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Purpose: Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissues are rare mesenchymal neoplasms, some of which are malignant. However, their clinical and pathological characteristics remain unclear. This study was performed to investigate the clinical and pathological characteristics of PEComas in bone and soft tissues by leveraging information from the Japanese Musculoskeletal Oncology Group. Methods: Ten patients, including four male and six female patients with a median age of 49 years, were retrospectively reviewed. PEComas of the visceral organs, including the uterus and retroperitoneum, were excluded. Results: Nine tumors arose in the soft tissue and one in the bone, with a mean size of 9.3 cm. Four patients showed local recurrence or distant metastasis. The 1-year survival rate was 77%. Pathologically, eight tumors were classified as malignant and two as having uncertain malignancy potential. Half of the tumors showed high MIB-1 index values of >30%. Immunohistochemically, the melanocyte marker HMB45 was expressed in 90% cases, and muscle-specific markers were expressed only in 30–50% cases. Transcription factor binding to IGHM enhancer 3 (TFE3) expression was positive in 89% of the patients. Tumors with high expression of TFE3 were classified as PEComas with malignant potential according to Folpe’s classification. Conclusions: Bone and soft tissue PEComas may have a higher malignancy potential than other visceral PEComas and are more likely to develop as TFE3-rearranged PEComas.

This study aimed to retrospectively analyze the clinical outcomes of patients with pelvic and retroperitoneal bone and soft tissue sarcoma (BSTS). Overall, 187 patients with BSTS in the pelvis and retroperitoneal region treated at 19 specialized sarcoma centers in Japan were included. The prognostic factors related to overall survival (OS), local control (LC), and progression-free survival (PFS) were evaluated. The 3-year OS and LC rates in the 187 patients were 71.7% and 79.1%, respectively. The 3-year PFS in 166 patients without any distant metastases at the time of primary tumor diagnosis was 48.6%. Osteosarcoma showed significantly worse OS and PFS than other sarcomas of the pelvis and retroperitoneum. In the univariate analyses, larger primary tumor size, soft tissue tumor, distant metastasis at the time of primary tumor diagnosis, P2 location, chemotherapy, and osteosarcoma were poor prognostic factors correlated with OS. Larger primary tumor size, higher age, soft tissue tumor, chemotherapy, and osteosarcoma were poor prognostic factors correlated with PFS in patients without any metastasis at the initial presentation. Larger primary tumor size was the only poor prognostic factor correlation with LC. This study has clarified the epidemiology and prognosis of patients with pelvic and retroperitoneal BSTS in Japan.

Background/aim This study aimed to evaluate the association of clinical characteristics with treatment outcomes to ascertain the appropriate treatment options for soft tissue sarcomas (STS) patients with brain metastasis (BM). Patients and methods Medical records of STS patients with BM who were treated in our institutions were retrospectively reviewed, and analyzed to identify the factors associated with post-BM survival. Results Among the 509 STS patients, BM occurred in five patients (0.98%). The median survival after BM was 1.5 months. Histological subtypes of the primary lesions in the five BM patients were: two synovial sarcomas, one myxoid liposarcoma, one alveolar soft part sarcoma, and one rhabdomyosarcoma. Among the five BM patients, the post-BM survival of two patients, who underwent surgery and postoperative radiotherapy, was longer than that of the other patients (p Conclusion Combined surgery and postoperative radiotherapy effectively managed symptoms and prolonged survival in STS patients with BM.

Soft tissue sarcomas (STSs) are rare heterogeneous malignancies of mesenchymal origin. Pulmonary metastases develop in approximately 50% of the patients with high-grade STS, being the major cause of mortality in patients with metastatic STS. Pulmonary metastasectomy has been reported to contribute to long-term survival; however, an appropriate treatment has not been established. We aimed to identify factors associated with post-metastasis survival in STS patients with pulmonary metastasis and determine the appropriate treatment for each patient. We retrospectively reviewed the records of metastatic STS patients treated between 2000 and 2017 and analyzed the clinico-pathologic variables to identify factors associated with the survival. The median survival after pulmonary metastasis was 20.6 months, and the 1-, 3-, and 5-year survival rates were 68.6%, 36.0%, and 25.1%, respectively. The survival was significantly greater in patients who underwent pulmonary metastasectomy than in those without surgery (38.9 months vs. 10.5 months; p < 0.0001). Among those who did not undergo surgery, the survival was significantly greater in patients who received chemotherapy than in those without chemotherapy (19.1 months vs. 6.3 months, p = 0.037). Multivariate analysis identified pulmonary metastasectomy as the most important prognostic factor for post-metastasis survival (Hazard ratio 5.623; 95% Confidence Interval 2.733-11.572; p < 0.0001). In conclusion, pulmonary metastasectomy was the most important prognostic factor for post-metastasis survival in patients with metastatic STS. In addition, chemotherapy could prolong survival in patients who were not eligible for pulmonary resection. Although we should carefully weigh the risks and benefits, appropriate treatment for pulmonary metastases could contribute to long-time survival.

