PET/MRI: Multiparametric imaging of brain tumors.

A combination of morphological imaging of the brain with microstructural and functional imaging provides a comprehensive overview of the properties of individual tissues. While diffusion weighted imaging provides information about tissue cellularity, spectroscopic imaging allows us to evaluate the integrity of neurons and possible anaerobic glycolysis during tumor hypoxia, in addition to the presence of accelerated synthesis or degradation of cellular membranes; on the other hand, PET metabolic imaging is used to evaluate major metabolic pathways, determining the overall extent of the tumor (18F-FET, 18F-FDOPA, 18F-FCH) or the degree of differentiation (18F-FDG, 18F-FLT, 18F-FDOPA and 18F-FET). Multi-parameter analysis of tissue characteristics and determination of the phenotype of the tumor tissue is a natural advantage of PET/MRI scanning. The disadvantages are higher cost and limited availability in all centers with neuro-oncology surgery. PET/MRI scanning of brain tumors is one of the most promising indications since the earliest experiments with integrated PET/MRI imaging systems, and along with hybrid imaging of neurodegenerative diseases, represent a new direction in the development of neuroradiology on the path towards comprehensive imaging at the molecular level.

Bilateral Gigantomastia, Multiple Synchronous Nodular Pseudoangiomatous Stromal Hyperplasia Involving Breast and Bilateral Axillary Accessory Breast Tissue, and Perianal Mammary-Type Hamartoma of Anogenital Mammary-Like Glands: A Case Report.

Mammary-type fibroepithelial lesions involving ectopic breast and anogenital region are rare and usually coexist with normal orthotopic breast. We present what we believe to be a unique case of synchronous bilateral gestational gigantomastia resembling fibrous mastopathy, synchronous rapidly growing pregnancy-associated nodular pseudoangiomatous stromal hyperplasia involving right breast and bilateral axillary ectopic breast tissue, and metachronous perianal mammary-type hamartoma involving anogenital mammary-like glands occurring in a 34-year-old patient with facioscapulohumeral muscular dystrophy. Also, we review the literature concerning these lesions.

The role of 18F-FDG accumulation and arterial enhancement as biomarkers in the assessment of typing, grading and staging of hepatocellular carcinoma using 18F-FDG-PET/CT with integrated dual-phase CT angiography.

AIM: The purpose of the present study was to evaluate the possibility of detection, staging and differentiation assessment of hepatocellular carcinoma (HCC) using a combination of dual-phase computed tomography (CT)-angiography in the arterial and portal phase with positron emission tomography (PET) imaging using (18)F-fluorodeoxyglucose ((18)FDG). PATIENTS AND METHODS: From a set of 10,000 patients who underwent (18)FDG-PET/CT, we examined a total of 65 patients (52 males, 13 females; mean age=61.7 years, ranging from 35-82 years) with HCC. The imaging included CT data acquisition after intravenous application of iodinated contrast material in arterial and portal phases, allowed to obtain data in CT angiography quality. Histological diagnosis of the resection sample (21), biopsy (37) or necropsy (7), including the evaluation of the hepatocytary origin of the tumor and the grade of its differentiation, was determined in all patients. RESULTS: The most sensitive sign in the detection of HCC was the alternative presence of hypervascularity or hyperaccumulation of (18)F FDG that reached 93.8%. The high level of (18)F-FDG accumulation showed sensitivity of 84.1% and specificity of 75.0% for distinguishing between well- and poorly differentiated HCC. CONCLUSION: The combination of the dual-phase CT angiography with (18)FDG PET helps in the assessment of staging and differentiation of HCC and has an important role in treatment decision-making.

18F-FDG-PET/CT in potentially advanced renal cell carcinoma: a role in treatment decisions and prognosis estimation.

