长骨骨纤维结构不良型釉质上皮瘤临床病理分析

目的探讨长骨骨纤维结构不良型釉质上皮瘤的临床病理特点、鉴别诊断及生物学特征。方法对2例长骨骨纤维结构不良型釉质上皮瘤进行病理学、免疫组织化学观察,结合临床资料进行分析并复习相关文献。结果2例长骨骨纤维结构不良型釉质上皮瘤患者均为年轻人,临床以小腿疼痛或无痛性肿胀为主,病程较长,影像学均示胫骨骨干病变。光镜下肿瘤主要由梭形细胞和散在的新生编织骨组成,梭形细胞密度较高,呈束状或编织状排列,纤维组织间见少数巢团状或裂隙状的上皮样细胞巢,梭形细胞和上皮样细胞均无明显异型性。免疫表型:上皮细胞巢表达CKpan、CK14和CK19,但不表达CK8、CK18。EGFR阳性表达主要见于上皮样细胞,Ki-67在上皮样细胞和梭形细胞均有少量散在阳性表达。结论长骨骨纤维不良型釉质上皮瘤是一种罕见的好发于年轻人胫骨骨干的低度恶性肿瘤,主要根据影像学、光镜及免疫组化确诊,应与骨纤维结构不良、转移癌、滑膜肉瘤等相鉴别。     

Primitive multipotential primary sarcoma of bone: a case report and immunohistochemical study.

A tumor initially presenting as a round cell sarcoma in the proximal tibia of a 42-yr-old male disseminated to involve the femur, multiple tarsal bones, and the lungs. In addition to round cell areas resembling lymphoma, other areas of the tumor produced osteoid matrix and still other areas suggested epithelial differentiation. Immunohistochemical staining revealed the presence of epithelial markers in areas of epithelial differentiation, leukocyte markers in areas of lymphomatous differentiation, and neither marker in areas of osteosarcomatous differentiation. The finding of epithelial elements in a primary bone tumor is rare, and this report is one of the first confirming their presence using immunohistochemical markers. The findings are consonant for neither epithelial nor lymphoid neoplasms and in all probability represent multipotential differentiation in a primitive mesenchymal tumor.     

Multifocal giant cell tumour of bone in a skeletally immature patient--a case report

Giant cell tumor of bone is usually seen in adults affecting a single bone. Multiple giant cell tumour of bone occurring in skeletally immature patients is extremely rare. Multifocal giant cell tumor of bone in a ten year old boy involving upper end of humerus and tibia is being reported for its extreme paucity in literature.     

Epidemiology of bone tumors in Mexico City: retrospective clinicopathologic study of 566 patients at a referral institution

A retrospective analysis of all bone tumors accessioned at a large referral center (Instituto Nacional de Rehabilitacion) in Mexico City between 2000 and 2005 is presented. A total of 6216 biopsies and surgical resection specimens were reviewed during this period, of which 566 corresponded to bone tumors. Benign bone tumors accounted for 71.6% of cases and malignant bone tumors for 28.4%. The tumors affected men in 53.7% of cases and women in 46.3% of cases, with an average age at presentation of 25 years. The femur was the most common location of the tumors (39.9%), followed by the tibia (17.7%) and humerus (11.8%). The commonest malignant bone tumors were osteosarcoma (46.6%) and chondrosarcoma (8.7%). Of malignant bone tumors, 18.6% corresponded to metastases of carcinomas from internal organs and 8.1% were multiple myeloma. The most common benign bone tumor was osteochondroma (43.7%) followed by giant cell tumor of bone (14.6%) and enchondroma (10.1%). The age distribution showed a peak in children and adolescents comprised predominantly of benign lesions and a second peak in young adults that corresponded to malignant bone tumors (principally osteosarcoma). Malignant bone tumors most often involved the femur, vertebra, and tibia. Our results parallel the findings previously reported in the world literature and show a similar distribution and epidemiology as in other developed and underdeveloped countries. Geographic location does not appear to represent a risk factor for any particular type of bone tumor and does not affect the age distribution, location, or histopathologic type of the lesions.     

