A new variant of Todani type I choledochal cyst. Imaging evaluation.

BACKGROUND AND AIM: Choledochal cysts are congenital segmental aneurysmal dilatations of any portion of bile ducts, most commonly of the main portion of the common bile duct. The classification system of choledochal cysts is based on the site of the cyst or dilatation and currently includes 5 major types. The purpose of our study is to describe the imaging findings of a new variant of choledochal cyst. MATERIALS AND METHODS: Six patients (4 children and 2 adults, 4 females and 2 males, 3-67 year old) were evaluated by US, CT, CT-cholangiography, MRI, magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP), because of jaundice, abdominal pain and a palpable mass. RESULTS: A new type of choledochal cyst was demonstrated in all cases. In this new variant apart from the dilatation of the common hepatic and the common bile duct, dilatation of the central portion of the cystic duct was also observed, giving a bicornal configuration to the cyst. CONCLUSION: The imaging findings of a new variant of choledochal cyst with participation of the cystic duct are described. We propose the classification of this type of choledochal cyst as a new subtype of Todani I cyst, namely Todani ID.

Orbital cavernous hemangiomas: ultrasound and magnetic resonance imaging evaluation.

Cavernous hemangioma is the most common intraorbital lesion in adults. The aim of our study was to evaluate the magnetic resonance imaging (MRI) and ultrasound (US) characteristics of cavernous hemangioma and their role in the differential diagnosis of orbital tumors. Eight patients with orbital cavernous hemangiomas, five women and three men with a mean age of 48 years were examined in a period of six years. All patients underwent MRI examination and four patients were also evaluated by US. In all cases MRI depicted a well-defined intraconal tumor. The lesions were homogeneous, isointense to muscle on T1-weighted sequence and hyperintense to muscle on T2-weighted sequence in six patients. In one patient the mass was isointense on T1WI with heterogeneous signal intensity on T2WI and in one patient the lesion had heterogeneous signal intensity on both T1- and T2-weighted sequences. After intravenous contrast medium administration, the tumors showed initial inhomogeneous enhancement with progressive accumulation of contrast material on delayed images in seven patients and initial homogeneous enhancement in one patient. On ultrasonography, the orbital masses appeared slightly hyperechoic, heterogeneous with small areas of slow blood flow. The analysis of imaging characteristics of a well-defined intraconal lesion in an adult patient with painless progressive proptosis can be highly suggestive of the diagnosis of cavernous hemangioma.

Magnetic resonance imaging and ultrasonographic evaluation of retinal detachment in orbital uveal melanomas.

We assess the role of ultrasonography (US) in the evaluation of retinal detachment in patients with uveal melanomas, in correlation with magnetic resonance imaging (MRI) findings. Twenty-six patients (13♂ and 13♀) with uveal melanomas underwent MRI and US examination. The signal intensity of the tumors and retinal detachments were depicted using MRI. The position of retinal detachment in correlation with the position of intraocular tumor was also evaluated performing ultrasound examination. MRI and US identified uveal melanoma in all 26 patients. In 11 of the 26 patients (42.3%) uveal melanoma was accompanied by retinal detachment. MRI demonstrated retinal detachment in nine patients. US disclosed retinal detachment in two more cases. US has an important role in the depiction of uveal melanomas and associated retinal detachment. It also appears to be more sensitive than MRI in detecting retinal detachment in patients with orbital uveal melanomas.

Evaluation of popliteal arteries with CT angiography in popliteal artery entrapment syndrome.

BACKGROUND: Popliteal artery entrapment is an uncommon clinical entity that occurs due to compression of the popliteal artery by adjacent muscle and tendinous structures. Early diagnosis should be established through a combined approach of careful physical examination and history-taking, duplex ultrasonography, and CT angiography. PATIENTS AND METHODS: We have studied retrospectively 16 patients of popliteal artery entrapment syndrome, 9 men and 7 women. All patients were scanned with a scanner Picker PQ 5000 after bolus injection of nonionic contrast medium and they all underwent a two-part examination first, with the knee in a neutral position, and, second, with the knee hyperextended. RESULTS: At the second phase of the examination 3 patients showed normal findings, 10 patients have shown mild stenosis of the popliteal artery or more severe stenosis due to compression, 2 patients have exhibited bilateral stenosis and 1 patient has also showed popliteal venous compression. CONCLUSION: CT angiography images and three-dimensional images are useful not only for depiction of the arterial changes but also identification of the abnormal anatomic structures responsible for the entrapment.

Craniosynostosis : correlation with cranial vault shape and osseous defects.

