrest, he understood spoken commands, and there was no abnormal motor activity or paresis. The episodes of speech arrest were thought to be short aphasic periods due to transient ischemic attacks in the left carotid territory. Computed tomography and magnetic resonance imaging demonstrated a small calcified lesion in the upper medial portion of the left frontal lobe. Left internal carotid angiography demonstrated no abnormal findings. After neuroradiological examination finished, he suddenly raised his right hand and followed it with his gaze and a right head turn. The EEG seizure pattern in which 20-25 Hz activity began in the left fronto-central region and spread rapidly to the right fronto-central region, which after about 8 seconds was replaced by 12-14 Hz flattening rhythmic polyspikes was detected 9 times within 60 minutes. It is most unusual for supplementary motor area seizure to present pure paroxysmal speech arrest without accompanying paroxysmal motor activity. As in our case, epileptic arrest of speech may be confused with a transient ischemic attack of the dominant hemisphere.

Successful multiple burr hole openings for limb-shaking transient ischemic attack due to moyamoya disease: illustrative case.

Limb-shaking transient ischemic attacks (LS-TIAs) are a rare form of TIAs that present as involuntary movements of the limbs and indicate severe cerebral hypoperfusion. LS-TIAs are often reported in patients with carotid artery stenosis but can also affect patients with intracranial artery stenosis and moyamoya disease (MMD). A 72-year-old woman presented with repeated episodes of involuntary shaking movements of the right upper limb. Cerebral angiography revealed complete occlusion of the M1 segment of the left middle cerebral artery (MCA), and the left hemisphere was supplied by moyamoya vessels. She was treated with left direct revascularization without complications, and her involuntary movements subsided. However, she demonstrated involuntary shaking movements of the right lower limb 2 months postoperatively. Cerebral angiography revealed complete occlusion of the A1 segment of the left anterior cerebral artery (ACA). The multiple burr hole opening (MBHO) procedure was performed to improve perfusion in the left ACA territory and after 3 months, the patient's symptoms resolved. This case demonstrated that LS-TIAs can also develop as ischemic symptoms due to MMD. Moreover, instances of LS-TIA of the upper and lower limbs developed separately in the same patient. The patient's symptoms improved with direct revascularization and MBHO.


After reading the question and the possible answers, we have two tumor and two non-tumor options. At no time are atypical cellular features mentioned, so we can safely rule out tumor lesions, leaving doubts between answers 2 and 3. In this case, we would have to opt for IMMATURE TERATOMA, mentioning the presence of embryonic remains.

After reading the question and the possible answers, we have two tumor and two non-tumor options. At no time are atypical cellular features mentioned, so [HIDDEN], leaving doubts between answers 2 and 3. In this case, we would have to opt for IMMATURE TERATOMA, mentioning the presence of embryonic remains.

A 20-year-old woman with a 15 cm solid-cystic ovarian tumor detected by ultrasound after presenting with non-specific abdominal symptoms. In the anatomopathological study of the corresponding specimen, teeth, hairs, areas of intestinal epithelium, areas of squamous epithelium (15%) and bronchial epithelium, as well as neuroectodermal and embryonic elements are found in several of the histological preparations. In reference to this case, point out the correct diagnosis:

Malignant transformation of ovarian mature cystic teratoma with a predominant pulmonary type small cell carcinoma component.

A 68-year-old woman was diagnosed with mature cystic teratoma of the left ovary when she was 44 years old. The tumor recently enlarged rapidly, and abdominal magnetic resonance imaging revealed an intrapelvic cystic lesion, which measured 123 × 120 × 107 mm and contained a mural nodule. Under a clinical diagnosis of malignant transformation of mature cystic teratoma, bilateral salpingo-oophorectomy with total hysterectomy and omentectomy were performed. The resected specimen showed a unilocular cystic lesion containing a well-demarcated mural nodule measuring 35 × 30 × 25 mm in the left ovary. A microscopic examination revealed various types of carcinoma in the mural nodule: pulmonary type small cell carcinoma (65%), adenocarcinoma (25%), squamous cell carcinoma (5%), and transitional cell carcinoma (5%). Small cell carcinoma was positive for CD56, synaptophysin, and chromogranin A. The adenocarcinoma component showed intestinal phenotypes; i.e. cytokeratin (CK) 7(-), CK20(+), CDX2(+), estrogen receptor(-), and progesterone receptor (-). Interestingly, CDX2 positivity was retained in all of the carcinomas. It was assumed that the adenocarcinoma had arisen from an intestinal epithelium in the mature cystic teratoma and then differentiated into the diverse histological types mentioned above.

Adenocarcinoma arising from the respiratory ciliated epithelium in a benign cystic teratoma of the ovary.

