Aneurysmal bone cyst secondary to infantile cartilaginous hamartoma of rib

We report the first case, in a 4-month-old girl, of an aneurysmal bone cyst secondary to infantile cartilaginous hamartoma of the rib. Infantile cartilaginous hamartoma of the rib (benign mesenchymoma) is a large, expansile tumour that is characterised by cartilaginous, vascular and primitive-appearing stromal and mesenchymal elements. Most cases are diagnosed at less than 1 year of age and may even be congenital. Aneurysmal bone cyst may be a secondary lesion to pre-existing tumours such as giant cell tumours, osteoblastomas, angiomas and chondroblastomas. This unique case is presented with radiological and pathological findings. Received: 15 November 1996 Accepted: 18 February 1997     

Multifocal metachronous giant cell tumor in a 15-year-old boy

We report a case of multifocal metachronous giant cell tumor (GCT) that involved the fibula, tibia, and sacrum of a 15-year-old boy. Multifocal GCT of bone presenting in children is an exceedingly rare phenomenon; however, there is evidence that multifocal GCT presents, on average, at a younger age than solitary GCT. Pediatric radiologists should be aware of this when encountering a single lesion with characteristic radiographic features of GCT and when encountering multiple lytic skeletal lesions.     

Congenital ossifying fibroma (osteofibrous dysplasia) of the tibia —a case report

Ossifying fibromas of the long bones of the leg are benign lesions occurring in the pediatric age group identical in histological appearance to the similarly named tumor of the jaw in adults. Most frequently presentation occurs after minor trauma with symptoms of a swelling of the tibia or fibula which may be painful. Pathological fracture or limp are also occasional presentations. Congenital cases are extremely rare. We describe an otherwise normal male neonate who presented at birth with a bowed right lower leg. The limb was 1 cm shorter than the other side, with tibia vara and a firm mass situated anteriorly. X-ray showed a mixed lytic and sclerotic lesion in the proximal metaphysis of the tibia. Biopsy showed collagenous stroma containing spindle cells and irregular trabeculae of woven bone rimmed by plump osteoblasts. As the appearances were typical of an ossifying fibroma (osteofibrous dysplasia) no surgical treatment was given. The patient was well with no growth of the tumor and with radiological evidence of healing at 1 year follow up. This case is presented to draw attention to the clinicopathological features of this unusual lesion which must be considered in the differential diagnosis of congenital lesions of the tibia.     

Occipital aneurysmal bone cyst secondary to eosinophilic granuloma

We describe the case of a 2-year-old male patient with an aneurysmal bone cyst (ABC) of the occiput secondary to unifocal eosinophilic granuloma (EG). The lesion presented as a painless mass of the scalp which had grown rapidly over the 2 weeks prior to admission. Radiologically, the lesion was osteolytic and multicystic with fluid-fluid levels. On histology, the cyst wall contained hemosiderin-laden histiocytes, spindle cells, multinucleated giant cells, Langerhans' cells and eosinophils. Surgical treatment consisted of en bloc resection. Fourteen months after surgery, the patient was well with no local recurrence. Association between EG and ABC is rare. To our knowledge, this is the first case involving the skull.     

小儿非骨化性纤维瘤的临床特点及诊治

目的：探讨小儿非骨化性纤维瘤的临床特点及治疗方法。方法：回顾性分析我院收治的小儿非骨化性纤维瘤20例。其中男12例，女8例，年龄1．5～14岁，平均8．5岁。病灶均在下肢长管状骨，其中股骨远端10例，股骨颈1例，胫骨近端7例，远端1例，腓骨1例。全部病例均经手术治疗，术式包括单纯病灶切除、病灶切除加自体髂骨或加异体骨移植术。结果：全部病例均经病理证实为非骨化性纤维瘤。术后随访1～5年，平均3．2年，无一例复发。结论：非骨化性纤维瘤的病因和发病机制不清。根据其临床特点，典型的X线片表现，诊断并不困难。但应与骨纤维异样增殖症、骨囊肿及骨巨细胞瘤相鉴别。因本病可引起疼痛及病理性骨折，确诊后应早期手术治疗。术式以病灶切除植骨术为主。     

