Showing papers in "Československá patologie in 2007"

[Perivascular epithelioid cell tumor (PEComa) of the liver: a case report and review of the literature].

Abstract: Perivascular epithelioid cell tumor (PEComa) is rare entity and has been described only recently. By immunohistochemistry and genetics it belongs to the family of tumours which comprises angiomyolipoma, clear cell "sugar" tumor of lung, lymphangioleiomyomatosis and clear cell myomelanotic tumor of ligamentum falciforme/teres hepatis. We describe an unusual case of hepatic PEComa arising in a 55-year-old woman with previous history of glioblastoma. Histologically the tumor grew in expansive way, and was composed of clear and eosinophilic epithelioid cels, without vascular or lipomatous component characteristic of angiomyolipoma. There was mild nuclear pleomorphism, sporadic mitotic activity and haemorrhage without necrosis. On immunohistochemistry, the tumor was HMB-45+50, Melan-A and smooth muscle actin positive. Tyrosinase, S-100 protein, cytokeratin coctail, EMA, vimentin, muscle specific actin, CD10, TTF-1, hepatocyte, desmin and cyclin D1 were negative. Sporadic nuclear p53 positivity was seen. The main differential diagnosis of hepatic PEComa includes clear cell variant of liver cell adenoma and hepatocellular carcinoma, metastases of various clear cell carcinomas and metastasis of malignant melanoma. In respect of uncertain biologic potential of PEComa, long term follow up is indicated.

Paraganglioma of the mesenterium: a case report.

Abstract: Summary Extra-adrenal paragangliomas constitute 10 % or less of phaeochromocytomas/ paragangliomas Even rarer is the occurrence of paragangliomas outside the usual distribution of paraganglionic tissue We report a case of extra-adrenal paraganglioma occurring in the small intestine mesentery in a 65-year-old man To our knowledge, there are only seven case reports of paraganglioma occurring in this non-typical site Computed tomography showed a solid expansive nonhomogenously enhancing mesenteric mass, measuring 10 x 8 cm with peripheral cystic component Histologically, the tumour had a typical organoid “zellballen” pattern, showed immunohistochemical positivity for synaptophysin, neuron specific enolase, CD-56, chromogranin, and focally vimentin, and was cytokeratin and EMA negative S-100 protein stained few sustentacular cells The patient was free from recurrence or metastasis three months after tumour resection Although rare, paraganglioma should be included in the preoperative differential diagnosis of solid mesenteric tumours, to prevent any potential life-threatening event peroperatively in the case of a catecholamines-producing tumour Suhrn Paragangliom mezenteria: kazuistika Extraadrenalne paragangliomy tvoria do 10 % feochromocytomov/paragangliomov Este vzacnejsi je výskyt paragangliomov mimo zvycajnej distribucie paraganglii Popisujeme pripad extraadrenalneho paragangliomu mezenteria tenkeho creva u 65 rocneho muža Podľa nasich vedomosti, existuje iba sedem popisaných pripadov výskytu paragangliomu v tejto atypickej lokalizacii Vysetrenie pocitacovou tomografiou zobrazilo solidny expanzivny kontrastom nehomogenne zvýraznený nador veľkosti 10 x 8 cm Nador bol periferne cysticky zmenený Histologicky mal nador typicku organoidnu „zellballen“ architektoniku, bol imunohistochemicky synaptofyzin, neuron specificka enolaza, CD-56, chromogranin a fokalne vimentin pozitivny Cytokeratin a EMA boli negativne S-100 protein zobrazil sporadicke sustentakularne bunky Pacient bol 3 mesiace po operacii bez znamok recidivy alebo metastaz Napriek tomu, že je výskyt paragangliomu v mezenteriu vzacny, mal by byť zahrnutý do predoperacnej diferencialnej diagnozy, aby sa predislo potencionalnej život ohrozujucej prihode pocas operacie tumoru produkujuceho katecholaminy Kľucove slova: extraadrenalny paragangliom – mezenterium – feochromocytom

Expression of E-cadherin and c-erbB-2/HER-2/neu oncoprotein in high-grade breast cancer.

