E. Moskovic

Aggressive Fibromatosis: MRI Features with Pathologic Correlation

OBJECTIVE We present the MRI features with pathologic correlation of aggressive fibromatosis, incorporating 203 cases over a 5-year period from the Royal Marsden Hospital Sarcoma Unit database. MATERIALS AND METHODS Sixty patients had imaging available for retrospective review of which 29 had preoperative MRI and final histopathologic diagnosis of aggressive fibromatosis. RESULTS The average age at diagnosis was 41.3 years with a female-to-male sex ratio of 1.2:1. Twenty lesions were extraabdominal; six, intraabdominal; and three, in the abdominal wall (classic desmoid). The average tumor size was 6.4 cm (range, 2.2-13.7 cm). Intraabdominal aggressive fibromatosis produced the largest tumors, averaging 9.5 cm. Most lesions were ovoid (52%) or infiltrative (34.5%) in outline with an irregular or lobulated contour (76%). The lesions crossed major fascial boundaries in 31% of cases overall and in 66% of patients referred for recurrent disease. On MRI, homogeneous isointensity or mild hyperintensity on T1-weighted images and heterogenous high signal on T2-weighted or STIR images were seen. All lesions enhanced after IV gadolinium, usually avidly. In contrast to previous reports, 38% of cases failed to show low signal on all pulse sequences and no abnormalities were seen in local bone structures. Histology showed sheets of bland spindle cells in dense collagen and did not vary with the MRI signal characteristics of the lesion. Patients referred for recurrent disease were most likely to have a recurrence after surgery. MRI and pathology findings did not predict recurrence. CONCLUSION Accurate diagnosis and staging of aggressive fibromatosis by MRI have important treatment and prognostic implications.

Preliminary results of a phase I dose escalation clinical trial using focused ultrasound in the treatment of localised tumours

OBJECTIVE The primary aim of this phase I trial was to assess the tolerance of cancer patients to focused ultrasound (FUS) treatment in a variety of different sites and to document any associated acute or delayed toxicity. This would appear to be the first time that treatment has been given without sedation or anaesthesia. METHODS Patients with advanced and/or metastatic disease were eligible for entry into this study. Previous work has established that an in situ ablative intensity (AI) of 1500 W/cm2 Isp for 1 s achieves coagulative necrosis at the focal spot. Ultrasonic exposures of 25-100% of AI for 1 s were delivered to preselected tissue volumes. Pain questionnaires recording any side effects were completed by the patient and the investigator separately. Ultrasound images of the target volume were taken before, immediately after, and 1 week after treatment. RESULTS A total of 14 patients have been entered into this study to date. Seven patients were treated at their primary site and seven received treatment to one of their metastases. No treatment needed to be stopped because of pain. Eight of the 14 patients did not complain of any side effect during or after the treatment. One patient complained of mild, and two of moderate pain during the week following treatment. One patient developed an asymptomatic blister on the skin. CONCLUSION Focused ultrasound is a safe, well-tolerated and non-invasive method of delivering ablative thermal energy to selected tumours. More clinical trials are needed to assess the role of this modality in the treatment of cancer.

Surgical management of primary retroperitoneal sarcoma

Local recurrence after surgical resection is the main cause of disease‐related mortality in patients with primary retroperitoneal sarcoma (RPS). This study analysed predictors of local recurrence and disease‐specific survival.