Mandibular aneurysmal bone cyst in a child misdiagnosed as acute osteomyelitis: a case report and a review of the literature

The aneurysmal bone cyst is a very infrequent, benign bone lesion in children which rarely can be found at the craniofacial skeleton. Here, we describe a case presenting in the mandible as an acute swelling of the cheek, which was initially misdiagnosed as osteomyelitis resulting in a delay to definitive surgical treatment. The cause of misleading diagnosis is often owing to the rapid growth of the lesion, sometimes associated with painful soft tissue swelling indicating an infectious origin or a malignant tumor. Magnetic resonance imaging revealed the classic characteristics of aneurysmal bone cyst with severe destruction of the mandible. Therefore, curative tumor embolization and complete surgical excision was successfully performed. The aneurysmal bone cyst is a curable condition by radical excision, although relapse may occur when only incomplete excision is obtained. Therefore, aneurysmal bone cyst should be considered early when children present with unusual, rapid-growing neoformations of the extremities or the facial region.     

Image diagnosis in McCune-Albright syndrome

McCune-Albright syndrome consists of polyostotic fibrous dysplasia, precocious puberty and cafè-au-lait skin lesions. Bone lesions are characterized by the presence of fibrous connective tissue with a characteristic whorled pattern and containing trabeculae of immature non-lamellar (woven) bone. They may be solitary (monostotic) or multiple (polyostotic). Commonly involved bones include the femur, tibia, ribs and facial skeleton. In the fibula pseudo-cystic areas and 'ground glass'-like areas, in the femur 'shepherd crook' deformation due to weight on a less resistant bone and secondary to many cortical microfractures, are typical. MRI gives the exact delimitation of the lesions and is especially indicated in the followup of monostotic forms and in the outcome of surgical corrections. Bone scintigraphy with technetium 99 is essential in the follow-up of the disease. Ultrasonography is very useful in ovarian cyst follow-up and in the detection of thyroid and adrenal nodules and testicular microlithiasis.     

Giant Cell Tumor in the Skull of a 9-year-old Child: Immunohistochemistry to Confirm a Diagnosis Rare for Age and Site

Giant cell tumor of the bone is usually located within the epiphysis of a long bone, the majority of the lesions occurring in the third and fourth decades of life. We report an unusual case of giant cell tumor (GCT) arising in the parietal skull bone of a 9-year-old girl. The tumor exhibited histologic findings typical for GCT, with conspicuous intravascular giant cells. Based on microscopic features, not only conditions like aneurysmal bone cyst or bone changes associated with hyperparathyroidism but also tumors such as chondroblastoma or osteosarcoma had to be considered. Immunohistochemistry revealed strong reactivity of the tumor giant cells and normal bone osteoclasts with CD68 but not Mac-387; tumor stromal cells were uniformly negative for both. The stromal cells exhibited two immunohistochemically distinct phenotypes. One, involving 50-80% of the tumor cells, exhibited negative lysozyme staining with positivity of proliferating cell nuclear antigen (PCNA) in about 30% of the nuclei. The other showed reactivity with lysozyme but negative PCNA staining. Immunohistochemistry thus helped to distinguish chondroblastoma and osteosarcoma, in which lysozyme positivity would reside in macrophages but not within stromal cells. Instead, chondroblastoma would exhibit protein S-100 positivity in the tumor cells. The biological behavior of GCT is difficult to predict based on morphology alone, although the malignant potential seems to rest in the stromal cells rather than the giant cells. Specifically, in reported cases, the intravascular occurrence of giant cells in GCT is not associated with an increased incidence of metastasis.     