Soft tissue sarcomas (STSs) are rare heterogeneous malignancies of mesenchymal origin. Pulmonary metastases develop in approximately 50% of the patients with high-grade STS, being the major cause of mortality in patients with metastatic STS. Pulmonary metastasectomy has been reported to contribute to long-term survival; however, an appropriate treatment has not been established. We aimed to identify factors associated with post-metastasis survival in STS patients with pulmonary metastasis and determine the appropriate treatment for each patient. We retrospectively reviewed the records of metastatic STS patients treated between 2000 and 2017 and analyzed the clinico-pathologic variables to identify factors associated with the survival. The median survival after pulmonary metastasis was 20.6 months, and the 1-, 3-, and 5-year survival rates were 68.6%, 36.0%, and 25.1%, respectively. The survival was significantly greater in patients who underwent pulmonary metastasectomy than in those without surgery (38.9 months vs. 10.5 months; p 0.0001). Among those who did not undergo surgery, the survival was significantly greater in patients who received chemotherapy than in those without chemotherapy (19.1 months vs. 6.3 months, p = 0.037). Multivariate analysis identified pulmonary metastasectomy as the most important prognostic factor for post-metastasis survival (Hazard ratio 5.623; 95% Confidence Interval 2.733-11.572; p 0.0001). In conclusion, pulmonary metastasectomy was the most important prognostic factor for post-metastasis survival in patients with metastatic STS. In addition, chemotherapy could prolong survival in patients who were not eligible for pulmonary resection. Although we should carefully weigh the risks and benefits, appropriate treatment for pulmonary metastases could contribute to long-time survival.

Clear cell sarcoma of tendons and aponeuroses (CCS) is a rare, malignant tumor arising in lower extremities with no effective treatment other than wide surgical resection. Here described is a case of primary CCS in the peroneal tendon of the right foot of a 54-year-old woman enrolled to undergo BNCT. The tumor mass post-BNCT disappeared totally without damage to other normal tissue, demonstrating, for the first time, the potential efficacy of BNCT in complete local control of CCS.

Synovial sarcoma is a rare tumor requiring new treatment methods. A 46-year-old woman with primary monophasic synovial sarcoma in the left thigh involving the sciatic nerve, declining surgery because of potential dysfunction of the affected limbs, received two courses of BNCT. The tumor thus reduced was completely resected with no subsequent recurrence. The patient is now able to walk unassisted, and no local recurrence has been observed, demonstrating the applicability of BNCT as adjuvant therapy for synovial sarcoma. Further study and analysis with more experience accumulation are needed to confirm the real impact of BNCT efficacy for its application to synovial sarcoma.

Clear cell sarcoma of tendons and aponeuroses (CCS) is a rare malignant tumor with no effective treatment. This study demonstrates the efficacy of BNCT with the use of human CCS-bearing nude mice. Groups A and C were administered saline, and groups B and D were injected with p-borono- l -phenylalanine-fructose complex. Groups C and D were then irradiated with thermal neutrons. The tumors in only group D disappeared, demonstrating that BNCT is a potentially new option for the treatment of human CCS.

Sphingolipids are ubiquitous components of cell membranes. Their metabolites ceramide, sphingosine, and sphingosine-1-phosphate (S1P) have important physiological functions, including regulation of cell growth and survival. S1P is generated by phosphorylation of sphingosine catalyzed by sphingosine kinase-1 (SPHK1). The purpose of this study is to explore the roles of S1P, S1P receptors, and sphingosine kinases in malignant musculoskeletal tumors. Twenty-one tumor samples (7 liposarcomas, 3 chondrosarcomas, 6 osteosarcomas, 5 MFH) obtained at open biopsy, and four human MFH cell lines (Nara H, Nara F, TNMY1, GBS-1) were used. We examined the mRNA expression of S1P receptors by RT-PCR, and the expression levels of SPHK by Real-time PCR. We used 4 MFH cell lines to analyze SPHK1 proteins by Western blotting. SPHK1 siRNA was transfected into MFH cell lines by lipofection method. Cell proliferation (control and transfected with siRNA) was assayed using WST-8 (Cell Counting Kit-8) assay. All high grade malignant tumors expressed S1P1, S1P2, S1P3 receptors, whereas the expression of S1P1 receptor was detected in 50% of low-grade malignant tumors, S1P2 receptor in 30%, and S1P3 in 50%. No statistically significant difference was found in the expression level of SPHK1 between high-grade and low-grade malignant tumors by Real-time PCR. By results of Western blotting, proteins of SPHK1 were expressed in all MFH cell lines. In MFH cell lines, transfection with SPHK1 siRNA oligonucleotides resulted in approximately 50 to 80% suppression of SPHK1 mRNA expression as determined by real-time PCR. Down-regulation of SPHK1 with small interfering RNA significantly reduced SPHK1 protein levels by Western blotting. Knock down of SPHK1 expression significantly decreased cell proliferation of all MFH cells. These results suggest that the expression of S1P receptors may play an important role for cell proliferation and may correlate with histologic grade in malignant bone and soft tissue tumors, and that SPHK1 may be one of essential molecules for cell proliferation in MFH cell lines.

