Histological and clinical characteristics of malignant giant cell tumor of bone

Malignant giant cell tumors of bone (MGCTB) are rare, and the diagnosis can be difficult due to the occurrence of a variety of malignant tumors containing giant cells. To better understand its clinicopathological features, we have reviewed our experience with 17 cases of MGCTB. Five cases were primary malignant giant cell tumor of bone (PMGCTB), and 12 cases were giant cell tumors of bone initially diagnosed as benign but malignant in a recurrent lesion (secondary MGCTB, SMGCTB). The patients included six women and 11 men (age ranged from 17 to 52 years; mean, 30.5 years). The tumor arose in the femur (six cases), the tibia (seven cases), the humerus (three cases), and the fibula (one case). Microscopically, PMGCTB showed both conventional giant cell tumor and malignant sarcoma features. SMGCTB were initially diagnosed as conventional giant cell tumor of bone, the recurrent lesion showing malignant features. Histologically, the malignant components included osteosarcoma (11 cases), undifferentiated high-grade pleomorphic sarcoma (two cases), and fibrosarcoma (four cases). SMGCTB cases showed strong expression of p53. Follow-up information revealed that four patients died of lung metastasis, two patients are alive with lung metastases, and 11 patients are alive without tumor. MGCTB should be considered as a high-grade sarcoma. It must be distinguished from GCTB and other malignant tumors containing giant cells. p53 might play a role in the malignant transformation of GCTB.     

Pattern of primary tumors and tumor-like lesions of bone in children: retrospective survey of biopsy results

Background: Although primary bone tumors are relatively uncommon, they constitute the most important tumors in patients less than 20 years. We aimed to determine the frequencies of primary bone tumors and tumor-like lesions of bone and the anatomical sites of their occurrence. Methods: A retrospective review of histopathology reports of all bone specimens received in a private pathology laboratory in Istanbul between 2009 and 2015. Results: A total of 57 patients (aged 5 to 18 years) with a mean of 13.12 years were studied. Thirty five patients (61.4%) were males and 22 (38.6%) were females. Fifty five (94.4%) of the tumors were benign. Osteochondroma was the commonest tumor accounting for 31 cases (54.3%) followed by osteoid osteoma, 9 cases (15.7%). Chondrosarcoma observed in two patients and Ewing sarcoma in one patient as malignant tumors. Of the 57 bone tumors 13 (22.8%) occurred in the upper extremities, while 44 (77.2%) were in the lower extremities. Proximal humerus was the most commonly involved site in upper extremity tumors, with osteochondromas representing the most frequent type of tumor (4 patients; 7%). In the lower extremities again osteochondromas were the most common type of tumor (8 cases, 14%), with the femur being the most common site of involvement (18 patients, 31.5%). Of the patients with tumor-like lesions; four patients had fibrous dysplasia, 4 patients had non-ossified fibromas, 4 patients had simple bone cysts and 3 had aneurismal bone cyst. Conclusion: This study showed that primary bone tumors were mainly benign, settled predominantly in the lower extremities mostly in the femur with a male preponderance. Osteochondroma was the most common benign bone tumor. We didn’t observed osteosarcoma, which is the most frequent malignant bone tumor.     

Bone tumors. Their prevalence

1,200 cases of bone tumors were reviewed from the Orthopedic Hospital "Magdalena de las Salinas" between 1982 and 1989. 66.7% of them were benign tumors, 14.3% pseudotumoral lesions, 10.1% metastatic tumors, 8.6% primary malignant tumors, and 0.3% were malignant invasive tumors to bone. The most frequently found benign tumors were: osteochondroma, enchondroma and giant cell tumor; the most frequent pseudotumoral lesions were metaphyseal fibrous defects, solitary bone cysts and fibrous dysplasia; the most frequent primary malignant tumor was osteosarcoma. Prevalence, frequency, distribution, sex, most frequent ages, affected bones, multicentricity, and aggregated fractures, as well as the site of the primary lesion in metastatic and invading tumors, were similar to those reported in the classical series.     