AIM: to assess the influence of positron emission tomography/computed tomography with (18)F-fluorodeoxyglucose ((18)F-FDG-PET/CT) on the treatment decision in renal cell carcinoma and to assess the prognostic value of the (18)F-FDG accumulation assessments. PATIENTS AND METHODS: Data from 60 patients were included. The cohort consisted of 43 males, 17 females, mean age 66.2 years (range=49-86 years). All patients underwent (18)F-FDG-PET/CT including two-phase CT-angiography of the kidneys. Locally advanced or generalized renal cell carcinoma was suspected in all patients. The level of the (18)F-FDG accumulation within the tumor was compared with the histological grading and the development of the disease was assessed 12 months after (18)F-FDG-PET/CT. RESULTS: Overall mortality reached 46.7%, the highest (18)F-FDG accumulation showed tumor of grade 4 (mean SUV(max)=10.7, range=5-23), the highest mortality was found for tumors exceeding SUV(max) value of 10 (mortality 62.5%). New information was brought by (18)F-FDG-PET/CT in 85% of cases. CONCLUSION: (18)F-FDG-PET/CT has the potential to estimate the patient's survival according to the (18)F-FDG accumulation measured in SUV(max). Depiction of occult metastatic disease has an emerging role in decision making regarding surgery.

A role of multifactorial evaluation of prostatic 3T MRI in patients with elevated prostatic-specific antigen levels: prospective comparison with ultrasound-guided transrectal biopsy.

AIM: To assess the role of multiparametric 3T magnetic resonance (3TMRI) of the prostate in detection of the prostatic carcinoma in a male population with elevated prostatic-specific antigen (PSA) and to compare the results with those of transrectal biopsies. MATERIALS AND METHODS: A prospectively collected cohort of 191 men underwent 3T MRI before transrectal biopsy. The evaluation consisted of the assessment of T2-weighted images, diffusion-weighted images, MR spectroscopy and the pharmacokinetic evaluation of the data obtained during the dynamic post-contrast T1 imaging. The assessment included the calculation of the blood volume and transfer constant evaluations. The diagnosis of prostate carcinoma was based on a minimum of three positive signs obtained from MR studies--hypointensive T2 lesion, diffusion restriction, elevated choline/creatine peak in spectrum and malignant type of saturation by contrast agents. All biopsies were evaluated by a specialist in uropathology. RESULTS: 164 patients underwent biopsy, in 27 the biopsy was omitted due to a lack or low probability of carcinoma: Overall, 84 carcinomas were found. Based on the comparison of biopsy results, 3T MRI reached a sensitivity of 97.6%, specificity of 85.0%, positive predictive value of 74.6% and negative predictive value of 96,3% respectively. There were only three false negative findings. In three patients with very suspicious MRI findings and PSA levels over 30 ng/ml, the biopsy did not confirm carcinoma, even though it was highly suspected. CONCLUSION: The implementation of 3T MRI in routine assessment of patients with elevated PSA should reduce the number of biopsies performed and improve the number of tumors detected due to better targeted biopsies.

Hybrid peripheral nerve sheath tumors, including a malignant variant in type 1 neurofibromatosis.

The authors report a small case series of hybrid nerve sheath tumors occurring in the setting of type 1 neurofibromatosis. Four lesions were benign and consisted of plexiform neurofibromas with considerable areas of perineuriomatous differentiation in patients with type 1 neurofibromatosis. In these lesions, biphasic (Schwannian and perineuriomatous) differentiation was apparent on immunohistochemistry, with the perineuriomatous areas staining for epithelial membrane antigen, glut-1, and claudin-1 and being negative for S-100 protein. Three patients were members of a single family, with a history of various malignant neoplasms. Included in the series is 1 hybrid lesion in which neurofibromatous and perineuriomatous areas were clearly visible on hematoxylin- and eosin-stained slides. The lesion was unique in that it manifested malignant change in the S-100 protein-positive component, which was classified as malignant peripheral nerve sheath tumor. The malignant component showed areas with an epithelioid cell morphology.

Three-tesla MRI biphasic angiography: a method for preoperative assessment of the vascular supply in renal tumours: a surgical perspective.

