Desmoid-type fibromatosis.

Desmoid-type fibromatosis is a rare, locally infiltrative, mesenchymal neoplasm that is associated with high rates of local recurrence but lacks the potential to metastasise. The disease affects younger individuals, with a peak age of 30 years, and is the most common cause of an anterior abdominal wall mass in young women of childbearing age. It may, however, involve nearly every body part, including the extremities, head and neck, trunk, and abdominal cavity; as such, desmoid-type fibromatosis may present to a range of general and subspecialty radiologists. These rare tumours have a widely variable clinical presentation and unpredictable natural history, hence input from a soft-tissue tumour centre is recommended, although much of the imaging may be performed at the patient's local hospital. The consensus for treatment has changed over the past decade, with most centres moving away from primary radical surgery towards a front-line 'watch-and-wait' policy. Therefore, imaging has an increasingly important role to play in both the diagnosis and follow-up of these patients. This review will discuss the typical imaging characteristics of these lesions and suggest diagnostic and follow-up magnetic resonance imaging protocols, with details of suitable sequences and scanning intervals.

Extragonadal teratomas of the adult abdomen and pelvis: a pictorial review.

Teratomas comprise a spectrum of tumours that have striking imaging appearances and are commonly considered when evaluating a mass in the female pelvis. A subgroup of these tumours located in an extragonadal abdominopelvic location, in contrast, are extremely rare and can affect both sexes. Extragonadal teratomas can occur at all ages, are particularly unusual in adults and can cause confusion in the differential diagnosis, especially in children. Familiarity with the imaging features of the spectrum of teratomas within the abdominal cavity is therefore of great importance, as radiological diagnosis can guide treatment, prevent delays in diagnosis and avoid sequelae. This article summarizes the radiological appearances of these rare extragonadal tumours in adults in relation to their pathology, malignant potential, location and behaviour. Although uncommon, teratomas should be considered in the differential diagnosis of extragonadal abdominal masses, particularly in young adults.

Tail-end troubles: imaging of soft-tissue buttock tumours.

Primary soft-tissue buttock tumours are relatively common entities, although they are infrequently reported in the literature. The buttock can be a difficult anatomical site to treat soft-tissue tumours due to the proximity of the sciatic nerve and the propensity of tumours at this site to extend into the pelvis and perineum. Therefore, the radiologist plays an important role in the multidisciplinary assessment of these lesions. Cross-sectional imaging, principally magnetic resonance imaging, is used to determine the exact location and extension of the tumour. Furthermore, certain tumours have characteristic imaging appearances that can help to establish a suitably ordered differential diagnosis. From our prospectively maintained database at The Royal Marsden Hospital, including 225 cases that were treated at the Sarcoma Unit over a 30 year period, we present examples of benign and malignant primary soft-tissue buttock tumours and describe the pertinent imaging characteristics, with emphasis on computed tomography and magnetic resonance imaging findings.

Surgical management of primary retroperitoneal sarcoma.

BACKGROUND: 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. METHODS: A prospective database was reviewed to identify patients who underwent surgery for primary RPS between 1990 and 2009. Patient demographics, operative outcomes and tumour variables were correlated with local recurrence and disease-specific survival. Multivariable analysis was performed to evaluate predictors for local recurrence and disease-free survival. RESULTS: Macroscopic clearance was achieved in 170 of 200 patients. The median weight of tumours was 4.0 kg and median maximum diameter 27 cm. Resection of adjacent organs was required in 126 patients. The postoperative mortality rate was 3.0 per cent. Seventy-five patients developed local recurrence during follow-up. At 5 years the local recurrence-free survival rate was 54.6 per cent and the disease-specific survival rate 68.6 per cent. Inability to obtain macroscopic clearance at resection and high-grade tumours were significant predictors for local recurrence and disease-specific survival. CONCLUSION: Complete macroscopic excision should be the goal of surgical resection. Ability to resect a RPS completely and tumour grade are the most important predictors of local recurrence and overall survival.

The radiological manifestations of intramuscular haemangiomas in adults: magnetic resonance imaging, computed tomography and ultrasound appearances.

