Stanisław Sporny

Primary perivascular epithelioid cell tumor (PEComa) of the liver: Report of a case

PEComa is very rare mesenchymal neoplasm which is formed by perivascular epithelioid cells and is characterized by dual melanocytic and myoid differentiation. Up to now only a very few cases of PEComa of the liver have been described worldwide. We herein present a patient who underwent a right hemihepatectomy for a huge tumor which could not be identified by imaging investigations. A final histopathologic examination revealed a benign epithelioid tumor with a solid growth pattern, abundant vascularity, and frequently dilated vascular channels. Immunohistochemically, the tumor cells were strongly positive for HMB-45, moderately positive for actin, and faintly positive for S-100, respectively. Based on the above findings, a diagnosis of a primary clear cell “sugar” tumor was established. Because the natural history of PEComas is mostly unpredictable, the patient has been closely followed up; however, no recurrence has so far been observed. Immunohistochemical findings play a crucial role in avoiding a misdiagnosis, and a surgical resection with an adequate margin of healthy tissue remains the gold standard of treatment. A long-term periodic follow-up is reasonable in all cases presenting with PEComa.

Single nucleotide polymorphisms of RAD51 G135C, XRCC2 Arg188His and XRCC3 Thr241Met homologous recombination repair genes and the risk of sporadic endometrial cancer in Polish women

Background:  The genes RAD51, XRCC2 and XRCC3 encode proteins that are important for the repair of double‐strand DNA breaks by homologous recombination. Therefore, genetic variability in these genes may contribute to the occurrence and progression of endometrial cancer.

The fine-needle aspiration biopsy efficacy of small thyroid nodules in the area of recently normalized iodine supply

OBJECTIVE To evaluate the incidence of focal lesions in the thyroid in the area of recently normalized iodine supply as well as to compare the efficacy of fine-needle aspiration biopsy (FNAB) of small (infracentimetric) and large thyroid lesions in this area. METHODS The outcomes of 13,646 ultrasound (US) examinations, 13,437 US-guided FNABs of the thyroid and 1694 results of post-operative histopathological examinations were analysed. RESULTS Infracentimetric nodules (INs < or = 10 mm) were revealed by US examinations in 43.5% of patients; in the majority of the cases (82.2%) INs were multiple. The percentage of revealed carcinomas by aspiration of INs is similar to that observed when large nodules (LNs > 10 mm) are examined cytologically. However, the efficiency of preoperative diagnosis of INs is lower than LNs with respect to both US selection of lesions for FNAB and the percentage of false negative results of FNAB (29.8 vs 5.4%, P<0.001). In post-operative histopathological examination, extrathyroidal extension of thyroid cancers was observed in nearly 30% of microcarcinomas. CONCLUSIONS In endemic or post-endemic areas, the efficiency of FNAB is lower in the case of small lesions than larger ones. In spite of this, the percentage of cytologically revealed carcinomas among small lesions is not lower than larger ones. Thus, it is particularly indicated to follow up small thyroid lesions with repeated US examinations in such areas.

