Imaging findings in Mazabraud's syndrome: seven new cases.

OBJECTIVE: To present seven new cases of Mazabraud's syndrome with particular observations on the magnetic resonance imaging findings and a review of the literature. DESIGN AND PATIENTS: A multi-institutional retrospective review was performed on seven patients with confirmed Mazabraud's syndrome. The patient group was composed of six women and one man, ranging in age from 39 to 65 years, with a mean age of 53 years. RESULTS: Fibrous dysplasia was more often polyostotic ( n=6) and right-sided ( n=4). Fibrous dysplasia involved the femur in five cases. The soft tissue myxomas were multiple in four cases and were intramuscular in origin. The most commonly affected location was the thigh ( n=4). On computed tomography, myxomas were well-circumscribed, low-attenuation masses. On magnetic resonance images, the lesions were significantly low in signal intensity on T1-weighted images and high in signal intensity on T2-weighted images relative to adjacent skeletal muscle. Enhancement of the myxomas was heterogeneous with irregular, peripheral rim enhancement, and a variable degree of central enhancement depending on the abundance of solid myxoid tissue and bridging fibrous septa. CONCLUSIONS: Knowledge of Mazabraud's syndrome and the imaging appearance of intramuscular myxoma is important in order to avoid unnecessary biopsies of the osseous and soft tissue lesions. The unique features of this disorder allow discrimination from soft tissue malignancies such as sarcoma.

Utility of chest computed tomography for staging in patients with T1 extremity soft tissue sarcomas.

BACKGROUND: National Cancer Center Network (NCCN) and Society of Surgical Oncology (SSO) practice guidelines recommend chest computed tomography (CT) as part of the staging evaluation of patients with extremity soft tissue sarcoma (STS). In the current study, the authors evaluated the use and yield of chest roentgenography (CXR) and selective chest CT to screen for pulmonary metastases in patients with T1 STS. METHODS: The utility of these staging studies was evaluated retrospectively in a cohort of 125 consecutive patients who presented to a tertiary care cancer center with T1 primary (nonrecurrent) extremity STS. Two diagnostic strategies (CXR alone vs. CXR plus chest CT) were evaluated using an incremental cost-effectiveness ratio. RESULTS: The majority of tumors (70%) were high grade. The median sarcoma size was 3.0 cm; 64 of the tumors (51%) were located deep to the investing fascia of the extremity. All patients underwent staging CXR; 1 CXR (< 1%) was suspicious for metastatic disease. Fifty-one patients (41%) also underwent chest CT; 1 chest CT, performed in the patient with a suspicious CXR, revealed metastatic disease. With a median follow-up of 76 months, 19 patients (15%) developed metachronous pulmonary metastases. The relatively low yield resulted in an incremental cost-effectiveness ratio of $59,772 per case of synchronous pulmonary metastasis detected by CXR plus chest CT. CONCLUSIONS: Less than 1% of patients with T1 primary extremity STS were found to have pulmonary metastases that were detectable using a staging algorithm that employs routine CXR with the selective use of chest CT. The findings of the current study do not support current NCCN or SSO practice guidelines for patients with high-grade T1 STS.

Limitations of size as a criterion in the evaluation of adrenal tumors.

BACKGROUND: Size has been considered to be the single best predictor of malignancy in adrenal neoplasms that have been identified incidentally. However, small adrenal cortical cancers have been reported from multiple centers. METHODS: We retrospectively evaluated the value of tumor size and other clinical parameters in the prediction of the presence of adrenal malignancy. RESULTS: The records of 117 patients who underwent evaluation for tumors of the adrenal gland were reviewed. The median tumor size of the adrenal cortical carcinomas (n = 38 carcinomas) was 9.2 cm (range, 1.7-30 cm); 5 cancers (13.5%) were smaller than 5.0 cm. The median overall size of the benign tumors, excluding pheochromocytomas, was 4.0 cm (n = 38 carcinomas); 10 benign tumors (26%) were larger than 5.0 cm. The imaging features of 4 of 5 small adrenal cancers predicted malignancy; the remaining patients had hormonally functioning tumors. The imaging features of 7 of 10 large benign adrenal tumors predicted benign histologic features, including 5 of 5 myelolipomas. CONCLUSIONS: Although size remains a good predictor of the histologic features and clinical behavior of adrenal neoplasms, both small adrenal cortical cancers and large benign tumors occur with measurable frequency. High-quality imaging studies may be helpful in the identification of relatively small adrenal cancers and of characteristic benign lesions that may be selectively followed.

Imaging of soft-tissue sarcomas.

