The Prognostic Significance of Surgical Treatment for Excessive Elderly Soft Tissue Sarcoma Patients over 90 Years Old: A Clinicopathological Study of 16 Cases.

OBJECTIVES: The incidence of soft tissue sarcomas (STSs) among older patients is increasing. Although surgical treatment of elderly patients with STS has been reported to improve their prognosis, most of these studies included patients with STS aged <85 years. This study aimed to analyze the clinical features and prognostic factors of STS in elderly patients aged ≥90 years. SUBJECT AND METHODS: We retrospectively identified patients aged ≥90 years with STS who were treated at our two hospitals between 1994 and 2022. Data on clinical information and detailed assessments were collected. We evaluated the features and factors affecting the prognosis of patients with older-extremity STS. In addition, we compared the clinical courses and results of patients treated with surgery and radiotherapy for primary tumors. RESULTS: Among 454 patients with STS, 16 were aged ≥90 years. Kaplan-Meier curves for overall survival showed a significantly poorer prognosis in patients who did not receive surgical treatment (p = 0.0348) and those who received radiotherapy (p = 0.0070). Moreover, we investigated the difference in prognosis between surgical treatment and radiotherapy, excluding two cases with distant metastasis at initial diagnosis and one case with no treatment. Kaplan-Meier curves for overall survival showed a significantly better prognosis in patients who underwent surgical treatment (p = 0.0161). Univariate analysis revealed that only primary tumor size was a significant predictor of poor prognosis (p = 0.0426). CONCLUSION: In patients with STS aged ≥90 years old, aggressive surgical treatment may improve the prognosis more than radiotherapy.

Clinical outcomes in patients with adamantinoma: Report from the bone and soft tissue tumor registry in Japan.

BACKGROUND: Adamantinomas are rare malignant bone tumors. Due to their low incidence, there are few reports on the clinical results of adamantinoma. OBJECTIVES: This study aims to clarify outcomes in patients with adamantinoma using data from the National Bone and Soft Tissue Tumor Registry. METHODS: From 2006 to 2019, 38 cases of tibial origin were included. Twenty-four were male and 14 were female, with a mean age of 37 (6-87) years and a mean follow-up of 35 (1-128) months. RESULTS: Surgery was performed in 33 cases (87%) (curettage: 4 cases, wide resection: 27 cases, amputation: 2 cases). Reconstruction was performed in 27 patients who underwent wide resection. A total of 12 additional surgeries were performed in 11 patients. The main reason for the additional surgeries was nonunion of grafting bone in 6 cases. Oncologic outcomes were DOC (death from other causes) in one case and NED (no evidence of disease) in 37 cases. CONCLUSIONS: The results of treatment of adamantinomas in Japan have been extremely favorable. This may be due in part to the large number of cases with wide resection.

Coefficient of variation of T2-weighted MRI may predict the prognosis of malignant peripheral nerve sheath tumor.

BACKGROUND: We investigated whether non-enhancement MRI features, including measurement of the heterogeneity of the tumor with MR T2 imaging by calculating coefficient of variation (CV) values, were associated with the prognosis of non-metastatic malignant peripheral nerve sheath tumors (MPNST). METHODS: This retrospective study included 42 patients with MPNST who had undergone surgical resection (mean age, 50 years ± 21; 20 male participants). Non-enhancement MR images were evaluated for signal intensity heterogeneity on T1- and T2-weighted imaging, tumor margin definition on T1- and T2-weighted imaging, peritumoral edema on T2-weight imaging, and CV. We measured the signal intensities of MR T2-weighted images and calculated the corresponding CV values. CV is defined as the ratio of the standard deviation to the mean. The associations between factors and overall survival (OS) were investigated via the Kaplan-Meier method with log-rank tests and the Cox proportional hazards model. RESULTS: The mean CV value of MR T2 images was 0.2299 ± 0.1339 (standard deviation) (range, 0.0381-0.8053). Applying receiver operating characteristics analysis, the optimal cut-off level for CV value was 0.137. This cut-off CV value was used for its stratification into high and low CV values. At multivariate survival analysis, a high CV value (hazard ratio = 3.63; 95% confidence interval = 1.16-16.0; p = 0.047) was identified as an independent predictor of OS. CONCLUSION: The CV value of the signal intensity of heterogenous MPNSTs MR T2-weighted images is an independent predictor of patients' OS.

