BRCA-associated Cancers: Role of Imaging in Screening, Diagnosis, and Management.

Harmful mutations of the BRCA tumor suppressor genes result in a greater lifetime risk for malignancy-breast and ovarian cancers in particular. An increased risk for male breast, fallopian tube, primary peritoneal, pancreatic, prostate, and colon cancers also has been reported. The BRCA gene is inherited in an autosomal dominant pattern and tends to be highly penetrant; thus, there is an increased incidence of these cancers in affected families. Compared with sporadic tumors, BRCA-associated malignancies have unique manifestations, clinical features, and pathologic profiles. Manifestation at an early patient age, high-grade tumors, and an aggressive clinical course are common features of BRCA-associated malignancies. Understanding the behavior of these cancers aids in identification of affected individuals and families, who can then make informed decisions regarding their future health. Enhanced screening, prophylactic surgery, and chemoprevention are options for managing cancer risk factors in these individuals. Imaging has an important role in the screening, evaluation, staging, and follow-up of BRCA-associated malignancies. Supplemental screening of BRCA mutation carriers often begins at an early age and is critical for early and accurate cancer diagnoses. The authors review the etiopathogenesis and imaging features of BRCA-associated malignancies, the importance of a multidisciplinary approach to determining the diagnosis, and the treatment of patients who have these mutations to improve their outcomes. © RSNA, 2017.

Characterization of Anthropometric Changes that Occur During Neoadjuvant Therapy for Potentially Resectable Pancreatic Cancer.

BACKGROUND: Little is known about changes in body composition that may occur during neoadjuvant therapy for pancreatic cancer. This study was designed to characterize these changes and their potential relationships with therapeutic outcomes. METHODS: The study population consisted of patients with potentially resectable pancreatic cancer treated on a phase II trial of neoadjuvant chemotherapy and chemoradiation. Skeletal muscle and adipose tissue compartments were measured before and after administration of neoadjuvant therapy using SliceOMatic software (TomoVision, 2012) and protocol-mandated CT scans. Sarcopenia was defined using gender-adjusted norms. RESULTS: Among 89 eligible patients, 46 (52 %) patients met anthropometric criteria for sarcopenia prior to the initiation of neoadjuvant therapy. Further depletion of skeletal muscle, visceral adipose tissue, and subcutaneous adipose tissue occurred during neoadjuvant therapy, but these losses did not preclude the performance of potentially curative surgery. Degree of skeletal muscle loss correlated with disease-free survival while visceral adipose loss was associated with overall and progression-free survival. However, completion of all therapy, including pancreatectomy, was the only independently significant predictor of outcome in a multivariate analysis of overall survival. DISCUSSION: These data suggest that body composition analysis of standard CT images may provide clinically relevant information for patients with potentially resectable pancreatic cancer who receive neoadjuvant therapy. Anthropometric changes must be considered in the design of preoperative therapy regimens, and further efforts should focus on maintenance of muscle and visceral adipose tissue in the preoperative setting.

Tumor thrombus as a rare presentation of lymphoma: a case series of 14 patients.

OBJECTIVE: We present a case series of 14 patients with lymphoma presenting with tumor thrombus to highlight this rare but important manifestation of lymphoma. CONCLUSION: The dominant mass leading to tumor thrombus formation was nodal disease in nine patients and extranodal disease in five patients. Lymphoma should be considered in the differential diagnosis in the setting of tumor thrombus with solid organ involvement when there are other imaging features suggestive of lymphoma.

CAIPIRINHA-VIBE and GRAPPA-VIBE for liver MRI at 1.5 T: a comparative in vivo patient study.