PURPOSE: The new generation of 3TMRI has improved spatial and time resolutions, which are favourable in imaging of the renal vasculature. In this study, we have compared the imaging findings of the renal blood vessels using 3TMRI and CT with intraoperative assessment of the renal vasculature as gold standard. METHODS: This prospective study was approved by the local ethical committee. Between 4/2011 and 12/2011, 80 patients with renal tumours underwent 3TMRA (angiography) (Magnetom SKYRA 3T, Siemens). Twenty of the patients were also examined with CT AG. The results of the CTA- and MRA-imaging studies were correlated with the intraoperative assessment of the renal vessels. RESULTS: Seventy patients (87.5 %) had a detailed intraoperative assessment of the renal vessels. The sensitivities for CTA and MRA were 88.2 and 88.6 %, respectively. All discrepancies between imaging studies and intraoperative findings were due to inability to identify small polar vessels. The results of MRA were concordant with CTA in 85.0 % of cases. The (three) discrepancies between MRI and CT were due to failure of MRI in identifying small polar vessels. CONCLUSIONS: (1) 3TMRA gives detailed information about the renal vasculature including its topographical anatomy. (2) With MRI, small aberrant vessels are more frequently missed than with CTA. (3) CTA remains the gold standard. However, MRA may be used for planning of laparoscopic operations. (4) The quality of the 3D reconstruction is highly depending on the skills of the radiologist.

Prognostic value of the number and volume of liver tumours on portal vein embolization outcomes.

BACKGROUND/AIMS: Portal vein embolization (PVE) extends the resecability of liver tumours.The issue of PVE is an insufficient growth of the liver parenchyma or a tumour progression in some patients. We evaluated the effect of the volume and the number of liver tumours on the effect of PVE. METHODOLOGY: PVE was performed in 40 patients with liver tumours due to an insufficient future remnant liver volume. The number and the volume of the tumours were evaluated and compared with the final PVE effect. RESULTS: In patients without any increase of the liver volume after PVE (n=3) the number and the volume of the tumours before PVE were 2.7±2.1 and 2205.1±2432.7mm3, respectively. In patients with sufficient growth of the liver (n=22) it was 3.8±2.2 (NS) and 1164.9±1392.1mm3 (NS), respectively. In patients with tumour progression (n=11) it was 5.6±2.2 and 6971.4±5189.5mm3, respectively (p<0.04 and p<0.005, respectively). Four patients were treated by radiofrequency ablation only due to worsening of their health state. Patients with >4 foci (OR 4.7) and a tumour volume >400mm3 (OR=13.0) had a higher probability of cancer progression or insufficient growth of the liver tissue. Patients with <6 foci and a tumour volume <3100mm3 had an 87.5% probability of a successful liver hypertrophy after PVE. CONCLUSIONS: The tumour number and volume were crucial for progression of a malignant disease and growth of the liver parenchyma after PVE.

Predictive value of serum biomarkers in patients after portal vein embolization (PVE): a pilot study.

BACKGROUND/AIM: Insufficient growth of the liver or tumor progression is an important issue of portal vein embolization (PVE) in some patients. This study evaluated the predictive value of serum biomarkers for liver hypertrophy and tumor progression after PVE. PATIENTS AND METHODS: Serum levels of tumor markers, growth factors and cytokines were determined in 40 patients with malignant liver tumors in the pre- and post-PVE period. The values were compared with contralateral liver hypertrophy and tumor progression. RESULTS: Liver tissue hypertrophy occurred in 26 (65%), tumor progression in 11 (27.5%) and insufficient liver hypertrophy in 3 (7.5%) of the patients. The significant predictive biomarkers of PVE included serum TPA levels, monototal, IGF-BP3, IGF1, TGF-α, EGF, HGF, VEGF, TNFa and IL-10 before PVE; and TK, TPA, monototal, IGF-BP3, TGFa and IL-8 over the course of 28 days after PVE. CONCLUSION: Certain serum biomarkers have an important predictive value for the result of PVE.

