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Biomedical subjects

E W Brien

Publications and source records attributed to E W Brien.

At least 19 recordsLinked to original sources

Giant notochordal hamartoma of intraosseous origin: a newly reported benign entity to be distinguished from chordoma. Report of two cases.

Two cases are reported of a newly described intraosseous entity of vertebral bodies deemed "giant notochordal hamartoma of intraosseous origin". This entity is commonly mistaken for chordoma and must be distinguished from it as the consequences of misinterpretation may be serious. The clinical, radiological and histologic criteria that can be used to distinguish these two entities are emphasized. Included is a proposed pathogenesis for this lesion, its probable notochordal origin, and a review of other probable cases.

Adult↗

Radiographic analysis to determine the treatment outcome in developmental dysplasia of the hip.

The purpose of this retrospective study was to determine whether pretreatment radiographic findings of patients less than 6 months old with developmental dysplasia of the hip can predict treatment outcome. Medical records and radiographs were reviewed in a cohort of 35 patients (44 hips) less than 6 months old with a diagnosis of hip dislocation at one institution. Using pretreatment anteroposterior radiographs, we measured the superior gap (distance between the proximal metaphysis to Hilgenreiner's line) and the medial gap (distance between the femoral calcar and the lateral pelvic wall at that level) and analyzed these data along with the type of splint used (Pavlik harness verses other abduction orthosis). Medical records and radiographs from an aged-matched cohort of 20 patients (23 hips) treated at a second institution were analyzed by using the same clinical and radiographic criteria of hip dislocation. Of the 44 dislocated hips, 29 failed with a trial of splintage (66%). Of the 23 dislocated hips from the second institution, 16 failed a trial of splintage (70%). Statistical analysis evaluating the age of the patient, medial and superior gaps, and harness type revealed that an abnormal superior and medial gap consistently predicted success or failure of splintage. We concluded that infants with pretreatment radiographs revealing a superior gap equal to or less than 3 mm, or a medial gap equal to or greater than 10 mm should not be treated with an initial trial of splintage because failure is likely.

Chi-Square Distribution↗

Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. II. Juxtacortical cartilage tumors.

In part I, we reviewed the varied clinical presentations, pathogenesis, histologic findings, radiologic findings, and treatment of intramedullary cartilaginous lesions of bone. In this section, we will evaluate our cases and consultations of juxtacortical cartilaginous tumors. Radiographic differential diagnosis includes the numerous juxtacortical lesions particularly osteochondroma, parosteal chondroma, Trevor's disease, trauma (fracture and periostitis ossificans), and the low- and high-grade surface osteosarcomas. By emphasizing pathogenesis in conjunction with radiographic and histologic findings, pitfalls in diagnosis and subsequent treatment can be avoided in such cases.

Bone Neoplasms↗

Benign giant cell tumor of bone with osteosarcomatous transformation ("dedifferentiated" primary malignant GCT): report of two cases.

It is not uncommon for sarcomatous transformation of giant cell tumor (GCT) of bone to occur after radiation, but rarely does malignant transformation occur spontaneously, with less than 15 cases reported up to 1995. Only four of these cases have been documented in detail. We report two additional cases of GCT of bone spontaneously transforming or "dedifferentiating" into osteosarcoma without radiation therapy. The first case is absolutely unique and most interesting in that the dedifferentiation process occurred in one of multiple GCT lung metastases 6 years after successful eradication of a primary tibial tumor. The right lung was resected due to development of a large tumor, and at pathologic examination, demonstrated several small nodules of conventional GCT and a much larger, 14-cm mass composed of a mixture of GCT and high-grade osteosarcoma. The second case involved a physician, who had a large tumor in the sacrum with vague symptoms for 8 years. Open biopsy revealed conventional, benign GCT of bone with a secondary aneurysmal bone cyst. Complete curettage 2 weeks later revealed, in addition to areas of conventional, benign GCT a second component of very high grade osteosarcoma. Both patients died less than 1.5 years from diagnosis. This report of osteosarcomatous transformation of a conventional GCT of bone strengthens the theory that there is a mesenchymal cell line in GCT that may spontaneously transform to sarcoma.

Adult↗

Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors.

