OBJECTIVE:To describe novel MR imaging features, and clinical characteristics of soft tissue angiomatoid fibrous histiocytoma (AFH) at presentation, local recurrence, and metastases.MATERIALS AND METHODS:We described the MRI findings of six cases of histologically proven AFH. Pathologic findings, clinical presentation, and outcome were reviewed.RESULTS:Lesions were primarily cystic. At initial presentation, tumors were surrounded by low signal intensity fibrous pseudocapsule. High signal intensity consistent with the lymphoplasmacytic infiltrate was seen in T2-weighted and post-contrast images as a rim over the hypointense pseudocapsule (double rim sign). High signal intensity infiltrating tumoral cords extended into adjacent tissues, through pseudocapsular defects on T2-weighted and post-contrast images. The cystic component and tumor cell nodularity were demonstrated at post-contrast images. Clinically, lesions were often thought to be benign, underwent marginal resection, developed local recurrence, and one developed second recurrence consisting of metastases. Recurrent tumors appeared as multiple masses, misinterpreted as post-surgical changes. An intramuscular recurrence demonstrated double rim and infiltrating margin.CONCLUSIONS:A predominantly well-circumscribed, primarily cystic mass with double-rim and marginal infiltration on MRI suggests the possibility of AFH, in particular in child or young adult. Inclusion of these novel observations in AFH differential diagnosis may have a significant impact on treatment and prevention of recurrence.
Leuprorelin is a luteinizing hormone-releasing hormone (LH-RH) agonist that is used as an agent of androgen deprivation in some patients with prostate cancer. When administered in depot form, local granulomatous reactions may occur at the injection site, which may mimic masses and which are associated with treatment failure. We present a patient who, over a period of 5 years, developed multiple intramuscular gluteal masses while receiving leuprorelin therapy via intramuscular depot injections; biopsy of one of the masses showed the specific histologic features of leuprorelin granuloma. To our knowledge, this entity has not been described in the radiology literature. Awareness of this entity is necessary to suggest the correct diagnosis in patients with a history of leuprorelin depot injections.
INTRODUCTION:Soft tissue sarcoma (STS) with extensive intra-tumoral hemorrhage is an infrequently described entity, usually misdiagnosed as intra-muscular hematoma. The outcomes in this group of patients have not been previously described.MATERIALS AND METHODS:We retrospectively identified 15 patients, with initial clinical or imaging diagnosis of hematoma, or hematoma versus hemorrhagic sarcoma, although final diagnosis of high-grade STS was established in all cases.RESULTS:The most common location was the thigh. Three patients had a bleeding predisposition. Ten patients were referred for further evaluation with the initial diagnosis of muscle strain/hematoma, hematoma versus abscess in one, whereas four were referred for soft tissue mass evaluation. Final diagnosis was made by one biopsy in only 53% of patients. Mean time to diagnosis for patients with two biopsies was 7 months from initial presentation. Histologic diagnosis was malignant fibrous histiocytoma in ten patients. Surgical treatment included tumor resection in eleven and amputation in three patients. One patient had lung metastatic disease at presentation and eight developed lung metastases within a median time of 7 months.CONCLUSION:We suggest that an STS masquerading as hematoma should be suspected when the mechanism and the energy of the trauma do not justify the clinically detected severity of the injury, or the lesion does not follow the expected clinical course of resolution after initial conservative management. Bleeding predisposition does not exclude malignancy. The evacuation of hematomas should include pathologic examination of tissue. Prognosis is dismal due to early metastatic disease.
Chondrosarcomas (CHS) represent a heterogeneous group of disorders ranging from indolent, low-grade tumors to aggressive, high-grade forms. Surgical resection represents the primary and preferred treatment modality for individuals with localized disease. Radiation therapy is appropriate for the treatment of positive surgical margins or palliation of disease-related symptoms. The treatment of advanced, metastatic disease is particularly challenging given the recognition that conventional chemotherapy has proven to be largely ineffective. Systemic chemotherapy may be considered in variant forms such as mesenchymal or dedifferentiated chondrosarcomas but high-quality data supporting its use is limited. There is universal agreement, however, that novel treatment strategies are desperately needed. This review will highlight the need for a coordinated multidisciplinary approach to optimize the management and care of patients.
We present the case of a 41-year-old man with known large cell lung cancer who had undergone left pneumonectomy 7 months prior and who presented with a large intramuscular mass involving the posterior left thigh and upper calf. This thigh mass was ultimately surgically explored, and specimens yielded both Escherichia coli organisms and cells reflecting a skeletal muscle metastasis from the patient's known lung cancer. The patient was also found to have a rectal metastasis from his lung cancer. Intramuscular abscesses produced by gastrointestinal tract flora are a well-known presentation of colon cancer. To our knowledge, this is the first case report of the simultaneous occurrence of a skeletal muscle metastasis and an E. coli abscess in the same anatomic location. We believe the patient's rectal metastasis may have been the intermediate step in this process.