Benign cystic teratoma of the ovary (BCTO) is the most common benign ovarian tumor, accounting for 15-20% of all ovarian tumors. It is usually diagnosed in the third and fourth decades of life. Malignant transformation is rare, occurring in approximately 1-2% of reported cases, with squamous cell carcinoma being the most common form. Adenocarcinoma arising from mature cystic teratoma is extremely rare. We present the patient with a BCTO, where a malignant transformation of respiratory ciliated epithelium resulted in well differentiated adenocarcinoma. Although respiratory epithelium is often found in BCTOs, adenocarcinoma arising from this cell type is uncommon. To our knowledge, this is the fourth reported case of adenocarcinoma arising from the respiratory epithelium of a BCTO.

Ovarian Teratoma in Routine Biopsy Material During a Five-Year Period.

Teratomas are tumors derived from germ cells, most frequently arising in the gonads. The aim of this study was to determine the number of ovarian teratomas diagnosed in the routine biopsy material at Ljudevit Jurak Clinical Department of Pathology, Sestre milosrdnice University Hospital Center during a 5-year period, as well as their clinical, gross and microscopic characteristics. Teratomas accounted for 48.6% (n=166) of primary ovarian tumors. The patient mean age was 34.74±12.37 years. Difference in the incidence of teratoma between the left and right ovary was not significant; bilateral teratoma was found in 13 patients. Teratomas were detected by ultrasonography in 115 (69.27%) cases and the rest were found during surgery performed for other indications. Most teratomas (n=161; 96.9%) were mature and cystic (dermoid cysts). Mature and solid teratomas were diagnosed in 5 (3.01%), ovarian struma in 2 (1.8%) cases and strumal carcinoid in 1 (1.2%) case. Mature cystic teratomas contained sebaceous material in 123 (76.8%) cases, and a total of 16 teeth were found; 157 (94.5%) teratomas measured <10 cm in largest diameter. Microscopically, mature cystic teratomas most frequently contained ectodermal (skin with appendages, mature glia and nerve ganglia) and mesodermal (fi brous, fat tissue, cartilage and bone) tissues. Frequently found tissues of endodermal origin were respiratory and intestinal epithelia. Small foci of thyroid tissue were found in 20 (12%) teratomas. Chronic granulomatous foreign body reaction in the wall of mature cystic teratomas was found in 11 (6.8%) tumors.

[Malignant transformation of thyroid tissue in an ovarian dermoid cyst: case report].

Mature cystic teratoma is the most common type of ovarian teratomas and also the most frequent tumor originating from germ cells. Usually unilateral, it is, by definition, composed of well-differentiated derivates which originate from all three layers. It is most frequently diagnosed in the female reproductive period; in its pure form, it is generally benign, but occasionally it may undergo malignant transformation in one of its elements. The authors report a case of a 43-year-old woman who underwent a thyroid regulation substitutive therapy 12 years after thyroidectomy which had been performed because of Basedow's disease. On the occasion of her fourth delivery by cesarean section, bilateral cystical formations in both ovaries were found. Upon ending the delivery, the right ovary with the cyst was surgically removed, as well as part of the cystically changed left ovary. The pathohistological analysis of the samples taken from the left ovary cyst pointed to the following diagnosis: dermoid cyst of the ovary a mature cystic teratoma, whereas the samples from the cystic tumor of the right ovary were diagnosed as: ovarian teratoma malignant a papillary carcinoma of the thyroid tissue and cystadenoma papillare serosum ovarii. The histological type of the malignant thyroid tissue neoplasm in the teratoma was a papillary carcinoma consisting of papillary proliferations lined by cuboidal, cylindrical and pseudostratified epithelium with charasteristic "ground glass" nuclei, but without invasion into the blood vessels. Malignant transformation of the ectopic thyroid tissue, which is one of the elements of the ovarian cystic teratoma, is extremely rare: it is diagnosed on basis of the histological picture and criteria which are the same as for the tumor of the thyroid gland. One should not exclude a pssibility that the identical or different pathological alterations characteristic of the thyroid gland may also be detected in the ectopic thyroid tissue of a dermoid cyst. The reported case was chosen due to its complex and rich clinical and pathohistological picture characterized by the occurrence of a bilateral dermoid cyst with the malignant transformation of the thyroid tissue. The malignant neoplasm in the mature ovarian cystic teratoma was papillary carcinoma of the thyroid tissue with all histological properties typical for the thyroid gland carcinoma. The possibility of the malignant transformation of the endodermal thyroid tissue in the mature ovarian cystic teratoma and changes in the thyroid gland of the Basedow's disease type diagnosed before are without clear causation.

Well-differentiated cerebellum in an ovarian mature cystic teratoma: a case report and review of the literature.