Clear cell sarcoma (CCS) is a rare malignant tumor with a poor prognosis. In the present study, we established a lung metastasis animal model of CCS and investigated the therapeutic effect of boron neutron capture therapy (BNCT) using p-borono-L-phenylalanine (L-BPA). Biodistribution data revealed tumor-selective accumulation of (10)B. Unlike conventional gamma-ray irradiation, BNCT significantly suppressed tumor growth without damaging normal tissues, suggesting that it may be a potential new therapeutic option to treat CCS lung metastases.

Purpose: Transforming growth factor β (TGF-β) is a multifunctional growth factor that variably affects proliferation, differentiation, and extracellular matrix formation. Little information is currently available on the TGF-β expression in malignant fibrous histiocytoma (MFH). The aims of the present study were to investigate the expression of TGF-β isoforms and their receptors in human MFH specimens. Experimental Design: The expression of TGF isoforms, and TGF-β receptors (TGF-βR1 and -βR2) were immunohistochemically evaluated in 43 paraffin-embedded MFH specimens. Furthermore, the correlation of the TGF-β and receptor expression with tumor proliferative activity assessed by MIB-1 indices was analyzed. Results: Positive immunoreactivity for TGF-β1, -β2, and -β3 was identified in tumor cells of 42, 40, and 38 of the 43 MFHs, respectively. In each TGF-β isoform immunostaining, the specimens were divided into two groups based on the number of positive tumor cells: those with low ( Conclusions: These findings strongly suggest an association of the TGF-β ligand/receptor system with a significantly higher MIB-1 index in human MFHs. Investigation of the TGF-βR1 and -βR2 coexpression might be useful in predicting tumor behavior of MFHs.

A solitary fibrous tumor is a relatively unusual neoplasm first described as a distinctive tumor arising from pleura. Some reports have shown that solitary fibrous tumors also affect extrathoracic regions. The current study presents a literature review with four additional patients with solitary fibrous tumor arising from the extremities to clarify clinicopathologic features. The current four patients were two males and two females, ranging from 17 to 60 years of age. Magnetic resonance imaging scans of the current patients showed inhomogeneous low to intermediate intensity signal on T1-weighted images and inhomogeneous intermediate to high intensity signal on T2-weighted images. Histologically, the tumors were composed of a haphazard proliferation of spindle cells, although cellularity was variable in each case. Two of the four tumors showed hypercellularity of spindle cells with focally myxomatous or hyaline changes, whereas myxomatous patterns with scattered spindle cells throughout the specimens were observed in the other two tumors. Immunohistochemically, all four patients showed positive immunoreactivity for CD34, and two tumors showed focally positive immunoreactivity for bcl-2 protein. During the followup of 12 to 54 months, neither local recurrence nor distant metastasis was detected after wide resection. Examination of the literature and the current patients suggests that solitary fibrous tumors in the extremities are likely to have a malignant potential, although most patients have a benign clinical course. Local wide resection and careful long-term followup are necessary for patients with solitary fibrous tumor in the extremities.

A patient with bone marrow edema syndrome of the hip associated with a uterine myoma is presented. A 51-year-old woman could not walk because of severe pain in both hips and had been referred to the authors' institute. Magnetic resonance imaging scans showed abnormal intensity on T1- and T2-weighted images in both femoral heads and a large mass arising from the uterus which was diagnosed as a uterine myoma. A 99m Tc-methylene diphosphonate scintigraph showed diffuse uptake in both femoral heads. The pain in both hips decreased shortly after a hysterectomy and the patient could walk without crutches within 2 weeks after the gynecologic surgery. Magnetic resonance imaging scans taken 8 months after surgery showed high signal intensity on T1- and T2-weighted images, indicating normal bone marrow in the femoral heads. To the authors' knowledge, this is the first case report showing a bone marrow edema syndrome of the hip associated with uterine myoma. The pathophysiologic mechanisms for bone marrow edema syndrome of the hip in the current patient and in pregnancy may be identical. More specifically, a large intrapelvic mass may cause an increase of intrapelvic pressure and subsequent blood stasis in both conditions. The current case suggests the possible factors of bone marrow edema syndrome of the hip which need to be investigated.

Malignant peripheral nerve sheath tumors (MPNST) are relatively rare neoplasms with poor prognosis. At present there is no effective treatment for MPNST other than surgical resection. Nonetheless, the anti-tumor effect of boron neutron capture therapy (BNCT) was recently demonstrated in two patients with MPNST. Subsequently, tumor-bearing nude mice subcutaneously transplanted with a human MPNST cell line were injected with p-borono-L-phenylalanine (L-BPA) and subjected to BNCT. Pathological studies then revealed that the MPNST cells were selectively destroyed by BNCT.

This report presents a case of neural fibrolipoma arising from the superficial peroneal nerve in the ankle. A 28-year-old woman was referred with a soft tissue mass in the anterior aspect of the right ankle, which had been gradually enlarging for the past 10 years. Magnetic resonance imaging showed a mass lesion, measuring approximately 8 x 3 x 2 cm, with high to partially low signal intensity on both T1- and T2-weighted images. A band of low signal intensity within the lesion, which is indicative of coexistence with the tumor and the superficial peroneal nerve, could be detected on both T1- and T2-weighted images. The patient underwent an excisional biopsy. The specimen microscopically consisted of nerve bundles and fibro-fatty proliferation with abundant collagen fibers. Immunoreactivity for CD34 antigen antibody was detected in fibrous spindle cells. This is the first report to present an immunohistochemical profile of neural fibrolipoma. Neural fibrolipoma should be considered as a differential diagnosis when a lipomatous lesion is encountered in the foot or ankle as well as in the upper extremities.

