Renal extramedullary hematopoiesis as an epiphenomenon of bone marrow dysfunction in a patient with primary myelofibrosis: A rare case report.

Extramedullary hematopoiesis represents a clinical compensatory condition characterized by the growth of hematopoietic tissue outside the bone marrow. It can mainly occur in patient with myeloproliferative disorders where alteration or neoplastic invasion of the bone marrow causes ineffective production of blood cells with the recruitment of progenitrix blood cells in non-hematopoietic organs, including kidneys. Renal extramedullary hematopoiesis is a rare condition manifesting as parenchymal or perirenal soft tissue masses with different patterns mimicking neoplasms, infectious or vascular diseases. We describe a unique case of a patient affected by primary myelofibrosis underwent ultrasound and magnetic resonance examinations showing bilateral perirenal alterations to be related to hemopoietic tissue. We also focused on the pathophysiology of this condition with imaging correlation. The case we present emphasises the importance of recognising the main radiological features of renal extramedullary hematopoiesis. MR examination should become part of the diagnostic pathway of the patient with primary myelofibrosis.

Quantitative Diffusion-Weighted MR Imaging: Is There a Prognostic Role in Noninvasively Predicting the Histopathologic Type of Uveal Melanomas?

Histopathologically, uveal melanomas (UMs) can be classified as spindle cell, mixed cell and epithelioid cell type, with the latter having a more severe prognosis. The aim of our study was to assess the correlation between the apparent diffusion coefficient (ADC) and the histologic type of UMs in order to verify the role of diffusion-weighted magnetic resonance imaging (DWI) as a noninvasive prognostic marker. A total of 26 patients with UMs who had undergone MRI and subsequent primary enucleation were retrospectively selected. The ADC of the tumor was compared with the histologic type. The data were compared using both one-way analysis of variance (ANOVA) (assessing the three histologic types separately) and the independent t-test (dichotomizing histologic subtypes as epithelioid versus non-epithelioid). Histologic type was present as follows: the epithelioid cell was n = 4, and the spindle cell was n = 11, the mixed cell type was n = 11. The mean ADC was 1.06 ± 0.24 × 10-3 mm2/s in the epithelioid cells, 0.98 ± 0.19 × 10-3 mm2/s in the spindle cells and 0.96 ± 0.26 × 10-3 mm2/s in the mixed cell type. No significant difference in the mean ADC value of the histopathologic subtypes was found, either when assessing the three histologic types separately (p = 0.76) or after dichotomizing the histologic subtypes as epithelioid and non-epithelioid (p = 0.82). DWI-ADC is not accurate enough to distinguish histologic types of UMs.

Hepatic Sarcoidosis: Lesson Based on a Case Report.

Sarcoidosis is a multisystemic disease of unknown etiology that can involve lungs, abdominal organs, and lymph nodes. The incidence of sarcoidosis is highest between the ages of 20 and 40 years, and it affects both sexes equally. The most frequent localization is in the lungs, and about half of the affected patients are generally asymptomatic but can involve in small percentages of various other parts such as the biliary tract, pancreas, stomach, and urinary tract. Hepatic and splenic localization is infrequent, and lesions are often mistaken for metastases due to their morphologic similarity. The histological lesion of sarcoidosis is a noncaseous granuloma always associated with high levels of angiotensin-converting enzyme, hypercalcemia, hypercalciuria, and, in a significant percentage of cases (35%-40%), liver enzyme alteration. The pathological evolution of granulomas is fibrosis, and the most severe cases of hepatic sarcoidosis can develop into cirrhosis or portal hypertension. Imaging is essential for lesion localization and is represented by ultrasound, computed tomography (CT), magnetic resonance imaging, and positron emission tomography/CT. The differential diagnosis is very difficult and is almost always histological. We describe a case of hepatic sarcoidosis in an asymptomatic patient with elevated liver enzymes.

Histopathologic and MR Imaging Appearance of Spontaneous and Radiation-Induced Necrosis in Uveal Melanomas: Initial Results.

