Overview of malignant soft-tissue sarcomas of the limbs.

Although soft-tissue masses are common, sarcomas are rare malignant neoplasms showing variable mesenchymal differentiation and can occur at any anatomical site. Limb soft-tissue sarcomas (STS) are rare, but often lethal tumours. Although there are scores of historical pathological subtypes, this article will deal with the commonest: liposarcoma, leiomyosarcoma (LMS), undifferentiated pleomorphic sarcoma (UPS), synovial sarcoma, myxofibrosarcoma, malignant peripheral nerve sheath tumour (MPNST), epithelioid sarcoma, alveolar rhabdosarcoma, angiosarcoma and radiation-induced sarcoma (RIS). Following a review of >4,000 adult patients with limb sarcoma from our specialist soft-tissue tumour database, we summarise the literature and their imaging findings, with emphasis on radiological hallmarks that can aide in diagnosis and management. Increased awareness of sarcoma when challenged with a new mass in the extremity can ensure timely and appropriate treatment.

Prospective cohort study of ultrasound surveillance of regional lymph nodes in patients with intermediate-risk cutaneous melanoma.

BACKGROUND: For patients with intermediate-thickness melanoma, surveillance of regional lymph node basins by clinical examination alone has been reported to result in a larger number of lymph nodes involved by melanoma than if patients had initial sentinel node biopsy and completion dissection. This may result in worse regional control. A prospective study of both regular clinical examination and ultrasound surveillance was conducted to assess the effectiveness of these modalities. METHODS: Between 2010 and 2014, patients with melanoma of thickness 1·2-3·5 mm who had under-gone wide local excision but not sentinel node biopsy were recruited to a prospective observational study of regular clinical and ultrasound nodal surveillance. The primary endpoint was nodal burden within a dissected regional lymph node basin. Secondary endpoints included locoregional or distant relapse, progression-free and overall survival. RESULTS: Ninety patients were included in the study. After a median follow-up of 52 months, ten patients had developed nodal relapse as first recurrence, four had locoregional disease outside of an anatomical nodal basin as the first site of relapse and six had relapse with distant disease. None of the patients who developed relapse within a nodal basin presented with unresectable nodal disease. The median number of involved lymph nodes in patients undergoing lymphadenectomy for nodal relapse was 1 (range 1-2; mean 1·2). CONCLUSION: This study suggests that ultrasound surveillance of regional lymph node basins is safe for patients with melanoma who undergo a policy of nodal surveillance.

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.

Soft-tissue masses in the abdominal wall.

Masses involving the abdominal wall arise from a large number of aetiologies. This article will describe a diagnostic approach, imaging features of the most common causes of abdominal wall masses, and highly specific characteristics of less common diseases. A diagnostic algorithm for abdominal wall masses combines clinical history and imaging appearances to classify lesions.

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.

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.

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.

Imaging of skeletal metastases in myxoid liposarcoma.

Unlike other soft tissue sarcomas, myxoid/round cell liposarcoma (MRCL) has a tendency to spread to extrapulmonary sites but bone metastases are thought to be uncommon. In case reports, negative bone scintigraphy has been noted in patients with myxoid/round cell liposarcoma and bone metastases but the prevalence and optimal method of diagnosis of bone metastases in this common subtype of liposarcoma are unclear. In an attempt to answer these questions, data were obtained from a prospective database of patients with sarcoma, including MRCL, and the diagnostic imaging used was examined. A variety of imaging tools were used including plain X-rays, bone scintigraphy, computed tomography (CT), and magnetic resonance imaging (MRI). Eight patients (4.3%) developed skeletal metastases all of which were positive on MRI. Bone scintigraphy was negative in two out of four cases, CT was negative in six out of seven, and X-rays were negative in four. Radiography and CT measure mainly cortical bone involvement, whereas MRI examines bone marrow. When investigating patients with MRCL for bone pain, negative X-rays and bone scans do not rule out bone metastases. In our experience, MRI provides the most sensitive technique for the diagnosis of bone metastases in MRCL.

CT imaging improves histopathological grading of retroperitoneal leiomyosarcomas.

