1 case and literature review of Rosai-Dorfman disease in children withintra-cranial primary disease

Rosai-Dorfman disease (RDD), also known as sinus tissue cell hyperplasia with great lymph node disease (sinus histiocytosis with masive lymphadenopathy, SHML), has a low incidence of a class of unexplained benign tissue cell hyperplasia, first reported in 1965 and was first identified as a class iadeThe incidence of intracranial primary RDD is lower, and rarely reported in literature at home and abroadBecause the imaging performance of the disease has no obvious characteristics, the initial diagnosis is often confused with meningioma, glioma or Langhans cell hyperplasiaIn this paper, the first case of intracranial primary RDD in children admitted to pediatric neurosurgery at Xiangya Hospital of South University is reported, and relevant literature reviews are carried out1Clinical information1.1 General informationchild, male, 12 years old, was admitted to hospital for 2 years, headache, dizziness and 1 month after the discovery of a lump in the lower part of the right forehead-stostleThere is no speciality in the pastPhysical examination: clear mind, normal intellectual development, right forehead -shin scalp can be about 2.5 cm x 2.5 cm bone defect area, the corresponding area can be a throbbing lump, soft toughness, poor mobility;1.2 Routine examinationskull 3D CT examination shows: the right forehead-stoic sand a size of about 10.4 cm x 5.1 cm x 8.6 cm of high density of the lump, surrounded by a large edema band, the middle line of the brain left shiftBone window shows that the tumor has invasive damage to the bone, the rest of the skull and jaw bone multiple bone-dissolving damage (Figure 1A, 1B)Magnetic resonance showed that the tumor strengthened obviously, the epidural had aggression, the tumor "burr" was obvious, suggesting that the soft meninges were damaged, and that the boundary with part of the brain tissue was unclear (Figure 1A)Chest and abdominal CT showed: two-sided tibia bone, two-sided shoulder blade, double-sided collarbone, chest bone, tibia, bi-sided tibia and multiple vertebral body appeared to varying degrees of ossotic damageBone scans of single-photon emission computer body imaging (single-photon computed tomography, SPECT) show increased and decreased metabolism in multiple areas of bone metabolism (Figure 1C) Figure 1 imaging data in children
laboratory examination showed that the red blood cell deposition rate was 76 mm/h, the level of leukocyl-6 was 31.9 pg/mL, all of which were moderately elevated There were no significant abnormalities in liver and kidney function, hematuria routine, alpha fetoprotein (AFP), cancer ouseroantigen (carcinoembryonic antigen, CEA), etc 1.3 Surgical treatment admitted to the hospital for a clear diagnosis, considering the possibility of non-surgical treatment of the child, in the bureau of the three-dimensional directional biopsy, take the skull defect smaller underthe skin 2 cm position small specimen sent to pathological examination Pathological results suggest that Rosai-Dorfman disease, accompanied by more lymphocytes, plasma cells soaked, visible tissue cells devourly lymphocytes After the biopsy 1 month after the child's conscious headache symptoms aggravated, the review of magnetic resonance showed that the tumor volume has increased, then performed a craniofacial tumor excision Take the right forehead -the large question mark flap into the road, see the lesions are located at the junction of the forehead-stoic, cross-front cranial nest, the size is about 10.1 cm x 5.2 cm x 9.3 cm, the quality is soft and strong, blood transport rich, the relevant area of the skull thinning combined with part of the bone defect Tumor and epidural is closely related, the main body is located under the epidural, invasion and soft meninges, and part of the brain tissue boundary is not clear, the relevant region of the epidural and the soft meninges are incomplete The tumor was completely excisioned under the microscope and sent for pathological examination, taking into account the benign nature of the pathology and the appearance and function of the child, the bone flap was preserved 1.4 Pathological Results the final pathological results of the child after surgery (Figure 2) suggest edgtorosai-Dorfman disease in the right frontal temporal lobe The chemical results of immunohistosis are as follows: positive for leukocyte differentiation antigen 163 (cluster of differentiation 163, CD163); CD68 negative; CD1a negative; CD207 negative; CD117 negative; soluble protein-100 (soluble protein-100, S-100) positive; cell proliferation antigen Ki-67 is about 10% positive; calcium adhesion Protein E (E-cadherin) negative; glial fibrillary fibrosprotein ,GFAP) negative; immunoglobulin G (immunoglobulin G, IgG) partially positive; IgG4 9 positive cells per high-spectroscopy field; platelet-derived growth factor alpha (plate-derived growth factor alpha, PDGFR) positive 2 tumor slicing pathology and immunohistochemical results 1.5 follow-up treatment children after surgery, subcutaneous fluid and repeated fever, repeated extraction of fluid pressure bandaged scalp can not be attached to the subcutaneous tube drainage, 1 week after the introduction of flow reduced, the child's subcutaneous fluid disappeared, removed the drainage tube did not recur Postoperative addition of hormone therapy (Ponisson 40 mg oral, 1 time per day) and chemical medication (ampicillin s6-pyridoxine and strong pine solution combined with dasatinib) The child's current chemical drug treatment ended, the follow-up condition was stable, followed by 8 months prognosis is good, the review did not show signs of tumor recurrence (Figure 1D) 2 Discussion