The term “lipoma arborescens” has been used to describe the diffuse infiltration of fat within hypertrophic synovial villi, a condition which has been most frequently described in the knee. We advocate the term “synovial lipomatosis arborescens” for this process and present what is, to our knowledge, the first reported case of this disorder isolated to the peroneal tendon sheath, with imaging, intraoperative, and histological correlation.
We describe a distinctly unusual MR appearance of the cancellous bone never before described in a patient with biopsy-proven fibrogenesis imperfecta ossium.
OBJECTIVE Acral myxoinflammatory fibroblastic sarcoma is a rare, recently described, low-grade sarcoma that involves mainly the distal extremities. The purpose of this study is to report the MRI findings in four cases of acral myxoinflammatory fibroblastic sarcoma. CONCLUSION Acral myxoinflammatory fibroblastic sarcomas may present with various MRI patterns that probably reflect their variable histologic composition. Differential diagnosis with other benign conditions, especially with ganglion cysts and giant cell tumors of the tendon sheath, may be difficult. We report tumoral invasion of the bone in one case, which to our knowledge has not been previously described.
Soft-tissue tumors are a large and heterogeneous group of neoplasms. Hence, classification is often difficult. The most effective management decisions are made when a working group participates in the same diagnostic standard criteria in the evaluation of soft-tissue tumors. The purpose of this pictorial review is to highlight the new and the less well-known features on magnetic resonance (MR) imaging of soft-tissue tumors according to the World Health Organization (WHO) classification established in 2002. The article depicts the major changes of the WHO classification since it was established in 2002 and the most significant findings on MR imaging, thereby providing an update.
During the past 2 years, we treated five patients with acral myxoinflammatory fibroblastic sarcoma at our institution. Four patients presented with a firm, painless mass in the hand that appeared over several months. One patient discovered a painless mass in his shoulder region. The five patients initially were diagnosed as having benign conditions and treated with intralesional or marginal excision by referring physicians, only to have the lesion reappear as sarcoma. Each patient was treated with wide resection of the tumor bed. Acral myxoinflammatory fibroblastic sarcoma is a rare, but increasingly recognized sarcoma of the distal extremities, which often is confused with benign lesions. Surgeons should be familiar with this tumor’s clinical, radiographic, and histologic appearances as it has a high rate of recurrence and can metastasize.
During the past 2 years, we treated five patients with acral myxoinflammatory fibroblastic sarcoma at our institution. Four patients presented with a firm, painless mass in the hand that appeared over several months. One patient discovered a painless mass in his shoulder region. The five patients initially were diagnosed as having benign conditions and treated with intralesional or marginal excision by referring physicians, only to have the lesion reappear as sarcoma. Each patient was treated with wide resection of the tumor bed. Acral myxoinflammatory fibroblastic sarcoma is a rare, but increasingly recognized sarcoma of the distal extremities, which often is confused with benign lesions. Surgeons should be familiar with this tumor's clinical, radiographic, and histologic appearances as it has a high rate of recurrence and can metastasize.
OBJECTIVE:To evaluate the magnetic resonance (MR) features of intramuscular myxoma (IM) compared with its pathological findings.DESIGN:Two radiologists retrospectively reviewed records and imaging studies of patients with histologically proven IM. Two radiologists also analyzed by consensus all the MR studies (pre- and post-contrast T1-weighted and T2-weighted sequences) and a pathologist reviewed the available histological material.PATIENTS:Seventeen patients with 18 histologically proven IM were reviewed. Histological samples of 11 of these 18 tumors were available for pathological analysis.RESULTS:There were 14 women and three men, with a mean age of 58.9 years. IM involved predominantly the thigh (n=10). MR imaging showed well-circumscribed intramuscular masses, hypointense on T1-weighted and hyperintense on T2-weighted images. Eleven masses were homogeneous and seven slightly heterogeneous due to fibrous septa. Enhanced MR imaging demonstrated three different patterns: peripheral enhancement (n=1), peripheral and patchy internal enhancement (n=7) or peripheral and linear internal enhancement (n=4). Intratumoral cysts were detected in four masses. MR imaging showed the presence of a pseudocapsule (n=12), fat around the lesion (n=16) and peritumoral edema (n=16). Histologically, all the tumors were hypocellular, hypovascular and myxoid. Peripheral areas of collagenous fibers formed a partial capsule and IM often merged into surrounding muscular fibers. More cellular tumors and those with scanty myxoid stroma tended to show a more prominent internal enhancement.CONCLUSION:IM shows several recognizable MR features which suggest its diagnosis.