BACKGROUND: Initial grading of retroperitoneal leiomyosarcoma (LMS) is performed by core biopsy (CB) however, discrepancy between grade of tumour at initial CB and surgical excision is recognised, raising concerns about the accuracy of CB for directing neoadjuvant therapy. The histological grading system used for staging LMS consists of 3 components: tumour differentiation, mitotic index and proportion of necrosis. We postulate that assessment of necrosis by histopathology alone is inadequate, resulting in under-grading of LMS. We propose and assess a combined grading system that incorporates CT scan findings into pre-surgical grading. METHODS: Retrospective, blinded review of CT, CB histology and final surgical histology of patients with retroperitoneal LMS was undertaken. A modified grading system, CTH-Grade, was derived by replacing the CB necrosis score with a CT-derived necrosis score. The sensitivity and specificity of CTH-Grade, the standard histopathology scoring, H-grade were compared. Inter-observer variability in assessment of CT necrosis was also assessed. RESULTS: 53 patients fulfilled criteria for inclusion. CT was more sensitive at detection of necrosis than CB histology alone with sensitivity of 100% vs 53%. The use of CTHGrade resulted in increased detection of high-grade tumours with CTH-grade having sensitivities of 80% and 35% for Grade 2 and 3 tumours respectively vs 53% and 15% with H-Grade. Assessment of reader agreement demonstrated Kappa scores of 0.8. CONCLUSION: Histology from CB under-grades LMS due to undersampling of tumour necrosis. CT is more sensitive in assessing necrosis and its incorporation into a modified CT-histopathology grading system (CTH-Grade) improves accuracy of grading with significant implications for patient management.

Aggressive fibromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response.

BACKGROUND: One of the commonly used systemic agents for the treatment of aggressive fibromatosis is the anti-oestrogen drug tamoxifen. However, data on efficacy and optimum methods of response assessment are limited, consisting mainly of small case series and reports. METHODS: A retrospective database was used to identify consecutive patients diagnosed with aggressive fibromatosis (AF) and treated with tamoxifen plus/minus non-steroidal anti-inflammatory drugs at our tertiary referral centre between 2007 and 2014. MRI and symptom changes were recorded. RESULTS: Thirty-two patients (13 male 19 female, median age 41 years) were included. Median duration of treatment with tamoxifen was 316 days. Of 9 patients with progressive disease by RECIST 1.1 (28%): 4 patients experienced worsening symptoms; 3 patients had improved symptoms and 2 had no change in symptoms. Of 22 patients with stable disease (69%): 11 had no change in symptoms; 6 had improved symptoms and 5 patients had worsening symptoms. One patient achieved a partial response with improved symptoms. CONCLUSIONS: No relationship was identified between symptomatic benefit and response by RECIST 1.1 on MRI. Prospective studies in AF should incorporate endpoints focusing on patient symptoms.

Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.

Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera.

Incidence and significance of abdominal lymphadenopathy in hairy cell leukaemia.

We have investigated the incidence and significance of abdominal lymphadenopathy in hairy cell leukemia (HCL) by routinely CT scanning 88 patients. These included 70 men and 18 women with a median age of 51 years (range 25-83). Abdominal CT scans were performed at diagnosis in 29 patients and in 59 during the course of the disease. Abdominal lymphadenopathy was documented in 25 patients (28%) overall; the incidence of abdominal lymphadenopathy was higher in relapse (56%) than at diagnosis (17%). All patients with lymphadenopathy had bone marrow disease. There is no association with age or sex but lymphadenopathy tends to be more common in patients with long-standing disease (median duration of disease 6 years v one year in those without nodes) and in patients with bulky disease, particularly in the relapse group (splenomegaly/splenectomy in 95% vs 40%). The presence of abdominal nodes is also associated with relative resistance to treatment, with more treatment failures and fewer complete responses seen in this group. Most patients with lymphadenopathy had large, immature-looking hairy cells present in both the bone marrow and lymph nodes, when these were examined. Abdominal lymphadenopathy in HCL is more common than previously recognised, particularly in relapsed patients, and is always associated with active disease. The presence of large hairy cells and the relative resistance to treatment suggest that this phenomenon represents a form of transformation of the disease. Longer follow up is required to confirm this.