Asymptomatic solid pancreatic hamartoma

Pancreatic hamartomas occur extremely rarely. They may appear as solid masses or cystic forms and should be regarded as malformations rather than neoplasms [1]. Due to absence of characteristic differential features they may be clinically and radiologically mistaken for a malig7-18062cy [2]. Misinterpretation of hamartoma as a true neoplasm may result in unnecessarily aggressive surgery. The authors herein present a case report of solid pancreatic hamartoma and demonstrate its characteristics along with a review of the literature and discuss the problem of potential overtreatment of these patients. A 69-year-old male patient was presented with a hypoechogenic, asymptomatic pancreatic body mass, incidentally revealed by ultrasonography. The patient gave no medical history of either chronic pancreatitis or other diseases of the pancreas. Pancreatitis and neoplasm (CEA, CA 19-9) markers were within the normal range; pancreatic exocrine and endocrine function was sufficient. Further endosonography scans revealed a 28 mm × 22 mm hypoechogenic tumour-like lesion within the body of the pancreas (Figure 1). Computed tomography scans confirmed a solid tumor with ill-demarcated margin (Figure 2). The patient was qualified for surgical treatment. Intraoperatively, the oval, pancreatic body mass of firm consistency was confirmed. Central pancreatic resection with Roux-en-Y pancreaticojejunostomy to the distal pancreatic rem7-18062t was performed. The postoperative period was complicated by leakage from the pancreatojejunostomy. Reoperation with re-suturing of the anastomosis was applied. The remaining postoperative time was uneventful. So far, the patient has been followed up for 55 months and has remained disease-free. Figure 1 7.5 MHz radial EUS view of a 28 mm × 22 mm pancreatic corpus tumour-like lesion Figure 2 Computed tomography revealing solid tumour within the body of the pancreas Macroscopically, a firm, solid, whitish, well-circumscribed, encapsulated mass measuring 3 cm in maximum diameter was confirmed. Histologically, the tumour was almost entirely composed of disorderly arranged, well-differentiated endocrine and exocrine pancreatic tissue (Figure 3). The spindle morphology of a small quantity of stroma cells and cystically dilated pancreatic ducts, filled with pancreatic juice, were observed (Figure 4). The tumour was surrounded by adjacent normal pancreatic parenchyma without significant fibrosis or features of chronic inflammation. Insulin, glucagon, chromogranin, somatostatin, amylase, CD34, CD117, S-100 and desmin immunostainings confirmed colocalization of the typical pancreatic cells. Surprisingly, immunohistochemical analysis of CD34 and CD117 showed a negative reaction in elongated stoma tumour cells. Therefore, the diagnosis of pancreatic hamartoma was established. Figure 3 Exocrine part of the pancreas with islets and adipose tissue. HE, 120× Figure 4 Dilated pancreatic ducts. HE, 120× Hamartomas of the pancreas are rarely reported, since only a few cases have been described in the literature. Clinicopathological features of all noted cases are summarized in Table I [2-10]. Some authors have observed that this kind of lesion includes spindle-shaped cells which are immunoreactive for CD34 and CD117 [9]. The descriptive term of these tumours as cellular hamartomas resembling gastrointestinal stromal tumours was suggested. In our case, pathological changes in the cellular shape of stroma cells were observed, although immunohistochemical tests for CD 34 and CD117 were non-contributory, which excludes such a pathological characterization. Table I Characteristics of pancreatic hamartomas reported in the literature The histogenetic concept of hamartomas is enigmatic. Hamartomas of the pancreas were thought to coexist with chronic pancreatitis. On the other hand, Pauser et al. excluded such cases from this group. In the authors’ opinion, the term of hamartoma should be reserved for asymptomatic patients. The hypothesis that inflammation is one of the factors that induce hamartoma development is not valid, since chronic pancreatitis may just mimic hamartoma lesions lacking acinar cells. The natural course of hamartoma is not characteristic, as in our described case. Routine laboratory tests and imaging studies are non-contributory in establishment of the final diagnosis. Needle biopsy is suggested to be feasible in differential diagnosis. However, false negative results may occur frequently, and it may be associated with tumour cells seeding in malig7-18062cy. Thus, in the authors’ opinion, patients with solitary incidentaloma such as hamartoma of the pancreas should not undergo routine pancreatic tumour biopsy. Currently, preoperative differentiation between hamartomas or other benign tumours and malig7-18062cies is very difficult, if not in many cases impossible. As a consequence, it raises a question about treatment of patients with incidentally diagnosed solitary pancreatic tumour of unknown character. In view of the fact that pre-malig7-18062t and malig7-18062t histology of pancreatic asymptomatic incidentalomas is far more frequent, still surgical excision should be the treatment of choice, even though aggressive surgery may be overtreatment in patients among whom postoperative histopathological examination revealed hamartoma or other benign tumour. In conclusion, pancreatic hamartoma is an extremely rare medical problem. Misinterpretation of asymptomatic hamartomas as a true neoplasms may result in unnecessarily aggressive surgery. Nevertheless, preoperative diagnostic difficulties may be finally resolved only with histopathological examination of the postoperative specimen.