OBJECTIVE: Three-dimensional T1-weighted (T1W) gradient recall echo volumetric interpolated breath-hold examination (VIBE) using generalized autocalibrating partially parallel acquisitions (GRAPPA) is one of the key sequences in liver magnetic resonance imaging (MRI) and is used for precontrast, dynamic postcontrast, and delayed postcontrast imaging. The purpose of this study is to compare image quality and liver lesion detection (LLD) on a shorter-duration T1W VIBE sequence using the controlled aliasing in parallel imaging results in higher acceleration (CAIPIRINHA) technique with the conventional T1W GRAPPA-VIBE sequence during a single liver MRI session on a 1.5-T Seimens scanner. METHODS: Twenty consecutive patients (9 women and 11 men; age range, 36-85 years) were included in this prospective study. All patients underwent a complete liver MRI on a 1.5-T magnet (Aera; Siemens Medical Systems, Erlangen, Germany) that consisted of a T1W (in/out-of-phase), T2W, DWI, and precontrast and postcontrast multiphasic images (late arterial, 50 seconds, 120 seconds, and 300 seconds) with GRAPPA-VIBE. The CAIPI-VIBE images were acquired for precontrast and at 300 seconds (5 minutes) postcontrast phases (6.9 seconds per phase) in addition to GRAPPA-VIBE (21 seconds per phase). The shorter time for the CAIPI-VIBE was selected to allow postprocessing of image acquisition in the setting of multi-late arterial phase (single breath hold) postcontrast images. Five radiologists independently analyzed image quality with predefined scores for liver edge sharpness, artifacts, fat saturation deficiency, visualization of the portal veins and hepatic veins, and LLD (size, <0.5-3.8 cm). Score 0 was suboptimal (inadequate), 1 was acceptable for diagnosis, and 2 was optimal (excellent). Kappa statistics were used to assess agreement among readers. Generalized linear mixed model with generalized estimation equation method was used to estimate and compare the LLD failure rates. RESULTS: No statistically significant difference was seen in the degree of reader variability between CAIPI-VIBE and GRAPPA-VIBE for all evaluated categories using multirater κ statistics. For the precontrast and 5-minutepostcontrast phase sequences, greater than 95% of images were considered to be of acceptable quality in all image quality categories for both sequences. Forty-one lesions were evaluated in 17 patients with total of 204 observations (n = 204) by 5 readers. For 5-minute postcontrast images, the LLD rate of CAIPI-VIBE (80%) was lower than GRAPPA-VIBE (84%) (P = 0.03) for small lesions (0.5-1.7 cm). There was no significant difference in lesion detection on precontrast images. CONCLUSIONS: At 1.5 T, the CAIPI-VIBE may be helpful in reducing scan time and demonstrates similar image quality compared with the traditional GRAPPA-VIBE. The CAIPI-VIBE has shorter breath-hold time requirement and thus can be an acceptable alternative for the precontrast and 5-minute postcontrast GRAPPA-VIBE in patients with breath-hold difficulties.

Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists.

Peritoneal disease can be caused by a wide spectrum of pathologies. While peritoneal disease is usually caused by primary or secondary malignancies, benign diseases can occur and mimic malignancies. This article begins with an overview of peritoneal embryology and anatomy followed by a detailed description of the multimodality imaging appearance of peritoneal diseases. Common diseases include peritoneal carcinomatosis, pseudomyxoma peritonei, lymphomatosis, sarcomatosis, and tuberculous peritonitis. The uncommon diseases which cause peritoneal disease include desmoid fibromatosis, desmoplastic small round cell tumor, malignant mesothelioma, well-differentiated mesothelioma, multicystic mesothelioma, papillary serous carcinoma, leiomyomatosis, extramedullary hematopoiesis, inflammatory pseudotumor and amyloidosis. This manuscript will help the radiologist become familiar with the different peritoneal spaces, pathways of spread, multimodality imaging appearance and differential diagnoses of peritoneal diseases in order to report the essential information for surgeons and oncologists to plan treatment.

Impact of ¹8F-FDG PET/CT on the management of adrenocortical carcinoma: analysis of 106 patients.

