Epithelioid hemangioendothelioma of bone

Epithelioid hemangioendothelioma is a low-grade malignant tumor with a histologic appearance and clinical course between that of a hemangioma and angiosarcoma. It is rarely encountered in the bone. A 48-year-old woman was examined following trauma. A cystic lesion was noted on a plain radiograph of the left foot, destructing the diaphysis of the first metatarsal bone. Magnetic resonance imaging showed a solid intramedullary lesion involving a large part of the bone. Scintigraphic examination showed uptake in the diaphysis of the left tibia and the first metatarsal bone of the left foot. Histopathologic examination showed a neoplastic lesion consisting of atypical endothelial cells lining vascular structures or forming solid nests in a myxoid stroma. The tumor was immunoreactive for factor VIII, CD31, CD34, and vimentin. A diagnosis of epithelioid hemangioendothelioma was made and the patient underwent subtotal resection of the metatarsal bone with reconstruction of the fibula, and a wide resection of the tibial lesion. No recurrences or metastasis were observed during a four-year follow-up.     

Solitary osseous hemangioma outside the spinal and craniofacial bones

Bone hemangioma is mainly seen in the skull and spine, and rarely occurs in other bones. We report herein four cases of osseous hemangioma arising in rare sites: In two cases, on a rib; a faintly painful mass in one case located on the scapula; and progressive pain in one case located on the ischium. The tumors presented clinically as incidental lesions on radiographs. All cases had an aggressive appearance, such as defect of the cortex, a soft-tissue mass, and a sunburst-like appearance. Markedly high signal intensity on T2-weighted magnetic resonance images was a characteristic finding. Open biopsy resulted in severe blood loss, but needle biopsy was performed safely under computed tomography guidance. It is important to note that bone hemangiomas may be misdiagnosed as malignant tumors. Received: 13 April 1999     

Posttraumatic necrosis of the talus: the Hawkins sign versus magnetic resonance imaging

Magnetic resonance imaging (MRI) is reported to be more sensitive than plain radiographs, computed tomography, or radionuclide bone scanning in detecting osteonecrosis. Few cases of MRI scans falsely negative for osteonecrosis have been reported. A 36-year-old man with posttraumatic necrosis of the body of the talus proven by biopsy had three serial MRI scans that were interpreted as showing the talus to be viable. Eight weeks after injury, the plain radiographs did not show subchondral resorption of bone in the talus (Hawkins sign) and, thus, did correctly indicate necrosis. Most likely a non-union of the talus neck fracture resulted in the false-negative MRI scans.     

Intraosseous cavernous hemangioma of the frontal bone

A 32-year-old man presented with a primary intraosseous cavernous hemangioma manifesting as a small painless swelling of the right forehead. Radiography revealed a radiolucent osteolytic lesion in the right frontal region. Bone window computed tomography demonstrated a 1.5 cm mass between the outer and inner tables just lateral to the right frontal sinus. The outer and inner tables were thin and partially defective, but without bone fracture. Magnetic resonance imaging revealed a small mass lesion with bone erosion of the posterior table of the frontal bone. Preoperative examination yielded no final diagnosis. En bloc resection was performed. The histological diagnosis was primary intraosseous cavernous hemangioma. Total resection is recommended for definitive diagnosis of intraosseous tumor.     

Total elbow arthroplasty as a reconstruction option for distal humerus osteosarcoma: A case report

