Huge Sellar Plasmacytoma as Differential Diagnosis of Invasive Pituitary Adenoma: A Case Report

Sellar plasmacytomas are rare tumors arising from plasma cells. They are often misdiagnosed as adenomas. We report the case of a 63-year-old woman with headache, cranial nerve III palsy and decreased visual acuity. Imaging revealed an extensive lesion centered on the clivus, extending to the cavernous sinus bilaterally and into the sphenoid sinus. The hormonal tests were compatible with panhypopituitarism and mild hyperprolactinemia. The first hypothesis was invasive pituitary adenoma. Partial resection was achieved, and the immunohistochemical evaluation was compatible with plasmacytoma. After a few weeks, she developed lumbar and hip pain, and the imaging confirming osteolytic lesions. The final diagnosis was multiple myeloma.

The stability of outcomes of cancellous autografts with acellular membrane for extended osteochondritis of the knee. Outcomes for 22 AMIC® grafts at an average follow-up of 4 years

Objectives: Loss of osteochondral substances resulting from osteochondritis dissecans (OCD) of the knee are arthrogenic in the long term. When they exceed 2 cm2, the Autologous Matrix Induced Chondrogenesis (AMIC®) technique is one of the methods used in France. The objective of this study was to evaluate the medium-term results of the AMIC® technique. Methods: This was a consecutive, prospective, single-center, single-operator series of 22 patients (13 men, 9 women, mean age 28 years (15-51)) treated by AMIC® (spongy bone graft + sutured collagen membrane) between September 2011 and November 2016. Previous surgery had been performed on 17 patients. According to the ICRS classification, the lesion was stage IV in 21 cases and stage III in 1 case. The sites were: condylar: 19 cases, patellar: 2 cases and trochlear: 1 case. The mean surface area was 3.6 cm2 (2-8) and the depth was 0.5 mm (0.4-0.8). All the patients were functionally assessed by an independent examiner using validated functional scores for these indications (KOOS, subjective IKDC). Student’s T tests were used. Results: At a mean follow-up of 4 years (minimum 2 years) all but 2 patients had significantly improved (In preop. IKDC: 44±14 and KOOS: 56±17). In these 2 cases, the postoperative scores remained unchanged over the years: one patient had had several surgeries before the graft and a 51-year-old female patient had an extensive lesion of 6.9 cm2. The mean IKDC and KOOS scores were 73±18 and 78±15 and then 77±16 and 81±14 respectively, at 1 and 4 years (p> 0.05 for IKDC and KOOS). Regrettably there were 2 complications: 1 arthrolysis 1 year after surgery and 1 algodystrophy. Conclusion: Few techniques are available in France for extensive symptomatic osteochondral lesions. AMIC® is a reliable, one-step, reproducible, inexpensive technique for loss of substance due to OCD with stable results as of 1 year after surgery.

Posterior reversible encephalopathy syndrome with spinal cord involvement as the first presentation of lupus nephritis

A 23-year-old woman was admitted to the emergency department with the history of headache, serial seizures, and decreased the level of consciousness from a week before. At admission, blood pressure was 230/170 mmHg, and creatinine level was 7.6 mg/dl. Initial brain and cervical magnetic resonance imaging (MRI) revealed hyperintense lesions on fluid-attenuated inversion recovery (FLAIR) in bilateral occipital lobes and a longitudinally extensive lesion in the spinal cord (Figure 1, A-C). In the laboratory investigations, the level of anti-double stranded DNA was 45 IU/ml (normal < 10 IU/ml) and anti-nuclear antibody titer was high (> 1/160). Moreover, in renal biopsy, lupus nephritis was reported. Two weeks later, after hypertension treatment, the hyperintense signals wholly disappeared (Figure 1, D-F). Figure 1. Axial fluid-attenuated inversion recovery (FLAIR) brain magnetic resonance imaging (MRI) indicating hypersignal lesions in parieto-occipital areas in favor of posterior reversible encephalopathy syndrome (PRES) at admission (A, B); sagittal T2 cervical MRI demonstrating a longitudinally extensive lesion in the spinal cord at admission (C); two weeks later, the hyperintense signals had completely disappeared on the brain and spinal cord MRIs (D-F).

Mesenchymal chondrosarcoma of maxilla in paediatric patient

Chondrosarcoma (CS) is a malignant tumour of long and flat bone characterised by the formation of cartilage. Mesenchymal chondrosarcoma (MCS) is a rare subtype of CS that is more aggressive and may lead to erroneous diagnosis in a limited biopsy. The diagnosis is mainly based on the histopathological appearance of biphasic pattern of undifferentiated small round cells separated by islands of well-differentiated hyaline cartilage. We report a case of 13-year-old boy who initially presented with gum swelling and the biopsy result suggested a benign fibrous lesion. Following an extensive lesion shown in radiologic findings, the tumour excision was done and finally was diagnosed as an MCS of the maxilla. The patient was given postoperative chemotherapy (EURO-EWING 99 regimen), and now on regular follow-up for monitoring of local recurrence or tumour metastasis.

Clinical features of demyelinating optic neuritis with seropositive myelin oligodendrocyte glycoprotein antibody in Chinese patients

Background/aimsTo investigate the clinical features of Chinese patients with seropositive myelin oligodendrocyte glycoprotein antibody (MOG-Ab) optic neuritis (ON) and patients with seropositive aquaporin-4 antibody (AQP4-Ab) ON.MethodsIn this retrospective observational study, sera from patients with demyelinating ON were tested for MOG-Ab and AQP4-Ab with a cell-based assay. Clinical characteristics were compared between MOG-Ab-related ON (MOG-ON) and AQP4-Ab-related ON (AQP4-ON), including visual performances, serum autoantibodies and features on MRI.ResultsA total of 109 affected eyes from 65 patients with demyelinating ON (20 MOG-ON and 45 AQP4-ON) were included. The onset age of MOG-ON was younger than AQP4-ON (MOG-ON: 20.2±17.4 years old, AQP4-ON: 35.6±15.7 years old, P=0.001). Onset severity was not different between these two groups (P=0.112), but patients with MOG-ON showed better visual outcomes (P=0.004). Half of the MOG-ON had a relapsing disease course. Nineteen per cent of patients with AQP4-ON presented coexisting autoimmune disorders, but there were no coexisting autoimmune disorders among patients with MOG-ON. Optic nerve head swelling was more prevalent in patients with MOG-ON (P<0.01). Retrobulbar segment involvement of the optic nerve were more common in patients with MOG-ON according to our MRI findings (P<0.01). Patients with MOG-ON showed longitudinally extensive lesion in 30% and chiasm and optic tract involvement in 5%.ConclusionsMOG-ON is not rare in Chinese demyelinating patients. It underwent a severe vision loss at onset but had relatively better visual recovery than patients with AQP4-ON. MOG-ON might have an unique pathogenesis different from AQP4-ON.

A Rare Case of Extensive Lichen Striatus

Lichen striatus is a rare dermatitis characterized by lichenoid papules arranged in a linear band along the Blaschko’s line. Extremities are commonly affected sites. Sometimes, abdomen, buttocks and thighs may also be involved in a single extensive lesion. But, multiple lesions involving many Blaschko’s lines are rare with only few publications till now. Hence, we are reporting a case of 14-year-old male presented with lichen striatus over left upper and lower extremities as well as left side of trunk.Nepal Journal of Dermatology, Venereology & Leprology, Vol.14(1) 2016, pp.48-50