[Sarcomatoid (metaplastic) spindle cell carcinoma arising in a phylloid tumor with massive squamous metaplasia - a case report and review of the literature]. / Sarkomatoidní (metaplastický) vretenobunecný karcinom prsu vznikající ve fyloidním tumoru s rozsáhlou skvamózní metaplázií - kazuistika a prehled literatury.

UNLABELLED: A 76-years-old woman underwent a partial mastectomy and a low-grade malignant homologous phyllodes tumor measuring 45 mm in maximum diameter was diagnosed. Beyond its typical dual composition the tumor displayed extensive intraductal squamous metaplasia. Approximately in one third of the lesion the original mesenchymal component cytologically and structurally changed which ultimately led to seeming stromal overgrowth. The loose storiform background contained isolated larger atypical elements with ample eosinophilic cytoplasm and obvious mitotic activity. This final fibromatosis-like arrangement was completed either by multiple dispersed abrupt squamous morules or just by pearl-like abortive form of squamous differentiation. Conventional in situ or invasive ductal carcinoma was not present. A combined expression of both low and high molecular weight cytoketatins, S100 protein, p63, CD10 and GFAP confirmed the incomplete basal/myoepithelial phenotype and ultimately led to the diagnosis of a spindle cell metaplastic carcinoma arising in a phyllodes tumor - a neoplasm unpublished so far. A review of the literature concerning epithelial malignancies originating from a milieu of phyllodes tumor guides discussion/speculation over the possible histopathogenesis of this vanishingly rare lesion. KEYWORDS: breast - phylloid tumor - phyllodes tumor - spindle cell sarcomatoid/metaplastic carcinoma - squamous metaplasia.

[Secondary angiosarcomas after conservation treatment for breast cancers]. / Sekundární angiosarkomy po konzervativn lécebe nádoru prsu.

BACKGROUNDS: The cancerogenic effect of ionizing radiation was documented just several years after it started to be used as a treatment option. Ionizing radiation produces a small but detectable risk of carcinoma as well as bone and soft tissue sarcomas. Over the past 20 years angiosarcomas arising from the area of the irradiated breast have been reported with increasing frequency as the number of women undergoing the breast conserving surgery with consecutive radiotherapy has increased also. Angiosarcomas can originate from either lymphatic or capillary endothelium, namely lymphangiosarcomas and haemangiosarcomas. The most of haemangiosarcomas arising from the breast skin developed in the irradiated area after breast conserving procedure--secondary angiosarcomas. Lymphangiosarcoma is typically associated with longstanding extremity lymphedema--Stewart-Treves syndrome. CASES: We report three cases of angiosarcomas which occured in this region after breast conserving treatment and we also review the literature. CONCLUSION: Paradoxically, the decrease in the use of radiotherapy to the post-mastectomy chest wall and the axillary area is expected to reduce the incidence of angiosarcomas, while the increase in the use of breast conserving procedure plus radiotherapy could lead to increased incidence of angiosarcomas in the residual breast tissue. Special attention should be paid to skin leasions and changes occuring after breast conserving treatment and especially to the ones with the skinthickening. The early detection and diagnosis has the crucial prognostic value.

[Primary sebaceous carcinoma of the breast; three casuistic reports]. / Primární sebaceózní karcinom prsu - klinicko-morfologická analogie kozní adnexální léze? Popis trí prípadu.

