[Diagnostic difficulties in diagnosis of Hodgkin's disease in a young man--a case report]. / Trudnosci diagnostyczne w rozpoznaniu ziarnicy zlosliwej sródpiersia u mlodego mezczyzny--opis przypadku.

UNLABELLED: Lymphomas are neoplasms of lymphocytes and their precursor cells. This disease develops from lymph nodes or extranodal lymphoid tissue. A common site for such a tumour is the chest. The authors describe the case of a young man, who was admitted do the thoracic surgery department with a mediastinal tumour and an induration of the base of the neck. Initial diagnostic tests gave the wrong diagnosis of actinomycosis. A lack of progress in the therapy and the occurrence of a life-threatening condition led to the implementation of more aggressive diagnostic methods. In biopsies taken during thoracotomy, the patient was finally diagnosed with Hodgkin's lymphoma of nodular sclerosis type. CONCLUSIONS: Mediastinal tumours may cause diagnostic difficulties and, to avoid mistakes, typical histological studies should be complemented by targeted immunohistochemical tests.

[Granulomatous pneumonia as a complication of intravesical BCG immunotherapy--a case report]. / Zapalenie ziarniniakowe pluc jako powiklanie leczenia dopecherzowymi wlewkami BCG--opis przypadku.

BCG (Bacillus Calmette-Guerin) comprises an attenuated strain of Mycobacterium bovis and is used for vaccination against tuberculosis. An additional use of BCG is for immunotherapy of cancer in which the vaccine is administered intravesically for the treatment of superficial bladder cancer. The efficacy of immunotherapy with BCG in the prevention of recurrence is estimated at 70-99%, which is higher than for local chemotherapy. The most frequent complications of such treatment include fever and urinary bladder inflammation, while serious complications of haematogenous organ inflammation, especially inflammation of the lungs with the formation of pulmonary caseosus granulomas, are rarely seen. The authors reported a case of a 68-year-old man who was treated with intravesical BCG instillations due to a superficial bladder cancer. The patient underwent transurethral resection of bladder cancer and then periodically received intravesical BCG instillations. A few days after one instillation, systemic symptoms with a high fever appeared. Further examinations showed features of hepatitis and spread pulmonary changes. The patient underwent videothoracoscopy, and a fragment of lung parenchyma was collected. The histopathological examination revealed the presence of granulomas with central caseosus necrosis. Suspecting BCG infection, diagnostics were enhanced to include bacteriological and genetic tests for the presence of acid-resistant bacilli, which finally gave negative results. The authors diagnosed granulomatous pneumonia as a complication of intravesical BCG immunotherapy. Treatment with antituberculous drugs was initiated. After completing pharmacological treatment, radiological control was performed, which showed significant but not complete remission of pulmonary changes.

[Nodular lymphoid hyperplasia - a rare case of lymphoproliferative disorder of the lungs]. / Guzkowy rozrost limfoidalny ­ rzadki przypadek chorobylimfoproliferacyjnej w plucach

Nodular lymphoid hyperplasia (NLH) belongs to a very rare, mild, lymphoproliferative disease of unestablished aetiology historically included in the group of pseudolymphomas. Its existence was controversial for many years, until modern techniques of pathomorphological diagnosis approved it as a separate entity of lung disease. It manifests in the form of well limited nodules localized in the lungs, which are mostly identified accidentally. Clinical symptoms are rare and nonspecific; the disease usually occupies only one lung. Pathomorphological diagnosis requires immunohistochemical designation of expressions of numerous antigens in order to exclude malignant lymphoma of the lungs. Surgical resection is used in cases of larger nodules; the smaller ones require periodic observation, and the prognosis is good. The authors describe the case of 65-year-old woman with pulmonary nodules which were detected accidentally in the right lung. The patient was qualified for right-sided videothoracoscopy and removal of the lung nodule. In classic HE staining of the histological material, the presence of lymphoid infiltration of the lungs was revealed, which formed lymph follicles with reactive germinal centres. In order to differentiate from the malignant lymphatic expansion, immunohistochemical designations were made, which showed positive expression of CD20 antigen in the B cell zone, positive expression of the CD3 antigen in the T cells zone, positive expression of CD23 antigen in the lymph follicles, negative expression of bcl-2 in the lymph follicles, and positive expression of MIB-1 in the germinal centres of lymph follicles. Such a histopathological and immunohistochemical picture provided the basis for diagnosis of nodular lymphoid hyperplasia of the lung.

CHEK2 mutations and HNPCC-related colorectal cancer.

Recently, the 1100delC variant of cell cycle checkpoint kinase 2 (CHEK2) has been reported to confer a colorectal cancer risk in hereditary non-polyposis-colorectal cancer (HNPCC) and HNPCC-related families in the Netherlands. To investigate whether CHEK2 mutations confer increased cancer risk in HNPCC and HNPCC-related families in Poland, we genotyped 463 probands from HNPCC and HNPCC-related families, and 5,496 controls for 4 CHEK2 alleles (1100delC, IVS2+1G>A, del5395, I157T). All 463 probands were screened for mutations in the HNPCC-related genes MSH2, MLH1 and MSH6. A positive association was observed for HNPCC-related cancer and the I157T missense CHEK2 mutation (OR = 1.7; p = 0.007), but not for the truncating alleles (OR = 1.0; p = 1.0). The association with the I157T was seen both for the 117 cases who fulfill Amsterdam criteria (OR = 1.9; p = 0.1) and for the 346 cases who do not fulfill the criteria (OR = 1.6; p = 0.03). One hundred forty-five of the 463 families had a mutation in MSH2, MLH1 or MSH6 (MMR-positive families). A positive association between the CHEK2 I157T mutation and HNPCC-related cancer was observed only for MMR-negative cases (OR = 2.1; p = 0.0004), but not for MMR-positive cases (OR = 0.8; p = 0.9). The association with I157T was particularly strong for MMR-negative cases with familial colorectal cancer (2 or more first-degree relatives affected) (OR = 2.5; p < 0.0001). We conclude that the I157T variant of CHEK2 increases the risk of colorectal cancer among MMR-negative, HNPCC/HNPCC-related families in Poland.