Fine-needle aspiration biopsy of tibial adamantinoma: a case report

Adamantinoma of long bones is a rare, primary bone tumor of controversial histogenesis, usually arising in the anterior midshaft of the tibia. This slowly growing, radiolucent, expansile tumor eventually causes thinning or destruction of overlying cortical bone. Fine-needle aspiration biopsy (FNAB) is a rapid, safe, and relatively painless means of obtaining diagnostic material from bone tumors, provided the mass has eroded through or markedly attenuated the overlying cortex. A case is presented of primary adamantinoma of the tibia which was sampled first by FNAB and subsequently by surgical biopsy.     

Adamantinoma of the tibia. An eccrine carcinoma

Recent debate concerning adamantinoma of long bone has centered around its histogenesis. The two major proposals have advocated endothelial or epithelial origin. Recent electron microscopic studies and immunoperoxidase evaluation using keratin and factor VIII-related antigen have supported an epithelial derivation. We have studied such a tumor and applied enzyme histochemical techniques in addition to previous methods. Electron microscopy disclosed desmosomes and intermediate-sized filaments. Positive carcinoembryonic antigen and keratin and negative factor VIII-related antigen confirm previous studies and support an epithelial origin. We demonstrated a correlation between enzyme staining of the tumor cells when compared with normal eccrine gland controls. Our evidence thus points to the adamantinoma of tibia as showing eccrine differentiation.     

Pleomorphic myofibrosarcoma of the tibia with aneuploid DNA content

Myofibrosarcomas of the tibia are exceedingly rare, with only one case reported in the literature. We describe DNA ploidy of high-grade myofibrosarcoma of the tibia in correlation with clinicomorphologic and ultrastructural features in a 16-year-old adolescent girl. Radiological studies revealed an expanding osteolytic bone lesion in the metaphysis of proximal tibia. A biopsy was consistent with malignant fibrous histiocytoma. The final diagnosis of myofibrosarcoma was supported by light microscopy and corroborated by electron microscopy and immunohistochemistry findings. The DNA content analysis showed an aneuploid tumor. She developed local recurrence at 6 months after initial treatment with no evidence of lung metastases and 16 months later is alive with persistence of disease. This is the second case reported in the literature with this location. In this case, the high grade correlated with recurrence behavior and aneuploidy DNA content but not with metastases. By ultrastructural analyses, fibronexus and intracellular collagen persisted in high-grade myofibrosarcoma.     

Primary malignant giant cell tumour of bone--a study of two cases with short review

Primary malignant giant cell tumour of bone is extremely rare. It is distinctly separate from benign metastasising giant cell tumour of bone and secondary malignant giant cell tumour which occurs in response to radiotherapy and repeated curettage of benign giant cell tumor. The tumor has high mortality rate. It usually affects lower end of femur and upper end of tibia. Two usually affects lower end of femur and upper end of tibia. Two cases, on involving upper end of tibia and other in vertebra are discussed. Extreme paucity of literature prompted to publish this article. A short review of radiological appearance, histopathological findings and treatment modalities is highlighted.     

Case 8

The patient was a 13-year-old girl with a 2-year history of pain and slowly growing tumor on the diaphysis of the left tibia. There was no history of previous trauma. On clinical examination a 6×4 cm hard swelling was found on the anteromedial aspect of the tibia. Serum alkaline phosphatase was moderately raised to 22.5 μkat/L (normal value > 16 μkat/L). Radiologic examination revealed a 6 × 4 × 4 cm, mainly osteolytic, destruction surrounded by sclerosing bone and periosteal thickening. Curettage was performed at the county hospital, but the patient was referred to the Karolinska Hospital 4 months later because of recurrence (Fig. 1). A radical local resection and autologous bone transplantation were performed (16 cm of tibia including the tumor and a good margin of healthy soft tissue were resected). Grossly, a soft reddish tumor causing destruction of the bone was found. Two and one-half years after surgery the patient was well without signs of recurrence or metastases.     