Mature teratoma is the most common germ cell tumor as it represents 95% of germ cell tumors. Although common in children and young adults, ovarian teratoma can occur at any age. Mature teratomas are composed of mature tissues representing elements derived from more than one embryonic germ layer (ectoderm, mesoderm, and endoderm), with ectodermal derivatives being the usual predominant component; however, the finding of a well-differentiated cerebellum is extremely rare. A 20-year-old Saudi female presented to the emergency department with severe abdominal pain of 1-day duration. Pelvic ultrasound showed a large ovoid- to bilobed-shaped cystic pelvic structure extending to the lower abdomen. The patient underwent left ovarian cystectomy. Microscopic examination showed a cyst wall with skin tissue, including adnexal structures (sebaceous glands), a well-differentiated cerebellum, and mature glial tissue. After extensive sampling, no immature component was identified. Thus, the final diagnosis of a mature cystic teratoma with well-differentiated cerebellum was established. The patient was well postoperatively and was discharged in a stable condition. We report this case of well-differentiated cerebellum within ovarian teratoma to expand the pool of cases reported in literature of this extremely rare entity, as only 22 cases with such findings have been reported in literature to the best of our knowledge. This finding poses a diagnostic challenge to the pathologist due to its rarity and its similarity to immature teratoma. We thus emphasize that thorough sampling of ovarian teratoma is of paramount importance and to keep the aforementioned diagnosis in mind and not confuse it with immature elements, especially in intraoperative consultation and frozen sections.

A case of concomitant occurrence of struma ovarii and malignant transformation of cystic teratoma.

A 77-year-old woman received a total abdominal hysterectomy and bilateral salpingo-oophorectomy because of a tumor in the left ovary. The surgical specimen measured 8.5x4.5x4.0 cm, and the solid lesion measured 4.0x3.5x3.5 cm. The solid lesion was diagnosed as struma ovarii. The cyst wall partially comprised squamous epithelium-like and ciliated columnar epithelium-like cells. The tumorous lesion of the cyst wall revealed a poorly differentiated adenocarcinoma. Immunohistochemically, the tumor cells were positive for cytokeratin7, and were negative for cytokeratin20 and thyroid transcription factor-1. The authors diagnosed that struma ovarii and other parats coexisted as a poorly differentiated adenocarcinoma that had arisen from a mature ovarian cystic teratoma. As for the identification of the origin of adenocarcinomas arising from mature ovarian cystic teratomas, more cases need to be identified and investigated.

Mature cystic teratoma of the fallopian tube in a postmenopausal woman: a case report and review of the literature.

Background. Mature cystic teratomas of the fallopian tube are extremely rare and only 54 cases have been reported in the literature. In this paper, we report a mature cystic teratoma of the fallopian tube in a postmenopausal woman and we report the review of literature of tubal cystic teratomas. Case. A 62-year-old, gravida 4 postmenopausal woman presented with pain in the right lower abdominal region for a long time. An 88 × 72 × 95 mm heterogeneous mass which contained calcifications and lipoid components was detected in the right adnexal region by transvaginal ultrasonogram (TV-USG). Serum tumour markers, namely, CA125, CA15-3, and CA19-9, were within normal range. A laparotomy revealed a 9 × 10 cm cystic mass within the fimbrial region in the right fallopian tube, and right salpingoopherectomy was performed consequently. Microscopic examination revealed squamous epithelium with sebaceous glands and hair follicles, and pseudostratified ciliated respiratory epithelium with cartilage and mucous glands. Because the frozen section resulted in a benign dermoid cyst, no further operative procedure was performed. The postoperative follow-up was uneventful and the patient was discharged on the second postoperative day. Conclusion. In cases of undetermined pelvic or abdominal masses, a teratoma of the fallopian tube should be considered.

A case of ovarian mature cystic teratoma presenting as a pedunculated ileal tumor.

We report on a 34-year-old Japanese woman who presented with a pedunculated ileal tumor and who was finally diagnosed as having a right ovarian mature cystic teratoma penetrating and protruding into the ileum. She had undergone laparoscopic left ovarian cystectomy, whose specimen was diagnosed as dermoid cyst when she was 27 years old. The colon fiberscope revealed an ileal polyp, diagnosed as mature teratoma. Because of adhesion to the necrotic nodule between the tumor and the right ovary, ileocecal resection and right ovarian cystectomy were performed. The ileal tumor contained tissues of skin, neuroglia, ganglion, choroid, retina, smooth muscle, as well as fibrous and adipose tissues, cartilage, bone, mucous epithelium, and bronchial structures with bronchial glands. The necrotic nodule showed abscess, granulation tissue, foreign body reaction, hairs, normal ileal epithelium, and the ovary with ovums.

Teratoma

Malignant transformation A "benign" grade 0 (mature) teratoma nonetheless has a risk of malignancy. Recurrence with malignant endodermal sinus tumor has been reported in cases of formerly benign mature teratoma, even in fetiform teratoma and fetus in fetu. Squamous cell carcinoma has been found in a mature cystic teratoma at the time of initial surgery. A grade 1 immature teratoma that appears to be benign (e.g., because AFP is not elevated) has a much higher risk of malignancy, and requires adequate follow-up. This grade of teratoma also may be difficult to diagnose correctly. It can be confused with other small round cell neoplasms such as neuroblastoma, small cell carcinoma of hypercalcemic type, primitive neuroectodermal tumor, Wilm's tumor, desmoplastic small round cell tumor, and non-Hodgkin lymphoma.