The effect of short-term intoxication of rats with pentabromodiphenyl ether (in mixture mimic commercial products)

Until quite recently, pentabromodiphenyl ether (PentaBDE) was most commonly used as a flame retardant. Due to the considerably long atmospheric half-life of PentaBDE and its contribution to environmental pollution, it is categorized as a persistent organic pollutant (POP). As the data on the toxicity of PentaBDE is rather scarce, its potential acute toxicity was the subject of this study. PentaBDE was administered intragastrically to female rats, in a single dose (25, 200 or 2000 mg/kg b.w.). PentaBDE administered to rats disturbed redox homeostasis, which was manifested by lower total antioxidant status (TAS) in serum and by higher liver glutathione reduced (GSH) concentration. The toxic effect of PentaBDE intensified lipid peroxidation. On histopathological examination, administration of the highest PentaBDE dose (2000 mg/kg b.w.) was seen to induce symptoms of fatty liver. PentaBDE caused an increase in relative liver mass, cytochromes P-450 (after two highest doses), a dose-dependent increase in the activity of CYP lA (12—26 fold) and CYP 2B (5—6 fold) as well as the levels of CYP lAl (16—50 fold) and CYP 4A (2—3 fold) in liver.

β-catenin as a prognostic factor for prostate cancer (PCa).

Introduction The prostate cancer is difficult to predict, and treatment failure is associated with local infiltration, as well as distant metastases. Adhesion and migration abilities to of cancer cells play a major role in formation of metastasis. The participation of β-catenin in pathogene-sis of many types of cancer and benign processes has been an important discovery of recent years. Material and methods The studied material was obtained by transrectal, sextant core biopsy from 102 patients hospitalized in Department of Urology, Regional Hospital in Kalisz (2001-2004). The aim of our study was to determine the predictive value of β-catenin immunoexpression in prostate cancer, to analyze the prognostic aspect of some histopathological features and finally to assess the relationship between β-catenin immunoreactivity and the microscopic image of the tumor. Relationships between the investigated variables were analyzed using the Chi2 test of compatibility. We used the Kaplan-Meier curves to assess survival differences between groups of patients. Finally we established which of the studied factors significantly affect the patient outcome, using the method of Cox proportional hazard regression. Results In prostate cancer in comparison with the normal epithelium, both the location and the strength of β-catenin immunoexpression are impaired. Conclusions Our results indicate that the presence of disorders in β-catenin immunoexpression in prostate cancer cells indicates a high risk of death due to tumor progression and makes it imperative for immediate treatment procedures.

Diagnostics and treatment of differentiated thyroid carcinoma in children - Guidelines of Polish National Societies.

1Department of Paediatric Endocrinology and Rheumatology, Poznan University of Medical Sciences, Poznan, Poland 2Department of Nuclear Medicine and Endocrine Oncology, Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Gliwice Branch, Gliwice, Poland. 3Department of Paediatrics and Paediatric Endocrinology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland 4Department of Oncological and Reconstructive Surgery, Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Gliwice Branch, Gliwice, Poland 5Department of Oncological Endocrinology and Nuclear Medicine, Centre of Oncology – Maria Sklodowska-Curie Memorial Institute, Warsaw, Poland 6Department of Tumour Pathology, Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Gliwice Branch, Gliwice, Poland 7Department of Paediatrics and Endocrinology, Medical University, Warsaw, Poland 8Department of General and Oncological Surgery, Medical University of Lodz, Lodz, Poland 9Private practice, Katowice, Poland 10Department of Paediatric Surgery and Urology, Wroclaw Medical University, Wroclaw, Poland 11Department of Morphometry of Endocrine Glands, Chair of Endocrinology, Medical University of Lodz, Lodz, Poland 12Department of Dental Pathology, Medical University of Lodz, Lodz, Poland 13Department of Tumour Pathology, Poznan University of Medical Sciences, Poznan, Poland 14Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, Poznan, Poland 15Department of Endocrinology and Metabolic Diseases, Polish Mother’s Memorial Hospital-Research Institute, Medical University of Lodz, Lodz, Poland