Giant cell tumor of bone is rarely seen in the rib, where it may present as a mediastinal mass. The diagnosis of giant cell tumor of bone is generally straightforward by fine-needle aspiration or needle core biopsy, but sampling problems may lead to confusion with other neoplasms or inflammatory processes. Here, we report a case of giant cell tumor of rib presenting as a mediastinal mass in a 36-year-old man. Because of inadequate sampling and inaccurate clinical information, the tumor was initially mistaken for thymoma. When the mass failed to respond to conventional chemotherapy, additional tissue was obtained and a giant cell tumor was diagnosed. Consequently, definitive therapy was delayed. The case illustrates an important diagnostic pitfall in the biopsy of mediastinal masses.
Objectives: To assess whether group exercise and coping classes reduce physical and psychological impairments and functional disability in older women with prevalent vertebral fractures (VFs).Design: Randomized, controlled trial (modified cross-over) with site as unit of assignment; testing at baseline and 3, 6, 9, and 12 months.Setting: Nine North Carolina retirement communities.Participants: One hundred eighty-five postmenopausal Caucasian women (mean age 81), each with at least one VFs.Intervention: The intervention group had 6 months of exercise (3 meetings weekly, 45 minutes each) and coping classes (2 meetings weekly, 45 minutes each) in Phase 1, followed by 6 months of self-maintenance. The control group had 6 months of health education control intervention (1 meeting weekly, 45 minutes) in Phase 1, followed by the intervention described above.Measurements: Change in trunk extension strength, change in pain with activities, and change in psychological symptoms.Results: Between-group differences in the change in trunk extension strength (10.68 foot pounds, P<.001) and psychological symptoms (-0.08, P=.011) were significant for Phase 1. Changes in pain with activities did not differ between groups (-0.03, P=.64); there was no change in the pain endpoint. In Phase 2, controls showed significant changes in trunk strength (15.02 foot pounds, P<.001) and psychological symptoms (-0.11, P=.006) from baseline. Change in pain with activities was not significant (-0.03, P=.70). During self-maintenance, the intervention group did not worsen in psychological symptoms, but improved trunk extension strength was not maintained.Conclusion: Weak trunk extension strength and psychological symptoms associated with VFs can be improved in older women using group treatment, and psychological improvements are retained for at least 6 months.
During the last 12 years, tumoral calcinosis (TC) has become an entity that is better understood by radiologists, clinicians, and pathologists. Several aspects of TC are well documented by the scientific community, although the most important, etiology, remains an enigma. As a consequence, the treatment of TC continues to be symptomatic. Imaging continues to be the best method for diagnosis, assessing extension, evaluating complications, monitoring treatment, and selecting patients for surgical excision. Investigators from different countries are reporting the most elusive component of TC: calcific myelitis. New systemic and cerebrovascular complications, even in the young, have been described recently. Similarly, ocular manifestation, which had not been reported previously, is well described in the recent literature. A few, poorly documented reports are confounding TC myelitis with osteomyelitis and TC periarticular soft tissue masses with tumoral calcium pyrophosphate dihydrate (CPPD) of the temporomandibular joint and spine. Advances in the clinical arena have resulted in a new approach and classification of several clinical forms of TC. Most of the recent literature has been related to the histopathology of TC. A new view of the old "traumatic theory" suggests microtrauma of connective tissues around the joints, causing bleeding and the formation of rudimentary bursae as the initial event. Perhaps, in the near future, research will define at the genetic and molecular levels the etiology and pathophysiology that will allow the treatment and prevention of TC.