Study design A case of multicentric osteosarcoma presenting with sacral radiculopathy is reported. Objective To present unusual clinical and radiologic findings of multicentric osteosarcoma. Summary of background data Multicentric osteosarcoma is a rare variant of osteosarcoma. To the authors' knowledge, no cases of multicentric osteosarcoma presenting as sacral radiculopathy have been reported previously. Methods A 14-year-old boy had a large sacral tumor extending into the spinal canal, which was found to account for the initial symptoms, which mimicked those of herniated nucleus pulposus. At diagnosis, a bone survey showed multiple foci of osteosarcoma in the long bones. Results The patient was treated with chemotherapy, but died of the disease 8 months after the initial presentation. Conclusion Multicentric osteosarcoma should be considered in the differential diagnosis for a pediatric patient with low back pain and sciatica.

The existence of an anterior cruciate ligament-hamstring reflex arc, the extent to which these reflexes can protect the knee, and the extent to which they are affected by rupture of the anterior cruciate ligament remain controversial. We evaluated the temporal components of the anterior cruciate ligament-hamstring synergy by simulating an injury to the ligament in a goat model. Reflexive hamstring activation in anesthetized goats was evaluated when the anterior cruciate ligament was loaded with static subfailure, dynamic subfailure, and dynamic failure loads. Reflexive hamstring activation was not found in response to static subfailure loading but was observed in response to dynamic subfailure and failure loading. The latency of the reflex evoked by dynamic failure loading was shorter than that evoked by dynamic subfailure loading. The findings suggest that the extent to which the hamstring reflexes can protect the knee may be bounded by the ability of these muscles to generate force rapidly and the amplitude and time-course of the loads applied to the knee joint. The present data present a framework for further investigation of the contribution of anterior cruciate ligament-hamstring reflexes to the stability of the knee joint under high loads and loading rates.

Optical absorption of ammonium dihydrogen phosphate (ADP) crystals doped with thallous ions is studied in comparison with those of potassium dihydrogen phosphate (KDP) and rubidium dihydrogen phosphate (RDP) crystals doped with thallous ions. It is experimentally confirmed that the ferroelectric dipole field in KDP and RDP is responsible for the clear doublet structure of the A xy bands of Tl + centers below the ferroelectric transition temperatures.

Fluorescence excitation spectroscopy at both vibrational and rotational resolution has been used to probe the changes in energy, electronic distribution, and geometry that occur when 1‐aminonaphthalene (1AN) absorbs light at ∼332 nm. The 000 band of the S1←S0 transition of 1AN is red shifted by nearly 2000 cm−1 with respect to the corresponding band of naphthalene. Additionally, it is mainly b‐axis polarized, unlike the corresponding bands of naphthalene and other 1‐substituted naphthalenes. Thus, 1La/1Lb state reversal occurs on 1‐substitution of naphthalene with an NH2 group. The S0 state of 1AN is pyramidally distorted at the nitrogen atom. Additionally, the NH2 group is rotated by ∼20° about the C–NH2 bond. Excitation of 1AN to the zero‐point vibrational level of its S1 state reduces the C–NH2 bond length by ∼0.2 A and flattens the NH2 group along both out‐of‐plane coordinates. Other vibronic bands in the S1←S0 transition exhibit significantly different rotational constants, inertial defects, and tran...

The most common types of tarsal coalition are talocalcaneal and calcaneonavicular coalition. This report presents a case of a patient with middle foot pain who was diagnosed as having first cuneometatarsal coalition.

Effects of a noncubic crystal field on the ${\mathit{s}}^{2}$ ion are investigated. Optical-absorption spectra of thallous ion centers produced in single crystals of ${\mathrm{KH}}_{2}$${\mathrm{PO}}_{4}$ (KDP) and ${\mathrm{RbH}}_{2}$${\mathrm{PO}}_{4}$ (RDP) are presented. They consist of polarized absorption bands. Since it is confirmed that the ${\mathrm{Tl}}^{+}$ centers in KDP and RDP exhibit quite similar absorption characteristics, a model is proposed to interpret the interaction mechanisms between the ${\mathit{s}}^{2}$ ions and the KDP-like crystal lattices. Among five typical absorption bands named ${\mathit{A}}_{\mathit{z}}$, ${\mathit{A}}_{\mathit{x}\mathit{y}}$, ${\mathit{B}}_{\mathit{x}\mathit{y}}$, ${\mathit{C}}_{\mathit{x}\mathit{y}}$, and ${\mathit{C}}_{\mathit{z}}$, the ${\mathit{A}}_{\mathit{x}\mathit{y}}$ band has a noticeable doublet structure and the ${\mathit{B}}_{\mathit{x}\mathit{y}}$ band is as intense as the ${\mathit{A}}_{\mathit{x}\mathit{y}}$ band even at liquid-nitrogen temperature and is strongly temperature dependent. The observed results are qualitatively explained by taking into account the spin-orbit, crystal-field, and electronic-vibrational interactions.