Necrosis in uveal melanomas can be spontaneous or induced by radiotherapy. The purpose of our study was to compare the histopathologic and MRI findings of radiation-induced necrosis of a group of proton beam-irradiated uveal melanomas with those of spontaneous necrosis of a control group of patients undergoing primary enucleation. 11 uveal melanomas who had undergone proton beam radiotherapy, MRI and secondary enucleation, and a control group of 15 untreated uveal melanomas who had undergone MRI and primary enucleation were retrospectively identified. Within the irradiated and nonirradiated group, 7 and 6 eyes with histological evidence of necrosis respectively, were furtherly selected for the final analysis; the appearance of necrosis was assessed at histopathologic examination and MRI. Irradiated melanomas showed a higher degree of necrosis as compared with nonirradiated tumors. Irradiated and nonirradiated lesions differed based on the appearance and distribution of necrosis. Irradiated tumors showed large necrotic foci, sharply demarcated from the viable neoplastic tissue; nonirradiated tumors demonstrated small, distinct foci of necrosis. Radiation-induced necrosis, more pigmented than surrounding viable tumor, displayed high signal intensity on T1-weighted and low signal intensity on T2-weighted images. The hemorrhagic/coagulative necrosis, more prevalent in nonirradiated tumors (4 out of 6 vs. 1 out of 7 cases), appeared hyperintense on T2-weighted and hypointense on T1-weighted images. Our study boosts the capability to recognize radiation-induced alterations in uveal melanomas at MRI and may improve the accuracy of radiologists in the evaluation of follow-up MR examination after radiotherapy.

True congenital multicystic disease of the pancreas in the infant: a very rare case.

The most frequent pancreatic cysts in patients of developmental age are pseudocysts, neoplastic cysts, posttraumatic cysts, and parasitic cysts. Retention cysts, duplication cysts, and congenital cysts are called true cysts. Among the true cysts, congenital cysts are very rare and include 1% of all pancreatic cysts. The finding of true congenital pancreatic cysts in children under 3 years represents a very rare event; less than 30 have been described in the literature. We describe the case of a 3-year-old male who came to our observation for abdominal pain and underwent an abdomen ultrasound examination. The ultrasound examination showed unilocular sub-centimeter cystic lesions, distributed throughout the pancreatic parenchyma. The patient was, therefore, not subjected to any treatment, and after a few days, the symptoms regressed spontaneously. This description probably represents the first case in the literature of "congenital multicystic disease" of the pancreas.

Non traumatic intrathoracic liver herniation mimicking a pulmonary metastasis in patient with breast cancer: A case report.

Non-traumatic hepatic hernia is defined as hepatic protrusion through acquired or congenital defects on diaphragm without prior trauma. This event is rare among adults and infrequently reported in literature. 52-year-old Caucasian woman with surgically treated breast cancer with suspected lung metastasis detected during a routine Multidetector Computed Tomography lung exam. Ultrasound and subsequently Magnetic Resonance Imaging (MRI) was performed which revealed an overdiaphragmatic mass in contiguity with liver parenchyma compatible with overdiaphragmatic hepatic hernia. Differential diagnosis should be made with diaphragmatic or pulmonary nodule. Correct diagnosis can avoid further diagnostic investigations or invasive procedures such as biopsy. Magnetic Resonance Imaging is a non-risky method and can clarify interpretative doubts. Currently there are still controversies about traumatic or idiopathic nature of this hernia.

Diagnostic methods and therapeutic options of uveal melanoma with emphasis on MR imaging-Part II: treatment indications and complications.