PURPOSE: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Limited data are available about on value of (18)F-FDG PET/CT in ACC. We evaluated the impact of PET/CT on the management of ACC. METHODS: We performed a retrospective review in patients with ACC who had undergone PET/CT. The impact of PET/CT on the management plan was evaluated by comparing the findings on PET/CT to the findings on contrast-enhanced CT. The sensitivity, specificity, and accuracy of each form of imaging were calculated. The correlations between PET/CT parameters, including maximum standardized uptake value (SUVmax), total lesion glycolysis, and decline in SUVmax after chemotherapy, and clinical outcome were evaluated. RESULTS: Included in the analysis were 106 patients with 180 PET/CT scans. Of the 106 patients, 7 underwent PET/CT only for initial staging, 84 underwent PET/CT only for restaging, and 15 underwent PET/CT for both initial staging and restaging. PET/CT changed the management plan in 1 of 22 patients (5%) at initial staging and 9 of 99 patients (9%) at restaging. In 5 of the patients in whom PET/CT changed the management plan, PET/CT showed response to chemotherapy but contrast-enhanced CT showed stable disease. Sensitivity, specificity, and accuracy were 100%, 100%, and 100% for PET/CT at initial staging; 92.6%, 100%, and 96.4% for CT at initial staging; 98.4%, 100%, and 99.5% for PET/CT at restaging; and 96.8%, 98.6%, and 98.0% for CT at restaging, respectively. No PET/CT parameters were associated with survival at either initial diagnosis or recurrence. CONCLUSION: PET/CT findings could substantially change the management plan in a small proportion of patients with ACC. Although lesion detection was similar between PET/CT and CT, PET/CT may be preferred for chemotherapeutic response assessment because it may predict response before anatomic changes are detected on CT.

Pelvic reconstruction with pedicled thigh flaps: indications, surgical techniques, and postoperative imaging.

OBJECTIVE: This article will describe and illustrate the relevant anatomy and surgical techniques used in pelvic reconstruction using regional pedicled thigh flaps, which is often necessary in oncologic surgeries. Examples of normal postoperative imaging and common complications that can accompany pelvic reconstruction with anterolateral, gracilis myocutaneous, and posterior thigh fasciocutaneous flaps will be provided. CONCLUSION: Pelvic reconstruction using regional pedicled thigh flaps is often needed with extirpative oncologic surgeries to eliminate dead space, provide pelvic organ support, restore form and function, and introduce vascularized tissue to promote wound healing. Radiologists need to be aware of the normal postoperative appearance of these flaps so that the flaps are not mistaken for residual or recurrent disease and so that residual or recurrent disease can be identified and treated.

Serum carbohydrate antigen 19-9 represents a marker of response to neoadjuvant therapy in patients with borderline resectable pancreatic cancer.

OBJECTIVES: The purpose of this study was to determine the relationship between carbohydrate antigen (CA) 19-9 levels and outcome in patients with borderline resectable pancreatic cancer treated with neoadjuvant therapy (NT). METHODS: This study included all patients with borderline resectable pancreatic cancer, a serum CA 19-9 level of ≥40 U/ml and bilirubin of ≤2 mg/dl, in whom NT was initiated at one institution between 2001 and 2010. The study evaluated the associations between pre- and post-NT CA 19-9, resection and overall survival. RESULTS: Among 141 eligible patients, CA 19-9 declined during NT in 116. Following NT, 84 of 141 (60%) patients underwent resection. For post-NT resection, the positive predictive value of a decline and the negative predictive value of an increase in CA 19-9 were 70% and 88%, respectively. The normalization of CA 19-9 (post-NT <40 U/ml) was associated with longer median overall survival among both non-resected (15 months versus 11 months; P = 0.022) and resected (38 months versus 26 months; P = 0.020) patients. Factors independently associated with shorter overall survival were no resection [hazard ratio (HR) 3.86, P < 0.001] and failure to normalize CA 19-9 (HR 2.13, P = 0.001). CONCLUSIONS: The serum CA 19-9 level represents a dynamic preoperative marker of tumour biology and response to NT, and provides prognostic information in both non-resected and resected patients with borderline resectable pancreatic cancer.

