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Biomedical subjects

Hirokazu Chuman

Publications and source records attributed to Hirokazu Chuman.

13 recordsLinked to original sources

Solitary intramuscular myxoma with monostotic fibrous dysplasia as a rare variant of Mazabraud's syndrome.

The rare coexistence of intramuscular myxoma (IM) and fibrous dysplasia (FD) is known as Mazabraud's syndrome. IM tends to occur multifocally and is associated most frequently with polyostotic FD in Mazabraud's syndrome. We present an extremely rare combination of a solitary IM and monostotic FD as a variant of Mazabraud's syndrome, and discuss the importance of recognizing this rare coexistence for appropriate management of the patient.

Biopsy↗

A case of resected huge ileocolonic mesenteric liposarcoma which responded to pre-operative chemotherapy using doxorubicin, cisplatin and ifosfamide.

Primary mesenteric liposarcoma is a rare entity that has been reported only 14 times in English literature. The treatment strategy for mesenteric liposarcoma is, if no distant metastases are detected, surgical resection with a wide surgical margin, often followed by radiation and/or adjuvant chemotherapy for high-risk patients. However, the efficacy of pre-operative chemotherapy is unknown. If the tumor is shrunk by pre-operative chemotherapy, we could achieve complete surgical resection, which is difficult when the tumor is too large or is invading neighboring organs. We herein describe a case of huge mesenteric liposarcoma that showed significant tumor shrinkage by pre-operative chemotherapy using doxorubicin, cisplatin and ifosfamide, allowing a margin-negative operation.

Adult↗

Cyclooxygenase-2 overexpression associated with a poor prognosis in chondrosarcomas.

Recent studies have shown increased levels of cyclooxygenase (COX)-2 in various human malignancies, including some bone and soft tissue tumors, but little is known about the presence of COX-2 in chondrosarcomas. We performed immunohistochemical staining for COX-2 in 74 chondrosarcomas and compared the staining results with the characteristics and outcome of the patients. Thirty-seven men and 37 women between the ages of 14 and 81 years (median, 50 years) participated in the study. The tumors were located in the axial skeleton in 47 cases and in the long bones in 27 cases. The largest diameters of the tumors ranged from 2.7 to 32 cm (median, 8.0 cm). The immunohistochemistry findings revealed the overexpression of COX-2 in 16 (22%) of the 74 patients. Thirteen (41%) of 32 grade 2 and 2 (67%) of 3 grade 3 chondrosarcomas showed overexpression for COX-2, whereas only 1 (3%) of 39 grade 1 chondrosarcomas showed overexpression. Overexpression of COX-2 was significantly associated with histologic grade (P < .001) and a decreased disease-specific survival (P < .001). These findings suggest that COX-2 overexpression in chondrosarcoma is a negative prognostic factor that may be strongly associated with histologic grade.

Adolescent↗

Interobserver variability in histologic recognition, interpretation of KIT immunostaining, and determining MIB-1 labeling indices in gastrointestinal stromal tumors and other spindle cell tumors of the gastrointestinal tract.