Pulmonary imaging using dual-energy CT, a role of the assessment of iodine and air distribution.

AIM: The aim of the study is to present the feasibility of using dual-energy CT and the evaluation of iodine and air distribution in differentiation of pathological conditions. MATERIAL AND METHOD: We used the data of 50 CT examinations performed due to suspected pulmonary embolism with any pathological finding except consolidation of the parenchyma. The patients underwent CT angiography of the pulmonary arteries on a dual-source CT (DSCT), with the two tubes independently operated at 140 and 80 kV. By exploiting the dual-energy information, iodine distribution maps were obtained in addition to the conventional CT images which served as a marker of pulmonary perfusion. Minimum intensity projections (MinIP) were used as a marker of air content. RESULTS: By comparing the iodine distribution maps and MinIP images, it was possible to differentiate between the following templates of lung parenchyma: A--normal iodine and air distribution; B--iodine content deficit with minimal or with no redistribution of air; C--reduced iodine content and increased content of air; D--deficit of iodine content and increased content of air; E--increased iodine content and normal content of air; F--increased iodine content and reduced content of air; G--reduced perfusion and reduced content of air. The type A (five cases) was typical for the pulmonary embolism with preserved normal conditions of perfusion and ventilation. Type B (18 cases) occurred in pulmonary embolism; type C was found in case of inflammation of small respiratory airways (five cases); emphysema was typical for type D (nine cases); increased perfusion was observed in the parenchyma preserved from emphysema or preserved from embolism in cases of emphysema or pulmonary embolism; type F occurred in pulmonary interstitial edema (four cases) both with pulmonary infection; finally type G was found in interstitial lung diseases (five cases). CONCLUSION: Imaging of the pulmonary circulation by means of dual-energy CT opens the potential to study pathological changes of circulatory and pulmonary perfusion impairments, our presented work signs the important relations between iodine and air distribution which have to be thought in the interpretation of dual-energy perfusion imaging of the lungs.

Tubulocystic renal carcinoma: a clinical perspective.

INTRODUCTION: Tubulocystic renal carcinoma (TCRC) is a recently described neoplastic entity. To date, clinicopathological features on less than hundred cases of these rare tumours have been characterized exclusively in the pathological literature. Herein, we present five additional cases emphasizing clinical aspects on these rare renal neoplasms. MATERIAL AND METHOD: Cases diagnosed as TCRC were retrieved and reviewed from the routine and consultation files of the Pilsen tumour registry comprising over 20,000 cases of renal tumours. RESULTS: All patients were men, mean age 56 years (range 29-70). Features on computed tomography (CT) were in two cases Bosniak III, one IV and two were solid tumours. In four patients, nephrectomy was performed, and one patient underwent resection. At the time of surgery, two patients had metastases. In one case, both primary tumour and metastases were active on FDG positron emission tomography (PET)/CT. Both patients with metastatic disease were treated with sunitinib with partial response. One patient died 26 months postoperatively and the other patient is alive 5 months after surgery. Three patients with localized tumours are without evidence of disease 31, 28 and 7 months after surgery. In one case, the resected tumour was histologically combined with a papillary renal cell carcinoma (PRCC). CONCLUSION: TCRC occurs predominantly in men with a wide age range. TCRC frequently displays a cystic component which may render a radiological classification of Bosniak III or IV. FDG PET/CT is helpful in the detection of metastases. TCRC has definitive malignant potential. Our findings support a possible relationship to PRCC. The tyrosine kinase inhibitor sunitinib may be used a therapeutical agent with partial response and temporary effect.

Prostatic-type tissue in the lower female genital tract: a morphologic spectrum, including vaginal tubulosquamous polyp, adenomyomatous hyperplasia of paraurethral Skene glands (female prostate), and ectopic lesion in the vulva.