Establishment and characterization of a novel dedifferentiated chondrosarcoma cell line, SMU-DDCS, harboring an IDH1 mutation.

Dedifferentiated chondrosarcoma (DDCS) is a high-grade subtype with a bi-morphic histological appearance of a conventional chondrosarcoma component and it can abruptly transition to a high-grade non-cartilaginous sarcoma. To better understand the biological features of DDCSs and to help develop new therapies, a novel DDCS cell line, SMU-DDCS, was established. Tissue from an open biopsy of a tumor resected from a 75-year-old patient was subjected to primary culture. The cell line was established and authenticated by assessing DNA microsatellite short tandem repeats. The cells maintained in monolayer cultures exhibited constant growth, spheroid formation, and high invasive capacity. Out of the four mice inoculated with SMU-DDCS cells, tumors developed in three mice after 2 weeks. R132C mutation was found in the IDH1 but not the IDH2 genomic DNA sequence of SMU-DDCS cells. SMU-DDCS cells exhibited low chemosensitivity to doxorubicin, methotrexate, and cisplatin. This SMU-DDCS cell line harboring an IDH1 mutation will be a useful tool for investigating DDCS development and for evaluating novel therapeutic agents against it.

A Comparative Study of Foot Range of Motion and Activities of Daily Living Status of Patients Following Ankle Arthrodesis and Tibiotalocalcaneal Arthrodesis.

Subtalar arthrodesis in addition to ankle arthrodesis (AA) should have effect on foot motion and functional capabilities pertaining to activities of daily living (ADL); however, it is not well characterized. We compared the foot range of motion and ADL-related functional capabilities between patients who had undergone AA and tibiotalocalcaneal arthrodesis (TTC). Twenty-one AA patients and 10 TTC patients were enrolled. Foot sagittal motion arc was measured by radiographs. Patient satisfaction, ADL, footwear restriction, and rating scale scores were compared between the 2 groups. The mean sagittal motion arc in the AA group (23.5 ± 6.2°) was significantly greater than that in the TTC group (15.3 ± 3.5°). Patient satisfaction and overall ADL status was comparable in the 2 groups. Difficulties in climbing stairs, wearing rubber boots, and sitting in cross-legged position were more frequently reported in the TTC group. Our findings may be valuable for both surgeons and patients in predicting post-treatment ADL status and avoiding over expectations.

Usefulness of SynCAM3 and cyclin D1 immunohistochemistry in distinguishing superficial CD34-positive fibroblastic tumor from its histological mimics.

Superficial CD34-positive fibroblastic tumor (SCPFT) is a fibroblastic/myofibroblastic soft tissue tumor of rarely metastasizing intermediate malignancy. Some recent studies have described a relationship between SCPFT and PRDM10-rearranged soft tissue tumor (PRT) based on SynCAM3 and PRDM10 expression on immunohistochemistry. We performed CD34, cytokeratin AE1/AE3, SynCAM3, and PRDM10 immunohistochemistry in SCPFT and its histological mimics, including myxoinflammatory fibroblastic sarcoma (MIFS), superficially localized myxofibrosarcoma (MFS), and undifferentiated pleomorphic sarcoma. We also examined cyclin D1 expression because it is expressed in MIFS and MFS. We conducted fluorescence in situ hybridization (FISH) of PRDM10 rearrangement in SCPFT cases. On immunohistochemistry, only SCPFT showed strong and diffuse SynCAM3 expression. SCPFT also exhibited strong nuclear and weak cytoplasmic cyclin D1 expression, which was similar to that observed in MIFS. Two of five SCPFT cases exhibited nuclear PRDM10 expression. FISH revealed PRDM10 split signals in 44% and 24% of tumor cells in two SCPFT cases showing nuclear PRDM10 expression on immunohistochemistry, respectively. A minority of non-SCPFT cases showed focal SynCAM3 expression, but a combination of SynCAM3 and cyclin D1 in addition to CD34 and cytokeratin AE1/AE3 may be useful for the differential diagnosis of SCPFT and its histological mimics.

