Factors Associated with Disease Stabilization of Desmoid-Type Fibromatosis.

BACKGROUND: Spontaneous disease stabilization of desmoid-type fibromatosis (DF) has been demonstrated in many reports, and the watchful waiting approach without any frontline treatment is becoming popular as an initial management strategy. In this study, we aimed to assess the disease stabilization rate and identify predictive factors for disease stabilization of DF in patients with conservative treatment. METHODS: We reviewed 76 patients with sporadic extra-abdominal DF who were managed with frontline conservative treatment in our institute. The minimum follow-up was 12 months. Stabilization was defined as radiological evidence of no change or continuous decrease in size of the tumor for six months or more. The primary endpoint was stabilization of DF. Possible patient-, disease-, and treatment-related factors predictive of disease stabilization were analyzed with multivariate analysis. RESULTS: At final follow-up, 54 of the 76 tumors (71%) were stable, and mean time to stabilization was 30.4 months (range, 7 to 112 months). On Kaplan-Meier survival analysis, the spontaneous stabilization rate was 25.4% at one year, 52.7% at two years, and 70.9% at three years. The mean time to spontaneous stabilization was longer in patients with ≤ 40 years of age (p = 0.022) or recurrence (p = 0.041). On multivariate analysis with the Cox proportional hazard method, recurrence (hazard ratio [HR], 1.79; p = 0.041) and younger age (HR, 2.04; p = 0.022) were identified as independent prognostic factors for longer time to disease stabilization. CONCLUSIONS: Frontline conservative treatment seems to be the optimal treatment for most patients with DF. Younger patients or those with recurrence may require longer time to spontaneous disease stabilization.

An unusual case of proximal humeral simple bone cyst in an adult from secondary cystic change.

BACKGROUND: Simple bone cysts (SBC) have been documented to occur in adults with closed physeal plates, most commonly affecting the calcaneus in this patient subset. Although most authors theorize an association to trauma, etiology of simple bone cysts remains an enigma up to now. CASE PRESENTATION: A 26-year-old kickboxing coach sought consult for a painful right shoulder which on radiographs and magnetic resonance (MR) imaging showed a proximal humeral lesion with signs of ossification. The patient was lost to follow-up but again sought consult after 3 years for the recurring complaint. On repeat radiographs, computed tomography (CT) scan, and MR images, tumor enlargement with cystic findings typical of simple bone cyst were documented. Diagnostic aspiration of the lesion was firstly done, revealing straw-colored fluid. The patient then underwent intralesional curettage with alpha-tricalcium phosphate cement reconstruction of the lytic defect. No perioperative complications were incurred, and on latest follow-up at 3 years postoperatively, Musculoskeletal Tumor Society (MSTS) and visual analog scale (VAS) pain scores were 30/30 and 0/10, respectively. CONCLUSIONS: The authors believe their report provides support to a possible association to trauma of simple bone cysts occurring in the adult population with closed physes and suggest this subset of patients may require a different treatment approach from that for juvenile simple bone cysts.

Functional and radiological outcomes of a minimally invasive surgical approach to monostotic fibrous dysplasia.

BACKGROUND: Reports showing high recurrence rates for intralesional curettage and bone grafting have made the current treatment principle for fibrous dysplasia controversial. This study aimed to report the postoperative clinical outcomes from three minimally invasive surgical strategies we use for monostotic fibrous dysplasia (MFD). PATIENTS AND METHODS: Twelve patients with MFD presenting with no pathologic fracture or deformity and treated with one of three surgical strategies-plain open biopsy, plain alpha-tricalcium phosphate (ATP) reconstruction, and prophylactic bridge plating-were included. There were nine men and three women, with median age of 38 years. Mean follow-up was 88 weeks. Five cases involved the proximal femur, two each involved the femoral and tibial diaphyses, and one each involved the distal humerus, radial diaphysis, and proximal tibia. All cases were reviewed for functional and radiological outcomes. RESULTS: Median time to full activity was 1 day (range 1 to 3) for the plain open biopsy group, while the prophylactic bridge-plating and plain ATP reconstruction groups had longer median recovery times (59 days, range 3 to 143, and 52 days, range 11 to 192, respectively). Musculoskeletal Tumor Society scores at last follow-up were excellent for all the cases (mean 29.6, range 25 to 30). Radiological analysis using Gaski et al.'s criteria showed plain open biopsy resulted in partial resolution of proximal femoral lesions, while ATP reconstruction and prophylactic plating resulted in no change and progression in this lesion site, respectively. For femoral diaphyseal lesions, prophylactic plating resulted in partial resolution, while ATP reconstruction resulted in no change. In the tibial diaphysis, prophylactic plating resulted in partial resolution, while plain open biopsy resulted in no change. For the lesions involving the distal humerus and the proximal tibia, plain open biopsy resulted in partial resolution, while for the radial diaphyseal lesion, ATP reconstruction resulted in no change. Radiological progression was limited in 11 (92%) cases, and none had postoperative complications. CONCLUSION: Plain open biopsies for asymptomatic lesions; prophylactic bridge plating for symptomatic, large diaphyseal lytic lesions; and plain ATP reconstructions for both small and large nondiaphyseal symptomatic lytic lesions may be acceptable alternatives to curettage-incorporating procedures for MFD.

A case of infected schwannoma mimicking malignant tumor.

BACKGROUND: Infected schwannoma has been reported, this being one of the four cases published in the literature. Infected schwannoma has proven to be a tough diagnostic challenge to the treating tumor surgeon, mimicking infectious entities and most essentially, a malignant tumor. CASE PRESENTATION: The authors report the case of a 64-year-old male with a soft tissue mass in his right gluteal area that presented initially with right leg pain, then later with signs of inflammation on the tumor area. Magnetic resonance imaging (MRI), computed tomography (CT), and thallium-201 scintigraphy studies confirm the presence of soft tissue mass which had continuity with sciatic nerve, with subsequent serial MRI findings suggesting tumor enlargement with cystic degeneration. Increased level of C-reactive protein (CRP) was observed before surgery. During an open biopsy upon tissue sampling, exudates with necrotic tissue were seen. Increased level of CRP and necrotic change suggested the possibility of malignant tumor. Histopathological diagnosis was schwannoma, and group B Streptococcus was detected by culture. After the confirmation of infected schwannoma, enucleation of the tumor was performed. CONCLUSIONS: The report concludes that establishment of a benign pathology is essential when presented with similar clinical findings prior to definitive enucleation of an infected schwannoma.