Adenocarcinoma arising from respiratory ciliated epithelium in benign cystic teratoma of the ovary: a case report with analyzes of the CT, MRI, and pathological findings.

The malignant transformation of mature cystic teratoma is rare, thus occurring in only 1-2% of all cases. The most common malignancy arising in mature cystic teratoma is squamous cell carcinoma. Adenocarcinoma occurs with less frequency. We herein present a patient with an ovarian mature cystic teratoma who demonstrated a malignant transformation to well-differentiated adenocarcinoma. Malignant transformation was diagnosed preoperatively by contrast enhanced computed tomography (CT) and magnetic resonance imaging (MRI). Microscopically and immunohistochemically, the adenocarcinoma was considered to have arisen from the ciliated respiratory epithelium. After a 28-month of follow-up period, she remains free of the disease. This is the third reported case of adenocarcinoma arising in the respiratory epithelium of an ovarian mature cystic teratoma. Contrast enhanced CT and MRI are useful for making a preoperative diagnosis and an immunohistochemical study is helpful for defining its origin.

[Pelvic retroperitoneal teratoma. A case report].

Teratomas are congenital tumors comprising of tissues arising from pluripotent embryonic cells. Their common sites of occurrences are ovary, testis, mediastinum and retroperitoneum. Retroperitoneal teratomas are extremely rare in adults but occur in 3% of children. The presence of a cystic or complex adnexal mass during pregnancy is common with mature teratoma reported in approximately 30-50% of pregnancies. The ultrasound is the number one method of detection. The use of tumor markers is limited because it alters its value during pregnancy. We report a case of a 18 year old female patient who came to the General Hospital Dr. Miguel Silva in Morelia, Michoacán with a gestation age of 10.2 weeks and a recent diagnosis of an asymptomatic adnexal mass. Using the transvaginal approach, the ultrasound reported an irregular gestational sac with the absence of cardiac activity in the embryo. A diffusely echogenic adnexal mass with oval appearance, wall regularity and homogeneous internal echos of 12.96 x 8.99 cm. Exploratory laparotomy was performed finding normal uterus and ovaries, with the posterior retroperitoneal resection of the 15 cm multi-lobed tumor. The surgical specimen measured 13 x 12 x 8 cm, had a brownish color, wall irregularity, soft consistency, multi-lobed, and a yellowish interior greasy material. Microscopically the tumor is delimited with a firm capsule composed of a hyaline-fibrous tissue, lined by squamous keratinized epithelium, with multiple layers of keratin. This anato-pathologic finding is diagnostic of retroperitoneum mature teratoma.

Ovarian cancer

Immature, or solid, teratomas are the most common type of ovarian germ cell tumor, making up 40–50% of cases. Teratomas are characterized by the presence of disorganized tissues arising from all three embryonic germ layers: ectoderm, mesoderm, and endoderm; immature teratomas also have undifferentiated stem cells that make them more malignant than mature teratomas (dermoid cysts). The different tissues are visible on gross pathology and often include bone, cartilage, hair, mucus, or sebum, but these tissues are not visible from the outside, which appears to be a solid mass with lobes and cysts. Histologically, they have large amounts of neuroectoderm organized into sheets and tubules along with glia; the amount of neural tissue determines the histologic grade. Immature teratomas usually only affect one ovary (10% co-occur with dermoid cysts) and usually metastasize throughout the peritoneum. They can also cause mature teratoma implants to grow throughout the abdomen in a disease

Immature teratoma

An immature teratoma is a teratoma that contains anaplastic immature elements, and is often synonymous with malignant teratoma. A teratoma is a tumor of germ cell origin, containing tissues from more than one germ cell line, It can be ovarian or testicular in its origin. and are almost always benign. An immature teratoma is thus a very rare tumor, representing 1% of all teratomas, 1% of all ovarian cancers, and 35.6% of malignant ovarian germ cell tumors. It displays a specific age of incidence, occurring most frequently in the first two decades of life and almost never after menopause. Unlike a mature cystic teratoma, an immature teratoma contains immature or embryonic structures. It can coexist with mature cystic teratomas and can constitute of a combination of both adult and embryonic tissue. The most common symptoms noted are abdominal distension and masses. Prognosis and treatment options vary and largely depend on grade, stage and karyotype of the tumor itself.