This study assessed the value of three-dimensional CT (3D CT) in the diagnosis of craniosynostosis, and correlated the cranial deformity with the presence of osseous defects in cranial vault's bones. One hundred and two children (55♀ and 47♂) with a clinical suspicion of craniosynostosis, underwent spiral computed tomography with 3D reconstruction using the shaded surface display (SSD) and volume rendering (VR) algorithms. We evaluated the presence of osseous defects in cranial bones in correlation with the type of craniosynostosis and the shape of the cranial vault. 3D CT allowed the evaluation of craniosynostosis in all patients. All patients had combined forms of craniosynostosis. Osseous defects in the bones of cranial vault were found in 56 patients of whom nine had scaphocephaly, eight plagiocephaly and one trigonocephaly. CT of the skull with three-dimensional reconstruction can safely and reliably identify craniosynostoses in children and could be used for the identification of osseous defects in the cranial vault.

Is preservation of the adrenal vein mandatory in laparoscopic adrenal-sparing surgery?

BACKGROUND AND OBJECTIVE: Adrenal tissue-sparing or partial adrenalectomy evolved initially for patients with bilateral synchronous adrenal surgical pathology to preserve vital adrenal volume. In the laparoscopic era, the exact criteria for performing such procedures laparoscopically have yet to be defined. Controversy exists regarding the importance of preserving the adrenal vein, main or accessory. The aim of this retrospective study was to present our short series of laparoscopic tissue-sparing adrenalectomies with vein preservation. Our main goal is not to support partial adrenalectomy as an alternative to total (this is already advocated by many surgeons) but to emphasize the vein-preserving technique. METHODS: Seven patients with peripherally located either aldosterone-producing adenomas (4 cases) or myelolipomas (4 cases) underwent laparoscopic lateral partial adrenalectomy. One patient harbored an aldosterone-producing adenoma and a myelolipoma as well. The main adrenal vein was identified and preserved in 6 patients and the accessory vein in one. RESULTS: No conversion to open adrenalectomy was necessary, and no perioperative morbidity or mortality occurred. Three adenoma patients are normotensive 44, 23, and 20 months postoperatively, while the fourth one's pressure is refractory. CONCLUSIONS: Surprisingly, total adrenalectomies preceded the partial ones, which is controversial compared with other procedures. Laparoscopic lateral partial adrenalectomy is a technically challenging tissue-sparing operation. Meticulous dissection allows preservation of the middle artery and main or accessory vein resulting in a functioning adrenal stump.

Feasibility of laparoscopic adrenalectomy for large pheochromocytomas.

BACKGROUND AND OBJECTIVES: The majority of surgeons consider large and potentially malignant pheochromocytomas an absolute contraindication for laparoscopic adrenalectomy (LA). The aim of this study was to evaluate the risks and outcomes of LA in patients with this anomaly. METHODS: Five patients (2 males, 3 females) with large (>6 cm) pheochromocytomas were selected. Preoperative investigation demonstrated no evidence of invasive carcinoma. All patients received alpha-blocker preparation for at least 20 days. Laparoscopic adrenalectomy via a lateral transperitoneal approach was performed in all cases. RESULTS: Patient's median age was 48 years, and the median tumor size was 10.8 cm. No capsular disruption and no hypertensive crises occurred during the operation. The median operating time was 148 minutes and blood loss was <150 mL. Conversion to open adrenalectomy occurred in 2 patients owing to intraoperative evidence of carcinoma. No postoperative morbidity or mortality occurred. All patients are disease free after a median follow-up of 13 months. CONCLUSIONS: In experienced hands, LA can be proposed for large and potentially malignant pheochromocytomas. Conversion to open adrenalectomy is mandatory if local invasion, capsular disruption, or technical difficulties are observed during the operation.

Topoisomerase I protein expression in primary colorectal cancer and recurrences after 5-FU-based adjuvant chemotherapy.

PURPOSE: Our aim was to investigate whether chemotherapy with 5-FU induces an alteration in the levels of topoisomerase I (topo I) in colorectal neoplastic tissues METHODS: Twenty-five colorectal cancer patients were included in our study; these had undergone surgical resection of the primary tumor, received post-operatively 5-FU-based adjuvant chemotherapy and then suffered from recurrences. In a standard three-step immunohistochemical procedure, a monoclonal antibody to topo I was applied in both specimens from each patient (one from the primary location and a second one from the recurrence). Statistical analysis was subsequently performed. RESULTS: Malignant cells from the recurrences displayed a statistical significant increase, concerning the levels of topoisomerase I, by comparison with the primary tumors (P=0.01). The increase in topo I levels did not demonstrate significant correlations with Duke's stage (Fisher's Exact Test P value=0.496), differentiation grade (P value=0.661), localization (P value=0.072), patient sex (P value=0.434), nor with relapse free interval (P value=0.493). There was a statistically significant relationship between the age of patients and increase in topo I levels (P=0.011). CONCLUSIONS: Topo I expression may be part of the malignant cells' phenotype in recurrent colorectal carcinomas, suggesting a potential role for Topo I in the acquisition of a metastatic phenotype. The increase of topo I immunohistochemical status is likely to be attributed to 5-FU and given the fact that high levels of topo I correlate with sensitivity to camptothecin, advanced colorectal cancer patients seem to benefit from topo I targeted anticancer drug therapy.

