Computerized tomography texture analysis of pheochromocytoma: relationship with hormonal and histopathological data.

OBJECTIVES: Pheochromocytomas are rare tumors which can present with heterogeneous secretion profiles, clinical manifestations, and radiologic appearance. Under a histopathological point of view, they can be characterized as more or less aggressive with the Pheochromocytoma of the Adrenal gland Scaled Score (PASS) and the Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP) score. The aim of this study is to analyze the texture analysis characteristics of pheochromocytoma and identify whether the texture analysis can yield information aiding in the diagnosis and the characterization of those tumors. METHODS: Radiological, biochemical, and histopathological data regarding 30 consecutive patients with histologically confirmed pheochromocytoma were analyzed. Images obtained in the unenhanced, late arterial, venous, and delayed phases were used for the texture analysis. RESULTS: Urinary epinephrine and metanephrine levels showed a significant correlation (R2 = 0.946; R2 = 699) in the multivariate linear model with texture features, as well as Ki-67 (R2 = 0.397), PASS score (R2 = 0.182), GAPP score (R2 = 0.705), and cellularity showed a significant correlation (R2 = 0.389). The cluster analysis based on radiomic features resulted in 2 clusters, with significative differences in terms of systolic and diastolic blood pressure values at the time of diagnosis (p = 0.025), GAPP score (4 vs 6, p = 0.05), histological pattern (1-2, p = 0.039), and comedonecrosis (0% vs 50%, p = 0.013). CONCLUSION: In conclusion, our study provides the proof of concept for the use of texture analysis on contrast-enhanced CT images as a noninvasive, quantitative tool for helping in the characterization of the clinical, biochemical, and histopathological features of pheochromocytoma.

Radiologically defined lipid-poor adrenal adenomas: histopathological characteristics.

BACKGROUND: Adrenal lipid-poor adenomas (LPA) are defined by high unenhanced density (≥ 10 HU), and absolute and relative contrast medium washout > 60% and > 40%, respectively, at computerized tomography (CT). To date, no thorough histopathological characterization has been performed in those frequent lesions (one-third of adrenal adenomas). Our aim was to analyze the histopathological characteristics of adrenal LPA. METHODS: Patients with LPA (n = 57) were selected among consecutive subjects referred for an adrenal incidentaloma or ACTH-independent Cushing syndrome. FluoroDeoxyGlucose-Positron Emission Tomography (FDG-PET) was performed in 37 patients. In patients treated by adrenalectomy (n = 17), Weiss score and Lin-Weiss-Bisceglia score (in tumors composed entirely or predominantly of oncocytes) were calculated. RESULTS: Radiological parameters did not differ among patients with ACTH-independent Cushing syndrome (n = 6) and those with adrenal incidentalomas associated with primary aldosteronism (n = 2), autonomous cortisol secretion (n = 14), or non-functioning (n = 35). Patients treated by adrenalectomy had larger tumors (28.9 ± 11.2 vs 17.3 ± 8.4 mm, P < 0.001), higher CT unenhanced density (29.1 ± 11.0 vs 23.1 ± 9.0 HU, P = 0.043), and FDG-PET adrenal uptake (9.0 ± 6.4 vs 4.4 ± 2.3 SUV, P = 0.003) than non-operated ones. Oncocytic features > 75% of the tumor were detected in 12/17 cases (70.6%). Five of those showed borderline-malignant histopathological characteristics by Lin-Weiss-Bisceglia score. Among remaining non-oncocytic tumors, 1/5 had a Weiss score ≥ 3. Overall, 6/17 tumors (35.3%) had borderline-malignant potential. Radiological parameters were similar between patients with benign and borderline-malignant tumors. CONCLUSIONS: Adrenal LPA are a heterogeneous group of tumors, mostly composed of oncocytomas. Up to 1/3 of those tumors may have a borderline-malignant potential at histopathology.