Study Design. A case of acute low back pain caused by pseudogout attack of the lumbar facet joint is reported. Objective. To report a new cause of acute low back pain previously unreported in the literature. Summary of Background Data. There have been some reports of lumbar spinal stenosis caused by calcium pyrophosphate dihydrate crystal deposition. However, there are no known reports of pseudogout attack of the lumbar facet joint. Methods. An axial magnetic resonance imaging scan demonstrated joint effusion at the level of the bilateral L4–L5 facet joint. Aspiration of the left L4–L5 facet joint yielded 1.5 mL of pus-like synovial effusion. Multiple cultures of synovial fluid tested negative for bacteria and fungi, whereas compensated polarized light microscopy revealed monoclinic or triclinic crystals with a positive birefringence. Results. The symptoms of acute low back pain lasted for 3 days. Local and systemic inflammatory signs, as well as symptoms, gradually improved after joint aspiration. A follow-up evaluation 8 months after lumbar facet joint aspiration showed complete resolution of pain and no neurologic deficit. Conclusions. Pseudogout attack of the lumbar facet joint is rare, but this clinical entity should be added to the differential diagnosis of acute low back pain.

Clear cell sarcoma (CCS) is a rare melanocytic malignant tumor with a poor prognosis. Our previous study demonstrated that in vitro cultured CCS cells have the ability to highly uptake l-BPA and thus boron neutron capture therapy could be a new option for CCS treatment. This paper proved that a remarkably high accumulation of (10)B (45-74 ppm) in tumor was obtained even in a CCS-bearing animal with a well-controlled biodistribution followed by intravenous administration of L-BPA-fructose complex (500 mg BPA/kg).

Clear cell sarcoma (CCS), a rare malignant tumor with a predilection for young adults, is of poor prognosis. Recently however, boron neutron capture therapy (BNCT) with the use of p-borono-L-phenylalanine (BPA) for malignant melanoma has provided good results. CCS also produces melanin; therefore, the uptake of BPA is the key to the application of BNCT to CCS. We describe, for the first time, the high accumulation of boron in CCS and the CCS tumor-bearing animal model generated for BNCT studies.

Nodular fasciitis is a rapidly growing mass, with high cellularity and mitotic activity, that can be both clinically and histologically misdiagnosed as a soft tissue sarcoma. Nodular fasciitis of the hand is an extremely rare condition. We report a 17-year-old male hand-ball player with nodular fasciitis in the dominant hand. The patient presented with a rapidly growing mass in his right hand and no history of major trauma. On physical examination, a painful mass measuring 2 cm in diameter was observed in the first web space. Magnetic resonance imaging (MRI) demonstrated a subcutaneous mass with isointensity on T1-weighted images and inhomogeneous high intensity on T2-weighted images. The lesion was inhomogeneously enhanced after intravenous administration of gadolinium. Moreover, thallium-201 scintigraphy showed high uptake at the early phase and no wash-out at the delayed phase. We performed an excisional biopsy. The mass was present subcutaneously and adhered to the interosseous muscle fascia. Although a pathological examination by frozen section during surgery showed a low-grade spindle cell sarcoma, the final histological diagnosis was nodular fasciitis. There was no evidence of local recurrence at the recent follow-up 2 years after the operation. We speculate that repeated small injuries as a result of sports activities played an important causative role in the nodular fasciitis.

Neutron-capture therapy with gadolinium (Gd-NCT) has therapeutic potential, especially that gadolinium is generally used as a contrast medium in magnetic resonance imaging (MRI). The accumulation of gadolinium in a human sarcoma cell line, malignant fibrosis histiocytoma (MFH) Nara-H, was visualized by the MRI system. The commercially available MRI contrast medium Gd-DTPA (Magnevist®, dimeglumine gadopentetate aqueous solution) and the biodegradable and highly gadopentetic acid (Gd-DTPA)-loaded chitosan nanoparticles (Gd-nanoCPs) were prepared as MRI contrast agents. The MFH cells were cultured and collected into three falcon tubes that were set into the 3-tesra MRI system to acquire signal intensities from each pellet by the spin echo method, and the longitudinal relaxation time (T1) was calculated. The amount of Gd in the sample was measured by inductively coupled plasma atomic emission spectrography (ICP-AES). The accumulation of gadolinium in cells treated with Gd-nanoCPs was larger than that in cells treated with Gd-DTPA. In contrast, and compared with the control, Gd-DTPA was more effective than Gd-nanoCPs in reducing T1, suggesting that the larger accumulation exerted the adverse effect of lowering the enhancement of MRI. Further studies are warranted to gain insight into the therapeutic potential of Gd-NCT.

We present a rare case of stress fracture of the second metacarpal bone. A 14-year-old girl felt pain on the dorsal aspect of the right wrist without any history of major trauma, when she played a smash during a game of badminton. On the radiographs, periosteal reaction was detected on the ulnar aspect of the base of the second metacarpal bone. She was treated conservatively and she returned to the original activity level.

