Diagnosis and treatment of acute hemorrhagic pineal region tumors

Pineal region tumors are clinically rare, for adults, these tumors account for about 0.5%-1% (Europe and the United States) and 3.2% (Asian countries) of intracranial tumors; while for adolescents, the incidence of pineal region tumors increases significantly, accounting for about 3%-11% of intracranial occupations in adolescents [1]. Although the treatment strategy of pineal region tumors is still controversial, with the rapid development of modern neurosurgery in many fields, such as neuroanesthesia, microsurgery and postoperative management, neurosurgeons have gradually made striving for total surgical resection as the primary treatment principle, and the mortality rate of total surgical resection has been reduced to about 5% [2]. Despite this, how to choose the timing and surgical approach for patients with acute stroke from pineal region tumors is rarely reported in the literature. Case 1, 41-year-old male, was first admitted to the hospital on December 19, 2008, with a headache for 8 days. After admission, a cranial MRI showed a tumor in the pineal region and obstructive hydrocephalus (Figure 1A, Figure 1B). Ventriculoperitoneal shunt was performed under general anesthesia on 2008-12-24, and postoperative follow-up cranial CT suggested relief of obstructive hydrocephalus. On 2009-01-16, the patient was discharged from the hospital and underwent gamma knife treatment, and then he was lost to the hospital. 2009-07-13, the patient was admitted to the hospital again as an emergency because of “sudden unconsciousness for 4 hours”, and at that time, he was in a deep coma, the diameter of the pupil on the right side was 4 mm, and the light reflex disappeared; the pupil on the left side was 2 mm, and light reflex existed, and he did not cooperate with the muscle examination, and he had high muscle tension in the left side, and high tone in the limbs. The left limb was uncooperative in muscle strength examination, the left limb had high muscle tone, and the right limb was flexed after tingling. Emergency cranial CT suggested a tumor stroke in the pineal region (Figure 1C, Figure 1D). Emergency Poppen approach pineal region tumor resection was performed, and the tumor was seen to be about 3cm×3cm in size, with rich blood supply, soft texture, and obvious adhesion with the surrounding tissues, and the tumor was resected in pieces, and the hematoma was about 25 ml, which was to the right, and compressed the right thalamus to the anterior, and it was removed during the operation. In the 2nd postoperative day, the patient’s consciousness turned clear, the muscle strength of the left limb was grade IV, and the muscle strength of the right limb was grade V. The postoperative pathology suggested that it was pineoblastoma. After the head incision was removed, the patient was transferred to the radiotherapy department, and is now under further follow-up, with no tumor recurrence. Case 2, male, 15 years old, was admitted to the hospital with “headache with nausea and vomiting for 10 days”. At the beginning of the disease, the patient felt dizziness, accompanied by cough, and consulted the local hospital with “cold”, and was given antiviral treatment without improvement, followed by nausea and vomiting and other symptoms, and then investigated the cranial CT suggesting “occupying lesion in pineal region and obstructive hydrocephalus”, and was given dehydration and other treatment. “He was given dehydration and other treatments, but his symptoms did not improve. On the day of admission, he had convulsions of the limbs and clenched teeth, which were considered as seizures, and he was transferred to our hospital after being given antiepileptic treatment. At the time of admission, the patient was unconscious, bilateral pupils were equal in size and round, with a diameter of 3mm, sensitive to light reflex, uncooperative in limb muscle strength examination, movable, normal limb muscle tone, and negative pathological reflexes. After admission, cranial MRI (Figure 2A, Figure 2B) showed a space-occupying lesion in the pineal region, with T1-weighted low signal and T2-weighted high signal, which could be uniformly strengthened. Aggressive preoperative preparations were made, and a limited surgical treatment was proposed. On the 2nd day after admission, the patient was suddenly in a deep coma, all physiological and pathological reflexes disappeared, basic vital signs were still stable, and an urgent cranial CT suggested that the peritumor space was narrowed, and the periphery was hyperdense, with obstructive hydrocephalus (Figure 2C). Unilateral lateral ventricular external drainage was performed urgently, and the cerebrospinal fluid was seen to have a high pressure and was in the form of a jet. After the operation, the patient was continuously observed for 4 hours, and there was no improvement in the patient’s conscious state, and the muscle strength of the limbs was still at grade 0. All the physiological reflexes and pathological reflexes were not induced, and the patient was in an endangered state. Following the application of Poppen approach for tumor resection in the pineal region, a small amount of occipital lobe tissue was resected during the operation due to high intracranial pressure, and after opening the occipital horn, normal brain pulsations were seen. The tumor of pineal region was resected according to the Poppen approach. During the operation, the tumor was seen to be about 4cmX3cm in size, rich in blood supply, hard and tough, with uneven distribution of texture, and the tumor was completely resected in pieces during the operation, and the outlet of the third ventricle and the midbrain conduit were revealed. During the operation, the tumor was seen to stroke, and the hematoma compressed the midbrain in an anterior direction, so the hematoma was removed. Postoperative follow-up CT (Figure 2D) showed complete resection of the tumor, relief of hydrocephalus, and no delayed hematoma in the operative field. Postoperative pathology suggested a germ cell tumor in the pineal region (immature teratoma grade II, partially embryonal carcinoma), and immunohistochemistry suggested scattered positivity for CK, EMA, SYN, GFAP, CEA, S-100, CD56, and Ki-67, and negativity for a-AFP, Desmin, and SMA. After the operation, the patient’s consciousness turned clear and there was no obvious obstacle in limb movement. After the head was dismantled, the patient was transferred to the Radiotherapy Department for radiation therapy, and is now under further follow-up, and no tumor recurrence has been seen. 2.Discussion: Reviewing the treatment history of pineal region tumor, we know that due to the deep location of the tumor in this region, it was often considered as a “forbidden area for surgery” by some