[Combined approach of laparoscopy and mini-laparotomy for surgery of a voluminous pancreatic cystic lymphangioma]. / Approccio combinato laparoscopico e mini-laparotomico in un voluminoso linfangioma cistico pancreatico.

Cystic lymphangioma is a rare disease of lymphatic system; in particular, pancreatic cystic lymphangioma is an unusual localization. A correct differential diagnosis with more common glandular lesions allows to plan a proper therapeutic approach. The Authors report the observed last case, a lesion in the head of the pancreas laparoscopically treated, and discuss this uncommon disease.

Prognostic factors in periampullary and pancreatic tumor resection in elderly patients.

BACKGROUND: More than half of all patients with pancreatic adenocarcinoma are over 70 years of age. Life expectancy for the elderly population is increasing and currently major pancreatic resection provides the only meaningful chance of cure for periampullary and pancreatic tumors. Controversy over what constitutes the correct treatment of these tumors in elderly patients continues to this day. The aim of our study was to determine whether age alone or age plus some prognostic factors constitute contraindications to major pancreatic resections. METHODS: Between 2000 and 2005, data from 88 consecutive patients who had major pancreatic resection for periampullary or pancreatic tumors were entered into a prospective database. Fifty-three patients under 70 years of age (young patients), and 35 patients 70 years of age or older (elderly patients) were compared with respect to several characteristics and the postoperative course. RESULTS: Postoperative mortality and morbidity, length of hospital stay, and long-term survival were similar in the two groups. In the elderly group, the mortality rate was significantly higher in patients with chronic obstructive pulmonary disease (COPD), and the morbidity rate was significantly higher in patients with ASA 3 than in patients with ASA 1-2, in whom a pancreaticoduodenectomy or total pancreatectomy had been performed. CONCLUSIONS: Age alone is not a contraindication for major pancreatic resection. In elderly patients a careful evaluation of the co-morbidities and of the type of surgical procedure is mandatory in order to allow the proper selection of those patients best suited for surgery in specialized centers.

Cystic lymphangioma of the pancreas: anatomoclinical, diagnostic and therapeutic considerations regarding three personal observations and review of the literature.

Cystic lymphangioma is a very rare pathology and the pancreatic ones represent an exceptional report that must be considered in the differential diagnosis with others and more frequent cystic lesions of the pancreas. In this paper we describe three cases of cystic lymphangiomas of the pancreas observed in our Institute and we report on the literature review. Anatomopathological, clinical and therapeutics aspects of pancreatic cystic lymphangioma were analyzed for a better knowledge of this cystic lesion and to recognize some specific findings that could allow a preoperative diagnosis and, subsequently, a proper treatment.

Laparoscopic body--tail pancreatic resection for insulinoma.

A case of pancreatic insulinoma with a neuroglycopenic syndrome was treated with laparoscopic body--tail pancreatic resection. An en bloc splenectomy was required due to the close anatomic relation of the insulinoma with the splenic vein, as shown on intraoperative ultrasonography. The operative time was 4 h, and blood loss was minimal (<200 ml). Laparoscopic coagulating shears were used for the pancreatic mobilization, and an endoGIA was used for the section of the splenic vessels and the central pancreatic transection. The postoperative course was uneventful, and the patient was discharged in good condition on the 7th postoperative day. We concluded that laparoscopic access in patients with pancreatic disease is not only a valuable way to establish or confirm a diagnosis and assess the severity of the disease but also a safe way to perform distal pancreatic resection.

Beta-galactoside alpha2,6 sialyltransferase in human colon cancer: contribution of multiple transcripts to regulation of enzyme activity and reactivity with Sambucus nigra agglutinin.

