Abdominal Imaging Findings in Neurocutaneous Syndromes: Looking Below the Diaphragm.

OBJECTIVE: Neurocutaneous syndromes are a heterogeneous group of multisystemic disorders. We review the multimodality imaging findings in the disorders with a propensity for intraabdominal disease. We highlight more recently recognized disease patterns and discuss imaging surveillance optimization. CONCLUSION: Knowledge of varied intraabdominal phenotypic expressions can increase diagnostic rates. Limitation of radiation dose must be considered in screening this tumor-prone population. Early detection of neoplastic findings is critical for morbidity and mortality reduction.

An Investigative Study of Hepatic Arterial Anomalies in a West Indian Population.

PURPOSE: There are many known variations in the arterial supply to the liver. We sought to document the incidence and details of anomalies of the extrahepatic arteries in an unselected population in the West Indies. METHODS: This study spanned 24 months. All 205 CT scans were evaluated at a hepatobiliary referral center in Trinidad and Tobago. We described the anomalies of the arterial supply to the liver using the conventional classification proposed by Michels. RESULTS: 205 CT scans were evaluated, and 112 persons (54.6%) had conventional Type 1 anatomy. However, compared to the incidence in the existing medical literature, we encountered a greater incidence of replaced right hepatic arteries (18.1% vs 11%; P 0.04) and a lower incidence of accessory right hepatic arteries (2.4% vs 7%; P 0.030). CONCLUSION: Although 54.6% of persons in this West Indian population have conventional hepatic arterial supply, the distribution of anatomic variants of the right hepatic artery is quite different to that seen in North American and European centers. We found a higher incidence of replaced right hepatic arteries and a lower incidence of accessory right hepatic arteries.

A pictorial review of the less commonly encountered patterns of metastatic prostate carcinoma.

Usually late in the course of advanced prostate carcinoma, atypical nodal and distant metastases may be encountered. Accurate characterisation of disease spread and assessment of disease response have significant treatment and prognostic implications. Surveillance imaging, therefore, along with clinical and biochemical parameters, is a key factor in directing appropriate management. Atypical metastases may also require histological re-evaluation, as they may indicate differentiation into aggressive histologic subtypes, which can lead to management alteration. We present a pictorial review of the less common patterns of metastatic prostate carcinoma, to aid in timely recognition and diagnosis.

Biparametric MRI prior to Radical Radiation Therapy for Prostate Cancer in a Caribbean Population: Implications for Risk Group Stratification and Treatment.

Purpose: To assess risk-group migration and subsequent management change following biparametric MRI using a risk-stratified approach in a group of Caribbean men with prostate adenocarcinoma being offered external beam radiation therapy (EBRT). Materials and Methods: This retrospective study assessed the biparametric MRI findings in men who opted for EBRT from January 2018 to June 2019 (n = 79; mean age, 67.9 years ± 6.2 [standard deviation]). Serum prostate-specific antigen level, digital rectal examination findings, histologic grade group (GG) from transrectal US-guided biopsy, prior androgen deprivation therapy (ADT), and any prior CT results were used to stratify patients into low-, intermediate-, and high-risk groups, according to the National Comprehensive Cancer Network risk categories. Risk-group stratification prior to MRI separated patients into low- (seven of 79 [8.9%]), intermediate- (36 of 79 [45.6%]) and high-risk (36 of 79 [45.6%]) groups. Following MRI, any risk group (low, intermediate, high, nodal involvement, and metastatic disease) or oncologic management changes were recorded. Multivariable binary logistic regression analyses were used to assess predictor of upgrade status, with adjustment for demographic covariates jointly. Results: Following MRI, 30 of 79 (38.0%) patients had risk-group upshifts compared with their original assessment. Patients were recategorized into low risk (one of 79, 1.3%), intermediate risk (19 of 79, 24.1%), high risk (51 of 79, 64.6%), nodal involvement (one of 79, 1.3%), and metastatic disease (seven of 79, 8.9%). From the original groupings, there were six of seven (85.7%) from the low group, 18 of 36 (50.0%) from the intermediate group, and six of 36 (16.7%) from the high group that had risk group upward shifts. There was no association with GG: GG2 versus GG1, P = .53; GG3 versus GG1, P = .98; or prior ADT (P = .37) and the adjusted odds of risk-group upshifts. MRI findings resulted in treatment plan modification for 39 of 79 (49.4%) men overall. Conclusion: Prostate MRI should be considered for patients in high-risk populations prior to EBRT because upstaging from MR image assessment may have implications for modification of treatment.Keywords: MR-Imaging, Prostate, Radiation Therapy© RSNA, 2020See the commentary by Davenport and Shankar in this issue.

