[Interdigitating dendritic cell sarcoma of lower extremities resistant to high dose chemotherapy BEAM with peripheral blood stem cell transplantation]. / Sarkom z interdigitujících dendritických bunek dolní koncetiny rezistentní k vysokodávkované chemoterapii BEAM s autologní transplantací kmenových krvetvorných bunek--popis prípadu a prehled literatury.

Interdigitating dendritic cell sarcoma is a rare neoplasm forming part of the group of malignancies derived from histocytic cell line. This nosological unit can be detected only by special immunohistochemical exams. A young man aged 25 found a tumorous swelling in the proximal part of his left crus. The pathological process affected proximal tibial epiphysis and adjacent soft tissues. The first FDG-PET examination performed in the process of determining the clinical stage of the disease showed a high activity in the site of primary tumour (SUV 7.71) and in the site of regional inguinal node (SUV 4.25). Histological examination of a diagnostic excision specimen of the tumour in the tibia and the extirpated enlarged regional nodes in the left groin led to the diagnosis of interdigitating dendritic cell sarcoma. The diagnosis was confirmed pathologically by another two centres in the Czech Republic and, due to the unusual nature of the diagnosis, also in Regensburg, Germany. Treatment started with chemotherapy, applied to patients with aggressive lymphomas in the framework of clinical studies, i.e. a combination of MegaCHOP. After 4 cycles, however, there was no visible response on the site of primary tumour. MegaCHOP therapy was therefore discontinued after the 4 cycles. Subsequently, we referred the patient for a high-dose chemotherapy with autologous bone marrow transplantation, similarly to aggressive lymphomas. The collection of blood producing stem cells from peripheral blood was successfully performed after ESHAP chemotherapy. A verificatoin FDG-PET examination was performed before high-dose chemotherapy. Increased activity was detected only in left proximal crus, with an SUV of 4.6. One month after ESHAP chemotherapy, BEAM high-dose chemotherapy with autologous transplantation of blood forming tissue was performed. High-dose chemotherapy was followed up by radiotherapy targeted on the primary tumour in the crus (70 Gy). The third verification FDG-PET examination was performed 3 months after radiotherapy. The examination showed a continuing higher activity in the region of the primary tumour (SUV 2.69) and a new centre of activity was detected in the left inguinal nodes region (SUV4.09). The activity corresponded to the presence of viable tumour tissue in the primary nidus and new metastases in inguinal nodes, without proofs of further proliferation at the time. Nodes of the left groin were removed. Histological examination showed affection of the node by the same type of tumour, i.e. a continuing activity of the disease despite chemotherapy. Due to suspected continuation of viable tumour in the crus judging by the intensity of accumulation of FDG-PET and the proof of a new affection of regional nodes, surgical treatment was preferred after the failure of chemotherapy. After the removal of inguinal nodes, left knee joint exarticulation was performed. This was followed by regional inguinal node region radiotherapy (56 Gy). The last fourth PET-CT examination carried out 4 months after the radiation therapy of the inguinal region showed massive dissemination into the region ofileac and paraaortic nodes (lymphadenopathy up to 6 cm in diameter) with an activity of 5.9 to 6.73 SUV units. Currently, we test the sensitiveness of the disease to 2-chlordeoxyadenosin and look for additional therapeutic options. To our knowledge, the above description is the first documented case of interdigitating dendritic cell sarcoma located in the tibia and crus soft tissue. We have not found any description of high-dose therapy supported by autologous transplantation of blood-forming tissue for this type of tumour in relevant literature. In this case, we record chemoresistance to high-dose chemotherapy and certain radiosensitivty of the tumour at the same time.

[Pseudolymphoma of the breast nipple. The problem overview]. / Pseudolymfom prsní bradavky. prehled problematiky.

Clinical, pathological, and molecular-genetic features as well as etiology of cutaneous pseudolymphoma (CPL, cutaneous lymphoid hyperplasia, lymphocytoma cutis) of the breast nipple are summarized. CPL presents as a nipple induration and it is often suspected to be Paget carcinoma pre-operatively. Histologically, atypical microscopic features of a dense lymhoid infiltrate with follicles often mislead to the diagnosis of a malignant lymphoma. However, CPL runs a benign course. Rare cases of CPL contain a clonal lymphoid population. A substantial number of CPL in the breast nipple is caused by antigenic stimulation by Borrelia burgdorferi. In some patients a tick bite is documented. CPL of the breast is commonly treated by excision, but some patients may be cured by antibiotic therapy. The presence of Borrelia burgdorferi should be detected using methods of serology, culture, and molecular biology. Beside CPL, the differential diagnosis of the breast nipple lesions further includes Paget carcinoma, eczema, and florid papillomatosis.