IntroductionOsteosarcoma is the second most common malignant bone tumor. The humerus is the third most common anatomical location for osteosarcoma, however, osteosarcoma around the elbow joint is uncommon. The intricacy of the elbow joint, limited soft tissue coverage, and proximity to nerves and arteries make the surgical resection and reconstruction complicated.Case reportA 17-year-old boy came with a chief complaint of lump and pain on his left elbow. One month later, the patient felt there was a lump with a size of a marble on the left elbow, which getting bigger to a size of a tenis ball. Physical examination showed mass on the posterior aspect of the elbow with the size 14x12cm. Plain radiographs revealed osteolytic lesion and periosteal reaction of the distal humerus and the magnetic resonance imaging (MRI) showed a low intensity on T2-weighted imaging. Histopathological examination suggested osteosarcoma. The patient underwent neoadjuvant chemotherapy for 3 cycles. The patient was treated with limb salvage surgery by wide excision, cryosurgery followed by total elbow arthroplasty and ORIF with plate and screw. Postoperative plain radiographs showed the plate and screws are well-fixated. The patient can slowly regain his elbow motion without limitation one month postoperatively.ConclusionsDistal humerus in an unusual site for osteosarcoma. Total elbow arthroplasty and ORIF with plate and screw is a favorable reconstruction option for distal humerus osteosarcoma with excellent postoperative functional outcomes.     

Classification,imaging, biopsy and staging of osteosarcoma

Osteosarcoma is the most common primary osseous malignancy excluding malignant neoplasms of marrow origin (myeloma, lymphoma and leukemia) and accounts for approximately 20% of bone cancers. It predominantly affects patients younger than 20 years and mainly occurs in the long bones of the extremities, the most common being the metaphyseal area around the knee. These are classified as primary (central or surface) and secondary osteosarcomas arising in preexisting conditions. The conventional plain radiograph is the best for probable diagnosis as it describes features like sun burst appearance, Codman''s triangle, new bone formation in soft tissues along with permeative pattern of destruction of the bone and other characteristics for specific subtypes of osteosarcomas. X-ray chest can detect metastasis in the lungs, but computerized tomography (CT) scan of the thorax is more helpful. Magnetic resonance imaging (MRI) of the lesion delineates its extent into the soft tissues, the medullary canal, the joint, skip lesions and the proximity of the tumor to the neurovascular structures. Tc99 bone scan detects the osseous metastases. Positron Emission Tomography (PET) is used for metastatic workup and/or local recurrence after resection. The role of biochemical markers like alkaline phosphatase and lactate dehydrogenase is pertinent for prognosis and treatment response. The biopsy confirms the diagnosis and reveals the grade of the tumor. Enneking system for staging malignant musculoskeletal tumors and American Joint Committee on Cancer (AJCC) staging systems are most commonly used for extremity sarcomas.     

Intraosseous neurilemmoma of the proximal ulna

INTRODUCTION

Neurilemmoma is a benign nerve sheath neoplasm commonly located in the soft tissue. Intraosseous neurilemmoma is rare, constituting less than 1% of primary bone tumors.

PRESENTATION OF CASE

A 21 year-old woman was presented with left elbow pain of 1-month duration. Plain radiographs showed a well-defined, lytic and expansile lesion of the proximal ulna. Computed tomography revealed cortical destruction and soft tissue extension. Because the tissue of origin for the tumor was uncertain, an open biopsy was performed. The specimens demonstrated a benign spindle cell tumor suggestive of a neurilemmoma, similar to a soft tissue neurilemmoma. The diagnosis of intraosseous neurilemmoma was established. Marginal excision of the soft tissue component and curettage of the lesion in the bone were performed. After 3.5 years of follow up, there is no clinical or radiographic finding to suggest any recurrence.

DISCUSSION

The major site of intraosseous neurilemmoma is the mandible. Occurrence in the long bone is particularly rare. Only two cases of intraosseous neurilemmoma involving the bones around the elbow have been reported to our knowledge; these cases arose in the distal humerus. We describe the first case of intraosseous neurilemmoma of the proximal ulna of the left elbow. The recommended treatment is conservative resection and bone grafting, as malignant change is extremely rare.

CONCLUSION

Although very rare, intraosseous neurilemmoma should be taken under consideration in the differential diagnosis of painful, radiographically benign-appearing osseous tumor around the elbow.     

