Fibromyxoma of bone: a case report and review of the literature

In this study, a case of fibromyxoma of the proximal femur in a 59-year old woman is reported. The classification of this rare bone tumour is still a matter of debate and some investigators have suggested that these lesions represent a degenerative form of fibrous dysplasia. Some authors make a further distinction between fibromyxoma and myxoma of bone. In a review of 23 cases of fibromyxoma and five cases of myxoma, no differences in clinical, radiographic and biologic behaviour between fibromyxoma and myxoma were found. Apart from the age at diagnosis, the most important difference between fibromyxoma and myxoma was the degree of myxoid matrix. Therefore, we suggest that extragnathic myxoma is a regressive variant of extragnathic fibromyxoma and should be termed as the same entity. In contrast to monostotic fibrous dysplasia fibromyxoma / myxoma often causes pain and presents as a Lodwick IC lesion with a soft tissue mass. Therefore, fibromyxoma / myxoma should be distinguished from fibrous dysplasia because of its different clinical and radiographic features.     

Osteogenic sarcoma and soft tissue myxoma in a patient with fibrous dysplasia and hemoglobins JBaltimore and S

A 41-year-old man with recognized polyostotic fibrous dysplasia since late childhood developed fibroblastic osteogenic sarcoma in the left tibia. Four months after the initial diagnosis, an intramuscular myxoma was discovered in the left thigh. Twenty years previously he had been found to be heterozygous for hemoglobins JBaltimore and S. Malignant transformation in fibrous dysplasia is unusual and may be associated in some individuals with prior irradiation. Soft tissue myxomas associated with fibrous dysplasia are even rarer. To the best of the authors' knowledge the occurrence of both of these lesions in a patient with fibrous dysplasia has been reported only once before. Patients with both fibrous dysplasia and myxomas may be at greater risk for malignant transformation than are individuals with only one of these lesions. There is no well-recognized association between hemoglobinopathies and either fibrous dysplasia or bone tumors. It is therefore probable that the rare constellation of findings is in this patient a stochastic event.     

Chondrosarcoma arising in monostotic fibrous dysplasia treated with total femur resection and megaprothesis: A case report

IntroductionFibrous dysplasia is tumor like lesions of bone which develop as substitution of bone by an expansion of fibrous connective tissue mixed with hard trabeculae. Chondrosarcomas is one of common malignant primary bone tumor derived from heterogenous group of neoplasm producing chondroid matrix. Chondrosarcoma arising in fibrous dysplasia, especially in monostotic fibrous dysplasia is a very rare case.Case reportA 54-year-old male presented with chief complaint of pain on left thigh. Patient with history of pathological fracture on left femoral diaphysis 3 years ago due to fibrous dysplasia and had underwent curettage, open reduction, and internal fixation at other hospital. Plain radiography revealed expansive lytic lesion, interrupted periosteal reaction with plate and screw attached to the lesion, and soft tissue mass. MRI T2FS sequence showed hyperintense mass extending from subtrochanteric to distal of left femoral diaphysis. Histopathological result from biopsy suggested chondrosarcoma.ConclusionsMalignant transformation of monostotic type was less frequently compared to polyostotic type. Among all malignant transformation cases, alteration to chondrosarcoma was more scarce than other malignancy such as osteosarcoma and fibrosarcoma. Wide surgical margin and reconstruction in chondrosarcoma provide good local control and functional outcome.     

Intramuscular myxoma simulating a cystic mass on computed tomography

A case of an intramuscular myxoma (IMM) of the thigh simulating a cystic mass is reported. A discussion of the computed tomography (CT) characteristics of benign and malignant soft tissue tumors are presented.     

An atypical monomelic presentation of Mazabraud syndrome

Mazabraud syndrome is a rare condition characterized by a combination of fibrous dysplasia and intramuscular myxomas. In Mazabraud syndrome, the distribution of fibrous dysplasia is mostly polyomelic and frequently located in the femur, with myxomas adjacent to the fibrous dysplasia lesion of bone (mostly in the quadriceps muscle). However, when presented as atypical clinical features, patients of Mazabraud syndrome is either misdiagnosed or difficult to diagnose. We report an atypical monomelic case of Mazabraud syndrome in the right upper arm and discuss the difficulties in making an accurate diagnosis.     

Intramuscular myxoma of the hypothenar muscles

Intramuscular myxomas of the hand are rare entities. Primarily found in the myocardium, these lesions also affect the bone and soft tissues in other parts of the body. This article describes a case of hypothenar muscles myxoma treated with local surgical excision after frozen section biopsy with tumor-free margins. Radiographic images of the axial and appendicular skeleton were negative for fibrous dysplasia, and endocrine studies were within normal limits. The 8-year follow-up period has been uneventful, with no complications. The patient is currently recurrence free, with normal intrinsic hand function.     