Ovarian germ cell tumors

Teratomas Teratoma are most common germ cell tumor of ovary. Teratomas can be divided into two types: mature teratoma (benign) and immature teratoma (malignant). Immature teratomas contain immature or embryonic tissue which significantly differentiates them from mature teratomas as they carry dermoid cysts. It is commonly observed in 15 to 19-year-old women and rarely in women after menopause. Immature teratomas are characterized with a diameter of 14–25 cm, encapsulated mass, cystic areas, and occasional appearance of hemorrhagic areas. The stage of immature teratomas is determined depending on the amount of immature neuroepithelium tissue detected. Yolk sac tumor

[Benign mature teratomas of the mediastinum].

Mature teratomas of the mediastinum are rare. However, they represent the most common mediastinal germ cell tumours. The aim of this study is to describe their clinical and pathological characteristics. Fourteen cases of mediastinal mature teratoma, diagnosed between January 1992 and December 2006, were reviewed retrospectively, noting the clinical, radiological, surgical, and pathological findings. The patient population consisted of 10 females and 4 males with mean age of 29 years (5-56 years). Chest pain was the main symptom. Imaging features comprised a heterogeneous anterior mediastinal mass containing soft-tissue, fluid, fat, or calcium attenuation, or any combination of the four. Macroscopically all the tumours were cystic, or predominantly cystic, containing mostly grumous material. The mean size was 9 cm (5-12 cm). Histological examination constantly revealed skin with or without appendages. Other components identified were bronchial mucosa (12 cases), fat (12 cases), muscle (10 cases), cartilage (8 cases), bone (7 cases), gastrointestinal mucosa (7 cases), pancreas (5 cases), urothelial epithelium (3 cases), nervous and prostatic tissues (one case each). All the teratomas were mature, and 7 of them were intrathymic. Every form of teratoma occurs in the mediastinum (mature, immature and with malignant transformation) but, in our study, they were exclusively mature. The histology is essentially similar to that of teratomas of gonads. However, pancreatic tissue occurs frequently in mediastinum (54% in literature, 25% in our study) but not in the gonads. On the other hand, thyroid follicles have not yet been seen in mediastinal teratomas.

Dermoid cyst

A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair, pockets of sebum, blood, fat, bone, nail, teeth, eyes, cartilage, and thyroid tissue. As dermoid cysts grow slowly and contain mature tissue, this type of cystic teratoma is nearly always benign. In those rare cases wherein the dermoid cyst is malignant, a squamous cell carcinoma usually develops in adults, while infants and children usually present with an endodermal sinus tumor. Location Due to its classification, a dermoid cyst can occur wherever a teratoma can occur. Vaginal and ovarian dermoid cysts

Immature teratoma

Ovarian cancer is staged using the FIGO staging system and uses information obtained after surgery, which can include a total abdominal hysterectomy via midline laparotomy, unilateral (or bilateral) salpingo-oophorectomy, pelvic (peritoneal) washings, assessment of retroperitoneal lymph nodes and/or appendectomy. The AJCC staging system, identical to the FIGO staging system, describes the extent of tumor (T), the presence of absences of metastases to lymph nodes (N), the presence or absence of distant metastases (M). Pathology An immature teratoma contains varying compositions of adult and embryonic tissue. The most common embryonic component identified in immature teratomas is the neuroectoderm. Occasionally, tumors may present neuroepithelium that resemble neuroblasts. Tumors may also present embryonic components such as immature cartilage and skeletal muscle of mesodermal origin. Immature teratomas composed of embryonic endodermal derivatives are rare.

[Laparoscopic extirpation of retroperitoneal bronchogenic cyst].

The retroperitoneal bronchogenic cyst is an extremely rare anomaly. During the examination of an 18-year-old female patient due to her extreme thinness, the abdominal ultrasound and later the CT indicated as an accidental finding--a left side adrenal/suprarenal adenoma, which turned out to be hormonally inactive. But while we were doing a laparoscopic surgical intervention we found a 6-8-cm cyst in the retroperitoneum, between the greater curvature and the spleen. It contained light grey, mucinous liquid. The left suprarenal gland had normal size and appearance. During the microscopic examination the removed terime showed cystic structures, which were covered inside with multilayer ciliated columnar epithelium, mucous excretory glands, a whole layer of connective/interstitial and smooth muscle tissue. Neither cartilage, nor gastrointestinal epithelium was found. Only a few publications have reported about retroperitoneal bronchogenic cyst so far. These lesions occur very rarely at the greater curvature. Although this alteration is really unusual, bronchogenic cysts have to be expected in the differential diagnostics of the retroperitoneal tumors.

Teratoma

Types Mature teratoma A mature teratoma is a grade 0 teratoma. They are highly variable in form and histology, and may be solid, cystic, or a combination of the two. A mature teratoma often contains several different types of tissue such as skin, muscle, and bone. Skin may surround a cyst and grow abundant hair (see dermoid cyst). Mature teratomas generally are benign, with 0.17-2% of mature cystic teratomas becoming malignant. Immature teratoma Immature teratoma is the malignant counterpart of the mature teratoma and contains immature tissues which typically show primitive or embryonal neuroectodermal histopathology. Immature teratoma has one of the lowest rates of somatic mutation of any tumor type and results from one of five mechanisms of meiotic failure.