Mammography in the Assessment of Response to Medical Treatment of Large Primary Breast Cancer

The sequential mammograms of 48 patients (median age: 54 years, range: 24-84 years) undergoing primary medical treatment for large operable breast cancer at this institution were reviewed, and compared to the findings at clinical assessment. Twenty-six patients underwent endocrine therapy using tamoxifen, and 22 chemotherapy (CMF or MMM). All had more than two mammograms which were reviewed blindly by two independent radiologists. Response to treatment by both evaluation modalities was recorded using WHO definitions (NC, PR, CR, PD). Some 81% of patients achieved an objective clinical response to treatment, whilst 63% showed mammographic response. Overall comparison of clinical examination with mammography showed agreement in 38 patients (79%) and disagreement in 10 (21%). Agreement in type of response but not in its degree was found in 22 cases (46%). The results suggest that in the majority of cases mammography provides a useful adjunct to clinical examination in monitoring response to primary medical treatment for breast cancer. However, radiological factors such as unchanging microcalcification, and the continuing presence of mammographic density contribute to the discrepancy with clinical response in some tumours, and the search for alternative modalities of assessment should continue.

Solitary Fibrous Tumors of the Soft Tissues: Review of the Imaging and Clinical Features With Histopathologic Correlation

OBJECTIVE Solitary fibrous tumors are rare soft-tissue tumors of submesothelial origin with variable malignant potential. Most of these tumors originate within the thoracic cavity, but they can occur in a variety of sites, including the abdomen, pelvis, and soft tissues and muscles. The purpose of this study was to review the imaging findings with clinicopathologic correlation in 34 cases. CONCLUSION The finding of a large, solid, vascular tumor, particularly with prominent feeding vessels or a visible fatty component, should alert the radiologist to the possible diagnosis of solitary fibrous tumor. Percutaneous biopsy carries minimal risk and should be used for definitive diagnosis of these lesions, which in many cases are curable with surgery. The prognosis is good for patients with benign tumors but variable for those with malignant tumors.

The Role of Ultrasound-guided Cytology of Groin Lymph Nodes in the Management of Squamous Cell Carcinoma of the Vulva: 5-Year Experience in 44 Patients

AIM To assess the accuracy of ultrasound combined with fine-needle aspiration cytology (FNAC) in the detection of lymph node metastasis in patients with squamous cell carcinoma of the vulva. MATERIALS AND METHODS The groin nodes of 44 consecutive patients with primary squamous cell carcinoma of the vulva undergoing groin node dissection were assessed with ultrasound and FNAC. The results were compared with histology from subsequent inguinofemoral lymph node dissection. Twenty-nine patients underwent bilateral groin node dissections and 15 unilateral providing comparable data for 73 groins. RESULTS Histology demonstrated metastatic disease in 28 groins and no evidence of metastatic disease in 45. Ultrasound agreed with the histology in 67 of the 73 groins (92%), with two false-positives, four false-negatives and two indeterminate appearances. Cytology agreed with the histology in 65 of 72 FNAC samples obtained (90%), with six false-negatives, and one indeterminate result. No false-positive cytology results were seen. Ultrasound and FNAC together failed to detect metastatic disease in four groins, one with an indeterminate ultrasound appearance, another with indeterminate cytology, the two others each having a single positive inguinal node despite a negative ultrasound and FNAC. CONCLUSION The combination of ultrasound and FNAC provides a sensitive and specific tool for pre-operative assessment and may prevent unnecessary groin dissection and the attendant morbidity in selected patients with vulval cancer.

Adult Rhabdomyosarcoma: Cross-Sectional Imaging Findings Including Histopathologic Correlation

OBJECTIVE The purpose of our study was to present the MRI and CT features of adult rhabdomyosarcomas with histopathologic correlation. Forty-nine sequential cases were incorporated over a 5-year period from the sarcoma unit database. Twenty-six patients had adequate imaging (16 MRI, 10 CT) and histopathology available for retrospective review. The alveolar subtype was present in 13 patients, embryonal subtype in four patients, and pleomorphic subtype in nine patients. On both CT and T1-weighted MRI, all tumors were isodense to skeletal muscle, although enhancement was variable after the administration of IV contrast material. Pleomorphic tumors were very high signal on T2-weighted/STIR imaging, and both pleomorphic and alveolar subtypes were extremely heterogeneous. Embryonal tumors were more homogeneous. CONCLUSION Although adult rhabdomyosarcomas have certain imaging appearances in common with other soft-tissue sarcomas, features at presentation such as tumor heterogeneity, site, regional lymphadenopathy, and pulmonary metastasis should make the radiologist consider this important diagnosis.

