An aggressive nodular fasciitis lesion protruding from the palm (USP6 gene fusion helps differentiate from sarcomas): A case report.

Nodular fasciitis is a mesenchymal lesion, which has been viewed as a reactive process. The MYH9-USP6 fusion gene was recently detected in nodular fasciitis, and nodular fasciitis is now considered to be a self-limiting neoplastic process. Recently, a case of nodular fasciitis that recurred a number of times and metastasized to soft tissues was reported, and the features of aggressive cases of nodular fasciitis are currently under investigation. Here, a case of locally aggressive nodular fasciitis is presented, in which the lesion grew rapidly and caused ulnar nerve palsy. The lesion was locally controlled via marginal excision, and no metastasis was identified at 24 months post-operation. Histologically, the lesion was consistent with nodular fasciitis, and the detection of the MYH9-USP6 fusion gene supported the diagnosis. Although most nodular fasciitis lesions are benign, some may be locally aggressive or even metastasize. In the case outlined in the present study, marginal excision was sufficient to locally control the lesion.

Bone abnormal signal incidentally found in pre-biopsy diffusion-weighted MRI for suspected prostate cancer: what does it reflect?

OBJECTIVE: To clarify the clinical significance of incidentally found diffusion-weighted MRI (DW-MRI)-positive findings on pre-biopsy MRI in patients with suspected prostate cancer. PATIENTS AND METHODS: 754 consecutive patients with suspected prostate cancer underwent pelvic MRI including DW-MRI. 43 DW-MRI-positive bone lesions were found in 27 patients. Imaging findings of these lesions were compared with the clinical diagnosis. RESULTS: Of the 43 DW-MRI-positive bone lesions, 21 (48.8%) were diagnosed as metastatic prostate cancer. The remaining 22 (51.2%) were diagnosed as red bone marrow in 17, enchondroma in 1, ganglion in 1, osteoma in 1, fibrous dysplasia in 1 and bone infarction in 1. Enchondroma, ganglion, osteoma and fibrous dysplasia all showed T1-weighted imaging (T1WI) low and T2-weighted imaging (T2WI) high signals, while others, including prostate cancer metastases, showed T1WI and T2WI low signals. Of the 40 lesions with T1WI and T2WI low signals, metastatic prostate cancer had higher apparent diffusion coefficient values (median 0.42 × 10(-3) mm(2)/s) than other lesions (0.26 × 10(-3) mm(2)/s; p < 0.0001). CONCLUSIONS: DW-MRI-positive bone lesions represent various coexisting types of bone lesions, including metastatic cancer in patients with suspected prostate cancer. T2WI findings and apparent diffusion coefficient values can be helpful in diagnosing metastatic cancer.

How long should we follow patients with soft tissue sarcomas?

BACKGROUND: Guidelines suggest that followup for low-grade soft tissue sarcomas should be every 3 to 6 months for 2 to 3 years and then annually, and for high-grade sarcomas every 3 to 6 months for 2 to 5 years, then every 6 months for the next 2 years, and then annually. However, there is only very limited evidence to support these strategies. QUESTIONS/PURPOSES: In a population of patients treated surgically for soft tissue sarcomas, we evaluated the (1) timing of diagnosis of local recurrences after sarcoma excision; (2) timing of diagnosis of distant metastases; and (3) the difference in those parameters based on tumor size and grade. METHODS: Patients diagnosed with soft tissue sarcomas and who underwent surgical excision between 1978 and 2008 were retrospectively reviewed. Age, histologic diagnosis, Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade, tumor location, and size were reviewed at a mean of 6 years (range, 1 month to 30 years). We met with patients every 3 months for 5 years, every 6 months for 10 years, and then annually until 15 years after surgery. Eight hundred sixty-seven patients with a median age at diagnosis of 52 years were eligible for analysis. The incidence of local recurrence and metastases was calculated for every 2-year period and presented per 1000 person-years. RESULTS: Ninety-eight patients (11%) developed local recurrence at a median time of 19 months; 90% of patients who had local recurrences had them within 7.1 years, and 95% occurred by 8.6 years. One hundred ninety-eight patients (23%) developed distant metastases at a median time of 12 months; 90% of patients who developed metastases developed them by 4.2 years and 95% did so by 7.3 years. High-grade tumors had a higher incidence of local recurrence and metastases in first 2 years, whereas low-grade tumors recurred at a constant rate throughout the followup period. CONCLUSIONS: Followup beyond 10 years does not yield a sufficient number of local recurrences or metastases to warrant further monitoring. LEVEL OF EVIDENCE: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Lymphadenectomy and histologic subtype affect overall survival of soft tissue sarcoma patients with nodal metastases.