The authors studied the concordance among seven pathologists for the histologic diagnosis, interpretation of KIT immunostaining, and determining MIB-1 labeling indices (LI) in 80 adult patients with primary spindle cell tumors, mainly of the gastrointestinal tract, mesentery, retroperitoneum, and pelvis, based on the review of tissue sections using an immunohistochemical panel of antibodies for KIT/CD117, CD34, desmin, smooth muscle actin (SMA), and S-100 protein. Tumors included 30 gastrointestinal stromal tumors (GISTs), 10 leiomyomas, 10 leiomyosarcomas, 10 schwannomas, 10 solitary fibrous tumors, and 10 desmoid-type fibromatoses. The overall concordance with the original diagnosis of each histologic type was 97.9%, the kappa value ranging from 0.95 to 1.00 (mean 0.97), indicating a perfect agreement. The overall interlaboratory concordance with the original interpretation of KIT immunostaining was 91.3%, the kappa value ranging from 0.77 to 0.90 (mean 0.86). The overall interlaboratory concordance with the original interpretation of KIT immunostaining was 91.9%, the kappa value ranging from 0.72 to 0.93 (mean 0.85). The overall concordance for determining MIB-1 LI was 90% with the original evaluation, and the overall kappa value ranged from 0.62 to 0.86 (mean 0.77). These results indicate that it is possible to reliably diagnose GIST and other spindle cell tumors of the gastrointestinal tract with the use of an immunohistochemical panel of antibodies for KIT, CD34, desmin, SMA, and S-100 protein. Although there is clearly unavoidable inter-observer and interlaboratory variability in the interpretation of KIT immunostained sections and interobserver variability in the determination of MIB-1 LI, the concordance between observes is very acceptable, and in most instances such variability can be eliminated by careful reviewing of the hematoxylin and eosin and immunostained sections.

Adult↗

Cytogenetic confirmation of a gastrointestinal stromal tumor and ewing sarcoma/primitive neuroectodermal tumor in a single patient.

We report a rare case in which two tumor entities, a gastrointestinal stromal tumor (GIST) and Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET), with distinct cytogenetic features occurred in a single patient. The patient was a 72-year-old woman. The first tumor was a submucosal gastric tumor and was diagnosed as a low-risk group GIST based on morphological characteristics and the results of an immunohistochemical analysis for c-kit and CD34. Further cytogenetic analysis revealed that this tumor had a point mutation (D842V substitution) in exon 18 of the platelet-derived growth factor receptor alpha gene. The second tumor was found more than 4 years after the appearance of the first tumor. ES/PNET was highly suspected both morphologically and immunohistochemically, and the diagnosis was confirmed by the detection of an EWS rearrangement using a fluorescence in situ hybridization technique. Although the cytogenetic correlations of these two tumors are unclear, accurate histologic recognition is of clinical importance because the treatments for these two tumors differ.

Aged↗

Expression of epidermal growth factor receptor, ERBB2 and KIT in adult soft tissue sarcomas: a clinicopathologic study of 281 cases.

BACKGROUND: Little is known about the expression of receptor tyrosine kinases in adult soft tissue sarcomas (STS). In the current study, the authors analyzed the expression of epidermal growth factor receptor (EGFR), ERBB2, and KIT in 281 patients with STS who were treated in a single institution. Verification of the presence of an association with prognosis was performed. METHODS: The current study included 281 adult patients with STS of the extremity and trunk who were diagnosed and treated in the National Cancer Center, Tokyo. Expression was assessed using immunohistochemical stains for EGFR, ERBB2, and KIT on formalin-fixed, paraffin-embedded tissue sections by standard avidin-biotin peroxidase complex technique and EGFR detection system. RESULTS: Positive staining of EGFR was observed in 168 of 281 (60%) patients. Positive staining was common in pleomorphic malignant fibrous histiocytomas (89%), myxofibrosarcomas (89%), synovial sarcomas (76%), malignant peripheral nerve sheath tumors (89%), and leiomyosarcomas (73%). It was less common in well differentiated liposarcomas (38%), fibrosarcomas (36%), and myxoid liposarcomas (6%). In contrast, positive staining of ERBB2 and KIT was very limited. Increased levels of EGFR were significantly associated with a decreased probability of overall survival (P = 0.01), although by univariate analysis; probability of overall survival at 5 years was 64% in patients with increased levels of EGFR and 79% in patients without such overexpression. The overexpression of EGFR was significantly associated with histologic grade (P < 0.001). Moreover, stratified log-rank test revealed that there is an interrelation between EGFR overexpression and histologic grade. CONCLUSIONS: EGFR overexpression was found to be a negative prognostic factor of adult STS, which is strongly associated with histologic grade. STS patients with EGFR overexpression may benefit from treatment with currently available biospecific inhibitors for EGFR.

