Taisuke Tanizawa

Malignant change secondary to fibrous dysplasia.

BackgroundMalignant change in fibrous dysplasia (FD) is very rare. This study was carried out to establish some characteristic clinical information about this disorder.MethodsFour cases with a malignant change in FD out of 128 cases with FD were surgically treated and followed up for a median period of 61.3 months. The mean age of the patients was 39.8 years. Clinical features, radiological findings, and the outcome were analyzed for each of the four cases.Results and conclusionThe sites of the lesions were tibia (2 cases), femur (1 case), and rib (1 case). The forms of FD were monostotic in one case and polyostotic in three cases. Radiologically, plain films and computed tomography (CT) showed osteolytic lesions with poorly delineated margins within and/or near areas having a ground-glass appearance. In the osteolytic lesions, simple cystic changes associated with old FD could be excluded by enhanced magnetic resonance imaging (MRI). Histopathologically, two cases were osteosarcoma, one case was malignant fibrous histiocytoma (MFH), and one case was fibrosarcoma. The management of this disease should be decided according to the type of primary high-grade bone sarcoma. One patient, with MFH, was dead of lung metastasis 13 months after surgery. The others are alive without disease.

SYT-SSX breakpoint peptide vaccines in patients with synovial sarcoma: A study from the Japanese Musculoskeletal Oncology Group†

In the present study, we evaluated the safety and effectiveness of SYT‐SSX‐derived peptide vaccines in patients with advanced synovial sarcoma. A 9‐mer peptide spanning the SYT‐SSX fusion region (B peptide) and its HLA‐A*2402 anchor substitute (K9I) were synthesized. In Protocols A1 and A2, vaccines with peptide alone were administered subcutaneously six times at 14‐day intervals. The B peptide was used in Protocol A1, whereas the K9I peptide was used in Protocol A2. In Protocols B1 and B2, the peptide was mixed with incomplete Freunds adjuvant and then administered subcutaneously six times at 14‐day intervals. In addition, interferon‐α was injected subcutaneously on the same day and again 3 days after the vaccination. The B peptide and K9I peptide were used in Protocols B1 and B2, respectively. In total, 21 patients (12 men, nine women; mean age 43.6 years) were enrolled in the present study. Each patient had multiple metastatic lesions of the lung. Thirteen patients completed the six‐injection vaccination schedule. One patient developed intracerebral hemorrhage after the second vaccination. Delayed‐type hypersensitivity skin tests were negative in all patients. Nine patients showed a greater than twofold increase in the frequency of CTLs in tetramer analysis. Recognized disease progression occurred in all but one of the nine patients in Protocols A1 and A2. In contrast, half the 12 patients had stable disease during the vaccination period in Protocols B1 and B2. Of note, one patient showed transient shrinkage of a metastatic lesion. The response of the patients to the B protocols is encouraging and warrants further investigation.

Definition of the target sign and its use for the diagnosis of schwannomas.

The target sign is one of the characteristic imaging findings in schwannomas that distinguishes it from other soft tissue tumors, but its definition has varied in past studies. We defined the characteristic target sign on MRI and clarified its sensitivity and specificity based on histology. Of the 199 schwannomas reviewed, 162 (81%) showed biphasic macroscopic and microscopic patterns of central Antoni A and peripheral Antoni B cells; 118 (59%) also showed a biphasic pattern on MRI. Gadolinium-enhanced T1-weighted images showed central high intensity and peripheral low intensity, whereas T2-weighted images showed peripheral high intensity and central low intensity. Seventy-eight cases had cystic, hemorrhagic, or necrotic degeneration, which corresponded to high intensity on T2-weighted images and low intensity on gadolinium-enhanced T1-weighted images. Nine cases in which the degenerative area was only in the central portion of Antoni A showed a triphasic pattern. We defined the target sign as the biphasic or triphasic pattern on MRI, and the sign correlated with macroscopic and microscopic findings. The specificity of the target sign in schwannoma was 100% and the sensitivity was 59%; therefore, the target sign was characteristic of and helpful for the diagnosis of schwannomas.Level of Evidence: Level II, diagnostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Surgical treatment of bone metastasis: indications and outcomes.

Due to advances in cancer treatment that prolong survival, there has been a noticeable increase in the prevalence of bone metastasis. Consequently, the likelihood of orthopedic surgeons encountering such tumors and the need for effective surgical treatment are also expanding. Although bone metastasis constitutes only one possible type of metastatic lesion, and while local control at a bone metastatic site alone may have little effect on total curability, the quality of life (QOL) of the patient cannot be ensured if local control is ignored. Therefore, it is important to achieve local control through multidisciplinary treatments aimed at both reduced surgery and at conservation of limb function. Surgical treatment may be indicated after a series of more conservative treatments. Among the main indications are pathologic fracture or a high risk of fracture of limb bones. Acute spinal cord injury caused by acute compression fracture from spinal metastasis is a relative indication. The occurrence of a solitary metastasis, for which extended survival may be anticipated, is also an indication for surgery to achieve local control. Appropriate local control is required to realize a predicted survival period. As well, the surgical method, resection margin, and supplementary treatments, and immediately effective reconstruction method should be selected accordingly.