Colon cancer tissues display an increased activity of beta-galactoside alpha2,6 sialyltransferase (ST6Gal.I) and an increased reactivity with the lectin from Sambucus nigra (SNA), specific for alpha2,6-sialyl-linkages. Experimental and clinical studies indicate a contribution of these alterations to tumor progression, but their molecular bases are largely unknown. In many tissues, ST6Gal.I is transcriptionally regulated through the usage of different promoters that originate mRNAs diverging in the 5;-untranslated regions. RT-PCR analysis of 14 carcinoma samples, all expressing an increased ST6Gal.I enzyme activity, and of the corresponding normal mucosa revealed the presence of at least 2 transcripts. One, containing the 5;-untranslated exons, Y+Z, is thought to represent the "housekeeping" expression, and another previously described in hepatic tissues. Both the Y+Z and the hepatic transcripts were detectable in normal and cancer tissues but that latter form had a marked tendency to accumulate in cancer. The extent of alpha2,6-sialylation of glycoconjugates, as determined by SNA-dot blot analysis, was markedly enhanced in all cancer specimens, but the level of reactivity only partially correlated with the level of enzyme expression. Western blot analysis revealed a strikingly heterogeneous pattern of SNA reactivity among cancer tissues. These data indicate that: i) during neoplastic transformation of colonic cells, ST6Gal.I expression may be modulated through a differential promoter usage; ii) the extent of alpha2,6-sialylation of cancer cell membranes is not a direct function of the ST6Gal.I activity, strongly suggesting the existence of other, more complex mechanisms of regulation.

Gastrointestinal autonomic nerve tumor of the jejunum. Case report and review of the literature.

Gastrointestinal autonomic nerve tumor is very rare and it is difficult to distinguish this tumor from other gastrointestinal tumors due to the absence of clinical, instrumental and macroscopic features which allow pre- or intraoperative diagnosis. Our aim was to recognize the characteristic features (preoperative, intraoperative, pathological) that would allow diagnosis of gastrointestinal autonomic nerve tumor. A case of gastrointestinal autonomic nerve tumor of the jejunum is reported. Surgical specimen was routinely processed. Immunohistochemical staining was performed according to modified immunoperoxidase Avidin-Biotin-Peroxidase Complex method. An electron microscopy study was also performed. The tumor mass showed some characteristic pathological findings: histologically, it was composed of spindle cells and epithelioid cells; immunohistochemically, a focal positivity for Neuron Specific Enolase was shown, and finally, ultrastructural examination showed neuron-like cells with long cytoplasmic processes containing microtubules and bulbouns synapse-like structures with dense core neurosecretory-type granules. Preoperatively gastrointestinal autonomic nerve tumor of the jejunum must be considered and treated as a malignant tumor. A correct diagnosis is possible only with immunohistochemical and ultrastructural studies. It is probable that this tumor is more common than previously thought.

Dieulafoy's disease associated with early gastric cancer.

In the past different terms have been used to define the vascular malformations of Dieulafoy's disease--for example, calibre persistent artery of the stomach, cirsoid aneurysm and gastric atherosclerosis. A case of Dieulafoy's disease is described in a 41 year old man, who presented with symptoms of anaemia and melaena, with particular attention paid to the morphological characterisation of the vascular histological lesions. Intimal hyperplasia with a non-concentric proliferation of myointimal cells, areas of muscular degeneration, aspects of vascular neoformation of the arterial wall, and other findings are reported. An association between an early diffuse adenocarcinoma and parietal anomalies of Dieulafoy's disease is illustrated.

Management of a large mediastinal cyst of thyroid origin.

Large mediastinal cysts of the thyroid are rare and it is difficult to make a definitive tissue diagnosis prior to surgical removal. Ultrasonography and computed axial tomography are useful in documenting the cystic nature of the lesion and demonstrating its relationship to other mediastinal structures. These studies may also suggest the tissue of origin. We report a case of massive mediastinal thyroid cyst situated in the right posterior mediastinum and causing significant tracheal compression. Because of this unusual location and lack of iodine uptake, preoperative diagnosis was not possible. At surgery, which we performed via right thoracotomy because of the location of the mass, its origin from thyroid tissue was demonstrated. Resection was accomplished with care taken not to injure the recurrent laryngeal nerve. The patient recovered uneventfully.