Imaging features of thalassaemia.

This article highlights the range of osseous findings that can be encountered as well as the imaging features of extramedullary haematopoiesis. As iron overload remains a major cause of morbidity and mortality in these disorders, we also discuss the MRI evaluation of hepatic and cardiac hemosiderosis, to aid in the optimization of iron chelation therapy. Future imaging use will be dictated by evolving clinical needs, such as in screening for emerging morbidities, including hepatic fibrosis and hepatocellular carcinoma.

Multi-organ benign and malignant tumors: recognizing Cowden syndrome: a case report and review of the literature.

BACKGROUND: Cowden syndrome is an autosomal dominant disorder with a predisposition to multiple benign and malignant tumors. In our patient, in addition to breast and endometrial malignancies as well as facial trichilemmomas, she was noted to have multiple meningiomas, pancreatic lipomas and lung cysts. These latter lesions have been noted in previous Cowden syndrome case reports, but are not included in the diagnostic criteria at this time. To our knowledge, this is the first case of multiple meningiomas in this syndrome. Further studies are therefore warranted to assess the significance of these findings in Cowden syndrome. CASE PRESENTATION: A middle-aged Afro-Caribbean known endometrial carcinoma patient (post surgery and adjuvant radiotherapy), presented with a locally advanced breast carcinoma. She received neoadjuvant chemotherapy followed by a modified radical mastectomy and axillary lymph node clearance. Her past medical history included a sphenoid wing meningioma for which she received definitive external beam radiotherapy. She was also known to have a multinodular goiter, anal polyp and longstanding mucocutaneous lesions. Further workup revealed additional smaller meningiomas, a parotid arteriovenous malformation, a lung cyst and pancreatic lipomas. Overall, consortium criteria were met for the diagnosis of Cowden syndrome. Furthermore, genetic testing identified a pathogenic mutation in the PTEN gene. She will be closely followed with annual clinical examination, dermatologic assessment and screening colonoscopies. She will perform interval whole body contrast enhanced CT for continued surveillance for metastatic disease. CONCLUSION: Cowden syndrome is likely to be an under diagnosed condition, but critically important to identify due to its cancer predisposition. When encountering multi-organ tumors, diagnostic criteria for Cowden syndrome should be sought in order to increase the diagnostic rates. Cancer surveillance for carcinoma detection in the early and curative stages remains the most critical aspect of management.

Encephalopathy and Neuropathy due to Glue, Paint Thinner, and Gasoline Sniffing in Trinidad and Tobago-MRI Findings.

A 29-year-old male petrol station pump attendant was admitted with ataxia and clinical evidence of a sensorimotor polyneuropathy which developed over the preceding 3 months. He had cognitive dysfunction, hearing loss, and cerebellar clinical abnormalities that came on slowly over the three years. He had a fifteen-year history of sniffing mostly glue, occasionally paint thinners, and, in the recent two years, gasoline. Magnetic resonance brain imaging showed abnormalities of the cerebral cortex, cerebral white matter, corpus callosum, hippocampus, brainstem and cerebellar atrophy, hypointensities of basal ganglia, red nuclei, and substantia nigra as previously described in toluene sniffing. Abstinence for six months led to partial clinical improvement. Clinicians need to be aware of this preventable entity which has peculiar radiological findings which are being increasingly accepted as typical.

Breast cancer and mammography: knowledge, attitudes, practices and patient satisfaction post-mammography at the San Fernando General Hospital, Trinidad.

Self-administered questionnaires were completed by 314 Trinidadian women, 40 years and older, to ascertain their breast cancer knowledge, attitudes, and practices. Those with higher education had greater knowledge of the benefits of early breast cancer detection (p < .01) and knew that an abnormal mammography result (p = .01) or recall (p < .01) was not necessarily indicative of breast cancer. Inaccurate beliefs particularly that breast compression causes cancer were more likely among those with the least education (p = .04). Obesity and alcohol were identified as risk factors by 29.3% and 12.4%, respectively. Over two-thirds (70%) of mammograms were primarily the doctor's decision. Over 90% of women were satisfied post-mammography, most (94.6%) with plans for future use. Some (15.6%) reported pain and 48.7% reported discomfort, with over 70% of these finding the examination less painful than anticipated. Targeted culturally sensitive awareness campaigns are needed. Clinicians must be sensitized to their importance in recommending mammography. Data on patient satisfaction and pain perception can be publicized to encourage women who are hesitant about mammography.