Tumor cell phagocytosis. Its occurrence in a patient with medulloblastoma

Phagocytosis was observed in tumor cells from a metastatic medulloblastoma to the bone marrow. Erythrocytes and leukocytes were seen in the cytoplasm of the tumor cells. Another abnormal finding was self-phagocytosis, in which tumor cells engulfed one another. The mechanism of tumor phagocytosis is not yet clearly understood. Only a few cases of erythrophagocytosis by epithelial tumor cells have been reported in the literature. To our knowledge, this is the first reported case of a medulloblastoma with hematophagocytosis.     

Atypical Carcinoid Tumor of the Lung, Associated with Giant-cell Transformation in Bone Metastasis

A case of neuroendocrine lung tumor located beneath the pleura in a 71 year old woman is reported. At autopsy, the tumor was found to have metastasized to the bones and liver without involving the hilar lymph nodes. Histological-ly, the tumor cells at the primary site and in the liver metastasis exhibited a carcinoid-like organoid structure, whereas pleomorphic giant cells were noted in the bone metastasis. The argyrophilic tumor cells were immuno-reactive for neuron specific enolase, chromogranin A, serotonin, calcitonin, calcitonin gene-related peptide, gas-trin-releasing peptide, neuropeptide Y, gastrin, pancreatic polypeptide, glicentin, the alpha subunit of human cho-rionic gonadotropin, keratin, epithelial membrane antigen, Leu M1 and carcinoembryonic antigen. Electron microscopy revealed abundant neurosecretory granules in the cytoplasm. This was considered to be a rare case of neuroendocrine lung tumor showing carcinoid like histology at the primary site and large-cell transformation in bone metastasis.     

Atypical carcinoid tumor of the lung, associated with giant-cell transformation in bone metastasis.

A case of neuroendocrine lung tumor located beneath the pleura in a 71-year-old woman is reported. At autopsy, the tumor was found to have metastasized to the bones and liver without involving the hilar lymph nodes. Histologically, the tumor cells at the primary site and in the liver metastasis exhibited a carcinoid-like organoid structure, whereas pleomorphic giant cells were noted in the bone metastasis. The argyrophilic tumor cells were immunoreactive for neuron-specific enolase, chromogranin A, serotonin, calcitonin, calcitonin gene-related peptide, gastrin-releasing peptide, neuropeptide Y, gastrin, pancreatic polypeptide, glicentin, the alpha-subunit of human chorionic gonadotropin, keratin, epithelial membrane antigen, Leu M1 and carcinoembryonic antigen. Electron microscopy revealed abundant neurosecretory granules in the cytoplasm. This was considered to be a rare case of neuroendocrine lung tumor showing carcinoid-like histology at the primary site and large-cell transformation in bone metastasis.     

Plexiform fibrohistiocytic tumor of soft tissues and bone

Plexiform fibrohistiocytic tumor is a rare lesion which has been reported only in the dermis and subcutis so far. We present two cases in this location and an additional one localized in the proximal epiphysis of the tibia. A case with crural location showed predominance of plexiform tumorous nodes with fibrohistiocytic arrangement. The nodes were composed of spindle-shaped myofibroblasts with admixture of histiocytes and giant multinucleated osteoclast-like cells. In another case the tumor of axillary soft tissues featured mostly plexiform bundles of spindle-shaped myofibroblast-like cells reacting positively with actin and CD 68 antibodies; ultrastructurally, they contained numerous dense lysosomal inclusions with myelin figures. The bone tumor was composed of hyalinized fibroblastic component with disperse fibrohistiocytic nodes. Despite a semimalignant character of the lesion there was no recurrence in our cases during the 2-6 year postoperative period.     

