Prediction of the future leg length after rotationplasty in a 3-year-old boy with extraskeletal Ewing sarcoma and long follow-up until skeletal maturity: a case report.

Rotationplasty is a useful option for limb salvage surgery in pediatric patients with malignant bone and soft tissue tumors. However, bone growth after rotationplasty remains poorly characterized. We present a case of a 3-year-old boy with extraskeletal Ewing sarcoma who underwent multidrug chemotherapy (vincristine, doxorubicin, ifosfamide and etoposide), wide resection and functional reconstruction with rotationplasty. No radiotherapy was administered during the treatment course and the patient has been followed up for 16 years after rotationplasty. Leg length at skeletal maturity was predicted using postoperative radiograph measurements of the affected leg at the age of 4 years. Two conventional prediction methods, the multiplier and straight-line graph methods were used for prediction. The predicted leg length was compared with the actual leg length to assess bone growth after rotationplasty. Immediately after surgery, the lengths of the affected lower leg, femur and tibia were 32.5, 15.0 and 17.6 cm, respectively, and the predicted leg length of the affected leg at skeletal maturity using the multiplier and straight-line graph methods was 44.6 and 44.2 cm, respectively. However, the actual length of the affected leg at skeletal maturity was 49.8 cm, and the gap between actual and predicted lengths was more than 5 cm. In conclusion, the affected leg after rotationplasty showed more than expected bone overgrowth compared with the predictions of the conventional methods.

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.

Clinical features of soft tissue sarcoma presenting intra-tumour haematoma: case series and review of the literature.

INTRODUCTION: Intra-tumour haematoma is an uncommon clinical presentation in malignant soft tissue tumours. This study aimed to highlight the clinical features of patients with soft tissue sarcomas with intra-tumour haematoma. METHODS: The patient group was composed of eight men and one woman aged between 29 and 83 years (mean 44.0 ± 20.8). The average follow-up was 29.8 months. Clinical information, including clinical features, radiological information and treatment course, was retrospectively investigated. RESULTS: Tumours were predominantly located in the chest wall and thigh, and average diameter was 10.3 cm. Six patients underwent needle biopsy with ultrasound sonography, and three underwent an open biopsy. Histological diagnoses indicated that all tumours were high grade, three of which were undifferentiated pleomorphic sarcomas, three synovial sarcomas, two pleomorphic liposarcomas, one a leiomyosarcoma and one a malignant peripheral nerve sheath tumour. The median diagnostic delay time was 3.0 months. Magnetic resonance imaging (MRI) indicated a haematoma area <25 % in three, 25-50 % in four and 50 % two. A wide resection was performed in eight patients, and radiotherapy was administered to one patient for the initial local treatment of a primary tumour. Local recurrence was detected in four patients, whilst five developed lung metastases. The five-year survival rate was 57.1 % and median survival 34.0 months. CONCLUSIONS: Soft tissue sarcomas with intra-tumour haematoma presented with locally aggressive and highly metastatic behavior, consistent with high-grade tumours.

Palliative surgery for acetabular metastasis with pathological central dislocation of the hip joint after radiation therapy: a case report.

Orthopedic surgery for bone metastases is mainly a palliative treatment. Pathological central dislocation of the hip joint secondary to osteonecrosis of acetabular metastasis after radiation therapy brings severe suffering to cancer patients. We performed minimally invasive palliative surgery for an elderly woman, and excellent pain relief was achieved. An 80-year-old female suffering from right hip pain was referred to our hospital. She had undergone surgery for lung cancer 5 years previously and her right acetabulum was subsequently affected by metastasis. With the aim of controlling the metastasis, radiation therapy was performed. Two years later, pathological central dislocation of the hip joint occurred with sudden onset of severe pain, and she was unable to maintain a sitting position and became bedridden. After she was referred to our hospital, we created an intentional pseudarthrosis in the femoral neck for palliation. After the surgery, excellent pain relief and remarkably improved mobility were achieved during her limited remaining lifetime. In this report, we introduce a novel method of producing a pseudarthrosis in the femoral neck for pathological dislocation. This procedure is a minimally invasive treatment and an alternative option for palliative surgery for pathological dislocation of the hip joint due to osteonecrosis after radiation therapy.

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.

Clinical management for spontaneous spinal epidural hematoma: diagnosis and treatment.