儿童胫腓骨骨与纤维发育不良的诊断与治疗

目的：探讨儿童胫骨骨与纤维发育不良的诊断、治疗和预后。方法：在复习文献的基础上,对1992年以来手术治疗和门诊观察的10例儿童胫腓骨骨与纤维发育不良的临床表现、X线特征和手术治疗结果进行回顾性分析。结果：7例手术刮除后都出现术后复发,1次复发者2例、2次复发者4例,3次复发者1例。结论：儿童胫骨骨与纤维发育不良是一种侵袭性瘤样病变。在发病年龄、X线及病理表现、临床过程等方面,与单发性长骨纤维发育不良均有明显的区别。儿童期手术治疗极易复发,因此,应将手术治疗推迟到骨骼成熟时实施。     

Simple (unicameral) bone cyst of the calcaneus: a pathologic variant revisited

A 17-year-old girl was admitted to the hospital for surgery of an enlarging, painful mass of the left calcaneus. Preoperative imaging studies suggested either a simple (unicameral) or aneurysmal bone cyst. Intraoperative biopsy of the lesion revealed a simple bone cyst with extensive cholesterol clefts. Such cysts are not uncommon in the calcaneus. However, the pathology of this case is unusual and often overlooked. The typical presentation, treatment, and pathology of these lesions are reviewed.     

Synchronous multicentric giant cell tumor in a 16-year-old boy

Synchronous multicenric giant cell tumor of the bone is a rare variant of a lesion appearing during childhood. The authors report clinical, radiological, and pathological features of a 16-year-old boy who was diagnosed with synchronous multicenric giant cell tumor, which originated in the right distal femur and the left fibula.     

Extraosseous aneurysmal bone cyst in a 12-year-old girl

A soft-tissue aneurysmal bone cyst located on the lateral aspect of the left thigh of a 12-year-old girl is described. Conventional radiography of the thigh was normal. Sonography showed a hypoechoic mass with a feeding vessel and intralesional vascularity. On MRI, it was of predominantly soft-tissue signal intensity with intense enhancement following administration of IV contrast medium. Histopathological examination diagnosed the lesion as a soft-tissue aneurysmal bone cyst. This is the fourth paediatric case of this very rare benign tumour.     

Adamantinoma in childhood: report of six cases and review of the literature

Background Adamantinoma is a rare slow-growing malignant bone tumour. Objective To describe the imaging appearances of six childhood cases and review the published literature. Materials and methods The database of the Working Group on Paediatric Oncology, Academic Medical Centre/Emma Children’s Hospital Amsterdam, was searched for cases of adamantinoma. Additionally a literature study was performed to identify cases of adamantinoma in childhood. Results We identified six local cases of adamantinoma of the long bones, two boys (age 3 and 8 years) and four girls (mean age 8.8 years, range 3.0–14.0 years). The location of the tumour was the tibia in five and the tibia and fibula in one patient. In two patients initially a different diagnosis was made, which led to a delay in appropriate treatment. None of the children showed pulmonary metastases and all underwent total gross resection. On follow-up (mean 6.1 years, range 1.6–12.0 years) all children remained disease-free. Besides a discussion of our six patients, imaging features, histopathology, surgical approach and a literature review of childhood adamantinomas is presented. Conclusion Although the incidence of adamantinoma is low, it is important to recognize this rare bone tumour, since in the early stages of the disease adequate treatment will result in an excellent prognosis. Part of this paper was presented at the Annual Meeting of the Radiological Society of North America, 2004, Chicago, USA.     

Aneurysmal bone cyst of the orbit

We report the case of an aneurysmal bone cyst of the left orbital roof in a 12-year-old boy who presented proptosis of the left eye and painless swelling of the left orbital rim. A 3-cm-large tumor developed in less than 3 months, with first visible signs about 2 months after a minor head injury. Radiologically, the lesion was osteolytic and multicystic. The cyst, filled with hematic fluid, was surgically removed via left frontal craniotomy and a simple curettage with high-speed bur. The patient recovered well and has been in good health throughout 14 months of follow-up.     