Multimodality imaging of vulvar cancer: staging, therapeutic response, and complications.

OBJECTIVE: Vulvar carcinoma is a rare malignancy, most commonly seen in elderly women. Survival depends on the lymph node status. Lymph node-negative patients have a 5-year survival of 90%, whereas survival drops to about 50% in patients with positive lymph nodes. The purpose of this article is to familiarize the reader with the revised International Federation of Gynecology and Obstetrics (FIGO) staging of vulvar cancer and incorporate it into daily practice when interpreting cross-sectional imaging studies. CONCLUSION: Although vulvar cancer is diagnosed clinically, cross-sectional imaging plays an important complimentary role in staging of the tumor, assessing extent of disease, and selecting operable versus inoperable candidates to ultimately help in decreasing morbidity and increasing survival in these patients.

Complications of Whipple surgery: imaging analysis.

BACKGROUND/AIMS: The purpose of this article is to describe and illustrate anatomic findings after the Whipple procedure, and the appearance of its complications, on imaging. CONCLUSION: Knowledge of the cross-sectional anatomy following the Whipple procedure, and clinical findings for associated complications, are essential to rapidly and accurately diagnose such complications on postoperative studies in order to optimize treatment.

Complications of oncologic therapy in the abdomen and pelvis: a review.

Cancer therapy has significantly improved in the past few decades with development of various newer classes of cytotoxic chemotherapy as well as novel, molecularly targeted chemotherapy. Similar to chemotherapy, radiotherapy is another important therapeutic option used in the curative and palliative management of various abdominal malignancies. However, both these treatments affect the tumor as well as the normal tissues, leading to significant toxicity. These side effects range from mild to life threatening, and may involve multiple organs. Imaging plays an important role in the early identification of such complications, which may allow more effective patient management. The aim of this article is to discuss and illustrate the wide spectrum of chemotherapy and radiotherapy induced complications in the abdomen and pelvis.

Solid pseudo-papillary tumors of the pancreas: current update.

Solid pseudo-papillary tumors are rare pancreatic tumors, which occur in females and are typically indolent neoplasms. However, atypical, aggressive variants can occur with locally advanced disease or metastases. They have characteristic imaging features, which vary according to size. This article provides a current update on the molecular biology, histopathology, clinico-radiological features, and management of these tumors.

Vascular pancreatic lesions: spectrum of imaging findings of malignant masses and mimics with pathologic correlation.

The differential diagnosis of hypervascular pancreatic lesions is complex, and includes endocrine and exocrine tumors of the pancreas, metastases to the pancreas, and masses, or mass-like lesions, arising from the neurovascular networks traversing the pancreas. In this manuscript, we will discuss salient imaging findings of these masses, pertinent differential diagnoses, as well as review clinical symptomatology that may aid in the diagnosis of some of these lesions.

Pancreatic neuroendocrine neoplasms: diagnosis and management.

Pancreatic neuroendocrine neoplasms are uncommon but rising in incidence. There have been recent changes in the WHO nomenclature and a newly proposed American Joint Committee on Cancer TNM staging, which complement each other. These neoplasms are of great medical and radiological interest because of their diverse presenting features and imaging appearances. There is an increased role for both anatomic and functional imaging in the assessment of these neoplasms. A review of the nomenclature, staging, and imaging is presented in this paper.

Response of borderline resectable pancreatic cancer to neoadjuvant therapy is not reflected by radiographic indicators.