Therapy of uveal melanoma aims to preserve the eye and its function and to avoid metastatic dissemination. The treatment choice is difficult and must keep into account several factors; the therapeutic strategy of uveal melanoma should therefore be personalized, sometimes requiring to combine different treatment techniques. Nowadays globe-sparing radiotherapy techniques are often preferred to enucleation. Plaque brachytherapy, the most commonly used eye-preserving therapy, is suitable for small- and medium-sized uveal melanomas. Proton beam radiotherapy is indicated for tumours with noticeable size, challenging shape and location, but is more expensive and less available than brachytherapy. Enucleation is currently restricted to advanced tumours, uveal melanomas with orbital or optic nerve involvement, blind and painful eyes because of treatment-related complications (neovascular glaucoma, chronic inflammatory processes). The effect of proton beam therapy on neoplastic tissue is related to direct cytotoxic action of the radiations, impairment of neoplastic vascular supply and immunologic response. Complications after radiotherapy are frequent and numerous and mainly related to tumour thickness, radiation dose and distance between the tumour and optic nerve. The purpose of this pictorial review is to provide the radiologists with awareness about diagnostic methods and therapeutic options of uveal melanoma. In the present second section, we discuss the therapeutic management of uveal melanoma, describing the main ocular-conserving radiotherapic techniques. We subsequently present an overview of the effects of radiations on neoplastic tissue. Lastly, we review ocular complications following radiotherapy that should be evaluated by radiologists during follow-up MRI examinations.

Diagnostic methods and therapeutic options of uveal melanoma with emphasis on MR imaging-Part I: MR imaging with pathologic correlation and technical considerations.

Uveal melanoma is a malignant neoplasm that derives from pigmented melanocytes of the uvea and involves, in order of decreasing prevalence, the choroid, ciliary body and iris. Its prognosis is related to histopathologic and genetic features, tumor size and location, extraocular extension. The diagnosis is fundamentally based on clinical evaluation (ophthalmoscopy, biomicroscopy) and ultrasonography. MRI is useful in case of untransparent lens or subretinal effusion. Moreover, MRI has a significant role to confirm the diagnosis, in the evaluation of the local extent of the disease with implications for treatment planning, and in the follow-up after radiotherapy treatment. Uveal melanoma can show different morphologic features (lentiform, dome or mushroom shape) and often determines retinal detachment. MR appearance of uveal melanoma mainly depends on the melanin content. Uveal melanoma typically displays high signal intensity on T1-weighted images and low signal intensity on T2-weighted images. Nevertheless, imaging appearance may be variable based on the degree of pigmentation and the presence of areas of necrosis or cavitation. Differential diagnosis includes other uveal lesions. The radiologists and in particular MRI play a significant role in the clinical management of uveal melanoma. The purpose of this pictorial review is to provide the radiologists with awareness about diagnostic methods and therapeutic options of uveal melanoma. In the present first section we summarize the MR anatomy of the eye and describe ophthalmological and radiological imaging techniques to diagnose uveal melanomas, with emphasis on the role of MR imaging. Additionally, we review MR imaging appearance of uveal melanomas.

Can Conventional and Diffusion-Weighted MR Enterography Biomarkers Differentiate Inflammatory from Fibrotic Strictures in Crohn's Disease?

Background and Objectives: To retrospectively assess the value of magnetic resonance enterography (MRE) parameters derived from conventional and diffusion weighted imaging (DWI) sequences to differentiate fibrotic strictures from inflammatory ones in adult patients with Crohn's disease (CD), using surgical specimens as the histopathological reference standard. Material and Methods: Twenty-three patients with CD who had undergone surgical resection of ileal strictures with full-thickness histopathologic analysis within 3 months from preoperative MRE were included. Two radiologists blinded to histopathology in consensus evaluated the following biomarkers on MRE images matched to resected pathological specimens: T1 ratio, T2 ratio, enhancement pattern, mural thickness, pre-stenotic luminal diameter, and apparent diffusion coefficient (ADC). A blinded pathologist graded stricture histological specimens with acute inflammation score (AIS) and fibrosis score (FS). MRE measurements were correlated with the reference standard. Results: Inflammation and fibrosis coexisted in 78.3% of patients. T2 ratio was reduced in patients with severe fibrosis (p = 0.01). Pre-stenotic bowel dilatation positively correlated with FS (p = 0.002). The ADC value negatively correlated with FS (p < 0.001) and was different between FS grades (p < 0.05). The area under the receiver operating characteristic curve for discriminating between none and mild/moderate-severe bowel wall fibrosis was 0.75 for pre-stenotic bowel dilatation (sensitivity 100%, specificity 44.4%) and 0.97 for ADC (sensitivity 80%, specificity 100%). Conclusions: Inflammation and fibrosis often coexist in CD bowel strictures needing surgery. The combination of parameters derived from conventional MR sequences (T2 ratio, pre-stenotic dilatation) and from DWI (ADC) may provide a contribution to detect and grade bowel fibrosis in adult CD patients.