A multidisciplinary approach that includes active participation by a radiologist is beneficial for optimal diagnostic evaluation and treatment of soft-tissue sarcomas. Recent advances in diagnostic imaging include a better understanding of the practical roles of ultrasonography, computed tomography, and magnetic resonance imaging in the evaluation of primary and recurrent soft-tissue sarcomas. The potential role of positron emission tomography scanning and dynamic magnetic resonance imaging in the evaluation of soft-tissue sarcomas has been preliminarily evaluated. The clinically practical utility of radiologic imaging in the evaluation of soft-tissue sarcomas is discussed, and ongoing research is briefly reviewed.

Magnetic resonance imaging and magnetic resonance angiography before postchemotherapy retroperitoneal lymph node dissection.

OBJECTIVES: Retroperitoneal lymph node dissection (RPLND) after primary chemotherapy is an accepted therapeutic approach for metastatic nonseminomatous germ cell testicular cancer. Because of the intense desmoplastic reaction and adherence to venous and arterial walls, accurate imaging of the retroperitoneal vasculature and its relation to residual tumor is essential. We report our experience with magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA), including the recently developed technique of bolus-contrast MRA, in patients undergoing postchemotherapy RPLND. METHODS: Eighteen patients underwent MRI of the retroperitoneal region before RPLND. In addition to routine sequences, MRA was performed in 10 patients, including 8 with a three-dimensional technique using bolus intravenous MR contrast. Results were compared with intraoperative and pathologic findings. RESULTS: MRI and MRA provided detailed information on retroperitoneal vasculature and its relation to tumor, including multiple renal vessels (n = 5), duplex inferior vena cava (n = 1), left retroaortic renal vein (n = 2), and common iliac vein thrombus (n = 1). In all cases, bolus-contrast MRA provided unique information on the location and number of renal and lumbar arteries, in addition to information on the aorta and the mesenteric and iliac vessels. The origin and number of renal arteries were accurately identified in all patients by bolus-contrast MRA; 2 patients had supernumerary renal arteries discovered at RPLND that had not been identified on non-bolus-contrast MRI. CONCLUSIONS: Bolus-contrast three-dimensional MRA provides unique information on renal and lumbar vessels. The potential benefit of avoiding vascular injury during dissection should be prospectively evaluated.

Optimal radiologic imaging of soft tissue sarcomas.

Radiologic evaluation of the patient with soft tissue sarcoma may include conventional radiography, scintigraphy, ultrasonography (US), computed tomography (CT), and magnetic resonance (MR) imaging. The radiologist also plays a critical role in the evaluation of the patient with soft tissue sarcoma by guiding and performing aspiration biopsies. This review discusses the use of radiological techniques to evaluate the primary lesion and to detect recurrences. In adults, soft tissue sarcoma most commonly occurs in the extremities, and this review emphasizes the role of MR imaging in the pre- and post-treatment evaluation of extremity soft tissue sarcomas. The role of imaging studies in the evaluation of soft tissue sarcomas in the abdomen, thorax, and the head and neck region is also discussed.

Preoperative chemotherapy for stage IIIB extremity soft tissue sarcoma: long-term results from a single institution.

PURPOSE: To review a single institution's long-term results with doxorubicin-based preoperative chemotherapy for American Joint Committee on Cancer (AJCC) stage IIIB extremity soft tissue sarcoma (STS). PATIENTS AND METHODS: The records of all patients with AJCC stage IIIB extremity STS treated with preoperative chemotherapy between 1986 and 1990 at The University of Texas M.D. Anderson Cancer Center were reviewed to assess rates of response, local recurrence-free survival (LRFS), distant metastasis-free survival (DMFS), disease-free survival (DFS), and overall survival (OS). RESULTS: Seventy-six patients with stage IIIB disease received preoperative chemotherapy. The median sarcoma size was 10 cm. Seventy-two patients (95%) had tumors located deep to the muscular fascia. Most patients received a median of three preoperative cycles of doxorubicin and dacarbazine (ADIC), cyclophosphamide and ADIC (CyADIC), or other doxorubicin-based regimens. Radiographic response rates were as follows: complete response (CR), 9%; partial response (PR), 19%; minor response, 13%; stable disease, 30%; and progression, 30%. The overall objective major response rate (CRs plus PRs) was 27%. At a median follow-up time of 85 months, 5-year actuarial rates of LRFS, DMFS, DFS, and OS with 95% confidence intervals (CIs) were 83% (CI, 73% to 94%), 52% (CI, 41% to 66%), 46% (CI, 35% to 60%), and 59% (CI, 48% to 72%), respectively. Comparison of responding patients (CRs plus PRs) and nonresponding patients did not show any significant differences in LRFS, DMFS, DFS, or OS. CONCLUSION: Preoperative doxorubicin-based chemotherapy was associated with response, DFS, and OS rates similar to those observed in randomized postoperative chemotherapy trials. Responding patients had rates of LRFS, DMFS, DFS, and OS comparable to those of nonresponders.