OBJECTIVE: Intramuscular haemangiomas in adults may appear similar to soft tissue sarcomas on imaging. This study evaluates the imaging characteristics of intramuscular haemangiomas on magnetic resonance imaging (MRI), computed tomography (CT) and ultrasound. SUBJECTS AND METHODS: Retrospective review of medical records, imaging and pathology details of 34 patients presenting with intramuscular haemangiomas was performed. Age of patient, size, site of lesion and type of imaging was recorded. Lesions were evaluated for calcification, presence of fat, outline, texture, vascularity, enhancement and involvement of local structures. RESULTS: There were 12 male and 22 female patients aged between 9 years and 90 years (mean 39 years). Twenty-two lesions involved muscles of the extremities and 12 involved muscles of the trunk. Mean duration of the lesions was long: 59.3 months (range 3-240 months), with an average size of 7.5 cm (1.5-15 cm). Imaging comprised 27 MR studies, seven CT scans, 19 ultrasounds, and eight plain films. All lesions were well defined, lobulated and heterogeneous. None showed local invasion. On MRI and CT, 93-100% showed the presence of fat and 100% showed vascular channels and enhancement on MRI, CT and ultrasound. Phleboliths were seen on four plain films (50%) and on the corresponding MR images. On MRI, 70% of lesions had mildly hyperintense signal on T1-weighted sequences and 96% had hyperintense signal on T2-weighted sequences (relative to skeletal muscle). Histopathology confirmed diagnosis in all cases, showing the presence of fat, medium/large vessels and skeletal muscle. CONCLUSION: Intramuscular haemangiomas have characteristic appearances on MRI, CT and ultrasound. Long history, presence of fat, calcification and internal vessels should alert the radiologist to this diagnosis prior to biopsy.

Imaging features of retroperitoneal and pelvic schwannomas.

AIM: To describe the imaging features of retroperitoneal and pelvic schwannomas. MATERIALS AND METHODS: The presenting cross-sectional imaging for 18 sequential patients with retroperitoneal or pelvic schwannomas was reviewed retrospectively. Note was made of tumour diameter, position, homogeneity, margin, shape, calcification and invasion into adjacent structures. Where MRI had been performed, T1 and T2 signal intensity relative to skeletal muscle, and the degree and pattern of enhancement with gadolinium, were also assessed. RESULTS: Imaging from 13 patients was available for review. The mean tumour diameter was 8.7 cm (range 4 to 15 cm); 9 schwannomas were located in the pelvis and 4 in the retroperitoneum; 12 cases showed smooth, regular margins and 1 case irregular, invasive margins. The tumours were homogeneous in 5 cases and heterogeneous with cystic change in 8; in 2 cases there was smooth expansion of a sacral nerve root exit foramen, and in 1 there was bony destruction of the sacrum and extension of tumour into the spinal canal. In 5 cases MRI was performed; on T1-weighted images all tumours were isointense; on T2-weighted images 4 tumours were hyperintense and 1 was isointense to skeletal muscle. In all cases the diagnosis was confirmed by core biopsy. CONCLUSION: Retroperitoneal and pelvic schwannomas typically form large, well-circumscribed masses in the retroperitoneum or presacral area, and frequently undergo cystic degeneration. They can occasionally cause bony changes in the spine, but otherwise do not invade or obstruct adjacent structures. Although they are rare, it is important for the radiologist to recognize the typical appearance of schwannomas because they can be mistaken for malignant tumours.

Imaging in vulval cancer.

Cancer of the vulva spreads locally and, almost without exception, to the regional nodes in the groin to the superficial then deep inguinal groups and the pelvic nodes in a step-wise fashion. Because the single most important prognostic factor is the presence or absence of nodal disease, accurate diagnosis of nodal involvement is paramount. In the past, the status of inguinal nodes in vulval cancer has been ascertained only following groin node dissection, except in those cases with clinically obviously groin nodes. However, as up to 70% of patients at all stages of disease have negative nodes histologically, and as up to 70% of patients have groin or lower-limb problems after radical groin surgery, this approach incurs 'unnecessary' surgery for the majority of patients with the attendant morbidity. Using new diagnostic imaging methods, detection and assessment of groin lymph nodes has been developing over the past few years with the ultimate intention of reducing groin node surgery in node-negative patients. In this chapter we review the role of imaging in patients with vulval cancer in which there is a greater role in the assessment of nodal disease rather than in the assessment of the cancer on the vulva.

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.

The role of high resolution ultrasound with guided cytology of groin lymph nodes in the management of squamous cell carcinoma of the vulva: a pilot study.

The accuracy of high resolution ultrasound with guided fine needle aspiration cytology in detecting inguinal lymph node involvement was assessed in 24 women undergoing radical vulvectomy and groin node dissection for squamous cell vulval cancer. Of the 43 groins dissected, ultrasound correctly diagnosed the lymph node status in 36, with five false positive and two false negative results. Cytology in 40 groins showed no false positive and five false negative results. The sensitivity and specificity for the combined techniques were 83% and 82% respectively. Assessed together, the combined technique failed to detect metastatic disease in two groins; in both cases the extent of nodal metastatic involvement was a solitary focus < 3 mm in diameter. The ultrasound and fine needle aspiration procedure is safe and well tolerated and can be repeated as needed for surveillance. The authors suggest that this procedure should be evaluated further to determine whether a policy of individual selection for lymphadenectomy can be implemented based on this technique.

