Pseudoprogression in patients with uterine leiomyosarcoma treated with first-line single-agent doxorubicin.

Aim: To evaluate the incidence of pseudoprogression in patients with metastatic or inoperable uterine leiomyosarcoma (LMS) treated with first-line single-agent doxorubicin.

Methods: The Royal Marsden NHS Foundation Trust Sarcoma Unit database was searched to identify all patients with metastatic or inoperable LMS treated with first-line doxorubicin from January 2006 to January 2022. Patients with available computed tomography scans performed at baseline and during doxorubicin therapy were included.

Imaging features of primary sites and metastatic patterns of angiosarcoma.

Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease.

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).

Descriptive analysis of MRI functional changes occurring during reduced dose radiotherapy for myxoid liposarcomas.

Objectives: Myxoid liposarcomas (MLS) show enhanced response to radiotherapy due to their distinctive vascular pattern and therefore could be effectively treated with lower radiation doses. This is a descriptive study to explore the use of functional MRI to identify response in a uniform cohort of MLS patients treated with reduced dose radiotherapy.

Methods: 10 patients with MLS were imaged pre-, during, and post-radiotherapy receiving reduced dose radiotherapy and the response to treatment was histopathologically assessed post-radiotherapy.

Imaging Soft-tissue Sarcomas of the Head and Neck: A Tertiary Soft-tissue Sarcoma Unit Experience.

Background/aim: To describe imaging features of head and neck soft-tissue sarcomas.

Patients And Methods: Patients with a diagnosis of head and neck sarcoma between 2011 and 2015 were reviewed.

Results: There were a total of 62 patients (24 female; median age=60 years).

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.

Outcome of Primary Desmoid Tumors at All Anatomic Locations Initially Managed with Active Surveillance.

Background: The behavior of desmoid tumors is unpredictable and varies from spontaneous remission to symptomatic and radiologic progression. This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance.

Methods: Patients with a primary desmoid tumor at any anatomic location diagnosed between 1998 and 2016 were identified in a prospectively maintained database from a single sarcoma reference center in the United Kingdom.

Utility of Multi-Parametric Quantitative Magnetic Resonance Imaging for Characterization and Radiotherapy Response Assessment in Soft-Tissue Sarcomas and Correlation With Histopathology.

To evaluate repeatability of quantitative multi-parametric MRI in retroperitoneal sarcomas, assess parameter changes with radiotherapy, and correlate pre-operative values with histopathological findings in the surgical specimens. Thirty patients with retroperitoneal sarcoma were imaged at baseline, of whom 27 also underwent a second baseline examination for repeatability assessment. 14/30 patients were treated with pre-operative radiotherapy and were imaged again after completing radiotherapy (50.

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.

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.

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.

Paratesticular Sarcoma: Typical Presentation, Imaging Features, and Clinical Challenges.

Objective: To describe the major imaging features, together with clinical data, of paratesticular sarcomas.

Materials And Methods: A retrospective analysis was performed of available imaging and clinical data of 77 consecutive cases of paratesticular sarcoma referred to the soft tissue sarcoma center at the Royal Marsden hospital between January 2006 and January 2015.

Results: Of the total cases, 87% had been referred postoperatively, 43% of which had been imaged preoperatively and 24% of which required re-resection due to incomplete initial excision.

Computerised cognitive testing of individuals with Down's syndrome and Alzheimer's disease.

Background: Although Alzheimer's disease neuropathology is present by age 40 years, in Down's syndrome, ante-mortem diagnosis is difficult. No standard for cognitive assessment is well established or easy to administer.

Method: We used a simple, widely available computerised test (NeuroTrax Moderate to Severe Impairment Assessment Battery, Mindstreams, Newark, NJ) to evaluate 14 patients in this population.

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.

MEN1 Syndrome and Hibernoma: An Uncommonly Recognised Association?

MEN1 syndrome is known to classically result in parathyroid, pituitary, and pancreatic islet cell tumours. However, the potential association of MEN1 syndrome with hibernoma, a benign tumour with differentiation towards brown fat, is far less well known, despite their genetic profile both being linked to deletion of the MEN1 gene. Herein, we describe a case with its key radiological and pathological findings.

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.

Fistulation as a complication of intra-abdominal soft-tissue sarcomas; a case series.

Soft-tissue sarcomas are rare, accounting for only one percent of all cancers. They can occur in retroperitoneal and intraperitoneal sites, including gastrointestinal stromal tumours (GIST), and have the potential to cause complications secondary to interaction with other abdominal viscera. Fistulation, or an abnormal communication between two epithelium-lined surfaces that do not usually connect, is a rare example of such a complication.

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.

Desmoplastic small round cell tumour: the radiological, pathological and clinical features.

Objectives: Desmoplastic small round cell tumours (DSRCTs) are rare aggressive tumours of young adults that present late and have poor prognosis. This review discusses distinctive radiological features, histopathology and clinical course of this soft-tissue sarcoma.

Methods: From 1991 to 2012, the radiology of 20 patients with pathologically proven DSRCT was independently reviewed by two experienced radiologists.

Screening for ovarian cancer in women with varying levels of risk, using annual tests, results in high recall for repeat screening tests.

Background: We assessed ovarian cancer screening outcomes in women with a positive family history of ovarian cancer divided into a low-, moderate- or high-risk group for development of ovarian cancer.

Methods: 545 women with a positive family history of ovarian cancer referred to the Ovarian Screening Service at the Royal Marsden Hospital, London from January 2000- December 2008 were included. They were stratified into three risk-groups according to family history (high-, moderate- and low-risk) of developing ovarian cancer and offered annual serum CA 125 and transvaginal ultrasound screening.

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.

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.