A Case of Nutcracker Syndrome Combined with Wilkie Syndrome with Unusual Clinical Presentation.

BACKGROUND Nutcracker syndrome and Wilkie's syndrome are rare vascular diseases due to the abnormal course of the superior mesenteric artery originating from the abdominal aorta with reduced angle (<22°) and consequent compression of the left renal vein (nutcracker) and duodenum (Wilkie). Here, we report the case of a patient with a rare combination of these 2 syndromes and with unusual clinical manifestation of post-prandial pain. CASE REPORT We describe the case of a young male patient with rapid weight loss, coupled with post-prandial abdominal pain, with sub-acute onset, not associated with other symptoms. The ultrasound examination found an aorto-mesenteric angle of 18° and compression of the left renal vein and left varicocele. A CT study was performed to exclude oncological diseases and/or other pathologies responsible for the pain and weight loss, which confirmed the ultrasound findings and showed compression of the third part of the duodenum. The patient underwent endovascular treatment, with stent placement in the left renal vein, which resolved the vascular compression and of the duodenum, with regression of symptoms. CONCLUSIONS The ultrasound scan promptly highlighted the reduction of the aorto-mesenteric angle and the signs of venous congestion of the left renal vein. Based on this experience, in patients with weight loss and post-prandial pain, in our opinion, diagnostic investigations should also be extended to the study of the aorto-mesenteric angle to confirm or exclude any vascular and/or duodenal compression.

Endometriosis: clinical features, MR imaging findings and pathologic correlation.

OBJECTIVE: We illustrate the magnetic resonance imaging (MRI) features of endometriosis. BACKGROUND: Endometriosis is a chronic gynaecological condition affecting women of reproductive age and may cause pelvic pain and infertility. It is characterized by the growth of functional ectopic endometrial glands and stroma outside the uterus and includes three different manifestations: ovarian endometriomas, peritoneal implants, deep pelvic endometriosis. The primary locations are in the pelvis; extrapelvic endometriosis may rarely occur. Diagnosis requires a combination of clinical history, invasive and non-invasive techniques. The definitive diagnosis is based on laparoscopy with histological confirmation. Diagnostic imaging is necessary for treatment planning. MRI is as a second-line technique after ultrasound. The MRI appearance of endometriotic lesions is variable and depends on the quantity and age of haemorrhage, the amount of endometrial cells, stroma, smooth muscle proliferation and fibrosis. The purpose of surgery is to achieve complete resection of all endometriotic lesions in the same operation. CONCLUSION: Owing to the possibility to perform a complete assessment of all pelvic compartments at one time, MRI represents the best imaging technique for preoperative staging of endometriosis, in order to choose the more appropriate surgical approach and to plan a multidisciplinary team work. TEACHING POINTS: • Endometriosis includes ovarian endometriomas, peritoneal implants and deep pelvic endometriosis. • MRI is a second-line imaging technique after US. • Deep pelvic endometriosis is associated with chronic pelvic pain and infertility. • Endometriosis is characterized by considerable diagnostic delay. • MRI is the best imaging technique for preoperative staging of endometriosis.

Thyroid adenolipoma: a case report.

Generally, small quantities of adipose tissue is present in the thyroid gland. The adenolipoma of the thyroid gland is considered a rare finding. It consists in a benign, encapsulated neoplasm composed of mature adipose tissue and glandular elements. We report a case of a 71 year-old female patient presenting with swelling of the anterior neck and history of airway obstruction. Ultrasound (US) examination showed a bulky multinodular goiter which caused dislocation and compression of the trachea. The scans performed at the level of the isthmic region showed the presence of a hyperechoic oval formation with a homogeneous echostructure and regular contours; these characteristics suggested the lipomatous nature of the nodule. The patient was subsequently subjected to a Computer Tomography (CT) of the neck for a pre-operative balance of the goitre and to exclude extra-thyroid pathologies. The CT scan confirmed the sonographic findings, and the probable adipose nature of the isthmic formation. After the patient has been subjected to total thyroidectomy and histological examination confirmed the diagnosis of adenolipoma.