OBJECTIVE: Presentation of three cases of primary sebaceous carcinoma of the breast particularly focusing on the clinical, biological and molecular genetic aspects regarding their possible pathogenetic relationship to the Muir-Torre and Lynch syndrome. Reviewed are basic principles of miscosatellite instability and dysregulations of mismatch repair genes by these inherited tumorous syndromes especially looking for morphologic and fenotypic parallels between sebaceous carcinomas of the breast and their cutaneous counterparts. DESIGN: Three casuistic reports. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: Three casuistic reports are covered in detail including broad immunohistochemistry (LSAB+, Dako). RESULTS: In three women aged 51 to 69 was diagnosed primary sebaceous carcinoma of the breast with maximum dimension ranged from 13 to 41 mm. Lumpectomy was performed at the smallest one and included sentinel lymph node examination turned out to be negative. The other two patients underwent modified mastectomy with axillary lymph node dissection. In tumor sized 25 mm, macrometastasis 4 mm in maximum dimension was identified in one axillary lymph node. Follow-up available in two women, both without regional metastasis, revealed no local or distant progression of the disease. The histology consisted of conventional G1-2 invasive duct carcinoma in all cases and sebaceous differentiation represented 10-40% of all neoplastic population. The patognomic features included cells with ample eosinophilic/clear foamy cytoplasm, partly with multiple crowded small vacuoles characteristically impressing the nuclei. All tumors were ER positive and Her2/neu 2+ lesion was not amplified. Strong diffuse nuclear expression of MLH1, PMS2, MSH2, MSH6 proteins in all cases confirmed unaltered mismatch repair genes pathway. Familial tumorous stigmas were not evident and subsequent close clinical monitoring in two of the patients tracked down no intern malignancy, including cutaneous sebaceous lesion.

[Metastases to the breast from primary extramammary tumors--real diagnostic dilemma]. / Metastázy extramamárních solidních tumoru do mlécné zlázy--skutecný diagnostický problem.

OBJECTIVE: To address the difficulty and possible pitfalls in diagnostics of breast mtttstases from extramanmnary primaries--a pathobiological phenomenon that is not always thought of. To underline the open-minded differential diagnostic algorithm that is naturally weakened by dominant straightaway logistics in a routine contemplation. To emphasize the importance of complete and relevant clinical data including the anamnesis. To evaluate the effectiveness velocity and cheapness of imunuohistochemistry at reaching the correct diagnosis. DESIGN: Comprehensive study of the unusual pathogenetic setting based on personal experience with eight observations and literature review. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: Detailed clinicopathologic characteristics and review of morphologic spectrum in nine cases of extramammary tumors metastatic to the breast (three melanomas, two small cell carcinomas, one carcinoma from salivary gland, ovary, kidney and prostate). Standard immunohistochemistry was used as a reliable tool for phenotypic evaluation. RESULTS: In total nine cases, eight women and one man, were identified among 3238 of malignant breast tumors in the years 2005-2010. There were three melanomas, two small cell carcinomas and by one carcinoma from salivary gland, ovary, kidney and prostate. The age ranged from 43 to 81 years and maximum size of lesion spanned 7-31 millimeters. All specimens were core needle biopsy and only in one patient the past medical history concerning the nonbreast malignancy was known (ovarian serous carcinoma). Two neoplasms (one of the melanomas and small cell carcinomas) were the first sign of underlying malignant process ever; in this melanoma the clinical workout to disclose primary skin lesion failed. These two tumors were also the only ones, where the multiple or bilateral involvement of breast was clinically documented. There was evident other organs spread in three cases at the time of diagnosis (generalization in melanoma and prostate carcinoma; skin metastasis in salivary gland carcinoma) and axillary lymph nodes involvement in two melanomas. In five patients where follow-up was available, three died of tumor in interval from five to eighteen months (in turn melanoma, small cell and prostate carcinoma). Four original pathologic verdicts turned out to be wrong (two melanomas, salivary gland and prostate carcinoma); in three of them final correct diagnosis was established even after additional clinical information about morphologically verified malignancy in the past.

[Ovarian tumor as a complication in diagnosis of the extraintestinal gastrointestinal stromal tumor--two case reports]. / Nádor ovaria jako komplikace diagnostiky extraintestinálního gastrointestinálního stromálního tumoru--dve kazuistiky.

