Multiple Bone Metastasis of Sclerosing Epithelioid Fibrosarcoma 12 Years after Initial Surgery—Increasing Ki-67 Labeling Index

Sclerosing epithelioid fibrosarcoma (SEF) is a rare sarcoma of low-grade malignancy. There has been no report to describe the comparison of histological features of SEF between primary and metastatic lesions in spite of high local recurrence rate. We report the histological changes and increasing Ki-67 labeling index of the primary and metastatic lesions of SEF. The patient was a 31-year-old man. At 18, a tumor in the abdominal wall was excised. At 23, the tumor recurred which was removed again. At 30, he was referred to our hospital because of swelling and pain in the chest. Histological examination of the chest wall tumor showed epithelioid cells arranged like alveolar pattern with dense collagen stroma. These findings were consistent with those of SEF. Abdominal and the rib tumors showed the same immunohistochemistrical expression. It is noteworthy that the tumor cells of the rib lesion showed increased cellularity, and its Ki-67 activity was higher as compared with the abdominal tumor, suggestive of progression of malignancy of SEF

Secondary osteosarcoma arising after treatment for childhood hematologic malignancies

Secondary osteosarcoma arising after the treatment of hematologic malignancies other than Hodgkin's lymphoma is rare. We report two cases of secondary osteosarcoma arising after treatment for childhood hematologic malignancies (non-Hodgkin's lymphoma and lymphoblastic leukemia). A 10-year-old boy, at the age of 3, was diagnosed with non-Hodgkin's lymphoma. He received chemotherapy, radiation, and bone-marrow transplantation and then was in complete remission. At 6 years, he complained of increasing pain of the right thigh and was diagnosed with osteoblastic osteosarcoma. A 26-year-old man, at the age of 6, was diagnosed as having acute lymphoblastic leukemia (ALL). He received chemotherapy, radiation, and peripheral blood stem cell transplantation (PBSCT). At 11 years after PBSCT, he visited with the complaint of left lumbar swelling. He was diagnosed with chondroblastic osteosarcoma. In both cases alkaline phosphatase (ALP) had already increased prior to the onset of the symptom. We should rule out secondary osteosarcoma at the abnormal elevation of ALP during clinical follow-up of patients after treatment of childhood hematologic malignancies

Clinical Outcome of Patients with Pelvic and Retroperitoneal Bone and Soft Tissue Sarcoma : A Retrospective Multicenter Study in Japan

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

Radiation Eye Dose for Physicians in CT Fluoroscopy-Guided Biopsy

It is important to evaluate the radiation eye dose (3 mm dose equivalent, Hp (3)) received by physicians during computed tomography fluoroscopy (CTF)-guided biopsy, as physicians are close to the source of scattered radiation. In this study, we measured the radiation eye dose in Hp (3) received by one physician during CTF in a timeframe of 18 months using a direct eye dosimeter, the DOSIRISTM. The physician placed eye dosimeters above and under their lead (Pb) eyeglasses. We recorded the occupational radiation dose received using a neck dosimeter, gathered CT dose-related parameters (e.g., CT-fluoroscopic acquisition number, CT-fluoroscopic time, and CT-fluoroscopic mAs), and performed a total of 95 procedures during CTF-guided biopsies. We also estimated the eye dose (Hp (3)) received using neck personal dosimeters and CT dose-related parameters. The physician eye doses (right and left side) received in terms of Hp (3) without the use of Pb eyeglasses for 18 months were 2.25 and 2.06 mSv, respectively. The protective effect of the Pb eyeglasses (0.5 mm Pb) on the right and left sides during CTF procedures was 27.8 and 37.5%, respectively. This study proved the existence of significant correlations between the eye and neck dose measurement (right and left sides, R2 = 0.82 and R2 = 0.55, respectively) in physicians. In addition, we found significant correlations between CT-related parameters, such as CT-fluoroscopy mAs, and radiation eye doses (right and left sides, R2 = 0.50 and R2 = 0.52, respectively). The eye dose of Hp (3) received in CTF was underestimated when evaluated using neck dosimeters. Therefore, we suggest that the physician involved in CTF use a direct eye dosimeter such as the DOSIRIS for the accurate evaluation of their eye lens dose

Adamantinoma arising in the distal end of the fibula

Adamantinoma is a rare, low-grade, malignant bone tumor. It frequently occurs in the tibia but rarely arise in the distal end of the fibula. This study reported a case of adamantinoma arising in the distal end of the fibula, resulting in good prognosis. A 38-year old female felt left ankle pain, and was suspected as having a bone tumor at the distal end of the fibula by X-ray. She was diagnosed as the classical adamantinoma of the fibula by open biopsy. En bloc wide resection of the tumor, primary arthrodesis of the ankle was performed. During the follow- up period of 7 years after the surgery, she has lived without any metastasis and local recurrence. A wide resection and arthrodesis of the ankle joint can provide a good outcome for adamantinoma arising in the end of the fibula

Arthroscopic Excision of Intra-Articular Osteoid Osteoma at the Elbow

Osteoid osteoma (OO) apparent in the intra-articular region of the elbow is very rare. Although computed tomography-guided excision and radiofrequency ablation have been recognized as useful treatment options, arthroscopic excision has recently received focus as an alternative strategy for lesions close to neurovascular structures or intra- and juxta-articular lesions. We herein report a 17-year-old female who underwent arthroscopic treatment for intra-articular OO located at the olecranon/coronoid fossa. Her symptoms included elbow pain that was exacerbated at night and contracture of elbow flexion-extension, and she was diagnosed with intra-articular OO after 12 months of symptomatic history. Arthroscopically, thorough synovectomy for both the anterior and posterior aspects of the joint enabled definition of the tumor margin with hyperemic alteration and excision of the lesion as an en bloc specimen. At the 12-month follow-up, the patient had no recurrence of elbow limitation or pain. This case report describes the advantages of arthroscopic treatment, including a low-invasive approach and easy accessibility to the whole intra-articular space, which can provide clear visualization of the tumorous lesion