A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up.
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.