OBJECTIVE: The aim of this study is to address difficulties in diagnosis of the extraintestinal gastrointestinal stromal tumor (GIST) when even in ovary is the tumor. One should ever think of the GIST in the case of peritoneal dissemination of the spindle/epithelioid cell tumor. In contrary, the late intraabdominal recurrence of primary both epithelial and non-epithelial tumor of genital tract can be undistinguishable by pure morphology. Complete clinical records and accurate operation field topography are both of great value. Documenting this phenomenon, presented is both late intraabdominal dissemination of primary uterine endometrial stromal sarcoma and massive ovarian involvement by extraintestinal GIST. DESIGN: Two case reports. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: The two casuistic reports are covered in detail including broad immunohistochemistry (LSAB+, Dako) and genetics (ABI Prism 310, PE/Applied Biosystems). RESULTS: In 75-year-old woman, 5 cm tumor in maximum diameter of the mesentery was diagnosed as an extraintestinal GIST. Multiple peritoneal recurrence occurred ten years later and the tumor has been reclassified as a disseminated endometroid stromal sarcoma. Ultimately, comprehensive search of clinical files confirmed hysterectomy and adnexotomy in 1973. The second case describes 49-year-old woman operated for tumorous obliteration of the small pelvis where a tumor 9 cm in maximum diameter was found. Initially, predominant epitheloid morphology of the tumor led to the diagnosis of primary epithelial malignancy, sex-cord stromal tumor or metastatic melanoma of the ovary. Finally, the help of immunohistochemistry and molecular biology rendered the tumor as an extraintestinal GIST, ovarian primary not excluded.

[Pleomorphic epithelioid/clear cell malignant tumor of the uterus exhibiting both myoid and melanocytic differentiation--leiomyosarcoma or PEComa? A case report and a review of the literature]. / Pleomorfni epiteloidni a svetlobunecný maligní tumor delohy s myoidní a melanocytární diferenciací--leiomyosarkom nebo PECom? Kazuistika a prehled literatury.

30-year-old woman admitted for acute abdominal pain with peritoneal signs was immediately operated with findings of hemorrhage in small pelvis. Laparotomy revealed hemorrhagic, friable mass 3 cm in maximum diammeter arising from the uterine horn. The neoplasm broadly invaded myometrium with no evidence of endometrial cavity involvement. Microscopically, the tumor displayed solid mosaic pattern and consisted of large epithelioid cells with ample eosinophilic, finely granular cytoplasm ongoing apparent clear cell change elsewhere. There was marked nuclear irregularity with numerous atypical mitotic figures and multiple bizarre giant elements dispersed throughout the lesion. Unusual complex phenotype included co-expression of vimentin, smooth muscle actin, desmin, HMB45, Melan A, CD10 and EMA. No obvious stigmata of tuberous sclerosis were found and a five months follow-up after chemotherapy indicated no progression of disease. With some uncertainty the tumor was finally rendered as pleomorphic leiomyosarcoma with peculiar melanocytic differentiation. PEComa as an alternative term was coined. Expressed are both difficulty in explaining the histogenesis and ambiguity of the existing terminology of the uterine tumors with mixed myoid and melanocytic phenotype. Reviewed is the literature and discussed is the differential diagnosis.

[Warfarin-induced hemorrhagic pseudocyst in the pelvis of a woman with an inherited disorder of blood coagulation, complicated by pelvic bone pseudoxanthoma mimicking Erdheim-Chester disease]. / Warfarinem vyvolaná hemoragická pseudocysta malé pánve u zeny s vrozeným genetickým defektem koagulace komplikovaná usuracním pseudoxantomem pánevní kosti napodobujícím Erdheim-Chesterovu nemoc.

A fifty-year-old woman with developmental dysplasia of the hip underwent total hip arhtroplasty, and subsequently developed recurrent venous thrombophilia of the lower extremities. Hematological examination revealed an inherited disorder of blood coagulation (homozygous mutation of the 5,10-methylenetetrahydrofolate reductase gene) and therefore longterm Warfarin anticoagulation therapy was started. A year later she was diagnosed with a large pelvic posthemorrhagic pseudocyst (hematoma) located below the musculus iliacus and adhering to bone in the region of posterior acetabulum. The condition was complicated by usuration and focal osteolysis of the adjacent pelvic bone. Histological examination of the hematoma showed characteristics of an unusual pseudoxanthoma mimicking Erdheim-Chester disease. The differential diagnosis of histological findings is discussed and recent relevant literature is reviewed.

[Basal-like carcinoma of the breast--the actual review and clinico-pathological corelations]. / Basal-like karcinom prsu--aktuální prehled problematiky a klinicko-patologické korelace.