Segmentation of mammograms using multiple linked self-organizing neural networks.

A possible first stage in the analysis of the mammographic scene is its segmentation into four major components: background (the nonbreast area), pectoral muscle, fibroglandular region (parenchyma), and adipose region. An algorithm has been developed for this task. It is based on the classification of a feature vector constructed from statistical measures of texture calculated at two window sizes. Separate self-organizing neural networks are trained on sample data taken from each of the four regions. The feature vectors from the entire mammogram are then classified with the trained networks linked via a decision logic. To overcome the variability of texture between mammograms the algorithm uses data from a mammogram to classify itself in a staged approach consisting of several binary decisions. The training regions for each successive stage are determined from geometric information produced by the previous stages. The dataset in the study consisted of thirty (fifteen pairs) digitized normal mammograms of variable radiographic appearance. As a measure of performance, the outlines of the parenchyma were compared to those drawn by a radiologist experienced in reading mammograms. Comparison of the areas and perimeters generated by the human and computer observers gives a relationship with correlation coefficients of 0.74 and 0.59 for each measure, respectively. The overlapping areas of the parenchymas segmented by the observers normalized by the combined area was also calculated for each case. The mean and standard deviation of this measure was 0.69 +/- 0.12.

Duodenal leiomyosarcoma as a cause of retroperitoneal sepsis.

The case of a patient with a duodenal leiomyosarcoma presenting with sepsis is presented. The necrotic centre of the tumour communicated with the lumen of the duodenum without causing duodenal obstruction. Resection of the duodenum with tumour offers the only chance of cure.

Measurement of tumour size in case selection for breast cancer therapy by clinical assessment and ultrasound.

AIMS: Clinical assessment of tumour size is often used to choose between mastectomy and primary medical therapy. Clinical and imaging modalities may have varying levels of accuracy across the range of tumour sizes. The aim of this study was to compare the accuracy of clinical measurement and ultrasound in discriminating palpable tumours up to 3 cm vs those greater than 3 cm. METHODS: A prospective analysis of 111 consecutive patients with palpable breast cancer was performed. All women had clinical measurement by caliper and ultrasound assessment prior to any needle biopsy. Clinical measurement and ultrasound assessment of size were compared to pathological tumour size of the surgical specimen. RESULTS: Both clinical and ultrasound measurement underestimate the size of larger tumours. The overall accuracy of clinical assessment and ultrasound examination in correctly identifying a 30 mm cut off was 70.3 and 77.5%, respectively. Ultrasound was significantly more accurate at determining the size of tumours <30 mm (p=0.007) but there was no significant difference between both modalities in assessing tumours greater than 30 mm. CONCLUSIONS: Ultrasound assessment of breast cancer size is more accurate than clinical assessment for tumours less than 30 mm. As clinical examination is as accurate on ultrasound for tumours greater than 30 mm, clinical assessment of tumour size alone is adequate to select patients for primary medical therapy or mastectomy.

The use of ultrasound by breast surgeons in outpatients: an accurate extension of clinical diagnosis.

AIMS: To assess the accuracy of breast ultrasound scan (USS) performed by a surgeon in outpatients and to evaluate the additional contribution of USS to clinical diagnosis. METHODS: A prospective study of 302 patients with symptomatic breast disease (322 lumps) was performed. Group 1 consisted of 213 clinic USS of lumps surgically removed for appropriate clinical indications. In Group 2, a USS was performed on 231 lumps by both the surgeon and radiologists as part of triple assessment. Each clinic USS was compared to the surgical pathology (Group 1) or USS performed by the radiologist (Group 2). RESULTS: In Group 1 (n=213), 89 lumps were proven benign and 124 malignant on histology. Ultrasound scans performed by the surgeon compared to histology had a sensitivity of 98.3% and specificity of 91.7%. An abnormal clinic USS heightened the index of suspicion in 22/213 (10.3%) of cases felt clinically to be benign but subsequently confirmed malignant on histology. Fifty-seven lumps felt to be indeterminate clinically were correctly identified on USS by surgeon as benign (n=56) or malignant (n=1). In Group 2 (n=231), there was complete concordance of USS scans by surgeon and radiologists in 197 (96%) and complete discordance in eight (3.9%) patients. Of the discordant scans, the surgeon correctly identified 7/8 diagnoses on histology. A USS examination by the radiologists provided a correct diagnosis of 6/14 scans scored by the clinician as indeterminate. CONCLUSION: USS performed in outpatients by a breast surgeon is accurate and a useful adjunct to clinical assessment. This enables rapid diagnosis in one-stop breast clinics, selecting difficult diagnostic procedures for USS by radiologists at the same visit.