Unsymmetrical fluoran dyes whose coloring-to-decoloring reversible reaction depends upon the acidity are used in heat-Sensitive recording systems. The reversible reaction corresponds to open-to-closed forms of the lactone ring. We observed remarkable chemical shift differences in 4 species of 13C NMR signals between open- and closed-forms of the lactone ring of an unsymmetrical fluoran dye, 3-diethylamino-6-methyl-7-chlorofluoran (DEAMCF) in CDCl3. According to the optimized molecular structure of DEAMCF by a semi-empirical molecular orbital (MO) Austin Model 1(AM 1) method, the xanthene moiety in colorless form is folded at 166 degree around the C(spiro)-O(xanthene) axis, and the benzolactone moiety is almost perpendicular to the xanthene moiety. The moiety in color form for a molar DEAMCF/H2SO4 ratio of 1/2.2 becomes almost planar and has canonical structure, while the lactone ring opens, according to NMR and MO analyses.

Synovial osteochondromatosis arising in the foot is a rare condition. We report a 69-year-old woman with synovial osteochondromatosis of the Lisfranc joint. The patient presented with a 10-year history of left foot pain. Imaging studies showed multiple calcified masses around the Lisfranc joint. We performed a synovectomy and removal of the loose bodies in the dorsal, lateral, and plantar aspects of the Lisfranc joint by dislocating the bases of the fourth and fifth metatarsal bones. To our knowledge, this is only the second case report of synovial osteochondromatosis involving the Lisfranc joint.

Malignant fibrous histiocytoma (MFH) is one of the most common high-grade sarcomas in bone and soft tissue and, due to its chemo-resistance, the prognosis of the disease is poor. ST1571 is a tyrosine kinase inhibitor that was initially developed as a BCR/ABL inhibitor for chronic myeloid leukemia patients. STI571 also selectively inhibits platelet-derived growth factor receptors (PDGFRs) and c-kit. We examined the expression of PDGFRs and c-kit in human MFH cell lines, and the effect of STI571 on cell proliferation.Four human MFH cell lines (TNMY1, GBS-1, Nara-F and Nara-H) were used. mRNA expression of the receptor tyrosine kinases (PDGFRs and c-kit) was analyzed using reverse transcription-polymerase chain reaction, and the inhibitory effect of STI571 on cell proliferation was analyzed using the MTS assay technique.PDGFRalpha mRNA was expressed in TNMY1 and GBS-1, and PDGFRbeta and c-kit mRNAs were expressed in TNMY1, GBS-1 and Nara-F. All three of these mRNAs were absent in Nara-H. STI571 inhibited cell proliferation of TNMY1, GBS-1 and Nara-F in a dose- and time-dependent manner, but cell proliferation of Nara-H was not inhibited by ST1571 at concentrations of 10 microM or less.STI571 significantly inhibited proliferation of the three human MFH cell lines that expressed mRNAs of target receptor tyrosine kinases. The inhibitory effect of ST1571 on cell proliferation in these three cell lines might be due to decreased tyrosine kinase activity. STI571 might be a potent chemotherapeutic agent for human MFHs.

Heparin-binding epidermal growth factor (HB-EGF), betacellulin (BTC) and epiregulin (EPR) are members of the EGF system and involved in the cell growth of various epithelial malignancies. There have been no reports on the HB-EGF, BTC and EPR expression in mesenchymal malignancies of fibrohistiocytic origin including malignant fibrous histiocytoma (MFH).We investigated the expression of HB-EGF, BTC, EPR and EGF-receptor (EGF-R) in 43 human MFH tissue samples using immunohistochemical techniques.Positive immuno-reactivity for HB-EGF, BTC, EPR and EGF-R was identified in 28 (65%), 7 (16%), 43 (100%) and 36 (84%) out of the 43 MFH cases analyzed, respectively. Coexpression of HB-EGF/BTC, BTC/EPR and HB-EGF/EPR was observed in 6 (14%), 7 (16%) and 28 (65%) of the MFHs, respectively. Coexpression of HB-EGF/EGF-R, BTC/EGF-R and EPR/EGF-R was observed in 25 (58%), 6 (14%) and 36 (84%) of the MFHs, respectively.These results revealed that HB-EGF, BTC and EPR are expressed not only by epithelial tumor cells, but also by MFH cells. It is suggested that HB-EGF and EPR might be more important tumor growth regulators of MFH through autocrine or paracrine pathways, when compared with BTC.

Hepatocyte growth factor (HGF) is a multifunctional cytokine that variably affects cell motility, proliferation, and morphogenesis. Little information is currently available on the HGF and its receptor c-Met expression in malignant fibrous histiocytoma (MFH). We immunohistochemically investigated the HGF and c-Met expression in 43 MFH tissue specimens. Furthermore, the correlation of the HGF and c-Met expression with tumor proliferative activity assessed by MIB-1 indices was analyzed. Our results showed that positive cytoplasmic immunoreactivity for HGF and c-Met was identified in tumor cells in 36 (84%) and 20 (47%) of the 43 MFH cases analyzed, respectively. Coexpression of HGF and c-Met was observed in 20 (47%) of the 43 MFHs, and was correlated with high MIB-1 proliferative indices (p = 0.0446). These findings strongly indicate that the HGF/c-Met signaling system plays an important role in promoting cell proliferation of human MFHs via an autocrine loop.

