Giant cell tumor of bone - Analysis of 213 cases involving extra-craniofacial bones.

We elucidated clinicopathological characteristics of giant cell tumor of bone (GCTB) in Japan, and significant clinicopathological factors for predicting local recurrence. Clinicopathological profiles of 213 patients with GCTB (100 male, 113 female) involving extra-craniofacial bones were retrieved. Pathological slides obtained at the initial surgery were reviewed. Fourteen pathological and five clinical features were statistically analyzed to disclose prognostic significance. Patient age ranged from 12-80 years (Average 38.7). Long bones were most frequently affected (86.4%), especially around the knee (62.9%). Histological features are basically similar to those previously reported. Within a follow-up period (24-316 months, average 106.1 months), the local recurrence rate is 29.1%. Metastasis has occurred in 9 patients. Cox regression analysis of representative clinicopathological features shows that younger age, higher mitotic count, smaller zones of stromal hemorrhage, considerable vascular invasion and absence of ischemic necrosis are significant predictors for local recurrence. Initial operative method (curettage) is a significant risk factor in univariate analysis but not by multivariate analysis (P = 0.053). Denosumab administration increases risk but not significantly (P = 0.053). Histone 3.3 G34W immunopositivity is not significant for predicting local recurrence.

Possible pathogenesis of calcaneal bone cysts.

INTRODUCTION: Recent literature indicates that calcaneal bone cysts have different clinical features compared to long bone cysts. Yet, previous studies have mainly focused on pathogenesis of long bone cysts. In this study, the pathogenesis of calcaneal bone cysts was investigated from radiological and pathological findings. MATERIALS AND METHODS: Patients comprised 18 males and 6 females diagnosed with calcaneal bone cysts at a median age ± standard deviation of 13.0 ± 3.6 years. Plain radiographs, computed tomography, magnetic resonance imaging, cystography, bone scintigraphy, and pathology were reviewed. RESULTS: All cysts occupied the central triangular area of the bone, with six extending posteriorly and two developing anteriorly. Fifteen of 20 cases (75%) showed the expanded foramen structures at the anterior margin of the posterior facet. According to cystography, four cysts showed absence of leakage to subtalar joint. Cystic fluid caused chronic haemorrhaging in 18 cases and pathologically detected cholesterol clefts were noted in 14 cases. DISCUSSION: The location of the expanded foramen structures was consistent with the nutrient foramens of the sinus tarsi artery in the unaffected population. Hence, initial cysts may originate from the collapse of the sinus tarsi artery. Subsequent haemorrhage may extend into the central triangle area, which is biomechanically free of stress. Because of poor bone remodelling, degenerative change of the haemorrhage is prolonged. The results of this study showed that circulation collapse of the sinus tarsi artery and mechanical factors are important in the formation of calcaneal bone cysts.

Secondary osteosarcoma in patients previously treated for childhood cancer: Three case reports.

The prognosis of childhood cancers has improved markedly, and the proportion of long-term survivors has increased in recent years. However, with the increase in the number of long-term survivors, the development of latent treatment-related adverse effects, such as secondary malignancies, has generated new problems. Secondary cancer is defined as a histologically distinct malignancy that develops at least 2 months after the completion of treatment for primary cancer. Genetic factors and acquired conditions associated with treatment modalities are possible causes of secondary malignancy development. Genetic factors include the presence of Li-Fraumeni syndrome (LFS) and retinoblastoma. In terms of acquired factors, radiation and chemotherapy have been reported to be the most strongly associated with secondary malignancy development. The use of alkylating agents and topoisomerase II inhibitors for the treatment of childhood cancer increases the subsequent risk of secondary tumors. We herein investigated three cases of secondary osteosarcoma several years after treatment for primary cancer. In the three patients, the familial history did not appear to fit the clinical diagnostic criteria of LFS or retinoblastoma. The patients had not received previous radiation therapy to the anatomical site of the secondary cancer. However, high dosages of alkylating agents and topoisomerase II inhibitors had been administered for the treatment of primary cancer. The exact link between chemotherapy and secondary cancer remains elusive, but the possibility of an association should be considered. Following the development of multidisciplinary therapies, long-term follow-up and monitoring of latent adverse effects may be necessary for childhood cancer survivors.

Diagnostic value of tumor-fascia relationship in superficial soft tissue masses on magnetic resonance imaging.