OBJECTIVE: Comprehensive review of current topic called basal-like carcinoma of the breast, focused on morphology, molecular biology and clinico-pathological aspects concerning biology, prognosis and rational therapy. DESIGN: Review article. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: Summarized are recent data, diagnostic principals and clinico-pathological recommendations of basal-like carcinoma and outlined is comprehensive guide for daily diagnostic and therapeutic practice. RESULTS: Basal-like carcinoma of the breast is a subset of tripple negative lesions (ER-/PR-/Her2-) and accounts for approximately 5-7% all ductal carcinomas. This is a highly aggressive tumor characterized by rapid systemic dissemination, especially to the lung and brain. By definition, there is expression of high molecular weight cytokeratins and in up to 50% of cases alteration of EGFR gene is found, which creates potential for targeted therapy with tyrosin kinase inhibitors. Basic morphology derives from medullary carcinoma with sharp margin from adjacent parenchyma and presents archetype of BRCA1 mutated familial cancer. Existence of the rare noninvasive in situ phase of the basal-like carcinoma having similar phenotype enables both early and reliable diagnosis even from small core biopsy specimens. Correct diagnosis of this relentless tumor not only brings the chance for effective monitoring specific organs apt to potential metastasis but also challenges for implementation of alternative therapeutic approach, because standard protocols usually fail.

[Benign mesenchymal stromal tumor of the breast simulating benign schwannoma in an 81-year-old male--a case report]. / Benigní vretenobunecný stromální tumor prsu podobný schwannomu u 81letého muze-- kazuistika.

Presented is an unusual case of a benign mesenchymal stromal tumor of the breast in an 81-year-old male. The basic appearance of the lesion simulated benign schwannoma and was misinterpreted as a low-grade myxoid liposarcoma initially. Well-circumscribed, gray-white mass measuring 35 mm in maximum diameter was discovered deep in the parenchyma of the completely removed breast. Microscopically, the lesion consisted of myxoid, richly vascular background where dominated oval or spindle cells with impressive palisading replicating that of benign schwannoma. Rarely, the large multinucleated (floret-like type) cells were visible; no nuclear atypia or mitotic figures were found. Immunohistochemical examination confirmed expression of estrogen and progesterone receptors, antigen Bcl2 and also focal desmin positivity. Clinical examinations disclosed no objective reason for possible hyperestrinism; no other therapy followed and the patient is free of disease 19 months after operation. On the background of both detailed review and differential diagnosis of benign, so-called stromal tumor of the female breast, the rarity of this microscopic finding in male is documented.

[Massive breast angiomatosis with dramatic course leading to acute total mastectomy--a case report]. / Masivní angiomatóza prsu s dramatickým prubehem vedoucím k akutní totální mastektomii--kazuistika.

OBJECTIVE: On the background of clinical, radiological and morphologic settings presented is unusual case of the rare angiomatosis of the female breast. Discussed are both clinicopathological aspects and differential diagnosis from the morphological point of view. DESIGN: Case report. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: As a case report described is a rare case of the angiomatosis of the female breast. Implemented illustrations consist of local findings, CT scan, specimen gross appearance and histological pictures including immunohistochemistry. Applied antibodies and detection system LSAB+ were provided by Dako, visualization was performed by chromogen DAB (diaminobenzidin tetrahydrochlorid). RESULTS: 30-year-old woman suffering of painful, enormous breast asymmetry underwent core needle biopsy of the right breast. The mastogram showed diffuse process of ambiguous significance. During the next few days her condition dramatically changed and under the circumstances of florid apoplexy and suspicion of tumor of her right breast, total mastectomy was carried out. Histological picture revealed widespread, unlimited vascular lesion dissecting throughout the stroma of the entire gland. Bland morphology and the nature of tissue involvement eventually led to diagnosis of benign angiomatosis. The woman underwent several local plastic operations but is out of final reconstruction of her breast so far. Thirteen months after mastectomy there are no signs of recurrence.

[Harmatoma of the breast--case report]. / Hamartom prsu--kazuistika.

OBJECTIVE: To describe rare hamartoma of the breast in a 31-year-old female. DESIGN: Case report. SETTINGS: Department of Special Diagnostics SPAU, Charles University Hospital Pilsen. SUBJECT AND METHOD: A 31-year-old female was examined for breast asymmetry. A huge tumor was revealed in right mammary gland using ultrasonography. Encapsulated tumor 10x10x10 cm was removed. Subsequent histological examination revealed breast hamartoma. The patient is alive and well without signs of disease one year after excision. CONCLUSION: Hamartoma of the breast is a rare benign tumor. Diagnostics is very complicated and it is almost impossible to establish correct diagnosis preoperatively. A simple excision is a sufficient treatment with good curative effect.