Carcinoma arising in ectopic hamartomatous thymoma. An ultrastructural study.

We present the ultrastructural features of a case of adenocarcinoma arising in ectopic hamartomatous thymoma in a 31 year old man, in the supraclavicular location. The tumor had a cribriform adenomatous component with a granular cell quality and mimicked a metastatic carcinoma. Ultrastructurally, the spindle cell component revealed clusters of tonofilaments and the tumor cells were attached by well developed desmosomes. The cytoplasm of adenocarcinoma cells contained peculiar cup-shaped bodies derived from the endoplasmic reticulum wrapped around the mitochondria.

Phyllodes tumour of the breast: immunohistochemical study of 37 tumours using MIB1 antibody.

In our study, the expression of Ki-67 antigen in phyllodes tumours of the breast was investigated in order to estimate its significance in assessing the grade of malignancy and in predicting the clinical behaviour of these tumours. Formalin-fixed and paraffin-embedded tissue specimens of 37 cases of phyllodes tumour of the breast (eight graded as malignant and 29 as benign according to histopathological criteria) and 20 breast fibroadenomas were stained by an immunoperoxidase technique, using the monoclonal antibody MIB1 directed against cell proliferation-associated Ki-67 antigen. A MIB1 index was calculated to express the percentage of MIB1 positive proliferating stromal cells. Our data indicate that there are statistically significant differences in MIB1 indices between histologically benign and malignant phyllodes tumours (alpha = 0.001), between benign phyllodes tumour and fibroadenoma (alpha = 0.01), and between malignant phyllodes tumour and fibroadenoma (alpha = 0.001). Our results also indicate a good correlation between the conventional grading of phyllodes tumours based on histological criteria and MIB1 indices. However, little predictive value of the MIB1 index in phyllodes tumours of the breast can be derived from our study.

Oncocytic cystadenoma of the parotid gland with tyrosine-rich crystals.

A case of benign oncocytic cystadenoma with abundant intraluminal tyrosine-rich crystals involving the parotid gland is described.

Protein histochemistry methods for colloid identification to metastases of thyroid carcinoma.

Evidence for a thyroid origin of tumorous metastases may be facilitated by a histochemical determination of colloid. It is supported by the presence of proteins, demonstrable especially by the reaction for tyrosine, and a simultaneous absence, or only traces of acid mucosubstances. Contrary to existing literary reports, an intraplasmatic formation of mucin has been detected in certain thyroid carcinomas and their metastases. The most difficult to diferentiate histochemically from thyroid colloid is the secrete of certain adenocarcinomas of the prostate.

Pleiomorphous histiocytic sarcoma arising in a patient with histiocytosis X.

An adult woman was followed up for the occurrence of multiple successive cutaneous and mucosal tumors of small size and classical histiocytosis X structure (histiocytoeosinophilic granuloma). In the second phase of the patient's clinical history there were successively appearing nodular or infiltrative cutaneous and subcutaneous tumors histologically resembling polymorphocellular sarcoma close either to malignant lymphogranuloma or to recently described "regressing atypical histiocytosis". Despite the marked cellular atypia and polymorphism the tumors exhibited a relatively favorable clinical course. Beside clearly sarcomatous structures, some of them displayed also transitional structures to histiocytosis X. Immunohistologically the atypical cells showed features of both the Langerhans' line and non-specific histiocytes. Ia-like antigen was positive in most of the elements. Quite occasional cells contained detectable amount of S-100 protein. Electronmicroscopy revealed quite sporadic structures resembling Langerhans granules.

Cytoplasmic liver cell inclusions--a typical feature of porphyria cutanea tarda--are absent in porphyria-related hepatic neoplasias.

Crystalline cytoplasmic needle-shaped inclusions in hepatocytes are considered to represent a specific morphological feature of porphyria cutanea tarda (PCT) and experimental PCT-like porphyrias. The cytoplasmic inclusions, however, are absent in hyperplastic hepatic nodules and hepatocellular carcinomas arising in the course of these conditions. It is assumed that porphyrins and related substances accumulated in hyperplastic and neoplastic hepatic lesions differ from those found in non-neoplastic liver tissue: the highly carboxylated porphyrins are stored in both sites, the crystal-forming substance only in non-proliferating liver tissue.