A retrospective study comparing the individual modalities of triple assessment in the pre-operative diagnosis of invasive lobular breast carcinoma.

AIMS: Early invasive lobular breast carcinoma (ILC) is associated with few symptoms and signs. The individual sensitivity of clinical examination, mammography, ultrasonography, cytology and core biopsy have each been reported to be of limited value. The aim of this study was to evaluate the accuracy of triple assessment in the pre-operative detection of patients identified to have ILC from their surgical pathology. METHODS: Pure ILC was defined as tumours containing at least 90% lobular features. The triple assessment of 273 patients diagnosed primarily at our institution were reviewed. RESULTS: 87.5% of women were symptomatic and 12.5% were screen detected. The mean patient age was 59 (range 30-81) years and the median tumour size was 26 (range 5-110) mm. The main mammographic abnormalities were a spiculated lesion (33.3%), an ill-defined mass (33.3%) or architectural distortion (23.5%). The sensitivities for detecting ILC of each modality were: clinical examination (76.6%), mammography (79.8%), ultrasound examination (93.9%), fine-needle aspiration cytology (FNAC) (60.5%) and core biopsy (90.8%). Combining the three modalities of clinical examination, imaging and cyto/pathology increased the pre-operative detection rate of ILC. CONCLUSION: Triple assessment is useful in the diagnosis of ILC. As the features of ILC may be subtle, a high index of suspicion is required to facilitate early diagnosis.

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.

Chest radiography in the management of breast cancer.

This study was undertaken to assess the utility of chest radiography (CXR) in the management of patients with breast cancer and to devise a suitable imaging policy for such patients. A retrospective analysis was performed of the case notes and the CXRs of a series of 141 patients presenting to this hospital in 1980 with a diagnosis of early breast cancer. Data retrieved covered the entire clinical course to date and included the number of CXRs performed for a clinical reason and the number performed as "routine". Correlation of the result of every CXR requested to subsequent therapeutic decision-making throughout the complete clinical course of each patient was undertaken and the proportion of CXRs initiating a management change, or performed for a clinical reason, out of the total requested was assessed. A total of 1161 CXRs were performed on the 141 patients studied. Of these, only 174 (15%) were undertaken for a direct clinical reason and 987 (85%) were undertaken as part of "routine" follow-up procedures and had no impact on patient management. Four "routine" CXRs (< 0.4%) demonstrated previously undiagnosed pulmonary metastases, in patients with no other history of metastatic disease. Thus, routine chest radiography is not a cost-effective method of monitoring asymptomatic patients with breast cancer for metastasis. It is recommended that outside staging procedures for clinical trials, the CXR is used only to address a clinical problem relating specifically to the thorax. It is anticipated that the cessation of all other chest radiography in patients being followed up for breast cancer at this institution will have significant budgetary implications.

Focal myositis, a benign inflammatory pseudotumour: CT appearances.

Focal myositis is a rare benign pseudotumour of skeletal muscle, of unknown cause. Clinically it presents as an enlarging mass within muscle, usually of an extremity, and is often mistaken for a soft tissue neoplasm. The diagnosis is made by biopsy which reveals characteristic histological changes of inflammation, focal degeneration and regeneration, and some evidence of denervation. The CT findings include irregularity and enlargement of the muscles involved, with diffuse, poorly defined fatty infiltration of the muscle planes, but no evidence of an associated mass. We present two cases of focal myositis of the calf, both of which mimicked a soft tissue neoplasm, and in which CT was helpful in determining the nature and extent of the abnormality, for needle biopsy, and follow-up.