Diagnostic utility of CSF1 immunohistochemistry in tenosynovial giant cell tumor for differentiating from giant cell-rich tumors and tumor-like lesions of bone and soft tissue.

BACKGROUND: Tenosynovial giant cell tumor (TSGCT) is a benign fibrohistiocytic tumor that affects the synovium of joints, bursa, and tendon sheaths and is categorized into localized TSGCT (LTSGCT) and diffuse TSGCT (DTSGCT). LTSGCT and DTSGCT are characterized by recurrent fusions involving the colony-stimulating factor 1 (CSF1) gene and its translocation partner collagen type VI alpha 3 chain. The fusion gene induces intratumoral overexpression of CSF1 mRNA and CSF1 protein. CSF1 expression is a characteristic finding of TSGCT and detection of CSF1 mRNA and CSF1 protein may be useful for the pathological diagnosis. Although there have been no effective anti-CSF1 antibodies to date, in situ hybridization (ISH) for CSF1 mRNA has been performed to detect CSF1 expression in TSGCT. We performed CSF1 immunohistochemistry (IHC) using anti-CSF1 antibody (clone 2D10) in cases of TSGCT, giant cell-rich tumor (GCRT), and GCRT-like lesion and verified its utility for the pathological diagnosis of TSGCT. METHODS: We performed CSF1 IHC in 110 cases including 44 LTSGCTs, 20 DTSGCTs, 1 malignant TSGCT (MTSGCT), 10 giant cell tumors of bone, 2 giant cell reparative granulomas, 3 aneurysmal bone cysts, 10 undifferentiated pleomorphic sarcomas, 10 leiomyosarcomas, and 10 myxofibrosarcomas. We performed fluorescence ISH (FISH) for CSF1 rearrangement to confirm CSF1 expression on IHC in TSGCTs. We considered the specimens to have CSF1 rearrangement if a split signal was observed in greater than 2% of the tumor cells. RESULTS: Overall, 50 of 65 TSGCT cases, including 35 of the 44 LTSGCTs and 15 of the 20 DTSGCTs, showed distinct scattered expression of CSF1 in the majority of mononuclear tumor cells. MTSGCT showed no CSF1 expression. Non-TSGCT cases were negative for CSF1. FISH revealed CSF1 rearrangement in 6 of 7 CSF1-positive cases on IHC. On the other hand, FISH detected no CSF1 rearrangement in all CSF1-negative cases on IHC. Thus, the results of IHC corresponded to those of FISH. CONCLUSION: We revealed characteristic CSF1 expression on IHC in cases of TSGCT, whereas the cases of non-TSGCT exhibited no CSF1 expression. CSF1 IHC may be useful for differentiating TSGCTs from histologically mimicking GCRTs and GCRT-like lesions.

Effect of Accelerated Rehabilitation on Early Return to Sport After Arthroscopic Ankle Lateral Ligament Repair.

