Fibro-osseous pseudotumor of the digit: Case report and surgical experience with extensive digital lesion abutting on neurovascular bundles.

BACKGROUND: Fibro-osseous pseudotumor (FOPD) of the digit is a rare benign lesion of subcutaneous tissue characterized by fibroblastic proliferation and osteoid formation. Herein, we present a case of massive FOPD lesion in the base of ring finger with extensive involvement of the neurovascular bundles with challenging surgical approach. CASE DESCRIPTION: A 27-year old female patient, presented with 7-months history of a progressively enlarging mass on her left hand. Upon assessment, the mass was located over the proximal phalanx of the left ring finger with extensive involvement of the 4th web space. Her neurovascular examination was normal. Radiological investigations showed partial involvement of the radial sided bundle together with complete involvement of the ulnar sided neurovascular bundle. The patient was bothered by the mass being painful with overlying skin ulceration. She was taken afterwards to the operating room where the mass was dissected freely from those bundles while preserving the radial and ulnar structures. The resected margins were however, positive for residual lesions due to the extensive nature of the mass. The patient was informed about the need for close follow-ups for both clinical and radiological signs of lesion recurrence pending early surgical intervention. CONCLUSION: FOPD although benign, a soft tissue osteosarcoma is one of the differential diagnosis. Meticulous attention to the clinical, pathological and histological features of FOPD is required. Early diagnosis and treatment of FOPD is very crucial in optimizing the overall outcome. Pre-operative planning with various radiological modalities was of great help anticipating the surgical course.

The Use of Recombinant nAG Protein in Spinal Cord Crush Injury in a Rat Model.

OBJECTIVE: To evaluate the therapeutic properties of nAG protein during the recovery following acute spinal cord injuries in the rat. STUDY DESIGN: An experimental study. PLACE AND DURATION OF STUDY: King Saud University, Riyadh, Saudi Arabia, from September 2014 to September 2015. METHODOLOGY: Eight rats were studied (4 control rats and 4 experimental rats; and hence 50% were controls and 50% were experimental). All rats were subjected to an acute spinal cord injury using the aneurysmal clip injury model. Immediately after the injury, a single intra-dural injection of either normal saline (in the control group) or the nAG protein (in the experimental group) was done. Assessment of both groups was done over a 6-week period with regard to weight maintenance, motor recovery scores, MRI and histopathology of the injury site. RESULTS: Weight maintenance was seen in the experimental and not in the control rats. Starting at 3 weeks after injury, the motor recovery was significantly (p<0.05) better in the experimental group. MRI assessment at 6 weeks showed better maintenance of cord continuity and less fluid accumulation at the injury site in the nAG-treated group. Just proximal to the injury site, there was less gliosis in the experimental group compared to the control group. At the crush injury site, there was less tissue architecture distortion, less vacuole formation, and less granulation tissue formation in the experimental group. CONCLUSION: The local injection nAG protein enhances neuro-restoration, reduces gliosis, and reduces vacuole/ granulation tissue formation following acute spinal cord crush injury in the rat aneurysmal clip animal model.

Two novel homozygous missense mutations in the GDF5 gene cause brachydactyly type C.

Mutations of the GDF5 gene cause a variable phenotype including brachydactyly type C. A review of the literature showed that it is caused either by heterozygous frameshift mutations within the prodomain or heterozygous missense/nonsense mutations within the active domain. Only a single patient with a homozygous mutation (c.517A > G, which predicts p. Met173Val) has been reported in this disorder. In this paper, we report two children with novel homozygous missense mutations in the GDF5 gene associated with brachydactyly type C: one mutation was within the region coding for the prodomain (c.608C > A, which predicts p.Thr203Asn) and the other was within the region coding for the active domain (c.1456 G > A, which predicts p.Val486Met). The genotype-phenotype correlations in the mutational spectrum of the GDF5 gene are discussed.

A solitary fibrous tumor of the upper limb.

Solitary fibrous tumors (SFT) of the upper limb are extremely rare, and we report this tumor in the arm of a 30-year-old male. He is presented with a 22 cm painless mass. Complete surgical excision was performed. The histological diagnosis of SFT was based on the presence of ectatic blood vessels and positive staining for CD34 and vimentin. He remains disease-free at the 3-year follow-up interval. The report aims to increase the awareness of the criteria for the histological diagnosis of SFT, as well as the principles of their surgical excision and follow-up.