Secondary osteosarcoma arising in fibrous dysplasia,case report

Malignant transformation of fibrous dysplasia is very rare. The frequency is increased in polyostotic forms, in McCune–Albright and Mazabraud’s syndromes and previously irradiated cases. Pain, which is rapidly becoming worse over a short period unrelated to trauma is the most alarming symptom. Early radiological features of sarcomatous transformation are moth-eaten or cystic areas of osteolysis, cortical destruction and gradual formation of a soft tissue mass. The prognosis is unfavorable as most of the cases are in an advanced stage in the time of diagnosis. We present an unusual case of unsuspected secondary osteosarcoma arising in a previously unirradiated, monostotic fibrous dysplasia. A 46-year-old woman was admitted with hip pain, which worsened after a minor trauma occurred 1 year ago. Plain graphies of left femur showed a well-delineated lesion with endosteal scalloping and areas having a ground-glass appearance. The MRI revealed minimal contrast enhancement but no heterogenous signal intensity, cortical destruction, periost reaction or accompanying soft tissue component was noted. The lesion was initially curetted. But being diagnosed as osteosarcoma histologically, classical osteosarcoma protocol pre and postoperative chemotherapy was applied. Resected femur showed areas of fibrous dysplasia admixed with osteosarcoma having fibroblastic, chondroblastic and osteoblastic areas that were focally invading the soft tissue. Tumor viability was estimated as 95%. The clinical course worsened rapidly after the operation. She did not respond to postoperative chemotherapy and lost with pulmonary metastases less than a years’ time after the operation. The case is presented to increase awareness on the possibility of malignant transformation in an otherwise unsuspected fibrous dysplasia.     

An intramuscular myxoma of the hand.

Myxomas of the upper limb are rare and are classified according to their tissue of origin into tumours arising from periosteum, bone or soft tissues such as skin, fascia and muscle. In the literature, one case of intramuscular myxoma of the forearm has been reported. The current report describes the first intramuscular myxoma within the hand muscles.     

颗粒打压植骨辅钢板内固定治疗股骨近端骨肿瘤或瘤样病损

目的 :评价颗粒打压植骨辅钢板内固定治疗股骨近端骨肿瘤或瘤样病损的临床可行性。方法 :2013年1月至2016年1月治疗股骨近端骨肿瘤或瘤样病损26例,均未发生病理性骨折,男12例,女14例;年龄8~62岁,平均34.2岁。病理结果:纤维结构不良11例,骨孤立性骨囊肿7例,骨巨细胞瘤3例,动脉瘤样骨囊肿3例,非骨化性纤维瘤1例,良性纤维组织细胞瘤1例。术前未进行病灶活组织检查,术后送慢病理,手术采取颗粒打压植骨辅钢板内固定。结果:26例均随访至恢复日常生活,随访时间8~42个月,平均25个月。参照骨与软组织肿瘤协会(MSTS)进行功能评估。术后末次复查股骨正侧位X线片,植骨边缘及植骨体部未见低密度影,植骨区骨愈合良好,所有患者未见复发及转移病灶,内固定物无松动、变形。髋关节功能恢复良好,所有患者无再骨折和畸形进展。结论:股骨近端肿瘤复发与病灶刮除植骨技术有关,刮除后采用化学、物理方法处理消灭残留的肿瘤细胞,利用此方法可以获得疾病的长期治愈,减少复发,恢复髋关节功能。     

The diagnosis,classification and grading of soft tissue tumors

An accurate diagnosis of soft tissue tumors is dependent on a thorough history and clinical examination, adequate organ imaging, and subsequent core needle biopsy under organ imaging control. Portions of the cores should be submitted for histology, cytogenetics, electron microscopy, microbiology, and any research requirements. Two recent, cautionary cases of Madelung's disease that were initially misdiagnosed because the history, clinical examination, and organ imaging were circumvented are documented. Single examples of malignant fibrous histiocytoma, Ewing's sarcoma, synovial sarcoma, and intramuscular myxoma, in which appropriate treatment was based on the histories, examination, organ imaging, and core needle biopsies are described. The most reliable classification applicable to the diagnosis and management of soft tissue tumors is found in Enzinger and Weiss' classic textbook,Soft Tissue Tumors (3rd ed. St Louis; CV Mosby, 1995). Provided that the pathologist is trained and experienced in the histological diagnosis of soft tissue tumors, that histological classification provides more accurate prognostic indicators for the established but rare clinico-pathological entities than the crude histological grade. The latter finds its greatest prognostic application with so-called malignant fibrous histiocytomas of adults. Lecture delivered at the 28th Musculoskeletal Tumor Meeting of the Japanese Orthopaedic Association Meeting in Tokyo on 15 July, 1995     

Low-grade central osteosarcoma of distal femur,resembling fibrous dysplasia

We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intra-operatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision.     