Percutaneous needle biopsy diagnosis of benign neurogenic neoplasms.

Preoperative diagnosis of benign neurogenic neoplasms (BNNs) provides useful information in guiding management. To assess the effectiveness of fine-needle aspiration (FNA) and needle core biopsy (NCB) in diagnosing schwannomas and neurofibromas, 40 percutaneous biopsies interpreted as BNNs or obtained from lesions subsequently shown by excision to be BNNs were reviewed. The 13 aspirates diagnostic of BNN revealed spindle cells arranged haphazardly in irregular tissue fragments and in parallel as elongated ropy fascicles, with a myxoid to fibrillary background. The nuclei were buckled, often with intranuclear cytoplasmic inclusions. Four lesions showed nuclear pleomorphism without mitoses. Of 19 schwannomas evaluated by FNA, four (21%) were diagnosed as schwannomas and seven (37%) as BNNs. Ten neurofibromas were aspirated, revealing two (20%) BNNs. Of seven nondiagnostic FNAs accompanied by NCB, three (43%) indicated a BNN. The sensitivities of FNA, NCB, and both modalities in diagnosing BNNs were 43,60, and 71%, respectively. For the 16 FNAs showing features of BNNs, subsequent excisions revealed 11 schwannomas, two neurofibromas, one neurogenic sarcoma, one fibromyxoid neoplasm of uncertain malignant potential, and one unclassified low-grade myxoid sarcoma. FNA can be effective in diagnosing BNNs. If collagenous or myxoid lesions yield paucicellular nondiagnostic aspirates, NCB is helpful. Lowgrade sarcoma and neurofibromatous areas of neurogenic sarcoma may be misinterpreted as BNNs by percutaneous biopsy. BNNs may show nuclear pleomorphism without mitotic activity, and should not be mistaken for sarcoma.

Fine-needle aspiration of pigmented villonodular synovitis of the temporomandibular joint masquerading as a primary parotid gland lesion.

The fine-needle aspiration findings in a case of pigmented villonodular synovitis of the temporomandibular joint are presented. The characteristic cytomorphologic and clinical features of this uncommon, benign fibrohistiocytic lesion are discussed. In addition, due to the initial clinical impression of a primary parotid gland lesion, the differential diagnosis for the cytomorphologic features observed (histiocytoid cells admixed with osteoclast-like giant cells) are discussed within the context of a primary salivary gland mass.

Chondromyxoid fibroma of paranasal sinuses: report of two cases presenting with nasal obstruction.

Chondromyxoid fibroma (CMF) is a rare, benign cartilaginous tumor that often occurs in the metaphyses of long bones. Tumors of the craniofacial bones are extremely rare and most often involve the mandible and the maxilla. This report presents the clinicopathological and radiological features of two unusual cases of CMF arising in the paranasal sinuses that presented with nasal obstruction. The tumors arose in the sphenoid and ethmoid sinuses and were treated by curettage and resection, respectively. One of the two patients was 20 days old, suggesting a possible congenital origin.

Remission of advanced uterine leiomyosarcoma with pulmonary metastases with carboplatin and paclitaxel.

A patient who had a high-grade uterine leiomyosarcoma (LMS) with extensive intra-abdominal and pulmonary metastases at the time of diagnosis underwent supracervical hysterectomy, bilateral salpingo-oophorectomy and tumor reductive surgery. She then received induction chemotherapy with paclitaxel 135 mg/m2 over 24 h and carboplatin (target AUC = 7.5 mg.ml/min) monthly for seven courses, achieving remission with a small amount of residual disease. The treatment was well tolerated except for peripheral neuropathy. Accordingly, the combination of carboplatin and paclitaxel may be considered in patients with advanced high-grade LMS of the uterus, and this regimen warrants further study in this disease.

Retroperitoneal granular cell tumor. Computed tomography findings with 5-year follow-up.

We describe the computed tomography (CT) appearance of an unusual, large retroperitoneal granular cell tumor and provide a 5-year follow-up. The tumor occurred in association with multiple, smaller, subcutaneous granular cell tumors. The CT findings were nonspecific, but the scans delineated the extent of the lesion and its relationship to adjacent vessels well. To our knowledge, this report is the first description of the CT appearance of retroperitoneal granular cell tumor in the medical literature.

Psoas sheath chemical neurolysis for management of intractable leg pain from metastatic liposarcoma.