Ganglia within the posterior tibial nerve is a rare condition. The authors report 2 cases of epineural ganglion of the posterior tibial nerve, causing tarsal tunnel syndrome. Both cases presented with numbness on the plantar surface of the foot. Magnetic resonance imaging showed the presence of the cyst within the tarsal tunnel. During surgery, these cysts were found within the epineurium of the posterior tibial nerve and were successfully removed without damage to nerve fibers. Both patients were free of symptoms after surgery. Ganglion cysts in the peripheral nerve are either intrafascicular or epineural. Intrafascicular ganglia present beneath the epineurium and involve the nerve fibers, whereas epineural ganglia are located in the epineurium and do not involve the nerve fibers. A review of the literature discusses these concepts. The authors suggest that epineural ganglion should be clinically distinctive from an intrafascicular ganglion because of the differences in surgical treatment, postoperative nerve function, and the recurrence rate.

Amphiregulin is a member of the epidermal growth factor (EGF) system and a potent mitogen for various epithelial tissues. Little information, however, is currently available on the amphiregulin and EGF receptor (EGF-R) expression in mesenchymal malignancies of a fibrohistiocytic origin including malignant fibrous histiocytoma (MFH).We investigated the amphiregulin and EGF-R expression in 43 human MFH tissues using immunohistochemical techniques. Furthermore, the correlation of the ligand and the receptor expression with tumor proliferative activity assessed by MIB-1 indices was analyzed.Positive immunoreactivity for amphiregulin and EGF-R was identified in 34 (79%) and 36 (84%) of the 43 MFH cases analyzed, respectively. Coexpression of amphiregulin and EGF-R was observed in 30 (70%) of the 43 MFHs analyzed. There were no significant differences in MIB-1 indices between both the amphiregulin and EGF-R-positive MFHs and the remaining MFHs.These results show that amphiregulin is expressed not only by epithelial tumor cells but also by MFH cells. Our data provide evidence indicating the presence of an autocrine mechanism of proliferation control involving the amphiregulin/EGF-R signaling system in human MFHs.

Little information is available regarding the expression of platelet-derived growth factor (PDGF) isoforms and their receptors in soft tissue malignant fibrous histiocytoma (MFH).We investigated expression of PDGF isoforms and their receptors (PDGF-R alpha and -R beta) in 43 MFH tissue specimens using immunohistochemical techniques. Furthermore, we examined the correlation of PDGF expression in MFHs with proliferative activity assessed by MIB-1 immunohistochemical staining.Positive cytoplasmic immunoreactivity for PDGF-AA, -BB and -AB was identified in tumor cells of 28 (66%), 4 (10%) and 26 (61%), respectively, of the 43 MFHs analyzed. Positive cytoplasmic immunoreactivity for PDGF-R alpha and -R beta was identified in tumor cells of 41 (95%) and 32 (74%), respectively, of the MFHs. Thirty-four (79%) MFHs coexpressed one or more PDGF isoforms and their corresponding receptors. In PDGF-AA immunostaining, MIB-indices in the high immunoreactivity group (10% of tumor cells) were significantly higher than those in the low immunoreactivity group (10% of tumor cells) (p = 0.031).Our data provide evidence to support the presence of an autocrine/paracrine mechanism of proliferation control involving the PDGF ligand/receptor system in human MFHs.

Little information is available on the expression of stem cell factor (SCF) and its receptor c-kit in soft tissue tumors of a fibrohistiocytic origin, including malignant fibrous histiocytoma (MFH).We investigated the endogenous expression of SCF and c-kit in 43 MFH tissue samples using immunohistochemical techniques. Furthermore, we examined the correlation of SCF expression in MFHs with proliferative activity assessed by mitotic indices and MIB-1 immunohistochemical staining.Positive immunoreactivity for c-kit was identified in tumor cells of only one MFH case, while the remaining 42 cases were negative. In the one positive case, immunohistochemical staining was focal. Positive immunoreactivity for SCF was identified in 31 out of 43 cases studied (72%, focal; 11, moderate; 6, diffuse; 14). There were no significant differences in the MIB-1 and mitotic indices between the SCF-positive and negative groups.Our data indicate that any direct autocrine effects of the SCF/c-kit system on cell growth regulation are precluded in most MFH cases studied, but it is speculated that SCF might indirectly influence tumor growth by promoting local neovascularization.