Frontoethmoidal Mucoceles: CT and MRI Evaluation.

Paranasal sinus mucocele is an expanded, airless, mucus-filled sinus caused by obstruction of the sinus ostium. It is a benign slow growing epithelial lined lesion, bulging against adjacent anatomical structures, without infiltrating them. The purpose of our study is to describe the CT and MR findings in 19 patients (ten women, nine men, 18-72 years, mean age: 48.1) with surgically confirmed frontoethmoidal mucoceles between 1999-2005. CT scans displayed mucoceles as non enhancing soft tissue density lesions, generally isodense to the brain parenchyma, expanding the sinuses in most cases, eroding adjacent bones and extending intraorbitally or intracranially. Signal intensity in T2WI and T1WI MR images varied, but generally lesions had high signal intensity in T2WI and low to intermediate signal intensity in T1WI. Some of the lesions demonstrated regular linear peripheral enhancement after administration of contrast medium. The causes of mucoceles included mucosal thickening from chronic sinusitis, adhesions from previous operation in the nasal cavity, previous trauma, small nasal polyps and a small osteoma, while in six patients (31.5%) the cause of the mucocele remained unrecognized even after surgery. No underlying malignant tumor was found in any of the cases as the cause of obstruction. CT and MRI established the correct diagnosis in all patients. CT was more sensitive in determining bone erosions, while MRI had the advantage of multiplanar imaging and was much more sensitive for differentiating mucocele from a tumor on the basis of MR signal intensity characteristics. In conclusion, CT and MRI are the methods of choice for diagnosing mucoceles of the paranasal sinuses and are of major importance for the treatment plan. Each method seems to have its own advantages and should be used as complementary investigations of sinonasal pathology. Enhanced CT scan should only be performed in the absence or contraindication for enhanced MR imaging.

Evaluation of the prognostic and predictive value of p53 and Bcl-2 in breast cancer patients participating in a randomized study with dose-dense sequential adjuvant chemotherapy.

PURPOSE: To assess the prognostic and predictive significance of p53 and Bcl-2 protein expression in high risk patients with breast cancer treated with dose-dense sequential chemotherapy. PATIENTS AND METHODS: From June 1997 until November 2000, 595 patients were randomized to three cycles of epirubicin (E) 110 mg/m2 followed by three cycles of paclitaxel (P) 250 mg/m2 followed by three cycles of 'intensified' CMF (cyclophosphamide 840 mg/m2, methotrexate 47 mg/m2 and fluorouracil 840 mg/m2) or to four cycles of E, followed by four cycles of CMF. p53 and Bcl-2 expression was investigated by immunohistochemistry in 392 and 397 patients respectively. RESULTS: Positive expression of p53 was detected in 104 (26.5%) patients and was significantly associated with negative hormonal status, worse histologic grade, higher incidence of disease relapse and higher rate of death. p53 positive expression was a significant negative predictor of overall survival (OS) (P = 0.002) and disease-free survival (DFS) (P = 0.001). Negative expression of Bcl-2 was detected in 203 (51%) patients and was significantly associated with negative hormonal status. Multivariate analysis revealed that, positive p53 expression, higher number of positive nodes and worse tumor grade were related to significantly poorer OS and DFS. CONCLUSIONS: For both treatments, p53 positive expression was a significant negative prognostic factor for OS and DFS while Bcl-2 was not. No predictive ability of p53 status or Bcl-2 status for paclitaxel treatment was evident.

Open surgical biopsy for nonpalpable breast lesions detected on screening mammography.