[Spontaneous remission of Kikuchi-Fujimoto disease (lymphadenopathy) with focal skin parakeratosis]. / Samoistna remisja choroby (limfadenopatii) Kikuchi-Fujimoto z ogniskowa parakeratoza skórna.

Kikuchi-Fujimoto disease is subacute, necrotizing lymphadenopathy affecting mainly young women, and manifested by cervical lymphadenopathy. It is often observed, and described in Asia, but in other world regions sporadically too. Etiology is unknown, but it has been postulated that this condition is induced by infectious factor. We described a case of 20-year-old Caucasian woman with diagnosis of Kikuchi-Fujimoto lymphadenopathy. First symptoms as a cervical lymph nodes enlargement 2 cm in diameter was associated with elevated body temperature, without associated symptoms of infection appeared about one year ago (data from history). Lymphadenopathy and general symptoms receded after empiric therapy with amoxicilin. Relapse of cervical lymphadenopathy alongshore sternocleidomastoid muscles bilateral to about 2 cm in diameter with pseudo-flu symptoms like fever and joints and muscles pains. Focal hiperpigmentation of abdomen, arms and legs skin appeared. In histopathology of collected lymph node histiocytic necrotizing lymphadenopathy without neutrophils infiltration Kikuchi type was diagnosed. In peripheral blood morphology transitional leucopenia 2.58 x 10(9)/l with granulocytopenia 0.64 x 10(9)/l was noted. Anemia and thrombocytopenia did not be observed. In serology active CMV, EBV or toxoplasmosis were excluded. In immunology presence of eleveted levels of anti-Jo, anti-nuclear and anticardiolipin antibodies was excluded too. Serum protein electrophoresis and additional biochemical parameters was normal. In chest X-ray and abdomen ultrasonography abnormalities was not found. In skin and muscles biopsy specimens focal parakeratosis was found. Despite no empiric antiinfectious therapy after 3-4 weeks remission of general symptoms and lymphadenopathy was noted.

Distal intramural spread of rectal cancer after preoperative radiotherapy: the results of a multicenter randomized clinical study.

PURPOSE: To evaluate the extent of distal intramural spread (DIS) after preoperative radiotherapy for rectal cancer. METHODS AND MATERIALS: A total of 316 patients with T(3-4) primary resectable rectal cancer were randomized to receive either preoperative 5x5 Gy radiation with immediate surgery or chemoradiation (50.4 Gy, 1.8 Gy per fraction plus boluses of 5-fluorouracil and leucovorin) with delayed surgery. The slides of the 106 patients who received short-course radiation and of the 86 who received chemoradiation were available for central microscopic evaluation of DIS. RESULTS: The length of DIS did not differ significantly (p = 0.64) between the short-course group and the chemoradiation group and was 0 in 47% vs. 49%; 1 to 5 mm in 41% vs. 42%; 6 to 10 mm in 8% vs. 9%, and greater than 10 mm in 4% vs. 0, respectively. Among the 11 clinically complete responders, DIS was found 1 to 5 mm from the microscopically detected ulceration of the mucosa in 5 patients. The discontinuous DIS was more frequent in the chemoradiation group as compared with the short-course group (i.e., 57% vs. 16% of cases, p < 0.001). CONCLUSIONS: Approximately 1 out of 10 advanced rectal cancers after preoperative radiotherapy or radiochemotherapy was characterized by DIS of over 5 mm. No significant difference was seen in the length of DIS between the 2 groups.

[Idiopathic retroperitoneal fibrosis--the Ormond's disease]. / Przypadek idiopatycznego, pozaotrzewnowego zwlóknienia--choroba Ormonda.

Idiopathic retroperitoneal fibrosis, so called "Ormond's disease" belongs to rare inflammatory processes of unknown etiology. As a result of this disease fibrotic masses causing compression of the ureters, vessels and alimentary canal appear in the retroperitoneal space. In this study we describe the case of 59 years old man. Diagnosis was confirmed with computed tomography of abdominal cavity. Patient was treated with corticosteroids, endoxan. Good course of disease was observed.

BRCA1 mutations and colorectal cancer in Poland.

Evidence to date that germline mutations in the tumor suppressor gene BRCA1 increase the incidence of colorectal cancer is mixed, and both positive and negative results have been reported. To establish whether or not inherited variation in BRCA1 influences the risk of colorectal cancer, we genotyped 2,398 unselected patients with colorectal cancer and 4,570 controls from Poland for three BRCA1 founder mutations (C61G, 4153delA and 5382insC). A BRCA1 mutation was present in 0.42% of unselected cases of colorectal cancer and in 0.48% of controls (OR = 0.8; P = 0.8). The mutation frequency was slightly higher (0.93%) in 321 cases who reported a family history of colon cancer in a first- or second-degree relative (OR = 1.9; P = 0.5). A BRCA1 mutation was also seen in excess (0.82%) in 851 cases who were diagnosed with colorectal cancer at age 60 or earlier (OR = 1.7; P = 0.3). The mean age at onset in carriers was 7 years younger than in non-carriers (57.0 years vs. 64.0) and the difference was significant (P = 0.05). This study suggests that BRCA1 mutations may be associated with early-onset of colorectal cancer.