scholars in the early stage of the development of neurosurgery, and was often described as “disastrous” and “incompetent” by Dandy in the beginning of the 20th century when mentioning the tumor of pineal region. Dandy, in the early 20th century, described pineal region tumors as “catastrophic” and “impotent” [3]; Cushing called them “tumors that can never be fully revealed” [4]. Early neurosurgeons were committed to total and subtotal resection of pineal region tumors, but patient mortality rates were consistently higher than 50% until the early 1970s, when radiotherapy and hydrocephalus shunting were introduced as the treatment of choice for pineal region tumors, with a concomitant 5-year survival rate of 58%-70% [5]. This view was maintained for about 10 years, and stereotactic biopsy was gradually added to the surgical strategy due to radiological brain damage and the presence of a subset of tumors that were insensitive to radiotherapy; combining the pathological findings of the biopsy, and then determining whether to perform radiotherapy, became the mainstay of treatment in the late 20th century [6]. Currently, although evidence-based treatment guidelines for pineal region tumors are still unpublished and the debate in this field is still intense, with the development of modern neurosurgery, especially the application of minimally invasive neurosurgery and surgical microscopy, neurosurgeons are gradually adopting the surgical total resection of tumors combined with postoperative radiotherapy as the preferred treatment for pineal region tumors. For the treatment of acute stroke in pineal region tumors, there are rare reports in the literature, and the two cases in our group were treated with emergency surgical craniotomy and total resection of the tumor, and the patients obtained a good prognosis, and the author believes that, for acute stroke in pineal region tumors, decisive measures should be taken to avoid missing the best time for surgery. Acute hemorrhage of tumor occurs due to internal and external factors during the growth of brain tumor, which manifests itself as acute intracranial pressure increase, resembling a stroke attack, and is called brain tumor stroke [7]. Brain tumor stroke is one of the serious complications of brain tumors, which is not uncommon in clinical practice, and is more common in metastatic tumors, gliomas, and pituitary tumors. Literature has reported that brain tumor hemorrhage accounts for about 1%-10% of all intracranial hemorrhages during the same period, and severe intracranial hemorrhage originating from tumors accounts for at least 10% [8]. The causes of brain tumor stroke are multiple [9]: the intrinsic factors of brain tumor stroke are mostly vascular factors, such as the tumor is often a pathological blood vessel, such as thin-walled, twisted, lumen dilation and distribution such as reticulation, containing many blood sinusoids, reflux veins are blocked by tumor cells, etc., which is prone to hemorrhagic; at the same time, in the process of tumor growth, the wall of the newborn tumor blood vessel is brittle, which can be manifested as aneurysm-like dilatation and lead to rupture Hemorrhage. Extrinsic factors are tumor infiltration, compression of adjacent tissue structures caused by degeneration, necrosis so that the blood vessels are stretched without support; or tumor compression of adjacent reflux vessels resulting in stagnation of local blood flow and increased pressure; there are also reports that tumors located in the ventricles of the brain and subarachnoid space are prone to rupture and hemorrhage [9], and hemorrhage-inducing factors may be the result of head trauma, exposure of the head to sunlight for too long, and radiotherapy, etc. [10]; and there are reports that stroke is associated with the overexpression of tumors. found that stroke is associated with tumor overexpression of vascular endothelial factor, which makes it promote tumor angiogenesis, but neovascularization tends to be underdeveloped, with thin walls and high permeability, which makes it easy to rupture and bleed [11]. In our case 1, there was a clear history of radiotherapy, which may be the causative factor of tumor stroke, while other causes may be related to the mechanical compression infiltration caused by tumor growth and pathophysiological changes resulting in the alteration of the blood vessels themselves or the blood flow status, together with the intrinsic factors of the tumor, which together lead to the occurrence of brain tumor stroke. The onset of both cases was characterized by impaired consciousness, and the reason for this was that case 1 was consistent with the pathophysiological changes caused by brain hernia, which was consistent with the large amount of hemorrhage in this case and the obvious stroke; case 2 did not have the typical manifestations of brain hernia when the consciousness was suddenly altered, and no obvious signs of stroke were found in the emergency CT examination, and only obstructive hydrocephalus was seen, and the treatment of external drainage was adopted in an attempt to improve the consciousness by relieving the intracranial high pressure, and the patient was kept in observation for 4 hours without seeing any results. The patient was kept under observation for 4 hours without success. After reviewing the history of recurrent epileptic seizures before the onset of the disease and carefully comparing the CT films of the patient examined before admission and before the sudden deterioration of the disease, we considered that the sudden deterioration of the disease might be due to the tumor having an acute stroke, which caused direct compression of the brainstem reticularis superior activation system and the cerebral peduncles. This was confirmed intraoperatively. There is no uniform guideline treatment plan for pineal region tumors due to the diverse pathologic diagnoses, and all treatment strategies are based on correct diagnosis.Currently, direct microsurgery has been shown to lead to a better prognosis for patients, but with further development of neurosurgical techniques, stereotactic radiotherapy and neuroendoscopy, it may further change the means of treatment for pineal region tumors, and the author It is believed that for non-acute patients, comprehensive treatment by multiple means will be the best choice for these patients; and for patients with acute stroke from pineal region tumors, emergency surgery should be performed, striving for the first time to completely excise the tumor and open the cerebrospinal fluid circulation pathway. It is worth mentioning the management of the second patient, in which lessons should be learned from the case when the condition and the imaging examination are not obviously consistent, so as not to miss the best time for successful resuscitation.