A rare cause of painful cervical swelling: myositis ossificans progressiva in childhood. Report of a case

Myositis ossificans progressiva (MOP) is a rare condition of which we report a case in a 13-year-old girl with involvement of the cervical paraspinal and periscapular muscles. No ectopic ossifications were visible on plain radiographs. Computed tomography disclosed diffuse inflammation. Plain radiographs of the toes confirmed the diagnosis by visualizing characteristic bone abnormalities. Knowledge of the digital abnormalities seen in MOP is important to avoid unnecessary diagnostic investigations, most notably a surgical biopsy, which may trigger a flare of the disease. The prognosis is guarded.     

Solitary eosinophilic granuloma of sternum

Primary bone tumors of the chest wall are uncommon, although a wide variety of both benign and malignant tumors arise within the chest wall. Among those tumors, sternal tumors are rare and usually malignant. We report an extremely rare case of eosinophilic granuloma developed in the sternum in a 30-year-old woman. She presented anterior chest pain and somewhat tender mass over the sternum. Chest roentgenogram, computed tomography scanning of the thorax, and total body bone scintigraphy revealed an isolated lytic lesion in the corpus sterni. A tru-cut biopsy of the mass exhibited the typical histologic appearance of eosinophilic granuloma. The solitary lesion was removed completely surgically. This rare condition should be kept in mind in differential diagnosis of sternal lesions.     

Diagnostik und Therapie primärer Knochentumoren

Primary bone tumors can be either benign or malignant. Metastization is a characteristic feature ofmalignant bone tumors. Malignant tumors are characterized by a local aggressive and destructive behavior. The behavior of a tumor is dependent on its entity, the differentiation grade and localization and these factors are of decisive importance for the correct therapy. Even benign tumors can behave very aggressively. Different stages are defined. Patient history and conventional radiographs are the most powerful primary diagnostic tools. Many tumors show typical characteristics and if a malignant lesion is suspected a biopsy should be carried out. Several quality standards have to be respected when making the biopsy. The approach to malignant tumors is always interdisciplinary. Several biological as well as alloplastic reconstruction techniques exist. The treatment of primary malignant bone tumors requires a lot of experience and should only be done in specialized centers.     

The Disappearing Phalanx: A Case Report of a Vascular Tumor of the Toe

We report a unique case of an epithelioid hemangioma of the third middle phalanx in which the lesion replaced the phalanx, became symptomatic, and then required resection, bone grafting, and joint arthroplasty. To the best of our knowledge, this is the first report of an epithelioid hemangioma in the toe that was treated using this approach.     

Metaphyseal fibrous defects

Nonossifying fibromas and fibrous cortical defects are the most common benign lesions of the skeletal system. They are frequently detected incidentally on radiographs taken for an unrelated reason. The diagnosis is routinely made solely on the basis of the history, physical examination, and radiographic appearance. The incidence of multifocal nonossifying fibroma is typically underestimated in patients initially found to have a solitary lesion; the use of a limited skeletal survey rather than bone scan may help detect multifocal lesions in this population. Small, asymptomatic lesions may be followed over time using serial radiographs. Incisional biopsy, curettage, and bone grafting are indicated for large lesions that raise concern for impending pathologic fracture, for lesions that have become painful, and for lesions whose characteristics prevent a definitive radiographic diagnosis. Pathologic fractures involving these lesions should be treated with cast immobilization until the fracture has healed, followed by biopsy, curettage, and bone grafting.     

Sclerosing hemangioma of the lung: a benign tumour with potential for malignancy?

Pulmonary sclerosing hemangioma represents a rare neoplasm with variable potential for progression. This case report of a 35-year-old female with left-sided thoracic pain. Computed tomography revealed a centrally located, well-circumscribed and partially calcified lesion. Intraoperative findings were suggestive of a carcinoid tumour. The tumour was completely removed by lobectomy followed by systematic lymphadenectomy. The histopathological analysis revealed a sclerosing hemangioma, a rare benign neoplasm. Sclerosing hemangiomas (SHs) are true neoplasms derived from alveolar pneumocytes. However, little data is available on the potential malignant behaviour, such as lymphnode metastases, local recurrence, and the appearance of SH's. Generally, wedge resection is justified in the majority of cases, but in cases of uncertain intraoperative diagnosis, anatomic resection with systematic lymphadenectomy is recommended.     