Pulmonary blastoma. Comparison between its epithelial components and fetal bronchial epithelium.

Three cases of pulmonary blastoma exhibiting biphasic epithelial and stromal patterns, and a case of fetal lung-type adenocarcinoma, were examined by immunohistochemistry and electron microscopy (EM) and compared with fetal bronchial epithelium in order to explore the multidirectional differentiation of their epithelial components. The glandular cells of all four tumors resembled fetal bronchial epithelial cells in the pseudoglandular stage. Neuroendocrine (NE) cells were also present; they were argyrophilic and expressed pan-NE markers, neurosecretory granules and peptide hormones. The neural cell adhesion molecule (NCAM) was strongly expressed on the cell membranes of glandular cells, as in the case of proximal bronchial epithelial cells at the pseudoglandular stage in fetal lung. Sialosylated Lewis X was also expressed, indicating that the epithelial cells were possibly of endodermal origin. Two of the four cases showed considerable immunoreactivity for alpha-fetoprotein (AFP). The epithelial cells of pulmonary blastomas may occasionally de-differentiate into cells functionally resembling fetal hepatic, foregut and yolk sac cells expressing AFP. Tumor examination by immunohistochemistry and EM suggested that the glandular cells of the tumors may differentiate to some extent like those of fetal large bronchi at the pseudoglandular stage, but there was concordance and discordance in the expression of neuroendocrine and oncofetal markers between blastomatous tumors and fetal bronchial epithelium.

Pathology_Robbins

Benign (mature) cystic teratomas are marked by the presence of mature tissues derived from all three germ cell layers: ectoderm, endoderm, and mesoderm. Usually these tumors contain cysts lined by epidermis replete with adnexal appendages—hence the common designation dermoid cysts. Most are discovered in young women as ovarian masses or are found incidentally on abdominal radiographs or scans because they contain foci of calcification produced by toothlike structures contained within the tumor. About 90% are unilateral, with the right side more commonly affected. Rarely do these cystic masses exceed 10 cm in diameter. On cut section, they often are filled with sebaceous secretion and matted hair that, when removed, reveal a hair-bearing epidermal lining ( Fig. 19.18 ). Sometimes there is a nodular projection from which teeth protrude. Occasionally, foci of bone and cartilage, nests of bronchial or gastrointestinal epithelium, or other tissues are present.

International Classification of Diseases for Oncology

M9064/3 Germinoma Germ cell tumor, NOS M9065/3 Germ cell tumor, nonseminomatous M9070/3 Embryonal carcinoma, NOS Embryonal adenocarcinoma M9071/3 Yolk sac tumor Endodermal sinus tumor Polyvesicular vitelline tumor Orchioblastoma Embryonal carcinoma, infantile Hepatoid yolk sac tumor M9072/3 Polyembryoma Embryonal carcinoma, polyembryonal type M9073/1 Gonadoblastoma Gonocytoma M9080/0 Teratoma, benign Adult cystic teratoma Adult/cystic teratoma, NOS Teratoma, differentiated Mature teratoma M9080/1 Teratoma, NOS Solid teratoma M9080/3 Teratoma, malignant, NOS Embryonal teratoma Teratoblastoma, malignant Immature teratoma, malignant or NOS M9081/3 Teratocarcinoma Mixed embryonal carcinoma and teratoma M9082/3 malignant teratoma, undifferentiated Malignant teratoma, anaplastic M9083/3 Malignant teratoma, intermediate M9084/0 Dermoid cyst, NOS Dermoid, NOS

[Bronchogenic cyst of the tongue].

Bronchogemc cyst is a rare aberration secondary to abnormal detachment of accessory lung buds from the primitive foregut. Through one case of bronchogenic cyst of the tongue, the authors made a review of this exceptional localisation. We report one case of bronchogenic cyst of the tongue treated in the Ibn Sina Hospital. Our patient was a new born admitted in our formation for a voluminous cystic mass ot the tongue, responsible of difficulty for mouth occlusion and feeding The radiology I confirms the liquid nature of this mass. The treatment was surgical, and the diagnosis of bronchogenic cyst was histological. Bronchogenic cysts are generally intrathoracic. The localization in the cervico-facial area is rare, and only 4 cases of bronchogenic cyst of the tongue were described in the literature. The clinical presentation is non specific, depending on the volume of the cyst. Radiology permits to confirm the nature of the mass. The certitude diagnosis is histological: bronchogenic cysts are lined by pseudostratified ciliated columnar epithelium with accessory tissues in the cyst wall, smooth muscle, seromucinous glands and cartilage, simulating a bronchus. Complete surgical excision is the base of the treatment.