Adult↗

Pelvic ring reconstruction with the double-barreled vascularized fibular free flap.

BACKGROUND: Although hemipelvectomy has been the standard treatment for malignant tumors of the pelvis, limb salvage surgery is now the treatment of choice, even for patients with advanced tumors. For these patients, pelvic reconstruction is needed to maintain the stability of the pelvis and the spinal column and to allow ambulation. In this report, the authors' experiences with pelvic ring reconstruction are described. METHODS: Pelvic ring reconstruction with free double-barreled vascularized fibular grafts was performed after resection of malignant pelvic tumors in five patients. The graft was fixed with a fixation plate and screws in three patients and with the Cotrel-Dubousset rod system in two patients. After surgery, perioperative and postoperative findings were evaluated. RESULTS: In one patient, a pedicled rectus abdominis musculocutaneous flap was transferred to repair defects of the skin and underlying soft tissue. The free fibular graft was transferred successfully in four of five patients; however, the graft was removed in one patient because of infection with methicillin-resistant Staphylococcus aureus. After surgery, three of the four patients with successful grafts could walk with full weight bearing and without a cane; the fourth patient died as a result of multiple metastases to the lung before walking was attempted. CONCLUSIONS: The double-barreled fibular graft is well vascularized and can achieve satisfactory bone union. It is a safe and effective method for reconstructing the pelvic ring. Furthermore, the Cotrel-Dubousset rod system can provide rigid fixation soon after surgery and is useful for early rehabilitation of walking.

Adolescent↗

Differential diagnosis of gastrointestinal stromal tumor and other spindle cell tumors in the gastrointestinal tract based on immunohistochemical analysis.

To confirm the usefulness of an immunohistochemical panel of antibodies for KIT (c-kit/CD117), CD34, desmin, smooth-muscle actin (SMA), h-caldesmon (HCD), S-100 protein, neuron-specific enolase (NSE), and beta-catenin, 297 mesenchymal and peripheral nerve-sheath tumors of the gastrointestinal tract and intra-abdominal locations including 211 gastrointestinal stromal tumors (GISTs), 12 leiomyomas, 18 leiomyosarcomas, 17 solitary fibrous tumors (SFTs), 14 schwannomas, and 25 desmoid-type fibromatoses (DTFs) were analyzed immunohistochemically. Consistent (100%) immunoreactivity for KIT, CD34, desmin and S-100, and nuclear accumulation of beta-catenin were detected in GISTs, SFTs, smooth-muscle tumors, schwannomas, and DTFs, respectively. Immunoreactivity for SMA, HCD, and NSE was observed in a wide range of these tumors. In addition, 418 bone and soft tissue tumors were enrolled in this study for KIT immunostaining. As a result, a limited number of these tumors were KIT positive, including synovial sarcoma that showed morphological similarity to GISTs. These findings suggest that KIT, CD34, desmin, S-100, and beta-catenin are key markers for clinical diagnosis of GISTs and other spindle cell tumors that may involve the gastrointestinal tract, whereas SMA, HCD, and NSE have only limited value.

Antigens, CD34↗

[Current strategy of chemotherapy for reflactory bone and soft tissue sarcomas].

Pulmonary metastases have been curative in some patients of osteosarcoma with aggressive surgical excision and intensive chemotherapy. But several retrospective studies and randomized II studies did not prove survival advantages of advanced cases with bone and soft tissue sarcoma. In treatment for refractory and advanced osteosarcoma after conventional treatments, clinical phase II trials showed 20-30% response ratio of high-dose ifosfamide and etoposide combination or carboplatin combination. In soft tissue sarcomas, high-dose ifosfamide and doxorubicin have over 50% response rate to advanced soft tissue sarcoma and total surgical excision of residual lung metastases prolong the survival. But new drugs active to refractory sarcoma had not reported in a recent decade.

Antineoplastic Combined Chemotherapy Protocols↗

Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature.