The typical manifestation of RDD is painless, symmetrical double-sided neck lymph nodes swollen, accompanied by fever and elevated white blood cells, often occurring in children and adolescents under 20 years of age However, RDD is a kind of benign tissue cell hyperplasia disease that can occur in various systems throughout the body, the proportion of RDD that occurs outside the lymphatic system is greater than 40% of all RDD, so the clinical manifestations of the disease are diverse, and THE RDD that occurs outside the lymphatic system shows atypical symptoms of the corresponding system intracranial RDD is often characterized by headache, vomiting and other cranial high-pressure symptoms, as well as a variety of occupancy effects, such as tumor-affected saddle areas may cause vision impairment and endocrine dysfunction, the ventricular system may lead to obstructive hydrocephalus, and even cause coma death at present, the initial screening and diagnosis of intracranial RDD still lack specific methods, imaging and intracranial meningioma, eosinophilic granuloma and other diseases are easy to be confused At present, some scholars have proposed magnetic resonance dispersion weighted imaging (diffusion weighted imaging, DWI) and magnetic resonance epigenetic diffusion coefficient (apparent diffusion diffusion coefficient( coefficient, aDC) to identify intracranial RDD and meningioma, but limited by sensitivity and technical level and other factors, there are still some difficulties in implementation pathology examination as the diagnostic gold standard for the disease, RDD under electron microscope histology manifests itself as different forms of tissue cells, plasma cells and lymphocytes, forming a "light and dark structure." In immunohistosis, the diagnostic specificity and sensitivity of RDD were higher in both S-100 and CD68 Surgical treatment is the most direct and effective method for treating intracranial RDD but in this case, because of the large size of the tumor, and the damage to the skull bone is more serious, the initial consideration is that the possibility of langerhans cell hyperplasia, blood system disease or invasive meningioma, etc is high, there is a possibility of non-surgical treatment, and direct surgical treatment needs to consider the scope of tumor removal, the degree of retention of nerve function, whether the skull should be discarded and so on The examination of the first stereotactic living tissue can help to judge its pathological properties and provide relevant basis for further treatment According to domestic and foreign research reports, in addition to surgical treatment, complementary treatment (radiation therapy, chemotherapy, hormone therapy) for intracranial RDD may also play a certain control and treatment effect, some cases of steroid treatment effect is significant Although the child in this case had done the tumor all-cut, but its tired bone and epidural range is wide, it is difficult to completely remove Therefore, chemical therapy and hormone therapy are still necessary after surgery at present, some scholars believe that RDD may be a class of IgG immunorelated diseases, reflected in hormone therapy for individual differences In this study, the patients with a high proportion of IgG4 positive cells in lymphocytes had a better steroid treatment effect than those with low proportion of IgG4-positive cells, and the therapeutic effect was positively correlated with the proportion of positive cells This also provides a reference value for hormone therapy for intracranial RDD , intracranial RDD in children is a type of benign disease that can be combined with multidisciplinary treatment, but the treatment of the disease is still lack of relevant standardized treatment guidelines, surgical treatment is the most direct and effective treatment, radiation, chemical and hormone therapy may be effective in some children At the same time, the authors believe that for children with tumors that are covered by a wider range of bone and epidurals, chemical and hormone therapy should also be considered to consolidate the efficacy For children with heavy illness, large tumor and progression trend (e.g this case), after clear diagnosis to surgery-based, combined radiation, chemical drugs and hormone therapy as the current treatment trend;