Angela A. Giardino

A to Z of Desmoid Tumors

OBJECTIVE The purpose of this article is to illustrate the common locations of desmoid tumors (deep fibromatosis), complications of intra- and extraabdominal desmoids, and treatment-related changes in their imaging appearance. CONCLUSION Desmoids are locally aggressive fibrous tumors with a tendency to recur. Desmoids can be intraabdominal, in the abdominal wall, or extraabdominal. Complications, such as compression or invasion of adjacent structures, and abscess formation can occur. Treatment options include observation, local treatment (surgery, radiotherapy), or systemic therapy (conventional chemotherapy, molecular targeted agents).

A to Z of Extraskeletal Ewing Sarcoma Family of Tumors in Adults: Imaging Features of Primary Disease, Metastatic Patterns, and Treatment Responses

OBJECTIVE Though classically thought of as an osseous-based sarcoma of children and adolescents, it is important to recognize that approximately 20% of all cases of Ewing sarcoma family of tumors (ESFT) occur in patients older than 18 years, often with extraskeletal disease. Understanding the specific imaging features, manners of presentation, and patterns of behavior of ESFT is therefore important in all settings. CONCLUSION This article presents the imaging findings of ESFT in the adult, with specific attention to extraskeletal manifestations.

Spleen in haematological malignancies: spectrum of imaging findings

Imaging morphology and metabolic activity of splenic lesions is of paramount importance in patients with haematological malignancies; it can alter tumour staging, treatment protocols and overall prognosis. CT, MRI and positron emission tomography (PET)/CT have been shown to be powerful tools for the non-invasive assessment of splenic involvement in various haematological malignancies. Since many haematological malignancies and non-neoplastic conditions can involve the spleen and imaging manifestations can overlap, imaging and clinical findings outside of the spleen should be looked for to narrow the differential diagnosis; confirmation can be obtained by pathological findings. Radiologists should be familiar with the cross-sectional imaging patterns of haematological malignancies involving the spleen as well as non-neoplastic splenic findings common in these patients to facilitate their care and follow-up. This pictorial review provides the common and uncommon imaging appearances and complications of various haematological malignancies involving the spleen on CT, MRI and PET/CT, and common pitfalls in diagnosis.

Typical and atypical metastatic sites of recurrent endometrial carcinoma

Abstract The purpose of this article is to illustrate the imaging findings of typical and atypical metastatic sites of recurrent endometrial carcinoma. Typical sites include local pelvic recurrence, pelvic and para-aortic nodes, peritoneum, and lungs. Atypical sites include extra-abdominal lymph nodes, liver, adrenals, brain, bones and soft tissue. It is important for radiologists to recognize the typical and atypical sites of metastases in patients with recurrent endometrial carcinoma to facilitate earlier diagnosis and treatment.

Imaging in Renal Cell Carcinoma

Imaging plays a central role in the detection, diagnosis, staging, and follow-up of renal cell carcinoma (RCC). Most renal masses are incidentally detected with modern, high-resolution imaging techniques and a variety of management options exist for the renal masses encountered today. This article discusses the role of multiple imaging modalities in the diagnosis of RCC and the imaging features of specific pathologic subtypes and staging techniques. Future directions in RCC imaging are presented, including dynamic contrast-enhanced and unenhanced techniques, as well as the development of novel tracers for positron emission tomography.

A phase 2 study of Rituximab-Bendamustine and Rituximab-Cytarabine for transplant-eligible patients with mantle cell lymphoma.