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.     

Epiphyseal osteosarcoma revisited: four illustrative cases with unusual histopathology and literature review

Osteosarcomas arising in the epiphysis are extremely rare and easily missed in the diagnostic consideration of epiphyseal tumors. It is the purpose of this study to delineate the clinical pathological characteristics of ‘epiphyseal osteosarcoma’ under the definition of ‘a solitary long bone osteosarcoma radiographically considered an epiphyseal tumor for which the main radiologic differential diagnosis would encompass giant cell tumor, chondroblastoma and clear cell chondrosarcoma’. Four such cases with unusual histopathology were retrieved among 110 cases of osteosarcoma. Their clinical, radiological and pathological features, together with all 10 reported cases, were analyzed. The radiographic diagnoses of our four cases include two giant cell tumors, one chondroblastoma and one clear cell chondrosarcoma but turn out to be fibroblastic, giant cell rich, telangiectatic and epithelioid variant of epiphyseal osteosarcoma. Including our patients, the 14 reported epiphyseal osteosarcomas comprise 8 males and 6 females, the age at presentation ranges from 11 to 39 years, two‐third in the second decade, 71.4% affect the femur. Due to their epiphyseal locations, many carry benign radiological diagnoses notably giant cell tumor and chondroblastoma. Epiphyseal osteosarcomas may not only masquerade as benign radiological bony lesions but also assume many histological patterns; orthopedic surgeons, radiologists and pathologists should be aware of such possibility. Their behavior and prognosis are dictated by the histologic types, grading and staging rather than location.     

Malignant fibrous histiocytoma of bone. Clinical pathology and histological diagnosis

Malignant fibrous histiocytoma of bone is a histologically well-defined tumor. Our aim is to describe five own cases and to analyze the published cases in order to demonstrate, the controversial aspects of clinical pathology. The essential histological criteria are the storiform tissue pattern and the presence of fibroblastic and histiocytic cells and giant cells. Inclusive of our cases, the total number reported stands at 196. There are features of malignant fibrous histiocytoma of bone about which there is almost general agreement: 1. The tumor occurs at all ages with an average onset from the age of 10 to the age of 50. 2. The tumor occurs in both the long and flat bones, but the main sites are the distal femur and the proximal tibia. 3. The tumor lacks any initial distinctive features in its clinical phase, but with respect to its biological behaviour, malignant fibrous histiocytoma of bone can be distinguished from osteosarcoma.     

Parosteal osteosarcoma of the distal tibia

We report on the case of a 24-year-old black male who presented to the orthopedic service with a parosteal osteosarcoma of the distal tibia. There have been 206 cases reported on this tumor in all the world's literature, with the distal femur being the most common location; this case report is rare, therefore, because of the unusual location of the tumor. This article was prepared in order to familiarize the general medical community with the occurrence of the tumor and to provide some general background information and treatment recommendations for parosteal osteosarcoma.     

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.     

BONE TUMORS AMONG THE ATOMIC BOMB SURVIVORS OF HIROSHIMA AND NAGASAKI

A study was made of 163 benign and malignant bone tumors found at autopsy and in surgical specimens in Hiroshima and Nagasaki during the period 1950–1965 in order to determine whether or not there is a relationship between atomic bomb radiation and formation of bone tumors.
Twenty-four bone neoplasms were found in a fixed population sample (the ST-100 sample), and nine of these (7 malignant and 2 benign tumors) were encountered at autopsy. The prevalence rate for malignant bone tumors in the autopsies in the fixed sample was estimated to be 0.26%.
No direct relationship was found between the frequency or type of bone tumor and exposure distance.
The case histories of 5 proximally exposed persons who died of malignant bone tumors (2 osteosarcoma and 3 multiple myeloma) were described.     