Copeman nodule: a case report.

The Copeman nodule is a disease of the subcutaneous soft tissue consisting in subcutaneous adipose tissue herniation through the superficial muscular fascia. A 30-year-old female patient presented with chronic left pain just above the iliac crest. Ultrasound examination showed subcutaneous adipose tissue herniation through the superficial muscular fascia with a hernia gap of 15.9 mm in diameter. Then the patient underwent surgery under local anesthesia with suturing of the hernia gap and immediate regression of chronic pain. Ultrasound can accurately highlight or exclude the presence of Copeman nodule and, in our view, should be used as first examination in patients with superficial chronic pain to the right or left side.

Uveal melanoma: quantitative evaluation of diffusion-weighted MR imaging in the response assessment after proton-beam therapy, long-term follow-up.

PURPOSE: The purpose of this prospective study was to investigate the proton-beam-induced changes in apparent diffusion coefficient (ADC) values of ocular melanoma treated with proton-beam therapy (PBT) in patients undergoing long-term magnetic resonance imaging (MRI) follow-up and to assess whether variations in ADC constitute a reliable biomarker for predicting and detecting the response of ocular melanoma to PBT. METHODS: Seventeen patients with ocular melanoma treated with PBT were enrolled. All patients underwent conventional MRI and diffusion-weighted imaging (DWI) at baseline and 1, 3, 6, and 18 months after the beginning of therapy. Tumor volumes and ADC values of ocular lesions were measured at each examination. Tumor volumes and mean ADC measurements of the five examination series were compared; correlation of ADC values and tumor regression was estimated. RESULTS: Mean ADC values of ocular melanomas significantly increased already 1 month after therapy whereas tumor volume significantly decreased only 6 months after therapy. Pretreatment ADC value of ocular melanomas and early change in ADC value 1 month after therapy significantly correlated with tumor regression. CONCLUSIONS: In ocular melanoma treated with PBT, ADC variations precede volume changes. Both pretreatment ADC and early change in ADC value may predict treatment response, thus expanding the role of DWI from diagnostic to prognostic.

Non-neoplastic diseases of the fallopian tube: MR imaging with emphasis on diffusion-weighted imaging.

OBJECTIVE: We illustrate the magnetic resonance imaging (MRI) features of non-neoplastic tubaric conditions. BACKGROUND: A variety of pathologic non-neoplastic conditions may affect the fallopian tubes. Knowledge of their imaging appearance is important for correct diagnosis. With recent advances in MRI, along with conventional MR sequences, diffusion-weighted imaging (DWI) sequences are available and may improve lesion characterization by discriminating the nature of the content of the dilated tube. Tubal fluid with low signal intensity on T1-weighted images, high signal intensity on T2-weighted images and no restricted diffusion on DWI is indicative of hydrosalpinx. Content with high signal intensity on T1-weighted images and restricted diffusion on DWI is suggestive of hematosalpinx associated with endometriosis or tubal pregnancy. A dilated tube with variable or heterogeneous signal intensity content on conventional MR sequences and restricted diffusion on DWI may suggest a pyosalpinx or tubo-ovarian abscess. We describe morphological characteristics, MR signal intensity features, enhancement behaviour and possible differential diagnosis of each lesion. CONCLUSION: MRI is the method of choice to study adnexal pelvic masses. Qualitative and quantitative functional imaging with DWI can be of help in characterization of tubaric diseases, provided that findings are interpreted in conjunction with those obtained with conventional MRI sequences. TEACHING POINTS: • Nondilated fallopian tubes are not usually seen on MR images. • MRI is the method of choice to characterize and localize utero-adnexal masses. • MRI allows characterization of lesions through evaluation of the fluid content's signal intensity. • DWI in conjunction with conventional MRI sequences may improve tissue characterization. • Pelvic inflammatory disease is the most common tubal pathology.

MR imaging of ovarian masses: classification and differential diagnosis.