PURPOSE: Many surgeons participate in the management of superficial soft tissue masses, and a preoperative incorrect diagnosis frequently results in dismal oncological outcomes. The aim of this study was to identify distinguishing magnetic resonance imaging features between malignant and non-malignant lesions. METHODS: The clinicopathological data for 219 patients (men 114; women 105) with superficial soft tissue masses treated from January 2007 to December 2016 in our institution were retrospectively analyzed. The median age at the first visit was 55.6 years (range 1-90 years). MRI findings of tumor size, margin, lobulation, intratumoral hemorrhage, peritumoral edema, and tumor-fascia relationship were compared with the final histological diagnosis and tumor grade. RESULTS: Univariate analysis revealed significant relationships between histologically malignant lesions and tumor size ≥5 cm (p = 0.035), positive peritumoral edema (p = 0.031), and tumor-fascia relationship (p<0.001), but not margin (p = 0.107), lobulation (p = 0.071), and intratumoral hemorrhage (p = 0.17). In addition, using multivariate analysis, the tumor-fascia relationship (p<0.001) and tumor size were significant factors. A significant correlation between tumor-fascia relationship and malignancy (p<0.001) was observed; such a relationship was, however, not observed for tumor grade (p = 0.43). CONCLUSIONS: Tumors measuring ≥5 cm and the tumor-fascia relationship on magnetic resonance imaging are highly indicative of malignancy. When superficial soft tissue masses cross the superficial fascia and form obtuse angles with the fascia, sarcoma should be considered. The tumor-fascia relationship can offer surgeons useful information regarding the status of superficial soft tissue masses.

Clinical experiences of focal periphyseal edema zones in adolescent knees: case reports.

Focal periphyseal edema (FOPE) zones were first described in 2011. The aim of this report was to investigate the clinical course of patients with FOPE zones. Three adolescent patients with a FOPE zone in the knee were treated and observed for a maximum of 2 years. No symptoms or leg-length discrepancy developed at the final follow-up after conservative therapies. This is the first report on the follow-up of FOPE zones with a maximum of 2 years. The results suggest that observation of FOPE zones may be sufficient without invasive examinations and treatment.

Two case reports of fair lower limb function after sciatic nerve sacrifice for the treatment of a malignant peripheral nerve sheath tumor.

Wide resection for malignant soft tissue tumors may require resection of a major nerve. When a limb salvage procedure is selected in these cases, the anticipated functional outcome of the procedure must be clearly discussed with the patient, as recovery of normal function cannot be expected. In this report, we describe our surgical management of two cases of malignant peripheral nerve sheath tumor, a 58-year-old man and a 10-year-old girl, requiring a limb salvage procedure with sacrifice of the sciatic nerve. Although both patients required a short-leg brace for walking, because of a drop foot, both patients reported satisfactory functional results and were able to perform their activities of daily living with only slight difficulties. Based on our experience, limb salvage surgery can be considered for the treatment of malignancies involving the sciatic nerve, with fair functional outcomes expected.

Chondroblastoma of extra-craniofacial bones: Clinicopathological analyses of 103 cases.

We elucidated clinicopathological characteristics of chondroblastoma (CB) in Japan, and reliable clinicopathologic parameters predicting local recurrence and/or metastasis. Clinicopathological profiles of 103 CB (80 male, 23 female) in extra-craniofacial bones were retrieved. Numerical scoring of nine pathological and five radiological features was statistically analyzed to determine prognostic significance. Age ranged 8-61 years (average 19.6 years). Frequently involved sites were femur, tibia, calcaneus, patella and humerus. Radiologically, tumors were 2-80 mm (average 31.1 mm) in size. Marginal sclerosis and calcification were common. Histologically, pink cartilage, mitoses, and chicken-wire calcification were often seen. Within a follow-up period [2-260 months (average 53.5 months)], the local recurrence rate was 15.5%. No patient had metastasis. Recurrence was most frequently observed at the femur. By log-rank analysis, only cyst formation in images was significant for predicting recurrence free survival (RFS). By Cox hazard analysis with representative clinico-radiological and pathological features, only age (≥16 years) and cyst formation were significant predictors for RFS. Pathological features were not significant in both uni- and multivariate analyses.

Prognostic factors for elderly patients with primary malignant bone and soft tissue tumors.