Primary Mature Teratoma of the Rectum: A Case Report.

BACKGROUND Teratomas are embryonal neoplasms that contain tissues derived from 1 or more of the 3 germ layers. They commonly are found in the sacrococcygeal-gonadal location, sometimes in midline locations such as the mediastinum, retroperitoneum, and head and neck region. Primary rectal teratomas are extremely rare. Extragonadal teratomas can originate from pluripotent germ cells present in abnormal embryonic rests. CASE REPORT Here, we report a rare case of a primary mature, solid teratoma of the rectum. A 68-year-old woman presented with hematochezia and denied any history of abdominal pain or a change in bowel habits. Colonoscopy revealed a 4-cm pedunculated polyp in the rectum. No hair was present on its surface. The polyp was completely removed by polypectomy. Histologically, the tumor consisted of mature components from all 3 germ layers. Its surface was covered by squamous epithelium with hair follicles and sweat glands. Adipose tissue, blood vessels, bone, and glandular epithelium were present inside the mass. No evidence was found of immature elements or malignant features. CONCLUSIONS When polypoid lesions are found in the rectum, teratoma should be considered in the differential diagnosis. Histopathological confirmation is necessary to diagnose teratoma. Primary rectal teratomas should be distinguished from other neoplastic polyps as well as from local spread of teratomas arising in adjacent organs. These neoplasms are usually mature (benign) but may undergo malignant transformation. Therefore, complete resection is recommended to alleviate symptoms and avoid the risk of malignancy.

[Mature cystic teratoma of the ovary with a small ganglioneuroma].

A case of ganglioneuroma arising within a cystic mature teratoma of the ovary in a 34-year-old woman is reported. Patient underwent right adnexectomy. The ovary was completely replaced by a bilocular cystic lesion, measuring 8 cm in diameter and filled with adipose tissue and pilosebaceous material. Microscopically the cyst was composed by a mature cystic teratoma containing skin with dermal appendages, fatty tissue and bronchial epithelium. Furthermore a nodule (0.5 cm in size) composed of mature ganglion cells, axons and Schwann cells, was identified. Ganglion cells were positive for NSE and synaptophysin, while Schwann cells stained positively with S100 protein and GFAP. To the best of our knowledgment this is the first reported cases of ganglioneuroma arisen within a cystic mature teratoma of the ovary.

Clinicopathological observation of mature teratoma with malignant transformation, a single center retrospective study.

To investigate the clinicopathological features of mature teratoma with malignant transformation. Retrospectively analysis of 1179 cases mature teratoma was done from August 1999 to December 2019 in Institution. 14 cases of mature teratoma with malignant transformation were discussed mainly for the pathological characteristics and clinical manifestations. 4 of them were less than 40 years old. All but one occurred in the ovaries, and the one was in the left anterior mediastinum which was the only male. The clinical manifestations of the patients were atypical. Imaging showed cystic solid mass. Surgery was performed. Polypoid mass, solid nodule and thickened area of cyst wall can be seen on the section of tumor. Pathological results show that there were 5 cases of squamous cell carcinoma, 3 cases of carcinoid, 2 cases of serous carcinoma and 2 cases of thyroid papillary carcinoma, 1 case of carcinosarcoma and 1 case of strumal carcinoid. Two cases of squamous cell carcinoma had pelvic and abdominal metastasis. Immunohistochemistry of case 14 showed that AE1/AE3, CD56, SYN, NSE, PSAP, CDX2 were positive in carcinoid. EMA and CK20 were positive in mucinous glands around carcinoid. Calretinin and inhibin were positive in the mesenchyme adjacent to intestinal mucinous gland. Teratoma with malignant transformation is a rare malignancy, although teratoma is a common germ cell tumor. And it's more common in patients over 40 years, especially those patients who were in menopause. Squamous cell carcinoma is the most common type and prone to metastasis. Strumal carcinoid was well-defined, but as an endocrine tumor, it may cause a series of digestive, respiratory or hormonal disorders. Therefore, the mature teratomas should be removed in time after detection.

[Mature benign teratomas with malignant tumors and malignant monodermal ovarian teratomas. Anatomo-clinical presentation of 10 cases].

The clinicopathologic features are presented of 8 malignant neoplasms occurring in adult teratomas, and of 2 malignant monodermal teratomas found in a continuous series of 1029 cases of ovarian adult teratomas. The age of the patients was 44--76 years. The first 8 observations included 6 epidermoid carcinomas, 1 undifferentiated carcinoma and 1 strumal carcinoid. In the epidermoid carcinomas malignancy was recognized peroperatively only in three instances. One tumor had infiltrated the broad ligament on one side and another the urinary bladder. The neoplasm appeared as a mass which thickened the wall of a typical dermoid cyst. Four patients died of the tumor from 3 months to 1 year after operation; one is alive more than 19 years later and one is lost to follow-up. The crucial factor in prognosis is the integrity of the ovarian capsule. The undifferentiated carcinoma, which had infiltrated the pelvic walls, could only be incompletely resected and caused death within 3 1/2 months. The strumal carcinoid was discovered by chance during laparotomy for a far-advanced adenocarcinoma of the pancreas which led to death within 2 months. The two cases of malignant monodermal teratoma comprised 1 thyroid papillary carcinoma and 1 carcinoid. Both patients are alive more than 8 and 5 years after operation. The carcinoid manifested itself by persistant diarrhea which ceased soon after operation and did not recur subsequently.