Role of mammalian target of rapamycin inhibitor in the treatment of metastatic epithelioid angiomyolipoma: A case report

Epithelioid angiomyolipoma has malignant potential; however, no effective therapy has been established for advanced cases. A 50‐year‐old woman with a history of right nephrectomy for epithelioid angiomyolipoma was referred to our institution. Computed tomography and magnetic resonance imaging showed multiple tumors in her lung, liver and pelvic cavity. The liver and pelvic tumor specimens obtained by needle biopsy confirmed the diagnosis of epithelioid angiomyolipoma recurrence. The patient was treated with everolimus (10 mg/day). Three months later, pulmonary lesions disappeared; liver and pelvic tumors significantly shrank in size, but the pelvic tumor gradually enlarged again. We carried out surgical resection of the residual liver and pelvic cavity tumors. Although the mammalian target of rapamycin inhibitor seems to be effective for treating epithelioid angiomyolipoma, its long‐term effects remain unknown. Thus, aggressive administration of a multidisciplinary treatment including molecular target therapy and surgical resection is required to improve the prognosis of epithelioid angiomyolipoma.     

Lytic bone lesions in an Australian population: the results of 100 consecutive biopsies

BACKGROUND: The identification of a lytic bone lesion is often perceived as a harbinger of malignancy, especially in an older patient. This study reviews the results of 100 consecutive biopsies of patients who presented with either a solitary lesion or multiple lytic bone lesions in the absence of a history of malignancy. METHODS: Following preoperative planning, including plain radiographs, bone scan and computed tomography scanning, an open biopsy was carried out. RESULTS: The most common site affected was the pelvis (20 patients), followed by the femur (19) and tibia (15). The lesions were solitary in 88 patients and multiple in 12. The most common diagnosis was a secondary carcinoma presenting as a bone lesion, which was found in 25 patients. The carcinomas were a widely distributed group, but the most common among them were renal cell carcinoma (seven patients), breast carcinoma (five patients) and lung carcinoma (five patients). The most common primary bone tumour was a giant cell tumour (16) followed by myeloma (10). Multiple lesions were not universally an indication of a malignant process. In 14 patients the lesion was not a tumour. CONCLUSION: This study emphasizes the importance of obtaining a tissue diagnosis.     

Osseous lipoma: eleven new cases and review of the literature

This study describes the clinical features, radiological appearance, and treatment of 11 new cases of osseous lipoma and reviews 301 other cases in the literature. Osseous lipomas are classified by the site of origin: either within bone (intraosseous lipoma) or on the surface of bone (juxtacortical). Intraosseous lipomas include intramedullary and intracortical lesions. Surface lipomas include subperiosteal and parosteal lesions. The authors added their cases to those found in the literature. Intramedullary osseous lipoma (n=262) : the most common presenting symptoms were pain and swelling (69%). The most frequent localisations were the calcaneus (24%) and the femur (22%). On plain radiographs, these lesions consisted of a well-circumscribed radiolucent area with central calcification and a sclerotic rim, occasionally with cortical expansion. Computed tomography (CT) and magnetic resonance imaging (MRI) showed that the lesions had attenuation values and a signal intensity identical to that of adipose tissue. In symptomatic lesions and in cases with impending fracture, operative treatment was indicated. Parosteal lipoma (n=47): the most common presenting symptoms were local swelling and pain (58%). This lesion was most frequent in the radius (31%) and in the femur (23%). The classic radiographic appearance of parosteal lipoma was that of an exostosis-like bony prominence capped with a radiolucent layer of fat. CT-scan and MRI showed similar features. Intracortical (n=2) and subperiosteal lipomas (n=11): only a few cases were found in the literature. Their localisation differs from that of the other osseous lipomas. The differential diagnosis of osseous lipoma in general is extensive and should include benign and malignant tumours. Combination of radiological and histological data is essential to determine whether an osseous lipoma is actually present.     