A clinicopathological study on soft tissue tumors of the head and neck.

The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fascitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence and/or metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described.     

Das Klarzellchondrosarkom

Clear-cell chondrosarcoma is a rare, low-malignant bone tumor. The lesion most commonly occurs in adults, generally in the 3rd or 4th decade. Clear-cell chondrosarcoma has a predilection for the epiphyses of long tubular bones, particularly the femoral head. Radiologically, it is a sharply defined radiolucent lesion. Histologically, it is characterized by large tumor cells with distinct boundaries and a clear cytoplasm. In addition to areas of conventional chondrosarcoma, there are partially mineralized trabecular osteoid formations. On the basis of 16 cases of clear-cell chondrosarcoma, we present the observations of the Hamburg Bone Tumor Register. The cases were examined according to age distribution, location, and radiological and pathomorphological criteria. The proximal femur was the most frequent location; rare manifestations in the rib, os ilium, and distal phalanx of the hand were also present. In 50% of the cases high-malignant osteosarcoma or conventional chondrosarcoma was considered. In addition to the epiphysis of the proximal femur, clear-cell chondrosarcoma can also involve other, rather unusual sites in the skeleton. Knowledge of the histomorphology of this tumor is therefore of crucial importance for the diagnosis.     

Loss of KAI1/CD82 expression in bone and soft tissue tumors is not associated with lung metastasis

The KAI1 gene has been identified as a metastasis suppressor gene in human prostate cancer. Decrease or loss of KAI1/CD82 expression has been shown to be associated with poorer prognosis and metastasis in carcinomas of various organs. The purpose of this study was to examine whether KAI1/CD82 is expressed in bone and soft tissue tumors, and whether it is associated with metastasis to the lungs. Immunohistochemically, KAI1/CD82 expression in benign and malignant soft tissue tumors was noted in 83% and 37% of cases, respectively. KAI1/CD82 was- also expressed in benign bone tumors and osteosarcomas in 67% and 36% of the cases, respectively. Four (40%) of 10 osteosarcoma cases with no lung metastasis and one (25%) of four osteosarcoma cases with lung metastasis were positive for KAI1/CD82, respectively. Metastasis of osteosarcoma cells to the lungs was not correlated with the loss of KAI1/CD82 in osteosarcoma cells.     

Differential diagnosis of giant cell-rich lesions of bone

The diagnosis of giant cell-rich lesions of bone is often problematic even for the experienced pathologist. The diagnostic key lies in multinucleated osteoclast-like giant cells and a mononuclear stroma. From the histological picture alone it is often difficult to distinguish between individual entities such as conventional giant-cell tumor of bone, non-ossifying fibroma, giant-cell tumor in hyperparathyroidism or an aneurysmal bone cyst. Furthermore, these lesions can be confused with malignant bone tumors such as giant cell-rich osteosarcoma. X-ray is important for a correct diagnosis since some of these tumors show a typical anatomic location, while age of the patient is also an important factor. In most cases diagnosis is made on the basis of intraoperative frozen-section, but even in this setting, due to the poor quality of the material, it is very difficult to make a correct diagnosis of giant cell-rich lesions without X-ray and clinical data.     

Roentgenographic diagnosis of bone tumors--its basic knowledge and clinical application

Roentgenographic diagnosis of bone tumors is considered to be extremely important to decide whether a new patient should be biopsied immediately to make the proper diagnosis or the patient could be followed up without operative treatment. The basis knowledge important in making the proper decision on the roentgenogram of the bone tumor, are presented and four cases of bone tumors or tumor-like conditions are presented to show how such knowledge becomes useful. First of all, the basic knowledge based on the anatomical peculiarities are described. Chordoma deriving from the remnant tissue, notochord, should be placed in the midline of the body. Metaphyseal region of the growing child showed a local immune deficient state because of the special anatomic arrangement of the vascular structure for the sake of the open epiphyseal line. This is thought to be related with the high rate of primary malignant bone tumors in this area and in this age group, for example, osteosarcoma. Next, roentgenographic changes are described separately: intracortical changes, cortical changes, periosteal reaction and soft tissue extension. These changes are summarized to differentiate the benign lesion from the malignant one. Thirdly, the peculiar location of the tumor is described. Examples are giant cell tumor, chondroblastoma and clear cell chondrosarcoma in the epiphyseal region, osteoid osteoma, intracortical abscess and the osteofibrous dysplasia in the intracortical region.     