The aim of this retrospective clinical study was the analysis of histologic findings of nonpalpable breast lesions managed by open surgical biopsy. A series of 630 women underwent 664 preoperative localizations of nonpalpable, mammographically detected breast lesions during the last 10-year period. Indications for biopsy were (1) clustered microcalcifications, (2) solid mass, and (3) radiologic parenchymal distortion. The lesions were localized preoperatively using hook-wire methods, and all biopsies were performed under general anesthesia. Histopathology revealed carcinoma in 172 (25.9%) cases; noninvasive in 114 (66.3%) cases and infiltrating in 58 (33.7%) cases. The highest malignancy rate was found in cases with microcalcifications (112 carcinomas out of 323 cases, 34.7%). Lymph node invasion was present in 25% of patients with invasive cancers. The hook-wire localization of nonpalpable breast lesions is a simple, accurate and safe method for detection of early breast cancers. Frozen section is feasible and accurate in the majority of these lesions, and therefore, diagnostic and therapeutic one-step surgical procedures could be performed.

Fibroadenomas of the breast: is there any association with breast cancer?

PURPOSE: The experience of our Breast Unit in the diagnosis and treatment of fibroadenomas is presented in this retrospective study, focusing specifically in cancer development within these common benign tumors of young age. MATERIAL-METHODS: 310 women with histologically verified breast fibroadenomas who had surgical management in our Unit over the last 14 years were included in the study. RESULTS: Most of them (n: 255) presented with palpable lesions and had triple assessment preoperatively, while the remaining 55 had nonpalpable lesions and underwent needle-wire localization biopsies. Nine cases had cancer development, usually in situ, within the fibroadenomas (2.9%). CONCLUSION: Coexistence of fibroadenomas and breast cancer is relatively rare, but it should not be ignored by breast surgeons, and patients should be properly informed.

Carcinosarcoma of the breast: report of two cases.

Carcinosarcoma is a rare, malignant tumor of the breast, consisting of intraductal or infiltrating carcinoma contiguous or subtly merged with a highly cellular, mitotically active pleomorphic spindle cell stroma (sarcoma). It is a form of the metaplastic mammary carcinomas and it is probably derived of myoepithelial cells. We report two cases of breast carcinosarcoma in two white women. The patients, following a frozen section positive for malignancy, had had a modified radical mastectomy. Paraffin sections and positive immunohistochemical cell staining (in our first case) confirmed the diagnosis of carcinosarcoma. This tumor should be distinguished from other forms of metaplastic carcinomas because it is very aggressive and has an ominous prognosis.

Computer-aided preoperative diagnosis of microcalcifications on mammograms.

PURPOSE: To evaluate of a computer-aided method for differentiating malignant from benign clustered microcalcifications. MATERIAL AND METHODS: Our material was 350 suspicious microcalcifications on mammograms from 330 female patients who underwent breast biopsy (after hook wire localization and under mammographic guidance). The histologic findings were malignant in 140 cases (40%) and benign in 210 cases (60%). Those clusters were manually detected, computer-aided analyzed and quantitatively estimated. Besides computer analysis, 3 physicians-observers (2 radiologists and 1 breast surgeon) evaluated the malignant or benign nature of the clustered microcalcifications. The performance of the artificial network, each observer and the three observers as a group was evaluated by receiver operating characteristics (ROC) curves. RESULTS: Comparison of the ROC curves revealed the following AUC values (area under the curve): computer - 0.950, physician 1 - 0.815, physician 2 - 0.830, physician 3 - 0.830, and physicians as a group - 0.825. The results, compared by the student t-test for paired data, showed a statistically significant difference between computer analysis and physicians' performance, independently and as a group. CONCLUSION: Our study showed that computer analysis achieved statistically significantly better performance than that of physicians in the classification of malignant and benign calcifications.

Use of artificial neural networks (computer analysis) in the diagnosis of microcalcifications on mammography.

INTRODUCTION/OBJECTIVE: The purpose of this study was to evaluate a computer based method for differentiating malignant from benign clustered microcalcifications, comparing it with the performance of three physicians. METHODS AND MATERIAL: Materials for the study are 240 suspicious microcalcifications on mammograms from 220 female patients who underwent breast biopsy, following hook wire localization under mammographic guidance. The histologic findings were malignant in 108 cases (45%) and benign in 132 cases (55%). Those clusters were analyzed by a computer program and eight features of the calcifications (density, number, area, brightness, diameter average, distance average, proximity average, perimeter compacity average) were quantitatively estimated by a specific artificial neural network. Human input was limited to initial identification of the calcifications. Three physicians-observers were also evaluated for the malignant or benign nature of the clustered microcalcifications. RESULTS: The performance of the artificial network was evaluated by receiver operating characteristics (ROC) curves. ROC curves were also generated for the performance of each observer and for the three observers as a group. The ROC curves for the computer and for the physicians were compared and the results are:area under the curve (AUC) value for computer is 0.937, for physician-1 is 0.746, for physician-2 is 0.785, for physician-3 is 0.835 and for physicians as a group is 0.810. The results of the Student's t-test for paired data showed statistically significant difference between the artificial neural network and the physicians' performance, independently and as a group. DISCUSSION AND CONCLUSION: Our study showed that computer analysis achieves statistically significantly better performance than that of physicians in the classification of malignant and benign calcifications. This method, after further evaluation and improvement, may help radiologists and breast surgeons in better predictive estimation of suspicious clustered microcalcifications and reduce the number of biopsies for non-palpable benign lesions.

