A 29-year-old male patient presented with right side weakness and left side numbness that occurred just after a car accident. Upon physical examination, there was no abnormal finding, except for tenderness in the upper cervical area. Routine laboratory tests demonstrated normal results. Motor weakness of right side extremity was disclosed as 1/5 for arm and 2/5 for leg with slightly hyperactivity of deep tendon reflex. Sensory examination showed significantly decreased pain, temperature, vibration, and discriminatory touch sensations below the left C2 dermatome. Babinski's reflex and Hoffman's sign were present on the left side. Based on the symptoms and neurological findings, the assumptive diagnosis of Brown-Sequard syndrome was made. The patient had no symptoms or past medical history related to a cervical spine lesion before the trauma. Cervical plain films revealed straightening of the cervical spine with loss of normal lordosis and widening between the posterior arch of the atlas and spinous process of the axis. A computed tomography (CT) scan of the upper cervical spine revealed a large round mass which measured 25×18×24 mm with relatively homogeneous strong enhancement and was located between the C1 and C2 interspinous space. In addition, the CT scan showed cortical osseous erosions at the lower C1 posterior arc and upper C2 spinous process. Cervical magnetic resonance images (MRI) revealed an epidural dumbbell-shaped mass traversing the C1 to C2 interspinous space, compressing the surrounding structures (). The spinal cord was compressed by the lesion which was isointense on T1-weighted image (WI) and mixed hypointense on T2WI with homogenous strong enhanced after gadolinium administration. Radiologic analysis indicated possible lymphoma, meningioma, schwannoma or other neuronal tumors.\nThe patient underwent a surgery immediately for decompression of the spinal cord. A total tumor resection was performed with only a partial laminectomy from the lower half of the C1 to upper half of C2 without any instrumentation and access to the tumor. After partial lamicectomy, an extradural and yellowish mass was exposed with well-demarcated from the surrounding structures but adhesive at the epidural area. Under microscopy, the tumor was delicately dissected, and en bloc removal was achieved (). The mass was ovoid, soft like gelatin, avascular, and well-encapsulated, measuring 25 mm at the largest diameter, and was adherented to the outer layer of the dura mater. And, there was no root invasion. On the basis of the clinical and operative features, we thought that the lesion originated from the outer aspect of the dura mater. Upon sectioning, the mass was particularly yellow and white with cystic components ().\nAfter surgery, the patient's right hemiparesis fully recovered in 2 weeks. However, a slightly tingling numbness on his left arm remained and was controlled through medication in the outpatient clinic for 4 months. Currently, the patient has no symptoms without instability of cervical spine. Cervical MRI with gadolinium enhancement at 2 years after surgery demonstrated no recurrent lesion ().\nHistological examination of the tumor uncovered focal aggregation cells with ample, clear, and foamy cytoplasm in a background of loose, fibrous stroma of xanthic appearance. Large, round cells had irregular vesicular nuclei which were regular and small with condensed chromatin (). The specimen was underwent to immunohistochemical testing, yielding strongly positive for CD 68 and lysozyme stain, which confirmed that the large foam cells were histiocytes. The test with CD-1a was negative, thus ruling out Langerhans' cell histiocytosis. GFAP stain and S-100 protein were negative. Thus, a diagnosis of xanthogranuloma was established.
The second child (Fig. ) of a 29-year-old woman was spontaneously born at 39 + 4 weeks of gestation after an unremarkable pregnancy. Birth weight was 3510 g (50th percentile), length 55 cm (75th percentile) and head circumference 33.5 cm (20th percentile). Apgar scores were 9/10/10. At the age of 3 months the girl was seen by a general pediatrician and consecutively referred to our hospital because of a recently developed mass on the left temple. The subcutaneous swelling was about 2 cm in diameter, non-moveable, not reddish or overheated and not painful. Furthermore, the mother reported recurrent fever spikes up to 38.5 °C without signs of inflammation for about 4 weeks. Defecation and drinking habits were adequate, vomiting was denied. However, a weight loss of 200 g within 3 weeks was obvious. In addition to a pale skin color and three pinhead-large livid subcutaneous lesions located on the trunk and the lower extremities, there was a left-sided rib hump situated at the level of Th6 to Th10; a secondary finding was oral candidiasis. Laboratory values on admission showed: hemoglobin 85 g/l, hematocrit 0.24 L/l, thrombocytes 380 G/l, lactate dehydrogenase 308 U/l, alpha-1-fetoprotein 225.6 ng/ml, beta-human chorionic gonadotropin < 1 mU/ml, c-reactive protein 10.13 mg/dl, interleukin-6 45.8 pg/ml and procalcitonin 0.31 ng/ml. To define the extent of disease, whole-body magnetic resonance imaging (MRI) (Fig. ) was performed. An intraosseous soft tissue lesion in the left sphenoid bone (diameter 18 × 20 mm), a big paravertebral thoracic tumor conglomerate (diameter 85 × 59 mm), multiple papules to nodules in the liver (7 mm), in both kidneys (6 mm) and lungs (3 × 4.3 mm) and in the pancreatic head (3.5 mm), as well as cutaneous (5 mm) and intraosseous lesions were found. A vertebra plana of Th9, together with infiltration of the adjacent Th8 and Th10, resulting in a kinking of the spinal column compromising the spinal canal and obliteration of nerve roots by soft tissue tumor mass was seen. Due to the lesion in the skull and the vertebra plana, Langerhans cell histiocytosis was one of the primary differential diagnoses. But the histology of one cutaneous lesion of the trunk did not confirm this diagnosis. Rapid deterioration with paraplegia prompted us to administer immunosuppressive treatment immediately. Based on the presumed diagnosis of a neoplasia of the Ewing / PNET group the patient was initially treated according to the Euro-Ewing protocol. After the third biopsy and histological examination two independent pathology centers confirmed the diagnosis of xanthosiderohistiocytosis, which is not well-defined and is regarded as a morphologic variant of xanthoma disseminatum – a type that most often occurs in adult patients with monoclonal gammopathy (Fig. ). In keeping with the established diagnosis, Langerhans cell histiocytosis-based chemotherapy treatment was administered. Following the arm for the high-risk group, the chemotherapy agents included prednisone, vinblastine, 6-mercaptopurine and methotrexate. With this therapy the primary tumor mass decreased. Clinical and radiologic examinations at the age of 3 years show partial remission after 1 year maintenance chemotherapy with puri-nethol and methotrexate.