BACKGROUND: Lymph node metastases in patients with soft tissue sarcomas are rare and these metastases are frequently associated with certain histologic subtypes. The survival is believed to be poor if lymph node metastases occur and the potential benefit of lymphadenectomy is unclear. QUESTIONS/PURPOSES: We determined whether lymph node metastases affect overall survival with regard to the status of lymphadenectomy, histologic subtypes, isolated or systemic metastasis, and the timing of presentation of lymph node metastases. METHODS: We retrospectively reviewed all 871 patients diagnosed with soft tissue sarcomas between 1983 and 2008 to determine whether they had lymph node metastases at diagnosis or subsequently and whether they had lymphadenectomy for treatment. Overall survival was assessed and the effect of prognostic variables was examined by a log rank test. RESULTS: Forty-nine of the 871 patients (6%) had lymph node metastases. The estimated 5-year survival rate for the group of 49 patients with lymph node metastases was 27%. Those who had lymphadenectomy had better survival at 1.5 years although there was no difference between those who did not undergo lymphadenectomy at 5 years. Patients with nonrhabdomyosarcoma had better overall survival than patients with rhabdomyosarcoma. Timing of development of lymph node involvement and whether lymph node metastases were an isolated site did not affect the overall survival. CONCLUSIONS: Overall survival of patients with lymph node metastases was related to histologic subtypes and patients with nonrhabdomyosarcoma had better survival than those with rhabdomyosarcoma. Resection of the metastatic lymph node did not improve long-term survival of patients with soft tissue sarcomas. LEVEL OF EVIDENCE: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Indications for and surgical complications of rotationplasty.

BACKGROUND: Rotationplasty is one treatment option for femoral bone sarcomas in skeletally immature patients. This procedure can also be used to save failed limb salvage procedures such as infected prostheses and failed bone grafts in adults. Rotationplasty is only rarely indicated, and the surgical complications and risk factors for failure of the procedure that might influence the treatment or patient choices have not been well described. METHODS: We retrospectively reviewed 19 patients who underwent rotationplasty focusing on surgical indications, surgical procedures such as the type of rotationplasty, and whether vessels were sacrificed and reconstructed with vascular anastomosis or preserved. Risk factors for failure of rotationplasty were categorized into either early or late postoperative complications. RESULTS: Thirteen of 19 rotationplasties were done for patients diagnosed with primary sarcoma, 3 patients for unplanned excisions of sarcomas, and 3 patients for infection. Two of 19 patients did not have sufficient circulation resulting in amputation. Six of 19 patients underwent vascular anastomoses, and all of these anastomoses were successful. Three patients had multiple operations for infection before rotationplasty, and one patient's treatment failed so an amputation was required. As late complications, one patient had delayed bone union and one had postoperative infection that healed after removal of plate fixation. Two patients had malrotation of the tibia that resolved with revision surgery. One patient had a skin ulcer that healed with conservative treatment. One patient who had an unplanned excision had a hemipelvectomy for non-union 11 months after rotationplasty. CONCLUSIONS: Rotationplasty was successfully accomplished in 16 of the 19 patients (84 %) with sarcomas, unplanned excision of sarcomas or infection. Vascular anastomosis did not increase the risk of rotationplasty failure in our series. Patients appeared to have a higher risk of failure of rotationplasty after multiple operations, possibly because of inflammation around the vessels. The surgeon should be aware of the higher potential risk and consider vascular anastomosis for these patients.

What are risk factors for local recurrence of deep high-grade soft-tissue sarcomas?

BACKGROUND: Patients with local recurrence of soft-tissue sarcomas have a poor overall survival. High-grade, soft-tissue sarcomas in deep locations may have a poorer prognosis regarding local recurrence than low-grade sarcomas or those located superficially. Although previous reports evaluated tumors at various depths, it is unclear what factors influence recurrence of deep, high-grade sarcomas. QUESTIONS/PURPOSES: We therefore determined whether possible risk factors (tumor size, location, histologic subtype, unplanned excision, local recurrence at presentation, metastasis at diagnosis, surgical procedure, surgical margin, and adjuvant treatments) influenced local recurrence of deep, high-grade, soft-tissue sarcomas. PATIENTS AND METHODS: We retrospectively reviewed 433 patients with deep, high-grade, soft-tissue sarcomas surgically treated between 1985 and 2005. For each patient, we reviewed tumor size, location, histologic subtype, unplanned excision, local recurrence at presentation, metastasis at diagnosis, surgical procedure, surgical margin, and adjuvant treatments and determined the effect of each prognostic variable on local recurrence. The minimum followup was 1 month (median, 51 months; range, 1-305 months). RESULTS: Forty-seven patients had local recurrence at a median of 10.7 months. Local recurrence at presentation, metastasis at diagnosis, and positive margins independently predicted local recurrence. No other factors independently predicted local recurrence. CONCLUSIONS: Unplanned excisions did not increase the rate of local recurrence of deep, high-grade, soft-tissue sarcomas if treated appropriately. Aggressiveness of tumor represented by metastasis or local recurrence at presentation may be a risk for local recurrence. LEVEL OF EVIDENCE: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Factors predicting local recurrence, metastasis, and survival in pediatric soft tissue sarcoma in extremities.