Breast cancer in ectopic breast tissue.

Ectopic breasts usually develop along the mammary ridges. Their incidence has been reported as up to 5-6%. Development of malignancy is rare. We report three cases of postmenopausal female patients with breast cancer which developed in the axillary accessory breast. They were all successfully treated by surgery and adjuvant therapy. A review of the literature on the incidence and pathology of ectopic mammary tissue is also presented.

Alterations of p16-pRb pathway and chromosome locus 9p21-22 in sporadic invasive breast carcinomas.

The p16-pRb pathway represents a vital cell-cycle checkpoint. In the present study we investigated the alterations of this G1-phase protein pathway using immunohistochemical and molecular methods in a series of 55 breast carcinomas and correlated the findings with clinicopathological features of the patients. Furthermore, we examined its relationship with the status of the chromosomal region 9p21-22 performing a deletion map analysis because there are indications that, in addition to CDKN2 and MTS2/p15(INK4B) tumor suppressor genes (TSGs), this area harbors other TSG(s). Aberrant expression (Ab) of p16 and pRb was observed in 26 (47%) and 16 (29%) of the carcinomas, respectively. A statistical trend pointing out an inverse relationship between p16 and pRb expression was found (p = 0.079). Analysis of the region that encodes for p16 by deletion mapping, a PCR-based methylation assay and PCR-SSCP, revealed that deletions and transcriptional silencing by methylation might represent the main mechanisms of CDKN2/p16(INK4A) inactivation in breast carcinomas. The results of deletion mapping also suggest that another TSG(s) may reside at the 9p21-22 area particularly at the D9S162 loci and that co-deletion of this putative gene with CDKN2/p16(INK4A) may play a role in breast carcinogenesis. In addition, microsatellite instability (MI), a marker of replication error phenotype (RER+), was observed with a frequency of 16% in the area examined and was inversely related with loss of heterozygosity (LOH). Interestingly, most cases with MI at the region encoding for p16 were aggregated in a subgroup of breast carcinomas with no other obvious genetic and/or epigenetic CDKN2/p16(INK4A) alterations. We speculate that there is an additional mechanism of CDKN2/p16(INK4A) inactivation. The relationship of p16 protein level pRb, status, the p16-pRb combined immunoprofiles, and the microsatellite alterations detected at the 9p21-22 locus with the patients' clinicopathological parameters revealed two significant correlations: one between normal pRb expression and lymph node involvement (p = 0.0263), and the other between microsatellite alterations (LOH and or MI) and tumor size (p = 9.2 x 10(-3)). In view of the heterogenous nature of breast cancer, we suggest that in a significant proportion of breast carcinomas, deregulation of the p16-pRb pathway in association with another, as-yet unidentified, TSG(s) of the 9p21-22 region may play a role in initiating or progressing the oncogenic procedure, while in other subgroups, alternative molecules may play this role.

Ductular structures in acute hepatitis with panacinar necrosis.

The development of ductular structures in acute hepatitis with panacinar necrosis was studied in 15 cases of fulminant hepatitis with variable clinical duration, using immunohistochemical markers. The immunophenotype of ductular structures was assessed by the expression of two bile duct epithelium determinants, wide spectrum cytokeratin and epithelial membrane antigen (EMA), and by their glycoconjugate expression using the specific binding lectins Dolichos biflorus agglutinin (DBA) and soybean agglutinin (SBA). Ductular structures showed a predilective, but not a strictly selective location in acinar zone 1 and at the periphery of newly formed parenchymal nodules. All were positive for keratin, while EMA and the lectins were identified less frequently. Cytokeratin expression was additionally observed in hepatic cells with no other phenotypic alteration: this occurred along isolated hepatic cords, within parenchymal remnants, in the spared parenchyma in acinar zone 1 and occasionally at the periphery of parenchymal nodules. The presence of cytokeratin expression in liver cell plates in association with intermediate morphological stages of tubular remodelling speaks in favour of biliary metaplasia of hepatocytes. This process may represent a phenotypic-functional accommodation of hepatocytes to an altered microenvironment, due to loss of parenchymal integrity. During the phenotypic shift, altered cytokeratin expression appears as one of the earliest biliary features, while EMA and the expression of glycoconjugates represent maturation markers.