A CLINICOPATHOLOGICAL STUDY ON SOFT TISSUE TUMORS OF THE HEAD AND NECK

The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fasciitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence andlor metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described. ACTA PATH. JAP. 29: 389 - 408, 1979.     

足踝部骨与关节肿瘤及瘤样病变103例分析

目的研究足踝部骨与关节肿瘤及瘤样病变的发病规律。方法总结20年来齐鲁医院经病理确诊的足踩郜骨肿瘤及瘤样病变103例，结合文献分析。结果最高发病年龄集中于10～39岁组之间，男性多于女性，其比例为1．45：1。病变多何于跗骨，跖骨，趾骨。良性肿瘤及瘤样病变明显多十恶性肿瘤，其比例为6．38：1。良性肿爝67例，其中骨软骨瘤、软骨瘤最多见（占良件肿瘤的55．22％）；瘤样病变16例中，骨囊肿9例，最多见（占56125％）：恶性肿瘤13例，以滑膜肉瘤和软骨肉瘤最多见（分别有5例及3例）。结论足踩部骨肿瘤与瘤样病变良性多于恶性，病变多位于跗骨与跖骨。对足部恶性肿瘤的诊断应重视。     

Histopathology of benign versus malignant sympathoadrenal paragangliomas: clinicopathologic study of 120 cases including unusual histologic features

The clinical and pathologic features of 120 adrenal and extraadrenal paragangliomas were studied in an attempt to identify features which might predict malignant behavior. Clinical follow-up was obtained in 98 cases (82%); 64 tumors were clinically benign, and 34 were malignant as evidenced by regional or distant metastases and/or extensive local invasion. Thirty-two of the 34 malignant tumors (94%) were functionally active. Features noted more frequently in malignant tumors included male predominance (74%; P2 [two-sided P value] = .002), extraadrenal location (52%; P2 less than .0001), greater tumor weight (mean 383 g versus 73 g for nonmalignant tumors), confluent tumor necrosis, and the presence of vascular invasion and/or extensive local invasion. Intracytoplasmic hyaline globules were seen in 59% and 32% of benign and malignant tumors, respectively (P2 = .001). Logistic regression analysis of 16 nonhistologic and histologic parameters showed four of them to be most predictive of malignancy--extraadrenal location, coarse nodularity of the primary tumor, confluent tumor necrosis, and absence of hyaline globules. Most malignant paragangliomas had two or three of these features (71%), while 89% of benign tumors had only one (or none; P less than .0001). According to the statistical model developed, there was better than a 95% probability that more than 70% of tumors could be classified correctly on the basis of the four factors indicated. Although limitations still exist, results of this study provide some basis for evaluating malignant potential of these tumors.     

Low-grade intraosseous osteosarcoma in northern Japan: advantage of AgNOR and MIB-1 staining in differential diagnosis