股骨近端纤维结构不良的手术治疗

目的探讨股骨近端纤维结构不良的手术治疗方式。方法对19例股骨近端纤维结构不良的不同手术治疗方式及术后疗效进行回顾性分析。结果1例术后3d引流管口渗出血清样物质，加强抗感染、营养支持及换药处理后愈合。19例均获随访，时间13—58个月。复查X线片见缺损修复区内有新骨生成改变，骨折处骨愈合；1例术后20个月因外伤致股骨转子下内固定物旁骨折再次手术发现肿瘤复发，行再次刮除植骨内固定术后14个月愈合；除1例未行内固定的病例外，余患者术后患肢功能均得到良好恢复，8—12个月可弃拐行走。结论股骨近端纤维结构不良应积极手术治疗，在彻底刮除病变和充分植骨的基础上，强调内固定的应用。     

Benign fibrous histiocytoma of bone

The cases of seven patients who had a lytic lesion that was histologically similar to a metaphyseal fibrous defect (non-ossifying fibroma) of bone were studied. The patients all were adults and had pain without a fracture. These features were considered distinctive for the lesion, which has the same histological appearance as benign fibrous histiocytoma of soft tissue. The lesion is a benign tumor with fibroblastic and histiocytic differentiation. This picture may be seen in foci in other lesions of bone (aneurysmal bone cyst, fibrous dysplasia, and giant-cell tumor). Ten cases of giant-cell tumor of bone that had a large component of the same foci were also reviewed. It should be emphasized that these areas are secondary reactive tissue rather than the true neoplastic tissue of benign fibrous histiocytoma.     

Fibrous dysplasia of a third toe

Fibrous dysplasia is a benign disorder of bone, consisting of one or more foci of fibro osseous tissue within the matrix of the affected bone. Fibrous dysplasia usually effects the femur, tibia, ribs, and facial bones and is rarely seen in feet. An unusual case of fibrous dysplasia involving the third toe is presented.     

Reconstruction of knee ligaments with a free tensor fascia lata myocutaneous flap transfer.

A 26-year-old woman suffered a grinding injury of the thigh and knee, resulting in the loss of a large amount of soft tissue and some of the femur. A contralateral tensor fascia lata myocutaneous free flap was used to reconstruct the soft tissues and the lateral ligament compartment around the knee. Later, a ceramic artificial bone was implanted. The patient was able to walk, and a stress radiograph showed that the implanted artificial bone and the knee joint were stable. There was no donor-site morbidity. The functional and cosmetic results were satisfactory.     

Fibromyxoma of the axis

Fibromyxoma of bone is a rare benign tumor of fibrous tissue origin. The typical location is the jaws. Sporadic extragnathic cases have been reported, but fibromyxoma of the spine has not been reported. The histological appearance of fibromyxoma is benign and includes abundant extracellular fibrous and myxoid stroma with varying amounts of calcification and ossification. Myxoid changes are usually extensive. Extragnathic fibromyxoma of bone should be distinguished from benign cartilage-forming bone tumors, such as chondromyxoid and myxoid chondrosarcoma and myxoma of bone. It has also been suggested that fibromyxoma is a variant of myxoid fibrous dysplasia, whereas other authors reported extragnathic fibromyxoma resulting from myxoid degeneration of bone tumors, such as chondrosarcoma or fibrosarcoma. The overtreatment of patients with fibromyxoma of bone due to an aggressive imaging appearance should be avoided; the prognosis is excellent compared with the jaw variant and depends on the location and extent of the tumor. This article describes a case of a 21-year-old woman with fibromyxoma of bone originating from the spinous process of the axis. Clinical examination showed a tender mass in the midline of the posterior aspect the neck and slight limitation of neck range of motion; neurologic examination was normal. Diagnosis was obtained with a preoperative biopsy. Marginal excision of the lesion with posterior laminectomy of the axis was performed. The facets were preserved, and no fusion was performed. At last follow-up 2 years after diagnosis and treatment, the patient was asymptomatic with no evidence of local recurrence.     

Low grade central osteosarcoma--a diagnostic dilemma

Low grade central osteosarcoma is a rare primary bone tumor. This fibro-osseous lesion shares some radiological and histopathological resemblance with fibrous dysplasia, which is a more common entity. Thus it may be mistaken as fibrous dysplasia and may receive inadequate treatment resulting in a more malignant recurrent bone tumor. We present a case of low grade central osteosarcoma of the right tibia, which was initially treated as fibrous dysplasia with curettage and bone grafting. Three years later the tumor recurred with greater soft tissue and bony involvement but without metastasis. The patient was treated with above knee amputation and has been asymptomatic for the last two years.