Ultrasound assessment of residual abnormalities following primary chemotherapy for breast cancer.

The purpose of this study was to assess the usefulness of ultrasonography (US) in the assessment of the breast following primary medical therapy (PMT) of large operable breast cancer. A total of 52 patients were studied; all had invasive breast cancer, confirmed by core biopsy, with initial size > 4 cm by palpation, T2-3, N0-1, M0. PMT was with epirubicin, cisplatin and continuous infusional 5-fluorouracil, as previously described (Jones et al, 1994, J Clin Oncol 12: 1259-1265). Independent clinical and US assessments were made during PMT before surgery or biopsy. A total of 31 (60%) patients achieved complete clinical response (cCR), but in only five of these was the post-treatment ultrasound normal. Post-treatment sonographic findings of diffuse parenchymal distortion or a mass lesion without Doppler signal were associated with more favourable histology (pathological CR, non-invasive or microinvasive carcinoma), whereas a mass with Doppler positivity was more often associated with residual macroscopic invasive carcinoma. Patients who did not achieve cCR had a high incidence of residual macroscopic carcinoma (71%) regardless of the sonographic characteristics. With median follow-up of 27 months (range 12-43), ten (19%) patients have relapsed and six (12%) have died, but only one relapse has occurred within treated breast. Ultrasonography is a sensitive technique for assessing the response to PMT, particularly in patients who achieve cCR. It may be helpful in selecting those patients who do not require post-PMT surgery and in localizing abnormalities in those who do, particularly in those with cCR. However, clinicians should be aware that a residual US abnormality is by no means pathognomonic of residual cancer.

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.

Surveillance after treatment for well differentiated thyroid cancer: audit for chest radiography.

Well differentiated thyroid carcinoma has an excellent prognosis, with many patients achieving cure. The incidence of pulmonary metastases is about 11%. When thyroid ablation has been used in the initial treatment, serial thyroglobulin (Tg) levels are a sensitive marker of recurrent disease. Data regarding the usefulness of chest radiography (CXR) in monitoring relapse in these patients does not exist and regular CXRs continue to be performed as routine surveillance. To assess their efficacy we have reviewed all serial CXRs and corresponding Tg levels on every patient with well differentiated thyroid cancer who was treated and followed up at the Royal Marsden Hospital between 1984 and 1987. A total of 369 CXRs were performed on 49 patients, with a median follow-up of 81 months. Of these, only 15% were undertaken for a clinical reason, with 85% being requested as routine. Seven patients developed lung deposits, six of whom had CXR performed because of elevated Tg, abnormal 131I uptake imaging or thoracic symptoms. In no case were lung metastases diagnosed by routine CXR in an asymptomatic patient. The cost of routine chest films for the duration of this study was approximately 13,500 pounds. It is concluded that routine chest radiography is neither cost effective nor a sensitive method of screening for relapse in asymptomatic well differentiated thyroid cancer. It should be reserved for patients with chest symptoms, or those in whom Tg has been shown not to be a useful marker of disease.

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.

Mammographic accuracy and patient age: a study of 297 patients undergoing breast biopsy.

This study was undertaken to compare the diagnostic accuracy of mammography in patients under 50 years with that of patients over 50, and with further subdivisions under 35 and 65 years. A retrospective review of the mammograms of 297 sequential patients undergoing mammography prior to surgical breast biopsy at the Breast Unit, Royal Marsden Hospital, London from 1988-1991 was performed. The films were reviewed blind by a single radiologist and correlated with histological findings at biopsy. The sensitivity and specificity of mammography compared with histology in four age-groups was assessed. There were 144 patients in the under-50 age group (< 35 years = 20, 35-49 years = 124), and 153 patients aged over 50 (50-64 years = 96, > 65 years = 57). Of all patients with histologically malignant biopsies 79% of the under-50s and 76% of the over-50s were diagnosed mammographically. Sensitivity of mammography was therefore 3% greater in the under-50 age-group (95% confidence interval (CI): +16% to -10%). Of patients who had benign biopsies, 76% of the under-50s and 75% of the over-50s were correctly diagnosed by mammography. Mammographic specificity was therefore 1% greater in the over-50 age-group (95% confidence interval: +15% to -14%). Accuracy of mammography was also comparable in the four subdivided age-groups. The results suggest that in patients requiring breast biopsy, contemporary mammography is similar in accuracy in the younger patient when compared to the older post-menopausal patient.

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.