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.

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.

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.

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.

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.

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.

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.

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.

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.

Routine computerized tomography scanning, groin ultrasound with or without fine needle aspiration cytology in the surgical management of primary squamous cell carcinoma of the vulva.

We set out to study whether computerized tomography (CT) scanning of the vulva and the groin and groin ultrasound scanning (USS) alone or with fine needle aspiration cytology (FNAC) (USS/FNAC) influenced or could influence the surgical management of primary squamous cell carcinoma of the vulva (SCCaV). Forty-four patients underwent surgery for primary SCCaV following radiologic imaging by one or more modalities. Patient details included the clinical assessment of the carcinoma, radiologic findings, the operation performed, and whether the decision regarding the type and extent of surgery for the vulval carcinoma and, in particular, for the groin node dissection was or could be influenced by the radiologic findings. The age range was 38-87 years, with a median of 74 years. A total of 75 groin dissections were performed. Twenty-five of the 44 patients (56.8%) did not have groin node metastasis, 14 had unilateral metastasis (31.8%), and 5 (11.4%) had bilateral metastasis. All cases with histologically proven nodal status were analyzed to compare the preoperative imaging status with the histology. The calculated sensitivity, specificity, negative predictive value, and positive predictive value for CT were 58%, 75%, 75%, and 58%, for USS alone-87%, 69%, 94%, and 48%, and for USS-guided FNAC-80%, 100%, 93%, and 100%, respectively. There was no patient in whom surgical planning for the vulval carcinoma or the groin nodes was or could be altered by the CT findings. The data do not support the routine use of CT scanning in patients with primary SCCaV, either in assessment of the primary vulval carcinoma or in detecting groin nodal metastases. For the groin nodes, USS/FNAC is superior to CT in assessing disease status. In contrast to CT, USS/FNAC may have a useful clinical role in the management of the groin nodes in vulval carcinoma.

The soft-tissue manifestations of neurofibromatosis type 1.

The radiological appearances of neurofibromatosis type 1 (NF-1) are numerous and variable, because of the widespread presence of peripheral nerves. Knowledge of this variability can prevent unnecessary intervention. For example, occasionally lesions can be misinterpreted and biopsies performed unnecessarily. Thus, familiarity with the manifestations of this disease and the spectrum of associated abnormalities is an important part of the radiologist's armamentarium. This paper explores the manifold radiological appearances of extracranial NF-1 as experienced by the Sarcoma and Soft Tissue Tumour Unit at the Royal Marsden Hospital.

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.

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.

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.