We reviewed 845 cases of benign and 356 cases of malignant cartilaginous tumors from a total of 3067 primary bone tumors in our database. Benign cartilaginous lesions are unique because the epiphyseal plate has been implicated in the etiology of osteochondroma, enchondroma (single or multiple), periosteal chondromas and chondroblastoma. In the first part of this paper, we will review important clinical, radiologic and histologic features of intramedullary cartilaginous lesions in an attempt to support theories related to anatomic considerations and pathogenesis.

Adolescent↗

Treatment of femoral Ewing's sarcoma.

BACKGROUND: The treatment of Ewing's sarcoma consists of chemotherapy for systemic and local disease. However, the role of radiation therapy, and/or surgical resection for definitive local treatment has yet to be determined. METHODS: A retrospective review of 32 patients (24 males and 8 females) treated for femoral Ewing's sarcoma between 1970 and 1985 was performed. Patients were divided into 3 treatment groups: chemotherapy and radiotherapy (CR) (10); chemotherapy and surgery (CS) (9); and chemotherapy, surgery, and radiotherapy (CSR) (13). Patients in the CR group received a mean of 5320 centigray (cGy) of radiation and patients in the CSR group received a mean of 3590 cGy. Multiagent cyclophosphamide/doxorubicin based chemotherapy was used in all cases. Surgery consisted of wide resection or amputation. RESULTS: Patients in the CR group had a higher risk of local recurrence than patients in the CS and CSR groups (P=0.02, log rank). The combination of local recurrences and treatment complications necessitated surgery for 7 of 10 CR patients, whereas 1 of 9 and 4 of 13 in the CS and CSR groups required additional surgery. The median survival for the entire group was 39 months. Minimum follow-up for surviving patients was 45 months. Five-year survival consisted of 1 of 10 patients in the CR group, 2 of 9 in the CS group, and 7 of 13 in the CSR group. There were no statistically significant differences among the three survival curves. Tumor location within the femur was a significant prognostic variable. Distal femoral location had a survival advantage compared with proximal and mid-femur locations (P = 0.049, log rank). CONCLUSIONS: Femoral Ewing's sarcoma remains a disease with a poor prognosis. Radiation alone for local treatment results in a high rate of local recurrence and complications. Our current local treatment strategy for femoral Ewing's sarcoma includes surgery in all and adjuvant radiotherapy in many of the patients.

Adolescent↗

Clear-cell chondrosarcoma with elevated alkaline phosphatase, mistaken for osteosarcoma on biopsy.

This paper report a case of a pelvic clear-cell chondrosarcoma that was initially diagnosed as clear-cell osteosarcoma after needle biopsy. Preoperatively, laboratory studies revealed an elevated alkaline phosphatase and needle biopsy showed clear cells producing osteoid. Internal hemipelvectomy was performed and final pathology was most consistent with clear-cell chondrosarcoma, despite a negative S-100 immunohistochemical stain. Distinguishing between these two entities is clinically important because treatment differs. In this case, the patient did not receive pre-or postoperative chemotherapy and is disease free more than 2 years later.

Acetabulum↗

Primary malignant mesenchymoma of bone: case report, literature review, and distinction of this entity from mesenchymal and dedifferentiated chondrosarcoma.

The seventh case of primary malignant mesenchymoma of bone is reported. It is compared to the other six cases in the literature and clinicopathologic pattern of this entity is developed. Primary malignant mesenchymoma of bone is compared to mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Differences as well as similarities are discussed and a more current classification scheme for malignant mesenchymoma of bone is proposed.

Bone Neoplasms↗

Giant bone island of femur. Case report, literature review, and its distinction from low grade osteosarcoma.

A 42-year old male with the largest reported giant bone island (10.5 cm in length) is presented. Due to its ominous size, association with some degree of pain and increased uptake on radionuclide bone scan, a biopsy was considered necessary definitively to rule out a slow-growing osteosarcoma or blastic metastasis. Documentation of growth in adult patients of conventional and giant bone islands, coupled with evidence of increased radionuclide uptake, makes the clinicoradiological distinction between bone islands and blastic malignancies difficult. Guidelines for biopsy versus serial radiographic follow-up of such lesions are addressed.

Adult↗

Paget's disease of bone: review with emphasis on radiologic features, Part I.