BACKGROUND: Pediatric soft tissue sarcomas are rare and differ from those in adults regarding the spectrum of diagnoses and treatment. Sarcomas in extremities may have different prognoses from those located elsewhere. QUESTIONS/PURPOSES: We sought risk factors predicting local recurrence, metastasis, and overall survival and asked whether radiation and chemotherapy influenced local recurrence, metastasis, and overall survival. METHODS: We retrospectively reviewed all 98 patients aged 18 years or younger diagnosed with soft tissue sarcomas in extremities from 1990 to 2008. Age, tumor size, depth, location, bone or neurovascular involvement, histologic subtypes, unplanned excision, surgical margins, metastasis at diagnosis, and adjuvant treatments were reviewed for each patient. We determined the effect of each prognostic variable on local recurrence, metastasis, and overall survival. RESULTS: Ninety-four patients underwent surgical excision and seven patients had local recurrence at a median time of 18.6 months. Radiation therapy reduced the rate of local recurrence. Fourteen patients had metastasis at diagnosis and seven patients later developed metastasis. The median time to metastasis was 20.9 months. Six patients died and the median time to death was 28.0 months. Metastasis at diagnosis was a predictive factor for death. CONCLUSIONS: When limited to extremities, radiation therapy reduced the rate of local recurrence in pediatric soft tissue sarcomas. Metastases at diagnosis predict death.

Complications and risk factors for failure of rotationplasty: review of 25 patients.

UNLABELLED: Rotationplasty is one treatment option for femoral bone sarcomas in skeletally immature patients. This procedure enables patients to avoid phantom pain, limb length discrepancy, or loosening of an endoprosthesis, and good functional outcome has been reported. However, rotationplasty is only rarely indicated and the surgical complications or risk factors for failure of the procedure that might influence choices of treatment or patient counseling have not been well described. We reviewed 25 patients who underwent rotationplasty focusing on risk factors for failure and postoperative complications. Three of 25 patients had vascular compromise resulting in amputation. All three had vascular anastomosis and were resistant to chemotherapy with less than 95% of tumor necrosis. Two of the three patients who underwent amputation had a pathologic fracture before surgery. Late complications included one patient with a tibial fracture, two with wound complications treated with skin grafts, one with nonunion, and one with subsequent slipped capital femoral epiphysis. Rotationplasty was successfully accomplished in 22 of the 25 patients. Patients with large tumors unresponsive to chemotherapy or preoperative pathologic fracture appear at higher risk for failure of rotationplasty presumably as a result of compromise of venous drainage of the leg. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Effect of in ovo immobilization on development of chick hind-limb articular cartilage: an evaluation using micro-MRI measurement of delayed gadolinium uptake.

To examine the effect of immobilization on the development of articular cartilage, we assessed glycosaminoglycan (GAG) content in the chick articular surface by delayed gadolinium-enhanced MRI of cartilage (dGEMRIC). Chick embryos were paralyzed by decamethonium bromide (DMB) from day 10 to either day 13 or day 16. The GAG content of the chick knee was compared with that of nonparalyzed chick embryos. Histologic analysis was unable to quantify GAG content; however, dGEMRIC demonstrated that GAG content was higher in the femoral condyles of the nonparalyzed embryos on day 13, and on day 16 the GAG content was lower in both the femoral condyles and the tibial plateaus of the nonparalyzed embryos. These results suggest that paralysis delays embryonic hind-limb development. Osteoblastic activity at the cartilage canal, as demonstrated by staining for alkaline phosphatase (ALP), was present only in the nonparalyzed chick embryos on day 16. The GAG content of the cartilage decreased when the cartilage canals began to form on day 16. The effect of immobilization on hind-limb development was indicated by the differences in the GAG content of the cartilage anlage measured by dGEMRIC in the developing knee joint of paralyzed and nonparalyzed embryonic chicks.