Abstract: Summary E-cadherin (E-CD) is an epithelial-specific cell adhesion molecule, whose expression is lost in invasive lobular (ILC) but not in invasive ductal carcinoma (IDC) of the breast. This cell adhesion system can be disrupted by tyrosine kinase c-erbB-2/HER-2/neu. We examined 106 cases of highgrade invasive breast cancer, including 91 IDCs, 12 ILCs and 3 pleomorphic lobular carcinomas (PLCs). We determined Nottingham histological grade and performed immunohistochemistry for estrogen and progesterone receptors (ER/PR), Ki-67, E-CD and c-erbB-2/HER-2/neu with subsequent fluorescence in situ hybridization. Amplification of c-erbB-2/HER-2/neu gene was observed in 55/91 (60.4%) of IDCs, 3/12 (25%) of ILCs and 1/3 (33.3%) of PLCs, and associated with positive axillary lymph nodes. E-CD expression was lost in 14/91 (15.4%) of IDCs, 10/12 (83.3%) of ILCs and 2/3 (66.7%) of PLCs. The loss of E-CD immunoreactivity in IDCs appeared to be associated with c-erbB-2/HER-2/neu gene amplification, negative ER/PR status and positive lymph nodes, whereas E-CD-positive ILCs tended to be HER-2/neu-positive. The biological significance of E-CD expression seems to be different in high-grade IDC and ILC. Oncogenic pathway mediated by cerbB-2/HER-2/neu may affect the E-CD expression in most invasive ductal breast carcinomas in vivo.

[Cutaneous sarcoidosis during pegylated interferon alpha and ribavirin treatment of chronic hepatitis C--a case report].

Abstract: Interferon-induced sarcoidosis is well documented. We report a case of sarcoidosis in a patient with hepatitis C virus infection treated with pegylated interferon alpha and ribavirin. The patient developed cutaneous sarcoidosis about 6 months after beginning of the combination therapy. Spontaneous regression of the lesions was noted after discontinuation of the treatment. Interferon-alpha is known to stimulate T helper cells with a TH-1 profile immune response, which is the key immunologic event of a sarcoid granuloma formation. In addition to its antiviral action, ribavirin also enhanced the TH-1 response. Incidence of drug induced sarcoidosis may be underreported, because many symptoms of sarcoidosis mimic the side-effects of interferon as fever, myalgias, arthralgias, fatigue and symptoms of pulmonary diseases.

Malignant fibrous histiocytoma of the parotid gland

Abstract: We described a rare malignant fibrous histiocytoma of the parotid gland in a 63-year-old woman. During six month the tumour size became 10 cm in diameter with skin ulceration. The tumour was examined morphologically, by immunohistochemistry and molecular biology methods-FASAY and CGH.

[Primary Hodgkin lymphoma of the stomach--a case report].

Abstract: A case of a 69-year-old male with a one-month history or recurrent attacks of GI bleeding, secondary anemic syndrome and epigastric pain is reported. Endoscopic examination revealed a tumorous lesion suspicious of malignancy. Neither lymphadenopathy nor any other tumorous lesion was identified by extensive clinical examination. In the biopsy specimens there was ulcerated gastric mucosa with an infiltration by lymphoid cells, predominantly of mature T-cell type. Atypical large cells with large nuclei with prominent nucleoli were dispersed among the mature lymphocytes. These atypical cells were both mono- and binucleated (Hodgkin and RS cells). Both cell types revealed immunohistochemically membranous and dot-like perinuclear positivity of CD30 and CD15 antigens. Based on morphologic features and immunohistochemical findings, a diagnosis of primary gastric Hodgkin lymphoma was established. Diagnostic approach, as well as differential diagnosis of primary Hodgkin lymphoma in this extremely rare location, are discussed.

[Extrapleural solitary fibrous tumor mimicking lateral neck cyst--a case report].