Histochemical and biochemical observations of the spleen in atypical Niemann-Pick disease and in idiopathic thrombocytopenic purpura.

In a case of adolescent Niemann-Pick disease (NP) and in a case of idiopathic thrombocytopenic purpura (ITP), the histologic picture of the spleen showed appreciable similarity in localization of sparing cells and in a number of histochemical tests. The sphingomyelin, which was the main organ phospholipid in both conditions, contained substantially elevated content of C24 fatty acids. Detailed analysis of spleen lipids showed great relative increase of lysobisphosphatidic acid and of cholesterol which was in NP mainly in free form but in ITP surprisingly mainly esterified, mostly to oleic and palmitic acid. Possible molecular mechanism of sphingomyelin storage was enzymologically followed in model conditions using separated lipid fractions from NP's spleen. The activity of sphingomyelinase (Cl. perfringens exotoxin) was in comparison to phospholipase C relatively specifically inhibited by lysobisphosphatidic acid.

Diagnostic value of PB/KOH/PAS method for identification of primary cancer from metastases.

The demonstration of 0-acylated sialic acids in the mucin of cancer metastases by Culling's periodic acid-borohydride-potassium hydroxide-periodic acid-Schiff method (PB/KOH/PAS) is helpful in distinguishing between mucin-producing primary colorectal adenocarcinoma (which will be in 60 to 70% positive) and mucin-producing primary lung adenocarcinoma (which will be negative). In addition to colorectal mucin, the 0-acylated sialic acids may be demonstrated in the mucin of some gastric and gallbladder carcinomas, and only exceptionally in pancreatic, ovarian, and prostatic cancers.

Liver cell cytoplasmic inclusions in experimental porphyrias: their demonstration with the ferric ferricyanide reduction reaction.

In the present paper we describe needle-shaped and granular cytoplasmic inclusions in the liver cells of mice and rats with experimental porphyria biochemically resembling human porphyria cutanea tarda. The inclusions were inconspicuous in routine histological slides. The ferric ferricyanide reduction reaction, however, enabled us to demonstrate their shape and location within the hepatic lobule. Needle-shaped inclusions are considered to represent a structure specifically seen in experimental porphyrias resembling porphyria cutanea tarda. These structures are similar to the inclusions seen in human porphyria cutanea tarda.

Liver preneoplastic changes in mice treated with the herbicide fomesafen.

1. Effect of the diphenyl ether herbicide fomesafen on liver preneoplastic changes and porphyrin biosynthesis was examined in male C57BL/6J mice (0.23% in the diet for 14 months) and ICR mice (0.3% in the diet for 50 weeks). Fomesafen treatment resulted in preneoplastic changes (liver nodules and foci of altered hepatocytes) in both strains, uroporphyria developed only in ICR mice. 2. Iron pretreatment (600 mg/kg as a single dose) accelerated the development of fomesafen-induced preneoplastic changes in both mouse strains. The number of foci containing altered hepatocytes, as well as the number and size of liver nodules, were increased in iron-pretreated animals. 3. A single injection of iron induced marked uroporphyria in C57BL/6J mice after 14 months (liver porphyrin content 102 nmol/g). This uroporphyria was further potentiated by fomesafen administration (208 nmol/g). 4. In ICR mice, liver histology was apparently normal after a 3 month recovery from fomesafen treatment (0.32% for 9 months). Liver porphyrin content (260 nmol/g) started to decrease immediately after fomesafen withdrawal, but was still significantly elevated after 3 months (5 nmol/g), as compared to controls (1 nmol/g). 5. It is concluded that the toxicological evaluation of fomesafen should focus on liver porphyrin biosynthesis.

[Primary cutaneous B-cell lymphoma: present views]. / Primární lymfomy kuze z b-bunek: soucasný pohled.

Most lymphoproliferative B cell cutaneous lesions do represent primary lymphomas, not a secondary lymphomatous spread or pseudolymphoma* A description of the development of classification systems for primary cutaneous B lymphomas is given concerning especially some newly defined lesions: primary cutaneous marginal zone B cell lymphoma, primary cutaneous follicular centre cell lymphoma and large B cell lymphoma of the legs. Majority of primary cutaneous B lymphomas answer well the treatment and have a favourable prognosis.

[Consultation (second opinion on biopsies) as an instrument of quality control in histopathologic diagnosis]. / Konzultacní cinnost (druhé ctení biopsií) jako nástroj kontroly kvality histopatologické diagnostiky.