The investigators report a series of prostatic-type lesions occurring in the lower female genital tract. The cases included a 4.5-cm mass representing hyperplasia of the glandular and stromal tissue of paraurethral Skene gland, a small ectopic prostatic lesion in the vulva, and 4 tubulosquamous vaginal polyps. All lesions were immunopositive for prostate-specific antigen and/or prostatic acid phosphatase. A brief discussion of the earlier published material is included.

Fever of unknown origin: a value of (18)F-FDG-PET/CT with integrated full diagnostic isotropic CT imaging.

AIM: The aim of presented work is to evaluate the clinical value of (18)F-FDG-PET/CT in patients with fever of unknown origin (FUO) and to compare PET/CT finding with the results of the following investigation. MATERIAL AND METHOD: 48 patients (24 men, 24 women, mean age 57.6 years with range 15-89 years) underwent (18)F-FDG-PET/CT due to the fever of unknown origin. All examinations were performed using complex PET/CT protocol combined PET and whole diagnostic contrast enhanced CT with sub-millimeter spatial resolution (except patient with history of iodine hypersensitivity or sever renal impairment). CT data contained diagnostic images reconstructed with soft tissue and high-resolution algorithm. PET/CT finding were compared with results of biopsies, immunology, microbiology or autopsy. RESULTS: The cause of FUO was explained according to the PET/CT findings and followed investigations in 44 of 48 cases-18 cases of microbial infections, nine cases of autoimmune inflammations, four cases of non-infectious granulomatous diseases, eight cases of malignancies and five cases of proved immunity disorders were found. In 46 cases, the PET/CT interpretation was correct. Only in one case, the cause was overlooked and the uptake in atherosclerotic changes of arteries was misinterpreted as vasculitis in the other. The reached sensitivity was 97% (43/44), and specificity 75% (3/4) respectively. CONCLUSION: In patients with fever of unknown origin, (18)F-FDG-PET/CT might enable the detection of its cause.

Aortic annulus and ascending aorta: comparison of preoperative and periooperative measurement in patients with aortic stenosis.

BACKGROUND: Precise determination of the aortic annulus size constitutes an integral part of the preoperative evaluation prior to aortic valve replacement. It enables the estimation of the size of prosthesis to be implanted. Knowledge of the size of the ascending aorta is required in the preoperative analysis and monitoring of its dilation enables the precise timing of the operation. Our goal was to compare the precision of measurement of the aortic annulus and ascending aorta using magnetic resonance (MR), multidetector-row computed tomography (MDCT), transthoracic echocardiography (TTE), and transoesophageal echocardiography (TEE) in patients with degenerative aortic stenosis. METHODS AND RESULTS: A total of 15 patients scheduled to have aortic valve replacement were enrolled into this prospective study. TTE was performed in all patients and was supplemented with TEE, CT and MR in the majority of patients. The values obtained were compared with perioperative measurements. For the measurement of aortic annulus, MR was found to be the most precise technique, followed by MDCT, TTE, and TEE. For the measurement of ascending aorta, MR again was found to be the most precise technique, followed by MDCT, TEE, and TTE. CONCLUSION: In our study, magnetic resonance was found to be the most precise technique for the measurement of aortic annulus and ascending aorta in patients with severe degenerative aortic stenosis.

Diffusion tensor magnetic resonance imaging of glial brain tumors.