Distinctive clinical, radiologic and pathologic features are seen in Paget's disease of bone. These distinct features can be divided into three phases; initial phase, midphase, and late phase. The clinical features may vary from patients being asymptomatic (involving a single bone) to patients having severe, multiple bone involvement with systemic disease. Radiologically, there are unique features which can differentiate Paget's disease from other bone diseases. The radiologic features follows the histologic findings on light microscopy. The correlation of the different phases with the clinical, radiologic and pathologic features of Paget's disease allows for early accurate diagnosis and treatment.

Adult↗

Paget's disease of bone: review with emphasis on radiologic features, Part II.

Part I discusses the correlation between the clinical, radiology and histologic features with the three phases (incipient, mid- and late phase) of Paget's disease. In this section, we will discuss in detail the radiologic features by location as well as aberrant radiographic presentations in addition to secondary tumors such as post radiation sarcomas and giant cells tumors which occur in Paget bone. Because Paget's disease generally affects people in their middle and late ages, the differential diagnosis often includes metastatic disease and the differentiation can often be quite challenging. Moreover, metastatic disease to bones afflicted with Paget's disease can further add diagnostic confusion. These critical aspects will be discussed in this section of Paget's disease of bone.

Adult↗

Chondroblastoma arising from a nonepiphyseal site.

Chondroblastoma is a rare, benign primary bone tumor usually involving secondary centers of enchondral ossification. The consistent epiphyseal location in the great majority of chondroblastomas signifies that the tumor may arise from an aberrant germ cell of the physeal plate. This case report describes a chondroblastoma located in a atypical nonphyseal location, namely in the right fourth metatarsal base. Cases such as these imply that the cell of origin may not exclusively be derived form the physeal plate. The clinical-radiographic significance is that historically chondroblastoma is considered an epiphyseal lesion, but rare cases such as the one reported here and those of the skull demonstrate that this is not always a characteristic of these tumors. The implication from a histogenetic standpoint is that chondroblastoma may, on rare occasions, occur in an area of an enchondrally formed bone other than adjacent to the physeal plate. We are presently investigating the histogenetic relationship of the chondroblastoma (CB) of bone to CB of soft tissue, giant-cell tumor of tendon sheath (GCT-TS), pigmented villonodular synovitis (PVNS) and chondroma of tendon sheath (chondroma of soft parts). We now have collected about 15 cases of GCT-TS and PVNS with extensive areas of chondroid and/or cartilage differentiation that cannot be distinguished from CB of bone by histologic or electron microscopic features alone. From these interesting observations we are developing the histogenetic concept that all of these lesions are interrelated to multipotential mesenchymal and/or synovial cells of the tendon sheath.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Treatment of soft-tissue sarcomas of the hand.

We studied the clinical features, radiographic and pathological findings, treatment, and results for twenty-three patients who had been managed for a soft-tissue sarcoma of the hand between 1982 and 1990. The ages of the patients ranged from sixteen to seventy-six years (median age, thirty-one years). The most common clinical finding was a small, painless soft-tissue mass. Twenty of the tumors were high-grade, and eighteen were less than five centimeters in diameter. The most common diagnosis was synovial sarcoma, which was identified in eight patients. Leiomyosarcoma, rhabdomyosarcoma, and malignant fibrous histiocytoma developed in three patients each; epithelioid sarcoma, in two patients; and angiosarcoma, liposarcoma, neuroectodermal tumor, and clear-cell sarcoma, in one patient each. Curative wide excision or amputation was attempted in twenty-two patients; the margins were positive for tumor cells in eight, and local recurrence was seen in nine. Of the twenty-three patients, fourteen had survived, without evidence of disease, after a median duration of follow-up of forty-nine months, and nine had died of disease. The median rate of survival did not differ significantly on the basis of the size or grade of the tumor or the use of adjuvant treatment. However, the rate of survival of the patients who had a soft-tissue sarcoma of the hand that was less than five centimeters in diameter was significantly lower (p = 0.0008) than that of 152 patients who had a similar tumor at another site in an extremity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Soft-tissue sarcomas of the hand and wrist.

Treatment of soft-tissue sarcomas of the hand cause the ultimate confrontation between oncologic outcome and function. The management of hand sarcomas is based on knowledge of the specific biology and location of each tumor. This article reviews the multimodality management of soft-tissue sarcomas of the hand and wrist.

Biopsy↗