Background: Although the minimal invasiveness of arthroscopic ankle lateral ligament repair (ALLR) means that an early return to sporting activities can be anticipated, studies have described postoperative cast immobilization and the avoidance of weightbearing for a certain period. Accelerated rehabilitation may be helpful for an early return to sport. Purpose: To investigate clinical outcomes of ALLR and accelerated rehabilitation with a minimum duration of postoperative ankle immobilization and proactive early weightbearing. Study Design: Case series; Level of evidence, 4. Methods: This study investigated 23 ankles of 22 patients (11 men, 11 women; mean age, 38.7 years) who underwent ALLR for chronic lateral ankle instability. Postoperative management included the avoidance of weightbearing until postoperative day 3, after which full weightbearing walking with a brace was permitted. The objective was to return to competitive sport 8 weeks after surgery. The following were evaluated: pre- and postoperative instability and pain symptoms, ankle range of motion, anterior drawer distance on stress radiograph, anterior translation measured with a capacitance-type strain sensor, the Ankle-Hindfoot Scale from the Japanese Society for Surgery of the Foot, and the SAFE-Q (Self-Administered Foot Evaluation Questionnaire). Results: Two male patients dropped out and were excluded from analysis. Postoperatively, instability and pain resolved or improved in all patients. There was no significant postoperative change in range of motion. There were significant pre- to postoperative improvements in talar tilt angle (from 12.2°-5.6°, P < .01), anterior drawer distance (8.2-4.4 mm, P < .01), and anterior translation (10.5-4.6 mm, P < .01) as well as the Ankle-Hindfoot Scale score (68.8-96.8, P < .01) and all subscales of the SAFE-Q (P ≤ .01 for all). Complete return to sport was achieved by 75% of the patients at 8 weeks postoperatively. Conclusion: When accelerated rehabilitation with proactive weightbearing exercises was implemented from postoperative day 3 without ankle immobilization after ALLR, there were significant improvements in objective assessments of ankle stability and clinical scores, and as many as 75% of the patients were able to make a complete return to sport within 8 weeks.

Risk Factors of Early Distant Metastasis After Primary Tumor Treatment in Soft Tissue Sarcoma.

BACKGROUND/AIM: Distant metastasis has a strong influence on prognosis in patients with soft tissue sarcoma (STS). While various risk factors have been reported for distant metastases, risk factors for distant metastases early after treatment of primary tumor have not been investigated. This study aimed to evaluate the factors of early distant metastasis after primary tumor resection in patients with STS. PATIENTS AND METHODS: We retrospectively identified patients with STS involving the extremities or trunk without any metastasis at the first visit. Data on clinical information and detailed assessment were collected. We evaluated clinical information as a risk factor for distant or lung metastases. Additionally, we evaluated risk factors for metastases in patients with distant metastases as early as 6 months after the initial resection of the primary tumor. RESULTS: A total of 337 patients were included in the study. Multivariate analysis revealed that the size of the primary tumor (p=0.0011 and p=0.0167), consultation in a short period after onset (p=0.0325 and p=0.0402), histological high grade (p=0.0006 and p=0.0002), and inadequate surgical margin (p=0.0151 and p=0.0055) were significant predictors for poor prognosis for all distant and lung metastases, respectively. However, the only risk factor for early metastases within 6 months was young age (p=0.0148). CONCLUSION: The only risk factor for early distant metastasis after primary tumor resection in patients with STS was young age, even though large tumor diameter and histological high grade were risk factors for distant metastasis.

Non-ossifying fibromas and fibrous cortical defects around the knee - an epidemiologic survey in a Japanese pediatric population.

BACKGROUND: The aim of the present study was to evaluate the prevalence of non-ossifying fibroma (NOF) and fibrous cortical defect (FCD) in a Japanese pediatric population and the association between the lesion size and pain. METHODS: This retrospective study, conducted across 10 Japanese institutions, included patients aged 5-15 years who had undergone standard antero-posterior and lateral view radiography of the knee. Using these radiographs, we diagnosed the lesion as a NOF or FCD. Patient demographics, including age, sex, the size and location of the NOF, and chief complaint were recorded. The lesion size was determined using radiographs. Student's t-test was used to compare the associations between the lesion size and spontaneous pain. RESULTS: A total of 6222 subjects (3567 boys and 2455 girls) were included in this study. The number of NOF and FCD cases was 143 and 437, respectively, and the prevalence of NOF and FCD was 2.3% and 7.0%, respectively. The average size of NOF and FCD was 22.1 mm (range: 4-102 mm) and 13.2 mm (range: 5-21 mm), respectively. Three patients (2.1%) had pathological fractures due to NOF. Of the 140 NOFs and 437 FCDs, we obtained complaints from the medical records of 126 and 393 patients, respectively. The number of patients with spontaneous pain or other problems with NOF was 68 (54%) and 58 (46%), respectively, that of patients with FCD was 195 (50%) and 198 (50%) patients, respectively. The lesion size was not associated with spontaneous pain in either lesion (p = 0.67 and p = 0.27, respectively). CONCLUSION: The prevalence of NOF and FCD around the knee was lower than that reported in previous studies. The prevalence of NOF increased and that of FCD decreased with advancing age. In both lesions, the lesion size may not be associated with pain.