[Isolated lymphadenopathy as the first presentation of systemic mastocytosis--description of two cases]. / Izolovaná lymfadenopatie jako první projev systémové mastocytózy--popis dvou prípadu.

Presented are two cases of systemic mastocytosis in 46- and 63-year-old women, where the correct diagnosis was established in randomly disclosed cervical respectively intraabdominal lymphadenopathy. Both cases lacked characteristic skin and systemic mast-cell mediator symptoms at the time of histologic diagnosis. The first case was classified as a indolent systemic mastocytosis without any proven genetic alteration, the second one met the criteria of aggressive systemic mastocytosis with eosinophilia, where the point mutation asp816val in c-kit gene was confirmed and the patient responded unexpectedly well to Gleevec. Discussed are both conventional morphological differential diagnosis of mastocytosis in lymph nodes and recent advances in genetics of these systemic clonal mast cell proliferations. The latter not only outlines the oncopathogenesis but, in particular, also provides important prognostic and biological implications of this peculiar disease.

[Intraductal papillary proliferation of brest. Actual review and clinicopathological corelation]. / Intraduktální papilární proliferace prsu. Aktuální prehled problematiky a klinicko-patologické korelace.

OBJECTIVE: Complex summary addressing current view of the intraductal papillary lesions of breast, especially focused on morphology, exact terminology and clinicopathological aspects of effective therapy. DESIGN: Review article. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: Summarized recent data, diagnostic principals and clinicopathological recommendations of papillary breast lesions created comprehensive guide for daily diagnostic and therapeutic practice. RESULTS: The group of papillary tumors of the breast includes various lesions with different biologic potential. They are all presenting as pure intraductal lesions, but in case of multiple peripheral distribution imply possible risk for aggressive behavior. The reason is frequent occurrence of atypical ductal hyperplasia/intraductal (in situ) carcinoma in the adjacent tissue. Spectrum of secondary changes further complicates the diagnosis of papillary tumors, especially in core needle biopsy. Atypical papilloma is still very controversial issue and it essentially means situation when atypical ductal hyperplasia/conventional ductal in situ carcinoma arises in preexisting papillomas or extends to it from neighborhood. Generally, papillary lesions are suitable for effective conservative treatment in the case of proper clear resection margin. Finding of benign papilloma in core biopsy remains unsolved question yet.

[Metastasing and relapsing "low grade" adenosquamous metaplastic breast cancer--is there a really indolent lesion? A description of three cases and review of literature]. / Metastazující a recidivující "low grade" adenoskvamózní metaplastický karcinom prsu--jde skutecne o indolentní lezi? Popis trí prípadu a rozbor literatury.

OBJECTIVE: To describe personnel experience with three unusual cases of low-grade adenosquamous carcinoma of the breast. Due to the aggressive course in two cases, the review of the literature does not concentrate on morphology and differential diagnosis only, but discuss overall biology of this lesion too. DESIGN: Three case reports. SETTING: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS: Included are cases from mammary register of Biopsy Lab s.r.o. and from personnel consulting registry of Prof. Michal. In details are documented both clinical presentations, including follow up, and spectrum of histologic patterns of the lesion. Immunohistochemistry performed by streptavidin-biotin system (LSAB+, Dako) as detection system and diaminobenzidin tetrahydrochlorid as chromogen, employed standard myoepitelial markers (actin, CD10, calponin and p63), hormonal receptors, oncogen Her-2/neu, CK7, CAM5.2, CD34 and CD117. RESULTS: Partial mastectomy and segmentectomy were performed in three women 46, 72 and 74 years-old resp. for tumor, which size ranged from 20-35 mm in maximum diameter (mean 28 mm). Histology revealed low-grade adenosquamous metaplastic carcinoma displaying typical biphasic appearance combining regular tubular structures with surrounding storiform, sclerosing fibrous stroma. Variably present was characteristic squamous metaplasia of the gland forming either luminal morules or evident squamous pearls. Using immunohistochemistry, a total absence of myoepithelial layer in epithelial structures was confirmed. The stromal component expressed smooth muscle actin in two cases, one of which contained areas of heterologous chondrosseal differentiation. There were recognized metastases by one woman in two ipsilateral axillary lymph nodes mimicking benign breast heterotopia in one of them. One patient experienced recurrence in the scar 37 months after mastectomy where only mesenchymal heterologous component element was found. In two women with aggressive course the original biopsy was falsely interpreted, once as phyllodes tumor and secondly as benign sclerosing pseudotumor. CONCLUSION: low-grade adenosquamous carcinoma, despite its bland histology, should be understood as regular malignancy prone to frequent recurrences and recognized metastatic potential. It arises in the deep breast tissue and structurally resembles the microcystic adnexal carcinoma of the skin. Low-grade adenosquamous carcinoma, however, has nothing to do with syringomatous adenoma of the nipple, which is a benign tumor of the skin adnexa. Differential diagnosis includes spectrum of non-neoplastic slerosing lesions and above-mentioned phylloid tumor. The rarity of this neoplasm does not exclude deep knowledge.