Abstract: A 62-year-old man was referred to the Department of Dentistry because of ultrasonographic finding of "cystoid lesion with relationship to right parotid gland". During operation, a tumor mass without any relationship to parotid gland but attached to the right internal carotid artery was found. Grossly, the tumor was well circumscribed, spheric, measuring 40 mm in diameter; it was of solid, firm appearance and tan-to-white color on cross section. Microscopically, the tumor cells were round to spindle-shaped with vesicular nuclei and eosinophilic cytoplasm, arranged in fascicular pattern. Immunohistochemically, the cells expressed vimentin, CD 34, smooth muscle actin, and bcl-2 protein. On the basis of microscopical appearance and results of immunohistochemistry, the diagnosis of solitary fibrous tumor (cellular variant) was established. One year after resection, the patient is free of disease. A new concept of this uncommon mesenchymal tumor is discussed.

Cutaneous angiosarcoma following conservative surgery and radiotherapy for breast carcinoma. A case report

Abstract: Breast angiosarcomas (AS) are very rare neoplasms, which can be divided into primary (or sporadic), and secondary AS, the latter arising either on the base of lymphoedema after mastectomy (so called AS with Stewart-Treves syndrome- ASSTS), or skin AS after breast conservation surgery with subsequent radiotherapy for breast cancer (KPRAS). The authors present a case of a 55-year-old female patient with a 17mm tumour in nipple region, developing 8 years after primary diagnosis of tubulolobular carcinoma of the breast which was treated by breast conservation surgery and radiotherapy. A probatory bioptic examination of the lesion proved well-differentiated KPRAS. The patient underwent mastectomy. The authors analyse the basic clinical and morphological features of KPRAS, which distinguish it from other forms of AS, e.g. occurrence in older age, shorter period of latency after radiation therapy when compared to ASSTS, absence of lymphoedema, and rare involvement of the breast tissue. Prognosis of this entity is very poor; today the most reliable prognostic marker is histological grading. However, it will be needed to assess in the future new indicators of prognosis of patients with this rare disease.

Angiogenesis in the bone marrow of patients with chronic lymphocytic leukaemia

Abstract: Angiogenesis is a process of formation of new vessels from the preexisting ones. It is involved in many physiological processes, at the same time, however, it is involved also in the progress of tumoral growth. Although a lot is known about angiogenesis in solid tumors where it plays a role in tumoral invasion and its metastatic potential, in hematological malignancies it has been appreciated only recently. However, the results of studies on abnormal angiogenesis in hematological malignancies are inconsistent. Angiogenesis can be studied at different levels; histologically, it is studied in the infiltrated tissues (lymph nodes, bone marrow) and quantified as microvessel density (MVD). The aims of our study were to introduce the method of MVD quantification in the bonemarrow using immunohistochemical detection of endothelial markers (fVIII) and then evaluate MVD in bone marrow samples in a group of patients with chronic lymphocytic leukaemia (CLL) and compare the results with a control group of patients (CON). CLL is a typical malignancy of the hematopoietic tissue but the course and the prognosis of patients with this disease vary considerably. For this reason there is urgent need for novel prognostic markers in order to assess individual patient prognosis and tailor treatment. Angiogenesis is one of the possible markers which may add more informations about the course of this disease. So far only few studies have been published about angiogenesis measured as MVD in CLL patients andthe results are inconsistent. In our study, both the number and the area of microvessels were increased in bone marrow of patients with CLL, but the number and area of sinuses were not. It can be concluded that there are signs of abnormal angiogenesis in bone marrow of patients with CLL but larger study with longer follow-up is needed to give more specific information about prognostic value of these findings.

[Problems of suitability laser's excision of pigmented dermal lesions: case report of minimal deviation melanoma].

Abstract: The paper describes a case of a 23-year-old woman, in whom an intradermal pigmented nevus was removed by an excision using a CO2 laser. In histological examination a minimal deviation melanoma (MDM) was found, occurring sparsely in the centre of the nevus. A sufficiently wide excision is important not only in terms of the pathologist who assesses the lesion but also, in particular, in terms of the prognosis to the patient. In problematic diagnoses where it is necessary to assess the extent of the lesion as well as its edges and base in particular, the sample taken by laser may be insufficient. The authors report widely used methods of nevus laser surgery and highlight the necessity of a sufficiently radical exstirpation of a nevus.