Chemoimmunotherapy followed by autologous stem cell transplantation (ASCT) is a standard therapy for transplant‐eligible patients with newly diagnosed mantle cell lymphoma (MCL). The achievement of complete remission (CR) and minimal residual disease (MRD) negativity are associated with better outcomes. We tested an induction regimen of rituximab/bendamustine followed by rituximab/high‐dose cytarabine (RB/RC). This phase 2 study (NCT01661881) enrolled 23 transplant‐eligible patients aged 42–69, of whom 70% were MCL international prognostic index low‐risk. Patients received three cycles of RB followed by three cycles of RC. The primary endpoint of the trial was the rate of CR after six cycles of therapy, with a rate of 75% considered promising. 96% of patients achieved a CR/unconfirmed CR after treatment, meeting the primary objective. One patient progressed on study, one declined ASCT in CR, and the remaining 21 underwent successful stem cell collection and ASCT. After a median follow‐up of 13 months, the progression‐free survival rate was 96%. Among 15 MRD‐evaluable patients who completed treatment, 93% achieved MRD negativity after RB/RC. In conclusion, RB/RC achieves very high CR and MRD negativity rates in transplant‐eligible patients, with a favourable safety profile. RB/RC warrants further comparative studies, and may become a useful alternative to RCHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)‐based induction regimens in this patient population.

Imaging features of primary and secondary malignant tumours of the sacrum

Malignant tumours of the sacrum may be primary or secondary. While sacral metastases are frequently encountered, a diagnostic dilemma can present when there is a single sacral bone tumour with no history or evidence of malignancy elsewhere in the body. Familiarity with the imaging features and clinical presentations of primary malignant bone tumours is helpful in narrowing the differential. This pictorial review will illustrate with both common and uncommon malignant sacral tumours CT, MRI and positron emission tomography/CT, highlighting the specific features of each.

Extrahepatic spread of hepatocellular carcinoma: spectrum of imaging findings.

OBJECTIVE The purpose of this article is to describe the imaging findings of extrahepatic spread of hepatocellular carcinoma (HCC). CONCLUSION Detecting extrahepatic metastases in HCC is critical in determining the optimal treatment plan for patients. Identifying the presence of extrahepatic metastases in patients with advanced disease may eliminate unnecessary surgery, such as liver transplantation or partial hepatic resection, and help direct the appropriate therapy.

The addition of sirolimus to the graft-versus-host disease prophylaxis regimen in reduced intensity allogeneic stem cell transplantation for lymphoma: a multicentre randomized trial

Inhibition of the mechanistic target of rapamycin (mTOR) pathway has clinical activity in lymphoma. The mTOR inhibitor sirolimus has been used in the prevention and treatment of graft‐versus‐host disease (GVHD) after allogeneic haematopoietic stem cell transplantation (HSCT). A retrospective study suggested that patients with lymphoma undergoing reduced intensity conditioning (RIC) HSCT who received sirolimus as part of their GVHD prophylaxis regimen had a lower rate of relapse. We therefore performed a multicentre randomized trial comparing tacrolimus, sirolimus and methotrexate to standard regimens in adult patients undergoing RIC HSCT for lymphoma in order to assess the possible benefit of sirolimus on HSCT outcome. 139 patients were randomized. There was no difference overall in 2‐year overall survival, progression‐free survival, relapse, non‐relapse mortality or chronic GVHD. However, the sirolimus‐containing arm had a significantly lower incidence of grade II‐IV acute GVHD (9% vs. 25%, P = 0·015), which was more marked for unrelated donor grafts. In conclusion, the addition of sirolimus for GVHD prophylaxis in RIC HSCT is associated with no increased overall toxicity and a lower risk of acute GVHD, although it does not improve survival; this regimen is an acceptable option for GVHD prevention in RIC HSCT. This trial is registered at clinicaltrials.gov (NCT00928018).

Extrapulmonary Small Cell Carcinoma: A Pictorial Review

OBJECTIVE Extrapulmonary small cell carcinoma (EPSCC) is a rare, aggressive neoplasm arising from virtually any organ. Numerous oncologic studies have addressed prognostic indicators and survival rates in EPSCC, however relatively little has been published regarding the imaging features and metastatic patterns of these uncommon tumors. This article provides a pictorial review of EPSCC in multiple organs, emphasizing the imaging appearance at presentation and the radiologic patterns of recurrence/metastasis. CONCLUSION Although the appearance of EPSCC is often nonspecific, the typical presentation is large aggressive tumors that, similar to small cell carcinoma in the lung, often respond well to local therapy but tend to recur relentlessly at distant sites.