As the whole spectrum of morphological patterns encountered in surgical pathology cannot be, in its entirety, diagnosed correctly by a general pathologist alone, one has to seek consultation for difficult or unusual cases with specialised experts. In the Czech Republic, the consultation practice performed by specialists from different medical institutions is the only way to assess the quality of histopathological diagnostic performance, i.e. it represents an audit system. Audit systems functioning in other countries are described here. A proposal of a standard consultation process as well as problems concerning the financial costs and increased workload of consultants are discussed.

[Inflammatory fibrous histiocytoma]. / Inflamatorní fibrózní histiocytom.

A busky tumour of yellow colour with pus-filled central cavities was removed from the left retroperitoneum of a 54 year old man. Histology revealed it to be a rare variant of fibrous histiocytoma characterized by an extremely large quantity of mature neutrophil granulocytes in all the tumour tissue messively phagocyted by tumour histiocytes. There was no relapse or dissemination when postmortem was performed 5 months after the operation.

[Myelosarcoma as the 1st manifestation of myelosis]. / Myelosarkom jako první projev myelózy.

Myelosarcoma of the nodes, middle ear, rib, and spinal epidural space was observed in five cases as preceding the diagnosis of myelosis. In two cases involving epidural localization malignant lymphoma was misdiagnosed and reclassification only at the time of postmortem. Myelosarcoma is best distinguished from other types of tumour by means of the histochemical proof of naphthol-AS-D-chloracetatesterase.

[The significance of enzyme cytochemical methods in the classification of hemoblastoses]. / Význam enzymových cytoc-emických metod v klasifikaci hemoblastóz.

Selected cytoenzymological methods together with the PAS reaction often permit more precise identifying normal and tumorous blood cells and thus determining the type of hemoblastosis. The cells of the myeloid neutrophil series are characterized by the activity of peroxidase and naphtol-AS-D-chloracetate esterase, monocytic elements by the activity of a nonspecific esterase sensitive to fluoride, the tumorous cells of the red series by the PAS positivity and paranuclear positivity of acid phosphatase. Some tumorous lymphoblasts have gross PAS--positive granules in the plasma, others, probably of T-origin, paranuclear focal positivity of acid phosphatase. Hematological classification of acute hemoblastoses does not exactly correspond to Löffler's cytochemical classification; of diagnostic and therapeutically considerable importance is the synthetic evaluation of the hematological finding and of interpretive possibilities of individual cytochemical methods.

[Anaplastic Ki-1/CD30 positive lymphoma]. / Anaplastický Ki-1/CD30 pozitivní lymfom.

Ki-1(CD30) positive lymphoma in its large cell variant represents a distinct histopathological entity. Its cells have a characteristic morphology, immunophenotype and karyotype. In the beginning the tumour infiltrates sinuses of the lymph node and it can be difficult to distinguish it from a metastatic carcinoma or melanoma. We present here six cases of this peculiar entity.

[Myelomonocytic chloroleukemia. Histochemical evaluation of two observations]. / Myelomonocytární chloroleukémie. Histochemické hodnocení dvou pozorování.

A myelosarcoma was diagnosed in the bioptic excision of an eight year old boy's middle ear. An autopsy of this case as well as that of a 47 year old man revealed the presence of tumoriform chloroleukaemia. The histochemical positivity of naphthol AS-D-chloracetate-esterase and NaF-sensitive non-specific esterase showed the simultaneous double differentiation of tumour cells thus serving as evidence of their myelomonocytic nature. The first observation also revealed the presence of atypical eosinophil leucocytes with PAS-positive, naftol AS-D-chloracetate-esterase-negative granules. Both observations brought evidence of the reticular as well as tumour cells showing signs of erythrocytophagia which may have a share in the development of the tumour tissue green coloration.

[Chronic granulomatosis in childhood]. / Chronická granulomatóza detskéhoveku.

In a boy aged 8 years suffering from chronic granulomatosis of childhood necrotizing pneumonie and small pulmonary tuberculoid granulomas containing filaments of moulds were found at autopsy. Necrotizing leucocytic granulomas were present in the liver, spleen and the lymph nodes. All the organs showed aggregates of histiocytes containing yellowish cytoplasmic deposits of lipogment surrounded by a high acid phosphatase activity. The NBT-reduction leucocytic tests was repeatedly negative in vivo. The activities of NADH- and NADPH-tetrazolium reductase and succinate dehydrogenase in the tissues were histochemically normal.