Plexiform neurofibroma of the wrist: imaging features and when to suspect malignancy.

Plexiform neurofibromas are essentially pathognomonic for neurofibromatosis type 1 (NF1), occurring when there is diffuse involvement along a nerve segment and its branches. Transformation into a malignant peripheral nerve sheath tumour (MPNST) is a major cause of mortality in NF1 patients. These tumours are highly aggressive and particularly difficult to diagnose in NF1 patients due to the clinical overlap between benign and malignant lesions. We present a case of a plexiform neurofibroma and discuss the typical imaging characteristics on ultrasound, CT, and MRI, including the target sign and continuity with the parent nerve. Certain imaging features should raise suspicion for malignancy however, these modalities may not always reliably differentiate between benign and malignant lesions. Recent studies show a very high negative predictive value for FDG-PET making it quite useful in excluding malignancy. In positive scans, PET/CT aids in guiding biopsy to the most metabolically active area of the tumour.

Peritoneal tuberculosis mimicking advanced ovarian carcinoma: an important differential diagnosis to consider.

BACKGROUND: Female patients who present with ascites, adnexal masses and elevated CA125 levels are typically presumed to have advanced ovarian carcinoma. This can lead to radical surgery with its associated morbidity. An important differential diagnosis to consider is tuberculous peritonitis which can present in a similar manner and responds well to medical treatment. CASE PRESENTATION: A 44 year old female presented with abdominal distension, weight loss and low grade fever. Her CA125 level was 909 U/ml. Imaging studies revealed an adnexal lesion and ascites. The lungs appeared normal and a Mantoux test was negative. Ovarian malignancy was highly suspected. Cytology of ascites was negative for malignant cells. The patient subsequently developed a large pleural effusion which was drained and negative for malignant cells and acid fast bacilli. Repeat imaging revealed a 'tree in bud' appearance of the lung parenchyma and dense ascites. Histology from diagnostic laparotomy revealed caseating granulomas with epithelioid cells and Langhan's type giant cells. The patient responded well to antituberculosis therapy with normalization of CA125 levels, confirming the diagnosis of peritoneal tuberculosis. CONCLUSION: CA125 levels lack specificity, with elevated levels encountered in many benign and malignant conditions, including tuberculosis. There are a few discriminating features that suggest a diagnosis of tuberculous peritonitis rather than ovarian carcinoma. Apart from chest findings which may not always be present, smooth peritoneal thickening and a dirty omentum on CT favours a diagnosis of peritoneal tuberculosis compared with nodular thickening of the peritoneum and omentum in peritoneal carcinomatosis. PCR and ADA testing of ascitic fluid can also be helpful. When these tests are negative or unavailable then diagnostic laparoscopy or laparotomy should be performed with the aid of frozen section to avoid unnecessary radical surgery in cases of peritoneal tuberculosis.

Tuberous sclerosis presenting with spontaneous pneumothorax secondary to lymphangioleiomyomatosis; previously mistaken for asthma.

A middle-aged female patient, previously diagnosed with asthma, presented with a large spontaneous left pneumothorax. She had a history of nephrectomy for a ruptured renal angiomyolipoma (AML) with a postoperative spontaneous pneumothorax when she was an adolescent. High-resolution CT chest revealed multiple scattered thin-walled lung parenchyma cysts consistent with lymphangioleiomyomatosis (LAM). Hepatic AMLs and adenoma sebaceum skin lesions were also noted, consistent with an overall diagnosis of tuberous sclerosis. Her acute management included lung re-expansion via chest tube insertion, antibiotics for concurrent chest infection, nebulisation and chest physiotherapy. Since discharge, the patient had only occasional shortness of breath, relieved by bronchodilators. She is considering expanded immunisation as well as enrolment in a clinical trial. Her hepatic AMLs will be monitored via ultrasound for growth. LAM treatment is generally aimed at its complications with lung transplantation reserved for severe disease; however, hormonal therapy and the mTOR inhibitor aim at targeting systemic disease.