WE EVALUATED A healthy male 59-year-old business executive for a swelling on the left side of the head. In 1991, he noticed some ill-defined sensations on the left side of his head. After a clinical examination and skull X-ray, the executive was diagnosed with Paget's disease of the bone involving the skull. At that time, his alkaline phosphatase (ALP) was 80 IU/liter. He was examined periodically without any treatment. The patient continued to see an enlargement of the left side of his head and decided to have a reevaluation in 1993. At this time, his skull X-ray was read as normal and the computed tomography (CT) scan (Fig. 1) showed sclerosis in the frontoparietal region with involvement of both the tables and the diploë, which was felt to be consistent with Paget's disease. His ALP was 100 IU/liter in 1993, 118 IU/liter in 1994, and 95 IU/liter in 1995. He moved to Pinehurst, NC in 1995 where he used etidronate for 6 months. His postdidronel treatment ALP read as 80 IU/liter. In 1996, the patient had a bone scan that revealed (Fig. 2) increased uptake only in the skull, which was thought to be monostotic Paget's disease of the skull. The patient began using alendronate at the time and his ALP went down to 61 IU/liter in 1997. During this time the swelling on his head continued to grow (Fig. 3), and he came to us in June 1998 for evaluation. Patient CT scan from 1993. Bone scan in 1996. Patient photograph from June, 1998. Note mass on left side of skull. On examination, the frontoparietal region appeared to be the size of a grapefruit that felt smooth and bony hard and lacked warmth or tenderness. He suffered a mild bilateral hearing loss and no other bony involvement with any focal neurological signs. Several bone radiologists examined a series of skull X-ray films (Figs. 4A-4C) from 1993, 1996, and 1998. The radiologists felt that it was a focal lesion rather than a diffuse process based on the typical appearance of the lesion on the skull X-ray (4C). (A) X-ray of skull taken in 1993 showing an ill-defined area of sclerosis seen in the frontoparietal region. (B) More obvious area of sclerosis and hair-on-end appearance in 1996. (C) Typical hair-on-end appearance of the skull suggesting bone spiculations is seen in 1998. A repeat CT scan showed that the lesion was consistent with intraosseous meningioma. The patient underwent a frozen section biopsy, which confirmed the CT findings. The defect was repaired by a wide excision and did well for the year after the operation. This case highlights the importance of differential diagnosis. Different physicians had followed the patient for several years with the primary diagnosis of Paget's disease without reevaluation. Although Paget's disease is a more common condition than intraosseous meningioma, the patient showed no sign of inflammation (warmth and tenderness) or elevation of serum ALP. Another indicator of failed diagnosis was the continued growth of the skull with no other bony involvement, which is not consistent with Paget's disease. Paget's disease usually involves the skull, pelvis, vertebrae, or femur. Multiple sites often are involved though monostatic lesions are common. Clinically, patients can have bone growth (frontal bossing), but they also can be asymptomatic with the only finding an elevated bone-specific ALP. Radiologically, there are diffuse changes with varied findings depending on the stage of the disease. They can have sclerotic and hyperdense areas corresponding to the increased bone turnover or osteolytic areas similar to cotton or wool spots. Early lesion of the skull may be a localized area of osteoporosis (Osteoporosis circumscripta). Conditions that mimic Paget's disease are benign tumors, such as meningioma and osteoma, osteoblastic metastasis, hypervitaminosis A or D, brown tumor of hyperparathyroidism, and fibrous dyplasia. Primary intraosseous meningiomas arise from arachnoid cap cells with diploë.1 There are 39 reported cases. The most common site of involvement is the orbit followed by the frontoparietal region.2 They are usually asymptomatic. They also can cause localized swellings and ill-defined sensations in the skull. Hyperostosis is the most common radiographic abnormality, which is found in 60% of the cases.2 CT scans and the typical hair-on-end appearance of the sclerotic skull lesions suggest diagnosis of intraosseous meningioma. Definite diagnosis occurs with a biopsy followed by a wide excision and reconstruction. If the tumor recurs, then radiotherapy is recommended.2 Although intraosseous meningioma usually is benign, its location can cause local compressive symptoms and disfigurement. Surgical resection is a curative treatment. Appropriate diagnosis would avoid unnecessary treatment with bisphosphonate with its short- and long-term side effects. Thus, we find that this radiological finding, which includes localized hyperdensity with continued bone enlargement, lack of other bony involvement, and characteristic spiculated hair-on-end appearance of the tumor, should prompt the diagnosis of intraosseous meningioma and avoid confusion with common conditions, such as Paget's disease.
Pseudosarcomatous lesions are benign neoplasms of the musculoskeletal system that are likely to be misdiagnosed as malignant, based on clinical and histologic features. These include soft-tissue "tumors" considered reactive or reparative lesions such as nodular fasciitis and myositis ossificans. Also included in the "pseudosarcoma" category are benign neoplasms which show "pseudoanaplastic" cytologic atypia. The latter include lipoma, leiomyoma, angiomyolipoma, and benign peripheral nerve-sheath tumors. These neoplasms, particularly the reparative processes and the nerve sheath tumors, are increasingly being subjected to initial diagnosis by fine-needle aspiration cytology. Even by conventional cytology this group of lesions represents a well-known pitfall for the diagnostic pathologist. We review some cytologic features: repair-like change, cohesion of cellular fragments, and presence of "normal" elements in the aspirate, which may help the cytopathologist avoid misdiagnosis of these notoriously difficult entities.