Osteochondral fractures of the dome of the talus

Twenty-four patients who had an osteochondral fracture of the dome of the talus were examined by plain radiography, magnetic resonance imaging, computerized tomography, and, when indicated, scintigraphy. When plain radiographs of the ankle are relied on for the diagnosis of an osteochondral fracture of the talus, many lesions remain undiagnosed. Stage-I osteochondral fractures show no diagnostic changes on plain radiographs, and Stage-II lesions are usually subtle and, therefore, are often overlooked by both radiologists and clinicians. The use of scintigraphy as a screening procedure and of magnetic resonance imaging for patients who have positive scintiscans showed that osteochondral fractures are more common than has previously been indicated in the literature. Scintigraphy should be used to assess patients when there is clinical suspicion of an osteochondral fracture but the plain radiographs appear to be negative. Patients who have positive scintiscans should be assessed by magnetic resonance imaging. Patients who have abnormal plain radiographs will derive no major benefits from magnetic resonance imaging; for all but one of these patients, computerized tomography was adequate for staging the fracture.     

Malignant intraosseous peripheral nerve sheath tumour of the proximal femur: a case report

We report a rare case of an intraosseous malignant peripheral nerve sheath tumour of the femoral head and neck. The tumour presented as a well-defined radiolucent lesion on plain radiography. Computed tomography showed aggressive destruction of the bone with no involvement of the adjacent soft tissues. Magnetic resonance imaging revealed an isointense signal intensity on T1-weighted images, hyperintensity on T2-weighted images, and non-homogeneously increased signal intensity after administration of contrast media. The final diagnosis was based on pathohistologic analyses due to the non-specific nature of the lesion.     

Early magnetic resonance imaging compared with bone scintigraphy in suspected scaphoid fractures

We evaluated 100 consecutive patients with a suspected scaphoid fracture but without evidence of a fracture on plain radiographs using MRI within 24 hours of injury, and bone scintigraphy three to five days after injury. The reference standard for a true radiologically-occult scaphoid fracture was either a diagnosis of fracture on both MRI and bone scintigraphy, or, in the case of discrepancy, clinical and/or radiological evidence of a fracture. MRI revealed 16 scaphoid and 24 other fractures. Bone scintigraphy showed 28 scaphoid and 40 other fractures. According to the reference standard there were 20 scaphoid fractures. MRI was falsely negative for scaphoid fracture in four patients and bone scintigraphy falsely positive in eight. MRI had a sensitivity of 80% and a specificity of 100%. Bone scintigraphy had a sensitivity of 100% and a specificity of 90%. This study did not confirm that early, short-sequence MRI was superior to bone scintigraphy for the diagnosis of a suspected scaphoid fracture. Bone scintigraphy remains a highly sensitive and reasonably specific investigation for the diagnosis of an occult scaphoid fracture.     

Die Wertigkeit der Magnetresonanztomografie (MRT) bei der Diagnostik von Übergangsfrakturen im Bereich der distalen Tibia

The rare transitional fractures occur in adolescents at a time of incomplete desis of the epiphysis. The anatomy of this fracture type is complex with the fracture line running in multiple planes. Conventional plain film radiographs often underestimate the extent and geometry of the fracture due to its transverse components. This study was performed to asses the benefit of MR-imaging compared to plain film radiographs in diagnosis and analysis regarding fracture-type, anatomy and dislocation of fracture. During a time period of 18 months we treated 15 patients with a fracture of the distal tibial epiphysis. In addition to plain film radiographs they got MRI of the distal tibia. Plain film radiographs and MRI were anonymized and diagnosed by 2 surgeons and 2 radiologists. 12 transitional fractures were diagnosed in plain radiographs as well as MRI, but regarding our criteria as above, we found two wrong classifications of fracture-type, an underestimation of fracture dislocation of an average of 0,5 mm in plain film radiographs and two rotational dislocations were missed. The MRI was found to provide anatomical detail and information superior to plain film radiographs.