Low-grade intraosseous osteosarcoma is an uncommon form of bone cancer. It is occasionally difficult to recognize as a malignant tumor and is commonly misdiagnosed as a benign fibrous lesion. We retrospectively studied the records of 8 patients with low-grade intraosseous osteosarcoma in the files of the Tohoku Musculoskeletal Tumor Society in Japan. All tumors arose in the lower limb. The most common symptom was pain, with a duration exceeding 2 years in 4 patients. Radiologic findings, including those at magnetic resonance imaging (MRI), suggested malignancy in 5 lesions, whereas 3 were diagnosed as benign. Two patients initially presented with pathological fracture. The initial pathological diagnosis was malignant in 5 patients and benign in 3. All eight tumors were grade 1 in Broders' classification. The tumor showed a permeative pattern in all eight cases, but this pattern could not be confirmed in the multiple tiny fragments obtained as biopsy specimens in 3 cases. The number of silver-staining nucleolar organizer regions (AgNOR) per nucleus and MIB-1-positive rate were significantly higher in low-grade intraosseous osteosarcoma than in fibrous dysplasia, offering an advantage in differential diagnosis. Three patients (38%) developed high-grade sarcoma at the site of local recurrence after multiple intralesional excisions, and one of them died of the disease. The other 5 patients had a good clinical course after surgery with a wide margin. These findings indicate that preoperative diagnosis with radiologic investigation, including magnetic resonance (MR) imaging and histologic examination of biopsy specimens is essential in preparation for surgery with a wide margin, assuring a good clinical course, and the results of AgNOR and immunohistochemical MIB-1 staining might be helpful in differentiating low-grade intraosseous osteosarcoma from fibrous dysplasia.     

侵袭性骨肿瘤中RB基因及其蛋白产物表达的研究

目的探讨抑癌基因ＲＢ的改变与骨肿瘤的关系。方法采用地高辛标记的ＲＢｃＤＮＡ探针对３４例侵袭性骨肿瘤组织及同体正常软组织进行ＳｏｕｔｈｅｒｎＢｌｏｔ检测，同时应用ＬＳＡＢ免疫组化法对９９例侵袭性骨肿瘤及１４例瘤旁软组织进行ＲＢ蛋白表达的检测。结果２１例骨肉瘤中有９例可检出ＲＢ基因的结构异常（４２．９％），ＲＢ蛋白的缺失仅在２６．９％（７／２６）的骨肉瘤和２１．７％（５／２３）的软骨肉瘤中检测到，良性的骨巨细胞瘤和软骨母细胞瘤中未检出ＲＢ蛋白的缺失，其二者差异有意义（Ｐ＜０．０５）；分化差、异型性明显的骨肉瘤细胞其ＲＢ蛋白表达为阴性，低分化的软骨肉瘤及间叶性软骨肉瘤其ＲＢ蛋白亦多为阴性。结论ＲＢ基因结构的异常、ＲＢ蛋白的缺失，可能与骨的恶性肿瘤的发生及其演进有关。     

制型肿瘤膝关节假体在膝关节周定围肿瘤中的应用体会

目的探讨采用国产定制型肿瘤膝关节假体治疗膝关节周围肿瘤的临床疗效.方法对2004年6月~2011年10月应用定制肿瘤型铰链膝关节假体置换膝关节周围肿瘤的15例病人进行回顾性分析,男6例,女9例;年龄27~76岁,平均51.4岁.骨肉瘤4例,骨巨细胞瘤6例,非典型性纤维组织细胞瘤2例,软骨肉瘤3例,均为股骨远端肿瘤.均行瘤段广泛切除国产骨水泥定制肿瘤型铰链膝关节假体重建.结果全部15例随访14~96个月,平均32个月.术后切口均一期愈合,未出现感染、免疫排斥、皮肤会死等近期并发症.1骨肉瘤患者术后10个月出现局部复发,拒绝手术,术后16个月死亡,1例骨肉瘤患者18个月肺转移死亡,1例骨肉瘤患者术后3年局部复发截肢.所有患者无关节脱位、假体松动、断裂、肢体短缩.术后膝关节主动屈曲活动度70~120°,平均89.4°,末次随访ISLOS影像评分24~31分,平均28.6分;骨肿瘤术后MSTS功能评分21~25分,平均22.8分.结论对膝关节周围恶性与侵袭性骨肿瘤切除后骨缺损采用国产定制肿瘤型人工假体重建是有效的保肢方法.