Differentiated adamantinoma: a case report and review of literature

Adamantinoma of the long bones are uncommon, slow growing tumors with a predilection for the tibia. A special rare variant known as the osteofibrous dysplasia-like adamantinoma or differentiated adamantinoma is thought to be a precursor of the classic adamantinoma. We report a case in a 24 year old female who presented with pain and lytic lesion of the left tibia. Microscopy showed a tumor composed predominantly of osteofibrous dysplasia-like areas with focal cytokeratin positive epithelial islands. This case is highlighted because of its rarity, its association with good prognosis and possible misdiagnosis as osteofibrous dysplasia.     

Malignant fibrous histiocytoma arising within a bone infarct in a patient with sickle cell trait

Sarcoma associated with osteonecrosis or bone infarction is a rare but well-documented pathological event. In this report, a 69-year-old man with sickle cell trait presented with malignant fibrous histiocytoma (MFH) in his distal tibia. The resected tumor was found in association with a large medullary infarct that extended 10 cm proximal from the tumor site. Bone infarcts can be caused by a number of processes including corticosteroid overuse, alcoholism, dysbarism, and hemoglobinopathies such as sickle cell disease. Patients with sickle cell anemia often develop osteonecrosis, but osteonecrosis has also been reported in people with sickle cell trait, albeit much more rarely. Our patient is only the third reported case of infarct-related bone sarcoma in a patient with sickle cell trait. Bone infarction may be a rare though serious consequence of sickle cell trait.     

Malignant fibrous histiocytoma of bone: a study of 35 cases.

Thirty-five cases of primary malignant fibrous histiocytoma of bone are reported. Twenty of these cases were collected from a retrospective analysis of other malignant bone tumors. The age range was from 11 to 69 years; the average age was 34 years. The tumor occurred most commonly in the distal femur and proximal tibia. The distinguishing histologic feature was a storiform arrangement of spindle cells. The differential diagnosis included fibrosarcoma, osteogenic sarcoma, malignant giant cell tumor, malignant lymphoma, and metastatic carcinoma. Follow-up of at least three years was available in 21 cases. Of these, nine patients were alive and free of metastases three and one-half to 12 years after treatment. Two were alive with solitary metastases at three years, and 10 patients died between three months and three years after treatment. In four cases the lesions were multicentric at the time of diagnosis and in four cases were associated with bone infarction. This tumor must be recognized as an important complication of bone infarction and should be suspected when a patient with a known history of bone infarction develops a change in symptoms. Because the prognosis of this tumor is significantly better than that in those tumors with which it had been previously grouped, and in view of its association with bone infarction, it deserves to be maintained as a distinct clinicopathologic entity. Amputation is the treatment of choice.     

Preliminary study on HA coating percutaneously implanted in bone

A comparative investigation on the possibility of hydroxyapatite (HA) coating and pure Ti column to form biological sealing with skin tissue was completed in this study. HA coating and pure Ti column were percutaneously implanted in the tibia of rabbits. Compared with titanium (Ti) implant, HA coating forms epithelial sealing with skin tissue at 6 weeks postoperatively, while the Ti implant may loosen from the implanted site and be lost. The Ti column loosing rate at this time was 50%. However, once the Ti implant becomes fixed with the bone tissue, it can form epithelial sealing with skin tissue just like the HA coating, at 8 weeks postoperatively. At 8 weeks postoperatively, the epithelial sealing is not destroyed in spite of the fact that the HA coating is biodegraded. Our results show that the HA coating can become fixed with the bone faster than the Ti, which is beneficial for epithelial sealing formation. The main role of HA coating for epithelial sealing is beneficial for sealing at the initial period after it is implanted.     