The number of patients with primary malignant bone and soft tissue tumors in Japan is increasing in line with the increasing size of the elderly population. The aim of the present study was to determine the prognostic factors of primary malignant bone or soft tissue tumors in elderly patients. Clinical data was obtained from 90 patients, aged ≥65 years, with primary malignant bone or soft tissue tumors (bone, 20 cases; and soft tissue, 70 cases), treated at the Osaka City University Hospital between 1993 and 2013. Clinical information prior to treatment and tumor type, location, size, depth, grade and American Society of Anesthesiologists-Physical Status (ASA-PS) score were evaluated in order to identify prognostic factors using the Cox proportional hazards regression model. In addition, 5-year survival rates were evaluated using the Kaplan-Meier method. The average follow-up period was 44.8 months and the 5-year overall survival rate was 77.5%. In the multivariate analysis, ASA-PS score and high-grade sarcoma were found to be associated with a poorer overall survival. No significant differences were observed between the patient group aged 65-74 years and that aged ≥75 years. In general, aging is associated with physically reduced function and an increased prevalence of comorbidities. It was therefore expected that increasing age may be a predictive factor for poor prognosis. However, the results of the present study suggested that ASA-PS score and tumor grade were significant factors associated with poor prognosis, whereas increasing age was not. Therefore, the treatment of elderly patients with primary bone and soft tissue tumors should not be based on age.

Primary central chondrosarcoma of long bone, limb girdle and trunk: Analysis of 174 cases by numerical scoring on histology.

The aims of this study were: (i) to elucidate clinicopathological characteristics of pcCHS of long bones (L), limb girdles (LG) and trunk (T) in Japan; (ii) to investigate predictive pathological findings for outcome of pcCHS of L, LG and T, objectively; and (iii) to elucidate a discrepancy of grade between biopsy and resected specimens. Clinicopathological profiles of 174 pcCHS (79 male, 95 female), of L, LG, and T were retrieved. For each case, a numerical score was given to 18 pathological findings. The average age was 50.5 years (15-80 years). Frequently involved sites were femur, humerus, pelvis and rib. The 5-year and 10-year disease-specific survival (DSS) rates [follow-up: 1-258 months (average 65.5)] were 87.0% and 80.4%, respectively. By Cox hazards analysis on pathological findings, age, sex and location, histologically higher grade and older age were unfavorable predictors, and calcification was a favorable predictor in DSS. The histological grade of resected specimen was higher than that of biopsy in 37.7% (26/69 cases). In conclusion, higher histological grade and older age were predictors for poor, but calcification was for good prognosis. Because there was a discrepancy in grade between biopsy and resected specimens, comprehensive evaluation is necessary before definitive operation for pcCHS.

Fertility following treatment of high-grade malignant bone and soft tissue tumors in young adults.

This study was conducted to investigate marriage and fertility in long-term survivors with high-grade bone and soft tissue tumors following chemotherapy. We reviewed issues related to fertility in 47 patients (24 men and 23 women) who had survived for >5 years. The median age at diagnosis was 18.0±8.9 years and the mean duration of follow-up 11.0 years. We investigated the proportions of married subjects (number of married/total number of subjects) and fertile subjects (number who had fathered or conceived offspring/total number of subjects), the interval between first delivery and last chemotherapy, type of delivery, congenital deformities in the offspring and the association between cumulative dose of chemotherapeutic agents and subsequent reproduction. The final proportions of married and fertile subjects following sarcoma treatment were 36.2% (17/47) and 29.8% (14/47), respectively. Two subjects had fathered and 8 had conceived a total of 15 offspring. The proportion of fertile men was significantly lower compared to that of women. The mean interval between the last chemotherapy and the first delivery was 9.5 years. Of the 10 first births, 6 were normal transvaginal deliveries and 4 were caesarean sections (1 was a medical termination due to lung metastases, 1 was due to infected amniotic fluid caused by uterine myomas and 2 were performed due to abnormal rotation of the fetus). No offspring presented with congenital deformities. Fertile men had received smaller cumulative chemotherapeutic agent doses, particularly of ifosfamide, compared to those who had not fathered any offspring. In conclusion, infertility occurs more frequently in men compared to women following intensive chemotherapy. All the patients should be offered counseling regarding the potential risk of infertility prior to cancer treatment.

Flail hip joint following periacetabular tumor resection of the pelvis using upper surface of the femoral neck as a saddle: A case report.

Reconstruction following periacetabular tumor resection of the pelvis is one of the most challenging issues in limb salvage surgery. An ideal procedure has still not been established. There are several reconstruction methods available, including arthrodesis, pseudoarthrosis, resection arthroplasty, prosthetic arthroplasty, saddle prosthesis, allograft and hip transposition. In our hospital, we have been using a flail hip joint procedure without reconstruction. Herein, we report good extremity function following a flail hip joint procedure which used the upper surface of the femoral neck as a saddle facing the remaining ilium. To the best of our knowledge, among the reported flail hip joint cases, this procedure has never been used. The patient is able to walk without any pain for several hundred meters with a shoe lift but without a cane, as observed at a 20-year follow-up. The authors consider that the flail hip joint using the upper surface of the femoral neck as a saddle is a simple and effective method.