Low mutational rate of K-ras codon 12 in singular bronchoscopy specimens in suspected lung cancer.

Mutations of the K-ras gene are found in a subset of non-small- cell lung carcinomas (NSCLC). The aim of our study was to determine the K-ras codon 12 mutation in the first, singular bronchoscopy specimen in parallel with the cytological examination for the diagnosis of lung cancer. Samples were obtained by diagnostic bronchoscopy in 140 patients with suspected lung tumors. The analysis of K-ras mutations was carried out by a sensitive two-step mutation- enriched polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) assay. This method has been confirmed earlier to be positive for mutated tumor cells and negative for normal lung parenchyma and bronchus. Of the 140 patients with suspected cancer, 93 were diagnosed as NSCLC by cytology or histology in either the same specimen used for the detection of K-ras mutation or in later biopsies. However, only four K-ras codon 12 mutations were detected in the first bronchoscopic material: one in adenocarcinoma, two in squamous cell tumors, and one mutation was found in a patient with dysplasia which was diagnosed later as a squamous cell carcinoma. Our findings indicate that although the K-ras (codon 12) mutation is a gene lesion infrequently detectable in a singular specimen taken at the first bronchoscopy examination in cases of clinically suspected lung cancer, the detection of this mutation can help to confirm the cytological diagnosis of NSCLC or may be even diagnostic in cytologically negative cases.

[Acinic cell-like differentiation in invasive ductal carcinoma and in ductal hyperplasia of the breast--report of two cases]. / "Acinic cell-like" diferenciace v invazivním duktálním karcinomu a duktální hyperlazii mlécné zlázy--popis dvou prípadu.

Described are two epithelial lesions of the breast displaying extremely rare, widespread acinic cell-like differentiation (metaplasia). Two women, 70 and 40-year-old, one with invasive ductal papillocarcinoma, the other one with conventional intraductal hyperplasia without atypia, both demonstrated massive diffuse, PAS positive, granular eosinophilic transformation of the cell cytoplasm. This unusual cell appearance closely simulated acinar cells in normal serous salivary gland/acinic cell carcinoma or Paneth cells. Both extensive expression of lysozyme and finding of numerous zymogen granules ultrastructurally confirmed the acinic cell-like fenotype. Discussed is differential diagnosis of the breast neoplasm containing overt eosinophilic and granular cytoplasm. Reviewing literature and comparing our unique finding of unusual salivary-type differentiation in conventional ductal hyperplasia of the breast, biologic implications are considered.

[Syndrome of pseudomyxoma peritonei--description of three cases and survey of the problem]. / Syndrom pseudomyxomu peritonea--popis trí prípadu a prehled problematiky.