Lung osteoma—a new benign lung lesion

Extraskeletal osteomas have not been described in the lung. Tumors with osseous elements can be found, such as hamartoma and amyloid tumor, and reactive lesions such as osseous metaplasia. A 39-year-old male patient was treated for multiple myeloma and got a bone marrow transplantation 2 years and a few months before he presented with a solitary well-circumscribed tumor in the right middle lobe. The patient underwent surgical resection. The tumor presented with a fibrous capsule and consisted of mature bone trabecules. Within the tumor, fatty tissue was seen. There were small bone spicules interpreted as areas of new bone formation and appositional growth. No amyloid deposition, no immature epithelial tubules as in hamartomas, and no normal lung structure as in osseous metaplasia were seen. Within the osseous elements, a positive reaction was seen with antibodies for osteonectin, whereas the reaction for calcitonin was negative. To the best of our knowledge, this is the first case of an osteoma being reported in the lung looking like any other extraskeletal osteoma. This tumor might have been induced by circulating stem cells; however, due to autologous bona marrow transplantation, this cannot be proven.     

Computed tomography based evaluation of the bone mineral density around the fixation area during knee ligament reconstructions: Clinical relevance in the choice of fixation method

IntroductionThis study examined the bone density around the fixation area during knee ligament reconstructions and assessed how this clinical relevance can be applied to a firm construction for a reconstructed ligament.Materials and methodsFifty consecutive patients (25 healthy men and 25 healthy women) were enrolled in this study. A quantitative computed tomography was used to determine the trabecular bone density at the 7 clinically relevant areas (anteromedial area of proximal tibia, anterolateral area of proximal tibia, posteromedial area of the proximal tibia, posterocentral area of the proximal tibia, posterolateral area of the proximal tibia, near femoral tunnel entrance of the ACL, near the femoral funnel entrance of the PCL). The means and standard deviations of the areas of interest were measured using a 10 mm diameter circle and the bone density was compared.ResultsA comparison of the fixation areas in the proximal tibia, anteromedial area of proximal tibia showed the highest bone density and posterocentral area showed the lowest bone density. A comparison of the PCL tibial fixation with interference screws or trans-condylar fixation revealed the posterocentral area to have the lowest bone density. A comparison of the femoral fixation areas in the ACL and PCL reconstruction revealed no differences in bone density.ConclusionThe anteromedial area of the proximal tibia was most acceptable in the interference screw fixation and the posterocentral area had the lowest bone density in the proximal tibia. There were no differences in the femoral fixation areas in the ACL and PCL reconstruction.     

Podoplanin expression in adamantinoma of long bones and osteofibrous dysplasia

Adamantinoma of long bones (ALB) and osteofibrous dysplasia (OFD) are rare osteolytic bone tumours that principally arise in the tibia. Both ALB and OFD contain epithelial and stromal elements, as well as areas of fibro-osseous proliferation. We assessed expression of podoplanin, a glycoprotein found in osteocytes, in OFD and ALB as well as in fibrous dysplasia and metastatic cancer. Forty-two cases of ALB and OFD, 20 cases of fibrous dysplasia and 20 cases of metastatic carcinoma to bone were stained by immunohistochemistry for expression of podoplanin, epithelial (cytokeratin, epithelial membrane antigen) and vascular (CD34, LYVE-1) markers. Podoplanin was expressed in epithelial cells and tumour glands in ALB as well as in scattered intertrabecular stromal cells in both ALB and OFD. Podoplanin was not expressed by intertrabecular stromal cells in fibrous dysplasia or in metastatic adenocarcinoma. Podoplanin was expressed by osteocytes but not osteoblasts of woven and lamellar bone trabeculae in ALB, OFD, fibrous dysplasia and skeletal metastases. The finding of a common osteocyte marker in OFD/ALB stromal cells is in keeping with a close histogenetic relationship between OFD and ALB; this may reflect the prominence of fibro-osseous proliferation in these tumours. The expression of podoplanin in an osteolytic tumour of the tibia may be useful as a diagnostic discriminant in distinguishing OFD from fibrous dysplasia and ALB from metastatic adenocarcinoma.