AIM: To evaluate the author's experience with the use of diffusion tensor magnetic resonance imaging (DTI) on patients with glial tumors. METHODS: A retrospective evaluation of a group of 24 patients with glial tumors was performed. There were eight patients with Grade II, eight patients with Grade III and eight patients with Grade IV tumors with a histologically proven diagnosis. All the patients underwent routine imaging including T2 weighted images, multidirectional diffusion weighted imaging (measured in 60 non-collinear directions) and T1 weighted non-enhanced and contrast enhanced images. The imaging sequence and evaluation software were produced by Massachusetts General Hospital Corporation (Boston, MA, USA). Fractional anisotropy (FA) maps were calculated in all patients. The white matter FA changes were assessed within the tumorous tissue, on the tumorous borderline and in the normally appearing white matter adjacent to the tumor. A three-dimensional model of the white matter tract was created to demonstrate the space relationship of the tumor and the capsula interna or corpus callosum in each case using the following fiber tracing parameters: FA step 0.25 and a tensor declination angle of 45 gr. An additional assessment of the tumorous tissue enhancement was performed. RESULTS: A uniform homogenous structure with sharp demargination of the Grade II tumors and the wide rim of the intermedial FA in all Grade III tumors respectively, were found during the evaluation of the FA maps. In Grade IV tumors a variable demargination was noted on the FA maps. The sensitivity and specificity for the discrimination of low- and high-grade glial tumors using FA maps was revealed to be 81% and 87% respectively. If the evaluation of the contrast enhancement was combined with the evaluation of the FA maps, both sensitivity and specificity were 100%. CONCLUSION: Although the evaluation of the fractional anisotropy maps is not sufficient for glioma grading, the combination of the contrast enhancement pattern and fractional anisotropy maps evaluation improves the possibility of distinguishing low- and high-grade glial tumors. Three-dimensional models of the white matter fibers in the corpus callosum and the internal capsule may be used in the presurgical planning.

(18)F-FDG-PET/CT of orofacial tumors, a value of whole-body imaging approach.

AIM: Staging of head and neck tumors is one of the most difficult tasks in imaging techniques, due to the very complicated head and neck anatomy and serious problems with the differentiation of reactive enlarged lymph nodes and lymph nodes involved with metastases. The aim of the study was to evaluate the validity of the whole-body approach in the assessment of head and neck malignancies using (18)F-FDG-PET/CT. MATERIALS AND METHODS: The analysis of a group of 1750 consecutive whole-body procedures in all indications of (18)F-FDG-PET/CT was made according to: the presence of orofacial tumors; their histology; findings concerning the spread outside head and neck region; and findings concerning the primary staging or restaging. The examinations of head and neck tumors were performed after intravenous application of the (18)F-FDG and its accumulation for one hour. Drinking and speaking is restricted during this accumulation to prevent artificial muscle (18)F-FDG uptake and to minimize false positive findings. In our hospital, high resolution PET is followed by the sub-millimeter isotropic acquisition of CT data after intravenous application of an iodinated contrast material. The acquisitions of head and neck region and trunk are performed separately to obtain optimal resolution in both regions. RESULTS: 105 examinations of the orofacial tumors were performed on 87 patients in a group of 1750 consecutive PET/CT examinations. The ratio between primary staging and restaging was 3:7. The most frequent indications were carcinomas of the tongue (19 examinations) and carcinomas of the salivary glands (19 examinations). The metastatic spread of the tumor outside the region of the head and neck was noted in 12 cases. CONCLUSION: Our findings of distant metastases confirmed the importance of the use of whole-body PET/CT in this indication.

TSC2/PKD1 contiguous gene syndrome: a report of 2 cases with emphasis on dermatopathologic findings.

The association of tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD), termed TSC2/PKD1 contiguous gene syndrome, is a result of molecular defect demonstrating by deletion disrupting TSC2 and PKD1. Dermatopathology of this syndrome has never been addressed. We report 2 sporadic cases form of TSC2/PKD1 contiguous gene syndrome, with emphasis on dermatopathologic findings. Both patients presented with a typical phenotype of TSC and early-onset renal polycystic requiring kidney transplantation in one of the patients. Of a total of 13 cutaneous lesions studied, there were 7 facial angiofibromas, 2 shagreen patches, 1 periungual fibroma, 1 hypopigmented macule, 1 epidermoid cyst, and 1 intradermal melanocytic nevus. The histological features were basically similar to those occurring in TSC, but some unusual features were identified. In both patients, deletions in the region of TSC2 and PKD1 were revealed performing by multiplex ligation probe amplification test. It is concluded that the histopathological features of skin lesions in this syndrome are similar to those encountered in TSC. Clinical awareness and appropriate molecular investigation of TSC2/PKD1 contiguous gene syndrome is necessary in all patients with a typical phenotype of TSC in infancy, adolescence, or adult age, because of severity of the renal alterations.