BACKGROUND: Experience with preoperative therapy for other cancers has led to an assumption that borderline resectable pancreatic cancers can be converted to resectable cancers with preoperative therapy. In this study, the authors sought to determine the rate at which neoadjuvant therapy is associated with a reduction in the size or stage of borderline resectable tumors. METHODS: Patients who had borderline resectable pancreatic cancer and received neoadjuvant therapy before potentially undergoing surgery at the authors' institution between 2005 and 2010 were identified. The patients' pretreatment and post-treatment pancreatic protocol computed tomography images were rereviewed to determine changes in tumor size or stage using modified Response Evaluation Criteria in Solid Tumors (RECIST) (version 1.1) and standardized anatomic criteria. RESULTS: The authors identified 129 patients who met inclusion criteria. Of the 122 patients who had their disease restaged after receiving preoperative therapy, 84 patients (69%) had stable disease, 15 patients (12%) had a partial response to therapy, and 23 patients (19%) had progressive disease. Although only 1 patient (0.8%) had their disease downstaged to resectable status after receiving neoadjuvant therapy, 85 patients (66%) underwent pancreatectomy. The median overall survival duration for all 129 patients was 22 months (95% confidence interval, 14-30 months). The median overall survival duration for the patients who underwent pancreatectomy was 33 months (95% confidence interval, 25-41 months) and was not associated with RECIST response (P = .78). CONCLUSIONS: Radiographic downstaging was rare after neoadjuvant therapy, and RECIST response was not an effective treatment endpoint for patients with borderline resectable pancreatic cancer. The authors concluded that these patients should undergo pancreatectomy after initial therapy in the absence of metastases.

Defined clinical classifications are associated with outcome of patients with anatomically resectable pancreatic adenocarcinoma treated with neoadjuvant therapy.

BACKGROUND: We previously introduced a classification system for patients with localized pancreatic adenocarcinoma that integrates assessments of tumor anatomy, cancer biology, and patient physiology. By means of this system, we sought to analyze outcomes of patients with resectable anatomy but heterogeneous biology and physiology who were treated with neoadjuvant therapy. METHODS: We evaluated consecutive patients (2002-2007) with anatomically potentially resectable cancers treated with chemotherapy or chemoradiation before potential pancreatectomy. We compared clinical factors and outcomes of patients classified as having disease that was clinically resectable (CR; no extrapancreatic disease, preserved performance status); suspicion for extrapancreatic disease (BR-B); or marginal performance status or significant comorbidity (BR-C). Patients with borderline resectable anatomy (BR-A) were excluded. RESULTS: Resection rates for 138 CR, 41 BR-B, and 38 BR-C patients were 75, 46, and 37%, respectively (P < 0.001). Metastases, detected during treatment in 23% of patients, were the most common contraindication to resection among CR (15%) and BR-B (46%) patients. Performance status rarely precluded surgery except among BR-C (32%) patients. Factors associated with selection against surgery were older age, poor performance status, pain, and therapeutic complications (P < 0.05). The median overall survival of all patients was 21 months. Resected and unresected BR-B and BR-C patients had median overall survival durations similar to those of resected and unresected CR patients, respectively (P > 0.22). CONCLUSIONS: This system describes discrete clinical subgroups of patients with pancreatic cancer who have similar, potentially resectable tumor anatomy but heterogeneous physiology and cancer biology. It may be used with neoadjuvant therapy to predict outcomes, individualize treatment algorithms, and optimize survival.

Venous tumor thrombus in nonfunctional pancreatic neuroendocrine tumors.

OBJECTIVE: We sought to determine the incidence of venous tumor thrombus in nonfunctioning pancreatic neuroendocrine tumors. MATERIALS AND METHODS: We reviewed CT images of patients with a diagnosis of nonfunctional pancreatic neuroendocrine tumors over a 4-year period. For patients who underwent surgery, changes to surgical plans related to the tumor thrombus were also recorded. RESULTS: CT showed venous tumor thrombi in 29 of the 88 patients (33%; 95% CI, 23-44%). This CT finding was not accurately reported in 18 of the 29 patients (62%; 95% CI, 42-79%). Of the 39 patients who underwent surgery, venous tumor thrombi were detected in 11 patients (28%; 95% CI, 15-45%) and were confirmed by pathology. Microscopic venous tumor thrombi in 10 patients were not detected by CT. Pathologic results showed venous tumor thrombi in 21 of the 39 patients (54%; 95% CI, 37-70%) who underwent surgery. The surgical plan was significantly changed in two of the 11 patients with gross thrombi (18%; 95% CI, 2-52%) who underwent surgery. There was no change in the surgical plan for the 10 patients with microscopic tumor thrombi. CONCLUSION: The incidence of venous tumor thrombus detected by imaging was 33% in our study. This imaging finding was not accurately reported on the radiology report in 62% of the patients. In 18% of the patients with gross venous tumor thrombi, there was a significant alteration in the surgical plan. It is critical for the radiologist to be aware of the association of venous tumor thrombi in patients with nonfunctioning pancreatic neuroendocrine tumors and to report these findings.