Computerised tomography in the evaluation of expansile lesions arising from the skull vault in childhood — a report of 5 cases

Expansile lesions of the skull vault are rare in childhood, and often present as relatively asymptomatic calvarial swellings. The cases of 5 children with expansile lesions of the skull vault due to both benign and malignant primary bone lesions are described. The value of computerised tomography in demonstrating that the “tumour” arises primarily from the skull vault as opposed to the underlying brain, and in demonstrating clinically unsuspected endocranial extension of the mass is described. The CT findings in 2 cases of aneurysmal bone cyst, including the significance of the presence of “fluid levels” in reaching a definitive diagnosis are discussed. The successful pre-operative embolisation in one case of aneurysmal bone cyst is reported.     

Aneurysmal bone cyst of the acromion: a case report

The case of 6-year-old girl with an asymptomatic aneurysmal bone cyst of the acromion is reported. Such tumors are rarely located in the scapula and are especially rare in the acromion. The diagnosis was confirmed by biopsy and surgical resection of the lesion. Roentgenographic, CT and histologic features of the cyst are discussed.

     

OSTEOBLASTOMA: A MIMIC OF OSTEOSARCOMA

Osteoblastoma, a bone - forming neoplasm without metastatic potential, can be mistaken histologically for various benign and malignant primary neoplasms of bone or mimic nonneoplastic conditions, including osteoid osteoma, giant cell tumor of bone, aneurysmal bone cyst, and chondroblastoma [1]. The most important diagnostic distinction is to distinguish osteoblastoma from osteosarcoma. Although both lesions usually have characteristic clinical, radiologic, and histologic findings, in certain cases the distinction between both can be difficult. This is complicated by lesions that morphologically and biologically appear to exist at the interface between the two. In this review, we describe the characteristics of osteoblastomas and review criteria that can prove helpful in the distinction among the various pathologic entities.     

Classic metaphyseal lesion following external cephalic version and cesarean section

We report a case of an otherwise healthy neonate diagnosed at birth with a classic metaphyseal lesion of the proximal tibia following external cephalic version for frank breech presentation and a subsequent urgent cesarean section. Although the classic metaphyseal lesion is considered highly specific for infant abuse, this case demonstrates the importance of obtaining a history of obstetric trauma for neonates presenting to the imaging department for suspected non-accidental injury.     

Mesenchymal Hamartoma of the Chest Wall: A Cooperative Study with Review of the Literature

The clinicopathologic features of three examples of mesenchymal hamartoma of the chest wall are described. The entity has been recorded under a number of names including osteochondroma, osteochondrosarcoma, benign chondroblastoma, mesenchymoma, and chondromatous hamartoma. The condition is manifest at birth or shortly thereafter with deformity of the chest wall and respiratory distress. Radiographic examination reveals a well-defined, partly calcified mass involving one or more ribs. The tumor is composed predominantly of chondroid tissue with large endothelium-lined blood spaces and immature mesenchyme with osteoclastic giant cells and osteoid. We review the literature and suggest that the lesion should be distinguished from aneurysmal bone cyst, chondroma, and other mesenchymal neoplasms. In order to avoid local recurrence the recommended treatment is complete surgical resection.     

儿童四肢长骨病理性骨折手术治疗60例

目的 总结儿童四肢长骨病理性骨折的手术治疗经验,探讨提高临床诊断和治疗水平的方法.方法 2005年1月至2010年3月作者收治长骨病理性骨折患儿74例,其中60例采用手术治疗,在确诊原发疾病的同时,给予病灶彻底清除及植骨,并对骨折进行一期内固定处理.患儿出现患肢疼痛等症状的时间为1 d至2年,出现病理性骨折的时间为2 ...