Surgically treated reactive arthritis of the ankle after COVID-19 infection: A case report.

A 37-year-old man developed right ankle pain and swelling six days after being diagnosed with coronavirus disease (COVID-19). Despite conservative treatment, his ankle symptoms persisted. Magnetic resonance imaging and computed tomography showed synovial hypertrophy and bone erosion in the ankle. Following arthroscopic synovectomy, performed 69 days after the COVID-19 diagnosis, the pain improved significantly. The clinical course was consistent with that of reactive arthritis following severe acute respiratory syndrome coronavirus 2 infection. The pathological findings resembled rheumatoid nodules. The bone erosion may have originated from the inflammatory pathway, which resembles the mechanism of rheumatoid arthritis.

Efficacy and safety of cyclooxygenase 2 inhibitors for desmoid tumor management: a systematic review.

The efficacy and safety of cyclooxygenase 2 (COX2) inhibitors for the treatment of desmoid-type fibromatosis (DF) are unclear. Therefore, we systematically reviewed related literature to assess the efficacy and safety of COX2 inhibitors for DF treatment. We searched pertinent literature between January 1999 and August 2017 to identify relevant studies using the keywords "Fibromatosis, aggressive" and "Cyclooxygenase inhibitors." Thereafter, we screened and determined the quality of the studies using the Grading of Recommendations Assessment, Development, and Evaluation system and extracted the article data. The critical outcomes selected were the efficacy and adverse effects of COX2 inhibitors. Efficacy was evaluated in terms of clinical benefit when patients showed complete response, partial response, and stable disease. Thirty-one articles were identified from the database search, and one was identified through the reviewers' manual search. Finally, we retrieved six studies, including three case reports, comprising 89 patients after the first and second screenings. Fifty-three patients were excluded because three studies were reported from the same institution; hence, in total, 36 patients were included. Clinical benefit was noted in 64% of the patients. Three adverse effects were identified from the records of the six extracted studies. The strategy of watchful waiting using COX2 inhibitors with few side effects is weakly recommended for DF, especially DF patients with pain.

Impact of Primary Tumor Resection on Metastasis to the Lung in Patients With Bone and Soft-tissue Sarcoma.

BACKGROUND: Few reports have investigated the effect of primary tumor resection on lung metastasis and prognosis in patients with bone and soft-tissue sarcoma, and we evaluated its effect on lung metastasis and prognosis. PATIENTS AND METHODS: We retrospectively identified patients with lung metastasis from bone and soft-tissue sarcoma. We examined the factors affecting prognosis and the rate of lung metastasis increase. RESULTS: A total of 48 patients were included. The multivariate analysis revealed that poor prognosis was significantly associated with a large maximum diameter of lung metastasis at the first visit and a rapid rate of increase (p=0.0400 and p=0.0003, respectively). The multivariate logistic regression analysis revealed that the rate of increase of size of lung metastases was only significantly associated with a large maximum diameter at the first visit (p=0.0245). CONCLUSION: Primary tumor resection of bone and soft-tissue sarcoma in patients with lung metastasis was not shown to affect their prognosis.

Oncological and prognostic analysis of soft tissue sarcoma of the elbow: report using the bone and soft tissue tumor registry in Japan.