OBJECTIVE: To describe personal experience with three heterogeneous cases of pseudomyxoma peritonei. Review of the literature concentrates on the pathogenesis and biologic spectrum of the rare syndrome. DESIGN: Three case reports. SETTING: Private Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS AND RESULTS: Included are cases from Biopsy Lab s.r.o. and from personal consulting registry of Prof. Michal, Sikl's Department of Pathology, Pilsen. Clinical presentation of three cases of pseudomyxoma peritonei documented is in details including long follow up. Broad variability of histologic appearance associated with different Clinical outcome is demonstrated. Immunohistochemistry performed by streptavidin-biotin system (LSAB+, Dako) as detection system, and diaminobenzidine tetrahydrochloride as chromogen, employed three monoclonal antibodies--MUC2 (Novocastra, 1:400), cytokeratin 7 (Dako, 1:200) and cytokeratin 20 (Dako, 1:100). CONCLUSION: Pseudomyxoma peritonei is a clinical syndrome defined as presence of massive mucinous, viscous material in the peritoneal cavity, both floating and adhering to serosal surface (jelly-belly). The histologic examination should always follow with detailed and precise description of the epithelial component. The cytological and structural quality of the epithelium constitutes two basic forms with entirely different nature biology and prognosis. First and more frequent, so-called disseminated peritoneal adenomucinosis, where primary low grade (benign) mucinous appendiceal tumor is almost constant finding, often recurs but displays favorable prognosis. Second, so-called peritoneal mucinous carcinomatosis is an extraordinary manifestation of peritoneal carcinosis following generalization of the gastrointestinal mucinous adenocarcinoma. Histopathogenesis in both types is thought to arise from MUC2 positive goblet cells in GI and ovarian involvement is secondary. A normal macroscopic finding on appendix or "uneventful" appendectomy in anamnesis is not unusual.

[Cystic hypersecretory carcinoma: rare and potentially aggressive variant of intraductal carcinoma of the breast. Report of five cases]. / Cystický hypersekretorický karcinom: vzácná a potenciálne agresivní varianta intraduktálního karcinomu prsu.

OBJECTIVE: To describe a poorly recognized histological and immunohistochemical features of cystic hypersecretory carcinoma of breast, including the overexpression of HER-2/neu protein and androgen receptors. SUBJECT: Review of clinical and pathological features of 5 cases of cystic hypersecretory carcinoma. SETTING: Private Bioptic Lab, Plzen and Department of Pathology, Charles University, Plzen. SUBJECT AND METHODS: Five cases of rare variant of intraductal carcinoma of the breast, so-called cystic hypersecretory carcinoma, are reported. The patients were females aged between 53 and 78 years (average 66.8 years). The size of the lesions ranged between 7 and 8 cm in largest dimension. RESULTS: In two cases, the development of high grade invasive ductal carcinoma was observed; in one additional case there was recurrence of high grade in situ carcinoma after three years. Strong overexpression of HER-2/neu protein was observed in three cases including those two with invasive component. Protein p53 was variably positive in all cases. Steroid receptor immunohistochemical study yielded variable results with only one case being positive for both estrogen and progesterone receptors. Interestingly, in most case (4/5) staining for androgen receptors was observed. CONCLUSION: The importance of correct diagnosis of this potentially aggressive lesion is emphasized. The differential diagnosis of breast lesions with predominantly cystic growth pattern is discussed in detail.

Cystic hypersecretory carcinoma: rare and poorly recognized variant of intraductal carcinoma of the breast. Report of five cases.

AIMS: To report five cases of a rare variant of intraductal carcinoma of the breast, so-called cystic hypersecretory carcinoma. The clinical and pathological characteristics of the lesion are described, along with a review of the literature. METHODS AND RESULTS: The patients were females aged between 53 and 78 years (average 66.8 years). The size of the lesions ranged between 70 and 80 mm in largest dimension. In two cases, the development of high-grade invasive ductal carcinoma was observed; in one additional case there was recurrence of high-grade in-situ carcinoma after 3 years. This emphasizes the importance of correct diagnosis of this potentially aggressive lesion. Strong over-expression of HER-2/neu protein was observed in three cases, including the two with an invasive component. Protein p53 was variably positive in all cases. Steroid receptor immunohistochemistry yielded variable results with only one case being positive for both oestrogen and progesterone receptors. Interestingly, in most cases (4/5) staining for androgen receptors was observed. CONCLUSIONS: Cystic hypersecretory ductal carcinoma of the breast is a rare distinctive variant of ductal carcinoma in situ. It has the potential for invasive growth and the development of metastases.