A Japanese patient with Li-Fraumeni syndrome who had nine primary malignancies associated with a germline mutation of the p53 tumor-suppressor gene

We describe a patient who had nine primary malignant tumors and a germline mutation in the p53 tumor-suppressor gene, characteristically found in the Li-Fraumeni syndrome (LFS). A 15-year-old girl with no family history of cancer was referred to our hospital because of pain and swelling of the right knee. Osteosarcoma was diagnosed. The patient received chemotherapy followed by surgery and had a remission. After the age of 28 years, nine primary malignant tumors developed successively, including right breast cancer, colon cancer, malignant fibrous histiocytoma (MFH) of the abdominal wall, right lung double cancers, bilateral breast cancers, and MFH of the left thigh. This is the second highest number of types of primary malignant tumors to be reported in LFS. All tumors were treated by a multidisciplinary approach, including surgery. Genetic analysis revealed a germline missense mutation in the p53 gene (c.659 A > G), resulting in Y220C, which has been reported in three families with LFS. The patient died of lung metastasis from MFH at the age of 37 years. Despite the multiple tumors, repeated induction of remissions resulted in long survival. Our findings suggest that a multidisciplinary approach to treatment, including surgery, is beneficial in patients with LFS.

Oncologic outcome of parosteal osteosarcoma.

BackgroundThis retrospective review evaluated the clinical features and surgical outcomes of parosteal osteosarcoma (POS).MethodsNine patients were surgically treated and followed up. Their mean age was 30.8 years. Clinical information and oncologic outcomes of each case were analyzed.ResultsSites of involvement were all in the femur, and all tumors arose from the metaphyseal area of the distal femur. Biopsies for definite diagnoses were performed in just two of the nine cases. Wide resection was applied for all tumors. Surgical evaluations were a 1-cm-wide procedure in six cases and a 2-cm-wide procedure (or more) in three cases. All patients were found to be continuously disease free during the follow-up period of 115.1 months.ConclusionPOS showed characteristic findings on radiographic images. Therefore, wide resection without biopsy could be performed in 77.8% (7/9) of the cases. This procedure may contribute to attaining better limb function, because of preventing contamination of healthy surrounding tissue and minimizing the extent of resection. The safety margin was evaluated as a 1-cm-wide procedure. For the choice of reconstruction, indication of autobone grafting (3/9) or total knee replacement (TKR) (6/9) depended on tumor size, location, and shape. With no adjuvant treatments, all cases have shown good clinical courses during the entire follow-up period of about 10 years.

Dedifferentiated liposarcoma with rhabdomyoblastic differentiation in an 8-year-old girl.

The development of a liposarcoma during childhood is extremely rare. We report a case of dedifferentiated liposarcoma arising in the left thigh of an 8-year-old girl. Morphological and immunohistochemical studies revealed occasional rhabdomyoblastic differentiation in the dedifferentiated component. The identical cells were co-mingling with the well-differentiated liposarcoma component. This is the first description of such a case of liposarcoma occurring in a child.

Incidence of Pneumothorax in Advanced and/or Metastatic Soft Tissue Sarcoma Patients during Pazopanib Treatment

Sird After pazopanib’s approval for the treatment of soft tissue sarcomas, pneumothorax was reported as an unexpected adverse event linked to pazopanib treatment. Pneumothorax was rarely reported in the pre-approval clinical trials of pazopanib [1], so the incidence of pneumothorax during pazopanib treatment has not yet been established. We retrospectively reviewed the cases of 32 soft tissue sarcoma patients treated with pazopanib between November 2012 and December 2013 at our institute. The median follow-up time was 3.1 months (range 0.3e9.6

Pulmonary metastasis from giant cell tumor of bone: clinical outcome prior to the introduction of molecular target therapy

Objective We analyzed the risk factors for pulmonary metastasis from giant cell tumor of bone and aimed to discuss their therapeutic strategy and appropriate follow-up period. Methods We analyzed 141 patients of giant cell tumor of bone. The variables analyzed included age, gender, primary site, Campanacci grading, surgical treatment on the primary lesion, radiotherapy and local recurrence. Results Pulmonary metastasis occurred in 12 patients. The risk factors were young age, Campanacci Grade III and local recurrence. Median time from initial surgery to metastasis was 1.3 years (0-3.1 years). Among them, eight patients experienced local recurrence of the primary tumor, and the median time from initial surgery to local recurrence was 0.8 years (0.3-2.9 years). Among seven patients who underwent wide resection, three patients showed local recurrence of the soft tissue. Nine patients underwent metastasectomy for pulmonary metastases. Of three patients who did not undergo metastasectomy, one patient died of uncontrollable metastases, and two patients showed no changes in their metastatic lesions. Conclusions Although we found a correlation between local recurrence and pulmonary metastasis, we were still unable to prevent local or metastatic recurrence by wide resection. Local recurrence and metastasis have been found within ~3 years after initial surgery, and routine image examinations of the primary site and chest after initial surgical treatment should be considered for at least 3 years postoperatively.

A new technique using mesh for extensor reconstruction after proximal tibial resection

BACKGROUND Proximal tibial reconstruction following wide resection in both malignant and benign tumors presents difficulties mainly due to both patellar tendon reconstruction and high risk of infection. The purpose of this study is to determine the efficacy of a new technique using a mesh for extensor reconstruction. METHODS We retrospectively reviewed nine consecutive patients who underwent resection of the proximal tibia with prosthetic reconstruction and reconstruction of the extensor using a mesh between 2009 and 2012. The surgical technique included the attachment of the mesh to the tibial component with a band of meshes looped over the patella and a gastrocnemius flap for coverage. RESULTS One patient had an above-the-knee amputation due to infection. Eight patients were followed up for 33 months (range, 20-50). In the eight patients, extensor lag had a mean of 5° (range, 0 to 20). Active flexion had a mean of 96.25° (range, 80 to 120) and ISOLS scores had a mean of 21/30 (range, 18 to 26). All patients were able to ambulate without crutches at the latest follow-up. CONCLUSION Extensor lag was significantly less compared to previous reports. No complications were observed in eight patients. Utilization of the mesh for extensor reconstruction after the proximal tibial resection is a simple, reliable and successful method.