OBJECTIVE: Soft tissue sarcomas in the elbow are extremely rare, and they have primarily been described in case series. Definitive concerning the prevalence and prognostic factors of elbow soft tissue sarcomas remain unknown. We examined the outcome of patients with elbow soft tissue sarcomas and identified the relevant prognostic factors. METHODS: In total, 219 patients with elbow soft tissue sarcomas were identified using data from the bone and soft tissue tumor registry in Japan. Differences in demographics, disease characteristics, treatment and survival were compared among the patients. Survival analyses including local recurrence-free survival, distant metastasis-free survival, and overall survival were performed using the Kaplan-Meier method with log-rank tests and the Cox proportional hazards model. RESULTS: Two hundred nineteen patients with elbow soft tissue sarcomas were identified, including 119 males (54.3%) and 100 females (45.7%). In total, 189 patients (86.3%) underwent surgery including re-excision. Of the surgically treated patients, 180 (95.2%) underwent limb salvage surgery, and nine patients (4.8%) underwent amputation. The 5-year overall survival, local recurrence-free survival, and distant metastasis-free survival rates for the entire patient cohort were 76.3, 70.1, and 69.3%, respectively. After adjusting for clinically relevant factors, overall survival was significantly worse among patients with tumors: >10 cm (hazard ratio = 4.34; 95% confidence interval = 1.03-18.2) and metastatic disease (hazard ratio = 6.94; 95% confidence interval = 1.55-31.0). CONCLUSIONS: Tumor size was identified as an independent risk factor for poor prognosis.

Relationship Between Plantar Callosity and Foot Deformity in Hallux Valgus Using Weightbearing Computed Tomography.

Plantar callosities under lesser metatarsals are often accompanied by the hallux valgus, and the cause of callosity is thought to be associated with the foot deformity, such as the metatarsal length discrepancy, the abnormal metatarsal head height, cavus, flat foot, and rheumatoid conditions. However, it is unclear which variable is most involved in the cause of callosity in hallux valgus deformity. To clarify the factors associated with the callosity with hallux valgus deformity, we conducted multiple image assessments based on weightbearing radiography and computed tomography. A retrospective review was performed based on the collection of clinical records from all patients with hallux valgus treated from 2010 to 2019 in our institution. We measured the hallux valgus angle, intermetatarsal angles, calcaneal pitch angles, talo-first metatarsal angles, metatarsal length, metatarsal head height, first metatarsal pronation angles, and sesamoid position with weightbearing radiography and computed tomography. We analyzed the relation between callosity formation and imaging assessments using univariate and multivariate logistic regression models. Fifty feet were retrospectively evaluated, and multiple logistic analyses by the stepwise method revealed that the first metatarsal-lateral-sesamoid distance was the only radiographical variable associated with callosity formation among all the tested variables (p < .001). As the grade of the callosity became more severe, the lateral shift of the lateral sesamoid increased. The position of the sesamoid bone appears to have a critical role in the assessment and choice of treatment protocols and further research needs to be conducted on the relationship with the position of sesamoid bone to elucidate the mechanism of callus formation.

Clinical features of bone and soft tissue tumors of the foot and ankle: Results from a retrospective single-center case-series.