A bronchogenic cyst, presenting as a retroperitoneal cystic mass.

Bronchogenic cysts are mostly benign, congenital abnormalities originating from the remnants of the primitive foregut. A retroperitoneal location is rare. Due to the mostly asymptomatic behavior and the historical confusion regarding histology, an exact prevalence is not known. We present here a case report of a retroperitoneal bronchogenic cyst. A literature review was performed for cases of retroperitoneal bronchogenic cysts written in English. Anatomopathological criteria for inclusion were pseudo stratified, ciliated, columnar epithelium together with the presence of at least one of the following: cartilage, smooth muscle or seromucous glands. In addition, the embryology, pathogenesis, radiological, clinical and suggested treatment modalities are reviewed. We report the surgical excision of a retroperitoneal bronchogenic cyst that presented as a non-functioning left adrenal mass. Our review of literature revealed only 62 potential cases of retroperitoneal bronchogenic cysts. After applying the strict anatomopathological criteria, only 30 cases of true retroperitoneal bronchogenic cysts could be identified. Retroperitoneal location of a bronchogenic cyst is rare. Despite the rarity of this pathologic entity, bronchogenic cysts should be considered in the differential diagnosis of retroperitoneal cystic lesions. Only histology can confirm definitive diagnosis. Surgery remains the recommended treatment of choice.

Retroperitoneal bronchogenic cyst: a case report.

Bronchogenic cysts are among developmental disorders of the primitive foregut which are typically found above the diaphragm. Bronchial cysts discovered in the abdominal cavity or retroperitoneum are extremely rare. We present a rare case of a retroperitoneal bronchogenic cyst which was incidentally detected after a wrestling injury in a 23-year-old man who had a negative medical history. Although initial imaging studies suggested an adrenal tumor, histopathological analysis provided a definite diagnosis of bronchogenic cyst. Though rare, bronchogenic cysts must be considered in the differential diagnosis of retroperitoneal cystic lesions. This is the first case of a retroperitoneal bronchogenic cyst reported in Iran.

Teratoma

Regardless of whether fetus in fetu and fetiform teratoma are one entity or two, they are distinct from and not to be confused with ectopic pregnancy. Struma ovarii A struma ovarii (also known as goitre of the ovary or ovarian goiter) is a rare form of mature teratoma that contains mostly thyroid tissue. Epignathus Epignathus is a rare teratoma originating in oropharyngeal area that occurs in utero. It presents with a mass protruding from the mouth at birth. Untreated, breathing is impossible. An EXIT procedure is the recommended initial treatment. Signs and symptoms Teratomas may be found in babies, children, and adults. Teratomas of embryonal origin are most often found in babies at birth, in young children, and, since the advent of ultrasound imaging, in fetuses.

Ovarian cancer

Histologically, embryonal carcinoma appears similar to the embryonic disc, made up of epithelial, anaplastic cells in disorganized sheets, with gland-like spaces and papillary structures. Polyembryoma Polyembryomas, the most immature form of teratoma and very rare ovarian tumors, are histologically characterized by having several embryo-like bodies with structures resembling a germ disk, yolk sac, and amniotic sac. Syncytiotrophoblast giant cells also occur in polyembryomas. Squamous cell carcinoma Primary ovarian squamous cell carcinomas are rare and have a poor prognosis when advanced. More typically, ovarian squamous cell carcinomas are cervical metastases, areas of differentiation in an endometrioid tumor, or derived from a mature teratoma.

[Bronchogenic cyst: a rare cause of a retroperitoneal mass].

Bronchogenic cysts are developmental abnormalities of the primitive foregut which typically occur in the lung. Subdiaphragmatic and, especially, retroperitoneal locations are rare. The histopathological definition consists of the presence of ciliated epithelium together with cartilage or bronchial mucous glands. We report on a 49-year-old patient with the incidental finding of a large cystic mass between the diaphragm and the stomach. Imaging studies suggested an adrenal tumour. Surgical resection and postoperative follow-up were uneventful. Histological examination revealed the surprising diagnosis of a bronchogenic cyst. Bronchogenic cysts must be considered in the differential diagnosis of retroperitoneal cystic lesions. Regardless of being asymptomatic most of the time, surgical resection is recommended to obtain definitive histological diagnosis and avoid future complications.