OBJECTIVE: We propose a Magnetic Resonance Imaging (MRI) guided approach to differential diagnosis of ovarian tumours based on morphological appearance. BACKGROUND: Characterization of ovarian lesions is of great importance in order to plan adequate therapeutic procedures, and may influence patient's management. Optimal assessment of adnexal masses requires a multidisciplinary approach, based on physical examination, laboratory tests and imaging techniques. Primary ovarian tumours can be classified into three main categories according to tumour origin: epithelial, germ cell and sex cord-stromal tumours. Ovarian neoplasms may be benign, borderline or malignant. Using an imaging-guided approach based on morphological appearance, we classified adnexal masses into four main groups: unilocular cyst, multilocular cyst, cystic and solid, predominantly solid. We describe MR signal intensity features and enhancement behaviour of ovarian lesions using pathologically proven examples from our institution. CONCLUSION: MRI is an essential problem-solving tool to determine the site of origin of a pelvic mass, to characterize an adnexal mass, and to detect local invasion. The main advantages of MRI are the high contrast resolution and lack of ionizing radiation exposure. Although different pathological conditions may show similar radiologic manifestations, radiologists should be aware of MRI features of ovarian lesions that may orientate differential diagnosis. TEACHING POINTS: • Diagnostic imaging plays a crucial role in detection, characterization and staging of adnexal masses. • Characterization of an ovarian lesion may influence patient's management. • Different pathological conditions may have similar radiologic manifestations. • Non-neoplastic lesions should always be taken into consideration.

Crohn's disease of the small bowel: evaluation of ileal inflammation by diffusion-weighted MR imaging and correlation with the Harvey-Bradshaw index.

PURPOSE: This study was undertaken to determine the diagnostic capabilities of diffusion-weighted magnetic resonance imaging (DWI) in detecting ileal inflammation in Crohn's disease (CD), and to verify the correlation between the DWI sequences and the Harvey-Bradshaw index (HBI). MATERIALS AND METHODS: Twenty patients with an endoscopic-histological diagnosis of CD of the terminal ileum and MR enterography with DWI sequences and HBI were retrospectively selected. Disease activity was visually evaluated on the DWI sequences. In quantitative analysis, the apparent diffusion coefficient (ADC) of the terminal ileum was compared with that of normal ileal loops. Pearson's r was used to verify the correlation between the DWI findings and the HBI. RESULTS: On visual assessment, the accuracy, sensitivity and positive predictive value of DWI for the detection of inflammation were 100%. In the quantitative assessment, the ADC value of the disease-active terminal ileum was significantly lower (p < 0.00001) than that of normal ileal loops. A correlation was found between visual assessment of the terminal ileum with the DWI sequences and HBI; no correlation was found between ADC of the terminal ileum and HBI. CONCLUSION: DWI sequences may be useful in differentiating actively inflamed small bowel segments from normal small bowel in CD. Though partial, the correlation between DWI sequences and HBI confirms the utility of this technique in the study of patients with CD.

Diffusion-weighted magnetic resonance imaging for predicting and detecting the response of ocular melanoma to proton beam therapy: initial results.

PURPOSE: The aim of this study was to investigate the utility of diffusion-weighted magnetic resonance (MR) imaging for prediction and early detection of response to proton beam therapy in ocular melanoma. MATERIALS AND METHODS: Ten ocular melanoma patients treated with proton beam therapy were enrolled in the study. All patients underwent conventional MR imaging and diffusion-weighted imaging (DWI) before the start of therapy, and after 1, 3 and 6 months of therapy. Tumour volumes and apparent diffusion coefficient (ADC) values of ocular lesions were measured at each examination. Tumour volumes and mean ADC measurements of the four examination series were compared; correlation of ADC values and tumour regression was investigated. RESULTS: Mean ADC value of ocular melanomas significantly increased as early as 3 months after therapy; tumour volume significantly decreased as early as 6 months after therapy. The ADC values of ocular melanomas before therapy significantly correlated with tumour regression. CONCLUSIONS: DWI may provide an early surrogate biomarker for prediction and early detection of tumour response to eye-preserving therapies in ocular melanoma.