Chinese Journal of Neurosurgery: An analysis of intracranial primary multiple Rosai-Dorfman disease

Fig.
1 Preoperative enhanced head MRI showed multiple flattened occlusive masses in the left occipital lobe and right frontal lobe, the base was located in the dura, and the surrounding brain tissue was compressed.
coronal position; c.
Sagittal position Fig.
2 Dural and subdural masses are mainly composed of hyperplastic tissue cells, focal lymphocytes and plasma cells, distributed in the form of sheets or nodules, and "protruding motion" (HE×400) can be seen.

The patient is female, 55 years old
.
He was hospitalized for "intermittent headache for 20 years, repeated sudden loss of consciousness and 14 days of limb rigidity", with a feeling of fatigue and low-grade fever
recently.
Systemic examination did not reveal peripheral enlarged lymph nodes, and specialist examination showed no obvious positive neurological signs
.
Complete blood analysis, erythrocyte sedimentation rate, and CRP are generally normal
.
Cranial CT showed flattened and slightly densely opacities on the convex surface of the right frontal lobe.
MRI of the head showed that the left occipital lobe was clearly bordered and the base was located in the dura mater mater, with a size of about 1.
2 cm× 1.
0 cm× 1.
0 cm (Figure 1a), and multiple masses of equal T1 and isoT2 signal sizes were visible in the right frontal lobe, the most about 3.
0 cm× 4.
0 cm×1.
5 cm, and after enhancement, all lesions were significantly uniformly strengthened, there was no meningocaudal sign, and the edema around the multiple masses of the right frontal lobe was severe (Fig.
1b, c).

Preoperative diagnosis is multiple intracranial masses, and meningiomas are possible
.
After the patient was admitted to the hospital, he improved various relevant examinations in the next stage of general anesthesia of tracheal intubation and performed "left occipital transverse sinus mass resection" and "right frontal multiple mass resection", during the operation, it was seen that the left occipital mass base was attached to the paratransverse sinus dura, the transverse sinus was not invaded, the right frontal lobe multiple mass base was also attached to the dura, all lesions were yellow, soft, not rich in blood supply, the mass was tightly adhesed to the occipital lobe and frontal cortex, there was a thin layer of yellow exudate under and around the tumor, and the mass was completely removed.
Yellow exudate is not treated
.

Postoperative pathological report showed multiple irregular masses attached to the dura mater on the left occipital and right forehead, and microscopic examination showed dura mater and subdural masses, mainly composed of hyperplastic tissue cells, focal lymphocytes and plasma cells, distributed in the form of sheets or nodules, and "protruding movement" (Figure 2).

Immunohistochemical staining showed CD68 (histiocyte+), S-100 (histiocyte+), consistent with dural Rosai-Doffman disease
.
The patient's headache was relieved after surgery, there was no nausea and vomiting, and there were no recurrent seizures, and on the third day after surgery, the head MRI with non-contrast scan and enhancement showed total resection of multiple intracranial lesions, and the skull MRI was re-examined 3 months after surgery with non-contrast scan and enhancement and no recurrence
.

discuss

Rosai-Dorfman disease (RDD) is a very rare benign tissue proliferative disorder first reported
by Rosai and Dorfman in 1969.
The disease tends to occur in children and adolescents, mostly located in the cervical lymph nodes, and the skin, salivary glands, orbits, upper respiratory tract and nervous system can be affected outside the nodes, while RDD primary to the central nervous system is rare
in domestic and foreign reports.
RDDs that originate in the skull tend to occur in men aged 40 to 50 years and are mostly solitary
.
This case is a middle-aged and elderly female patient with multiple RDDs originating in the skull, which is clinically very rare
.

At present, the cause of RDD is unknown, and some authors believe that it may be related to viral infection and/or immunodeficiency, and this patient has fatigue and low-grade fever, and no history of immunodeficiency, which cannot exclude the possibility of
viral infection.
Intracranial RDD is usually clinically manifested as headache and seizures, according to the location of the lesion can be manifested as aphasia, cranial nerve dysfunction, progressive vision loss, hemiplegia and other symptoms, a small number of patients can appear fever, fatigue, anemia and other discomfort Some patients can have increased erythrocyte sedimentation rate and elevated CRP, clinically erythrocyte sedimentation rate and CRP changes can assist the diagnosis of RDD, but there is no specificity
.

Most of the intracranial RDD imaging is manifested as a single mass with the dura as the base and clear boundaries, mainly located on the convex surface of the brain, a small part can be located in the sellar area, cavernous sinus, brain parenchyma and slope, local brain tissue is compressed and displaced, peritumoral edema is obvious, tumor enhancement can be seen after obvious uniform strengthening, some people may have meningeal tail sign
.
The imaging of this patient basically meets the above characteristics, but the lesions are frequent and there is no obvious meningeal tail sign is its characteristics
.
Therefore, the presence or absence of meningocaudal signs cannot be used as the basis for distinguishing intracranial RDD from meningioma, and it is difficult to distinguish from meningiomas on imaging, and the diagnosis of RDD can only rely on pathological examination
.
The positive expression of S-100 protein and CD68 in histiocytes is a reliable basis for the diagnosis of RDD, and the diagnosis of this patient is relatively clear
.

Current treatments for intracranial RDDs include surgical resection, radiation therapy, chemotherapy, and hormonal therapy
.
Most of the literature reports that surgical resection is the treatment of choice and can achieve satisfactory results
.
In this case, the patient had multiple RDDs in different parts of the skull through the first stage of total resection, and the headache symptoms of the patient were completely relieved after surgery, and there were no seizures and no symptoms
of neurological deficit.
After surgery, no radiotherapy, chemotherapy and other adjuvant treatments were given, and 3-month reexamination of the head MRI showed that intracranial RDD resection was complete, and no residue or recurrence
was seen.
A small number of literature reports that low-dose radiotherapy and hormonal therapy
may be considered for cases of partial resection, incomplete surgical resection, and recurrence.