Pathologic complete response to neoadjuvant therapy in patients with pancreatic ductal adenocarcinoma is associated with a better prognosis.

In patients with pancreatic ductal adenocarcinoma (PDA) who received neoadjuvant therapy and pancreatectomy, pathologic complete response (pCR) is rarely observed and the prognostic significance of pCR is not clear. In this study, we identified 11 patients with pCR (2.5%) from 442 patients with PDA who received neoadjuvant treatment and pancreatectomy from 1995 to 2010. There were 6 men and 5 women, with a median age of 61 years. Four patients had either synchronous or history of extrapancreatic cancer. Five patients received neoadjuvant chemotherapy followed by chemoradiation, and 6 received chemoradiation alone. Ten patients had pancreaticoduodenectomy, and 1 had distal pancreatectomy. Scar and chronic pancreatitis consistent with therapy effect were present in all cases (100%). Pancreatic intraepithelial neoplasia (PanIN) 3/carcinoma in situ was present in 5 cases, and PanIN1 and PanIN2 in 5 cases. However, no residual invasive carcinoma or lymph node metastasis was identified in all cases. Follow-up information was available in 10 patients. Follow-up time ranges from 6 to 194 months (median, 63 months). During the follow-up, 3 patients died of other causes, and 1 developed a second primary PDA in the tail of the pancreas at 84 months after the initial pancreaticoduodenectomy and died at 105 months after the initial diagnosis of PDA. The other 6 patients were alive with no evidence of disease. Patients with pCR had a better survival than did those who had posttherapy stage I or IIA disease (P < .001). Patients with PDA who received neoadjuvant therapy and had pCR in pancreatectomy are rare but have a better prognosis.

Role of magnetic resonance imaging as an adjunct to clinical staging in cervical carcinoma.

Magnetic resonance imaging depicts the morphological details of the female pelvis and is useful for evaluating both benign and malignant cervical masses. Clinical assessment of the extent of cervical cancer is crucial in determining the optimal treatment strategy, but clinical staging by itself has limitations. Clinical staging, as defined by FIGO (International Federation of Gynecologic Oncology), is based on the findings of physical examination, lesion biopsies, chest radiography, cystoscopy, and renal sonography and can be erroneous, depending on the stage of the disease, by 16% to 65%. The prognosis of cervical cancer is determined not only by stage, but also by nodal status, tumor volume, and depth of invasion, none of which are included in the FIGO guidelines. Magnetic resonance imaging has been described as the most accurate, noninvasive imaging modality in staging cervical carcinoma. This review outlines the magnetic resonance features of normal cervix, primary disease (by stage), and recurrent disease and discusses the role of magnetic resonance imaging in staging and clinical decision making.

Pancreas: peritoneal reflections, ligamentous connections, and pathways of disease spread.

The pancreas is a retroperitoneal organ with a close anatomic relationship to the peritoneal reflections in the abdomen, including the transverse mesocolon and the small bowel mesentery, and is directly contiguous to peritoneal ligaments such as the hepatoduodenal ligament, gastrohepatic ligament, splenorenal ligament, gastrocolic ligament, and the greater omentum. Understanding of these anatomic relationships of the pancreas is aided by knowledge of its embryologic development. These reflections and ligaments are potential pathways for spread of disease processes such as pancreatitis and pancreatic carcinoma. One can recognize these ligaments and reflections by identifying the blood vessels that traverse them.