The distribution of neural elements in the triangular fibrocartilage complex (TFCC) of the human wrists was studied via immunohistochemical staining of protein gene product (PGP) 9.5 and calcitonin gene-related peptide (CGRP). Articular branches projecting to the TFCC arose from the dorsal branch of the ulnar nerve in all wrists examined. The TFCC is subdivided into the following six regions: the articular disc proper (ADP), meniscus homolog (MH), radio-ulnar ligament (RUL), loose part of ulnar collateral ligament (lUCL), dense part of ulnar collateral ligament (dUCL), and internal portion (IP). The IP consists of a mixture of dense and loose connective tissues enclosed by the ADP, MH, RUL, and UCL, and resides deep in the prestyloid recess, which is a pit in the MH. The densities of PGP 9.5-positive neural elements, including free nerve endings, single nerve fibers, nerve fascicles, and perivascular neural nets, were significantly higher in the IP than in other regions. Some of the neural elements except for the perivascular neural nets were positive for CGRP. The high density of neural elements in the IP suggests that sensory nerves projecting to the TFCC enter into the IP and from there distribute to adjacent regions such as the MH and RUL. Free nerve endings are responsible for pain transmission. The high density of free nerve endings in the IP suggests that the IP is a source of ulnar side wrist pain.
The 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.
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.
In recent years, calcium phosphate cements (CPCs) have frequently been used as bone substitutes in the field of orthopedic surgery. When CPC is used as a bone substitute in vivo, blood contamination is unavoidable. To date, however, no detailed study has been conducted focusing on how the physical properties of CPCs would change under the influence of blood. In this study, the effects of blood contamination on Biopex-R (BPR, PENTAX, Tokyo) are examined in vitro and in vivo. The compressive strength of BPR after setting decreased depending on the amount of contaminating blood. The BPR, which has set in vivo, not only has a fragile surface due to the contamination by blood, but also has a propensity to shorten and be destroyed during the early postoperative stage, especially in the bone exposed to loads. On the other hand, radiographic and histological features in vivo indicated that the absorption and the bone replacement of BPR were stimulated by blood contamination. In the clinical evaluation, the patient's own peripheral venous blood was added to the BPR. One year after the surgery, the absorption was noted around the hardened BPR. To advance CPCs (including BPR) as bioabsorbable bone replaceable materials, it is essential to utilize the patient's own blood in combination with the CPC.
POS 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.
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