A case of excessive autophagocytosis with multiorgan involvement and low clinical penetrance.

Abstract: Summary An autopsy and microscopic analyses of a 74-year-old female with a clinical history of cardiac hypertrophy and hypertension disclosed a pronounced distension of lysosomal compartment with signs of excessive autophagocytosis, predominantly in cardiomyocytes, hepatocytes and smooth muscle cells of the small intestine. The histological storage pattern did not correspond to the usual changes seen in defined lysosomal storage disorders. The amount of age-related lipopigment was low in all tissues. Confocal microscopy of liver tissue samples documented a progressive loss of mitochondrial epitopes in the distended lysosomal compartment along the porto-central axis of hepatic lobules. The possibility to detect subunit c of mitochondrial ATP synthase (SCMAS) indicated extensive intra-lysosomal degradation of mitochondria, both in hepatocytes and smooth muscle cells. The SCMAS epitope can thus be considered a valuable immunohistochemical marker of autophagocytic mitochondrial degradation. The distended lysosomes also displayed tissue specific ubiquitination. Absence of immuno-detectable p62 protein excluded aggresome formation. An inherent dysfunction of the late endosomal/lysosomal LAMP2 protein (Danon disease), was excluded on the basis of LAMP2 gene sequence analysis and LAMP2 protein levels. Whether the observed process reflects a primary dysregulation of the constitution of the autophagosomal membrane or was induced by defects in other cellular components, remains unanswered. Whatever mechanism involved, the findings should be considered relevant in differential diagnostics, despite their low clinical penetrance, should be registered and thus rendered available for future definition.

Histopathologic changes in gastroesophageal reflux disease. A study of 126 bioptic and autoptic cases.

Abstract: The histologic diagnosis of reflux esophagitis is still complicated by the lack of a consensus opinion on what is the normal mucosa in the area of the gastroesophageal junction (GEJ). Most authors consider GEJ as the junction between the squamous and the cardiac epithelium. The cardiac mucosa is composed of mucinous or mixed mucinous-oxyntic glands. These glands are in fact indistinguishable from metaplastic mucosa that arises in the distal esophagus in consequence of gastroesophageal reflux (GER). The cardiac mucosa shows invariably chronic inflammatory changes referred to as "carditis". The cause of "carditis" is GER and/or Helicobacter pylori (HP) infection. In our series of 120 endoscopic biopsies of the GEJ and distal esophagus the cardia type mucosa (CM) was always present. In 15 cases, it was accompanied by oxyntocardiac mucosa. Both mucosa types showed chronic inflammation that is after exclusion of HP infection regarded as a strong diagnostic sign of the gastroesophageal reflux disease (GERD). In two cases with clinical symptoms of GERD, a few HP were found on the CM. Therefore we diagnosed them as GERD with secondary HP infection. In 17 cases, CM displayed intestinal metaplasia (IM) predominantly of incomplete type and no dysplasia. This IM expressed MUC6 in the glandular zone of the mucosa like it did in the neighboring glands, whereas in the surface and foveolar epithelium the MUC6 was negative or only slightly and focally positive. On the other hand, IM in the surface and foveolar epithelium was reactive for MUC5AC. The positivity and distribution of CK7 and CK20 was very similar in the Barrett's mucosa, cardiac mucosa and antral mucosa. In one specimen of esophagus resected for adenocarcinoma, CM with incomplete IM was found in the vicinity of the tumor. Squamous metaplastic epithelium was often seen near the orifices of submucosal esophageal glands in these areas, indicating the metaplastic nature of the glandular mucosa in the distal esophagus. In the GEJ of 5 autopsy cases of children with spastic quadriplegia (age range 7-10 years) CM in a short segment (0.5-3 mm in length), probably of metaplastic origin was identified, showing chronic inactive inflammation.