E-cadherin expression is more associated with histopathological type of thyroid cancer than with the metastatic potential of tumors

The aim of this study was to evaluate the relationship between abnormal expression of E-cadherin (E-CAD) and the extracapsular extension of tumors, lymph node involvement and the presence of metastasis in various types of thyroid cancers. Histopathological specimens of 35 benign thyroid lesions and 122 malignant tumors (papillary, follicular, poorly differentiated and undifferentiated cancers) were analyzed. E-CAD immunostaining intensity, its subcellular localization, homogeneity within lesion, and the relation of staining intensity between tumor and surrounding thyroid parenchyma were evaluated. The obtained results show that the variants of differentiated cancers with a poorer prognosis (i.e. tall cell and follicular variants of papillary cancer and widely invasive follicular cancers) present reduced intensity of E-CAD expression, its abnormal localization or heterogeneity of staining more frequently than classical papillary cancers and minimally invasive follicular cancers. However, the assessment of E-CAD expression does not allow the prediction of extrathyroidal growth of thyroid cancers.

Synchronous occurrence of multiple focal nodular hyperplasia and huge hepatic Perivascular epithelioid cells tumor (PEComa) in young woman after oral contraceptive use – is there a common pathogenesis?

The association of focal nodular hyperplasia (FNH) and various neoplasms was described, but coincidence of multiple FNH and hepatic perivascular epithelioid cells tumor (PEComa) has not been reported. The clinical debate of oral contraceptive (OC) influence on FNH growth is ongoing, but no evidence exists about association of hepatic PEComa with OC use. Herein, we report a case of two FNH lesions and huge (150x100x80 mm) left hepatic lobe PEComa that occurred simultaneously in 18-year-old female with previous two year history of OC use, who underwent left hemihepatectomy and right hepatic FNH enucleation. Up to date, the patient has been followed-up for 65 months and remained disease-free. FNH and PEComa have a common vascular cytogenetic denominator. Our case raising a question of a causal relationship of FNH and PEComa with OC use that might be attributed to vascular changes. Future researches of larger sample sizes should further address this issue.

Non-diagnostic cytological outcome of thyroid biopsy and the risk of thyroid malignancy.

This study assessed the incidence of neoplasms, including malignant tumors, in lesions within the thyroid gland from which non-diagnostic biopsy aspirates were obtained. An auxiliary goal of the study was an evaluation of the diagnostic efficacy of repeated biopsy in cases when the first biopsy was non-diagnostic. Thus, results of 4603 fine-needle aspiration biopsies (FNABs) were submitted to histopathological verification. The verification revealed the rate of malignancy at 7.1% for non-diagnostic biopsies, i.e., significantly higher (p<0.001) than that in cases FNAB-diagnosed as benign lesions (2.1%). Repeated biopsy, performed when inadequate material has been collected, seems to be less effective than the first biopsy (non-diagnostic specimens; 14.4% vs 8.9%; p<0.01). The occurrence of neoplasms in the goiter was significantly higher (p < 0.0001) in patients with non-diagnostic first FNAB than in those with diagnostic one (50.7% vs 33.5%, p<0.001). And again, in patients with two non-diagnostic FNABs, the occurrence of neoplasms was higher than that in patients with the second diagnostic cytology (63% vs 41.2%, p<0.05). According to our data, patients with non-diagnostic FNAB results should be very carefully monitored, especially when the repeated biopsy is either non-diagnostic again or not performed at all.