The role of percutaneous nephrostomy in malignant urinary tract obstruction

Percutaneous nephrostomy is a well established technique for rapid relief of ureteric obstruction and improvement of renal function. However, its role in the management of renal failure resulting from advanced abdominopelvic malignancy is controversial and there are no clear guidelines to predict which patients benefit from such intervention both in terms of survival time and quality of life. To establish a protocol for selection of patients with abdominopelvic malignancy most likely to benefit from nephrostomy for renal obstruction, the medical records of 50 consecutive patients undergoing this procedure at the Royal Marsden Hospital were reviewed. The patients were divided into four groups: Group I, renal obstruction caused by a nonmalignant complication as a result of previous surgery or radiotherapy (n = 8); Group II, renal obstruction due to untreated primary malignancy (n = 16); Group III, renal obstruction from relapsed disease with a viable treatment option (n = 8); and Group IV, relapsed disease with no conventional treatment option (n = 18). There was significant benefit from percutaneous nephrostomy in Groups I-III. The overall median survival time of Group IV patients was extremely poor: 38 days (range 6-143 days) with no long-term survivors. The results suggest that strict selection criteria should be applied to patients with a history of abdominopelvic malignancy before proceeding to percutaneous nephrostomy. No worthwhile benefit is obtained if nephrostomy is used as a palliative measure in the absence of definitive treatment.

Computed tomography in the assessment of response to limb compression in unilateral lymphoedema

In this prospective study computed tomography (CT) was used to monitor the response of compression therapy in 27 patients with chronic unilateral lymphoedema over a 12 week period. Computed tomography examination of abnormal and normal limbs (proximal and distal portions) was performed in the first, third and 12th weeks of treatment. Changes in cross-sectional area (CSA) and average densities of the different compartments within the proximal and distal portions of the abnormal limb were compared with the normal side. The most significant decrease in CSA occurred within the subcutaneous compartment of the distal portion (P = 0.002); the decrease in CSA of the proximal portion was also significant (P = 0.02) but changes in muscle and bone compartments were not significant. Significant differences in average density measurements of the subcutaneous and muscle compartments remained between normal and abnormal limbs following the conclusion of the study (P = 0.001 and P = 0.01, respectively). This study demonstrates that CT is a useful method for monitoring therapeutic response to compression therapy.

Leiomyosarcomas of the inferior vena cava: diagnostic features on cross-sectional imaging

AIM To evaluate the cross-sectional radiological appearances and to review the clinical presentation and outcome of patients with leiomyosarcomas of the inferior vena cava (IVC LMS). These are rare aggressive tumours that present late with non-specific symptoms and have a poor prognosis. MATERIALS AND METHODS From January 2002 to December 2008, the radiological images of 23 sequential patients with pathologically proven IVC LMS were independently reviewed by two experienced radiologists. The clinical presentation, treatment including surgical details, and outcome were recorded. RESULTS There were 19 females and four males with a mean age of 53 years. CT typically demonstrated a large, lobulate, non-calcified heterogeneous mass with peripheral enhancement. T1-weighted magnetic resonance imaging (MRI) images demonstrated a mass with a low signal intensity and T2-weighted MRI images demonstrated a mass with a high signal intensity. Clinical presentation included leg oedema, back and abdominal pain with almost 50% of patients presenting with metastases. Eleven patients underwent ablative surgery. The mean survival time of all patients in the study was 34 months and that of the 11 post-surgical patients was 56 months. CONCLUSION There are a variety of diagnostic features on both computed tomography (CT) and MRI which aid the diagnosis of this unusual vascular neoplasm. CT is vital in determining the location of the tumour within the IVC and MRI accurately depicts its extent and the potential for surgical resectability, which offers the only chance of survival.