Expression of UDP-GalNAc:NeuAc alpha 2,3Gal beta-R beta 1,4(GalNAc to Gal) N-acetylgalactosaminyltransferase involved in the synthesis of Sda antigen in human large intestine and colorectal carcinomas.

N-Acetylgalactosamine beta 1,4-linked to a galactose residue substituted in O-3 with one N-acetylneuraminic acid residue is the immunodominant sugar of the human blood group Sda antigen which is also largely present in the kidney medulla and colon mucosa. A beta 1,4-N-acetylgalactosaminyltransferase very similar to that previously described in urine of Sd(a+) individuals (F. Serafini-Cessi, N. Malagolini, and F. Dall'Olio. Arch. Biochem. Biophys., 266: 573-582, 1988) has been identified in cells released from human large intestine. The higher values of beta 1,4-N-acetylgalactosaminyltransferase activity were detected in proximal and medial segments of the large intestine, suggesting a proximal-distal gradient of the enzyme expression. When the beta 1,4-N-acetylgalactosaminyltranferase activity of colorectal carcinoma specimens from 18 patients was compared with that of the normal mucosa surrounding the tumor, a constant and in several cases drastic reduction of the activity was detected in tumor cells. Three human colorectal adenocarcinoma cell lines (Colo-205, SW-48, and SW-948) have been found to lack the beta 1,4-N-acetylgalactosaminyltransferase activity. Altogether, these results support the notion that the malignant transformation drastically affects the expression of this glycosyltransferase in large bowel cells.

Increased CMP-NeuAc:Gal beta 1,4GlcNAc-R alpha 2,6 sialyltransferase activity in human colorectal cancer tissues.

The sialyltransferase activities of 10 human colorectal specimens were compared with those of the corresponding adjacent normal mucosa. Using asialofetuin as an acceptor we found, in tumor tissues of 9 out of 10 patients, an increased sialyltransferase activity towards the N-linked chains as determined upon peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase (PNGase) treatment. On the contrary, the activity towards the O-linked chains was not significantly changed. When the specificity of the sialyltransferase acting on N-linked chains was investigated by using N-acetyl-lactosamine (Gal beta 1,4GlcNAc) as an acceptor, we found that the alpha 2,6 sialyltransferase activity expressed by both normal and tumor colorectal tissues was far higher than the alpha 2,3-activity and that alpha 2,6 was the only sialyltransferase activity increased in tumor tissues. Kinetic analysis revealed that normal and tumor alpha 2,6 sialyltransferases have the same apparent Km for the acceptor substrate (469 and 465 microns), but normal enzyme has a higher Km for CMP-NeuAc (303 microns) than the tumor enzyme (50 microns). The higher affinity of tumor enzyme for the nucleotide-sugar might partially explain its increased activity in tumor tissues. In addition, tumor tissues contain a lower amount of sialic acid despite the increase in alpha 2,6 sialyltransferase activity.

[Isobutyl-2-cyanoacrylate in resection of the upper pole of the kidney in rats and rabbits: surgical technic and histological followup (author's transl)]. / Isobutil-2-cianoacrilato nelle resezioni polari superiori di rene nel ratto e nel coniglio: tecnica e note istostrutturali.

The authors explored the possibility of using isobutyl-2-cyanoacrylate, a fast-setting plastic adhesive, for controlling parenchymal bleeding after resection of the upper pole of the kidney in rats and rabbits. They describe the immediate results of this technic and histological findings elicited at various removes after experimental surgery.

[Isobutyl-2-cyanoacrylate in atypical liver resection in rats and rabbits: surgical technic and histostructural observations (author's transl)]. / isobutil-2-cianoacrilato nelle resezioni epatiche atipiche nel ratto e nel coniglio: tecnica e note istostrutturali.

The authors examined the behavior of isobutyl-2-cyanoacrylate in connection with atypical liver resection in rats and rabbits, both in terms of immediate hemostatic results and in regard to possible tissue toxicity and degradability of the monomer through serial histological examinations of the cut surface over time.