Knee reconstruction with endoprosthesis after extra-articular and intra-articular resection of osteosarcoma.

OBJECTIVE: Extra-articular resection is necessary to obtain a wide margin for primary osteosarcomas invading the knee joint, and the limb is often reconstructed using a prosthesis. Here, outcomes of extra-articular and intra-articular procedures were compared. METHODS: Between 1999 and 2012, 14 patients with osteosarcoma underwent extra-articular excision (n = 6; ages 23-65 years; mean follow-up 82.8 months) or intra-articular excision (n = 8; ages 8-58 years; mean follow-up 96.4 months). In the extra-articular group, there was one Enneking Stage IIA case and five Enneking Stage IIB cases. No local recurrences were noted in either group. RESULTS: Patient outcomes were as follows (extra-articular cases vs. intra-articular cases): continuous disease-free (2 vs. 7), alive with disease (2 vs. 0) and death from disease (1 vs. 1). Average Musculoskeletal Tumor Society functional scores were 69.4% (range 63.3-83.3%) and 88.3% (range 70.0-96.7%) in the extra-articular and intra-articular groups, respectively, constituting a significant difference (P < 0.05). The 5-year oncological overall survival exceeded 80% in both groups. Amputation was necessary for one patient in the extra-articular group. The 5-year event-free prosthesis survival rates in the extra-articular and intra-articular groups were 33.3 and 75.0%, respectively (P < 0.05). CONCLUSION: The extra-articular excision group developed more complications than the intra-articular excision group, as a result of extensive bone and muscle excision. The limb survival rates were similar in both groups. Our results suggest that extra-articular resection was a necessary and clinically acceptable procedure.

Bisphosphonate-related atypical femoral fracture with bone metastasis of breast cancer: case report and review.

BACKGROUND: Intravenous bisphosphonates (BPs) have been used to reduce the frequency of skeletal-related events due to bone metastases of several kinds of cancers. Although many studies on BP-related atypical fractures (BRAFs) due to the use of BP for osteoporosis treatment have been reported, few reports on BRAFs arising as a complication of long-term BP use for bone metastasis of cancer are available. CASE REPORT: A 62-year-old woman with a history of breast cancer presented with right thigh pain after she had a fall. Radiographs indicated a transverse fracture in the shaft of the right femur. She had been on zoledronate treatment for six years. Based on radiographic and histopathological findings, we concluded that the fracture was not a pathological fracture associated with metastasis but was a complication of long-term BP treatment. CONCLUSION: Clinical oncologists should consider the possibility of BRAFs in patients on long-term zoledronate treatment for bone metastases.

Clinical problems after initial unplanned resection of sarcoma.

OBJECTIVE: Unplanned resection of a sarcoma is often chosen in the early phase by general physicians without any imaging scrutiny. The present study aimed to highlight the clinical problems associated with unplanned resection of sarcomas. METHODS: Thirty-eight patients who underwent unplanned resection of a sarcoma and additional treatment were examined. The definite histological grading was high in 31 patients and low in 7 patients. RESULTS: The tumors were located in the depth of the subfascia in 13 patients. The maximal tumor sizes exceeded 5 cm in 16 patients. Preoperative MRI was only performed in six patients. The previous surgical margins were intralesional in 20 patients and marginal in 18 patients. Inappropriate transverse skin incisions were found in 21 patients. Extensive hematoma at the initial surgical site was seen in five patients. Thirty-three patients accepted additional wide resection due to the insufficient removal of malignancy. During an average follow-up of 42.7 months, seven patients died of lung and brain metastases. CONCLUSIONS: On excision of any soft tissue tumor, surgeons should be aware of the potential risk for erroneous management of malignancy. If not, careless surgery may render the treatment protocol complicated and require excessive additional tissue resection with poor function and prognosis. Appropriate salvage treatment may have a significant role to play after unplanned resection of the sarcoma.

Synovial chondromatosis of the acromioclavicular joint.

A 53-year-old woman presented with swelling of 3 years' duration on the right anterior chest wall. A radiograph showed coarse calcifications around the subclavicular region and erosion of the ipsilateral acromioclavicular joint. Computed tomography also showed calcifications in soft tissue. Magnetic resonance imaging revealed a tumor around the clavicle extending to the anterior aspect of chest wall, which had low signal intensity on T1-weighted imaging and high signal intensity on T2-weighted imaging. The histologic findings were of a hyaline cartilage-like mass consisting of mature chondrocytes and an extracellular matrix. The histologic diagnosis of synovial chondromatosis was made. The present case is unusual in respect of the location and size of the tumor.