CASE REPORT: A 56-year-old man with widely metastatic liposarcoma, after left Tower extremity amputation, complained of severe right lower extremity pain. Trials of systemic opioids had resulted in poor pain control while introducing intolerable dose-limiting side effects. METHODS AND RESULTS: Initial inpatient management consisted of a lumbar epidural infusion of a dilute local anesthetic and preservative-free morphine. This provided satisfactory relief but was discontinued because of recrudescence of phantom limb pain. A lumbar epidural infusion of preservative-free morphine sulfate was associated with poor pain relief, central nervous system (CNS) side effects, and severe urinary retention resulting in acute renal failure. A repeated trial of parental opioids provided marginal pain relief with persistent CNS side effects. Chemical neurolysis of the lumbar plexus was performed with 10 ml of 10% aqueous phenol injected into the psoas muscle sheath. The pain gradually resolved over a 2-day period without apparent side effects. Motor function was preserved, pain was resolved, and as systemic opioids were reduced, cognitive function and overall well-being were improved.

Soft-tissue sarcoma of the extremities. MR appearance of post-treatment changes and local recurrences.

MR imaging has the potential to provide valuable insights into intratumoral changes following preoperative therapy and has proven beneficial in the diagnosis of recurrent soft-tissue sarcoma of the extremities and differentiation of recurrence from postsurgical/postradiation changes. As advances occur in surgical and adjuvant treatment programs, further refinements of qualitative and quantitative MR imaging parameters should continue to enhance the role of MR imaging in the post-treatment evaluation of patients with soft-tissue sarcoma of the extremities.

Leuprolide acetate and intravascular leiomyomatosis.

BACKGROUND: Intravascular leiomyomatosis is an uncommon uterine tumor characterized by grossly visible intravascular proliferation of benign smooth muscle. Based on its role in reducing the size of leiomyomas, leuprolide acetate was given as induction therapy for extensive inoperable intravascular leiomyomatosis. CASE: A 44-year-old woman, gravida 1, para 1-0-0-1, presented in July 1992 with abnormal uterine bleeding. Pelvic examination and ultrasonography revealed the presence of a large irregular pelvic mass. At laparotomy, uterine and bilateral adnexal masses were noted extending up to the pelvic inlet and into the broad and infundibulopelvic ligaments. This tumor was not resectable. Based on histologic and immunoperoxidase studies, the lesion was interpreted as a plexiform epithelioid smooth-muscle tumor of uncertain malignant potential. Leuprolide acetate depot therapy (7.5 mg every 4 weeks) was begun in September 1992 and continued for a total of 20 months. Maximal tumor regression was achieved after 9 months. Subsequent reexploration at 20 months revealed a resectable tumor. Resection was accomplished successfully, leaving no apparent residual disease. CONCLUSION: Leuprolide acetate induced tumor regression and rendered debulking surgery feasible in a patient with previously unresectable, widespread, retroperitoneal intravascular leiomyomatosis. Primary hormone therapy may provide alternative therapeutic options for certain cases of intravascular leiomyomatosis.

Unsuppressed fat in the right anterior diaphragmatic region on fat-suppressed T2-weighted fast spin-echo MR images.

The authors retrospectively evaluated magnetic resonance images of the abdomen obtained in 52 consecutive patients. All cases included fast spin-echo (FSE) T2-weighted images acquired with a frequency-selective fat saturation technique. All imaging was performed with a 1.5-T unit. In 42 patients (81%), fat was not suppressed in the right anterior diaphragmatic region on the T2-weighted FSE images with fat suppression. In 11 (26%) of these 42 patients, subcutaneous fat adjacent to the unsuppressed anterior diaphragmatic fat was well suppressed. Hence, the fat in the diaphragmatic region mimicked fluid or peritoneal implants. The cause of the artifact appears to be the juxtaposition of liver, fat, and lung parenchymal air within a small anatomic space, creating a localized inhomogeneity of the magnetic field and susceptibility effects. Radiologists should be aware of this phenomenon to prevent confusion of an artifact with fluid or neoplasms.

Computerized scientific exhibit in radiology: a valuable format for delivering scientific information.

The computerized scientific exhibit (CSE) is gaining acceptance as a tool for delivering scientific information at meetings of radiologic societies. CSEs allow presentation of more material in a more space-efficient manner than do conventional exhibits, and the viewer can control the order and detail in which material is reviewed. As a disadvantage, currently, the radiologist must use a support team and learn new tools to create a CSE. The preparation and cost may also be greater. Meeting attendees must overcome a reluctance to use computers to benefit from a CSE. Creation of a CSE has design, production, and presentation phases. A team of content authors, software author, graphics acquisition person, and project manager works together through brainstorming, conceptual ordering, storyboarding, prototyping, and selection of authoring tools to design the exhibit. Production must include use of common word processing and image file formats, as well as standardization of image resolution. Accurate equipment specification is needed to ensure that the exhibit can be run on the equipment provided by the meeting organizers. In addition, some security steps are needed to prevent misuse of the exhibit. In addition to display at assemblies, the CSE can be made available to a larger audience by delivery on media such as compact disks and the Internet and can be continually modified as new material becomes available.