The assessment of intracranial bleeding with virtual unenhanced imaging by means of dual-energy CT angiography.

The purpose of this study was to assess virtual unenhanced brain computed tomography (CT) images obtained by dual-energy CT angiography (CTA) for the detection of intracranial bleeding. In total, 25 patients were included in the study (average age 53.2 years, range 25-75 years, 14 male, 11 female), all with intracranial bleeding on unenhanced brain CT and who underwent additional CTA performed on a dual-source CT in a dual-energy acquisition mode. The two X-ray tubes were operated at 140 and 80 kV, respectively. Data were analyzed using dual-energy evaluation software. Virtual unenhanced images were calculated by removing the relative iodine content from each voxel. The virtual unenhanced images were evaluated by a radiologist blinded to the findings of the conventional images related to the presence of intracranial bleeding. The image quality and contrast-to-noise ratio (CNR) between bleeding and brain tissue were assessed. The virtual image quality was found to be sufficient in 96%. The agreement in detection of intracranial bleeding on virtual and conventional unenhanced images reached 96% in per-lesion analysis and 100% in per-patient analysis. The averaged CNR reached 2.63 in virtual unenhanced images and 3.27 in conventional. Virtual unenhanced images are sufficient for the detection of intracranial bleeding.

Crystal-deficient alveolar soft-part sarcoma with cutaneous involvement: a case report.

Alveolar soft-part sarcoma (ASPS) is a rare distinctive sarcoma, in most cases involving deep soft tissues of the extremities. It is associated with a specific unbalanced translocation, der(17)t(X;17)(p11;q25) that results in the formation of an ASPL-TFE3 fusion gene. Microscopically, it is typified by an alveolar growth of large cells containing typical periodic acid-Schiff-positive rod-shaped crystals, often serving as a diagnostic clue. Other distinctive features include nuclear immunoreactivity for transcription factor 3 (TFE3) protein and a typical ultrastructural finding of large crystals with a rectangular or rhomboid shape. The authors present an unusual case of ASPS with cutaneous involvement, which did not exhibit typical large crystals; there were striking round granules. Molecular genetic study revealed fusion transcript ASPL-TFE3, type 2. To the best of our knowledge, cutaneous involvement of a crystal-deficient ASPS has not been reported.

Acral calcified vascular leiomyoma of the skin: a rare clinicopathological variant of cutaneous vascular leiomyomas: report of 3 cases.

BACKGROUND: Several histopathologic variants of cutaneous leiomyomas have been recognized. In our consultation dermatopathology practice, we encountered a variant of cutaneous leiomyoma which, to our knowledge, has not been reported. OBJECTIVE: We report 3 cases of vascular leiomyomas, all of them manifesting prominent intratumoral calcifications dominating over the residuum of the tumors and occurring on the acral sites. METHODS: We conducted a clinicopathological and immunohistochemical study, which is complemented by a literature review. RESULTS: All 3 patients were women ranging in age from 57 to 72 years. Each presented clinically with a slowly growing, firm mass. The lesion was painful in two cases. None of the patients had renal disease or endocrine abnormalities. Microscopically, the lesions were a well-circumscribed, non-encapsulated neoplasm composed of mature smooth muscle cells and vascular pattern, which was inconspicuous, but focally dilated blood vessels were present. In all cases, prominent calcifications were present. Immunohistochemically the spindle cells were positive for alpha-smooth muscle actin. LIMITATIONS: This study utilizes tissue specimens that all came as consultations; therefore, some inherent selection bias exists. CONCLUSION: Acral calcified vascular leiomyoma is a rare clinicopathological variant of leiomyoma which has predilection for acral sites and shows massive calcifications prevailing over the tumor itself.