The expression of c-kit and/or its ligand, stem cell factor (SCF), has been related to tumor proliferation, in several tumor systems.We analyzed the expression of the SCF/its receptor (c-kit) mRNA and the production of soluble SCF in a human malignant fibrous histiocytoma (MFH) cell line (TNMY1).Immunocytochemical analysis revealed that the TNMY1 cells were positive for both SCF and c-kit. Reverse transcriptase-polymerase chain reaction (RT-PCR) showed that the TNMY1 cell line expressed mRNA for SCF and c-kit. By using an enzyme-linked immunosorbent assay (ELISA), the TNMY1 cells were found to produce relatively high amounts of soluble SCF. However, the addition of soluble SCF to the TNMY1 cells did not alter the in vitro growth ability of the cells.Our data showed that the MFH cells produced consistent amounts of SCF but did not demonstrate autocrine growth modulation. Thus, SCF secretion may have a paracrine activity in the growth of MFH cells.

Osteoblastomas located on the surface of the cortical bone, so-called periosteal osteoblastomas, are extremely rare. We report on a case of periosteal osteoblastoma arising from the posterior surface of the right distal femur in a 17-year-old man. Roentgenographic, computed tomographic, magnetic resonance imaging, and histologic features of the case are presented. Periosteal osteoblastoma should be radiologically and histologically differentiated from myositis ossificans, avulsive cortical irregularity syndrome, osteoid osteoma, parosteal osteosarcoma, periosteal osteosarcoma, and high-grade surface osteosarcoma. Although periosteal osteoblastoma is rare, this tumor should be included in the differential diagnosis of surface-type bone tumors.

Ischiogluteal bursitis is a rare, infrequently recognized condition. Two cases of ischiogluteal bursitis in an 80-year-old man and a 75-year-old woman are presented. Both patients presented with a painful buttock mass. Contrast-enhanced multiplanar reconstruction computed tomography scans revealed a lesion with peripheral enhancement inferior to the ischial tuberosity. Magnetic resonance imaging revealed a lesion with low or intermediate signal intensity on T1-weighted images. T2-weighted images revealed a lesion with high signal intensity. Postcontrast magnetic resonance imaging scans revealed peripheral enhancement of the lesion. The multimodality imaging findings of ischiogluteal bursitis have not been described previously. Ischiogluteal bursitis should be considered in the differential diagnosis of a buttock mass.

Introduction Anatomical abnormalities in the lower limb vessels are uncommon. However, the preoperative evaluation of the anatomical variations is very important for planning the operation procedure to prevent jeopardizing the donor leg. Case presentation In this case report, a 23-year-old Asian woman who was scheduled to have vascularized free fibula transplantation for reconstruction of her wrist after excision of bone tumor in her distal radius, was found to have congenital aplastic posterior tibial arteries in both legs. These findings were found on magnetic resonance angiography (our preferred methodology due to its simplicity). We planned testing the sufficiency of her pedal pulses after temporarily clamping her peroneal artery but prior to harvesting, to ensure minimal risk to the longevity of her donor leg. During the operation, after dissection of a 10cm segment of her fibula with the peroneal artery, the peroneal artery proximal to the graft was temporarily clamped and the tourniquet was released. As adequate sustainable pedal pulses were confirmed, the graft was harvested and transplanted to her wrist. There was no morbidity in her right leg postoperatively and the union of the grafted fibula was substantiated 10 months postoperatively. Conclusions We concluded two findings: firstly, for accurate preoperative planning of a vascularized free fibula procedure, examination of the bilateral lower leg vasculature either by angiography or other imaging should be performed. Secondly, abnormalities are not in themselves reason to abandon the vascularized free fibula procedure. We contend that pedal pulses should be evaluated preoperatively and provided that adequate foot circulation can be confirmed (by temporarily clamping the vessels and releasing the tourniquet during the operation prior to harvesting the free vascularized fibula) the procedure should be successful without jeopardizing the donor leg.

The existence of an anterior cruciate ligament-hamstring reflex arc, the extent to which these reflexes can protect the knee, and the extent to which they are affected by rupture of the anterior cruciate ligament remain controversial. We evaluated the temporal components of the anterior cruciate ligament-hamstring synergy by simulating an injury to the ligament in a goat model. Reflexive hamstring activation in anesthetized goats was evaluated when the anterior cruciate ligament was loaded with static subfailure, dynamic subfailure, and dynamic failure loads. Reflexive hamstring activation was not found in response to static subfailure loading but was observed in response to dynamic subfailure and failure loading. The latency of the reflex evoked by dynamic failure loading was shorter than that evoked by dynamic subfailure loading. The findings suggest that the extent to which the hamstring reflexes can protect the knee may be bounded by the ability of these muscles to generate force rapidly and the amplitude and time-course of the loads applied to the knee joint. The present data present a framework for further investigation of the contribution of anterior cruciate ligament-hamstring reflexes to the stability of the knee joint under high loads and loading rates.

A speech model preparing method capable of easily preparing a new Hidden Marcov Model (HMM) of an input speech with a very small number of utterances such as once or twice , and a speech recognition apparatus using this method. A speech recognition apparatus uses, as a speech model, a continuous distribution type HMM defined by three parameters of a state transition probability, an average vector and a variance. The apparatus computes (7) an average vector of an input speech to be learned, selects an HMM approximate to the input to-be-learned speech as an initial model from a registration dictionary (3), replaces at least an average vector of the selected HMM with the computed average vector of the to-be-learned speech and adds an obtained HMM as an HMM for the input to-be-learned speech in the dictionary (3).