November 27, 2022

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Case Study for primary and multifocal intradural extramedullary hair cell astrocytoma

Case Study for primary and multifocal intradural extramedullary hair cell astrocytoma


Case Study for primary and multifocal intradural extramedullary hair cell astrocytoma. Pilocytic astrocytoma (PA) is a common central nervous system (CNS) tumor affecting children, but it is rare in the elderly. It is histologically equivalent to a WHO-grade tumor, usually with clear boundaries and slow growth. Tumors are commonly found in the cerebellum, brainstem, hypothalamus, optic nerve, or intramedullary spinal cord. Surgical removal as much as possible can be considered a cure.

Pilocytic astrocytoma (PA) is a common central nervous system (CNS) tumor affecting children, but it is rare in the elderly. It is histologically equivalent to a WHO-grade tumor, usually with clear boundaries and slow growth. Tumors are commonly found in the cerebellum, brainstem, hypothalamus, optic nerve, or intramedullary spinal cord. Surgical removal as much as possible can be considered a cure.

The dissemination of craniospinal axis of trichomycosis is a rare event, which may occur through the cerebrospinal fluid (CSF) pathway and is its main mechanism. When this happens, the distal tumor can manifest as discrete nodular disease in the intradural extramedullary space, that is, descending metastasis or diffuse pial medullary glioma.

We report here a rare case of multifocal intradural extramedullary hair cell astrocytoma. The location of the tumor suggests that they are descending metastases of the primary tumor, but the image of the entire craniospinal axis shows no other tumor locations. This is a rare manifestation of hair cell astrocytoma. In this report, we discuss this case and review relevant literature, focusing on possible mechanisms.

Patient history

The patient was a 69-year-old woman who presented to the doctor with a worsening cough and pain from chest to back. Chest pain was initially thought to be a symptom of pleurisy and was related to her chronic obstructive pulmonary disease (COPD) worsening. When the treatment of her chronic obstructive pulmonary disease did not improve her symptoms, and considering that the nerve root distribution of her pain was disordered, the doctor performed a spine MRI to explore other possible causes of her pain.

Before the scheduled MRI examination, the patient was diagnosed with myocardial infarction in the emergency department and was sent to the emergency department for hypertension. During the hospitalization, the patient was found to have neck pain, bilateral upper limb pain (C8-T1 skin distribution) and urinary incontinence. Upon examination, the patient’s upper limb flexion and abduction were slightly reduced. The strength of her lower limbs weakened. Bilateral deep tendon reflex of upper limbs is 3+, bilateral deep tendon reflex of lower limbs is 3+, and there is clonus at the ankle joint. She has a bilateral toe extension reaction.

Case Study for primary and multifocal intradural extramedullary hair cell astrocytoma
figure 1. Magnetic resonance imaging of cervical-thoracic intradural extramedullary hair cell astrocytoma. The initial MRI of the cervical and thoracic spine showed that T1 cervical spine posterior contrast agent, b thoracic spine posterior contrast agent, c thoracic spine sagittal T2, multiple enhanced intradural extramedullary tumors in the lower, upper and middle segments of the cervical spine (White arrows indicate tumors). These factors lead to multifocal severe spinal stenosis, the spinal cord is significantly flattened, and the axial position is compared with the T9 level after T1.

 

 

MRI of the entire spine before and after the initial angiography showed extensive, mostly bright-enhanced intradural extramedullary nodules, extending from C5-6 to T11 (Figure 1). Part of the T6 segment nodules only slightly enhanced, and showed obvious enhancement before T1 angiography. The lesions can cause severe central canal stenosis in multiple segments with multiple cervical and thoracic spinal cord compression. The largest mass gathering is from C6 to T4, according to the Associated Press 1.9 cm 1.2 cm and 10.0 cm wide.

An MRI follow-up of the spine was performed 10 days later to determine the patient’s response to steroid treatment. The results showed no significant changes. Due to the patient’s age, intradural extramedullary location, and uniform enhancement seen on MRI, multiple spinal meningiomas are also considered. It is worth noting that the patient has no history of neurofibromatosis or any other neurocutaneous disease.

The patient also had no history of tumors and had never received chemotherapy or radiotherapy before. This patient did have a history of polio, and she recalled being infected when she was about two years old. The patient’s mother was diagnosed with a rapidly growing brain tumor in her 80s, but other than that, the patient’s family history was not significant.

Surgical treatment of pilocytic astrocytoma

For tissue diagnosis, a T7-T8 laminectomy was performed to partially remove the tumor. Once the lesion is determined to be pilocytic astrocytoma, the tumor is removed. The residual tumor is removed in two stages. In the first operation, multilayer laminectomy was performed from T4-T10. After opening the midline of the dura mater, the tumor was found to be light silver attached to the spinal cord (Figure 2).

We performed a meticulous microsurgical resection to separate the tumor from the spinal cord. There is an identifiable plane between the tumor and the spinal cord, which facilitates the safe removal of the tumor, except for certain areas where the tumor appears to be more adherent and may invade the pial coverage of the spinal cord. At these locations, the tumor is removed from the spinal cord, leaving only a very thin layer of tumor. Subsequently, we performed a C4-T3 laminectomy to perform a second resection and removal of the remaining tumor.

Case Study for primary and multifocal intradural extramedullary hair cell astrocytoma
figure 2. Intraoperative image. The intraoperative image (magnified 5 times) shows the tumor (*), the plane is identifiable, and it is easily separated from the normal spinal cord (↑)

 

None of the patients cited in this report were found to have NF1 or any other tumor susceptibility syndrome, which suggests that these tumors may have carcinogenic molecular mechanisms that are more closely related to the occasional pilocytic astrocytoma. On the basis of such a small sample size, no clear treatment recommendations can be made. Our case is a nodular spinal cord tumor with spinal cord compression. Taking into account the patient’s age and the histological characteristics of the tumor’s focal anaplasticity, we decided to choose the combination of radiotherapy and chemotherapy after extremely safe resection.

This is a rare case of an elderly pilocytic astrocytoma found in the extramedullary space in the dura. This finding supports previous research that the glioma originating from this anatomical location may be caused by ectopic glial cells. Genetic research can clarify the development and clinical processes of these tumors.

 

(source:internet, reference only)


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