BACKGROUND CONTEXT: Spontaneous spinal epidural hematoma (SSEH) is a very rare condition, so there were few studies assessing the management criteria of SSEH. PURPOSE: To assess the differential diagnosis and clinical results of treatment for SSEH. STUDY DESIGN: A retrospective chart and radiograph review of the patients with SSEH. PATIENT SAMPLE: Seven consecutive patients with SSEH who were treated in our institute. OUTCOME MEASURES: Differential diagnosis, severity of the paresis, and treatment selection were assessed preoperatively and postoperatively. METHODS: We assessed the relationship between the following parameters and clinical results: (1) the initial symptoms, (2) imaging diagnosis of magnetic resonance imaging (MRI), (3) treatment selection (conservative or surgical treatment), (4) the interval of surgery, and (5) the severity of paresis using ASIA impairment scale (AIS) grading. RESULTS: In all patients, the symptoms at onset were severe neck and back pain. MRI showed isointensity to the spinal cord in the T1-weighted view and iso- or high intensity in the T2-weighted view. A solid pattern in MRI was shown in 4 patients, and a mosaic pattern was shown in 3 patients. Decompression was performed in five cases, and spontaneous recovery appeared in two cases. The mean interval time for operation was 29.8 hours. The severity of paresis was grade B in 3 cases and grade C in 4 cases at onset. These cases recovered to become grade E in 3 cases and grade D in 4 cases. Neurological deficits were present in two patients with conservative therapy and in two patients with a long interval for operation. CONCLUSIONS: Precise diagnosis without delay and rapid surgical treatment are essential for the management of SSEH.

Pleomorphic malignant fibrous histiocytoma: response of bone, lung, and brain metastases to chemotherapy.

We present a case of pleomorphic malignant fibrous histiocytoma arising from the left forearm in a 45-year-old man who had undergone resection and radiotherapy for a tumor 3 years previously. At his first visit, he had multiple lung and bone metastases. Although these metastases responded well to systemic chemotherapy, brain metastases newly appeared and caused the death of the patient. These findings demonstrate that individual sarcomatous metastatic organs exhibit different sensitivities to chemotherapy. The mechanism of this phenomenon is discussed with a review of previous reports. It is suggested that the blood-brain barrier may play an important role in sensitivity to chemotherapy.

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.

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.

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.

Radiation Therapy without Surgery for Spinal Metastases: Clinical Outcome and Prognostic Factors Analysis for Pain Control.

The purpose of radiation therapy (RT) for patients with spinal metastases is pain relief and control of paralysis. The aim of the present study was to assess pain relief using RT and to evaluate prognostic factors for pain control. We evaluated 97 consecutive patients, of mean age 62.7 years (range 28 to 86), with spinal metastases that had been treated by RT. We evaluated the effects of RT using pain level assessed using a drug grading scale based on the World Health Organization standards. The following potential prognostic factors for pain control of RT were evaluated using multivariate logistic regression analysis: age, gender, tumor type, performance status (PS), number of spinal metastases, and a history of chemotherapy. Among the 97 patients who underwent RT for pain relief, 68 patients (70.1%) presented with pain reduction. PS (odds ratio: 1.931; 95% confidence interval: 1.244 to 2.980) was revealed by multivariate logistic regression analysis to be the most important prognostic factor for pain control using RT. In conclusion, we found that RT was more effective for patients with spinal metastases while they maintained their PS.

A case of multiple skeletal lesions of brown tumors, mimicking carcinoma metastases.

Brown tumor is not a true tumor, being an unusual reactive lesion in association with primary or secondary hyperparathyroidism. We report a 23-year-old woman, who initially presented with lower back pain caused by ureterolithiasis. The initial diagnosis of brown tumor was delayed, but later pain in her leg worsened and a sacral lesion was incidentally discovered on lumbar magnetic resonance imaging (MRI); multiple destructive bone lesions were then found radiologically. The radiological features of the multiple bone lesions, which mimicked multiple metastatic tumors, seemed to be those of the terminal stage of malignancy. However, pathological examination and abnormal laboratory data showing elevated serum calcium, alkaline phosphatase, and parathyroid hormone and low serum phosphate confirmed the diagnosis of brown tumor. Adenoma in the parathyroid gland was confirmed and surgically resected. The clinical symptoms of bone pain, and abnormal radiological findings and laboratory data were resolved 6 months after surgery. Synthetic analysis of the clinical, radiological, and laboratory findings was necessary for the definite diagnosis of brown tumor.

Spontaneous regression of osteochondromas.

Spontaneous regression of an osteochondroma is an infrequent event. In this report, two cases with spontaneous regression of osteochondromas are presented. The first case was a solitary osteochondroma of the pedunculated type involving the right proximal humerus in a 7-year-old boy. This lesion resolved over 15 months of observation. The second case was a 3-year-old girl with multiple osteochondromatosis, in whom sessile osteochondromas of the right tibia and left fibula regressed over 33 months. The mechanism of this phenomenon is discussed with a review of previous reports. Regarding treatment, careful observation may be acceptable for typical osteochondromas, especially in young children.