STUDY DESIGN: Report of four cases and a review of the literature. OBJECTIVES: To study the clinical features and prognosis of primitive neuroectodermal tumor or extraskeletal Ewing sarcoma arising around the spinal column. SUMMARY OF BACKGROUND DATA: Primitive neuroectodermal tumor or extraskeletal Ewing sarcoma that originates around the spinal column is very rare, and its prognosis is very poor. METHODS: Four patients were diagnosed and underwent treatment. RESULTS: Although all the patients received high-dose chemotherapy with or without radiotherapy after surgery, three patients died of the disease. Only one patient who received en bloc resection of the tumor combined with multiagent chemotherapy followed by high-dose chemotherapy with peripheral blood stem cell transplantation remains alive and continues to be disease free. CONCLUSION: The prognosis of the patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column is very poor. Multiagent chemotherapy combined with en bloc resection and radiation therapy is the preferred treatment for patients with primitive neuroectodermal tumor or extraskeletal Ewing sarcoma around the spinal column.

Adolescent↗

Sclerosing perineurioma: a clinicopathological study of five cases and diagnostic utility of immunohistochemical staining for GLUT1.

We reviewed five cases of sclerosing perineurial tumor of the hand. Four patients were male and one was female with ages ranging from 11 years to 49 years (mean 26 years). The predominant reason for consultation at the outpatient clinic was a slowly growing painless mass. The sites of involvement were the thumb in two cases, and the ring finger, middle finger and palm in one case each. The lesions were hard and firm, well-circumscribed white masses with a fibrous consistency ranging from 1.2 cm to 4.0 cm (mean 2.5 cm) in maximum dimension. Microscopically, all the tumors were composed of thick collagen and variable numbers of small, epithelioid cells exhibiting corded, trabecular and whorled growth patterns. Electron microscopy showed long cytoplasmic processes with a discontinuous basal lamina and occasional pinocytotic vesicles in the tumor cells. Immunohistochemically, most of the tumor cells were positive for epithelial membrane antigen, vimentin, collagen type IV and CD10, but not for S-100 protein, CD34, desmin and cytokeratin. We also observed that the tumor cells were positive for the human erythrocyte glucose transporter (GLUT1) antigen, suggesting that GLUT1 may be a useful marker for the identification of sclerosing perineurioma.

Adolescent↗

[Current topics in the diagnosis and treatment of malignant fibrous histiocytoma].

Malignant fibrous histiocytoma (MFH) is a tumor about which much remains unknown. The cell origin, molecular mechanism of pleomophism and mechanism of pleomorphic change in a cell undergoing malignant change have not been elucidated. MFH-like histological changes are observed in many bone and cartilage sarcomas, and some renal cell carcinomas and malignant lymphomas. These changes occur in many subtypes of sarcomas such as osteogenic sarcoma, chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, and liposarcoma. MFH has been regarded as one tumor classification from its special histopathological features. In clinical pathological studies these tumors are divided into low-grade fibrous tumors and fibrous histiocytic tumors. With the establishment of molecular biological diagnostic methods, MFH-like histological features can be seen in changes in cellular differentiation of many sarcomas. Patients with MFH often have repeated recurrences before a diagnosis is made, and the tumor is partially resected. Furthermore, distance metastasis develops and the prognosis is poor. The sensitivity of MFH to radiotherapy and chemotherapy is insufficient, and evidence is lacking for adjuvant treatment. Rescue following initial treatment failure is extremely difficult. Local control of 70% to 90% can be achieved if a correct diagnosis is made, and a curative wide resection or salvage wide resection are done early. For treatment of bone and soft tissue sarcoma, a correct diagnosis and initial treatment are extremely important. MFHs are rare tumors that occur in every part of the body. Many cases need to be accumulated in joint clinical studies across fields according to organ and specialty, and effective treatment method developed. We need to advance the standardization of treatment for MFH, and eliminate wrong initial treatment through the active provision of information.

Antineoplastic Combined Chemotherapy Protocols↗