BACKGROUND: The malignant potential of the musculoskeletal tumors of the foot and ankle has often been underestimated because of their rarity. The current study reviewed the clinical features of the tumors of the foot and ankle, and evaluated the tumor size via imaging-based analysis to distinguish between benign and malignant lesions. METHODS: A retrospective review was performed using the clinical records of all patients with histologically confirmed musculoskeletal tumors of the foot and ankle, treated between 1998 and 2020 at our institution. We examined the distribution of tumors, rate of unplanned excision for primary surgery, and subsequent outcomes. In addition, the tumor size was examined via magnetic resonance imaging, and the cut-off value was determined via receiver operating characteristic (ROC) curve. RESULTS: A total of 103 bone and soft tissue tumors of the foot and ankle were included, of which 78 were soft tissue tumors and 25 were bone tumors. Of the 14 cases of malignant bone and soft tissue tumors, 6 (42.9%) received unplanned excision in the primary surgery, followed by amputation in 3 cases. Tumor size of malignant soft tissue tumors was significantly larger than that of benign soft tissue tumors (47.6 mm vs. 31.0 mm, respectively, P < .001). However, the difference between benign and malignant bone tumors was not statistically significant with the numbers available. ROC curve determined that the optimum diagnostic cutoff value for soft tissue tumor size was 40 mm, with a high area under the ROC curve 0.816 (95% CI: 0.711-0.921, sensitivity 91.7%, specificity 70.5%) CONCLUSIONS: We highlighted that bone and soft tissue tumors of the foot and ankle were often misdiagnosed and initially inadequately treated. We suggest that a cutoff value of 40 mm may be a useful index for prediction of malignancy in soft tissue tumors of the foot and ankle. LEVEL OF EVIDENCE: Ⅲ.

Impact of Acridine Orange in Patients With Local Recurrent Soft Tissue Sarcoma.

BACKGROUND/AIM: Local recurrence in soft tissue sarcoma (STS) is a risk factor of worse prognosis. Although a few studies have shown that adjuvant therapy with acridine orange (AO) is effective for local control of primary STS, there have been no reports examining its effectiveness for local recurrence. PATIENTS AND METHODS: This retrospective study included 36 patients with first local recurrence of STS. Of them, 23 patients received wide excision without AO therapy (Wide group); the other 13 patients received marginal excision with AO therapy (AO group). We compared re-recurrence rates between these two groups. RESULTS: The total re-recurrence rate was 43.5% in the Wide group and 46.2% in the AO group. There was no significant difference in local re-recurrence-free survival and overall survival between the two groups. CONCLUSION: Adjuvant AO therapy combined with a marginal excision suppresses local re-recurrence rates of individuals with local STS recurrence.

Osteofibrous dysplasia-like adamantinoma treated via intercalary segmental resection with partial cortex preservation using pedicled vascularized fibula graft: a case report.

BACKGROUND: Morphologically, osteofibrous dysplasia-like adamantinoma is thought to be intermediate between osteofibrous dysplasia and adamantinoma. Its treatment is not well established owing to its rarity. CASE PRESENTATION: We report about of a 10-year-old girl with osteofibrous dysplasia-like adamantinoma initially diagnosed as osteofibrous dysplasia and treated via intercalary segmental resection with partial cortex preservation using a pedicled vascularized fibula graft for reconstruction. Bone union was observed 9 weeks after surgery. Twenty-two months after the definitive surgery, no recurrence was observed. CONCLUSION: This case illustrates the upgrade from osteofibrous dysplasia to osteofibrous dysplasia-like adamantinoma. The surgical method may aid the treatment of osteofibrous dysplasia-like adamantinoma with incomplete cortex involvement of the tumor.

Prognostic Significance of Histological Subtype in Soft Tissue Sarcoma With Distant Metastasis.

BACKGROUND/AIM: Few studies have examined the prognostic significance of histological subtypes in patients with soft tissue sarcoma (STS) and distant metastasis, and we evaluated the relationship between the histological subtypes and clinical outcomes. PATIENTS AND METHODS: This retrospective study evaluated the histological subtypes of 105 patients with STS and distant metastasis. The STS histological subtypes were compared based on the clinical information. RESULTS: The Kaplan-Meier curves for overall survival revealed that myxoid liposarcoma had a significantly better prognosis compared to Malignant Peripheral Nerve Sheath Tumor (MPNST) (p=0.0221). In the multivariate logistic regression analyses, the independent predictors of a poor prognosis were: i) large size, ii) advanced stage, and iii) non-surgical treatment for metastasis (p<0.05). The presence or absence of lung metastasis was not significantly associated with prognosis (p=0.4452). CONCLUSION: Myxoid liposarcoma had a better prognosis compared to MPNST in STS patients with distant metastasis. The surgical removal of distant metastatic lesions may improve the patient's prognosis.